Progressive destruction or the absence of all or part of the extrahepatic BILE DUCTS, resulting in the complete obstruction of BILE flow. Usually, biliary atresia is found in infants and accounts for one third of the neonatal cholestatic JAUNDICE.
Operation for biliary atresia by anastomosis of the bile ducts into the jejunum or duodenum.
The degeneration and resorption of an OVARIAN FOLLICLE before it reaches maturity and ruptures.
Congenital obliteration of the lumen of the intestine, with the ILEUM involved in 50% of the cases and the JEJUNUM and DUODENUM following in frequency. It is the most frequent cause of INTESTINAL OBSTRUCTION in NEWBORNS. (From Stedman, 25th ed)
Congenital abnormality characterized by the lack of full development of the ESOPHAGUS that commonly occurs with TRACHEOESOPHAGEAL FISTULA. Symptoms include excessive SALIVATION; GAGGING; CYANOSIS; and DYSPNEA.
The channels that collect and transport the bile secretion from the BILE CANALICULI, the smallest branch of the BILIARY TRACT in the LIVER, through the bile ductules, the bile ducts out the liver, and to the GALLBLADDER for storage.
Impairment of bile flow due to obstruction in small bile ducts (INTRAHEPATIC CHOLESTASIS) or obstruction in large bile ducts (EXTRAHEPATIC CHOLESTASIS).
Yellow discoloration of the SKIN; MUCOUS MEMBRANE; and SCLERA in the NEWBORN. It is a sign of NEONATAL HYPERBILIRUBINEMIA. Most cases are transient self-limiting (PHYSIOLOGICAL NEONATAL JAUNDICE) occurring in the first week of life, but some can be a sign of pathological disorders, particularly LIVER DISEASES.
A congenital heart defect characterized by the narrowing or complete absence of the opening between the RIGHT VENTRICLE and the PULMONARY ARTERY. Lacking a normal PULMONARY VALVE, unoxygenated blood in the right ventricle can not be effectively pumped into the lung for oxygenation. Clinical features include rapid breathing, CYANOSIS, right ventricle atrophy, and abnormal heart sounds (HEART MURMURS).
Passages external to the liver for the conveyance of bile. These include the COMMON BILE DUCT and the common hepatic duct (HEPATIC DUCT, COMMON).
Inflammation of the biliary ductal system (BILE DUCTS); intrahepatic, extrahepatic, or both.
A clinical manifestation of HYPERBILIRUBINEMIA, characterized by the yellowish staining of the SKIN; MUCOUS MEMBRANE; and SCLERA. Clinical jaundice usually is a sign of LIVER dysfunction.
A congenital abnormality that is characterized by a blocked CHOANAE, the opening between the nose and the NASOPHARYNX. Blockage can be unilateral or bilateral; bony or membranous.
Imino acids are organic compounds containing a nitrogen atom in their structure, classified as derivatives of amino acids, where the carbon atom adjacent to the carboxyl group is bonded to a nitrogen atom instead of a hydrogen atom, forming a characteristic imino functional group.
The BILE DUCTS and the GALLBLADDER.
The transference of a part of or an entire liver from one human or animal to another.
A radiopharmaceutical used extensively in cholescintigraphy for the evaluation of hepatobiliary diseases. (From Int Jrnl Rad Appl Inst 1992;43(9):1061-4)
Impairment of bile flow due to injury to the HEPATOCYTES; BILE CANALICULI; or the intrahepatic bile ducts (BILE DUCTS, INTRAHEPATIC).
Absence of the orifice between the RIGHT ATRIUM and RIGHT VENTRICLE, with the presence of an atrial defect through which all the systemic venous return reaches the left heart. As a result, there is left ventricular hypertrophy (HYPERTROPHY, LEFT VENTRICULAR) because the right ventricle is absent or not functional.
INFLAMMATION of the LIVER.
A congenital anatomic malformation of a bile duct, including cystic dilatation of the extrahepatic bile duct or the large intrahepatic bile duct. Classification is based on the site and type of dilatation. Type I is most common.
An infant during the first month after birth.
Abnormal passage between the ESOPHAGUS and the TRACHEA, acquired or congenital, often associated with ESOPHAGEAL ATRESIA.
A genus of primitive fish in the family Petromyzontidae. The sole species is Petromyzon marinus, known as the sea lamprey. The adult form feeds parasitically on other fish species.
A congenital abnormality in which organs in the THORAX and the ABDOMEN are opposite to their normal positions (situs solitus) due to lateral transposition. Normally the STOMACH and SPLEEN are on the left, LIVER on the right, the three-lobed right lung is on the right, and the two-lobed left lung on the left. Situs inversus has a familial pattern and has been associated with a number of genes related to microtubule-associated proteins.
A large lobed glandular organ in the abdomen of vertebrates that is responsible for detoxification, metabolism, synthesis and storage of various substances.
A bile pigment that is a degradation product of HEME.
Pathological processes of the LIVER.
Infection with any of the rotaviruses. Specific infections include human infantile diarrhea, neonatal calf diarrhea, and epidemic diarrhea of infant mice.
A genus of REOVIRIDAE, causing acute gastroenteritis in BIRDS and MAMMALS, including humans. Transmission is horizontal and by environmental contamination. Seven species (Rotaviruses A thru G) are recognized.
Semisynthetic penicillin-type antibiotic.
Precipitin tests which occur over a narrow range of antigen-antibody ratio, due chiefly to peculiarities of the antibody (precipitin). (From Stedman, 26th ed)
Passages within the liver for the conveyance of bile. Includes right and left hepatic ducts even though these may join outside the liver to form the common hepatic duct.
Predominantly extrahepatic bile duct which is formed by the junction of the right and left hepatic ducts, which are predominantly intrahepatic, and, in turn, joins the cystic duct to form the common bile duct.
Impairment of bile flow in the large BILE DUCTS by mechanical obstruction or stricture due to benign or malignant processes.
Non-cadaveric providers of organs for transplant to related or non-related recipients.
Pathologic processes that affect patients after a surgical procedure. They may or may not be related to the disease for which the surgery was done, and they may or may not be direct results of the surgery.
Diseases of newborn infants present at birth (congenital) or developing within the first month of birth. It does not include hereditary diseases not manifesting at birth or within the first 30 days of life nor does it include inborn errors of metabolism. Both HEREDITARY DISEASES and METABOLISM, INBORN ERRORS are available as general concepts.
A congenital abnormality characterized by the persistence of the anal membrane, resulting in a thin membrane covering the normal ANAL CANAL. Imperforation is not always complete and is treated by surgery in infancy. This defect is often associated with NEURAL TUBE DEFECTS; MENTAL RETARDATION; and DOWN SYNDROME.
A condition characterized by an abnormal increase of BILIRUBIN in the blood, which may result in JAUNDICE. Bilirubin, a breakdown product of HEME, is normally excreted in the BILE or further catabolized before excretion in the urine.

Liver transplantation in patients with situs inversus. (1/274)

Two patients with situs inversus and biliary atresia were treated with hepatic transplantation, one with an auxiliary liver and the other with an orthotopic graft which was placed using a piggy-back technique. Both transplants functioned well initially. The auxiliary liver was rejected after 1 1/2 months, and the patient died after an attempt at retransplantation many months later. The recipient of the orthotopic liver has perfect liver function 10 months postoperatively.  (+info)

Screening of newborn infants for cholestatic hepatobiliary disease with tandem mass spectrometry. (2/274)

OBJECTIVE: To assess the feasibility of screening for cholestatic hepatobiliary disease and extrahepatic biliary atresia by using tandem mass spectrometry to measure conjugated bile acids in dried blood spots obtained from newborn infants at 7-10 days of age for the Guthrie test. SETTING: Three tertiary referral clinics and regional neonatal screening laboratories. DESIGN: Unused blood spots from the Guthrie test were retrieved for infants presenting with cholestatic hepatobiliary disease and from the two cards stored on either side of each card from an index child. Concentrations of conjugated bile acids measured by tandem mass spectrometry in the two groups were compared. MAIN OUTCOME MEASURES: Concentrations of glycodihydroxycholanoates, glycotrihydroxycholanoates, taurodihydroxycholanoates, and taurotrihydroxycholanoates. Receiver operator curves were plotted to determine which parameter (or combination of parameters) would best predict the cases of cholestatic hepatobiliary disease and extrahepatic biliary atresia. The sensitivity and specificity at a selection of cut off values for each bile acid species and for total bile acid concentrations for the detection of the two conditions were calculated. RESULTS: 218 children with cholestatic hepatobiliary disease were eligible for inclusion in the study. Two children without a final diagnosis and five who presented at <14 days of age were excluded. Usable blood spots were obtained from 177 index children and 708 comparison children. Mean concentrations of all four bile acid species were significantly raised in children with cholestatic hepatobiliary disease and extrahepatic biliary atresia compared with the unaffected children (P<0.0001). Of 177 children with cholestatic hepatobiliary disease, 104 (59%) had a total bile acid concentration >33 micromol/l (97.5th centile value for comparison group). Of the 61 with extrahepatic biliary atresia, 47 (77%) had total bile acid concentrations >33 micromol/l. Taurotrihydroxycholanoate and total bile acid concentrations were the best predictors of both conditions. For all cholestatic hepatobiliary disease, a cut off level of total bile acid concentration of 30 micromol/l gave a sensitivity of 62% and a specificity of 96%, while the corresponding values for extrahepatic biliary atresia were 79% and 96%. CONCLUSION: Most children who present with extrahepatic biliary atresia and other forms of cholestatic hepatobiliary disease have significantly raised concentrations of conjugated bile acids as measured by tandem mass spectrometry at the time when samples are taken for the Guthrie test. Unfortunately the separation between the concentrations in these infants and those in the general population is not sufficient to make mass screening for cholestatic hepatobiliary disease a feasible option with this method alone.  (+info)

Partial left lateral segment transplant from a living donor. (3/274)

A shortage of liver donors for low-weight transplant recipients has prompted the development of procedures for liver-reduction, split-liver, and living related donor transplantations. For pediatric recipients weighing less than 10 kg, the left lateral segment is often still too large. We describe the procedure of monosegmental transplantation using segment II after segment III was resected in situ from a living related donor. Successful monosegmental transplantation is technically feasible and is a valid alternative to be considered for cases of size discrepancy between the recipient's volume and the donor's left lateral segment.  (+info)

Extrahepatic metabolism of sevoflurane in children undergoing orthotopic liver transplantation. (4/274)

BACKGROUND: Sevoflurane is metabolized by cytochrome P450 and produces inorganic fluoride. The anhepatic phase of liver transplantation provides a useful tool to study the extrahepatic metabolism of drugs. The authors therefore studied the extrahepatic metabolism of sevoflurane by measuring the fluoride production in children receiving sevoflurane solely during the anhepatic phase of orthotopic liver transplantation. METHODS: Children with end-stage liver disease undergoing orthotopic liver transplantation were studied. Anesthesia was provided with isoflurane, sufentanil, and pancuronium. In one group, isoflurane was replaced by sevoflurane as soon as the liver was removed from the patient and maintained until reperfusion of the new liver. Arterial blood samples were drawn at induction, before removal of the liver, 15 min and 30 min after the beginning of the anhepatic phase, at the unclamping of the new liver, and finally 60 and 120 min after the unclamping. Plasma fluoride concentrations were determined by ion-selective electrode. RESULTS: No differences between the two groups (n = 10) regarding age, weight, duration of the anhepatic phase, or basal level of inorganic fluoride were found. The fluoride concentration increased significantly as soon as sevoflurane was introduced; it remained stable in the group receiving isoflurane. The peak fluoride concentration was also significantly higher in the first group (mean +/- SD: 5.5 +/- 0.8 microM (sevoflurane group) versus 1.4 +/- 0.5 microM (isoflurane group) P < 0.05). CONCLUSIONS: These results demonstrate the existence of an extrahepatic metabolism of sevoflurane at least in children with end-stage liver disease.  (+info)

Health-related quality of life in long-term survivors of pediatric liver transplantation. (5/274)

The purpose of this study is to measure the health-related quality of life (HRQOL) in children who are long-term survivors of liver transplantation and to pilot the Liver Transplant Disability Scale (LTDS), a newly developed 12-point scale that quantifies chronic medical disability related to liver transplantation. This study is a cross-sectional survey of 51 children surviving liver transplantation by at least 2 years, with a median age of 4.94 years. Functional capacity and utility scores were measured by the Health Utilities Index Mark II (HUI2), and chronic disease-specific medical disability was measured by the LTDS. HUI2 results were compared with a reference population. LTDS scores were compared with utility scores and patient survival 3 years later. Ninety percent of the study patients had functional deficits compared with 50% of controls. Functional impairment was typically mild. The resulting mean utility score, 0.86 +/- 0.13 (0 = dead, 1 = perfect health), was significantly less than that of the reference population, 0.95 +/- 0. 07 (P <.001). LTDS scores ranged from 0 (no disability) to 6 (moderate disability). Seventy-one percent of the children had mild disability (scores 0 to 3), and 29% had moderate disability (scores 4 to 6). LTDS scores did not correlate with utility scores but were predictive of survival. The majority of pediatric liver transplant recipients have mild functional deficits. Their utility scores reflected a high level of HRQOL but were significantly less than those of a reference population. The majority also had mild medical disability, predominantly delayed growth. Medical disability did not correlate with HRQOL but predicted survival 3 years later.  (+info)

The significance of functioning gallbladder visualization on hepatobiliary scintigraphy in infants with persistent jaundice. (6/274)

The purpose of this study was to determine whether gallbladder visualization can help exclude biliary atresia in hepatobiliary scintigraphic studies of infants with persistent jaundice. METHODS: One hundred fifty-two infants with persistent jaundice (49 patients with a final diagnosis of biliary atresia and 103 with biliary patency) were studied using both hepatobiliary scintigraphy and abdominal sonography. Food was withheld for 4 h before the examination, and the infants were fed nothing but glucose until 6 h after the initial injection of (99m)Tc-disofenin or until the gallbladder was seen. If the gallbladder was seen, the infants were fed milk, and imaging was continued to observe gallbladder contractility. RESULTS: In none of the 49 patients with biliary atresia could the gallbladder be seen with hepatobiliary scintigraphy, but abdominal sonography revealed 9 normal-sized gallbladders. Of the 103 patients with biliary patency, hepatobiliary scintigraphy detected the gallbladder more frequently (74%, 76/103) than did abdominal sonography (63%, 65/103). All visualized gallbladders contracted after the infants were fed milk. If we include visualization of both the gallbladder and bowel radioactivity as criteria, the specificity of biliary atresia on hepatobiliary scintigraphy increases to 86% (89/103). CONCLUSION: Gallbladders were usually visible on hepatobiliary scintigraphy of fasting patients with biliary patency. A functioning gallbladder, with or without visualization of bowel radioactivity, indicated biliary patency.  (+info)

Characteristics of Malaysian infants with biliary atresia and neonatal hepatitis. (7/274)

Cholestatic disorders of infancy (viz neonatal hepatitis and biliary atresia) have not been well studied in Malaysia. In a retrospective study in the Department of Paediatrics, University Hospital, Kuala Lumpur from January 1982 through December 1991, a total of ninety-three infants with such conditions were identified: 35 (38%) had biliary atresia, 58 (62%) neonatal hepatitis. There was a statistically significant male preponderance in the neonatal hepatitis group (P = 0.020). There was no significant difference in the racial distribution and in the proportions of low birthweight infants between the two groups of disorders. When the biliary atresia group was compared with the neonatal hepatitis group, significant differences were observed in the age of presentation (mean +/- SD) 9.8 +/- 6.8 VS 20 +/- 17.3 weeks (P < 0.001), proportion of infants with prolonged jaundice (> seven weeks) 28/35 (80%) VS 20/58 (34.5%) (P < 0.00001), occurrence of alcoholic stools 26/35 (74.3%) VS 27/58 (46.6%) (P = 0.020), liver size (mean +/- SD): 4.3 (1.6 cm VS 3.3 +/- 1.8 cm (P < 0.01) and splenic size: 2.5 (1.8 cm VS 1.4 (1.2 cm (P < 0.001). There was however considerable overlap between the two groups in these features at presentation, making clinical differentiation between the two conditions difficult. Infants with cholestasis tended to present late, compromising the chance of survival. In order to improve the medical care of these patients, these conditions must be emphasised during the training of medical practitioners, and efforts to increase public awareness of these conditions must be created.  (+info)

Paediatric liver transplantation: Queen Mary Hospital experience. (8/274)

OBJECTIVE: To assess the results of paediatric liver transplantation in our institution. METHODS: From September 1993 to November 1996, 10 living-related liver transplants (LRLT) and 3 reduced-size liver transplants (RSLT) were performed on 12 children at our hospital. The medical records of the patients were reviewed. All patients suffered from end-stage liver disease resulting from biliary atresia with failed Kasai's operations. Their ages at initial transplantation ranged from 8 months to 11 years. Excluding the 2 older children aged 7.5 and 11 years, the remaining patients were aged 10.5 months on the average and weighed 6 to 9.5 kg (mean: 6.8 kg) at the time of initial transplantation. RESULTS: All living donors were discharged on postoperative day 4 to 8 and resumed their previous normal activities. All recipients were alive with normal liver function and growing after a follow-up period of 3-40 months (mean: 21 months). The patient survival rate was 100%. One patient with RSLT had hepatitis of undetermined aetiology and underwent retransplant with a graft from her mother. The graft survival rate was 92%. Postoperative complications included: postoperative bleeding (n = 3), hepatic vein stenosis (n = I), biliary-enteric anastomotic stenosis (n = 3), intestinal perforation (n = I) and portal vein thrombosis (n = I). They were all treated promptly. In all patients, the hepatic artery (diameter ranged from 1.5 to 2.5 mm) anastomosis was achieved by microvascular technique. There was no hepatic artery thrombosis in our patients. CONCLUSION: With technical refinements, early detection and prompt treatment of complications, and advances in immunotherapy, excellent results can be achieved in paediatric liver transplantation.  (+info)

Biliary atresia is a rare, progressive liver disease in infants and children, characterized by the inflammation, fibrosis, and obstruction of the bile ducts. This results in the impaired flow of bile from the liver to the intestine, leading to cholestasis (accumulation of bile in the liver), jaundice (yellowing of the skin and eyes), and eventually liver cirrhosis and failure if left untreated.

The exact cause of biliary atresia is not known, but it is believed to be a combination of genetic and environmental factors. It can occur as an isolated condition or in association with other congenital anomalies. The diagnosis of biliary atresia is typically made through imaging studies, such as ultrasound and cholangiography, and confirmed by liver biopsy.

The standard treatment for biliary atresia is a surgical procedure called the Kasai portoenterostomy, which aims to restore bile flow from the liver to the intestine. In this procedure, the damaged bile ducts are removed and replaced with a loop of intestine that is connected directly to the liver. The success of the Kasai procedure depends on several factors, including the age at diagnosis and surgery, the extent of liver damage, and the skill and experience of the surgeon.

Despite successful Kasai surgery, many children with biliary atresia will eventually develop cirrhosis and require liver transplantation. The prognosis for children with biliary atresia has improved significantly over the past few decades due to earlier diagnosis, advances in surgical techniques, and better postoperative care. However, it remains a challenging condition that requires close monitoring and multidisciplinary management by pediatric hepatologists, surgeons, and other healthcare professionals.

A hepatic portoenterostomy, also known as Kasai procedure, is a surgical operation performed on infants with extrahepatic biliary atresia. This condition is characterized by the absence or abnormal formation of the bile ducts that carry bile from the liver to the small intestine, leading to obstruction and damage to the liver.

During a hepatic portoenterostomy, the surgeon creates an anastomosis (connection) between the portal vein, which brings blood to the liver, and a loop of intestine. This connection allows bile to flow directly from the liver into the intestine, bypassing the blocked or absent bile ducts. The goal of the procedure is to restore bile flow and prevent further damage to the liver.

The success of the procedure varies, but it can help improve the child's quality of life and delay or prevent the need for a liver transplant in some cases. However, many children with biliary atresia will eventually require a liver transplant as the disease progresses.

Follicular atresia is a physiological process that occurs in the ovary, where follicles (fluid-filled sacs containing immature eggs or oocytes) undergo degeneration and disappearance. This process begins after the primordial follicle stage and continues throughout a woman's reproductive years. At birth, a female has approximately 1 to 2 million primordial follicles, but only about 400 of these will mature and release an egg during her lifetime. The rest undergo atresia, which is a natural process that helps regulate the number of available eggs and maintain hormonal balance within the body.

The exact mechanisms that trigger follicular atresia are not fully understood, but it is believed to be influenced by various factors such as hormonal imbalances, oxidative stress, and apoptosis (programmed cell death). In some cases, accelerated or excessive follicular atresia can lead to infertility or early menopause.

Intestinal atresia is a congenital condition characterized by the absence or complete closure of a portion of the intestine, preventing the passage of digested food from the stomach to the remaining part of the intestines. This results in a blockage in the digestive system, which can be life-threatening if not treated promptly after birth. The condition can occur anywhere along the small or large intestine and may affect either a single segment or multiple segments of the intestine.

There are several types of intestinal atresia, including:

1. Jejunal atresia: A closure or absence in the jejunum, a part of the small intestine located between the duodenum and ileum.
2. Ileal atresia: A closure or absence in the ileum, the lower portion of the small intestine that connects to the large intestine (cecum).
3. Colonic atresia: A closure or absence in the colon, a part of the large intestine responsible for storing and eliminating waste.
4. Duodenal atresia: A closure or absence in the duodenum, the uppermost portion of the small intestine that receives chyme (partially digested food) from the stomach.
5. Multiple atresias: When more than one segment of the intestines is affected by atresia.

The exact cause of intestinal atresia remains unclear, but it is believed to be related to disruptions in fetal development during pregnancy. Treatment typically involves surgical correction to reconnect the affected segments of the intestine and restore normal digestive function. The prognosis for infants with intestinal atresia depends on the severity and location of the atresia, as well as any associated conditions or complications.

Esophageal atresia is a congenital condition in which the esophagus, the tube that connects the throat to the stomach, does not develop properly. In most cases, the upper esophagus ends in a pouch instead of connecting to the lower esophagus and stomach. This condition prevents food and liquids from reaching the stomach, leading to difficulty swallowing and feeding problems in newborn infants. Esophageal atresia often occurs together with a congenital defect called tracheoesophageal fistula, in which there is an abnormal connection between the esophagus and the windpipe (trachea).

The medical definition of 'Esophageal Atresia' is:

A congenital anomaly characterized by the absence of a normal connection between the upper esophagus and the stomach, resulting in the separation of the proximal and distal esophageal segments. The proximal segment usually ends in a blind pouch, while the distal segment may communicate with the trachea through a tracheoesophageal fistula. Esophageal atresia is often associated with other congenital anomalies and can cause serious complications if not diagnosed and treated promptly after birth.

Bile ducts are tubular structures that carry bile from the liver to the gallbladder for storage or directly to the small intestine to aid in digestion. There are two types of bile ducts: intrahepatic and extrahepatic. Intrahepatic bile ducts are located within the liver and drain bile from liver cells, while extrahepatic bile ducts are outside the liver and include the common hepatic duct, cystic duct, and common bile duct. These ducts can become obstructed or inflamed, leading to various medical conditions such as cholestasis, cholecystitis, and gallstones.

Cholestasis is a medical condition characterized by the interruption or reduction of bile flow from the liver to the small intestine. Bile is a digestive fluid produced by the liver that helps in the breakdown and absorption of fats. When the flow of bile is blocked or reduced, it can lead to an accumulation of bile components, such as bilirubin, in the blood, which can cause jaundice, itching, and other symptoms.

Cholestasis can be caused by various factors, including liver diseases (such as hepatitis, cirrhosis, or cancer), gallstones, alcohol abuse, certain medications, pregnancy, and genetic disorders. Depending on the underlying cause, cholestasis may be acute or chronic, and it can range from mild to severe in its symptoms and consequences. Treatment for cholestasis typically involves addressing the underlying cause and managing the symptoms with supportive care.

Neonatal jaundice is a medical condition characterized by the yellowing of a newborn baby's skin and eyes due to an excess of bilirubin in the blood. Bilirubin is a yellowish substance produced by the normal breakdown of red blood cells, which are then processed by the liver and excreted through the bile. In neonatal jaundice, the liver is not yet fully developed and cannot process bilirubin quickly enough, leading to its accumulation in the body.

Neonatal jaundice typically appears within the first 2-4 days of life and can range from mild to severe. Mild cases may resolve on their own without treatment, while more severe cases may require medical intervention such as phototherapy or a blood transfusion. Risk factors for neonatal jaundice include prematurity, bruising during birth, blood type incompatibility between mother and baby, and certain genetic disorders.

It is important to monitor newborns closely for signs of jaundice and seek medical attention if concerned, as untreated neonatal jaundice can lead to serious complications such as brain damage or hearing loss.

Pulmonary atresia is a congenital heart defect where the pulmonary valve, which controls blood flow from the right ventricle to the lungs, doesn't form properly and instead of being open, there is a membranous obstruction or atresia. This results in an absence of communication between the right ventricle and the pulmonary artery.

The right ventricle is often small and underdeveloped due to this condition, and blood flow to the lungs can be severely limited. In some cases, there may be additional heart defects present, such as a ventricular septal defect (a hole between the two lower chambers of the heart) or patent ductus arteriosus (an abnormal connection between the pulmonary artery and the aorta).

Pulmonary atresia can range from mild to severe, and treatment options depend on the specific anatomy and physiology of each individual case. Treatment may include medications, catheter-based procedures, or open-heart surgery, and in some cases, a heart transplant may be necessary.

Extrahepatic bile ducts refer to the portion of the biliary system that lies outside the liver. The biliary system is responsible for producing, storing, and transporting bile, a digestive fluid produced by the liver.

The extrahepatic bile ducts include:

1. The common hepatic duct: This duct is formed by the union of the right and left hepatic ducts, which drain bile from the corresponding lobes of the liver.
2. The cystic duct: This short duct connects the gallbladder to the common hepatic duct, allowing bile to flow into the gallbladder for storage and concentration.
3. The common bile duct: This is the result of the fusion of the common hepatic duct and the cystic duct. It transports bile from the liver and gallbladder to the duodenum, the first part of the small intestine, where it aids in fat digestion.
4. The ampulla of Vater (or hepatopancreatic ampulla): This is a dilated area where the common bile duct and the pancreatic duct join and empty their contents into the duodenum through a shared opening called the major duodenal papilla.

Extrahepatic bile ducts can be affected by various conditions, such as gallstones, inflammation (cholangitis), strictures, or tumors, which may require medical or surgical intervention.

Cholangitis is a medical condition characterized by inflammation of the bile ducts, which are the tubes that carry bile from the liver to the small intestine. Bile is a digestive juice produced by the liver that helps break down fats in food.

There are two types of cholangitis: acute and chronic. Acute cholangitis is a sudden and severe infection that can cause symptoms such as abdominal pain, fever, jaundice (yellowing of the skin and eyes), and dark urine. It is usually caused by a bacterial infection that enters the bile ducts through a blockage or obstruction.

Chronic cholangitis, on the other hand, is a long-term inflammation of the bile ducts that can lead to scarring and narrowing of the ducts. This can cause symptoms such as abdominal pain, itching, and jaundice. Chronic cholangitis can be caused by various factors, including primary sclerosing cholangitis (an autoimmune disease), bile duct stones, or tumors in the bile ducts.

Treatment for cholangitis depends on the underlying cause of the condition. Antibiotics may be used to treat bacterial infections, and surgery may be necessary to remove blockages or obstructions in the bile ducts. In some cases, medications may be prescribed to manage symptoms and prevent further complications.

Jaundice is a medical condition characterized by the yellowing of the skin, sclera (whites of the eyes), and mucous membranes due to an excess of bilirubin in the bloodstream. Bilirubin is a yellow-orange pigment produced when hemoglobin from red blood cells is broken down. Normally, bilirubin is processed by the liver and excreted through bile into the digestive system. However, if there's an issue with bilirubin metabolism or elimination, it can accumulate in the body, leading to jaundice.

Jaundice can be a symptom of various underlying conditions, such as liver diseases (hepatitis, cirrhosis), gallbladder issues (gallstones, tumors), or blood disorders (hemolysis). It is essential to consult a healthcare professional if jaundice is observed, as it may indicate a severe health problem requiring prompt medical attention.

Choanal atresia is a medical condition where the back of the nasal passage (choana) is blocked or narrowed, usually by bone, membrane, or a combination of both. This blockage can be present at birth (congenital) or acquired later in life due to various reasons such as infection, injury, or tumor.

Congenital choanal atresia is more common and occurs during fetal development when the nasal passages fail to open properly. It can affect one or both sides of the nasal passage and can be unilateral (affecting one side) or bilateral (affecting both sides). Bilateral choanal atresia can cause breathing difficulties in newborns, as they are obligate nose breathers and cannot breathe through their mouth yet.

Treatment for choanal atresia typically involves surgical intervention to open up the nasal passage and restore normal breathing. The specific type of surgery may depend on the location and extent of the blockage. In some cases, follow-up surgeries or additional treatments may be necessary to ensure proper functioning of the nasal passage.

Imino acids are organic compounds that contain a nitrogen atom as part of an amide-like structure. They are structurally similar to amino acids, which contain a carboxyl group and an amino group, but instead of the amino group, imino acids have a structural unit known as an imine or Schiff base, which is a carbon-nitrogen double bond with a hydrogen atom attached to the nitrogen atom.

One example of an imino acid is proline, which is a cyclic imino acid that plays important roles in protein structure and function. Proline is unique among the 20 standard amino acids because its side chain is linked to the nitrogen atom of the backbone, forming a ring-like structure. This structural feature gives proline unique properties, such as restricted rotation around the bond between the nitrogen and alpha carbon atoms, which can affect protein folding and stability.

Other imino acids may be formed through chemical reactions or enzymatic processes, and they can play important roles in various biological pathways, including the biosynthesis of amino acids, nucleotides, and other biomolecules. However, imino acids are not typically considered to be part of the standard set of 20 amino acids that make up proteins.

The biliary tract is a system of ducts that transport bile from the liver to the gallbladder and then to the small intestine. Bile is a digestive fluid produced by the liver that helps in the breakdown and absorption of fats in the small intestine. The main components of the biliary tract are:

1. Intrahepatic bile ducts: These are the smaller branches of bile ducts located within the liver that collect bile from the liver cells or hepatocytes.
2. Gallbladder: A small pear-shaped organ located beneath the liver, which stores and concentrates bile received from the intrahepatic bile ducts. The gallbladder releases bile into the small intestine when food is ingested, particularly fats, to aid digestion.
3. Common hepatic duct: This is a duct that forms by the union of the right and left hepatic ducts, which carry bile from the right and left lobes of the liver, respectively.
4. Cystic duct: A short duct that connects the gallbladder to the common hepatic duct, forming the beginning of the common bile duct.
5. Common bile duct: This is a larger duct formed by the union of the common hepatic duct and the cystic duct. It carries bile from the liver and gallbladder into the small intestine.
6. Pancreatic duct: A separate duct that originates from the pancreas, a gland located near the liver and stomach. The pancreatic duct joins the common bile duct just before they both enter the duodenum, the first part of the small intestine.
7. Ampulla of Vater: This is the dilated portion where the common bile duct and the pancreatic duct join together and empty their contents into the duodenum through a shared opening called the papilla of Vater.

Disorders related to the biliary tract include gallstones, cholecystitis (inflammation of the gallbladder), bile duct stones, bile duct strictures or obstructions, and primary sclerosing cholangitis, among others.

Liver transplantation is a surgical procedure in which a diseased or failing liver is replaced with a healthy one from a deceased donor or, less commonly, a portion of a liver from a living donor. The goal of the procedure is to restore normal liver function and improve the patient's overall health and quality of life.

Liver transplantation may be recommended for individuals with end-stage liver disease, acute liver failure, certain genetic liver disorders, or liver cancers that cannot be treated effectively with other therapies. The procedure involves complex surgery to remove the diseased liver and implant the new one, followed by a period of recovery and close medical monitoring to ensure proper function and minimize the risk of complications.

The success of liver transplantation has improved significantly in recent years due to advances in surgical techniques, immunosuppressive medications, and post-transplant care. However, it remains a major operation with significant risks and challenges, including the need for lifelong immunosuppression to prevent rejection of the new liver, as well as potential complications such as infection, bleeding, and organ failure.

Technetium Tc 99m Disofenin is not a medical condition, but rather a radiopharmaceutical used in diagnostic imaging. It is a radioactive tracer used in nuclear medicine scans, specifically for liver and biliary system imaging. The compound consists of the radioisotope Technetium-99m (Tc-99m) bonded to the pharmaceutical Disofenin.

The Tc-99m is a gamma emitter with a half-life of 6 hours, making it ideal for diagnostic imaging. When administered to the patient, the compound is taken up by the liver and excreted into the bile ducts and gallbladder, allowing medical professionals to visualize these structures using a gamma camera. This can help detect various conditions such as tumors, gallstones, or obstructions in the biliary system.

It's important to note that Technetium Tc 99m Disofenin is used diagnostically and not for therapeutic purposes. The radiation exposure from this compound is generally low and considered safe for diagnostic use. However, as with any medical procedure involving radiation, the benefits and risks should be carefully weighed and discussed with a healthcare professional.

Intrahepatic cholestasis is a medical condition characterized by the interruption or reduction of bile flow within the liver. Bile is a digestive fluid produced by the liver that helps in the absorption of fats and fat-soluble vitamins. Intrahepatic cholestasis occurs when there is a problem with the transport of bile components inside the liver cells (hepatocytes). This can lead to an accumulation of bile acids, bilirubin, and other substances in the liver, which can cause damage to liver cells and result in symptoms such as jaundice, itching, and dark urine.

Intrahepatic cholestasis can be caused by various factors, including medications, alcohol abuse, hepatitis viruses, autoimmune disorders, genetic defects, and cancer. Depending on the underlying cause, intrahepatic cholestasis can be acute or chronic, and it can range from mild to severe. Treatment typically involves addressing the underlying cause of the condition, as well as providing supportive care to manage symptoms and prevent complications.

Tricuspid atresia is a congenital heart defect where the tricuspid valve, which regulates blood flow between the right atrium and right ventricle, fails to develop properly. As a result, there is no direct pathway for blood to move from the right atrium to the right ventricle and then to the lungs for oxygenation.

In this condition, blood from the body returning to the heart enters the right atrium but cannot flow through the tricuspid valve into the right ventricle. Instead, it flows through an opening in the interatrial septum (atrial septal defect) into the left atrium and then into the left ventricle. The left ventricle pumps this blood to the body and a portion of it goes to the lungs via a patent ductus arteriosus or other collateral vessels.

Tricuspid atresia is often associated with other heart defects, such as transposition of the great arteries, pulmonary stenosis, or total anomalous pulmonary venous return. Symptoms can vary depending on the severity and associated defects but may include cyanosis (bluish discoloration of the skin), shortness of breath, fatigue, and poor growth. Treatment typically involves surgical interventions to create a path for blood to flow to the lungs and establish proper oxygenation.

Hepatitis is a medical condition characterized by inflammation of the liver, often resulting in damage to liver cells. It can be caused by various factors, including viral infections (such as Hepatitis A, B, C, D, and E), alcohol abuse, toxins, medications, and autoimmune disorders. Symptoms may include jaundice, fatigue, abdominal pain, loss of appetite, nausea, vomiting, and dark urine. The severity of the disease can range from mild illness to severe, life-threatening conditions, such as liver failure or cirrhosis.

A Choledochal cyst is a congenital dilatation or abnormal enlargement of the bile ducts, which are the tubes that carry bile from the liver to the small intestine. Bile is a digestive juice produced by the liver that helps in the digestion of fats.

Choledochal cysts can be classified into several types based on their location and the anatomy of the biliary tree. The most common type, called Type I, involves dilatation of the common bile duct. Other types include dilatation of the intrahepatic bile ducts (Type II), dilatation of both the intrahepatic and extrahepatic bile ducts (Type III), and multiple cystic dilatations of the bile ducts (Type IV).

Choledochal cysts are more common in females than males, and they can present at any age. Symptoms may include abdominal pain, jaundice, vomiting, and fever. Complications of choledochal cysts can include bile duct stones, infection, and cancer. Treatment typically involves surgical removal of the cyst, followed by reconstruction of the biliary tree.

A newborn infant is a baby who is within the first 28 days of life. This period is also referred to as the neonatal period. Newborns require specialized care and attention due to their immature bodily systems and increased vulnerability to various health issues. They are closely monitored for signs of well-being, growth, and development during this critical time.

A tracheoesophageal fistula (TEF) is an abnormal connection between the trachea (windpipe) and the esophagus (tube that carries food from the mouth to the stomach). This congenital anomaly is usually present at birth and can vary in size and location. It can cause complications such as respiratory distress, feeding difficulties, and recurrent lung infections. TEF is often treated surgically to separate the trachea and esophagus and restore their normal functions.

"Petromyzon" is the name of a genus that includes the sea lampreys, an ancient type of jawless fish. They are eel-like aquatic creatures with circular mouths filled with teeth, which they use to attach themselves to other fish and feed on their blood. They have a long, slender body, and can grow up to 20-100 cm in length depending on the species. Sea lampreys are considered parasites and can cause significant damage to commercial fisheries.

Situs Inversus is a congenital condition in which the major visceral organs are situated in mirror-image positions to their normal locations. Instead of being on the left side, the heart and its large blood vessels are on the right side, while the liver is on the left side and the lungs are reversed. The stomach, spleen, and pancreas may also be affected. It's important to note that this condition is generally asymptomatic and often goes unnoticed unless there are complications or associated abnormalities.

There are two types of Situs Inversus: total (complete reversal of all organs) and partial (reversal of only some organs). Total Situs Inversus is also sometimes referred to as "mirror-image dextrocardia" because the heart, which is usually on the left side, is located on the right side in a mirrored position.

While Situs Inversus itself does not typically cause health problems, people with this condition may have an increased risk for certain medical conditions, such as congenital heart defects or primary ciliary dyskinesia (PCD), which can lead to chronic respiratory infections and infertility.

The liver is a large, solid organ located in the upper right portion of the abdomen, beneath the diaphragm and above the stomach. It plays a vital role in several bodily functions, including:

1. Metabolism: The liver helps to metabolize carbohydrates, fats, and proteins from the food we eat into energy and nutrients that our bodies can use.
2. Detoxification: The liver detoxifies harmful substances in the body by breaking them down into less toxic forms or excreting them through bile.
3. Synthesis: The liver synthesizes important proteins, such as albumin and clotting factors, that are necessary for proper bodily function.
4. Storage: The liver stores glucose, vitamins, and minerals that can be released when the body needs them.
5. Bile production: The liver produces bile, a digestive juice that helps to break down fats in the small intestine.
6. Immune function: The liver plays a role in the immune system by filtering out bacteria and other harmful substances from the blood.

Overall, the liver is an essential organ that plays a critical role in maintaining overall health and well-being.

Bilirubin is a yellowish pigment that is produced by the liver when it breaks down old red blood cells. It is a normal byproduct of hemoglobin metabolism and is usually conjugated (made water-soluble) in the liver before being excreted through the bile into the digestive system. Elevated levels of bilirubin can cause jaundice, a yellowing of the skin and eyes. Increased bilirubin levels may indicate liver disease or other medical conditions such as gallstones or hemolysis. It is also measured to assess liver function and to help diagnose various liver disorders.

Liver diseases refer to a wide range of conditions that affect the normal functioning of the liver. The liver is a vital organ responsible for various critical functions such as detoxification, protein synthesis, and production of biochemicals necessary for digestion.

Liver diseases can be categorized into acute and chronic forms. Acute liver disease comes on rapidly and can be caused by factors like viral infections (hepatitis A, B, C, D, E), drug-induced liver injury, or exposure to toxic substances. Chronic liver disease develops slowly over time, often due to long-term exposure to harmful agents or inherent disorders of the liver.

Common examples of liver diseases include hepatitis, cirrhosis (scarring of the liver tissue), fatty liver disease, alcoholic liver disease, autoimmune liver diseases, genetic/hereditary liver disorders (like Wilson's disease and hemochromatosis), and liver cancers. Symptoms may vary widely depending on the type and stage of the disease but could include jaundice, abdominal pain, fatigue, loss of appetite, nausea, and weight loss.

Early diagnosis and treatment are essential to prevent progression and potential complications associated with liver diseases.

According to the World Health Organization (WHO), Rotavirus is the most common cause of severe diarrhea among children under 5 years of age. It is responsible for around 215,000 deaths among children in this age group each year.

Rotavirus infection causes inflammation of the stomach and intestines, resulting in symptoms such as vomiting, watery diarrhea, and fever. The virus is transmitted through the fecal-oral route, often through contaminated hands, food, or water. It can also be spread through respiratory droplets when an infected person coughs or sneezes.

Rotavirus infections are highly contagious and can spread rapidly in communities, particularly in settings where children are in close contact with each other, such as child care centers and schools. The infection is usually self-limiting and resolves within a few days, but severe cases can lead to dehydration and require hospitalization.

Prevention measures include good hygiene practices, such as handwashing with soap and water, safe disposal of feces, and rotavirus vaccination. The WHO recommends the inclusion of rotavirus vaccines in national immunization programs to reduce the burden of severe diarrhea caused by rotavirus infection.

Rotavirus is a genus of double-stranded RNA virus in the Reoviridae family, which is a leading cause of severe diarrhea and gastroenteritis in young children and infants worldwide. The virus infects and damages the cells lining the small intestine, resulting in symptoms such as vomiting, watery diarrhea, abdominal cramps, and fever.

Rotavirus is highly contagious and can be spread through contact with infected individuals or contaminated surfaces, food, or water. The virus is typically transmitted via the fecal-oral route, meaning that it enters the body through the mouth after coming into contact with contaminated hands, objects, or food.

Rotavirus infections are often self-limiting and resolve within a few days to a week, but severe cases can lead to dehydration, hospitalization, and even death, particularly in developing countries where access to medical care and rehydration therapy may be limited. Fortunately, there are effective vaccines available that can prevent rotavirus infection and reduce the severity of symptoms in those who do become infected.

I'm sorry for any confusion, but "Sulbenicillin" is not a recognized or established term in medical science or pharmacology. It seems that there might be a spelling mistake or a mix-up with the names of antibiotics. If you meant "Subenicillin," it also doesn't exist in medical literature as a known drug.

If you have any other questions about medical definitions, please provide the correct term, and I will be happy to help.

Flocculation tests are diagnostic procedures used in medical laboratories to detect and measure the presence of certain substances, such as proteins or bacteria, in a sample. These tests work by adding a reagent to the sample that causes any targeted substances to clump together (flocculate) and become visible or easily measurable.

For example, in a coagulation or flocculation test for proteinuria (protein in urine), a reagent such as sulfosalicylic acid is added to a urine sample. If proteins are present in the sample, they will react with the reagent and form a white precipitate that can be seen with the naked eye or measured with a spectrophotometer.

Flocculation tests are commonly used in clinical chemistry and microbiology to diagnose various medical conditions, monitor treatment progress, and assess overall health status.

Intrahepatic bile ducts are the small tubular structures inside the liver that collect bile from the liver cells (hepatocytes). Bile is a digestive fluid produced by the liver that helps in the absorption of fats and fat-soluble vitamins from food. The intrahepatic bile ducts merge to form larger ducts, which eventually exit the liver and join with the cystic duct from the gallbladder to form the common bile duct. The common bile duct then empties into the duodenum, the first part of the small intestine, where bile aids in digestion. Intrahepatic bile ducts can become obstructed or damaged due to various conditions such as gallstones, tumors, or inflammation, leading to complications like jaundice, liver damage, and infection.

The common hepatic duct is a medical term that refers to the duct in the liver responsible for carrying bile from the liver. More specifically, it is the duct that results from the convergence of the right and left hepatic ducts, which themselves carry bile from the right and left lobes of the liver, respectively. The common hepatic duct then joins with the cystic duct from the gallbladder to form the common bile duct, which ultimately drains into the duodenum, a part of the small intestine.

The primary function of the common hepatic duct is to transport bile, a digestive juice produced by the liver, to the small intestine. Bile helps break down fats during the digestion process, making it possible for the body to absorb them properly. Any issues or abnormalities in the common hepatic duct can lead to problems with bile flow and potentially cause health complications such as jaundice, gallstones, or liver damage.

Extrahepatic cholestasis is a medical condition characterized by the impaired flow of bile outside of the liver. Bile is a digestive fluid produced by the liver that helps in the absorption and digestion of fats. When the flow of bile is obstructed or blocked, it can lead to an accumulation of bile components, such as bilirubin, in the bloodstream, resulting in jaundice, dark urine, light-colored stools, and itching.

Extrahepatic cholestasis can be caused by various factors, including gallstones, tumors, strictures, or inflammation of the bile ducts. It is essential to diagnose and treat extrahepatic cholestasis promptly to prevent further complications, such as liver damage or infection. Treatment options may include medications, endoscopic procedures, or surgery, depending on the underlying cause of the condition.

A living donor is a person who voluntarily donates an organ or part of an organ to another person while they are still alive. This can include donations such as a kidney, liver lobe, lung, or portion of the pancreas or intestines. The donor and recipient typically undergo medical evaluation and compatibility testing to ensure the best possible outcome for the transplantation procedure. Living donation is regulated by laws and ethical guidelines to ensure that donors are fully informed and making a voluntary decision.

Postoperative complications refer to any unfavorable condition or event that occurs during the recovery period after a surgical procedure. These complications can vary in severity and may include, but are not limited to:

1. Infection: This can occur at the site of the incision or inside the body, such as pneumonia or urinary tract infection.
2. Bleeding: Excessive bleeding (hemorrhage) can lead to a drop in blood pressure and may require further surgical intervention.
3. Blood clots: These can form in the deep veins of the legs (deep vein thrombosis) and can potentially travel to the lungs (pulmonary embolism).
4. Wound dehiscence: This is when the surgical wound opens up, which can lead to infection and further complications.
5. Pulmonary issues: These include atelectasis (collapsed lung), pneumonia, or respiratory failure.
6. Cardiovascular problems: These include abnormal heart rhythms (arrhythmias), heart attack, or stroke.
7. Renal failure: This can occur due to various reasons such as dehydration, blood loss, or the use of certain medications.
8. Pain management issues: Inadequate pain control can lead to increased stress, anxiety, and decreased mobility.
9. Nausea and vomiting: These can be caused by anesthesia, opioid pain medication, or other factors.
10. Delirium: This is a state of confusion and disorientation that can occur in the elderly or those with certain medical conditions.

Prompt identification and management of these complications are crucial to ensure the best possible outcome for the patient.

A "newborn infant" refers to a baby in the first 28 days of life outside of the womb. This period is crucial for growth and development, but also poses unique challenges as the infant's immune system is not fully developed, making them more susceptible to various diseases.

"Newborn diseases" are health conditions that specifically affect newborn infants. These can be categorized into three main types:

1. Congenital disorders: These are conditions that are present at birth and may be inherited or caused by factors such as infection, exposure to harmful substances during pregnancy, or chromosomal abnormalities. Examples include Down syndrome, congenital heart defects, and spina bifida.

2. Infectious diseases: Newborn infants are particularly vulnerable to infections due to their immature immune systems. Common infectious diseases in newborns include sepsis (bloodstream infection), pneumonia, and meningitis. These can be acquired from the mother during pregnancy or childbirth, or from the environment after birth.

3. Developmental disorders: These are conditions that affect the normal growth and development of the newborn infant. Examples include cerebral palsy, intellectual disabilities, and vision or hearing impairments.

It is important to note that many newborn diseases can be prevented or treated with appropriate medical care, including prenatal care, proper hygiene practices, and timely vaccinations. Regular check-ups and monitoring of the newborn's health by a healthcare provider are essential for early detection and management of any potential health issues.

Imperforate anus is a congenital condition in which the opening of the anus is absent or abnormally closed or narrowed, preventing the normal passage of stool. This results in a blockage in the digestive tract and can lead to serious health complications if not treated promptly.

The anus is the external opening of the rectum, which is the lower end of the digestive tract. During fetal development, the rectum and anus normally connect through a canal called the anal canal or the recto-anal canal. In imperforate anus, this canal may be completely closed or narrowed, or it may not form properly.

Imperforate anus can occur as an isolated condition or as part of a genetic syndrome or other congenital abnormalities. The exact cause is not fully understood, but it is believed to result from a combination of genetic and environmental factors.

Treatment for imperforate anus typically involves surgery to create an opening in the anus and restore normal bowel function. In some cases, additional procedures may be necessary to correct related abnormalities or complications. The prognosis for individuals with imperforate anus depends on the severity of the condition and any associated abnormalities. With prompt and appropriate treatment, most people with imperforate anus can lead normal lives.

Hyperbilirubinemia is a medical condition characterized by an excessively high level of bilirubin in the bloodstream. Bilirubin is a yellowish pigment produced by the liver when it breaks down old red blood cells. Normally, bilirubin is conjugated (made water-soluble) in the liver and then excreted through the bile into the digestive system. However, if there is a problem with the liver's ability to process or excrete bilirubin, it can build up in the blood, leading to hyperbilirubinemia.

Hyperbilirubinemia can be classified as either unconjugated or conjugated, depending on whether the bilirubin is in its direct (conjugated) or indirect (unconjugated) form. Unconjugated hyperbilirubinemia can occur due to increased production of bilirubin (such as in hemolytic anemia), decreased uptake of bilirubin by the liver, or impaired conjugation of bilirubin in the liver. Conjugated hyperbilirubinemia, on the other hand, is usually caused by a problem with the excretion of conjugated bilirubin into the bile, such as in cholestatic liver diseases like hepatitis or cirrhosis.

Symptoms of hyperbilirubinemia can include jaundice (yellowing of the skin and eyes), dark urine, light-colored stools, itching, and fatigue. Treatment depends on the underlying cause of the condition and may involve medications, dietary changes, or surgery.

... is most common in East Asia, with a frequency of one in 5,000. The cause of biliary atresia in Egyptian infants ... If left untreated, biliary atresia can lead to liver failure. Unlike other forms of jaundice, however, biliary-atresia-related ... Syndromic biliary atresia (e.g. Biliary Atresia Splenic Malformation (BASM)) has been associated with certain genes (e.g. ... transposition of the great vessels and the syndromic form of biliary atresia. Chardot, Christophe (2006). "Biliary atresia". ...
Biliary atresia is not usually observed in patients with right atrial isomerism. Random positioning of the stomach is often one ... Biliary atresia, or inflammation and destruction of the bile ducts, may lead to jaundice. Vomiting and swelling of the ... This biliary atresia can lead to acute problems such as nutrient malabsorption, pale stools, dark urine, and abdominal swelling ... Following cholangiogram, a Kasai procedure is usually performed in cases of biliary atresia. In this surgery, a Y-shaped shunt ...
She was also part of the team that analyzed the handling of Biliary Atresia patients in England and Wales from 1999 to 2002 in ... McKiernan, P. J.; Baker, A. J.; Kelly, D. A. (1 January 2000). "The frequency and outcome of biliary atresia in the UK and ... McKiernan, P. J., Baker, A. J., & Kelly, D. A. (2000). The frequency and outcome of biliary - atresia in the UK and Ireland. ... Hepatology 2006 May; 43(5): 1109-1117 Hartley, J. L., Davenport, M., & Kelly, D. A. (2009). Biliary atresia. The Lancet, 374( ...
Examples of atresia include: Aural atresia (anotia), a congenital deformity where the ear canal is underdeveloped. Biliary ... "Biliary atresia". PubMed Health. Retrieved 11 September 2012. Zieve, David. "Choanal atresia". Pubmed Health. Retrieved 11 ... Congenital bronchial atresia, a rare congenital abnormality Choanal atresia, blockage of the back of the nasal passage, usually ... "Pulmonary atresia". PubMed Health. Retrieved 11 September 2012. "Tricuspid atresia". PubMed Health. Retrieved 11 September 2012 ...
Kasai and a colleague, Sozo Suzuki, worked together in the 1950s to devise a surgery to treat babies born with biliary atresia ... "About the Biliary Atresia Clinical Care Program". www.chop.edu. Children's Hospital of Philadelphia. Retrieved August 18, 2018 ... While the procedure is not a definitive cure for biliary atresia and about half of patients require liver transplantation by ... Bessho, Kazuhiko (December 2015). "Complications and quality of life in long-term survivors of biliary atresia with their ...
Govindarajan, Krishna Kumar (2016-12-28). "Biliary atresia: Where do we stand now?". World Journal of Hepatology. 8 (36): 1593- ... "Malnutrition in Biliary Atresia: Assessment, Management, and Outcomes". Liver Transplantation. 28 (3): 483-492. doi:10.1002/lt. ...
Chang has researched hepatitis B vaccination, biliary atresia in infants, and led the Children's Liver Foundation. She was ... Lee, I-chia (17 October 2016). "Parents warned against biliary atresia in infants". Taipei Times. Retrieved 20 February 2023. ...
Patman, G. (2015). "Biliary tract: Newly identified biliatresone causes biliary atresia". Nat Rev Gastroenterol Hepatol. 12 (7 ... It has been found to cause extrahepatic biliary atresia in a zebrafish model. The enone moiety of biliatresone is particularly ... May 2015). "Identification of a plant isoflavonoid that causes biliary atresia". Sci Transl Med. 7 (286): 286ra67. doi:10.1126/ ... 2016). "Reactivity of biliatresone, a natural biliary toxin, with glutathione, histamine, and amino acids". Chem. Res. Toxicol ...
"Management of Biliary Atresia". In: Surgical Disease of the Biliary Tract and Pancreas (1994) "Management of Primary Hepatic ... "Transplantation for Biliary Atresia". In: Transplantation of the Liver (1996) "Liver Transplantation". In: Maingot's Abdominal ... new techniques of liver surgery that improved outcomes following liver transplantation and non transplant liver and biliary ...
Timmy was diagnosed with biliary atresia upon birth. The condition required a liver transplant for survival and he underwent ...
Some of the causes of neonatal cholestasis are listed below: Biliary atresia Choledochal cyst Cholelithiasis Malignancy Alpha-1 ... Biliary atresia requires urgent surgical intervention with intraoperative cholangiography. A hepatoportoenterostomy (HPE) ... of neonatal cholestasis cases are caused by biliary atresia. This is one of the most common causes for neonatal cholestasis. ... and alternatives to Kasai hepato-portoenterostomy to improve the outcome in biliary atresia". Pediatric Surgery International. ...
Davit-Spraul A, Baussan C, Hermeziu B, Bernard O, Jacquemin E (2008). "CFC1 gene involvement in biliary atresia with ...
Recently, some natural isoflavonoids have been identified as toxins, including biliatresone which may cause biliary atresia ... "Identification of a plant isoflavonoid that causes biliary atresia". Science Translational Medicine. 7 (286): 286ra67. doi: ...
It is contraindicated in obstruction of biliary tracts such as biliary atresia. It's not effective in liver allograft rejection ... Kotb MA (July 2008). "Review of historical cohort: ursodeoxycholic acid in extrahepatic biliary atresia". Journal of Pediatric ... UDCA is used as therapy in primary biliary cholangitis (PBC; previously known as primary biliary cirrhosis) where it can ... which commonly occurs due to the rapid weight loss producing biliary cholesterol oversaturation and also biliary dyskinesia ...
"The extended Kasai portoenterostomy for biliary atresia: A preliminary report". Journal of Indian Association of Pediatric ...
... biliary atresia) and chromosome 22 aneuploidy. Allotey J, Lacaille F, Lees MM, Strautnieks S, Thompson RJ, Davenport M. J ... Hernias Biliary atresia Rarer malformations can affect almost any organ Intellectual disability - many are intellectually ... 1965). "Chromosomes in Coloboma and Anal Atresia". Lancet. 2 (7406): 290. doi:10.1016/S0140-6736(65)92415-3. PMID 14330081. ... Anal atresia (abnormal obstruction of the anus) Downward-slanting palpebral fissures (openings between the upper and lower ...
Mutations in this gene have also been associated with biliary atresia. Ho M, Kim H (February 2011). "Glypican-3: a new target ... "Evidence from human and zebrafish that GPC1 is a biliary atresia susceptibility gene". Gastroenterology. 144 (5): 1107-1115.e3 ...
Gómez-Bravo (Nov 2015). "Outcomes of Liver Transplantation During Adulthood After Kasai Portoenterostomy Due to Biliary Atresia ... cholangitis and malabsorption are common Many children with biliary atresia will require liver transplantation despite the ... or Kasai portoenterostomy is a surgical treatment performed on infants with Type IVb choledochal cyst and biliary atresia to ... This procedure was developed in 1951 by Japanese biliary and hepatic pediatric surgeon Morio Kasai (1922-2008). Prognosis of ...
Patients with biliary atresia may require a Kasai procedure to improve bile drainage; however, later liver transplantation is ... biliary atresia). Bile duct paucity results in the reduced absorption of fat and fat-soluble vitamins (A, D, E and K), which ... Partial biliary diversion has been used to significantly reduce pruritus, jaundice, and xanthoma caused by poor bile flow in ...
T cells recognize biliary epithelial cell antigens causing injury and eventual atresia. Primary biliary cirrhosis Primary ... Intrahepatic bile duct atresia (Alagille syndrome) (ALGS2 MIM:610205 and ALGS1 MIM:118450) Extrahepatic bile duct atresia ...
... can be carried out to relieve the symptoms of biliary obstruction. In infants with biliary atresia, hepatoportoenterostomy is ... Biliary drainage is performed with a tube or catheter (called a biliary drain, biliary stent or biliary catheter) by a surgeon ... A biliary drain can also be used to take bile samples for diagnostic workup or disease monitoring, as well as providing a route ... The biliary tree (see below) is the whole network of various sized ducts branching through the liver. The path is as follows: ...
His son was diagnosed with biliary atresia soon after birth, and required a liver transplant. This was a contributing factor ...
reported that the proteolytic activity of MMP7 plays major role in tissue remodeling in biliary atresia-associated liver ... is a major matrix metalloproteinase upregulated in biliary atresia-associated liver fibrosis". Mod. Pathol. 18 (7): 941-50. doi ...
... biliary atresia, and several cardiac malformations. Associated cardiac conditions include dextrocardia, atrial situs ambiguus, ... such as intestinal malrotation or biliary atresia, as well as cardiac abnormalities, such as dextrocardia. There are frequent ...
... and biliary atresia. Neonatal jaundice Pediatric Gastrointestinal and Liver Disease (5 ed.). Elsevier. 2016. pp. 823-837. Burt ...
Diseases associated with this genus include mild upper respiratory tract disease, gastroenteritis, and biliary atresia. ...
The camp is "A Special Camp for Special Kids." In 1984, Wolff's youngest son, Nicholas, was born with biliary atresia, an ...
... biliary atresia, and primary biliary cholangitis. Odevixibat was granted its initial approval in July 2021, in the European ... "Ileal bile acid transporter inhibition as an anticholestatic therapeutic target in biliary atresia and other cholestatic ...
Ben Hardwick was a two-year-old toddler dying of biliary atresia, with only a few weeks to live. He was being treated by ...
Structural abnormalities such as biliary atresia and choledochal cysts can lead to cholestatic liver injury leading to neonatal ... Autoimmune hepatitis is distinct from the other autoimmune diseases of the liver, primary biliary cirrhosis and primary ...
Biliary atresia is most common in East Asia, with a frequency of one in 5,000. The cause of biliary atresia in Egyptian infants ... If left untreated, biliary atresia can lead to liver failure. Unlike other forms of jaundice, however, biliary-atresia-related ... Syndromic biliary atresia (e.g. Biliary Atresia Splenic Malformation (BASM)) has been associated with certain genes (e.g. ... transposition of the great vessels and the syndromic form of biliary atresia. Chardot, Christophe (2006). "Biliary atresia". ...
Biliary atresia is characterized by obliteration or discontinuity of the extrahepatic biliary system, resulting in obstruction ... Postnatal biliary atresia. Patients with isolated biliary atresia (postnatal form) account for 65-90% of cases. The neonatal ... Patients with biliary atresia can be subdivided into 2 distinct groups: isolated biliary atresia (postnatal form) and ... No primary medical treatment is relevant in the management of extrahepatic biliary atresia. Once biliary atresia is suspected, ...
Biliary atresia is a condition that causes blockage of the bile ducts in newborn babies. This is a rare condition that appears ... What Is Pediatric Biliary Atresia?. Biliary atresia is a condition that causes blockage of the bile ducts in newborn babies. ... Causes of Pediatric Biliary Atresia. The cause of biliary atresia is unknown. The condition develops during the neonatal period ... When biliary atresia is diagnosed early, patients are more likely to be candidates for surgery and may be able to delay a liver ...
Congenital absence or closure of the bile ducts, biliary atresia, is a disease state of unknown etiology, controversial ...
A to Z: Atresia, Biliary. Atresia (ah-TREE-zhah) is a condition in which a baby is born with a missing or closed valve or tube ... Choanal atresia is a defect of the nasal passages (choana).. *Anal atresia (imperforate anus) and esophageal atresia are ... Tricuspid atresia, pulmonary atresia, and aortic atresia involve valves in the heart. ... Biliary atresia, a defect in the liver or bile system, means the ducts that carry bile from the liver to the gallbladder are ...
Stanford Medicine Childrens Health Biliary atresia is a rare liver disease that occurs in infants. It is often found shortly ... Biliary Atresia in Children. What is biliary atresia in children?. Biliary atresia is a rare liver disease that occurs in ... What causes biliary atresia in a child?. Experts dont know what causes biliary atresia. It is not passed down from parent to ... How is biliary atresia treated in a child?. Without surgery, biliary atresia can be fatal. Two types of surgery are used to ...
Biliary AtresiaHealth Professionals Blog Biliary atresia is associated with polygenic susceptibility in ciliogenesis and planar ... Biliary AtresiaHealth Professionals Blog Genetic contributions to biliary atresia: a developmental cholangiopathy ... Biliary AtresiaHealth Professionals Blog Cytomegalovirus in biliary atresia is associated with increased pretransplant death, ... Biliary AtresiaHealth Professionals BlogLiver Transplantation Cost-effectiveness analysis of liver transplantation in biliary ...
Biliary atresia or obliterative cholangiopathy is a multifactorial fibrosing and destructive disease that involves both ... duodenal atresia and anomaly of the portal vein * Cystic biliary atresia * Comprises 5 - 10% of patients and is identified on ... A. Biliary atresia. The patient is presenting with jaundice, cholestatic pattern of liver function test, nonvisualization of ... Mutations leading to laterality defects like CFIC and ZIC3 are identified in ~10% of biliary atresia patients (J Hepatobiliary ...
Importance: Biliary atresia is the most common cause of end-stage liver disease in children. Controversy exists as to whether ... Design, setting, and patients: The multicenter, double-blind Steroids in Biliary Atresia Randomized Trial (START) was conducted ... Use of corticosteroids after hepatoportoenterostomy for bile drainage in infants with biliary atresia: the START randomized ... Conclusions and relevance: Among infants with biliary atresia who have undergone hepatoportoenterostomy, high-dose steroid ...
Is it really biliary atresia_?. Author: Pediatric Oncall Question A 10 years old boy presented with ascites and joint stiffness ... Is this biliary atresia with neonatal ichthyosis and alopecia_? 2 Expert Opinion : The child seems to fit into Neonatal ... He was operated at 4 months of age for biliary atresia and a Kasai surgery was done. There is a history of neonatal ichthyosis ...
Overview of Biliary Atresia. Biliary Atresia (https://www.youtube.com/watch?v=pyT95PVUINI) by Osmosis (https://open.osmosis.org ...
We Think its Biliary Atresia. by Band Back Together , Jan 29, 2020 , Biliary Atresia, Birth Defects, Birth Trauma, NICU, ... And thats just not common in Biliary Atresia.. In "classic" biliary atresia, by the time most kids are born, their bile duct ... Our NICU surgeon made the comment to us that is the title of this entry, "We think its Biliary Atresia, but thats really bad ... And thats when he said that he hoped that this wasnt Biliary Atresia. He and the Pediatric GI attending to us in the NICU, ...
Archive institutionnelle de lUniversité de Genève - Institutional Repository of the University of Geneva
... Arch Dis Child. 2013 May;98(5):381-3. doi ... Aims: To evaluate the outcome of Scottish children with extra hepatic biliary atresia (EHBA) since rationalisation of Kasai ...
This issue contains information on: Biliary atresia in children under 6 monthsQuantiferon testingAllergy skin testingLabtests ...
Biliary Atresia, Diagnostic Errors, Humans, Infant, Male, Physical Examination, Referral and Consultation ...
... is a rare gastrointestinal disorder in which the bile ducts outside the liver are missing or damaged, so that ... Born with biliary atresia, a rare gastrointestinal disorder, Aaron Jackson has undergone 17 surgical procedures and 5 major ... "Usually for people who have biliary atresia, theyre in and out of the hospital, and they experience certain medical setbacks ... Not realising the connection to his biliary atresia, he assumed he was overworked and just needed more exercise. ...
Biliary atresia is characterized by obliteration or discontinuity of the extrahepatic biliary system, resulting in obstruction ... encoded search term (Pediatric Biliary Atresia) and Pediatric Biliary Atresia What to Read Next on Medscape ... Does the morphology of the extrahepatic biliary remnants in biliary atresia influence survival? A review of 205 cases. J ... Pediatric Biliary Atresia Differential Diagnoses. Updated: Nov 17, 2014 * Author: Steven M Schwarz, MD, FAAP, FACN, AGAF; Chief ...
Introduction For parents, children are the most valuable assets. They leave no stones unturned to provide them with necessities and facilities. They make no compromise to their childs health and provide with them the best healthcare facilities. They search for the best pediatric doctor and ... Read More ...
Biliary atresia. Biliary atresia is a congenital abnormality in which the bile duct is missing or malformed. The symptoms ... Biliary atresia. (2019).. https://rarediseases.org/rare-diseases/extrahepatic-biliary-atresia/. *. Cells that maintain and ...
"Biliary Atresia" by people in this website by year, and whether "Biliary Atresia" was a major or minor topic of these ... "Biliary Atresia" is a descriptor in the National Library of Medicines controlled vocabulary thesaurus, MeSH (Medical Subject ... Usually, biliary atresia is found in infants and accounts for one third of the neonatal cholestatic JAUNDICE. ... Cytomegalovirus in biliary atresia is associated with increased pretransplant death, but not decreased native liver survival. ...
Biliary Atresia - Etiology, pathophysiology, symptoms, signs, diagnosis & prognosis from the MSD Manuals - Medical Professional ... Symptoms and Signs of Biliary Atresia Infants with biliary atresia are jaundiced Neonatal Hyperbilirubinemia Jaundice is a ... Prognosis for Biliary Atresia Biliary atresia is progressive and, if untreated, results in cirrhosis with portal hypertension ... Infants with presumed biliary atresia require surgical exploration with an intraoperative cholangiogram. If biliary atresia is ...
CBAR was founded through the generous support of the CHILD Foundation and the Anthopoulos family donation to the Montreal Childrens Hospital Foundation. ...
On a trip in March 2023, Chad and Ethan Glaser soaked in the breathtaking views, marveling at the majestic, snow-capped peaks of the French Alps before gliding side by side down the powdered slopes. As Chad soared down the alpine landscape in Chamonix, France, glancing at his 22-year-old son, Ethan, he felt immense gratitude.
Biliary atresia symptoms are yellow skin, eyes in kids, due to bile buildup in liver from bile duct faults, with its treatment ... Causes Of Biliary Atresia:. The exact cause of biliary atresia is not known, but medical experts ascertain that the ... Biliary atresia is a liver condition in children wherein the bile fluids cannot flow from the hepatic tissues to the intestines ... Like Alagille syndrome, biliary atresia is also a rare ailment, medically termed as extrahepatic ductopenia and progressive ...
What is biliary Atresia?. Biliary Atresia:. Biliary atresia is a liver disease affecting small children. It causes damage, ... How is Biliary Atresia treated?. Biliary Atresia treated:. If the testing and cholangiogram show biliary atresia, infants need ... What causes an infant to have biliary Atresia?. Causes of Biliary Atresia:. The cause of biliary atresia is unknown. A number ... What are the symptoms of Biliary Atresia?. Symptoms of Biliary Atresia:. Infants with biliary atresia have jaundice (yellowing ...
She was diagnosed as Biliary atresia. Biliary atresia is a condition where the bile duct is not formed by birth; bile duct ... Biliary Atresia Case Study. Gayatri T Rao May 10, 2016 Case Study, Health Leave a comment 5,804 Views ... Biliary Atresia Case Study Liver Transplantation Medanta-the Medicity 2016-05-10 +Gayatri T Rao ... In Biliary atresia, the liver will gradually become cirrhotic and go towards failure. Paridhi underwent Kasai surgery for ...
  • The cause of biliary atresia in Egyptian infants has been proven to be as a result of aflatoxin induced cholangiopathy acquired prenatally in infants who have glutathione S transferase M1 deficiency. (wikipedia.org)
  • citation needed] The cause of biliary atresia in most infants is not fully understood and it is well possible that a number of factors may play a role, but especially maternal rotavirus infection during pregnancy and subsequent transmission of the virus to the child resulting in infection of the biliary epithelium and subsequent occluding fibrosis may be important in this respect. (wikipedia.org)
  • The cause of biliary atresia is unknown. (memorialhermann.org)
  • Genetic reason or a combination of these things also been a postulated cause of biliary atresia. (faisaldar.com)
  • There is no clear cause of biliary atresia. (drcolinknight.com)
  • Surgical intervention is the only means available for a definitive diagnosis of biliary atresia (intraoperative cholangiography) and therapy (Kasai portoenterostomy). (medscape.com)
  • He was operated at 4 months of age for biliary atresia and a Kasai surgery was done. (pediatriconcall.com)
  • To evaluate the outcome of Scottish children with extra hepatic biliary atresia (EHBA) since rationalisation of Kasai services to three English centres in 2002 (The 'Group A' centres). (nih.gov)
  • The Anatomic Pattern of Biliary Atresia Identified at Time of Kasai Hepatoportoenterostomy and Early Postoperative Clearance of Jaundice Are Significant Predictors of Transplant-Free Survival. (medscape.com)
  • Serum bile acids as a prognostic biomarker in biliary atresia following Kasai portoenterostomy. (ucdenver.edu)
  • Our patient, a 6-year-old girl, underwent a porto-enterostomy (Kasai) operation on the 45 th day of delivery due to biliary atresia. (transpopmed.org)
  • She had a Kasai operation on the 45 th day after birth to treat biliary atresia. (transpopmed.org)
  • While the Kasai procedure does not cure biliary atresia, it allows the child to develop healthily. (drcolinknight.com)
  • Describe how surgical techniques and multidisciplinary care can improve biliary drainage after Kasai portoenterostomy, thus avoiding rapid progression of liver disease. (cloud-cme.com)
  • Small Intestinal Adenocarcinoma Arising at the Anastomotic Site after Kasai Operation for Biliary Atresia: A Case Report and Literature Review. (bvsalud.org)
  • Polycystic Kidney Disease 1 Like 1 - PKD1L1), and some infants with isolated biliary atresia may arise as a result of an autoimmune inflammatory response, possibly due to a viral infection of the liver soon after birth. (wikipedia.org)
  • Initially, the symptoms of biliary atresia are indistinguishable from those of neonatal jaundice, a usually harmless condition commonly seen in infants. (wikipedia.org)
  • However, infants with biliary atresia develop progressive conjugated jaundice, pale white stools, and dark urine. (wikipedia.org)
  • citation needed] Egyptian infants with biliary atresia were found to have null GSTM1 genotype while all their mothers were heterozygous for GSTM1. (wikipedia.org)
  • Hepatobiliary scintiscanning is useful in evaluating infants with suspected biliary atresia. (medscape.com)
  • Although histopathologic features of biliary atresia have been extensively studied in surgical specimens from excised extrahepatic biliary systems of infants undergoing portoenterostomy, the pathogenesis of this disorder remains poorly understood. (medscape.com)
  • In 10% to 15% of infants, biliary atresia can be associated with other abnormalities of the spleen, intestines, heart or urinary system. (memorialhermann.org)
  • Biliary atresia is a rare liver disease that occurs in infants. (stanfordchildrens.org)
  • Premature babies are at greater risk for biliary atresia than full-term infants. (stanfordchildrens.org)
  • Among infants with biliary atresia who have undergone hepatoportoenterostomy, high-dose steroid therapy following surgery did not result in statistically significant treatment differences in bile drainage at 6 months, although a small clinical benefit could not be excluded. (nih.gov)
  • Beyond the Pediatric end-stage liver disease system: solutions for infants with biliary atresia requiring liver transplant. (medscape.com)
  • Usually, biliary atresia is found in infants and accounts for one third of the neonatal cholestatic JAUNDICE. (ucdenver.edu)
  • Biliary atresia only occurs in young infants (less than 3-4 months of age). (faisaldar.com)
  • About 10-20% of infants with biliary atresia abnormalities in other organs. (faisaldar.com)
  • Infants with biliary atresia have jaundice (yellowing of eyes and skin). (faisaldar.com)
  • Infants with biliary atresia may also have a stool color that is lighter than normal. (faisaldar.com)
  • Infants with biliary atresia may also dark urine, a large liver, or a large spleen. (faisaldar.com)
  • Objectives: To evaluate MRI findings and to generate a decision tree model for diagnosis of biliary atresia (BA) in infants with jaundice. (elsevierpure.com)
  • Biliary atresia is a rare disease that strikes the liver and bile ducts of infants, typically diagnosed between two to eight weeks old. (drcolinknight.com)
  • Why Biliary Atresia Demands Our Respect Dr William Balistreri explains how interceding quickly can help limit the devastating effects of this rare disease in infants. (medscape.com)
  • Unlike other forms of jaundice, however, biliary-atresia-related cholestasis mostly does not result in kernicterus, a form of brain damage resulting from liver dysfunction. (wikipedia.org)
  • Many healthy newborns experience jaundice during the first week of life, and without other symptoms it usually does not indicate biliary atresia. (memorialhermann.org)
  • The characteristic indications of biliary atresia closely resemble those of newborn jaundice , including excess bilirubin in blood along with the yellowing of skin and eyes. (netmeds.com)
  • Jaundice part of biliary atresia caused by a substance called "bilirubin" building up in the blood. (faisaldar.com)
  • Jaundice resulting from biliary atresia tends to occur two to three weeks being born. (drcolinknight.com)
  • Apply a set of diagnostic tests available in his/her clinical practice to rapidly distinguish biliary atresia from other causes of neonatal jaundice. (cloud-cme.com)
  • This is because in biliary atresia, the liver, although diseased, is still able to conjugate bilirubin, and conjugated bilirubin is unable to cross the blood-brain barrier. (wikipedia.org)
  • Babies with biliary atresia have high levels of direct bilirubin. (memorialhermann.org)
  • Diagnostic yield of newborn screening for biliary atresia using direct or conjugated bilirubin measurements. (msdmanuals.com)
  • There is no cure for biliary atresia, but it can be treated to improve symptoms. (memorialhermann.org)
  • There exists no cure for biliary atresia in newborns and young children. (netmeds.com)
  • Bile ductular proliferation in liver biopsy specimen (hematoxylin and eosin stain) from patient with biliary atresia. (medscape.com)
  • The presence of a hypoplastic left ventricle in a patient with biliary atresia has previously been reported only in the context of a heterotaxy syndrome. (aku.edu)
  • If these tests point to a possible diagnosis of biliary atresia, your doctor may order the intraoperative cholangiogram. (faisaldar.com)
  • Clinical diagnosis of biliary atresia or primary sclerosing cholangitis. (stanford.edu)
  • MRI has comparable performance to ultrasonography for diagnosis of biliary atresia. (elsevierpure.com)
  • We present data on increased likelihood of unpalliated and late diagnosis biliary atresia among non-white patients, approaches to improve early diagnosis across all populations, and controversies in surgical management among patients with late diagnoses. (cloud-cme.com)
  • Biliary atresia is characterized by obliteration or discontinuity of the extrahepatic biliary system, resulting in obstruction to bile flow. (medscape.com)
  • Biliary atresia is obstruction of the biliary tree due to progressive sclerosis of the extrahepatic bile duct. (msdmanuals.com)
  • In neonatal cholestasis syndromes, ultrasonography can exclude specific anomalies of the extrahepatic biliary system. (medscape.com)
  • Biliary atresia represents the most common surgically treatable cause of cholestasis encountered during the newborn period. (medscape.com)
  • The viral association of neonatal cholestasis in Sweden: a possible link between cytomegalovirus infection and extrahepatic biliary atresia. (medscape.com)
  • MRI-based decision tree model reliably differentiates biliary atresia in neonatal cholestasis. (elsevierpure.com)
  • Biliary atresia associated with hypoplastic left heart syndrome: A cas" by Daniel J. Becker, Saleem Islam et al. (aku.edu)
  • The authors describe an infant with both biliary atresia and hypoplastic left heart syndrome (HLHS) in the absence of a laterality defect or evidence of any other morphologic defect. (aku.edu)
  • Babies with biliary atresia often have a gallbladder that is small or collapsed. (memorialhermann.org)
  • Biliary atresia, a defect in the liver or bile system, means the ducts that carry bile from the liver to the gallbladder are blocked. (akronchildrens.org)
  • In "classic" biliary atresia, by the time most kids are born, their bile duct structure (gallbladder included) has shriveled up and is not working. (bandbacktogether.com)
  • If not surgically corrected, secondary biliary cirrhosis invariably results. (medscape.com)
  • However, in instances of biliary atresia, owing to faulty or absent bile duct structures, bile cannot pass through to the intestines, thereby accumulating in the liver and resulting in fatty liver, haemochromatosis, hepatic cirrhosis and liver damage. (netmeds.com)
  • A 6-year-old female (height: 115 cm, weight: 22 kg) patient with pediatric living donor LT for biliary atresia (cirrhosis with portal hypertension) with a pulmonary AVF, HPS, was admitted to an intensive care unit (ICU) transplantation. (transpopmed.org)
  • Bittmann S. Surgical experience in children with biliary atresia treated with portoenterostomy. (medscape.com)
  • Patients with biliary atresia can be subdivided into 2 distinct groups: isolated biliary atresia (postnatal form) and associated situs inversus or polysplenia / asplenia with or without other congenital anomalies (fetal/embryonic form). (medscape.com)
  • Intraoperative cholangiography definitively demonstrates the anatomy and patency of the extrahepatic biliary tract. (medscape.com)
  • The goals of the proposed work are two fold: Firstly, to see if the antibiotic vancomycin may be used for the early treatment of Biliary Atresia (BA) and Primary Sclerosing Cholangitis (PSC). (stanford.edu)
  • The investigators will focus on primary sclerosing cholangitis, biliary atresia, as well as states of health. (stanford.edu)
  • The Human Gastrointestinal Tract Microbiota in the Setting of Treating Primary Sclerosing Cholangitis and Biliary Atresia With Vancomycin. (stanford.edu)
  • Clinical controls who are undergoing upper endoscopy or colonoscopy and do not have biliary atresia or primary sclerosing cholangitis. (stanford.edu)
  • Malnutrition in Biliary Atresia: Assessment, Management, and Outcomes. (ucdenver.edu)
  • Please join us in the discussion of paradigms in care to improve outcomes for children with biliary atresia across the US and beyond. (cloud-cme.com)
  • Explain the emerging framework of the pathogenesis of biliary atresia and how racial inequities may affect surgical outcomes and survival with the native liver. (cloud-cme.com)
  • The rhesus rotavirus gene encoding VP4 is a major determinant in the pathogenesis of biliary atresia in newborn mice. (medscape.com)
  • Biliary atresia: pathogenesis and treatment. (medscape.com)
  • Biliary atresia is a condition that causes blockage of the bile ducts in newborn babies. (memorialhermann.org)
  • Biliary atresia is a rare but serious liver disorder that affects newborn babies. (kidshealth.org.nz)
  • What are the symptoms of biliary atresia in a child? (stanfordchildrens.org)
  • Symptoms of biliary atresia may look like other liver conditions or health problems. (stanfordchildrens.org)
  • With the assigned delivery doctor on holiday, a stand-in recognised Aaron's symptoms straight away, having seen a colleague's notes about a condition called biliary atresia. (pagesuite.com)
  • Here, we report a false-positive case of hepatobiliary scintigraphy in a child with a choledochal cyst, which mimicked biliary atresia. (ajou.ac.kr)
  • Primary vs. salvage liver transplantation for biliary atresia: A retrospective cohort study. (ucdenver.edu)
  • Biliary atresia, also known as extrahepatic ductopenia and progressive obliterative cholangiopathy, is a childhood disease of the liver in which one or more bile ducts are abnormally narrow, blocked, or absent. (wikipedia.org)
  • Like Alagille syndrome, biliary atresia is also a rare ailment, medically termed as extrahepatic ductopenia and progressive obliterative cholangiopathy. (netmeds.com)
  • Perinatal biliary atresia . (stanfordchildrens.org)
  • Sox17 haploinsufficiency results in perinatal biliary atresia and hepatitis in C57BL/6 background mice. (medscape.com)
  • There are three main types of extra-hepatic biliary atresia:[citation needed] Type I: Atresia is restricted to the common bile duct. (wikipedia.org)
  • 90% of patients) involves atresia of the right and left hepatic ducts to the level of the porta hepatis. (medscape.com)
  • Biliary atresia is a liver condition in children wherein the bile fluids cannot flow from the hepatic tissues to the intestines, due to the bile ducts being rather narrow, obstructed or entirely missing. (netmeds.com)
  • Relatively good hepatic uptake with no evidence of excretion into the bowel for up to 24 h is a representative finding of biliary atresia. (ajou.ac.kr)
  • For children who have biliary atresia, the bile ducts between the liver and the small intestine are blocked by scar tissue. (memorialhermann.org)
  • Children with biliary atresia and significant liver damage may need a liver transplant by about age 2. (memorialhermann.org)
  • Children and young adults with biliary atresia can still experience a good quality of life. (memorialhermann.org)
  • Which children are at risk for biliary atresia? (stanfordchildrens.org)
  • Biliary atresia is the most common cause of end-stage liver disease in children. (nih.gov)
  • Steroid treatment was associated with earlier onset of serious adverse events in children with biliary atresia. (nih.gov)
  • Effect of ursodeoxycholic acid on liver function in children after successful surgery for biliary atresia. (medscape.com)
  • Risk of variceal hemorrhage and pretransplant mortality in children with biliary atresia. (ucdenver.edu)
  • Biliary atresia is a liver disease affecting small children. (faisaldar.com)
  • New Studies on Pediatric Liver Diseases Dr William Balistreri shares new data from this year's Liver Meeting on fatty liver disease and biliary atresia in children and young adults. (medscape.com)
  • The liver biopsy can help the pathologist look for other causes of liver failure or determine if the scarring around the small bile tubes in the liver is consistent with biliary atresia. (memorialhermann.org)
  • These variants should not be confused with intrahepatic biliary hypoplasia, which comprises a group of distinct and surgically noncorrectable disorders. (medscape.com)
  • Type III: Atresia involves the most proximal part of the bile ducts (>95% of all cases). (wikipedia.org)
  • To determine whether the addition of high-dose corticosteroids after hepatoportoenterostomy is superior to surgery alone in improving biliary drainage and survival with the native liver. (nih.gov)
  • The fetal/embryonic form of atresia is associated with other congenital anomalies. (medscape.com)
  • Anal atresia (imperforate anus) and esophageal atresia are defects of the digestive tract. (akronchildrens.org)
  • We reviewed the literature to identify GWAS on oral clefts , congenital heart defects (CHDs) , biliary atresia , pyloric stenosis , hypospadias , craniosynostosis , and clubfoot . (cdc.gov)
  • Mogul D, Zhou M, Intihar P, Schwarz K, Frick K. Cost-Effective Analysis of Screening for Biliary Atresia With The Stool Color Card. (medscape.com)
  • In biliary atresia, the bile ducts are absent (that's what atresia means) so there is no way for the bile produced in the liver to reach the intestines. (drcolinknight.com)
  • Many newborns with biliary atresia appear healthy when they are born. (memorialhermann.org)
  • In addition to the routine physical exam all newborns undergo at birth, doctors may recommend any of the following tests if biliary atresia is suspected. (memorialhermann.org)
  • Tricuspid atresia , pulmonary atresia , and aortic atresia involve valves in the heart . (akronchildrens.org)
  • We present a 6-year-old female patient with successful anesthetic management of pediatric living donor LT with HPS and pulmonary arteriovenous fistula (AVF) due to biliary atresia. (transpopmed.org)
  • Herein, we present the successful anesthetic management of a pediatric living donor LT for biliary atresia with a pulmonary AVF and HPS in the light literature. (transpopmed.org)
  • Biliary Atresia" is a descriptor in the National Library of Medicine's controlled vocabulary thesaurus, MeSH (Medical Subject Headings) . (ucdenver.edu)
  • In animals plant toxins have been shown to cause biliary atresia. (wikipedia.org)
  • The plants were later found to contain a toxin, now called biliatresone Studies are ongoing to determine whether there is a link between human cases of biliary atresia and toxins such as biliatresone. (wikipedia.org)

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