Excretion of an excessive amount of OXALATES in the urine.
A genetic disorder characterized by excretion of large amounts of OXALATES in urine; NEPHROLITHIASIS; NEPHROCALCINOSIS; early onset of RENAL FAILURE; and often a generalized deposit of CALCIUM OXALATE. There are subtypes classified by the enzyme defects in glyoxylate metabolism.
Derivatives of OXALIC ACID. Included under this heading are a broad variety of acid forms, salts, esters, and amides that are derived from the ethanedioic acid structure.
The calcium salt of oxalic acid, occurring in the urine as crystals and in certain calculi.
Formation of stones in the KIDNEY.
A subclass of enzymes of the transferase class that catalyze the transfer of an amino group from a donor (generally an amino acid) to an acceptor (generally a 2-keto acid). Most of these enzymes are pyridoxyl phosphate proteins. (Dorland, 28th ed) EC 2.6.1.
Stones in the KIDNEY, usually formed in the urine-collecting area of the kidney (KIDNEY PELVIS). Their sizes vary and most contains CALCIUM OXALATE.
A condition characterized by calcification of the renal tissue itself. It is usually seen in distal RENAL TUBULAR ACIDOSIS with calcium deposition in the DISTAL KIDNEY TUBULES and the surrounding interstitium. Nephrocalcinosis causes RENAL INSUFFICIENCY.
The sole species of the genus Oxalobacter consisting of straight or curved gram-negative rods with rounded ends. Cells are nonmotile, nonsporing, and use oxylates as the only source of CARBON and energy, with formate and CARBON DIOXIDE as end products. They are isolated from lake sediments and from the rumen or large bowel of humans and animals. (From Bergey's Manual of Determinative Bacteriology, 9th ed)
Formation of stones in any part of the URINARY TRACT, usually in the KIDNEY; URINARY BLADDER; or the URETER.
A strong dicarboxylic acid occurring in many plants and vegetables. It is produced in the body by metabolism of glyoxylic acid or ascorbic acid. It is not metabolized but excreted in the urine. It is used as an analytical reagent and general reducing agent.
Excretion of abnormally high level of CALCIUM in the URINE, greater than 4 mg/kg/day.
Low-density crystals or stones in any part of the URINARY TRACT. Their chemical compositions often include CALCIUM OXALATE, magnesium ammonium phosphate (struvite), CYSTINE, or URIC ACID.
Derivatives of ACETIC ACID which contain an hydroxy group attached to the methyl carbon.
A colorless, odorless, viscous dihydroxy alcohol. It has a sweet taste, but is poisonous if ingested. Ethylene glycol is the most important glycol commercially available and is manufactured on a large scale in the United States. It is used as an antifreeze and coolant, in hydraulic fluids, and in the manufacture of low-freezing dynamites and resins.
Glyoxylates are organic compounds that are intermediate products in the metabolic pathways responsible for the breakdown and synthesis of various molecules, including amino acids and carbohydrates, and are involved in several biochemical processes such as the glyoxylate cycle.
A procedure consisting of the SURGICAL ANASTOMOSIS of the proximal part of the JEJUNUM to the distal portion of the ILEUM, so as to bypass the nutrient-absorptive segment of the SMALL INTESTINE. Due to the severe malnutrition and life-threatening metabolic complications, this method is no longer used to treat MORBID OBESITY.
A condition that is characterized by chronic fatty DIARRHEA, a result of abnormal DIGESTION and/or INTESTINAL ABSORPTION of FATS.
Surgical creation of an opening (stoma) in the URINARY BLADDER for drainage.
A species of gram-positive, rod-shaped LACTIC ACID bacteria that is frequently used as starter culture in SILAGE fermentation, sourdough, and lactic-acid-fermented types of beer and wine.
An enzyme that catalyzes the oxidation of D-glycerate to hydroxypyruvate in the presence of NADP.
A hydroxylated form of the imino acid proline. A deficiency in ASCORBIC ACID can result in impaired hydroxyproline formation.
A powder that dissolves in water, which is administered orally, and is used as a diuretic, expectorant, systemic alkalizer, and electrolyte replenisher.
Enzymes that catalyze the cleavage of a carbon-carbon bond of a 3-hydroxy acid. (Dorland, 28th ed) EC 4.1.3.
A key intermediate in metabolism. It is an acid compound found in citrus fruits. The salts of citric acid (citrates) can be used as anticoagulants due to their calcium chelating ability.
The end-stage of CHRONIC RENAL INSUFFICIENCY. It is characterized by the severe irreversible kidney damage (as measured by the level of PROTEINURIA) and the reduction in GLOMERULAR FILTRATION RATE to less than 15 ml per min (Kidney Foundation: Kidney Disease Outcome Quality Initiative, 2002). These patients generally require HEMODIALYSIS or KIDNEY TRANSPLANTATION.
The formation of crystalline substances from solutions or melts. (McGraw-Hill Dictionary of Scientific and Technical Terms, 4th ed)
The transference of a kidney from one human or animal to another.
A subclass of enzymes which includes all dehydrogenases acting on primary and secondary alcohols as well as hemiacetals. They are further classified according to the acceptor which can be NAD+ or NADP+ (subclass 1.1.1), cytochrome (1.1.2), oxygen (1.1.3), quinone (1.1.5), or another acceptor (1.1.99).
Electron-dense cytoplasmic particles bounded by a single membrane, such as PEROXISOMES; GLYOXYSOMES; and glycosomes.

Plasma calcium oxalate supersaturation in children with primary hyperoxaluria and end-stage renal failure. (1/80)

BACKGROUND: Children with primary hyperoxaluria type 1 (PH 1) are at great risk to develop systemic oxalosis in end-stage renal disease (ESRD), as endogenous oxalate production exceeds oxalate removal by dialytic therapy. As oxalate accumulates, calcium oxalate (CaOx) tissue deposition occurs. Children with other causes of ESRD, however, are not prone to CaOx deposition despite elevated plasma oxalate (POx) levels. METHODS: Our study objective was to examine the potential mechanisms for these observations. We measured POx, sulfate, citrate, and calculated CaOx saturation (betaCaOx) in 7 children with ESRD caused by PH 1 and in 33 children with non-PH-related ESRD. Maintenance hemodialysis (HD) was performed in 6 PH 1 and 22 non-PH patients: Pre- and post-HD levels were analyzed at this point and were repeated twice within 12 months in 5 PH 1 and 14 non-PH patients. Samples were obtained only once in 12 patients (one PH 1) on peritoneal dialysis (PD). After liver-kidney or kidney transplantation, plasma levels were measured repetitively. RESULTS: The mean POx was higher in PH 1 (125.7 +/- 17.9 micromol/liter) than in non-PH patients (44.2 +/- 3.3 micromol/liter, P < 10(-4)). All other determined anions did not differ between the two groups. betaCaOx was higher in PH 1 (4.71 +/- 0.69 relative units) compared with non-PH children (1.56 +/- 0.12 units, P < 10(-4)). POx and betaCaOx were correlated in both the PH 1 (r = 0.98, P < 2 x 10(-4)) and the non-PH group (r = 0.98, P < 10(-4)). POx and betaCaOx remained stable over time in the non-PH children, whereas an insignificant decline was observed in PH 1 patients after six months of more aggressive dialysis. betaCaOx was supersaturated (more than 1) in all PH 1 and in 25 out of 33 non-PH patients. Post-HD betaCaOx remained more than 1 in all PH 1, but in only 2 out of 22 non-PH patients. In non-PH children, POx and betaCaOx decreased to normal within three weeks after successful kidney transplantation, whereas the levels still remained elevated seven months after combined liver-kidney transplantation in two PH 1 patients. CONCLUSION: Systemic oxalosis in PH 1 children with ESRD is due to higher POx and betaCaOx levels. As betaCaOx remained supersaturated in PH 1 even after aggressive HD, oxalate accumulation increases, and CaOx tissue deposition occurs. Therefore, sufficient reduction of POx and betaCaOx is crucial in PH 1 and might only be achieved by early, preemptive, combined liver-kidney transplantation or liver transplantation alone.  (+info)

Transplantation for primary hyperoxaluria in the United States. (2/80)

BACKGROUND: Transplantation (TX) has become an acceptable treatment for renal failure in primary hyperoxaluria (PH). We have analyzed data from three U.S. sources to estimate the success or failure of different modes of management in PH patients. METHODS: The United States Renal Data System (USRDS) tapes provided coded medical record data, with PH assigned to 235 patients from 1974 to 1996. Another 45 patients were found from USRDS hospitalization records. We limited patients to those developing end-stage renal disease at <55 years of age after 1984 (95 PH patients). The North American Pediatric Renal Transplantation Cooperative Study (NAPRTCS) identified 34 (11 new) PH patients, and the United Network for Organ Sharing (UNOS) database identified PH in 34 (16 new, 5 more in both UNOS and NAPRTCS) patients. These secondary sources were used to correct some data from the USRDS and to add 32 more patients, with a total of 128 PH patients. Considering kidney TX (KTX) prior to combined kidney/liver TX (K/LTX) as a separate record for some calculations, the total "cases" were 138. RESULTS: By life table analysis, the 94 total TX patient survival was better than for the 34 NoTX patients (P<0.001). The 52 KTX patients' survival was better than either the 32 primary K/LTX (P<0.001) or the 10 K/LTX that following KTX (P<0.001). The 62 KTX cases' survival was better than the 42 K/LTX cases (P<0.005), which did not differ from the 34 NoTX (P<0.67). The overall survival of these 62 KTX patients was 76%. The survival of 42 K/LTX was 69%, and the survival of 34 NoTX patients was 44%. Kidney graft life table projected survival curves for TX patients did not differ between K/LTX (56% at 6 years) and isolated KTX (51% at 6 years, 35% at 10 years, P<0.91). CONCLUSION: KTX offers better patient survival in the United States then either K/LTX or NoTX. Graft survival does not differ between KTX and K/LTX. Because K/LTX can still follow a failed KTX, isolated living related donor KTX is still a reasonable first option for PH type 1 if a strictly managed protocol is followed.  (+info)

Effects of amlodipine on tubulointerstitial lesions in normotensive hyperoxaluric rats. (3/80)

Although controversial, a number of reports have suggested that calcium antagonists can retard or prevent the progression of various renal diseases in experimental models. Nevertheless, there are few data related to tubulointerstitial changes in these studies. On the other hand, hyperoxaluria is a recognized cause of tubulointerstitial lesions, and this could contribute to the development of hypertension and chronic renal failure. The aim of the present study was to evaluate a possible beneficial effect of amlodipine, a 1,4-dihydropyridine class of calcium antagonist, in a model of primary tubulointerstitial lesion produced by hyperoxaluria. Two-month-old male Sprague-Dawley rats were separated into 4 groups for a 4-week period: G1 (control; tap water only); G2 (hyperoxaluric); G3 (hyperoxaluric plus amlodipine treatment); and G4 (amlodipine treatment). G2 and G3 rats were given 1% ethylene glycol (a precursor for oxalates) in drinking water, and G3 and G4 rats were given amlodipine 2 mg. kg(-1). d(-1) by gavage. At the end of the study, we evaluated by semiquantitative scores (0 to 4) the different renal tubulointerstitial lesions, urinary albumin excretion, renal function by creatinine clearance, and blood pressure. Rats belonging to the hyperoxaluric group treated with amlodipine (G3) had fewer tubulointerstitial lesions, as follows: (1) inflammatory infiltrate score: 3.31+/-0.07 versus 0.23+/-0.12; P<0.05; (2) tubular atrophy score: 3.33+/-0.33 versus 0.50+/-0.22, P<0.05; (3) interstitial fibrosis score: 2.76+/-0.34 versus 0.31+/-0. 16, P<0.05; (4) oxalate deposits score: 3.66+/-0.33 versus 0.09+/-0. 08, P<0.05; (5) lower urinary albumin excretion (11.3+/-2 versus 27+/-4.5 mg/d, P<0.01); and (6) higher creatinine clearance (1. 22+/-0.08 versus 1.13+/-0.08, P<0.01) compared with the hyperoxaluric group untreated with amlodipine (G2). On the other hand, there were no significant changes in blood pressure in any group. In view of these data, we suggest that amlodipine, probably by nonhemodynamic mechanisms of action, can provide an important benefit in the prevention of epithelial tubular cell injury and inflammatory response and therefore in the prevention of the progressive tubulointerstitial fibrosis caused by oxalates.  (+info)

Identification and expression of a cDNA for human hydroxypyruvate/glyoxylate reductase. (4/80)

The isolation and expression of a human liver cDNA encoding a 40-kDa protein with glyoxylate and hydroxypyruvate reductase activities is described. The cDNA (GLXR) is 1235 bp and consists of a predicted open reading frame of 987 bp with a 225-bp 3'-untranslated region. The 328-amino acid protein has partial sequence similarity to hydroxypyruvate and glyoxylate reductases from a variety of plant and microbial species.  (+info)

Primary hyperoxaluria type I: a model for multiple mutations in a monogenic disease within a distinct ethnic group. (5/80)

Primary hyperoxaluria type 1 is an autosomal recessive inherited metabolic disease in which excessive oxalates are formed by the liver and excreted by the kidneys, causing a wide spectrum of phenotypes ranging from renal failure in infancy to mere renal stones in late adulthood. Mutations in the AGXT gene, encoding the liver-specific enzyme alanine:glyoxylate aminotransferase, are responsible for the disease. Seven mutations were detected in eight families in Israel. Four of these mutations are novel and three occur in children living in single-clan villages. The mutations are scattered along various exons (1, 4, 5, 7, 9, 10), and on different alleles comprising at least five different haplotypes. All but one of the mutations are in a homozygous pattern, reflecting the high rate of consanguinity in our patient population. Two affected brothers are homozygous for two different mutations expressed on the same allele. The patients comprise a distinct ethnic group (Israeli Arabs) residing in a confined geographic area. These results, which are supported by previous data, suggest for the first time that the phenomenon of multiple mutations in a relatively closed isolate is common and almost exclusive to the Israeli-Arab population. Potential mechanisms including selective advantage to heterozygotes, digenic inheritance, and the recent emergence of multiple mutations are discussed.  (+info)

Functional synergism between the most common polymorphism in human alanine:glyoxylate aminotransferase and four of the most common disease-causing mutations. (6/80)

The autosomal recessive disorder primary hyperoxaluria type 1 (PH1) is caused by a deficiency of the liver-specific pyridoxal-phosphate-dependent enzyme alanine:glyoxylate aminotransferase (AGT). Numerous mutations and polymorphisms in the gene encoding AGT have been identified, but in only a few cases has the causal relationship between genotype and phenotype actually been demonstrated. In this study, we have determined the effects of the most common naturally occurring amino acid substitutions (both normal polymorphisms and disease-causing mutations) on the properties, especially specific catalytic activity, of purified recombinant AGT. The results presented in this paper show the following: 1) normal human His-tagged AGT can be expressed at high levels in Escherichia coli and purified in a correctly folded, dimerized and catalytically active state; 2) presence of the common P11L polymorphism decreases the specific activity of purified recombinant AGT by a factor of three; 3) AGTs containing four of the most common PH1-specific mutations (G41R, F152I, G170R, and I244T) are all soluble and catalytically active in the absence of the P11L polymorphism, but in its presence all lead to protein destabilization and aggregation into inclusion bodies; 4) naturally occurring and artificial amino acid substitutions that lead to peroxisome-to-mitochondrion AGT mistargeting in mammalian cells also lead to destabilization and aggregation in E. coli; and 5) the PH1-specific G82E mutation abolishes AGT catalytic activity by interfering with cofactor binding, as does the artificial K209R mutation at the putative site of cofactor Shiff base formation. These results are discussed in the light of the high allelic frequency ( approximately 20%) of the P11L polymorphism and its importance in determining the phenotypic manifestations of mutations in PH1.  (+info)

Small intestinal infarction: a fatal complication of systemic oxalosis. (7/80)

Primary hyperoxaluria is a rare genetic disorder characterised by calcium oxalate nephrolithiasis and nephrocalcinosis leading to renal failure, often with extra-renal oxalate deposition (systemic oxalosis). Although ischaemic complications of crystal deposition in vessel walls are well recognised clinically, these usually take the form of peripheral limb or cutaneous ischaemia. This paper documents the first reported case of fatal intestinal infarction in a 49 year old woman with systemic oxalosis and advocates its consideration in the differential diagnosis of an acute abdomen in such patients.  (+info)

AGXT gene mutations and their influence on clinical heterogeneity of type 1 primary hyperoxaluria. (8/80)

Primary hyperoxaluria type 1 (PH1) is an autosomal recessive disorder that is caused by a deficiency of alanine: glyoxylate aminotransferase (AGT), which is encoded by a single copy gene (AGXT). Molecular diagnosis was used in conjunction with clinical, biochemical, and enzymological data to evaluate genotype-phenotype correlation. Twenty-three unrelated, Italian PH1 patients were studied, 20 of which were grouped according to severe form of PH1 (group A), adult form (group B), and mild to moderate decrease in renal function (group C). All 23 patients were analyzed by using the single-strand conformation polymorphism technique followed by the sequencing of the 11 AGXT exons. Relevant chemistries, including plasma, urine and dialyzate oxalate and glycolate assays, liver AGT activity, and pyridoxine responsiveness, were performed. Both mutant alleles were found in 21 out of 23 patients, and 13 different mutations were recognized in exons 1, 2, 4, and 10. Normalized AGT activity was lower in the severe form than in the adult form (P < 0.05). Double heterozygous patients presented a lower age at the onset of the disease (P = 0.025), and they were more frequent in group A (75%) than in the group B (14%; P = 0.0406). The T444C mutation was more frequent in the severe form (P < 0.05), and the opposite was observed for G630A (P < 0.05). G630A mutation homozygotes had a higher AGT residual activity (P = 0.00001). This study confirms the allelic heterogeneity of the AGXT, which could to some extent be responsible for the phenotypic heterogeneity in PH1.  (+info)

Hyperoxaluria is a medical condition characterized by an excessive excretion of oxalate in the urine. Oxalate is a naturally occurring substance found in some foods and can also be produced by the body. When oxalate combines with calcium in the urine, it can form kidney stones or calcium oxalate deposits in the kidneys and other tissues, leading to kidney damage or systemic oxalosis. There are three types of hyperoxaluria: primary, secondary, and enteric. Primary hyperoxaluria is caused by genetic defects that affect the body's ability to regulate oxalate production, while secondary hyperoxaluria results from increased dietary intake or absorption of oxalate, or from other medical conditions. Enteric hyperoxaluria occurs in individuals with malabsorption syndromes, such as inflammatory bowel disease or after gastric bypass surgery, where excessive amounts of oxalate are absorbed from the gut into the bloodstream and excreted in the urine.

Primary hyperoxaluria is a rare genetic disorder characterized by overproduction of oxalate in the body due to mutations in specific enzymes involved in oxalate metabolism. There are three types of primary hyperoxaluria (PH1, PH2, and PH3), with PH1 being the most common and severe form.

In primary hyperoxaluria type 1 (PH1), there is a deficiency or dysfunction in the enzyme alanine-glyoxylate aminotransferase (AGT), which leads to an accumulation of glyoxylate. Glyoxylate is then converted to oxalate, resulting in increased oxalate production. Oxalate is a compound that naturally occurs in the body but is primarily excreted through the kidneys. When there is an overproduction of oxalate, it can lead to the formation of calcium oxalate crystals in various tissues, including the kidneys. This can cause recurrent kidney stones, nephrocalcinosis (calcium deposits in the kidneys), and eventually chronic kidney disease or end-stage renal failure.

Primary hyperoxaluria type 2 (PH2) is caused by a deficiency or dysfunction in the enzyme glyoxylate reductase/hydroxypyruvate reductase (GRHPR), leading to an accumulation of glyoxylate, which is subsequently converted to oxalate. PH2 has a milder clinical presentation compared to PH1.

Primary hyperoxaluria type 3 (PH3) is a rare form caused by mutations in the gene HOGA1, which encodes for 4-hydroxy-2-oxoglutarate aldolase. This enzyme deficiency results in an increase in glyoxylate and, subsequently, oxalate production.

Early diagnosis and management of primary hyperoxaluria are crucial to prevent or slow down the progression of kidney damage. Treatment options include increased fluid intake, medications to reduce stone formation (such as potassium citrate), and in some cases, liver-kidney transplantation.

Oxalates, also known as oxalic acid or oxalate salts, are organic compounds that contain the functional group called oxalate. Oxalates are naturally occurring substances found in various foods such as spinach, rhubarb, nuts, and seeds. They can also be produced by the body as a result of metabolism.

In the body, oxalates can bind with calcium and other minerals to form crystals, which can accumulate in various tissues and organs, including the kidneys. This can lead to the formation of kidney stones, which are a common health problem associated with high oxalate intake or increased oxalate production in the body.

It is important for individuals with a history of kidney stones or other kidney problems to monitor their oxalate intake and limit consumption of high-oxalate foods. Additionally, certain medical conditions such as hyperoxaluria, a rare genetic disorder that causes increased oxalate production in the body, may require medical treatment to reduce oxalate levels and prevent complications.

Calcium oxalate is a chemical compound with the formula CaC2O4. It is the most common type of stone found in kidneys, also known as kidney stones. Calcium oxalate forms when there is too much calcium or oxalate in the urine. This can occur due to various reasons such as dietary habits, dehydration, medical conditions like hyperparathyroidism, or genetic factors.

Calcium oxalate stones are hard and crystalline and can cause severe pain during urination or while passing through the urinary tract. They may also lead to other symptoms like blood in the urine, nausea, vomiting, or fever. Prevention strategies for calcium oxalate stones include staying hydrated, following a balanced diet, and taking prescribed medications to control the levels of calcium and oxalate in the body.

Nephrolithiasis is a medical term that refers to the presence of stones or calculi in the kidney. These stones can form anywhere in the urinary tract, including the kidneys, ureters, bladder, and urethra. Nephrolithiasis is also commonly known as kidney stones.

Kidney stones are hard deposits made up of minerals and salts that crystallize in the urine. They can vary in size from tiny sand-like particles to larger pebble or even golf ball-sized masses. Kidney stones can cause pain, bleeding, and infection if they block the flow of urine through the urinary tract.

The formation of kidney stones is often associated with a variety of factors such as dehydration, high levels of calcium, oxalate, or uric acid in the urine, family history, obesity, and certain medical conditions like gout or inflammatory bowel disease. Treatment for nephrolithiasis depends on the size and location of the stone, as well as the severity of symptoms. Small stones may pass spontaneously with increased fluid intake, while larger stones may require medication, shock wave lithotripsy, or surgical removal.

Transaminases, also known as aminotransferases, are a group of enzymes found in various tissues of the body, particularly in the liver, heart, muscle, and kidneys. They play a crucial role in the metabolism of amino acids, the building blocks of proteins.

There are two major types of transaminases: aspartate aminotransferase (AST) and alanine aminotransferase (ALT). Both enzymes are normally present in low concentrations in the bloodstream. However, when tissues that contain these enzymes are damaged or injured, such as during liver disease or muscle damage, the levels of AST and ALT in the blood may significantly increase.

Measurement of serum transaminase levels is a common laboratory test used to assess liver function and detect liver injury or damage. Increased levels of these enzymes in the blood can indicate conditions such as hepatitis, liver cirrhosis, drug-induced liver injury, heart attack, and muscle disorders. It's important to note that while elevated transaminase levels may suggest liver disease, they do not specify the type or cause of the condition, and further diagnostic tests are often required for accurate diagnosis and treatment.

Kidney calculi, also known as kidney stones, are hard deposits made of minerals and salts that form inside your kidneys. They can range in size from a grain of sand to a golf ball. When they're small enough, they can be passed through your urine without causing too much discomfort. However, larger stones may block the flow of urine, causing severe pain and potentially leading to serious complications such as urinary tract infections or kidney damage if left untreated.

The formation of kidney calculi is often associated with factors like dehydration, high levels of certain minerals in your urine, family history, obesity, and certain medical conditions such as gout or inflammatory bowel disease. Symptoms of kidney stones typically include severe pain in the back, side, lower abdomen, or groin; nausea and vomiting; fever and chills if an infection is present; and blood in the urine. Treatment options depend on the size and location of the stone but may include medications to help pass the stone, shock wave lithotripsy to break up the stone, or surgical removal of the stone in severe cases.

Nephrocalcinosis is a medical condition characterized by the deposition of calcium salts in the renal parenchyma, specifically within the tubular epithelial cells and interstitium of the kidneys. This process can lead to chronic inflammation, tissue damage, and ultimately impaired renal function if left untreated.

The condition is often associated with metabolic disorders such as hyperparathyroidism, distal renal tubular acidosis, or hyperoxaluria; medications like loop diuretics, corticosteroids, or calcineurin inhibitors; and chronic kidney diseases. The diagnosis of nephrocalcinosis is typically made through imaging studies such as ultrasound, CT scan, or X-ray. Treatment usually involves addressing the underlying cause, modifying dietary habits, and administering medications to control calcium levels in the body.

"Oxalobacter formigenes" is a type of gram-negative, anaerobic bacteria that resides in the human gastrointestinal tract. It is commonly found in the large intestine and plays a role in the metabolism of oxalate, a compound found in many foods that can contribute to kidney stone formation when present in high levels in the body.

"Oxalobacter formigenes" has the ability to break down and utilize oxalate as a source of energy, which can help to reduce the amount of oxalate that is absorbed into the bloodstream and excreted by the kidneys. Some research suggests that the presence of "Oxalobacter formigenes" in the gut may be associated with a lower risk of kidney stone formation, although more studies are needed to confirm this association.

It's worth noting that while "Oxalobacter formigenes" is considered a beneficial bacteria, it is not currently used as a probiotic or therapeutic agent in clinical practice.

Urolithiasis is the formation of stones (calculi) in the urinary system, which includes the kidneys, ureters, bladder, and urethra. These stones can be composed of various substances such as calcium oxalate, calcium phosphate, uric acid, or struvite. The presence of urolithiasis can cause symptoms like severe pain in the back or side, nausea, vomiting, fever, and blood in the urine. The condition can be managed with medications, increased fluid intake, and in some cases, surgical intervention may be required to remove the stones.

Oxalic acid is not a medical term, but it is a chemical compound with the formula HOOC-COOH. It is a white crystalline solid that is soluble in water and polar organic solvents. Medically, oxalic acid is relevant due to its presence in certain foods and its potential to form calcium oxalate stones in the kidneys when excreted in urine.

Hyperoxaluria is a medical condition characterized by increased levels of oxalate in the urine, which can lead to the formation of kidney stones. This condition can be caused by genetic factors or excessive intake of oxalate-rich foods such as spinach, rhubarb, and certain nuts and beans. In severe cases, it may require medical treatment to reduce oxalate levels in the body.

Hypercalciuria is a medical condition characterized by an excessive amount of calcium in the urine. It can occur when the body absorbs too much calcium from food, or when the bones release more calcium than usual. In some cases, it may be caused by certain medications, kidney disorders, or genetic factors.

Hypercalciuria can increase the risk of developing kidney stones and other kidney problems. It is often diagnosed through a 24-hour urine collection test that measures the amount of calcium in the urine. Treatment may include changes in diet, increased fluid intake, and medications to help reduce the amount of calcium in the urine.

Urinary calculi, also known as kidney stones or nephrolithiasis, are hard deposits made of minerals and salts that form inside the urinary system. These calculi can develop in any part of the urinary system, which includes the kidneys, ureters, bladder, and urethra.

The formation of urinary calculi typically occurs when there is a concentration of certain substances, such as calcium, oxalate, uric acid, or struvite, in the urine. When these substances become highly concentrated, they can crystallize and form small seeds that gradually grow into larger stones over time.

The size of urinary calculi can vary from tiny, sand-like particles to large stones that can fill the entire renal pelvis. The symptoms associated with urinary calculi depend on the stone's size, location, and whether it is causing a blockage in the urinary tract. Common symptoms include severe pain in the flank, lower abdomen, or groin; nausea and vomiting; blood in the urine (hematuria); fever and chills; and frequent urge to urinate or painful urination.

Treatment for urinary calculi depends on the size and location of the stone, as well as the severity of symptoms. Small stones may pass spontaneously with increased fluid intake and pain management. Larger stones may require medical intervention, such as extracorporeal shock wave lithotripsy (ESWL), ureteroscopy, or percutaneous nephrolithotomy (PCNL) to break up or remove the stone. Preventive measures include maintaining adequate hydration, modifying dietary habits, and taking medications to reduce the risk of stone formation.

Glycolates are a type of chemical compound that contain the group COOCH2, which is derived from glycolic acid. In a medical context, glycolates are often used in dental and medical materials as they can be biodegradable and biocompatible. For example, they may be used in controlled-release drug delivery systems or in bone cement. However, it's important to note that some glycolate compounds can also be toxic if ingested or otherwise introduced into the body in large amounts.

Ethylene glycol is a colorless, odorless, syrupy liquid with a sweet taste, which makes it appealing to animals and children. It is commonly used in the manufacture of antifreeze, coolants, deicers, hydraulic brake fluids, solvents, and other industrial products. Ethylene glycol is also found in some household items such as certain types of wood stains, paints, and cosmetics.

Ingesting even small amounts of ethylene glycol can be harmful or fatal to humans and animals. It is metabolized by the body into toxic substances that can cause damage to the central nervous system, heart, kidneys, and other organs. Symptoms of ethylene glycol poisoning may include nausea, vomiting, abdominal pain, decreased level of consciousness, seizures, coma, acidosis, increased heart rate, low blood pressure, and kidney failure.

If you suspect that someone has ingested ethylene glycol, it is important to seek medical attention immediately. Treatment typically involves administering a medication called fomepizole or ethanol to inhibit the metabolism of ethylene glycol, as well as providing supportive care such as fluid replacement and dialysis to remove the toxic substances from the body.

Glyoxylates are organic compounds that are intermediates in various metabolic pathways, including the glyoxylate cycle. The glyoxylate cycle is a modified version of the Krebs cycle (also known as the citric acid cycle) and is found in plants, bacteria, and some fungi.

Glyoxylates are formed from the breakdown of certain amino acids or from the oxidation of one-carbon units. They can be converted into glycine, an important amino acid involved in various metabolic processes. In the glyoxylate cycle, glyoxylates are combined with acetyl-CoA to form malate and succinate, which can then be used to synthesize glucose or other organic compounds.

Abnormal accumulation of glyoxylates in the body can lead to the formation of calcium oxalate crystals, which can cause kidney stones and other health problems. Certain genetic disorders, such as primary hyperoxaluria, can result in overproduction of glyoxylates and increased risk of kidney stone formation.

A jejunoileal bypass is a surgical procedure that was once used to treat morbid obesity, but it is now rarely performed due to the high risk of serious complications. This procedure involves dividing the small intestine into two parts: the proximal jejunum and the distal ileum. The proximal jejunum is then connected to the colon, bypassing a significant portion of the small intestine where nutrient absorption occurs.

The goal of this surgery was to reduce the amount of food and nutrients that could be absorbed, leading to weight loss. However, it was found that patients who underwent jejunoileal bypass were at risk for developing severe malnutrition, vitamin deficiencies, bone disease, kidney stones, and liver problems. Additionally, many patients experienced unpleasant side effects such as diarrhea, bloating, and foul-smelling stools. Due to these significant risks and limited benefits, jejunoileal bypass has largely been replaced by other weight loss surgeries such as gastric bypass and sleeve gastrectomy.

Steatorrhea is a medical condition characterized by the excessive amount of fat in stools, which can make them appear greasy, frothy, and foul-smelling. This occurs due to poor absorption of dietary fats in the intestines, a process called malabsorption. The most common causes of steatorrhea include conditions that affect the pancreas, such as cystic fibrosis or chronic pancreatitis, celiac disease, and other gastrointestinal disorders. Symptoms associated with steatorrhea may include abdominal pain, bloating, diarrhea, weight loss, and vitamin deficiencies due to malabsorption of fat-soluble vitamins (A, D, E, K). The diagnosis typically involves testing stool samples for fat content and further investigations to determine the underlying cause. Treatment is focused on addressing the underlying condition and providing dietary modifications to manage symptoms.

A cystostomy is a surgical procedure that creates an opening through the wall of the bladder to allow urine to drain out. This opening, or stoma, is usually connected to a external collection device, such as a bag or a tube. The purpose of a cystostomy is to provide a stable and reliable way for urine to leave the body when a person is unable to urinate naturally due to injury, illness, or other medical conditions that affect bladder function.

There are several types of cystostomies, including temporary and permanent procedures. A temporary cystostomy may be performed as a short-term solution while a patient recovers from surgery or an injury, or when a person is unable to urinate temporarily due to an obstruction in the urinary tract. In these cases, the cystostomy can be closed once the underlying issue has been resolved.

A permanent cystostomy may be recommended for individuals who have irreversible bladder damage or dysfunction, such as those with spinal cord injuries, neurological disorders, or certain types of cancer. In these cases, a cystostomy can help improve quality of life by allowing for regular and reliable urinary drainage, reducing the risk of complications like urinary tract infections and kidney damage.

It's important to note that a cystostomy is a significant surgical procedure that carries risks and potential complications, such as bleeding, infection, and injury to surrounding tissues. As with any surgery, it's essential to discuss the benefits and risks of a cystostomy with a healthcare provider to determine whether it's the right option for an individual's specific medical needs.

Lactobacillus brevis is a species of gram-positive, rod-shaped, facultatively anaerobic bacteria that belongs to the lactic acid bacteria group. It is commonly found in various environments such as plants, soil, and fermented foods like sauerkraut, pickles, and sourdough bread. Lactobacillus brevis is also part of the normal microbiota of the human gastrointestinal tract and vagina.

This bacterium is known for its ability to produce lactic acid as a metabolic end-product, which contributes to the preservation and fermentation of food. Lactobacillus brevis can also produce other compounds with potential health benefits, such as bacteriocins, which have antibacterial properties against certain pathogenic bacteria.

In some cases, Lactobacillus brevis has been investigated for its probiotic potential, although more research is needed to fully understand its effects on human health. It's important to note that while some strains of Lactobacillus brevis may have beneficial properties, others can cause infections in individuals with weakened immune systems or underlying medical conditions.

Hydroxypyruvate Reductase is an enzyme involved in the metabolism of carbohydrates. Specifically, it catalyzes the conversion of hydroxypyruvate to glycerate during the photorespiratory cycle in plants and some bacteria. This reaction is a part of the process that recovers carbon from the 2-phosphoglycolate generated by the oxygenase activity of ribulose-1,5-bisphosphate carboxylase/oxygenase (RuBisCO) during photosynthesis.

The enzyme Hydroxypyruvate Reductase belongs to the family of oxidoreductases, more specifically those acting on the CH-OH group of donor with NAD+ or NADP+ as acceptor. The systematic name of this enzyme class is hydroxypyruvate:NAD(P)+ 2-oxidoreductase. Other common names include D-glycerate dehydrogenase, serine glyoxalate transaminase, and L-serine transaminase.

Hydroxyproline is not a medical term per se, but it is a significant component in the medical field, particularly in the study of connective tissues and collagen. Here's a scientific definition:

Hydroxyproline is a modified amino acid that is formed by the post-translational modification of the amino acid proline in collagen and some other proteins. This process involves the addition of a hydroxyl group (-OH) to the proline residue, which alters its chemical properties and contributes to the stability and structure of collagen fibers. Collagen is the most abundant protein in the human body and is a crucial component of connective tissues such as tendons, ligaments, skin, and bones. The presence and quantity of hydroxyproline can serve as a marker for collagen turnover and degradation, making it relevant to various medical and research contexts, including the study of diseases affecting connective tissues like osteoarthritis, rheumatoid arthritis, and Ehlers-Danlos syndrome.

Potassium citrate is a medication and dietary supplement that contains potassium and citrate. Medically, it is used to treat and prevent kidney stones, as well as to manage metabolic acidosis in people with chronic kidney disease. Potassium citrate works by increasing the pH of urine, making it less acidic, which can help to dissolve certain types of kidney stones and prevent new ones from forming. It is also used as an alkalizing agent in the treatment of various conditions that cause acidosis.

In addition to its medical uses, potassium citrate is also found naturally in some fruits and vegetables, such as oranges, grapefruits, lemons, limes, and spinach. It is often used as a food additive and preservative, and can be found in a variety of processed foods and beverages.

It's important to note that taking too much potassium citrate can lead to high levels of potassium in the blood, which can be dangerous. Therefore, it is important to follow the dosage instructions carefully and talk to your doctor before taking this medication if you have any medical conditions or are taking any other medications.

Oxo-acid lyases are a class of enzymes that catalyze the cleavage of a carbon-carbon bond in an oxo-acid to give a molecule with a carbonyl group and a carbanion, which then reacts non-enzymatically with a proton to form a new double bond. The reaction is reversible, and the enzyme can also catalyze the reverse reaction.

Oxo-acid lyases play important roles in various metabolic pathways, such as the citric acid cycle, glyoxylate cycle, and the degradation of certain amino acids. These enzymes are characterized by the presence of a conserved catalytic mechanism involving a nucleophilic attack on the carbonyl carbon atom of the oxo-acid substrate.

The International Union of Biochemistry and Molecular Biology (IUBMB) has classified oxo-acid lyases under EC 4.1.3, which includes enzymes that catalyze the formation of a carbon-carbon bond by means other than carbon-carbon bond formation to an enolate or carbonion, a carbanionic fragment, or a Michael acceptor.

Citric acid is a weak organic acid that is widely found in nature, particularly in citrus fruits such as lemons and oranges. Its chemical formula is C6H8O7, and it exists in a form known as a tribasic acid, which means it can donate three protons in chemical reactions.

In the context of medical definitions, citric acid may be mentioned in relation to various physiological processes, such as its role in the Krebs cycle (also known as the citric acid cycle), which is a key metabolic pathway involved in energy production within cells. Additionally, citric acid may be used in certain medical treatments or therapies, such as in the form of citrate salts to help prevent the formation of kidney stones. It may also be used as a flavoring agent or preservative in various pharmaceutical preparations.

Chronic kidney failure, also known as chronic kidney disease (CKD) stage 5 or end-stage renal disease (ESRD), is a permanent loss of kidney function that occurs gradually over a period of months to years. It is defined as a glomerular filtration rate (GFR) of less than 15 ml/min, which means the kidneys are filtering waste and excess fluids at less than 15% of their normal capacity.

CKD can be caused by various underlying conditions such as diabetes, hypertension, glomerulonephritis, polycystic kidney disease, and recurrent kidney infections. Over time, the damage to the kidneys can lead to a buildup of waste products and fluids in the body, which can cause a range of symptoms including fatigue, weakness, shortness of breath, nausea, vomiting, and confusion.

Treatment for chronic kidney failure typically involves managing the underlying condition, making lifestyle changes such as following a healthy diet, and receiving supportive care such as dialysis or a kidney transplant to replace lost kidney function.

Crystallization is a process in which a substance transitions from a liquid or dissolved state to a solid state, forming a crystal lattice. In the medical context, crystallization can refer to the formation of crystals within the body, which can occur under certain conditions such as changes in pH, temperature, or concentration of solutes. These crystals can deposit in various tissues and organs, leading to the formation of crystal-induced diseases or disorders.

For example, in patients with gout, uric acid crystals can accumulate in joints, causing inflammation, pain, and swelling. Similarly, in nephrolithiasis (kidney stones), minerals in the urine can crystallize and form stones that can obstruct the urinary tract. Crystallization can also occur in other medical contexts, such as in the formation of dental calculus or plaque, and in the development of cataracts in the eye.

Kidney transplantation is a surgical procedure where a healthy kidney from a deceased or living donor is implanted into a patient with end-stage renal disease (ESRD) or permanent kidney failure. The new kidney takes over the functions of filtering waste and excess fluids from the blood, producing urine, and maintaining the body's electrolyte balance.

The transplanted kidney is typically placed in the lower abdomen, with its blood vessels connected to the recipient's iliac artery and vein. The ureter of the new kidney is then attached to the recipient's bladder to ensure proper urine flow. Following the surgery, the patient will require lifelong immunosuppressive therapy to prevent rejection of the transplanted organ by their immune system.

Alcohol oxidoreductases are a class of enzymes that catalyze the oxidation of alcohols to aldehydes or ketones, while reducing nicotinamide adenine dinucleotide (NAD+) to NADH. These enzymes play an important role in the metabolism of alcohols and other organic compounds in living organisms.

The most well-known example of an alcohol oxidoreductase is alcohol dehydrogenase (ADH), which is responsible for the oxidation of ethanol to acetaldehyde in the liver during the metabolism of alcoholic beverages. Other examples include aldehyde dehydrogenases (ALDH) and sorbitol dehydrogenase (SDH).

These enzymes are important targets for the development of drugs used to treat alcohol use disorder, as inhibiting their activity can help to reduce the rate of ethanol metabolism and the severity of its effects on the body.

Microbodies are small, membrane-bound organelles found in the cells of eukaryotic organisms. They typically measure between 0.2 to 0.5 micrometers in diameter and play a crucial role in various metabolic processes, particularly in the detoxification of harmful substances and the synthesis of lipids.

There are several types of microbodies, including:

1. Peroxisomes: These are the most common type of microbody. They contain enzymes that help break down fatty acids and amino acids, producing hydrogen peroxide as a byproduct. Another set of enzymes within peroxisomes then converts the harmful hydrogen peroxide into water and oxygen, thus detoxifying the cell.
2. Glyoxysomes: These microbodies are primarily found in plants and some fungi. They contain enzymes involved in the glyoxylate cycle, a metabolic pathway that helps convert stored fats into carbohydrates during germination.
3. Microbody-like particles (MLPs): These are smaller organelles found in certain protists and algae. Their functions are not well understood but are believed to be involved in lipid metabolism.

It is important to note that microbodies do not have a uniform structure or function across all eukaryotic cells, and their specific roles can vary depending on the organism and cell type.

NIH/UW entry on Primary Hyperoxaluria Type 1 GeneReview/NCBI/NIH/UW entry on Primary Hyperoxaluria Type 2 Primary hyperoxaluria ... Peroxisomal disorder "LEARN Oxalosis & Hyperoxaluria , Oxalosis & Hyperoxaluria Foundation". www.ohf.org. "Primary ... Oxalate stones in primary hyperoxaluria tend to be severe, resulting in relatively early kidney damage (in teenage years to ... Primary hyperoxaluria is a rare condition (autosomal recessive), resulting in increased excretion of oxalate (up to 600 mg a ...
Primary hyperoxaluria Enteric hyperoxaluria Idiopathic hyperoxaluria Oxalate poisoning The main therapeutic approach to primary ... Hyperoxaluria can be primary (as a result of a genetic defect) or secondary to another disease process. Type I primary ... Secondary hyperoxaluria is much more common than primary hyperoxaluria, and should be treated by limiting dietary oxalate and ... A child with primary hyperoxaluria was treated with a liver and kidney transplant. A favorable outcome is more likely if a ...
Primary Hyperoxaluria type 2 is caused by any one of several mutations to the GRHPR gene and results in the accumulation of ... Primary hyperoxaluria is a condition that results in the overproduction of oxalate which combines with calcium to generate ... "Primary hyperoxaluria". Retrieved 4 March 2013. Holzer H, Holldorf A (1957). "[Isolation of D-glycerate dehydrogenase, some ... Rumsby, G; Pagon, RA; Bird, TD; Dolan, CR; Stephens, K; Adam, MP (1993). "Primary Hyperoxaluria Type 2". PMID 20301742. {{cite ...
260000 HYPEROXALURIA, PRIMARY, TYPE II; HP2". OMIM. Retrieved 2023-10-05. "Primary Hyperoxaluria Type 2". GeneReviews®. ... D-Glyceric acidemia should not be confused with L-Glyceric acidemia (a.k.a. L-glyceric aciduria, a.k.a. primary hyperoxaluria ...
Nishiyama K, Funai T, Katafuchi R, Hattori F, Onoyama K, Ichiyama A (Jul 1991). "Primary hyperoxaluria type I due to a point ... von Schnakenburg C, Rumsby G (1997). "Primary hyperoxaluria type 1: a cluster of new mutations in exon 7 of the AGXT gene". J. ... Disease: primary hyperoxaluria type I". Hum. Genet. 104 (5): 441. doi:10.1007/s004390050984. PMID 10394939. S2CID 34307977. ... Danpure CJ (1993). "Primary hyperoxaluria type 1 and peroxisome-to-mitochondrion mistargeting of alanine:glyoxylate ...
Danpure CJ (2005). "Primary hyperoxaluria: From gene defects to designer drugs?". Nephrology Dialysis Transplantation. 20 (8): ...
Bogle MA, Teller CF, Tschen JA, Smith CA, Wang A (October 2003). "Primary hyperoxaluria in a 27-year-old woman". J. Am. Acad. ... Marconi V, Mofid MZ, McCall C, Eckman I, Nousari HC (February 2002). "Primary hyperoxaluria: report of a patient with livedo ... cutaneous manifestations of primary hyperoxaluria". Arch Dermatol. 136 (10): 1272-4. doi:10.1001/archderm.136.10.1272-a. PMID ... Primary hyperoxaluria, oxalosis (oxalate vasculopathy) Cytomegalovirus infection (very rare clinical form, presenting with ...
"Mutations in DHDPSL are responsible for primary hyperoxaluria type III". American Journal of Human Genetics. 87 (3): 392-9. doi ... a condition known as primary hyperoxaluria type III. GRCh38: Ensembl release 89: ENSG00000241935 - Ensembl, May 2017 GRCm38: ...
Danpure CJ (August 2005). "Primary hyperoxaluria: from gene defects to designer drugs?". Nephrology, Dialysis, Transplantation ...
This medication is used for the treatment of primary hyperoxaluria type 1 (PH1) in pediatric and adult populations. The drug is ... primary hyperoxaluria (PH) and hereditary ATTR amyloidosis as well as other cardiometabolic diseases such as hypertension and ... an RNAi Therapeutic for Primary Hyperoxaluria Type 1". New England Journal of Medicine. 384 (13): 1216-1226. doi:10.1056/ ... The primary transcripts from such genes are first processed to form the characteristic stem-loop structure of pre-miRNA in the ...
... is indicated for the treatment of primary hyperoxaluria type 1 (PH1) in adults and children of all ages. PH1 is a ... Lumasiran, sold under the brand name Oxlumo, is a medication for the treatment of primary hyperoxaluria type 1 (PH1). The most ... Clinical trial number NCT03681184 for "A Study to Evaluate Lumasiran in Children and Adults With Primary Hyperoxaluria Type 1 ( ... D'Ambrosio V, Ferraro PM (2022). "Lumasiran in the Management of Patients with Primary Hyperoxaluria Type 1: From Bench to ...
Belostotsky, Ruth; Pitt, James Jonathon; Frishberg, Yaacov (2012-12-01). "Primary hyperoxaluria type III-a model for studying ... Glyoxylate is involved in the development of hyperoxaluria, a key cause of nephrolithiasis (commonly known as kidney stones). ... These glyoxylate molecules can be oxidized into oxalate increasing its concentration and causing hyperoxaluria. Glyoxylic acid ... The disruption of glyoxylate metabolism provides an additional mechanism of hyperoxaluria development. Loss of function ...
"A Study to Evaluate DCR-PHXC in Children and Adults With Primary Hyperoxaluria Type 1 and Primary Hyperoxaluria Type 2 (PHYOX2 ... Nedosiran, sold under the brand name Rivfloza, is a medication used for the treatment of primary hyperoxaluria. It is an LDHA- ... Nedosiran is indicated to lower urinary oxalate levels in people with primary hyperoxaluria type 1. "Rivfloza (nedosiran) ... November 2022). "Nedosiran, a Candidate siRNA Drug for the Treatment of Primary Hyperoxaluria: Design, Development, and ...
Factors that promote the precipitation of oxalate crystals in the urine, such as primary hyperoxaluria, are associated with the ... Hoppe B, Langman CB (October 2003). "A United States survey on diagnosis, treatment, and outcome of primary hyperoxaluria". ... primary hyperoxaluria, or medullary sponge kidney. 3-20% of people who form kidney stones have medullary sponge kidney. Kidney ... Primary hyperoxaluria is a rare autosomal recessive condition that usually presents in childhood. Calcium oxalate crystals can ...
The reduced enzyme function can be caused by a rare inherited autosomal recessive disorder known as primary hyperoxaluria type ... is mutated in patients with primary hyperoxaluria type II". Hum. Mol. Genet. 8 (11): 2063-9. doi:10.1093/hmg/8.11.2063. PMID ... "Novel mutation in the GRHPR gene in a Chinese patient with primary hyperoxaluria type 2 requiring renal transplantation from a ... gene and description of mutations underlying primary hyperoxaluria type 2". Hum. Mutat. 22 (6): 497. doi:10.1002/humu.9200. ...
GeneReviews/NCBI/NIH/UW entry on Primary Hyperoxaluria Type 2 Takayama T, Nagata M, Ozono S, et al. (2007). "A novel mutation ... is mutated in patients with primary hyperoxaluria type II". Hum Mol Genet. 8 (11): 2063-9. doi:10.1093/hmg/8.11.2063. PMID ... and deletion mutations in the GRHPR gene in patients with primary hyperoxaluria type II (PH2)". Hum. Genet. 107 (2): 176-85. ... Type II hyperoxaluria is caused by mutations in this gene. GRHPR mutation analysis needs to pay attention to primer design, ...
Primary hyperoxaluria is a rare, inherited condition, resulting in increased excretion of oxalate, with oxalate stones being ... An excess oxalate level in the blood is termed hyperoxalemia, and high levels of oxalate in the urine is termed hyperoxaluria. ... Many metal ions form insoluble precipitates with oxalate, a prominent example being calcium oxalate, the primary constituent of ...
Gruessner, Rainer W. (1998). "Preemptive Liver Transplantation from a Living Related Donor for Primary Hyperoxaluria Type I". ...
... however if not treated it can lead to renal dysfunction this includes primary hyperoxaluria, hypomagnesemic hypercalciuric ... Suh, Jane M.; Cronan, John J.; Monchik, Jack M. (September 2008). "Primary hyperparathyroidism: is there an increased ... Medullary sponge kidney Distal renal tubular acidosis Hyperoxaluria Renal papillary necrosis And other causes of hypercalcaemia ... These include tumor lysis syndrome, acute phosphate nephropathy, and occasional cases of enteric hyperoxaluria. ...
... type 6 Mitochondrial trifunctional protein deficiency Nonsyndromic deafness Photic sneeze reflex Primary hyperoxaluria Primary ... hyperoxaluria I; glycolicaciduria; serine-pyruvate aminotransferase) ALS2: amyotrophic lateral sclerosis 2 (juvenile) ALS2CR8: ... type 4 Hereditary nonpolyposis colorectal cancer Infantile-onset ascending hereditary spastic paralysis Juvenile primary ...
... nonsyndromic deafness OCD polycythemia vera porphyria primary hyperoxaluria Tangier's disease tetrasomy 9p thrombotic ...
... hyperoxaluria, primary MeSH C12.777.419.331 - hypertension, renal MeSH C12.777.419.331.490 - hypertension, renovascular MeSH ...
Some of these diseases include: · Hepatitis A, Hepatitis B, and Hepatitis C · Primary biliary cholangitis and Primary ... sclerosing cholangitis · Liver cancer, Bile duct cancer, and Liver cell adenoma · Hemochromatosis, Hyperoxaluria, and Wilson's ...
... hyperoxaluria, primary MeSH C18.452.565.100 - anemia, iron-deficiency MeSH C18.452.565.500 - iron overload MeSH C18.452.565.500 ... hyperoxaluria, primary MeSH C18.452.648.202.589 - lactose intolerance MeSH C18.452.648.202.607 - mannosidase deficiency ...
... hyperoxaluria, primary MeSH C16.320.565.202.589 - lactose intolerance MeSH C16.320.565.202.607 - mannosidase deficiency ...
Infiltrative Amyloidosis Sarcoidosis Primary hyperoxaluria Storage diseases Fabry disease Gaucher disease Hereditary ... Thus it is possible to divide the causes into primary and secondary. The common modern organization is into Infiltrative, ...
Hyperostosis corticalis generalisata Hyperostosis frontalis interna Hyperoxaluria type 1 Hyperoxaluria type 2 Hyperoxaluria ... Hyperparathyroidism Hyperparathyroidism, familial, primary Hyperparathyroidism, neonatal severe primary Hyperphalangism ... primary or idiopathic Hypertropia Hypertropic neuropathy of Dejerine-Sottas Hypertryptophanemia Hypervitaminosis A ... male mental retardation skeletal anomaly Hypogonadism mitral valve prolapse mental retardation Hypogonadism primary partial ...
... a candidate to treat primary hyperoxaluria type 1. Precision is in the process of developing multiple candidates targeting non- ...
... sarcoma Primary hyperoxaluria Primary hyperparathyroidism Primary lateral sclerosis Primary malignant lymphoma Primary muscular ... Griscelli syndrome Primary agammaglobulinemia Primary aldosteronism Primary alveolar hypoventilation Primary amenorrhea Primary ... Primary orthostatic tremor Primary progressive aphasia Primary pulmonary hypertension Primary sclerosing cholangitis Primary ... 2 Primary ciliary dyskinesia Primary craniosynostosis Primary cutaneous amyloidosis Primary granulocytic ...
Although the association is greater in the context of ulcerative colitis, Crohn's disease may also be associated with primary ... Calcium oxalate is due to hyperoxaluria typically associated with either distal ileal CD or ileal resection. Oxalate absorption ... "Primary sclerosing cholangitis, inflammatory bowel disease, and colon cancer". Seminars in Liver Disease. 26 (1): 31-41. doi: ...
NIH/UW entry on Primary Hyperoxaluria Type 1 GeneReview/NCBI/NIH/UW entry on Primary Hyperoxaluria Type 2 Primary hyperoxaluria ... Peroxisomal disorder "LEARN Oxalosis & Hyperoxaluria , Oxalosis & Hyperoxaluria Foundation". www.ohf.org. "Primary ... Oxalate stones in primary hyperoxaluria tend to be severe, resulting in relatively early kidney damage (in teenage years to ... Primary hyperoxaluria is a rare condition (autosomal recessive), resulting in increased excretion of oxalate (up to 600 mg a ...
Primary hyperoxaluria type 1 is a rare hereditary condition characterized by excessive oxalate formation. Learn how PH1 affects ... Primary Hyperoxaluria Type 1: https://www.kidney.org/atoz/primary-hyperoxaluria. Primary hyperoxaluria: https://radiopaedia.org ... Primary hyperoxaluria type 1: https://rarediseases.info.nih.gov/diseases/2835/primary-hyperoxaluria-type-1/. Primary ... There are two types of hyperoxaluria: primary and secondary. Hyperoxaluria is a condition in which the urine contains an ...
Background/Aims: Primary hyperoxaluria (PH) is an inherited disorder that causes calcium urolithiasis and renal failure. Due to ... Cochat P, Deloraine A, Rotily M, Olive F, Liponski I, Deries N: Epidemiology of primary hyperoxaluria type 1. Société de ... Monico CG, Milliner DS, Wilson DM, Rumsby G: Atypical primary hyperoxaluria: PH type III? Nephrol Dial Transplant 1999;14:2784- ... Hoppe B, Langman CB: A United States survey on diagnosis, treatment, and outcome of primary hyperoxaluria. Pediatr Nephrol 2003 ...
OxThera reports results from Phase 3 ePHex study with Oxabact in primary hyperoxaluria patients with maintained kidney function ... OxThera AB is a Swedish biopharmaceutical company developing a new treatment for primary hyperoxaluria (PH) - a rare genetic ... OxThera reports results from Phase 3 ePHex study with Oxabact in primary hyperoxaluria patients with maintained kidney function ... a privately-held biopharmaceutical company dedicated to improving the lives of people with Primary Hyperoxaluria (PH), ...
Primary hyperoxaluria is caused by pathogenic (disease-causing) variants in the AGXT (type 1), GRHPR (type 2), or HOGA1 (type 3 ... Primary hyperoxaluria is a rare condition where people get kidney and bladder stones over and over again. This can lead to a ... There are three types of primary hyperoxaluria, and theyre different in how severe they are and what causes them. In type 1, ... The main issue in primary hyperoxaluria is that the body makes too much of something called oxalate. This oxalate goes through ...
Plasma and urinary amino acids in children with primary hyperoxaluria and in normal children. ... Plasma and urinary amino acids in children with primary hyperoxaluria and in normal children. ...
The 4 main types of hyperoxaluria-primary hyperoxaluria, enteric hyperoxaluria, dietary hyperoxaluria, and idiopathic or mild ... Primary hyperoxaluria. Primary hyperoxaluria is a group of rare disorders due exclusively to genetic defects that cause a loss ... Treatment of Primary Hyperoxaluria. Patients with primary hyperoxaluria usually present with a urinary oxalate level in excess ... In primary hyperoxaluria, urinary oxalate excretion is typically more than 100 mg/d. Primary hyperoxaluria may result in kidney ...
Primary hyperoxaluria. More than 175 mutations in the AGXT gene have been found to cause primary hyperoxaluria type 1. This ... Primary Hyperoxaluria Type 1. 2002 Jun 19 [updated 2022 Feb 10]. In: Adam MP, Feldman J, Mirzaa GM, Pagon RA, Wallace SE, Bean ... Cochat P, Rumsby G. Primary hyperoxaluria. N Engl J Med. 2013 Aug 15;369(7):649-58. doi: 10.1056/NEJMra1301564. No abstract ... Phenotype-Genotype Correlations and Estimated Carrier Frequencies of Primary Hyperoxaluria. J Am Soc Nephrol. 2015 Oct;26(10): ...
Primary Hyperoxaluria Precision Panel Primary Hyperoxaluria (PH) is a group of inherited metabolic diseases of the liver ... The Igenomix Primary Hyperoxaluria Syndrome Precision Panel is indicated for those patients with a clinical suspicion or ... Primary Hyperoxaluria (PH) is a group of inherited metabolic diseases of the liver characterized by increased formation of ... The Igenomix Primary Hyperoxaluria Precision Panel can be used to make a directed and accurate diagnosis and aid in the ...
Oxlumo 2021 U.S. PROMOTIONAL AUDIT REPORT Published July 2022 • 24 Pages The 5 Key Questions Addressed by this Report:... ...
... Dec 21 ... Here we evaluate the therapeutic efficacy of an in vivo CRISPR/Cas9-mediated SRT to treat primary hyperoxaluria type I (PH1), a ...
Combined liver/kidney transplant is the preferred transplant option for most patients with primary hyperoxaluria type 1 (PH1) ... Combined Liver-Kidney Transplantation for Primary Hyperoxaluria Type 2: A Case Report. E. C. Lorenz, J. C. Lieske, D. S. ... However, primary hyperoxaluria type 2 (PH2) is caused by deficient glyoxylate reductase/hydroxypyruvate reductase (GRHPR), and ... Combined liver/kidney transplant is the preferred transplant option for most patients with primary hyperoxaluria type 1 (PH1) ...
Find additional resources about primary hyperoxaluria type 1 (PH1) and hear from people about their experiences living with ... Support for the primary hyperoxaluria type 1 (PH1) community, gathered into one place. Dont miss our caregivers resources ... Having a difficult time explaining primary hyperoxaluria type 1 (PH1) to your child? Watch this animated video series, ... developed by Alnylam in partnership with The Oxalosis and Hyperoxaluria Foundation (OHF), starring kids from around the world ...
Vitamin B6 in primary hyperoxaluria I: first prospective trial after 40 years of practice. Clinical Journal of the American ... Lumasiran, an RNAi therapeutic for primary hyperoxaluria type 1. The New England Journal of Medicine, 385(20): e69. doi: ... Multiplex gene editing reduces oxalate production in primary hyperoxaluria type 1. doi: 10.24272/j.issn.2095-8137.2022.495 * ... Molecular basis of primary hyperoxaluria: clues to innovative treatments. Urolithiasis, 47(1): 67−78. doi: 10.1007/s00240-018- ...
Primary Hyperoxaluria. Niranjan Khandelwal, Kushaljit Singh Sodhi, Vivek Virmani, Navneet Sharma An 18-year-old male presented ... Improving the Uptake of Human Immunodeficiency Virus (HIV) Antenatal Screening in a Primary Healthcare Setting. E G Tay, S C ... Immunoglobulin (Ig) A nephropathy is the most common primary glomerulonephritis in various parts of the world, and it was ... IgA nephropathy (IgAN) is the most common primary glomerulonephritis in Singapore1 and in many parts of the world, contributing ...
Primary Hyperoxaluria Type 1. Primary hyperoxaluria type 1 (PH1) is an ultra-rare genetic disease that leads to frequent kidney ...
Primary Hyperoxaluria Type 2 and our test PH2 is a rare genetic disorder. It is characterized by frequent kidney stones that ... Primary Hyperoxaluria Type 2 Pyruvate Kinase Deficiency Rhizomelic Chondrodysplasia Punctata Type 1 Salla Disease Sickle Cell ...
... an experimental once-monthly RNAi treatment for primary hyperoxaluria, which met both primary and secondary endpoints. ... Primary hyperoxaluria (PH) is a family of ultra-rare, life-threatening genetic disorders that initially manifest with ... Dicerna Reports Positive Results from Pivotal Trial of Experimental RNAi Therapy for Primary Hyperoxaluria. August 6, 2021 ... Still, the primary endpoint of the study, percent change from baseline in 24-hour urinary oxalate excretion as assessed by area ...
Primary hyperoxaluria: MedlinePlus Genetics (National Library of Medicine) * Prolidase deficiency: MedlinePlus Genetics ( ...
9 Years and Adults with Primary Hyperoxaluria Type 1. ... Novo Nordisk Rivfloza Primary Hyperoxaluria Type 1 Regulatory ... Novo Nordisk Rivfloza (nedosiran) Receives the US FDAs Approval for Children Aged ≥9 Years and Adults with Primary ...
Cutaneous Oxalosis Due to Primary Hyperoxaluria. Am J Dermatopathol 2022;44:981-983.. PMID: 36395453 ...
Management of Primary Hyperoxaluria Type 1 in Italy]. Ferraro PM, Gambaro G, Mandrile G, Montini G, Pasini A, Peruzzi L. ... Genetic assessment in primary hyperoxaluria: why it matters. Mandrile G, Beck B, Acquaviva C, Rumsby G, Deesker L, Garrelfs S, ... Primary hyperoxaluria Type 1: indications for screening and guidance for diagnosis and treatment. Cochat P, Hulton SA, ... Clinical practice recommendations for primary hyperoxaluria: an expert consensus statement from ERKNet and OxalEurope. ...
... primary hyperoxaluria and cystinuria, in patients attending kidney stone clinics is ∼15%. However, for the majority of ... Primary hyperoxaluria type 1: indications for screening and guidance for diagnosis and treatment. Nephrol. Dial. Transpl. 27, ... Phenotype-genotype correlations and estimated carrier frequencies of primary hyperoxaluria. J. Am. Soc. Nephrol. 26, 2559-2570 ... Primary hyperoxaluria type III gene HOGA1 (formerly DHDPSL) as a possible risk factor for idiopathic calcium oxalate ...
Primary hyperoxaluria Donald R. Steinmuller, M.D.. Cleveland Clinic Journal of Medicine March 1985, 52 (1) 27-30; ...
Novo Nordisk gains FDA approval for primary hyperoxaluria therapy News Basilea pre-emptively seeks US partner for ceftobiprole ...
Primary Hyperoxaluria *Tubulointerstitial Nephritis. Systemic Disease. *Renal Vasculitis *Henoch-Schonlein Purpura *Systemic ...
Download and share our brochure for healthcare professionals on Alnylam Act® for primary hyperoxaluria type 1. ... Download and share our brochure for patients on Alnylam Act® for primary hyperoxaluria type 1. ... or primary hyperoxaluria type 1.. The Alnylam Act® program was developed to reduce barriers to genetic testing and counseling, ...
  • A single kidney stone in children or recurrent stones in adults is often the first warning sign of primary hyperoxaluria. (wikipedia.org)
  • Here we evaluate the therapeutic efficacy of an in vivo CRISPR/Cas9-mediated SRT to treat primary hyperoxaluria type I (PH1), a rare inborn dysfunction in glyoxylate metabolism that results in excessive hepatic oxalate production causing end-stage renal disease. (cun.es)
  • Combined liver/kidney transplant is the preferred transplant option for most patients with primary hyperoxaluria type 1 (PH1) since orthotopic liver transplantation replaces the deficient liver-specific AGT enzyme, thus restoring normal metabolic oxalate production. (researcher-app.com)
  • Increased oxalate levels in the blood can lead to systemic oxalosis, particularly affecting bones and the walls of blood vessels in people with primary hyperoxaluria type 1. (medlineplus.gov)
  • Watch this animated video series, developed by Alnylam in partnership with The Oxalosis and Hyperoxaluria Foundation (OHF) , starring kids from around the world who have been diagnosed with PH1. (livingwithph1.ca)
  • Cutaneous Oxalosis Due to Primary Hyperoxaluria. (amedeo.com)
  • Lumasiran, an RNA interference therapeutic drug, is indicated for the treatment of primary hyperoxaluria type 1 (PH1) in adults and children of all ages and is available under the UK Early Access to Medicines Scheme (EAMS). (wikipedia.org)
  • What Is Primary Hyperoxaluria Type 1 (PH1)? (medicinenet.com)
  • While kidney stones are the most common sign of primary hyperoxaluria type 1 (PH1), they do not occur in all patients. (medicinenet.com)
  • Primary hyperoxaluria type 1 (PH1) is an extremely rare hereditary condition that is characterized by excessive oxalate formation -a waste product usually removed by the kidneys and discharged in the urine. (medicinenet.com)
  • What are the symptoms of primary hyperoxaluria type 1 (PH1)? (medicinenet.com)
  • Primary hyperoxaluria is caused by pathogenic (disease-causing) variants in the AGXT (type 1), GRHPR (type 2), or HOGA1 (type 3) genes and exhibits autosomal recessive inheritance . (jewishgenetics.org)
  • More than 175 mutations in the AGXT gene have been found to cause primary hyperoxaluria type 1. (medlineplus.gov)
  • Primary Hyperoxaluria Type 1. (medlineplus.gov)
  • However, primary hyperoxaluria type 2 (PH2) is caused by deficient glyoxylate reductase/hydroxypyruvate reductase (GRHPR), and this enzyme is widely distributed throughout the body. (researcher-app.com)
  • Support for the primary hyperoxaluria type 1 (PH1) community, gathered into one place. (livingwithph1.ca)
  • Having a difficult time explaining primary hyperoxaluria type 1 (PH1) to your child? (livingwithph1.ca)
  • Targeting key enzymes that generate oxalate precursors or substrates is an alternative strategy to eliminate primary hyperoxaluria type I (PH1), the most common and life-threatening type of primary hyperoxaluria. (zoores.ac.cn)
  • Primary hyperoxaluria Type 1: indications for screening and guidance for diagnosis and treatment. (nih.gov)
  • Alnylam Act® is a sponsored, no-charge, third-party genetic testing and counseling program for patients with a family history or suspected diagnosis of hereditary ATTR (hATTR) amyloidosis, acute hepatic porphyria, or primary hyperoxaluria type 1. (alnylam.com)
  • Download and share our brochure for patients on Alnylam Act ® for primary hyperoxaluria type 1 . (alnylam.com)
  • The ILLUMINATE-A trial of lumasiran in patients with primary hyperoxaluria type 1 supported the FDA approval of this drug. (urologytimes.com)
  • What type of diet may be appropriate in hyperoxaluria? (betterhealthguy.com)
  • Dicerna Pharmaceuticals will license Tekmira's lipid nanoparticle (LNP) technology for delivery of DCR-PH1, Dicerna's investigational drug for the rare liver disorder primary hyperoxaluria type 1 (PH1), Dicerna said today. (genengnews.com)
  • Primary hyperoxaluria is a rare condition (autosomal recessive), resulting in increased excretion of oxalate (up to 600 mg a day from normal 50 mg a day), with oxalate stones being common. (wikipedia.org)
  • The buildup of oxalate in the body causes increased renal excretion of oxalate (hyperoxaluria), which in turn results in kidney and bladder stones. (wikipedia.org)
  • Oxalate stones in primary hyperoxaluria tend to be severe, resulting in relatively early kidney damage (in teenage years to early adulthood), which impairs the excretion of oxalate leading to a further acceleration in accumulation of oxalate in the body. (wikipedia.org)
  • Hyperoxaluria-that is, elevated urinary excretion of the metabolic end product oxalate-can contribute to kidney stone formation and other health problems. (medscape.com)
  • Reflecting these normal values, the usual definition of hyperoxaluria is urinary oxalate excretion that exceeds 40 mg/day. (medscape.com)
  • Nedosiran achieved the primary endpoint in the PHYOX2 trial, demonstrating a statistically significant reduction from baseline in urinary oxalate (Uox) excretion compared to placebo. (globalgenes.org)
  • Still, the primary endpoint of the study, percent change from baseline in 24-hour urinary oxalate excretion as assessed by area under the curve (AUC) from Day 90 to Day 180, was met, with nedosiran resulting in a statistically significant reduction in Uox of a 57.5 percent greater daily average reduction over Day 90 to Day 180 compared to placebo. (globalgenes.org)
  • Exclusion criteria included age below 18 years at the time of diagnosis, presentation with a diagnosis and or treatment of recurrent disease within 5 years of primary and second primary cancers, and initial referral for diagnosis or palliative treatment of advanced unresectable/metastatic cancer. (ajmc.com)
  • metastatic cancer, and primary hyperoxaluria. (msdmanuals.com)
  • Primary hyperoxaluria is caused by genetic defects that result in the overproduction of oxalate. (wikipedia.org)
  • Dicerna Pharmaceuticals reported positive top-line results from the PHYOX2 pivotal clinical trial of nedosiran, an experimental once-monthly RNAi treatment for primary hyperoxaluria, which met both primary and secondary endpoints. (globalgenes.org)
  • This is different from secondary hyperoxaluria, which is caused by the increase in dietary and intestinal absorption of oxalate or excessive intake of oxalate precursors. (wikipedia.org)
  • In these patients, stone analysis and urine analysis are recommended to rule out secondary causes of hyperoxaluria. (wikipedia.org)
  • There are two types of hyperoxaluria: primary and secondary. (medicinenet.com)
  • Primary hyperoxaluria is an autosomal recessive disease, meaning both copies of the gene contain the mutation. (wikipedia.org)
  • OxThera AB is a Swedish biopharmaceutical company developing a new treatment for primary hyperoxaluria (PH) - a rare genetic and devastating disease with fatal outcomes. (biospace.com)
  • Primary Hyperoxaluria (PH) is a group of inherited metabolic diseases of the liver characterized by increased formation of calcium-oxalate stones in kidneys with the subsequent development of nephrolithiasis and chronic kidney disease. (igenomix.it)
  • The Igenomix Primary Hyperoxaluria Precision Panel can be used to make a directed and accurate diagnosis and aid in the differential diagnosis of recurrent kidney stones ultimately leading to a better management and prognosis of the disease. (igenomix.it)
  • These included one patient with proven primary hyperoxaluria, one suspected of having this disease and 11 patients in whom no information was available as to their oxalate metabolism. (portlandpress.com)
  • The RNAi drug nedosiran is a once-monthly subcutaneous injection under investigation for all 3 types of primary hyperoxaluria. (urologytimes.com)
  • Primary hyperoxaluria is a rare condition where people get kidney and bladder stones over and over again. (jewishgenetics.org)
  • Primary hyperoxaluria (PH) is an inherited disorder that causes calcium urolithiasis and renal failure. (karger.com)
  • The three main types of primary hyperoxaluria (PH1, PH2, and PH3) are each associated with mutations in specific genes involved in the metabolism of glyoxylate, the precursor of oxalate. (wikipedia.org)
  • Of the remaining 11 patients, one was shown to exhibit a transient hyperoxaluria, but the others showed a normal oxalate metabolism. (portlandpress.com)
  • A definitive diagnosis of primary hyperoxaluria requires genetic testing. (wikipedia.org)
  • Primary hyperoxaluria (PH) is a family of ultra-rare, life-threatening genetic disorders that initially manifest with complications in the kidneys. (globalgenes.org)
  • Clinical practice recommendations for primary hyperoxaluria: an expert consensus statement from ERKNet and OxalEurope. (nih.gov)
  • Hyperoxaluria is a condition in which the urine contains an excessive amount of oxalate. (medicinenet.com)
  • This oxalate goes through the kidneys and comes out in urine, making the urine have too much oxalate (hyperoxaluria). (jewishgenetics.org)
  • 1. In order to find out whether hyperoxaluria can be demonstrated in patients on chronic (twice a week) haemodialysis, a group of 13 patients was investigated. (portlandpress.com)
  • As a physician treating patients with PH, it is of primary importance to me that my patients are stable. (biospace.com)
  • Management of patients with hyperoxaluria. (igenomix.it)
  • 5. Hyperoxaluria can still be demonstrated in patients, who because of renal failure are subjected to haemodialysis. (portlandpress.com)
  • About 1 to 2% of patients with calcium calculi have primary hyperparathyroidism. (msdmanuals.com)
  • Treatment depends to some extent on the underlying etiology and severity of the hyperoxaluria. (medscape.com)
  • Treatment of the primary hyperoxalurias: A new chapter. (igenomix.it)
  • It had co-primary outcomes of treatment-related adverse events (AEs) and dose-limiting toxicities. (pharmacytimes.com)
  • TTI, which was the primary outcome of the study, was defined as time from histopathologic diagnosis to initiation of the first treatment measured in days. (ajmc.com)
  • If you have a history of calcium-oxalate kidney stones or suspect you have hyperoxaluria, there are a few more things you can do such as limit oxalate as much as possible, add citrate to your diet (through orange or lemon juice, or calcium citrate), minimize added fructose and sodium, or try a probiotic supplement as described below. (veganhealth.org)
  • According to LowOxalate.info , leaky gut syndrome, in which molecules are absorbed from the digestive tract at a higher than normal rate, can cause hyperoxaluria. (veganhealth.org)
  • citation needed] A diagnosis of primary hyperoxaluria is suspected based on presenting patient characteristics such as kidney stones in infants or children, recurrent kidney stones in adults, or family history of hyperoxaluria. (wikipedia.org)
  • This is performed using a gene panel covering known mutations for all three types of primary hyperoxaluria. (wikipedia.org)
  • There are three types of primary hyperoxaluria, and they're different in how severe they are and what causes them. (jewishgenetics.org)
  • The mean Pox concentration, the primary endpoint of the study, separated after week 24 in favor of Oxabact. (biospace.com)
  • The study did not demonstrate a significant improvement in progression-free survival (PFS), the primary endpoint of the trial. (delveinsight.com)
  • However, many treatments (eg, dietary measures) can be used in any case of hyperoxaluria, and treatments can be combined for increased efficacy. (medscape.com)
  • 3. The patient with primary hyperoxaluria had a plasma oxalate concentration before dialysis above 100 μmol/l and after dialysis above 25 μmol/l, while the oxalate concentration in haemodialysate at the start of dialysis was above 25 μmol/l and at the end above 10 μmol/l. (portlandpress.com)
  • 4. A plasma oxalate/creatinine concentration ratio exceeding 0.1, and the calculated total quantity of oxalate removed by dialysis exceeding 2 mmol, also enabled a diagnosis of hyperoxaluria to be made. (portlandpress.com)
  • Plasma and urinary amino acids in children with primary hyperoxaluria and in normal children. (bmj.com)
  • The patient suspected of hyperoxaluria had similar values. (portlandpress.com)
  • Measurements of oxalate in haemodialysate and plasma are valuable in cases where kidney transplantations are considered, especially when the particular patient exhibits hyperoxaluria. (portlandpress.com)
  • Conversely, a restriction in oxalate intake is of limited use as the main source of oxalate is endogenous in primary hyperoxaluria. (wikipedia.org)
  • The main issue in primary hyperoxaluria is that the body makes too much of something called oxalate. (jewishgenetics.org)
  • Why are sulfate levels important in hyperoxaluria? (betterhealthguy.com)
  • PLP is the primary active pyridoxal form, and serum PLP is used as the primary index of whole-body pyridoxal levels. (medscape.com)