Guest contributor. What if you were suddenly diagnosed with a potentially fatal disease just when your life, work and marriage were on track and your plans to start a family were underway?. Thats what happened to Sue R. Levy.. In 2008, at age 37, she was diagnosed with Pulmonary Lymphangioleiomyomatosis, otherwise known as LAM, a rare, chronic, progressive lung disease in which the lungs fill up with cysts. The result is gradual destruction of the normal lung architecture, compromised breathing and, in many cases, an eventual lung transplant - a procedure with major risks. The LAM Foundation reports 10-year survival, following a lung transplant, at 47 percent.. Fueled by estrogen, LAM primarily affects women in their childbearing years. With only 1,300 documented cases in North America, LAM is poorly understood; currently, there are a few experimental medications in use, but no proven treatments exist.. Prior to the diagnosis, Sue, who lives in Brookline, Mass., had a successful career as a ...

Lymphangioleiomyomatosis (LAM) is a low-grade neoplasm characterized by the pulmonary infiltration of smooth muscle-like cells (LAM cells) and cystic destruction. Patients usually present with airway obstruction in pulmonary function tests (PFTs). Previous studies have shown correlations among histological parameters, lung function abnormalities and prognosis in LAM. We investigated the lung tissue expression of proteins related to the mTOR pathway, angiogenesis and enzymatic activity and its correlation with functional parameters in LAM patients. We analyzed morphological and functional parameters of thirty-three patients. Two groups of disease severity were identified according to FEV1 values. Lung tissue from open biopsies or lung transplants was immunostained for SMA, HMB-45, mTOR, VEGF-D, MMP-9 and D2-40. Density of cysts, density of nodules and protein expression were measured by image analysis and correlated with PFT parameters. There was no difference in the expression of D2-40 between the more

Lymphangioleiomyomatosis is caused by mutations (usually sporadic, sometimes in tuberous sclerosis) of the TSC1 or TSC2 genes and results in cystic destruction of the lungs, with CT features being sufficiently characteristic to establish the diagnosis in many cases. Two-thirds of patients suffer pneumothoraces. Hormonal therapy with progesterone or tamoxifen is usually given for progressive disease; medical or surgical pleurodesis is advisable; lung transplantation is the main option for advanced disease....

Lymphangioleiomyomatosis LymphangioleiomyomatosisClassification & external resources CT scan of a lung with LAM. ICD-O: 9174/1 OMIM 606690 DiseasesDB 30755

TY - JOUR. T1 - Distinguishing between lymphangioleiomyomatosis and carcinomatous peritonitis in a patient with ovarian cancer. AU - Hirasawa, Akira. AU - Sato, Takashi. AU - Ueno, Mari. AU - Akahane, Tomoko. AU - Susumu, Nobuyuki. AU - Betsuyaku, Tomoko. AU - Aoki, Daisuke. PY - 2013/10/1. Y1 - 2013/10/1. UR - http://www.scopus.com/inward/record.url?scp=84891522232&partnerID=8YFLogxK. UR - http://www.scopus.com/inward/citedby.url?scp=84891522232&partnerID=8YFLogxK. U2 - 10.1200/JCO.2012.45.3019. DO - 10.1200/JCO.2012.45.3019. M3 - Short survey. C2 - 23752115. AN - SCOPUS:84891522232. VL - 31. SP - e427-e429. JO - Journal of Clinical Oncology. JF - Journal of Clinical Oncology. SN - 0732-183X. IS - 28. ER - ...

TY - JOUR. T1 - Distinguishing between lymphangioleiomyomatosis and carcinomatous peritonitis in a patient with ovarian cancer. AU - Hirasawa, Akira. AU - Sato, Takashi. AU - Ueno, Mari. AU - Akahane, Tomoko. AU - Susumu, Nobuyuki. AU - Betsuyaku, Tomoko. AU - Aoki, Daisuke. PY - 2013/10/1. Y1 - 2013/10/1. UR - http://www.scopus.com/inward/record.url?scp=84891522232&partnerID=8YFLogxK. UR - http://www.scopus.com/inward/citedby.url?scp=84891522232&partnerID=8YFLogxK. U2 - 10.1200/JCO.2012.45.3019. DO - 10.1200/JCO.2012.45.3019. M3 - Short survey. C2 - 23752115. AN - SCOPUS:84891522232. VL - 31. SP - e427-e429. JO - Journal of Clinical Oncology. JF - Journal of Clinical Oncology. SN - 0732-183X. IS - 28. ER - ...

What every physician needs to know: Lymphangioleiomyomatosis (LAM) is a rare cystic lung disease that is associated with progressive dyspnea on exertion, recurrent pneumothorax, chylous thoracic and abdominal effusions, and thoracic and abdominal tumors, including lymphangioleiomyomas and angiomyolipomas. LAM occurs predominantly in young and middle-aged women, but it has also been reported in children, post-menopausal…. ...

A total of 532 patients (64.5% males) from 32 hospitals were included. The mean (SD) ages of both study groups were similar [68 (10) vs 67 (10) years, P = NS). A total of 1050 lung resections were performed (90% segmentectomies or wedge, n = 946 and 10% lobectomies or greater, n = 104). Group A included 83 (15.6%) patients who developed a total of 100 complications. These included persistent air leaks in 18, atelectasis in 13, pneumonia in 13, paralytic ileum in 12, arrhythmia in 9, acute respiratory distress syndrome in 4 and miscellanea in 31. Reoperation was performed in 5 (0.9%) patients due to persistent air leaks in 4 and lung ischaemia in 1. The mortality rate was 0.4% (n = 2). Causes of death were sepsis in 1 patient and ventricular fibrillation in 1. In the multivariate analysis, lobectomy or greater lung resection [odds ration (OR) 1.9, 95% confidence interval (95% CI) 1.04-3.3, P = 0.03], respiratory co-morbidity (OR 2.3, 95% CI 1.1-4.6, P = 0.01) and cardiovascular co-morbidity (OR ...

Bitte beachten Sie diesen Artikel im Zusammenhang des Gesamtwerks. Eine ärztliche Plausibilitätsprüfung im Kontext dieses Cockpits ist unerlässlich. Die Anzeige von Inhalten ist insbesondere bei den Dropdowns zu Therapie und Medikamenten keinesfalls als Anwendungsempfehlung oder Indikation zu verstehen, sondern soll Ihnen lediglich die Suche erleichtern. Häufig werden ganze Medikamenten-/Themengruppen angezeigt, die im gegebenen Zusammenhang möglicherweise von Interesse sein könnten. Für Vollständigkeit kann keine Gewähr übernommen werden. ...

The LAM Foundation urgently seeks safe and effective treatments, and ultimately a cure, for lymphangioleiomyomatosis (LAM) through advocacy and the funding of promising research.

Research program, lymphangioleiomyomatosis, smoke induced lung injury, cigarette smoke related vascular disease, tissue destruction in alternative lung diseases

TY - JOUR. T1 - Pulmonary lymphangioleiomyomatosis in a man. AU - Aubry, M. C.. AU - Myers, J. L.. AU - Ryu, J. H.. AU - Henske, E. P.. AU - Logginidou, H.. AU - Jalal, S. M.. AU - Tazelaar, H. D.. PY - 2000. Y1 - 2000. N2 - Pulmonary lymphangioleiomyomatosis (LAM) is an uncommon disease reported to occur exclusively in women. We describe a pheno-typically normal man with pulmonary LAM. Fluorescence in situ hybridization (FISH) studies performed on the lung biopsy confirmed a normal XY genotype. Our patient also had stigmata of tuberous sclerosis complex (TSC), including facial angiofibromas and renal angiomyolipoma. Immunohistochemical stains of both Lam and renal angiomyolipoma showed positive immunoreactivity for hamartin (TSC1) and loss of immunoreactivity for tuberin (TSC2). Loss of heterozygosity (LOH) for TSC2 was further demonstrated in the renal angiomyolipoma. Coupled with the results of immunostains, these findings are consistent with TSC2 mutation.. AB - Pulmonary ...

The benign, non-invasive lesions of tuberous sclerosis can appear in any organ like the brain, heart, skin, eyes, kidney, lung, and liver. Therefore, TS has a wide clinical spectrum. The diagnosis of definitive TS is based on specific clinical features and requires the presence of two major criteria, or one major and two minor [3]. Pulmonary lymphangioleiomyomatosis, renal angiomyolipoma and facial angiofibroma are some of the major clinical features.. The most frequent cause of death in patients with TS is renal complication [3, 5]. Multifocal, bilateral angiomyolipomas are found in about 70-90% of adult patients [3], and the prevalence increases with age, being less frequent in children [3, 4]. These lesions are more often prevalent in women, suggesting a hormonal component to the tumor growth [6]. The angiomyolipomas are composed of varying amounts of mature adipose tissue, smooth muscle, and abnormal blood vessels [3, 6]. The demonstration of intratumoral fat with negative attenuation values ...

Lymphangioleiomyomatosis: a case report and review of diagnosis and treatment Yi Liu,* Zhibin Guo,* Chenlong Zhao, Xin Li, Hongyu Liu, Jun Chen Department of Lung Cancer Surgery, Tianjin Key Laboratory of Lung Cancer Metastasis and Tumor Microenvironment, Tianjin Lung Cancer Institute, Tianjin Medical University General Hospital, Tianjin 300052, China *These authors contributed equally to this work Abstract: Lymphangioleiomyomatosis (LAM) is a rare disease that generally affects young women and involves the abnormal proliferation of smooth muscle-like cells (LAM cells) in the lungs (pulmonary LAM) and extrapulmonary sites (extrapulmonary LAM). This disease is rare in males. It is hard to distinguish between lung cancer and pulmonary LAM, especially during early stages. Herein, we present a case of a 66-year-old man with a small nodule in the right upper lobe that was first diagnosed as a lung malignancy using a chest CT scan. After a wedge dissection, a pathologist performed a histologic and

Specific Aim 1: To investigate whether, in Lymphangioleiomyomatosis (LAM) patients, the combination of sirolimus and hydroxychloroquine is safe and well tolerated. Specific Aim 2: To investigate whether, in LAM patients, 6 months of combination therapy with sirolimus and hydroxychloroquine results in improvement of indicators of disease, and whether the gains are sustained after stopping therapy.. Specific Aim 3: To investigate the potential role of a LAM-specific peripheral blood signature to predict rates of disease progression and determine responsiveness to combination therapy.. This will be a phase I dose escalation study of the combination of sirolimus (2 mg adjusted to keep trough levels between 5-15 ng/ml) and hydroxychloroquine (200 mg or 400 mg) taken orally daily. Up to 18 adult women with LAM will be enrolled. ...

Specific Aim 1: To investigate whether, in Lymphangioleiomyomatosis (LAM) patients, the combination of sirolimus and hydroxychloroquine is safe and well tolerated. Specific Aim 2: To investigate whether, in LAM patients, 6 months of combination therapy with sirolimus and hydroxychloroquine results in improvement of indicators of disease, and whether the gains are sustained after stopping therapy.. Specific Aim 3: To investigate the potential role of a LAM-specific peripheral blood signature to predict rates of disease progression and determine responsiveness to combination therapy.. This will be a phase I dose escalation study of the combination of sirolimus (2 mg adjusted to keep trough levels between 5-15 ng/ml) and hydroxychloroquine (200 mg or 400 mg) taken orally daily. Up to 18 adult women with LAM will be enrolled. ...

AbstractThe authors discuss the case of a 21-year-old female of Afro-Caribbean origin, who presented with primary amenorrhoea, and was found to have congenital absence of the uterus and fallopian tubes in the presence of a normal vaginal cavity.

Lymphangioleiomyomatosis (LAM) is a rare, low-grade multisystem neoplastic disease. Most LAM patients are at a high risk of losing lung function at an accelerated rate and developing progressive dyspnea. Recently, several studies have reported their experience with pharmacological treatments for LAM. Therefore, we conducted a systematic review and meta-analysis to assess the efficacy and safety of these therapies. PubMed (Medline), EMBASE, Cochrane Library, Web of Science and EBSCO Host were searched (until March 31, 2019) for eligible prospective studies regarding LAM patients treated with pharmacological treatments. Random effect models were used for quantitative analysis. Fourteen prospective studies regarding five pharmacological treatments (including sirolimus, everolimus, doxycycline, triptorelin, and a combination therapy of sirolimus and hydroxychloroquine) were enrolled in our systematic review, and ten of them were used for the meta-analysis. Seven prospective studies reported that sirolimus

Lymphangioleiomyomatosis (LAM) is a rare progressive cystic lung disease affecting young women. The pivotal observation that LAM occurs both spontaneously and as part of the tuberous sclerosis complex (TSC) led to the hypothesis that these disorders share common genetic and pathogenetic mechanisms. …

Relevant aspects of lymphangioleiomyomatosis: almost exclusively affects women of child-bearing age it is a multi-system disorder and can affect many organs recurrent pneumothorax can occur in up to 80% of cases in the lungs it manifests as m...

EAML is the least common subtype of AML. This tumor is generally regarded as one tumor type in a family of neoplasms known as perivascular epithelioid. cell tumors or PEComas. In addition to the classic triphasic AML with a mixture of smooth muscle, fat and blood vessels, the family of PEComas also includes MK-2206 chemical structure the myomelanocytic tumor of the falciform ligament, so-called clear cell tumor of the lung, lymphangiomyomatosis, and EAML of the liver. The corroboration of the diagnosis of EAML generally relies upon the immunohistochemical expression of a melanocyte marker-MART-1/Melan-A, Human Melanoma Black-45, or both.4 Smooth muscle actin expression is variable from one case to another; there was only minimal and quite localized staining in our case. Classic AMLs of the kidney are initially recognized at or before the age of 10 years in approximately 10%-15% of TSC cases. Individuals with TSC have multifocal AMLs measuring 4 cm or less in most cases detected in the first ...

LAM is a rare disorder that occurs almost exclusively in women and is generally diagnosed in women of childbearing age. LAM mainly affects the lungs, where an abnormal type of cell (called a LAM cell) builds up. This build up of LAM cells occurs around the airways (breathing tubes) and also around the blood vessels and the lymph vessels (lymph vessels drain excess fluid from the lungs). The LAM cells lead to cysts developing in the lung and these may be seen on a chest x-ray and are shown very clearly on a CT lung scan. The main effects of LAM are in the lungs but around half of patients also have tumours in the kidneys; they are not malignant but can cause bleeding. Lymph nodes may also be enlarged, usually in the chest or abdomen, but they dont normally cause problems.. The name lymphangioleiomyomatosis reflects the different components of the disease. Lymph and angio refer to the lymph and blood vessels that are involved and leiomyo refers to smooth muscle which LAM cells resemble. LAM that ...

Home spirometry is being added as a secondary end point in a lymphangioleiomyomatosis (LAM) trial soon to begin enrollment at the University of Cincinnati (UC) Medical Center. Its inclusion is aimed at helping to assess the feasibility and reliability of performing home spirometry among patients with LAM.

Doctors who treat patients with a severe and progressive respiratory disease called lymphangioleiomyomatosis (LAM) can face an agonizing treatment decision.

TY - JOUR. T1 - Rapid growth and regression of intracranial meningiomas in lymphangioleiomyomatosis. T2 - case report. AU - Pozzati, Eugenio. AU - Zucchelli, Mino. AU - Schiavina, Mario. AU - Contini, Paola. AU - Foschini, Maria Pia. PY - 2007/12. Y1 - 2007/12. N2 - Background: Lymphangioleiomyomatosis is a progressive interstitial lung disease that affects young women. It has been suggested that estrogens play a role in its evolution, and progesterone therapy is often provided in these cases. Case Description: We present a case of a postmenopausal woman with LAM treated with progesterone; subsequently, rapid growth of multiple intracranial meningiomas was observed. One prominent lesion was excised, and 3 other lesions regressed spontaneously over 2 years. Conclusions: This is a rare case of a non-pregnancy-related regression of meningiomas in a woman affected by LAM. The significance of this association and the hormonal treatment of the disease are discussed.. AB - Background: ...

Clinical record. A 22-year-old Taiwanese woman on a working holiday in Australia was admitted to hospital in March 2011 with a cervical spine fracture from a motor vehicle accident. During her hospital stay it was noted that she had metabolic alkalosis with significant hypokalaemia.. Her past medical history included presentation at 16 years of age with primary amenorrhoea that was attributed to pubertal delay. At that time, she was given oestrogen-progesterone replacement therapy, which induced withdrawal bleeding. She noted an increase in stature after commencing hormone replacement therapy, growing to the height of her two brothers. She was diagnosed with mild hypertension at a pre-immigration health check in Taiwan. After arriving in Australia, 12 months before the accident, she had stopped taking hormonal therapy and experienced complete cessation of withdrawal bleeds. She was ethnically Han Chinese with no family history of consanguinity.. Physical examination revealed she was tall and ...

A 17-year-old Asian man presented with a spontaneous pneumothorax, which was treated initially by needle aspiration. It recurred within a week with complete right-sided pneumothorax requiring water-sealed intercostal drainage. The air leak persisted after one week and the patient developed pyrexia with associated neutrophilia and raised serum inflammatory markers. A chest radiograph showed a hydro-pneumothorax and culture of the pleural fluid grew Pseudomonas aeruginosa and Methicillin-resistant Staphylococcus aureus (MRSA). He was treated with intravenous Piperacillin-Tazobactam resulting in a full clinic-radiological recovery.. A week later his fever returned and repeat chest radiograph showed right middle and lower lobe consolidation associated with recurrent hydro-pneumothorax. A new intercostal drain was inserted and he was intubated and ventilated due to the rapid onset of severe respiratory distress. Computed tomogram (CT scan) of the chest confirmed bilateral patchy consolidation in ...

Lu,C.; Lee,H. S.; Pappas,G. P.; Dilling,D. F.; Burger,C. D.; Shifren,A.; Veeraraghavan,S.; Chapman,J. T.; Parambil,J.; Ruoss,S. J.; Young,L. R.; Hammes,S. R.; Kopras,E. J.; Roads,T.; Krischer,J. P.; McCormack,F. X.; Trial of an Aromatase Inhibitor in Lymphangioleiomyomatosis GroupA Phase II Clinical Trial of an Aromatase Inhibitor for Postmenopausal Women with Lymphangioleiomyomatosis Annals of the American Thoracic Society 2017 ;14(6):919- ...

LAM Australasia Research Alliance (LARA) funds research into lymphangioleiomyomatosis, a rare and devastating lung disease striking women mostly in their 30s.

I am very lucky to be alive after a double-lung transplant in July 2006. I suffered the rarest lung disease called, Lymphangioleiomyomatosis (Lam). I kept fighting for my life and trying to breathe each time my lung collapsed (15 times). I used to be on 24 hour oxygen to help me breathe and also wheelchair bound. At one stage I ended up in a coma (3weeks) and remained on life support for almost 2 months. I had to learn how to walk again with a zimaframe, and start to re-build my life/confidence/strength up. I want to enjoy my life with the extension this transplant has given me. My journey post transplant has been challenging with health issues, but, I try to remain as positive as possible ...

I am very lucky to be alive after a double-lung transplant in July 2006. I suffered the rarest lung disease called, Lymphangioleiomyomatosis (Lam). I kept fighting for my life and trying to breathe each time my lung collapsed (15 times). I used to be on 24 hour oxygen to help me breathe and also wheelchair bound. At one stage I ended up in a coma (3weeks) and remained on life support for almost 2 months. I had to learn how to walk again with a zimaframe, and start to re-build my life/confidence/strength up. I want to enjoy my life with the extension this transplant has given me. My journey post transplant has been challenging with health issues, but, I try to remain as positive as possible ...

TGLBF : Triglyceride concentration in body fluids is correlated to the presence of chylomicrons and can be useful when diagnosing chylous effusion or differentiating from pseudochylous effusion.(1) Chylous effusions are characterized by the presence of chyle which contains chylomicrons circulating through the lymphatic system. Pseudochylous effusions do not have chylomicrons. These fluids have a milky appearance and can be confused with chylous effusions. While chylous effusions often have elevated triglyceride concentrations and decreased cholesterol concentrations, identification of chylomicrons is considered the gold standard for the diagnosis. Pleural fluid: Chylothorax is the name given to pleural effusions containing chylomicrons. They develop when chyle accumulates from disruption of the lymphatic system, often the thoracic duct, caused mainly by malignancy or trauma.(1) Lymph contains chylomicron rich chyle characterized by high concentrations of triglycerides. Pseudochylous

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LAM Australasia Research Alliance (LARA) funds research into lymphangioleiomyomatosis, a rare and devastating lung disease striking women mostly in their 30s.

Lymphangioleiomyomatosis or LAM is a rare lung disease that occurs only in women and nearly always starts before the menopause. LAM mainly affects the lungs, where an abnormal type of cell (called a LAM cell) builds up around the airways and the lymph vessels. The LAM cells lead to cysts developing in the lung. One of the main symptoms is breathlessness due to the effect of the cells on the airways and also to the cysts which take up space in the lungs. Click here for more information.. ...

CHICAGO, IL--(Marketwired - Mar 23, 2015) - About 1,400 American women and some 3,000 globally have been diagnosed with LAM or Lymphangioleiomyomatosis, a progressive and rare deadly lung disease which affects women during their child bearing years. One in 10 Americans is affected by a rare disease, and there are...

Differential diagnosis of multiple lung cysts on HRCT: Lymphangioleiomyomatosis, emphysema (cysts and walls usually less well-defined), chronic interstitial lung diseases (honeycombed cysts usually peripheral), Langerhans cell histiocytosis (nodules ± cavities, presence of thicker-walled cysts, upper lung predominance), bronchiectasis (tree-and-bud pattern often present), sarcoidosis (cysts usually apical), and pneumatoceles (commonly seen with pneumocystis pneumonia) Histologic differential diagnosis: Lymphangioleiomyomatosis; emphysema (if smooth muscle nodules not recognized); smooth muscle proliferations such as benign metastasizing leiomyoma, metastatic stromal sarcoma of the uterus, and metaplastic muscle bundles in fibrosing lung diseases; and alveolar hemorrhage syndromes (if hemosiderin deposits frequent) Diagnosis: Lymphangioleiomyomatosis. Diagnostic features of lymphangioleiomyomatosis on HRCT. ...

Common treatment options for persistent air leaks involve continued tube thoracostomy drainage, pleurodesis, and surgical repair.1 Endobronchial valve placement remains the only endobronchial intervention available, with documented literature supporting use in air leaks.2-4 Although other interventions such as glue and coiling have been reported, the American College of Chest Physicians statement on prolonged air leaks in pneumothorax suggest avoidance of these endobronchial interventions.5 Our multidisciplinary team pursued endobronchial valve placement in this patient with a persistent air leak after recurrent pneumothorax in the setting of an advanced refractory malignancy. Standard management options appeared unfavorable due to his underlying comorbidities; prolonged chest tube drainage via an out-patient Heimlich valve was undesirable due to persistent neutropenia and infectious complications, and thoracoscopic surgery in transfusion-dependent acute myelogenous leukemia carries significant ...

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Large infarcts, produce multiple myocardiocytolysis ,local expansion and sometimes even perforation of the myocard wallfollowed by haemopericardium acute and sudden death by

Antibodies , Primary Antibodies , Cytoskeleton , Anti-Actin (alpha Smooth muscle specific), HiLyte Fluor 647-labeled; Host: rabbit polyclonal; Species Reactivity: human, mouse; Application: WB, IHC

Trauma is the third most common cause of death in the West. In the US, approximately 90,000 deaths annually are traumatic in nature and over 75% of casualties from blunt trauma are due to chest injuries. Cardiac injuries from rib fractures following blunt trauma are extremely rare. We report the unusual case of a patient who fell from a height and presented with haemopericardium and haemothorax as a result of left ventricular and lingular lacerations and was sucessfully operated upon.

Introduction. Chylous effusions, particularly chylothorax, are frequently reported in dogs and cats. Chyle is the term used to denote lymphatic fluid arising from the intestine and therefore containing a high quantity of fat. Chyle is normally transported to the venous system by a network of lymphatics in the mesentery (intestinal trunk). These lymphatics arborize in the cisterna chyli, a large dilated sac that lies adjacent to the aorta at L1-L4. The thoracic duct (TD) is the cranial continuation of the cisterna chyli and is generally said to begin between the crura of the diaphragm. In dogs (cats), the thoracic duct lies between the aorta and the azygous vein on the right side (left side) of the thorax and terminates in the venous system of the neck (left external jugular vein or jugulo-subclavian angle). Regardless of the etiology, chylothorax is a potentially devastating disease. Chylothorax results in both compromised respiration and debilitation because of loss of large amounts of ...

The jugular vein is the most frequent site of intravenous injection and catheter placement in the horse.. The most common cause of clotting of the jugular vein is irritation and clot formation from an intravenous catheter. Horses that are very systemically ill are much more likely to have this complication. It is especially common in horses that have intestinal inflammation (colitis and enteritis). Horses that are very ill post-colic surgery are also at higher risk for this problem.. Horses can live a normal life with only one jugular vein. Most even survive loss of both jugulars. The jugular vein is the main avenue for return of blood to the heart from the head. When a horse loses its jugular vein, the head swells transiently because of back pressure in the vessels from the damming effect of the clot. Horses that lose both jugular veins may have chronically swollen heads.. ...

The purpose of this medical research study is to evaluate the safety and effectiveness of a new medication called imatinib mesylate in the treatment of Lymphangioleiomyomatosis (LAM). LAM is a rare disease in which abnormal cells (called LAM cells) grow out of control. Over time, LAM cells destroy healthy lung tissue and cause respiratory disease or failure. Many patients with LAM are currently treated with a medication called sirolimus (rapamycin). Sirolimus slows the growth of LAM cells. Imatinib mesylate (hereafter called imatinib) is approved by the Food and Drug Administration (FDA) for the treatment of some cancers that share common pathways with LAM cells. Laboratory studies suggest that imatinib could completely block the growth of LAM cells through initiation of targeted cell death.. An important purpose of this research is to determine the safety of imatinib in people with LAM. This study will also evaluate the short-term effectiveness of imatinib. Participants will be randomized to ...

Message from Frank McCormack, MD, Division Director of Pulmonary, Critical Care & Sleep Medicine. Dear Colleague:. Greetings from the University of Cincinnati Medical Center Division of Pulmonary, Critical Care, & Sleep Medicine. Starting in 2015, we are pleased to share Pulmonary Insights, an educational resource with information on our ongoing research in rare lung disease, as well as innovative diagnostics and therapeutics that have resulted. Our goal is to collaborate with our peers, using Pulmonary Insights as one of many ways to create a dialogue with you - fellow physicians who seek the best ways to care for patients.. Research in lymphangioleiomyomatosis (LAM) has been particularly rewarding. We studied the safety and efficacy of the drug sirolimus (rapamycin) in an NIH-funded randomized controlled trial called MILES based at our academic health center and conducted by physician scientists here and around the world. Since the New England Journal of Medicine published the findings in ...

This is a report of the June 11, 2012 American Thoracic Society Public Advisory Roundtable Meeting that Karen Deitemeyer attended in Washington, DC, on behalf of EFFORTS. If you do not have time to read the entire report, please be sure to read the four Q & A at the end. Dr. Tony Punturieri, Program Director, Division of Lung Diseases at the NHLBI answered questions provided by EFFORTS members .. There were 21 people present, representing the ATS staff and staff or patients from various lung disease patient organizations. The attendees with COPD groups were Karen Deitemeyer, representing EFFORTS, Vlady Rosenbaum from COPD-ALERT and Miriam O Day, representing the Alpha-1 Foundation.. Other attendees represented the Hermansky-Pudlak Syndrome Network, Inc; the Asthma and Allergy Foundation of America; the Lymphangiomatosis and Gorham s Disease Alliance; the Pulmonary Hypertension Association, Inc.; the LAM Foundation (lymphangioleiomyomatosis); the Coalition for Pulmonary Fibrosis; the Children s ...

OBJECTIVES: We examined the long-term effects of everolimus in patients with renal angiomyolipoma associated with tuberous sclerosis complex or sporadic lymphangioleiomyomatosis. METHODS: Following favorable results from the double-blind core phase of EXIST-2 (NCT00790400), patients were allowed to receive open-label everolimus (extension phase). Patients initially randomly assigned to everolimus continued on the same dose; those who were receiving placebo crossed over to everolimus 10 mg/day. Dose modifications were based on tolerability. The primary end point was angiomyolipoma response rate, defined as a ≥50% reduction from baseline in the sum volume of target renal angiomyolipomas in the absence of new target angiomyolipomas, kidney volume increase of >20% from nadir, and angiomyolipoma-related bleeding grade ≥2. The key secondary end point was safety. RESULTS: Of the 112 patients who received ≥1 dose of everolimus, 58% (95% CI, 48.3% to 67.3%) achieved angiomyolipoma response. Almost ...

乳び腹水に対して腹腔頸静脈シャント術を施行したリンパ脈管筋腫症の1剖検例 [in Japanese] Lymphanigiomyomatosis with Chylous Ascites Treatment Successfully by Peritoneo-Venous Shunting [in Japanese] ...