Developmental Disorders of the Lymphatics

An information blog for disorders of the lymphatics. For all articles, please click on "Archives" - Due to spammers, I will no longer allow comments, sorry.

Saturday, June 30, 2007

A case of lymphangioleiomyomatosis found due to chylous ascites, pleural effusion and pelvic lymphadenopathy

A case of lymphangioleiomyomatosis found due to chylous ascites, pleural effusion and pelvic lymphadenopathy

Nihon Kokyuki Gakkai Zasshi. 2007

Yano T, Hasizume I, Kasamatsu N, Kato T, Shibata M, Ashinuma N, Kobayashi K, Yasuda T, Nakamura A.
Department of Respiratory Medicine, Hamamatsu Medical Center.

We report a case of lymphangioleiomyomatosis, complaining initially of abdominal distension due to massive chylous ascites. The patient was a 28-year-old woman in whom abdominal ultrasound had strongly suggested the existence of both pelvic lymphadenopathy and massive ascites, the latter subsequently turning out to be chylous. Pelvic lymph node biopsy yielded a diagnosis of lymphangioleiomyomatosis. High-resolution computed tomography (HRCT) of the chest showed no remarkable findings except for very few cystic changes in the lung parenchyma.

Pulmonary function had remained normal except for a temporary constrictive pattern when chylous pleural effusion developed. No airflow obstruction was detected on pulmonary function tests.

Although lymphangioleiomyomatosis is often associated with pulmonary symptoms, we should bear in mind the possibility of lymphangioleiomyomatosis even in the absence of such symptoms when facing any woman of child-bearing age with abdominal chylous ascites of unknown etiology.

PMID: 17554983 [PubMed - indexed for MEDLINE]

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Sunday, June 24, 2007

Antioxidants suppress lymphoma and increase longevity in Atm-deficient mice.

Antioxidants suppress lymphoma and increase longevity in Atm-deficient mice.
J Nutr. 2007 Jan
Reliene R, Schiestl RH.
Department of Pathology, Geffen School of Medicine and School of Public Health, University of California, Los Angeles, CA 90095, USA.

* To whom correspondence should be addressed. E-mail:

Ataxia telangiectasia (AT), a human hereditary disorder resulting from mutations in the ATM gene, is characterized by a high incidence of lymphoid malignancies, neurodegeneration, immunodeficiency, premature aging, elevated radiosensitivity, and genomic instability. Evidence has been accumulating that ATM-deficient cells are in a continuous state of oxidative stress. A variety of markers of oxidative stress were detected in AT patients as well as Atm-deficient mice, used as an animal model of AT. Since then, it has been proposed that oxidative stress contributes to the clinical phenotype of AT, especially carcinogenesis and neurodegeneration, and several animal studies were conducted to determine whether exogenous antioxidants mitigate the symptoms of AT. Tempol, EUK-189, and N-acetyl cysteine have been tested as chemopreventive antioxidants in Atm-deficient mice. We review these findings, mainly focusing on the effect of N-acetyl cysteine, which is known as a safe and efficient drug and nutritional supplement.

Full Text Article:

Journal of Nutrition

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Monday, June 18, 2007

Kawasaki disease: diagnosis, management and cardiac sequelae

Kawasaki disease: diagnosis, management and cardiac sequelae

May 2007, Vol. 5, No. 3, Pages 553-561

† Author for correspondence

Kawasaki disease (KD) is an acute self-limiting systemic vasculitis of unknown etiology and the most common cause of acquired coronary disease in children aged 6 months to 5 years. The inflammatory process results in coronary arteritis, aneurysmal lesions, arterial thrombotic occlusion or even sudden death. The diagnostic tests are unknown but treatment with immunoglobulin and aspirin is effective at reducing cardiac complications from 25 to 4.7% in the UK. Myocardial, endocardial or pericardial inflammation may occur acutely or many years later and abnormalities of myocardial blood flow may require ongoing medication, interventional catheterization or even cardiac surgery. There are several new drugs that may have important roles to play in managing KD in children and young adults.

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Sunday, June 10, 2007

Generalized lymphangiomatosis presenting as cardiomegaly

Generalized lymphangiomatosis presenting as cardiomegaly

J Formos Med Assoc. 2007 Mar
Chen YL, Lee CC, Yeh ML, Lee JS, Sung TC.
Department of Pediatrics, Shin Kong Wu Ho-Su Memorial Hospital, Taipei, Taiwan.

Lymphangioma refers to the local proliferation of well-differentiated lymphatic tissue.

Generalized lymphangiomatosis is rare. We report a previously healthy 8-month-old infant who suffered from tachypnea with mild fever for 2 weeks. Imaging studies revealed a well-defined, large mass occupying the mediastinum, which presented as cardiomegaly. The disseminated mass extended to the thymus, lung, and spleen. Lymphangiomatosis was diagnosed by biopsy.

Drainage of the pericardial fluid and total parenteral nutrition did not result in improvement of chylopericardium.

Secondary hypogammaglobulinemia and septic shock developed sequentially. Surgical removal of the mediastinal mass and spleen were performed. Daily subcutaneous injection of interferon (IFN) alpha-2b was then given for 3 months. No recurrence was noted during 2 years of follow-up. IFN alpha-2b may be considered as an alternative for the treatment of generalized lymphangiomatosis.


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Saturday, June 02, 2007

New Treatment for Noonan Syndrome


June 1, 2007

WASHINGTON, June 1 (UPI) -- The U.S. Food and Drug Administration Friday approved Copenhagen-based Novo Nordisk's Norditropin for children with Noonan syndrome.

The genetic disorder is characterized by short stature, congenital heart defects and unique facial features, notably widely set eyes.

The use of Norditropin -- somatropin injection -- is aimed at treatment of short stature in these individuals.

Fewer than 200,000 people have the condition, prompting the FDA to give Norditropin an orphan drug designation. No other treatment for short stature in these patients is available.

The prevalence of Noonan syndrome has not been determined accurately to date, but most authors report 1 in 1,000 to 1 in 2,500 live births, affecting males and females equally. In addition to heart abnormalities and short stature -- affecting 80 percent of people with Noonan syndrome -- patients may also have visual and hearing deficits, bleeding abnormalities and lack muscle tone.

"Noonan syndrome is a heterogeneous genetic condition in which the clinical features are quite variable," said Alicia Romano, a pediatric endocrinologist at the New York Medical College, in a statement issued by Novo Nordisk.

"Short stature, which can be severe, is one of the most common characteristics. Treatment with Norditropin may help children with Noonan syndrome improve one of the most concerning physical features of the condition."

Norditropin is also approved for treatment of children with growth failure due to inadequate secretion of endogenous growth hormone and for replacement of endogenous growth hormone in adults with growth hormone deficiency.

United Press International

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