Computed tomography after lymphangiography in the diagnosis of intestinal lymphangiectasia with protein-losing enteropathy in Noonan's syndrome

Department of Radiology, Universität Würzburg, Germany.
European Radiology (Impact Factor: 4.01). 02/2000; 10(10):1591-3. DOI: 10.1007/s003300000384
Source: PubMed


Noonan's syndrome is a rare congenital disorder that may be associated with abnormalities in the lymphatic drainage. In this case of a 21-year-old man CT after bipedal lymphangiography confirmed the diagnosis of intestinal lymphangiectasy causing protein-losing enteropathy in Noonan's syndrome by showing contrast-enhanced abnormal lymphatic vessels in the mesentery and the intestinal wall. Because of the benefit of diet in case of intestinal involvement, we recommend a thorough documentation of the lymphatic drainage with lymphangiography followed by CT, if clinical signs of lymphatic dysplasia, such as pleural effusions, lymphedema, or hypoproteinemia are present.

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    • "PIL, also known as Waldmann's disease, is a rare disease in humans characterized by dilated lymphatics in the duodenum, jejunum, or mesentery (Buohnik et al. 2000; Koo et al. 2005). It is congenital or idiopathic, and has been linked to several syndromes including Milroy's, Von Recklinghausen, Turner, Klippel Trenaunay, Hennekam, and Noonan's (Keberle et al. 2000; Suresh et al. 2009). An immunohistochemical study by Hokari et al. (2008) identified several growth factors and receptors significant in lymphangiogenesis and lymphangiectasia, namely VEGF-C and -D, FOXC2, SOX18, Prox 1, and VEGFR3 and LYVE-1, respectively, the latter two of which were specific and sensitive markers for lymphatics. "
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    ABSTRACT: To investigate the toxicity and carcinogenic potential of indole-3-carbinol (I3C), the National Toxicology Program has conducted 13-week subchronic studies in Fisher 344 rats and B6C3F1 mice, and chronic 2-year bioassays in Sprague-Dawley rats and B6C3F1 mice. While the chronic study results are not yet available, subchronic study results and short-term special evaluations of interim sacrifices in the 2-year rat bioassay are presented. F344 rats were orally gavaged ≤300 mg I3C/kg body weight 5 days a week for 13 weeks. Rats treated with ≥150 mg/kg demonstrated a dose-related dilation of lymphatics (lymphangiectasis) of the duodenum, jejunum, and mesenteric lymph nodes. Material within dilated lacteals stained positively for Oil Red O and Sudan Black, consistent with lipid. Electron microscopic evaluation confirmed extracellular lipid accumulation within the villar lamina propria, lacteals, and within villar macrophages. Analyses of hepatic and pulmonary CYP1A enzymes demonstrated dose-dependent I3C induction of CYP1A1 and 1A2. B6C3F1 mice orally gavaged ≤250 mg I3C/kg body weight did not demonstrate histopathological changes; however, hepatic CYP induction was similar to that in rats. The histopathologic changes of intestinal lymphangiectasis and lipidosis in this study share similarities with intestinal lymphangiectasia as observed in humans and dogs. However, the resultant clinical spectrum of protein-losing enteropathy was not present.
    Toxicologic Pathology 02/2012; 40(4):561-76. DOI:10.1177/0192623311436178 · 2.14 Impact Factor
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    • "Although protein-losing enteropathy (PLE) occurs in post-cardiac operations, such as the Fontan operation, inflammatory bowel syndrome, connective tissue diseases and parasitic disease (4,5,6,7,8,9), PLE with Noonan syndrome is rarely reported (10). "
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    ABSTRACT: Noonan syndrome is characterized by facial dysmorphology, congenital heart disease and growth failure. Although it is also accompanied by deranged lymph-vessel formation, protein-losing enteropathy (PLE) with Noonan syndrome is rarely reported. We report clinical information about a boy with Noonan syndrome and late-onset lymphedema and PLE after standing for long periods of time during athletic practice sessions. The boy recovered from lymphedema and PLE after administration of 2.5 g of albumin followed by resting and raising his legs. They did not recur after he began walking again. Standing for long periods of time congested the lymph stream at the abdominal lymph vessel, whose formation is frequently disturbed in Noonan syndrome, and the increased pressure caused lymphedema and PLE. PLE is one of the clinical manifestations of Noonan syndrome.
    Clinical Pediatric Endocrinology 07/2009; 18(3):87-93. DOI:10.1297/cpe.18.87
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