Information on all the types of lymphangiectasia, including intestinal, pulmonary, renal, cutaneous (skin). Sponsored by Pat O'Connor

Tuesday, October 20, 2009

Cytomegalovirus-associated protein-losing enteropathy resulting from lymphangiectasia in an immunocompetent child.

Cytomegalovirus-associated protein-losing enteropathy resulting from
lymphangiectasia in an immunocompetent child.

Department of Pediatrics, Graduate School of Medical Sciences, Kyushu University, Fukuoka 812-8582, Japan.

An immunocompetent 8-year-old boy with cytomegalovirus (CMV)-associated transient protein-losing enteropathy (PLE) is described. Colonoscopic examination revealed lymphoid hyperplasia of the terminal ileum. Histological examination of the biopsied specimens showed marked dilation of the lymphatic vessels. Primary CMV infection was demonstrated by serological test and polymerase chain reaction. The child had complete resolution of the disease without antiviral treatment. The present case suggests the etiologic role of CMV infection in PLE resulting from intestinal lymphangiectasia in childhood.


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Monday, October 12, 2009

P13K 1-phosphatidylinositol 3-kinase definition and studies

P13K 1-phosphatidylinositol 3-kinase definition

<cell biology> An enzyme that catalyses the conversion of phosphatidylinositol to phosphatidylinositol 3-phosphate. This is the first committed step in the biosynthesis of phosphatidylinositol 3,4-bisphosphate and phosphatidylinositol 3,4,5-trisphosphate. This pathway is thought to play a critical role in DNA repair, v(d)j recombination and cell cyle checkpoints.37.

Chemical name: ATP:1-phosphatidyl-1D-myo-inositol 3-phosphotransferase

Registry number: EC

Online Medical Dictionary

Additional studies on its role:

Effects of Tyroserleutide on Gene Expression of Calmodulin and P13K in Hepatocellular Carcinoma

Oncogenic P13K Deregulates Transcription and Translation

A role for phosphoinositol-3-kinase (P13K) in the stimulation of hepatic taurocholate uptake by cyclic AMP

Laminin-332 promotes the invasion of oesophageal squamous cell carcinoma via P13K activation


Expression Status and Mutational Analysis of the PTEN and P13K Subunit Genes in Ovarian Granulosa Cell Tumors

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Organ-specific lymphangiectasia, arrested lymphatic sprouting, and maturation defects P13K gene

Organ-specific lymphangiectasia, arrested lymphatic sprouting, and maturation defects resulting from gene-targeting of the PI3K regulatory isoforms p85, p55, and p50

Dev Dyn. 2009 Oct

Center for Molecular Medicine, Maine Medical Center Research Institute, Scarborough, Maine, USA.

email: Calvin P.H. Vary (

The phosphoinositide 3-kinase (PI3K) family has multiple vascular functions, but the specific regulatory isoform supporting lymphangiogenesis remains unidentified. Here, we report that deletion of the Pik3r1 gene, encoding the regulatory subunits p85alpha, p55alpha, and p50alpha impairs lymphatic sprouting and maturation, and causes abnormal lymphatic morphology, without major impact on blood vessels. Pik3r1 deletion had the most severe consequences among gut and diaphragm lymphatics, which share the retroperitoneal anlage, initially suggesting that the Pik3r1 role in this vasculature is anlage-dependent. However, whereas lymphatic sprouting toward the diaphragm was arrested, lymphatics invaded the gut, where remodeling and valve formation were impaired. Thus, cell-origin fails to explain the phenotype. Only the gut showed lymphangiectasia, lymphatic up-regulation of the transforming growth factor-beta co-receptor endoglin, and reduced levels of mature vascular endothelial growth factor-C protein. Our data suggest that Pik3r1 isoforms are required for distinct steps of embryonic lymphangiogenesis in different organ microenvironments, whereas they are largely dispensable for hemangiogenesis.

Wiley InterScience

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Reversal of protein-losing enteropathy after heart transplantation in young patients.

Reversal of protein-losing enteropathy after heart transplantation in young patients

Rev Esp Cardiol. 2009 August

Servicio de Cardiología, Hospital Universitario La Fe, Valencia, Spain.

Protein-losing enteropathy is a rare but life-threatening complication that occurs in some patients who develop intestinal lymphangiectasis secondary to increased systemic venous pressure. Although different forms of treatment have been tried, with varying results, the majority were reported to be unsuccessful. The aim of this study was to demonstrate that heart transplantation may be an appropriate therapeutic option for patients who do not respond to medical treatment. At our center, we performed heart transplantations in three patients with this condition. The mean follow-up period was 11+/-2 months. No patient died and the enteropathy regressed in all three.

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