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

Wednesday, September 26, 2007

Hypocalcaemic seizures: sign of intestinal disease lymphangiectasia?

Hypocalcaemic seizures: sign of intestinal disease?

Acta Gastroenterol Belg. 2007 Apr-Jun

Van Biervliet S, Velde SV, Robberecht E, Van Winckel M.
Department of paediatric gastroenterology, Ghent University Hospital, Ghent, Belgium.

We describe a baby admitted with convulsions, fever, low protein level and coagulation abnormalities where congenital intestinal lymphangiectasia was confirmed by endoscopy and histology. Treatment with a low fat diet, supplemented with medium chain triglycerides (MCT), resulted in a disappearance of the symptoms and normal growth. When confronted with seizure-like attacks, electrolyte disturbances and hypo-albuminemia one should consider the possibility of protein losing enteropathy.

PMID: 17715644
PubMed - in process]


Wednesday, September 12, 2007

Clinico-pathological Characteristics of Congenital Pulmonary Lymphangiectasis: Report of Two Cases.

Clinico-pathological Characteristics of Congenital Pulmonary Lymphangiectasis: Report of Two Cases.

J Korean Med Sci. 2007 Aug

Eom M, Choi YD, Kim YS, Cho MY, Jung SH, Lee HY.

Department of Forensic Medicine, Western District Office, National Institute of Scientific Investigation, Jangseong, Korea.

Department of Pathology, Wonju College of Medicine, Yonsei University, Wonju, Korea.

Congenital pulmonary lymphangiectasis (CPL) is a rare, poorly documented disease, characterized by abnormal dilatation of pulmonary lymphatics without lymphatic proliferation. This disease is seen almost exclusively in infancy and early childhood. It can usually be divided into primary (congenital) and secondary forms. The primary form presents in neonates, and the patients mostly die due to the respiratory distress, shortly after birth. The authors experienced two cases of primary CPL in a 13-day-old male neonate and a one-day-old male neonate, showing prominent lymphatic dilatation in the septal, subpleural, and peri-bronchial tissue throughout both lungs. The latter case was associated with congenital cardiac anomaly including single ventricle. These are unique cases of CPL in Korea of which the diagnosis was established through post-mortem examination. Therefore, the authors report these two cases with primary CPL with a review of the literature.

Journal of Korean Medical Science