A first report of familial visceral myopathy with additional chylous ascites in two siblings.
BACKGROUND: Familial visceral myopathy in two siblings, involving the intestine with concurrent chylous ascites and chylous pleural effusion, is reported. In this connection a review of the recent gastroenterologic literature was performed. PATIENTS: In two siblings, admitted between 1992 and 1995 with increasing ascites, loss of weight, and attacks of subileus, the diagnosis of familial visceral myopathy was established. CASE ONE: During the initial preoperative examination hepatogenous ascites was diagnosed. Visceral myopathy and chylous ascites were subsequently established by means of an explorative laparotomy in conjunction with histologic examination of specimens taken from the small-bowel wall. CASE TWO: The patient's brother was admitted 3 years later with similar symptoms. Complaints of chylous ascites and chylous pleural effusion in connection with attacks of subileus are still present. OUTCOME: The female sibling was discharged from hospital with unchanged complaints and died only a few months later; the male sibling is still alive, but with unchanged complaints. CONCLUSIONS: Rare cases of familial and sporadic myopathy have been reported in the recent literature, but to our knowledge these are the first cases with concurrent chylous ascites and chylous pleural effusion in this uncommon disorder.[1]References
- A first report of familial visceral myopathy with additional chylous ascites in two siblings. Jatzko, G., Lisborg, P., Siebert, F., Denk, H., Klimpfinger, M. Scand. J. Gastroenterol. (1997) [Pubmed]
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