Alpha-Chain Disease Causing Kaliopenic Nephropathy and Fatal Intestinal Perforations

Overview

Journal Am J Dig Dis

Specialty Gastroenterology

Date 1976 Jul 1

PMID 821342

Authors

J C Economidou

O N Manousos

D Katsaros

Affiliations

Soon will be listed here.

Abstract

A case of alpha-chain disease (alpha-CD) is described presenting as a malabsorption syndrome complicated by severe kalipenic nephropathy. Antibiotics and cyclophosphamide were effective in controlling the malabsorption and thr renal dysfunction but did not affect the basic pathological lesion. Eventually the disease became generalized, affected lymph nodes, liver and bone marrow and caused multiple intestinal perforations. Detailed histological studies failed to demonstrate the development of reticulum-cell sarcoma as a terminal complication of the disease and are suggestive of a single malignant plasmacytic cell process.

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