All issues > Volume 27(6); 1984

Case Report

J Korean Pediatr Soc. 1984;27(6):628-634. Published online June 30, 1984.

Two Cases of Gaucher's Disease in Brothers.

Jeong Sick Min¹, Il Whan Kim¹, Dae Young Hwang¹, Hyun Gi Jeong¹, Jae Sun Park¹, In Sun Jun², Man Ha Huh²

¹Department of Pediatrics,Kosin Medical College, Pusan Korea
²Department of Pathology, Kosin Medical College, Pusan Korea

Abstract

We experienced 2 cases of Gaucher' s disease of adult type in brothers when their ages were 4 year 1 month and 3 year 10 month respectively. They showed remarkable hepatosplenomegaly with mild hematologic complications and characteristic histiocytoid cells in the spleen and liver biopsies. Splenectomy was followed by improvement of anemia and labored respiration in both patients. In case 1, however, two episode of fever and leg pain with suspected aseptic osteomyelitis and increased hepatomegaly was noticed after splenectomy. A brief review of literature was made.

Keywords :Hepatosplenomegaly; Anemia, Gaucher' s disease; Splenectomy