All issues > Volume 31(2); 1988

Original Article

J Korean Pediatr Soc. 1988;31(2):202-211. Published online February 28, 1988.

A Clinical Study and Comprehensive Total Care in Hemophilia.

Shin Heh Kang¹, Chang Hyun Yang¹, Kir Young Kim¹

¹Department of Pediatrics, Yonsei University, College of Medicine, Seoul Korea

Received: October 2, 1987;

Abstract

Hemophilia is one of the hereditary coagulation disorders characterized by deficiency in plasma clotting facotrs such as factor VIII and IX. As life long bleeding occurs in hemophiliacs, continous comprehensive total care is required for the patients. After the self therapy, home care program was introduced in the 1970s’ in the United States, there was significant reduction in absentism, hospitalized days, outpatient visits, decrease in health costs and complications including hemophilic arthropathy. The present investigation was performed to study the clinical aspects regarding severity, symp- toms, various laboratory profiles and complications on 200 hemophiliacs who had visited outpatient clinic of department of Pediatrics and Hemophilia special clinic of Severance hospital since July, 1978 till March, 1987. The results are summarized as follows; 1) 174 cases (87%) were diagnosed as hemophilia A and the remainder 26 cases (13%) as hemophilia B. 2) All patients were male and 82 cases (41%) belonged to severe form (factor less than 1 %), whereas 75 cases (37.5%) and 43 cases (21.5%) belonged to moderate form (factor 2-5%) and mild form (factor 6-30%), respectively. 3) The age at the time of diagnosis was before the age of 11 in 69%, before the age of 3 in 88%, and before the age of 5 in 92.5%. All of the severe cases were diagnosed before the age of 5. 4) Family history was positive in 122 cases (61%) and the involved members were brothers, maternal cousin, maternal uncle and maternal grandfather in descending order of frequency. There was no correlation between the presence of family history and the severity of disease. 5) The major symptoms of the patients were hemorrhagic symptoms such as easy brusing and hemarthrosis followed by prolonged bleeding after trauma and soft tissue hematoma. 6) Acute arthropathy was present in 187 cases (93.5%) and the involved joints were knee, ankle, elbow and hip in descending order of frequency. Most of the cases were managed with factor replacement and conservative care. 7) Chronic arthropathy was present in 114 cases (57%) and the incidence increased significantly with age. But there was no correlation between the presence of chronic arthropathy and the severity of disease. 8) The management of chronic alrthropathy most commonly employed was rehabilitation in 56 cases (49%), but in 58 cases (51%) no management was given at all. 9) PTT was prolonged over 40 sec in all cases and 120 cases (60%) showed severe prolongation over 80 second. 10) Liver function tests were performed in 126 cases, and elevations of SGOT and SGPT were noted in 34% and 31%, respectively. 11) HBs Ag was positive in 9 (7.4%) out of 122 cases tested an anti-HBs was positive in 100 cases (82%). About 90% had evidence of exposure to hepatitis B virus. 12) The complications were intracranial hemorrhage in 21 cases, overt hepatitis in 16 cases, GI bleeding in 15 cases and nerve palsy in 14 cases.

Keywords :Hemophilia, Arthropathy, Comprehensive total care