| A Clinical Study of Hereditary Spherocytosis. |
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Ki Ho Kim, Kun Soo Lee, Haeng Mi Kim, Doo Hong Ahn |
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Department of Pediatrics, School of Medicine, Kyungpook National University, Taegu, Korea |
| 유전성구상적혈구증에 대한 임상적 고찰 |
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김기호, 이건수, 김행미, 안두홍 |
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경북대학교 의과대학 소아과학교실 |
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Received: 11 May 1989 • Accepted: 24 July 1989 |
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| Abstract |
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The following results were obtained from eight cases of hereditary spherocytosis (HS) who were
admitted at the Department of Pediatrics, Kyung-pook National University Hospital from July 1984
to January 1989.
The percentage of HS among the pediatric hematology patients during the same period was 2.6%.
Male amd female were one and seven respectively.
The mean age at the diagnosis and at onset of symptom was 7.1 years (8 months-13.4 years) and
4.9 years (2 months-10.4 years). The duration from initial symptom to diagnosis was 2.2 years (5
months-5 years).
The chief complaint was jaundice in 2, hepatosplenomegaly in 2, splenomegaly in 1, easy
fatigability in 1, and pallor in 1.
The liver and spleen were enlarged in 6 cases with 2.2 cm and 5.3 cm below the costal margin
respectively.
The mean hemoglobin was 8.2 g/dl, the reticulocyte count 12.3%, the mean reticulocyte production
index 4.4, the mean MCV 78u3, the mean MCH 28 pg, the mean MCHC 34 g/dl. The mean spherocyte
was 33% and the mean bilirubin 2.4 mg% with indirect predominant. The bone marrow aspiration
was done in 7 cases and showed erythroid hyperplasia in all.
In osmotic fragility test, the hemolysis was started in the mean concentration of 0.7%NaCl solution
and was completed in 0.48%. The autohemolysis test at 48 hours showed 9.8%, 4.2%, 2.6%, 6.6%,
40.8%, and 60.8% of hemolysis in 6 cases and showed partial correction by adding glucose in 2 cases.
Aplastic crisis was deveploped in one case and was recovered with packed cell transfusion after
several days.
Splenectomy was performed in 5 cases (10,11,13.4,5.9, and 10.6 years old) and cholecystectomy was
done in one of five concomitantly. |
| Key Words:
Hereditary Spherocytosis, Reticulocyte production index, Aplastic crisis, Splenectomy,
Cholecystectomy |
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