Journal of Yeungnam Medical Science
- 제11권1호
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- Pages.42-48
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- 1994
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- 2799-8010(eISSN)
소아에서 유전성 구상 적혈구증의 비장 적출술
Splenectomy in Hereditary Spherocytosis in Childhood
- 허영수 (영남대학교 의과대학 외과학교실) ;
- 김창식 (영남대학교 의과대학 외과학교실) ;
- 도병수 (영남대학교 의과대학 외과학교실) ;
- 서보양 (영남대학교 의과대학 외과학교실) ;
- 하정옥 (영남대학교 의과대학 소아과학교실)
- Huh, Young-Soo (Department of Surgery, College of Medicine, Yeungnam University) ;
- Kim, Chang-Sig (Department of Surgery, College of Medicine, Yeungnam University) ;
- Do, Byung-Soo (Department of Surgery, College of Medicine, Yeungnam University) ;
- Suh, Bo-Yang (Department of Surgery, College of Medicine, Yeungnam University) ;
- Hah, Jeong-Ok (Department of Pediatrics, College of Medicine, Yeungnam University)
- 발행 : 1994.06.30
초록
저자들은 1987년 12월부터 1993년 8월까지 영남대학교 의과대학 부속병원에 입원하여 유전성 구상 적혈구증으로 진단받고 비장적출술을 시행한 9례를 대상으로 임상분석하여 다음과 같은 결론을 얻었다. 1. 총 9명중 남아 5명, 여아 4명으로 남녀비가 비슷하였으며, 6세에서 10세 사이가 9례중 5례로 전체의 반이상을 차지하였다. 2. 가족력을 나타낸 경우는 9례중 4례(44.4%)이었다. 3. 주요임상 증상으로는 빈혈, 황달 및 비장종대가 주증상이었으며, 담석증 및 총담관결석을 동반하였던 2례에서는 우상복부 동통을 나타내었다. 4. 검사실 소견으로는 말초혈액 도말검사상 구상 적혈구를 발견할 수 있었으며, 혈색소
Among the erythrocyte membrane defects, hereditary spherocytosis is the most common. The erythrocyte membrane defect results from a deficiency of spectrin, the most important structural protein in red cell. Hereditary spherocytosis often presents with hemolytic anemia, jaundice, moderate splenomegaly. Diagnosis is established by the presence of spherocytes in the peripheral blood, reticulocytosis, an increased osmotic fragility, and a negative Coombs test. In children, splenectomy is usually performed after age 6 years but can be done at a younger age if warranted by the severity of the anemia and the need for frequent transfusions. In the period December 1987 to Agust 1993, 9 patients with hereditary spherocytosis underwent splenectomy and the following results were obtained. 1. Nine patients were comprised of five males and four females. 2. Five patients(55.6%) had been admitted to our hospital during age 6-10 years. 3. Four of the nine patients had autosomal dominant inheritance with variable expression. The other five patients had no known inheritance. 4. The diagnosis of the spherocytosis was based on the increased osmotic fragility and increased autohemolysis of the erythrocytes, as well as on the appearance of spherocytes in the peripheral blood smear. 5. In all cases splenectomy was performed. Two patients had concomitant gall stones and choledocholithiasis, respectively. One patient with concomitant gall stones underwent simultaneous cholecystectomy and splenectomy. The other patient associated with choledocholithiasis underwent splenectomy, cholecystectomy, choledocholithotomy, and T-tube drainage. 6. Complete hematologic recovery was obtained by the splenectomy in all cases. 7. Postoperative complication was not occurred.