• Pediatric Gastroenterology, Hepatology & Nutrition
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• 제17권4호
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• pp.266-269
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• 2014

Gilbert syndrome is the most common inherited disorder of bilirubin glucuronidation. It is characterized by intermittent episodes of jaundice in the absence of hepatocellular disease or hemolysis. Hereditary spherocytosis is the most common inherited hemolytic anemia and is characterized by spherical, osmotically fragile erythrocytes that are selectively trapped by the spleen. The patients have variable degrees of anemia, jaundice, and splenomegaly. Hereditary spherocytosis usually leads to mild-to-moderate elevation of serum bilirubin levels. Severe hyperbilirubinemia compared with the degree of hemolysis should be lead to suspicion of additional clinical conditions such as Gilbert syndrome or thalassemia. We present the case of a 12-year-old boy with extreme jaundice and nausea. The diagnosis of hereditary spherocytosis was confirmed by osmotic fragility test results and that of Gilbert syndrome by genetic analysis findings.

• Pediatric Infection and Vaccine
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• 제16권2호
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• pp.215-219
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• 2009

5-14세에 호발하는 마이코플라즈마 폐렴은 20-25%에서 폐외 증상을 일으키는 것으로 알려져 있으며 이 중 혈액계 질환에는 용혈성 빈혈, 혈소판 감소증, 혈구포식세포 증식증(hemophagocytosis) 등이 있다. 마이코플라즈마 폐렴에서의 냉항체에 의한 자가면역성 용혈로 용혈성 빈혈이 발생할 수 있으며, 이 때 항체의 역가와 용혈의 정도에 상관관계가 있을 수 있다. 저자들은 마이코플라즈마 폐렴에 의해 용혈성 빈혈이 발현된 유전성 구상 적혈구증 1례를 경험하였기에 보고하는 바이다.

• Journal of Chest Surgery
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• 제46권2호
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• pp.156-158
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• 2013

Thoracic extramedullary hematopoiesis (EMH) is a rare disease entity that is usually associated with hematologic disorders, such as myelodysplastic or hemolytic disease. Because thoracic EMH is usually encountered as a mass during radiologic examinations, it should be differentiated from posterior mediastinal neurogenic tumors. Here, the authors report a case of EMH associated with hereditary spherocytosis. The patient underwent a complete excision by thoracoscopic surgery to differentiate it from other mediastinal tumors.

• Journal of Yeungnam Medical Science
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• 제11권1호
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• pp.42-48
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• 1994

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 $8.1{\pm}2.4gm/dl$, 헤마토크리트 $23.4{\pm}10.2%$ 망상적혈구 $15.9{\pm}11.7%$이었으며, 삼투압 취약성은 모두에서 증가되어 있었다. 혈청 전 빌리루빈 $8.6{\pm}10.9mg/dl$, LDH $370.1{\pm}169.1IU/L$으로 증가되었다. Coombs 검사상 9례 모두 음성반응을 보였다. 5. 9례 모두 비장적출술을 시행하였으며 이들중 담석증을 동반한 1례에서는 담낭절제술, 총담관 결석을 동반한 1례에서는 담낭절제술, 총담관 결석제거술 및 T-tube 담관조루술을 동시에 시행하였다. 수술 소견상 비장의 무게는 350(150-600)gm이었고, 9례중 3례에서 부비장(accessory spleen)이 관찰되었다. 6. 비장 적출후 유전성 구상 적혈구증 9례에서 수술전 혈색소 $8.1{\pm}2.4gm/dl$, 헤마토크리트 $23.4{\pm}10.2%$, 수술후 혈색소 $12.3{\pm}1.2gm/dl$, 헤마토크리트 $37.0{\pm}5.3%$로 증가되어 술후 빈혈이 교정되었음을 관찰할 수 있었다. 수술후에 수혈의 필요성은 없었으며, 술후 특별한 합병증없이 9명 모두 현재까지 건강하게 잘 자라고 있다.

• Clinical and Experimental Pediatrics
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• 제46권11호
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• pp.1139-1142
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• 2003

저자들은 유전성 구상적혈구증으로 추적 관찰 중인 7세 남아와 용혈성빈혈의 기왕력이 없던 11세, 8세 환아가 심한 범혈구 감소증으로 내원하여 parvovirus B19 감염에 의한 골수무형성 위기로 진단하고 수혈 및 정주용 면역글로불린으로 1-2주만에 모두 호전된 유전성 구상적혈구증 환아 3례를 보고하는 바이다.

• Clinical and Experimental Pediatrics
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• 제50권6호
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• pp.511-518
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• 2007

To understand the hemolytic anemia (HA) in children, the diagnostic approach and management of hereditary and acquired HA are described. The hereditary hemolytic anemia (HHA) can be classified according to the pathogenesis into three types : RBC membrane defects, hemoglobinopathies, and RBC enzymopathies. Clinical characteristics, laboratory findings and molecular defects of these three types are presented briefly. In Korea, HHA due to the RBC membrane defect, hereditary spherocytosis had been reported often but HHA due to hemoglobinopathies and RBC enzymopathies had been thought to be relatively rare. With recent development in the molecular diagnosis, ${\beta}$ thalassemia, mostly heterozygote, G6PD and pyruvate kinase deficiency have been reported with gene characterization. If the patients with microcytic hypochromic anemia show unproportionally low MCV or MCH or refractory to the iron therapy, hemoglobin electrophoresis and gene analysis for thalassemia or other unstable hemoglobinopathies need to be done accordingly. The global movement of the population especially from the region prevalent of hemoglobinopathies or enzymopathies to Korea warrants considering broad spectrum of etiology for the diagnosis of HHA. Aquired HA resulting from extracellular factors such as autoimmune HA from warm antibody, cold agglutinin and paroxysmal cold hemoglobinuria as well as nonimmune HA are described briefly.

• Advances in pediatric surgery
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• 제3권2호
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• pp.160-163
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• 1997

Three cases of laparoscopic splenectomy for hereditary spherocytosis are reported. The average operation time was 100 minutes. This was longer than traditional open splenectomy for the same entity(63 minutes). Average hospitalization period was 3 days. This was shorter than the hospitalization period for the traditional group(6.2 days).

• Advances in pediatric surgery
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• 제5권1호
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• pp.53-57
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• 1999

Pediatric laparoscopic splenectomy has been gradually accepted as the surgical management of a various splenic disorders, particularly in hematologic diseases. We report our experience with 16 patients who underwent this procedure because of hematologic disorders during the past 3 and a half years at the Department of Surgery, St. Mary's Hospital, the Catholic University Medical College. The mean age was 10 years(range 6-16 years) and the mean spleen weight was 210 gm(range 85-500 g). The indication for splenectomy were hereditary spherocytosis(6 cases), idiopathic thrombocytopenic purpura(8 cases), autoimmune hemolytic anemia(1 case), and idiopathic splenomegaly(1 case). All splenectomies were performed safely with mean estimated blood loss of 233 ml. Mean operative time and mean postoperative hospital stay were 157 min and 4.5 days, respectively. Postoperative pain, medication was needed in 3 cases, just one injection in immediate postoperative period. Diet was started on the second or third postoperative day. In conclusion, laparoscopic splenectomy in pediatric patients is a safe procedure, offering a small of abdominal scar, much less pain, a shorter hospital stay and car the lower postoperative morbidity.

• Advances in pediatric surgery
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• 제10권1호
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• pp.31-34
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• 2004

The laparoscopic splenectomy (LS) became popular over the last 10 years. The advantage of LS over open splenectomy (OS) includes short hospital stay, improved cosmesis, less development of postoperative intestinal ileus, and less analgesics required. The purpose of this study is to evaluate the outcome of LS at Asan Medical Center from January 1999 to January 2003. The records of 14 consequent children who underwent splenectomy were reviewed retrospectively. Patients characteristics, morbidity, mortality, operative time, blood loss, and hospital stay were analyzed., Seven patients age 5 to 15 years underwent LS under the indications: idiopathic thrombocytic purpura (ITP, n=3), hereditary spherocytosis (n=3), and myelodysplastic syndrome (n=l). Seven patients, age 7 to 16 years, underwent OS during the same period for ITP (n=7). Median operative time was 120 mInutes (80 to 170 mins.) in OS, and 270 minutes (110 to 480 mins,) in LS (p<0.05). Median length of hospital stay was 6 days (3 to 8) in OS, and 4 days (3 to 6) in LS (p>0.05). Median splenic length was 12.0cm (9.2 to 18.0) in OS, 14.0 cm (10.0 to 19.5) (p>0.05). Accessory spleens were identified in 3 of 7 LS and 1 of 7 OS cases. In the LS group, there was no conversion to open surgery. Two patients in LS required blood transfusion postoperatively. LS in children can be performed as effectively and safely as OS.

• Pediatric Infection and Vaccine
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• 제27권2호
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• pp.111-116
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• 2020

목적: 인간 파르보바이러스 B19 감염은 무증상에서 생명을 위협하는 합병증에 이르기까지 광범위하고 다양한 증상을 나타낸다. 우리는 파르보바이러스 B19 감염의 지역사회 내 집단발생 시기 동안, 파르보바이러스 B19 감염의 다양한 임상 양상에 대해 조사하였다. 방법: 2017년 12월부터 2019년 4월까지 부산대학교 어린이병원에서 파르보바이러스 B19 면역 글로불린 M (IgM) 또는 중합효소 연쇄 반응(polymerase chain reaction, PCR)에 의해 검출된 파르보바이러스 B19 DNA를 가진 소아의 임상적 특징을 후향적 차트 분석을 통해 알아보았다. 결과: 24명의 어린이가 파르보바이러스 B19 감염으로 진단되었다. 24명의 환자 중 12명(50%)은 레이스 형태 발진이 있었고, 24명의 환자 중 4명(16.7%)은 점상출혈 발진, 24명의 환자 중 2명(8.3%)은 무병소 발열로 진단되었다. 24명 중 6명의 환자(25%)는 골수무형성위기가 발생하였고, 이 중 3명은 이전에 유전 구형적혈구증, 3명은 이전에 급성 림프모구 백혈병으로 진단되었던 환자이다. 결론: 감염홍반 외에도 파르보바이러스 B19 감염의 임상적 특징은 다양한 종류의 발진과 무병소 발열으로 나타날 수 있다. 또한, 혈액학적 증상으로 중성구 감소증, 골수무형성 위기와 같이 발생할 수 있다.