• Neonatal Medicine
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• 제15권2호
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• pp.190-195
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• 2008

저자들은 항-E항체에 의한 신생아 동종 면역성 용혈성 질환 환아에서 생후 8일째 담낭내 오니가 있고 4개월, 9개월, 11개월 및 50개월 후 추적 초음파에서 담석을 보인 1례를 경험하였기에 보고하는 바이다

• Childhood Kidney Diseases
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• 제8권1호
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• pp.86-90
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• 2004

저자들은 복통과 구토, 혈변을 주소로 내원한 환아의 바륨 대장 조영술에서 무지문양(thumb-printing)을 확인하여 허혈성 대장염 진단하에 치료 중 미세혈관성 용혈성 빈혈과 혈소판 감소, 전해질 불균형과 급격한 소변량 감소의 급성 신부전 소견을 확인하고 허혈성 대장염에 동반된 용혈성 요독 증후군을 경험하였기에 문헌고찰과 함께 보고하는 바이다.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• 제9권1호
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• pp.108-113
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• 2006

윌슨병에서 용혈성 빈혈과 전격성 간부전이 동반되면 혈장교환술이나 간이식이 필수적이다. 저자들은 간염과 용혈위기가 동반된 전격성 윌슨병 환아에서 혈장교환술을 계속하였으나 호전을 보이지 않아 교환수혈을 시행한 결과 용혈위기를 극복하였다. 현재까지 약물치료와 혈장교환술에 뒤이은 간이식이 일차 치료로 되어 있지만, 전격성 간부전이 응급으로 간이식을 해야 할 만큼 심하지 않은 경우에는 혈장교환술후 교환수혈을 시도할 수 있을 것으로 생각한다.

• Clinical Psychopharmacology and Neuroscience
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• 제16권4호
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• pp.501-504
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• 2018

Autoimmune hemolytic anemia is a disease characterized with destruction of erythrocytes as a result of antibody produce against patient's own erythrocytes and anemia. Autoimmune hemolytic anemia can be roughly stratified into two groups according to serological features and secondary causes including drugs induced hemolytic anemia. Drugs induced autoimmune hemolytic anemia is very rare in pediatric patients. Even though hematological side effects such as leucopenia, agranulocytosis, eosinophilia, thrombocytopenic purpura and aplastic anemia might occur due to psychotropic drug use; to the best of our knowledge there is no autoimmune hemolytic anemia case due to quetiapine, an atypical antipsychotics, in literature. We hereby describe the first child case of autoimmune hemolytic anemia during quetiapine treatment.We also are pointing out that one should keep in mind serious hematological side effects with atypical antipsychotic drug use with this case report.

• Clinical and Experimental Pediatrics
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• 제46권7호
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• pp.718-721
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• 2003

저자들은 신생아 용혈성 질환을 일으킨 아기를 분만한 산모와 신생아에서 $anti-Jk^b$를 동정하여 진단하였으며, 이에 대해 그 임상경과를 문헌고찰과 함께 보고하는 바이다. 따라서, 생후 24시간이내 신생아 황달이 있는 경우 군소 혈액형 부적합증에 의한 신생아 용혈성 질환을 감별하여야 하며, 불규칙항체 선별 검사나 불규칙항체 동정 검사를 통하여 항체를 규명하고, 광선요법이나 교환수혈 등을 실시하여 핵황달 예방에 주의함과 동시에 지연성 빈혈이 없는지 지속적인 관찰이 필요할 것으로 사료된다.

• Journal of Yeungnam Medical Science
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• 제30권1호
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• pp.21-24
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• 2013

Hemolytic disease in a newborn that causes early jaundice is common. It is often due to the Rh (D) and ABO incompatibility, but rarely due to unexpected antibodies. Among these unexpected antibodies, the anti-$Di^a$Dia antibody rarely occurs. The anti-$Di^a$ antibody was observed in the serum and red-cell eluate of an infant, and in the serum of his mother. The frequency of the appearance of the $Di^a$ antigen in the Korean population is estimated to be 6.4-14.5%. This paper reports a case of hemolytic disease in a newborn associated with the anti-$Di^a$ antibody. A full-term male infant was transferred to the authors' hospital due to hyperbilirubinemia the day after his birth. The laboratory data indicated a hemoglobin value of 11.6 g/dL, a reticulocyte count of 10.6%, a total bilirubin count of 14.4 mg/dL, a direct bilirubin count of 0.6 mg/dL, and a positive result in the direct Coombs' test. Due to the identification of an irregular antibody from the maternal serum, an anti-$Di^a$ antibody was detected, which was also found in the eluate made from the infant's blood. The infant had been treated with phototherapy and intravenous immunoglobulin since the second day after his birth and was discharged due to an improved condition without exchange transfusion. Therefore, in cases of iso-immune hemolytic disease in a newborn within 24 hours from birth who had a negative result in an antibody screening test, the conduct of an anti-$Di^a$ antibody identification test is recommended due to the suspicion of an anti-$Di^a$ antigen, followed by early administration of intravenous immunoglobulin.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• 제23권2호
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• pp.180-187
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• 2020

Giant cell hepatitis with autoimmune hemolytic anemia (AHA) is a rare disease of infancy characterized by the presence of both Coombs-positive hemolytic anemia and progressive liver disease with giant cell transformation of hepatocytes. Here, we report a case involving a seven-month-old male infant who presented with AHA followed by cholestatic hepatitis. The clinical features included jaundice, pallor, and red urine. Physical examination showed generalized icterus and splenomegaly. The laboratory findings suggested warm-type AHA with cholestatic hepatitis. Liver biopsy revealed giant cell transformation of hepatocytes and moderate lobular inflammation. The patient was successfully treated with four doses of rituximab. Early relapse of hemolytic anemia and hepatitis was observed, which prompted the use of an additional salvage dose of rituximab. He is currently in clinical remission.

• Clinical and Experimental Pediatrics
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• 제61권2호
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• pp.37-42
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• 2018

Hemolytic uremic syndrome (HUS) is often encountered in children with acute kidney injury. Besides the well-known shiga toxin-producing Escherichia coli-associated HUS, atypical HUS (aHUS) caused by genetic complement dysregulation has been studied recently. aHUS is a rare, chronic, and devastating disorder that progressively damages systemic organs, resulting in stroke, end-stage renal disease, and death. The traditional treatment for aHUS is mainly plasmapheresis or plasma infusion; however, many children with aHUS will progress to chronic kidney disease despite plasma therapy. Eculizumab is a newly developed biologic that blocks the terminal complement pathway and has been successfully used in the treatment of aHUS. Currently, several guidelines for aHUS, including the Korean guideline, recommend eculizumab as the first-line therapy in children with aHUS. Moreover, life-long eculizumab therapy is generally recommended. Further studies on discontinuation of eculizumab are needed.

• Pediatric Infection and Vaccine
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• 제5권2호
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• pp.302-307
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• 1998

Hemolytic anemia due to cold agglutinin disease is a known complication of Mycoplasma pneumoniae infection but is rarely observed, particularly in children. A case of Mycoplasma pneumonia complicated with hemolytic anemia is presented. A 7 year-old girl was adimitted because of fever, cough, sputum and pale appearance. Chest X-ray showed pneumonic consolidation of Rt. upper lobe, lingular division. Laboratory studies disclosed the following values : Hb 5.3g/dL, Hct 11.1%, reticulocyte 2.9%, indirect Coombs test negative, direct Coombs test(monovalent) Anti-C3d positive, Anti-IgG negative, Anti-IgM negative, cold agglutinin titer 1 : 256, mycoplasma antibody titer 1 : 640, total bilirubin 1.0mg/dL. Initial PBS before wanning showed agglutination of red blood cells. The diagnosis of cold agglutinin hemolytic anemia complicating mycoplasma pneumonia was made. And treatment with roxithromycin, prednisolone and avoiding cold exposure was initiated, and complete recovery ensued. We report a case of cold agglutinin hemolytic anemia complicating mycoplasma pneumonia in children.

• Childhood Kidney Diseases
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• 제26권1호
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• pp.58-62
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• 2022

Atypical hemolytic uremic syndrome (aHUS) is a rare disease characterized by microangiopathic hemolytic anemia, thrombocytopenia, and acute kidney injury without any association with preceding diarrhea. Dysregulation of the complement system is the most common cause of aHUS, and monoclonal humanized anti-C5 antibodies are now recommended as the first-line treatment for aHUS. However, if the complement pathway is not the cause of aHUS, C5 inhibitors are ineffective. In this study, we report the second reported case of aHUS caused by DGKE mutations in Republic of Korea. The patient was an 11-month-old infant who presented with prodromal diarrhea similar to typical HUS, self-remitted with conservative management unlike complement-mediated aHUS but recurred with fever. While infantile aHUS often implies genetic dysregulation of the complement system, other rare genetic causes, such as DGKE mutation, need to be considered before deciding long-term treatment with C5 inhibitors.