• Childhood Kidney Diseases
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• 제3권2호
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• pp.227-231
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• 1999

Hemolytic uremic syndrome is a clinical syndrome with various etiology and pathogenesis. And pneumococcal neuraminidase has been known to play a pathogenetic role in some cases with this syndrome. We experienced two children with hemolytic uremic syndrome complicated by pneumococcal infection. One was 21-month-old girl with pneumococcal pneumonia, and the other was 7-month-old girl with pneumococcal meningitis and sepsis. Both of them showed typical clinical manifestations of hemolytic uremic syndrome with prolonged anuria during the course of pneumococcal infection. The renal functions of both cases did not recovered after resolution of acute hemolytic episode and chronic renal failure developed.

• Childhood Kidney Diseases
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• 제3권1호
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• pp.104-108
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• 1999

Atypical hemolytic uremic syndrome associated with neuraminidase-producing Streptococcus pneumoniae usually associated with invasive infection such as fulminant pneumonia, sepsis, and meningitis and may occur earlier in lift and has a higher mortality rate than typical hemolytic uremic syndrome. We have experienced a 22-month-old female patient with hemolytic uremic syndrome associated with S. pneumoniae pneumonia and empyema. The patient was treated with ceftriaxone and washed red blood cell transfusion. As the disese course could be aggravated by the use of blood products containing anti-Tomsen-Friedenreich antigen, early recognition and sensible use of blood products such as washed RBC might lead to the improved outcome.

• Childhood Kidney Diseases
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• 제19권1호
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• pp.48-52
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• 2015

Streptococcus pneumoniae associated hemolytic uremic syndrome (SpHUS) is one of the causes of atypical hemolytic uremic syndrome, and increasingly reported. They are more severe and leave more long-term sequelae than more prevalent, typical hemolytic uremic syndrome. But it is not so easy to diagnose SpHUS for several reasons (below), and there was no diagnostic criteria of consensus. A 18 month-old-girl with sudden onset of oliguria and generalized edema was admitted through the emergency room. She had pneumonia with pleural effusion and laboratory findings of HUS, DIC, and positive direct Coombs' test. As DIC or SpHUS was suspected, we started to treat her with broad spectrum antibiotics, transfusion of washed RBC and replacement of antithrombin III. On the $3^{rd}$ day, due to severe hyperkalemia and metabolic acidosis, continuous renal replacement therapy (CRRT) was started. She showed gradual improvement in 4 days on CRRT and discharged in 16 days of hospital care. At the follow up to one year, she has maintained normal renal function without proteinuria and hypertension. We report this case with review of articles including recently suggested diagnostic criteria of SpHUS.

• 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.

• Childhood Kidney Diseases
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• 제10권2호
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• pp.249-256
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• 2006

Diarrhea-associated hemolytic uremic syndrome(D+ HUS) is induced by enterohemorrhagic Escherichia coli(EHEC) and is characterized by the triad of microangiopathic hemolytic anemia, thrombocytopenia, and acute renal failure. The disease is usually transmitted by meat and water contaminated by excreta of domestic animals. We report a son and his mother with diarrhea-associated hemolytic uremic syndrome that spread within the family.

• Childhood Kidney Diseases
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• 제6권2호
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• pp.237-242
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• 2002

용혈성 요독 증후군은 용혈성 빈혈, 혈소판 감소증, 급성신부전의 임상양상을 보이는 질환으로서 전형적인 경우 EHEC에 의해 유발되며, 설사(특히 혈변) 등의 전구증상과 연관된다. 비전형적으로 드물게 폐구균 감염으로도 유발되며, 이 경우 매우 불량한 예후를 보이는 것으로 알려져 있고, 아직 국내엔 폐구균 감염과 연관된 HUS에 대해 보고된 바가 없다. 저자들은 폐구균 감염이 진단된 후 급격히 심한 비전형적 용혈성 요독 증후군이 진행되었으나 성공적으로 치료한 1례를 경험하였기에 보고하는 바이다.

• Childhood Kidney Diseases
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• 제7권1호
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• pp.82-85
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• 2003

저자들은 18개월된 남자 환아에서 아메바성 장염 후 급성 신부전, 미세혈관성 용혈성 빈혈, 혈소판 감소증의 전형적 용혈성 요독 증후군이 발생한 증례를 경험하였기에 문헌 고찰과 함께 보고하는 바이다.

• 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.

• Clinical and Experimental Pediatrics
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• 제50권12호
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• pp.1261-1265
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• 2007

근래 용혈요독증후군의 신외 증상에 대한 인식이 점점 더 증가하고 있으며 이환률과 사망률의 주요 결정인자가 되고 있다. 용혈요독증후군 환자의 심장동맥순환계에서 미소혈전이 발견되는 일은 흔하지만 실제 임상적으로 명백하게 발현하는 폐 혹은 심장 합병증은 흔하지 않다. 저자들은 용혈요독증후군으로 치료 받던 10개월 영아에서 폐출혈, 급성호흡곤란증후군, 확장심근병증 등이 발생하여 지지요법 후 회복된 1례를 경험하여 문헌고찰과 함께 보고하는 바이다.

• Childhood Kidney Diseases
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• 제7권1호
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• pp.77-81
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• 2003

설사연관형 용혈성 요독 증후군은 수술적 치료가 필요한 외과적 합병증이 드물게 동반된다. 저자들은 급성 출혈성 장염으로 진단된 후 동시에 설사 연관형 용혈성 요독 증후군과 급성 충수염으로 진행한 1례를 경험하였기에 보고하는 바이다.