• 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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• 제25권1호
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• pp.14-21
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• 2021

Chronic kidney disease, the presence of structural and functional abnormalities in the kidneys, is associated with a lower quality of life and increased morbidity and mortality in children. Genetic etiologies account for a substantial proportion of pediatric chronic kidney disease. With recent advances in genetic testing techniques, an increasing number of genetic causes of kidney disease continue to be found. Genetic testing is recommended in children with steroid-resistant nephrotic syndrome, congenital malformations of the kidney and urinary tract, cystic disease, or kidney disease with extrarenal manifestations. Diagnostic yields differ according to the category of clinical diagnosis and the choice of test. Here, we review the characteristics of genetic testing modalities and the implications of genetic testing in clinical genetic diagnostics.

• Clinical and Experimental Pediatrics
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• 제57권2호
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• pp.96-99
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• 2014

Hemolytic uremic syndrome (HUS) is one of the most common causes of acute renal failure in childhood and is primarily diagnosed in up to 4.5% of children who undergo chronic renal replacement therapy. Escherichia coli serotype O157:H7 is the predominant bacterial strain identified in patients with HUS; more than 100 types of Shiga toxin-producing enterohemorrhagic E. coli (EHEC) subtypes have also been isolated. The typical HUS manifestations are microangiopathic hemolytic anemia, thrombocytopenia, and renal insufficiency. In typical HUS cases, more serious EHEC manifestations include severe hemorrhagic colitis, bowel necrosis and perforation, rectal prolapse, peritonitis, and intussusceptions. Colonic perforation, which has an incidence of 1%-2%, can be a fatal complication. In this study, we report a typical Shiga toxin-associated HUS case complicated by small intestinal perforation with refractory peritonitis that was possibly because of ischemic enteritis. Although the degree of renal damage is the main concern in HUS, extrarenal complications should also be considered in severe cases, as presented in our case.