• Childhood Kidney Diseases
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• v.3 no.2
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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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• v.3 no.1
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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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• v.19 no.1
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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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• v.8 no.1
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• pp.86-90
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• 2004

Hemolytic uremic syndrome(HUS) is characterized by acute renal failure, microangiopathic hemolytic anemia, and thrombocytopenia and the most common pathogen is Escherichia coli (E. coli) O157 : H7. Ischemic colitis, which rarely occurs in children, is due to the reduced local blood flow to the intestine, tissue necrosis and secondary bacterial infection. We describe a patient who was admitted with abdominal pain, vomiting and hematochezia, and diagnosed as ischemic colitis by barium enema. This patient showed hemolytic anemia, thrombocytopenia and progressive renal failure and was subsequently diagnosed as hemolytic uremic syndrome. After hemodialysis, the patient showed improvement of symptoms and resolution of renal failure and ischemic colitis.

• Childhood Kidney Diseases
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• v.10 no.2
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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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• v.6 no.2
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• pp.237-242
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• 2002

Hemolytic Uremic Syndrome (HUS) is the most common cause of acute renal failure in children and is comprised of the combination of hemolytic anemia, thrombocytopenia, and acute renal failure. Atypical HUS, rare in childhood, has worse prognosis than that of typical HUS and is associated with chemotherapy drug, other bacterial (especially Streptococcus pneumoniae) or viral infections, and so on. We report a case of HUS caused by pneumococcal infection in 4-year-old boy. While he was admitted with pneumonia and pleural effusion, pneumococcal infection could be revealed. Although HUS progressed rapidly, he immediately received 3-time hemodialysis and recovered completely after two weeks.

• Childhood Kidney Diseases
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• v.7 no.1
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• pp.82-85
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• 2003

The hemolytic uremic syndrome(HUS), a heterogenous group of disorders characterized by micorangiopathic hemolytic anemia, thrombocytopenia, and azotemia, is the most frequent cause of acute renal failure in children. The association of the HUS with E. coli O157 : H7 has been well-described, but the other intestinal infection have been relatively less reported to date. We report a 18-month-old boy presenting with typical clinical characteristics of HUS associated with amoebic dysentery with a brief review of literatures.

• Childhood Kidney Diseases
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• v.26 no.1
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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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• v.50 no.12
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• pp.1261-1265
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• 2007

Extrarenal manifestations of hemolytic uremic syndrome (HUS) have increasingly been recognized and may be major determinants of mortality and morbidity. Although microthrombi are often found in the pulmonary and coronary circulation, apparent lung and cardiac involvement are clinically infrequent. We describe here a 10-month-old boy with HUS who developed pulmonary hemorrhage, acute respiratory distress syndrome and dilated cardiomyopathy. Complete renal as well as clinical recovery from these very uncommon complications was achieved by optimum supportive care.

• Childhood Kidney Diseases
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• v.7 no.1
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• pp.77-81
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• 2003

Diarrhea-associated hemolytic uremic syndrome is rarely accompanied by complications requiring surgical intervention. We report a case of the diarrhea-associated hemolytic uremic sydrome which developed simultaneously with an acute appendicitis.