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 3rd 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.
Acidosis ; Anti-Bacterial Agents ; Antithrombin III ; Child ; Consensus ; Coombs Test ; Dacarbazine* ; Disseminated Intravascular Coagulation ; Edema ; Emergency Service, Hospital ; Follow-Up Studies ; Hemolytic-Uremic Syndrome* ; Humans ; Hyperkalemia ; Hypertension ; Oliguria ; Pleural Effusion ; Pneumonia ; Proteinuria ; Renal Replacement Therapy ; Streptococcus pneumoniae*

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 3rd 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.
Acidosis ; Anti-Bacterial Agents ; Antithrombin III ; Child ; Consensus ; Coombs Test ; Dacarbazine* ; Disseminated Intravascular Coagulation ; Edema ; Emergency Service, Hospital ; Follow-Up Studies ; Hemolytic-Uremic Syndrome* ; Humans ; Hyperkalemia ; Hypertension ; Oliguria ; Pleural Effusion ; Pneumonia ; Proteinuria ; Renal Replacement Therapy ; Streptococcus pneumoniae*

Hemolytic anemia and thrombocytopenia are rare clinical manifestations of acute glomerulonephritis. Initially, in all such cases, a diagnosis of hemolytic uremic syndrome, thrombotic thrombocytopenic purpura, systemic lupus erythematosus, and amyloidosis should be ruled out. The presence of hemolytic anemia and thrombocytopenia is rare, but possible, in a case of acute poststreptococcal glomerulonephritis, and may result in delayed diagnosis or misdiagnosis. Correct and timely diagnosis would ensure adequate treatment in such patients. We report of a 22-month-old boy with acute glomerulonephritis coexistent with hemolytic anemia and idiopathic thrombocytopenia.
Amyloidosis ; Anemia, Hemolytic* ; Delayed Diagnosis ; Diagnosis ; Diagnostic Errors ; Glomerulonephritis* ; Hemolytic-Uremic Syndrome ; Humans ; Infant* ; Lupus Erythematosus, Systemic ; Male ; Purpura, Thrombotic Thrombocytopenic ; Thrombocytopenia*

Hemolytic anemia and thrombocytopenia are rare clinical manifestations of acute glomerulonephritis. Initially, in all such cases, a diagnosis of hemolytic uremic syndrome, thrombotic thrombocytopenic purpura, systemic lupus erythematosus, and amyloidosis should be ruled out. The presence of hemolytic anemia and thrombocytopenia is rare, but possible, in a case of acute poststreptococcal glomerulonephritis, and may result in delayed diagnosis or misdiagnosis. Correct and timely diagnosis would ensure adequate treatment in such patients. We report of a 22-month-old boy with acute glomerulonephritis coexistent with hemolytic anemia and idiopathic thrombocytopenia.
Amyloidosis ; Anemia, Hemolytic* ; Delayed Diagnosis ; Diagnosis ; Diagnostic Errors ; Glomerulonephritis* ; Hemolytic-Uremic Syndrome ; Humans ; Infant* ; Lupus Erythematosus, Systemic ; Male ; Purpura, Thrombotic Thrombocytopenic ; Thrombocytopenia*

Henoch-Schonlein purpura can result from exposure to an antigen after infection with several types of organisms. However, Henoch-Schonlein purpura caused by a primary Epstein-Barr virus infection has been rarely reported. Here, we report the case of a 32-month-old female patient who presented with Henoch-Schonlein purpura. Based on abnormal liver function test results and positive results for Epstein-Barr virus infection markers, a diagnosis of Epstein-Barr virus hepatitis manifesting as Henoch-Schonlein purpura was made. Treatment with methylprednisolone and hydration improved the symptoms, and a switch to oral steroids was effective in completely alleviating the purpura. No recurrence was noted and no liver function abnormalities were detected during the follow up period.
Arthritis ; Child, Preschool ; Diagnosis ; Female ; Follow-Up Studies ; Hepatitis* ; Herpesvirus 4, Human* ; Humans ; Liver ; Liver Function Tests ; Methylprednisolone ; Purpura ; Purpura, Schoenlein-Henoch* ; Recurrence ; Steroids

Henoch-Schonlein purpura can result from exposure to an antigen after infection with several types of organisms. However, Henoch-Schonlein purpura caused by a primary Epstein-Barr virus infection has been rarely reported. Here, we report the case of a 32-month-old female patient who presented with Henoch-Schonlein purpura. Based on abnormal liver function test results and positive results for Epstein-Barr virus infection markers, a diagnosis of Epstein-Barr virus hepatitis manifesting as Henoch-Schonlein purpura was made. Treatment with methylprednisolone and hydration improved the symptoms, and a switch to oral steroids was effective in completely alleviating the purpura. No recurrence was noted and no liver function abnormalities were detected during the follow up period.
Arthritis ; Child, Preschool ; Diagnosis ; Female ; Follow-Up Studies ; Hepatitis* ; Herpesvirus 4, Human* ; Humans ; Liver ; Liver Function Tests ; Methylprednisolone ; Purpura ; Purpura, Schoenlein-Henoch* ; Recurrence ; Steroids

PURPOSE: We evaluated the long-term results of endoscopic Deflux(R) injection for treating vesicoureteral reflux (VUR) in children. METHODS: Between September 2004 and September 2014, 243 children (137 boys and 106 girls) with a mean age of 53 months underwent Deflux(R) injection. Our clinical protocol included radionuclide voiding cystography (RNC) at postoperative 3 months, 1 year and 3 years to assess the VUR resolution. RESULTS: The cure rates at 3 months, 1 year, and 3 years by patients were 70.8%, 64.3%, and 65.6% for the total patients and 79.2%, 75.2%, and 76.4%, for the ureters, respectively. The recurrence rate of postoperative febrile urinary tract infection (UTI) was 20% in patients without VUR at postoperative 1 year. Twenty patients undergoing ureteroneocystostomy (UNC) significantly had younger age (P=0.003), higher VUR grade (P<0.001), and lower success rates of Deflux(R) injection (P<0.05). On univariate analysis, older age (P=0.014) and lower grade of VUR (P=0.031) were the significant predictors of a successful outcome. But there was none on multivariate analysis. Younger age, especially age of 0-12 month-old, was the only significant predictor of postoperative febrile UTI recurrence on both univariate and multivariate analysis. CONCLUSION: Deflux(R) injection is efficacious with a low complication rate for the anti-reflux procedure in children. There is low recurrence rate of UTI though VUR persists, and high probability of no VUR at 3 years if no VUR at 1 year. It is recommendable not to perform follow-up RNC at 3 years routinely if no VUR at 1 year.
Child* ; Clinical Protocols ; Humans ; Multivariate Analysis ; Recurrence ; Ureter ; Urinary Tract Infections ; Vesico-Ureteral Reflux*

PURPOSE: We evaluated the long-term results of endoscopic Deflux(R) injection for treating vesicoureteral reflux (VUR) in children. METHODS: Between September 2004 and September 2014, 243 children (137 boys and 106 girls) with a mean age of 53 months underwent Deflux(R) injection. Our clinical protocol included radionuclide voiding cystography (RNC) at postoperative 3 months, 1 year and 3 years to assess the VUR resolution. RESULTS: The cure rates at 3 months, 1 year, and 3 years by patients were 70.8%, 64.3%, and 65.6% for the total patients and 79.2%, 75.2%, and 76.4%, for the ureters, respectively. The recurrence rate of postoperative febrile urinary tract infection (UTI) was 20% in patients without VUR at postoperative 1 year. Twenty patients undergoing ureteroneocystostomy (UNC) significantly had younger age (P=0.003), higher VUR grade (P<0.001), and lower success rates of Deflux(R) injection (P<0.05). On univariate analysis, older age (P=0.014) and lower grade of VUR (P=0.031) were the significant predictors of a successful outcome. But there was none on multivariate analysis. Younger age, especially age of 0-12 month-old, was the only significant predictor of postoperative febrile UTI recurrence on both univariate and multivariate analysis. CONCLUSION: Deflux(R) injection is efficacious with a low complication rate for the anti-reflux procedure in children. There is low recurrence rate of UTI though VUR persists, and high probability of no VUR at 3 years if no VUR at 1 year. It is recommendable not to perform follow-up RNC at 3 years routinely if no VUR at 1 year.
Child* ; Clinical Protocols ; Humans ; Multivariate Analysis ; Recurrence ; Ureter ; Urinary Tract Infections ; Vesico-Ureteral Reflux*

NPHP is the most common monogenic cause of CKD in children or adolescents. Extra-renal symptoms often accompany, therefore examination of retina, hearing, and skeleton is necessary in patients with CKD with insidious onset. Genes involved in NPHP-RC are mostly related in primary cilia. While genetic diagnosis is necessary for definitive diagnosis, there is no curative treatment.
Adolescent ; Child ; Cilia ; Diagnosis ; Hearing ; Humans ; Renal Insufficiency, Chronic ; Retina ; Skeleton

NPHP is the most common monogenic cause of CKD in children or adolescents. Extra-renal symptoms often accompany, therefore examination of retina, hearing, and skeleton is necessary in patients with CKD with insidious onset. Genes involved in NPHP-RC are mostly related in primary cilia. While genetic diagnosis is necessary for definitive diagnosis, there is no curative treatment.
Adolescent ; Child ; Cilia ; Diagnosis ; Hearing ; Humans ; Renal Insufficiency, Chronic ; Retina ; Skeleton