• Childhood Kidney Diseases
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• v.9 no.1
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• pp.31-37
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• 2005

Puruose : Thin glomerular basement membrane disease(TGBMD) is found in patients with family history of hematuria. TGBMD is autosomal dominant and is known to be one of the commonest causes of asymptomatic hematuria. This study was conducted to evaluate the histological and clinical features of patients with TGBMD. Methods : 150 cases diagnosed with TGBMD by renal biopsy while admitted in the department of pediatrics, Kyungpook National University Hospital between January 1999 and December 2003 comprised the study group. The following parameters were retrospectively anaIyzed age of onset, hematuria pattern, existence of proteinuria, process of diagnosis, laboratory findings, thickness and character of basement membrane and family history. Results : The mean age at the time of diagnosis was 7.9 years. The male to female ratio was 65:77. 94 patients or 66% visited the hospital with a chief complaint of persistent microscopic hematuria. Gross hematuria accounted for 13 cases or 9%. 78 cases(55%) were found to have hematuria for the first time from a routine school urinalysis screening. The renal biopsy showed the thickness of basement membrane to be 186$\pm$36 nm. Focal lamellation of the basement membrane was found in eight cases. In the family history, hematuria was shown in 10 cases on the Paternal side, 13 on The maternal side and none on both sides. In seven cases, hematuria was shown among siblings. No significant differences were found among the laboratory test results which were conducted at an average interval of fifteen months. Conclusion : TGBMD is one of the major causes of asymptomatic hematuria in children, which was diagnosed in increasing numbers since school urinary mass screening test started in 1998. In cases with familial progressive renal disease or focal duplication in the basement membrane Alport syndrome should be considered.

• Childhood Kidney Diseases
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• v.8 no.2
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• pp.223-228
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• 2004

Purpose and Methods: Urinary tract infection(UTI) is one of the most important diseases of childhood, especially for young infants. To characterize the patients diagnosed with febrile UTI in their first 6 months of life and to explore the risk factors of recurrent UTI, a retrospective study was performed. Results: Among the 90 patients studied, 77 were boys(86%). First episodes of UTI were diagnosed at the age of $2.5{\pm}1.4$ months. These patients underwent ultrasonographic evaluation of urinary tract(n=90) and voiding cystourethrography(n=81) where 53 and 35 studies showed abnormal findings respectively, and a total of 45 cases of urinary tract anomaly including vesicoureteral reflux(VUR, n=35) were diagnosed. Normal findings on ultrasonography indicated decreased risk of VUR in boys of 1-3 months of age(n=30). 53 patients were followed up more than 6 months and 45 episodes of subsequent UTI developed in 29 patients during the first 6-month period. Patients with relapse were older than patients without relapse at the diagnosis of first UTI, but other clinical parameters including abnormal findings on the imaging studies and prophylactic antibiotics prescription were not different between the two groups. Conclusion: UTI in early infancy occurred mainly in male infants and half of the patients had structural anomalies. USG was of clinical value in detecting anomalies requiring surgical intervention, and to rule out high grade VUR in 1-3 months old boys. Results of the imaging study or prophylactic antibiotics could not modify the risk of recurrent UTI. (J Korean Soc Pediatr Nephrol 2004;8:223-228)

• Childhood Kidney Diseases
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• v.2 no.2
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• pp.95-103
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• 1998

Purpose : To find and solve the common problems of peritoneal dialysis(PD) by analysing the clinical data of pediatric PD performed in Korea. Methods : 264 cases of CAPD and acute PD had been performed from Nov.1987 to Oct. 1997 in 17 institutions of pediatric nephrology in Korea. Results : CAPD was performed in 114 cases. The mean age of the patients was $10.5{\pm}6.6$ years and male to female ratio was 1.4:1. The original renal diseases of ESRD were proven in 92 cases($80\%$). The common renal disease of ESRD were FSGS($17\%$), reflux nephropathy ($11\%$), chronic glomerulonephritis($9.6\%$). Mean duration of CAPD was $20{\pm}16.9$ months. Peritonitis was the most common complication and incidence was one episode/18.2 patient-months. Other complications were exit site infection in 10 cases, obstruction in 7 cases, leakage of dialysate in 6 cases. The most common etiologic organism of peritonitis was staphylococcus aureus and the next was staphylococcus coagulase(-). Acute PD was performed in 150 cases. Most common underlying causes were congenital heart disease, hemolytic uremic syndrome, sepsis and dehydration. The mean duration was $10.3{\pm}11.3$ days. The most common complication was peritonitis($20.6\%$). The most common etiologic organism was staphylococcus aureus and coagulase(-), acinetobactor and pseudomonas. Conclusion : Reflux nephropathy should be emphasized in early diagnosis and treatment to prevent ESRD. Incidence of congenital anomaly($7\%$) as a original disease of ESRD was relatively low in Korea. Growth status was not significantly improved after CAPD. In acute PD, the incidence of peritonitis was rapidly increased at 2weeks after beginning of dialysis.

• Childhood Kidney Diseases
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• v.17 no.2
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• pp.49-56
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• 2013

Purpose: IgA nephropathy (IgAN) is one of the major causes of end-stage renal disease. Mass school urine screening (SUS) has been performed to enable early detection of chronic renal diseases, including IgAN. We wanted to evaluate the patients with IgAN, including those diagnosed through SUS. Methods: Between 1998 and 2010, 64 children were diagnosed with IgAN based on renal biopsy results obtained at the Pediatric Nephrology Department, ${\bigcirc\bigcirc}$ University Hospital. We divided these patients into the SUS group (37 cases), diagnosed through SUS, and the symptomatic (Sx) group (27 cases), diagnosed clinically. The medical records of both groups were analyzed retrospectively. Results: The mean age of the SUS and Sx groups was $10.8{\pm}2.7$ and $9.5{\pm}3.4$ years (P >0.05), respectively. Both groups had a higher proportion of male patients. The time from the notification of an abnormal urinary finding to a hospital visit or renal biopsy was shorter in the Sx group than in the SUS group. Regarding clinical manifestations, there were fewer cases with gross hematuria (P <0.001) and edema (P =0.008) in the SUS group, but there were no differences in terms of the therapeutic regimen and treatment duration. Regarding laboratory parameters, the Sx group had a higher white blood cell count (P =0.007) and lower hemoglobin (P =0.007) and albumin (P =0.000) levels. There were no differences in the renal biopsy findings in both groups, based on the history of gross hematuria or the severity of proteinuria. However, in all 64 patients with IgAN, the light microscopy findings (Hass classification) were related to a history of gross hematuria or the severity of proteinuria. Conclusion: There were no significant clinical and histological differences between the groups, as both had early stage IgAN. Although SUS facilitates the early detection of IgAN, long-term, large-scale prospective controlled studies are needed to assess the benefits of early diagnosis and treatment in chronic renal disease progression.

• Childhood Kidney Diseases
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• v.9 no.2
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• pp.222-230
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• 2005

Purpose : ARC syndrome refers to an association of arthrogryposis, renal tubular dysfunction, and cholestasis. The VPS33B gene was recently identified as the causative gene. So far, 41 cases of ARC syndrome have been reported worldwide, and it has rarely been reported in Korea. This study was conducted to report the clinical findings of seven ARC syndrome cases in Korean children, focusing especially on renal tubular dysfunction. Methods : The hospital records of 7 cases diagnosed as ARC syndrome at Severance Hospital between Mar. 1995 and Aug. 2005 were reviewed and analyzed. Results : Of the 7 cases, 4 were boys and 3 were girls. Six patients(85$\%$) were born with normal birth weight at term, and one was born at preterm. All cases presented with cholestasis and severe jaundice. According to the type of arthrogryposis described by Brown et al, type 3 and 4 were found in 2 patients and type,6, 7, and the undistributed type in one patient respectively. Other associated clinical findings were as follows : failure to thrive in 6(85$\%$), lax skin in 5(71$\%$), and gray platelet syndrome in 4(57$\%$). Urine analysis revealed 6 cases(85$\%$) with proteinuria, 3(43$\%$) with hematuria, 5(71$\%$) with glycosuria, 2(29$\%$) with phosphaturia and 2(29$\%$) with calciuria. Serum electrolytes showed 4 cases(57$\%$) with hyponatremia, 3(43$\%$) with hypokalemia, and 1(14$\%$) with creatinine elevation. Renal tubular dysfunctions were diagnosed as renal tubular acidosis in 6 cases(85$\%$), nephrogenic diabetes insipidus in 2(29$\%$), and Fanconi syndrome in 2(29$\%$). During the follow-up period, 2(29$\%$) had no treatment, 5(85$\%$) had continuous supplementation to correct the electrolyte imbalance and acidosis, and 1(14$\%$) had dialysis. Only one patient had a family history of ARE syndrome in a sibling. Four cases(57$\%$) were diagnosed at the mean age of 8.2 months, and one case was lost during follow-up. Ages of the survived 2 cases were 13 and 25 months, respectively. Conclusion : The rare disease of ARC syndrome Is associated with severe renal dysfunction. However, this study revealed that the renal manifestation of ARC syndrome in Korean children is relatively mild and survival rate is higher than that of previous studies. Contrary to previous reports, this study showed that familial cases are rare and sporadic occurence is possible in Korea. Thus, the diagnosis of this syndrome requires a careful evaluation of the venal function in cases of congenital arthrogryposis, and a mandatory genetic counseling of affected family for prevention of familial occurance. (J Korean Soc Pediatr Nephrol 2005;9:222-230)

• Childhood Kidney Diseases
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• v.5 no.1
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• pp.9-14
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• 2001

Purpose : This study was undertaken to investigate clinical aspects of nutcracker syndrome in children including sexual distribution, age of onset, the ratio of peak flow velocity between the narrowed and dilated portions in the left renal vein and prevalence of hematuria or proteinuria. Materials and Method : The subjects included 30 patients diagnosed with nutcracker syndrome using Doppler sonography from September 1999 to January 2001 in Severance Hospital, Department of Pediatric Nephrology. The diagnostic criteria for nutcracker syndrome was a peak flow velocity of ratio is more than 5.0. Results : Patients consisted of 14 males and 16 females. The mean age was 9.19${\pm}$2.31 years old and the ratio of peak flow velocity was 8.52${\pm}$2.24. Age of onset us higher in males 9.68${\pm}$2.82 year old than in females, 8.76${\pm}$1.72 year old respectively(P<0.05). But there was no difference in the ratio of peak flow velocities ; 8.45${\pm}$2.31 in the male group , 8.58${\pm}$2.25 in the female group(P>0.05). There was no difference in the age of onset and the ratio of peak flow velocity between patients with hematuria and without hematuria. Among patients with nutcracker syndrome, 24 cases of hematuria, 3 cases of proteinuria only, and 3 cases of hematuria with proteinuria. Conclusion : In children of school age, early diagnosis of nutcracker syndrome and consistent follow up is worthy of consideration, especially ill the prevention of sequelae. (J. Korean Soc Pediatr Nephrol 5 : 9-14, 2001)

• Childhood Kidney Diseases
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• v.8 no.2
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• pp.229-238
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• 2004

Purpose: This study was perfonned to elucidate the clinical pictures of acute focal bacterial nephritis(nephronia) in children. Methods: We reviewed 9 children with nephronia diagnosed by ultrasonography or computed tomography of kidneys from September 1994 to August 2004. Results: The overall male to female ratio was 2:1, and the age distribution ranged from 0.1 to 6 years(mean $2.8{\pm}2.2$). The cardinal symptoms were fever, chills, abdominal pain and dysuria/frequency. The initial leukocyte count was $21,000{\pm}5,600/uL$, ESR $60{\pm}23mm/hr$, CRP $17{\pm}10\;mg/dl$. Pyuria was noted in every patient and persisted for $10.5{\pm}7.8$ days after antimicrobial treatment. Abdominal sonography demonstrated focal lesion of ill-defined margin and low echogenicity in 5 of 9 patients(55.6%), while computed tomography revealed nonenhancing low density area in all patients(100%). Three of 9 patients(33.3%) had vesicoureteral reflux, greater than grade III. The initial $^{99m}Tc-DMSA$ scan showed one or multiple cortical defects in every patient, and improvements were noted in 2(33.3%) of 6 patients who received follow up scan after 4 months. Intravenous antibiotics was given in every patient under admission. Total febrile period was $11.8{\pm}6.3$ days(pre-admission, $4.0{\pm}3.0;$ post-admission, $7.8{\pm}5.5$ days) and the patients needed hospitalization for $17.2{\pm}8.1$ days. Conclusion: For the early diagnosis of 'acute focal bacterial nephritis' we should perform renal computed tomography first rather than ultrasonography, when the child has toxic symptoms and severe inflammatory responses in blood and urine.

• Childhood Kidney Diseases
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• v.3 no.2
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• pp.117-122
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• 1999

Purpose : We studied the correlation between urinalysis and radiologic findings in infants and children with urinary tract infection. Method : Urine Dipstick test and unstained urine microscopic examination were carried out in 56 infants and children who were hospitallized with the diagnosis of urinary infection by pocitive urine culture at Seoul Adventist from September 1996 through August 1998. Urine was collected by midsream, catheter, urine bag after cleansing or bladder puncture. Renal sonography and $^{99m}Tc-DMSA$ renal scan or voiding cystoureterography were studied. Results : 1)In dipstick analysis, leukocyte esterase(LE) were positive in 30 cases of 56 patients(54%) and Nitrite were positive in 20 cases of 56 patients(36%) and LE or Nitrite were positive in 38 cases of 56 children with UTI(68%). 2)In microscopic analysis, WBC were positive in 38 cases of patients(68%) and bacteria were positive in 23 cases of 56 patients(41%) and WBC or bacteria were positive in 41 cases of 56 children with UTI(71%). 3)Ten cases of 56 UTI patients(18%) showed negative finding in Dipstick and microscopic analysis. 4)There was no significant difference in positive rate of dipstick(71% vs 66%) and microscopia analysis(83% vs 66%) between two group with or without renal scar on $^{99m}Tc-DMSA$ scan (P=0.117). 5)There was no significnt difference in positive rate of dipstick(100% vs 91%) and microscopic analysis(100% vs 100%) between two groups with or without vesicoureteral reflux on VCUG. Conclusion : There was no specific relationships between the abnormal urinalysis and the abnormal findings on $^{99m}Tc-DMSA$ scan or VCUG.

• Childhood Kidney Diseases
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• v.3 no.2
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• pp.209-216
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• 1999

Purpose : Nephrogenic diabetes insipidus (NDI) is a rare X-linked disorder associated with renal tubule resistance to arginine vasopressin (AVP). The hypothesis that the defect underlying NDI might be a dysfunctional renal AVPR2 has recently been proven by the identification of mutations in the AVPR2 gene in NDT patients. To investigate the association of mutations in th AVPR2 gene with NDI, we analyzed the AVPR2 gene located on the X chromosome. Methods : We have analyzed the AVPR2 gene in a kindred with X-linked NDI. The proband and proband's mother were analyzed by polymerase chain reaction-single strand conformational polymorphism(PCR-SSCP) and DNA sequencing of the AVPR2 gene. We also have used restriction enzyme analysis of genomic PCR product to evaluate the AVPR2 gene. Results : C to T transition at codon 202, predictive of an exchange of tryptophan 202 by cysteine(R202C) in the third extracellular domain was identified. This mutation causes a loss of Hae III site within the gene. Conclusion : We found a R202C missense mutation in the AVPR2 gene causing X-linked NDI, and now direct mutational analysis is available for carrier screening and early diagnosis.

• Childhood Kidney Diseases
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• v.8 no.2
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• pp.176-185
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• 2004

Purpose: Rapidly progressive glomerulonephritis (RPGN) is a clinicopathologic entity characterized by extensive crescent formation and rapid deterioration of renal function within few months. For better understanding of its clinical course and designing better treatment strategies, a clinicopathological study of childhood RPGN was performed. Methods: The clinical manifestations and pathological findings were reviewed retrospectively in 12 children who were diagnosed as having RPGN by clinical manifestations and renal biopsy during a period from 1991 to 2003. Several clinicopathological parameters were analyzed as prognostic factors. Results: Among a total of 12 patients, 4 were male and 8 were female. The median onset age was 11.5 years(range 5.5-14.6 years), and the median period of follow-up was 25 months(range 7 months-6.6 years). According to the pathological classification, 10 patients (83%) were type II RPGN(immune-complex mediated glomerulonephritis), 2 patients were type III RPGN(pauci-immune glomerulonephritis), and none was type I RPGN(anti-glomerular basement membrane nephritis). All patients were treated with oral steroid in various combinations with methylprednisolone pulse therapy(10 patients, 83%), cyclophosphamide(8 patients, 67%), or plasmapheresis(4 patients, 33%). Clinical outcomes of 12 patients were complete remission in 1(8%), end-stage renal disease in 2(17%), chronic renal insufficiency with persistent proteinuria in 2(17%), and normal renal function with persistent proteinuria in 7(58%) at the last follow-up. Poor prognosis is associated with increased serum creatinine level, severe anemia and younger age at the time of diagnosis. Conclusion: Immune-complex mediated glomerulonephritis is the major cause RPGN in children and most cases showed improvement of renal function with aggressive management. For better understanding of this rare disease, a prospective multicenter study should be done.