• Childhood Kidney Diseases
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• v.15 no.1
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• pp.81-85
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• 2011

IgA nephropathy is the most common form of primary glomerulonephritis and chronic glomerular disease worldwide including Korea. Familial gathering of IgA nephropathy suggests that genetic factors contribute to the development of this disease. Although there have been many reports on familial IgA nephropathy with genetic analysis and their pedigrees, there has been few reports in Korea. We reported a partial familial IgA nephropathy pedigree with a brief review of the literatures.

• Childhood Kidney Diseases
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• v.13 no.2
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• pp.271-277
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• 2009

Kimura's disease is a chronic inflammatory disorder of unknown etiology. A 14 year old boy suffering from steroid dependant nephrotic syndrome, was presented with relapsing painless subcutaneous masses on the left buccal area. Blood analysis showed increased IgE and eosinophilia. During 4 years follow up, he was been treated by low dose steroid and short term cyclosporine. Consequently, frequent relapses of subcutaneous masses and nephrotic syndrome has been relieved. Cyclosporine treatment combined with steroid may be useful for preventing frequent relapse of Kimura's disease.

• Childhood Kidney Diseases
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• v.20 no.2
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• pp.74-78
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• 2016

Purpose: Early diagnosis and treatment of urinary tract infection have been emphasized to prevent renal scarring. If untreated, acute pyelonephritis could cause renal injury, which leads to renal scarring, hypertension, proteinuria, and chronic renal failure. The purpose of this study was to assess risk factors of renal scarring after treatment of acute pyelonephritis (APN). Methods: The medical records of 59 patients admitted at Daegu Fatima Hospital because of APN between March 2008 and April 2015 whose renal cortical defects were confirmed by using initial technetium-99m dimercaptosuccinic acid (DMSA) scans were reviewed retrospectively. We divided 59 patients into 2 groups according to the presence of renal scar and assessed risk factors of renal scar, including sex, age at diagnosis, feeding method, hydronephrosis, bacterial species, vesicoureteral reflux, and vesicoureteral reflux grade. Results: Of 59 patients (41%), 24 showed renal scar on follow-up DMSA scan. No significant differences in sex, hydronephrosis, bacterial species, and fever duration were found between the renal-scarred and non-scarred groups. As for age at diagnosis, age of >12 months had 5.8 times higher incidence rate of renal scarring. Vesicoureteral reflux (VUR) affected renal scar formation. VUR grade III or IV had 14.7 times greater influence on renal scar formation than VUR grade I or II. Conclusion: Our data suggest that the presence of VUR and its grade and age at diagnosis are risk factors of renal scar on follow-up DMSA scan after APN.

• Childhood Kidney Diseases
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• v.24 no.1
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• pp.58-61
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• 2020

Pseudohypoaldosteronism type 1 (PHA1) is a rare salt-wasting disorder caused by resistance to mineralocorticoid action. PHA1 is of two types with different levels of disease severity and phenotype as follows: systemic type with an autosomal recessive inheritance (caused by mutations of the epithelial sodium channel) and renal type with an autosomal dominant inheritance (caused by mutations in the mineralocorticoid receptor). The clinical manifestations of PHA1 vary widely; however, PHA1 commonly involves hyponatremia, hyperkalemia, metabolic acidosis and elevated levels of renin and aldosterone. The earliest signs of both type of PAH1 also comprise insufficiency weight gain due to chronic dehydration and failure to thrive during infancy. Here, we report a case of renal PAH1 in a 28-day-old male infant harboring a novel heterozygous mutation in NR3C2 gene (c.1341_1345dupAAACC in exon 2), showing only failure to thrive without the characteristic of dehydration.

• Childhood Kidney Diseases
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• v.1 no.2
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• pp.183-188
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• 1997

Familial juvenile hyperuricemic nephropathy is an autosomal dominant disease characterized by progressive renal disease and hyperuricemia or gout, affecting young people of either sex equally. There are two biochemical markers of this disorder. The first is hyperuricemia disproportionate to the degree of renal dysfunction; the second is a grossly reduced clearance of uric acid relative to creatinine, dispropotionate to age, sex and degree of renal failure. We experienced 2 family members with hyperuricemia. One family member, a 13-year-old girl who had suffered from tophaceous gout and chronic renal failure. Her younger brother also had hyperuricemia and moderately reduced renal function. Their urinary excretion fractions of uric acid($FE_{uric\;acid}$) were reduced and renal biopsy specimens showed interstitial fibrosis with tubular atrophy and interstitial urate crystal deposition. We have treated these two patients with allopurinol but we have done renal transplantation because she progressed to end stage renal disease at 16 year old age.

• Childhood Kidney Diseases
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• v.22 no.2
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• pp.81-85
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• 2018

Amyloidosis is a rare disease that results from the deposition of extracellular protein in various body tissues, causing progressive organ dysfunction. Secondary renal amyloidosis is a rare but serious complication of chronic inflammatory bowel disease, particularly in patients with Crohn's disease or ulcerative colitis. We report a case of secondary renal amyloidosis in a pediatric patient who reported a 16-year history of "very early onset inflammatory bowel disease". Intensive treatment including repeated infliximab infusions improved clinical parameters of inflammatory bowel disease, although renal dysfunction showed progression. Amyloidosis should be considered in patients with IBD, particularly if they suffered disease progression.

• Childhood Kidney Diseases
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• v.7 no.1
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• pp.103-105
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• 2003

Scrotal swelling may be acute or chronic, painful or painless. Common causes of scrotal swelling in newborns are hydrocele, inguinal hernia, testicular torsion, testicular tumor, scrotal hematoma, meconium peritonitis and epididymitis. Abrupt onset of a painful scrotal swelling necessitates prompt evaluation. Testicular torsion and incarcerated inguinal hernia require urgent surgical management. We report a case of scrotal swelling caused by a tunica vaginalis abscess in a 20-days-old boy. He was admitted to the hospital due to fever, irritability and left scrotal swelling with local heat, tenderness and redness. Exploratory laparotomy was performed to rule out testicular torsion. On the operative field, congestive erythematous inflammation on the left tunica vaginalis was noted and it was filled with a pus like discharge. The cultured organism was Streptococcus agalactiae(group B). He recovered quickly after debridement and administration of empirical antibiotics.

• Childhood Kidney Diseases
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• v.9 no.1
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• pp.102-107
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• 2005

With the advent of hemodialysis, the success of renal transplants in the 1960s and the wide use of continuous ambulatory peritoneal dialysis at the end of the 1970s, children with renal failure now enjoy an extended life span. As a result, several children experience renal osteodystrophy and growth retardation. Renal osteodystrophy is induced by phosphorus retention, hypocalcemia, low vitamin D levels and hyperparathyroidism. The pharmacologic interventions are used to prevent bone deformities and to normalize growth velocity. But surgical intervention is required sometimes whorl osteodystrophy is severe and poorly controlled. We report an eight-year-old boy with ctironic renal failure who developed severe bone deformities and needed osteotomy.

• Childhood Kidney Diseases
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• v.9 no.1
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• pp.108-111
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• 2005

Nephrotic syndrome is a common chronic disease in childhood. Patients with nephrotic syndrome are at an increased risk of bacterial Infections due to immunological changes secondary to proteinuria. Primary bacterial peritonitis is one of the most serious and common infectious complications. The rate of peritonitis is, 2-6% and overwhelming infection still carries a mortality rate of 1.5%. We experienced a rare case of nephrotic syndrome complicated with severe peritonitis and Peritoneal empyema in a 10-year old girl after 2 months of medical neglect by parents. Here we emphasize thf: importance of early detection and treatment of peritoneal infection in nephrotic syndrome.

• Childhood Kidney Diseases
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• v.25 no.2
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• pp.92-111
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• 2021

Purpose: Nephrotic syndrome (NS) is the most common form of glomerulopathy in children. Most pediatric patients respond to glucocorticosteroid treatment (steroid-sensitive NS, SSNS), while approximately 10-15% will remain unresponsive or later become steroid-resistant. There has been a long-standing effort to find biomarkers that may predict steroid responsiveness. Methods: We systematically reviewed current studies which investigated clinically relevant biomarkers for predicting steroid responsiveness in pediatric NS. We performed a PubMed and EMBASE search to identify eligible articles. We collected data on urinary markers, blood/serum markers (including cellular phenotypes and mRNA expression), genotypes and HLA allele frequency. Results: A total of 659 articles were identified following electronic and manual searches. After reviewing the titles, abstracts, and full texts, 72 eligible articles were finally included. Vitamin D-binding protein (VDBP) seemed to be significantly elevated in SRNS than in SSNS, in both serum and urine specimen, although further validation is required. Conclusions: The present paper narratively illustrates current understandings of potential biomarkers that may help predict steroid responsiveness. Further investigation and collaboration involving a larger number of patients are necessary.