Multilocular cystic nephroma is a rare disease, noninherited benign renal neoplasm occurring in both children and adults. It is necessary to make a differential diagnosis from all renal diseases with a cystic component, such as Wilms tumor, armatoma or polycystic dysplastic kidney in childhood. There are about only 200 case reports in the world since Walter Edmunds had described it first. We report a case of multilocular cystic nephroma presented with painless abdominal mass, treated with nephrectomy and confirmed with pathology.
Adult ; Child ; Diagnosis, Differential ; Humans ; Kidney ; Kidney Neoplasms ; Nephrectomy ; Pathology ; Rare Diseases ; Wilms Tumor

Antiphospholipid syndrome is a thrombotic disorder characterized by the association of arterial and venous thrombosis with the antibodies directed toward phospholipids. The presence of these antibodies in systemic lupus erythematosus(SLE) has been shown to be related to several clinical and analytical alterations. We experienced one case of lupus nephritis with positive antiphospholipid antibodies in a 10-year-old girl whose chief complaint was persistent microscopic hematuria. We report this case with a brief review of related literatures.
Antibodies ; Antibodies, Antiphospholipid* ; Antiphospholipid Syndrome ; Child ; Female ; Hematuria ; Humans ; Lupus Erythematosus, Systemic ; Lupus Nephritis* ; Mass Screening* ; Phospholipids ; Venous Thrombosis

Microscopic Polyangiitis(MPA) belongs to a spectrum of systemic vasculits, and particularly antineutrophil cytoplasmic autoantibodies(ANCA)-associated small-vessel vasculitis which is characterized by involvement of the lung and kidney. The diagnosis of MPA is often difficult to make, and delayed because of the variability of the clinical presentation. Renal biopsies have a very important diagnostic and prognostic value in MPA. We experienced a case of microscopic polyangiits which was confirmed by renal biopsy and positive serum perinuclear ANCA, associated with alveolar hemorrhage and gastrointestinal bleeding. We began methylprednisolone pulse therapy, combined with a low dose of cyclophosphamide and plasmapheresis therapy. ACE inhibitor and Ca channel blocker were used when proteinuria and hypertention developed. On admission, the patient's lab findings showed BUN 117 mg/dL, Cr 2.3 mg/dL, while on the 60th hospital day BUN/Cr values fell to 20.8 mg/dL / 1.6 mg/dL and though proteinuria persisted, the patient's condition was tolerable and is currently under observation on an out-patient basis. The last lab values were BUN 26 mg/dL / Cr 1.6 mg/dL.
Antibodies, Antineutrophil Cytoplasmic ; Biopsy ; Cyclophosphamide ; Cytoplasm ; Diagnosis ; Glomerulonephritis* ; Hemorrhage* ; Humans ; Kidney ; Lung ; Methylprednisolone ; Microscopic Polyangiitis* ; Outpatients ; Plasmapheresis ; Proteinuria ; Systemic Vasculitis ; Vasculitis

Retinal detachment is a rare disease in children but cases of serous retinal detachment due to use of systemic corticosteroids have been reported in and out country. Rhematogenous retinal detachment has developed in a child with oral and steroid pulse therapy due to nephrotic syndrome. He was treated with laser and buckling in right and left eye respectively. Currently he is under observation through the out patient department.
Adrenal Cortex Hormones ; Child* ; Humans ; Nephrotic Syndrome* ; Rare Diseases ; Retinal Detachment* ; Retinaldehyde*

C1q nephropathy is an immune complex glomerulonephritis defined by the presence of mesangial C1q deposits in immunofluorescence microscopy and electron dense deposits on electron microscopy. It was described as a distinct disease entity in 1985 by Jennette and Hipp. Thirty four cases were reported in the literature but there has been no pediatric case reported in Korea yet. It commonly presents with steroid-resistent nephrotic syndrome in older children and young adults, and occasionally nephritic-nephrotic syndrome or rapidly progressive glomerulonephritis. We report a case of C1q nephropathy in a 23-month-old girl with steroid-dependent nephrotic syndrome.
Antigen-Antibody Complex ; Child ; Female ; Glomerulonephritis ; Humans ; Infant ; Korea ; Microscopy, Electron ; Microscopy, Fluorescence ; Nephrotic Syndrome* ; Young Adult

PURPOSE: Membranous glomerulopathy is a glomerular disease characterized by the presence of subepithelial immune deposits with thickening of the capillary wall of the glomerulus without inflammatory change. The pathogenesis of membranous glomerulopathy is still unknown. Its incidence is higher in males, and it is rarely found in infants and adolescents. Among the clinical manifestations proteinuria is most common, while edema and hematuria are present. According to reports from other countries, among few patients diagnosed with membranous glomerulopathy by renal biopsy, show isolated microscopic hematuria without the clinical manifestations. Little research in this area has been performed in Korea, and so we conducted retrograde studies on membranous glomerulopathy associated with isolated microscopic hematuria. MATERIALS AND METHODS: We analyzed retrogradely 109 cases of asymptomatic isolated microscopic hematuria that were diagnosed as membranous glomerulopathy by renal biopsy at Yonsei University Severance hospital from January, 1992 to July, 2001. RESULTS: In 87 of the 109 cases patients were over 15 years old while in 22 cases patients were under 15 at the time of dignosis. Only three patients showed isolated microscopic hematuria without the clinical manifestations and abnormal laboratory findings and they were all male patients under 15 years old. CONCLUSION: Few cases of the membranous glomerulopathy show only asymptomatic isolated microscopic hematuria. However, since membranous glomerulopathy can be found in patients who present with asymptomatic isolated microscopic hematuria only, if adequate indication for renal biopsy is present, we conclude that renal biopsy must be aggresively pursued in order to find the underlying disease.
Adolescent ; Biopsy ; Capillaries ; Edema ; Glomerulonephritis, Membranous* ; Hematuria* ; Humans ; Incidence ; Infant ; Korea ; Male ; Proteinuria

Type II membranoproliferative glomerulonephritis (Dense deposit disease ) is an acquired primary glomerular disease characterized by electron microscopic evidence of a continuous dense membrane deposition replacing the lamina densa. It is a subtype of idiopathic membra- noproliferative glomrulonephritis, and was described as a separate entity by Berger and Galle in 1963. It frequently occurs in older chilren and young adults and the clinical course is variable, but is generally progressive. The presenting feature is nephrotic syndrome in many patients, and proteinuria and hematuria are also seen frequently. The purpose of this paper is to present a case of DDD (Dense deposit disease) from a 10 year old boy who was diagnosed as a acute poststreptococcal glomurulonephritis with protenuria, hematuria, and facial edema by renal biopsy 4 years ago.
Biopsy ; Child ; Dichlorodiphenyldichloroethane* ; Edema ; Glomerulonephritis, Membranoproliferative* ; Hematuria ; Humans ; Male ; Membranes ; Nephrotic Syndrome ; Proteinuria ; Young Adult

PURPOSE: To evaluate the clinical significance of antenatally detected mild fetal pelviectasia and the role of voiding cystourethrography (VCUG). METHODS: From January 1999 to September 1999, 28 neonates (44 pelviectatic kidneys) with mild fetal pelviectasia (anteroposterial diameter <10 mm) were evaluated by postnatal renal sonography and voiding cystourethrography. RESULTS: Among 44 mild pelviectatic kidneys, 29 (65.9%) kidneys had no pelviectasia and 15 (34.1%) kidneys had persistent mild pelviectasia on postnatal renal sonography. Four (9.1%) kidneys of 3 males (10.7%) without pelviectasia on postnatal renal sonography had vesicoureteral reflux (VUR) on voiding cystourethrography. Vesicoureteral reflux was not correlated with prenatal progression or regression of pelviectasia and was not correlated with postnatal pelviectasia. CONCLUSION: All neonate with mild fetal pelviectasia need to undergo voiding cystoure- thrography for early diagnosis of vesicoureteral reflux even though postnatal renal sonography shows no pelviectasia.
Early Diagnosis ; Humans ; Infant, Newborn ; Kidney ; Male ; Vesico-Ureteral Reflux

PURPOSE: Urinary tract infection is a common problem in children. To evaluate for reflux most authorities recommend a voiding cystourethrogram 3 to 6 weeks after the first urinary tract infection. But during the 3 to 6 weeks interval, patients may fail to show up for the scheduled VCUG and thus risk for loss of follow up. We analyzed patient's records to evaluate whether the timing of VCUG after UTI influenced the prevalence or severity of VUR. METHODS: We retrospectively reviewed 213 children diagnosed with UTI from March 1997 to December 2000. These children were divided into 2 groups according to whether they had VCUG scheduled to be performed either within 1 weeks after the diagnosis of UTI (Group A) or later than 1 week after the diagnosis(Group B). We compared the presence and severity of reflux in the 2 groups. RESULTS: Reflux was present in 19% of the patients studied within 1 week after UTI and in 18% of those studied after 1 week. This difference was not statistically significant. Whereas 100% of the scheduled VCUGs in the Group A were performed, only 48% of those scheduled in the Group B were performed. This difference is statistically significant. CONCLUSION: Because there was no significant difference between the presence or severity of reflux and timing of VCUG after UTI, we suggest that a hospitalized patient with UTI should have VCUG performed before discharge.
Child ; Diagnosis ; Follow-Up Studies ; Humans ; Prevalence ; Retrospective Studies ; Urinary Tract Infections* ; Urinary Tract* ; Vesico-Ureteral Reflux

There are several diseases characterized by neurologic abnormalities and renal disease. Joubert syndrome is one of them. Joubert syndrome is a relatively rare autosomal recessive syndrome. The most significant and constant neurologic finding is hypoplasia of the cerebellar vermis. Joubert syndrome is associated with hypotonia, retinal dystrophy, abnormal eye movement, delayed development, abnormal respiratory pattern (neonatal episodic tachypnea or apnea) and nephronophthisis. We report a boy with Joubert syndrome associated with nephrocalcinosis and agenesis of the cerebellar vermis. This patient had also abnormal eye movement, hypotonia, abnormal respiratory pattern, delayed development and chronic renal failure.
Eye Movements ; Humans ; Kidney Failure, Chronic ; Male ; Muscle Hypotonia ; Nephrocalcinosis* ; Neurologic Manifestations ; Retinal Dystrophies ; Tachypnea