Flank Pain is a leading indicator of renal and upper urinary tract disease or trauma, and rarely results from pelvic congestion syndrome. Although pelvic congestion syndrome occurs commonly in multi-parous women, pelvic congestion syndrome should also be considered as the cause of flank pain in an adolescent girl. We report the first case of pelvic congestion syndrome presenting with chronic left flank pain in an adolescent girl.
Adolescent* ; Estrogens, Conjugated (USP)* ; Female* ; Flank Pain* ; Humans ; Urologic Diseases

Wegener's granulomatosis(WG) is a necrotizing granulomatous small vessel vasculitis with a clinical predilection for involvement of the upper airways, lungs and kidneys. The disease usually manifests in adults between 25 and 50 years of age, but it can also rarely occur-in childhood with some features different from those of adults. WG may be easily overlooked in young patients by misinterpretion of the symptoms as caused by an infectious disease of the respiratory tract. Delayed diagnosis and treatment of the disease may cause more rapid progression of the glomerulonephritis to end stage renal disease. We report a boy who was diagnosed with WG with involvement of multiple organs at 13 years of age.
Adult ; Communicable Diseases ; Delayed Diagnosis ; Glomerulonephritis ; Humans ; Kidney ; Kidney Failure, Chronic ; Lung ; Male ; Respiratory System ; Vasculitis ; Wegener Granulomatosis*

Although juvenile nephronophthisis(NPHP) is one of the most frequent genetic causes of chronic renal failure, it has very rarely been reported in Korean children. Most NPHP patients are found to have chronic renal failure, since there are no distinct clinical symptoms for NPHP except polydipsia, polyuria and enuresis in the early stage of disease. Ten percent of NPHP patients manifest retinitis pigmentosa, called Senior-Loken syndrome. We experienced 2 cases of Senior-Loken syndrome that occurred in siblings(a 10 year-old boy and a 14-year-old girl) who were diagnosed with Leber's amaurosis. They were found to have severe renal impairment without polydipsia and polyuria. However, no large homogenous deletion of the NPHP1(2q13) gene was not identified in these patients. We report here on these cases and we review the literature to emphasize the association between Leber's amaurosis and the development of chronic renal failure.
Adolescent ; Blindness ; Child ; Enuresis ; Humans ; Kidney Failure, Chronic ; Leber Congenital Amaurosis ; Male ; Polydipsia ; Polyuria ; Retinitis Pigmentosa ; Siblings*

Henoch-Schonlein Purpura(HSP) is a form of vasculitis that typically affects small arteries in the skin, joints, intestinal tract and kidneys. It usually resolves spontaneously but sometimes can cause serious problems in the kidneys and intestinal tract. A 6-year-old girl with purpura, arthralgia and abdominal pain for 2 weeks was admitted. She also showed gross hematuria, generalized edema and decreased urine output. Blood pressure was in the upper normal range. Initial laboratory findings showed hypoalbuminemia, hyperlipidemia, microhematuria and nephrotic-range proteinuria(27.2 g/day). Initially, she was treated with pulse methylprednisolone, azathioprine, albumin and furosemide. Her renal biopsy revealed diffuse mesangial proliferation with strong IgA deposition. There were no crescents. On the third hospital day, she complained of severe abdominal pain and free peritoneal air was seen on abdominal X-ray. Primary repair of small bowel was performed and two pin-point sized holes were found. One week later, she still showed heavy proteinuria. Therefore, we added an ACE inhibitor and dipyridamole, and changed azathioprine to cyclosporine. One month later, the urine protein/creatinine ratio was decreased to 17.8 from 57, but heavy proteinuria has been still persisted. Here we report a rare case of a patient with HSP who had both severe nephrritc-nephrotic syndrome and small bowel perforation.
Abdominal Pain ; Arteries ; Arthralgia ; Azathioprine ; Biopsy ; Blood Pressure ; Child* ; Cyclosporine ; Dipyridamole ; Edema ; Female ; Furosemide ; Hematuria ; Humans ; Hyperlipidemias ; Hypoalbuminemia ; Immunoglobulin A ; Intestinal Perforation ; Joints ; Kidney ; Methylprednisolone ; Nephritis ; Proteinuria ; Purpura ; Purpura, Schoenlein-Henoch* ; Reference Values ; Skin ; Vasculitis

Cockayne syndrome is a rare autosomal recessive disorder characterized by cachectic dwarfism, mental retardation, loss of facial subcutaneous adipose tissue, microcephaly and photosensitive dermatitis. It is associated with renal abnormalities characterized by hyalinization of glomeruli, atrophy of tubules and interstitial fibrosis. To our knowledge, this is the first report of a case of Cockayne syndrome with FSGS in Korea. A 7-year old boy was admitted for evaluation of hypertension and proteinuria, which were detected 2 month ago. He was followed for short stature(<3 percentile), mental retardation(IQ 55), strabismus and dental caries since 3 years ago. He also showed microcephaly, a bird-like face and relatively large hands and feet. Laboratory findings showed decreased creatinine clearance(CCr 76.1 mL/min/1.73m2) and proteinuria(1,548 mg/day). Renal biopsy demonstrated focal segmental glomerulosclerosis of the hilar type with large hyaline deposits, moderate tubular atrophy and interstitial fibrosis. His cardinal features, mental retardation, and renal biopsy findings were consistent with Cockayne syndrome. We report here a very rare case of Cockayne syndrome with FSGS presenting with proteinuria and hypertension.
Atrophy ; Biopsy ; Child ; Cockayne Syndrome* ; Creatinine ; Dental Caries ; Dermatitis ; Dwarfism ; Fibrosis ; Foot ; Glomerulosclerosis, Focal Segmental* ; Hand ; Humans ; Hyalin ; Hypertension ; Intellectual Disability ; Korea ; Male ; Microcephaly ; Proteinuria ; Strabismus ; Subcutaneous Fat

The posterior reversible encephalopathy syndrome(PRES) is characterized clinically by a combination of acute or subacute confusion, lethargy, visual disturbance, and seizures. PRES has been described in various clinical settings, including severe hypertension, chemotherapy, eclampsia, and seizure. We report a case of a 7-year-old girl who had taken cyclosporine for steroid resistant nephrotic syndrome. Twenty one days after the cyclosporine therapy, she was admitted due to generalized tonic clonic seizure and headache. Her blood pressure was 170/90 mmHg. Magnetic resonance(MR) imaging showed necrotic/cystic lesions involving the bilateral parieto-occipital region. After discontinuation of cyclosporine, and control of blood pressure, she had no more seizure and headache. The follow-up MR examination which was performed 6 months later showed the decreased extent of the lesion.
Blood Pressure ; Child* ; Cyclosporine* ; Drug Therapy ; Eclampsia ; Female ; Follow-Up Studies ; Headache ; Humans ; Hypertension ; Lethargy ; Nephrotic Syndrome* ; Posterior Leukoencephalopathy Syndrome* ; Pregnancy ; Seizures

PURPOSE: Childhood primary VUR is generally diagnosed after urinary tract infection, is more prevalent among girls and has a low spontaneous resolution rate in cases of severe VUR. The aim of the present study is to examine the age and gender-related characteristics and the spontaneous resolution rate of infantile primary VUR. METHODS: The medical records of 96 infants with primary VUR, diagnosed after their first UTI, were retrospectively reviewed(1995-2004). The clinical characteristics including gender, the degree of VUR and presence of renal scars were evaluated. The spontaneous resolution rate and contributing factors were also analyzed. RESULTS: Infantile primary VUR was more prevalent in males than females. The percentage of atrophic scarred kidney was significantly higher in males than females(17.2% vs 3.4%)(P<0.05). The cumulative spontaneous resolution rate in 3 years was very high(89.1%), and was not significantly different between gender and among VUR grades. But in the first year, the spontaneous resolution rate of severe refluxing ureters was significantly higher in males than in females(46.2% vs 7.1%)(P<0.05) and the spontaneous resolution rate of refluxing ureters with no scarred kidneys was significantly higher than those associated with atrophic scarred kidneys(76.6% vs 20%)(P<0.05). CONCLUSION: Infantile primary VUR was more prevalent among males and tends to be associated with atrophic scarred kidneys in male infants. The cumulative spontaneous resolution rate in 3 years was very high, even in high-grade VUR and associated atrophic scarred kidneys. In infantile primary VUR, surgery should be withheld even in infants with high-grade VUR with atrophic scarred kidneys.
Cicatrix ; Female ; Humans ; Infant ; Kidney ; Male ; Medical Records ; Retrospective Studies ; Ureter ; Urinary Tract Infections ; Vesico-Ureteral Reflux*

PURPOSE: To analyze the clinical characteristics, spontaneous resolution rate and predictive factors of resolution in children with primary vesicoureteral reflux(VUR). METHODS: Between October 1991 and July 2003, 149 children diagnosed with primary VUR at Chonbuk National University Hospital were reviewed retrospectively. All of the patients were maintained on low-dose antibiotic prophylaxis and underwent radionuclide cystograms at 1- year intervals over 3 years after the initial diagnosis of VUR by voiding cystourethrogram was made. RESULTS: The median time to resolution of VUR was 24 months and the total 3 year-cumulative resolution rate of VUR was 61.7%. The following variables were associated with resolution of VUR according to univariate analysis-; age<1 year, male gender, mild grade of reflux, unilateral reflux, congenital hydronephrosis as clinical presentation at time of diagnosis of VUR, absence of focal defects in the renal scan at diagnosis, absence of recurrent UTI, renal scars and small kidney during follow-up. After adjustment by Cox regression model, five variables remained as independent predictors of VUR resolution; age<1 year, relative risk 1.77(P<0.05), VUR grade I+II 2.98(P<0.05), absence of renal scars 2.23(P<0.05), and absence of small kidney 5.20(P<0.01) during follow-up. CONCLUSION: In this study, spontaneous resolution rate of VUR, even high grade reflux, is high in infants during medical management, and it was related to age, reflux grade at diagnosis, absence of renal scars and small kidney during follow-up. Therefore early surgical intervention should be avoided and reserved for the selected groups.
Antibiotic Prophylaxis ; Child* ; Cicatrix ; Diagnosis ; Follow-Up Studies ; Humans ; Hydronephrosis ; Infant ; Jeollabuk-do ; Kidney ; Male ; Retrospective Studies ; Urinary Tract Infections ; Vesico-Ureteral Reflux*

PURPOSE: Voiding cystourethrography(VCUG) is a commonly performed diagnostic procedure in children with urinary tract infections. Recently, with the widespread use of prenatal ultrasonography, VCUG is performed as part of the postnatal radiological evaluation of asymptomatic infants with prenatally detected hydronephrosis. The procedure is relatively simple but it involves discomfort and some complications. We studied post procedural symptoms and complications in children who underwent VCUG. METHODS: This study reviewed 269 patients who underwent VCUG in our hospital between October 2005 and September 2006. We did a chart review and a telephone interview with the patients' parents about symptoms and complications associated with VCUG. RESULTS: Among 269 children, 217 patients(80.7%) were under 2 years of age and 5 patients (1.9%) were over 8 years of age. Their mean age was 13.1+/-22.9 months. After VCUG, dysuria was found in 49 patients presented with dysuria, and irritability in 36 patients with irritability. Other complications were hematuria, fever, frequency, bladder rupture and urinary tract infection. Mean symptoms duration was 1.4+/-0.7 days. There was no significant relationship between prophylactic antibiotics use and complication rate associated with VCUG. CONCLUSION: Our study demonstrated that 32.7% of patients showed complications including bladder rupture and urinary tract infection after VCUG. We also found that prophylactic antibiotics use did not prevent urinary tract infection nor decrease the rate of complications associated with VCUG. Therefore, we suggest that the procedure must be done carefully and aseptically, and we should closely observe the children who undergo VCUG for development of possible complications.
Anti-Bacterial Agents ; Child ; Dysuria ; Fever ; Hematuria ; Humans ; Hydronephrosis ; Infant ; Interviews as Topic ; Parents ; Rupture ; Ultrasonography, Prenatal ; Urinary Bladder ; Urinary Tract Infections

PURPOSE: Suprapubic aspiration(SPA) has been considered the "gold standard" for obtaining urine in non-toilet trained infants. Ultrasound(US)-guided SPA improves the success rate of the procedure and reduces the complications. However, many physicians perceive SPA as invasive and prefer the use of urethral catheterization (Ucat). We compared the success rate, complications and accuracy of US-guided SPA and Ucat. METHODS: 121 infants who visited Ewha Womans University Mokdong Hospital with suspected urinary tract infection(UTI) were investigated. For the first study, the study infants were randomly assigned to either the US-guided SPA(n=32) or Ucat(n=32) groups. The success rate and complications of both procedures were compared. For the second study, US-guided SPA and Ucat were performed simultaneously(n=57). The accuracy of urethral catheterization was subsequently analyzed. The criteria for success was defined as the collection of more than 0.5 mL of urine. UTI was diagnosed by the presence of uropathogens over 105 colony-forming units(CFU)/mL. RESULTS: The overall success rate of the US-guided SPA was 96.9%(71.9% in first attempts, 25.0% in second attempts) which was not significantly different compared to 96.9%(90.6% in first attempts, 6.3% in second attempts) in the Ucat(P>0.05) group. The aspirated urine volume was 7.4+/-3.7 mL in the US-guided SPA group, which was not significantly different to 4.5+/-2.6 mL in the Ucat(P>0.05) group. The accuracy of Ucat in comparison to the US-guided SPA was low with sensitivity 59.5%, specificity 86.6%, false-positive rate 13.3% and false-negative rate 40.5%. CONCLUSION: US-guided SPA should be encouraged as the best method to collect the urine in non-toilet trained infants with UTI.
Female ; Humans ; Infant* ; Sensitivity and Specificity ; Urinary Catheterization* ; Urinary Catheters* ; Urinary Tract