• Childhood Kidney Diseases
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• v.10 no.2
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• pp.201-212
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• 2006

Purpose : Vesicoureteral reflux(VUR) is the major risk factor of urinary tract infection(UTI) in children and may result in serious complications such as renal scarring and chronic renal failure. The purpose of this study was to evaluate the relationship between VUR and renal scar formation, the usefulness and correlation of various imaging studies in reflux nephropathy, and the spontaneous resolution of VUR. Methods : We retrospectively reviewed 106 patients with VUR with no accompanying urogenital anomalies in the Department of Pediatrics, Bundang CHA Hospital during the period from Jan. 1996 to Mar. 2005. Ultrasonography and $^{99m}Tc$-dimercaptosuccinic acid(DMSA) scan were performed in the acute period of UTI. Voiding cystourethrography(VCUG) was performed 1 to 3 weeks after treatment with UTI. Follow-up DMSA scan was performed 4 to 6 months after treatment and a follow-up VCUG was performed every 12 months. Results : The mean age at detection of VUR was $13.8{\pm}22.2$ months and the male to female ratio was 2:1. The incidence of renal scarring showed a tendency of direct correlation between severity of VUR(P<0.001) and abnormal findings of renal ultrasonography(P<0.01). 63.2%(24 of 38 renal units) of renal parenchymal defects present in the first DMSA scan disappeared on follow-up DMSA scans. Follow-up DMSA scans detected renal scars in 7(14%) of 50 renal units with ultrasonographically normal kidneys. Meanwhile, ultrasonography did not show parenchymal defects in 7(36.8%) of 19 renal units where renal scarring was demonstrated on a follow-up DMSA scan. The spontaneous resolution rate of VUR was higher(75%) in cases with low grade(I to III) VUR(P<0.01). Conclusions : The presence and severity of VUR and abnormal findings of renal ultrasonography significantly correlated with renal scar formation. DMSA scan was useful in the diagnosis of renal defects. Meanwhile renal ultrasonography was an inadequate method for evaluating renal parenchymal damage. Therefore, follow-up DMSA scans should be performed to detect renal scars even in children with low-grade VUR and normal renal ultrasonography.

• Journal of Chest Surgery
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• v.25 no.12
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• pp.1550-1555
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• 1992

From June 1989 to July 1992, we used centrifugal Biomedicus pump[CBP] in 20 patients In 9 cases, CBP was used as ventricular assistance after heart surgery for those who could not be weaned off bypass even with intra-aortic balloon counter-pulsation and with maximal inotropic support In 8 patients, CBP was used as partial left heart bypass during repair of aortic aneurysms or congenital aortic anomalies. And in 3 patients, CBP was used as vena caval bypass during resection of renal cell carcinoma with tumor extension into the inferior vena cava. In 2 of 9 patients with ventricular assistance, they were weaned off the device successfully after 16 hours and 7 days respectively. But the patients died of intracranial hemorrhage and sepsis, 7 and 29 days after weaning from cardiac support, respectively. In all the patients who underwent aortic of vena caval surgery using CBP as shunt, there were no complications such as postoperative bleeding necessitating reoperation, renal failure or neurologic sequelae. In conclusion, the centrifugal type of ventricular assistance may be potentially life saving treatment modality in patients with severe postoperative low cardiac output syndrome. The CBP can be safely employed for resection of renal cell carcinoma with vena caval tumor extension and for repair of aortic aneurysms.

• Childhood Kidney Diseases
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• v.1 no.1
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• pp.67-72
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• 1997

Purpose : Congenital urinary tract anomaly is the most common anomaly in the childhood and progress to chronic renal failure and growth retardation. Therefore, early diagnosis arid treatment of urinary tract anomaly are important. Method : We reviewed medical records of 124 patients who had urinary tract anomalies on radiologic studies from Jan. 1986 to Dec. 1996. We analyzed demography and clinical characteristics of urinary tract anomalies. Results : 1) The age distributions were as follows ; 61 cases of 124 patients (49%) were under 1 year, 11 cases (8.8%) from 1 to 3 years, 20 cases (16%) from 4 to 6 years, 10 cases (8%) from 7 to 9 years, 9 cases (7.2%) from 10 to 12 years, 10 cases (8%) from 13 to 15 years, and 3 cases (2.4%) from 16 to 18 years. 2) Chief complaints in patients with urinary tract anomalies were fever, flank pain, prenatally diagnosed hydronephrosis, abdominal mass, dysuria and hematuria. 3) Of 124 patients, 68 cases(54.8%) were combined with urinary tract infection, and main causative organism was E.coli, and the most frequently associated anomaly was vesicoureteral reflux. 4) Most of the urinary tract anomalies were VUR, UPJ obstruction, congenital hydronephrosis and double ureter in order of sequence. 5) Whereas the frequency of simple urinary tract anomaly was 87.9%, that of complex anomaly was 12%. 6) Operative corrections were needed in 47 cases and 7 cases were progressed to renal insufficiency. Conclusion : We emphasize that early detection of urinary tract anomaly, appropriate treatment and regular follow-up are needed.

• Childhood Kidney Diseases
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• v.1 no.1
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• pp.53-59
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• 1997

There have been many recent reports that unilateral renal agenesis and multicystic dysplastic kidneys are accompanied by contralateral vesicoureteral reflux leading to its injury. We grouped the children with unilateral renal agenesis, renal hypoplasia, multicystic dysplastic kidney into abnormal unilateral renal development and investigated whether it was accompanied with contralateral vesicoureteral reflux. We retrospectively reviewed 96 pediatric cases of unilateral renal agenesis, hypoplasia, multicystic dysplastic kidney diagnosed at Shinchon Severance Hospital, Yongdong Severance Hospital from 1987 to 1996 and Ajou University Hospital from 1994 to 1996. Diagnosis was based on radiological findings, renal hypoplasia being defined as small renal size with no apparent renal scarring and no irregularity of the calyceopelvic system on abdominal sonography or intravenous pyelography. Among the 96 cases,48 cases carried out voiding cystourethrography. 58 cases were male(60%) and 38 cases were female(40%). The cases of abnormal unilateral development on the left side were 45(47%) and that on the right side were 51(53%). Although there were diverse reasons leading to diagnosis, the major ones included were prenatal sonography, urinary tract infection, and other congenital anomalies. In cases of unilateral renal agenesis & hypoplasia the leading factors were urinary tract infection & other congenital anomalies and in cases of multicystic dysplastic kidney that was prenatal sonography. There was a chronological gap between the mean age of diagnosis(1.8 year) and voiding cystouerthrography(2.5 year, P < 0.01). 9 of the 18 unilateral renal agenesis cases, 5 of the 11 unilateral renal hypoplasia cases, and 3 of the 19 unilateral multicystic dysplastic kidney cases showed contralateral vesicoureteral reflux. Average reflux grade was above G III.Among the 17 children who had contralateral vesicoureteral reflux, 3 children had chronic renal failure and ureteroneocystostomy was carried out in 6 children. From the above results we conclude that screening voiding cystourethrography should be performed in children with abnormal unilateral renal development for early detection of vesicoureteral reflux in the contralateral kidney.

• Journal of Chest Surgery
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• v.23 no.6
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• pp.1139-1145
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• 1990

Seven infants less than age 3 months underwent patch aortoplasty and tube graft bypass for relief of coarctation of aorta. All had intractable congestive heart failure, despite aggressive medical therapy Each infant had other cardiac anomalies including patent ductus arteriosus, ventricular septal defect, atrial septal defect and congenital mitral stenosis. All patients underwent closure of the ductus arteriosus and patch angioplasty of the aorta to produce a luminal diameter of at least 15mm or tube graft interposition utilizing the Gortex tube graft diameter larger than 10mm. In 5 patients who had ventricular defect, they underwent pulmonary arterial banding. &ere was one hospital death 17 days after operation secondary to the hydronephrosis and renal failure. Hospitalization was less than 10 days after operation except one case. In 3 patients who had associated VSD, open heart surgery[VSD closure+PA debanding]was done without difficulty. Surgical repair of critical coarctation of the aorta in infants can safely be offered despite the poor preoperative condition and presence of other cardiac anomalies.

• Journal of Genetic Medicine
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• v.11 no.2
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• pp.74-78
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• 2014

Sotos syndrome (SS, OMIM 117550) is characterized by prenatal and postnatal overgrowth with multiple congenital anomalies. However, there have been few cases of growth retardation caused by renal failure from infancy. We report a case of dysplasia of the bilateral kidneys with renal failure and poor postnatal growth. A 2-month-old boy visited the emergency room owing to poor oral intake and abdominal distension. He was born at the gestational age of 38 weeks with a birth weight of 4,180 g. After birth, he had feeding difficulty and abdominal distension. Upon physical examination, his height and weight were in less than the 3rd percentile, while his head circumference was in the 50th percentile on the growth curve. He also showed a broad and protruding forehead and high hairline. Blood laboratory tests showed severe azotemia; emergent hemodialysis was needed. Abdominal ultrasonography revealed bilateral renal dysplasia with multiple cysts and diffuse bladder wall thickening. A posterior urethral valve was suggested based on vesicoureterography and abdominal magnetic resonance findings. Results of a colon study to rule out congenital megacolon did not reveal any specific findings. The conventional karyotype of the patient was 46, XY. Array comparative genomic hybridization study revealed a chromosome 5q35 microdeletion including the NSD1 gene, based on which SS was diagnosed. We describe a case of SS presenting with end stage renal disease due to posterior urethral valve. The typical somatic overgrowth of SS in the postnatal period was not observed due to chronic renal failure that started in the neonatal period.

• Childhood Kidney Diseases
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• v.23 no.2
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• pp.124-127
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• 2019

Herlyn-Werner-Wunderlich (HWW) syndrome is a rare congenital anomaly of the genitourinary tract comprising uterus didelphys, obstructed hemivagina, and ipsilateral renal agenesis. Patients with HWW syndrome usually present symptoms such as dysmenorrhea, abdominal pain, pelvic mass, and purulent vaginal discharge. If not treated at an appropriate time, complications such as infertility, endometriosis, pyosalpinx, and subsequent pelvic adhesions may occur. Here, we report a case of HWW syndrome in a 7-year-old-girl who was also diagnosed as having central precocious puberty. She was brought to the pediatric department with chief complaints of lump in her breast and vaginal discharge. When she was around 2 months old, she was confirmed to have a single kidney on ultrasonography. We checked her past medical history and diagnosed her as having HWW syndrome based on the results of imaging studies, including abdominal ultrasonography and pelvic magnetic resonance imaging. She underwent treatment with gonadotropin-releasing hormone analogue for 2 years. During 24 months of follow-up, she showed no serious problems or complications. If renal anomalies are identified immediately after birth or in infancy, further screening tests should be conducted prior to menstruation for determining congenital abnormalities of the reproductive tract and vice versa.

• Childhood Kidney Diseases
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• v.18 no.1
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• pp.42-46
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• 2014

Kawasaki disease (KD) is a systemic vasculitis that can affect many organ systems. Renal manifestations include pyuria, hematuria, proteinuria, tubulointerstitial nephritis, acute renal failure, hemolytic uremic syndrome, or renal scarring. Although its precise pathogenesis remains unknown, it is considered an autoimmune disease. In the literature, it has been reported that KD may develop in conjunction with urinary tract infections. However, many of these previous studies did not use imaging methods such as renal sonograms, dimercaptosuccinic acid renal scans, and voiding urethrocystograms. We report a case of an 8-month old male infant with high grade vesicoureteral reflux, who developed incomplete KD after recurrent pyelonephritis. Acute pyelonephritis can be an early manifestation of KD. Such cases require the evaluation of urinary tract anomalies according to the guidelines for the management of urinary tract infections.

• Childhood Kidney Diseases
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• v.2 no.2
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• pp.169-177
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• 1998

Purpose : Renal duplication is a common occurrence, and the range of ureteral and renal anomalies draining the two poles of the duplex kidney leads to a variety of clinical presentations. Method : A series of 61 children with duplicated systems was studied retrospectively who were admitted during last 12years. Results : Of the children $60.6\%$ were accompanied with urologic anomalies that required surgical treatment, and most children initially were presented with urinary tract infection. $42.6\%$ of children were either diagnosed incidentally during evaluation of other non-urologic disease or during follow-up evaluation of abnormal antenatal renal sonogram. Conclusion : From our point view, children with history of abnormal antenatal renal sonogram, or with symptoms such as urinary tract infection, hematuria, abdominal pain should be evaluated radiologially fully to confirm further abnormality and accompanied complications. And routine follow-up using abdominal ultrasonogram, VCUG, and urine culture should be used to prevent and detect early treatable complications.

• Childhood Kidney Diseases
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• v.3 no.1
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• pp.88-94
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• 1999

Purpose : Malformation of urinary tract is among the most common of all congenital anomalies and can progress to irreversible renal damage before diagnosis due to difficulty of early diagnosis. Present study was undertaken to determine the clinical characteristics of urinary tract anomaly and to find out the most appropriate diagnostic and therapeutic measures for children with these anomalies. Methods : During the past 10 years from 1987 to 1998, review of medical records revealed 65 children with congenital anomaly of urinary tract and the following results were obtained. Results : The most common anomalies were ureteropelvic junction obstruction occuring in 26 cases ($36\%$), followed by ureteral duplication in 11 cases, renal agenesis in 10 cases and ureterovesical function obstruction in 7 cases. Complex anomaly of urinary tract was found in 8 cases and anomaly of other systems such as congenital heart disease was detected in 11 cases. The most frequent age group at the time of diagnosis was below 1 year of age constituting 39 cases ($60\%$) and male preponderance was noted as male to female ratio being 2.25:1. Presenting symptoms were urinary tract infection in 25 cases, followed by hematuria, abdominal mass, abdominal pain and voiding difficulty, etc, and in 11 cases, the anomaly was picked up by routine prenatal ultrasonography. Azotemia was noted in 9 cases and the underlying anomaly was obstructive uropathy in 4 out of these 9 cases. Surgical correction was undertaken in 38 cases (most frequently in cases of obstructive uropathy) and in 2 out off cases with obstructive uropathy in whom surgical correction was done, azotemia disappeared during follow up period of 1-5years. No new cases of deteriorating renal function appeared during follow-up period. Conclusion : In spite of high incidence of congenital malformation of urinary tract, early diagnosis is still hampered by nonspecific symptoms and signs. Therefore, in patients with symptoms such as urinary tract infection, abdominal pain and voiding problems, etc, it Is advisable to take various diagnostic tests promptly to pick up any urinary tract anomaly and to apply proper therapy in order to avoid progression to irreversible renal damage. In this regard, prenatal ultrasonography should be utilized more widely as a routine procedure to detect any urinary tract anomalies before birth.