• Childhood Kidney Diseases
• /
• v.4 no.1
• /
• pp.63-68
• /
• 2000

Purpose: MCDK is regarded as a common cause of abdominal masses in children. And the presentation of the MCDK is usually a unilateral flank mass in the a newborn. Bialteral disease results in either fetal demise or necessity fer renal replacement therapy at birth. This study is designed to assess the clinical features and natural history of the unilateral multicystic dysplastic kidney. Patients and Methods: From January 1987 to January 2000 data were obtained retrospectively on 57 patients (28 boys and 29 girls, age ranged 1day-11years) who had a diagnosis of multicystic dysplastic kidney. The diagnosis of multicystic dysplastic kidney was confirmed by a combination of ultrasonography and radionuclide scan. Voiding cystourethrogram study in 31 patients were done to determine the condition of the contalateral kidney. Restllts: $84\%$ of the patients were diagnosed before birth by antenatal ultrasonography Clinical manifestations of children with postnatal diagnoses were palpable abdominal mass($3.5\%$), abdominal distension($17\%$), and incidental($10.5\%$). The abnormalities in contralateral kidney were hydronephrosis($21\%$), compensatory hypertrophy($12\%$), simple cyst($2\%$), bifid pelvis($2\%$). Surgical management was performed in 20 patients($35\%$) due to recurrent infection, for diagnostic purpose to differentiate from malignancy and abdominal distention. Follow-up in the remaining 37 patients continued (mean 18 months) and results of sonogram findings were involution change in 23 patients($40\%$) and no interval changes in 13 patient($23\%$). Conclusions : The apparent tendency to regression of the dysplastic kidney and no difference in the number of complications justify a conservative management rather than operative intervention except in associated severe complications such as urinary tract infection or rupture of cysts.

• Childhood Kidney Diseases
• /
• v.17 no.1
• /
• pp.25-28
• /
• 2013

There have only been 35 pediatric cases and one adult case reported on segmental multicystic dysplastic kidney (MCDK) from our search in PubMed, including 19 cases detected antenatally. There is little documentation of segmental MCDK, particularly concerning its natural history. Segmental MCDK can be presented atypically, making diagnosis more difficult. We report an another case with segmental MCDK. Multicystic abdominal mass detected on antenatal sonogram in this infant was diagnosed as segmental MCDK by renal ultrasonography and computed tomography. If a definitive diagnosis of segmental MCDK can be made on imaging, surgery is not required for a diagnostic biopsy.

• Proceedings of the Korean Society of Veterinary Clinics Conference
• /
• 2008.05a
• /
• pp.106-106
• /
• 2008

• Clinical and Experimental Pediatrics
• /
• v.45 no.9
• /
• pp.1141-1145
• /
• 2002

Klinefelter syndrome is the most common chromosomal abnormality, with a 47, XXY karyotype and typical clinical findings of infertility, hypogonadism, reduced body hair, gynecomastia, tall stature, and incresed gonadotropins and decreased testosterone levels. In addition to this classic description, several other diseases have been discribed in Klinefelter syndrome such as unilateral renal aplasia, autoimmune disease, diabetes mellitus, sexual precoxity, renal cell carcinoma, intravesical ureterocele, and osteoporosis. The incidence is 1 in 400-1,000 of the population and urological abnormalities are not common. However a case of Klinefelter syndrome associated with multicystic dysplastic kidney has not been not reported up to date. Therefore, we describe a 1-day-year old baby boy who presented with Klinefelter syndrome with unilateral multicystic kidney dysplastic disease, plus with a brief review of the literature.

• Clinical and Experimental Pediatrics
• /
• v.49 no.7
• /
• pp.796-799
• /
• 2006

Multicystic dysplastic kidney and congenital cystic adenomatoid malformation of the lung are independent disorders, but both result from abnormal morphogenesis during embryogenesis. Congenital cystic adenomatoid malformation of the lung is associated with renal anomalies as well as other extrapulmonary anomalies and almost all cases with these anomalies are stillborn. We report a case of a 21-month-old male who was admitted with the impression of acute infectious gastroenteritis; multicystic dysplastic kidney with congenital cystic adenomatoid malformation of the lung was detected incidentally during evaluation.

• Childhood Kidney Diseases
• /
• v.12 no.2
• /
• pp.262-266
• /
• 2008

Crossed renal ectopia is a congenital malformation in which both kidneys lie on the same side of the spine, usually side by side longitudinally. More often on the right side. Fusion of the two renal units is eight times more common than nonfusion. Although crossed renal ectopia is uncommon, this unusual entity must be considered in an infant when cystic mass in the abdomen or pelvis paticularly if no kidney can be found on the opposite side. In many cases of crossed fused ectopia with multicystic dysplastic kidney(MCDK), the diagnosis can be strongly suspected from the sonogram, and no other studies may be necessary. However, both intravenous urography and isotope renography is useful to assess the function of the crossed kidney. Crossed renal ectopia and MCDKs are associated with a greater incidence of ureteropelvic junction obstruction and reflux. So, screening voiding cystourethrography should be performed. Very few studies of MCDK in the setting of crossed fused ectopia have been reported. We have experienced a 3-year-old boy with crossed fused renal ectopia with multicystic dysplasia.

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

• Childhood Kidney Diseases
• /
• v.21 no.1
• /
• pp.1-7
• /
• 2017

Mitogen-activated protein kinases (MAPKs) play important roles in various cellular functions including proliferation, differentiation, and apoptosis. We showed that MAPKs are developmentally regulated in the rat kidney. p38 MAPK (p38) and extracellular signal-regulated kinase (ERK) were strongly expressed in the fetal kidney, whereas c-Jun N-terminal kinase (JNK) was detected predominantly in the adult kidney. The inhibition of p38 or ERK in organ culture resulted in reduced nephron formation with or without reduced kidney size. On the other hand, persistent fetal expression pattern of MAPKs, i.e., upregulation of p38 and ERK and downregulation of JNK, was observed in the cyst epithelium of human renal dysplasia, ovine fetal obstructive uropathy, and pcy mice, a model of polycystic kidney disease. Furthermore, activated p38 and ERK induced by cyclic stretch mediated proliferation and $TGF-{\beta}1$ expression in ureteric bud cells, probably leading to cyst formation and dysplastic changes. Inhibition of ERK slowed the disease progression in pcy mice. Finally, ERK and p38 were inactivated in the early embryonic kidney subjected to maternal nutrient restriction, characterized by reduced ureteric branching and nephron number. Thus, MAPKs mediate the development of normal and diseased kidney. Their modulation may result in novel therapeutic strategies against developmental abnormalities of the kidney.

• Childhood Kidney Diseases
• /
• v.5 no.2
• /
• pp.225-230
• /
• 2001

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, harmatoma 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. (J. Korean Soc Pediatr Nephrol 2001 ;5 : 219-24)

• Childhood Kidney Diseases
• /
• v.1 no.1
• /
• pp.31-37
• /
• 1997

The DMSA scan is a useful radiologic study in diagnosis of morphologic and functional diseases of kidney. We evaluated the distribution of sex and age, clinical manifestations, diagnosis, combined diseases, treatment and prognosis of the 61 patients with non-functioning kidney(no isotope uptake or uptake below 5% in DMSA scan) who admitted in our hospital from 1980 to 1995. The proportion of patients under 1 year old age was 46%. Sex ratio was 1.4:1 with male predominance. Most diagnosis of non-functioning kidneys were congenital such as multicystic dysplastic kidney, hydronephrosis due to ureteropelvic junction obstruction, renal agenesis and renal hypoplasia. In order of frequency thirty one percent of them were previously detected on antenatal ultrasonogram. Treatment consisted of operation in 47.5%, mostly nephrectomy and 32.8% of patients were followed up at OPD base without definite treatment. The most common combined diseases was hydronephrosis, in 4patients who had both kidneys inveloved progressed to chronic renal failure, but the prognosis in most cases were good. It is important to evaluate renal diseases in perinatal periods, and we believe that highly sensitive diagnostic study contribute to early treatment plan and thus to good prognosis.