• Childhood Kidney Diseases
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• v.2 no.2
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• pp.161-168
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• 1998

Purpose : Lots of congenital anomalies of urinary tract including hydronephrosis are detected in fetus and neworn by popular use of prenatal ultrasonography. But there are little data available in Korea about natural course of hydronephrosis diagnosed antenatally by ultrasonography. So we intended to help management of these patients by analizing the follow up data of the neonates with hydronephrosis diagnosed antenatally. Methods : We evaluated 22 patients with neonatal hydronephrosis(33 renal units) who were diagnosed prenatally and confirmed postnatally. Especially patients with suspected ureteropelvic junction obstruction were followed regulary with renal ultrasonography and diuretic renography for 8-24 months. Results : 1) The etiologies of neonatal hydronephrosis diagnosed prenatally were suspected ureteropelvic junction obstruction($69.9\%$), vesicoureteral reflux($15.1\%$), primary megaureter($3.0\%$), double ureter with ureterocele($3.0\%$), ureteral stricture($3.0\%$), multicystic dysplastic kidney(3.0$\%$), and ureterovesical junction obstruction(3.0$\%$). 2) The follow up results of 23 renal units of suspected ureteropelvic junction obsruction: Except 4 renal units with palpable abdominal mass, of the remained 19 renal units, 14 units($73.6\%$) were improved spontaneously, 3 units($15.7\%$) remained stable, only2 units($10.5\%$) were aggravated. Conclusion : We concluded that in most cases of hydronephrosis there is no need for immediate surgery, and that nonoperative approach, using serial ultrasonography and diuretic renogram, is safe management of neonatal hydronephrosis diagnosed anteratally.

• Clinical and Experimental Pediatrics
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• v.59 no.sup1
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• pp.25-28
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• 2016

Phelan-McDermid syndrome is a rare genetic disorder caused by the terminal or interstitial deletion of the chromosome 22q13.3. Patients with this syndrome usually have global developmental delay, hypotonia, and speech delays. Several putative genes such as the SHANK3, RAB, RABL2B, and IB2 are responsible for the neurological features. This study describes the clinical features and outcomes of Korean patients with Phelan-McDermid syndrome. Two patients showing global developmental delay, hypotonia, and speech delay were diagnosed with Phelan-McDermid syndrome via chromosome analysis, fluorescent in situ hybridization, and multiplex ligation-dependent probe amplification analysis. Brain magnetic resonance imaging of Patients 1 and 2 showed delayed myelination and severe communicating hydrocephalus, respectively. Electroencephalography in patient 2 showed high amplitude spike discharges from the left frontotemporoparietal area, but neither patient developed seizures. Kidney ultrasonography of both the patients revealed multicystic kidney disease and pelviectasis, respectively. Patient 2 experienced recurrent respiratory infections, and chest computed tomography findings demonstrated laryngotracheomalacia and bronchial narrowing. He subsequently died because of heart failure after a ventriculoperitoneal shunt operation at 5 months of age. Patient 1, who is currently 20 months old, has been undergoing rehabilitation therapy. However, global developmental delay was noted, as determines using the Korean Infant and Child Development test, the Denver developmental test, and the Bayley developmental test. This report describes the clinical features, outcomes, and molecular genetic characteristics of two Korean patients with Phelan-McDermid syndrome.

• The Journal of the Korean life insurance medical association
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• v.3 no.1
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• pp.187-208
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• 1986

The conclusions which was acquired one renal cell carcinoma and renal disease 132 cases that was tested renal echogram among 4,499 cases for recent 16 months at medical department, Dae Han Kyoyuk Insurance company from August, 1984 to November, 1985 are as follows: 1. On bur ultrasonography, the echo of tumor was demonstrated with echogenicity as compared with renal parenchyme. 2. The case was stage I by Robson's modification method for pathologic histology. 3. There is no fever, typical triad of renal cell carcinoma and the result of serum biochemical test was within normal limit. 4. The frequency with disease was renal cell carcinoma(0.76%), ureteral stone(1.5%), multicystic kidney(2.27%), hydroureter(2.27%), Bilateral poly cystic kidney(4.55%), hydronephrosis(4.55%), renal agenesis(6.06%) renal calculi(18.18%), simple cyst(60.61%). 5. The frequency with age was 55/1200 case(4.58%) in $41{\sim}50$ years, 13/296 cases(4.39%) in $51{\sim}60$ years, 43/2144 cases(2.01%) in $31{\sim}40$ years, 14/791 cases(1.77%) in $21{\sim}30$ years, 7/53 cases(1.32%) more than 61 years and 0/15(0%) under 20 years. 6. The affected site of renal agenesis 8 cases was right side all. 7. In total renal disease 132 cases, the affected site of 126 cases except bilateral polycystic kidney 6 cases was right kidney 72 cases, left kidney the proportion of right to left 1.6:1 8. In total renal disease 132 cases except bilateral polycystic kidneys 6 cases, the patients affected with both side kidneys were 14 cases. 9. The affection rate with sex in total renal disease 132 cases was 98/2860 cases in male, 34/1819 cases in female and the former was about 2 times than the latter. 10. Classifying the stone with part, nephrolithiasis 24 cases were appeared high frequency, on the contray, ureteral stone 2 cases. 11. 2 cases of ureteral stone developed complication, hydronephrosis and hydroureter. 12. The linear array type transducer was not helpful for the diagnosis of lower ureteral calculi but for the lower ureteral calculi, we could see the stone with high echo in accompanying with acoustic shadowing. 13. In 24 cases of renal calculi, both side nephrolithiasis was 3 cases(12.5%). 14. In renal calculi, solitary stone could be seen extremely much and the number of stone was so much variable from 2 to 10. 15. In 26 cases with renal calculi and ureteral stone, the common clinical manifestation was a intermittent and slight pain. 16. In 80 cases of renal cyst, as one's get older, the affection rate of cyst extremely rised. 17. In bilateral polycystic kidney, large cyst had septum on the whole. 18. The patients with complication were 14 cases(10.6%) of total patients.

• Applied Microscopy
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• v.18 no.1
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• pp.92-102
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• 1988

The ototoxic effects of salicylate on the ultrastructure of spiral ganglion cells were examined. Sodium salicylate($50{\sim}60\/kg$ body weight, once a day for 7 days) were injected subcutaneously to $5{\sim}6$ week-old fifteen Sprague-Dawley rats. Animals were sacrificed 24 hours (group 1), 6 weeks (group 2) or 10 weeks (group 3) after the last injection. In group 1 animals, distention of membranous cisternae was found in the cytoplasm of ganglion cells, satellite cells and Schwann cells in which enlargement or multicystic cytosome formation of the mitochondria were shown. In group 2 animals, membranous cisternae became larger or fused to form larger vacuoles or cysts. Shrinkage of spiral ganglion cell cytoplasm and loosening of myelin sheath were seen. In group 3 animals, extensive swelling or loss of nerve fibers were shown along with the folding or partial loss of myelin sheath which caused leakage of ganglion cell cytoplasm. It was concluded that the ototoxicity of salicy-late caused the ultrastructral changes of the spiral ganglion cells which became more severe in group 2 and 3 animals. The possibility of retrograde degeneration following the sensory cell changes was suggested.

• Journal of Korean Academy of Oral and Maxillofacial Radiology
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• v.12 no.1
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• pp.27-33
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• 1982

The purpose of this study is to obtain some informations for the differential diagnosis of ameloblastoma from dentigerous cyst by analysis of the radiographic findings of these lesions. The author studied age and sex distribution, the site of the lesion, tooth behavior and several radiographic features of ameloblastoma and dentigerous cyst. The material consisted of 65 patients of ameloblastoma and 37 patients of dentigerous cyst. The results were obtained as followings. 1) The incidence was highest in 2nd decade (29.2%) and total 65 cases consists of 35 males (53.8%) and 30 females (46.2%) in ameloblastoma. 62 cases were found in lower jaw (95.4%) and the highest site of occurence of ameloblastoma was mandibular molar. region 27 cases. (41.5%) 2) In 65 cases of amelobl!!stoma, 18 cases were seen in association with tooth and 15 cases (83.2%) out of those were associated with mandibular molar teeth. Mandibular molar were most frequently involved in dentigerous cyst (11/31 cases, 29.7%). 3) (a) 23 cases (35.3%) of tooth resorption were found in ameloblastoma and 11 cases (29.7%) of tooth resorption were found in dentigerous cyst. (b) 15 cases (23.1%) of tooth migration were found in ameloblastoma and 10 cases (27.0%) of tooth migration were found in dentigerous cyst. 4) Several radiographic features. (a) Monolocular type ameloblastoma were seen in 23 cases (35.4) and multilocular type of ameloblastoma were seen in 42 cases (64.6%). Monolocular type of dentigerous cyst were seen in 33 cases (89.2%) and multilocular type was seen in 4 cases. (b) Monolocular type ameloblastoma showed 20 cases (87.0%) of scalloped border but 32 cases (97.0%) of dentigerous cyst showed smooth border. (c) 34 cases (81.0%) of ameloblastoma showed honey-comb appearance, soap-bubble appearance or mixed appearance. but all 4 cases of dentigerous cyst showed multicystic appearance. (d) 12 cases (52.2%) of monolocular type ameloblastoma showed slightly increased radiopacity in surrounding bone, and 22 cases (66.7%) of monolocular type dentigerous cyst showed sharp osteosclerotic border. 27 cases (64.3%) of multilocular type ameloblastoma and 3 cases (75.0%) of multilocular type dentigerous cyst showed no changes in surrounding bone.

• Childhood Kidney Diseases
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• v.9 no.1
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• pp.69-75
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• 2005

Purpose : The most important management of congenital hydronephrosis consists of the early diagnosis and evaluation of the pathologic abnormalities of congenital hydronephrosis. This study was conducted to investigate the different causes of hydronephrosis and its clinical outcome. Methods : 54 live neonates who were hospitalized and diagnosed with congenital hydronephrosis at Chungnam National University Hospital from Aug. 1998 to Aug. 2003 were retrospectively analyzed. Results : Hydronephrosis(renal pelvic AP diameter $gt;5 mm) was postnatally detected in 54 cases(2.1%) among 2,539 neonates who were hospitalized from Aug. 1998 to Aug. 2003. There were three times more males than females. Additional imaging studies revealed that ureteropelvic junction obstruction was the most common postnatal diagnosis(48.7%), followed by multicystic dysplastic kidney, vesicoureteral reflux and duplication kidney with ureterocele. Spontaneous regression of hydronephrosis was revealed in 25 renal units(75.8% ) of mild hydronephrosis, 14 renal units of moderate hydronephrosis and 1 renal unit of severe hydronephrosis. Operative correction were carried out in 14 renal units(70%) of severe hydronephrosls. Conclusion : The most common established cause of congenital hydronephrosis in this study was ureteropelvic junction obstruction. There are many cases of spontaneous regression in mild to moderate congenital hydronephrosis. Urinary tract infections occur in many neonates with hydronephrosis. Therefore, early detection and evaluation of congenital hydronephrosis and continuous follow-up at regular intervals are necessary for conservation of renal function. (J Korean Soc Pediatr Nephrol 2005;9:69-75)

• Childhood Kidney Diseases
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• v.20 no.1
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• pp.29-32
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• 2016

Purpose: Congenital anomalies of the kidneys and urinary tract (CAKUT) are the most common anomalies identified in newborns. This study aims to demonstrate the prevalence of CAKUT including hydronephrosis diagnosed by antenatal and postnatal ultrasound over a five-year period. Methods: The records of births between May $1^{st}$, 2009 and April $30^{th}$, 2014 at our hospital were collected. The number of infants who underwent renal ultrasound after birth for the detection of CAKUT was counted. The incidence of each type of CAKUT such as hydronephrosis, size abnormality, horseshoe kidney, and Multicystic dysplastic kidney (MCDK) was retrospectively evaluated for antenatal screening and postnatal follow-up examination. Results: During the study period, 33,276 infants were born and 521 neonates underwent postnatal renal ultrasound. 183 cases of CAKUT were detected prenatally and 140 postnatally using ultrasonographic examinations at the following time: (i) 3-7 days postnatally in 123 newborns (87.9%), (ii) during 1-3 months in 11 newborns (7.9%), and (iii) later than 3 months in 6 newborns (4.3%). Among diagnosed CAKUT, hydronephrosis was the most common anomaly with 113 newborns diagnosed prenatally and 46 postnatally. Duplex kidney was the second most common anomaly followed by horseshoe kidney, simple cysts in the kidney and so on. Conclusion: The detection of CAKUT is an important part of the prenatal ultrasound. This study analyzed the prevalence of CAKUT detected on prenatal screening and compared the results to those detected postnatally. Prenatal ultrasound screening fulfills the needs of postnatal examinations and therefore, both antenatal and postnatal sonographic investigations are of vital importance for diagnosis of renal and urinary tract anomalies.

• Clinical and Experimental Pediatrics
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• v.52 no.4
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• pp.464-470
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• 2009

Purpose : This study assessed the incidence and outcome of congenital anomalies of the kidney and urinary tract (CAKUT) detected by prenatal ultrasonography Methods : There were 906 cases of CAKUT detected by prenatal ultrasonography and postnatally confirmed at the Asan Medical Center from October 1989 to October 2007. We investigated the incidence and outcome of these cases by reviewing medical records retrospectively. Results : The order of incidence was hydronephrosis, multicystic dysplastic kidney (MCDK), duplex kidney, vesico-ureteral reflux (VUR), single kidney, hydroureteronephrosis, ectopic kidney, polycystic kidney, ureterocele, and posterior urethral valve (PUV). There were 520 cases (57.4%) of hydronephrosis, and 20% of these needed an operation due to significant obstruction. MCDK was associated with other CAKUT in 25.4% of all cases. Approximately 57.9% of duplex kidney cases needed surgical treatment due to ureterocele and VUR. VUR had a male: female ratio of 10:1. Two out of seven cases of autosomal recessive polycystic kidney had progressed to chronic renal failure. Patients with PUV were relatively uncommon, and one out of nine cases progressed to end-stage renal disease. Conclusion : CAKUTs detected by prenatal ultrasonography were composed of various anomalies, and almost all of them had a good outcome without any intervention. However, in some cases, recurrent urinary tract infection or renal failure occurred, especially in bilateral cases. For further management, a long-term multicenter study is needed to investigate the precise incidence and outcome of each anomaly in the general population.

• Childhood Kidney Diseases
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• v.2 no.1
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• pp.60-68
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• 1998

Purpose: To determine the various prenatal factors related to the prenatal hydronephrosis diagnosed on prenatal ultrasonography. We also attempted to correlate the prenatal and neonatal renal pelvic anteroposterior diameter with the outcome in infancy Methods: Between 1985 and 1997. We retrospectively reviewed 105 renal unit (75 patient) with fetal hydronphrosis persisting postnatally. Investigation consisted of renal ultrasonography, voiding cystourethrography, diurectic renogram, and DMSA scan. Results: The 75 patient assesed had the following underlying cause: UPJ(Ureteropelvic juction) obstruction(52%). multicystic dysplastic kidney(10%). UVJ (Ureterovesicai juction) obstruction (10%) and no underlying cause (25%). Of theses cases 36 cases (40 renal unit) underwent operation, while 28 cases (50 renal unit) resoled spontaneously. 12% of mild hydornephrosis deteriorated. whereas 50% of modrate hydrophrosis and 81% of severe hydronephrosis required surgical correction. Attempting to find the renal unit that were at risk for deterioration. our study showed that urinary tract infection group and calyceal blunting group had a predictive role. Conclusion: It is necessary to follow up after birth dilatation of caylx or urinary tract infection are present. Early operation is considered when prenatal pelvic AP diameter greater is than 22 mm and postnatal diameter greater than 17 mm. This may make it possible to prevent further progression of renal damage and prompt treatment of asymptomatic hydronephrosis before complications occur.

• Childhood Kidney Diseases
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• v.3 no.2
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• pp.180-186
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• 1999

Purpose : It has been well known that urinary tract infection(UTI) in infants and children is frequently associated with vesicoureteral reflux(VUR). However, the publishied papers dealing with congenital anomalies associated with UTI emphasized the importance of VUR only. The aim of our study was to evaluate the type, incidence and spectrum of urologic anomalies associated with UTI. Methods : Medical records of clinical, bacteriologic and radiologic study were assessed retrospectively in 65 infants or children with documented UTI who were admitted to the Department of Pediatrics, Samsung Seoul Hospital from March 1996 to February 1998. Results : Spectrum of anomalies were associated with UTI as follows: VUR(n=23), both ectopic kidney(n=1), ureterovesical junction(UVJ) obstruction(n=1), multicystic dysplastic kidney(n=1), ureteropelvic junction(UPJ) obstruction with hydronephrosis(n=1), hutch diverticulum(n=1), UPJ stenosis(n=1), posterior urethral valve(n=1), urachal remnant(n=1) and bladder diverticula(n=1). Congenital urinary anomalies other than VUR were detected in 9 children among 65 patients with UTI(13.8%). 4 children among 9 congenital urinary anomalies other than VUR were combined with VUR. Sex distribution with congenital urinary anomalies other than VUR was more prevalent in male than female (7 males : 2 females). Age distribution at the time of UTI was less than 5 years in most patient (under 1 year in 1 patient, 1-2 year in 5 patients, 3-5 year in 1 patient, and above 5 year in 2 patients). And age distribution at the time of UTl associated with VUR was less than 5 years in most patient (under 1 year in 6 patients, 1-2 year in 8 patients, 3-5 year in 5 patients, and above 5 year in 4 patients), too Conclusion : Because congenital urinary anomalies other than VUR are seen in as high as 13.8% of patients, more careful evaluation of all possible congenital urinary anomalies as well as VUR is mandatory in pediatric patient with UTI.