• Childhood Kidney Diseases
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• v.9 no.1
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• pp.21-30
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• 2005

Purpose : Metabolic bone diseases have been mai or problems in children with renal diseases and steroid treatment is the main precipitating factor reducing bone mineral density(BMD). This study was performed to assess the prevalence of osteoporosis and to evaluate the clinical factors associated with decreased BMD in children with renal diseases. Methods : Forty-four children with renal diseases who were diagnosed at the Pediatric no phrology division of Ajou University hospital since Oct. 1994 were included. Using a new quantitative ultrasound device, BMD and the prevalence of osteoporosis were evaluated. The clinical and serological data were analyzed in association with decreased BMD. Results : A total of 44 patients were evaluated. The age at initial diagnosis was 6.7$\pm$4.2 years. At the time of evaluation, the chronological and bone age was 9.3$\pm$4.2 years and 8.2 $\pm$ 4.6 years, respectively. The renal diseases included nephrotic syndrome 24(54.5%), Henoch Schonlein purpura nephritis 7(15.9%), IgA nephropathy 6(13.9%), reflux nephropathy(RN) 2 (4.5%), and other renal disease 5(%). The prevalence of osteoporosis was 11%. There was no difference in the clinical factors between the long-term and the short-term treated steroid groups. Conclusion : The prevalence of osteoporosis was 12% in 44 children with renal diseases No significant factor was found in association with decreased BMD and there was no relationship between osteoporosis and steroid usage duration or cumulative dose. A new quantitative ultrasound, which is relatively easy to perform, especially in children, is expected to be in common use and will enable clinicians to evaluate metabolic bone disorders with ease.

• Childhood Kidney Diseases
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• v.10 no.1
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• pp.27-32
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• 2006

Purpose : Conservative management of multicystic dysplastic kidney(MCDK) without nephrectomy has recently been advocated. The purpose of this study was to determine the clinical course of conservatively managed MCDK and to find out possible predictive factors for involution of MCDK by ultrasonography(US). Methods : A retrospective analysis was made on 45 patients(26 boys and 20 girls) in whom MCDK was detected and had been traced by US between Dec. 1993 and Aug. 2005 at Severance Hospital. Results : Median follow-up time was 30 months(range 2-102 months). All patients under-went radionuclide scans and voiding eystourethrograms. The serial follow-up US showed complete involution in 11(24%), partial involution in 19(41%), and no interval change or increased in cyst size in 13(28%) patients. Nephrectomy was done in 3 patients(7%) due to relapsing urinary tract infection(UTI) and severe abdominal distension. The mean age of complete involution of MCDK was 37 months(range 12-84 months). Episodes of UTI were present in 17 patients(37%) and additional genitourinary(GU) abnormalities were found in 22 patients(44%). Hypertension and renal insufficiency was complicated in one patient. No child developed malignant tumor. Univariate analysis showed that five variables were associated with complete involution of the MCDK; gender, site, UTI episode, additional GU abnormalities, and renal length on initial US. After adjusting using the Pearson model, the presence of additional GU abnormalities was exclusively associated with complete involution among the 5 variables(P=0.034). Conclusion : In our review of 46 cases of MCDK, non-surgical approach for patients with MCDK was advisable and we could predict poor prognosis when MCDK is associated with other GU anomalies.

• Childhood Kidney Diseases
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• v.8 no.2
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• pp.205-213
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• 2004

Purpose: To evaluate the clinical manifestations of various glomerular diseases in children, a clinicopathological study was performed in 52 children who had renal biopsy. The type and relative incidence of the glomerular pathologies were analyzed, and the clinical predictability and usefulness of renal biopsy in glomerular diseases were assessed. Methods: Medical records of fifty two children with renal disease who had undergone percutaneous renal biopsy under ultrasonic guidance at Chungnam University Hospital from October 1995 to August 2003 were reviewed. In addition, we compared the clinical findings before renal biopsy with the pathological diagnosis. Results: The male to female ratio was 1.6:1 and they were $9.8\pm2.6$ years old on average. The chief complaints for biopsy were hematuria in 22 cases which was the most common (42.3%), proteinuria in 16 cases(30.8%), and hematuria & proteinuria(26.9%). Among the 22 cases of hematuria, there were 15 cases of gross hematuria(68.2%) and 7 cases of microscopic hematuria(31.8%). In terms of histopathologic diagnosis, most of them were primary glomerular diseases(84.6%), which included IgA nephropathy(28.8%), thin glomerular basement membrane disease(25.0%), focal segmental glomerulosclerosis(FSGS)(11.5%), membranous proliferative glomerulonephritis(7.7%), minimal change lesion(3.8%), acute poststreptococcal glomerulonephritis(3.8%) and membranous glomerulonephritis(3.8%). The clinical manifestations and pathologic diagnosis were not correlated. Conclusion: The clinical manifestations could not predict the pathological diagnosis. Therefore, renal biopsy would be inevitable in diagnosis of glomerular diseases for effective management and assessment of prognosis.

• Clinical and Experimental Pediatrics
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• v.45 no.2
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• pp.232-239
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• 2002

Purpose : Renal cystic diseases comprise a mixed group of heritable, developmental and acquired disorders. Recently the use of imaging modalities such as ultrasonography and radionuclide scanning has increased the detection rate of renal cystic diseases. We studied to review the clinical features and treatment of renal cystic diseases in children. Methods : This study was performed in 95 children with renal cystic diseases in the Department of Pediatrics, Asan Medical Center from October 1989 to June 2001. Results : In 95 patients, there were 55 cases(58.0%) with multicystic dysplastic kidney(MCDK), 19 cases(20.0%) with simple renal cysts, 13 cases(13.7%) with hereditary polycystic kidney diseases( 7 with autosomal recessive type, 5 with autosomal dominant type, 1 with undetermined), 6 cases(6.3%) with renal cysts in tuberous sclerosis and 1 case(1.0%) with medullary cystic disease. All MCDK patients had no renal dysfunction and hypertension during the follow-up period. Three out of 13 with polycystic kidney diseases had progressed to end-stage renal disease during the follow-up period. One case with a simple cyst underwent laparoscopic malsupialization for decompression. Conclusion : Renal cystic diseases have diverse clinicopathologic features and variable prognosis. We emphasize that routine follow-up should be performed to prevent and to detect early treatable complication in renal cystic diseases. Therefore, their natural history and treatment need further investigation and long term follow-up is required.

• Tuberculosis and Respiratory Diseases
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• v.45 no.4
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• pp.736-745
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• 1998

Background: Respiratory muscle interaction is further profoundly affected by a number of pathologic conditions. Hyperinflation may be particularly severe in chronic obstructive pulmonary disease(COPD) patients, in whom the functional residual capacity(FRC) often exceeds predicted total lung capacity(TLC). Hyperinflation reduces the diaphragmatic effectiveness as a pressure generator and reduces diaphragmatic contribution to chest wall motion. Ultrasonography has recently been shown to be a sensitive and reproducible method of assessing diaphragmatic excursion. This study was performed to evaluate how differences of diaphragmatic excursion measured by ultrasonography associate with normal subjects and COPD patients. Methods: We measured diaphragmatic excursions with ultrasonography on 28 healthy subjects(l6 medical students, 12 age-matched control) and 17 COPD patients. Ultrasonographic measurements were performed during tidal breathing and maximal respiratory efforts approximating vital capacity breathing using Aloka KEC-620 with 3.5 MHz transducer. Measurements were taken in the supine posture. The ultrasonographic probe was positioned transversely in the midclavicular line below the right subcostal margin. After detecting the right hemidiaphragm in the B-mode the ultrasound beam was then positioned so that it was approximately parallel to the movement of middle or posterior third of right diaphragm. Recordings in the M-mode at this position were made throughout the test. Measurements of diaphragmatic excursion on M-mode tracing were calculated by the average gap in 3 times-respiration cycle. Pulmonary function test(SensorMedics 2800), maximal inspiratory(PImax) and expiratory mouth pressure(PEmax, Vitalopower KH-101, Chest) were measured in the seated posture. Results: During the tidal breathing, diaphragmatic excursions were recorded $1.5{\pm}0.5cm$, $1.7{\pm}0.5cm$ and $1.5{\pm}0.6cm$ in medical students, age-matched control group and COPD patients, respectively. Diaphragm excursions during maximal respiratory efforts were significantly decreased in COPD patients ($3.7{\pm}1.3cm$) when compared with medical students, age-matched control group($6.7{\pm}1.3cm$, $5.8{\pm}1.2cm$, p< 0.05}. During maximal respiratory efforts in control subjects, diaphragm excursions were correlated with $FEV_1$, FEVl/FVC, PEF, PIF, and height. In COPD patients, diaphragm excursions during maximal respiratory efforts were correlated with PEmax(maximal expiratory pressure), age, and %FVC. In multiple regression analysis, the combination of PEmax and age was an independent marker of diaphragm excursions during maximal respiratory efforts with COPD patients. Conclusion: COPD subjects had smaller diaphragmatic excursions during maximal respiratory efforts than control subjects. During maximal respiratory efforts in COPD patients, diaphragm excursions were well correlated with PEmax. These results suggest that diaphragm excursions during maximal respiratory efforts with COPD patients may be valuable at predicting the pulmonary function.

• Clinical and Experimental Pediatrics
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• v.50 no.12
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• pp.1231-1240
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• 2007

Purpose : Bartter syndrome is a renal tubular defect in electrolyte transport characterized by hypokalemia, metabolic alkalosis and other clinical signs and symptoms. The aims of this study were to analyze the clinical manifestations and the short- and long-term outcomes of Bartter syndrome. Methods : We retrospectively reviewed clinical history, laboratory finding of blood and urine, renal ultrasonography, and hearing tests of five patients who were diagnosed and managed with Bartter syndrome at Asan Medical Center from April 1992 to May 2007. We also evaluated height and body weight periodically after institution of therapy. Results : All patients had poor oral intake, failure to thrive and polyuria. Three of them had maternal history of polyhydramnios and premature delivery. The mean age at diagnosis was 11.8 months. All children presented with hypokalemia, metabolic alkalosis, hyperreninemia. Their blood pressures were normal. One patient had nephrocalcinosis on renal ultrasonography and all of them had normal result in hearing tests. After treatment with indomethacin or other prostaglandin inhibitors and potassium supplementation,their clinical features improved with catch-up growth and improvement in the development during long-term follow-up. Conclusion : We emphasize that early diagnosis and proper treatment in patient with Bartter syndrome are related to better prognosis.

• Journal of Veterinary Clinics
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• v.30 no.4
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• pp.292-295
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• 2013

A 9-year-old, male, Doberman pinscher dog with 5-month history of intermittent hematuria, vomiting and glucosuria was referred to local animal hospital. Abdominal ultrasonography showed an irregular and hyperechoic mass in the renal medulla of the enlarged left kidney. Grossly atrophied renal cortex and medulla and marked hydronephrosis were observed on the cut surface of kidney. A single, numerous papillary projected, pedunculated mass 4~5.5 cm in diameter was occupied in renal pelvis, and extended from pelvis to the inlet of ureter. Histopathologically, the mass had numerous papillary structures with arboriform pattern. These papillae were consisted of fibro-vascular stalks that were lined by multiple layers of neoplastic urothelium (transitional epithelium) with marked cellular atypia. Immnohistochemical (IHC) staining demonstrated that the neoplastic cells showed strong positive reactions for cytokeratin (CK) 7, CK 19, CK clone MNF116 and CK high molecular weight, but negative signals for CK 8 low molecular weight. Based on the gross findings, histopathology and CKs profile using IHC staining, this mass was diagnosed as renal pelvis transitional cell carcinoma in a dog.

• Childhood Kidney Diseases
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• v.17 no.2
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• pp.122-126
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• 2013

Cystinuria is an autosomal recessive disease characterized by impaired transport of cystine and dibasic amino acids in the proximal renal tubule, resulting in the formation of cystine stones. It is believed to account for about 1% of all kidney stones and up to 10% of pediatric stones. Here we report a case of cystinuria with multiple renal stones confirmed by genetic mutational analysis. An 8-month-old girl was admitted to AMC with persistent fever and multiple renal stones. A renal sonogram showed multiple stones at the right renal pelvis, right distal ureter, and left renal medullary portion. An approximately 1 cm renal stone was extracted spontaneously, and stone analysis revealed it to be composed entirely of cystine. Cystinuria was confirmed by increased urine dibasic amino acid levels, including cysteine, and genetic mutational analysis showed the patient to be a homozygote for the pathogenic c. 1820del (p.L607fs) of SLC3A1. Despite treatment with oral hydration and urinary alkalinization, and restricted intake of animal protein, the stones increased in size and number. The patient has since been treated with tiopronin.

• Clinical and Experimental Pediatrics
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• v.49 no.1
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• pp.87-92
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• 2006

Purpose : It is difficult to make a distinction between lower urinary tract infection(UTI) and acute pyelonephritis(APN) during the acute phase of febrile UTI due to nonspecific clinical symptoms and laboratory findings, especially among young children. We measured the serum procalcitonin(PCT) in children with UTI to distinguish between acute pyelonephritis and lower UTI, and to determine the accuracy of PCT measurement compared with other inflammatory markers. Methods : Serum samples were taken from children who admitted with unexplained fever or were suspected of having UTI. 51 children(mean $12.2{\pm}11.4$ months) were enrolled in this study. Leukocyte counts, erythrocyte sedimentation rates(ESR) and C-reactive protein(CRP) were also measured. Renal parenchymal involvement was assessed by $^{99m}Tc$ DMSA scintigraphy in the first 7 days after admission. PCT was measured by immunoluminometric assay. Results : PCT values were significantly correlated with the presence of renal defects in children with UTI(n=16)($5.06{\pm}12.97{\mu}g/L$, P<0.05). However, PCT values were not significantly different between children with UTI without renal damage(n=18) and children without UTI(n=17). Using a cutoff of $0.5{\mu}g/L$ for PCT and 20 mm/hr for ESR, 20 mg/L for CRP, sensitivity and specificity in distinguishing between UTI with and without renal involvement were 81.3 percent and 88.9 percent for PCT 87.5 percent and 72.2 percent for ESR, and 87.5 percent and 55.6 percent for CRP, respectively. Positive and negative predictive values were 86.7 percent and 84.2 percent for PCT and 60.9 percent and 81.8 percent for CRP, respectively. Conclusion : In febrile UTI, PCT values were more specific than CRP, ESR and leukocyte count for the identification of patients who might develop renal defects.

• Childhood Kidney Diseases
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• v.15 no.2
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• pp.184-190
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• 2011

Scrotal involvement has been reported from 2% to 38% of males with Henoch-Sch$\ddot{o}$nlein purpura (HSP). It may present before the rash occurs or even long time after it has disappeared. Scrotal involvement of HSP usually results in pain, tenderness, swelling or discoloration of scrotum. Scrotal pain sometimes mimics testicular torsion to various degrees, which can be followed by unnecessary operation. In our 2 cases, one was a 5-year and 11-month-old boy who came to our emergency department due to scrotal pain before the diagnosis of HSP, and the other was a 5-year and 1-month-old boy who came to the outpatient clinic due to scrotal pain after the resolution of HSP about 1 month before the visit. We performed Doppler ultrasonography (USG) to evaluate the acute scrotal pain in the two boys. On Doppler USG, epididymis showed increased blood flow, and testis showed normal blood flow. These findings enabled the diagnosis of acute epididymitis and excluded testicular torsion. Epididymitis was improved by conservative management including shortterm steroid therapy within 5 days. It is important to perform adequate evaluation with tools such as Doppler USG in the early course of acute scrotum of HSP, to avoid unnecessary scrotal exploration.