• Title/Summary/Keyword: 수신증

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Case of Partial Trisomy 9q Derived from Paternal Chromosome (아버지로부터 유래된 9번 염색체 장완의 부분 세염색체 1례)

  • Jung, Ji-Eun;Song, Eun-Jeong;Park, Hye-Jin;Lee, Kye-Hyang;Lee, Kyung-Hoon;Choi, Eun-Jin;Kim, Jin-Kyung;Chung, Hai-Lee;Seo, Eok-Su;Kim, Woo-Taek
    • Neonatal Medicine
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    • v.16 no.1
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    • pp.71-75
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    • 2009
  • There are few cases of partial trisomy of 9q, known as partial 9q trisomy syndrome with low birth weight, microcephaly, hypotelorism, beaked nose, small lip, long finger, hypertrophic pyloric stenosis, ventricular septal defect, and mental retardation. We report partial trisomy of 9q derived from a paternal chromosome, which has different features of other syndromes, including prematurity, atrial and ventricular septal defect, patent ductus arteriosus, persistent left superior vena cava, congenital hydronephrosis, and scrotal hernia.

A Case of Transient Pseudohypoaldosteronism Secondary to Ureteropelvic Junction Obstruction (신우요관이행부폐색과 동반된 일과성 가성저알도스테론혈증 1례)

  • Choi Jun-Gi;Hahn Hye-Won;You Han-Wook;Park Young-Seo
    • Childhood Kidney Diseases
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    • v.8 no.1
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    • pp.91-95
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    • 2004
  • We report a 2-month-old boy who presented with severe hyponatremia and hyperkalemia secondary to ureteropelvic junction(VPJ) obstruction. By prenatal ultrasonography at 19 weeks of gestation, severe hydronephrosis was found which was confirmed postnatally Pyeloplasty was done on the 45th day of life, and fifteen days after pyeloplasty, non-bilious vomiting, decreased activity and dehydration developed. Severe hyponatremia and hyperkalemia were observed, as a result of elevated serum aldosterone and plasma renin activity. The anterior posterior pelvic diameter(APPD) and Society for Fetal Urology(SFU) grade measured showed no interval change before and after pyeloplasty. Pseudohypoaldosteronism was diagnosed, and 2M NaCl was administrated orally for 7 days. The electrolyte imbalance was corrected, and 8 weeks later, the elevated levels of aldosterone and plasma renin activity were normalized. The left hydronephrosis was improved at 5 months of age. We hereby report a transient pseudohypoaldosteronism secondary to UPJ obstruction with a review of the literature.

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A Case of Schinzel-Giedion Syndrome (Schinzel-Giedion 증후군 1례)

  • Jeoung Min-Jee;Yim Hyung-Eun;Hong Young-Sook;Lee Joo-Won;Kim Soon-Kyum;Yoo Kee-Hwan
    • Childhood Kidney Diseases
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    • v.8 no.1
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    • pp.57-62
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    • 2004
  • Schinzel-Giedion syndrome is a rare, distinct dysmorphic syndrome characterized by congenital hydronephrosis, skeletal dysplasia, and severe developmental retardation, likely to be inherited as an autosomal recessive trait, but not yet confirmed. This syndrome is characterized by coarse facial features such as midfacial retraction, bulging forehead, short nose with anteverted nostrils, low-set malformed ears, protruding large tongue, and hypertelorism. Skeletal and limb defects, choanal stenosis, simian creases, hypospadias, microphallus, hypertrichosis, and intractable seizures are the frequently associated clinical findings. Urogenital involvement is a major component of the syndrome, and this problem sometimes is associated with nephrocalcinosis and urinary tract infection in the clinical course of the disease. We report a 22 month-old girl with Schinzel-Giedion syndrome complicated by medullary nephrocalcinosis and urinary tract infection due to Klebsiella pneumoniae. This patient had also been suffering from postnatal growth deficiency, intractable seizure, spastic tetraplegia, delayed development and severe mental retardation.

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Treatment of Experimental Hydronephrosis Using Renal Artery Embolization and Sclerotherapy in Beagle Dogs (신동맥색전술과 신우경화술을 이용한 비글견의 실험적 수신증 치료)

  • Chang Dong-woo;Lee Young-won;Shin Sang-tae
    • Journal of Veterinary Clinics
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    • v.22 no.2
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    • pp.119-124
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    • 2005
  • This study was performed to validate the procedure of transarterial embolization of the renal artery (TAE-RA) and sclerotherapy of renal pelvis using iohexol-ethanol solution in dogs with unilateral experimental hydronephrosis. Experimental hydronephrosis was induced by unilateral ureter ligation for 20 days in five Beagle dogs. Renal artery embolization with iohexol-ethanol solution was performed using selective catheterization technique in the hydronephrotic kidney and sclerotherapy was done by injection of the iohexol-ethanol solution through percutaneously placed pig-tail catheter. EKG, $SpO_2$ body temperature, pulse, and respiratory rate were within normal ranges during procedures. Average pure ethanol dose for renal artery embolization was $1.1\pm0.3ml/kg$. Renal artery embolization was confirmed by the detection of no blood flow signal at the interlobar and arcuate artery using color Doppler ultrasonography. There were no dogs expired after TAE-RA and sclerotherapy and no side effects associated with regurgitation of iohexol-ethanol solution. The value of BUN, creatinine, ALT, AST, Ca, P in five dogs were within normal range during the experiment period. Ultrasonographically, the mean longitudinal and transverse length and the depth of the embolized kidney significantly decreased at 28 days after TAE-RA. We may conclude that TAE-RA and sclerotherapy with iohexol-ethanol solution is an effective methods for the treatment of unilateral hydronephrosis in dogs.

Unusual Manifestation of Immunoglobulin G4-Related Disease Involving the Retroperitoneum: A Case Report (후복막강에 발생한 Immunoglobulin G4 연관 질환의 비전형적 발현: 증례 보고)

  • Byungsoo Kim;Jung Wook Seo
    • Journal of the Korean Society of Radiology
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    • v.82 no.2
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    • pp.487-492
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    • 2021
  • Immunoglobulin G4 (IgG4)-related disease is a systemic disease characterized by dense lymphoplasmacytic infiltrates with abundant IgG4-positive plasma cells and fibroblast proliferation. The retroperitoneal involvement of IgG4-related disease usually appears as a soft-tissue mass covering the abdominal aorta or entrapping the ureters, resulting in hydronephrosis. Here, we present a case of IgG4-related disease with retroperitoneal involvement in a 75-yearold woman with an unusual manifestation. A preoperative computed tomography (CT) scan revealed an irregular infiltrative retroperitoneal mass invading the normal anatomic barriers, raising the suspicion of malignancy or inflammation. Contrast-enhanced CT revealed a homogeneous progressive enhancement of the mass.

Incidence and outcome of congenital anomalies of the kidney and urinary tract detected by prenatal ultrasonography: a single center study (산전 초음파로 발견된 선천성 신 요로 기형의 발생률과 임상 결과: 단일 기관 연구)

  • Lim, Gina;Lee, Joo Hoon;Park, Young Seo;Kim, Kun Seok;Won, Hye-Sung
    • 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.

Urinary tract infections in infants under six months of age (6개월 미만 영아의 요로 감염)

  • Kang, Min Joo;Shin, Hye Kyung;Yim, Hyung Eun;Je, Bo-Kyung;Eun, So Hee;Choi, Byung Min;Park, Jong-Tae;Eun, Baik Lin;Yoo, Kee Hwan
    • Clinical and Experimental Pediatrics
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    • v.49 no.3
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    • pp.278-286
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    • 2006
  • Purpose : Although suprapubic bladder aspiration(SBA) is the most reliable technique for identifying bacteriuria in young infant, no report has been published about the clinical manifestations of urinary tract infection(UTI) diagnosed by aspirated urine in Korea. This study was performed to examine clinical manifestations and related factors of UTI confirmed by a positive urine culture obtained by SBA in young infants. Methods : We reviewed medical records of 159 infants younger than six months of age, who had been admitted for UTI to Korea University Ansan Hospital from January 2002 to June 2005. Results : The male:female ratio was 5.1 : 1. The most common pathogen in urine culture was Escherichia coli(92.5 percent). Most of the gram-negative pathogens had high sensitivity to amikacin, or third generation cephalosporins and had low sensitivity to ampicillin, trimethoprim-sulfamethoxazole, or gentamicin. Hydronephrosis and vesicoureteral reflux(VUR) were present in 32.7 percent and 27.7 percent respectively. Renal cortical defects observed on DMSA scans were detected in 37.1 percent. Prolonged fever duration and hospital day, high erythrocyte sedimentation rate(ESR) and C-reactive protein(CRP) levels, hydronephrosis and VUR were related to the renal cortical defects(P<0.05). Rates of UTI-associated bacteremia and aseptic meningitis were 8.3 percent and 6.6 percent. Conclusion : Early differential diagnosis is very important in infants younger than 6 months of age with UTI because the clinical findings are non-specific and UTI-associated bacteremia and aseptic meningitis are concomitantly found. Because prolonged fever and higher ESR and CRP levels are risk factors of the renal cortical defects, radiologic evaluations and nephrologic follow-up were needed in identifying the predisposing congenital abnormalities and chronic renal scarring.

Handoff Method Supporting LBS Information in Mobile Clouding Computing (이동 클라우딩 환경에서 LBS 지원 핸드오프 기법)

  • Kim, Ki-Young;Kim, Sun-Jib
    • Journal of the Korea Society of Computer and Information
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    • v.20 no.2
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    • pp.89-97
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    • 2015
  • In this paper, we propose a handoff method supporting LBS (Location Based Services) Information in mobile clouding environment. In mobile clouding computing, handoff delay and re-authentication is occurred. A mobile node needs re-authentication procedure from cloud server whenever it arrives new AP. But Using of location information of node enables to reduce delay time due to re-authentication. To reduce re-authentication delay time, proposed method stores location information of APs on WiFi based location server to complement. GPS-based technology which can't receive satellite signal in indoor and then node collects location information of AP at handoff time. And also enables to process LBS without increasing handoff delay by splitting the process of handoff from process of requesting location information. For analysis of proposed method, We analyze handoff delay and location information process time and have compared previous handoff method in cloud environment. We confirmed that proposed method shows lower delay time without increasing LBS process time than previous method because node receives location information from location information server when handoff is occurred.

Congenital Urinary Tract Anomalies Associated with Urinary Tract Infection in Infants and Children (요로감염증과 연관된 방광요관역류 이외의 선천성 요로계 이상에 관한 고찰)

  • Chung So-Hee;Kim Jung-Sim;Kim Hyun-Jung;Lee Mi-Na;Uhm Mee-Ryung;Jin Dong-Kyu;Shin Hwa-Sook
    • 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.

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Clinical Manifestation and Associated Cardiac Anomalies of Dextrocardia (우심증에 동반된 심기형과 임상양상)

  • Kim, Sung Hoon;Hong, Seung A;Cho, Yoon Jung;Lee, Sang Lak;Kwon, Tae Chan
    • Clinical and Experimental Pediatrics
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    • v.45 no.5
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    • pp.622-628
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    • 2002
  • Purpose : The purpose of this study is to ascertain the clinical manifestations and associated cardiac anomalies of dextrocardia. Methods : Twenty-four pediatric patients, who were admitted to, or visited, Dongsan Medical Center Keimyung University and were diagnosed with dextrocardia between January 1996 and July 2001, were enrolled in this study. All patients received echocardiogram and abdominal ultrasonogram to identify structural cardiac anomalies and visceral position. Results : Among 24 patients, 7 cases were situs solitus(29.2%), 10 cases(41.6%) were situs inversus and 7 cases(29.2%) were situs ambiguous. Most were diagnosed within a week of life(87.5%). They were most commonly cyanotic(45.8%), but eleven cases(45.8%) had no specific symptoms or signs. Nineteen cases(79.2%) had accompanied cardiac anomalies, and pulmonary stenosis or atresia were the most common among them. All the cases of situs solitus and ambiguous had associated cardiac anomalies, but half of the situs inversus had that. Eleven cases were dead by cardiac or extracardiac anomalies during follow up and the mortality rate was higher in situs solitus or ambiguous group than situs inversus group. Conclusion : Dextrocardia accompanies different cardiac and extracardiac anomalies. It's very important to diagnose dextrocardia and associated cardiac or extracardiac anomalies in the early stages of life to improve prognosis.