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    ๊ณ ์ถ”์˜ ์ž๊ฐ€ํ™œ์„ฑ Nucleotide-binding Leucine-rich Repeat ์œ ์ „์ž๊ฐ€ ์œ ๋„ํ•˜๋Š” ์„ธํฌ์‚ฌ๋ฉธ๊ธฐ์ž‘ ์—ฐ๊ตฌ

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    ํ•™์œ„๋…ผ๋ฌธ (๋ฐ•์‚ฌ)-- ์„œ์šธ๋Œ€ํ•™๊ต ๋Œ€ํ•™์› : ๋†์—…์ƒ๋ช…๊ณผํ•™๋Œ€ํ•™ ์›์˜ˆํ•™๊ณผ, 2019. 2. ์ตœ๋„์ผ.Plants possess hundreds of intracellular immune receptors encoding nucleotide-binding domain and leucine-rich repeat (NLR) proteins that can recognize pathogen effectors. Activated NLRs confer disease resistance that is often accompanied by localized cell death termed by hypersensitive response. NLR proteins typically consist of three major domains, an N-terminal TOLL/interleukin-1 receptor (TIR) or coiled-coil (CC) domain, a central nucleotide binding (NB-ARC) domain, and a C-terminal leucine-rich repeat (LRR) domain. The CC domain is known to function as a signal inducer and remains in an auto-inhibited state through intramolecular interaction with NB-ARC and LRR domains in absence of pathogen infection. In this study, cell death induced by genome-wide autoactive pepper NLRs and their N-terminal domain was analyzed by transient overexpression in Nicotiana benthamiana. The screening assay revealed that CC domain of group 10 (G10)-NLRs specifically trigger HR-like cell death. Moreover, G10-CC domain from other Solanaceae plants (tomato and tobacco) induced cell death. The G10-NLR or G10-CC domain-mediated cell death appears to mimic the HR cell death triggered by resistance protein and effector as demonstrated by the requirement of molecular chaperone complex for NLR immune signaling and upregulation of HR- and defense-related genes. VIGS-based screen designed to identify G10-NLR signaling components revealed that cell death induced by G10-NLR and G10-CC domain occurred in a SA/JA-independent manner. In addition, deletion and mutation analyses showed that the primary ฮฑ-helix in G10-CC domains contribute to cell death signaling rather than to the proper targeting of the protein to the plasma membrane. To understand the molecular basis of G10-NLR-mediated ell death, I identified S-adenosyl homocysteine hydrolase (SAHH) as a candidate of interacting protein with G10-CC domain using pull-down experiment coupled with LC-MS/MS analysis. Cell death induced by G10-NLR and G10-CC domain was compromised by co-expression with SAHH, suggesting that SAHH may function as a negative regulator of G10-NLRs. In addition, the SAHH-silenced plants exhibited constitutive H2O2 accumulation in absence of pathogen, implying that SAHH is a negative regulator of reactive oxygen species production. How G10-NLRs trigger cell death response remains unclear, but this study gives a clue to understand the molecular mechanisms underlying HR cell death induced by activated NLRs and the distinct role of G10-NLRs in plant immune responses.์‹๋ฌผ์€ ์˜ค๋žœ ์‹œ๊ฐ„ ๋™์•ˆ ๋ณ‘์›๊ท ์— ๋Œ€์‘ํ•ด์˜ค๋ฉด์„œ ๋‹ค์–‘ํ•œ ์ €ํ•ญ์„ฑ ๊ธฐ์ž‘์„ ์ง„ํ™”์‹œ์ผœ ์™”๋‹ค. ๊ทธ ์ค‘ nucleotide-binding domain and leucine-rich repeat (NLR) ์œ ์ „์ž๋Š” ๋ณ‘์›๊ท ์ด ์‹๋ฌผ์— ๋ณ‘์„ ๋‚ด๊ธฐ ์œ„ํ•ด ์‹๋ฌผ ์„ธํฌ ๋‚ด๋กœ ๋ถ„๋น„ํ•˜๋Š” effector ๋‹จ๋ฐฑ์งˆ์„ ์ธ์ง€ํ•˜์—ฌ, ์‹๋ฌผ์ด ์ €ํ•ญ์„ฑ ๋ฐ˜์‘๋“ค์„ ๋‚˜ํƒ€๋‚ผ ์ˆ˜ ์žˆ๋„๋ก ํ•˜๋Š” ๋Œ€ํ‘œ์ ์ธ ์ €ํ•ญ์„ฑ ์œ ์ „์ž์ด๋‹ค. Effector๋ฅผ ์ธ์ง€ํ•œ NLR ๋‹จ๋ฐฑ์งˆ์€ ๊ตฌ์กฐ ๋ณ€ํ™”๋ฅผ ํ†ตํ•ด ํ™œ์„ฑ ์ƒํƒœ๋กœ ์ „ํ™˜๋˜๊ณ  ๊ทธ ์ดํ›„ ์ €ํ•ญ์„ฑ ๋ฐ˜์‘์„ ์œ ๋„ํ•˜๊ฒŒ ํ•˜๋Š”๋ฐ, ๊ทธ ์ค‘ ๊ฐ€์žฅ ๊ฐ•๋ ฅํ•œ ์ €ํ•ญ์„ฑ ๋ฐ˜์‘์œผ๋กœ ์„ธํฌ์‚ฌ๋ฉธ (hypersensitive cell death)์ด ์žˆ๋‹ค. NLR์€ ํฌ๊ฒŒ 3๊ฐœ์˜ ์ฃผ์š” ๋„๋ฉ”์ธ์œผ๋กœ ๊ตฌ์„ฑ๋˜์–ด ์žˆ๋‹ค. C-๋ง๋‹จ์—๋Š” LRR๋„๋ฉ”์ธ, ์ค‘์•™์—๋Š” NB-ARC ๋„๋ฉ”์ธ, ๊ทธ๋ฆฌ๊ณ  N-๋ง๋‹จ์—๋Š” TIR ๋˜๋Š” CC๋„๋ฉ”์ธ์ด ์กด์žฌํ•œ๋‹ค. NLR์€ N-๋ง๋‹จ ๋„๋ฉ”์ธ์— ๋”ฐ๋ผ TNL ๊ณผ CNL ๋กœ ๋‚˜๋ˆ„์–ด ์ง„๋‹ค. N-๋ง๋‹จ ๋„๋ฉ”์ธ์€ NLR ๋‹จ๋ฐฑ์งˆ์˜ ์‹ ํ˜ธ๋ฅผ ์ „๋‹ฌํ•˜๋Š” ์—ญํ• ์„ ํ•˜๋ฉฐ ๋ณ‘์›๊ท ์ด ์กด์žฌํ•˜์ง€ ์•Š์€ ์ƒํ™ฉ์—์„œ๋Š” NB-ARC ๋˜๋Š” LRR๋„๋ฉ”์ธ๊ณผ ์ƒํ˜ธ์ž‘์šฉ์„ ํ†ตํ•ด ๊ทธ ๊ธฐ๋Šฅ์ด ์–ต์ œ ๋˜๊ณ  ์žˆ๋‹ค๊ณ  ์•Œ๋ ค์ ธ ์žˆ๋‹ค. ๋ณธ ์—ฐ๊ตฌ์—์„œ๋Š” ๊ณ ์ถ”์— ์กด์žฌํ•˜๋Š” NLR ์œ ์ „์ž 415๊ฐœ์— ๋Œ€ํ•˜์—ฌ NLR์ „์ฒด์™€ N-๋ง๋‹จ ๋„๋ฉ”์ธ์˜ ์„ธํฌ์‚ฌ๋ฉธํ™œ์„ฑ์„ Agrobacterium์„ ์ด์šฉํ•œ ์ผ์‹œ์  ๊ณผ๋ฐœํ˜„์„ ํ†ตํ•ด ์Šคํฌ๋ฆฌ๋‹ํ•˜์˜€๋‹ค. ๊ทธ ๊ฒฐ๊ณผ ๊ทธ๋ฃน10๋ฒˆ NLR์˜ CC๋„๋ฉ”์ธ์ด ํŠน์ด์ ์œผ๋กœ ๋†’์€ ๋น„์œจ๋กœ ์„ธํฌ์‚ฌ๋ฉธํ™œ์„ฑ์„ ๊ฐ€์ง€๋Š” ๊ฒƒ์œผ๋กœ ํ™•์ธ๋˜์—ˆ๋‹ค. ๊ณ ์ถ” ์™ธ ๋‹ค๋ฅธ ๊ฐ€์ง€๊ณผ ์‹๋ฌผ์ธ ํ† ๋งˆํ† ์™€ ๋‹ด๋ฐฐ์˜ ๊ทธ๋ฃน10๋ฒˆ NLR์˜ CC๋„๋ฉ”์ธ๋„ ์„ธํฌ์‚ฌ๋ฉธํ™œ์„ฑ์„ ๊ฐ€์ง€๋Š” ๊ฒƒ์œผ๋กœ ๋‚˜ํƒ€๋‚ฌ๋‹ค. ๊ทธ๋ฃน10๋ฒˆ NLR์˜ CC๋„๋ฉ”์ธ์ด ์œ ๋„ํ•˜๋Š” ์„ธํฌ์‚ฌ๋ฉธ์ด ์ผ์–ด๋‚˜๋Š”๋ฐ ์žˆ์–ด NLR์˜ molecular chaperone complex ๊ฐ€ ํ•„์š”ํ•˜๋ฉฐ, ์„ธํฌ์‚ฌ๋ฉธ์ด ์ง„ํ–‰๋˜๋Š” ๋™์•ˆ ์‹๋ฌผ์˜ ์ €ํ•ญ์„ฑ๋ฐ˜์‘์— ๊ด€์—ฌํ•˜๋Š” ์œ ์ „์ž๋“ค์˜ ๋ฐœํ˜„์ด ์ฆ๊ฐ€๋จ์„ ํ†ตํ•ด ๊ทธ๋ฃน10๋ฒˆ NLR์˜ CC๋„๋ฉ”์ธ์˜ ๊ณผ๋ฐœํ˜„์„ ํ†ตํ•ด ์œ ๋„๋œ ์„ธํฌ์‚ฌ๋ฉธ์ด ์‹๋ฌผ์˜ ์ €ํ•ญ์„ฑ ๊ธฐ์ž‘๊ณผ ๊ด€๋ จ ์žˆ์Œ์„ ์ž…์ฆํ•˜์˜€๋‹ค. ๋‹ค์Œ์œผ๋กœ ๊ทธ๋ฃน10๋ฒˆ NLR์˜ CC๋„๋ฉ”์ธ์ด ์œ ๋„ํ•˜๋Š” ์„ธํฌ์‚ฌ๋ฉธ์— ๊ด€์—ฌํ•˜๋Š” ํ•˜์œ„ ์‹ ํ˜ธ์ „๋‹ฌ ์ฒด๊ณ„๋ฅผ ๊ทœ๋ช…ํ•˜๊ธฐ ์œ„ํ•˜์—ฌ VIGS๋ฅผ ์ด์šฉํ•œ ์Šคํฌ๋ฆฌ๋‹์„ ์ง„ํ–‰ํ•˜์˜€๋‹ค. NLR ํƒ€์ž… ์ €ํ•ญ์„ฑ ์œ ์ „์ž๊ฐ€ ๋งค๊ฐœํ•˜๋Š” ์ €ํ•ญ์„ฑ์˜ ์ค‘์š” ์กฐ์ ˆ์ธ์ž์ธ EDS1๊ณผ NDR1, ๊ทธ๋ฆฌ๊ณ SA ํ˜ธ๋ฅด๋ชฌ ์ƒํ•ฉ์„ฑ ๋ฐ ํ•˜์œ„ ์‹ ํ˜ธ์ „๋‹ฌ์€ ๊ทธ๋ฃน10๋ฒˆ NLR์˜ CC๋„๋ฉ”์ธ์ด ์œ ๋„ํ•˜๋Š” ์„ธํฌ์‚ฌ๋ฉธ์— ์žˆ์–ด ํ•„์ˆ˜์ ์ด์ง€ ์•Š์Œ์„ ๋ฐํ˜”๋‹ค. ๋˜ํ•œ ๊ทธ๋ฃน10๋ฒˆ NLR์˜ CC๋„๋ฉ”์ธ๋“ค์˜ ๊ตฌ์กฐ ๋ถ„์„์„ ํ†ตํ•œ ๊ธฐ๋Šฅ ์—ฐ๊ตฌ๋ฅผ ํ†ตํ•ด CC๋„๋ฉ”์ธ์˜ ์ฒซ๋ฒˆ์งธ ฮฑ-helix๊ฐ€ ์„ธํฌ์‚ฌ๋ฉธํ™œ์„ฑ์— ์ค‘์š”ํ•จ์„ ํ™•์ธํ•˜์˜€๋‹ค. ๊ทธ๋ฃน10๋ฒˆ NLR์˜ CC๋„๋ฉ”์ธ์˜ ์„ธํฌ์‚ฌ๋ฉธ ๊ธฐ์ž‘์„ ์ดํ•ดํ•˜๊ธฐ ์œ„ํ•˜์—ฌ CC ๋„๋ฉ”์ธ์˜ ์ƒํ˜ธ์ž‘์šฉ์ธ์ž๋ฅผ ๋™์ •ํ•˜๊ธฐ ์œ„ํ•œ pull-down ๋ฐ LC-MS/MS์‹คํ—˜์„ ์ง„ํ–‰ํ•œ ๊ฒฐ๊ณผ S-adenosyl homocysteine hydrolase (SAHH) ๊ฐ€ CC๋„๋ฉ”์ธ์˜ ์ƒํ˜ธ์ž‘์šฉ ํ›„๋ณด ์œ ์ „์ž๋กœ ๋™์ •๋˜์—ˆ๋‹ค. SAHH๋Š” CC๋„๋ฉ”์ธ๊ณผ ๋™์‹œ ๊ณผ๋ฐœํ˜„์‹œ CC๋„๋ฉ”์ธ์ด ์œ ๋„ํ•˜๋Š” ์„ธํฌ์‚ฌ๋ฉธ์„ ์–ต์ œํ•˜๋Š” ๊ฒƒ์œผ๋กœ ๋ณด์•„ CC๋„๋ฉ”์ธ์˜ ์„ธํฌ์‚ฌ๋ฉธํ™œ์„ฑ์˜ negative regulator๋กœ์„œ์˜ ์—ญํ• ์„ ํ•˜๋Š” ๊ฒƒ์œผ๋กœ ์ถ”์ธก๋œ๋‹ค. SAHH์œ ์ „์ž์˜ ๋ฐœํ˜„์ด ๊ฐ์†Œํ•œ ๋‹ด๋ฐฐ์‹๋ฌผ์ฒด์—์„œ๋Š” ๋ณ‘์›๊ท  ์ ‘์ข… ์—†์ด๋„ ROS๊ฐ€ ๊ณผ๋‹ค ์ถ•์ ๋˜์—ˆ๊ณ , ์ด๋ฅผ ํ†ตํ•ด CC๋„๋ฉ”์ธ์ด SAHH๋ฅผ ๋น„ํ™œ์„ฑํ™” ์‹œํ‚ด์œผ๋กœ์จ ROS๊ฐ€ ์ฆ๊ฐ€๋˜์–ด ์„ธํฌ์‚ฌ๋ฉธ์„ ์œ ๋„ํ•˜๋Š” ๊ฒƒ์œผ๋กœ ์œ ์ถ”ํ•  ์ˆ˜ ์žˆ์—ˆ๋‹ค. ๋ณธ ์—ฐ๊ตฌ๋ฅผ ํ†ตํ•ด ๊ทธ๋ฃน 10๋ฒˆ NLR์˜ CC๋„๋ฉ”์ธ์ด ํŠน์ด์ ์œผ๋กœ ์„ธํฌ์‚ฌ๋ฉธ์„ ์œ ๋„ํ•œ๋‹ค๋Š” ๊ฒƒ์ด ๋ฐํ˜€์กŒ๋‹ค. ๊ทธ๋ฃน 10๋ฒˆ NLR์˜ CC ๋„๋ฉ”์ธ์ด ์œ ๋„ํ•˜๋Š” ์„ธํฌ์‚ฌ๋ฉธ๊ธฐ์ž‘์— ๋Œ€ํ•œ ์—ฐ๊ตฌ๋Š” NLR ๋‹จ๋ฐฑ์งˆ์ด ์–ด๋–ป๊ฒŒ ์„ธํฌ์‚ฌ๋ฉธ์„ ์œ ๋„ํ•˜๋Š” ์ง€, ๋” ๋‚˜์•„๊ฐ€ ์‹๋ฌผ์˜ ๋ณ‘ ์ €ํ•ญ์„ฑ์— ์žˆ์–ด ๊ณ ์ถ”์˜ ๊ทธ๋ฃน 10๋ฒˆ NLR์œ ์ „์ž๋“ค์˜ ๊ธฐ๋Šฅ์— ๋Œ€ํ•œ ์ดํ•ด๋ฅผ ๋•๋Š” ๋ฐ ์‹ค๋งˆ๋ฆฌ๊ฐ€ ๋  ๊ฒƒ์ด๋‹ค.ABSTRACT CONTENTS LIST OF TABLES LIST OF FIGURES LIST OF ABBREVIATIONS LITERATURE REVIEW Plant immune system Nucleotide-binding domain and leucine-rich repeat (NLR) genes in plant immunity Hypersensitive response in disease resistance Autoactivation of NLRs Cell death induced by autoactive N-terminal domain of NLRs INTRODUCTION MATERIALS AND METHODS RESULTS The autoactive N-terminal domain of pepper G10-NLRs causes cell death in N. benthamiana Cell death induced by G10-NLR or CC domain is associated with defense signaling pathway The N-terminal motif of G10-CCs is critical for autoactivity The TAILSP motif does not affect the localization of G10-CCs Defining minimal region of G10-CC as a signaling module The G10-NLR mediated cell death does not require EDS1, NDR1 and SA-related signaling pathway SAHH is a candidate interacting protein of G10-CCs DISCUSSION REFERENCES ABSTRACT IN KOREANDocto

    Appropriate Follow-up Ultrasonography Interval after Pyeloplasty in Children with Ureteropelvic Junction Obstruction

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    PURPOSE: Serial ultrasonography(US) is routinely performed after pyeloplasty in the setting of pediatric ureteropelvic junction obstruction(UPJO). We evaluated the adequacy of the follow-up US interval we are currently using, which calls for US at 1, 3, 6, 9, and 12 months following surgery. MATERIALS AND METHODS: Between January 2002 and August 2005, 102 patients underwent dismembered pyeloplasty for unilateral UPJO. Within this group, we selected 95 patients with high grade hydronephrosis to participate in this study. The degree of hydronephrosis was graded according to the classification issued by the Society for Fetal Urology(SFU). Improvement was defined as at least one grade of reduction. Serial sonograms were performed at 1, 3, 6, 9, and 12 months postoperatively. RESULTS: On follow-up US, 33.7%, 69.5%, 77.9%, 80.0%, and 83.2% of the patients showed improvement in their hydronephrosis at 1, 3, 6, 9, and 12 months, respectively. One patient presented with aggravation at 1 month. However, at 3 months, this patient had returned to the preoperative grade. There was no significant difference between the mean hydronephrosis grades at 6 and 12 months. No patient showed hydronephrosis aggravation at the 12-month follow-up examination. CONCLUSIONS: US at 1, 3, and 6 months revealed significant improvements in hydronephrosis. However, no significant change in hydronephrosis occurred beyond 6 months. Therefore, US performed between 6 and 12 months after pyeloplasty may be inefficient, and we propose follow-up US at the following time points: 1 month, 3 to 6 months, 12 months, and then annuallyope

    Atrial fibrillation and the risk of myocardial infarction : a nation-wide propensity-matched study

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    Department of Medicine/๋ฐ•์‚ฌ๋ชฉ์  : ์‹ฌ๋ฐฉ์„ธ๋™์€ ์‹ฌ๊ทผ๊ฒฝ์ƒ‰ ํ™˜์ž์—์„œ ๊ทธ ์˜ˆํ›„์— ์ข‹์ง€ ์•Š์€ ์˜ํ–ฅ์„ ๋ฏธ์น˜๋Š” ์š”์ธ์œผ๋กœ ์ด๋ฏธ ์•Œ๋ ค์ ธ ์žˆ๋‹ค. ์ตœ๊ทผ ์—ฐ๊ตฌ์—์„œ ์‹ฌ๋ฐฉ์„ธ๋™์ด ์‹ฌ๊ทผ๊ฒฝ์ƒ‰์˜ ์œ„ํ—˜์„ ๋†’์ธ๋‹ค๊ณ  ๋ณด๊ณ ํ•œ ๋ฐ” ์žˆ๋‹ค. ๋ณธ ์—ฐ๊ตฌ๋Š” ์‹ฌ๋ฐฉ์„ธ๋™๊ณผ ์—ฐ๊ด€๋œ ์‹ฌ๊ทผ ๊ฒฝ์ƒ‰์˜ ์œ„ํ—˜๋„๋ฅผ ์ „์ฒด ์ธ๊ตฌ๋ฅผ ๋Œ€์ƒ์œผ๋กœ ํ•˜์—ฌ ์กฐ์‚ฌํ•˜๊ณ ์ž ํ•˜์˜€๋‹ค ๋ฐฉ๋ฒ• : ํ•œ๊ตญ ๊ตญ๋ฏผ๊ฑด๊ฐ•๋ณดํ—˜ ์ž๋ฃŒ๋ฅผ ๋ถ„์„ํ•˜์—ฌ ๊ธฐ์กด์— ์‹ฌ๋ฐฉ์„ธ๋™์ด๋‚˜ ์‹ฌ๊ทผ๊ฒฝ์ƒ‰์˜ ๊ธฐ์™•๋ ฅ์ด ์—†๋Š” 497,366๋ช…(ํ‰๊ท ์—ฐ๋ น 47.6์„ธ, 250,569 ์—ฌ์„ฑ)์„ 5๋…„๊ฐ„ ์ถ”์  ๊ด€์ฐฐํ•˜์˜€์œผ๋ฉฐ ์ด ๊ธฐ๊ฐ„๋™์•ˆ ์‹ฌ๋ฐฉ์„ธ๋™์ด ์ƒˆ๋กœ ๋ฐœ์ƒํ•œ 3,295๋ช…๊ณผ ์„ฑํ–ฅ์ ์ˆ˜ ๋งค์นญ์œผ๋กœ ์„ ๋ณ„๋œ ์‹ฌ๋ฐฉ์„ธ๋™์ด ์—†๋Š” 13,159๋ช…์„ ๋Œ€์ƒ์œผ๋กœ ์‹ฌ๊ทผ๊ฒฝ์ƒ‰ ๋ฐœ์ƒ์œจ์„ ๋น„๊ต ๋ถ„์„ํ•˜์˜€๋‹ค. ๊ฒฐ๊ณผ : 4.2๋…„์˜ ์ถ”์ ๊ด€์ฐฐ ๊ธฐ๊ฐ„ ๋™์•ˆ ์ด 137๊ฑด์˜ ์‹ฌ๊ทผ๊ฒฝ์ƒ‰์ด ๋ฐœ์ƒํ•˜์˜€์œผ๋ฉฐ ์‹ฌ๋ฐฉ์„ธ๋™ ์ง„๋‹จ์‹œ์ ์—์„œ ์‹ฌ๊ทผ๊ฒฝ์ƒ‰ ๋ฐœ์ƒ๊นŒ์ง€์˜ ๊ธฐ๊ฐ„์€ 4๋…„์ด์—ˆ๋‹ค. ์—ฐ๋ น ๋ณด์ • ์‹ฌ๊ทผ๊ฒฝ์ƒ‰ ๋ฐœ์ƒ์œจ์€ ์‹ฌ๋ฐฉ์„ธ๋™์ด ์žˆ๋Š” ๊ตฐ์—์„œ ๊ทธ๋ ‡์ง€ ์•Š์€ ๊ตฐ๋ณด๋‹ค ๋” ๋†’์•˜๋‹ค [๋ฐœ์ƒ์œจ, 4.42 (95% ์‹ ๋ขฐ๊ตฌ๊ฐ„; 3.25-5.98 ๋Œ€ 1.42 (95% ์‹ ๋ขฐ๊ตฌ๊ฐ„. 1.07-1.87)/1000 ์ธ๋…„]. ์‹ฌ๋ฐฉ์„ธ๋™์€ ์‹ฌ๊ทผ๊ฒฝ์ƒ‰ ๋ฐœ์ƒ ์œ„ํ—˜์„ 3๋ฐฐ ์ฆ๊ฐ€์‹œํ‚ค๋ฉฐ [์œ„ํ—˜๋น„์œจ, 3.25 (95% ์‹ ๋ขฐ๊ตฌ๊ฐ„; 2.24-4.7), ์ถ”์  ๊ธฐ๊ฐ„ ๋™์•ˆ ์‹ฌ๊ทผ๊ฒฝ์ƒ‰์˜ ๋ˆ„์  ๋ฐœ์ƒ์œจ์€ ํ•ญ์‘๊ณ ์ œ์™€ ์ง€์งˆ ๊ฐ•ํ•˜์ œ๋ฅผ ๋ณต์šฉํ•œ ๊ตฐ์—์„œ ๊ทธ๋ ‡์ง€ ์•Š์€ ๊ตฐ์— ๋น„ํ•ด ์œ ์˜ํ•˜๊ฒŒ ์ž‘์•˜๋‹ค. ๊ฒฐ๋ก  : ์‹ฌ๋ฐฉ์„ธ๋™์€ ์‹ฌ๊ทผ๊ฒฝ์ƒ‰์˜ ์œ„ํ—˜์„ ์ฆ๊ฐ€์‹œํ‚ค๋ฉฐ, ๊ทธ ์œ„ํ—˜๋„๋Š” ํ•ญ์‘๊ณ ์ œ์™€ ์ง€์งˆ ๊ฐ•ํ•˜์ œ์˜ ์‚ฌ์šฉ์— ์˜ํ•ด ๊ฐ์†Œ๋œ๋‹ค. ๋ณธ ์—ฐ๊ตฌ์˜ ๊ฒฐ๊ณผ๋Š” ์‹ฌ๋ฐฉ์„ธ๋™์˜ ํ•ฉ๋ณ‘์ฆ์ด ๋‡Œ์กธ์ค‘๋ฟ๋งŒ ์•„๋‹ˆ๋ผ ์‹ฌ๊ทผ๊ฒฝ์ƒ‰์— ์˜ํ•œ ์‚ฌ๋ง๋ฅ ๊นŒ์ง€ ๊ณ ๋ คํ•ด์•ผ ํ•จ์„ ์‹œ์‚ฌํ•œ๋‹ค.open๋ฐ•

    Ureteropelvic Junction Obstruction: What We Know and What We Don't Know

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    PURPOSE: Ureteropelvic junction (UPJ) obstruction is by far the most common cause of pediatric hydronephrosis. The widespread use of antenatal ultrasonography and modern imaging techniques has resulted in earlier and more common diagnosis of hydronephrosis. However, compared with this increased earlier detection, little has changed regarding the management of hydronephrosis. Through this review, we wish to provide an overview of the studies done to date and search for areas that warrant further study. MATERIALS AND METHODS: Through PubMed, we reviewed the literature on the subject of UPJ obstruction in the pediatric population. We also present data from our institution regarding recent trends in the evaluation and treatment of UPJ obstructions. RESULTS: In addition to conventional imaging studies, attempts are being made at making use of biochemical parameters (e.g., beta2 m, N-acetyl-beta-D-glucosaminidase [NAG], transforming growth factor [TGF]-beta, etc.) as not only indicators of intervention but also prognostic factors during follow-up. Although we routinely use radionuclide imaging to evaluate renal function, a more accurate novel tool that can represent true renal function is needed. With the development in the field of laparoscopic and minimally invasive surgery, the role of laparoscopy and robot-assisted laparoscopic pyeloplasty is expanding, even in the pediatric population. However, relatively little is known about the factors that might be associated with postoperative outcomes. CONCLUSIONS: Not missing the optimal time for surgical intervention is as important as avoiding unnecessary surgery. Continuous development and refining of surgical skills for UPJ obstruction repair are requisites for (pediatric) urologists as is in-depth basic research of the disease.ope

    Postoperative Outcome of Upper Pole Kidney with a Complete Ureteral Duplication and Complicated with Ureterocele or Ectopic Ureter after Pyeloureterostomy

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    Purpose Non conservative treatment such as heminephrectomy is considered the treatment of choice when the upper pole kidney in children with a complete ureteral duplication complicated with ureterocele or ectopic ureter is nonfunctional. The postoperative outcome of the upper pole kidney with a complete ureteral duplication after pyeloureterostomy was evaluated, and we focused on those children with undetected functions on the upper pole kidney before surgery. Materials and Methods Between May 2002 and March 2006, we evaluated 28 children, 10 boys and 18 girls, who had undergone pyeloureterostomy for a complete ureteral duplication that was complicated with ureterocele or ectoplic ureter. Their mean age was 12.3 months. Ultrasound, 99mTc-dimercaptosuccinic acid (DMSA) scanning and voiding cystourethrography (VCUG) were performed preoperatively. The clinical courses were followed up with ultrasound at 1, 3, 6 and 12 months, and DMSA scanning was done between 6 and 12 months after surgery. The median follow-up period was 21.5 months. Results Among the 12 children with cortical thinning seen on ultrasound, 10 showed thickening of the renal cortex within 12 months. All 16 children with Grade III or less hydronephrosis on the upper pole kidney were downgraded, and 10 out of them showed complete resolution or Grade I hydronephrosis within 6 months. 4 cases with no visible photon uptake at the upper pole kidney preoperatively showed hot uptakes at the defective spot between 6 to 12 months after pyeloureterostomy. 5 out of 28 children had urinary tract infection (UTI) within 3 months after surgery; however, this was easily controlled with oral antibiotics. Conclusions The function of the upper pole kidney is recovered within 1 year after pyeloureterostomy in most of the cases with a complete ureteral duplication, regardless of the severity of hydronephrosis at the upper pole kidney. Pyeloureterostomy can be an alternative treatment modality for treating a complete ureteral duplication that's complicated with ureterocele or ectopic ureter due to pyeloureterostomy's lower morbidity compared to non-conservative surgery such as heminephrecotmy.ope

    ๋Œ€๊ธฐํ™˜๊ฒฝ์ •์ฑ… ์„ฑ๊ณผ์˜ ์˜ํ–ฅ์š”์ธ ๋ถ„์„

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    ํ•™์œ„๋…ผ๋ฌธ (์„์‚ฌ)-- ์„œ์šธ๋Œ€ํ•™๊ต ํ–‰์ •๋Œ€ํ•™์› : ๊ณต๊ธฐ์—…์ •์ฑ…ํ•™๊ณผ, 2014. 8. ๋ฐ•์ •ํ›ˆ.๋ณธ ์—ฐ๊ตฌ์˜ ๋ชฉ์ ์€ ๋Œ€๊ธฐํ™˜๊ฒฝ์— ๊ด€ํ•œ ๋Œ€ํ‘œ์  ์ •์ฑ…์ธ ๋Œ€๊ธฐ์˜ค์—ผ๋ฌผ์งˆ ๋ฐฐ์ถœํ—ˆ์šฉ๊ธฐ์ค€๊ณผ ์ˆ˜๋„๊ถŒ ์‚ฌ์—…์žฅ ๋Œ€๊ธฐ์˜ค์—ผ๋ฌผ์งˆ ์ด๋Ÿ‰๊ด€๋ฆฌ์ œ ๋‘ ์ •์ฑ…์˜ ๋น„๊ตยท๋ถ„์„์„ ํ†ตํ•ด ํ™˜๊ฒฝ์ •์ฑ…์˜ ์„ฑ๊ณต์ ์ธ ์ง‘ํ–‰ ์š”์ธ์„ ๋„์ถœํ•˜๋Š” ๊ฒƒ์ด๋‹ค. ์—ฐ๊ตฌ์˜ ์™ธ์ ํƒ€๋‹น์„ฑ์„ ๋†’์ด๊ณ  ๊ฐ๊ด€์ ์ธ ๋น„๊ต๋ฅผ ์œ„ํ•ด ์ •์ฑ…์ง‘ํ–‰์—ฐ๊ตฌ์˜ ์ด๋ก ์  ํ‹€์ธ ํ•˜ํ–ฅ์‹ ์ ‘๊ทผ๋ฐฉ๋ฒ•๊ณผ ๊ณผ๊ฑฐ ์„ ํ–‰์—ฐ๊ตฌ๋ฅผ ํ†ตํ•ด ์ œ์‹œ๋œ ๋ณ€์ˆ˜๋ฅผ ๊ธฐ์ค€์œผ๋กœ ๋ถ„์„ ๋ณ€์ˆ˜๋ฅผ ์„ค์ •ํ•˜์˜€๋‹ค. ํ•ด๋‹น ๊ธฐ์ค€๋ณ€์ˆ˜๋กœ ์ •์ฑ…์˜ ๋‚ด์šฉ์—์„œ๋Š” ๋ถ„๋ช…ํ•˜๊ณ  ์ผ๊ด€๋œ ์ •์ฑ…๋ชฉํ‘œ, ์ธ๊ณผ์ด๋ก  ๊ทธ๋ฆฌ๊ณ  ์ •์ฑ…์˜ ํ•ฉ๋ฆฌ์„ฑ, ํ˜•ํ‰์„ฑ์„, ์ •์ฑ…์˜ ์ง‘ํ–‰ํ™˜๊ฒฝ์—์„œ๋Š” ์ •์ฑ…์˜ ์ง‘ํ–‰์ง‘๋‹จ์˜ ์ „๋ฌธ์„ฑ ๋ฐ ๊ธฐ์ˆ ์„ฑ, ์ •์น˜์  ์ง€์ง€์™€ ์ •์ฑ…์ด ๊ฐˆ๋“ฑ, ๋ฐ˜๋ฐœ ๋“ฑ์„ ํ•ด๊ฒฐํ•  ์ˆ˜ ์žˆ๋Š” ์ œ๋„๋ฅผ ํฌํ•จํ•˜๊ณ  ์žˆ๋Š”์ง€์˜ ์—ฌ๋ถ€๋ฅผ ์„ค์ •ํ•˜์˜€๋‹ค. ์ด๋Ÿ‰๊ด€๋ฆฌ์ œ๋Š” ๊ฐ ๋ณ€์ˆ˜์— ๋Œ€ํ•ด ๊ทœ์ œ๊ธฐ์ค€์„ ๋†๋„์—์„œ ์ด๋Ÿ‰์œผ๋กœ ๋ณ€๊ฒฝํ•จ์œผ๋กœ์จ, ์ •์ฑ…๋ชฉํ‘œ์™€ ๊ด€๋ จ๋œ ํ™˜๊ฒฝ๊ธฐ์ค€์˜ ์„ค์ •์ด ๊ฐ€๋Šฅํ•˜๊ฒŒ ๋จ์— ๋”ฐ๋ผ ํ•ฉ๋ฆฌ์„ฑ์ด ์ œ๊ณ ๋œ ๊ฒƒ์œผ๋กœ ํŒ๋‹จํ•˜์˜€์œผ๋ฉฐ ๋˜ํ•œ ์ง‘๋‹จ๋ณ„ ์ผ๊ด€๋œ ๊ธฐ์ค€์„ ์ ์šฉํ•˜๋Š” ๋†๋„๊ทœ์ œ์™€ ๋‹ฌ๋ฆฌ ์‹œ์„ค๋ณ„ ํŠน์„ฑ์ด ๊ณ ๋ ค๋œ ํ• ๋‹น๋Ÿ‰์„ ๊ธฐ์ค€์œผ๋กœ ์„ค์ •ํ•จ์œผ๋กœ์จ ํ˜•ํ‰์„ฑ์ด ์ œ๊ณ ๋œ ๊ฒƒ์œผ๋กœ ๋ณด์•˜๋‹ค. ์ง‘ํ–‰ํ™˜๊ฒฝ์— ์žˆ์–ด์„œ๋„ ์ด๋Ÿ‰๊ด€๋ฆฌ์ œ๋Š” ๊ทธ ์‹ค์‹œ ๊ทผ๊ฑฐ๋กœ ์ˆ˜๋„๊ถŒ๋Œ€๊ธฐํ™˜๊ฒฝ๊ฐœ์„ ์— ๊ด€ํ•œ ํŠน๋ณ„๋ฒ•(2003)์˜ ์ œ์ • ๊ทธ๋ฆฌ๊ณ  ์ˆ˜๋„๊ถŒ ๋Œ€๊ธฐํ™˜๊ฒฝ์ฒญ์˜ ์‹ ์„ค(2005)์„ ํ†ตํ•ด ์ •์ฑ…์ ์ง€์ง€ ๋ฐ ์ •์ฑ…์— ๋Œ€ํ•œ ์‹ค์ฒœ์˜์ง€๋ฅผ ํ™•๋ณดํ–ˆ์Œ์„ ํ™•์ธํ•˜์˜€๊ณ  ๋งˆ์ง€๋ง‰์œผ๋กœ ์ด๋Ÿ‰๊ด€๋ฆฌ์ œ๋Š” ๊ธฐ์กด์˜ ๋†๋„๊ทœ์ œ์™€ ๋‹ฌ๋ฆฌ ๋ฐฐ์ถœ๊ถŒ๊ฑฐ๋ž˜์ œ, ์ž๋ฐœ์  ๊ฐ์ถ•ํ˜‘์•ฝ ๋“ฑ์„ ์šด์˜ํ•จ์œผ๋กœ์จ ์ง‘ํ–‰๋Œ€์ƒ์ž์˜ ์ˆœ์‘์„ ํ™•๋ณดํ•  ์ˆ˜ ์žˆ๋Š” ์ œ๋„๊ฐ€ ์šด์˜๋˜๊ณ  ์žˆ์Œ์„ ํ™•์ธํ•˜์˜€๋‹ค. ์ •์ฑ…์˜ ์„ฑ๊ณผ๋ถ„์„์—์„œ๋Š” ์„ฑ๊ณต์ ์ธ ์ง‘ํ–‰์— ๋Œ€ํ•œ ๊ธฐ์ค€์„ ์ •์ฑ…์˜ ์ง€์†๊ฐ€๋Šฅ์„ฑ์œผ๋กœ ๋ณด์•˜์œผ๋ฉฐ ์ด๋Š” ์ •์ฑ…์˜ ๋ชฉํ‘œ์™€ ์‚ฐ์ถœ๋ฌผ์ด ์ผ์น˜ํ•˜๋Š”์ง€, ๊ทธ๋ฆฌ๊ณ  ์ˆœ์‘์ด ์ œ๊ณ ๋˜์—ˆ๋Š”์ง€๋ฅผ ๊ทธ ๊ธฐ์ค€์œผ๋กœ ์‚ผ์•˜๋‹ค. ์ด๋Ÿ‰๊ด€๋ฆฌ์ œ๊ฐ€ ์ ์šฉ๋˜๋Š” ์ˆ˜๋„๊ถŒ์ง€์—ญ์—์„œ์˜ ๋Œ€๊ธฐ์˜ค์—ผ๋ฌผ์งˆ์˜ ๊ฐ์ถ•๋Ÿ‰์ด ๋†๋„๊ทœ์ œ๊ฐ€ ์ ์šฉ๋˜๋Š” ๋‹ค๋ฅธ ์ง€์—ญ์— ๋น„ํ•ด ๋” ํฌ๊ฒŒ ์‚ฐ์ •๋˜์—ˆ์œผ๋ฉฐ ์ˆœ์‘์ œ๋„์—์„œ ๋ฐฐ์ถœ๊ถŒ๊ฑฐ๋ž˜์ œ ๋˜ํ•œ ์•„์ง ํ™œ์„ฑํ™”๋˜์ง„ ์•Š์•˜์ง€๋งŒ ๋ฐฐ์ถœ๊ถŒ์ด ๊ฑฐ๋ž˜๋˜๋Š” ์–‘์ด๋‚˜ ๊ฑด์ˆ˜๊ฐ€ ์ง€์†์ ์œผ๋กœ ์ฆ๊ฐ€ํ•˜๋Š” ๊ฒƒ์„ ํ™•์ธํ•  ์ˆ˜ ์žˆ์—ˆ๋‹ค. ์ด๋ฅผ ํ†ตํ•ด ์ด๋Ÿ‰๊ด€๋ฆฌ์ œ๋ฅผ ๋†๋„๊ทœ์ œ์™€ ๋น„๊ตํ•ด ์„ฑ๊ณตํ•œ ์ •์ฑ…์œผ๋กœ ํŒ๋‹จํ•˜์˜€์œผ๋ฉฐ ๊ทธ ๊ฒฐ๊ณผ ์ •์ฑ…์ด ๊ฐ€์ง€๋Š” ํ•ฉ๋ฆฌ์„ฑ, ์ •์น˜์ ์ง€์ง€ ๊ทธ๋ฆฌ๊ณ  ์ˆœ์‘์„ ์ œ๊ณ ํ•  ์ˆ˜ ์žˆ๋Š” ์ œ๋„๋ฅผ ํฌํ•จํ•˜๊ณ  ์žˆ์Œ์„ ์ด๋Ÿ‰๊ด€๋ฆฌ์ •์ฑ…์˜ ์„ฑ๊ณต์  ์ง‘ํ–‰ ์š”์ธ์œผ๋กœ ์ œ์‹œํ•˜์˜€๋‹ค. ๊ณผ๊ฑฐ ์—ฐ๊ตฌ์—์„œ๋Š” ํ™˜๊ฒฝ์ •์ฑ…์— ๋Œ€ํ•ด ๋ณ€ํ™”๊ฐ€ ์ง€์†์ ์œผ๋กœ ์š”๊ตฌ๋˜์–ด์ ธ ์™”๋‹ค. ๋ณธ ์—ฐ๊ตฌ์—์„œ๋Š” ์ด๋Ÿ‰๊ด€๋ฆฌ์ œ๊ฐ€ ๊ธฐ์กด์˜ ํ™˜๊ฒฝ์ •์ฑ…๋“ค๊ณผ ๋‹ฌ๋ฆฌ ๊ณผ๊ฑฐ ์—ฐ๊ตฌ์—์„œ ์š”๊ตฌ๋˜์—ˆ๋˜ ๋‚ด์šฉ๋“ค์ด ์ผ๋ถ€ ํฌํ•จ๋˜์–ด ์žˆ๋Š” ์ •์ฑ…์œผ๋กœ์„œ ์„ฑ๊ณต์  ์ง‘ํ–‰์— ๋”ฐ๋ฅธ ๊ทธ ์˜ํ–ฅ์š”์ธ์„ ๋„์ถœํ•˜์˜€๊ณ  ์ด๋ฅผ ํ†ตํ•ด ์•ž์œผ๋กœ ํ™˜๊ฒฝ์ •์ฑ…์— ์žˆ์–ด ๋” ๊ฐœ์„ ๋œ ๋ฐฉํ–ฅ์œผ๋กœ์˜ ์ •์ฑ… ์ˆ˜๋ฆฝ ๋ฐ ์‹œํ–‰์— ์˜ํ–ฅ์„ ๋ฏธ์น  ๊ฒƒ์œผ๋กœ ์˜ˆ์ƒํ•œ๋‹ค.1. ์„œ ๋ก  ................................................................... 1 2. ์—ฐ๊ตฌ๋ชฉ์  ๋ฐ ํ•„์š”์„ฑ................................................. 3 3. ์ด๋ก  ๋ฐ ์„ ํ–‰์—ฐ๊ตฌ.................................................... 4 3.1 ์ด๋ก ์ ๋ฐฐ๊ฒฝ .......................................................... 4 3.1.1 ๊ตด๋š์›๊ฒฉ๊ฐ์‹œ์ฒด๊ณ„์ œ๋„.......................................... 4 3.1.2 ์ˆ˜๋„๊ถŒ ์‚ฌ์—…์žฅ ๋Œ€๊ธฐ์˜ค์—ผ๋ฌผ์งˆ ์ด๋Ÿ‰๊ด€๋ฆฌ์ œ.................. 6 3.2 ์ด๋ก ์  ๋…ผ์˜ .......................................................... 9 3.2.1 ํ™˜๊ฒฝ์ •์ฑ… ........................................................... 9 3.2.2 ํ™˜๊ฒฝ๊ทœ์ œ .......................................................... 10 3.2.3 ์ •์ฑ…์ˆœ์‘ ........................................................... 11 3.2.4 ์ง€์†๊ฐ€๋Šฅ์„ฑ ....................................................... 13 3.3 ์„ ํ–‰์—ฐ๊ตฌ ............................................................. 14 3.3.1 ํ™˜๊ฒฝ์ •์ฑ… .......................................................... 14 3.3.2 ๋Œ€๊ธฐ์˜ค์—ผ .......................................................... 17 3.3.3 ๋Œ€๊ธฐํ™˜๊ฒฝ์ •์ฑ… .................................................... 18 3.3.4 ์ •์ฑ…์ง‘ํ–‰์—ฐ๊ตฌ .................................................... 20 3.3.5 ๊ธฐ์กด์—ฐ๊ตฌ์˜ ํ•œ๊ณ„ ................................................ 21 4. ์—ฐ๊ตฌ๋Œ€์ƒ ๋ฐ ์—ฐ๊ตฌ๋ฐฉ๋ฒ• ............................................. 23 4.1 ์—ฐ๊ตฌ๋Œ€์ƒ ............................................................. 23 4.2 ์—ฐ๊ตฌ๋ฐฉ๋ฒ• ............................................................. 24 5. ๋Œ€๊ธฐํ™˜๊ฒฝ์ •์ฑ…์˜ ํ‰๊ฐ€ .............................................. 27 5.1 ํ™˜๊ฒฝ์ •์ฑ…์˜ ํŠน์„ฑ ................................................... 27 5.1.1 ์ •์ฑ…๋ชฉํ‘œ์˜ ํŠน์„ฑ ................................................. 27 5.1.2 ํ™˜๊ฒฝ์ •์ฑ… ๋””์ž์ธ ................................................ 30 5.2 ์ •์ฑ…์ง‘ํ–‰ํ™˜๊ฒฝ์˜ ํŠน์„ฑ ............................................. 34 5.2.1 ์ •์ฑ…์ง‘ํ–‰ํ™˜๊ฒฝ .................................................... 34 5.2.2 ๋ฒ•์ ๊ตฌ์กฐํ™” ๋Šฅ๋ ฅ ................................................ 37 5.3 ํ™˜๊ฒฝ์ •์ฑ…์˜ ์ง€์†๊ฐ€๋Šฅ์„ฑ .......................................... 41 5.3.1 ํ™˜๊ฒฝ์ •์ฑ…์˜ ๋ชฉํ‘œ๋‹ฌ์„ฑ .......................................... 41 5.3.2 ๋Œ€๊ธฐ์˜ค์—ผ๋ฌผ์งˆ ๋ฐฐ์ถœ๊ถŒ๊ฑฐ๋ž˜์ œ.................................. 47 5.3.3 ์ž๋ฐœ์  ๊ฐ์ถ•ํ˜‘์•ฝ ................................................ 49 5.3 ์„ฑ๊ณต์  ์ง‘ํ–‰์— ๋”ฐ๋ฅธ ์š”์ธ ๋ถ„์„ ................................. 51 6. ๊ฒฐ๋ก  ๋ฐ ๊ณ ์ฐฐ ......................................................... 54 7. ์ฐธ๊ณ  ๋ฌธํ—Œ ............................................................. 59 Abstract .................................................................. 64Maste

    Hinman Syndrome: Long Term Follow up of 14 Cases

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    Purpose Hinman syndrome is the most severe form of nonneurogenic neurogenic bladder causing damage of the upper urinary tract. Fourteen patients with Hinman syndrome followed at our institution were evaluated for their clinical characteristics and prognosis. Here we report the findings of this series of patients for this poorly understood syndrome. Materials and Methods The medical records of 14 patients, 8 boys and 6 girls, diagnosed with Hinman syndrome from March 1993 to June 2006 were reviewed. The mean duration of follow up was 69 months. The ultrasonography, 99m Tc-dimercaptosuccinic acid renal scan (DMSA), voiding cystourethrogram (VCUG), and urodynamic study (UDS) results were retrospectively analyzed and efficacy of each treatment method was evaluated based on the medical records on follow up. Results Hydronephrosis of grade III or greater on ultrasonography and renal scarring of both kidneys on the DMSA renal scan were observed in 12 and 14 patients, respectively. Severe bladder trabeculation and high grade VUR (IV, V) were observed on the VCUG in 14 and 8 patients, respectively. Decreased bladder compliance on the UDS was noted in 13 and detrussor-sphincter dyssynergia (DSD) was observed in eight. Medical treatment was not effective in all 14 cases and six patients who underwent botulinum injection of the bladder were unresponsive to that treatment as well. In spite of conservative treatments such as clean intermittent catheterization (CIC), seven patients eventually underwent bladder augmentations after a mean period of thirty-seven months from diagnosis because of concern about the loss of bladder capacity and renal function. One patient who did not perform CIC progressed to end-stage renal disease and had to be transferred to pediatric nephrology for dialysis. Conclusions Patients diagnosed with the Hinman syndrome were treated similar to patients with neurogenic bladder. However, from the long-termfollow up data at our outpatient clinic, many patients eventually had bladder augmentation to prevent further loss of bladder capacity and renal function. Therefore, urologists must not hesitate in performing such treatment when necessary.ope

    The Significance of the Parents' Description of Constipation in Pediatric Voiding Dysfunction

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    PURPOSE: Dysfunctional voiding is closely related to constipation, and accurate evaluation of constipation is important. We investigated the accuracy and reliability of several methods that are used to evaluate constipation. MATERIALS AND METHODS: We collected data from the parents of 168 dysfunctional voiders, and the data was collected using the Bristol stool form scale and the Leech method. We then analyzed the results of the questionnaire and compared them with the Paris Consensus on Childhood Constipation Terminology(PACCT). RESULTS: Among the 168 dysfunctional voiders, 71(42%) were diagnosed with constipation according to PACCT. Of the 39 children who were considered constipated, none were constipated according to PACCT. However, 35 of the 129 children whose parents denied they were constipation were in fact diagnosed with constipation. When the Leech method and the Bristol stool form scale were both used, the sensitivity of diagnosis rose to as high as 75%, and when the statement of the guardian was added, the sensitivity rose to as high as 85%. CONCLUSIONS: Since constipation can be a rather subjective condition, it would be appropriate to respect and incorporate the parents' statement into the diagnosis. However, for making a more accurate diagnosis, comprehensive evaluation based on specific symptoms and the Bristol stool form scale and the Leech method may be helpful.ope

    Clinical Characteristics of Children with Persistent Cloaca: A Urological Perspective

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    PURPOSE: Persistent cloaca is a rare multiple anomalous condition which involves the gastrointestinal, neurological and urogenital systems. We evaluated the clinical characteristics and urogenital anomalies of patients with persistent cloaca, and we investigated the factors that must be considered from a urological perspective. MATERIALS AND METHODS: We retrospectively reviewed the medical records of 11 patients who were diagnosed with persistent cloaca at our institution during the last 7 years. RESULTS: Nine of the 11 patients who were followed up at the urology clinic were subjected to this study. Nonspecific abnormalities, such as antenatal hydronephrosis, were noted in 5 of 9 patients. The chief complaints upon visits to the urologic clinic were recurrent urinary tract infections in 3, preoperative evaluation before neurosurgery in 3, urinary incontinence in 1, urinary retention in 1 and an elevated creatinine level in 1. The urological anomalies included 2 cases of renal agenesis and 1 horseshoe kidney. Vesicoureteral reflux was noted in 6 patients, and 4 of these patients underwent antireflux surgery. Videourodynamic study was performed in 8 patients and all of them were diagnosed with neurogenic bladder. Currently, 5 children are on intermittent catheterization, 2 have undergone vesicostomy and 2 void spontaneously. CONCLUSIONS: Most patients with persistent cloaca had urological anomalies of the upper urinary tract and neurogenic bladder. Therefore, a multidisciplinary approach for diagnosis and treatment from various departments, including the urology, pediatric surgery, neurosurgery departments is mandatoryope

    Long-term Outcome and Parentsโ€™ Satisfaction after the Correction of Concealed Penis

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    Purpose: Although there are multiple published report of surgical technique for concealed penis and the short-term result of the surgical correction, but there is a paucity of report concerning of the long term follow up data and parentsโ€™ satisfaction. We report our long-term results of the surgical treatment of concealed penis focus on parent satisfaction Patients and Methods: Medical data of 45 patients treated for concealed penis in Severance hospital from 1999 to 2001 were collected and reviewed retrospectively. The parents were questioned about the initial problem that they associated with concelaled penis, such as appearance, hygiene problem and infection problem via telephone interview. Results: Thirty two patients visited the OPD of pediatric urology because of the problem of hygiene, negative appearance and medical problem such as balanoposthitis (group 1) while 13 patients were referred by primary physician (group 2). Mean age was 2.6 yrs and mean duration of follow up was 7.8 yrs. All patients in group 1 and 89% of group 2 complained of difficulty with hygiene before surgery. Following surgery, group 1 patients fared better reporting improvements in hygiene (91%), accessibility (86%) and improved appearance of the penis (97%). Results from surgical intervention were less successful in group 2, with improved hygiene in 85%, improved penile lengthening in 75% and improved appearance in 77% of patients. Conclusions: Our long term outcome data demonstrate that most parents thought that surgical correction is helpful to keep hygiene of penis and improve the appearance of penis. Most parents still viewed surgery as a positive intervention and would recommend it to a friend with similar conditionope
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