• Journal of Korean Medical Science
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• 제33권52호
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• pp.336.1-336.12
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• 2018

Background: We aimed to investigate mucosal immunity related to forkhead box P3 ($FOXP3^+$) regulatory T (Treg) cells, T helper 17 (Th17) cells and cytokines in pediatric inflammatory bowel disease (IBD). Methods: Mucosal tissues from terminal ileum and colon and serum samples were collected from twelve children with IBD and seven control children. Immunohistochemical staining was done using anti-human FOXP3 and anti-$ROR{\gamma}t$ antibodies. Serum levels of cytokines were analyzed using a multiplex assay covering interleukin $(IL)-1{\beta}$, IL-4, IL-6, IL-10, IL-17A/F, IL-21, IL-22, IL-23, IL-25, IL-31, IL-33, interferon $(IFN)-{\gamma}$, soluble CD40L, and tumor necrosis factor-${\alpha}$. Results: $FOXP3^+$ Treg cells in the lamina propria (LP) of terminal ileum of patients with Crohn's disease were significantly (P < 0.05) higher than those in the healthy controls. $ROR{\gamma}t^+$ T cells of terminal ileum tended to be higher in Crohn's disease than those in the control. In the multiplex assay, serum concentrations (pg/mL) of IL-4 ($9.6{\pm}1.5$ vs. $12.7{\pm}3.0$), IL-21 ($14.9{\pm}1.5$ vs. $26.4{\pm}9.1$), IL-33 ($14.3{\pm}0.9$ vs. $19.1{\pm}5.3$), and $IFN-{\gamma}$ ($15.2{\pm}5.9$ vs. $50.2{\pm}42.4$) were significantly lower in Crohn's disease than those in the control group. However, serum concentration of IL-6 ($119.1{\pm}79.6$ vs. $52.9{\pm}39.1$) was higher in Crohn's disease than that in the control. Serum concentrations of IL-17A ($64.2{\pm}17.2$ vs. $28.3{\pm}10.0$) and IL-22 ($37.5{\pm}8.8$ vs. $27.2{\pm}3.7$) were significantly higher in ulcerative colitis than those in Crohn's disease. Conclusion: Mucosal immunity analysis showed increased $FOXP3^+$ T reg cells in the LP with Crohn's disease while Th17 cell polarizing and signature cytokines were decreased in the serum samples of Crohn's disease but increased in ulcerative colitis.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• 제21권1호
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• pp.34-42
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• 2018

Purpose: Monogenic inflammatory bowel disease (IBD) patients do not respond to conventional therapy and are associated with a higher morbidity. We summarized the clinical characteristics of monogenic IBD patients and compared their clinical outcomes to that of non-monogenic IBD patients. Methods: We performed a retrospective cohort study of all children <18 years old who were diagnosed with IBD between 2005 and 2016. A total of 230 children were enrolled. Monogenic IBD was defined as a presentation age less than 6 years old with confirmation of a genetic disorder. We subdivided the groups into monogenic IBD (n=18), non-monogenic very early-onset IBD (defined as patients with a presentation age <6 years old without a confirmed genetic disorder, n=12), non-monogenic IBD (defined as all patients under 18 years old excluding monogenic IBD, n=212), and severe IBD (defined as patients treated with an anti-tumor necrosis factor excluding monogenic IBD, n=92). We compared demographic data, initial pediatric Crohn disease activity index/pediatric ulcerative colitis activity index (PCDAI/PUCAI) score, frequency of hospitalizations, surgical experiences, and height and weight under 3rd percentile among the patients enrolled. Results: The initial PCDAI/PUCAI score (p<0.05), incidence of surgery per year (p<0.05), and hospitalization per year (p<0.05) were higher in the monogenic IBD group than in the other IBD groups. Additionally, the proportion of children whose weight and height were less than the 3rd percentile (p<0.05 and p<0.05, respectively) was also higher in the monogenic IBD group. Conclusion: Monogenic IBD showed more severe clinical manifestations than the other groups.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• 제17권3호
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• pp.147-154
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• 2014

Purpose: Gender differences in paediatric patients with inflammatory bowel disease (IBD) are frequently reported as a secondary outcome and the results are divergent. To assess gender differences by analysing data collected within the Swiss IBD cohort study database since 2008, related to children with IBD, using the Montreal classification for a systematic approach. Methods: Data on gender, age, anthropometrics, disease location at diagnosis, disease behaviour, and therapy of 196 patients, 105 with Crohn's disease (CD) and 91 with ulcerative or indeterminate colitis (UC/IC) were retrieved and analysed. Results: The crude gender ratio (male : female) of patients with CD diagnosed at <10 years of age was 2.57, the adjusted ratio was 2.42, and in patients with UC/IC it was 0.68 and 0.64 respectively. The non-adjusted gender ratio of patients diagnosed at ${\geq}10$ years was 1.58 for CD and 0.88 for UC/IC. Boys with UC/IC diagnosed <10 years of age had a longer diagnostic delay, and in girls diagnosed with UC/IC >10 years a more important use of azathioprine was observed. No other gender difference was found after analysis of age, disease location and behaviour at diagnosis, duration of disease, familial occurrence of IBD, prevalence of extra-intestinal manifestations, complications, and requirement for surgery. Conclusion: CD in children <10 years affects predominantly boys with a sex ratio of 2.57; the impact of sex-hormones on the development of CD in pre-pubertal male patients should be investigated.

• 대한소아치과학회지
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• 제41권1호
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• pp.80-84
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• 2014

난치성 궤양성 소장결장염은 영아에게서 드물게 나타나는 만성 염증성 장 질환으로 1991년 처음으로 보고되었다. 난치성 궤양성 소장결장염은 주로 영아기에 구강 내 궤양에서 시작되어 항문주위 및 장 질환으로 진행되는 특징을 갖는다. 생후 18일 된 환아가 구강 내 궤양으로 감별할 수 있는 전신질환에 대해 알아보고자 본과로 의뢰 되었다. 당시 구강 내 궤양을 제외한 특이 사항은 없었으며 3주 뒤 혈변이 발생하였다. 대장 내시경 상에서 대장 내 거대 궤양이 발견되었으며 여러검사 결과 난치성 궤양성 소장결장염으로 진단되었다. 치과의사가 난치성 궤양성 소장결장염을 조기에 진단하는 것은 면역 억제제에 반응이 없는 본 질환에 대해 결장절제술을 신속하게 시행함으로써 증상의 완화와 질병의 진행을 막는데 효과적으로 대처할 수 있으므로 매우 중요하다. 또한 대부분의 난치성 궤양성 소장결장염 환아에게서 구강 내 궤양이 가장 먼저 발현되므로 이 질환의 증상과 특징에 대해 아는 것은 매우 의미 있을 것으로 사료되어 보고하는 바이다.

• Clinical and Experimental Pediatrics
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• 제61권10호
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• pp.327-331
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• 2018

Purpose: The incidence of inflammatory bowel disease (IBD) is rapidly increasing, and several reports have described the renal complications of IBD. We sought to evaluate the clinical manifestations of renal complications in children with IBD in order to enable early detection and prompt treatment of the complications. Methods: We retrospectively reviewed the medical records of 456 children and adolescents aged <20 years who had been diagnosed with IBD since 2000. We analyzed patient age, sex, medication use, IBD disease activity, and clinical manifestations of renal symptoms. Results: Our study comprising 456 children with IBD included 299 boys (65.6%) and 157 girls (34.4%). The study included 346 children with Crohn disease and 110 children with ulcerative colitis. The incidence of kidney-related symptoms was 14.7%, which was significantly higher than that in normal children. We observed 26 children (38.8%) with isolated hematuria, 30 children (44.8%) with isolated proteinuria, and 11 children (16.4%) with hematuria and concomitant proteinuria. A renal biopsy was performed in 7 children. Histopathological examination revealed immunoglobulin A nephropathy in 5 children (71.4%). All children presented with mild disease and well-controlled disease activity of IBD. Conclusion: Children with IBD are more likely to show kidney-related symptoms than healthy children and adolescents are. Therefore, regular screening of urine and evaluation of renal function in such children are necessary for early detection of renal complications.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• 제9권2호
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• pp.176-182
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• 2006

목 적: 급성 복통을 주소로 내원한 소아 환아에서 위장관 내시경검사상 위장관 점막 병변을 조사하고 위장관 내시경 검사의 유용성을 알고자 본 연구를 시행하였다. 방 법: 1995년 1월부터 2004년 5월까지 한양대학 병원에 급성복통을 주소로 내원하여 진료받은 환아들 중 내시경 검사를 받은 위장관 점막 고유의 질환으로 간주된 환아31명을 후향적으로 조사하여 성별 및 나이의 분포, 복통의 기간, 동반증상, 복통의 부위, 위장관 내시경 검사상 점막 병변의 종류, Helicobacter pylori (H. pylori) 양성 환아의 유무 및 양성 환아들의 복통의 특성 등에 관해 조사하였다. 결 과: 남아가 16명, 여아가 15명이었으며 6~10세 환아가 15명으로 가장 많았고 그 다음이 11~15세 환아였다. 복통의 기간은 1주 이하가 23명으로 가장 많았다. 동반된 증상은 구토(15예), 설사(4예), 혈변(2예), 발열(2예) 등이었다. 위장관 내시경 검사 소견으로는 급성 출혈성 위염(6명), 결정성 위염(5명), 십이지장 궤양(4명), 위궤양(3명), 역류성 식도염(3예), 결절성 십이지장염(2예), 표재성 위염(2명), 궤양성 대장염(2명), 미란성 십이지장염(2명), 식도용종(1명), 십이지장위역류(1명) 등이었다. 10명의 환아가에서 H. pylori가 양성이었다. H. pylori가 음성인 환아들과 비교하여 복통의 기간, 환아의 연령, 성별의 차이는 있었으나 통계학적 의의는 없었고 H. pylori 양성 환아 모두에서 복통의 위치가 심와부라는 것에 대해서는 통계학적 의의가 있었다. 위장관 내시경 검사를 받고 위장관 점막 질환에 대한 치료를 받은 환아들 전체에서 증상의 호전이 관찰되었다. 결 론: 급성 복통은 원인이 다양하다. 급성 복통에서 점막 병변의 조기진단을 위해 위장관 내시경 검사가 우선적 검사로서 중요하며, 급성 복통 시에도 H. pylori 위장관 감염에 대해서 관심을 가지는 것이 감별진단에 도움이 된다.