• Clinical and Experimental Pediatrics
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• 제49권2호
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• pp.203-207
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• 2006

위장관계의 신경섬유종의 발생빈도는 드물다고 알려져 있다. 저자들은 제1형 신경섬유종증의 15세 남자 환아에서 상부위장관 출혈로 발현한 위에 생기는 신경섬유종을 내시경 및 상부위장관 조영술을 통해 진단을 내리고, 수술적 제거를 시행하였던 1례를 경험하였기에 문헌 고찰과 함께 보고하는 바이다.

• Journal of Gastric Cancer
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• 제18권1호
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• pp.99-107
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• 2018

Immunoglobulin G4-related disease (IgG4-RD) is an immune-mediated fibro-inflammatory disorder characterized by specific pathological findings and elevated serum IgG4 level. IgG4-RD in the stomach is rare, and occasionally diagnosed as gastric subepithelial tumor (SET) by endoscopy or computed tomography scan. Two female patients in the age group of 40-50 years were diagnosed with 4 cm sized gastric SET. One underwent laparoscopic gastric wedge resection. Another one had a history of subtotal gastrectomy for early gastric cancer and idiopathic thrombocytopenic purpura with oral steroids administration. She underwent a completion total gastrectomy with splenectomy for the gastric SET and ITP. The pathology showed storiform fibrosis, and IgG4 was positive in immunohistochemistry (IHC) stain. IgG4-RD is known as a medical disease that could be treated with oral steroids. The difficulty in preoperative diagnosis of the disease occasionally causes unnecessary gastric resection. Thus, preoperative diagnostic methods for IgG4-RD such as deep biopsy with IHC stain or magnetic resonance imaging are needed.

• Journal of Gastric Cancer
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• 제15권2호
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• pp.113-120
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• 2015

Purpose: Although several clinical trials have proven the efficacy of adjuvant S-1 treatment in gastric cancers, it is still unclear which patients receive the most benefit. In this study, we prospectively recruited patients with locally advanced gastric cancer who had undergone curative resection followed by adjuvant S-1 administration to investigate which factors affect the outcomes. Materials and Methods: Between July 2010 and October 2011, we enrolled 49 patients who underwent curative resection for stage II or III gastric cancer and who subsequently received adjuvant S-1 treatment for 1 year. Results: Twenty-nine patients (59.2%) continued S-1 treatment for 1 year, and 12 patients (24.5%) experienced recurrent disease during the follow-up period. Patients with continuation of S-1 for 1 year had significantly increased rates of disease-free survival (P<0.001) and overall survival (P=0.001) relative to the patients who discontinued S-1 during year 1. Multivariate analysis indicated poor outcomes for patients with stage III disease and those who discontinued S-1 treatment. Excluding patients who discontinued S-1 due to cancer progression (n=7), adjuvant treatment with S-1 still demonstrated a significant difference in the disease-free survival rate between the patients who continued treatment and those who discontinued it (P=0.020). Conclusions: S-1 is tolerated as adjuvant treatment in gastric cancer patients. However, discontinuing S-1 treatment may be an unfavorable factor in the prevention of recurrence. S-1 adjuvant treatment should be continued for 1 year if possible through the proper management of toxicities.

• Tuberculosis and Respiratory Diseases
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• 제79권4호
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• pp.267-273
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• 2016

Background: Cystic fibrosis (CF) is an autosomal recessive disorder with several clinical presentations. This study was undertaken in the Azeri Turkish population in Iran, to investigate gender differences in the age at onset and diagnosis, age of death, and duration of illness of CF. Methods: The data of 331 CF patients from 2001 to 2015 was surveyed. Parameters including age, sex, ${\Delta}F508$ mutation, age at onset, age at diagnosis, age of death and clinical presentations were evaluated for both sexes, using descriptive analysis. The association of gender with these variables was studied using logistic regression, chi-square test and Mann-Whitney U test by SPSS version 18. Odds ratio with a confidence interval of 95% and $p{\leq}0.05$ was considered statistically significant. Results: The study included 191 males (57.7%) and 140 females (42.3%), all showing statistically significant difference (p<0.001). Age duration differed between genders. Male and female patients were further under 9 and 4 years, respectively. The occurrence of ${\Delta}F508$ mutation was 0.51 times more in females than in males. Age, diagnosis and sex were closely associated: males were diagnosed at a significantly later age than females (p=0.05). While this compression performed based on clinical presentations, males with respiratory disease had a later median age at diagnosis than females at lifespan (p=0.001). The risk of infertility in males was approximately two times greater than in females (p=0.02). Conclusion: These findings indicate gender differences in CF patients. Future studies are needed to establish other differences and evaluate the causes for the gender variations.

• Clinical and Experimental Pediatrics
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• 제62권8호
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• pp.287-291
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• 2019

Fecal calprotectin (FC) is a calcium- and zinc-binding protein of the S100 family, mainly expressed by neutrophils and released during inflammation. FC became an increasingly useful tool both for gastroenterologists and for general practitioners for distinguishing inflammatory bowel disease (IBD) from irritable bowel syndrome. Increasing evidences support the use of this biomarker for diagnosis, follow-up and evaluation of response to therapy of several pediatric gastrointestinal diseases, ranging from IBD to nonspecific colitis and necrotizing enterocolitis. This article summarizes the current literature on the use of FC in clinical practice.

• Clinical and Experimental Pediatrics
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• 제65권1호
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• pp.21-28
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• 2022

Crohn disease (CD) is a multifactorial inflammatory disorder that can affect all segments of the gastrointestinal (GI) tract but typically involves the ileum and/or colon. To assess patient prognosis and choose appropriate treatment, it is necessary to accurately evaluate the factors influencing poor outcomes, including disease phenotype. Pediatric CD involving the upper GI (UGI) tract has become increasingly recognized with the introduction of routine upper endoscopy with biopsies for all patients and the increased availability of accurate small bowel evaluations. Most clinical manifestations are mild and nonspecific; however, UGI involvement should not be overlooked since it can cause serious complications. Although controversy persists about the definition of upper GI involvement, aphthoid ulcers, longitudinal ulcers, a bamboo joint-like appearance, stenosis, and fistula are endoscopic findings suggestive of CD. In addition, the primary histological findings, such as focally enhanced gastritis and noncaseating granulomas, are highly suggestive of CD. The association between UGI involvement and poor prognosis of CD remains controversial. However, the unstandardized definition and absence of a validated tool for evaluating disease severity complicate the objective assessment of UGI involvement in CD. Therefore, more prospective studies are needed to provide further insight into the standardized assessment of UGI involvement and long-term prognosis of CD. Our review summarizes the findings to date in the literature as well as UGI involvement in CD and its clinical implications.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• 제11권2호
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• pp.122-129
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• 2008

목 적: 만성 설사, 흡수 장애 등의 소화기 질환에서 경구 영양 공급이 불충분할 경우 미량원소 결핍 발생의 빈도가 높으나, 이러한 환아에서 미량원소 결핍 증상에도 불구하고 혈액 검사 결과 정상으로 나타나기도 한다. 따라서 미량원소 결핍 고위험군 환아에서 결핍된 미량원소를 확인하고, 적절한 공급 및 추적 관찰 지표로서 모발 검사의 유용성을 알아보고자 하였다. 방 법: 서울대학교 어린이병원에 내원하여 만성 소화기 질환으로 경구 영양 장애 또는 성장 발육 부전을 보였던 13명을 대상으로 모발과 혈액의 미량원소 검사를 시행하여, 결핍 소견을 보인 모발과 혈액의 미량원소에 대해 장기 정맥영양군과 경구영양군, 그리고 증상군과 무증상군으로 나누어 비교, 분석하였다. 결 과: 전체 13명 중 11명에서 모발 또는 혈액 검사에서 미량원소 결핍이 있었으며, 결핍된 미량원소는 아연, 셀레늄, 구리였다. 모발 내 아연 결핍은 8명(62%), 모발 내 셀레늄 결핍은 6명(46%)이었고, 혈액 내 아연농도가 정상인 환자의 67% (8/12명), 혈액 내 셀레늄 농도가 정상 환자의 57% (4/7명)에서 각각 모발 내 아연, 모발 내 셀레늄 결핍을 보였다. 모발 내 아연 농도는 장기 정맥영양군에서 경구영양군에 비해 유의하게 낮았으며(p=0.015) 결핍 발생 빈도 또한 유의하게 높았다(Fisher's exact test, p=0.032). 모발 내 셀레늄 농도는 증상군에서 무증상군에 비해 유의하게 낮았다(p=0.034). 결 론: 만성 소화기 질환으로 경구 영양 장애 또는 성장 발육 부전이 있을 경우 증상 여부에 상관없이 혈액검사와 함께 모발 미네랄 검사로 결핍 여부를 평가하는 것이 필요할 것으로 보인다.

• Journal of Gastric Cancer
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• 제15권2호
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• pp.75-76
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• 2015

• Pediatric Gastroenterology, Hepatology & Nutrition
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• 제17권1호
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• pp.52-56
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• 2014

We present a case of a 13-year-old boy with Gorham's disease involving the thoracic and lumbar spine, femur, and gastrointestinal (GI) tract, which was complicated by recurrent chylothorax and GI bleeding. The presenting symptoms were intermittent abdominal pain, back pain, and melena. Esophagogastroduodenoscopy and colonoscopy showed no abnormal lesions, but duodenal biopsy showed marked dilation of the lymphatics in the mucosa and submucosa, which revealed positive staining with a D2-40 immunohistochemical marker. In cases of GI bleeding with osteolysis, the expression of a D2-40 marker in the lymphatic endothelium of the GI tract may help to diagnose GI involvement in Gorham's disease. To the best of our knowledge, this is the first case report to pathologically demonstrate intestinal lymphatic malformation as a cause of GI bleeding in Gorham's disease.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• 제11권1호
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• pp.80-83
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• 2008

소아 베체트병은 상당히 드문 질환으로 전체 베체트병의 약 2~3%에 해당한다. 이 중 장 점막과 관련된 경우는 2~50%까지 다양하게 보고되나, 대부분이 40~50대 성인에서 보고되는 내용이며 소아 베체트병 환자에서 장 천공이 보고된 증례는 극히 드물다. 본 증례는 4년 9개월된 남아로, 베체트병을 진단받은 후 약물 치료하며 추적 관찰 중, 지속되는 복통이 있어 시행한 대장 내시경상에 다발성 장점막 궤양이 관찰되었고, 이후 스테로이드 용량 감량하며 경과 관찰하던 중 장 천공이 발생하여 수술한 1예를 경험하였기에 보고하는 바이다.