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A Case of Lipoprotein Lipase Deficiency in an Infant with Recurrent Pancreatitis 复发性胰腺炎婴儿脂蛋白脂肪酶缺乏1例
Pub Date : 2009-03-01 DOI: 10.5223/KJPGN.2009.12.1.79
H. Park, Byeong Sam Choi, H. Yang, J. Chang, J. Ko, C. Shin, S. Yang, J. Seo
Familial chylomicronemia syndrome is a rare disorder characterized by severe hypertriglyceridemia and fasting chylomicronemia. Causes of the syndrome include lipoprotein lipase (LPL) deficiency, apolipoprotein C-II deficiency, or the presence of inhibitors to LPL. We managed a 3-month-old girl who had recurrent acute pancreatitis caused by chylomicronemia. We report the first case of familial chylomicronemia in Korea caused by LPL deficiency in an infant with recurrent acute pancreatitis. (Korean J Pediatr Gastroenterol Nutr 2009; 12: 79∼83)
家族性乳糜微粒血症综合征是一种罕见的疾病,其特征是严重的高甘油三酯血症和空腹乳糜微粒血症。该综合征的原因包括脂蛋白脂肪酶(LPL)缺乏,载脂蛋白C-II缺乏或LPL抑制剂的存在。我们处理一个3个月大的女孩谁有复发急性胰腺炎引起的乳糜微粒血症。我们报告的第一例家族性乳糜微粒血症在韩国引起的LPL缺乏症的婴儿与复发性急性胰腺炎。韩国儿科胃肠病学杂志2009;12: 79∼83)
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引用次数: 0
Serum Lipid Profile and Nutritional Status in 6~7 Year Old Obese Children 6~7岁肥胖儿童血脂及营养状况分析
Pub Date : 2008-09-01 DOI: 10.5223/KJPGN.2008.11.2.160
J. Rim, J. Moon, Chul-Gab Lee, K. Moon
Purpose: This study was designed to characterize the nutritional status and assess obesity to determine the relationship between obesity and serum lipid profiles in 6 ∼ 7 year old children. Methods: In 2007, we surveyed 483 children (233 boys and 250 girls) aged 6 ∼ 7 years. The total cholesterol, triglyceride levels and HDL-cholesterol were measured in the fasting state. Dietary information was obtained by a questionnaire. Results: The prevalence of obesity was 9.9%. There was no significant difference between genders. The mean caloric intake was 1,781 kcal in boys and 1,640 kcal in girls. The prevalence of excessive calories was 33% in boys and 30% in girls. The prevalence of a total cholesterol ≥ 200 mg/dL was 8.4%, TG ≥ 130 mg/dL was 5.0%, LDL-cholesterol ≥ 130 mg/dL was 3.1%, and HDL-cholesterol <35 mg/dL was 4.4%. The prevalence of hypertension was 2.1%. There was no significant difference between genders. The systolic blood pressure, triglyceride levels and LDL-cholesterol were significantly related to an increased obesity index ( p < 0.05). The mean caloric intake and nutritive component were not related to the obesity index. The obesity group was compared to the control group: for triglycerides ≥ 130 mg/dL the odds ratio was 4.08; for LDL-cholesterol ≥ 130 mg the odds ratio was 2.85; for a TC/HDL-cholesterol ≥ 4.0 the odds ratio was 1.16. The BMI and triglyceride levels in the group with hypertension were higher than control group ( p < 0.05). There were significant positive correlations between the BMI and blood pressure as well as the LDL-cholesterol and triglycerides ( p < 0.05). The BMI was not correlated with the mean caloric intake or nutrition.
目的:本研究旨在描述6 ~ 7岁儿童的营养状况并评估肥胖,以确定肥胖与血脂之间的关系。方法:2007年,我们调查了483名6 ~ 7岁的儿童(233名男孩和250名女孩)。空腹时测定总胆固醇、甘油三酯和高密度脂蛋白胆固醇水平。饮食信息通过问卷调查获得。结果:肥胖率为9.9%。性别间无显著差异。男孩的平均卡路里摄入量为1781千卡,女孩为1640千卡。过量卡路里的患病率在男孩中为33%,在女孩中为30%。总胆固醇≥200mg /dL的占8.4%,TG≥130mg /dL的占5.0%,ldl -胆固醇≥130mg /dL的占3.1%,hdl -胆固醇< 35mg /dL的占4.4%。高血压患病率为2.1%。性别间无显著差异。收缩压、甘油三酯和低密度脂蛋白胆固醇水平与肥胖指数升高有显著相关性(p < 0.05)。平均热量摄入和营养成分与肥胖指数无关。肥胖组与对照组比较:甘油三酯≥130 mg/dL的比值比为4.08;ldl -胆固醇≥130 mg的优势比为2.85;TC/ hdl -胆固醇≥4.0的优势比为1.16。高血压组BMI和甘油三酯水平均高于对照组(p < 0.05)。BMI与血压、低密度脂蛋白胆固醇、甘油三酯呈显著正相关(p < 0.05)。体重指数与平均热量摄入或营养无关。
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引用次数: 1
A Case of Grayanotoxin Intoxication Presenting with Mental Changes and Vomiting 灰鱼毒素中毒致精神改变及呕吐1例
Pub Date : 2008-09-01 DOI: 10.5223/KJPGN.2008.11.2.223
Curie Kim, Dong Soon Kim, H. Lee, Y. Ahn, J. Uhm
Rhododendron species is one of the largest and most diverse genera in the plant kingdom, comprising over 800 species and existing all over the globe. Grayanotoxin intoxication is caused by ingestion of honey and flowers. Grayanotoxin exists in honey, flowers, pollen, and nectar of the Rhododendron species. Grayanotoxin-intoxicated patients may present with nausea, vomiting, dizziness, weakness, hypotension, bradycardia, and syncope for several hours. We report a case of grayanotoxin intoxication associated with mental changes. A 9-year-old male presented with impaired consciousness and delirium 26 hours after eating about 10 rhododendron sclippenbachii flowers. A brain MRI and EEG were normal. Parenteral fluids were administered and these symptoms resolved completely in 17 hours. (Korean J Pediatr Gastroenterol Nutr 2008; 11: 223∼225)
杜鹃花属是植物界最大、种类最多的属之一,有800多种,分布在世界各地。灰色毒素中毒是由摄入蜂蜜和花朵引起的。灰色毒素存在于杜鹃属植物的蜂蜜、花朵、花粉和花蜜中。格雷诺毒素中毒患者可出现恶心、呕吐、头晕、虚弱、低血压、心动过缓和晕厥数小时。我们报告一例灰色毒素中毒与精神变化。一名9岁男性在食用约10朵杜鹃花26小时后出现意识受损和谵妄。脑部核磁共振和脑电图正常。给予肠外液体,这些症状在17小时内完全消失。韩国儿科胃肠病学杂志2008;11: 223∼225)
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引用次数: 4
A Case of Crigler-Najjar Syndrome Type 2 Diagnosed Using Genetic Mutation Analysis 基因突变分析诊断2型克里格勒-纳贾尔综合征1例
Pub Date : 2008-09-01 DOI: 10.5223/KJPGN.2008.11.2.219
Sang Yee Kim, S. Lee, H. Koh, Seung-Tae Lee, C. Ki, Jong-Won Kim, K. Chung
Crigler-Najjar syndrome is a rare inherited disease associated with unconjugated hyperbilirubinemia. It is inherited via an autosomal recessive pattern and is caused by mutation in one of the five exons of the bilirubin uridine-diphosphoglucuronate glucuronosyltransferase (UGT1A1) gene. The synthesis of inactive isoforms of bilirubin uridine-diphosphoglucuronate glucuronosyltransferase (B-UGT) results in unconjugated hyperbilirubinemia. A 13-year-old boy with jaundice for 4 months was admitted to our hospital. He had unconjugated hyperbilirubinemia with no evidence of infection, hemolysis, or structural abnormalities on abdominal ultrasonography or 99mTc-DISIDA scan. The authors identified a missense mutation of Tyr486Asp in the fifth exon of the UGT1A1 gene and diagnosed the patient with Crigler-Najjar syndrome type II. This is the first reported case of Crigler-Najjar syndrome in a Korean child, and it is also the first reported case of a genetic mutation leading to Crigler-Najjar syndrome in Korea. (Korean J Pediatr Gastroenterol Nutr 2008; 11: 219∼222)
Crigler-Najjar综合征是一种罕见的遗传性疾病,与非结合性高胆红素血症相关。它通过常染色体隐性模式遗传,由胆红素尿苷-二磷酸葡萄糖醛酸葡萄糖醛酸转移酶(UGT1A1)基因的五个外显子中的一个突变引起。胆红素-尿苷-二磷酸葡萄糖醛酸葡萄糖醛酸转移酶(B-UGT)失活异构体的合成导致非共轭高胆红素血症。我院收治一名13岁男童,黄疸4个月。他有非结合性高胆红素血症,腹部超声或99mTc-DISIDA扫描无感染、溶血或结构异常的证据。作者在UGT1A1基因的第5外显子发现Tyr486Asp错义突变,并诊断该患者为Crigler-Najjar综合征II型。这是国内首次报道的克里格勒-纳贾尔综合征,也是国内首次报道的因基因突变导致克里格勒-纳贾尔综合征的病例。韩国儿科胃肠病学杂志2008;11: 219∼222)
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引用次数: 3
Effect of Lamivudine Treatment on Chronic Hepatitis B Infection in Children Unresponsive to Interferon 拉米夫定治疗对干扰素无反应儿童慢性乙型肝炎感染的疗效
Pub Date : 2008-09-01 DOI: 10.5223/KJPGN.2008.11.2.137
G. Yeon, H. Kim, J. Park
Purpose: Interferon is a widely used treatment for chronic hepatitis B in children. However, additional treatment options are needed because more than 50% of hepatitis B patients are unresponsive to interferon. Although lamivudine is widely used to treat hepatitis B, there are few studies on the effect of lamivudine in hepatitis B patients unresponsive to interferon. Methods: Eight interferon unresponsive patients (6 males and 2 females) were treated with lamivudine (3 mg/kg/day, maximum 100 mg/day) from 6∼12 months after interferon treatment was discontinued among 33 children with chronic hepatitis B. They were treated with interferon (interferon α-2b, 10 MU/m or pegylated interferon 1.5μg/kg) for 6 months from January 2000 to December 2007 at the Pusan National University Hospital. The medical records were analyzed retrospectively. Results: The age at treatment with interferon and lamivudine was 4.9±3.1 and 6.1±3.2 years, respectively. The serum ALT level before treatment with interferon was 148.1±105.8 IU/L and the log HBV-DNA PCR mean value was 6.95±0.70 copies/mL. The serum ALT level after treatment with interferon was 143.1±90.4 IU/L and the log HBV-DNA mean PCR value was 6.46±2.08. HBeAg negativization occurred in 2 patients. For all patients, normalization of the serum ALT levels and HBeAg seroconversion (except 2 patients with HBeAg negativization) occurred at 7.4±2.1 and 7.9±2.1 months respectively after lamivudine treatment. The HBV-DNA PCR became negative in 7 patients (87.5%) at 2.4±2.8 months. Complete response was achieved in 7 patients and no recurrence was observed in 2 patients for 3 years after the completion of treatment. Five patients are still under treatment for a mean treatment duration of 24.4±9.1 months. In one patient, viral breakthrough occurred and the treatment was stopped. Conclusion: The number of patients was small, however, lamivudine treatment in patients with chronic hepatitis B who were unresponsive to interferon was highly effective. (Korean J Pediatr Gastroenterol Nutr 2008; 11: 137∼142)
目的:干扰素是一种广泛应用于儿童慢性乙型肝炎的治疗药物。然而,由于50%以上的乙型肝炎患者对干扰素无反应,需要额外的治疗方案。虽然拉米夫定被广泛用于治疗乙型肝炎,但关于拉米夫定在对干扰素无反应的乙型肝炎患者中的作用的研究很少。方法:对33例慢性乙型肝炎患儿,于2000年1月至2007年12月在釜山国立大学医院接受干扰素(干扰素α-2b、10 μg/ m或聚乙二醇化干扰素1.5μg/kg)治疗6个月,在停止干扰素治疗6 ~ 12个月后,8例干扰素无反应患者(男6例,女2例)给予拉米夫定治疗(3mg /kg/d,最大100mg /d)。对病历进行回顾性分析。结果:干扰素治疗年龄为4.9±3.1岁,拉米夫定治疗年龄为6.1±3.2岁。干扰素治疗前血清ALT水平为148.1±105.8 IU/L,对数HBV-DNA PCR平均值为6.95±0.70 copies/mL。干扰素治疗后血清ALT水平为143.1±90.4 IU/L,对数HBV-DNA平均PCR值为6.46±2.08。2例患者出现HBeAg阴性。所有患者(除2例HBeAg阴性患者外)在拉米夫定治疗后7.4±2.1和7.9±2.1个月血清ALT水平和HBeAg血清转化正常化。7例(87.5%)在2.4±2.8个月后HBV-DNA PCR转为阴性。7例患者完全缓解,2例患者治疗结束后3年无复发。5例患者仍在治疗中,平均治疗时间24.4±9.1个月。在一名患者中,病毒发生突破并停止了治疗。结论:患者数量较少,但拉米夫定治疗对干扰素无反应的慢性乙型肝炎患者疗效显著。韩国儿科胃肠病学杂志2008;11: 137∼142)
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引用次数: 0
Prevalence and Risk Factors Associated with Esophagitis in Children with Abdominal Pain 腹痛患儿食管炎的患病率及相关危险因素
Pub Date : 2008-09-01 DOI: 10.5223/KJPGN.2008.11.2.103
Hyun jung Kwon, D. Yi, E. Ryoo, K. Cho, D. Son, Hann Tcha
목 적: 소아에서 식도염은 성인과 달리 나이별로 다양하고, 비특이적인 증상이나 징후를 보인다. 또한 식도염을 진단하기 위한 상부 위장관 내시경과 조직 생검을 시행하는 것도 쉽지 않다. 이에 본 연구에서는 소아에서 식도염의 유병률과 조직 검사의 필요성 및 위험 인자에 대해 알아보고자 하였다. 방 법: 2006년 1월부터 2007년 12월까지 가천의대 길병원 소아과에 내원하여 급만성 상복부 복통을 보인 환자들 266명을 대상으로 문진 및 신체 검사, 상부 위장관 내시경과 함께 식도 및 위 조직 생검을 시행하였다. 식도염의 진단은 조직 검사로 확진 하였으며, H. pylori 감염 여부는 CLO 검사, H&E 염색과 Giemsa 염색 혹은 요소호기검사로 확인하였다. 식도염과 관련된 위험인자 및 내시경 소견과 조직학적 소견의 관련성을 비교분석하였다. 결 과: 상부 내시경과 조직 생검을 시행 환자 266명 중 조직학적 식도염 환자는 전체 266명 중 53명(19.9%)이었으며, 남자 18명(34.0%), 여자 35명(66.0%)으로 평균 나이는 11.6 ${pm}$ 0.87세였다. 내시경 소견의 민감도는 41.5%, 특이도는 77.0%, 양성 예측도 31.0%였다. 조직학적 식도염 환자 중 역류성 식도염은 50명, 호산구성 식도염은 2명, 캔디다 감염증에 의한 식도염은 1명이었다. 모든 연령에서 복통이 가장 흔한 증상으로 나타났으며, 8세 이하 환아들에서 구토가 의미 있게 많았다(p 【Purpose: Children with esophagitis express a variety of nonspecific symptoms and signs depending on their age, and diagnosis is limited because gastrointestinal endoscopy (GFS) and biopsy are difficult to perform. The aim of this study was to examine the prevalence of esophagitis in children with upper abdominal pain, to determine the necessity of esophageal biopsy, and to evaluate the associated risk factors. Methods: We reviewed 266 pediatric patients with upper abdominal pain who underwent history-taking, physical examination, and GFS with esophageal and gastric biopsies between January 2006 and December 2007. Esophagitis was confirmed on biopsy. We analyzed the risk factors for histologic esophagitis and the necessity of esophageal biopsy. Results: The prevalence of esophagitis was 19.9% (53/266 patients). The sensitivity and specificity of endoscopic diagnosis were 41.5% and 77%. Of 53 patients with histologic esophagitis, reflux esophagitis was seen in 50 patients, eosinophilic esophagitis was seen in 2 patients, and esophageal candidiasis was seen in 1 patient. Vomiting was a significant factor in patients under 8 yr of age (p】
颈迹:小儿食道炎与成人不同,根据年龄表现出多种多样的非特异性的症状或征兆。另外,为诊断食道炎,进行上胃肠内视镜和组织活检也不容易。为此,本研究试图了解小儿食道炎的发病率和组织检查的必要性及危险因素。方法:2006年1月至2007年12月,到嘉泉医科大学吉医院小儿科就诊,对266名急慢性上腹部腹痛患者进行问诊及身体检查,上胃肠内窥镜及食道及胃组织活检。食道炎的诊断通过组织检查确诊,是否感染H. pylori通过CLO检查、h&e染色、Giemsa染色或尿素检查确认。比较分析食道炎相关的危险因素及内窥镜诊断与组织学诊断的关联性。结科:266名上内窥镜和组织活检患者中,组织学食道炎患者在全部266名患者中有53名(19.9%),男性18名(34.0%),女性35名(66.0%),平均年龄为11.6美元(pm) 0.87美元。内窥镜诊断的敏感度为41.5%,特异度为77.0%,阳性预测为31.0%。组织学食道炎患者中,反流性食道炎有50人,湖酸组成食道炎有2人,坎迪达感染症引起的食道炎有1人。在所有年龄层中,腹痛是最常见的症状,在8岁以下的患者中,呕吐是有意义的。Children with esophagitis express a variety of nonspecific symptoms and signs depending on their age;and diagnosis is limited because gastrointestinal endoscopy (GFS) and biopsy are difficult to perform。The aim of this study was to examine The prevalence of esophagitis in children with upper abdominal pain, to determine The necessity of esophageal biopsy, and to evaluate The associated risk factors。methods:We reviewed 266 pediatric patients with upper abdominal pain who underwent history-taking, physical examination, and GFS with esophageal and gastric biopsies between January 2006 and December 2007。Esophagitis was confirmed on biopsy。我们analyzed the risk factors for histologic esophagitis and the necessity of esophageal biopsy。The prevalence of esophagitis was 19.9% (53/266 patients)。The sensitivity and specificity of endoscopic diagnosis were 41.5% and 77%。reflux esophagitis was seen in 50 patients, eosinophilic esophagitis was seen in 2 patients, and esophageal candidiasis was seen in 1 patient。Vomiting was a significant factor in patients under 8 yr of age (p)
{"title":"Prevalence and Risk Factors Associated with Esophagitis in Children with Abdominal Pain","authors":"Hyun jung Kwon, D. Yi, E. Ryoo, K. Cho, D. Son, Hann Tcha","doi":"10.5223/KJPGN.2008.11.2.103","DOIUrl":"https://doi.org/10.5223/KJPGN.2008.11.2.103","url":null,"abstract":"목 적: 소아에서 식도염은 성인과 달리 나이별로 다양하고, 비특이적인 증상이나 징후를 보인다. 또한 식도염을 진단하기 위한 상부 위장관 내시경과 조직 생검을 시행하는 것도 쉽지 않다. 이에 본 연구에서는 소아에서 식도염의 유병률과 조직 검사의 필요성 및 위험 인자에 대해 알아보고자 하였다. 방 법: 2006년 1월부터 2007년 12월까지 가천의대 길병원 소아과에 내원하여 급만성 상복부 복통을 보인 환자들 266명을 대상으로 문진 및 신체 검사, 상부 위장관 내시경과 함께 식도 및 위 조직 생검을 시행하였다. 식도염의 진단은 조직 검사로 확진 하였으며, H. pylori 감염 여부는 CLO 검사, H&E 염색과 Giemsa 염색 혹은 요소호기검사로 확인하였다. 식도염과 관련된 위험인자 및 내시경 소견과 조직학적 소견의 관련성을 비교분석하였다. 결 과: 상부 내시경과 조직 생검을 시행 환자 266명 중 조직학적 식도염 환자는 전체 266명 중 53명(19.9%)이었으며, 남자 18명(34.0%), 여자 35명(66.0%)으로 평균 나이는 11.6 ${pm}$ 0.87세였다. 내시경 소견의 민감도는 41.5%, 특이도는 77.0%, 양성 예측도 31.0%였다. 조직학적 식도염 환자 중 역류성 식도염은 50명, 호산구성 식도염은 2명, 캔디다 감염증에 의한 식도염은 1명이었다. 모든 연령에서 복통이 가장 흔한 증상으로 나타났으며, 8세 이하 환아들에서 구토가 의미 있게 많았다(p 【Purpose: Children with esophagitis express a variety of nonspecific symptoms and signs depending on their age, and diagnosis is limited because gastrointestinal endoscopy (GFS) and biopsy are difficult to perform. The aim of this study was to examine the prevalence of esophagitis in children with upper abdominal pain, to determine the necessity of esophageal biopsy, and to evaluate the associated risk factors. Methods: We reviewed 266 pediatric patients with upper abdominal pain who underwent history-taking, physical examination, and GFS with esophageal and gastric biopsies between January 2006 and December 2007. Esophagitis was confirmed on biopsy. We analyzed the risk factors for histologic esophagitis and the necessity of esophageal biopsy. Results: The prevalence of esophagitis was 19.9% (53/266 patients). The sensitivity and specificity of endoscopic diagnosis were 41.5% and 77%. Of 53 patients with histologic esophagitis, reflux esophagitis was seen in 50 patients, eosinophilic esophagitis was seen in 2 patients, and esophageal candidiasis was seen in 1 patient. Vomiting was a significant factor in patients under 8 yr of age (p】","PeriodicalId":212346,"journal":{"name":"Korean Journal of Pediatric Gastroenterology and Nutrition","volume":"3 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2008-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"125342299","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 10
Clinical Features of Cricopharyngeal Incoordination in Newborns and Infants 新生儿和婴儿环咽不协调的临床特点
Pub Date : 2008-09-01 DOI: 10.5223/KJPGN.2008.11.2.116
Y. Han, J. Park
Purpose: Cricopharyngeal incoordination is a rare cause of swallowing difficulties in newborns and infants; it is characterized by delayed pharyngeal contractions related to cricopharyngeal relaxation. Dysphagia and repeated aspiration are common findings despite normal sucking. We conducted this study to assess the clinical features of cricopharyngeal incoordination in newborns and infants. Methods: An analysis of the clinical data from 17 patients with cricopharyngeal incoordination who were admitted to the Department of Pediatrics, Pusan National University Hospital, between 2000 and 2006 was conducted retrospectively. The diagnosis of cricopharyngeal incoordination was established by the clinical characteristics and the videofluoroscopic swallowing studies. Results: The male to female ratio was 1:1.1 (males 8, females 9) the age range 1 to 60 days. The body weight of 11 patients (64.7%) was less than the 10 percentile at diagnosis. Six patients (35.3%) were born prematurely. The associated anomalies or diseases were chromosomal anomaly (2 cases), congenital heart disease (3 cases), and laryngomalacia, hypoxic brain damage or neonatal seizures (1 case each). The chief complaints of patients were recurrent aspiration pneumonia (10 cases), feeding difficulty (9 cases), dyspnea (4 cases), and chocking (4 cases). The severity of aspiration on the videoesophagogram or esophagogram was mild in 12 cases. The correlation between the severity of aspiration and the duration of tube feeding after the diagnosis was significant (p<0.05). Conclusion: Cricopharyngeal incoordination should be considered in the differential diagnosis of newborns and infants, without known risk factors associated with swallowing dysfunction, when they present with unexplained respiratory problems. Although the prognosis of cricopharyngeal incoordination is good, early diagnosis and tube feeding are recommended to prevent the complications associated with this disorder. (Korean J Pediatr Gastroenterol Nutr 2008; 11: 116∼121)
目的:环咽不协调是新生儿和婴儿吞咽困难的罕见原因;其特征是与环咽松弛有关的咽收缩延迟。吞咽困难和反复吸痰是正常吸痰的常见表现。我们进行了这项研究,以评估新生儿和婴儿环咽不协调的临床特征。方法:回顾性分析釜山大学附属医院儿科2000 ~ 2006年收治的17例环咽不协调患者的临床资料。环咽不协调的诊断是通过临床特征和透视吞咽检查建立的。结果:雌雄比为1:1.1(男8只,女9只),年龄1 ~ 60日龄。11例(64.7%)患者的体重在诊断时小于10百分位。早产6例(35.3%)。相关异常或疾病为染色体异常(2例)、先天性心脏病(3例)、喉软化、缺氧性脑损伤或新生儿癫痫各1例。患者主诉为反复吸入性肺炎(10例)、进食困难(9例)、呼吸困难(4例)、窒息(4例)。食管影像或食管造影显示的误吸严重程度为轻度12例。诊断后误吸严重程度与管饲时间的相关性有统计学意义(p<0.05)。结论:在没有已知危险因素与吞咽功能障碍相关的新生儿和婴儿出现不明原因的呼吸问题时,应将环咽不协调作为鉴别诊断的考虑因素。虽然环咽不协调的预后良好,但建议早期诊断和管饲以预防与此疾病相关的并发症。韩国儿科胃肠病学杂志2008;11: 116∼121)
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引用次数: 0
A Case of Gastric MALT Lymphoma Presenting as Nodular Gastritis in a Child 儿童胃MALT淋巴瘤表现为结节性胃炎1例
Pub Date : 2008-09-01 DOI: 10.5223/KJPGN.2008.11.2.187
Kun-Song Lee, H. Yang, J. Ko, J. Seo, H. Lee
Most cases of mucosa-associated lymphoid tissue (MALT) lymphoma occur in adults. MALT lymphoma is very rare in children. Helicobacter pylori ( H. pylori ) infection is known to be an important etiologic factor predisposing to the development of gastric MALT lymphoma. A 12-year-old girl was admitted because of intermittent abdominal pain occurring over the preceding 2 years. Nodular gastritis of the stomach was demonstrated on endoscopy. H. pylori infection was confirmed using the rapid urease test and histopathology. Histopathological examination of gastric biopsy specimens revealed lymphoepithelial lesions pathognomonic of MALT lymphoma, and immunohistochemical staining for CD20 was diffusely positive. Therefore, the patient was diagnosed with gastric MALT lymphoma. Clinical manifestations and histopathologic findings compatible with MALT lymphoma improved with the eradication of H. pylori infection. We report a case of primary gastric MALT lymphoma in a child, associated with H. pylori infection and presenting as nodular gastritis. (Korean J Pediatr Gastroenterol Nutr 2008; 11: 187 ∼ 192)
大多数粘膜相关淋巴组织(MALT)淋巴瘤发生在成人。MALT淋巴瘤在儿童中非常罕见。幽门螺杆菌(h.p ylori)感染被认为是诱发胃MALT淋巴瘤的重要病因。一名12岁女孩因前2年出现间歇性腹痛而入院。胃镜检查显示结节性胃炎。通过快速脲酶试验和组织病理学证实幽门螺杆菌感染。胃活检标本组织病理学检查显示淋巴上皮病变为MALT淋巴瘤的病理特征,CD20免疫组化染色弥漫性阳性。因此,患者被诊断为胃MALT淋巴瘤。随着幽门螺杆菌感染的根除,与MALT淋巴瘤相符的临床表现和组织病理学结果得到改善。我们报告一例儿童原发性胃MALT淋巴瘤,伴幽门螺杆菌感染,表现为结节性胃炎。韩国儿科胃肠病学杂志2008;[11: 187 ~ 192]
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引用次数: 2
Risk Factors for the Failure of Non-operative Reduction of Intussusceptions 非手术套叠复位失败的危险因素分析
Pub Date : 2008-09-01 DOI: 10.5223/KJPGN.2008.11.2.110
K. Ko, Y. W. Song, Bo-Kyung Je, J. Han, C. Woo, B. Choi, Jung Hwa Lee
Purpose: Intussusceptions are one of the most common causes of intestinal obstruction in infants and young children. Although it is easily treated by non-operative reduction using barium, water or air, this treatment is very stressful for young patients and may cause bowel perforation, peritonitis and shock. In this study, we identified the risk factors associated with the failure of non-operative reduction, to identify a group of children that would benefit from the procedure and those who would not. Methods: We reviewed the medical records of patients with intussusception who were treated at the Korea University Medical Center Ansan hospital from March 1998 to July 2006. Three hundred fourteen children with intussusception were identified. Among them, non-operative reductions were performed in three hundred. Clinical and radiological variables were compared according to the failure or success of the non-operative reduction. Results: Non-operative reductions were successful in 243 (81%) and failed in 57 (19%). The group that had failed procedures had a younger age (12.3±17.2 months vs. 18.0±15.8 months, p=0.03), longer symptom duration before reduction (33.6±29.0 hr vs. 21.5±20.3 hr, p<0.01), more vomiting and lethargy (p<0.01), but less abdominal pain and irritability (p<0.01), compared with the group that had a successful procedure. Logistic regression analysis showed that the factors associated with the failure of non-operative reductions were a younger age, less than 6 months of age (odds ratio: 2.5, 95% confidence interval: 1.2∼ 5.2, p=0.01), duration of symptoms, longer than 24 hrs before reduction (odds ratio: 2.1, 95% confidence interval: 1.2∼4.2, p=0.03), bloody stool (odds ratio: 4.8, 95% confidence interval: 1.9∼12.2, p<0.01), lethargy (odds ratio: 3.4, 95% confidence interval: 1.1∼10.4, p=0.04), and abdominal pain or irritability (odds ratio: 0.2, 95% confidence interval: 0.1∼0.4, p<0.01). Conclusion: For children with intussusception, an age younger than 6 months, and duration of symptoms more than 24 hrs before reduction, as well as the presence of bloody stools, lethargy and abdominal pain or irritability were variables associated with failure of a non-operative reduction. Knowledge of these variables should be considered in making clinical decisions for therapeutic interventions. (Korean J Pediatr Gastroenterol Nutr 2008; 11: 110∼115)
目的:肠套叠是婴幼儿肠梗阻最常见的原因之一。虽然它很容易通过使用钡、水或空气的非手术复位来治疗,但这种治疗对年轻患者来说压力很大,可能导致肠穿孔、腹膜炎和休克。在这项研究中,我们确定了与非手术复位失败相关的危险因素,以确定一组儿童将从手术中受益,而那些不会。方法:回顾性分析1998年3月至2006年7月在高丽大学医院安山医院收治的肠套叠患者的病历。发现了314例肠套叠患儿。其中非手术复位300例。根据非手术复位的成功或失败比较临床和放射学变量。结果:非手术复位成功243例(81%),失败57例(19%)。与手术成功组相比,手术失败组患者年龄更小(12.3±17.2个月vs. 18.0±15.8个月,p=0.03),减轻前症状持续时间更长(33.6±29.0小时vs. 21.5±20.3小时,p<0.01),呕吐和嗜睡发生率更高(p<0.01),腹痛和烦躁发生率更低(p<0.01)。Logistic回归分析显示,与非手术复位失败相关的因素有:年龄较小,小于6个月(优势比:2.5,95%可信区间:1.2 ~ 5.2,p=0.01)、症状持续时间,复位前时间大于24小时(优势比:2.1,95%可信区间:1.2 ~ 4.2,p=0.03)、带血便血(优势比:4.8,95%可信区间:1.9 ~ 12.2,p<0.01)、嗜睡(优势比:3.4,95%可信区间:1.1 ~ 10.4, p=0.04),腹痛或易怒(优势比:0.2,95%可信区间:0.1 ~ 0.4,p<0.01)。结论:对于年龄小于6个月的肠套叠患儿,在复位前症状持续时间超过24小时,以及出现血便、嗜睡、腹痛或易怒是非手术复位失败的相关变量。在制定治疗干预的临床决策时,应考虑到这些变量的知识。韩国儿科胃肠病学杂志2008;11: 110∼115)
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引用次数: 2
Primitive Neuroectodermal Tumor of the Liver in a 13-year-old Boy: A Case Report 13岁男孩原发性肝脏神经外胚层肿瘤1例报告
Pub Date : 2008-09-01 DOI: 10.5223/KJPGN.2008.11.2.214
Sang Lee, Ji Eun Kim, Ji Hyuk Lee, H. Lee, Jong Seung Lee, Jee Hyun Lee, Y. Choe
Primary primitive neuroectodermal tumor (PNET) of the liver is a rare disease with aggressive behavior and poor prognosis. We report a case of a PNET of the liver in a 13-year-old boy. The patient was admitted to the hospital with fever and abdominal pain. Abdominal CT and MRI revealed a 5.5 cm sized, septated, non-enhancing mass in the hepatic hilum. The patient was initially diagnosed with an inflammatory pseudotumor. Despite 9 days of antibiotic therapy, the patient’s clinical symptoms did not improve. A liver biopsy was performed in the interest of formulating a differential diagnosis. This procedure revealed tumor cells positive for CD99 on immunohistochemistry. The patient was diagnosed with a PNET. (Korean J Pediatr Gastroenterol Nutr 2008; 11: 214∼218)
肝脏原发性原始神经外胚层肿瘤(PNET)是一种罕见的疾病,具有侵袭性,预后差。我们报告一个13岁男孩的肝脏PNET病例。病人因发烧和腹痛住进医院。腹部CT和MRI显示肝门有一个5.5厘米大小的分隔性无增强肿块。患者最初被诊断为炎性假瘤。经过9天的抗生素治疗,患者的临床症状未见改善。肝活检是在制定鉴别诊断的利益。结果显示肿瘤细胞CD99免疫组化阳性。患者被诊断为PNET。韩国儿科胃肠病学杂志2008;11: 214∼218)
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引用次数: 0
期刊
Korean Journal of Pediatric Gastroenterology and Nutrition
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