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Hypocalcemic Tetany in a 13-Year-Old Girl with Wilson's Disease 13岁威尔逊氏病女童的低钙性手足搐搦症
Pub Date : 2011-03-01 DOI: 10.5223/KJPGN.2011.14.1.86
C. Ra, Sang Yong Kim, H. Koh
Wilson’s disease is an autosomal recessive disorder marked by disruptions in copper metabolism which leads to accumulation of copper in the liver, brain, cornea, and other tissues. Manifestations of this disease are more likely to be hepatic during early childhood and neurologic in adolescent. In addition, abnormalities that develop during disease progression may result in other manifestations such as hematologic, endocrine, or renal findings. Here we report a 13-year-old girl who presented with hypocalcemic tetany shortly after being diagnosed with Wilson’s disease. Despite aggressive calcium, magnesium, and vitamin D replacement therapy, the hypocalcemia and hypomagnesemia did not promptly respond. Mineral levels in the blood took longer than 3 weeks to normalize. We speculate that a parathyroid insufficiency and disrupted vitamin D metabolism caused by copper accumulation and hepatic dysfunction resulted in hypocalcemic tetany. (Korean J Pediatr Gastroenterol Nutr 2011; 14: 86∼90)
威尔逊氏病是一种常染色体隐性遗传病,其特征是铜代谢紊乱,导致铜在肝脏、大脑、角膜和其他组织中积累。这种疾病的表现在儿童早期更可能是肝脏和青少年的神经系统。此外,在疾病进展过程中出现的异常可能导致其他表现,如血液学、内分泌或肾脏方面的发现。在此,我们报告一位13岁的女孩,在被诊断为威尔逊氏病后不久,出现了低钙性手足搐缩。尽管积极的钙、镁和维生素D替代治疗,低钙和低镁血症并没有迅速反应。血液中的矿物质水平需要超过3周的时间才能恢复正常。我们推测,由铜积累和肝功能障碍引起的甲状旁腺功能不全和维生素D代谢紊乱导致低钙性手足搐缩。韩国儿科胃肠病学杂志2011;14: 86∼90)
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引用次数: 0
The Importance of Nutritional Assessment and Dietary Counseling in Infants and Young Children with Common Illnesses 常见病婴幼儿营养评估与饮食咨询的重要性
Pub Date : 2011-03-01 DOI: 10.5223/KJPGN.2011.14.1.33
S. Jeong
The influence of nutrition during early life on physical growth as well as mental development has been thoroughly discussed in the literature. The physical dimensions of the body are greatly influenced by nutrition, particularly during the period of rapid growth in early childhood. Nutritional status affects every pediatric patient's response toillness. Good nutrition is important for achieving normal growth and development. It is indicated that permanent impairment of the central nervous system may result from dietary restriction of imbalance during certain periods of life. If children under 3 years of age show a good nutritional status, it may be assumed that they are well nourished. Several common diseases of children such as iron deficiency, chronic constipation and atopic dermatitis are known food related diseases. Patients with chronic illness and those at risk of malnutrition should have detailed nutritional assessments done. Components of a complete nutritional assessment include a medical history, nutritional history including dietary intake, physical examination, anthropometrics (weight, length or stature, head circumference, midarm circumference, and triceps skinfold thickness), pubertal staging, skeletal maturity staging, and biochemical tests of nutritional status. The use of age, gender, and disease-specific growth charts is essential in assessing nutritional status and monitoring nutrition interventions. Nutrition assessment and dietary counseling is helpful for the cure of disease, and moreover, the prevention of illness. (Korean J Pediatr Gastroenterol Nutr 2011; 14: 33∼44)
早期营养对身体发育和智力发育的影响已经在文献中得到了深入的讨论。身体的尺寸很大程度上受营养的影响,特别是在儿童早期的快速生长时期。营养状况影响着每个儿科患者对疾病的反应。良好的营养是实现正常生长发育的重要条件。这表明,中枢神经系统的永久性损害可能是由于在生命的某些时期饮食限制或不平衡造成的。如果3岁以下的儿童表现出良好的营养状况,可以认为他们营养良好。一些常见的儿童疾病,如缺铁、慢性便秘和特应性皮炎,都是已知的与食物有关的疾病。慢性疾病患者和有营养不良风险的患者应进行详细的营养评估。完整营养评估的组成部分包括病史、营养史(包括膳食摄入)、体格检查、人体测量(体重、身高或身高、头围、中臂围和肱三头肌皮褶厚度)、青春期分期、骨骼成熟度分期和营养状况的生化测试。使用年龄、性别和疾病特异性生长图表对于评估营养状况和监测营养干预措施至关重要。营养评估和饮食咨询有助于疾病的治疗,更有助于疾病的预防。韩国儿科胃肠病学杂志2011;14: 33∼44)
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引用次数: 2
Pediatric Inflammatory Bowel Disease (IBD): Phenotypic, Genetic and Therapeutic Differences between Early-Onset and Adult-Onset IBD 儿童炎症性肠病(IBD):早期发病与成人发病IBD的表型、遗传和治疗差异
Pub Date : 2011-03-01 DOI: 10.5223/KJPGN.2011.14.1.1
J. Seo
Inflammatory bowel disease (IBD) develops during childhood or adolescence in approximately 25% of patients with IBD. Recent studies on pediatric IBD have revealed that early-onset IBD has distinct phenotype differences compared to adult onset IBD. Pediatric early-onset IBD differs in many aspects including disease type, location of the lesions, disease behavior, gender preponderance and genetically attributable risks. This review examines the currently published data on the clinical, epidemiological and genetic differences between early-onset and adult-onset IBD. And finally, therapeutic considerations in the management of pediatric-onset IBD are also discussed. (Korean J Pediatr Gastroenterol Nutr 2011; 14: 1∼25)
大约25%的炎症性肠病(IBD)患者在儿童期或青春期发病。最近对儿童IBD的研究表明,与成人IBD相比,早发性IBD具有明显的表型差异。儿童早发性IBD在许多方面存在差异,包括疾病类型、病变部位、疾病行为、性别优势和遗传归因风险。这篇综述检查了目前发表的关于早发性和成年性IBD的临床、流行病学和遗传差异的数据。最后,在儿科发病的IBD管理的治疗考虑也进行了讨论。韩国儿科胃肠病学杂志2011;14: 1∼25)
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引用次数: 16
Antimicrobial Resistance of Helicobacter pylori Isolated from Korean Children 韩国儿童幽门螺杆菌的耐药性研究
Pub Date : 2011-03-01 DOI: 10.5223/KJPGN.2011.14.1.45
Y. Kim, Y. Lee, S. Oh, H. Sung, Mina Kim, K. Kim
Purpose: The antimicrobial resistance of Helicobacter pylori is one of the critical factors in failure of eradication therapy. The purpose of this study was to evaluate antimicrobial resistance of H. pylori in Korean children. Methods: Gastric mucosal specimens for H. pylori were obtained from children with dyspepsia who were cared for at Asan Medical Center Children’s Hospital in Seoul, Korea between 2003 and 2009. Antimicrobial resistance tests were performed using the disk diffusion method for clarithromycin and amoxicillin and the E-test for metronidazole and tetracycline. Most children with H. pylori infections were treated using triple therapies. Results: Thirty-three children had positive H. pylori cultures, although a resistance test was only performed in 28 patients. Resistant strains were found in 9 children (32.1%). The resistance rates to clarithromycin and metronidazole were 25% and 17.8%, respectively. There was no resistance to amoxicillin or tetracycline. The resistance rates decreased from 44.4% (2003∼2006) to 26.3% (2006∼2009) during the study period. Conclusion: Korean children demonstrated relatively high antimicrobial resistance to H. pylori in this study. However, there was a temporarily decreasing trend during the study period. A larger multi-regional study may be needed to determine the optimal antimicrobial treatment for pediatric patients infected with H. pylori. (Korean J Pediatr Gastroenterol Nutr 2011; 14: 45∼51)
目的:幽门螺杆菌的耐药性是导致根除治疗失败的关键因素之一。本研究的目的是评估韩国儿童幽门螺杆菌的耐药性。方法:对2003年至2009年在韩国首尔峨山医疗中心儿童医院就诊的消化不良患儿进行胃黏膜幽门螺杆菌标本采集。采用纸片扩散法对克拉霉素和阿莫西林进行耐药试验,采用e -法对甲硝唑和四环素进行耐药试验。大多数幽门螺杆菌感染儿童采用三联疗法治疗。结果:33名儿童幽门螺杆菌培养呈阳性,尽管只有28名患者进行了耐药试验。耐药菌株9例(32.1%)。对克拉霉素和甲硝唑的耐药率分别为25%和17.8%。对阿莫西林和四环素均无耐药。在研究期间,耐药率从44.4%(2003 ~ 2006年)下降到26.3%(2006 ~ 2009年)。结论:本研究中韩国儿童对幽门螺杆菌的耐药性较高。但在研究期间,出现了暂时下降的趋势。可能需要更大的多区域研究来确定幽门螺杆菌感染儿科患者的最佳抗菌治疗。韩国儿科胃肠病学杂志2011;14: 45∼51)
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引用次数: 3
Diagnostic Approaches to Chronic Abdominal Pain in Children 儿童慢性腹痛的诊断方法
Pub Date : 2011-03-01 DOI: 10.5223/KJPGN.2011.14.1.26
J. Park
Chronic abdominal pain (CAP) is a common complaint encountered in pediatric clinics and a great concern for patients and their caretakers as well as health care professionals. A constant challenge is detecting individuals with organic diseases or psychosomatic disorders from the majority of patients who have a functional disorder including functional dyspepsia, irritable bowel syndrome, functional abdominal pain, and abdominal migraine. Beginning with a detailed history and physical examination, physicians must determine a differential diagnosis of CAP by applying the symptom-based Rome III criteria to positively identify a functional disorder. These findings should then be further analyzed based on diagnostic clues and red flags that indicate the presence of specific organic diseases and/or the need for further testing. Once a functional diagnosis has been made or an organic disease is suspected, physicians can initiate an empiric therapeutic trial. Since psychological distress accompanies both organic and non-organic abdominal pain in children, a cooperative diagnostic approach involving pediatricians and psychiatrists is recommended. (Korean J Pediatr Gastroenterol Nutr 2011; 14: 26∼32)
慢性腹痛(CAP)是儿科诊所常见的主诉,也是患者及其护理人员以及卫生保健专业人员非常关注的问题。从功能性消化不良、肠易激综合征、功能性腹痛和腹部偏头痛等大多数功能性疾病患者中检测出器质性疾病或心身疾病患者是一个持续的挑战。从详细的病史和体格检查开始,医生必须通过应用基于症状的Rome III标准来确定CAP的鉴别诊断,以确定功能性障碍。然后应根据诊断线索和提示存在特定器质性疾病和/或需要进一步检测的危险信号对这些发现进行进一步分析。一旦做出功能诊断或怀疑器质性疾病,医生就可以开始经验性治疗试验。由于儿童有器质性和非器质性腹痛都伴有心理困扰,建议采用儿科医生和精神科医生合作的诊断方法。韩国儿科胃肠病学杂志2011;14: 26∼32)
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引用次数: 2
Meconium Obstruction in Very Low Birth Weight Infants 极低出生体重儿的胎便梗阻
Pub Date : 2011-03-01 DOI: 10.5223/KJPGN.2011.14.1.52
Ki Bae Hong, I. Seong, Kun-Song Lee, Y. Chang, H. Song
Purpose: Mecnoium obstruction in very low birth weight infants (VLBWI), which delays enteral feeding and is one of the major causes of bowel obstruction, can be diagnosed and treated with hyperosmolar water-soluble contrast enema. The purpose of this study was to observe the clinical findings of meconium obstruction, the improvement of small bowel obstruction after contrast enema, and the complications related to the enema. Methods: Hypersolmolar water-soluble contrast enemas were performed in 14 VLBWIs with meconium obstruction. Clinical findings, radiologic findings, feeding intolerance, effectiveness, and complications of enemas were observed. Also, clinical findings related to meconium obstruction were compared with 18 VLBWIs without meconium obstruction. Results: 1) Fourteen VLBWIs with meconium obstruction had significantly lower 5 minutes Apgar scores than 18 VLBWIs without meconium obstruction (p<0.05). Moreover, the day of last meconium passing, and the day of the first trial and full enteral feeding were delayed significantly. 2) A total of 18 enemas were performed in the 14 infants. The contrast medium passed the ileocecal valve and reached the terminal ileus in 12 enemas. Of the 12 enemas, 11 were successful, but 1 infant underwent an ileotomy, even though the contrast medium reached the terminal ileum. 3) Intestinal obstruction was not relieved in three of five infants, in whom the contrast medium failed to pass the ileocecal valve. Obstruction was relieved after repeated enemas in which the contrast medium reached the terminal ileum. 4) No complications associated with water-soluble contrast enemas were observed. Conclusion: Hyperosmolar water-soluble contrast enema is considered to be safe and therapeutic for meconium obstruction in VLBWIs. (Korean J Pediatr Gastroenterol Nutr 2011; 14: 52∼58)
目的:极低出生体重儿(VLBWI)绒毛膜梗阻是肠内喂养延迟的主要原因之一,可通过高渗水溶性对比灌肠进行诊断和治疗。本研究的目的是观察胎便梗阻的临床表现,对比灌肠后小肠梗阻的改善情况,以及与灌肠相关的并发症。方法:对14例胎便梗阻的vlbwi患者行高溶剂水溶性造影剂灌肠。观察灌肠的临床表现、影像学表现、喂养不耐受、效果及并发症。同时,比较了18例无胎便梗阻的vlbwi与胎便梗阻的临床表现。结果:1)有胎便梗阻的产妇14例,其5分钟Apgar评分明显低于无胎便梗阻的产妇18例(p<0.05)。最后一次胎便排出日、初试日和全肠喂养日均明显推迟。2) 14例患儿共行18次灌肠。造影剂经回盲瓣到达末端肠梗阻12例。在12例灌肠中,11例成功,但1例婴儿进行了回肠切开术,尽管造影剂已到达回肠末端。3) 5例婴儿中有3例肠梗阻未缓解,造影剂未能通过回盲瓣。反复灌肠后,造影剂到达回肠末端,阻塞得以缓解。4)水溶性对比剂灌肠未见并发症。结论:高渗水溶性对比灌肠剂是治疗vlbwi胎便梗阻的安全有效的方法。韩国儿科胃肠病学杂志2011;14: 52∼58)
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引用次数: 3
Acute Intermittent Porphyria Presented with Recurrent Abdominal Pain and Hypertension 急性间歇性卟啉症表现为反复腹痛和高血压
Pub Date : 2011-03-01 DOI: 10.5223/KJPGN.2011.14.1.81
M. Park, J. Seo, J. Ko, J. Chang, H. Yang
Acute intermittent porphyria (AIP) is a rare disorder characterized biochemically by the increased excretion of porphyrins and porphyrin precursors, including delta-aminolevulinic acid (ALA) and porphobilinogen (PBG). AIP has variable clinical manifestations, such as acute abdominal pain, vomiting, nausea, constipation, peripheral neuropathy, seizures, tachycardia, and hypertension. A 16-year-old girl presented with recurrent abdominal pain, vomiting, hypertension, seizures, hypercholesterolemia, and red urine. AIP was confirmed by clinical features and increased 24-hour urine ALA and PBG. AIP should be considered in the differential diagnosis of patients who have abdominal pain, hypertension, and seizures when the results of all other tests are normal. (Korean J Pediatr Gastroenterol Nutr 2011; 14: 81∼85)
急性间歇性卟啉症(AIP)是一种罕见的疾病,其生化特征是卟啉和卟啉前体(包括δ氨基乙酰丙酸(ALA)和卟啉胆色素原(PBG))的排泄增加。AIP有多种临床表现,如急性腹痛、呕吐、恶心、便秘、周围神经病变、癫痫发作、心动过速、高血压等。一个16岁的女孩表现为反复腹痛,呕吐,高血压,癫痫发作,高胆固醇血症和红尿。临床表现和24小时尿液ALA和PBG升高证实了AIP。在其他检查结果正常的情况下,有腹痛、高血压和癫痫的患者应考虑AIP的鉴别诊断。韩国儿科胃肠病学杂志2011;14: 81∼85)
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引用次数: 2
Gaps between Infant Diarrheal Disease and Frequent Loose Stool 婴儿腹泻病与频繁稀便之间的差距
Pub Date : 2010-12-01 DOI: 10.5223/KJPGN.2010.13.SUPPL1.S44
Jee Hyun Lee
Chronic diarrhea in children has a long differential diagnosis, but the clinician can usually distinguish disease from functional diarrhea. A careful history will provide information on medical and dietary problem, growth disturbance, associated symptoms and signs. The extent of laboratory evaluation should be guided by common sense. In the absence of the worrisome historical items noted, or abnormal findings on examination or growth history, reassurance may be all that is required. This article reviewed the diagnostic approach and differential diagnosis of chronic diarrhea, and summarized common non pathologic conditions of chronic diarrhea such as chronic nonspecific diarrhea (toddler’s diarrhea) and secondary lactase deficiency. [Korean J Pediatr Gastroenterol Nutr 2010; 13(Suppl 1): 44∼50]
儿童慢性腹泻的鉴别诊断由来已久,但临床医生通常可以将其与功能性腹泻区分开来。仔细的病史将提供有关医疗和饮食问题、生长障碍、相关症状和体征的信息。实验室评价的范围应以常识为指导。如果没有令人担忧的病史记录,或检查或生长史上的异常发现,可能需要的只是保证。本文综述了慢性腹泻的诊断方法和鉴别诊断,并总结了慢性非特异性腹泻(幼儿腹泻)和继发性乳糖酶缺乏等慢性腹泻的常见非病理情况。中华儿科杂志[J];13(增刊1):44 ~ 50]
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引用次数: 0
Diagnostic Approach of Lower GI Bleeding in Children 儿童下消化道出血的诊断方法
Pub Date : 2010-12-01 DOI: 10.5223/KJPGN.2010.13.SUPPL1.S51
S. Jeong
Lower GI bleeding is one of the common and difficult problems in the practice of general pediatrics. Causes of bleeding are various but somewhat age-specific in children. A specific diagnosis can usually be made with a accurate history taking, physical examination, included rectal exam, simple laboratory investigations, and appropriate diagnostic studies. Further evaluations can be unnecessary if the patient have a small amount of bleeding and stable vital sign. But precise investigation included abdominal sonography, endoscopy, Meckel’s scan, and bleeding scan, are needed on a case by case. Treatment should be directed at the underlying cause. In most children, bleeding ceases spontaneously, and only supportive therapy is necessary. If there is evidence of hypovolemia, the patient must be hemodynamically stabilized, active bleeding stopped, and recurrent bleeding prevented. This review included age-specific cases such as Allergic proctocolitis, Meckel’s diverticulum, Juvenile polyps, Henoch-scholein purpura, and Crohn disease, of lower gastrointestinal bleeding in children. Also it will assist the physician in determining appropriate assessment and treatment for children with lower GI bleeding through the usual cases. [Korean J Pediatr Gastroenterol Nutr 2010; 13(Suppl 1): 51∼58]
下消化道出血是普通儿科实践中常见和困难的问题之一。出血的原因多种多样,但在儿童中有一定的年龄特异性。具体的诊断通常可以通过准确的病史、体格检查(包括直肠检查)、简单的实验室检查和适当的诊断研究来做出。如果患者有少量出血且生命体征稳定,则无需进一步评估。但精确的检查包括腹部超声检查、内窥镜检查、梅克尔扫描和出血扫描,需要逐案进行。治疗应针对根本原因。在大多数儿童中,出血会自发停止,只需要支持性治疗。如果有低血容量的证据,必须稳定患者的血流动力学,停止活动性出血,防止复发性出血。本综述包括儿童下消化道出血的年龄特异性病例,如过敏性直结肠炎、梅克尔憩室、少年型息肉、过敏性紫癜和克罗恩病。此外,它将帮助医生确定适当的评估和治疗下消化道出血的儿童通过通常的情况。中华儿科杂志[J];13(增刊1):51 ~ 58]
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引用次数: 0
Current Nutritional Status of Korean Adolescents and Countermeasures 韩国青少年营养现状及对策
Pub Date : 2010-12-01 DOI: 10.5223/KJPGN.2010.13.SUPPL1.S10
J. Moon
The importance of healthier nutrition and lifestyle in children and adolescents is growing more and more nowadays in the era of exponential increase of chronic diseases, such as cardiovascular diseases, diabetes mellitus, hypertension and hyperlipidemia. But the situation of the adolescent nutrition in Korea looks no so good. According to the several recent surveys in Korea, 67% increase in obesity during the past decade was striking. Prevalence of wasting in school girls was increasing, which were accompanied by high prevalence of eating disorder prone factors. Unhealthy eating behavior and selective deficiency of nutrient intake were common. Calcium and potassium were the most common deficient minerals of adolescents, whose usual dietary intake was below Korean Recommended Guidelines. To meet the upcoming challenges of nutrition in adolescence, we should prepare the new multidisciplinary policy including healthier school nutrition environment and enrollment of the health care clinics or hospitals as primary prevention providers. [Korean J Pediatr Gastroenterol Nutr 2010; 13(Suppl 1): 10∼14]
在心血管疾病、糖尿病、高血压和高脂血症等慢性疾病呈指数级增长的今天,健康的营养和生活方式对儿童和青少年的重要性越来越大。但是韩国青少年的营养状况却不容乐观。根据最近在韩国进行的几项调查,在过去的十年里,韩国的肥胖人口增加了67%,这是惊人的。在校女生的消瘦率正在上升,同时伴有易患饮食失调的因素的高发。不健康的饮食行为和选择性营养摄入不足是常见的。钙和钾是青少年中最常见的缺乏矿物质,他们的日常饮食摄入量低于韩国推荐指南。为了应对即将到来的青少年营养挑战,我们应该准备新的多学科政策,包括更健康的学校营养环境和卫生保健诊所或医院作为初级预防提供者的登记。中华儿科杂志[J];[13](增刊1):10 ~ 14]
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引用次数: 0
期刊
Korean Journal of Pediatric Gastroenterology and Nutrition
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