• 아동학회지
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• 제29권3호
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• pp.1-21
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• 2008

This research explored links between mother/child personality characteristics based on their diagnostic and clinical pathology. The 232 participants, selected from mother-child pairs receiving psychological care in a hospital setting, were tested and interviewed by clinical practitioners. Correlations were found between mothers' Psychopathic Deviate, Paranoia, Psychasthenia, and Schizophrenia and sons' Psychasthenia, aggression, alienation, depression, and anger. Mothers classified with Hysteria were likely to have aggressive children. Mothers with low self-esteem, repression, frustration, and strong levels of extroversion had daughters with anger/rage issues. Sons of mothers diagnosed with Hypochondriasis, Depression, Hysteria, Psychopathic Deviate, Paranoia, and Psychasthenia had high rates of Oppositional Defiant Disorder and ADHD. Children of mothers diagnosed with depression had high rates of ADHD. Specifically, mothers with Hypochondriasis, Hysteria, and Psychopathic Deviate had daughters with high rates of ADHD.

• Journal of Genetic Medicine
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• 제18권2호
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• pp.83-93
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• 2021

Charcot-Marie-Tooth disease (CMT) is the most common hereditary motor and sensory peripheral neuropathy. CMT is usually classified into two categories based on pathology: demyelinating CMT type 1 (CMT1) and axonal CMT type 2 (CMT2) neuropathy. CMT1 can be distinguished by assessing the median motor nerve conduction velocity as greater than 38 m/s. The main clinical features of axonal CMT2 neuropathy are distal muscle weakness and loss of sensory and areflexia. In addition, they showed unusual clinical features, including delayed development, hearing loss, pyramidal signs, vocal cord paralysis, optic atrophy, and abnormal pupillary reactions. Recently, customized treatments for genetic diseases have been developed, and pregnancy diagnosis can enable the birth of a normal child when the causative gene mutation is found in CMT2. Therefore, accurate diagnosis based on genotype/phenotypic correlations is becoming more important. In this review, we describe the latest findings on the phenotypic characteristics of axonal CMT2 neuropathy. We hope that this review will be useful for clinicians in regard to the diagnosis and treatment of CMT.

• 대한미생물학회지
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• 제15권1호
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• pp.3-8
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• 1980

Yersinia enterocolitica has been known to be an important enteric pathogen especially in Scandinavian countries and Canada. In Korea, the authors reported the first case of Y. pseudotuberculosis septicemia in 1979. In 1980, three isolates of Y enterocolitica were obtained from 3 adult patients with enteritis, besides the already reported one in a 5-month-old child, during March to June 1980. Difficulty in the isolation was experienced; ie., the organism was isolated only from the SS primary isolation plate in one case and in the other two cases only from the SS plates inoculated with overnight culture of selenite broth. The isolates showed typical cultural and biochemical characteristics except for the nonmotility even at room temperature. Two isolates were indole negative possibly belonging to Wauter's biotype 3 and the other one was indole positive belonging to biotype 2. One patient was tested for the serum agglutinin titer on the 8th hospital day and it was found to be 1:128. All of the isolates were susceptible to chloramphenicol, colistin, gentamicin, kanamycin, tetracyclne, and tobramycin by the Kirby-Bauer disc diffusion method. All of the infections were controled by ampicillin, amoxicillin, amikacin, or gentamicin treatment. It is considered urgent to broaden our knowledge on yersiniosis in Korea not only by isolating, serotyping and biotyping of the organism, but also by surveying serum agglutinin titer of enteritis patients and normal individuals.

• Clinical and Experimental Pediatrics
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• 제56권1호
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• pp.19-25
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• 2013

Purpose: Childhood obesity is associated with nonalcoholic fatty liver disease (NAFLD), and it has become one of the most common causes of childhood chronic liver diseases which significant as a cause of liver related mortality and morbidity in children in the United States. The development of simpler and easier clinical indices for medical practice is needed to identify advanced hepatic fibrosis in childhood NAFLD instead of invasive method like liver biopsy. FibroScan and aspartate aminotransferase (AST)-to-platelet ratio index (APRI) have been proposed as a simple and noninvasive predictor to evaluate hepatic fibrosis in several liver diseases. APRI could be a good alternative to detect pathologic change in childhood NAFLD. The purpose of this study is to validate the efficacy of APRI for assessing hepatic fibrosis in childhood NAFLD based on FibroScan. Methods: This study included 23 children with NAFLD who underwent FibroScan. Clinical, laboratory and radiological evaluation including APRI was performed. To confirm the result of this study, 6 patients received liver biopsy. Results: Factors associated with hepatic fibrosis (stiffness measurement >5.9 kPa Fibroscan) were triglyceride, AST, alanine aminotransferase, platelet count, APRI and collagen IV. In multivariate analysis, APRI were correlated with hepatic fibrosis (>5.9 kPa). In receiver operating characteristics curve, APRI of meaningful fibrosis (cutoff value, 0.4669; area under the receiver operating characteristics, 0.875) presented sensitivity of 94%, specificity of 66%, positive predictive value of 94%, and negative predictive value of 64%. Conclusion: APRI might be a noninvasive, simple, and readily available method for medical practice to predict hepatic fibrosis of childhood NAFLD.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• 제17권3호
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• pp.170-177
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• 2014

Purpose: Choledochal cyst is a cystic dilatation of common bile duct. Although the etiology is presently uncertain, anomalous pancreaticobiliary ductal union (APBDU) is thought to be a major etiology of choledochal cyst. In this study, we analyzed the clinical and anatomical characteristics and pathologies of patients diagnosed with choledochal cyst in a single institute for 25 years. Methods: A total of 113 patients, diagnosed with choledochal cyst and who received an operation in Severance Children's Hospital from January 1988 to May 2013, were included. Medical records were reviewed, including clinical and demographic data, surgical procedures. Abdominal ultrasonography, magnetic resonance cholangiopancreatography, and intraoperative cholangiography were used as diagnostic tools for evaluation and classification of choledochal cyst and the presence of anomalous pancreaticobiliary ductal union. Todani's classification, and relationship between APBDU and surgical pathology. Results: Among 113 patients, 77 patients (68.1%) presented symptoms such as hepatitis, pancreatitis and/or cholecystitis. Eighty three patients (73.5%) had APBDU, and 94 patients (83.2%) showed inflammatory pathologic changes. APBDU, pathologic inflammation, and serological abnormalities such as hepatitis or pancreatitis showed a statistically significant correlation to one another. Conclusion: APBDU is thought to be one of the etiologic factors of choledochal cyst. It is related to the inflammatory changes in bile duct that can lead to the cystic dilatation.

• Clinical and Experimental Pediatrics
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• 제49권8호
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• pp.875-881
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• 2006

목 적 : KD는 주로 동양의 젊은 성인 여성에게서 경부 림프절 종대로 발현하는 것이 특징이며 수개월 내에 자연 치유되는 양호한 경과를 가지는 질환이다, 저자들은 KD로 진단된 소아환아의 임상적 특징을 알아보고, 환아들의 림프절 조직에서 바이러스 검출을 시도하여 KD와 HHV 8, EBV와의 연관성을 알아보기 위하여 본 연구를 시행하였다. 방 법 : 인제대학교 상계백병원에서 1998년 1월부터 2005년 12월까지 KD로 진단되어 치료받은 17세 이하 소아 26례를 대상으로 임상적 특징을 고찰하였다. 병력지 고찰을 통하여 후향적으로 분석하였고 추적 조사는 외래 병력 기록지와 전화 방문을 통하여 분석하였다. KD 환아의 림프절 조직으로부터 DNA를 추출하여 HHV 8 DNA를 검출하기 위해 PCR, EBV RNA를 검출하기 위해 ISH가 시행되었다. 결 과 : KD로 진단된 26명 중 남아 11례, 여아 15례로 성비는 1:1.4였고, 평균 연령은 13세였다. 환아의 연도별 분포는 2000년에 7례로 가장 많았으며, 여름에 가장 많이 진단되었다. 주증상은 발열(8/26)과 림프절의 동통(11/26)으로 발열의 기간은 평균 7.3일 이었다. 목빗근 뒷부위의 림프절 종대가 72%(18/24)였고 1례에서 경부 이외의 림프절 종대로 나타났다. 림프절의 크기는 $1cm{\times}1cm$에서부터 $6cm{\times}6cm$까지로 다양하게 나타났다. 백혈구 감소가 46%(6/13)에서 있었고 적혈구 침강 속도상승(>20 mm/hr)이 62%(8/13)에서 나타났다. 검체 확보가 가능하였던 20례 모두에서 HHV 8 DNA는 검출되지 않았으며, ISH를 이용한 EBV RNA 검사 결과도 음성이었다. KD 26례 모두 임상 경과는 양호하였으며, 1례(4%)만이 추적 중 발열과 함께 재발하였다. 전신성 홍반성 루프스를 포함한 결체 조직 질환으로의 이환은 관찰되지 않았다. 결 론 : KD는 소아에서 드물지 않게 발생하므로, 림프절 종대를 호소하는 환아에서 감별 진단에 포함되어야 한다. HHV 8과 EBV는 소아에서 진단된 KD의 원인 병원체로 작용할 가능성이 적을 것으로 생각된다.