• Clinical and Experimental Pediatrics
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• v.55 no.3
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• pp.83-87
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• 2012

Several authors suggested that the clinical characteristics of incomplete presentation of Kawasaki disease are similar to those of complete presentation and that the 2 forms of presentation are not separate entities. Based on this suggestion, a diagnosis of incomplete Kawasaki disease in analogy to the findings of complete presentation is reasonable. Currently, the diagnosis of incomplete Kawasaki disease might be made in cases with fewer classical diagnostic criteria and with several compatible clinical, laboratory or echocardiographic findings on the exclusion of other febrile illness. Definition of incomplete presentation in which coronary artery abnormalities are included as a necessary condition, is restrictive and specific. The validity of the diagnostic criteria of incomplete presentation by the American Heart Association should be thoroughly tested in the immediate future.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.22 no.4
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• pp.392-399
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• 2019

Wilson disease a rare autosomal recessive inherited disorder of copper metabolism, is characterized by excessive deposition of copper in the liver, brain, and other tissues. Wilson disease is often fatal if it is not recognized early and treated when it is symptomatic. Gitelman syndrome is also an autosomal recessive kidney disorder characterized by low blood levels of potassium and magnesium, decreased excretion of calcium in the urine, and elevated blood pH. Hereditary sensory autonomic neuropathy type IV (HSAN-IV), a very rare condition that presents in infancy, is characterized by anhidrosis, absence of pain sensation, and self-mutilation. It is usually accompanied by developmental delay and mental retardation. We report a case of Wilson disease manifested as fulminant hepatitis, acute pancreatitis, and acute kidney injury in a 15-year-old boy comorbid with HSAN-IV and Gitelman syndrome. Such concurrence of three genetic diseases is an extremely rare case.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.21 no.1
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• pp.43-50
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• 2018

Purpose: Clostridium difficile colonization and infection are commonly associated with poor outcomes in patients with pediatric inflammatory bowel disease (PIBD). We aimed to investigate the prevalence of C. difficile colonization and infection at the time of diagnosis and to evaluate risk factors associated with the development of C. difficile infection during the course of PIBD treatment. Methods: We retrospectively enrolled a total of 59 children who were newly diagnosed with PIBD at the tertiary medical center. All patients underwent C. difficile toxin assays and cultures initially and at every follow-up during the disease course. Kaplan-Meier survival analysis and Cox regression test were used for statistical analysis. Results: Initial cultures for C. difficile were positive in 13 (22.0%) of 59 PIBD patients, whereas initial toxin assays were positive in 3 patients (5.1%). During treatment, C. difficile cultures converted to positive in 28 (47.5%) in addition to 13 patients who were initially culture-positive, and C. difficile toxins converted to positive in 13 (22.0%) in addition to 3 originally toxin-positive patients. Antibiotic usage alone was significantly associated with the development of C. difficile colonization (p=0.011), and the length of hospitalization was associated with the development of C. difficile infection (p=0.032). Conclusion: C. difficile colonization and infection occur frequently during the disease course of PIBD. Antibiotic usage and longer hospital stay were significant risks factors for the conversion of C. difficile status in PIBD patients undergoing treatment.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.24 no.3
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• pp.295-305
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• 2021

Purpose: Hepcidin levels have previously been reported to be correlated with liver damage. However, the association between hepcidin levels and liver fibrosis in children with fatty liver disease remains unclear. This study therefore aimed to investigate the pathophysiology of fibrosis in children with fatty liver disease and its association with hepcidin levels. Methods: This retrospective case series included 12 boys aged 6-17 years who were diagnosed with nonalcoholic fatty liver disease (NAFLD) or nonalcoholic steatohepatitis (NASH) at the Tokyo Medical University Hospital. Sixteen liver biopsy samples from 12 subjects were analyzed. Serum hepcidin levels were assayed using enzyme-linked immunosorbent assay. Immunostaining for hepcidin was performed, and the samples were stratified by staining intensity. Results: Serum hepcidin levels were higher in pediatric NAFLD/NASH patients than in controls. Conversely, a significant inverse correlation was observed between hepcidin immunostaining and Brunt grade scores and between hepcidin scores and gamma-glutamyltranspeptidase, hyaluronic acid, and leukocyte levels. We observed inverse correlations with a high correlation coefficient of >0.4 between hepcidin immunostaining and aspartate aminotransferase, alanine aminotransferase, total bile acid, and platelet count. Conclusion: There was a significant inverse correlation between hepcidin immunoreactivity and fibrosis in pediatric NAFLD patients; however, serum hepcidin levels were significantly higher, suggesting that these patients experienced a reduction in the hepcidin-producing ability of the liver in response to iron levels, leading to subsequent fibrosis. Therefore, hepcidin levels can be used as markers to identify the progression of fibrosis in patients with NAFLD.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.24 no.6
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• pp.518-527
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• 2021

Purpose: The incidence of hepatic steatosis among children has been increasing; however, data distinguishing simple steatosis from a more complex disorder are lacking. Methods: This study identified the etiologies resulting in hepatic steatosis through a retrospective review of pediatric liver biopsies performed in the last 10 years. A total of 158 patients with hepatic steatosis proven by histopathological evaluation were enrolled in the study, and baseline demographic features, anthropometric measurements, physical examination findings, laboratory data, ultrasonographic findings, and liver histopathologies were noted. Results: The two most common diagnoses were inborn errors of metabolism (IEM) (52.5%) and nonalcoholic fatty liver disease/steatohepatitis (NAFLD/NASH) (29.7%). The three most common diseases in the IEM group were glycogen storage disorders, Wilson's disease, and mitochondrial disease. The rates of consanguineous marriage (75.6%; odds ratio [OR], 26.040) and positive family history (26.5%; OR, 8.115) were significantly higher (p=0.002, p<0.001, respectively) in the IEM group than those in the NAFLD/NASH group. Younger age (p=0.001), normal anthropometric measurements (p=0.03), increased aspartate aminotransferase levels (p<0.001), triglyceride levels (p=0.001), and cholestatic biochemical parameters with disrupted liver function tests, as well as severe liver destruction of hepatic architecture, cholestasis, fibrosis, and nodule formation, were also common in the IEM group. Conclusion: Parents with consanguinity and positive family history, together with clinical and biochemical findings, may provide a high index of suspicion for IEM to distinguish primary steatosis from the consequence of a more complex disorder.

• Pediatric Infection and Vaccine
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• v.10 no.1
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• pp.123-126
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• 2003

Kikuchi disease(subacute necrotizing lymphadenitis), first reported by Kikuchi and Fujimoto in 1972, is a benign self-limiting illness characterized by fever, neutropenia and cervical lymphadenopathy and develops predominantly in young women, especially in Asia. The cause of Kikuchi disease is unknown, but postinfectious(virus or bacteria) hyperimmune reaction has been suggested. Few pediatric cases have been reported. We experienced a case of Kikuchi disease associated with Yersinia pseudotuberculosis infection in 12-year-old girl. After she was admitted with cervical lymphadenopathy and headache, followed by sustained fever with leukopenia. All symptoms and signs did not seem to be responded to antimicobial treatment. Open biopsy of cervical lymph node was performed and showed findings consistent with subacute necrotizing lymphadenitis. Serologic studies were all negative except for Y. pseudotuberculosis. Fever subsided and lymphadenopathy improved after administration of oral steroid for 1 week.

• The Journal of Pediatrics of Korean Medicine
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• v.15 no.2
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• pp.209-220
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• 2001

Objectives : The aim of this study was to investigate the attitude toward the oriental hospital among 100 pediatric outpatients. Methods : A questionnaire about the general characteristics of patients. the reason of choice or alteration was implemented. Results : 1. In distribution of sex & age, 60% was men and 40% women was between 2 and 6 years old. 2. In determinant factor for using Oriental medicine, 43.8% was tolerance increase of the western medicine and 33.0% was recommened by others. 3. In distribution of disease & therapeutic method, 62.5% was respiratory disease and 11.7% gastrointestinal disease, 64.3% was treated with oriental after Western medical therapy and 20.9% only Oriental medical care. 4. The changing causes for using Oriental after Western medicine, 35.9% was ineffective affect and 26.5% side effect. 5. The preference for using Oriental medicine, 41.6% was respiratory disease and 21.0% gastrointestinal disease. 6. The peculiarity of Oriental medicine compared with western medicine, 36% was effectiveness on the cronic disease and 14% for fundamental remedy. 7. The prodominance disease in Western medicine compared with Oriental medicine, 28% was operation, 18% common cold and 15% inflammatory disease 8. The questions about Oriental medicine, 22% was medical material's sanitation and place of origin, 18% harmness or not administered during long time. 9. The controversial point of Oriental medicine, 33.5% was expensive price and 27.7% difficulty of taking medicine. 10. In satisfaction degree of Oriental medicine, 69% was satisfied and 19% was very satisfied. Conclusions : Considering the above results, we have concluded that proper recognition about Oriental Pediatric medicine will be needed among patients.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.17 no.1
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• pp.23-30
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• 2014

Manometry is a noninvasive diagnostic tool for identifying motility dysfunction of the gastrointestinal tract. Despite the great technical advances in monitoring motility, performance of the study in pediatric patients has several limitations that should be considered during the procedure and interpretation of the test results. This article reviews the clinical applications of conventional esophageal and anorectal manometries in children by describing a technique for performing the test. This review will develop the uniformity required for the methods of performance, the parameters for measurement, and interpretation of test results that could be applied in pediatric clinical practice.

• Childhood Kidney Diseases
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• v.19 no.1
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• pp.14-22
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• 2015

Renal scintigraphic methods, which are physiologic and minimally invasive, have played important role in the management of various renal diseases in children, providing sensitive tool for early detection of disease even before structural changes become prominent and giving valuable functional and anatomical information to aid medical decision makings in the treatment and follow-up of patients. This review article focuses on the concept and advantages of renal scintigraphy in pediatric patients with various urologic diseases.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.10 no.1
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• pp.1-10
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• 2007

Outcome of liver transplantation for children with end liver disease has been improved markedly during last two decades. Improvement of immunosuppressive agent and its strategy to use in children, development of innovative surgical technique, and better understanding of the course of liver transplantation attributed to better outcome of pediatric liver transplantation. Therefore this review article will focus on the problems which can occur during pre- and post-transplantation period, current strategy to use immunosuppressive agent for the better understanding of pediatrician who is not involved in transplantation but takes care of the children pre- and postoperatively.