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Background: The Identification and Management of Feeding Difficulties (IMFeD™) tool was introduced in 2011 for management of children with feeding difficulties and has been used in various countries around the world. This study aimed to assess its acceptance and effectiveness in clinical practice in Taiwan. Methods: This was a prospective, observational, multicenter study where subjects were followed over 3 months. Outpatients identified as picky eaters, having poor appetite, poor weight gain, or weight loss were enrolled. The acceptance and effectiveness of IMFeD tool were evaluated by caregivers and pediatricians. Results: Among 423 children who completed the study (235 boys, 188 girls; 78.8% aged

Wilson's disease (WD) is an autosomal recessive defect of cellular copper export. Early diagnosis in children is difficult due to its obscure clinical presentations. The efficacy of zinc salts is well documented, although there are limited data concerning zinc use in pediatric patients with WD. Methods: We performed a retrospective analysis of clinical features, laboratory results and treatment responses in children with WD diagnosed at Taichung Veterans General Hospital between 1996 and 2008. Diagnosis was established by low serum ceruloplasmin, high 24-hour urinary copper excretion, presence of Kayser-Fleischer rings, and mutation analysis. Results: Eleven children were included in this study. The main initial presentations were impaired liver function tests (6/11) and hemolytic anemia (2/11). Gene studies in seven children showed six different mutations (G934D, R778Q, C490X, 304insC, IVS4-1 G > C, P992I) and one possible novel mutation (L1181P). All patients had improved liver function tests and hemoglobin levels after treatment with D-penicillamine, trientine and zinc supplement therapy. During a mean period of 3.4 ± 2.1 years with zinc therapy, six patients had serum zinc levels above the normal limit, and seven patients had serum copper levels below the normal range. Conclusion: Serum ceruloplasmin and 24-hour urinary copper examinations could be used to rule out WD in children with chronic hepatitis and hemolytic anemia. Gene analysis is helpful for prompt diagnosis of asymptomatic siblings and patients with atypical features. Zinc treatment is generally safe in pediatric patients with WD. However, its adverse effects should be monitored.

Hepatic osteodystrophy is a common complication in patients with chronic liver disease, however, bone mineral status in patients with biliary atresia has rarely been investigated. Methods: Twenty-nine children with biliary atresia were enrolled in our study and their demographic data, bone mineral density (BMD) of lumbar spine and bilateral femoral neck, and biochemical parameters were measured and analyzed. Results: The majority of our patients had osteopenia or osteoporosis over at least one part of the skeleton although none had jaundice. Instead of T helper 1 cell cytokine, interleukin (IL)-4 had a significant negative correlation with BMD of the right femoral neck (β = −0.251, p = 0.027) and left femoral neck (β = −0.299, p = 0.012) independently by multiple linear regression analysis. Conclusion: We conclude that chronic inflammation with increased expression of IL-4 may be an important factor for compromised bone health in patients with biliary atresia.

Background: The Identification and Management of Feeding Difficulties (IMFeD™) tool was introduced in 2011 for management of children with feeding difficulties and has been used in various countries around the world. This study aimed to assess its acceptance and effectiveness in clinical practice in Taiwan., Methods: This was a prospective, observational, multicenter study where subjects were followed over 3 months. Outpatients identified as picky eaters, having poor appetite, poor weight gain, or weight loss were enrolled. The acceptance and effectiveness of IMFeD tool were evaluated by caregivers and pediatricians., Results: Among 423 children who completed the study (235 boys, 188 girls; 78.8% aged <5 years, 21.3% aged 6-10 years), the most common feeding difficulties were poor appetite in a fundamentally vigorous child (85.3%) and highly selective intake (70.2%). Mean percentiles for height and weight were 34.6 ± 25.3 and 20.3 ± 21.3 at enrolment, and increased by 5.4 and 5.9 (p < 0.0001) at the last visit, respectively. Most caregivers (86.5%) thought that the IMFeD tool helped them to understand and better identify picky eating behaviors. Pediatricians reported that the IMFeD tool was effective in 99.3% of patients for managing picky eaters in clinical practice., Conclusions: The IMFeD tool was effective in addressing differences in needs according to type of feeding difficulty by providing a systematic approach to manage feeding difficulty in Taiwanese children. It is applicable to the clinical practice and well-accepted by pediatricians and caregivers., (Copyright © 2018. Published by Elsevier B.V.)

Background: Hepatic osteodystrophy is a common complication in patients with chronic liver disease, however, bone mineral status in patients with biliary atresia has rarely been investigated., Methods: Twenty-nine children with biliary atresia were enrolled in our study and their demographic data, bone mineral density (BMD) of lumbar spine and bilateral femoral neck, and biochemical parameters were measured and analyzed., Results: The majority of our patients had osteopenia or osteoporosis over at least one part of the skeleton although none had jaundice. Instead of T helper 1 cell cytokine, interleukin (IL)-4 had a significant negative correlation with BMD of the right femoral neck (β = -0.251, p = 0.027) and left femoral neck (β = -0.299, p = 0.012) independently by multiple linear regression analysis., Conclusion: We conclude that chronic inflammation with increased expression of IL-4 may be an important factor for compromised bone health in patients with biliary atresia., (Copyright © 2016. Published by Elsevier B.V.)

Background: Wilson's disease (WD) is an autosomal recessive defect of cellular copper export. Early diagnosis in children is difficult due to its obscure clinical presentations. The efficacy of zinc salts is well documented, although there are limited data concerning zinc use in pediatric patients with WD., Methods: We performed a retrospective analysis of clinical features, laboratory results and treatment responses in children with WD diagnosed at Taichung Veterans General Hospital between 1996 and 2008. Diagnosis was established by low serum ceruloplasmin, high 24-hour urinary copper excretion, presence of Kayser-Fleischer rings, and mutation analysis., Results: Eleven children were included in this study. The main initial presentations were impaired liver function tests (6/11) and hemolytic anemia (2/11). Gene studies in seven children showed six different mutations (G934D, R778Q, C490X, 304insC, IVS4-1 G > C, P992I) and one possible novel mutation (L1181P). All patients had improved liver function tests and hemoglobin levels after treatment with D-penicillamine, trientine and zinc supplement therapy. During a mean period of 3.4 +/- 2.1 years with zinc therapy, six patients had serum zinc levels above the normal limit, and seven patients had serum copper levels below the normal range., Conclusion: Serum ceruloplasmin and 24-hour urinary copper examinations could be used to rule out WD in children with chronic hepatitis and hemolytic anemia. Gene analysis is helpful for prompt diagnosis of asymptomatic siblings and patients with atypical features. Zinc treatment is generally safe in pediatric patients with WD. However, its adverse effects should be monitored., (Copyright 2010 Taiwan Pediatric Association. Published by Elsevier B.V. All rights reserved.)