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Dan-Nielsen S.,Hvidovre University Hospital | Wewer V.,Hvidovre University Hospital | Paerregaard A.,Hvidovre University Hospital | Hansen L.F.,Hvidovre University Hospital | And 3 more authors.
Journal of Pediatric Gastroenterology and Nutrition | Year: 2014

OBJECTIVES:: The aim of the study was to evaluate clinical response, use of colectomy, and adverse events related to infliximab (IFX) treatment in acute and chronic active ulcerative colitis (UC) in children. METHODS:: Children from 3 centers, who had received IFX for UC, were identified, and patient charts were reviewed retrospectively. Data concerning symptoms, biochemistry, concomitant medical treatment, colectomy, and adverse events were registered. RESULTS:: A total of 45 patients with UC (median age at diagnosis 12 years, interquartile range 10-14) were included, and studied for a median of 15 months (interquartile range 4.5-29) after first IFX infusion. The cumulative 1- and 2-year risks of colectomy were 21% and 26%, respectively. The cumulative 1- and 2-year risks of receiving a new course of systemic corticosteroids were 32% and 48%, respectively. Twenty-one patients (46%) experienced adverse events. Most common were mild infusion reactions, but 3 (7%) had serious adverse events. CONCLUSIONS:: IFX was efficient in preventing colectomy in children with UC. The risk of receiving systemic corticosteroids was lower than that reported in other studies. Most adverse events were mild to moderate and self-limiting. © 2014 by ESPGHAN and NASPGHAN. Source


Ravn K.,Copenhagen University | Neland M.,Hans Christian Andersen Childrens Hospital | Wibrand F.,Copenhagen University | Duno M.,Copenhagen University | Ostergaard E.,Copenhagen University
American Journal of Medical Genetics, Part A | Year: 2016

Recently, two research groups reported that mutations in RMND1 were associated with encephalopathy, elevated lactate, hypotonia, and in some patients seizures or myoclonia in individuals from two consanguineous families. A combined respiratory chain deficiency and a defect in mitochondrial protein translation was found. In this study, we report two siblings who are compound heterozygous for the mutations, c.713A>G and c.1003delG, in RMND1. Respiratory chain enzymatic analysis and BN-PAGE showed a combined OXPHOS deficiency. Western blot analysis indicated normal levels of RMND1, but the assembly of the RMND1 homopolymeric complex was highly impaired. The two siblings had a markedly milder phenotype and longer survival compared to previously reported patients. In addition, they had renal failure and hearing impairment. These two newly described patients contribute to delineation of the clinical spectrum associated with RMND1 aberrations. © 2015 Wiley Periodicals, Inc. Source


Wittenhagen P.,University of Southern Denmark | Thiesson H.C.,University of Southern Denmark | Baudier F.,University of Southern Denmark | Pedersen E.B.,University of Southern Denmark | Neland M.,Hans Christian Andersen Childrens Hospital
Pediatric Transplantation | Year: 2014

Increased focus on the potential negative side effects of steroid usage in pediatric transplantation has led to steroid minimization or steroid-free transplantation. In this study, we report results after complete steroid avoidance in renal transplantation in the period 1994-2009. We evaluate the effects of complete steroid avoidance on allograft function, BMI, and linear growth. The majority of transplanted children were induced with antithymocyte globulin and immunosuppressed with a calcineurin inhibitor and mycophenolate mofetil. Steroids were given only when rejection occurred or due to comorbidities. Anthropometric data were collected from 65 transplantations in 60 children. Patient survival was 93%; graft survival was 81% after five yr (N = 42) and 63% after 10 yr (N = 16). Acute rejection within the first year of transplantation was 9%. The distribution of the children's BMI before transplantation was normal; the mean BMI-SDS was 0.21 before transplantation, and this value remained stable during the next five yr. Post-transplantation the children demonstrated significant improved growth as the mean height-SDS increased significantly from -1.7 to -1.1. Catch-up growth was most pronounced in the youngest (< six yr). Steroid-free immunosuppression in pediatric renal transplantation is safe and protects against steroid-induced obesity and short stature. © 2013 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd. Source


Koletzko S.,Ludwig Maximilians University of Munich | Niggemann B.,Red Cross | Arato A.,Semmelweis University | Dias J.A.,Hospital S. Joao | And 8 more authors.
Journal of Pediatric Gastroenterology and Nutrition | Year: 2012

OBJECTIVES:: This guideline provides recommendations for the diagnosis and management of suspected cow's-milk protein allergy (CMPA) in Europe. It presents a practical approach with a diagnostic algorithm and is based on recently published evidence-based guidelines on CMPA. DIAGNOSIS:: If CMPA is suspected by history and examination, then strict allergen avoidance is initiated. In certain circumstances (eg, a clear history of immediate symptoms, a life-threatening reaction with a positive test for CMP-specific IgE), the diagnosis can be made without a milk challenge. In all other circumstances, a controlled oral food challenge (open or blind) under medical supervision is required to confirm or exclude the diagnosis of CMPA. TREATMENT:: In breast-fed infants, the mother should start a strict CMP-free diet. Non-breast-fed infants with confirmed CMPA should receive an extensively hydrolyzed protein-based formula with proven efficacy in appropriate clinical trials; amino acids-based formulae are reserved for certain situations. Soy protein formula, if tolerated, is an option beyond 6 months of age. Nutritional counseling and regular monitoring of growth are mandatory in all age groups requiring CMP exclusion. REEVALUATION:: Patients should be reevaluated every 6 to 12 months to assess whether they have developed tolerance to CMP. This is achieved in >75% by 3 years of age and >90% by 6 years of age. Inappropriate or overly long dietary eliminations should be avoided. Such restrictions may impair the quality of life of both child and family, induce improper growth, and incur unnecessary health care costs. Copyright © 2012 by European Society. Source


Poorisrisak P.,Naestved Hospital | Schroeder A.M.,Medicine Information Center | Greisen G.,Copenhagen University | Zachariassen G.,Hans Christian Andersen Childrens Hospital
BMJ Case Reports | Year: 2014

Preterm twin sisters (monozygotic) were born at gestational age 27 weeks and 5 days with birth weights of 935 and 735 g. They were admitted to our neonatal intensive care unit for a period of 1 month. Their parents were Jehovah's Witnesses and refused blood transfusion for their preterm daughters. Subcutaneous erythropoietin and intravenous iron were given as a prophylactic to avoid anaemia. Copyright 2014 BMJ Publishing Group. All rights reserved. Source

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