Growth, tolerance and safety outcomes with use of an extensively hydrolyzed casein-based formula in infants with cow’s milk protein allergy

ObjectiveTo evaluate growth, tolerance and safety outcomes with use of an extensively hydrolyzed casein-based formula (eHCF) in infants with cow’s milk protein allergy (CMPA).MethodsA total of 226 infants (mean ± SD age: 106.5 ± 39.5 days, 52.7% were girls) with CMPA who received eHCF comprising at least half of the daily dietary intake were included. Data on anthropometrics [weight for age (WFA), length for age (LFA) and weight for length (WFL) z-scores] were recorded at baseline (visit 1), while data on infant feeding and stool records, anthropometrics and Infant Feeding and Stool Patterns and Formula Satisfaction Questionnaires were recorded at visit 2 (on Days 15 ± 5) and visit 3 (on Days 30 ± 5).ResultsFrom baseline to visit 2 and visit 3, WFA z-scores (from −0.60 ± 1.13 to −0.54 ± 1.09 at visit 2, and to −0.44 ± 1.05 at visit 3, p < 0.001) and WFL z-scores (from −0.80 ± 1.30 to −0.71 ± 1.22 at visit 2, and to −0.64 ± 1.13 at visit 3, p = 0.002) were significantly increased. At least half of infants never experienced irritability or feeding refusal (55.7%) and spit-up after feeding (50.2%). The majority of mothers were satisfied with the study formula (93.2%), and wished to continue using it (92.2%).ConclusionsIn conclusion, eHCF was well-accepted and tolerated by an intended use population of infants  ≤ 6 months of age with CMPA and enabled adequate volume consumption and improved growth indices within 30 days of utilization alongside a favorable gastrointestinal tolerance and a high level of parental satisfaction

The Frequency of Lysosomal Acid Lipase Deficiency in Children With Unexplained Liver Disease

WOS: 000461077600024PubMed: 30540705Objectives: Evidence suggests that lysosomal acid lipase deficiency (LAL-D) is often underdiagnosed because symptoms may be nonspecific. We aimed to investigate the prevalence of LAL-D in children with unexplained liver disease and to identify demographic and clinical features with a prospective, multicenter, cross-sectional study. Methods: Patients (aged 3 months-18 years) who had unexplained transaminase elevation, unexplained hepatomegaly or hepatosplenomegaly, obesity-unrelated liver steatosis, biopsy-proven cryptogenic fibrosis and cirrhosis, or liver transplantation for cryptogenic cirrhosis were enrolled. A Web-based electronic data collection system was used. LAL activity (nmol/punch/h) was measured using the dried blood spot method and classified as LAL-D(0.37). Asecond dried blood spot sample was obtained from patients with intermediate LAL activity for confirmation of the result. Results: A total of 810 children (median age 5.6 years) from 795 families were enrolled. The reasons for enrollment were unexplained transaminase elevation (62%), unexplained organomegaly (45%), obesity-unrelated liver steatosis (26%), cryptogenic fibrosis and cirrhosis (6%), and liver transplantation for cryptogenic cirrhosis (<1%). LAL activity was normal in 634 (78%) and intermediate in 174 (21%) patients. LAL-D was identified in 2 siblings aged 15 and 6 years born to unrelated parents. Dyslipidemia, liver steatosis, and mild increase in aminotransferases were common features in these patients. Moreover, the 15-year-old patient showed growth failure and microvesicular steatosis, portal inflammation, and bridging fibrosis in the liver biopsy. Based on 795 families, 2 siblings in the same family were identified as LAL-D cases, making the prevalence of LAL-D in this study population, 0.1% (0.125%-0.606%). In the repeated measurement (76/174), LAL activity remained at the intermediate level in 38 patients. Conclusions: Overall, the frequency of LAL-D patients in this study (0.1%) suggests that LAL-D seems to be rare even in the selected high-risk population.Alexion Pharmaceuticals Inc.Fukuda Foundation for Medical TechnologyKayamori FoundationKayamori Foundation of Informational Science AdvancementSENSHIN Medical Research FoundationSasagawa Scientific Research GrantThis investigator sponsored research was funded by Alexion Pharmaceuticals Inc

Cytomegalovirus hepatitis in 49 pediatric patients with normal immunity

Background/aim: Cytomegalovirus (CMV) hepatitis is generally asymptomatic or rarely can lead to severe complications in immunocompetent hosts. This study aims to evaluate CMV hepatitis in immunocompetent young children, which is discussed relatively rarely in the literature. Materials and methods: A retrospective review of 49 pediatric patients with CMV hepatitis from January 2005 to December 2010 was performed. Results: The median age of the patients was 5.81 +/- 6.49 months and 57.1\% were female. Complaints were prolonged jaundice, vomiting, diarrhea, and abdominal distension. Seventeen patients (34.6\%) had congenital or probable congenital CMV infection, while 32/49 (65.3\%) had perinatal CMV infection. CMV hepatitis was accompanied by other system findings in 22 patients (44.9\%), and only liver involvement was present in 27/49 (55.1\%). Alanine aminotransferase and aspartate aminotransferase were elevated together in all patients. Cholestatic hepatitis was present in 13 patients (26.5\%). Four patients (8.16\%) were treated with ganciclovir. Complete improvement of hepatitis occurred in 48/49 (97.95\%). The recovery time of liver function tests was 7-180 days (mean: 53.92 +/- 40.8). Conclusion: CMV hepatitis is usually mild and has a good outcome in immunocompetent individuals. However, cases should be carefully evaluated due to the important role of CMV in the etiology of infantile and neonatal hepatitis

The Frequency of Lysosomal Acid Lipase Deficiency in Children With Unexplained Liver Disease

Objectives: Evidence suggests that lysosomal acid lipase deficiency (LAL-D) is often underdiagnosed because symptoms may be nonspecific. We aimed to investigate the prevalence of LAL-D in children with unexplained liver disease and to identify demographic and clinical features with a prospective, multicenter, cross-sectional study