First-year growth in children with Noonan syndrome: Associated with feeding problems?

Autor: Nel Roeleveld, Ineke van der Burgt, Cees Noordam, Jos M. T. Draaisma, Ellen A. Croonen
Rok vydání: 2018
Předmět:
Male
medicine.medical_specialty
Pediatrics
Time Factors
Genotype
Birth weight
030209 endocrinology & metabolism
First year of life
Healthcare improvement science Radboud Institute for Health Sciences [Radboudumc 18]
03 medical and health sciences
0302 clinical medicine
Surveys and Questionnaires
030225 pediatrics
Odds Ratio
Genetics
medicine
Humans
Longitudinal Studies
Growth Charts
Child
Alleles
Genetics (clinical)
Retrospective Studies
Neurodevelopmental disorders Donders Center for Medical Neuroscience [Radboudumc 7]
business.industry
Noonan Syndrome
Infant
Vascular damage Radboud Institute for Molecular Life Sciences [Radboudumc 16]
Gestational age
Feeding Behavior
medicine.disease
Cardiac surgery
Reconstructive and regenerative medicine Radboud Institute for Health Sciences [Radboudumc 10]
PTPN11
Phenotype
lnfectious Diseases and Global Health Radboud Institute for Health Sciences [Radboudumc 4]
Feeding problems
Child
Preschool

Mutation
Noonan syndrome
Female
medicine.symptom
business
Weight gain
Zdroj: American Journal of Medical Genetics. Part A, 176, 4, pp. 951-958
American Journal of Medical Genetics. Part A, 176, 951-958
ISSN: 1552-4825
DOI: 10.1002/ajmg.a.38649
Popis: Item does not contain fulltext Children with Noonan syndrome show rapid decline of growth in the first year of life and feeding problems are present in over 50%. The aim of this study was to explore whether growth decelerates because of feeding problems or other Noonan syndrome-related factors. We performed a retrospective, longitudinal cohort study of clinically and genetically diagnosed subjects with Noonan syndrome (n = 143). Questionnaires about the phenotypic-genotypic profile and reported feeding problems were sent to eligible subjects. Data on first-year growth was obtained from growth charts. Ninety-one participants were excluded because of different criteria. A total of 52 subjects with Noonan syndrome were included. The largest decline in weight and length standard deviation score (SDS) occurred in the first 2.5 months after birth (-1.93 and -1.15, respectively), with feeding problems causing a decline of 0.57 SDS in the remaining months. At 1 year, children with feeding problems were on average 290 g lighter and 0.8 cm shorter than children without feeding problems. Weight gain was also negatively influenced by having a PTPN11 mutation (n = 39) and a higher gestational age, whereas children of parents with Noonan syndrome and with a higher birth weight gained more weight. Growth in length was reduced by having cardiac surgery and a higher gestational age, but positively influenced by birth length and maternal height. Growth in children with Noonan syndrome is impaired right after birth and only partially associated with feeding problems. In addition, several specific Noonan syndrome-related factors seem to influence growth in the first year.
Databáze: OpenAIRE