BSPED2010 Poster Presentations (1) (59 abstracts)
Feeding problems and their associated predictive factors in congenital hyperinsulinism of infancy (CHI)
A Subbarayan , L Forsythe , L Rigby , J Marks , S Ehtisham , L Patel , R Amin , M Didi , J Blair , S Ellard , P Clayton , I Banerjee & M Skae
1Royal Manchester Children’s Hospital, Manchester, UK; 2Alderhey Children’s Hospital, Liverpool, UK; 3Peninsula Medical School, Exeter, UK; 4University of Manchester, Manchester, UK.
Congenital Hyperinsulinism (CHI), a common cause of persistent hypoglycaemia in infancy can be associated with feeding problems (FP). The extent of FP in CHI is not known. The commonest genetic cause of CHI is mutations in ATP-sensitive potassium (K+ATP) channel genes (ABCC8 and KCNJ11).
Aims: To define FP in CHI patients presenting to a regional centre, in relation to medication and K+ATP mutations.
Patients and methods: We retrospectively analysed data in 35 genotyped patients with persistent CHI, with National Research Ethics Service (NRES) approval and consent. Children with FP were formally assessed by a Speech and Language Therapy (SALT) service (recurrent vomiting or inability to tolerate prescribed feed volumes) and classified into 4 categories (poor suck, swallowing in-coordination, vomiting and food refusal). Children with FP were compared with children without FP with regard to mutation analysis, medication, presence of diffuse CHI and birth weight.
Results: Seventeen (48%) CHI patients were identified with FP. Positive K+ATP mutations (ABCC8=17, KCNJ11=4) were identified in 21 cases. When compared to those without FP, patients with FP had significantly higher K+ATP channel mutations (Chi-square test; P<0.001), genetically proven diffuse disease (Chi-square test; P<0.001) and birthweights (Chi-square test; P<0.05). Severe FP (FP ≥2 categories) were associated with homozygous/compound heterozygous mutations in the ABCC8 gene (Chi-square test; P<0.001), diffuse disease (Chi-square test; P<0.002) and Diazoxide unresponsiveness (Chi-square test; P<0.001). Fourteen (82%) patients required gastrostomy feeding and at 6 months follow-up, 12 (71%) continued to have 1 or more FP.
Conclusions: Feeding problems occur in approximately half of CHI patients. Patients who are K+ATP channel mutation positive, Diazoxide unresponsive or have diffuse disease are more likely to have severe FP. We recommend early feeding assessment and intervention in children with CHI as these problems persist in the first year of life.
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Endocrine Abstracts
ISSN 1470-3947 (print) | ISSN 1479-6848 (online)
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