مقالة علمية
Longitudinal Auxological recovery in a cohort of children with Hyperinsulinaemic Hypoglycaemia

Worth, Chris.


 

Longitudinal Auxological recovery in a cohort of children with Hyperinsulinaemic Hypoglycaemia

Worth, Chris.

Background Hypoglycaemia due to hyperinsulinism (HI) is the commonest cause of severe, recurrent hypoglycaemia in childhood. Cohort outcomes of HI remain to be described and whilst previous follow up studies have focused on neurodevelopmental outcomes, there is no information available on feeding and auxology. Aim We aimed to describe HI outcomes for auxology, medications, feeding and neurodevelopmental in a cohort up to age 5 years. Method We reviewed medical records for all patients with confirmed HI over a three-year period in a single centre to derive a longitudinal dataset. Results Seventy patients were recruited to the study. Mean weight at birth was − 1.0 standard deviation scores (SDS) for age and sex, while mean height at 3 months was − 1.5 SDS. Both weight and height trended to the population median over the follow up period. Feeding difficulties were noted in 17% of patients at 3 months and this reduced to 3% by 5 years. At age 5 years, 11 patients (15%) had neurodevelopmental delay and of these only one was severe. Resolution of disease was predicted by lower maximum early diazoxide dose (p = 0.007) and being born SGA (p = 0.009). Conclusion In a three-year cohort of HI patients followed up for 5 years, in spite of feeding difficulties and carbohydrate loading in early life, auxology parameters are normal in follow up. A lower than expected rate of neurodevelopmental delay could be attributed to prompt early treatment.

Background Hypoglycaemia due to hyperinsulinism (HI) is the commonest cause of severe, recurrent hypoglycaemia in childhood. Cohort outcomes of HI remain to be described and whilst previous follow up studies have focused on neurodevelopmental outcomes, there is no information available on feeding a...

مادة فرعية

المؤلف : Worth, Chris.

مؤلف مشارك : Al Hashmi, Laila
Yau, Daphne
Salomon Estebanez, Maria
Ruiz, Diego Perez
Hall, Caroline

بيانات النشر : Orphanet Journal of Rare Diseases، 24 يونيو 2020مـ.

التصنيف الموضوعي : العلوم التطبيقية|العلوم الطبية .

المواضيع : Children - diseases .

Hypoglycaemia .

رقم الطبعة : 162

المصدر : Europe PMC : .

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