ESPE Abstracts (2022) 95 P1-246

ESPE2022 Poster Category 1 Diabetes and Insulin (86 abstracts)

Developmental surveillance in children with Congenital Hyperinsulinism (CHI) - Personalising care

Carey Eldred , Clare Gilbert , Kate Morgan & Antonia Dastamani

Great Ormond Street Hospital, London, United Kingdom

Background: CHI is a rare condition which can be related to neurological damage due to hypoglycaemic brain injury. Long hospital admissions maybe necessary which can impact babies’ experience of posture and movement, translating to a motor delay. A physiotherapy developmental assessment is a vital part of a multidisciplinary team approach to personalising care. Assessment during admission is essential to provide appropriate developmental support, particularly as repeated hypoglycaemic episodes can lead to lethargy, further impacting opportunities to explore and engage with their environment.

Aim: To assess initial physiotherapy developmental status and progress of CHI infants.

Methodology: Retrospective data collection of infants (0-12 months old) diagnosed with CHI between April 2020 to April 2022 and admitted in our tertiary CHI centre, excluding those with syndromes. All patients assessed for: 1) neurological impairment using standardised assessment Hammersmith Neonatal Neurological Examination (HNNE) and 2) motor development by using the Alberta Motor Infant Score. Both methods used as it can be difficult to get discerning data from a motor assessment tool for newborns, as postural development is the main parameter of motor skills, which may not offer sufficient information. The HNNE looks at quality and quantity of spontaneous movement which can give us some further insight into motor skills.

Results: Eleven infants with genetic defect in one of the known genes (ABCC8, KCNJ11, HNF4A) implemented in CHI included in our study. Four patients have longitudinal assessment data. All had uncomplicated deliveries, one (KCNJ11) at 34 weeks and 3 (ABCC8) at 37 weeks+, with mean birth weight 3.94kg, presented day one of life. Mean length of initial hospital admission was 115.5 days. All the infants had initial newborn developmental assessment and follow-up assessment completed between ages of 6-12 months. At newborn assessment, the data showed postural developmental within age-appropriate norms, but with a reduction in quality and quantity of movement. In all cases, follow up assessment showed a drop in percentiles, some with delay well below normative. In these patients we use an equivalent 50th percentile age match to describe extent of delay.

Conclusion: These findings prompted further questions about the incidence of developmental delay in CHI and potential influencing factors. Results highlight the need for ongoing support of infants to ensure their developmental needs are met. Personalised care with developmental surveillance of CHI patients could improve long term outcomes. Prospective studies data are essential to formally assess the developmental needs of CHI patients.

Volume 95

60th Annual ESPE (ESPE 2022)

Rome, Italy
15 Sep 2022 - 17 Sep 2022

European Society for Paediatric Endocrinology 

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