Feeding Problems Are Persistent in Children with Severe Congenital Hyperinsulinism

Abstract

BackgroundCongenital hyperinsulinism (CHI) is a rare but severe disorder of hypoglycemia in children, often complicated by brain injury. In CHI, the long-term prevention of hypoglycemia is dependent on reliable enteral intake of glucose. However, feeding problems (FPs) often impede oral glucose delivery, thereby complicating the management of hypoglycemia. FPs have not been systematically characterized in follow-up in a cohort with CHI.AimsWe aimed to determine the prevalence, types, and persistence of FPs in a cohort of children with CHI and investigate potential causal factors.MethodsFPs were defined as difficulty with sucking, swallowing, vomiting, and food refusal (or a combination) in an observational study in 83 children in a specialized CHI treatment center. The prevalence of FPs at diagnosis, 6, and 12 months after diagnosis were noted. Genetic mutation status and markers of severity of CHI were tested for association with FPs.ResultsA third of children with CHI had FPs (n = 28), of whom 93% required antireflux medication and 75% required nasogastric and gastrostomy tube feeding. Sucking and swallowing problems were present at diagnosis but absent later. Vomiting was present in 54% at 6 months, while food refusal was present in 68% at 6 months and 52% at 12 months. The age at commencing and stopping nasogastric tube feeding did not correlate with FPs frequency at 6 and 12 months. Children with FPs had severe hypoglycemia at diagnosis and required glucagon infusion more often [odds ratio (OR) (95% confidence intervals) (95% CI) 28.13 (2.6–300.1), p = 0.006] to normalize glucose levels. FPs were more frequent in those with diffuse CHI undergoing subtotal pancreatectomy [n (%) = 10 (35%) vs. 0 (0%), p < 0.001], in contrast to those with spontaneous resolution [6 (22%) vs. 32 (58%), p = 0.002]. Those undergoing focal lesionectomy also had reduced FPs at 6 months after diagnosis [OR (95% CI) 0.01 (0.0–0.2), R2 = 0.42, p = 0.004]. These observations suggest that persistence of hyperinsulinism was associated with FPs.ConclusionFPs occur in a significant proportion of children with CHI. Severe hyperinsulinism, rather than nasogastric tube feeding or medications, is the main factor associated with FPs.

Therapies and outcomes of congenital hyperinsulinism‐induced hypoglycaemia

“…Feeding difficulties occur in a significant proportion of children (33-45%) [59] and are associated with more severe forms of CHI. Feeding difficulties occur in a significant proportion of children (33-45%) [59] and are associated with more severe forms of CHI.…”

“…Feeding difficulties, such as sucking and swallowing difficulties, persistent vomiting, gastro-oesophageal reflux and food refusal, represent another burden for families with CHI. Feeding difficulties occur in a significant proportion of children (33-45%) [59] and are associated with more severe forms of CHI. The cause is not clear, but likely to be multifactorial.…”

Complexities in the medical management of hypoglycaemia due to congenital hyperinsulinism

“…Further, oral feeds partly increase insulin secretion, possibly through sensitivity to milk protein triggering insulin, to worsen the risk of hypoglycaemia. This is further complicated by the high rate of feeding problems in patients with CHI . The exact causes for feeding problems in patients with CHI are not clear but are likely to be multifactorial, particularly at a time when children are vulnerable to the stress of illness, painful stimuli, suppression of appetite by high‐concentration dextrose and medication.…”

Delayed Resolution of Feeding Problems in Patients With Congenital Hyperinsulinism

“…Our group has previously found that severity of CHI is a strong predictor for the prevalence of feeding problems (7). Feeding problems can persist beyond resolution of hypoglycaemia in patients with diffuse forms of CHI (6,7) with variations between those undergoing surgery and those treated medically (4). The long-term outcome of feeding problems in CHI has not been characterized.…”

“…We did not aim to address predictive factors for development of feeding problems as our group has addressed this previously (7).…”