AUTHOR=Banerjee Indraneel , Forsythe Lynette , Skae Mars , Avatapalle Hima Bindu , Rigby Lindsey , Bowden Louise E. , Craigie Ross , Padidela Raja , Ehtisham Sarah , Patel Leena , Cosgrove Karen E. , Dunne Mark J. , Clayton Peter E. 

TITLE=Feeding Problems Are Persistent in Children with Severe Congenital Hyperinsulinism

JOURNAL=Frontiers in Endocrinology

VOLUME=7

YEAR=2016

URL=https://www.frontiersin.org/journals/endocrinology/articles/10.3389/fendo.2016.00008

DOI=10.3389/fendo.2016.00008

ISSN=1664-2392

ABSTRACT=<sec id="ST1"><title>Background</title><p>Congenital 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.</p></sec><sec id="ST2"><title>Aims</title><p>We aimed to determine the prevalence, types, and persistence of FPs in a cohort of children with CHI and investigate potential causal factors.</p></sec><sec id="ST3"><title>Methods</title><p>FPs 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.</p></sec><sec id="ST4"><title>Results</title><p>A third of children with CHI had FPs (<italic>n</italic> = 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), <italic>p</italic> = 0.006] to normalize glucose levels. FPs were more frequent in those with diffuse CHI undergoing subtotal pancreatectomy [<italic>n</italic> (%) = 10 (35%) vs. 0 (0%), <italic>p</italic> &lt; 0.001], in contrast to those with spontaneous resolution [6 (22%) vs. 32 (58%), <italic>p</italic> = 0.002]. Those undergoing focal lesionectomy also had reduced FPs at 6 months after diagnosis [OR (95% CI) 0.01 (0.0–0.2), <italic>R</italic><sup>2</sup> = 0.42, <italic>p</italic> = 0.004]. These observations suggest that persistence of hyperinsulinism was associated with FPs.</p></sec><sec id="ST5"><title>Conclusion</title><p>FPs 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.</p></sec>