Foregut Dysmotility Complicating Persistent Hyperinsulinaemic Hypoglycaemia of Infancy

Cade, Alan; Abel, Gill; Stringer, Mark D.; Milla, Peter J.; Puntis, John

doi:10.1097/00005176-199809000-00018

Cited by 3 publications

(1 citation statement)

References 17 publications

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“…They are often intolerant of oral feeding and may require parenteral nutrition to ensure adequate carbohydrate intake to prevent hypoglycemia. Feeding problems (FPs) are common in many seriously ill children, but are particularly frequent and complex in children with CHI, a feature that has been recognized in case reports ( 4 , 5 ), and in a recent retrospective observational study ( 6 ). However, the prevalence, types, extent, and natural history of FPs in follow-up assessments have not been described in children with CHI.…”

Section: Introductionmentioning

confidence: 99%

Feeding Problems Are Persistent in Children with Severe Congenital Hyperinsulinism

et al. 2016

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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.

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Section: Introductionmentioning

confidence: 99%

Feeding Problems Are Persistent in Children with Severe Congenital Hyperinsulinism

et al. 2016

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Hypoglycemia: Assessment and Management

Cotterill

Cowley

Greer

2009

Brook's Clinical Pediatric Endocrinology

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When It Is More than Transient Neonatal Hypoglycemia: Hyperinsulinemia— A Case Study Challenge

Shirland

2001

Neonatal Network

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Persistent uncontrolled neonatal hypoglycemia may cause irreversible brain damage. Hyperinsulinemia is a rare cause of persistent hypoglycemia, diagnosed by excluding other etiologies. Inappropriately high fasting serum insulin levels with concurrent hypoglycemia confirm the diagnosis. Initial interventions for hyperinsulinemia are conservative. The first line of therapy is administration of adequate intravenous (i.v.) glucose to maintain serum or whole blood glucose levels at or greater than 40 mg/dl. When enteral feedings are tolerated, schedules and caloric concentration are adjusted. Pharmacologic therapy is added to facilitate weaning from i.v. glucose. The drug of first choice is diazoxide. Octreotide is added if diazoxide therapy fails. Partial or complete pancreatectomy is the final treatment option. Nursing care for infants with hyperinsulinemia must also focus on the support and education of families. Family education must be individualized and should cover feeding regimes, administration of medication, proper use of equipment, and care during illness.

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Foregut Dysmotility Complicating Persistent Hyperinsulinaemic Hypoglycaemia of Infancy

Cited by 3 publications

References 17 publications

Feeding Problems Are Persistent in Children with Severe Congenital Hyperinsulinism

Feeding Problems Are Persistent in Children with Severe Congenital Hyperinsulinism

Hypoglycemia: Assessment and Management

When It Is More than Transient Neonatal Hypoglycemia: Hyperinsulinemia— A Case Study Challenge

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