Effects of Hypoglycemia on Developmental Outcome in Children With Congenital Hyperinsulinism

Steinkrauss, Linda; Lipman, Terri H.; Hendell, Christine D.; Gerdes, Marsha; Thornton, Paul; Stanley, Charles A.

doi:10.1016/j.pedn.2004.12.009

Cited by 83 publications

(66 citation statements)

References 23 publications

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“…The outcome of our patients is comparable with that of patients with early onset of the disease, who underwent pancreatectomy, described recently by three groups from specialized centers (18,19,21). Menni et al (18) described 90 CH patients treated between 1982 and 1998 and followed for at least 10 years.…”

Section: Discussionsupporting

confidence: 79%

“…There was no description of differences between medical and surgical patients, but they did note that among the patients with psychomotor or mental retardation, 51% had undergone surgery. Steinkrauss et al (21) described 68 CH patients treated between 1980 and 2000. Twenty-six patients were treated medically with diazoxide, 35 underwent surgery and 7 patients had transient hyperinsulinism.…”

Section: Discussionmentioning

confidence: 99%

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Long-term neurodevelopmental outcome in conservatively treated congenital hyperinsulinism

Mazor-Aronovitch

Gillis²,

Lobel³

et al. 2007

eur j endocrinol

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Section: Discussionsupporting

confidence: 79%

Section: Discussionmentioning

confidence: 99%

Long-term neurodevelopmental outcome in conservatively treated congenital hyperinsulinism

Mazor-Aronovitch

Gillis²,

Lobel³

et al. 2007

eur j endocrinol

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“…Both have only modest success and are technically difficult and highly invasive. More recently, PET scans with 18-fluoro-L-3,4-dihydroxyphenylalanine ( 18 F-DOPA) have been shown to accurately discriminate focal HI from diffuse HI (55)(56)(57). In a recent study including 50 patients with HI, the positive predictive value of 18 F-DOPA in diagnosing focal adenomatosis was 100% and the negative predictive value was 81% (58 ).…”

Section: Imaging In Himentioning

confidence: 99%

Hyperinsulinism in Infancy and Childhood: When an Insulin Level Is Not Always Enough

2008

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BACKGROUND: Hypoglycemia in infants and children can lead to seizures, developmental delay, and permanent brain damage. Hyperinsulinism (HI) is the most common cause of both transient and permanent disorders of hypoglycemia. HI is characterized by dysregulated insulin secretion, which results in persistent mild to severe hypoglycemia. The various forms of HI represent a group of clinically, genetically, and morphologically heterogeneous disorders.CONTENT: Congenital hyperinsulinism is associated with mutations of SUR-1 and Kir6.2, glucokinase, glutamate dehydrogenase, short-chain 3-hydroxyacylCoA dehydrogenase, and ectopic expression on ␤-cell plasma membrane of SLC16A1. Hyperinsulinism can be associated with perinatal stress such as birth asphyxia, maternal toxemia, prematurity, or intrauterine growth retardation, resulting in prolonged neonatal hypoglycemia. Mimickers of hyperinsulinism include neonatal panhypopituitarism, drug-induced hypoglycemia, insulinoma, antiinsulin and insulin-receptor stimulating antibodies, Beckwith-Wiedemann Syndrome, and congenital disorders of glycosylation. Laboratory testing for hyperinsulinism may include quantification of blood glucose, plasma insulin, plasma ␤-hydroxybutyrate, plasma fatty acids, plasma ammonia, plasma acylcarnitine profile, and urine organic acids. Genetic testing is available through commercial laboratories for genes known to be associated with hyperinsulinism. Acute insulin response (AIR) tests are useful in phenotypic characterization. Imaging and histologic tools are also available for diagnosing and classifying hyperinsulinism. The goal of treatment in infants with hyperinsulinism is to prevent brain damage from hypoglycemia by maintaining plasma glucose levels above 700 mg/L (70 mg/dL) through pharmacologic or surgical therapy.

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“…Meissner et al [19] described 114 patients and noted poor psychomotor outcome with infancy onset and 51% patients with mental retardation had undergone surgery. Steinkrauss et al [20] reported 68 patients with CH; one third had developmental delay which was more prevalent in surgical group. Mazor et al [21] studied 21 Ashkenazi children 12 with diffuse and 9 with focal disease; all of them received medical treatment.…”

Section: Discussionmentioning

confidence: 99%

Long Term Medical Treatment of Congenital Hyperinsulinemic Hypoglycemia

Ahmad¹

2014

JED

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Effects of Hypoglycemia on Developmental Outcome in Children With Congenital Hyperinsulinism

Cited by 83 publications

References 23 publications

Long-term neurodevelopmental outcome in conservatively treated congenital hyperinsulinism

Long-term neurodevelopmental outcome in conservatively treated congenital hyperinsulinism

Hyperinsulinism in Infancy and Childhood: When an Insulin Level Is Not Always Enough

Long Term Medical Treatment of Congenital Hyperinsulinemic Hypoglycemia

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