Ten years’ experience of persistent hyperinsulinaemic hypoglycaemia of infancy

Vj, Tyrrell; Ambler, Geoffrey; Wh, Yeow; Cowell, CT; Silink, Martin

doi:10.1046/j.1440-1754.2001.00748.x

Cited by 17 publications

(31 citation statements)

References 38 publications

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“…Other studies have shown that only 20% of infants with hyperinsulinaemia are LGA at birth. 25 Our study supports the recent advice to refrain from routine blood glucose measurements in healthy term infants who are not small for gestational age. 9 Our study sample comprised infants born in hospital.…”

Section: Discussionsupporting

confidence: 88%

Neurodevelopmental outcome of hypoglycaemia in healthy, large for gestational age, term newborns

Brand¹

2005

Archives of Disease in Childhood

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Aims: To evaluate the effects of transient hypoglycaemia on the first day of life in 75 healthy term large for gestational age (LGA) infants, born to non-diabetic mothers, on their neurodevelopmental outcome at the age of 4 years. Methods: Screening for hypoglycaemia was performed 1, 3, and 5 hours after birth, and continued if blood glucose levels were low. Treatment with intravenous glucose for hypoglycaemia was started if hypoglycaemia was severe or symptomatic. Patients' development and behaviour was examined at the age of 4 years by the Denver Developmental Scale, a non-verbal intelligence test, and the Child Behaviour Check List. Results: There were no significant differences between children with neonatal normoglycaemia (n = 15) and hypoglycaemia (plasma glucose ,2.2 mmol/l 1 hour after birth, or ,2.5 mmol/l subsequently; n = 60) in Denver developmental scale scores and child behaviour checklist scores. Although total IQ did not differ between hypoglycaemic and normoglycaemic children, one subscale (reasoning) did (mean difference 9.3, 95% CI 1.3 to 17.2). The correlation between reasoning IQ and neonatal blood glucose levels was weak and not statistically significant. When other definitions for hypoglycaemia were applied, the difference in reasoning IQ was not found. There were no differences in any of the test scores between hypoglycaemic children who had and who had not been treated with intravenous glucose. Conclusion: Transient mild hypoglycaemia in healthy, term LGA newborns does not appear to be harmful to psychomotor development at the age of 4 years.

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Section: Discussionsupporting

confidence: 88%

Neurodevelopmental outcome of hypoglycaemia in healthy, large for gestational age, term newborns

Brand¹

2005

Archives of Disease in Childhood

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“…Similarly, trends indicate an increased prevalence of developmental delay and psychomotor retardation in infants undergoing surgery at a later age [16,17] . The effi cient diagnosis and surgical management of HI in the present case stands in contrast to the variable time to diagnosis and surgery in other studies of HI (median: 1-180 days; median: 30-354 days, respectively) [3,11,12,14,[16][17][18][19][20] . The in utero diagnosis of HI in our case allowed for the immediate initiation of medical management at birth with a quicker progression to surgical management when medical management failed.…”

Section: Discussioncontrasting

confidence: 77%

“…Similarly, other studies have demonstrated an increased risk of persistent neurodevelopmental delays, reduced head circumference, reduced IQ scores and an increased rate of clinical complications at the time of birth associated with a delay in diagnosis and adequate management of hypoglycemia in the neonate [7][8][9][10] . Studies of infants with HI also indicate an increased incidence of neurodevelopmental problems associated with a delay in the recognition and management of hypoglycemia [1,[11][12][13][14][15] . This is exemplifi ed by the study of Jack et al [14], which supports improved neurodevelopmental outcomes in patients euglycemic within 35 days of presentation.…”

Section: Discussionmentioning

confidence: 99%

Prenatal Diagnosis and Postnatal Management of Diffuse Congenital Hyperinsulinism: A Case Report

Peranteau

Ganguly²,

Steinmuller³

et al. 2006

Fetal Diagn Ther

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We present the first case of the prenatal diagnosis of congenital hyperinsulinism based on the genetic analysis of known family mutations in the SUR1 gene. An amniocentesis was performed at 16 weeks gestation at which time two mutations in the SUR1 gene were identified consistent with the diagnosis of diffuse hyperinsulinism. The mother was transported to our facility and underwent an elective cesarian section at 38 weeks gestation. The diagnosis was confirmed and treatment was initiated within the first minutes of life. After a short course of failed medical management, the patient underwent a 98% pancreatectomy with subsequent good glycemic control. This case highlights the benefits of the timely in utero diagnosis of hyperinsulinism by mutational analysis.

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“…Furthermore, repeated episodes of severe, prolonged, sublethal hypoglycemia can result in permanent neurological damage, including developmental delay, mental retardation, and/or focal CNS deficits. Complications of neonatal hyperinsulinism are found in up to 50% of survivors, and this incidence has changed little during the past 20 years, reflecting the major problems that are faced in managing and treating this condition (5,8,9,29,166,173,181,196,201,253,284,313).…”

Section: Early Complications Of Hyperinsulinism In Infancymentioning

confidence: 99%

Hyperinsulinism in Infancy: From Basic Science to Clinical Disease

Dunne

Cosgrove²,

Shepherd³

et al. 2004

Physiological Reviews

263

277

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Dunne, Mark J., Karen E. Cosgrove, Ruth M. Shepherd, Albert Aynsley-Green, and Keith J. Lindley. Hyperinsulinism in Infancy: From Basic Science to Clinical Disease. Physiol Rev 84: 239–275, 2004; 10.1152/physrev.00022.2003.—Ion channelopathies have now been described in many well-characterized cell types including neurons, myocytes, epithelial cells, and endocrine cells. However, in only a few cases has the relationship between altered ion channel function, cell biology, and clinical disease been defined. Hyperinsulinism in infancy (HI) is a rare, potentially lethal condition of the newborn and early childhood. The causes of HI are varied and numerous, but in almost all cases they share a common target protein, the ATP-sensitive K+ channel. From gene defects in ion channel subunits to defects in β-cell metabolism and anaplerosis, this review describes the relationship between pathogenesis and clinical medicine. Until recently, HI was generally considered an orphan disease, but as parallel defects in ion channels, enzymes, and metabolic pathways also give rise to diabetes and impaired insulin release, the HI paradigm has wider implications for more common disorders of the endocrine pancreas and the molecular physiology of ion transport.

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Ten years’ experience of persistent hyperinsulinaemic hypoglycaemia of infancy

Cited by 17 publications

References 38 publications

Neurodevelopmental outcome of hypoglycaemia in healthy, large for gestational age, term newborns

Neurodevelopmental outcome of hypoglycaemia in healthy, large for gestational age, term newborns

Prenatal Diagnosis and Postnatal Management of Diffuse Congenital Hyperinsulinism: A Case Report

Hyperinsulinism in Infancy: From Basic Science to Clinical Disease

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