Persistent hyperinsulinemic hypoglycemia of infancysuccessful therapy with nifedipine

Shanbag, Preeti; Pathak, Ashish; Vaidya, Mamta; Shahid, Sukhbir Kaur

doi:10.1007/bf02734240

Cited by 32 publications

(27 citation statements)

References 5 publications

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“…Nifedipine is a calcium channel antagonist and has been used in some patients with CHI, although the vast majority of patients fail to show any response. Despite this, there have been several reports of nifedipine-responsive CHI patients [61, 62,] but the underlying molecular pathophysiology of CHI in these reported cases is unclear.…”

Section: Medical Managementmentioning

confidence: 97%

Diagnosis and Management of Hyperinsulinaemic Hypoglycaemia of Infancy

Hussain¹

2007

Horm Res Paediatr

150

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Hyperinsulinaemic hypoglycaemia is a cause of persistent hypoglycaemia in the neonatal and infancy periods. Prompt recognition and management of patients with hyperinsulinaemic hypoglycaemia are essential, if brain damage and long-term neurological sequelae are to be avoided. Hyperinsulinaemic hypoglycaemia can be transient, prolonged, or persistent (congenital). Advances in the fields of molecular biology, genetics, and pancreatic β-cell physiology are beginning to provide novel insights into the mechanisms causing congenital forms of hyperinsulinism. So far mutations in six different genes have been described that lead to unregulated insulin secretion. The histological differentiation of focal and diffuse congenital hyperinsulinism has radically changed the surgical approach to this disease. Until recently, highly invasive investigations were performed to localize the focal lesion, but recent experience with ¹⁸F-L-dopa positron emission tomography scanning suggests that this technique is highly sensitive for differentiating diffuse from focal disease as well as for accurately locating the focal lesion. Despite recent advances, the genetic basis of congenital hyperinsulinism is still unknown in about 50% of the patients, and the management of medically unresponsive diffuse disease remains a real challenge.

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Section: Medical Managementmentioning

confidence: 97%

Diagnosis and Management of Hyperinsulinaemic Hypoglycaemia of Infancy

Hussain¹

2007

Horm Res Paediatr

150

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“…In the paradigm outlined, blockers of VGCCs should be therapeutically efficacious in all cases of HI. A review of the literature suggests, however, that this in not the case, and whilst some patients are clearly responsive (12,76,171,256,269), in others, nifedipine is ineffective in the control of hypoglycemia (71, 291). This may, in part, be related to the dose of nifedipine that can be safely administered to neonates, but there is also evidence that the activity of voltage-gated Ca 2ϩ channels in HI ␤-cells is functionally heterogeneous (48).…”

Section: Depolarization-response Coupling In Hi-katp ␤-Cellsmentioning

confidence: 99%

“…No adverse effects have so far been reported in patients with HI. However, the response rates of patients are variable, blood pressure monitoring is mandatory, and because only a limited number of centers have reported clinical experiences of nifedipine, there is no data on long-term use in the treatment of hyperinsulinism (12,71,76,256,269,291). Figure 18 summarizes the key therapeutic options and long-term strategies for patients with the different hyperinsulinism syndromes.…”

Section: E Nifedipinementioning

confidence: 99%

Hyperinsulinism in Infancy: From Basic Science to Clinical Disease

Dunne

Cosgrove²,

Shepherd³

et al. 2004

Physiological Reviews

262

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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“…Nifedipine has been successfully used in patients with HI [27,28,29]. In summary, we report a case of severe hyperinsulinaemic hypoglycaemia in a baby born to a mother on long-term oral ritodrine therapy for preterm labour.…”

Section: Discussionmentioning

confidence: 98%

Severe Hyperinsulinaemic Hypoglycaemia in a Baby Born to a Mother Taking Oral Ritodrine Therapy for Preterm Labour

Kurtoğlu

Akçakuş²,

Keskın³

et al. 2005

Horm Res Paediatr

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Hyperinsulinism of infancy is a major cause of persistent hypoglycaemia in the newborn period. Transient mild self-limiting hyperinsulinaemia and hypoglycaemia have been described in neonates born to mothers taking ritodrine therapy for premature labour. Ritodrine crosses the placental barrier and enters the fetal circulation readily but the mechanism of how it causes hyperinsulinaemia and hypoglycaemia is unclear. We report the case of severe prolonged hyperinsulinaemic hypoglycamia in a neonate born to a mother taking ritodrine therapy from 16 weeks’ gestation for preterm labour. The hyperinsulinaemic hypoglycaemia was managed with oral nifedipine as diazoxide was contraindicated due to fluid overload. Possible mechanisms of ritodrine-induced hypoglycaemia and insulin secretion are discussed.

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Persistent hyperinsulinemic hypoglycemia of infancysuccessful therapy with nifedipine

Abstract: Recent studies have demonstrated a role for calcium channel blocking agents in the treatment of persistent hyperinsulinemic hypoglycemia of infancy. We report a 30 day old infant with PHHI whom we successfully treated with oral nifedipine alone.

Cited by 32 publications

References 5 publications

Diagnosis and Management of Hyperinsulinaemic Hypoglycaemia of Infancy

Diagnosis and Management of Hyperinsulinaemic Hypoglycaemia of Infancy

Hyperinsulinism in Infancy: From Basic Science to Clinical Disease

Severe Hyperinsulinaemic Hypoglycaemia in a Baby Born to a Mother Taking Oral Ritodrine Therapy for Preterm Labour

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