A Combination of Nifedipine and Octreotide Treatment in an Hyperinsulinemic Hypoglycemic Infant

Durmaz, Erdem; Flanagan, Sarah E.; Parlak, Mesut; Ellard, Sian; Akçurin, Sema; Bircan, İffet

doi:10.4274/jcrpe.1230

Cited by 19 publications

(22 citation statements)

References 17 publications

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“…Nifedipine is another off-label drug for neonatal use in cases where a response to diazoxide and octreotide are not seen (57). Nifedipine has been used in babies at a dose of 0.3-0.8 mg/kg/day by Bas et al (58) and was effective when other therapies failed, but this medication is not the mainstay of treatment due to its cardiovascular side effects.…”

Section: Approach To Diagnosis and Management Of Hypoglycemia In The mentioning

confidence: 99%

Hypoglycemia in the preterm neonate: etiopathogenesis, diagnosis, management and long-term outcomes

Sharma¹,

Davis²,

Shekhawat³

2017

Transl. Pediatr

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Glucose, like oxygen, is of fundamental importance for any living being and it is the major energy source for the fetus and the neonate during gestation. The placenta ensures a steady supply of glucose to the fetus, while birth marks a sudden change in substrate delivery and a major change in metabolism. Hypoglycemia is one of the most common pathologies encountered in the neonatal intensive care unit and affects a wide range of neonates. Preterm, small for gestational age (GA) and intra-uterine growth restricted neonates are especially vulnerable due to their lack of metabolic reserves and associated co-morbidities. Nearly 30-60% of these high-risk infants are hypoglycemic and require immediate intervention. Preterm neonates are uniquely predisposed to developing hypoglycemia and its associated complications due to their limited glycogen and fat stores, inability to generate new glucose using gluconeogenesis pathways, have higher metabolic demands due to a relatively larger brain size, and are unable to mount a counter-regulatory response to hypoglycemia. In this review we will discuss the epidemiology; pathophysiology; clinical presentation; management and neurodevelopmental outcomes in affected infants and summarize evidence to develop a rational and scientific approach to this common problem.

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Section: Approach To Diagnosis and Management Of Hypoglycemia In The mentioning

confidence: 99%

Hypoglycemia in the preterm neonate: etiopathogenesis, diagnosis, management and long-term outcomes

Sharma¹,

Davis²,

Shekhawat³

2017

Transl. Pediatr

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“… Nifedipine is a calcium antagonist, which is thought to act by blocking the influx of calcium into the pancreatic β cells ( 31 ). The efficacy is usually limited but successful glycemic control for residual hypoglycemia after pancreatectomy has been reported ( 75 , 76 , 77 ). Approximately 90% of patients with diazoxide unresponsive CHI have mutations in the K ATP channel genes, ABCC8 or KCNJ11 ) ( 78 , 79 ).…”

Section: Second Line Treatmentmentioning

confidence: 99%

Section: Second Line Treatmentmentioning

confidence: 99%

Clinical practice guidelines for congenital hyperinsulinism

Yorifuji

Horikawa

Hasegawa

et al. 2017

Clin Pediatr Endocrinol

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Abstract.Congenital hyperinsulinism is a rare condition, and following recent advances in diagnosis and treatment, it was considered necessary to formulate evidence-based clinical practice guidelines reflecting the most recent progress, to guide the practice of neonatologists, pediatric endocrinologists, general pediatricians, and pediatric surgeons. These guidelines cover a range of aspects, including general features of congenital hyperinsulinism, diagnostic criteria and tools for diagnosis, first- and second-line medical treatment, criteria for and details of surgical treatment, and future perspectives. These guidelines were generated as a collaborative effort between The Japanese Society for Pediatric Endocrinology and The Japanese Society of Pediatric Surgeons, and followed the official procedures of guideline generation to identify important clinical questions, perform a systematic literature review (April 2016), assess the evidence level of each paper, formulate the guidelines, and obtain public comments.

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“…11 The calcium channel blocker nifedipine has also been reported as an alternative in unresponsive cases. 12 Focal lesions usually warrant surgical excision, whereas for therapy-resistant diffuse presentations the only treatment option available is subtotal or total pancreatectomy. 13 Definitive success rates are high for the former, but about one-third of patients remain hyperinsulinemic after subtotal pancreatectomy, many develop exocrine pancreatic insufficiency, and nearly all will develop diabetes by adolescence.…”

Section: Figurementioning

confidence: 99%

Severe Hyperinsulinemic Hypoglycemia in a Neonate: Response to Sirolimus Therapy

et al. 2015

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Hyperinsulinemic hypoglycemia (HH) is one of the most common causes of persistent hypoglycemic episodes in neonates. Current pharmacologic treatment of neonatal HH includes diazoxide and octreotide, whereas for diffuse, unresponsive cases a subtotal pancreatectomy may be the last resort, with questionable efficacy. Here we report a case of congenital diffuse neonatal HH, first suspected when severe hypoglycemia presented with extremely high serum insulin levels immediately after birth. Functional imaging and genetic tests later confirmed the diagnosis. Failure to respond to a sequence of different treatments and to avoid extensive surgery with predictable morbidity prompted us to introduce a recently suggested alternative therapy with sirolimus, a mammalian target of rapamycin inhibitor. Glucose intake could be reduced gradually while euglycemia was maintained, and we were able to achieve exclusively enteral feeding within 6 weeks. Sirolimus was found to be effective and well tolerated, with no major adverse side effects attributable to its administration. PATIENT PRESENTATIONOur patient, a male infant, was born by normal vaginal delivery during the 37th week of gestation after an uneventful pregnancy. Birth weight was 4400 g, and 1-and 5-minute Apgar scores were 10 and 10. Both parents and the baby's 2 older siblings had unremarkable medical histories. A right-sided clavicular fracture was noted on first examination, which did not warrant additional intervention. Two hours after delivery, tremor and irritability were observed, with severe hypoglycemia (0.5 mmol/L, 9 mg/dL). High doses of intravenous glucose (up to 20 mg/kg per minute) and occasional glucagon boluses were needed to normalize the persistently low blood glucose levels. The diagnosis of hyperinsulinemic hypoglycemia (HH) was based on the clinical picture and on the laboratory values (glucose, 0.5 mmol/L; concomitant serum insulin, 130.7 mU/mL; growth hormone, 18.3 ng/mL; thyroidstimulating hormone, 8.2 mU/L; free thyroxine, 20.5 pmol/L; cortisol, 835 nmol/L), and samples were sent for genetic testing.Diazoxide therapy (with hydrochlorothiazide 1 mg/kg per day) was commenced on day 4 and was gradually increased to a maximum dose of 20 mg/kg per day without any effect. Somatostatin treatment (introduced as intravenous, subsequently modified to subcutaneous) was initiated on week 2 and increased to a maximum dose (35 mg/kg per day), but only a 20% reduction in total glucose requirements was achieved. On week 4 nifedipine was added to the therapeutic regimen, but it was discontinued after a week because of lack of response (Fig 1).

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A Combination of Nifedipine and Octreotide Treatment in an Hyperinsulinemic Hypoglycemic Infant

Cited by 19 publications

References 17 publications

Hypoglycemia in the preterm neonate: etiopathogenesis, diagnosis, management and long-term outcomes

Hypoglycemia in the preterm neonate: etiopathogenesis, diagnosis, management and long-term outcomes

Clinical practice guidelines for congenital hyperinsulinism

Severe Hyperinsulinemic Hypoglycemia in a Neonate: Response to Sirolimus Therapy

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