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Nifedipine in Congenital Hyperinsulinism - A Case Report.

Khawash P, Hussain K, Flanagan SE, Chatterjee S, Basak D - J Clin Res Pediatr Endocrinol (2015)

Bottom Line: Oral nifedipine has been effective in isolated cases of CHI.Octreotide was tapered off by one year age and thereafter the child is euglycaemic on oral nifedipine alone.Continuous glucose monitoring sensor was used as an aid to monitor glycaemic control and was found to be a safe and reliable option reducing the number of needle-pricks in small children.

View Article: PubMed Central - PubMed

Affiliation: Park Clinic, Clinic of Paediatrics and Neonatology, Kolkata, India Phone: +91 9831135350 E-mail: pkhawash@gmail.com.

ABSTRACT
Congenital hyperinsulinism (CHI) is the commonest cause of persistent hypoglycemia in neonates. Diazoxide is the first-line drug in its treatment, but the more severe cases are usually diazoxide-resistant. Recessive ABCC8 and KCNJ11 mutations are responsible for most (82%) of the severe diazoxide-unresponsive CHI. Oral nifedipine has been effective in isolated cases of CHI. Successful treatment of diazoxide-unresponsive CHI with a combination of octreotide and nifedipine has been reported in a single isolated case so far. We report here a case of diazoxide-resistant CHI due to homozygous ABCC8 nonsense mutation. In this case, hypoglycaemia uncontrolled by pancreatectomy and octreotide alone showed a good response to a combination of nifedipine and octreotide. Octreotide was tapered off by one year age and thereafter the child is euglycaemic on oral nifedipine alone. Continuous glucose monitoring sensor was used as an aid to monitor glycaemic control and was found to be a safe and reliable option reducing the number of needle-pricks in small children.

No MeSH data available.


Related in: MedlinePlus

CGMS data at 11/2 year age showing hypoglycaemia on tapering off oral nifedipine with recovery on re-starting nifedipine in previous dosage.
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f2: CGMS data at 11/2 year age showing hypoglycaemia on tapering off oral nifedipine with recovery on re-starting nifedipine in previous dosage.

Mentions: After 4 months, S/c octreotide was gradually tapered off and stopped by one year of age. The child thereafter maintained euglycaemia on oral nifedipine only. Follow-up visits were difficult to arrange and at 11/2 years of age, the baby was admitted to determine if the dose of nifedipine also needed tapering. A CGMS monitor was inserted and oral nifedipine was tapered under supervision. However, following a hypoglycaemic episode, nifedipine was restarted in the previous dosage of 0.8 mg/kg/day. A high insulin level during the hypoglycaemic episode (insulin-19 μIU/mL when lab glucose was 29 mg/dL) confirmed the persistent hyperinsulinaemic state. CGMS data after readjusting nifedipine dosage to previous levels shows good glycaemic control (vide Figure 2).


Nifedipine in Congenital Hyperinsulinism - A Case Report.

Khawash P, Hussain K, Flanagan SE, Chatterjee S, Basak D - J Clin Res Pediatr Endocrinol (2015)

CGMS data at 11/2 year age showing hypoglycaemia on tapering off oral nifedipine with recovery on re-starting nifedipine in previous dosage.
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4563189&req=5

f2: CGMS data at 11/2 year age showing hypoglycaemia on tapering off oral nifedipine with recovery on re-starting nifedipine in previous dosage.
Mentions: After 4 months, S/c octreotide was gradually tapered off and stopped by one year of age. The child thereafter maintained euglycaemia on oral nifedipine only. Follow-up visits were difficult to arrange and at 11/2 years of age, the baby was admitted to determine if the dose of nifedipine also needed tapering. A CGMS monitor was inserted and oral nifedipine was tapered under supervision. However, following a hypoglycaemic episode, nifedipine was restarted in the previous dosage of 0.8 mg/kg/day. A high insulin level during the hypoglycaemic episode (insulin-19 μIU/mL when lab glucose was 29 mg/dL) confirmed the persistent hyperinsulinaemic state. CGMS data after readjusting nifedipine dosage to previous levels shows good glycaemic control (vide Figure 2).

Bottom Line: Oral nifedipine has been effective in isolated cases of CHI.Octreotide was tapered off by one year age and thereafter the child is euglycaemic on oral nifedipine alone.Continuous glucose monitoring sensor was used as an aid to monitor glycaemic control and was found to be a safe and reliable option reducing the number of needle-pricks in small children.

View Article: PubMed Central - PubMed

Affiliation: Park Clinic, Clinic of Paediatrics and Neonatology, Kolkata, India Phone: +91 9831135350 E-mail: pkhawash@gmail.com.

ABSTRACT
Congenital hyperinsulinism (CHI) is the commonest cause of persistent hypoglycemia in neonates. Diazoxide is the first-line drug in its treatment, but the more severe cases are usually diazoxide-resistant. Recessive ABCC8 and KCNJ11 mutations are responsible for most (82%) of the severe diazoxide-unresponsive CHI. Oral nifedipine has been effective in isolated cases of CHI. Successful treatment of diazoxide-unresponsive CHI with a combination of octreotide and nifedipine has been reported in a single isolated case so far. We report here a case of diazoxide-resistant CHI due to homozygous ABCC8 nonsense mutation. In this case, hypoglycaemia uncontrolled by pancreatectomy and octreotide alone showed a good response to a combination of nifedipine and octreotide. Octreotide was tapered off by one year age and thereafter the child is euglycaemic on oral nifedipine alone. Continuous glucose monitoring sensor was used as an aid to monitor glycaemic control and was found to be a safe and reliable option reducing the number of needle-pricks in small children.

No MeSH data available.


Related in: MedlinePlus