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Nifedipine in Congenital Hyperinsulinism- A Case Report

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-un...

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Autores principales: Khawash, Papiya, Hussain, Khalid, Flanagan, Sarah E., Chatterjee, Sudip, Basak, Dhananjoy
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Galenos Publishing 2015
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4563189/
https://www.ncbi.nlm.nih.gov/pubmed/26316440
http://dx.doi.org/10.4274/jcrpe.1978
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author Khawash, Papiya
Hussain, Khalid
Flanagan, Sarah E.
Chatterjee, Sudip
Basak, Dhananjoy
author_facet Khawash, Papiya
Hussain, Khalid
Flanagan, Sarah E.
Chatterjee, Sudip
Basak, Dhananjoy
author_sort Khawash, Papiya
collection PubMed
description 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.
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spelling pubmed-45631892015-09-24 Nifedipine in Congenital Hyperinsulinism- A Case Report Khawash, Papiya Hussain, Khalid Flanagan, Sarah E. Chatterjee, Sudip Basak, Dhananjoy J Clin Res Pediatr Endocrinol Case Report 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. Galenos Publishing 2015-06 2015-06-03 /pmc/articles/PMC4563189/ /pubmed/26316440 http://dx.doi.org/10.4274/jcrpe.1978 Text en © Journal of Clinical Research in Pediatric Endocrinology, Published by Galenos Publishing. http://creativecommons.org/licenses/by/2.5/ This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
spellingShingle Case Report
Khawash, Papiya
Hussain, Khalid
Flanagan, Sarah E.
Chatterjee, Sudip
Basak, Dhananjoy
Nifedipine in Congenital Hyperinsulinism- A Case Report
title Nifedipine in Congenital Hyperinsulinism- A Case Report
title_full Nifedipine in Congenital Hyperinsulinism- A Case Report
title_fullStr Nifedipine in Congenital Hyperinsulinism- A Case Report
title_full_unstemmed Nifedipine in Congenital Hyperinsulinism- A Case Report
title_short Nifedipine in Congenital Hyperinsulinism- A Case Report
title_sort nifedipine in congenital hyperinsulinism- a case report
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4563189/
https://www.ncbi.nlm.nih.gov/pubmed/26316440
http://dx.doi.org/10.4274/jcrpe.1978
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