Successful Use of Long Acting Octreotide in Treatment of Congenital Hyperinsulinism

Pratik Shah; Clare Gilbert; Kate Morgan; Louise Hinchey; Senthil Senniappan; Ved Arya; Hannah Levy; Khalid Hussain

doi:10.1530/endoabs.33.OC3.4

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Endocrine Abstracts (2013) 33 OC3.4 | DOI: 10.1530/endoabs.33.OC3.4

BSPED2013 Oral Communications Oral Communications 3 (6 abstracts)

Successful Use of Long Acting Octreotide in Treatment of Congenital Hyperinsulinism

Pratik Shah , Clare Gilbert , Kate Morgan , Louise Hinchey , Senthil Senniappan , Ved Arya , Hannah Levy & Khalid Hussain

Err

Author affiliations

Great Ormond Street Hospital NHS Foundation Trust, London, UK.

Introduction/Aim: Congenital hyperinsulinism (CHI) is a cause of severe hypoglycaemia in infancy. Treatment of diazoxide unresponsive patients includes the use of somatostatin analogues (octreotide given either as four s.c. injections daily or via a pump). We aimed to evaluate the use of a long acting somatostatin analogue (Lanreotide) in children with CHI, switching them from daily oral diazoxide or s.c. Octreotide injections to 4 weekly Lanreotide injections.

Methods: Children with diffuse CHI on high dose diazoxide or daily octreotide injection were recruited. Lanreotide 30 mg was injected by deep s.c. route and the daily diazoxide/octreotide was weaned after the first dose of Lanreotide as per standard protocol. Each patient had height/weight, baseline USS abdomen, TFT, IGF1/IGFBP3, liver function, HbA1c and continuous glucose monitoring pre and post-Lanreotide therapy. Quality of life questionnaire was completed. Lanreotide levels for all the patients were measured.

Results: Four boys and four girls have been started on Lanreotide in the ongoing study. The mean age is 6.4 years (3.5–14 years). Three of them are currently being weaned off their continuous overnight feeds. Results as below.


	1	2	3	4	5	6	7	8
Treatment	Diazoxide 6.7 mg/kg per day	Octreotide 7.6 μg/kg per day	Octreotide 12 μg/kg per day	Octreotide 10 μg/kg per day	Diazoxide 13.5 mg/kg per day	Octreotide 24 μg/kg per day	Octreotide 22.7 μg/kg per day	Octreotide 29 μg/kg per day
Genetics	None	Homozyg-ous mutation ABCC8	Homozyg-ous mutation ABCC8	None	None	None	Heterozygous mutation ABCC8	Homozyg-ous mutation ABCC8
Previous pancreatec-tomy	No	No	No	No	No	No	Yes	Yes
Time to come off above treatment (in weeks)	8	4	4	12	12 (on 5 mg/kg per day diazoxide)	4	4	4

Conclusion: Lanreotide is a safe and effective alternative to octreotide/diazoxide therapy in patients with CHI, offering an improved quality of life. Long term follow up is required. The preliminary data on quality of life does suggest that both parents and children are very satisfied with the response.