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
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.
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Endocrine Abstracts
ISSN 1470-3947 (print) | ISSN 1479-6848 (online)
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