title: Use of Long-Acting Somatostatin Analogue (Lanreotide) in an Adolescent with Diazoxide-Responsive Congenital Hyperinsulinism and Its Psychological Impact.
abstract: Congenital hyperinsulinism (CHI) is a common cause of hypoglycaemia due to unregulated insulin secretion from pancreatic β cells. Medical management includes use of oral diazoxide (a KATP channel agonist) and daily injectable octreotide (somatostatin analogue) therapy. However, diazoxide is associated with severe sideeffects such as coarse facies, hypertrichosis and psychosocial/compliance issues in adolescents. Lanreotide (a long-acting somatostatin analogue) is used in adults with neuroendocrine tumours; however, its role in patients with CHI has not been well described. A 15-year-old girl with diazoxide-responsive CHI had severe hypertrichosis secondary to diazoxide and subsequent compliance/psychosocial issues. She was commenced on 30 mg of lanreotide every 4 weeks as a deep subcutaneous injection, in an attempt to address these issues. She was able to come off diazoxide treatment 2 months after starting lanreotide. Presently, after 2.5 years of lanreotide treatment, her blood glucose control is stable with complete resolution of hypertrichosis. Clinically significant improvements in the self-reported Paediatric Quality of Life (PedsQL) questionnaire and Strengths and Difficulties Questionnaire (SDQ) were reported after 1 year on lanreotide. No side effects were found, and her liver/thyroid function and abdominal ultrasound have been normal. We report the first case on the use of lanreotide in an adolescent girl with diazoxide-responsive CHI with significant improvement of quality of life.
authors.affiliation: Developmental Endocrinology Research Group, Genetics and Genomic Medicine Programme, Institute of Child Health, University College London, London, UK.
authors.forename: Pratik
authors.initials: P
authors.lastname: Shah
authors.forename: Sofia A
authors.initials: SA
authors.lastname: Rahman
authors.forename: Sharon
authors.initials: S
authors.lastname: McElroy
authors.forename: Clare
authors.initials: C
authors.lastname: Gilbert
authors.forename: Kate
authors.initials: K
authors.lastname: Morgan
authors.forename: Louise
authors.initials: L
authors.lastname: Hinchey
authors.forename: Senthil
authors.initials: S
authors.lastname: Senniappan
authors.forename: Hannah
authors.initials: H
authors.lastname: Levy
authors.forename: Rakesh
authors.initials: R
authors.lastname: Amin
authors.forename: Khalid
authors.initials: K
authors.lastname: Hussain
mesh_terms: D000293:Adolescent; D001786:Blood Glucose / Q000378:metabolism; D044903:Congenital Hyperinsulinism / Q000150:complications / Q000188:drug therapy* / Q000523:psychology*; D003981:Diazoxide / Q000008:administration & dosage / Q000627:therapeutic use*; D004232:Diuretics / Q000008:administration & dosage / Q000627:therapeutic use*; D005260:Female; D006801:Humans; D006983:Hypertrichosis / Q000188:drug therapy / Q000209:etiology / Q000523:psychology; D007279:Injections, Subcutaneous; D010456:Peptides, Cyclic / Q000008:administration & dosage / Q000627:therapeutic use*; D011788:Quality of Life; D012919:Social Behavior; D013004:Somatostatin / Q000008:administration & dosage / Q000031:analogs & derivatives* / Q000627:therapeutic use; D016896:Treatment Outcome
chemical_list: D001786:Blood Glucose; D004232:Diuretics; D010456:Peptides, Cyclic; C060347:lanreotide; D013004:Somatostatin; D003981:Diazoxide