Treatment with long-acting lanreotide autogel in early infancy in patients with severe neonatal hyperinsulinism
- PDF / 521,915 Bytes
- 8 Pages / 595.276 x 790.866 pts Page_size
- 72 Downloads / 176 Views
RESEARCH
Open Access
Treatment with long-acting lanreotide autogel in early infancy in patients with severe neonatal hyperinsulinism Heike Corda1*, Sebastian Kummer1, Alena Welters1, Norbert Teig2, Dirk Klee3, Ertan Mayatepek1 and Thomas Meissner1
Abstract Background: Treatment of severe diffuse congenital hyperinsulinism (CHI) without sufficient response to diazoxide is complicated by the lack of approved drugs. Therefore, patients are often hospitalized long-term or have to undergo pancreatic surgery if episodes of severe hypoglycaemia cannot be prevented. A long-acting somatostatin analogue, octreotide, has been reported to be an effective treatment option that prevents severe hypoglycaemia in children with CHI, and its off-label use is common in CHI. However, octreotide requires continuous i.v. or s.c. infusion or multiple daily injections. Here, we report our experiences with the use of a monthly application of a long-acting somatostatin analogue, lanreotide autogel® (LAN-ATG), in early infancy. Results: The mean blood glucose concentration within 7 days before the first LAN-ATG administration were compared to 7 days after the first LAN-ATG administration and increased by 0.75 mmol/L (range 0.39–1.19 mmol/L). In the following weeks intravenous glucose infusions, octreotide, and glucagon treatment could be successfully stopped in all patients 3–20 days after the first LAN-ATG injection without substantial worsening of the hypoglycaemia rate. Increased carbohydrate requirements could be normalized with an average reduction in the carbohydrate-intake of 7 g/kg body weight/d (range 1.75–12.8 g/kg body weight/d). Over a total of 52 treatment months, no serious adverse effects occurred. Conclusion: Long-term LAN-ATG treatment improved blood glucose concentrations, lowered the frequency of hypoglycaemia or allowed for normalization of oral carbohydrate intake in infants with CHI younger than 6 months of age. No severe side effects were observed. LAN-ATG might be an alternative treatment option in infants with severe CHI who lack risk factors for necrotizing enterocolitis and are not responding to current treatment regimens as an alternative to surgery after careful individual evaluation. Keywords: Congenital hyperinsulinism, Beckwith-Wiedemann syndrome, Hyperinsulinaemic hypoglycaemia, Somatostatin analogue, Adverse event
Background Congenital hyperinsulinism (CHI) is the most frequent cause of persistent hypoglycaemia in newborns [1] that can be differentiated into a diffuse (affecting the entire pancreas), focal, or atypical form (affecting one or multiple smaller regions in the pancreas) [2, 3].
* Correspondence: [email protected] 1 Department of General Pediatrics, Neonatology and Pediatric Cardiology, University Children’s Hospital Duesseldorf, Moorenstrasse 5, 40225 Duesseldorf, Germany Full list of author information is available at the end of the article
The most frequent genetic causes are mutations in ABCC8 and KCNJ11, coding for the subunits of the ATP-sensitive potassium channel in
Data Loading...
