Use of Long-Acting Somatostatin Analogue (Lanreotide) in an Adolescent with Diazoxide-Responsive Congenital Hyperinsulinism and Its Psychological Impact

2015 ◽  
Vol 84 (5) ◽  
pp. 355-360 ◽  
Author(s):  
Pratik Shah ◽  
Sofia A. Rahman ◽  
Sharon McElroy ◽  
Clare Gilbert ◽  
Kate Morgan ◽  
...  
2017 ◽  
Vol 89 (2) ◽  
pp. 82-89 ◽  
Author(s):  
Ivo van der Steen ◽  
Mirjam E. van Albada ◽  
Klaus Mohnike ◽  
Henrik Thybo Christesen ◽  
Susann Empting ◽  
...  

Background/Aims: Congenital hyperinsulinism (CHI) is a rare disease characterized by recurrent severe hypoglycemia. In the diffuse form of CHI, pharmacotherapy is the preferred choice of treatment. Long-acting somatostatin analogues have been used in children as off-label medication. However, the efficacy, outcomes, and adverse effect profiles of long-acting somatostatin analogues have not been described in multicentered studies. The aim of this retrospective study is to summarize the experience with long-acting somatostatin analogues in a large group of children with CHI. Methods: Data were obtained retrospectively from 27 patients with CHI who received long-acting somatostatin analogues in 6 different centers in Europe. These included information on glycemic stability, auxology, and adverse effect profile in clinical follow-up assessments. Results: Blood glucose control improved in most patients (89%). No life-threatening side effects occurred. Thirteen patients (48%) experienced side effects; in 3 patients (11%), the side effects were the main reason for discontinuation of the treatment. The most frequent side effect was elevated liver enzymes (n = 10, 37%). Conclusion: Long-acting somatostatin analogues are effective in glycemic control of patients with CHI. However, in 37% of all patients increased liver enzymes were observed. It is important to monitor liver function in all patients receiving long-acting somatostatin analogue therapy.


2012 ◽  
Vol 166 (2) ◽  
pp. 333-339 ◽  
Author(s):  
Kim-Hanh Le Quan Sang ◽  
Jean-Baptiste Arnoux ◽  
Asmaa Mamoune ◽  
Cécile Saint-Martin ◽  
Christine Bellanné-Chantelot ◽  
...  

ContextCongenital hyperinsulinism (HI) is a common cause of hypoglycemia in infancy. The medical treatment of diazoxide-unresponsive HI is based on a somatostatin analogue.ObjectiveThis study aims at replacing three daily s.c. octreotide (Sandostatin, Novartis) injections by a single and monthly i.m. injection of long-acting release (LAR) octreotide (Sandostatin LP, Novartis) in HI patients.Subjects and methodLAR octreotide was injected every 4 weeks during 6 months and s.c. octreotide injections were stopped after the third injection of LAR octreotide. After this 6-month study, LAR octreotide was continued, with an average follow-up of 17 months. Ten HI pediatric patients unresponsive to diazoxide and currently treated with s.c. octreotide were included in the trial. Glycemias and other parameters (HbA1c, IGF1, height, weight, quality of life (QoL), and satisfaction) were monitored at each monthly visit.ResultsFor all ten patients, glycemias were maintained in the usual range, HbAlc (mean 5.5%; 95% CI: 4.6–6.2) and IGF1 (mean 89.7 ng/ml; 95% CI: 26–153) were unchanged. Patients gained height significantly (mean 2.7 cm; 95% CI: 1.9–3.4) and no side effect was noted during the study and the later follow-up. Plasma octreotide levels were stable under LAR octreotide. Parents' questionnaires of general satisfaction were highly positive whereas children's QoL evaluation remained unchanged.ConclusionIn these diazoxide-unresponsive HI patients, LAR octreotide was efficient, well tolerated and contributed to a clear simplification of the medical care.


1988 ◽  
Vol 27 (3) ◽  
pp. 311-316 ◽  
Author(s):  
Masayuki TSUDA ◽  
Shigehisa TAMAKI ◽  
Eiichi IWASAKI ◽  
Seikou MURASHIMA ◽  
Katsumi DEGUCHI ◽  
...  

2011 ◽  
Vol 120 (02) ◽  
pp. 68-72 ◽  
Author(s):  
A. Jawiarczyk ◽  
M. Bolanowski ◽  
J. Syrycka ◽  
G. Bednarek-Tupikowska ◽  
M. Kałużny ◽  
...  

AbstractWe are reporting a case of 68-year-old woman with insulinoma, after a non-successful tumor surgery and a long-term diazoxide treatment. She had a lot of hypoglycemia cases, and a weight gain of 50 kg. An abdominal CT scan demonstrated a tumor 28 mm in the diameter, in the head of the pancreas. The patient did not agree for the repeated insulinoma surgery. Furthermore, we found a lesion in the left adrenal gland (14 mm in the diameter) and in the right lung (8 mm in the diameter). Pheochromocytoma was diagnosed on the basis of hypertension, elevated levels of normetanephrine in the 24-h urine collection, and an elevated level of norepinephrine in a plasma sample. After the left adrenal gland removal we observed lower blood pressure. Since we had revealed the presence of somatostatin receptors by the somatostatin receptors scintigraphy, we decided to control hypoglycemia by a monthly subcutaneous administration of the long-acting lanreotide. Because of higher glucose levels (300–400 mg/dl) we started an intense insulin therapy. Nowadays, the patient feels better, she has lost 20 kg of her body weight, and we have observed normal blood glucose levels during the long-term lanreotide treatment. We have noticed neither side effects nor hypoglycemic episodes and we have reduced the dose of insulin. The presented case can be an evidence of the effective treatment of the pancreatic neuroendocrine tumor of insulinoma type, with somatostatin analogue.


Pancreas ◽  
1991 ◽  
Vol 6 (6) ◽  
pp. 668-672 ◽  
Author(s):  
L. Gullo ◽  
R. Pezzilli ◽  
Dvora Ancona ◽  
A. Morselli Labate ◽  
L. Barbara

Author(s):  
Jordan Gabrielsen, PharmD ◽  
Gianna Girone, PharmD ◽  
Bonita Bennett, BSN ◽  
Anna Jung, PharmD, BCPS

2013 ◽  
Vol 154 (39) ◽  
pp. 1535-1540 ◽  
Author(s):  
László Herszényi ◽  
Emese Mihály ◽  
Zsolt Tulassay

The effect of somatostatin on the gastrointestinal tract is complex; it inhibits the release of gastrointestinal hormones, the exocrine function of the stomach, pancreas and bile, decreases motility and influences absorption as well. Based on these diverse effects there was an increased expectation towards the success of somatostatin therapy in various gastrointestinal disorders. The preconditions for somatostatin treatment was created by the development of long acting somatostatin analogues (octreotide, lanreotide). During the last twenty-five years large trials clarified the role of somatostatin analogues in the treatment of various gastrointestinal diseases. This study summarizes shortly these results. Somatostatin analogue treatment could be effective in various pathological conditions of the gastrointestinal tract, however, this therapeutic modality became a part of the clinical routine only in neuroendocrine tumours and adjuvant treatment of oesophageal variceal bleeding and pancreatic fistulas. Orv. Hetil., 2013, 154, 1535–1540.


2018 ◽  
Vol 24 (6) ◽  
pp. 542-547 ◽  
Author(s):  
Mehrnaz Imani ◽  
Mohammad Ebrahim Khamseh ◽  
Poopak Asadi ◽  
Mohammad Ghorbani ◽  
Hamideh Akbari ◽  
...  

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