A Multicenter Experience with Long-Acting Somatostatin Analogues in Patients with Congenital Hyperinsulinism

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.

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Somatostatin and gastrointestinal tract. Clinical experiences

Orvosi Hetilap ◽

10.1556/oh.2013.29721 ◽

2013 ◽

Vol 154 (39) ◽

pp. 1535-1540 ◽

Cited By ~ 3

Author(s):

László Herszényi ◽

Emese Mihály ◽

Zsolt Tulassay

Keyword(s):

Gastrointestinal Tract ◽

Gastrointestinal Hormones ◽

Gastrointestinal Diseases ◽

Somatostatin Analogues ◽

Therapeutic Modality ◽

Exocrine Function ◽

Somatostatin Analogue ◽

Clinical Experiences ◽

Pancreatic Fistulas ◽

Long Acting

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.

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Aggravation of hypoglycemia in insulinoma patients by the long-acting somatostatin analogue octreotide (Sandostatin®)

Acta Endocrinologica ◽

10.1530/acta.0.1210034 ◽

1989 ◽

Vol 121 (1) ◽

pp. 34-40 ◽

Cited By ~ 48

Author(s):

C. D. A. Stehouwer ◽

W. F. Lems ◽

H. R. A. Fischer ◽

W. H. L. Hackeng ◽

M. A. B. Naafs

Keyword(s):

Blood Glucose ◽

Hormone Secretion ◽

Somatostatin Analogues ◽

Serum Glucose ◽

Somatostatin Analogue ◽

Transient Decrease ◽

Glucoregulatory Hormones ◽

Octreotide Therapy ◽

Long Acting ◽

Analogue Octreotide

Abstract. Recently somatostatin analogues were successfully used to control insulin-induced hypoglycemia in patients with insulinoma. We observed a transient decrease in glucose levels and symptomatic hypoglycemia after administration of the long-acting somatostatin analogue octreotide (Sandostatin®) in two insulinoma patients. We studied the acute effects of octreotide (administered before breakfast) on blood glucose and glucoregulatory hormones in these patients. In one patient, we studied the effects of glucagon replacement and changing the time of breakfast (relative to octreotide administration) on octreotide-associated changes in blood glucose and glucoregulatory hormones. Compared with control levels, octreotide therapy reduced insulin levels. During hypoglycemia glucagon and growth hormone levels were suppressed, but cortisol levels appropriately increased. The increase in catecholamine levels was normal in one patient, but markedly attenuated in the other. A transient decrease in serum glucose after octreotide was absent after glucagon replacement, but present when breakfast was taken before administration of octreotide. We conclude that in patients with insulinoma, octreotide therapy may be associated with clinically important hypoglycemia, during which counterregulatory hormone secretion may be attenuated.

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

Hormone Research in Paediatrics ◽

10.1159/000439131 ◽

2015 ◽

Vol 84 (5) ◽

pp. 355-360 ◽

Cited By ~ 12

Author(s):

Pratik Shah ◽

Sofia A. Rahman ◽

Sharon McElroy ◽

Clare Gilbert ◽

Kate Morgan ◽

...

Keyword(s):

Psychological Impact ◽

Somatostatin Analogue ◽

Congenital Hyperinsulinism ◽

Long Acting

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Clinical and pathophysiological aspects of somatostatin and the gastrointestinal tract

Acta Endocrinologica ◽

10.1530/acta.0.115s019 ◽

1987 ◽

Vol 116 (4_Suppla) ◽

pp. S19-S25 ◽

Cited By ~ 14

Author(s):

C. B.H.W. Lamers

Keyword(s):

Gastrointestinal Tract ◽

Gastrointestinal Haemorrhage ◽

Somatostatin Analogues ◽

Exocrine Secretion ◽

Somatostatin Analogue ◽

Cell Hyperplasia ◽

Secretory Diarrhoea ◽

D Cell ◽

Long Acting ◽

Clinical Conditions

Abstract. Somatostatin is present in the gastrointestinal tract in appreciable amounts. The highest concentrations of the polypeptide are found in the stomach, the upper small intestine, and the pancreas. Within the gastrointestinal tract, somatostatin inhibits various functions, including endocrine and exocrine secretion, motility, blood flow, absorption, and growth. The polypeptide regulates these functions by endocrine, paracrine, neurocrine or luminal mechanisms. Abnormalities of endogenous somatostatin have been implicated in several gastrointestinal disorders, including the somatostatinoma syndrome, antroduodenal D-cell hyperplasia, peptic ulcer, obesity, and liver cirrhosis. Because of its potent inhibitory effects, somatostatin or somatostatin-analogues have been used as therapeutic agents in various clinical conditions, such as upper gastrointestinal haemorrhage, endocrine pancreatic tumours, gastrointestinal and pancreatic fistulas, pancreatitis, secretory diarrhoea, and dumping syndrome. The recent availability of the synthetic long-acting somatostatin-analogue SMS 201-995 (Sandostatin) has greatly facilitated the therapeutical application of somatostatin-polypeptides.

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007–Long Acting Somatostatin Analogues: Early Experience in the Treatment of 5 patients with Congenital Hyperinsulinism

Journal of Pediatric Nursing ◽

10.1016/j.pedn.2015.01.006 ◽

2015 ◽

Vol 30 (2) ◽

pp. 428-429

Author(s):

Lisa Truong ◽

Paul Thornton

Keyword(s):

Early Experience ◽

Somatostatin Analogues ◽

Congenital Hyperinsulinism ◽

Long Acting

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Self-Administration of Long-Acting Somatostatin Analogues in NET Patients—Does It Affect the Clinical Outcome?

Medicina ◽

10.3390/medicina57121287 ◽

2021 ◽

Vol 57 (12) ◽

pp. 1287

Author(s):

Anna Sowa-Staszczak ◽

Marta Opalińska ◽

Anna Kurzyńska ◽

Karolina Morawiec-Sławek ◽

Aleksandra Gilis-Januszewska ◽

...

Keyword(s):

Medical Staff ◽

Somatostatin Receptor ◽

Locally Advanced ◽

Progression Free Survival ◽

Somatostatin Analogues ◽

Somatostatin Analogue ◽

Self Administration ◽

Good Expression ◽

Well Differentiated ◽

Long Acting

Background and Objectives: Long-acting somatostatin analogues (SSA) (octreotide LAR and lanreotide Autogel) are recommended as first line treatment of locally advanced or metastatic well-differentiated neuroendocrine tumors (NETs) with a good expression of somatostatin receptor (SSTR). Both of these SSAs are usually administered via injections repeated every 4 weeks. The purpose of the study was to compare the route of SSA administration (injection performed by professional medical staff and self-administration of the drug) with progression-free survival. Materials and methods: 88 patients in 2019 and 96 patients in 2020 with locally advanced or metastatic well-differentiated NETs were included in the study. All patients had a good expression of SSTR type 2 and had been treated for at least 3 months with a stable dose of long-acting somatostatin analogue every 4 weeks. All of them had received training on drug self-injections from professional NET nurses at the beginning of the COVID-19 epidemic. Results: The rate of NET progression in the study group in 2020 was higher than in 2019 29.1% vs. 18.1% (28 vs. 16 cases), p = 0.081. Conclusions: The method of administration of long-acting SSA injection performed by professional medical staff vs. self-injection of the drug may significantly affect the risk of NET progression. The unequivocal confirmation of such a relationship requires further observation.

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Long-acting somatostatin analogues in the treatment of children with congenital hyperinsulinism. Literature review

Pharmateca ◽

10.18565/pharmateca.2020.9.38-41 ◽

2020 ◽

Vol 9_2020 ◽

pp. 38-41

Author(s):

A.R. Savkina Savkina ◽

M.A. Kareva Kareva ◽

M.A. Melikyan Melikyan ◽

◽

Keyword(s):

Literature Review ◽

Somatostatin Analogues ◽

Congenital Hyperinsulinism ◽

Long Acting

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Hyperthyroidism due to non-tumoural inappropriate TSH secretion

Acta Endocrinologica ◽

10.1530/acta.0.1130042 ◽

1986 ◽

Vol 113 (1) ◽

pp. 42-46 ◽

Cited By ~ 10

Author(s):

Gareth Williams ◽

Marius Kraenzlin ◽

Laurence Sandier ◽

Jacky Burrin ◽

Adam Law ◽

...

Keyword(s):

Pituitary Tumour ◽

Somatostatin Analogues ◽

Molar Ratio ◽

Somatostatin Analogue ◽

Axial Tomography ◽

Α Subunit ◽

Tsh Secretion ◽

Long Acting ◽

Inappropriate Tsh Secretion ◽

Tsh Levels

Abstract. Inappropriate hypersecretion of TSH was investigated in a 25 year old man whose hyperthyroidism had relapsed 4 years after subtotal thyroidectomy. Serum TSH levels were further increased by both TRH and metoclopramide and were partially suppressed by triiodothyronine (120 μ/day). The serum α-subunit: TSH molar ratio was < 1.0, and computerised axial tomography showed no evidence of a pituitary tumour. These features are characteristic of inappropriate TSH secretion due to thyrotroph resistance to thyroid hormones. A long-acting somatostatin analogue (SMS 201-995), 50 μg injected sc twice-daily for three days, suppressed TSH levels and nearly normalised thyroid hormone levels. Somatostatin analogues may be therapeutically useful in thyrotoxicosis due to non-tumoural inappropriate TSH hypersecretion.

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Laboratory and clinical adverse events following initiation of dimethyl fumerate

Canadian Journal of Neurological Sciences / Journal Canadien des Sciences Neurologiques ◽

10.1017/cjn.2015.70 ◽

2015 ◽

Vol 42 (S1) ◽

pp. S9-S9

Author(s):

YM Al Malik ◽

J Greenfield ◽

W Wall ◽

LM Metz

Keyword(s):

Side Effects ◽

Adverse Events ◽

Real World ◽

Clinical Setting ◽

Liver Enzymes ◽

First Line Therapy ◽

Elevated Liver Enzymes ◽

Relapsing Remitting ◽

Treatment Naïve ◽

Relapsing Remitting Multiple Sclerosis

Background: Dimethyl fumerate (DF) is a first line therapy for relapsing remitting multiple sclerosis (RRMS). This retrospective cohort study aims to determine adverse events (AEs) after initiation of DF in a real world clinical setting. Methods: Data from patients at the Calgary MS Clinic with RRMS who initiated DF between July 1, 2013 and December 31, 2014 were analyzed. Demographic, clinical and lab information were collected from patient electronic medical records and the clinic database. Results: This analysis included 170 patients. At treatment initiation mean age was 42.1 years, 75% were women, mean disease duration was 12.5 years, median EDSS was 2.0, and 24% were treatment naïve. Median follow-up was 6.4 months (range: 1.5-17.7). AEs occurred in 101 (59%); the most common were flushing (31%), gastrointestinal (GI) side effects (24%), and elevated liver enzymes (18%). Other less frequent AEs included lymphopenia (lymphocyte count < 0.5) (4%) and proteinuria (4%). DF was discontinued by 17 (10%); median time to discontinuation was 3.1 months. Fifteen (9%) discontinued due to AE. Conclusions: AE associated with DF in a real world clinical setting is comparable to the Canadian monograph for flushing, GI side effects, and lymphopenia but lower for elevated liver enzymes and proteinuria.

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