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

Pharmateca ◽  
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

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

2017 ◽  
Vol 89 (2) ◽  
pp. 82-89 ◽  
Author(s):  
Ivo van der Steen ◽  
Mirjam E. van Albada ◽  
Klaus Mohnike ◽  
Henrik Thybo Christesen ◽  
Susann Empting ◽  
...  
Keyword(s):  
Adverse Effect ◽  
Side Effects ◽  
Liver Enzymes ◽  
Blood Glucose Control ◽  
Severe Hypoglycemia ◽  
Somatostatin Analogues ◽  
Somatostatin Analogue ◽  
Congenital Hyperinsulinism ◽  
Elevated Liver Enzymes ◽  
Long Acting

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.

scholarly journals Hepatoblastoma and Wilms’ tumour in an infant with Beckwith–Wiedemann syndrome and diazoxide resistant congenital hyperinsulinism

2019 ◽  
Vol 2019 ◽  
Author(s):  
Saurabh Uppal ◽  
James Blackburn ◽  
Mohammed Didi ◽  
Rajeev Shukla ◽  
James Hayden ◽  
...  
Keyword(s):  
Surgical Resection ◽  
Poor Response ◽  
Somatostatin Analogues ◽  
Early Infancy ◽  
Congenital Hyperinsulinism ◽  
List Type ◽  
Wilms Tumour ◽  
Intravenous Glucose ◽  
Long Acting ◽  
First Time

Summary Beckwith–Wiedemann syndrome (BWS) can be associated with embryonal tumours and congenital hyperinsulinism (CHI). We present an infant with BWS who developed congenital hepatoblastoma and Wilms’ tumour during infancy. The infant presented with recurrent hypoglycaemia requiring high intravenous glucose infusion and was biochemically confirmed to have CHI. He was resistant to diazoxide but responded well to octreotide and was switched to Lanreotide at 1 year of age. Genetic analysis for mutations of ABCC8 and KCNJ11 were negative. He had clinical features suggestive of BWS. Methylation-sensitive multiplex ligation-dependent probe amplification revealed hypomethylation at KCNQ1OT1:TSS-DMR and hypermethylation at H19 /IGF2:IG-DMR consistent with mosaic UPD(11p15). Hepatoblastoma was detected on day 4 of life, which was resistant to chemotherapy, requiring surgical resection. He developed Wilms’ tumour at 3 months of age, which also showed poor response to induction chemotherapy with vincristine and actinomycin D. Surgical resection of Wilms’ tumour was followed by post-operative chemotherapy intensified with cycles containing cyclophosphamide, doxorubicin, carboplatin and etoposide, in addition to receiving flank radiotherapy. We report, for the first time, an uncommon association of hepatoblastoma and Wilms’ tumour in BWS in early infancy. Early onset tumours may show resistance to chemotherapy. UPD(11p15) is likely associated with persistent CHI in BWS. Learning points: Long-acting somatostatin analogues are effective in managing persistent CHI in BWS. UPD(11)pat genotype may be a pointer to persistent and severe CHI. Hepatoblastoma and Wilms’ tumour may have an onset within early infancy and early tumour surveillance is essential. Tumours associated with earlier onset may be resistant to recognised first-line chemotherapy.

scholarly journals The Dose of Somatostatin Analogues during Pre-Surgical Treatment Is a Key Factor to Achieve Surgical Remission in Acromegaly

Endocrines ◽  
2021 ◽  
Vol 2 (3) ◽  
pp. 241-250
Author(s):  
Marta Araujo-Castro ◽  
Eider Pascual-Corrales ◽  
Héctor Pian ◽  
Ignacio Ruz-Caracuel ◽  
Alberto Acitores Cancela ◽  
...  
Keyword(s):  
Retrospective Study ◽  
Surgical Treatment ◽  
Surgical Outcomes ◽  
Pituitary Adenomas ◽  
Somatostatin Analogues ◽  
Beneficial Effects ◽  
Key Factor ◽  
Long Acting ◽  
First Time ◽  
Endocrine Complications

Purpose: to determine whether pre-surgical treatment using long-acting somatostatin analogues (SSAs) may improve surgical outcomes in acromegaly. Methods: retrospective study of 48 patients with acromegaly operated by endoscopic transsphenoidal approach and for first time. Surgical remission was evaluated based on the 2010 criteria. Results: most patients, 83.3% (n = 40), harbored macroadenomas and 31.3% (n = 15) invasive pituitary adenomas. In this case, 14 patients were treated with lanreotide LAR and 6 with octreotide LAR, median monthly doses of 97.5 [range 60–120] and 20 [range 20–30] mg, respectively, for at least 3 months preoperatively. Presurgical variables were comparable between pre-treated and untreated patients (p > 0.05). Surgical remission was more frequent in those pre-treated with monthly doses ≥90 mg of lanreotide or ≥30 mg of octreotide than in untreated or pre-treated with lower doses (OR = 4.64, p = 0.025). However, no differences were found between pre-treated and untreated patients when lower doses were included or between those treated for longer than 6 months compared to those untreated or pre-treated for shorter than 6 months. Similarly, no differences were found either in terms of surgical or endocrine complications (OR = 0.65, p = 0.570), independently of the doses and the duration of SSA treatment (p > 0.05). Conclusions: the dose of SSAs is a key factor during pre-surgical treatment, since the beneficial effects in surgical remission were observed with monthly doses equal or higher than 90 mg of lanreotide and 30 mg of octreotide, but not with lower doses.

scholarly journals Prescription patterns of long-acting somatostatin analogues

2017 ◽  
Vol 5 ◽  
pp. 205031211769479 ◽  
Author(s):  
Jorge Enrique Machado-Alba ◽  
Manuel Enrique Machado-Duque
Keyword(s):  
Somatostatin Analogues ◽  
Prescription Patterns ◽  
Long Acting

scholarly journals Somatostatin and gastrointestinal tract. Clinical experiences

2013 ◽  
Vol 154 (39) ◽  
pp. 1535-1540 ◽  
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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