Octreotide-Induced Long QT Syndrome in a Child with Congenital Hyperinsulinemia and a Novel Missense Mutation (p.Met115Val) in the ABCC8 Gene

Çelik, Nurullah; Cınaz, Peyami; Emeksiz, Hamdi Cihan; Hussain, Khalid; Çamurdan, Orhun; Bıdecı, Aysun; Döğer, Esra; Yüce, Özge; Türkyılmaz, Zafer; Aydın, Oğuz

doi:10.1159/000354666

Cited by 15 publications

(11 citation statements)

References 23 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Pericardial and pleural effusions and wound-healing complications reported for a mammalian-target-ofrapamycin inhibitor after cardiac transplantation, but we didn't see these complications (6) . A prolonged QT interval has been observed with Octreotide use (7) , but we didn't see this complication.…”

Section: Discussioncontrasting

confidence: 53%

Successful anesthetic management of a 14 year old boy undergoing tricuspid valvular surgery for carcinoid heart disease

Fırat¹,

Özkan²,

İlkay³

et al. 2019

JARSS

View full text Add to dashboard Cite

Objective:We present our strategy for successful management of pediatric patients with carcinoid heart disease when weaning from cardiopulmonary bypass (CPB) during tricuspid valve surgery. Case Presentation: The patient was a 14-year-old boy who had been diagnosed with a grade IV neuroendocrine tumor. The patient was premedicated with oral midazolam, hydroxyzine, somatostatin analogue (Octreotide®), and pheniramine maleate. Octreotide® and serotonin 5-hydroxytryptamine receptor antagonist were also added during the rewarming period. Dopamine was continued as an infusion (5 µg kg -1 min -1 ) for the first day. Bioprosthetic valve placement and transannular right ventricular outflow tract patch augmentation were performed. He was extubated within 20 hours and discharged from the hospital at postoperative 6 day. Discussion: Thanks to the premedication of this case and the addition of somatostatin analogue, pheniramine maleate and methylprednisolone to the pump reservoir and avoiding agents such as atracurium, morphine and meperidine, which may cause histamine release, the surgery was accomplished have been accomplished without development of carcinoid crisis. ÖZAmaç: Bu olgu sunumunda karsinoid kalp tutulumu nedeniyle triküspid kapak replasmanı yapılan çocuk hastada kardiyopulmoner baypastan (KPB) ayrılma sırasında uygulanan strateji anlatılmaktadır. Olgu Sunumu: On dört yaşında olan hastamızın evre IV karaciğer metastazı olan nöroendokrin tümörü vardı. Hastaya premedikasyon amacıyla peroral verilen midazolam ve hidroksizinin yanına somatostatin analogu (Octreotid®) ve feniramin maleat ilave edildi. Octreotid® ve 5-hidroksitriptamin reseptör antagonisti aynı zamanda pompa rezervuarına da yapıldı ve ısınma döneminde doz yinelendi. Triküspid kapak yerine biyolojik kapak yerleştirildi ve pulmoner çıkıma krosanüler yama koyuldu. Postoperatif ilk gün 5 µkg -1 dk -1 dopamin infüzyonu devam etti. Cerrahi sonrası 20. saatte ekstübe edilen hasta, postoperatif 6. gün taburcu oldu. Tartışma: Bu olgunun premedikasyonuna ve pompa rezervuarına somatostatin analogu, feniramin maleat ve metilprednizolon ilave edilmesi ve histamin salımınına neden olabilecek atrakuryum, morfin ve meperidin gibi ajanlardan kaçınılması ile karsinoid kriz gelişmeden cerrahinin tamamlanması sağlanmıştır.Anahtar kelimeler: Karsinoid kalp hastalığı, karsinoid kriz, kardiyopulmoner baypas, anestezi, paraneoplastik sendrom Alındığı tarih: 23.11.2018

show abstract

Section: Discussioncontrasting

confidence: 53%

Successful anesthetic management of a 14 year old boy undergoing tricuspid valvular surgery for carcinoid heart disease

Fırat¹,

Özkan²,

İlkay³

et al. 2019

JARSS

View full text Add to dashboard Cite

show abstract

“…Rarer but more serious side effects include hepatitis [20], NEC [21] and long QT syndrome [22]. NEC is a devastating gastrointestinal disease among neonates with high morbidity and mortality [23].…”

Section: Discussionmentioning

confidence: 99%

Experience of Octreotide Therapy for Hyperinsulinemic Hypoglycemia in Neonates Born Small for Gestational Age: A Case Series

Pan¹,

Zhang²,

Li³

2015

Horm Res Paediatr

View full text Add to dashboard Cite

Aims: Hyperinsulinemic hypoglycemia (HH) is common in small-for-gestational-age (SGA) neonates. Diazoxide is often used as the first-line medication for HH in SGA neonates. Unfortunately, diazoxide is not authorized in China. We examined the effectiveness of octreotide as an alternative therapy to treat HH in SGA neonates. There is limited data on the use of octreotide in HH of SGA neonates. Methods: Seven SGA neonates with HH who were admitted to the Department of Neonatology at the Third Affiliated Hospital of Sun Yat-sen University between January 2013 and December 2014 received octreotide at an initial dose of 5 μg/kg/day through subcutaneous injection at 8-hour intervals. Depending on the glycemic control, the dose of octreotide was increased in increments of 2-5 μg/kg/day every 3-5 days to the maximum dose of 30 μg/kg/day. Results: The age of neonates with HH diagnosis ranged from 1 to 4 days. The maximum dose of octreotide ranged from 8 to 18 μg/kg/day. The duration of octreotide therapy ranged from 9 to 45 days. All patients had a clear glycemic response to octreotide, and no major adverse events were observed during the treatment. Conclusions: Octreotide may be a useful alternative therapy in HH of SGA neonates when diazoxide is unavailable.

show abstract

“…Common adverse events include gastrointestinal symptoms, white stool, dilated gall bladder with or without gall stones, and growth deceleration after 2 years of age. Rarer, but more serious side effects, include hepatitis70), necrotizing enterocolitis71) and long QT syndrome72).…”

Section: Current Treatment Strategiesmentioning

confidence: 99%

Congenital hyperinsulinism: current status and future perspectives

Yorifuji

2014

Ann Pediatr Endocrinol Metab

147

View full text Add to dashboard Cite

The diagnosis and treatment of congenital hyperinsulinism (CHI) have made a remarkable progress over the past 20 years and, currently, it is relatively rare to see patients who are left with severe psychomotor delay. The improvement was made possible by the recent developments in the understanding of the molecular and pathological basis of CHI. Known etiologies include inactivating mutations of the KATP channel genes (ABCC8 and KCNJ11) and HNF4A, HNF1A, HADH, and UCP2 or activating mutations of GLUD1, GCK, and SLC16A1. The understanding of the focal form of KATP channel CHI and its detection by 18F-fluoro-L-DOPA positron emission tomography have revolutionized the management of CHI, and many patients can be cured without postoperative diabetes mellitus. The incidence of the focal form appears to be higher in Asian countries; therefore, the establishment of treatment systems is even more important in this population. In addition to diazoxide or long-term subcutaneous infusion of octreotide or glucagon, long-acting octreotide or lanreotide have also been used successfully until spontaneous remission. Because of these medications, near-total pancreatectomy is less often performed even for the diazoxide-unresponsive diffuse form of CHI. Other promising medications include pasireotide, small-molecule correctors such as sulfonylurea or carbamazepine, GLP1 receptor antagonists, or mammalian target of rapamycin inhibitors. Unsolved questions in this field include the identification of the remaining genes responsible for CHI, the mechanisms leading to transient CHI, and the mechanisms responsible for the spontaneous remission of CHI. This article reviews recent developments and hypothesis regarding these questions.

show abstract

Octreotide-Induced Long QT Syndrome in a Child with Congenital Hyperinsulinemia and a Novel Missense Mutation (p.Met115Val) in the ABCC8 Gene

Cited by 15 publications

References 23 publications

Successful anesthetic management of a 14 year old boy undergoing tricuspid valvular surgery for carcinoid heart disease

Successful anesthetic management of a 14 year old boy undergoing tricuspid valvular surgery for carcinoid heart disease

Experience of Octreotide Therapy for Hyperinsulinemic Hypoglycemia in Neonates Born Small for Gestational Age: A Case Series

Congenital hyperinsulinism: current status and future perspectives

Contact Info

Product

Resources

About