Serbian
Albanian
Arabic
Armenian
Azerbaijani
Belarusian
Bengali
Bosnian
Catalan
Czech
Danish
Deutsch
Dutch
English
Estonian
Finnish
Français
Greek
Haitian Creole
Hebrew
Hindi
Hungarian
Icelandic
Indonesian
Irish
Italian
Japanese
Korean
Latvian
Lithuanian
Macedonian
Mongolian
Norwegian
Persian
Polish
Portuguese
Romanian
Russian
Serbian
Slovak
Slovenian
Spanish
Swahili
Swedish
Turkish
Ukrainian
Vietnamese
Български
中文(简体)
中文(繁體)
Journal of Clinical Endocrinology and Metabolism 2016-Dec

mTOR Inhibitors for the Treatment of Severe Congenital Hyperinsulinism: Perspectives on Limited Therapeutic Success.

Само регистровани корисници могу преводити чланке
Пријави се / Пријави се
Веза се чува у привремену меморију
Marie Szymanowski
Maria Salomon Estebanez
Raja Padidela
Bing Han
Karolina Mosinska
Adam Stevens
Lena Damaj
Florence Pihan-Le Bars
Emilie Lascouts
Rachel Reynaud

Кључне речи

Апстрактан

Congenital hyperinsulinism (CHI) is the most common cause of persistent hypoglycemia in neonates and infants. In medically unresponsive CHI, subtotal pancreatectomy is performed to achieve euglycemia with consequent diabetes in later life. Sirolimus, a mammalian target of rapamycin (mTOR) inhibitor, has been reported to obviate the need for pancreatectomy, but experience is limited.

We have investigated the efficacy and adverse effect profile of mTOR inhibitors in the treatment of severe CHI.

This was an observational review of 10 severe CHI patients treated with mTOR inhibitors, in France and the United Kingdom, with the intention of achieving glycemic control without pancreatectomy. Safety information was recorded.

We examined whether mTOR inhibitors achieved glycemic control, fasting tolerance, and weaning of supportive medical therapy.

mTOR inhibition achieved euglycemia, fasting tolerance, and reduced medical therapy in only three patients (30%). Triglyceride levels were elevated in five patients (50%). One child required a blood transfusion for anemia, four had stomatitis, two had sepsis, one developed varicella zoster, and two patients developed gut dysmotility in association with exocrine pancreatic insufficiency. In silico analysis of transcriptome arrays from CHI patients revealed no significant association between mTOR signaling and disease. Pancreatic tissue from two patients who did not respond to sirolimus showed no reduction in cell proliferation, further suggesting that mTOR signaling did not down-regulate proliferation in the CHI pancreas.

mTOR inhibitor treatment is associated with very limited success and must be used with caution in children with severe CHI.

Придружите се нашој
facebook страници

Најкомплетнија база лековитог биља подржана науком

  • Ради на 55 језика
  • Биљни лекови потпомогнути науком
  • Препознавање биљака по слици
  • Интерактивна ГПС мапа - означите биље на локацији (ускоро)
  • Читајте научне публикације повезане са вашом претрагом
  • Претражите лековито биље по њиховим ефектима
  • Организујте своја интересовања и будите у току са истраживањем вести, клиничким испитивањима и патентима

Упишите симптом или болест и прочитајте о биљкама које би могле да помогну, укуцајте неку биљку и погледајте болести и симптоме против којих се користи.
* Све информације се заснивају на објављеним научним истраживањима

Google Play badgeApp Store badge