Anesthetic management of a patient with maple syrup urine disease undergoing liver transplantation from living related donor
المؤلفون المشاركون
al-Shahrani, Muhannad
al-Dosri, Mubarak
Sierra, Plinio
المصدر
العدد
المجلد 43، العدد 2 (30 يونيو/حزيران 2021)، ص ص. 534-536، 3ص.
الناشر
تاريخ النشر
2021-06-30
دولة النشر
البحرين
عدد الصفحات
3
التخصصات الرئيسية
الملخص EN
Maple syrup disease is a rare metabolic condition affecting the pediatric population.
It is related to reduction or deficiency of branched chain a-ketoacid dehydrogenase (BCKD).
The mainstay of management is by strict dietary protein restriction with branch chain amino acid (BCAA) free diet along with supplementation of other essential amino acids and frequent monitoring of BCAA levels.
However, the approach should be through multidisciplinary team addressing dietary requirement, growth and psychomotor development.
This can be challenging due to compliance with such regimens as well as metabolic decompensations during physiological stress that can occur in fasting, surgery, infection and inflammation.
Liver transplantation has been shown to enable MSUD patients to have unrestricted diet and normal BCAA post-operatively.
Although MSUD is a rare disease, there is extensive literature published related to it from pediatrics, gastroenterology, hepatology and transplantation point of view.
We describe a case report of peri-operative management of 4 years old MSUD patient undergoing living related liver transplantation (LRLT) with normal BCAA levels post-operatively as this type of surgery is relatively recent in addition to the limited data on the peri-operative management of MSUD patients undergoing surgical procedures.
نمط استشهاد جمعية علماء النفس الأمريكية (APA)
al-Dosri, Mubarak& al-Shahrani, Muhannad& Sierra, Plinio. 2021. Anesthetic management of a patient with maple syrup urine disease undergoing liver transplantation from living related donor. Bahrain Medical Bulletin،Vol. 43, no. 2, pp.534-536.
https://search.emarefa.net/detail/BIM-1493293
نمط استشهاد الجمعية الأمريكية للغات الحديثة (MLA)
al-Dosri, Mubarak…[et al.]. Anesthetic management of a patient with maple syrup urine disease undergoing liver transplantation from living related donor. Bahrain Medical Bulletin Vol. 43, no. 2 (Jun. 2021), pp.534-536.
https://search.emarefa.net/detail/BIM-1493293
نمط استشهاد الجمعية الطبية الأمريكية (AMA)
al-Dosri, Mubarak& al-Shahrani, Muhannad& Sierra, Plinio. Anesthetic management of a patient with maple syrup urine disease undergoing liver transplantation from living related donor. Bahrain Medical Bulletin. 2021. Vol. 43, no. 2, pp.534-536.
https://search.emarefa.net/detail/BIM-1493293
نوع البيانات
مقالات
لغة النص
الإنجليزية
الملاحظات
Includes bibliographical references : p. 536
رقم السجل
BIM-1493293
قاعدة معامل التأثير والاستشهادات المرجعية العربي "ارسيف Arcif"
أضخم قاعدة بيانات عربية للاستشهادات المرجعية للمجلات العلمية المحكمة الصادرة في العالم العربي
تقوم هذه الخدمة بالتحقق من التشابه أو الانتحال في الأبحاث والمقالات العلمية والأطروحات الجامعية والكتب والأبحاث باللغة العربية، وتحديد درجة التشابه أو أصالة الأعمال البحثية وحماية ملكيتها الفكرية. تعرف اكثر