Neonatal Nutritional Carnitine Deficiency: A Piglet Model

نویسندگان
چکیده

برای دانلود باید عضویت طلایی داشته باشید

برای دانلود متن کامل این مقاله و بیش از 32 میلیون مقاله دیگر ابتدا ثبت نام کنید

اگر عضو سایت هستید لطفا وارد حساب کاربری خود شوید

منابع مشابه

The neonatal piglet as a model for human neonatal carnitine metabolism.

Investigations concerning carnitine metabolism and possible requirements for exogenous carnitine in human preterm neonates are limited by ethical considerations. The neonatal piglet is a potential animal model for these investigations. Tissue carnitine concentrations were determined in fetuses from cross-bred domestic gilts at stages of gestation corresponding to those of neonates found in neon...

متن کامل

Carnitine palmitoyltransferase II deficiency

The authors investigated 32 patients with the muscle form of CPT II deficiency. Total carnitine palmitoyltransferase enzyme system (CPT) activity was normal but abnormally inhibited by malonyl-CoA, palmitoyl-CoA, and the detergents Triton X and Tween 20. Mutation analysis identified three described mutations (S113L, P50H, and F448L) and two novel mutations (M214T and Y479F). Using modeling tech...

متن کامل

Carnitine Deficiency and Pregnancy

We present two cases of carnitine deficiency in pregnancy. In our first case, systematic screening revealed L-carnitine deficiency in the first born of an asymptomatic mother. In the course of her second pregnancy, maternal carnitine levels showed a deficiency as well. In a second case, a mother known with carnitine deficiency under supplementation was followed throughout her pregnancy. Both pr...

متن کامل

A surviving 24-month-old patient with neonatal-onset carnitine palmitoyltransferase II deficiency

The early-onset form of carnitine palmitoyltransferase (CPT) II deficiency has severe outcomes; patients typically die during the newborn period. We report a case of neonatal-onset CPT II deficiency with prolonged survival, exceeding 24 months. The patient was successfully treated by continuous hemodialysis (CHD), which enabled her to overcome repeated crises. We suggest that early intensive tr...

متن کامل

Gluconeogenesis in Neonatal Piglet Liver 1

The gluconeogenic capacity of livers from day old and 5-day-old piglets has been studied. The data show infused piglets and perfused livers convert lactate to glucose. Studies with isolated liver mitochondria show a large capacity to flux pyruvate through the pyruvate carboxylase reaction causing an accumulation of malate, citrate and phosphoenolypyruvate. Factors that affect accumulation of ma...

متن کامل

ذخیره در منابع من


  با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید

ژورنال

عنوان ژورنال: Pediatric Research

سال: 1997

ISSN: 0031-3998,1530-0447

DOI: 10.1203/00006450-199707000-00018