Fat oxidation defect presenting with overwhelming ketonuria
نویسندگان
چکیده
منابع مشابه
Ketonuria and Urinary Acidity.
The significance of acetone bodies in the urine subsequent to surgical operations has long been the cause of controversy and is not as yet settled. It is now well recognised that ketone bodies are not necessarily associated with acidosis. Although a ketosis usually accompanies an acidosis, the reverse is by no means the case. Despite the fact that a true acidosisa lepletion of the alkali reserv...
متن کاملChemical tests for ketonuria.
Variations in the concentration of taurine have been noted by several workers during chromatographic investigation of urinary amino-acids (Kay and Entenman, 1954; Souchon and Grunau, 1952; Ishihara, Komori, and lida, 1951 ; Nardi, 1954; Beerstecher, Sutton, Berry, Brown, Reed, Rich, Berry, and Williams, 1950). Taurine is present in high concentration in liver (Tallan, 1954), and it is in the li...
متن کاملDisrupted fat distribution and composition due to medium-chain triglycerides in mice with a β-oxidation defect.
BACKGROUND Because of the enhanced recognition of inherited long-chain fatty acid oxidation disorders by worldwide newborn screening programs, an increasing number of asymptomatic patients receive medium-chain triglyceride (MCT) supplements to prevent the development of cardiomyopathy and myopathy. OBJECTIVE MCT supplementation has been recognized as a safe dietary intervention, but long-term...
متن کاملKetonæmia and Ketonuria in Childhood.
The formation of ketone bodies and their excretion in the urine in albnormall amounts have for many years been problems of much interest to both physiologist and clinician. Much of our present knowledge of the subject has been gained from the study of carbohydrate metabolism in diabetes, where ketonuria in its classical formi is frequently observed. From this it has been established that an exc...
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ژورنال
عنوان ژورنال: Archives of Disease in Childhood
سال: 2002
ISSN: 0003-9888,1468-2044
DOI: 10.1136/adc.87.5.428