Abstract
Electron microscopical (EM) and lipid histochemical studies have been made of liver biopsies from 3 abetalipoproteinemic (AB-LP) and 2 hypobetalipoproteinemic (HypoB-LP) children. All AB-LP livers were enlarged and grossly fatty (38% lipid, 91% triglyceride [TG] ). By EM, endogenous tri-glyceride particles were absent. Trans-Golgi vesicles and circum-Golgi smooth endoplasmic reticulum were absent. Most lipid vacuoles were non-membrane-bounded. This Golgi lesion has not been seen in other childhood fatty livers. One AB-LP infant was biopsied at 10, 12, 24, 30 and 38 months, before, during and after treatment with a low-fat MCT diet. During the interval of observation, he had persistent elevation of transaminases and he developed severe micronodular cirrhosis. Alcoholic hyalin (AH) appeared in hepatocytes after MCT treatment; mitochondrial dense bodies increased in size and density. Eight months after MCT was stopped, AH was greatly reduced and mitochondrial dense bodies normalized. It is speculated the alcoholic hyalin was related to accumulation of acetate from MCT-fatty acids in the face of defective TG secretion in a manner biochemically analogous to hepatic ethanol metabolism. MCT should be used cautiously in AB-LP infants until more data is available. Support: NIH #RR-123
Article PDF
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
Partin, J., Partin, J. & Schubert, W. MICRONODULAR CIRRHOSIS IN ABETALIPOPROTEINEMIA: POSSIBLE EXACERBATION BY MEDIUM CHAIN TRIGLYCERIDE (MCT) FEEDING. Pediatr Res 8, 384 (1974). https://doi.org/10.1203/00006450-197404000-00264
Issue Date:
DOI: https://doi.org/10.1203/00006450-197404000-00264