Abstract
1) clinical and biological tolerances were good (no hepatic nor cerebral abnormality); cutaneous xanthomas slowly decreased.
2) cholesterolemia decreased (pre-operative levels: respectively 20.6 - 23.2 and 25.8 mmo1/1; post-operative levels: 10.3, 15.5 and 12.9 mmo1/1.
3) insulin and glucagon levels progressively increased (present basal insulin level: 20 μU/ml, glucagon: 500 pg/ml in case 1);results were normal in the 3rd case 2 months after shunt (insulin: 6 μU/ml, glucagon: 34 pg/ml).
4)in case 2, PCS thrombosed during the post-operative period; 18 months later a mesoiliac shunt was as efficient as PCS.
5) in cases 1 and 2 coronarography showed no change of coronary lesions, however; stabilization might be considered as favourable.
6) a diet low in lipids and cholesterol, inefficient before PCS became indispensable in the post-operative period. An increase of 30% of blood cholesterol occurred when the patient returned on a free diet. Continuous noctural gastric drip feeding (with a follow-up of 12 months in case 1) did not give better results than a well controlled diet.
7) a decrease of blood cholesterol during total parenteral feeding in the pre-operative period was a good index of the success of the PCS in the 3 cases.
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Farriaux, J., Ribet, M., Dhondt, J. et al. Portacaval shunt (PCS) in type IIa hypercholesterolemia; results from three cases: 21. Pediatr Res 14, 169 (1980). https://doi.org/10.1203/00006450-198002000-00048
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DOI: https://doi.org/10.1203/00006450-198002000-00048