Portacaval shunt in siblings for type I glycogenosis.
The paper reports two siblings with type I glycogenosis, presenting supportive laboratory data before and after 1 mo of central venous nutrition and later after surgical portal diversion. Both children dramatically improved with central venous nutrition, allowing safe and technically easy portacaval shunts to be constructed. Their smooth postoperative courses are documented. Currently, both patients are at home pursuing relatively normal lives.
D002648:Child; D002675:Child, Preschool; D005260:Female; D005953:Glycogen Storage Disease Type I / Q000178:diet therapy / Q000235:genetics / Q000601:surgery*; D006801:Humans; D007223:Infant; D008099:Liver / Q000473:pathology; D008297:Male; D010288:Parenteral Nutrition; D010289:Parenteral Nutrition, Total; D011167:Portacaval Shunt, Surgical; D012279:Rickets / Q000150:complications