Aciduria glutárica tipo I con fenotipo bioquímico de baja excreción asociado a una nueva mutación / Glutaric aciduria type I with a low-excretion biochemical phenotype associated to a new mutation

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Subclinical hepatic encephalopathy predicts the development of overt hepatic encephalopathy.

OBJECTIVES: In patients with compensated liver cirrhosis the clinical repercussions of detecting subclinical hepatic encephalopathy (SHE) are unclear. We present a long-term follow-up study in cirrhotic patients to examine the relationship between SHE and subsequent episodes of overt hepatic encephalopathy. METHODS: A total of 63 cirrhotic patients were studied by Number Connection Test and auditory evoked potentials. We determined glutamine, ammonia, zinc, glutamate, urea, and ratio of branched chain amino acids to aromatic amino acids, and Child-Pugh classification. RESULTS: Of 63 patients, 34 (53%) exhibited SHE. Nineteen out of 63 (30%) developed overt hepatic encephalopathy during follow-up. Hepatic encephalopathy in follow-up was related to alcoholic etiology, ammonia, glutamine, zinc, ratio of branched chain amino acids to aromatic amino acids, liver function, presence of esophageal varices, and detection of SHE (84% of patients who exhibited hepatic encephalopathy in follow-up showed SHE). In Cox-regression, glutamine levels, SHE, esophageal varices, and Child-Pugh class were the independent variables related to hepatic encephalopathy in follow-up. CONCLUSIONS: SHE (defined on the basis of number connection test or auditory evoked potentials alteration) could predict a subsequent episode of overt hepatic encephalopathy. Lower glutamine levels, presence of esophageal varices, and liver dysfunction were also related to the development of overt hepatic encephalopathy.

[Hypocaloric peripheral parenteral nutrition in postoperative patients (the Europan Project) (II)]. / Estudio de la nutrición parenteral periférica hipocalórica en pacientes posquirúrgicos (proyecto Europan) (II).

Hypocaloric peripheral parenteral nutrition (HPPN) appears to be indicated in patients in a situation of moderate malnutrition who are to undergo a short period of fasting following surgery. Our aim was to determine the utility of the contribution of parenteral solutions of amino acids (AA) with limited caloric supply in the post-surgical patient, using different nutritional evaluation parameters. We examined 75 post-surgical patients who met at least two of the three criteria established as malnutrition: 1) albumin < 3 g/dl; 2) pre-albumin < 21 mg/dl; 3) bodyweight of less than 95% of the ideal weight. They were divided into four groups: a control group, of 15 patients undergoing standard fluid therapy; Group I, 20 patients with nutritional support of glucose +AA; Group II, 20 patients with glycerol +AA; and Group III of 20 patients with sorbitol-xylitol +AA. The most significant data encountered were a rapid recovery of short half-life proteins (pre-albumin and retinol), a less negative nitrogen balance, and a greater decrease of urinary 3-methylhistidine, when HPPN was used. A notable increase was also obtained in the majority of AAs and of the G and M immunoglobulin plasmatic figures in the groups treated. In terms of complications, a greater percentage of wound dehiscences appeared in the control group than in those treated (13.3 vs 5%) while, on the other hand, there was a higher incidence of catheter-induced phlebitis in groups undergoing HPPN. We conclude that HPPN is a valid nutritional support in post-surgical patients with more or less significant malnutrition, and when the gastro-intestinal tract cannot be used, for whatever reason, during the first week following the operation.