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E Monteiro

Publications and source records attributed to E Monteiro.

79 records · Page 5Linked to original sources
PubMedDate not supplied

[Activity of beta-N-acetylglucosaminidase in chronic alcoholic liver disease].

Serum N-acetyl-B-glucosaminidase (NAG) activity was determined in a control group of 76 normal people and in 23 patients with hepatic cirrhosis: 15 non-compensated patients and 8 with compensated chronic liver fibrosis. NAG is significantly increased in the non-compensated group--mean value of 931.67 units vs 637.71 units for the controls (p less than 0.001). There is no significant elevation regarding the compensated group. No significant correlations were observed between NAG activity and hepatic functional tests. In the follow-up of the non-compensated group, NAG activity returned to normal with the clinical improvement; in 7 cases where this goal was not achieved there was elevation of NAG activity, with higher values in photo-systemic encephalopathy.

Acetylglucosaminidase↗
PubMedDate not supplied

Liver transplantation for familial amyloid polyneuropathy.

Familiar Amyloid Polyneuropathy (FAP), an autosomal dominant inherited multisystemic disorder was first observed by Corino de Andrade, a Portuguese neurologist, in 1939. This disease of Portuguese origin was probably spread by fishermen, mainly to Sweden and Japan. It is characterized by a progressive peripheral polyneuropathy and autonomic neuropathy (erectile sexual disfunction, gastrointestinal disfunction, bladder dysfunction and cardio vascular disease) and malnutrition. There are neural and systemic amiloid deposits. Type I FAP, of Portuguese origin, is the most common variety. The amyloid protein is the variant transthyretin (TTR) in which methionine (MET) is a substitute for valine in position 30 (TTR MET 30). It is mainly produced by the liver (90%) and, in small amounts, by the choroidal plexus. Symptoms usually start in the 3rd and 4th decade of life and the patients usually die within 10-15 years. From the therapeutic options--plasmapheresis, immunoadsorption and liver transplantation; the latter seems to be the only one, which stops the production of TTR MET 30 in a permanent way, by means of the liver. The lack of any other effective therapy and the success of the first liver transplantation performed in Sweden arouse great hope. So far, around 300 patients have been transplanted all over the world. A hundred and thirty of them were transplanted in Portugal. A Kaplan Meier survival curve of the Portuguese patients shows a survival rate of 78% at 5 years. However, in spite of the progression of the disease being halted, the irreversibility of some neurological lesions seems to persist. This fact raises the problem of the timing of the transplantation. It seems that the patients should be transplanted as soon as the symptoms start, since mortality and severe morbidity seems to mainly involve those in whom symptomatic disease has lasted longer than six years. As the explanted liver is a morphologic normal liver, a sequential (domino) transplant has been carried out in 16 cases so far done--by one of the authors (ALF) on patients with either hepatocellular carcinoma or liver metastatic disease.

Adult↗