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Biomedical subjects

C A Pierach

Publications and source records attributed to C A Pierach.

At least 73 records · Page 4Linked to original sources
PubMed1977

Postulated deficiency of hepatic heme and repair by hematin infusions in the "inducible" hepatic porphyrias.

There is compelling, indirect evidence of hepatic heme deficiency due primarily to the respective genetic errors of the three inducible hepatic porphyrias, acute intermittent porphyria, porphyria variegata, and hereditary coproporphyria. The induction is enhanced by exogenous inducers such as barbiturate, estrogens and other "porphyrogenic" chemicals and factors, including glucose deprivation. The newer knowledge of the induction of delta-aminolevulinic acid synthetase [delta-aminolevulinate synthase; succinyl--CoA:glycine C-succinyltransferase (decarboxylating), EC 2.3.1.37] in relation to inadequate heme, and repression by heme, stimulated early trials of hematin infusions to overcome the acute relapse in the foregoing inducible porphyrias. Recently this experience has been considerably expanded, 143 infusions of hematin having been given in 22 cases. Studies of the effect on the serum concentrations of delta-aminolevulinic acid and porphobilinogen have shown a highly significant decline, often to 0, especially of delta-aminolevulinic acid. A distinct relationship to the clinical severity of the attack has been evident in the frequency and magnitude of decline of serum delta-aminolevulinic acid and porphobilinogen. This was regularly associated with objective clinical improvement.

5-Aminolevulinate Synthetase↗
PubMed1977

Effect of hematin in porphyric neuropathy.

Early, intravenous administration of hematin in a patient with acute intermittent porphyria and severe quadriparesis may have produced partial but remarkable improvement of neuropathy, and resulted in simultaneous decline of porphyrin precursors in the blood. Intermittent, biweekly hematin infusions given 1 month after the onset of the porphyric relapse had no effect on recovery of the residual neuropathy. We believe hematin may be effective in the treatment of porphyric neuropathy, if administered before irreversible neuronal damage has occured.

Adult↗
PubMed1977

Comparison of the Hoesch and the Watson-Schwartz tests for urinary porphobilinogen.

A comparison of the Hoesch and the Watson-Schwartz tests shows that the latter, although slightly more complicated, generally yields more concise results and is superior in sensitivity and specificity for porphobilinogen. The recommendation of the Hoesch test for use as a "bedside screening" method seems unrealistic. Before the diagnosis of an "inducible" porphyria is made, a positive Hoesch test requires that indoles, indoleacetic acid, methyldopa, end-stage alcoholic malnutrition, and phenazopyridine HCl be excluded, to avoid misinterpretation.

Chromatography, Thin Layer↗
PubMed1976

[Electrocardiographic changes after surgical manipulation of the carotid arteries (author's transl)].

Seven patients showed deep symmetrical T wave inversions in their electrocardiograms after bilateral carotid endarterectomy. There was no evidence for myocardial ischaemia. These changes reversed completely within three months. Similar disturbances of repolarisation were seen in rats after bilateral denervation of the carotid sinus. These changes are probably of neurogenic origin, like those seen with central nervous system disorders. Their origin seems to be correlated to the acute imbalance of the autonomic innervation of the heart after surgical disturbance of baro- and chemoreceptors.

Aged↗
PubMed1975

[Haemolysis after heart valve replacement (author's transl)].

Intravascular haemolysis is frequently seen in patients after heart valve replacement, but is often compensated. The cause of the haemolysis is traumatic damage of the red cells. It is more often seen after aortic, than after mitral replacement, because the transvalvular pressure gradient is higher and is more severe in ball valves than in disc valves. If substitution therapy with iron, folic acid, or even red cells fails, re-operation should be seriously considered.

Anemia, Hemolytic↗
PubMed1975

Severe hemolysis with a fabric-worn cloth-covered aortic valve prosthesis.

A patient developed severe hemolytic anemia one year after insertion of a cloth-covered aortic valve prosthesis (Starr-Edwards No. 2320). The cloth over the three struts was disrupted but showed coverage with mostly organized collagen. Hemolysis stopped after replacement with a porcine heterograft. Fabric wear seems to augment the hemolysis in patients with cloth-covered artificial valves.

Adult↗