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C A Pierach

Publications and source records attributed to C A Pierach.

At least 19 recordsLinked to original sources
PubMed1992

And nor are we.

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Clinical Competence↗
PubMed1991

Bile porphyrin analysis in the evaluation of variegate porphyria.

BACKGROUND: Variegate porphyria is a genetic disorder of porphyrin metabolism in which patients may have both neurologic dysfunction and photocutaneous lesions. Biochemical confirmation of the diagnosis can be difficult, particularly in patients without neurologic dysfunction at the time of testing. The demonstration of increased fecal excretion of porphyrin is frequently used for this purpose, but levels may be normal. Since elevated fecal porphyrin levels in variegate porphyria are presumably a consequence of increased biliary excretion, we evaluated whether analysis of porphyrins in bile distinguishes better between patients with variegate porphyria and controls. METHODS: Bile samples were collected by duodenal aspiration from 10 patients with proved variegate porphyria who had no neurologic symptoms when they were studied and 17 control subjects. Bile and fecal porphyrin levels were measured fluorometrically. RESULTS: The mean total porphyrin concentration in bile in the patients with variegate porphyria was significantly higher than that in the controls (67.8 vs. 0.71 mumol per liter; P less than 0.00002). There was more than a ninefold difference between the highest level in any control subject and the lowest level in any patient with variegate porphyria. The mean fecal porphyrin level in the patients with variegate porphyria also differed significantly from that in the controls (0.79 vs. 0.14 mumol per gram of dry weight; P less than 0.007), but four patients had levels within the control range. CONCLUSIONS: The concentration of porphyrin in bile is higher in patients with variegate porphyria than in controls, and the difference is greater than that for fecal porphyrin. Bile porphyrin measurements may be helpful in the evaluation of asymptomatic patients suspected of having variegate porphyria.

Adolescent↗
PubMed1988

Relation between serum digoxin concentration and the electrocardiogram.

The relation between serum digoxin concentration and the electrocardiogram was assessed by correlating computerized measurements of electrocardiographic parameters (PR, QRS, QT and QTc intervals, ST segment, and T-wave amplitude) with the serum digoxin concentration in 97 patients on digoxin maintenance therapy and in 40 nondigitalized control subjects. None of the patients had unstable ischemic heart disease, electrolyte disorders, medication known to influence the ST segment, and/or the presence of a bundle-branch block or ventricular hypertrophy. We found a trend toward lengthened PR interval and shortened QT and QTc intervals in digitalized versus nondigitalized patients. Increasing serum digoxin concentrations were associated with progressive depression of the ST segment and decreased T-wave amplitude (p less than 0.001). A normal ST segment in four leads (I, aVF, V5, V6) excluded the presence of a serum digoxin concentration greater than 1.3 ng/ml in our patients, whereas severe ST-segment depression with a J point of greater than or equal to 100 microV was a strong indicator for the presence of a serum digoxin concentration greater than 2.0 ng/ml in our selected patient population (specificity 99%, sensitivity 30%, predictive accuracy 85%). We conclude that computerized electrocardiographic analysis of the ST segment may provide clinically useful information for the management of selected patients on digitalis therapy and may therefore increase the diagnostic yield of the electrocardiogram in predicting the presence of higher serum digoxin concentrations in a small but significant percentage of patients.

Adult↗
PubMed1979

Electrocardiographic changes after bilateral carotid sinus denervation in the rat.

The effect of bilateral carotid sinus denervation (CSD) on the ECG and on the monophasic ventricular action potential (MAP) was studied in 28 and 4 rats, respectively. After CSD, T wave changes, similar to those seen in man after carotid endarterectomy, were observed and mean Q-T prolongations of 19 ms were recorded (P less than 0.001). Mean MAP increased by 10 ms (P less than 0.02). P-QRS and heart rate remained stable. Propranolol, 10 mg/kg iv, before CSD and 10 mg.kg-1.day-1 iv for 2 days prevented all ECG abnormalities. Atropine, 1 mg/kg iv, before CSD and 2.5 mg.kg-1.day-1 iv for 2 days had no effect. Isoproterenol, 0.02-0.06 microgram iv, after CSD produced further lengthening of the MAP and Q-T interval. Blood gases and electrolytes remained unchanged, and cardiac histology was unremarkable. These results suggest that CSD produces alterations in cardiac sympathetic activity in the rat leading to MAP and Q-T prolongations and to changes in the T wave form. Similar mechanisms may be operative after carotid endarterectomy in man.

Action Potentials↗