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Biomedical subjects

A Langslet

Publications and source records attributed to A Langslet.

At least 19 recordsLinked to original sources

A new syndrome: thrombocytopathia, muscle fatigue, asplenia, miosis, migraine, dyslexia and ichthyosis.

A new multifacetted syndrome inherited as an autosomal, dominant trait is described encompassing not only two hitherto undescribed hereditary defects--thrombocytopathia and asplenia--but also muscle contractile defect, migraine-like headache, miosis, dyslexia and ichthyosis. None of these defects has so far been assigned to a specific chromosome or linkage group. Further studies on the various aspects of the syndrome are in progress.

Child

Demonstration of an alpha adrenoceptor-mediated inotropic effect of norepinephrine in human atria.

It has been claimed by other investigators that norepinephrine does not evoke a significant alpha adrenergic inotropic effect in human atria in contrast to epinephrine and phenylephrine, indicating a limitation of a possible functional role of the cardiac alpha adrenoceptors. We therefore characterized the inotropic effects of norepinephrine in isometrically contracting muscle strips from human atria obtained during open heart surgery. Both contraction and relaxation were studied by measuring developed tension and its first and second derivatives. Both the influence of propranolol and prazosin upon the inotropic responses to norepinephrine and the qualitative characteristics of the responses revealed that norepinephrine evoked both alpha and beta adrenergic inotropic effects. The alpha adrenergic response to norepinephrine was qualitatively different from the beta adrenergic effect and qualitatively similar to the alpha adrenergic effect of norepinephrine observed in other mammalian species. Although the alpha adrenergic effect was marked, the beta adrenergic effect was the dominating one as has also been found in other species. It is concluded that also in human atria norepinephrine evokes inotropic effects through both alpha and beta adrenoceptors.

Heart Atria

Surgical repair of isolated ventricular septal defects in the first year of life.

Since October 1975, 6 infants ranging in age from 5 to 9 months and weighing from 5.2 to 7.8 kg have been treated with primary closure of ventricular septal defect (VSD) at Ullevål Hospital. The indications for operation were large left-to-right shunts combined with persistence of heart failure in 4 patients, a large left-to-right shunt only in one and elevated pulmonary arterial resistance in one patient. Conventional cardiopulmonary bypass was used in all cases. There were no early or late deaths during the mean observation period of 17.3 months (range 3--25 months). One patient developed a recurrent VSD and was successfully re-operated on 8 months after the first operation; otherwise no signs of recurrence were found. The growth and weight gains have been satisfactory and the psychosomatic development of all the infants has been normal. All are in sinus rhythm with right bundle branch block in 4. Cardiac arrhythmias have not been in evidence.

Cardiopulmonary Bypass

Plasma concentration of diazepam and N-desmethyldiazepam in children after a single rectal or intramuscular dose of diazepam.

The absorption of diazepam and N-desmethyldiazepam after administration of diazepam solution for parenteral injection per rectum and intramuscularly was studied in 9 children (ages 3--12 years). Rectal administration of diazepam 1 mg/kg led to rapid absorption with plasma levels of 270--320 ng/ml within 5 min, and peak levels of 600--1300 ng/ml 10--60 min after administration. The absorption was comparable to that after intramuscular administration. A second peak in plasma diazepam concentration 6--12 h after dosing was observed in 6 children, which may have been due to mobilization of diazepam from the gastrointestinal mucosa produced by feeding 4 h after administration of the drug. A slowly increasing plasma level of N-desmethyldiazepam was observed during the first 24 h after administration of diazepam.

Child

Plasma concentrations of diazepam and N-desmethyldiazepam in newborn infants after intravenous, intramuscular, rectal and oral administration.

Five newborn infants (birth weight 2900--3600 g) were given diazepam (Valium, LaRoche) for convulsive disorders in 4 equal doses intravenously, intramuscularly, rectally and orally with at least 24 hours intervals. Three infants were given doses of 1 mg diazepam/kg body weight, and 2 0.5 mg/kg. The parenteral solution of the drug was given intravenously, intramuscularly and rectally. Powder of tablets was given orally. After intravenous administration very high peak values of plasma-diazepam concentration were obtained (5775--10800 ng/ml after 1 mg/kg, 2750 and 6450 ng/ml after 0.5 mg/kg). Next to intravenous administration rectal administration caused the most rapid increase in plasma-diazepam concentration. Presumed anticonsulsive concentrations (150--300 ng/ml) were obtained within 5 min with 1 mg/kg as well as 0.5 mg/kg rectally. Rectal administration therefore could be a suitable treatment for seizures in the newborn infant. Accumulation of the main depressive metabolite N-desmethyldiazepam occurred in all infants. This phenomenon must be taken into account when repeated doses of diazepam are administered.

Administration, Oral

Surdocardiac syndrome of Jervell and Lange-Nielsen, with prolonged QT interval present at birth, and severe anaemia and syncopal attacks in childhood.

A case of the surdocardiac syndrome of Jervell and Lange-Nielsen, with prolonged QT interval in the electrocardiogram at birth, is described. The affected girl presented 3 1/2 years later with severe iron deficiency anaemia, despite apparently adequate nutrition. At the age of 4 1/2 years she had a sudden attack of unconsciousness. Treatment with the beta-adrenoceptor blocker practolol was started and 3 years after initiation of this treatment she has been free from syncopal attacks. The QT interval remains prolonged. Her brother also had severe iron deficiency anaemia and had several attacks of unconsciousness before he died suddenly at the age of 3 1/2 years.

1-Propanol