Biomedical subjects

S J Sorland

Publications and source records attributed to S J Sorland.

15 recordsLinked to original sources

PubMed1977

Hereditary cholestasis combined with peripheral pulmonary stenosis and other anomalies.

A syndrome consisting of chronic intrahepatic cholestasis with retention of bile acids but with normalization of bile pigment excretion ahd blood lipids, peripheral pulmonary stenosis, vertebral anomalies and a characteristic facies is described in six patients, including a father and his daughter. An autosomal dominant mode of inheritance is suggested.

Abnormalities, Multiple↗

PubMed1976

The young platelet population in children with cyanotic congenital heart disease.

Some morphological, biochemical and functional parameters of platelet population in children with cyanotic congenital heart disease (CCHD) were studied by making comparisons of the normal platelet population in both CCHD patients and controls. The mean volume of the platelets from cyanotic patients was greater than from normals. The platelet size distribution curves demonstrated a shift towards larger than normal size in the case of CCHD. The mean protein content, as well as the mean PF3 content of platelets was increased in CCHD. Following addition of kaolin, PF3 release was more rapid and of shorter duration with platelets from CCHD patients as compared to normal platelets. They also released more PF3 than did normal platelets. After addition of ADP, collagen, or adrenalin, platelets of CCHD patients were more responsive than similarly treated platelets from normals. Platelets from CCHD showed an increased initial rate of aggregation and greater maximum aggregation. These data suggested that the platelet population of CCHD patients consists of larger, younger and functionally more active platelets than does the platelet population of normals.

Adolescent↗

PubMed1976

Transposition of great arteries. Early results of Mustard's operation in paediatric patients.

Between May 1969 and June 1975, 43 patients with d-transposition of the great arteries, ranging in age from 2 months to 13 years (mean 19 months), underwent surgical correction. In the first 3 patients Mustard's original method was followed. Later a modified procedure was performed using a Teflon patch. Forty-two of the 43 patients had previously had 50 various palliative procedures, of which 30 were balloon atrial septostomy only. Five patients died in hospital (up to 5 weeks after operation). Of the 32 patients with simple repair of transposition of the great arteries (including 3 with ligation of a persistent ductus arteriosus), 3 died. One patient died as a result of complete AV block, one as a result of renal damage associated with unrelieved coarctation of the aorta, and one of cerebral infarction and peritonitis which probably were initiated preoperatively. Six patients who had additional surgery for pulmonary stenosis survived, including 3 patients who had closure of ventricur septal defect, including 2 who had debanding of the pulmonary artery, 2 died in a low cardiac output state. One infant operated on for subpulmonary stenosis developed permanent complete AV block and was successfully treated with a pacemaker. All the patients with combined operations had some problems postoperatively. The survivors improved greatly, but 2 patients died suddenly one, and one and a half years after the operation respectively.

Adolescent↗

PubMed1975

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↗

PubMed1974