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Biomedical subjects

S Friedman

Publications and source records attributed to S Friedman.

At least 433 records · Page 24Linked to original sources

Variations in globin synthesis in delta-beta-thalassaemia.

Peripheral blood globin synthesis studies were done in 11 patients with delta beta-thalassaemia trait, Hb S-delta beta-thalassaemia or delta beta/betao-thalassaemia from two black and two Caucasian families. All patients had elevated Hb F and normal or decreased Hb A2 levels and 10 had family studies confirming the diagnosis. In addition, four unrelated non-thalassaemic patients with elevated Hb F levels also had peripheral blood globin synthesis studies. The beta/alpha specific activity globin synthesis ratios in the three blacks with delta beta-thalassaemia trait were 0.60--1.04. In the four Caucasians with delta beta-thalassaemia traint, the beta/alpha ratios were 0.58--1.02. These results demonstrate a wide range of ratios overlapping those of normal controls (0.99 +/- 0.06). The betas/alpha ratios in three blacks with Hb S-delta beta-thalassaemia ranged from 0.66 to 1.00, similar to those of patients with delta beta-thalassaemia trait. In the black patient with delta beta/betao-thalassaemia, the gamma/alpha ratio was 0.67. The beta/alpha peripheral blood ratios in the four non-thalassaemia patients with elevated Hb F ranged from 1.00 to 1.11, similar to those of normal controls. These studies indicate that a decreased beta/alpha ratio is not an invariable finding in delta beta-thalassaemia in blacks or Caucasian patients and that globin synthesis data alone is insufficient to diagnose definitively heterozygotes for delta beta-thalassaemia or to distinguish this trait from non-thalassaemic haematological disorders associated with a normal percentage of Hb A2 and an elevated level of Hb F.

Adolescent↗

A multi-method investigation of two family constructs.

Two family constructs--prominence and interpersonal distance--are examined. Their origins are traced in the experimental and theoretical literature. The validity of each construct is investigated using data obtained from 15 families with a symptom-bearing child. Validity is supported in both cases through the occurrence of significant correlations among different measures of the same construct. It is suggested that the current distress of the families being studied may have increased the clarity of the prominence hierarchies and dyadic distances that emerged.

Dominance-Subordination↗

Long-term mitral valve replacement in young children. Influence of somatic growth on prosthetic valve adequacy.

Long-term clinical and laboratory findings in three children who required mitral valve replacement below age four years are reported. In each instance a second valve replacement was necessary approximately 8 1/2 years after the initial one, following a two and one-half fold increase in body weight. Inadequate mitral valve orifice size was found in each instance, producing a hemodynamic picture equivalent to mitral stenosis: congestive heart failure, pulmonary hypertension and atrial fibrillation. A second valve was placed without mortality in each instance and relieved the mitral valve obstruction. Pulmonary vascular resistance increased postoperatively in two patients and failed to decrease in the third. Pulmonary arterial hypertension and left ventricular hypertrophy persisted as long as 13 to 37 months after the second valve placement in all patients. The consequences of increasing body size and the long-term interposition of a rigid prosthesis in a growing heart introduce additional complications to mitral valve replacement in childhood. Frequent hemodynamic observations and the use of a prosthesis other than the ball-cage variety is recommended for improved management.

Adolescent↗

Staged surgical management of tetralogy of Fallot in infants.

A cohort of 61 consecutive patients 24 months of age of younger had palliative shunts for symptoms of tetralogy of Fallot during a 12-year period. Thirty-six of these patients have been followed through definitive intracardiac repair or to death. For analysis palliative operations were separated into two six-year periods, 1965--1970. During the first period seven of 30 infants operated on died; all 31 infants operated on during the second period survived. The Waterston anastomosis was performed most frequently (67%) during the first period; the Blalock-Taussig anastomosis was performed in 68% of infants during the second period. Of 54 hospital survivors, three died before definitive intracardiac repair. Two of the three interim deaths were related to heart disease. Twenty-six of the remaining 51 patients have had definitive intracardiac repair with two deaths (8%). Twenty-four in this group had intracardiac repair since 1973 with one hospital death (4%). The cumulative mortality for the entire cohort is 25%, but more recent experience (1971--77) indicates a cumulative mortality near 5%. The recent mortality rate for staged management is less than the 14% rate reported by others for primary intracardiac repair of tetralogy of Fallot in 205 infants. We conclude that primary intracardiac repair has important advantages for infants with tetralogy of Fallot who have favorable anatomic features and no other associated cardiac lesions or medical problems. Staged management of tetralogy of Fallot is still recommended for infants with unfavorable anatomy, additional lesions or associated medical problems.

Age Factors↗

Hormonal regulation of trehalose metabolism in the blowfly Phormia Regina Meig.: effects of cardiacectomy and allatectomy at the subcellular level.

1. Kinetic properties of adult Phormia fat body glycogen synthetase were studied and compared to other animals. The KM for UDPG is 2.82 mM, decreasing to 0.58 mM in the presence of G-6-P. 2. The specific activity of fat body glycogen synthetase shows a reduction of 30% within 2 days after allatectomy. 3. Fat body T-6-P synthetase activity decreases to 70% of the control value after cardiacectomy. 4. Corpus cardiacum homogenate fails to induce higher T-6-P synthetase activity in cell-free preparations from cardiacectomized flies. 5. Interactions between corpus cardiacum and corpus allatum in regulating carbohydrate metabolism are discussed.

Animals↗

Congenital left aortic sinus-left ventricle fistula and review of aortocardiac fistulas.

A fistula from the left aortic sinus to the left ventricle is reported that was successfully repaired using cardiopulmonary bypass. In addition, 175 cases of fistula to the heart from the aortic sinuses published in the English literature from 1839 through 1972 were studied, with particular emphasis on the 126 patients who underwent operative repair. The major cause of the fistulas (76%) was found to be rupture of a congenital aortic sinus aneurysm, usually during the third or fourth decade of life. The remainder of these fistulas to the heart consisted of simply a tract in an otherwise normal sinus. Ventricular septal defect was the most common associated defect and, when present, was nearly always related to a fistula arising from the right aortic sinus. Repair was successful in 86% of the 126 operated patients. The principles of operative treatment of these fistulas are reviewed.

Aorta, Thoracic↗

Globin biosynthesis in sickle cell, Hb SC, and Hb C diseases.

The wide range of globin synthesis ratios reported in patients with sickle cell disease casts doubt on whether the presence of genes for alpha- or beta-thalassemia in combination with Hb S can be detected by globin synthesis studies. We have studied globin synthesis in 20 patients with Hb SS who had a mean betaA/alpha ratio of 1.05+/-0.04, similar to that of 28 control children. In nine of these patients the percentage of newly synthesized radioactive alpha-chains in dimer or monomer forms was 16.3%+/-1.3, also similar to the control subjects. The remainder of alpha-chain was in hemoglobin tetramer. In nine patients with Hb SC, the (non-alpha)/alpha ratio was 0.97+/-0.04, and the free alpha-chain pool radioactivity in four patients was 14.1%+/-4.2. In three patients with Hb CC, betac/alpha ratios were 0.99, 1.07, and 1.10. These results indicate that globin synthesis ratios and alpha-chain radioactivity in the free alpha-chain pool of peripheral blood of patients with Hb SS, Hb SC, and Hb CC have narrow ranges, close to those of nonthalassemic controls. The data provide a basis for detecting syndromes with Hb S or Hb C associated with alpha- or beta-thalassemia. This precise differentiation is important for clinical studies of severity in sickle cell disease and for genetic counseling.

Adolescent↗

Alterations of tRNA modification in mammalian systems: the effect of ethionine.

The relationship between the modification of tRNA and its ability to act as a substrate for homologous tRNA modification enzymes in vitro was studied. The tRNA extracted from the livers of rats was active as a substrate for in vitro methylation with extracts from normal rat liver 19 h after treatment with L-ethionine (35 mg/100 g/24 h). After 4 weeks of feeding a diet containing o.25% DL-ethionine, the tRNA was a poor substrate for methylation in vitro, even though it was deficient in methylated nucleosides. Only 18% and 7% of the available sites could be methylated after 67 h and 4 weeks, respectively, of ethionine treatment. 3-(3-amino-3-carboxypropyl)uridine, a nucleoside that is also synthesized from S-adenosylmethionine, was assayed in individual tRNAs by their reactivity with the N-hydroxysuccinimide ester of phenoxyacetic acid. The reactivity of tRNAIle, tRNAAsn, and tRNAThr was decreased by treatment with ethionine at 67 h as well as at 2 and 4 weeks, although no difference could be detected at 19 h.

Amino Acids↗

A system for tailoring change measures to the individual family.

The focus of this project is the problem of evaluating change in families. Prior work has tended to limit itself to anecdotal or self-report data. By and large, the studies that have evaluated a family intervention by observing family interactions have failed to demonstrate change. A major drawback in such studies has been the assumption that the few interaction variables selected (e.g., amount of silent time) have roughly the same salience and meaning for all the families studied. The measurement system described here was developed to fulfill four methodological requirements: (a) to represent the family as a whole; (b) to be sensitive to change; (c) to contain multiple measures; and (d) to be adaptable to the variation among families. The solution to these requirements, presented here, is a system for tailoring measures to individual families.

Aspirations, Psychological↗

Diagnosis and treatment planning.

A diagnosis based upon a haphazard examination is an invitation to failure. A thorough diagnostic service is essential for optimal results. Successful resolution of the complete denture problem depends upon many factors. The dentist's knowledge, possession of the necessary technical skills, sound clinical judgment, experience, and psychological management of the case, combined with the patient's willingness and capacity to persevere and master the use of well constructed dentures, are factors of major consideration which are vital to the ultimate result.

Attitude to Health↗

Maternal language to prelinguistic infants: syntactic aspects.

Maternal speech to children has been shown to vary by age and language ability of the children. Previous studies have usually involved children over 1 year of age. In this study maternal speech to male and female 4-, 6-, and 8-month-old infants was recorded in the laboratory. Mothers used shorter utterances to 8-month-olds than to 4- or 6-month-olds, presumably in response to the infant's changing level of comprehension. Mothers used more sentences with subjects, verbs, or objects deleted to 8-month-olds and more complex sentences to 4-month-olds.

Female↗