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Biomedical subjects

S Harel

Publications and source records attributed to S Harel.

At least 109 records · Page 6Linked to original sources
PubMed1981

Urinary excretion of desmosine in patients with severe burns.

Urinary excretion of total desmosine was measured by a radioimmunoassay in severely burned adult males, as well as in normal adult males. Total urinary desmosine was significantly elevated in all the samples in the burned patients, who had injuries involving more than 19% of total body surface area. The values of 24-hr urinary desmosine for the burned patients ranged from 250--1,411 nmoles, as compared with 82--142 nmoles for normal controls. These were equivalent to 14--78 mg of elastin degraded for the burned patients and 5--8 mg for normal controls. Urinary desmosine values expressed as nmoles per g of creatinine were also higher than the corresponding normal values, ranging from 110--768 nmoles versus 63 +/- 6 nmoles for normal controls. Urinary excretion of total hydroxyproline in the burned patients was also higher than in normal controls, ranging from 56--471 mg per 24 hrs, or 36 to 413 mg per g of creatinine, vs. 31 +/- 6 mg per 24 hr, or 23 +/- 2 mg per g of creatinine, in burned patients and normal controls, respectively. These values of hydroxyproline were equivalent to 413--3,623 mg of collagen and 238 mg of collagen, respectively. In the burned patients, both urinary desmosine and hydroxyproline values were elevated from day 1 post-burn, and reached peak levels in days 2--12, declining thereafter but remaining higher than values for normal controls through day 60. The metabolism of elastin and collagen in skin of burned patients was probably highly accelerated for a long time, at least through day 60 post-burn.

Adult↗
PubMed1980

Desmosine radioimmunoassay for measuring elastin degradation in vivo.

Desmosine is a cross-link amino acid unique to elastin. Previous work has shown that during turnover in the body, desmosine is not reused, and that desmosine is not absorbed from the intestine. Instead, all desmosine released in the course of elastin metabolism is excreted in the urine attached to low molecular weight peptides. Therefore, measurement of desmosine in acid-hydrolysates of urine might be used to monitor elastin breakdown in several pathologic states, including pulmonary emphysema. In the present report, we have described a sensitive, highly specific radioimmunoassay capable of detecting as little as 200 pg of desmosine in acid-hydrolysates of urine. The assay was specific for desmosine; cross-reactivity with merodesmosine, isodesmosine, lysine, and mixed amino acids was 0.25%, 0.1%, less than 0.0003%, and 0%, respectively. Twenty-three normal, nonsmoking subjects had a mean 24-hr desmosine excretion of 47 +/- 15 microgram. In a group of smokers with evidence of chronic obstructive disease and/or lung infection, the values for desmosine excretion ranged from 40 to 400 microgram/24 h. Desmosine radioimmunoassay may find application in the study of diseases involving increased destruction of elastin in the body.

Adolescent↗
PubMed1980

Ultrastructure of the conjunctiva, skin, and gingiva: a case of Sandhoff's disease in a Jewish patient.

Pleomorphic membranous cytoplasmic bodies that indicated glycolipid storage were found in the conjunctiva, skin, and gingiva of a Jewish patient with Sandhoff's disease. The clinical symptoms were typical of GM2 gangliosidosis. Both hexosaminidase A and hexosaminidase B activities were deficient in the leukocytes and serum. Glycosaminoglycan levels in cultured fibroblasts were elevated. Membranous cytoplasmic bodies were observed in high concentrations in a large proportion of the vascular endothelial cells, pericytes, and Schwann cells and to a somewhat lesser extent in the fibrocytes of all tissues studied. Ultrastructural analysis of the conjunctiva, skin, and gingiva as an aid for the diagnosis of Sandhoff's disease is suggested.

Capillaries↗
PubMed1979

Decreased serum 24,25-dihydroxy vitamin D concentrations in children receiving chronic anticonvulsant therapy.

Serum 24,25-dihydroxy vitamin D (24,25(OH)2D) and 25-hydroxy vitamin D (25-OHD) concentrations and the ratio between the two were measured in 31 Israeli children and adolescents receiving long-term treatment with phenobarbitone or phenytoin and in controls. 24,25 (OH)2D concentrations were significantly depressed in the patients, although the 25-OHD concentrations were similar to those in the healthy controls. In four patients with radiological evidence of osteopenia very low serum 24,25(OH)2D concentrations and serum 24,25(OH)2D: 25-OHD ratios were recorded. The findings suggest that 24,25(OH)2D deficiency may play an important part in the pathogenesis of osteomalacia in patients treated with anticonvulsant drugs and provide further indirect evidence that 24,25(OH)2D is important for normal bone structure.

Adolescent↗
PubMed1979

Myotonia congenita (Thomsen's disease). Early diagnosis in infancy.

A family with myotonia congenita. (Thomsen's disease) is reported in which the father and his two offspring are affected. The course was characterized by the early onset of clinical manifestations in both the father and his two children. In one child, a clinical and electrical diagnosis of the disease was made as early as two weeks of age. Early manifestations were breathing difficulty and eye closure myotonia. The importance of early recognition of the disorder is emphasized.

Adult↗
PubMed1979

Lung injury induced by leukocytic proteases.

Human polymorphonuclear neutrophilic leukocytes (PMNs) contain large amounts of neutral proteases that can degrade elastin, collagen, proteoglycan, and basement membrane. The instillation of one of the purified enzymes (elastase) into dog lungs in vivo causes degradation of elastic fibers and other alveolar septal components and results in anatomic changes similar to those of human pulmonary emphysema. Cigarette smoking is a major risk factor associated with pulmonary emphysema in man. One mechanism for this association may be interference with the regulation of PMN elastase activity by alveolar antiproteases. This possibility is supported by the observation that the oxidizing activity of tobacco smoke inactivates alpha 1-proteinase inhibitor in vitro. Macrophages also secrete an elastolytic protease, albeit at low levels. The short-term exposure of cultured mouse macrophages to cigarette smoke augments the rate of elastase secretion by these cells. Mouse macrophage elastase is not inhibited by alpha 1-proteinase inhibitor or alpha 2-macroglobulin. This unusual property of macrophage elastase may facilitate its attack upon elastin over prolonged intervals despite very low levels of macrophage elastase production. A unified hypothesis of lung injury in pulmonary emphysema is presented, involving both PMN and macrophage elastases and the actions of cigarette smoke. (Am J Pathol 97:111--136, 1979).

Animals↗
PubMed1979

[Reye's syndrome].

Explore the source record for details and available documents.

Child, Preschool↗
PubMed1978

Neuromotor development in relation to birth weight in rabbits.

The development of neuromotor patterns in relation to birth weight was studied in the rabbit, a perinatal brain developer. In order to induce intrauterine growth retardation and to increase the number of low birth weight rabbits, experimental ischemia to half the fetuses in each doe was achieved by total ligation of approximately 30% of spiral vessels to the placenta, during the last trimester of gestation. Following natural delivery, the rabbit pups were periodically observed for the appearance of eye-opening and righting reflex, and for the cessations of falling, circling and dragging of hind limbs. An index of neuromotor development was assigned to each rabbit by summing up the age (in days) of appearance of each of the neuromotor milestones. An association was found between low birth weight and delayed neuromotor development at 2 weeks of age. The most significant correlation was found between low birth weight and delayed disappearance of falling. The latter may represent incoordination as an expression of cerebellar dysfunction.

Age Factors↗
PubMed1976

Relations between birth weight and learning ability in young rabbits.

Performance on visual discrimination learning was studied in 41 rabbit pups mainly in relation to their birth weight, but also as functions of their neonatal motor development and weight gain between birth and training time. Partial placental vascular ligation was performed on some fetuses to increase the number of small-for-gestational-age pups. No relation was found between learning ability and either birth weight or rate of motor development. Low birth weight showed some association with slightly delayed motor development, but resulted in no lasting motor handicap. Between two subgroups of low birth weight pups, those who showed substantial weight gain by training time learned faster than those who remained relatively small.

Animals↗
PubMed1975

Experimental intrauterine growth retardation.

The effects of experimental intrauterine growth retardation on subsequent fetal development, especially with respect to brain development, were studied in a new animal model. The rabbit was chosen since it has a perinatal pattern of brain development similar to that of the human. Experimental ischemia was induced during the last trimester by ligation of spiral arterioles and the differential effects on fetal development at term (30th gestational day) are reported. Specific brain regions were examined for wet weight, total cell number (DNA) and total protein content. Highly significant decreases in all these parameters were found in both the cortex and cerebellum following experimental intrauterine growth retardation; these two organs were differentially affected. The prospects and advantages of using this animal model for the study of the postnatal "catch-up growth" are discussed.

Animals↗