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Biomedical subjects

J C Fratantoni

Publications and source records attributed to J C Fratantoni.

51 records · Page 3Linked to original sources
PubMed1969

The defect in Hurler and Hunter syndromes. II. Deficiency of specific factors involved in mucopolysaccharide degradation.

Cultured fibroblasts, derived from patients with the Hurler and Hunter syndromes, show defective degradation of sulfated mucopolysaccharide. The aberrant metabolism of Hurler cells can be corrected by secretions of fibroblasts of genotype other than Hurler, and similarly, the defect of Hunter cells can be corrected by secretions of fibroblasts of genotype other than Hunter. The active factors in these secretions, which are heat labile and associated with macromolecules, accelerate the degradation of mucopolysaccharide.

Culture Techniques↗
PubMed1968

Hurler and Hunter syndromes: mutual correction of the defect in cultured fibroblasts.

The biochemical defect of cultuired skin fibroblasts from Hurler or Hunter patients (faulty degradation of sulfated mucopolysaccharide, resulting in excessive intracellular accumulation) may be corrected if cells of these two genotypes are mixed with each other or with normal cells. The effect is mediated by substances released into the medium.

Carbohydrate Metabolism, Inborn Errors↗
PubMedDate not supplied

Survey on the current use of leukapheresis and the collection of granulocyte concentrates.

This study was designed to define leukapheresis practice. A voluntary questionnaire on leukapheresis was sent to 280 FDA-registered blood-collecting establishments performing leukapheresis. Of the facilities questioned, 67.9 percent responded. The survey results indicate that most facilities use intermittent-flow blood cell separators, while 22.6 percent use more than one separation method. Establishments routinely use 6% hydroxyethyl starch (HES 450/0.70) and the majority using trisodium citrate as the anticoagulant. Forty-eight percent use corticosteroids, primarily dexamethasone, to pretreat the donor. The frequency of donation was not specified by 25.3 percent of the report. Forty-two percent chose an individual donation frequency of two times per week. A limit on the total number of donations allowed per donor was not specified by 78.4 percent of the facilities. Community blood banks (including regional centers) performed 55.6 percent of all leukocyte concentrate collections. The donor reaction incidence was of 3.64 percent. Hospitals, of all types, performed 37.8 percent of the collections. The adverse reaction rate ranged from 2.84 to 9.72 percent. Adverse reactions occurred in donors 54.9 times per 1000 procedures. Ninety-four percent of reported reactions were mild, whereas moderate and severe reactions accounted for 5.9 and 0.4 percent, respectively. Granulocyte yields varied by the type procedure and the use of corticosteroids as well as among facilities. The majority (56.3%) held leukocytes at 22 to 25 degrees C prior to transfusion, while most of the remainder stored at 4 degrees C.

Adrenal Cortex Hormones↗
PubMedDate not supplied

Dimethyl sulfoxide: effects on function of fresh platelets and on the viability of platelets in storage.

Dimethyl sulfoxide (DMSO) is used as a cryoprotective agent when platelets are frozen. We examined the effect of DMSO (0.1 to 10%) on platelet aggregation, release, and prostaglandin synthesis (as indicated by malondialdehyde formation) in response to thrombin, collagen, arachidonic acid and calcium ionophore. Inhibition was observed at the lowest levels of DMSO, varied with the type of stimulus, and was reversed by washing the platelets. Inhibition of aggregation, release, and malondialdehyde formation were dose-dependent with thrombin or collagen. DMSO did not inhibit malondialdehyde formation stimulated by arachidonic acid, nor did it consistently inhibit any function stimulated by calcium ionophore. When platelets were stored as platelet-rich plasma at 20 to 24 degrees C for 48 hours, with and without 5 percent DMSO, and subsequently washed, the platelets stored with DMSO were more reactive in vitro. These results indicate that platelet function inhibition by DMSO not only is reversible, but protects the platelets during storage. The factor limiting the use of DMSO in platelet storage is potential systemic toxicity, not its effects on platelets.

Arachidonic Acid↗