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Biomedical subjects

C Sultan

Publications and source records attributed to C Sultan.

At least 181 records · Page 10Linked to original sources
PubMed1983

Haematological features of primary myelodysplastic syndromes (PMDS) at initial presentation: a study of 118 cases.

The haematological features of 118 cases of primary myelodysplastic syndromes (PMDS) were reviewed to see how these could be related and classified according to the recent FAB proposals. A majority of the cases were elderly who presented with a macrocytic or normocytic anaemia and reticulocytopenia. Cases of acquired idiopathic sideroblastic anaemia (AISA) usually had normal leucocyte and platelet counts, erythroid hyperplasia, marked dyserythropoiesis and more than 20% ringed sideroblasts. Cases of refractory anaemia with excess of blasts (RAEB) had frequent neutropenia and thrombopenia usually with prominent dysgranulopoiesis and dysthrombopoiesis. Refractory anaemia or refractory cytopenia appeared morphologically to be a heterogeneous group. Leukaemic transformation did not occur in any of these 16 cases of AISA whereas six of the 34 cases of RAEB transformed into acute leukaemia. It appears that the cases of PMDS present with well defined haematological features which permit recognition of different groups; these latter groups appear to be morphologically and prognostically distinct.

Adult↗
PubMed1983

Prevalence and distribution of ringed sideroblasts in primary myelodysplastic syndromes.

In order to determine the prevalence and percentage distribution of ringed sideroblasts in primary myelodysplastic syndromes, the results of Prussian blue staining were analysed in 133 cases. Ringed sideroblasts ranging from 1 to 86% of cells were found in 76 (57%) cases. The cases of primary myelodysplastic syndrome corresponding to the group entitled "acquired idiopathic sideroblastic anaemia" had between 21 and 86% ringed sideroblasts; these were also found in 40% (26/65) cases corresponding to refractory anaemia with excess of blasts. Seven of the 22 cases having morphological features of refractory anaemia with excess of blasts in transformation had ringed sideroblasts. It would appear that cases of acquired idiopathic sideroblastic anaemia have at least 20% ringed sideroblasts; they also seem to occur frequently in refractory anaemia with excess of blasts.

Anemia, Aplastic↗
PubMed1983

[Metabolism of dihydrotestosterone in cultured skin fibroblasts: reduction to 5 alpha-androstane-3 alpha, 17 beta-diol].

Reduction of dihydrotestosterone into 5 alpha-androstan-3 alpha, 17 beta diol by 3 alpha hydroxysteroid dehydrogenase was studied in human cultured skin fibroblasts. Characterization of the 3 alpha diol was performed by gas-liquid chromatography coupled with mass spectrometry. Sex skin fibroblasts enzyme has the same Km (9.10 X 10(-6) M) as that of non sex skin fibroblasts (8.75 X 10(-6) M). On the other hand, reduction of DHT is 3 times higher in sex skin fibroblasts (Vmax = 223.9) than in non sex skin fibroblasts (Vmax = 87.9). Thus, human culturel fibroblasts appear again to be an useful tool for the study of a key-enzyme of androgen metabolism in target cells.

Androstane-3,17-diol↗
PubMed1982

Androgen receptors in cultured human skin fibroblasts.

Human skin may be considered as a target organ for androgens, since events characteristic of androgen action have been described in this tissue, as well as in cultured human skin fibroblasts. The culture of human skin fibroblasts gives the opportunity to work on living cells, under controlled conditions, in a renewable material from a single skin biopsy. Using this method, we showed the presence od DHT-receptors in the human fetus and we studied the ontogenesis of androgen receptors in relation to sexual differentiation. In the neonatal period, the physiological T rise was not concurrent with a variation in sex skin androgen receptors. The evolution of DHT binding during puberty is now under investigation. These data suggest that the androgen receptor is not modulated by plasma androgens. Androgen receptor determination is of value in defining the biochemical defects involved in partial and complete androgen insensitivity syndrome (twenty-six cases in our study). In idiopathic hirsutism, DHT binding is used to detect an eventual local hypersensitivity. Fibroblast cultures have also been shown to be an excellent model for the screening of compounds which might block the expression of androgen action by competing for the androgen receptors. Cultured skin fibroblasts are a valuable model for the study of androgen and antiandrogen action in human skin.

3-Oxo-5-alpha-Steroid 4-Dehydrogenase↗
PubMed1982

[Effect of cyproterone acetate on the 3 alpha-hydroxysteroid-dehydrogenase activity of cutaneous fibroblasts in culture].

In men, parallel variations in urinary 3 alpha 5 alpha androstane 17 beta diol with the amount of sex skin fibroblast DHT receptors rise the question of a relationship of 3 ketosteroid reductase activity and specific DHT binding. To test this hypothesis, we measured 3 alpha 5 alpha androstane 17 beta diol biosynthesis in cultured sex skin fibroblasts, in the presence of high amounts of cyproterone acetate which competes with DHT for its receptor: when 95% of DHT is displaced by cyproterone acetate, the 3 keto steroid reductase activity remains unchanged. These results suggest that the relationship of 3-keto-steroid reductase activity and androgen receptors is unlikely.

Animals↗
PubMed1982

Proposals for the classification of the myelodysplastic syndromes.

New diagnostic criteria for the diagnosis of the various myelodysplastic syndromes (MDS) are proposed, and a detailed description is given of the features that may help define MDS. Five MDS are described: (1) refractory anaemia (RA), (2) RA with ring sideroblasts, (3) RA with excess of blasts (RAEB), (4) chronic myelomonocytic leukaemia (CMML), and (5) RAEB 'in transformation'. One of the main distinguishing features of these conditions is the proportion of blast cells in the peripheral blood (PB) and/or bone marrow (BM). The morphological features of the blast cells that are of diagnostic importance have been redefined. In RA, with or without ringed sideroblasts, there are fewer than 1% of blasts in the PB and fewer than 5% in the BM; RAEB is defined as having between 5% and 20% of blasts in the BM and fewer than 5% in the PB; RAEB in transformation (a newly defined category) will be considered when any of the following features is present: (i) more than 5% of blasts in the PB, (ii) 20-30% in the BM, and (iii) the presence of Auer rods in granulocyte precursors in BM or PB. In accordance with these newly defined criteria, it is now proposed that over 30% of bone marrow blasts will suffice for the diagnosis of acute myeloid leukaemia (AML) in any of its forms (M1-M6). The proposed descriptions of the MDS should facilitate the interpretation of data emerging from cytogenetic and bone marrow culture studies and the search for features of possible prognostic significance. Recognition of the new category, RAEB in transformation, may throw light on the pathogenesis of AML.

Anemia, Aplastic↗
PubMed1981

Inhibition of androgen binding in human foreskin fibroblasts by antiandrogens.

Antiandrogen effects on androgen receptor binding and androgen metabolism were studied in cultured human newborn foreskin fibroblasts. Three different antiandrogens were tested in this system: (a) cyproterone acetate (CA); (b) RU23908; and (c) R2956. CA and R2956 were equipotent inhibitors of androgen binding to its intracellular receptor. The magnitude of this action was nearly twice as great against the endogenous androgen ligands, dihydrotestosterone (DHT) or testosterone (T), than with the synthetic ligand, methyltrienolone (R1881). Whereas the relative binding affinities of CA and R2956 were approximately 5-10 times less than T or DHT, RU23908 was another order of magnitude less effective as an inhibitor of androgen binding. The lower relative binding affinity determined for RU23908 could not be explained on the basis of a requirement for metabolic activation. Subcellular fractionation studies and sucrose density gradient analysis further confirmed the rank order of antiandrogenic potency. None of the antiandrogens influenced the rate or profile of metabolites from cellular metabolism of T or DHT. We propose that cultured human genital skin fibroblasts may serve as a valuable system for the future evaluation of antiandrogens in intact ells under physiologic conditions.

Androgen Antagonists↗
PubMed1981

Distribution of 250 cases of acute myeloid leukaemia (AML) according to the FAB classification and response to therapy.

250 consecutive cases of AML in adults were diagnosed over a 6 year period in the same institution. Pretreatment blood and bone marrow smears and also cytochemical markers were taken into account in the study. All cases were reviewed by three different observers and classified according to the FAB criteria. The final diagnosis resulted from agreement of at least two observers. Some discrepancies occurred in the classification and are discussed. The prognostic implication of AML subclassification was assessed as the percentage of complete remission in 100 cases.

Adolescent↗
PubMed1981

The morphological classification of acute lymphoblastic leukaemia: concordance among observers and clinical correlations.

The degree of concordance in the morphological classification of ALL was assessed by the FAB group after two successive reviews of 200 and 100 slides respectively. As a result, a simple scoring system for types L1 and L2 is proposed based on the following four features: (1) nuclear cytoplasmic ratio, (2) presence, prominence and frequency of nucleoli, (3) regularity of nuclear membrane outline, and (4) cell size. By this method, the overall concordance by seven observers (agreement of 7:0 or 6:1 only) increased from 63% to 84%. A significant difference in the incidence of the ALL morphological types in children (less than or equal to 15 years) and adults (greater than 15 years) was found: 74% of L1 cases were children while 66% of L2 cases were adults (P less than 0.001). No significant differences were observed in the incidence of L3 in children and adults or between L1 and L2 according to the membrane phenotype of the blast cells. All L3 cases had B-cell characteristics. A better prognosis for L1 and a higher relapse rate for L2 has been found in several recent reports; The present study may facilitate the morphological analysis of ongoing clinical trials in ALL by improving the reproducibility of the FAB classification.

Acute Disease↗
PubMed1981

Acute myelodysplasia with myelofibrosis: a report of eight cases.

Eight patients with acute myelodysplasia and myelofibrosis are described. Four cases were secondary to long-term therapy with cytotoxic agents and four were idiopathic. All cases presented with an abrupt onset of the illness, absence of organomegaly and severe pancytopenia. Bone marrow aspirate yielded adequate material in four cases and showed myelodysplasic features. Study of histological sections indicated that the bone marrow was cellular in every case, including numerous dystrophic megakaryocytes, erythroblasts, immature cells of the granulocytic series and blast cells which were difficult to identify. The reticulin network was always increased. In each case the disease was rapidly fatal. No improvement was noted with chemotherapy. In three cases an overt leukaemia developed with marked pleomorphism of blast cells. The nosology of this syndrome is discussed.

Adult↗
PubMed1981

[Androgen receptors in the fetus of a primate (Macaca fascicularis) : preliminary results].

Skin fibroblasts raised from Macaca fascicularis skin samples were tested for specific androgen receptors. By the 100th day of gestation male and female skin fibroblasts androgen receptors were detected. Higher levels of androgen receptors were found in sex skin fibroblasts. These preliminary results suggest that this primate could be used as a good model for the study of androgen receptors ontogenesis and regulation.

Animals↗
PubMed1980

Phenotypic variation in a family with partial androgen insensitivity syndrome.

A family with partial androgen insensitivity syndrome exhibited considerable variation in phenotypic expression of their androgen resistance. One subject died at 2 1/2 years of age of a Wilms' tumor. In the two living members, one had a micropenis with otherwise normal genitalia, while the other had a small phallus, perineoscrotal hypospadias, bifid scrotum, and persistence of a vaginoutricular pouch. At puberty, plasma androgens and serum gonadotropins increased to normal or elevated values. However, despite adequate endogenous plasma testosterone levels and testosterone therapy, these patients showed poor virilization and were sterile. Studies of cultured sexual skin fibroblasts showed adequate 5 alpha-reductase activity and normal receptor affinity and capacity for dihydrotestosterone. An X-linked mode of inheritance is postulated, although autosomal dominance cannot be ruled out.

Child↗