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Biomedical subjects

C Sultan

Publications and source records attributed to C Sultan.

At least 109 records · Page 6Linked to original sources

[Leukocyte differential of the Coulter VCS. Evaluation and modalities of use in comparison with the Coulter STKR and manual count].

The Coulter VCS is a flow cytometer which performs, from a 100 microliter blood sample, a full five-part differential by assessing the volume (V), high frequency conductivity (C) and laser light scatter (S) on each white cell counted. The authors evaluated the Coulter VCS and compared its results with those of the Coulter STKR (three-part differential) and of the manual count. Reproducibility (ten replicate analyses on six different normal samples) was studied by the three methods and showed coefficients of variation closed to the manufacturer's specifications except for monocytes. The correlation coefficients obtained from 345 normal samples were the following: VCS/manual count: 0.97 for neutrophils, 0.70 for eosinophils, 0.97 for lymphocytes and 0.57 for monocytes; VCS/STKR: 0.99 for granulocytes, 0.99 for lymphocytes and 0.70 for monocytes. Comparisons of means displayed statistical differences for some cell categories but without clinical consequences. The flag analysis of 313 abnormal samples showed a false positive rate of 3.1 p. cent for the VCS and 1.8 p. cent for the STKR, the false negative rate was 2.1 p. cent for the VCS and 3.6 p. cent for the STKR. Starting from total blood count parameters, the authors propose guidelines for appropriate use of the different leucocyte differential methods.

Algorithms↗

Proposals for the classification of chronic (mature) B and T lymphoid leukaemias. French-American-British (FAB) Cooperative Group.

Peripheral blood, bone marrow films, and bone marrow biopsy specimens from 110 patients, well characterised by clinical and laboratory studies, including electron microscopy, were reviewed, to determine proposals for the classification of chronic (mature) B and T cell leukaemias. On the basis of cytology and membrane phenotype the following disorders were defined: (i) B cell type: chronic lymphocytic leukaemia (CLL); CLL of mixed cell type, which includes cases with more than 10% and less than 55% prolymphocytes (CLL/PL), and a less well defined form with pleomorphic lymphocytes but less than 10% prolymphocytes; prolymphocytic leukaemia (PLL); hairy cell leukaemia (HCL); HCL variant; splenic lymphoma with circulating villous lymphocytes; leukaemic phase of non-Hodgkin's lymphoma (follicular lymphoma, intermediate, or mantle zone lymphoma and others); lymphoplasmacytic lymphoma with peripheral blood disease (mostly Waldenström's macroglobulinaemia); and plasma cell leukaemia. (ii) T cell type: T/CLL, which was differentiated from reactive T/lymphocytosis; T/PLL; adult T cell leukaemia/lymphoma; and Sézary's syndrome. The recognition of distinct entities within the B and T cell leukaemias seems to have clinical and epidemiological connotations. It is hoped that these proposals may serve as the basis for further work, discussion, and improved management of patients.

Adult↗

An expert system applied to the diagnosis of anemia with special reference to myelodysplastic syndromes.

An expert system is described that includes interpretation of the results from a complete blood count as well as data from bone marrow aspiration. The system utilizes Bayes' rule. It has previously been tested on 180 cases of anemia including 20 benign and malignant hematologic disorders. On the data set, the system achieved 84% satisfactory diagnoses. In the present study, patients with myelodysplastic syndromes and with disorders of heme synthesis have been added to the test cases. For support, the expert system requires an IBM Personal Computer or equivalent. The program is available commercially (Coulter Electronics, Hialeah, FL).

Anemia↗

Adult patients presenting with pancytopenia: a reappraisal of underlying pathology and diagnostic procedures in 213 cases.

We reviewed 213 consecutive adult pancytopenic patients to determine the frequency of underlying pathology, to analyze our diagnostic procedure, and to determine the value of peripheral blood data for diagnosis. Pancytopenia was defined as the association of hemoglobin level below 12 g/dl in males and 11.5 g/dl in females, leukopenia below 4 x 10(9)/L, and thrombocytopenia below 150 x 10(9)/L. The bone marrow aspirates were normo- or hypercellular in 140 cases (66%). Bone marrow biopsies, performed in 93 cases, documented the presence of myelofibrosis in 67 cases. Aplastic anemia was diagnosed in 10% of the cases. Malignant myeloid disorders (acute myeloid leukemias, myelodysplastic syndromes, acute myeloid disorders with myelofibrosis) represented 42% of the cases and various malignant lymphoid disorders 18%. Vitamin deficiencies accounted for 7.5% and nonhematological pathology 10% of the cases. The bone marrow aspirate was sufficient for the diagnosis in 55% of the cases, and the trephine biopsy was necessary in 30%. In the remaining cases, other complementary tests were necessary to achieve final diagnosis. A discriminant analysis, focused on the hemogram data, showed that parameters obtained by analysis of blood smears were helpful for the diagnosis, especially the presence or absence of blast cells and/or of abnormal lymphoid cells.

Adolescent↗

[Bioluminescence assay of luteinizing hormone in plasma and urine].

The authors describe a bioluminescent immunoassay of LH in plasma and urine. It uses two monoclonal antibodies, one is labelled with glucose-6-phosphate-dehydrogenase, the other one is coimmobilized together with bioluminescent enzymes from marine bacteria on the same adsorbent (Sepharose). This assay can be performed directly on 20 microliters plasma or 10 microliters urine. The protocol is very fast, no separation step is required to remove the excess labeled antibodies. The inhibitory effect of the biological sample on luminescent reaction is determined by adding NADH to the assay tubes. The working range of this assay is 3 to 300 Ul/l, with a sensitivity of detection of 0.5 Ul/l. Recovery, linearity, within and between assay precision were evaluated and appeared to be satisfactory. The authors have observed a good correlation between results obtained with our method and with radioimmunoassay.

Female↗

Correlations between free plasma estradiol and estrogens determined by bioluminescence in saliva, plasma, and urine during spontaneous and FSH stimulated cycles in women.

Estrone and estradiol (E1 + E2) concentrations in saliva were compared with four other parameters of estrogen status in five normal ovulatory women and ten FSH stimulated women selected for an in vitro fertilization program. E1 + E2 in saliva, plasma, and urine were assessed by a rapid, specific and sensitive enzymatic assay using bioluminescence. The free fraction of plasma estradiol was determined by equilibrium dialysis and total plasma estradiol by conventional radioimmunoassay. The pattern of E1 + E2 variation in saliva was similar to that of free plasma estradiol and the two parameters were correlated in both spontaneous and stimulated cycles. However, the lower correlation coefficient (r = 0.52, P less than 0.001) in spontaneous cycles compared with the high (r = 0.96, P less than 0.001) in the stimulated cycles shows that salivary E1 + E2 could be representative of plasma free estradiol in stimulated cycles but not in normal cycles. The free fraction of plasma estradiol reproduced the variation of total plasma estradiol in spontaneous as well as in FSH stimulated cycles and both parameters were strongly correlated (r = 0.91, P less than 0.001 and r = 0.90, P less than 0.001), respectively. The data show that salivary E1 + E2 concentrations are highly representative of the free fraction of E2 in plasma and at a lesser extend (r = 0.72, P less than 0.001) of total plasma E2 in FSH stimulated cycles.

Adult↗

Androgen and erythropoiesis: evidence for an androgen receptor in erythroblasts from human bone marrow cultures.

The techniques for culturing erythroid precursors made possible the study of the effect of steroids on these cells, and it has been well established that androgens and 5 beta-steroids have a direct effect on erythroid precursor cells from animal or human bone marrow. By contrast, their mechanisms of intracellular action remain poorly understood. We used tritiated methyltrienolone (R1881), a synthetic androgen that binds strongly to the androgen receptor, to characterize the binding activity in nuclear extracts of erythroblasts from human bone marrow cultures. The nuclear extracts contained binding sites that were saturable at low concentrations of 3H-R1881 (8-12 nM). Scatchard analysis revealed that the dissociation constant of the hormone-receptor complexes (Kd) was 10-20 nM, and the number of binding sites was 64-103 fmol/mg of protein. On linear sucrose density gradient analysis (5-20%), the hormone-receptor complexes sedimented in the region of 3.9 S. Finally, 5 beta-dihydrotestosterone had also a strong affinity for the binding sites. The nuclear component binding has all the physicochemical characteristics usually attributed to androgen receptors. These data strongly suggest that androgen action on erythropoiesis is mediated by a nuclear androgen receptor.

Bone Marrow Cells↗

Congenital adrenal hyperplasia associated with hyperphosphatemic rickets.

The authors describe a boy with precocious puberty due to adrenal hyperplasia associated with rickets, hypocalcemia, hyperphosphatemia, elevated PTH and alkaline phosphatase levels, and concentrations of 25-OH-D and 1,25-(OH)2D at the upper limit or above normal range. Treatment with hydrocortisone for 9 months did not normalize hypocalcemia and hyperphosphatemia. The addition of 1,25-(OH)2D3 (0.5-2 micrograms/day) to the corticoid treatment for 1 year was followed by a progressive normalization of plasma calcium, phosphorus, PTH and alkaline phosphatase concentrations with improvement of the osteomalacia on bone biopsy.

Adrenal Hyperplasia, Congenital↗

[Malignant arterial hypertension disclosing late congenital adrenal hyperplasia due to 17 alpha-hydroxylase deficiency].

17 alpha-hydroxylase deficiency is a rare form of congenital abnormality in steroid synthesis, usually associated with moderate arterial hypertension and suppression of the renin-angiotensin system in a young adult. We report on a 45 years old woman with malignant hypertension (220/135 mmHg, severe retinopathy with papilledema, progressive renal insufficiency with serum creatinine over 300 mumol/l) of recent onset. Biological exploration revealed a metabolic alkalosis, a moderate hypokalemia (3 mmol/l), with elevated urinary excretion of potassium. Plasma aldosterone concentration (33 ng/dl) and plasma renin activity (17 ng/ml/h) were elevated. Acute captopril administration was followed by a marked (-29 p. 100) decrease in mean arterial pressure. In this 46 XX patient, a primary amenorrhea had never been explored; clinical examination disclosed the absence of female secondary sex characteristics. Plasma cortisol was low (203 mmol/l) as were plasma androgens (testosterone 0.55, androstene dione 0.19, delta HEA less than 0.1 nmol/l respectively) and oestrogens (oestradiol 59 nmol/l). Elevated levels of progesterone and pregnenolone sulfate (12.1 and 2027 nmol/l respectively) contrasted with decreased levels of 17 OH progesterone (0.35 nmol/l). Computed tomography revealed a subnormal right adrenal gland and a pseudo-tumoral aspect on the left side. Treatment with dexamethasone and combined antihypertensive drugs (captopril, nifedipine and atenolol) resulted in normalisation of blood pressure and secretion of renin and aldosterone but renal function did not fully recovered. Thus, the hypertension of 17 alpha-hydroxylase deficiency can follow a malignant course in association with a marked activation of the renin-angiotensin system.

Adrenal Hyperplasia, Congenital↗

Decision-making system (DMS) applied to hematology. Diagnosis of 180 cases of anemia secondary to a variety of hematologic disorders.

We have developed a new decision-making system which includes interpretation of complete blood count (CBC). The system works using Bayes' rule. We tested the CBC program for the diagnosis of 180 cases of anemia covering 20 benign and malignant hematological disorders. The data entered were obtained from a Coulter S + IV/HD and the interpretation of blood smears. Clinical information was not used. In 64.5% of cases, the correct diagnosis was displayed in first rank and in 20%, in second or third rank, giving a total of 84% of quite satisfactory responses. There were only 5% incorrect responses, but the proposed complementary tests rectified the error. Computer-aided diagnosis can help pathologists, clinicians, students, and technicians to make rapid correct diagnoses and choose the appropriate tests to perform. These programs run on IBM PC or similar microcomputers and are available from Coulter Electronics, Hialeah, FL.

Anemia↗

[An aid in decision-making in hematology: characteristics and performances of the program. 200 cases of anemia].

An aid to decision programme has been applied to 200 cases of anaemia. Evaluation by the bayesian method rested on clinical data (age, sex, race) and laboratory data (blood count and differential, erythrocyte morphology). An accurate diagnosis was made initially in 107 cases, and for the first 5 diseases in 173 cases. The programme proved more effective than two clinicians recently trained in haematology. Devised for micro-computers, it can be used in routine practice and for teaching purposes.

Anemia↗

Partial androgen receptor deficiency and mixed gonadal dysgenesis in Drash syndrome.

Drash syndrome associates a nephropathy characterized by a diffuse mesangial sclerosis of early onset, Wilms tumor, and male pseudohermaphroditism (MPH). A patient with Drash syndrome is reported with the following: karyotype 46,XY, external genitalia near normal female, mixed gonadal dysgenesis, severe androgen receptor deficiency demonstrated for the first time in this syndrome. The possibility of a common genetic denominator with the del 11p13 WAGR complex is suggested. MPH/nephroblastoma association is common. Androgen receptor deficiency has been observed in one case of each syndrome, respectively.

Disorders of Sex Development↗

Determination of glycogen and enzymes of glycogen metabolism in human hair follicles.

The skin epithelium and its organelles use glycogen as well as glucose as source of energy. Therefore the characterisation of glycogen metabolism and the enzymes involved is important in the study of mechanisms regulating the normal or abnormal differentiation of skin organelles such as sebaceous glands and hair follicles. The present paper describes fluorimetric methods for the determination of glycogen and for the measurements of phosphorylase and phosphorylase kinase activity in one and the same lysate of minute tissue samples. The methods were tested for their suitability on freshly isolated human hair follicles and cultured hair follicle cells. The possible use of these techniques for studies on the pathophysiology of acne and hirsutism is discussed.

Cells, Cultured↗

Bioluminescence: an improvement in the enzymatic assay of dehydroepiandrosterone sulfate in biological fluids.

Dehydroepiandrosterone sulfate (DHEAS) determination in biological fluids was carried out by enzymatic hydrolysis and conversion into estrogens [estrone (E1) and estradiol (E2)] by the multienzyme system of human placental microsomes. The enzymatic complex consists of sulfatase, 3 beta-hydroxysteroid oxido reductase and 5en----4en isomerase which converts DHEAS into androstenedione (A); the latter component is further converted into estrogens by the aromatase. The resulting estrogens were determined from the NADH formed by the transhydrogenation reactions of human placental dehydrogenase. NADH was measured by bioluminescence. As little as 4 pg was assayable by this rapid enzymatic method, with a coefficient of variation of 8%. The results are in good agreement with radioimmunoassay and the method is suitable for routine use.

Dehydroepiandrosterone↗