PubMed Health⌕ Search

Biomedical subjects

S Safai-Kutti

Publications and source records attributed to S Safai-Kutti.

At least 19 recordsLinked to original sources

The incidence and survival of acute de novo leukaemias in Estonia and in a well-defined region of western Sweden during 1982-1996: a survey of patients aged > or =65 years.

OBJECTIVES: To compare the incidence and survival of acute de novo leukaemias with particular reference to political/socio-economic and environmental factors in two neighbouring countries over the three 5-year periods (1982-1996). PATIENTS: The present report covers only patients diagnosed when aged > or =65 years. SETTING: A well-defined area of Sweden, the so-called Western Swedish Health Care Region and Estonia. Population-wise, the western Swedish Region and Estonia are very similar; area-wise they are also well comparable. RESULTS: The number of acute de novo leukaemias was quite dissimilar in the two countries (Estonia, n = 137, Sweden, n = 354). The age standardized incidence rates regarding the total number of acute de novo leukaemias was 5.31 per 100,000 inhabitants/year for Estonia and 7.99 for Sweden, this difference being statistically significant. However, the difference was merely attributable to incidence rates as regards acute myeloblastic leukaemias (AML); on the contrary, differences as regards acute lymphoblastic leukaemias (ALL) and non-classifiable, undifferentiated or biphenotypic acute leukaemias (uAL) were negligible. The relative survival for the total material of patients was significantly higher for Swedish when compared with Estonian patients (P < 0.001). Thus, the relative survival for the total material of patients aged > or =65 years in Estonia at 1 year was 8.5% and at 3 years 3.5% respectively. The corresponding figures for the Swedish patients were considerably higher, 22.7 and 7.7% respectively. This difference, however, applied only for patients with AML (P < 0.001), whereas the results for patients with ALL and uAL were equally dismal. CONCLUSION: The results clearly reflect how political and socio-economic factors may influence the survival of acute leukemia patients in two neighbouring countries.

Aged↗

Trends in the incidence of chronic Philadelphia chromosome negative (Ph-) myeloproliferative disorders in the city of Göteborg, Sweden, during 1983-99.

OBJECTIVE: In the literature the incidence rates for the chronic Philadelphia chromosome negative (Ph-) myeloproliferative disorders (MPD) are known to vary extensively; only a few studies have, however, been concerned with incidence trends over time. Therefore, the aim of the present work was to investigate possible trends as regards incidence rates over time for Ph-MPD. DESIGN: Herein, we carried out a retrospective population-based survey on the incidence of polycythaemia vera (PV), essential thrombocythaemia (ET) and chronic idiopathic myelofibrosis (IMF), in the city of Göteborg (Sweden), covering the years 1983-99. RESULTS: The study comprised 416 patients with Ph-MPD. There were 205 patients with PV, 153 patients with ET, 34 with IMF and 24 with unclassified MPD. The annual incidence for PV was 1.97 per 10(5) inhabitants; the corresponding figures for ET and IMF were 1.55 per 10(5) and 0.30 per 10(5) inhabitants, respectively. There was a significant increase in the annual incidence rate for ET (P = 0.008); this increase was significant for male subjects (P = 0.015) but did not reach significance for females (P = 0.118). No such increase over time was recorded as regards PV and IMF. CONCLUSIONS: The increasing annual incidence rate for ET is most possibly explained by the more frequent use of automated platelet counts whenever a patient consults a doctor. Thereby, an increasing number of patients with overt thrombocytosis of unknown origin are discovered and will be referred to specialists within the field of haematology for a correct diagnosis.

Adult↗

On the diagnosis of polycythaemia vera as assessed in the health and medical care in the Västra Götaland region, Sweden.

OBJECTIVES: The aim was to assess how the diagnosis of polycythaemia vera (PV) was established and to study to which extent the classic Polycythemia Vera Study Group (PVSG) criteria and the revised criteria for the diagnosis of PV as proposed by Pearson and Messinezy in 1996 (PM criteria) were fulfilled when the diagnosis of PV was made. DESIGN AND SETTING: A questionnaire was sent to physicians in charge of haematological patients at the departments of medicine in 12 hospitals in the Health and Medical Care in the Västra Götaland Region (VGR), Sweden, with a population of 1.5 million inhabitants; they were asked to provide reports as regards all patients with an unequivocal diagnosis of PV during a 5-year period from January 1994 to December 1998. SUBJECTS: Full reports were obtained from six hospitals, which serve about 1.1 million inhabitants. The results from a total of 129 PV patients, 62 from two university hospitals (UHs) and 67 from four county hospitals (CHs), were the subject of the present analysis. Results. It was shown that measurement of the red cell mass (RCM) had been carried out in 61 of 62 (98%) patients in UHs compared with 24 of 67 (36%) patients at CHs (P < 0.01). By using ultrasound imaging and/or scintigraphy the spleen size had been determined in 55 of 62 (89%) patients at UHs and in 24 of 67 (36%) patients at CHs (P < 0.01). At the UHs, arterial oxygen saturation had been measured in 32 of 62 (52%) patients; the corresponding figure for the CH patients was 22 of 67 (33%). Plasma or serum erythropoietin (EPO) concentrations had been measured in 44 of 62 (71%) of PV patients at UHs and in 31 of 67 (46%) of patients at CHs; in all these cases the EPO concentrations were subnormal or not measurable. CONCLUSIONS: At the UHs only 37% of the PV patients fulfilled the PVSG criteria for the diagnosis of PV whereas the corresponding figure for CHs was 4% (P < 0.01). The adherence to PM criteria was, however, 71% at UHs compared with 16% at CHs (P < 0.01).

Adult↗

No increased frequency of trisomies 8 and 9 by fluorescence in situ hybridization in untreated patients with essential thrombocythemia.

Essential thrombocythemia (ET) is one of the diseases included among the myeloproliferative disorders in which trisomies for chromosomes 8 and 9 commonly occur. In ET, only a few patients are known to show clonal abnormalities. With fluorescence in situ hybridization (FISH), interphase cells can be evaluated and clones can be detected even though not revealed by conventional cytogenetic methods. By using FISH for enumeration of chromosomes 8 and 9 in bone marrow cells, we studied 22 patients with ET; 20 of them were investigated at the time of diagnosis when they were still untreated with myelosuppressive agents. Only two patients had trisomy 8; one of them was also found to have +8 with conventional cytogenetics. None of the patients had trisomy 9; two patients had borderline values in comparison to a control group. Thus, in ET, no increased frequency of patients with trisomy for 8 or 9 at the time of diagnosis could be detected with FISH.

Adult↗

The red cell mass, plasma erythropoietin and spleen size in apparent polycythaemia.

It has been shown previously that measurement of the spleen size and plasma erythropoietin (EPO) concentration are valuable adjuncts in the diagnostic work-up of patients with polycythaemia vera. The aim of the present work was to evaluate their value in the assessment of apparent polycythaemia (AP). Therefore, over a 24-month period we routinely performed bone marrow biopsies, measurement of red cell mass (RCM) and plasma volume (PV), spleen size determination by gamma camera scintigraphy and determination of the plasma EPO concentration in consecutive patients referred to us because of elevated values for packed cell volume (>0.48 in females and >0.51 in males). After having excluded patients with clonal and secondary polycythaemias we were left with 38 patients (27 males and 11 females) with AP. In all of them the measured RCM was within normal range, i.e. <36 ml/kg for males and <32 ml/kg for females. The subjects were characterized by moderate increase in RCM and a concomitant moderate decrease in PV. Thus, as an average the measured RCM exceeded the predicted values by 14% in males and by 12% in females; conversely, as compared to the predicted values the average measured value for PV was reduced by 17% in males and by 8% in females. The average RCM for males was 29+/-3 ml/kg; the corresponding figure for females was 23+/-4 ml/kg. It was shown that 86% of the subjects had plasma EPO concentrations within the control range; the remaining had values slightly above or below the control range. The mean posterior spleen scan area was 57+/-16 cm2 and mean left lateral area 57+/-17 cm2; the reference value for spleen scan area (for both projections) is 57+/-12 cm2. Of the patients 35/38 (92%) had a spleen scan area within the mean+2SD for controls and 38 subjects (100%) had values within the mean+3SD. It is concluded that measurement of plasma EPO and a careful assessment of the spleen size should always be considered in the evaluation of patients with elevated values for venous packed cell volume.

Adult↗

Zinc therapy in children with cystic fibrosis.

The effect of oral zinc supplementation in patients with cystic fibrosis (CF) was investigated in a placebo-controlled, double-blind, crossover study with each treatment period covering 6 months. CF patients (n = 13, aged 2 years, 3 months to 19 years, 1 month) started with placebo and after 6 months, they received zinc therapy. Another 13 patients (aged 3 years, 5 months to 16 years, 10 months) started in the reverse order. Before zinc supplementation, all CF patients had low plasma levels of zinc which normalized during treatment. This effect was, however, transient. CF patients also had low concentrations of plasma selenium. A small decrease in the number of leukocytes was also noted during zinc therapy. In response to zinc treatment, no changes in the clinical status of the patients were observed either by the investigators or by the patients. Growth velocity was the same during the placebo and zinc treatment periods. No significant changes in lung function occurred in response to either placebo or zinc. It appears that the observed low plasma zinc concentration in CF patients was due to an impaired zinc absorption from the gut which was counteracted by an increased supply of oral zinc. No beneficial effect from zinc supplementation on clinical status, growth velocity, or lung function was found in this study.

Adolescent↗

Oral zinc supplementation in anorexia nervosa.

There is evidence to suggest that zinc (Zn) deficiency may be involved in the pathogenesis of anorexia nervosa (AN). In an open study of 20 females, aged 14-26 years, afflicted with AN the effect of oral zinc supplementation was investigated. In each case the diagnosis of AN was based on the criteria of DSM-III-R. After a careful history, complete physical examination and laboratory screening the subjects were started on 45-90 mg of Zn2+, as zinc sulfate, (SolvezinkR, Tika, Sweden) per day. During a follow-up period of 8-56 months 17 patients increased their body weight by more than 15%. The maximum gradual weight gain of 57% was encountered in one patient after 24 months of zinc therapy. The most rapid weight gain was recorded in a patient who increased her body weight by 24% over a period of 3 months. After the institution of zinc, weight loss was not registered in any of our patients. In 13 subjects the menstruation returned 1-17 months after the initiation of zinc therapy. None of our patients developed bulimia. The design of an ongoing multicenter placebo-controlled clinical trial of zinc supplementation to patients with AN is described.

Administration, Oral↗

In vitro platelet function in infantile autism.

It has previously been demonstrated that patients with infantile autism demonstrate impaired in vivo platelet behaviour. Therefore, in 14 children (13 boys and 1 girl) with infantile autism (aged 2-14, mean 6 years) and 12 healthy control boys (aged 6-15, mean 11 years) we studied in vitro platelet reactivity using ADP- and collagen-induced platelet aggregation. In each child a total of 7 different final concentrations of ADP and 4 different concentrations of collagen were employed. At all concentrations of ADP and collagen used the autistic children consistently exhibited diminished platelet aggregability; the differences, however, did not reach statistical significance. Therefore a wider panel of in vitro tests is apparently required and a larger group of patients be studied to help elucidate the functional/metabolic platelet defect met in infantile autism.

Adolescent↗

Splenic platelet kinetics in systemic lupus erythematosus (SLE).

The splenic blood flow, intrasplenic platelet kinetics and spleen size were determined in 8 females with systemic lupus erythematosus (SLE), all without signs of active disease, by using gamma-camera scintigraphy with 111In-labelled platelets and 99mTc-stannous colloid. The results for splenic blood flow, intrasplenic platelet transit time and splenic platelet pool size, obtained by compartmental analysis of the initial distribution of radiolabelled platelets between blood and spleen, did not differ from those of a control group. In all SLE patients the spleen size was within normal limits. There was a significant relationship between the spleen volume and the splenic platelet pool size (r = 0.75; p less than 0.05), and between the spleen volume and splenic blood flow (r = 0.76; p less than 0.05). A borderline, inverse correlation was present between an estimate of splenic perfusion and intrasplenic platelet transit time (r = 0.62; p = 0.1). It is concluded that the splenic function, measured as splenic blood flow and intrasplenic platelet kinetics, is not disturbed in SLE patients without active disease.

Adult↗

Plasma concentrations of platelet-specific proteins and serum thromboxane B2 production in response to treatment with dipyridamole. A pilot study of 27 post-myocardial infarction patients.

In the present study 27 post-myocardial infarction patients were treated with Persantin capsules (Depot-Kapseln, sustained release form), containing 200 mg dipyridamole, b.i.d. over a period of 3 weeks. Baseline levels for plasma beta-thromboglobulin (BTG), platelet factor 4 (PF4), and serum thromboxane B2 (TXB2) were obtained on day 0 and subsequently on days 1, 3, 5, 7, 14 and 21. The baseline levels for plasma BTG and PF4 as well as for serum TXB2 significantly exceeded those for a control group consisting of healthy subjects. The plasma values for BTG and PF4 remained unchanged during the whole study period. During the first week of study the levels for serum X TXB2 were unchanged; however, on days 14 and 21 the means for TXB2 dropped significantly. There is experimental work to suggest that dipyridamole may exert an inhibitory effect on platelet thromboxane biosynthesis. The present results support this concept.

Adult↗

The effect of acetylsalicylic acid in 3 different formulations on in vitro and in vivo platelet function tests. An experimental study on healthy male volunteers.

The template bleeding time (TBT), ADP-induced platelet aggregation, and serum production of TXB2 were measured in healthy young male subjects immediately before, and on days 1, 4 and 6 after the ingestion of 1 single dose of 500 mg acetylsalicylic acid (ASA) in 3 different formulations: Aspirin (Bayer), and the 2 enteric-coated formulations Reumyl (Hässle) and Premaspin (Lääke). The ingestion of Aspirin resulted in a significant prolongation of the TBT over a period of 6 d. However, after the ingestion of the same amount of ASA in the 2 enteric coated formulations, the TBT as measured on day 6 had become normalized. After the ingestion of Aspirin, there was no reappearance of the second wave of ADP-induced platelet aggregation during the study period; however, after the ingestion of the 2 enteric-coated formulations, secondary platelet aggregation occasionally returned on day 6. In response to the intake of each of the 3 ASA formulations, the serum TXB2 production as measured 24 h later was almost completely inhibited. In each of the 3 study groups, the TXB2 formation as measured on day 6 was still significantly impaired.

Adult↗

Successful treatment of refractory autoimmune haemolytic anaemia by plasmapheresis.

A 16-year-old female was admitted because of rapidly progressive fatigue, severe anaemia and icterus. The S-bilirubin was 190 mumol/l, COHb 7.3% and high amounts of free Hb in plasma were present. The Coombs' direct test was strongly positive with anti-IgG but negative with anti-IgM and anti-C3. Conventional therapy with very high doses of hydrocortisone i.v., cyclophosphamide, azathioprine, and transfusions of washed packed red cells proved ineffective. During 5 consecutive days she also received i.v. infusions of gamma-globulin (25 g each day). Nevertheless, her condition deteriorated and 3 plasma exchanges were carried out with impressive clinical and laboratory effects. After the 3rd plasma exchange, the patient did not require further transfusions of packed red cells. Therapy with corticosteroids could be rapidly reduced and she was discharged after 5 weeks with a normal blood picture. Since then she has remained in excellent health.

Adolescent↗

Plasma platelet factor 4: a potentially useful predictor of ischaemic heart disease?

The aim was to investigate whether plasma platelet factor 4 (PF-4) in suspected acute myocardial infarction (AMI) patients could serve as a prognostic tool and identify patients at risk for future death from AMI or ischaemic heart disease (IHD). Therefore, upon admission to our coronary care unit plasma PF-4 was measured on 109 consecutive patients. 53 of them proved to have AMI, and 50 IHD but no AMI; the remaining 6 had no evidence of IHD. 24 patients died in hospital or during the follow-up period which was an average of 16.7 +/- 2.4 months. The decreased were subgrouped into those dying of AMI (n = 16), and those dying of IHD but with no AMI (n = 8). No deaths from other causes were recorded. As compared with survivors there was a tendency towards higher PF-4 values among those who died of AMI. However, patients who during follow-up suffered death from IHD proved to have significantly (p less than 0.05) higher PF-4 levels than survivors.

Adult↗

The platelet factor 3 assay and circulating immune complexes as studied on non-thrombocytopenic patients with systemic lupus erythematosus.

Sera from 21 patients with systemic lupus erythematosus (SLE) were tested with a platelet factor 3 (PF3) assay. A control serum (pooled blood group AB serum) was always run in parallel with the test sera. The test and control sera were run in duplicate and for each patient the mean recalcification time was used for the calculation of the test serum: AB serum ratio. Concomitantly, the patients' serum concentrations of circulating immune complexes (CIC) were measured by using the Raji cell radioimmunoassay. 20 healthy hospital employees served as controls. In the latter subjects the mean test serum: AB serum ratio was 0.99 (range 0.92-1.09), their CIC concentrations being less than or equal to 16 micrograms/ml. 7 of the SLE patients had CIC values less than or equal to 16 micrograms/ml, and their test serum: AB serum ratios ranged 0.95-1.09. All the remaining SLE patients had elevated values for CIC. In the total material of SLE there was a highly significant (r = -0.87; P less than 0.001) negative correlation between the test serum: AB serum ratios and the values for CIC. It is widely held that the so-called PF3 immunoinjury test detects circulating antiplatelet antibodies. The present results, however, strongly suggest that the assay rather or also detects CIC which like antiplatelet antibodies may damage platelets as we and others have shown in prior publications.

ABO Blood-Group System↗