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Query: UMLS:C0002895 (sickle cell disease)
 11,747 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Defects in neutrophil adhesion and migration may contribute to the susceptibility to infection seen in sickle cell anaemia (SCA). These dynamic defects may be influenced by abnormalities in blood rheology found in this disorder. A whole blood model was used to study neutrophil adhesion in SCA patients: neutrophil adhesion to protein coated glass was quantitated by measuring the rate of disappearance of neutrophils from heparinized whole blood circulating through a perfusion chamber. Twenty-three adult patients (Hb SS) were studied in asymptomatic steady state, of whom nine were also studied during pain crisis, both before and 4-7 d after conventional therapy. Red cell and granulocyte filterability and whole blood and plasma viscosity were also measured. The half-time for disappearance from the perfusion system (t1/2) of neutrophils from patients in the steady-state was 93.5 +/- 8.4 min, compared to 49.1 +/- 2.8 min in normal age-matched controls (P = 0.001). In crisis t1/2 was further prolonged to 170.0 +/- 16.1 min (P = 0.01 v. steady state). After therapy, t1/2 decreased to 57.0 +/- 4.5 min (P = 0.001 v. pre-therapy state and P = 0.009 v. steady state) and was comparable to Hb AA controls. These findings reveal a neutrophil adhesion defect in SCA which worsens in crisis but is corrected following supportive therapy. Red cell filterability (expressed as average resistance to flow and pore-clogging particles) and white cell filterability (expressed as pore-clogging particles) were also abnormal in SCA and were found to correlate with neutrophil adhesion. Plasma viscosity also correlated with adhesion t1/2. The defect appears to be related to abnormal blood flow properties in SCA but the rheological factors cannot fully explain either the steady-state defect or the marked changes in neutrophil adhesion during crisis.
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PMID:Abnormal neutrophil adhesion in sickle cell anaemia and crisis: relationship to blood rheology. 187 28

To investigate the combined effect of pneumococcal vaccination and penicillin prophylaxis on the progress of sickle cell disease, two groups of sickle cell disease patients, presenting with severe clinical manifestations of the disease were selected as cohorts. One group was vaccinated with the polyvalent pneumococcal vaccine and given penicillin prophylaxis, while the other group was not given the vaccine and penicillin. Clinical manifestations, frequency of hospitalization, crises and blood transfusion requirements, haematological parameters, and differential counts were recorded for each patient in the two groups. The 'severity index' of the sickle cell disease was calculated for each patient. No significant differences were encountered in the values of the haematological parameters except for the white cell count which was significantly higher in the non-vaccinated group. However, the frequency of hospitalization, crisis, and blood transfusion were significantly higher in the non-vaccinated group compared to the vaccinated group. Clinical symptoms frequently associated with severe sickle cell disease, were also encountered at a higher frequency in the non-vaccinated group. These results show that pneumococcal vaccination and penicillin prophylaxis increase the crisis-free interval in sickle cell disease patients, and play a significant role in decreasing the morbidity associated with sickle cell disease.
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PMID:Symptom-free intervals in sicklers: does pneumococcal vaccination and penicillin prophylaxis have a role? 235 5

Fifty-nine children with sickle cell anaemia (HbSS) or associated haemoglobinopathies were studied prospectively using a chromogenic Limulus amoebocyte lysate assay to detect circulating endotoxin. The 41 children with HbSS (mean age 8 years 9 months) had more serious disease than the 18 with HbSC disease (n = 14) or HbS-beta-thalassaemia (n = 4) (mean age 7 years 2 months), with a greater degree of splenomegaly, lower haemoglobin, and higher white cell counts, platelet counts and bilirubin values (P less than 0.05 for all). Twenty-nine children with HbSS had evidence of poor reticuloendothelial function, with red cell pitting of greater than or equal to 2%. Three of these 29 had low levels of endotoxin in plasma (0.12-0.24 endotoxin units (EU)/ml); two were clinically well, one had a painful crisis. Eight of 18 children with other sickle haemoglobinopathies had greater than or equal to 2% pitted red cells; none was endotoxinaemic. Therefore, in 37 patients with reticuloendothelial dysfunction, three were endotoxinaemic; all had sickle cell anaemia. Although not statistically significant, this suggests that endotoxinaemia may occur predominantly in patients with reticuloendothelial dysfunction, and is compatible with the hypothesis that systemic endotoxinaemia can derive from the intestine especially when reticuloendothelial function is depressed.
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PMID:Endotoxinaemia in sickle cell disease. 307 92

The iron status in children with sickle cell anaemia has been studied in 45 patients. The mean haemoglobin level was 7.4 g/dl (s.d. 1.7) and the white cell count more than 11 X 10(9)/l in 35 (78%). Bone marrow examination showed depletion of iron stores of 21 (47%). The total iron binding capacity was elevated in most of the children and serum iron was below the normal mean level for age in 14 (31%) children. The possible causes of iron deficiency in some of these children include dietary deficiency, infections, malabsorption, blood loss through hookworm infestation and growth spurt. In the management of a child with sickle cell disease, iron administration is not recommended unless there is evidence of coexisting iron deficiency.
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PMID:Iron deficiency in sickle cell anaemia in Nigerian children. 618 85

Partial red cell exchange transfusion is used to manage several complications of sickle cell anemia. Reports have stressed the safety, ease, and speed of red cell exchange procedures performed with blood cell separators, but little information exists concerning operating conditions for the most efficient removal of hemoglobin S (HbS) containing cells. We performed 13 red blood cell exchange transfusions with the IBM 2997 continuous flow blood cell separator on two previously transfused adult homozygous (SS) sickle cell patients. We used both a single stage and a dual stage disposable pathway and varied rotor speed, collection port, and collection interface independently. The patients' baseline percentage HbS ranged from 28 to 64% (mean = 46%); post exchange values ranged from 19.1 to 46% (mean = 32%). Thirty-three of 34 specimens collected from the white cell or platelet port showed a higher percentage of HbS in the collection line compared to simultaneous patient samples. Mean enrichment was 9.4% (range = -4.0 to 35.5%). There was an inverse relationship between the hemoglobin concentration and the percentage of HbS in the collected specimens (p = 0.001; N = 45). Differential separation of sickle cells should decrease the volume of blood required for partial red cell exchange of selected SS patients.
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PMID:Selective removal of sickle cells with the IBM 2997 continuous flow blood cell separator. 668 83

Haplotypes of the beta-like globin gene cluster in individuals with sickle cell disease appear to be important as prognostic factors for the severity of the disease. The Hpa I polymorphic site 3' of the beta-globin gene is very often involved in haplotyping. However, this restriction site cannot be ascertained by conventional PCR, as it is in the middle of a long repetitive sequence. Long distance PCR was employed to amplify this Hpa I-containing region. By positioning either one or both primers outside the repetitive sequence, specific amplification was achieved. Moreover, sufficient amplification can be obtained by using a white cell lysate instead of purified DNA, thereby significantly reducing the amount of blood needed, and the total procedure time.
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PMID:Haplotypes of the human beta-like globin gene cluster: analyzing the 3' Hpa I polymorphic site by long distance PCR. 766 26

Psoas abscess is usually associated with tuberculous spondylitis, but also occurs in relation to inflammatory bowel disease. We present 17 cases of primary pyogenic psoas abscess seen during a 10 year period at the King Fahd Hospital, Al-Khobar. There were 13 males and 4 females with a mean age of 24.9 years (range one to 55 years). Five patients had sickle cell disease. The average delay in presentation was 4.5 weeks (range 2 to 9 weeks). Pyrexia and a painful hip with a flexion deformity were the most obvious signs. The sedimentation rate and white cell count were markedly raised in every case. Staphylococci were cultured in 10 and anaerobic streptococci, salmonella and E coli in 2 each. Ultrasonography, CT and MR imaging were used to confirm the diagnosis. Early recognition and drainage are important to ensure a rapid recovery.
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PMID:Retrofascial nontuberculous psoas abscess. 856 44

Erythrocytes resistant to standard lysing reagents are known to occur in sickle cell disease. These lyse-resistant erythrocytes can cause aberrant automated leucocyte counts and differentials. The ability of the Cell-Dyn 3500 automated haematology analyser to eliminate resistant erythrocytes and accurately count and differentiate leucocytes was evaluated. Samples were obtained from paediatric patients with sickle cell disease or haemoglobin SC disease. The Cell-Dyn 3500, using impedance and optical counting with a hypotonic salt "extended lyse mode', was compared to the Cell-Dyn 3000, an optical analyser that also uses a hypotonic salt lyse, the Cell-Dyn 400, a "hard detergent lyse' impedance counter, and a reference 400-cell manual white cell differential (National committee for Clinical Laboratory Standards [NCCLS] Approved Guideline H20-A). Seventy-five samples from patients with sickle cell disease or haemoglobin SC disease were evaluated for total leucocyte count, percentage of lymphocytes, percentage of neutrophils, and nucleated red blood cells (NRBC) flags. The Cell-Dyn 3500 correlated well with Cell-Dyn 400 leucocyte counts, with a correlation coefficient of 0.95. When compared to the manual differential, the correlation coefficient for lymphocytes was 0.93 and for neutrophils 0.95. The Cell-Dyn 3500 NRBC flag had a sensitivity of 47.7% and a specificity of 80.6%. The predictive value of a positive flag was 77.7%. The Cell-Dyn 3500's extended lyse mode clearly enhances the accuracy of leucocyte counts and differentials in patients with sickle cell disease.
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PMID:Evaluation of the automated leucocyte count and differential from the Cell-Dyn 3500 in sickle cell disease. 869 28

This study examined the presence of a persistent state of low-grade inflammation in sickle cell anemia patients by measuring circulating sHLA-I heterodimers and C-reactive protein during the steady state and after recent crises. Thirty-nine pediatric sickle hemoglobinopathy patients were studied during the steady state and 11 patients were evaluated within 1 month of a painful crisis. A disease severity score was generated for each patient, and soluble HLA-I (sHLA-I) and C-reactive protein levels were determined. Soluble HLA-I was significantly elevated in 55% of the steady-state group and in 36% of the recent-crisis group. The percentage of patients with elevated sHLA-I differed in the various disease subgroups in the steady state: 46% of Hb SS patients, 70% of Hb SC patients, 75% of Hb S beta-thal patients, and 20% of Hb SSF patients. Steady-state and recent-crisis sHLA-I levels were not significantly different. C-reactive protein levels were elevated in 11% of steady-state patients and in 9% of recent-crisis patients. Soluble HLA-I levels did not correlate with C-reactive protein levels or disease severity score, age, hemoglobin, reticulocyte count, platelet count, or white cell count. These results show that the majority of sickle hemoglobinopathy patients have elevated sHLA-I levels during the steady state and after recent crisis, suggesting the presence of chronic inflammation during the steady state.
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PMID:Increased circulating levels of soluble HLA class I heterodimers in patients with sickle cell disease. 954 79

We prospectively evaluated the results of ultrasonography in 53 patients of sickle cell disease suspected to have vasoocclusive crisis/acute hematogenous osteomyelitis. The average age was 8.4 +/- 3.40 years (range, 1-14). Twenty-six children were boys and 27 were girls. Seventeen (32%) patients had ultrasonographic changes that suggested acute osteomyelitis. The minimal white cell count was 7,200/mm3, and maximal, 9,900/mm3 (mean, 8,190/mm3) in uninfected patients and in 17 patients, the mean was 10,300/mm3 (7,200-13,600/mm3). The mean erythrocyte sedimentation rate in uninfected patients was 32 for the first hour (19-36 mm), and in infected patients, it was 43 for the first hour (35-38 mm). Pus culture was positive in all infected patients, and the infective organism was Salmonella enteriditis in eight, staphylococcal species in six (S. aureus in four and S. epidermidis in two), and Streptococcus species 1 and 2, anaerobic streptococci. All patients with vasoocclusive crisis were treated with analgesics and intravenous fluids and did not require any further treatment. In patients with acute osteomyelitis, the treatment was incision, drainage and drilling of bone, and antibiotic therapy. We conclude that ultrasonography clearly and decisively differentiated acute osteomyelitis from vasoocclusive crisis in patients with sickle cell disease.
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PMID:Ultrasonography: can it differentiate between vasoocclusive crisis and acute osteomyelitis in sickle cell disease? 966 72


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