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

A rheological study has been made in 20 patients with sickle cell anaemia in the steady state and in the prodromal and established phases of 12 vaso-occlusive crises. Rheology of sickle cells was studied by discontinuous density gradient fractionation and by filtration through pores of 5 microns diameter. The prodromal phase of crisis (day 1), when compared with mean steady state values, was associated with the development of a sub-population of poorly deformable dense cells. This sub-population appeared 1 or more days before the acute-phase rise in C-reactive protein, orosomucoid, fibrinogen, plasma viscosity and leucocytes, and before the rise in serum lactate dehydrogenase. As crisis evolved, the sub-population decreased to steady-state values, or below, by days 6-7. Identification of the prodromal phase of sickle cell crisis has allowed the detection of rheological changes of potential aetiological significance.
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PMID:Rheological changes in the prodromal and established phases of sickle cell vaso-occlusive crisis. 139 Feb 48

Metabolic and serum changes during steady-state homozygous sickle cell (SS) disease are consistent with an acute-phase response and raise the possibility that inflammation occurs in SS disease even during the steady state. To test this hypothesis, we measured concentrations of acute phase reactants in patients with SS disease, in patients with sickle cell haemoglobin C (SC) disease, and in normal (AA) control subjects. The concentrations of C-reactive protein and serum amyloid A were increased above 10 mg/L and 5 mg/L, respectively (our definition of an acute-phase response) in 18% (26/143) of subjects with SS disease even when they were symptom free, in 17% (6/35) of subjects with SC disease, and in 1% (1/80) of AA controls (p < 0.001). We suggest that subclinical vaso-occlusion may generate a covert inflammatory response and that the cytokine mediators of this response may contribute to the metabolic abnormalities and growth failure in sickle cell disease.
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PMID:Is there an acute-phase response in steady-state sickle cell disease? 809 78

Serum concentrations of seven acute-phase reactants: albumin, transferrin (Tf), alpha-1-antitrypsin (AIAT), caeruloplasmin (Cp), alpha 2-macroglobulin (alpha 2-MG), haptoglobin (hp) and C-reactive protein (CRP) were determined in 73 subjects with varying severities of homozygous sickle cell (HbSS) disease. Fifty healthy subjects of comparable sex, age and socio-economic class distributions as the HbSS subjects served as controls. Albumin and alpha 2-MG were comparable in all the subject groups. Tf and hp levels were significantly reduced in the HbSS groups relative to the control group. Conversely, AIAT, CRP and CP were significantly elevated. However only Tf and CRP manifested significant correlations with any of the indices of disease severity employed. Transferrin and CRP are suggested as plasma proteins worthy of further evaluation as indicators of severity in homozygous sickle cell disease.
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PMID:Acute phase reactants and severity of homozygous sickle cell disease. 768 69

Two acute phase reactants (C-reactive protein: CRP and alpha 1-acid glycoprotein: alpha 1-AGP) and transferrin (Tf) levels change were measured in order to evaluate their clinical or prognostic value during the vaso-occlusive crisis. Measurements were performed in normal controls, painful crisis with or without treatment and during steady state. Immunochemical determinations indicate that markedly acute inflammation occurred in sickle cell painful crisis, as revealed CRP and alpha 1-AGP levels. Compared to controls, during periods of steady state in the absence of any intercurrent condition, a lesser but significative increase of alpha 1-GPA was observed. Transferrin level remained lower and did not sufficient to discriminate between crisis and steady state. Compared to the vasodilatator drug effect, non steroidal anti-inflammatory drug showed more efficiency in the sickle cell disease inflammatory process. Taken together, these findings indicate that serial determinations of CRP, alpha 1-GPA and Tf may be helpful in monitoring the course of sickle cell disease and response to treatment. Then, non steroidal anti-inflammatory drugs appear necessary to reduce the length of the sickle cell crisis.
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PMID:[Clinical value of C-reactive protein, alpha 1-glycoprotein acid and transferrin assay in homozygous sickle cell disease]. 829 21

Mycoplasma pneumoniae, a gram-negative bacteria, is an important cause of lower respiratory tract infection in children (20% of cases). The infection tends to be endemic and is punctuated by epidemic episodes every 4 to 7 years. Its frequency seems to be higher in children between 5 and 9 years of age, but is probably underestimated before 5 years. M.pneumoniae may cause multisystem infection. Diagnosis is established upon clinical data and laboratory findings. Usually, the infection is associated with leucocyte count under 15,000/mL and C-reactive protein under 50 m/L. Detection of M. pneumoniae DNA in clinical samples appears to have advantages over serological tests. Severe infections have been described in patients with humoral and cellular immunodeficiencies, sickle cell disease, cystic fibrosis. Treatment with macrolids and tetracyclines (after 8 years of age) is indicated. Respiratory functional sequelae are possible.
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PMID:[Bronchopulmonary infections caused by Mycoplasma pneumoniae in children]. 897 63

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

The occurrence of a fat embolism syndrome (FES) can be explained by two hypothetic mechanisms. In the mechanical hypothesis, bone marrow enters into the cardiovascular system during an intramedullary peak pressure. This peak could occur during either long bone fracture and/or intramedullary nailing or cemented or noncemented arthroplasty. According to the biochemical hypothesis, the FES could occur in nontraumatic conditions such as lipid emulsion infusion or sickle cell disease. The C-reactive protein is a possible factor for destabilizing plasma fat (chylomicrons or Intralipid liposomes). Treatment with heparin has been reported to interfere with lipid metabolism through a "creaming" phenomenon. Plasma fatty acids increase lipid peroxidation, with potential severe oxidative stress of lung. Vascular lung injury is increased by granulocytes and the clotting cascade is activated by neutral fat. After a symptom-free period, the full clinical picture is characterized by pulmonary insufficiency with hypoxaemia, neurological impairment, pyrexia and petechial haemorrhages. The accurate incidence cannot be assessed as many subclinical forms remain unrecognized. Transoesophageal echocardiography with color-flow Doppler allows considerable insight into the sequence of embolic events and patent foramen ovale (PFO). A PFO induces an increase in right-to-left shunt in case of an elevated intrapulmonary pressure. PFO might elicit systemic manifestations of the FES, particularly with neurological impairment. Carotid ultrasonography helps to visualize embolism. Magnetic resonance imaging of cerebral fat emboli is a better diagnostic tool for detecting brain embolism than computerized tomography. Quantification of cells containing fat droplets in bronchoalveolar lavage material could also be helpful. Pulmonary microvascular cytology analysis of capillary blood samples obtained through a pulmonary artery catheter in combination with blood gas changes are of value for earlier stage FES. Prophylactic and therapeutic measures are aimed to counteract the various mechanisms leading to FES. The decrease in time delay of fracture management is probably the most effective prophylactic means. A reaming procedure can be noxious, particularly in a patient with a severe thoracic trauma. The insertion without reaming of a small diameter nail, plating or external fixation have several advantages. Albumin infusion is recommended for restoration of blood volume and binding of fatty acids. Among pharmacologic measures, only corticosteroids have a proven benefit, not only for prophylaxis but also for therapy. Aprotinin and heparin are beneficial in counteracting blood cell aggregation. A prophylactic use of vena cava filters has been advocated. Prevention or early treatment of hypovolaemia and hypoxaemia are essential.
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PMID:[From fat emboli to fat embolism syndrome]. 968 75

To identify a possible acute phase response during the steady state of sickle cell disease, we estimated the serum alterations of acute phase proteins, beta2-microglobulin (beta2M), kappa and lambda light chains, interleukins (ILs) and tumor necrosis factor-alpha (TNFalpha) in 21 patients. Increased concentrations of C-reactive protein (CRP) were found in 5 patients, alpha-1-acid-glycoprotein (AGP) in 3, alpha-1-antitrypsin (AAT) in 8, ceruloplasmin (CER) in 2, alpha-2-macroglobulin (AMG) in 14 and decreased haptoglobin (HPT) and transferrin (TFR) in 11 and 9, respectively. Increased beta2M was found in 10 patients and kappa and lambda light chains in 11. IL-1beta, IL-2, IL-4, IL-10 and TNFalpha were not detected in any of the patients. However, significantly increased values of IL-6 and sIL-2r were found. This study has demonstrated increased serum levels of some of the acute phase proteins in patients during the steady state of sickle cell disease. This may be a result of a subclinical vaso-occlusion which in turn leads to a covert inflammatory response. Cytokines, and in particular IL-6, produced after this response, seem to be responsible for the high levels of acute phase proteins in the steady state of this disease.
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PMID:Acute phase proteins and interleukins in steady state sickle cell disease. 968 92

Sickle cell anemia is characterized by painful vaso-occlusive crises. It is hypothesized that monocytes are activated in sickle cell disease and can enhance vaso-occlusion by activating endothelium. To test this hypothesis, human umbilical vein endothelial cells (HUVEC) and human microvascular endothelial cells (MVEC) with sickle and normal mononuclear leukocytes were incubated, and endothelial activation was measured. Endothelial cells incubated with sickle mononuclear leukocytes were more activated than those incubated with normal mononuclear leukocytes, as judged by the increased endothelial expression of adhesion molecules and tissue factor and the adhesion of polymorphonuclear leukocytes (PMNL). Monocytes, not lymphocytes or platelets, were the mononuclear cells responsible for activating endothelial cells. Sickle monocytes triggered endothelial nuclear factor-kappa B (NF-kappaB) nuclear translocation. Cell-to-cell contact of monocytes and endothelium enhanced, but was not required for, activation. Antibodies to tumor necrosis factor-alpha (TNF-alpha) and interleukin-1-beta (IL-1beta) blocked activation of the endothelium by monocytes. Peripheral blood monocytes from patients with sickle cell disease had 34% more IL-1beta (P =.002) and 139% more TNF-alpha (P =.002) per cell than normal monocytes. Sixty percent of sickle monocytes expressed the adhesion molecule ligand CD11b on their surfaces compared with only 20% of normal monocytes (P =.002). Serum C-reactive protein, a marker of systemic inflammation, was increased 12-fold in sickle serum than in normal serum (P =.003). These results demonstrate that sickle monocytes are activated and can, in turn, activate endothelial cells. It is speculated that vascular inflammation, marked by activated monocytes and endothelium, plays a significant role in the pathophysiology of vaso-occlusion in sickle cell anemia.
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PMID:Activated monocytes in sickle cell disease: potential role in the activation of vascular endothelium and vaso-occlusion. 1100 97

In sub-Saharan Africa, anaemia in pregnancy results from multiple causes including malaria, iron deficiency and haemoglobinopathies. In a cross-sectional study among 530 pregnant women in Ghana in November-December 1998, red blood cell indices were analysed with respect to malaria, serum concentrations of ferritin and C-reactive protein (CRP), and the haemoglobin and alpha-globin genotypes. Anaemia (haemoglobin [Hb] < 11 g/dL) was found in 54% of the women; 63% harboured malaria parasites at predominantly low numbers. Ferritin levels were considerably influenced by malaria and inflammatory processes (CRP > 0.6 mg/dL). Depending on the definition applied, the prevalence of iron deficiency ranged between 5% and 46%. The HbAS trait was observed in 14%, HbAC and elevated HbF in 7% each, and sickle cell disease in 1%. Heterozygous beta-thalassaemia was present in 1% of the women and alpha(+)-thalassaemia in 33% (29% heterozygous, 4% homozygous). Women with HbAS had higher malaria parasite densities than those with HbAA. In individuals with highly elevated HbF (> 10%), parasitaemia occurred in 27% only. Low gravidity, second trimester of pregnancy, malaria, raised CRP levels, and homozygous alpha(+)-thalassaemia were independent risk factors for anaemia in multivariate analysis. alpha(+)-Thalassaemia, however, was associated with a lesser degree of malarial anaemia when compared to non-thalassaemic women. Iron deficiency appears not to be a major health problem in this population. Haemoglobinopathies are common but, except for homozygous alpha(+)-thalassaemia, do not substantially contribute to anaemia in pregnancy. alpha(+)-Thalassaemia ameliorates malarial anaemia in pregnant women.
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PMID:Anaemia in pregnant Ghanaian women: importance of malaria, iron deficiency, and haemoglobinopathies. 1113 70


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