Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0002895 (sickle cell disease)
 11,747 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The relationship between the number of units of blood transfused and indicators of iron status in 37 patients with sickle cell anaemia (Hb SS), SC disease (Hb SC) or S beta-thalassaemia has been studied. The correlation coefficient between serum ferritin and the number of units transfused was good (r = 0.86), provided that ferritin samples taken within one week following a crisis were excluded. The relationship of transfusion history to serum ferritin in the steady state showed a similar relationship to that previously observed for other multiply transfused patients. The serum ferritin taken within 7 days of a painful crisis was significantly greater than the serum ferritin from the same patients in the steady state (p less than 0.025). The serum alanine transaminase did not rise as consistently as the serum ferritin during crises; it correlated with the serum ferritin but not the transfusion burden in the steady state. Transferrin iron saturation correlated less clearly with transfusion history than serum ferritin (r = 0.62). Patients who had received exchange transfusions were less likely to be iron-overloaded (ferritin increment per unit of blood = 9.9 +/- 3.8 micrograms/l) than patients who had received an equivalent number of units by conventional transfusion (ferritin increment per unit of blood transfused = 25.1 +/- 2.42 micrograms/l).
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PMID:Transfusion and exchange transfusion in sickle cell anaemias, with particular reference to iron metabolism. 312 Apr 72

Iron studies were performed in 22 pregnant and 18 non-pregnant women with haemoglobinopathies. Mean packed cell volume and mean haemoglobin concentration were significantly lower (p < 0.001) in haemoglobin SS patients than in haemoglobin SC patients, in both the pregnant and non-pregnant groups. Transferrin saturation was significantly lower in pregnant patients (haemoglobin SS and SC) than in the non-pregnant group (p < 0.001). Serum ferritin values in the haemoglobin SS and SC pregnant patients were not significantly different (p > 0.05). There was a strong correlation between serum ferritin levels and transferrin saturation in the pregnant group (r = 0.71; p < 0.001). Fourteen of the 22 pregnant women (63 per cent) and 9 of the 18 non-pregnant women (50 per cent) had scanty or no iron in the bone marrow; the serum ferritin levels increased progressively with greater amount of haemosiderin in the bone marrow. There was evidence of iron deficiency in both the pregnant and non-pregnant women with haemoglobinopathies and this suggests the need for further study on the routine administration of iron in the management of patients with sickle cell disease.
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PMID:Iron studies in pregnant and non-pregnant women with haemoglobin SS or SC disease. 743 71

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

It is becoming increasingly accepted that various diseases have a capacity to alter the composition of plasma proteins. This alteration in protein composition may consequently change the targeting capacity of nanoparticles (NPs). In this study, the impact of a model targeting ligand's (i.e., Transferrin; Tf) concentration in human plasma on the targeting capacity of gold NPs (Au NPs), pre-conjugated with Tf, is investigated. Our findings demonstrate that the protein corona formation by both healthy and Tf depleted human plasma diminishes the targeting efficacy of Au NPs within human cancer cells despite a preservation of targeting ability by plasma with excess Tf (10-fold). Moreover, the plasma samples obtained from patients with various Tf levels (e.g., thalassemia major, sickle cell anemia, aplastic anemia, and iron deficiency anemia) have affected the accessibility of the targeting Tf in the corona layer and subsequently affected their targeting ability, which emphasizes the critical role of disease-specific protein corona on the efficacy of Au NPs. Ultimately, variations of protein concentration (e.g., due to disease occurrence and progress) in plasma affect its recruiting in corona formation, and in turn, affect the targeting and therapeutic efficacies of Au NPs.
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PMID:Impact of plasma concentration of transferrin on targeting capacity of nanoparticles. 3205 94