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)

Vaccine-induced levels of antibody to Streptococcus pneumoniae of approximately 250-300 ng of antibody nitrogen/ml are protective against pneumococcal disease. Side effects of vaccination are not severe and are generally confined to local reactions at the site of inoculation. Patients with a documented high risk of acquiring pneumococcal disease include the elderly, especially those with underlying cardiopulmonary disease, and those with sickle cell anemia, Hodgkin's disease, a renal transplant, multiple myeloma, asplenia, and nephrotic syndrome. People with insulin-dependent diabetes mellitus or renal failure do not appear to be at high risk. All of these groups, except those with multiple myeloma, respond to vaccine with levels of antibody that are protective for many but not all of the serotypes included in the vaccine. Immunosuppression, splenectomy, and hemoglobinopathy depress antibody response. Duration of vaccine-induced antibody is unknown but may be shorter than that in normal persons. Preliminary guidelines for vaccination are proposed.
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PMID:Assessment of the antibody response to pneumococcal vaccine in high-risk populations. 702 58

High-risk patients who should receive the pneumococcal vaccine include asplenic patients, those with sickle cell anemia, nephrotic syndrome, congestive heart failure, pulmonary or renal disease, insulin-dependent diabetics, alcoholics with cirrhosis and immunocompromised patients. The vaccine should not be used in children under two years of age. It is safe, relatively inexpensive and effective when antibody response can be generated.
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PMID:Use of the pneumococcal vaccine. 729 14

A concentrated, DNA-free extract of histone acetylase A was prepared from calf thymus tissues in two simple steps, which exploit the ability of polyethylene glycol to precipitate both nucleic acids and proteins from solutions containing high concentrations of salt (Alberts, B., and Herrick, G. (1971) Methods Enzymol. 21, 198-217). This extract was then chromatographed on four successive columns. The use of 75 microgram/ml of insulin as a carrier protein in all of these later steps, plus the inclusion of 1 M urea in some column buffers, has been useful in improving both the yield and reproducibility of the purification. The highly active enzyme obtained has a molecular weight of about 70,000, and the best fractions could be about 30% pure. Our data indicate that the acetylase A is only a very minor protein in cells, being present in perhaps a few thousand molecules per cell.
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PMID:Extensive purification of histone acetylase A, the major histone N-acetyl transferase activity detected in mammalian cell nuclei. 744 May 47

We interviewed 170 sickle cell disease (SCD) patients (mean age 25 years) with a modified version of the Frankfurter Befindlichkeitskala (FBS, 33-item) and the 12-item General Health Questionnaire (GHQ-12), with a view to highlighting the psychosocial issues which worry them, the way they cope with these problems, and the factors associated with these issues. The mean FBS score of SCD patients was comparable with those of insulin dependent diabetics, but significantly higher than that of non-insulin dependent diabetics. The FBS scores were significantly correlated with GHQ-12 scores. Feelings of inadequacy of social contact were significantly associated with high FBS and GHQ scores. Some common complaints were: the limitations illness placed on social life; depressive feelings; abnormal habitus; suicidal ideation during crises; and the burden of illness on the family. They frequently resorted to prayers as a method of coping, as most had no clear ideas on how to deal with these issues. Worries over psychosocial consequences of SCD, seem to add considerably to the burden of illness, and clinicians will offer better care to patients if they routinely enquire into some of these issues and offer health education and counselling in a group setting.
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PMID:The psychosocial problems of sickle cell disease sufferers and their methods of coping. 779 34

Serum growth hormone (GH), cortisol, free thyroxine (FT4), thyroid-stimulating hormone (TSH), and insulin like growth factor I (IGF-I) concentrations were measured in 15 children with sickle cell disease (SCD) together with their heights < 5th percentile for age and gender, and in 15 healthy age-matched children who had normal variant short stature (NVSS). GH response to an oral dose of clonidine (0.15 mg/m2) and cortisol response to ACTH stimulation were determined in the two groups. Children with SCD had significantly lower serum concentrations of IGF-I and decreased GH response to stimulation. Eight out of the 15 children with SCD did not mount an appropriate GH response to clonidine provocation (> 10 micrograms/l). CT scanning of the hypothalamic-pituitary area in those eight children with SCD revealed a partial or complete empty sella in all of them. It appears that defective GH release, and consequently low IGF-I production and slow growth velocity in children with SCD might be secondary to hypoxic-vascular insults to their hypothalamic-pituitary axis during one or more of the sickling episodes.
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PMID:Circulating growth hormone (GH), insulin-like growth factor-I (IGF-I) and free thyroxine, GH response to clonidine provocation and CT scanning of the hypothalamic-pituitary area in children with sickle cell disease. 853 Dec 60

Between 1989 and 1995, 32 patients underwent combined kidney-pancreas transplantation for diabetic chronic renal failure. Only one of these patients received an isolated pancreas following cessation of function of a previously implanted segmental pancreas. The surgical technique always consisted of pure retroperitoneal transplantation into the right iliac fossa of a total pancreas transplant with duodenovesical anastomosis. The postoperative complications included one death on D10 from pulmonary vein thrombosis in a patient with sickle cell anaemia and early loss of the transplanted pancreas due to venous thrombosis. Nine patients underwent at least one surgical revision, due to a leaking duodenovesical anastomosis in 8 cases. With a mean follow-up of 33 +/- 20 months, the results demonstrate, apart from the early death indicated above, another death at 50 months of a patient who had lost his pancreas due to early venous thrombosis and who died with a functioning kidney. 23 of the 30 surviving patients have a functioning kidney and pancreas (79%), i.e. 74% of the total population of 32 patients. Loss of pancreatic function was surgical in two cases (one case of infection of the transplant site, one case of thrombosis), vascular in one case due to rupture of a mycotic aneurysm into the duodenum and immunological in three cases: two of these pancreases retained partial function allowing perfect blood glucose control with less than 10 units of ordinary insulin per day. Lastly, a perfectly functioning pancreas was removed 13 months after transplantation because of renal rejection not controlled by reinforced immunosuppression. Compared to the data of the international registry, these results demonstrate the value of the retroperitoneal approach used in this series and the improvement of the results obtained with increasing experience of the transplant team.
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PMID:[Combined renal-pancreas transplantation in the treatment for chronic renal insufficiency of diabetic origin . Results from the Pitie Urology Clinic]. 862 26

Pyomyositis is the primary infection of skeletal muscles, accompanied by abscess formation in the suppurative phase but may be without a focal fluid collection in the presuppurative phase. We describe three patients, one with insulin-dependent diabetes mellitus, another with sickle cell disease, and the third a previously healthy child with varicella infection who developed pyomyositis. Ultrasound or magnetic resonance imaging suggested the diagnosis in each case. The patients were treated with intravenous antibiotic therapy and two required abscess drainage. The infection in the third resolved without surgical drainage. None of our patients had residual functional limitations. We believe that a high index of suspicion and prompt diagnosis can prevent complications from pyomyositis.
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PMID:Pyomyositis: report of three patients and review of the literature. 886 99

With the two-dimensional Doppler echocardiogram and M-mode echocardiogram, one can study maternal and fetal cardiovascular physiology during rest and exercise. Using such noninvasive techniques, studies indicate that left ventricular function is maintained even during vigorous bicycle exercise in healthy pregnant subjects during the second half of pregnancy. In early pregnancy, the left ventricle adapts to strenuous bicycle exercise by increasing its contractile reserve, enhancing ventricular emptying, whereas in late pregnancy, the left ventricle increases its preload reserve without significantly increasing its contractile reserve. Thus, women are "cardiovascularly" disadvantaged early in pregnancy. Using Doppler signals, early (E-passive) flow and late peak (A-active) flow reflect left ventricular diastolic filling properties. Using such techniques, we found that diastolic filling patterns are significantly influenced by pregnancy and that each trimester influences these diastolic filling patterns during upright bicycle exercise. Doppler studies of uteroplacental circulation during or after exercise have yielded conflicting results. Some have described an increase in "the vascular resistance" of this pelvic bed during strenuous exercise, whereas others have not. It seems safe to conclude that more studies are needed to elucidate this problem. Exercise does not seem to influence the resistivity index of the umbilical artery in either singleton or twins, and may even cause it to decrease. Ventricular diastolic filling properties of the fetal heart do not seem to be influenced by maternal bicycle exercise. Further studies are needed to determine if less active pregnant subjects, women with chronic hypertensive disorders, women with sickle cell anemia, or women with insulin-dependent diabetes adapt to exercise as well as their "normal" counterparts.
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PMID:Maternal and fetal cardiovascular response to exercise during pregnancy. 888 51

Acyl-CoAsynthetase (ACS) is a key gene for cellular utilization of long-chain fatty acids. We characterized its regulation by physiological concentrations of insulin that acutely regulate metabolism. Our results demonstrate that subnanomolar insulin rapidly and maximally stimulates ACS gene transcription in the absence of protein synthesis; 0.5 nM insulin produced a 2.3 +/- 0.1-fold increase in ACS mRNA levels and induced ACS gene transcription 2.4 +/- 0.3-fold. The insulin sensitivity of ACS was compared with lipoprotein lipase (LPL) and stearoyl-CoA desaturase-1 (SCD-1), which were both less sensitive to insulin. Physiological triiodothyronine (10 nm) also induced ACS mRNA 2.4 +/- 0.1-fold and gene transcription 2.8 +/- 0.3-fold and coordinately induced LPL and SCD-1 mRNA and gene transcription. Because insulin and adenosine 3',5'-cyclic monophosphate often regulate genes involved in lipid and carbohydrate metabolism in a reciprocal manner, we evaluated effects of 1-methyl-3-isobutylxanthine (MIX).ACS mRNA levels were strongly downregulated by MIX in a dose-dependent manner, and ACS gene transcription inhibited in a coordinate manner with LPL and SCD-1. These data demonstrate a uniquely sensitive pattern of stimulation of ACS gene transcription by insulin with reciprocal regulation by MIX, and they suggest a significant role for ACS as a tightly regulated "gatekeeper" gene participating in the control of adipocyte metabolism.
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PMID:Physiological concentrations of insulin and T3 stimulate 3T3-L1 adipocyte acyl-CoA synthetase gene transcription. 896 77

Several transport pathways are involved in the regulation of cell volume and ion content in the human erythrocyte. Studies of these pathways have shown that K-Cl contransport and the Ca-gated K channel (Gardos channel) play an important role in the dehydration of sickle erythrocytes. Therapeutic strategies based on the specific blockade of these pathways have been tested in transgenic sickle mice and in patients with sickle cell disease, using oral Mg pidolate to inhibit K-Cl cotransport and oral clotrimazole to inhibit the Gardos channel. Studies on the erythrocyte Na-H (Na-Li) exchanger have uncovered an important role for insulin and blood pressure in mediating the activity of this pathway. These findings explain the reports of abnormal Na-Li exchange in diabetic nephropathy and essential hypertension. Targeted mutagenesis of the anion exchanger protein band 3 in mice has demonstrated that absence of this protein is compatible with life and leads to severe hemolytic anemia, normal assembly of the cytoskeleton, and reduced mechanical stability of the erythrocyte. Identification of the molecular identity of all the major erythrocyte transporters is imminent and will provide the bases for future studies of these proteins in the normal and abnormal erythrocyte.
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PMID:Erythrocyte membrane transport physiology. 910 29


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