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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 function of the pituitary-adrenal axis and leukocyte alkaline phosphatase activity were evaluated in eight patients with sickle cell disease during a painful crisis and when crisis-free. The leukocyte alkaline phosphatase (LAP) score did not increase during crisis; the scores were in the low-normal range during crisis and noncrisis periods. Insulin-induced hypoglycemia produced normal growth hormone responses during both crisis and crisis-free periods. Plasma cortisol concentrations were diminished in the crisis group. Also impaired was 11-deoxycortisol production in both groups after metyrapone. These findings indicate that a mild defect in the hypothalamic-pituitary-adrenal axis exists in sickle cell disease patients.
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PMID:Pituitary-adrenal axis function in sickle cell anemia and its relationship to leukocyte alkaline phosphatase. 701 Oct 1

Twenty-seven patients were seen and followed at our Sickle Cell Center over a period of seven years. Their clinical, hematological, and biochemical features were determined and compared to those of patients with sickle cell anemia who were concurrently investigated. The data indicate that the mild anemia of hemoglobin (Hb) SC disease is slightly microcytic and hyperchromatic. Parameters of hemolysis and the complications of chronic hemolytic anemia (cholelithiasis, leg ulcers, hepatomegaly, and cardiomegaly) are milder in Hb SC disease than in sickle cell anemia. Asplenia and its sequelae (increased platelet count and reduced serum IgM levels) are less frequent in Hb SC disease. Cerebrovascular accidents and the decreased leukocyte alkaline phosphatase scores are similar in both diseases. Thromboembolic complications, retinopathy, and renal papillary necrosis are more frequent in Hb SC disease.
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PMID:Clinical, hematological, and biochemical features of Hb SC disease. 713 65

In this paper clinical similarities between sickle cell anemia patients and zinc deficient subjects, the latter as reported from the Middle East have been presented. Zinc levels in plasma, red cells, hair and neutrophils were decreased in our adult patients with SCA. The activities of certain zinc dependent enzymes such as plasma RNase, red cell carbonic anhydrase, leucocyte alkaline phosphatase, and deoxythymidine kinase activity in freshly synthesized collagen connective tissue were consistent with the concept that indeed zinc deficiency occurred in SCA patients. Zinc supplementation under controlled conditions showed that the SCA patients gained weight, their serum testosterone level increased and plasma ammonia level decreased. Finally, we also observed abnormal dark adaptation in some SCA patients which improved following zinc supplementation. Inasmuch as we have previously reported that the number of irreversible sickle cells decrease following zinc supplementation, we would like to suggest that zinc supplementation at earlier age may be benefical in preventing organ damage. In conclusion, zinc supplementation should be prescribed for patients with SCA, particularly if they show evidences for zinc deficiency.
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PMID:Zinc deficiency and effects of zinc supplementation on sickle cell anemia subjects. 729 Dec 6

The blood biochemistry of 84 young homozygous sickle cell patients aged 1 to 11 years was analysed for evidence of liver disease. A comparison of the values found in their blood with those seen in normal children from an identical age group, assessed at the same time, shows no convincing evidence of liver cell damage except in 12% of cases. The normal transaminase observed in many of the patients assessed, together with the high alkaline phosphatase activity which seemed to be out of proportion to plasma bilirubin, is a picture compatible with localised obstructive lesions of the liver or bone lesions, both of which are common in sickle cell disease. This biochemical pattern suggests that the conjugated bilirubin, which dominates the picture in 40% of patients who have 'haemolytic jaundice', is due largely, not to liver cell damage, but to a combination of two factors, namely, intrahepatic cholestasis and the presence of actively functioning liver cells. Adequate albumin synthesis found in these patients, together with normal thymol reactions, provides further evidence of the absence of severe liver cell damage.
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PMID:Liver function and the diagnostic significance of biochemical changes in the blood of African children with sickle cell disease. 743 Mar 68

Thirty children with sickle cell anaemia had their serum alanine aminotransferase, alkaline phosphatase, total protein, albumin and bilirubin, assayed during vaso-occlusive crisis and at recovery. Alanine aminotransferase, alkaline phosphatase and bilirubin levels were significantly higher during crisis than at recovery, (p < 0.005) especially in the young patient. However, the total protein and albumin levels were not significantly different in crisis and at recovery. A transient hepatic functional derangement during vaso-occlusive crisis is a probable explanation for the reported changes.
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PMID:Hepatic function tests in children with sickle cell anaemia during vaso occlusive crisis. 788 14

Fifty-nine patients with homozygous sickle cell anaemia, 17 heterozygous individuals and 22 controls were investigated in respect to serum (S) 5'nucleotidase (5'NT, EC 3.1.3.5). The patients showed a significantly higher mean value of S-5'NT compared to the controls. However, this rise was heterogeneous as it occurred only among a subgroup of patients. The heterozygous individuals were not different from either the patients or the controls generating a situation which puts the heterozygous individuals in an intermediate position between the patients and the controls. S-5'NT showed significant correlation with S-bilirubin, S-aspartate aminotransferase, S-alanine aminotransferase and especially S-gammaglutamyl transferase. However, it was not correlated with S-alkaline phosphatase, which is another marker for hepatobiliary disease. These results suggest that the liver involvement in a subgroup of patients with sickle cell anaemia is a mixture of hepatocyte damage and the biliary tree involvement.
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PMID:Increased activity of 5' nucleotidase in serum of patients with sickle cell anaemia. 790 22

Patients with sickle cell disease (SCD) frequently have bone disorders of multifactorial aetiology. We attempted to analyse the relationships between bone mineral density (BMD) on the one hand and auxologic parameters, degree of siderosis, function of the growth hormone (GH)/insulin-like growth factor-I (IGF-I)/IGF-binding protein 3 (IGFBP3) axis, and calcium-phosphate balance in 28 prepubertal children with SCD and 15 age-matched children with constitutional short stature (CSS). Children with SCD had significantly decreased BMD (77.9 +/- 11.9 per cent of normal BMD for age and sex) and circulating concentrations of IGF-I (91 +/- 31 ng/ml) and IGFBP3 (1.7 +/- 0.44 mg/l) compared with the control group (BMD = 93.5 +/- 8.2 per cent of normal BMD for age and sex, IGF-I = 221 +/- 48 ng/ml, and IGFBP3 = 2.3 +/- 0.34 mg/ml). GH response to provocation was defective (peak below 10 micrograms/l) in 40 per cent of children with SCD. Those with SCD with defective GH secretion had significantly lower circulating IGF-I concentration and BMD than those with normal GH secretion. Serum calcium, phosphate and alkaline phosphatase concentrations were normal in all children with SCD. BMD was correlated significantly with height, weight, and body mass index as well as with the circulating concentrations of IGF-I and IGFBP3. It is suggested that increasing the circulating IGF-I concentration, either through increasing the caloric intake of subjects and/or via GH/IGF-I therapy, may improve growth and bone mineralization in these patients.
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PMID:Decreased bone mineral density in prepubertal children with sickle cell disease: correlation with growth parameters, degree of siderosis and secretion of growth factors. 971 3

Cholelithiasis is a common clinical condition in patients with sickle cell disease and there are conflicting reports on laboratory indices useful in predicting those patients who are likely to have gallstones. There is however lack of similar studies from Kenya. We therefore studied the role of clinical (Body Mass Index), haematological (reticulocyte count, haemoglobin level), and biochemical (serum bilirubin: direct and indirect, serum alkaline phosphatase, serum transaminase) indices in predicting sickle cell anaemia patients likely to develop gallstones. A cross sectional descriptive study was conducted from October 1993 to December 1994 on consecutive male and female patients of all ages with homozygous sickle cell disease (HbSS) confirmed by cellulose acetate paper electrophoresis. A total of 64 patients aged between three and 37 years were recruited into the study. They were classified into two groups: stone formers and non-formers. The difference in the two groups with respect to clinical, haematological and biochemical indices were determined by Chi-square contingency test. Body mass index (BMI), reticulocyte count and alkaline phosphatase were found to have a significant positive association with increased likelihood of gallstone formation at p values of 0.004, 0.007 and 0.007, respectively. The rest of the study indices had no association. The cut-off points were reticulocyte counts above ten per cent and alkaline phosphatase levels above 13 K.A. units. Though sickle cell anaemia patients with BMI > 20 had significant increased likelihood of cholelithiasis, we could not determine its cut-off value.
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PMID:Clinical and laboratory predictors of cholelithiasis in patients with sickle cell anaemia. 980 18

The cytotoxicity of hydroxyurea (HU), currently used to combat various cancers, sickle cell anemia and human immunodeficiency infection, was assessed by exposing decidualized and pregnant uteri of Sprague-Dawley rats to this drug. Consecutive daily doses of HU (500 mg/kg(-1)) for 4 days were injected subcutaneously during decidualization when proliferation of the deciduoma was biochemically analyzed on pseudopregnancy day 9, or injected intraperitoneally during pregnancy when uterine developmental processes were evaluated on gestation day 16. Hydroxyurea displayed prominent antiproliferative effects on decidual growth. These actions were comparable to significantly impaired (P<0.001) developmental responses (increases in post-implantation losses, in resorbed fetuses and in reduced fetal and placental weights) during pregnancy. The cellular components inhibited by HU were DNA, protein, nitric oxide synthase, a matrix metalloproteinase and decidual prolactin-related protein mRNA (P<0.05). Steroid-related endocrine events (serum progesterone concentrations, estrogen receptor and mRNA levels) were unaffected by HU, implying direct cellular action by the drug. Interestingly, endometrial alkaline phosphatase bioactivity was enhanced by HU (P<0.05). Subsequently, the reproductive toxicity of HU was apparently related to mitogenic and differentiation-induced endometrial cellular activities.
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PMID:Hydroxyurea inhibition of cellular and developmental activities in the decidualized and pregnant uteri of rats. 1113 71

Serum bone-specific alkaline phosphatase protein (bAP) was evaluated as indicator of bone turnover by immunoradiometric assay (IRMA) in twenty patients with sickle cell disease and in twenty healthy control subjects. Serum bAP was also compared with serum total alkaline phosphatase activity and serum lactate dehydrogenase in the same group. The concentrations of serum bAP and serum lactate dehydrogenase were significantly higher in the study group than in the control group (p < 0.05, p < 0.01, respectively). The serum total alkaline phosphatase activity showed no significant difference with the control healthy subjects. There was no correlation between serum bAP and total alkaline phosphatase or lactate dehydrogenase levels in the patient group. In conclusion, serum bAP protein measured by IRMA can be considered a sensitive marker of bone turnover and could be especially useful as valuable non-invasive biochemical marker for identifying sickle cell patients with skeletal complications.
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PMID:Bone-specific alkaline phosphatase protein, total alkaline phosphatase activity and lactate dehydrogenase in sera of patients with sickle cell disease. 1134 4


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