Sickle cell anemia as a syndrome: A review of diagnostic features

Huisman, T. H. J.

doi:10.1002/ajh.2830060210

Cited by 38 publications

(11 citation statements)

References 63 publications

(26 reference statements)

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“…Hb and Hct were used to eliminate subjects with anemia, which has been reported to be frequent in SCT [24]. Hb and MCV were studied for the indirect diagnosis of alpha-thalassemia, also reported to be frequent in SCT [11].…”

Section: Hematological Variablesmentioning

confidence: 99%

Alactic Anaerobic Performance in Subjects with Sickle Cell Trait and Hemoglobin AA

Hue¹,

Julan²,

Blonc³

et al. 2002

Int J Sports Med

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The aim of the present study was to assess the performance of subjects with sickle cell trait (SCT) during brief and explosive exercise involving mainly anaerobic metabolism. One hundred and ninety-six black subjects underwent SCT screening, which revealed the presence of 16 subjects with SCT and 180 subjects with normal hemoglobin (HbAA). All subjects performed four tests: 1) a 100-m sprint, 2) a long-jump, 3) a Leger-Boucher shuttle test and 4) a jump-and-reach test. A control group (n = 18) selected from the 180 subjects with HbAA was matched according to the sex, age, weight and height of the SCT subjects (SCTs). The performances of the SCTs (n = 16) were compared with those of the control group. The performances were similar between the SCTs and control group for the sprint test, long-jump and the Leger-Boucher shuttle test. There was, however, a significant difference for the jump-and-reach test between the two groups: the SCTs (i. e., males plus females, and males and females considered separately) reached a significantly greater height (p < 0.05) than the matched subjects of the control group (63.7 +/- 3.6 vs. 58.6 +/- 3.1 cm, 72.3 +/- 3.9 vs. 67.1 +/- 2.4 cm and 52.7 +/- 3.2 vs. 45.3 +/- 2.0 cm for SCTs versus non-SCTs, for the group, the males and the females, respectively). The results of the present study suggest that the performance of brief and explosive exercise may be enhanced by HbS.

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Section: Hematological Variablesmentioning

confidence: 99%

Alactic Anaerobic Performance in Subjects with Sickle Cell Trait and Hemoglobin AA

Hue¹,

Julan²,

Blonc³

et al. 2002

Int J Sports Med

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“…Our results show that the ratio ofabnormal to normal insulin in the pancreas is 4:5, and support the idea that both normal and abnormal insulins are secreted in almost equal proportions, indicating the codominant expression ofboth alleles ofthe insulin gene. However, we cannot insist that the expression ofboth alleles is exactly the same, because it was known that the quantities of HbS heterozygotes, which also have a point mutation of an allele like an abnormal insulin gene, are only 20-50% of all hemoglobins (27).…”

Section: Discussionmentioning

confidence: 99%

Structurally abnormal insulin in a diabetic patient. Characterization of the mutant insulin A3 (Val----Leu) isolated from the pancreas.

Sakura¹,

Iwamoto²,

Sakamoto³

et al. 1986

J. Clin. Invest.

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We have recently identified a diabetic patient with marked fasting hyperinsulinemia. Family study revealed that the abnormality was an autosomal dominant trait. High-performance liquid chromatography (HPLC) profile of the patient's serum insulin showed that she had an abnormal insulin in addition to a normal insulin. We have purified her insulin(s) from the specimen of her pancreas, which was biopsied during an operation of cholelithiasis. Insulin was also immunologically purified from the serum of her portal vein. The reverse-phase HPLC analysis revealed that the ratios of normal to abnormal insulin in the pancreas, portal vein, and peripheral vein were 5:4, 4:5, and 1:7, respectively. Radioreceptor assay for insulin using guinea pig kidney membrane revealed that the binding activities of the normal component insulin, the abnormal component insulin and her pancreatic insulin containing, both components were 100, 5, and 50% of standard human insulin, respectively. The biological activities of the normal components the abnormal component and her pancreatic insulin to stimulate glucose oxidation in rat adipocytes were found to be 100, 8, and 60% of standard human insulin, respectively. Analysis of amino acid sequences of the abnormal insulin purified from her pancreas strongly suggested the substitution of leucine for valine at the third position of the A chain, A3 (Val --Leu).

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“…Several investigations have demonstrated how the clinical status of patients with β-globin disorders can be improved by pharmacologically increased expression of foetal γ-globin genes (Jouini et al, 2012, Musielak, 2011, Huisman, 1979, Stamatoyannopoulos and Nienhuis, 1992and Gallo et al, 1979. Moreover, foetal haemoglobin levels greater than 9% could reduce early mortality (Platt et al, 1994).…”

Section: Introductionmentioning

confidence: 98%

Erythroid differentiation ability of butyric acid analogues: Identification of basal chemical structures of new inducers of foetal haemoglobin

Bianchi

Chiarabelli

Zuccato

et al. 2015

European Journal of Pharmacology

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Sickle cell anemia as a syndrome: A review of diagnostic features

Cited by 38 publications

References 63 publications

Alactic Anaerobic Performance in Subjects with Sickle Cell Trait and Hemoglobin AA

Alactic Anaerobic Performance in Subjects with Sickle Cell Trait and Hemoglobin AA

Structurally abnormal insulin in a diabetic patient. Characterization of the mutant insulin A3 (Val----Leu) isolated from the pancreas.

Erythroid differentiation ability of butyric acid analogues: Identification of basal chemical structures of new inducers of foetal haemoglobin

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