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纯合子β地中海贫血患者的血浆环核苷酸水平

Plasma cyclic nucleotide levels in patients with homozygous beta-thalassaemia.

作者信息

Peracchi M, Toschi V, Lombardi L, Bamonti-Catena F, Maiolo A T, Bareggi B, Massaro P, Polli E E

出版信息

Scand J Haematol. 1985 Apr;34(4):348-52. doi: 10.1111/j.1600-0609.1985.tb00761.x.

Abstract

To investigate the possibility that a proliferative non-neoplastic process influences extracellular cyclic nucleotide concentrations, we measured plasma cyclic AMP and cyclic GMP levels in 38 patients with homozygous beta-thalassaemia. This group consisted of 20 patients with thalassaemia major transfused regularly (mean pre transfusion Hb levels, 11 g/dl), and 18 patients with thalassaemia intermedia who did not require regular blood transfusion (mean Hb levels, 8.7 g/dl). In the patient group, plasma cyclic AMP levels were similar to those of 37 normal subjects matched for age and sex, whereas plasma cyclic GMP levels were markedly higher. Moreover, in the thalassaemic patients there was a significant negative correlation between plasma cyclic GMP levels and haemoglobin concentrations, suggesting that their marked erythroid hyperplasia may play a role in determining alterations in extracellular cyclic GMP levels.

摘要

为了研究增殖性非肿瘤性过程影响细胞外环核苷酸浓度的可能性,我们检测了38例纯合子β地中海贫血患者的血浆环磷酸腺苷(cAMP)和环磷酸鸟苷(cGMP)水平。该组包括20例定期输血的重型地中海贫血患者(输血前血红蛋白平均水平为11 g/dl)和18例无需定期输血的中间型地中海贫血患者(血红蛋白平均水平为8.7 g/dl)。在患者组中,血浆cAMP水平与37名年龄和性别匹配的正常受试者相似,而血浆cGMP水平明显更高。此外,在地中海贫血患者中,血浆cGMP水平与血红蛋白浓度之间存在显著负相关,这表明其明显的红系增生可能在决定细胞外环GMP水平的改变中起作用。

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