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Long-term treatment of anemia with recombinant human erythropoietin in familial amyloidosis TTR V30M.

作者信息

Beirão Idalina, Lobato Luísa, Moreira Luciana, Mp Costa Paulo, Fonseca Isabel, Cabrita António, Porto Graça

出版信息

Amyloid. 2008 Sep;15(3):205-9. doi: 10.1080/13506120802195840.

DOI:10.1080/13506120802195840
PMID:18925459
Abstract

Familial amyloidosis or familial amyloid polyneuropathy (FAP) TTR V30M is a hereditary disease presented, in most cases, as a sensorimotor and autonomic neuropathy. Normocytic and normochromic anaemia was found in 24.8% of symptomatic FAP patients associated to lower serum erythropoietin (Epo) levels. Erythropoietin has been reported as efficient in anaemia correction in this disease. To evaluate the tolerance and efficacy of this treatment, a retrospective longitudinal study with 24 patients was undertaken. Patients were followed for at least 6 months. Haemoglobin, hematocrit, iron status, serum creatinine and urea and r-HuEPO doses were monitored, at 0, 3 months, 6 months and at the end of the follow-up. Long-term use of r-HuEPO proved to be efficient in the treatment of anaemia in familial amyloidosis TTR V30M and, despite the disease progression, no resistance cases to this treatment were observed. Positive side effects, like improvement on orthostatic hypotension symptoms and well-being sensation, contributing to confirm erythropoietin as a drug of choice to treat anaemia in amyloidosis TTR V30M.

摘要

相似文献

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引用本文的文献

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Orthostatic hypotension in hereditary transthyretin amyloidosis: epidemiology, diagnosis and management.遗传性转甲状腺素蛋白淀粉样变性的直立性低血压:流行病学、诊断和治疗。
Clin Auton Res. 2019 Sep;29(Suppl 1):33-44. doi: 10.1007/s10286-019-00623-x. Epub 2019 Aug 26.
2
Evidence-based treatment of neurogenic orthostatic hypotension and related symptoms.神经源性直立性低血压及相关症状的循证治疗。
J Neural Transm (Vienna). 2017 Dec;124(12):1567-1605. doi: 10.1007/s00702-017-1791-y. Epub 2017 Oct 22.