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纯合子β地中海贫血中的急性脑血管损伤:一例报告

Acute cerebrovascular insults in homozygous beta-thalassaemia: a case report.

作者信息

Paolino E, Monetti V C, Granieri E, Boldrini P

出版信息

J Neurol. 1983;230(1):37-41. doi: 10.1007/BF00313595.

DOI:10.1007/BF00313595
PMID:6194271
Abstract

Neurological complications following vascular thrombotic crises are frequent and occur in sickle cell anaemia and its variants, but are less well recognized in thalassaemias. A case of beta-thalassaemia major is reported complicated by an ischaemic lesion involving the right cerebellar hemisphere. The probable pathogenesis is considered and the literature on the matter briefly reviewed. In agreement with other reports, the interference of risk factors provided by or occurring in association with multiple blood-transfusions is hypothesized. Consequently, caution is recommended in giving large blood-transfusions to thalassaemic patients.

摘要

血管血栓形成危象后的神经并发症很常见,发生于镰状细胞贫血及其变体中,但在地中海贫血中认识较少。本文报道了1例重型β地中海贫血合并右小脑半球缺血性病变的病例。文中探讨了可能的发病机制,并简要回顾了相关文献。与其他报道一致,推测多次输血所带来的或与之相关的危险因素的干扰作用。因此,建议在给地中海贫血患者大量输血时要谨慎。

相似文献

1
Acute cerebrovascular insults in homozygous beta-thalassaemia: a case report.纯合子β地中海贫血中的急性脑血管损伤:一例报告
J Neurol. 1983;230(1):37-41. doi: 10.1007/BF00313595.
2
Neurological complications of beta-thalassaemia major.重型β地中海贫血的神经并发症
Arch Dis Child. 1977 Dec;52(12):977-9. doi: 10.1136/adc.52.12.977.
3
Clinical manifestation and laboratory findings of sickle cell anaemia in association with alpha-thalassaemia in Saudi Arabia.沙特阿拉伯镰状细胞贫血与α地中海贫血相关的临床表现及实验室检查结果
Acta Haematol. 1985;74(3):155-60. doi: 10.1159/000206194.
4
Transfusion support for haemoglobinopathies.血红蛋白病的输血支持
Clin Haematol. 1984 Feb;13(1):151-65.
5
[Homozygous beta-thalassemia in Algeria. Study of 50 cases].
Presse Med. 1985 Nov 30;14(41):2089-92.
6
Homozygous beta thalassaemia and pregnancy.纯合子β地中海贫血与妊娠
Aust N Z J Obstet Gynaecol. 1984 Feb;24(1):45-8. doi: 10.1111/j.1479-828x.1984.tb03321.x.
7
[Acute erythroblastopenia disclosing homozygous beta-thalassemia. Role of parvovirus infection].[急性成红细胞减少症揭示纯合子β地中海贫血。细小病毒感染的作用]
Presse Med. 1985 Oct 5;14(33):1743-5.
8
[Influence of alpha-thalassemia on the hematologic expression of homozygote drepanocytosis].
Arch Fr Pediatr. 1987 Aug-Sep;44(7):517-9.
9
Hypertension, convulsion, and cerebral haemorrhage in thalassaemic patients after multiple blood transfusions.
Helv Paediatr Acta. 1980 Jul;35(3):269-71.
10
Homozygous beta delta-thalassaemia. Description of a case and review of the literature.
Acta Haematol. 1974;51(3):185-91. doi: 10.1159/000208292.

引用本文的文献

1
Protein C and Protein S: causative factor for developing a hemorrhagic infarct in a HbE/Beta thalassemia child.蛋白 C 和蛋白 S:导致 HbE/β 地中海贫血儿童发生出血性梗死的原因。
Indian J Pediatr. 2010 Mar;77(3):316-7. doi: 10.1007/s12098-009-0297-5. Epub 2010 Jan 20.
2
Cerebral thrombosis in beta-thalassaemia major.重型β地中海贫血中的脑血栓形成。
Indian J Pediatr. 2001 Nov;68(11):1081-2. doi: 10.1007/BF02722363.

本文引用的文献

1
Hypertension, convulsion, and cerebral haemorrhage in thalassaemic patients after multiple blood transfusions.
Helv Paediatr Acta. 1980 Jul;35(3):269-71.
2
[Nervous manifestations associated with thalassemia (critical study of the concept of thalassemic neurohemolytic syndrome)].[与地中海贫血相关的神经表现(对地中海贫血神经溶血性综合征概念的批判性研究)]
Pediatrie. 1967 Oct-Nov;22(7):771-84.
3
Thalassemia major (homozygous beta-thalassemia). A survey of 138 cases with emphasis on neurologic and muscular aspects.重型地中海贫血(纯合子β地中海贫血)。138例病例的调查,重点关注神经和肌肉方面。
Neurology. 1972 Mar;22(3):294-304. doi: 10.1212/wnl.22.3.294.
4
Spinal cord compression due to extramedullary hematopoiesis in homozygous thalassemia. Case report.
J Neurosurg. 1975 Feb;42(2):212-6. doi: 10.3171/jns.1975.42.2.0212.
5
Immunological impairment and susceptibility to infection after splenectomy.
JAMA. 1976 Sep 20;236(12):1376-7.
6
Neurological complications of beta-thalassaemia major.重型β地中海贫血的神经并发症
Arch Dis Child. 1977 Dec;52(12):977-9. doi: 10.1136/adc.52.12.977.
7
A syndrome of hypertension, convulsion, and cerebral haemorrhage in thalassaemic patients after multiple blood-transfusions.
Lancet. 1978 Sep 16;2(8090):602-4. doi: 10.1016/s0140-6736(78)92824-6.