Literature DB >> 23340961

Sickle cell disease with double stroke in a Moroccan family.

Khalil Hamzi1, Afaf Ben Itto, Zineb Jouhadi, Ilham Slassi, Sellama Nadifi.   

Abstract

The sickle-cell disease is a group of chronic hemolytic diseases which associates three types of injuries: severe anemia, severe infections, and ischemic vaso-occlusive crisis that are secondary to conflicts between small vessels and red blood cells too deformable. Thus, organic various complications may arise. Its prevalence in Europe is estimated to be about 1/150 and reaches 15 % in the Mediterranean areas. Clinical manifestations vary widely from one person to another and from one moment to another. In addition to anemia and bacterial infections, vaso-occlusive crisis may manifest by focal ischemia. In the long term, the VOC may compromise the function of a particular tissue or organ. The transmission is autosomal recessive. The sickle-cell diseases are determined by combinations of two abnormal alleles of beta globin gene including at least one which carries the mutation beta 6 glu-val (Hb S). We report the case of a girl aged 11 years, who presented two strokes in the interval of 8 months, which manifested by a complete right hemiplegia and aphasia confirmed by head CT scan; the electrophoresis of the hemoglobin and the molecular test had confirmed the diagnosis of sickle-cell disease, and we were allowed to spread better reflection on the prevention of stroke, which remains a frequent and serious complication of sickle-cell disease.

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Year:  2013        PMID: 23340961     DOI: 10.1007/s12031-013-9967-x

Source DB:  PubMed          Journal:  J Mol Neurosci        ISSN: 0895-8696            Impact factor:   3.444


  9 in total

1.  Sickle cell anemia: severe ischemic colitis responding to conservative management.

Authors:  A A Moukarzel; M Rajaram; A Sundeep; L Guarini; F Feldman
Journal:  Clin Pediatr (Phila)       Date:  2000-04       Impact factor: 1.168

2.  Abnormal hemoglobin as a cause of neurologic disease.

Authors:  M GREER; D SCHOTLAND
Journal:  Neurology       Date:  1962-02       Impact factor: 9.910

Review 3.  Sickle cell anemia and other hemoglobinopathies.

Authors:  D C Hess; R J Adams; F T Nichols
Journal:  Semin Neurol       Date:  1991-12       Impact factor: 3.420

4.  Sickle erythrocyte adherence to vascular endothelium. Morphologic correlates and the requirement for divalent cations and collagen-binding plasma proteins.

Authors:  N Mohandas; E Evans
Journal:  J Clin Invest       Date:  1985-10       Impact factor: 14.808

5.  Vascular lesions in the central nervous system in sickle cell disease (neuropathology).

Authors:  M Koshy; C Thomas; J Goodwin
Journal:  J Assoc Acad Minor Phys       Date:  1990

6.  Preventing stroke among children with sickle cell anemia: an analysis of strategies that involve transcranial Doppler testing and chronic transfusion.

Authors:  Maitreyi Mazumdar; Matthew M Heeney; Colin M Sox; Tracy A Lieu
Journal:  Pediatrics       Date:  2007-09-03       Impact factor: 7.124

7.  Cerebrovascular disease in sickle cell anemia: a clinical, pathological and radiological correlation.

Authors:  K H Merkel; P L Ginsberg; J C Parker; M J Post
Journal:  Stroke       Date:  1978 Jan-Feb       Impact factor: 7.914

8.  Mortality in sickle cell disease. Life expectancy and risk factors for early death.

Authors:  O S Platt; D J Brambilla; W F Rosse; P F Milner; O Castro; M H Steinberg; P P Klug
Journal:  N Engl J Med       Date:  1994-06-09       Impact factor: 91.245

9.  Sickle cell anemia and central nervous system infarction: a neuropathological study.

Authors:  S M Rothman; K H Fulling; J S Nelson
Journal:  Ann Neurol       Date:  1986-12       Impact factor: 10.422

  9 in total

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