Sickle cell disease with double stroke in a Moroccan family.

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.

• Authors: Khalil Hamzi, Afaf Ben Itto, Zineb Jouhadi, Ilham Slassi, Sellama Nadifi
• Journal: Journal of molecular neuroscience : MN (J Mol Neurosci) Vol. 50 Issue 2 Pg. 311-3 (Jun 2013) ISSN: 1559-1166 [Electronic] United States
• PMID: 23340961 (Publication Type: Case Reports, Journal Article)

Chemical References

• Hemoglobins

Topics

• Anemia, Sickle Cell (blood, complications, diagnosis)
• Child
• Female
• Hemiplegia (diagnosis, etiology)
• Hemoglobins (analysis)
• Humans
• Molecular Diagnostic Techniques
• Pedigree
• Stroke (blood, diagnosis, etiology)