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1.
Maroc Medical. 2009; 31 (3): 206-212
in French | IMEMR | ID: emr-133534

ABSTRACT

It is also known as hemophagocyticc syndrome, defined as pathology group characterized in common by the proliferation and the accumulation of macrophages in the bone marrow and the lymphoid system, leading to an abnormal phagocytosis of the blood cells and the production of proinflammatory cytokines. It has a non specific clinical picture as well as a perturbed laboratory findings. We differentiate the hereditary primary forms and the secondary related to several etiologies. Its pathophysiology is not yet clear the treatment remains poorly codified. It is a serious like-threatening disease but recent advances in the comprehension of its physiopathologic mechanisms will allow for a better adapted treatment and improve the survival rate of these patients

2.
Maroc Medical. 1999; 21 (3): 177-183
in French | IMEMR | ID: emr-51721

ABSTRACT

Homocystinuria is a rare inherited metabolic disease transmitted as an autosomal recessive trait. Arterial and venous thromboembolic events are frequent and life-threatening complications in homocystinuric patient. It has been suggested that mild homocysteinemia could be a risk factor for vascular disease. Homocysteine can promote lipid peroxidation and damage vascular endothelial cells. Moreover, homocysteine interferes with natural anticoagulant system and the fibrinolytic system. Homocysteinemia should be known in patients with premature vascular disease, especially in subjects with no risk factors. Folic acid, vitamin B6 can lower homocysteine levels


Subject(s)
Humans , Risk Factors , Cardiovascular Diseases/epidemiology , Thrombosis , Atherosclerosis , Homocysteine/metabolism , Homocysteine/blood , Hyperhomocysteinemia/etiology , Hyperhomocysteinemia/therapy , Venous Thrombosis
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