Relationship between serum visfatin and vascular inflammation markers level in beta thalassemia major patients

Understanding the possible role of visfatin in the pathogenesis of beta-thalassemia major [BTM] and its relationship with markers of endothelial function could help us to provide more effective therapeutic approaches for treatment of patients with BTM and its related complications. The aim of current study was to compare serum level of visfatin between patients with BTM and control group and determine its correlation with markers of endothelial function, intracellular adhesion molecule [ICAM] and vascular adhesion molecule [VCAM]. In this case-control study, patients with BTM receiving regular blood transfusion aged 10-20 years and a group of healthy subjects were enrolled. Selected subjects examined clinically and venous blood samples obtained for visfatin, ICAM, VCAM, cholesterol, triglyceride, high density lipoprotein cholesterol, low density lipoprotein cholesterol and ferritin measurements. Mean [standard deviation] of studied laboratory measurements compared in two studied groups and the relation between visfatin and ICAM, VCAM, ferrittin, body mass index determined. In this study 31 patients with BTM and 30 healthy controls studied. Mean of visfatin was significantly higher in patients with BTM than control group [133.9 +/- 60.1 vs. 43.3 +/- 27.9, P < 0.001]. The higher level of visfatin among patients with BTM indicated the possible inflammatory role of this adipocytokine in BTM. It seems that for understanding the underlying mechanisms and its relation with vascular inflammatory markers and endothelial function further studies with larger sample size is needed

case of atypical mccune-albright syndrome with vaginal bleeding

McCune-Albright syndrome [MAS] is a rare non-inherited disorder characterized by the clinical triad of precocious puberty, cafe-au-lait skin lesions, and fibrous dysplasia of bone. We report a girl with MAS, presenting initially with vaginal bleeding at the age of 17 months. Ultrasonography revealed unilateral ovarian cysts and ureteral and ovarian enlargement. Considering the clinical and paraclinical findings, the patient diagnosed as a case of gonadotropin-independent precocious puberty was treated with medroxy-progestrone acetate [MPA] for three months. During the follow up, recurrent episodes of bleeding, ovarian activation and cyst formation, as well as breast size development were reported. At the age of 5.5 years, fibrous dysplasia was detected, which in coexistence with precocious puberty confirmed the diagnosis of MAS. The patient had no cafe-au-lait skin macles during follow up. Considering that clinical manifestations of MAS appear later in the course of recurrent periods of ovarian activation and cyst formation, a careful clinical observation and follow up of patients is necessary and the diagnosis of MAS must be kept in mind in cases with gonadotropin-independent precocious puberty