Increased serum levels of progranulin (PGRN) in patients with haemophilic arthropathy

Haemophilia A and B are rarely occurring X chromosome‐linked congenital coagulation disorders dominated by spontaneous joint bleedings and chronic synovitis, leading to development of haemophilic arthropathy (HA). Progranulin (PGRN) is a growth factor with anti‐inflammatory and immunomodulatory properties. PGRN is an important molecule in the pathogenesis of osteoarthritis (OA) and rheumatological disorders. This study was aimed at investigating the potential role of PGRN in the mechanisms underlying the pathogenesis of HA. The serum levels of PGRN were measured by enzyme‐linked immunosorbent assay (ELISA) in patients with end‐stage knee joint HA (n = 20) and end‐stage primary knee joint OA (n = 20) who met the inclusion and exclusion criteria. The clinical and radiological assessment of disease severity was evaluated by the Knee Society Score (KSS) and Kellgren‐Lawrence scale. Median PGRN levels in HA patients was 349.1 ng/mL (232.8–415.6 ng/mL) and in OA patients 148.3 ng/mL (112.1‐275.3 ng/mL) with statistically significant differences between both groups (P < 0.015). Further analysis revealed no correlation between PGRN levels and any of the patient demographics and clinical parameters. This study demonstrates increased PGRN serum levels in patients with HA and provides new insights into the mechanisms underlying the pathogenesis of HA indicating a new potential target for therapeutic intervention.     

Perforation of the aorta after percutaneous closure of an atrial septal defect with an Amplatz prosthesis,presenting with acute severe hemolysis

We report a case of aortic perforation three weeks after transcatheter occlusion of an atrial septal defect (ASD) by an Amplatzer device. Revealed by acute hemolysis, this complication needed an emergency surgical operation. The fistula between the no coronary Valsalva sinus of the aorta and the left atrium was repaired. The ASD was closed by patch. This serious accident should consider a short antero-superior rim as a risk factor for aortic perforation in transcatheter closure for ASD.     

Acquired protein S deficiency in HIV infections]

A prospective study of protein S was carried out in 17 consecutive HIV-infected patients. One subject was excluded because of severe chronic liver disease. Of the 16 evaluable patients (10 men, 6 women), 3 had AIDS, 7 ARC, 4 lymphadenopathy and 2 were asymptomatic. Total protein S and C4bBP (bound protein) were normal in all of them. In contrast, all but one subject had a free protein S deficiency: 35.5% (range: 0-68) for the whole group; 12.4% (range: 0-29) for the women: 50.9% (range: 27-68) for the men. From these results, we concluded that: a) free protein S deficiency is common in HIV-infected patients, b) this deficiency is more pronounced in female than male subjects, and c) this deficit is not linked to elevated C4bBP levels. The mechanism and the clinical consequences of this deficiency are discussed.