background: alkaptonuria (aku) or ochronosis is a rare progressive degenerative arthropathy that results from deficiency of enzyme homogentisate 1,2 dioxygenase (hgd). the features include arthritis of the spine and in larger peripheral joints, with chondrocalcinosis. in this paper, we present a case of alkaptonuria in a 54 year old woman in tehran, iran. case presentation: a 54 year old woman ...

Radiosynovectomy is a novel method of treatment for several acute and chronic inflammatory joint disorders. A small amount of a beta-emitting radionuclide is injected into the affected joint delivering a radiation dose of 70 to 100 Gy to the synovia. The proliferative tissue is destroyed, secretion of fluid and accumulation of inflammation causing cellular compounds stops and the joint surfaces...

OBJECTIVE To determine the frequency and character of arthropathy in hereditary hemochromatosis (HH) and to investigate the relationship between this arthropathy, nodal interphalangeal osteoarthritis, and iron load. METHODS Participants were recruited from the community by newspaper advertisement and assigned to diagnostic confidence categories for HH (definite/probable or possible/unlikely)....

Abstract Background The purpose of this study was to assess the prevalence of major depressive and anxiety disorders in hemophilic and major beta thalassemic patients related to education of their mothers as a family’s agent. Materials and Methods A case-control study was performed on 34 major beta thalassemic patients. For each patient the control group was selected and matched (with age a...

Background: Hemophilic children must be thought of as special patients. Although there have been number of studies regarding with oral surgical, periodontal management of hemophilia there is paucity for the dental caries and its severity in hemophiliac children. The objective of this study is to assess prevalence of dental caries and treatment needs among hemophilic children attending Rajasthan...

OBJECTIVE To characterize the articular manifestations of cat-scratch disease (CSD) and to evaluate the long-term clinical outcome of those manifestations. METHODS A community- and hospital-based surveillance study of CSD was conducted in Israel between 1991 and 2002. CSD was defined as present in a patient when a compatible clinical syndrome and a positive confirmatory finding of Bartonella ...

BACKGROUND AND AIMS Hemophilic patients are faced with poor oral hygiene due to concerns about their dental care. The present study assessed the knowledge of hemophilic patients about oral hygiene and the effect of oral hygiene instruction in patients referred to Iranian Hemophilia Society. MATERIALS AND METHODS This cross-sectional study was carried out on 30 hemophilic patients randomly sel...

Ever since Sahli ( 1 ) demonstrated that the coagulation of hemophilic blood is accelerated by the addition of normal blood 2 many workers (2 to 9) have focused attention on its clot-promoting property. Since cell-free plasma seemed as effective as platelet-rich plasma this action could not be directly attributed to any of the cellular components of blood (2). Hemophilic plasma ws found to be i...

Hemophilic pseudotumor gradually erodes bone and induces fracture or deformity, causing joint dysfunction or destructive osteoarthropathy. Reports about surgery for hemophilic pseudotumor complicated by destructive osteoarthropathy are scarce. The object of this study was to evaluate the results and complications of surgical management for patients of pseudotumor complicated by destructive oste...

The metabolism of human prothrombin labeled with radioactive iodine was studied in seven normal subjects and four hemophilic patients. Results in the normal subjects were: plasma volume, 37.6+/-3.8 ml/kg; plasma prothrombin concentration, 303+/-40 U/ml (0.153+/-0.02 mg/ml); prothrombin half-life, 2.81+/-0.51 days; total plasma prothrombin pool, 5.72+/-0.62 mg/kg, representing 64.1+/-9.1% of tot...