OBJECTIVE To test the reproducibility of tests and criteria for generalized joint hypermobility (GJH) and benign joint hypermobility syndrome (BJHS). METHODS A standardized protocol for clinical reproducibility studies was followed using a three-phase study (with a training, an overall agreement and a test phase). An overall agreement of at least 0.80 was required to proceed to the test phase...

BACKGROUND The majority of individuals with joint hypermobility remain asymptomatic. However, those associated with Benign Joint Hypermobility Syndrome (BJHS), develop a number of systemic manifestations. Our objective was to determine the relationship between joint hypermobility and musculoskeletal problems, and frequency of BJHS in children and adolescents. METHOD This cross-sectional obser...

Idiopathic hypereosinophilic syndrome (HES) and Gleich's syndrome are related disorders characterized by persistent or recurrent hypereosinophilia of unknown origin. Elevated IgE levels and polyclonal hypergammaglobulinaemia are considered as markers of benign outcome in this setting as they are generally associated with predominant cutaneous manifestations and favourable response to glucocorti...

Dubowitz syndrome is a rare, autosomal recessive disorder characterized by intrauterine growth retardation, short stature, microcephaly, distinct facial dysmorphism, and psychomotoric retardation. The hyper-immunoglobulin E (hyper-IgE or Job syndrome) is a primary immunodeficiency characterized by recurrent staphylococcal abscesses, recurrent cyst-forming pneumonia, and an elevated serum IgE le...

The hyper-IgE syndromes are rare, complex primary immunodeficiencies characterized by clinical manifestation diversity, by particular susceptibility to staphylococcal and mycotic infections as well as by a heterogeneous genetic origin. Two distinct entities--the classical hyper-IgE syndrome which is inherited in an autosomal dominant pattern and the autosomal recessive hyper-IgE syndrome--have ...

Recent studies show that most patients with X-linked hyper IgM syndrome have defects in the gene for CD40 ligand. We evaluated 17 unrelated males suspected of having X-linked hyper IgM syndrome. Activated T cells from 13 of the 17 patients failed to bind a soluble CD40 construct. In these patients, the sequence of CD40 ligand demonstrated mutations. By contrast, T cells from the remaining four ...

Kluver Bucy Syndrome is a rare neuropsychiatric disorder and a sequel of limbic encephalitis. Its presentation may vary in different ages. The patients may have all the features of Kluver Bucy Syndrome like – Hyper orality, Hyper sexuality, hyper metamorphosis, affective blunting, memory difficulties, eating disturbances etc; or may have only few of the symptoms which are commonly known as part...

conclusions physicians should be aware of the signs and symptoms of different pain sources to prevent misdiagnosis and maltreatment. introduction fracture of the styloid process (sp) of the temporal bone is a rare traumatic injury in normal individuals who are not suffering from eagle’s syndrome. diagnosis and management of this problem requires comprehensive knowledge about its signs and sympt...

Introduction: Frozen shoulder syndrome (FSS) is a common shoulder disease that causes pain and has progressive loss of shoulder motion. Codman's pendulum exercises are commonly prescribed after a shoulder injury to provide early joint mobilization, increase the flow of nutrients into the joint space, and decrease pain. This study aimed to compare the effect of Cadman's exercises and scapular rh...