Results 42 children with HAIDS in age between 2 and 17 years were identified in 3 years. Age of onset ranged from 0 to 16 years, on an average 5.1 years old. The average age at diagnosis was 8.2 years old. The following diseases were identified: Behcet’s disease (BD) 16 children (male/female (M/F) 12/4); Cryopyrin-Associated Periodic Syndromes (CAPS): Muckle-Wells syndrome 4 (M/F 0/4 ); CINCA/N...

Familial aggregation and the effect of parenting styles on three dispositions toward ridicule and being laughed at were tested. Nearly 100 families (parents, their adult children, and their siblings) completed subjective questionnaires to assess the presence of gelotophobia (the fear of being laughed at), gelotophilia (the joy of being laughed at), and katagelasticism (the joy of laughing at ot...

Nylon-3 polymers (poly-β-peptides) have been investigated as synthetic mimics of host-defense peptides in recent years. These polymers are attractive because they are much easier to synthesize than are the peptides themselves, and the polymers resist proteolysis. Here we describe in vitro analysis of selected nylon-3 copolymers against Clostridium difficile, an important nosocomial pathogen tha...

Introduction Interleukin-36-receptor antagonist deficiency (DITRA) is a recently described auto-inflammatory disease, characterized by repeated flares of generalized pustular psoriasis, high fever, asthenia and systemic inflammation. This condition is caused by homozygous missense mutation in the IL36RN gene, encoding the interleukin-36-receptor antagonist (IL-36Ra), an anti-inflammatory cytoki...

The fate of the oxoanion arsenate in diverse systems is strongly affected by its adsorption on the surfaces of iron (oxyhydr)oxide minerals. Predicting this behavior in the environment requires an understanding of the mechanisms of arsenate adsorption. In this study, the binding site and adsorption geometry of arsenate on the hematite (012) surface is investigated. The structure and termination...

Introduction Blau syndrome (BS) is a rare autosomal dominant, autoinflammatory syndrome characterized by the clinical triad of granulomatous, recurrent uveitis, dermatitis and symmetric arthritis. The arthritis is usually a polyarticular exuberant synovitis and tenosynovitis and represents the characteristic phenotypic feature. Uveitis occurs in most patients and commonly evolves to a panuveiti...