Guidelines for the management and treatment of periodic fever syndromes: periodic fever, aphthous stomatitis, pharyngitis and adenitis syndrome.

Objective: To establish guidelines based on scientific evidence for the management of periodic fever, aphthous stomatitis, pharyngitis and adenitis (PFAPA) syndrome.

Description Of The Evidence Collection Method: The Guideline was prepared from 5 clinical questions that were structured through PICO (Patient, Intervention or indicator, Comparison and Outcome), to search in key primary scientific information databases. After defining the potential studies to support the recommendations, these were graduated considering their strength of evidence and grade of recommendation.

Guidelines for the management and treatment of periodic fever syndromes: Cryopyrin-associated periodic syndromes (cryopyrinopathies - CAPS).

Objective: To establish guidelines based on cientific evidences for the management of cryopyrin associated periodic syndromes.

Description Of The Evidence Collection Method: The Guideline was prepared from 4 clinical questions that were structured through PICO (Patient, Intervention or indicator, Comparison and Outcome), to search in key primary scientific information databases. After defining the potential studies to support the recommendations, these were graduated considering their strength of evidence and grade of recommendation.

Guidelines for the management and treatment of periodic fever syndromes familial Mediterranean fever.

Objective: To establish guidelines based on scientific evidence for the management of familial Mediterranean fever.

Description Of The Evidence Collection Method: The Guideline was prepared from 5 clinical questions that were structured through PICO (Patient, Intervention or indicator, Comparison and Outcome), to search key primary scientific information databases. After defining the potential studies to support the recommendations, these were graduated considering their strength of evidence and grade of recommendation.

Reduced ovarian reserve in patients with adult polymyositis.

Polymyositis (PM) affects female gender during reproductive age; however, there is no study assessing ovarian reserve in these patients to evaluate ovarian reserve markers in PM. Eight female patients with PM (Bohan and Peter criteria, 1975) with aged 18-40 years, followed at our tertiary centre from March 2011 to May 2014, were invited to participate. They were age-matched with 16 healthy individuals (control group).

Musculoskeletal manifestations and autoantibodies in children and adolescents with leprosy.

Objective: To evaluate musculoskeletal involvement and autoantibodies in pediatric leprosy patients.

Methods: 50 leprosy patients and 47 healthy children and adolescents were assessed according to musculoskeletal manifestations (arthralgia, arthritis, and myalgia), musculoskeletal pain syndromes (juvenile fibromyalgia, benign joint hypermobility syndrome, myofascial syndrome, and tendinitis), and a panel of autoantibodies and cryoglobulins. Health assessment scores and treatment were performed in leprosy patients.

Thrombotic thrombocytopenic purpura at presentation of juvenile systemic lupus erythematosus patients.

Thrombotic thrombocytopenic purpura (TTP) is a rare and life-threatening hematological abnormality characterized by thrombocytopenia and microangiopathic hemolytic anemia, with neurological abnormalities and/or renal disease. TTP has been rarely reported in juvenile systemic lupus erythematosus (JSLE) patients and, to our knowledge, its prevalence in a paediatric lupus population has not been studied. Therefore, from January 1983 to December 2010, we reviewed the charts of 5,508 patients followed-up at the Paediatric Rheumatology Unit of our university hospital.

Severe intestinal involvement as initial manifestation of systemic childhood polyarteritis nodosa: report of two cases.

Systemic childhood polyarteritis nodosa (C-PAN) is a rare primary vasculitis involving medium or small sized arteries. Abdominal angina is an important and serious complication of PAN, occurring usually 15 to 30 min after food intake, and particularly in adult patients. However, to our knowledge, this involvement as the first manifestation of C-PAN was not described.

Autoimmune disease and multiple autoantibodies in 42 patients with RASopathies.

The association of RASopathies [Noonan syndrome (NS) and Noonan-related syndromes] and autoimmune disorders has been reported sporadically. However, a concomitant evaluation of autoimmune diseases and an assessment of multiple autoantibodies in a large population of patients with molecularly confirmed RASopathy have not been performed. The clinical and laboratory features were analyzed in 42 RASopathy patients, the majority of whom had NS and five individuals had Noonan-related disorders.

Electrochromic properties of a metallo-supramolecular polymer derived from tetra(2-pyridyl-1,4-pyrazine) ligands integrated in thin multilayer films.

The electrochromic behavior of iron complexes derived from tetra-2-pyridyl-1,4-pyrazine (TPPZ) and a hexacyanoferrate species in polyelectrolytic multilayer adsorbed films is described for the first time. This complex macromolecule was deposited onto indium-tin oxide (ITO) substrates via self-assembly, and the morphology of the modified electrodes was studied using atomic force microscopy (AFM), which indicated that the hybrid film containing the polyelectrolyte multilayer and the iron complex was highly homogeneous and was approximately 50 nm thick. The modified electrodes exhibited excellent electrochromic behavior with both intense and persistent coloration as well as a chromatic contrast of approximately 70%.

Osteonecrosis of the jaw on imaging exams of patients with juvenile systemic lupus erythematosus.

Objective: The objective of the present study was to evaluate radiographic changes of the temporomandibular joint (TMJ) in patients with juvenile systemic lupus erythematosus (JSLE) and a control group.

Patients And Methods: Panoramic radiographies of the TMJ of 26 JSLE patients and 28 healthy individuals were evaluated. Multislice computed tomography (MCT) was performed on those patients who presented flattening and/or destruction of mandibular condyles.

Effect of musculoskeletal pain on sexuality of male adolescents and adults with juvenile idiopathic arthritis.

Objective: To develop a questionnaire for the evaluation of sexuality of male patients with juvenile idiopathic arthritis (JIA).

Methods: A cohort of male patients with rheumatoid factor (RF)-negative polyarticular JIA according to the 2004 revised ILAR criteria and inactive disease was studied. The Health Assessment Questionnaire (HAQ) was applied to all patients.

Sexuality in teenagers with epilepsy.

Objectives: In adults with epilepsy it is well known that the epileptic syndrome, seizure frequency and antiepileptic drug use may influence sexual function and behavior. However, knowledge acquired with adult populations has been extrapolated to teenagers, based on the supposition that these patients are influenced by similar factors. This study aimed to evaluate aspects related to sexuality obtained from female adolescents with epilepsy.

Williams-Beuren syndrome: cardiovascular abnormalities in 20 patients diagnosed with fluorescence in situ hybridization.

Objective: To evaluate the cardiovascular findings and clinical follow-up of patients with Williams-Beuren syndrome.

Methods: We studied 20 patients (11 males, mean age at diagnosis: 5.9 years old), assessed for cardiovascular abnormalities with electrocardiography and Doppler echocardiography.