Preliminary evidence that etanercept may reduce radiographic progression in juvenile idiopathic arthritis.

OBJECTIVE: To investigate the rate of radiographic progression, as measured with the carpo-metacarpal ratio (Poznanski score), during etanercept (ETN) therapy in children with polyarticular juvenile idiopathic arthritis (JIA). METHODS: Patients included in the Italian ETN registry who had a standard radiograph of both hands and wrists in the posteroanterior view made at start of treatment and after 1 year were included in the study. The clinical response was assessed by means of the ACR Pediatric definition of improvement. Radiographic progression was determined by calculating the change in the Poznanski score between the baseline and the 1-year radiographs. RESULTS: A total of 40 patients were studied. The frequency of ACR pediatric 30, 50, and 70 response at 1 year was 77%, 72%, and 50%, respectively. The median change in the Poznanski score between baseline and 1 year was + 0.3 units, meaning that, on average, patients experienced improvement in radiographic progression. CONCLUSION: Our pilot study provides evidence that ETN is potentially capable of reducing the progression of radiographic joint damage in JIA. This finding deserves confirmation in a controlled trial.

Mycoplasma pneumonias distribution, epidemiology and prevalence in a triennial survey.

OBJECTIVES: To evaluate: (1) the incidence of pneumonia monthly distribution; (2) the rate of pneumonitis due to Mycoplasma Pneumoniae; (3) the suitability of choosing an empirical-based antibiotic-therapy; (4) the need of a critical revision of Mycoplasma serological data. PATIENTS AND METHODS: We studied 188 patients admitted to the Palermo University Pediatrics Department, from september 1998 to august 2001, with admission diagnosis of pneumonia. RESULTS: The highest incidence of pneumonia was in december and march as in both months 28 cases occurred in the whole period 1998-2001 (average of 9.3 cases per each month). The highest rate of pneumonias by Mycoplasma Pneumoniae was in may with a total number of 11/21 cases (52.3%, average of 3.67 cases per month) in the above mentioned three-year period. CONCLUSIONS: Incidence of Mycoplasma pneumonitis is more represented in subjects that are at school age. Our study confirms the enormous variability of the prevalence of the infection by Mycoplasma Pneumoniae and how difficult it is to make an accurate diagnosis lacking standardized, rapid, specific and comparative methods.

Persistent efficacy of anakinra in patients with tumor necrosis factor receptor-associated periodic syndrome.

OBJECTIVE: To evaluate the efficacy and safety of treatment with the interleukin-1 receptor antagonist anakinra in patients with tumor necrosis factor receptor-associated periodic syndrome (TRAPS) requiring high cumulative doses of steroids. METHODS: Four children (mean age 9.1 years [range 4-13 years]) and 1 adult (age 33 years) with TRAPS were enrolled in the study. The 3 children with cysteine mutations (C52Y, C55Y, C43R) had prolonged and frequent attacks of fever. One child with the R92Q mutation and the adult patient with the C43R mutation displayed a more chronic disease course, with fluctuating, nearly continuous symptoms and persistent elevation of acute-phase reactant levels (including serum amyloid A [SAA]). All patients were treated with anakinra (1.5 mg/kg/day). RESULTS: All of the patients had a prompt response to anakinra, with disappearance of symptoms and normalization of acute-phase reactant levels, including SAA. In all pediatric patients, anakinra was withdrawn after 15 days of treatment. After a few days (mean 5.6 days [range 3-8]) a disease relapse occurred, which dramatically responded to reintroduction of anakinra. During the following period of observation (mean 11.4 months [range 4-20 months]), the patients did not experience episodes of fever or other disease-related clinical manifestations. Levels of acute-phase reactants remained in the normal range. No major adverse reactions or severe infections were observed. CONCLUSION: Continuous treatment with anakinra effectively controlled both the clinical and laboratory manifestations in patients with TRAPS and prevented disease relapses.

[Rheumatoid-like syndrome as a symptom of agammaglobulinemia]. / Sindrome simil-reumatoide, esordio di agammaglobulinemia.

A case of rheumatoid-like arthritis as a symptom of primary agammaglobulinemia X-linked in a 24-month-old child is reported. The treatment protocol is also discussed.

[Diagnosis in pediatric "allergic" patients]. / Problematiche di diagnostica allergologica in pediatria.

BACKGROUND: A clinician who deals with allergic patients needs the help of in vivo and in vitro allergometric tests in order to decide difficult therapeutic choices. METHODS: In the section of Clinical Immunology of the Institute of Paediatrics (Palermo University) two allergologic tests, prick and MAST-CLA, have been studied retrospectively, they were performed on patients observed for allergic disorders over 5 years. The aim of this study was to evaluate the diagnostic capacity of these tests "on the field". The study population is made of 459 patients (age range from 6 months to 16 years) affected with asthma, rhinitis, presumably allergic dermatitis, association of asthma and dermatitis, urticaria, selected on the basis of anamnesis and clinical examination. Eosynophils, basophils, total IgE, specific IgE with DHS-MAST-CLA and prick tests (Bayropharm) for the allergens most frequently responsible of allergic disease in our country, have been evaluated. A double controlled assay of 1040 pairs of allergens with MAST-CLA has been performed. RESULTS: The agreement between prick and MAST appeared to be satisfactory for inhaled allergens. Eosynophils count and total IgE levels showed a significative difference between patients negative versus patients positive for at least one allergen. CONCLUSIONS: The pattern of the allergens most frequently positive changes with age and patients positive for pollens showed positivity for vegetal food allergens with a frequency higher than expected.