A longitudinal analysis of physical functional disability over the course of juvenile idiopathic arthritis.

OBJECTIVE: To describe the longitudinal course of physical functioning in children with juvenile idiopathic arthritis (JIA) and identify predictors of long-term functional impairment. METHODS: Between January 1987 and December 2002, 227 patients had two or more functional ability questionnaires completed by a parent. The total number of questionnaires was 1356 and the follow-up between first and last questionnaire administration was 949.7 patient years. At each questionnaire administration, patients were assigned to one of three functional disability states (1 = no disability; 2 = mild to moderate disability; 3 = severe disability), based on their functional ability score. Predictor variables included sex, onset age, JIA category, age at visit, disease duration, presence of antinuclear antibodies, joint counts, acute phase reactants and initial disability state. RESULTS: Despite patient variability in the course of physical functioning, the following three longitudinal patterns were observed: (1) a stable state of disability throughout the entire study period, with continued absence of disability in 27.8% of patients and persistently moderate disability in 3.5% of patients; (2) a steady improvement (22.9% of patients) or deterioration (5.7% of patients) in disability over time; (3) a fluctuating course of disability, with deterioration and improvement (40.1% of patients). Younger age at disease onset and a greater restricted joint count were the strongest predictors of long-term functional impairment. CONCLUSION: A wide within-patient and between-patient variability in the longitudinal course of functional disability was found. Children with early disease onset and a greater number of restricted joints had the highest risk of developing long-term physical disability.

PRINTO/PRES international website for families of children with rheumatic diseases: www.pediatric-rheumatology.printo.it.

OBJECTIVE: To prepare a website for families and health professionals containing up to date information about paediatric rheumatic diseases (PRD). METHODS: Firstly, paediatric rheumatology centres and family self help associations were surveyed to characterise current clinical practice of physicians providing care for children with PRD, research activities, and training facilities of each centre. Secondly, international consensus was reached on the content of the website. Finally, the website was developed and the texts translated. RESULTS: The web page contains three main sections: (a) description for families of the characteristics of 15 PRD; (b) list of paediatric rheumatology centres; (c) contact information for family self help associations. A version for 45 countries in 52 languages (with another three in progress) is now available on the web. 291 surveys from 171 centres and 102 family associations were received from 42 countries. The median proportion of time spent in paediatric practice in the centres examined was 100%, with 70% of this time dedicated to paediatric rheumatology. 90% of the centres were willing to perform clinical trials in the future. CONCLUSIONS: The PRINTO/PRES website provides a well defined and competent set of information about PRD, with appropriate multiple translated versions and easy web navigational direction.

Neumonías recurrentes / Recurrent pneumonia

La neumonía recurrente (NR) es un motivo deconsulta ocasional en pediatría ambulatoria. Es necesario diferenciar al niño sano con intercurrencias de quien tiene trastornos predisponentes. Con el objetivo de investigar causas de NR y valorar los métodos de estudio evaluamos 42 niñosen forma prospectiva desde octubre de 1997 hasta noviembre de 1998. Incluimos niños derivados por NR, sin otra patología crónica conocida. Se definió NR a 2 neumonías en el lapso de 1 año ó 3 episodios en cualquier período de tiempo. Confeccionamos una historia clínica para NR. Con las Rx. previas evaluamos la existencia de NR. El diagnóstico clínico (D1) surgió de la anamnesis y el examen físico. Se solicitó a todos PPD, Rx. de Tórax F y P, hemograma, proteinograma, dosaje de Ig A, E, G, M, a los mayores de 2 años subclases de Ig G, títulos de isohemaglutininas, Ac antitetánicos, poblaciones linfocitarias, grupo y factor y test del sudor. Como segunda línea de estudio orientada por la evaluación clínica se solicitó endoscopía, complemento, NTB, espirometría, seriada esófago-gastro-duodenal y TAC de tórax. Con ello se estableció el diagnóstico final (D2) y el elemento diagnóstico clave. La edad media fue de 58.35 meses (rango 10 á 167). Hubo 23 mujeres y 19 varones. El número medio de neumonías fue de 4.2 (rango 2 á 13). En8 casos las neumonías fueron de una misma localización. Hubo internaciones previas en 27 niños. La causa de las NR fue única en 33 casos: asma (n:17), normal con intercurrencias (n:4), aspiración de cuerpo extraño (n:2), microaspiración por RGE (n:2), FQP (n:2), déficit de Ig G2 (n:1), inmunodeficiencia común variable(n:1), inmunodeficiencia combinada (n:1), colaterales aortopulmonares (n:1), bronquiectasias (n:1), causa indeterminada (n:1) y en 4 casos la causa fue múltiple con asma asociada a: déficit de IG A (n:3) y déficit de Ig G2 (n:1). En 5 casos se descartaron las NR. En el 35 por ciento de los caso D1 no concordó con D2. No hubo un único método diagnóstico clave. Concluimos que se deben confirmar losdiagnósticos de NR, es necesario un plan de estudio sistemático que complementeel examen clínico y que el asma es la causa más frecuente de NR.