RESUMO
OBJECTIVES: To determine whether demographic, clinical and immunological features may predict the outcome in juvenile SSc (JSSc). METHODS: Clinical and laboratory characteristics of patients with JSSc collected from paediatric rheumatology centres worldwide were analysed. First, univariate tests identified those features significantly related with fatal outcome, and then multivariate logistic regression analysis was applied to determine the predictors of mortality. RESULTS: One hundred and thirty-four patients from 40 centres were eligible for the analysis. Sixteen patients died and a rapidly fatal course was observed in most of them: 4/16 died within 1 yr after diagnosis and 10/16 within 5 yrs. At the moment of diagnosis, patients with poor outcome showed a significantly higher frequency of internal organ involvement, particularly cardiac, respiratory and gastrointestinal systems. No significant difference emerged for entity of skin, vascular and musculo-skeletal involvement, nor for auto-antibodies profile and laboratory tests. Multivariate analysis showed the following factors to be significant predictors of mortality: fibrosis on chest X-rays [odds ratio (OR) 11.2], raised creatinine levels (OR 22.7) and pericarditis (OR 41.3), while a short disease duration at diagnosis conferred protection (OR 0.3). CONCLUSIONS: All patients with JSSc and fatal outcome were affected by the diffuse form of the disease, and most of them showed a very rapid progression and early signs of internal organ involvement. This suggests that, in children, SSc may have two possible courses: a rapid development of internal organ failure leading to severe disability and eventually to death, or a slow course of the disease with lower mortality.
Assuntos
Escleroderma Sistêmico/mortalidade , Adolescente , Distribuição de Qui-Quadrado , Criança , Europa (Continente) , Seguimentos , Humanos , Análise Multivariada , América do Norte , Pericardite/complicações , Pericardite/mortalidade , Prognóstico , Fibrose Pulmonar/complicações , Fibrose Pulmonar/mortalidade , Estudos Retrospectivos , Escleroderma Sistêmico/complicações , América do Sul , SobrevidaRESUMO
Clinical, laboratory and radiological findings were evaluated in 26 children with Reiter's syndrome, all of whom met the 1982 diagnostic criteria of A. Calin. Twenty-two of the patients (85%) were male and 4 were female (15%); the mean age at onset was 10.5 years (range 4-15.5 yrs). Mean follow-up time was 28.6 months. Diarrhea prior to onset was observed in 18 cases (69%), but there was no report of venereal disease. The full classic triad was initially observed in only 9 patients (35%), urethritis alone in 6 (23%) and conjunctivitis alone in 4 (15%). Arthritis was present in all patients, with the lower limb joints involved in 25 cases (96%); the pattern was pauciarticular in 18 (69%), polyarticular in 7 (27%) and monoarticular in one (4%). There was complete remission in 15 out of the 26 patients (58%), while a sustained and fluctuating course was seen in 7 (27%) and 3 (11.5%) patients, respectively; a single patient showed a remitting course. Balanitis was present in 11 out of the 22 male (50%) cases. Twelve out of 18 patients tested (67%) proved to be HLA B27 positive and there was radiological evidence of sacroiliitis in 5 out of 24 patients (21%). Reiter's syndrome should be included in the differential diagnosis of the arthritic child. As a rule, the course of joint involvement is remittent and sequelae affecting functional capacity are indeed exceptional.
Assuntos
Artrite Reativa/diagnóstico , Adolescente , Artrite/diagnóstico por imagem , Artrite/etiologia , Artrite Reativa/diagnóstico por imagem , Artrite Reativa/etiologia , Artrite Reativa/imunologia , Artrite Reativa/patologia , Criança , Pré-Escolar , Feminino , Antígenos HLA/análise , Humanos , Masculino , Radiografia , Estudos RetrospectivosRESUMO
The normal values of the radiological lines most frequently used as references in the diagnosis of adult protrusio acetabuli were prospectively determined in 150 children (300 hips) without femoral pathology and aged between 2 and 15 years, mean age 8 years. The teardrop shape was modified according to the child's age, the "crossed" type predominating (48.7%). The mean centre edge (CE) angle fluctuated, with a median value of 31.2 degrees +/- 6.2 degrees, increasing with age and with slightly greater values in females; a large spread of CE angle values was observed within age groups. Acetabular ilioischial line values ranged from +7 to -5 mm, with a mean of +1.8 +/- 2 mm. This last method was the most suitable because it was not modified by changes in incidence of X-rays in radiological studies. With this method protrusio is diagnosed in children when the acetabular line is projected medially, provided that the ilioischial line is 1 or 3 mm or more for boys and girls, respectively. We conclude that the most trustworthy method available to evaluate PA in children should be "line crossing".
Assuntos
Acetábulo/diagnóstico por imagem , Cabeça do Fêmur/diagnóstico por imagem , Acetábulo/anatomia & histologia , Acetábulo/patologia , Adolescente , Criança , Pré-Escolar , Feminino , Cabeça do Fêmur/anatomia & histologia , Cabeça do Fêmur/patologia , Humanos , Masculino , Estudos Prospectivos , Radiografia , Valores de ReferênciaRESUMO
El prognostico de la capacidad funcional en artritis reumatoidea juvenil (ARJ) no ha podido ser establecido con seguridad y distintos son los factores que se han involucrado en el. En este trabajo se evaluo la capacidad funcional en 100 pacientes con ARJ, luego de un tiempo promedio de evolucion de 10 anos, que fueron seguidos en este Servicio. La capacidad funcional fue vinculada con el sexo, la edad de comienzo, la forma de comienzo, la forma de evolucion de la enfermedad y la presencia de factores reumatoideos. Luego de 10 anos de evolucion, 4 de cada 10 pacientes se encontraban en clases funcionales III y IV No se observo relacion entre el grado de capacidad funcional y el sexo, la edad de comienzo y la forma de comienzo de la enfermedad. Los pacientes con evolucion poliarticular y seropositivos para factores reumatoideos tuvieron peor pronostico funcional
Assuntos
Adolescente , Adulto , Humanos , Masculino , Feminino , Artrite Juvenil , PrognósticoRESUMO
En el periodo comprendido entre 1974 y 1981, de un total de 1050 ninos que consultaron por distintas manifestacions osteoarticulares en la Seccion de Estudio y Prevencion de Fiebre Reumatica del Hospital de Pediatria Pedro de Elizalde, 7 tuvieron un diagnostico final de leucosis.Cinco fueron leucemias linfociticas agudas (LLA) y 2, leucemias mieloides agudas (LMA). En todos los casos el diagnostico fue realizado por puncion aspirativa de medula osea. Artritis fue la primera manifestacion de enfermedad en 6 de 7 pacientes; en 5 asociada a fiebre y en 1 a adenomegalia. El compromiso fue poliarticular en 4 pacientes, oligoarticular en 2 y monoarticular en 1. El curso de estas manifesticones fue persistente en 5 pacientes y remitente en 2. Las grandes articulaciones fueron las mas afectadas frecuentemente. Estos casos demuestran que la leucemia aguda debe incluirse en el diagnostico difencial de las enfermedades reumaticas de la infancia. El mielograma es un estudio que debe ser considerado en todo nino afectado de patologia osea o articular cuyo diagnostico sea dudoso
Assuntos
Pré-Escolar , Criança , Humanos , Masculino , Feminino , Artrite , Leucemia , Doenças Reumáticas , Diagnóstico DiferencialRESUMO
El prognostico de la capacidad funcional en artritis reumatoidea juvenil (ARJ) no ha podido ser establecido con seguridad y distintos son los factores que se han involucrado en el. En este trabajo se evaluo la capacidad funcional en 100 pacientes con ARJ, luego de un tiempo promedio de evolucion de 10 anos, que fueron seguidos en este Servicio. La capacidad funcional fue vinculada con el sexo, la edad de comienzo, la forma de comienzo, la forma de evolucion de la enfermedad y la presencia de factores reumatoideos. Luego de 10 anos de evolucion, 4 de cada 10 pacientes se encontraban en clases funcionales III y IV No se observo relacion entre el grado de capacidad funcional y el sexo, la edad de comienzo y la forma de comienzo de la enfermedad. Los pacientes con evolucion poliarticular y seropositivos para factores reumatoideos tuvieron peor pronostico funcional
Assuntos
Adolescente , Adulto , Humanos , Masculino , Feminino , Artrite Juvenil , PrognósticoRESUMO
En el periodo comprendido entre 1974 y 1981, de un total de 1050 ninos que consultaron por distintas manifestacions osteoarticulares en la Seccion de Estudio y Prevencion de Fiebre Reumatica del Hospital de Pediatria Pedro de Elizalde, 7 tuvieron un diagnostico final de leucosis.Cinco fueron leucemias linfociticas agudas (LLA) y 2, leucemias mieloides agudas (LMA). En todos los casos el diagnostico fue realizado por puncion aspirativa de medula osea. Artritis fue la primera manifestacion de enfermedad en 6 de 7 pacientes; en 5 asociada a fiebre y en 1 a adenomegalia. El compromiso fue poliarticular en 4 pacientes, oligoarticular en 2 y monoarticular en 1. El curso de estas manifesticones fue persistente en 5 pacientes y remitente en 2. Las grandes articulaciones fueron las mas afectadas frecuentemente. Estos casos demuestran que la leucemia aguda debe incluirse en el diagnostico difencial de las enfermedades reumaticas de la infancia. El mielograma es un estudio que debe ser considerado en todo nino afectado de patologia osea o articular cuyo diagnostico sea dudoso
Assuntos
Pré-Escolar , Criança , Humanos , Masculino , Feminino , Artrite , Leucemia , Doenças Reumáticas , Diagnóstico DiferencialRESUMO
The Hermansky-Pudlak syndrome is a form of oculocutaneous albinism, characterized by a qualitative platelet defect and deposition of ceroid-like material throughout the reticuloendothelial system. During a 16 month period five patients with Hermansky-Pudlak syndrome presented with symptoms, chest films and pulmonary function studies consistent with restrictive pulmonary disease. In two patients, lung biopsies revealed diffuse interstitial fibrosis. However, light and electron microscopy demonstrated ceroid-like material within alveolar macrophages. In addition, two patients presented with inflammatory bowel disease with deposition of ceroid-like material in the colon. This disorder appears to be more common than is currently recognized and should be considered in the differential diagnosis of diffuse interstitial pulmonary disease and inflammatory bowel disease. A relationship between the deposition of ceroid-like material and pulmonary fibrosis is discussed in light of recent research concerning inflammatory processes. In view of the serious pulmonary, gastrointestinal and hematologic consequences of this syndrome, there is a need for genetic counseling of these patients.