Design and validation of a new scale to assess the functional ability in children with juvenile idiopathic arthritis (JIA).

OBJECTIVE: The assessment of the functional ability is one of the items of the core set to define improvements in patients with JIA, CHAQ being the most used scale already validated in 32 countries. The aim of this study was to design and validate a new scale named CAPFUN (capacidad funcional = functional ability) to assess functional ability in children with JIA. METHODS: This scale includes 20 items, 8 of upper limbs, 8 of lower limbs, 3 combined, and 1 of cervical spine, developed in two steps according with OMERACT. Each item is scored: 0 when it is impossible to be performed, 1 when it is performed incompletely or with difficulties, and 2 when it is well performed. Seventy three patients with JIA according to ILAR criteria were assessed: 25 boys (34.2%) and 48 girls (65.8%) whose aver-age age was 12.8 years (95% CI 11.8 - 13.8) and the time from disease onset was 5.02 years (95% CI 3.9 - 6.1). For validation purposes, it was applied to 91 healthy children and adolescents. In every patient, correlation with active joints count and functional class according to Steinbrocker was assessed and with CHAQ in 31 patients in this series. RESULTS: The CAPFUN index obtained in all healthy children was 2. Patients' media CAPFUN index was 1.54 (95%CI 1.38 - 1.68). The CAPFUN index for Steinbrocker's class I was 1.84 +/- 1.8; for class II 1.60 +/- 1.5 and for class III 0.91 +/- 1 (F 24.1 p < 0.001). CAPFUN showed significant correlation with CHAQ (Spearman coefficient -0.79 p < 0.001), with active joints count (Spearman coefficient -0.72 p < 0.001) and with Steinbrocker functional classes (Spearman coefficient -0.69 p < 0.001). This scale showed a good internal reliability (alpha coefficient equal to 0.94), its construct validity is demonstrated by its good correlation with Steinbrocker's scale and with CHAQ. CONCLUSION: CAPFUN is a new instrument in order to assess functional ability in children with JIA. This scale showed a good internal reliability. Construct validity is demonstrated by its high correlation with Steinbrocker's scale and with CHAQ. This study demonstrates the usefulness of CAPFUN for the assessment of functional ability in children with JIA.

Triamcinolone into the knee joint in juvenile chronic arthritis.

Sixty children aged less than 10 years with pauci-articular onset juvenile chronic arthritis and knee joint involvement received local injections with triamcinolone hexacetonide into the knee (total 83 knees). One year after injection, 77% of knees were excellent with no soft tissue swelling, no deformity and full range of movement, or good with slight swelling or deformity, but functioning well. Eleven knees which had relapsed within one year improved after a second injection and 2 after a third. Complications were limited to small areas of subcutaneous tissue atrophy in 2 patients. Intra-articular injection of long-acting steroids is a useful means of controlling disease activity in the knee joints of young children with pauci-articular juvenile chronic arthritis.

Juvenile onset Reiter's syndrome. A retrospective study of 26 patients.

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.

Vasculitis, Kawasaki disease, and pseudovasculitis.

Vasculitis is the inflammation and necrosis of vessel wall and may be a primary disease or secondary to another condition. At present, there is not an accurate classification; rather, classification depends on the changing nomenclature of these conditions and it is difficult to categorize some syndromes because they involve several vessel sizes. The most interesting type of vasculitis for the pediatrician is Kawasaki disease. New developments in this field are showing a relationship between this disease and superantigens. Some patients are resistant to high doses of intravenous gammaglobulin; in such cases, when some risk factors are present, some authors suggest the use of steroid pulse therapy. Inflammation of vessel walls results in occlusion of the lumen with necrosis; many other diseases have similar findings, such as infective endocarditis, Sneddon's syndrome, and others discussed here that should be included in the differential diagnosis.

Soluble intercellular adhesion molecule-1 in paediatric connective tissue diseases.

The intercellular adhesion molecule-1 (ICAM-1) is a cytokine-induced glycoprotein involved in the recruitment of cells into tissues undergoing inflammatory responses. The aim of this study was to compare the levels of soluble ICAM-1 (s-ICAM-1) in children with juvenile chronic arthritis (JCA) and systemic lupus erythematosus (SLE) and to evaluate the usefulness of this molecule as marker of disease activity. Levels of s-ICAM-1 were measured in sera using a monoclonal antibody sandwich enzyme-linked immunoassay. Serum levels (mean+/-SD) of s-ICAM-1 in 37 children with JCA, 18 patients suffering from SLE and 25 healthy controls were 609+/-184, 513+/-139 and 210+/-95 ng/ml, respectively. A significant difference could be demonstrated between the levels of s-ICAM-1 in sera from each disease, as a group, and those of healthy controls. Higher levels of s-ICAM-1 were recorded in JCA patients with systemic features and patients who had polyarthritis than in children who were pauciarticular. A positive correlation was observed between s-ICAM-1 levels and disease activity score in SLE patients. Moreover, s-ICAM-1 levels closely followed clinical conditions in five children with SLE during follow-up. The data show that s-ICAM-1 levels are increased in children suffering from connective tissue diseases and reflect disease status or activity, suggesting the usefulness of this molecule in the follow-up of these diseases.

Chondrosarcoma of the proximal phalanx.

An 87-year-old male presented with a painless, large mass on the dorsum of the left foot. He reported that the mass had first appeared 10 years ago and now had become so large that he could no longer tie his shoe. The mass originated from the proximal aspect of the second digit, encompassing the second web space and distal one third of the second and third metatarsals. Surgical excision of the mass was performed and pathologic diagnosis of the specimen confirmed a grade 1 chondrosarcoma. As expected with a lower grade chondrosarcoma, the patient did not have metastasis and fully recovered. While the occurrence of chondrosarcoma is not uncommon, it rarely affects the foot. This appears to be the third case of chondrosarcoma appearing in the proximal phalanx second digit of the foot.

Favourable outcome in 135 children with juvenile systemic sclerosis: results of a multi-national survey.

OBJECTIVE: To increase the current knowledge of the outcome of juvenile systemic sclerosis (jSSc), which is currently limited. METHODS: In order to investigate the patient outcome and prognostic factors, starting October 1994, we distributed questionnaires to 324 paediatric rheumatology centres. RESULTS: Until 15 May 1998 responses from 46 centres were received, 34 of which returned completed questionnaires on a total of 135 patients. One hundred and twenty-two of the 135 patients were Caucasian, 100 were female. The mean age at disease onset was 8.8 yr (S.D. +/- 3.3 yr). The mean disease duration at the last follow-up was 5 yr(S.D. +/- 3.3 yr). At the last follow-up the disease was still active and required medication in 82 patients, 36 had inactive disease on medication, and 16 were in remission. Ninety per cent of the living patients were fully active in daily life at the last follow-up. Eight of the 135 patients had died. These patients had a median age at onset of the disease of 10.5 yr (range 6.7-15.8 yr). The median disease duration until death was 2 yr (range 1-8 yr). The causes of death were heart failure (five), renal failure (one), sepsis (one) and in one case the cause was not defined. The 1 yr survival rate was 99%, the 2 yr was 97% and the 4 yr was 95%. CONCLUSIONS: At a mean follow-up of 5 yr, the current results show a favourable outcome in most patients with childhood onset jSSc and a significantly better survival than in the adult SSc patients.

Pronostico funcional en artritis reumatoidea. / Functional prognosis in rheumatoid arthritis

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

Enfermedad de Kawasaki. Comunicacion de un caso. / Kawasaki's disease. Report of a case

Se presenta un caso de enfermedad de Kawasaki en una nina de 13 meses de edad. Clinicamente presentaba exantema, adenopatia unilateral cervical, hipertemia que no remitia con antipireticos, lesiones en labios y mucosas, inyeccion conjuntival, artritis y artralgias, eritema de palmas y plantas, descamacion periungueal, estrias longitudinales en las unas, trombocitos y eritrosedimentacion elevada.En nuestro conocimiento este seria el cuarto caso publicado en nuestro medio y el primero en la ciudad de Buenos Aires

Pronostico funcional en artritis reumatoidea. / Functional prognosis in rheumatoid arthritis

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

Enfermedad de Kawasaki. Comunicacion de un caso. / Kawasakis disease. Report of a case

Se presenta un caso de enfermedad de Kawasaki en una nina de 13 meses de edad. Clinicamente presentaba exantema, adenopatia unilateral cervical, hipertemia que no remitia con antipireticos, lesiones en labios y mucosas, inyeccion conjuntival, artritis y artralgias, eritema de palmas y plantas, descamacion periungueal, estrias longitudinales en las unas, trombocitos y eritrosedimentacion elevada.En nuestro conocimiento este seria el cuarto caso publicado en nuestro medio y el primero en la ciudad de Buenos Aires

Manifestaciones osteoarticulares en leucemias agudas de la infancia. / Osteoarticular manifestations in acute leukemia in childhood

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

Manifestaciones osteoarticulares en leucemias agudas de la infancia. / Osteoarticular manifestations in acute leukemia in childhood

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