RESUMEN
OBJECTIVE: To evaluate the clinical features and outcome of antiphospholipid syndrome (APS) in children. STUDY DESIGN: Retrospective chart review of patients seen at the Children's Hospital of Philadelphia and Children's Seashore House Pediatric Rheumatology Center between 1988 and 1993. RESULTS: Nine patients with ages ranging from 8 months to 17 years are presented. Clinical features of five patients with primary APS, described in detail, were digital ischemia, stroke, chorea, Addison disease, and pulmonary vaso-occlusive disease. The four children with secondary APS had systemic lupus erythematosus. Clinical features of these patients include livedo reticularis, deep venous thrombosis, and pulmonary hypertension. Antiphospholipid titers, results of coagulation studies, and serologic findings did not predict outcome. CONCLUSION: APS in children has diverse clinical features similar to those in adults and should be considered in cases of unexplained vaso-occlusive disease.
Asunto(s)
Síndrome Antifosfolípido/diagnóstico , Adolescente , Síndrome Antifosfolípido/complicaciones , Niño , Preescolar , Femenino , Humanos , Lactante , Masculino , Estudios RetrospectivosRESUMEN
A number of conditions can cause color and temperature changes in the extremities. These include physical and chemical insults (eg, frost-bite; ergot), vasculitis (eg, systemic lupus erythematosus), or conditions associated with vascular tone. This article reviews two conditions that are commonly seen in children (Raynaud syndrome and reflex sympathetic dystrophy) and a recently recognized condition associated with antiphospholipid antibody. The emphasis is on differences between the adult and childhood forms of these disorders.
Asunto(s)
Enfermedad de Raynaud/etiología , Distrofia Simpática Refleja/etiología , Capilares/patología , Niño , Diagnóstico Diferencial , Humanos , Pronóstico , Enfermedad de Raynaud/diagnóstico , Enfermedad de Raynaud/terapia , Distrofia Simpática Refleja/diagnóstico , Distrofia Simpática Refleja/terapia , SíndromeRESUMEN
We describe five children with varied rheumatic manifestations, including fibromyalgia and arthralgias, ultimately proved to be associated with hypothyroidism. All musculoskeletal symptoms improved after thyroid replacement therapy. We conclude that rheumatic manifestations of hypothyroidism can be as varied in children as in adults.
Asunto(s)
Hipotiroidismo/complicaciones , Artropatías/etiología , Enfermedades Musculares/etiología , Adolescente , Niño , Preescolar , Diagnóstico Diferencial , Femenino , Humanos , Hipotiroidismo/diagnóstico , Hipotiroidismo/epidemiología , Masculino , Estudios RetrospectivosRESUMEN
Many children with acute leukemia have musculoskeletal complaints and non-specific hematologic abnormalities. A total of 10 children with acute leukemia and 10 with systemic juvenile rheumatoid arthritis were compared to delineate which early features could differentiate these diagnoses. Attention to evolving hematologic abnormalities and musculoskeletal findings may expedite diagnosis of these diseases.
Asunto(s)
Artritis Juvenil/diagnóstico , Leucemia-Linfoma Linfoblástico de Células Precursoras/diagnóstico , Artritis/etiología , Artritis Juvenil/epidemiología , Niño , Diagnóstico Diferencial , Femenino , Humanos , Masculino , Enfermedades Musculares/etiología , Leucemia-Linfoma Linfoblástico de Células Precursoras/complicaciones , Leucemia-Linfoma Linfoblástico de Células Precursoras/epidemiología , Estudios RetrospectivosRESUMEN
Twenty-nine children with juvenile rheumatoid arthritis were studied to determine the safety and efficacy of methotrexate therapy. The initial dose of methotrexate averaged 7.1 mg/m2/wk and was given as a single, oral weekly dose or as three divided doses, each separated by 12 hours. Current antiinflammatory medications were continued; 25 of 29 children had had lack of efficacy, and 8 of 29 had toxic effects, with one or more prior drugs such as intramuscularly or orally administered gold, hydroxychloroquine, or D-penicillamine. Intolerable corticosteroid dependency or toxic effects were present in 18 of 29 cases. Methotrexate-treated patients were examined monthly; minimum treatment duration required to assess efficacy and toxicity was 6 months. The range of treatment duration was 8 to 39 months (mean 18.5 months). Efficacy was assessed by comparing pretreatment versus posttreatment fever and rash, swollen-joint counts, articular indexes, duration of morning stiffness, functional class, hemoglobin levels, and platelet counts. Treatment with methotrexate effectively controlled fever and rash in 83% of children with systemic juvenile rheumatoid arthritis, reduced morning stiffness by 63%, eliminated recalcitrant joint restriction in 48%, and reduced numbers of swollen joints and swelling indexes by 46% and 52%, respectively. No significant toxic effects were observed. Juvenile rheumatoid arthritis of long duration, or with major erosions, was more likely to be refractory to methotrexate therapy. We recommend earlier consideration of methotrexate in place of other slow-acting antirheumatic drugs for juvenile rheumatoid arthritis not responding well to usual therapy. Future studies should address potential methotrexate toxic effects in the lungs and reproductive system, as well as outcome after discontinuation of methotrexate treatment.
Asunto(s)
Artritis Juvenil/tratamiento farmacológico , Metotrexato/uso terapéutico , Adolescente , Antiinflamatorios no Esteroideos/uso terapéutico , Niño , Preescolar , Quimioterapia Combinada , Femenino , Humanos , Masculino , Metotrexato/administración & dosificación , Metotrexato/efectos adversosRESUMEN
Although routinely used in health surveys, the factors affecting maternal perceptions of health in children, especially those with health problems, have received little examination. This question has been addressed using a telephone interview of families of 483 children followed in one of three clinics at Children's Hospital of Philadelphia: the Neonatal Follow-up Program, the Pediatric Rheumatology Center and the Spina Bifida Program. Limitations in activities of daily living due to the health of the child, medical care use and variables indicative of socio-economic disadvantage were associated with a maternal rating of child health as fair/poor in all groups. The relationship was strongest in the rheumatologic group and less strong in the other two, suggesting that mothers may distinguish between a chronic active disease in a previously healthy child and the disability following a neonatal event in conceptualizing health. The results add to our information about maternal perceptions in assessing child health and suggest ways in which such ratings may prove useful in assessing health and health services for children with health problems.
Asunto(s)
Actitud Frente a la Salud , Estado de Salud , Salud , Relaciones Madre-Hijo , Actividades Cotidianas , Niño , Servicios de Salud del Niño/estadística & datos numéricos , Enfermedad Crónica , Encuestas Epidemiológicas , Humanos , Lactante , Entrevistas como Asunto/métodos , Medicina , Servicio Ambulatorio en Hospital , Pennsylvania , Factores Socioeconómicos , EspecializaciónRESUMEN
We report 25 children with oligoarticular arthritis associated with Lyme disease. There were 16 boys (male/female ratio 1.8:1); ages ranged from 2 to 15 years. Thirteen (52%) children had no history of erythema chronicum migrans or other rash. Thirteen had temperatures as high as 41 degrees C for up to 2 months before the onset of arthritis. Twelve recalled definite tick bites. Ten (40%) children, of whom seven had no history of rash, were hospitalized for presumed septic arthritis. Another four had diagnoses of pauciarticular juvenile rheumatoid arthritis for as long as 3 years. Seven patients had less acute, recurrent episodes of synovitis. Two children had seventh nerve palsies 2 months before onset of arthritis. All patients had antibodies to the Lyme spirochete. In 14 patients, synovial fluid white blood cell counts ranged from 180 to 97,700/mm3 (greater than or equal to 76% polymorphonuclear leukocytes). Antibiotic therapy was effective in all patients; in 13, orally administered therapy alone resulted in elimination of synovitis and recurrent attacks. Lyme arthritis may be confused with acute bacterial septic arthritis or recurrent "pauciarticular juvenile rheumatoid arthritis," particularly when there is no history of erythema chronicum migrans.