RESUMO
The safety and efficacy of mycophenolate mofetil (MMF) were evaluated in adolescent patients with systemic lupus erythematosus and active or active/chronic class III-V lupus nephritis. During the 24-week induction phase, patients were randomized to oral MMF (target dose 3.0 g/day) or intravenous cyclophosphamide (IVC) (0.5-1.0 g/m(2)/month), plus prednisone. Response was defined as a decrease in 24-hour urine protein:creatinine ratio (P:Cr) to < 3 in patients with baseline nephrotic range proteinuria, or by ≥ 50% if subnephrotic baseline proteinuria, and stabilization (± 25%) or improvement in serum creatinine. In the 36-month maintenance phase, induction therapy responders were randomized 1:1 to MMF (1.0 g twice daily) or oral azathioprine (AZA) (2 mg/kg/day), plus prednisone. In the induction phase, 10 patients received MMF and 14 received IVC; 15 (62.5%) achieved treatment response (MMF, 7 (70%); IVC, 8/15 (57.1%); p = 0.53, odds ratio (95% confidence interval) 2.0 (0.2, 15.5)). There was a non-statistically significant difference in maintenance of response to MMF (7/8; 87.5%) versus AZA (3/8; 37.5%). Seven patients withdrew (MMF, 2; AZA, 5). During both phases, rates of serious adverse events were similar in both arms. During both phases treatment response with MMF was as effective as the comparator.
Assuntos
Imunossupressores/uso terapêutico , Nefrite Lúpica/tratamento farmacológico , Ácido Micofenólico/análogos & derivados , Administração Oral , Adolescente , Fatores Etários , Ásia , Azatioprina/uso terapêutico , Biomarcadores/sangue , Creatinina/sangue , Ciclofosfamida/uso terapêutico , Método Duplo-Cego , Quimioterapia Combinada , Europa (Continente) , Feminino , Humanos , Imunossupressores/administração & dosagem , Imunossupressores/efeitos adversos , Nefrite Lúpica/sangue , Nefrite Lúpica/complicações , Nefrite Lúpica/diagnóstico , Masculino , Ácido Micofenólico/administração & dosagem , Ácido Micofenólico/efeitos adversos , Ácido Micofenólico/uso terapêutico , Razão de Chances , Prednisona/uso terapêutico , Estudos Prospectivos , Proteinúria/tratamento farmacológico , Proteinúria/etiologia , América do Sul , Fatores de Tempo , Resultado do Tratamento , Estados UnidosRESUMO
We describe 10 patients who developed a psoriatic skin eruption during either the acute or convalescent phase of Kawasaki disease. The skin eruption was pustular in 3 patients, but more typical psoriasiform skin lesions were seen in the remaining 7 patients. No patient has yet developed chronic psoriasis.
Assuntos
Síndrome de Linfonodos Mucocutâneos/complicações , Psoríase/etiologia , Doença Aguda , Criança , Pré-Escolar , Humanos , LactenteRESUMO
BACKGROUND: Current American Heart Association guidelines indicate that patients with Kawasaki disease and no coronary artery abnormalities on echocardiography at any stage of illness may be discharged from cardiologic follow-up 1 year after onset of illness. METHODS AND RESULTS: To determine whether coronary artery dimensions in patients with Kawasaki disease whose vessels are classified as "normal" by Japanese Ministry of Health criteria have a distribution similar to expected population norms when adjusting for body surface area, we studied 125 patients during 4 intervals from onset of illness: (1) 10 days or less, (2) 2 weeks (11 to 21 days), (3) 6 weeks (22 days to 3 months), and (4) 1 year (4 months to 1.5 years). Using two-dimensional echocardiography, we measured the internal lumen diameter of the left main, proximal left anterior descending, and proximal right coronary arteries. Mean body surface area-adjusted dimensions of the proximal left anterior descending and right coronary arteries were significantly larger (P < .01) in patients with Kawasaki disease than those in subjects in all periods, except for a marginal difference at 6 weeks for the proximal right coronary artery (P = .02); for the left main coronary artery, this difference achieved statistical significance in the period of 10 days or less, with a trend at 2 weeks (P = .02). Among patients classified as having normal coronary arteries on all echocardiograms by the Japanese Ministry of Health criteria, 27% had at least 1 body surface area-adjusted coronary dimension more than 2 standard deviations above the expected mean. CONCLUSIONS: Coronary artery dilation in Kawasaki disease is thus more prevalent than previously reported, highlighting the need for systematic long-term surveillance of this population.
Assuntos
Vasos Coronários/patologia , Síndrome de Linfonodos Mucocutâneos/patologia , Adolescente , Superfície Corporal , Criança , Pré-Escolar , Vasos Coronários/diagnóstico por imagem , Dilatação Patológica , Humanos , Lactente , Síndrome de Linfonodos Mucocutâneos/diagnóstico por imagem , Valores de Referência , UltrassonografiaRESUMO
We describe four children with Kawasaki disease resistant to treatment with intravenously administered immune globulin who were treated with high doses of methylprednisolone. All four patients apparently responded with normalization of symptoms, and none had significant progression of coronary artery abnormalities or adverse events. We recommend pulse methylprednisolone therapy (30 mg/kg per day) during a 1- to 3-day period for patients with Kawasaki disease who do not respond to intravenous immune globulin therapy or who have recrudescent disease after adequate therapy.
Assuntos
Metilprednisolona/uso terapêutico , Síndrome de Linfonodos Mucocutâneos/tratamento farmacológico , Pré-Escolar , Esquema de Medicação , Resistência a Medicamentos , Feminino , Humanos , Imunoglobulinas Intravenosas/uso terapêutico , Lactente , Masculino , Metilprednisolona/administração & dosagem , Resultado do TratamentoRESUMO
We retrospectively reviewed the effects of intravenous gamma-globulin (IVGG) re-treatment of 13 children with Kawasaki disease and persistent or recrudescent fever. Fever and mucocutaneous inflammation resolved within 48 hours in nine patients; fever abated in two other children after a third course of IVGG. We conclude that IVGG re-treatment of Kawasaki disease appears to be safe and may improve the clinical course.
Assuntos
Imunoglobulinas Intravenosas/uso terapêutico , Síndrome de Linfonodos Mucocutâneos/terapia , Pré-Escolar , Feminino , Humanos , Masculino , Síndrome de Linfonodos Mucocutâneos/epidemiologia , Estudos Retrospectivos , Falha de TratamentoRESUMO
Because patients with Kawasaki disease have low serum concentrations of salicylates despite high doses, and because the free (unbound) drug is responsible for the pharmacologic effects of salicylates, we assessed salicylate protein binding in patients with Kawasaki disease. During the acute phase of the disease, protein binding of salicylate in 36 children with Kawasaki disease was 73 +/- 12%, significantly lower than during the subacute phase (90.4 +/- 8.7%; p less than 0.0005). Mean serum albumin concentration was 29.2 +/- 6.4 gm/L during the acute phase and 36.7 +/- 7.8 gm/L during the subsequent subacute phase (p less than 0.005). Salicylate protein binding was affected independently by both serum albumin and total salicylate levels. During the acute phase of Kawasaki disease, children had an average twofold increase in free salicylate compared with normoalbuminemic control subjects. A nomogram has been devised to derive free salicylate levels from the known total salicylate and serum albumin concentrations.
Assuntos
Síndrome de Linfonodos Mucocutâneos/sangue , Salicilatos/sangue , Albumina Sérica/metabolismo , Doença Aguda , Adulto , Radioisótopos de Carbono , Criança , Humanos , Síndrome de Linfonodos Mucocutâneos/tratamento farmacológico , Ligação Proteica , Salicilatos/administração & dosagem , Salicilatos/análise , Albumina Sérica/análise , UltrafiltraçãoRESUMO
In five children who met the diagnostic criteria for Kawasaki Disease, sensorineural hearing loss developed in association with the acute illness. The children, aged 7 months to 13 years, had deficits ranging from mild to profound bilateral sensorineural hearing loss. There were no associated neurologic abnormalities, and immunologic investigations and magnetic resonance imaging failed to reveal a cause. Treatment regimens differed among the children, but none had high salicylate levels (greater than 20 mg/dl) or received other ototoxic medications. Antiinflammatory therapy was not obviously beneficial in any case, and four of the children have persistent hearing deficits. We conclude that auditory involvement may be a complication of Kawasaki disease; screening of clinically affected children should be considered.