Mycophenolate mofetil in children with steroid/cyclophosphamide-resistant nephrotic syndrome.

The purpose of this study was to assess the results of therapy with mycophenolate mofetil (MMF) in children with idiopathic nephrotic syndrome (INS) who were both steroid- and cyclophosphamide-resistant. Treatment lasted a minimum of 6 months, and follow-up data were collected over a 2-year period. The children were divided into two groups: Group 1 (n=34) comprised patients who had received cyclosporine A (CsA) before the initiation of MMF therapy; Group 2 (n=18) comprised patients who received only MMF. Among the 34 patients of Group 1, complete and partial remission were achieved in seven (20.6%) and 13 patients (38.6%), respectively; there was no response in 14 patients (41.2%). Among the 18 patients in Group 2, complete and partial remission occurred in five (27.8%) and six (33.3%) patients, respectively; there was no response in seven patients (38.9%). Eight patients developed chronic kidney disease. The main side-effects were gastrointestinal complaints (n=11, 21%), recurring severe infections (n=1, 1.9%), and mild thrombocytopenia/leucopenia (n=1, 1.9%). MMF proved to be therapeutically effective in 59.5% of the cases. These beneficial effects need to be confirmed in studies with a long-term follow-up after discontinuation of the treatment. Our statistical analysis of the results of therapy with MMF did not reveal any significant difference between its use alone or following CsA administration.

Arterite de takayasu na infância: revisäo de literatura a propósito de 6 casos / Takayasus's arteritis in childhood: review of the literature apropos of 6 cases

Analisaram-se, retrospectivamente, aspectos epidemiológicos, clínico-laboratoriais e terapêuticos em 6 crianças portadoras de Arterite de Takayasu (AT), além de revisao da literatura. Todos os casos preenchiam os critérios do ACR. A idade da apresentaçao diagnóstica variou de 7 a 14 anos (x = 10,3+2,8), sendo 4 pacientes do sexo feminino. O tempo de início da sintomatologia até o diagnóstico variou de 1 mês a 5 anos. Astenia, emagrecimento e hipertensao arterial estiveram presentes em todos os pacientes. Outras manifestaçoes clínicas observadas foram cefaléia, dor abdominal, insuficiência cardíaca, alteraçao dos pulsos periféricos, sopro abdominal e crise convulsiva. A classificaçao angiográfica de Ueno modificada, demonstrou 5 pacientes portadores do tipo III e 1 do tipo II. Corticoterapia foi utilizada em 4 pacientes com a doença em atividade, observando-se remissao das manifestaçoes sistêmicas. Terapia antituberculosa foi utilizada em 5 pacientes com PPD fortemente reator (>15 mm). Controle da hipertensao arterial foi obtido com auxílio de terapêutica anti-hipertensiva. Angioplastia luminal foi realizada em 4 pacientes, com bons resultados em 1 paciente, com remissao de insuficiência renal aguda associada a rim único. Obito ocorreu em 1 criança devido complicaçao de abdome agudo vascular. A investigaçao da AT deve ser realizada nos pacientes com hipertensao renovascular, particularmente quando associada a manifestaçoes sistêmicas e/ou presença de sopro abdominal, alteraçao de pulsos periféricos e manifestaçoes cardiovasculares.