Rheumatic and connective tissue diseases in children at Santiago, ChileA collaborative ep.cerniclogical study o" pediatric rheumctic end connective tissue d'seases that were identified in patients aged 15 years or !ess (children] along year • 9

Thirty one patients, aged 4 to 15 years old with Systemic Lupus Eiythematosus (SLE) were evaluated retrospectively in order to analyse the clinical course of the disease. Twenty six patients were female and 5 males. All of them fulffled the criterions stated by the American Rheum atolo gist Academy for the diagnosis of S.L.E. Clinical and laboratory diagnostic parameters were similar to those of adult type. Our patients had mainly articular and cutaneous lessions. Whole survival rates were 68 and 40 per cent at five and ten years respectively. In children with renal lupus lesions the survival rates diminished to 57 and 28 per cent Patients without renal involvement had 90 per cent survival rates along the whole 10 years period. The authors rise the possibility to lower mortality rates, in those patients presenting initially with renal S.L.E. damage, by adding to their treatment other inmunosuppressors to the usually prescribed adrenal steroid derivates, because among 19 patients with such an evidence, only 5/12 (42^ receiving steroids alone survived at 5 years follow up in contrast with 7/7 of those receiving combined schedules.La sobrevida del paciente con Lupus Eritematoso Diseminado (LED) infantil ha sido materia de controversia debido a los diferentes resultados que se ban publicado en los ultimos afios. En efecto, los primeros trabajos 1 -demostraban sobrevidas a 5 afios de 72% y 42% en nifios sin y con compromise renal respectivamente, en cambio publicaciones mas recientes dan sobrevidas de 100% en pacientes con Iesi6n renal 2 .Estos resultados pueden explicarse, en gran medida, por los avances alcanzados en el diagnos-tic© mediante la introduction de nuevas te"cnicas inmunologicas que permiten hacerlo mas precozmente, un mejor manejo de la actividad lupica y por otra parte, el uso de corticosteroides y andbioticos que ha significado controlar en mejor forma la enfermedad y evitar las complicaciones infecciosas que constituyen una de las principles causas de mortalidad en estos pacientes 3 .No obstante estos adelantos, queda aun por resolver el papel que tienen los nuevos tratamientos, especialmente las drogas inmunosupresoras

Congenital deficiency of the C3 and C4 fractions of complement. A clinical report

No abstract

A 6 month old boy with severe combined immunodeficiency disease was treated successfully with a bone marrow 'ranspiantaiion (BMT) from his HLA-icntical dizyqotic female twin. Development of immune functions was achieved within 3 months with full reconstilulion of T-and B-lympnocyfc-mcdiated responses. The donor was Iransfused with irradiated self fresh whole blood, she showed good tolerance for the procedure, without complications and was discharged 24 hours later without changes in her hematological parameteis. The receptor showed clinical and bacteriological evidences of disseminated infection by bacillus of CalmeUe-Guerin before and after bone marrow transplantation, and was treated with rifanpicine, isoniazid, ethambutol. The clinical pre.sentclion of BCG infection was like that seen in similar cases and mimiked some aspects of local reactions at BCG vaccination site that have been described in patients with Kawasaki disease. No conditioning treatment was given to ihe patient and a mild graft versus host disease was seen one month after bone marrow infusion, consisting of rash, hepatomegaly, thrombocytopenia and increased scrum activity of liver enzymes. He was successfully treated with hydrocortisone and prcdnisonc for three months. One year after bone marrow trnsplantation the patient remains clinically well and free of any significant infection.