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GOODPASTURE'S SYNDROME IN A CHILD: NATURAL HISTORY AND EFFECT OF TREATMENT

ABSTRACT. Martini, A., Binda, S., Mariani, G., Scotta, M. S. and Ruberto, G. (Department of Pediatrics, University of Pavia, Italy). Goodpasture's syndrome in a child: natural history and effect of treatment. Acta Paediatr Scand, 70:435, 1981.–An 8‐year‐old girl with microhematuria of recent onset developed a picture of pulmonary hemosiderosis in the space of 2 months. Some months later while pulmonary involvement was improving severe extracapillary glomerulonephritis developed. Circulating anti‐glomerular basement membrane antibody was detected and linear deposition of IgG along glomerular basement membrane was observed. A diagnosis of Goodpasture's syndrome was made and treatment was started with prednisone, cyclophosphamide and periodic plasmapheresis with complete progressive disappearance of circulating anti‐glomerular basement membrane antibody. After suspension of plasmapheresis despite immunosuppressive therapy and lack of evidence for circulating anti‐glomerular basement membrane antibody, the child went into terminal renal failure. The natural history of the disease in this case and the results of treatment are discussed. To our knowledge this is the first case of Goodpasture's syndrome reported in childhood with demonstration of the presence of anti‐glomerular basement membrane antibody.