Prognosis of the Nephrotic Syndrome in Children

Abstract

Nephrosis, as a pathologic concept, was first described in 1905 when Muller suggested the term to designate purely degenerative conditions of the kidney. In 1906 Munk pointed out the clinical significance of lipoid bodies in the urinary sediment, and later he gave the name "lipoid nephrosis" to conditions in which they were found. In 1914 Volhard and Fahr accepted Muller's definition of nephrosis and used it to denote one of their three major divisions of renal disease. As such it included all types of renal disease in which at autopsy the lesions were found to be solely of degenerative type. The pathologic changes were not necessarily restricted to the tubules, but, if glomerular changes were present, they were of a noninflammatory nature. At this stage in the evolution of the term, therefore, "nephrosis" was used to describe a group of conditions covering all cases of degenerative Bright's disease, from the simple cloudy swelling of febrile illness to the necrotic lesions of mercurial poisoning. During the following decade and a half the use of the term was a continued subject of controversy.1 With an increasing number of pathologic reports and clinical observations it was gradually accepted in common medical usage, but most investigators restricted it, even when used without qualifying adjectives, to a definite clinical and pathologic entity. Clinically, the entity was characterized by a gradual insidious development of anasarca, massive albuminuria and lipoiduria, further by an absence of retention of nitrogen, elevation of the blood pressure and hematuria (gross or microscopic) ; pathologically, it was characterized by degenerative lesions in the kidney without evi¬ dence of inflammatory change.Recently, however, confusion has been injected by the recognition that the syndrome, as described, rarely continues in pure form, if it is possible to observe a patient over long enough periods of time, and by the recognition that at postmortem examination the lesion is rarely