Introduction: Imperfect past, subjunctive future

Abstract

The concept that focal segmental glomerulosclerosis (FSGS) represents a distinct pathologic syndrome was not due to single insight, but was developing over a period of nearly 50 years. The following highlights may be mentioned. •Fahr 1 Fahr T Pathologische anatomie des morbus brightii. in: Handbuch der speziellen pathogogischen anatomie und. vol 6. Springer, Berlin1925: 156 Google Scholar (1925) showed that patients with lipoid nephrosis who progressed to renal failure showed focal glomerular damage; •Rich 2 Rich AR A hitherto undescribed vulnerability of the juxtamedullary glomeruli in lipoid nephrosis. Bull Johns Hopkins Hosp. 1957; 100: 173 PubMed Google Scholar (1957) examined autopsy tissue from 20 children with nephrotic syndrome and otherwise typical lipoid nephrosis and described progressive sclerosis of glomeruli, affecting first the juxtamedullary glomeruli; •Heptinstall 3 Heptinstall RH Nephrotic syndrome, in Pathology of the Kidney. in: Little Brown, Boston1966: 355-396 Google Scholar (1966) confirmed that some patients with lipoid nephrosis had hyalinization of the glomerular tuft, particularly affecting deep cortical glomeruli. He showed the case of a 12-year-old boy diagnosed as lipoid nephrosis on biopsy examination who died in renal failure within 12 months—at autopsy virtually all glomeruli were hyalinized; •McGovern 4 McGovern VJ Persistent nephrotic syndrome: A renal biopsy study. Australas Int Med. 1964; 13: 306 PubMed Google Scholar (1964) and Hayslett et al 5 Hayslett JP Krassner LS Bensch KG et al. Progression of “lipoid nephrosis” to renal insufficiency. N Engl J Med. 1969; 281: 181-187 Crossref PubMed Scopus (76) Google Scholar (1969) showed that in some patients whose initial biopsy examination showed minimal changes, a later biopsy examination showed focal sclerosing glomerulonephritis; •Churg et al 6 Churg J Habib R White RH Pathology of the nephrotic syndrome in children: A report for the International Study of Kidney Disease in Children. Lancet. 1970; 760: 1299-1302 Abstract PubMed Scopus (442) Google Scholar (1970), writing for the International Study of Kidney Disease in Children, described biopsy examination findings in 127 children with nephrotic syndrome and found that focal sclerosing glomerular lesions were the second most common finding, after minimal changes; •White et al 7 White RH Glasgow EF Mills RJ Clinicopathological study of nephrotic syndrome in childhood. Lancet. 1970; 1: 1353-1359 Abstract PubMed Scopus (268) Google Scholar (1970), describing biopsy examination findings in 145 children, found that focal glomerulosclerosis was also the second most common finding in childhood nephrotic syndrome.