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Based on the histologic aspects of the lesions, primary nephrosis with focal glomerular sclerosis has been classified into focal segmental glomerulosclerosis (FSG) and focal global glomerulosclerosis (FGG). Several reports [1–14] define its clinical presentation and attempt to identify the initial signs that indicate a poor prognosis. The criteria indicating poor prognosis were reported to be age of onset [15, 16], the presence of a nephrotic syndrome [10, 15, 17], mesangial proliferation [14], the presence of focal segmental glomerulosclerosis on early biopsies [17], the association of glomerulotubular imbalance [18], the severity of the lesions on an early biopsy [1–3, 5, 6, 8, 10, 12, 13, 17], and especially the resistance of these patients to corticosteroid and immunosuppressive therapy [1–3, 6, 8, 9, 11–13, 16, 17]. The long-term followup of children with focal glomerular sclerosis has been analyzed in detail, however, only in a few articles [2, 3, 17], and these reports have concluded that the prognosis is generally poor. The purpose of our study is to report the outcome of observing 32 children with primary nephrosis and focal glomerular sclerosis over a period of several years. We observed that the long-term prognosis in our series is more favorable than that described by other reports. We believe that this discrepancy is due to the referral profile of our institution and that our population of patients more accurately reflects the full spectrum of the natural history of the disease.

Erythrocyte Na+ and K+ transport systems in children with Bartter syndrome: Increase in passive sodium permeability. Na+ and K+ intracellular content was studied in five children with Bartter syndrome and their age and race-paired controls. Na+ and K+ pump (ouabain sensitive) fluxes, Na+-K+ co-transport (furosemide sensitive), and rate constants of passive Na+ and K+ permeability were determined in each patient and control and also in six parents. The results show that in Bartter syndrome, there is a significant increase in the rate constant of passive Na+ permeability without any change in passive K+ permeability. This increase in the rate constant of passive permeability might explain at least partially the increased intracellular Na+ concentration also found in these patients. Moreover, the maximal rate of ouabain sensitive Na+ efflux was increased slightly, and co-transport fluxes were variable. Parents of patients had normal erythrocyte fluxes. Etude de divers systemes de transport du Na+ et du K + a travers la membrane erythrocytaire chez des enfants souffrant du syndrome de Bartter: Augmentation specifique de la permeabilite passive du sodium. La concentration intra-erythrocytaire en Na+ et K+ a ete mesuree chez cinq enfants souffrant du syndrome de Bartter couples a des temoins du meme âge et de la meme race. Les flux de la pompe a sodium (sensibles a la ouabaine), le co-transport du Na+ et du K+ (sensible a la furosemide) et le taux de permeabilite passive du Na+ et du K+ ont ete determines chez chaque patient et leur temoin ainsi que chez six parents de patients. Nos resultats demontrent que dans le syndrome de Bartter, le taux de la permeabilite passive du Na+ est eleve par rapport aux temoins, sans elevation toutefois du taux de la permeabilite passive du K+. Cette elevation du taux de la permeabilite passive du Na+ peut expliquer en partie les concentrations intra-erythrocytaires elevees de Na+ retrouvees chez nos patients. Quant aux autres systemes de transport, nos patients presentaient un taux maximal d'efiiux de Na+ sensible a la ouabaine legerement eleve et des flux des au co-transport variables. Les flux de Na+ et K+ chez les parents de patients etaient normaux.

Amoxicillin was used in treatment of 20 children suffering from recurrent urinary tract infection. Patients were divided into three groups: those without obstruction, those with obstruction, and those with renal parenchymal damage. Amoxicillin eradicated organisms from over 80% of urine samples within 48 hr. Three weeks after onset of therapy, most of the patients were again infected either with the same organism (which had become resistant to therapy) or with a new organism. Amoxicillin was well tolerated by the children; diarrhea, the only side effect of the drug, was observed in two instances.

Of renal biopsies from 300 children with idiopathic nephrotic syndrome (INS), 32 showed histological lesions of focal glomerular sclerosis. From this latter group, 25 had segmental and focal hyalinosis (FSH) with or without mesangial hypercellularity, and 7 focal glomerular obsolescence (FGO). The overall outcome after a mean observation of 7 years (0.5-18) was as follows: 11 patients (34%) were in complete remission, 12 (38%) had persistent proteinuria and/or recurrent nephrotic syndrome, 6 (19%) were in chronic renal failure and 3 patients (9%) died from non renal disease. Patients with FGO had a lower incidence of hematuria, hypertension and tubular defects, a better response to steroid and cyclophosphamide therapy and a better overall outcome. These results are similar to those reported by Habib and Gubler (Ped. Nephrology, Ed. Rubin and Barratt, p. 499-515) and indicate a more favorable outcome than that reported by Cameron et al. (Clin. Nephrol. 6:213, 1978). A progressive decrease in GFR was observed in some patients with persisting proteinuria or recurring nephrosis indicating that ultimate outcome of FGS in children cannot be established from pediatric age population studies.