ExtractThe accumulation of literature on hereditary nephritis with or without nerve deafness attests to the increased awareness of the existence of this syndrome. I t is the purpose of this report to show that there are distinguishing histologic findings in young patients with minimal disease and normal renal function. Eleven patients were studied; 4 were females and 7 were males. Relevant family histories and clinical laboratory findings are shown in table I. I n all patients, hematuria, gross or microscopic, was present. Exacerbations of the hematuria occurred as a part of the exaggerated response of these patients to upper respiratory infection. Renal functional data are shown in table 11. These data were generally within normal limits for our laboratory. Table I11 outlines the morphologic findings observed on renal biopsy. In renal biopsies obtained from 10 patients, there was observed persistence of fetal-like glomeruli characterized by the circumferential crowding of visceral epithelium and an apparent decrease in the number of patent capillaries. The most striking finding in the tubules was the presence of red cells and red blood cell casts seen mainly in the distal and collecting tubules. Foam cells were an infrequent finding but, when present, were unassociated with interstitial fibrosis. Examination of kidney specimens under the electron microscope revealed the presence of at least three glomeruli in seven patients and of fetal-like glomeruli in all patients. There was also circumferential crowding of visceral epithelial cells, particularly at the surface, facing Bowman's capsule. These epithelial cells were characterized by large nuclei, small amounts of cytoplasm, apparently poorly developed intracytoplasmic organelles, and frequent villous-like cytoplasmic projections on the surface. I n families from whom past histories are unavailable, recognition of distinct renal biopsy characteristics would help identify this syndrome in the early stages of disease. At this stage, the combination of morphologic features considered to be characteristic would include the presence of a) fetal-like glomeruli, which were seen by electron microscopy in 7 patients and by light microscopy in 10 patients; b) mild to moderate hypercellularity ; c) large numbers of tubules filled with red blood cells and red blood cell casts; and d) foam cells in the absence of interstitial fibrosis. The fetal-like glomeruli present in our patients did not correspond to any of the normal stages of glomerular development observed under electron microscopy by other investigators, but rather, appeared to represent dysgenesis. Most patients from whom data are reported were more than 2 years of age, at which time fetal glomeruli are exceptional. Of interest was the observation that almost identical histology was found to exist among the siblings. ANTONOVYCH, DEASY, TINA, D'ALBORA, HOLLERMAN a n d CALCAGNO SpeculationT h e fetal-like glomeruli observed o n renal biopsy might b e the result of either a developmental abnormality or dysgenesis. T ...
ExtractThe present studies were designed to elucidate renal functional changes in infants and children with urinary tract infections. Twenty-three infants and children ranging in age from 4 months to 13 6 / 12 years with urinary tract infections were studied. Four children ranging in age from 2 to 6 10 / 12 years without documented urinary tract infections served as controls. Inulin clearances (Gi) were utilized for measurement of glomerular filtration rate. Renal plasma flow and tubular maximum excretory capacity were ascertained by the intravenous administration of para-aminohippurate (PAH). Second determinations performed in eight patients and a third determination in one patient provided longitudinal follow-up. Renal plasma flow measurements were low in 4 of 6 infants and in 6 of 17 children on initial testing; these decreased values were associated with normal glomerular filtration rates except in 3 subjects. In the 4 patients without documented urinary tract infections, G PAH values were within the normal range. G PAH values obtained initially in all subjects were compared on a surface area basis to those characteristic of normal children. Of the 27 experimental points, 21 were below the line representing the normal average C PAH for children. In this small series, only one of three infants failed to recover this function at the time of repeat testing. The recovery rate was less in the children studied. The data indicate that a decrease in renal plasma flow occurs prior to significant alterations in the other renal functional parameters measured.Twenty-two subjects were tested for both G PAH and Tm P AH-The ratio (G PAH /Tm PAH ) was lower in all three groups than the reported normal value of 7.7. In those patients demonstrating an initially reduced value, 13 of 16 showed normal G PAH values. These findings could represent renal ischemia of proximal tubular tissue.The persistence of renal vascular changes in the face of absent clinical and/or laboratory evidence of urinary tract infections emphasizes the need for caution in identifying the prognosis of children with pyelonephritis and the need for careful long-term evaluation. SpeculationAlthough it has been well documented that the renal medulla is the primary site of involvement in pyelonephritis, the current data suggest that the post-glomerular capillaries and tubular structures in the renal cortex are also early sites of involvement in infants and children. A question raised by the study is the relation of reduced renal plasma flow measurements to the natural history and pathogenesis of recurrent pyelonephritis. It is suggested that agents promoting a change toward normal renal circulation may reverse the pathologic process.
scite is a Brooklyn-based organization that helps researchers better discover and understand research articles through Smart Citations–citations that display the context of the citation and describe whether the article provides supporting or contrasting evidence. scite is used by students and researchers from around the world and is funded in part by the National Science Foundation and the National Institute on Drug Abuse of the National Institutes of Health.
hi@scite.ai
10624 S. Eastern Ave., Ste. A-614
Henderson, NV 89052, USA
Copyright © 2024 scite LLC. All rights reserved.
Made with 💙 for researchers
Part of the Research Solutions Family.