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The mammalian kidney is a complex organ, requiring the concerted function of up to millions of nephrons. The number of nephrons is constant after nephrogenesis during development, and nephron loss over a life span can lead to susceptibility to acute or chronic kidney disease. New technologies are under development to count individual nephrons in the kidney in vivo. This review outlines these technologies and highlights their relevance to studies of human renal development and disease.

Background: Premature and/or intrauterine growth-restricted neonates have an increased risk of developing postnatal renal injuries in later life. Studies on renal physiology in these neonates at a corrected age of 30-40 days are scarce and mostly relate to preterm infants. The data from these studies often lack the results of correlation analyses between biochemical parameters and nephron number-data which could provide additional insight and/or improve recognition of individuals at higher risk of renal failure., Methods: Urinary total protein and albumin levels and N-acetyl-β-D-glucosaminidase and cathepsin B activity were evaluated in preterm and intrauterine growth-restricted infants at a corrected age of 30-40 days and compared to data from a healthy control neonate population. The data were then associated with predominant susceptibility factors of renal damage related to low nephron number, such as gestational age, birth weight, total renal volume and renal cortex volume., Results: Compared to the control neonate population, we found significantly increased levels of all biochemical parameters tested in the intrauterine growth-restricted neonates, whereas in the preterm infants we observed a significant increase in cathepsin B activity, total protein level and, to a lesser extent, albumin level. Cathepsin B activity showed a significant, strong and inverse correlation with all surrogate markers of nephron number and was also strongly and positively correlated with urinary albumin level., Conclusions: At this postnatal age, we found that lower nephron number in low birth weight neonates was associated to tubular impairment/injury that could be concurrent with a dysfunction of glomerular permeability. Urinary cathepsin B activity may be a candidate marker for the early prediction of renal susceptibility to damage in low birth weight neonates.

MRI can provide excellent detail of renal structure and function. Recently, novel MR contrast mechanisms and imaging tools have been developed to evaluate microscopic kidney structures including the tubules and glomeruli. Quantitative MRI can assess local tubular function and is able to determine the concentrating mechanism of the kidney noninvasively in real time. Measuring single nephron function is now a near possibility. In parallel to advancing imaging techniques for kidney microstructure is a need to carefully understand the relationship between the local source of MRI contrast and the underlying physiological change. The development of these imaging markers can impact the accurate diagnosis and treatment of kidney disease. This study reviews the novel tools to examine kidney microstructure and local function and demonstrates the application of these methods in renal pathophysiology., (Copyright © 2016 the American Physiological Society.)

Recent advances in molecular genetics and immunocytochemistry have clarified the cell of origin in many renal disorders. Several renal disorders are thought to involve specific segments of the nephron. Renin-secreting tumours arise from juxtaglomerular cells. Clear cell and papillary renal cell carcinoma (RCC) recapitulate the epithelium of the proximal tubules. Oncocytoma and chromophobe RCC differentiate towards Type A and Type B intercalated cells of the cortical collecting duct, respectively. Medullary collecting ducts are the target sites for the development of autosomal recessive polycystic kidney disease, collecting duct carcinoma and medullary carcinoma. Renal papillae are susceptible to unique changes such as necrosis or papillitis. The purpose of our article is threefold: to illustrate the imaging findings of renal disorders that show segmental involvement of the nephron, to describe proximal and distal nephron disorders and to correlate imaging findings of some entities with histopathological features.

In 50 patients with obstructive uropathy, nuclear imaging supplementary to excretory urography did not improve the detection of obstruction or the determination of the site or etiology of the lesion. However, rapid sequential radionuclide images showed decreased renal perfusion in 64%, more frequently than did an obstructive nephrogram (40%); this finding appeared to occur with more severe degrees of obstruction. Functional impairment of the obstructed kidney was demonstrated more often with 131I Hippuran (o-iodohippurate sodium) (91%) than with 99mTc glucoheptonate (68%) or excretory urography (66%). Neither nuclear nor urographic studies could predict the degree of functional recovery of the kidney until the increased intrapelvic pressures was relieved.