Summary and Conclusions
An electrolyte‐losing syndrome is described in three siblings, including one set of twins, and in an unrelated infant who was the product of incest. In most respects the clinical and laboratory findings in these patients were compatible with the condition which has come to be known as Bartter's syndrome. In all four of our patients, however, symptoms were manifested early in life, the disease ran a malignant course and death occurred early. Electron microscopic examination of the kidneys from two of our patients showed characteristic structural changes in the glomeruli including immaturity of the podocytes and extensive loss or fusion of their foot processes. Light microscopy of the kidneys from all four cases showed an unusually large proportion of immature glomeruli that seemed to diminish slowly with age. Some ninety per cent of these small glomeruli were located peripherally. The incidence and size of hypertrophied JGA, also the degree and extent of hyperplastic arterial thickening, appeared to increase with age. These findings are discussed in relation to the hypothesis that the basic abnormality in Bartter's syndrome may involve a defect of sodium reabsorption in the proximal tubule, and that this leads to hypertrophy of the JGAs of functioning nephrons with excessive production and release of renin, which causes vasoconstrictive dormancy of the more peripheral glomeruli.
By the use of the Bowie staining technique, juxtaglomerular cells or their homologues were identified in almost every member of Osteichthyes studied. Despite the fact that juxtaglomerular cells in the mammal, at least, belong to the sodium-retaining rennin–angiotensin–aldosterone system they occurred with frequency in salt-water as well as freshwater fish. Early evidence for the presence of renin in the kidney of certain freshwater fish was strengthened by the identification of granulated afferent arteriolar cells in the kidneys of these species. In an attempt to determine which structural components are necessary for the development of juxtaglomerular cells, their anatomical relationships in the fish were compared with the ones existing in higher forms.
A fluorescent antibody method of staining the juxta glomerular cells (JG-cells) of wax-embedded renal tissue, initially prepared by freeze-substitution, was developed. Specimens of antisera to pig renin, specially absorbed by low-renin pig kidney powder stained only the granules of the JG-cells of the pig kidney. These antisera also cross-reacted with the JG-granules of dog and rabbit. When plasma globulins were elevated in the dog, globulin deposits could be stained with conjugated antibodies to canine globulins inside the JG-cells. Because of this finding the JG-cells were judged to be highly permeable to plasma constituents and perhaps angiotensinogen.
Earlier work failed to establish the presence of juxtaglomerular cells (JG-cells) in the kidney of the hog embryo, although renin was found in both the embryonic metanephros and mesonephros. By the use of the fluorescent-antibody technique and the Bowie stain, mature granulated JG-cells were found in both instances. These cells possessed the same anatomical relationships as the JG-cells in the adult hog. These findings offer further evidence for the presence of renin in juxtaglomerular cells. JG-cells were also identified in the embryonic metanephros of the rat.
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