The Congenital Nephrotic Syndrome

Lange, Kurt; Wachstein, M.; Wasserman, Edward; Alptekin, Fahrettin; Slobody, Lawrence B.

doi:10.1001/archpedi.1963.02080040340003

Cited by 25 publications

(9 citation statements)

References 16 publications

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“…Other authors have found 'immature' glomeruli in Alport's syndrome [8], in the Fanconi syndrome [34,401, and in congenital nephrosis [39]. The existence of these glomeruli in association with disease suggests that dysgenesis may be one of the possible mechanisms in the causation of hereditary nephritis.…”

Section: Discussionmentioning

confidence: 99%

Hereditary Nephritis: Early Clinical, Functional, and Morphological Studies

et al. 1969

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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 ...

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Section: Discussionmentioning

confidence: 99%

Hereditary Nephritis: Early Clinical, Functional, and Morphological Studies

et al. 1969

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“…The variation in glomerular alteration from superficial to deep cortex lends weight to this possibility. Whilst the lesion, as visualized clinically and by light (case 1), electron (cases 2 and 3), and fluorescent microscopy (Lange, Wachstein, Wasserman, Alptekin, and Slobody, 1963), best ties in with a glomerulonephritic lesion in the present state of our knowledge, we must also consider the possibility of an inborn error of protein synthesis with a defect in basement membrane production as suggested by Hallman, Norio, and Kouvalainen (1967). The very unusual findings in the basement membrane described may represent a formation defect.…”

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confidence: 80%

“…If we consider the congenital nephrotic syndrome to be a glomerulonephritic lesion then an antigen-antibody reaction must take place either in the blood stream, with localization in the basement membrane, or the antibodies must be directed against renal tissue or react with it. Lange et al (1963) have suggested that the antibody formation occurs in the mother. Immunological incompatibility between mother and infant born with this syndrome is suggested by the 'second set' reaction observed by Kouvalainen et al (1962) following the transplantation of skin from the nephrotic infant to the mother.…”

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confidence: 99%

Congenital nephrotic syndrome and renal vein thrombosis in infancy

Alexander

Campbell

1971

J Clin Pathol

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Three of the four cases of the nephrotic syndrome in infancy described show the typical clinical and pathological features of the commonly termed congenital nephrotic syndrome, and two of them abnormal immunoglobulins. Two of the infants were siblings. The placental abnormalities and renal electron microscopic changes are reported and are believed to be involved in antigen-antibody reactions. The literature is reviewed and the possible aetiology of these lesions is discussed. The fourth case is considered to be due to thrombosis of the inferior vena cava and renal veins, an extremely rare cause of the nephrotic syndrome in infants.

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“…Autosomal recessive trans mission of CNF has been established [3], Attempts to explain the pathogenetic mechanisms of CNF has so far proved unsuccessful. Signs and findings have been noted in CNF, which could point to possible immunological patho genesis [4][5][6] or could be secondary phenomena [5], The latter possibility seems to be correct in the light of later studies [1,7]. An interesting point is that a girl who was nephrectomized at the age of 4 months lived for 9 months on haemodialysis, and her serum chemistry normalized completely and the general condition improved until the unsuccessful renal transplantation.…”

Section: Introductionmentioning

confidence: 99%

Number of Nephrons in Normal Human Kidneys and Kidneys of Patients with the Congenital Nephrotic Syndrome

Tryggvason

Kouvalainen

1975

Nephron

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A method of graded sieving for counting of glomeruli in removed kidneys is described. The number of nephrons (glomeruli) in six normal kidneys deriving from autopsies of three children 22–73 months of age was 1.82 ± 0.35 × 10⁶. The results give a little higher number than most previous studies with which they are compared. In the kidneys of two 10- and 15-month-old patients with the congenital nephrotic syndrome of the Finnish type the mean number of nephrons was 3.17 ± 0.52 × 10⁶ which is on the average 73 % higher than in the normal kidneys studied. The number of nephrons in kidneys of five other patients showed an average of at least 3.42 ± 0.30 × 10⁶ the counting method being a little different. The unusually high number of nephrons in CNF kidneys seems to be fully evident.

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The Congenital Nephrotic Syndrome

Cited by 25 publications

References 16 publications

Hereditary Nephritis: Early Clinical, Functional, and Morphological Studies

Hereditary Nephritis: Early Clinical, Functional, and Morphological Studies

Congenital nephrotic syndrome and renal vein thrombosis in infancy

Number of Nephrons in Normal Human Kidneys and Kidneys of Patients with the Congenital Nephrotic Syndrome

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