Morphometric Studies on Glomeruli in the Congenital Nephrotic Syndrome

Tryggvason, Karl

doi:10.1159/000181531

Cited by 6 publications

(6 citation statements)

References 12 publications

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“…Kjessler et al [7] reported a slight increase in glomerular size and number of CNF fetuses of identical gestational age as in this study. The increase in glomerular size in their report was mostly due to the increase in the volume of Bowman's space and not that of the capillary tuft as in Tryggvason s [15] report. The results of the present study are similar to those of Kjessler et al [7].…”

Section: Discussionmentioning

confidence: 77%

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Renal Pathology of Fetuses with Congenital Nephrotic Syndrome of the Finnish Type

Autio–Harmainen¹,

Rapola²

1981

Nephron

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A light microscopic study was performed on 10 fetal kidneys of congenital nephrosis of the Finnish type (CNF) and compared to 22 controls. The gestational age of the fetuses ranged from 16 to 24 weeks. A quantitative morphometric analysis was carried out on 6 CNF fetuses and 6 age-matched controls. The organogenesis of the kidney and the nephronogenesis proceeded normally in CNF. The size and the number of glomeruli were the same in CNF and controls. An increase in the proportion of mature glomeruli and a decrease in the percentage of visceral epithelial cells in them suggested accelerated glomerular maturation in CNF. Focal mesangial cell hyperplasia and dilatation of glomerular capillaries with congestion of blood were more often obvious in CNF than in controls. Marked dilatation of proximal tubular lumens and Bowman’s spaces were typical of CNF.

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Section: Discussionmentioning

confidence: 77%

“…by different techniques [8,14,15], Huttunen et al [6] re ported a lower percentage of immature fetal glomeruli in CNF children under the age of 6 months, but not there after. They suggested that the accelerated maturation of glomeruli possibly begins in the fetal period.…”

Section: Introductionmentioning

confidence: 99%

Renal Pathology of Fetuses with Congenital Nephrotic Syndrome of the Finnish Type

Autio–Harmainen¹,

Rapola²

1981

Nephron

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“…2 Proportion of "normal," microcystic, fetal, and sclerotic glomerular subtypes with advancing ages of patients with congenital nephrotic syndrome of Finnish type (CNF). The mean glomerular volume was significantly higher in CNF than in MCNS patients glomerular size and number in CNF [13,14]. The proportion of "normal" glomeruli was inversely correlated with increasing age, while that of microcystic glomeruli directly correlated with increasing age Fig.…”

Section: Discussionmentioning

confidence: 99%

“…The gene product, nephrin, localizes to the slit pores of the podocytes and has been shown to associate with a ligand, called CD2-associated protein (CD2AP), and to participate in the formation of the filtration barrier [7,8]. The histopathology of the kidney in CNF varies according to the severity and duration of disease, and may show either glomeruli of normal appearance or a combination of fetal, microcystic, and sclerosed glomeruli [1,12,13,14]. Despite these recent advances in understanding the molecular basis of the disease, very little information is available on the structural factors involved in the progression of CNF towards renal insufficiency.…”

Section: Introductionmentioning

confidence: 99%

“…Reduced glomerular number has also been recognized as a risk factor for progression of several renal diseases [20,21]. Previous studies have reported increased glomerular size in CNF, but this was in association with increased glomerular number [13,14]. Previous studies have reported increased glomerular size in CNF, but this was in association with increased glomerular number [13,14].…”

Section: Introductionmentioning

confidence: 99%

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Glomerular structural factors in progression of congenital nephrotic syndrome

2003

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The glomerular structural factors associated with progression of congenital nephrotic syndrome of Finnish type (CNF) are incompletely understood. We studied glomerular volume and the proportion of various glomerular subtypes in seven CNF patients (aged 4 months to 3 years). Glomerular tubular connections (GTC) were analyzed in two patients early and late in the disease spectrum (creatinine 0.2 and 2.8 mg/dl, respectively). The proportions of "normal" glomeruli decreased (r=-0.91, P=0.003) and microcystic glomeruli increased (r=0.85, P=0.015), while the fetal and sclerosed glomeruli remained stable (r=0.073 and 0.08 respectively, P=NS for both) with age. The "normal" glomeruli were larger in CNF than in six age-matched minimal change nephrotic patients (P=0.009). GTC analyses in the mild disease showed that 75% of the 12 "normal" glomeruli had normal tubules, and 25% were atubular or were attached to atrophic tubules, while 88% of the 12 fetal glomeruli were either atubular or were attached to atrophic tubules. In the advanced disease, 67% of 12 "normal" glomeruli had normal tubules, and 33% were atubular or atrophic, while none of the fetal or microcystic glomeruli had "normal tubules". Thus atubular glomeruli may play an important role in disease progression in CNF, as it is associated with a progressive decrease in hypertrophied "normal" glomeruli and an increase in largely atubular microcystic glomeruli.

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Glomerular number and size: Facts and artefacts

Pesce

1998

Anat. Rec.

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This review article is concerned with clinical and experimental data on glomerular number and size and includes a critical discussion of the relevant stereological methods. There is considerable evidence supporting the view that the size of the glomerular filter, in toto, is related to the inception and development of glomerular disease. Major divergences from the filter size are incompatible with normal structure and function. Furthermore, the hypothesis has been put forward that there exists a subset of individuals with minor divergences from the norm that is more susceptible to glomerular disease than the general population. Stereological methods for estimating glomerular number and mean glomerular size could be applied to set standard values. In addition, if the above hypothesis is confirmed, then they could identify on biopsy specimens the patients that belong to cohorts at risk of developing glomerular disease. Unfortunately, despite the development of new, robust methods for particle counting and size determination, such as the fractionator and the dissector, no universal standards of glomerular number and size have been agreed on to date. The major difficulties depend on 1) establishing a standard protocol of tissue acquisition and processing and 2) defining possible variations across age, sex, and ethnic subgroups. The considerable effort required to overcome these difficulties should be rewarded, however, by important advances in the understanding of the morphogenesis of glomerular disease and in the diagnostic and prognostic yield of renal biopsy. Anat. Rec. 251:66-71, 1998Rec. 251:66-71, . 1998 Wiley-Liss, Inc. Key words: glomerulus; size; number; quantitation; stereology EXPECTATIONS AND REALITYThe glomeruli are easily recognizable units of similar size and shape that naturally call the microscopist's attention as prime sources of quantitative data relevant to the kidney filtering function. This has given rise to an enormous, often misleading, amount of literature on glomerular stereology. Measuring the glomeruli is indeed a good idea, and there is considerable evidence that changes in glomerular number and size do relate to kidney function. Nonetheless, deriving reproducible, quantitative information from the kidney is far more difficult than expected at first sight.The size of the glomerular filter, in toto, depends on the number, mean dimension, and structural integrity of the individual glomeruli. Unfortunately, a stereological paradigm weighing these three variables is still a goal that is far from being obtained. In fact, no universally accepted standards are available, because neither a technical protocol for tissue processing nor a standard method for data collection has been established to date. Furthermore, the total number of glomeruli has been considered seriously only in the last decade, whereas much has been published in the past and is still being published on the mean size of the glomeruli and on the conditions of the glomerular filter. These considerations do not invali...

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Morphometric Studies on Glomeruli in the Congenital Nephrotic Syndrome

Cited by 6 publications

References 12 publications

Renal Pathology of Fetuses with Congenital Nephrotic Syndrome of the Finnish Type

Renal Pathology of Fetuses with Congenital Nephrotic Syndrome of the Finnish Type

Glomerular structural factors in progression of congenital nephrotic syndrome

Glomerular number and size: Facts and artefacts

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