Microcystic Renal Disease and Its Relation to "Infantile Nephrosis"

Abstract

In recent pediatric literature there have appeared several reports of cases in which a renal disturbance was the chief clinical problem; in some instances they have been described as examples of "infantile nephrosis"1; in others as examples of the "nephrotic syndrome." 2,3 Although the term "nephrosis" continues to appear in received classifications of renal disease along and coequal with such well-defined entities as glomerulonephritis and nephrosclerosis of arteriosclerotic origin, it will be generally agreed that practical difficulties arise for both pathologist and clinician when this term is applied in the designation of a particular case. The original concept of the renal lesions of nephrosis as "degenerative" rather than "inflammatory" is no longer pertinent to modern problems of clinicopathological correlation. More recent suggestions 4 that the term "indicates tubular disease" are difficult to accept since physiological and electromicroscopic findings 5 show that the most prominent feature of its clinical aspects, proteinuria, is due to a glomerular lesion, namely, an increased permeability to protein associated with structural changes in the filtering membranes.Nor do even the remote clinical origins of the renal disturbance suggest a mean¬ ingful unity when they are found to arise in and to be a result of such varied situations as pregnancy, suicidal intent with poisons, therapeutic procedures such as transfusions and surgical mishaps, or even the simpler catastrophy of the roof falling on one's head. And finally, as a general cloud over the whole concept drifts the suspicion that in many instances the lesions in the kidney may not be the primary source of the trouble but only effects of some distant, possibly "metabolic" disturbance.In contrast to these uncertainties and ambiguities in the pathological aspect of nephrosis is the clear-cut and well-established clinical fact that a certain collocation of functional disturbances frequently presents itself and demands attention; the situation is commonly designated the "nephrotic syndrome." Appearing thus in adjectival form, the renal implication of this term may seem relatively harmless, as it does not imply, as does the noun "nephrosis," that we are dealing with an entity, either morphological or clinical, of renal disease. The hazard in logic of its use is that, in spite of protestations by the user to the contrary, common sense Downloaded From: http://archpedi.jamanetwork.com/ by a University of British Columbia Library User on 06/18/2015

Congenital nephrotic syndrome and renal vein thrombosis in infancy

“…They attributed the clinical findings to the tubular dilatation and agreed with Oliver (1960) who believed that certain cases of infantile nephrosis could be descriptively designated as 'infantile microcystic renal disease'. In the absence of a family history Hallman et al (1962) did not consider their case as a true example of the familial form of the congenital nephrotic syndrome.…”

Congenital nephrotic syndrome and renal vein thrombosis in infancy

“…They attributed the clinical findings to the tubular dilatation and agreed with Oliver (1960) who believed that certain cases of infantile nephrosis could be descriptively designated as 'infantile microcystic renal disease'. In the absence of a family history Hallman et al (1962) did not consider their case as a true example of the familial form of the congenital nephrotic syndrome.…”

The Nephrotic Syndrome of Infancy

“…Ectasia of the tubules is an interesting histological finding confirmed by microdissection studies (6, 8, 35, 36) and gave rise to the term "microcystic renal disease" (35). It is not seen in all of the Finnish cases (39, 19) and to much lesser extent in cases outside Finland (13, 31).…”

La biopsia renal en el diagnóstico y evolución anatomoclínica de las nefropatías

“…El cuadro clínico albuminúrico-edematoso, junto con la disproteinemia e hipercolesterinemia hacen sencillo el diagnóstico sindrómico. Sin embargo, la posibilidad de que un S. N. pueda ser la expresión clínica de una enfermedad sistemática, incluso en niños 22 • 2 1, la evolución nefrósica de algunas glomerulonefritis y nefritis in-tersticiales 7 • 32 • 03 , los cuadros nefrósicos que pueden aparecer en el curso de tratamientos prolongados con sales de oro y tridiona 7 y sobre todo, la posibilidad de encontrarnos con S. N. resistentes al tratamiento con esteroides en recién nacidos 48 , son argumentos en favor de que la biopsia renal en un elevado número de casos, es absolutamente imprescindible para el diagnóstico de estos enfermos y puede ser de extraordinaria utilidad en el pronóstico y control terapéutico de estos síndromes 6 1 .…”

Congenital nephrotic syndrome and renal vein thrombosis in infancy

“…They attributed the clinical findings to the tubular dilatation and agreed with Oliver (1960) who believed that certain cases of infantile nephrosis could be descriptively designated as 'infantile microcystic renal disease'. In the absence of a family history Hallman et al (1962) did not consider their case as a true example of the familial form of the congenital nephrotic syndrome.…”

The Nephrotic Syndrome of Infancy

“…Ectasia of the tubules is an interesting histological finding confirmed by microdissection studies (6, 8, 35, 36) and gave rise to the term "microcystic renal disease" (35). It is not seen in all of the Finnish cases (39, 19) and to much lesser extent in cases outside Finland (13, 31).…”

La biopsia renal en el diagnóstico y evolución anatomoclínica de las nefropatías

“…El cuadro clínico albuminúrico-edematoso, junto con la disproteinemia e hipercolesterinemia hacen sencillo el diagnóstico sindrómico. Sin embargo, la posibilidad de que un S. N. pueda ser la expresión clínica de una enfermedad sistemática, incluso en niños 22 • 2 1, la evolución nefrósica de algunas glomerulonefritis y nefritis in-tersticiales 7 • 32 • 03 , los cuadros nefrósicos que pueden aparecer en el curso de tratamientos prolongados con sales de oro y tridiona 7 y sobre todo, la posibilidad de encontrarnos con S. N. resistentes al tratamiento con esteroides en recién nacidos 48 , son argumentos en favor de que la biopsia renal en un elevado número de casos, es absolutamente imprescindible para el diagnóstico de estos enfermos y puede ser de extraordinaria utilidad en el pronóstico y control terapéutico de estos síndromes 6 1 .…”

Congenital nephrotic syndrome and renal vein thrombosis in infancy

“…They attributed the clinical findings to the tubular dilatation and agreed with Oliver (1960) who believed that certain cases of infantile nephrosis could be descriptively designated as 'infantile microcystic renal disease'. In the absence of a family history Hallman et al (1962) did not consider their case as a true example of the familial form of the congenital nephrotic syndrome.…”

The Nephrotic Syndrome of Infancy

“…Ectasia of the tubules is an interesting histological finding confirmed by microdissection studies (6, 8, 35, 36) and gave rise to the term "microcystic renal disease" (35). It is not seen in all of the Finnish cases (39, 19) and to much lesser extent in cases outside Finland (13, 31).…”

La biopsia renal en el diagnóstico y evolución anatomoclínica de las nefropatías

“…El cuadro clínico albuminúrico-edematoso, junto con la disproteinemia e hipercolesterinemia hacen sencillo el diagnóstico sindrómico. Sin embargo, la posibilidad de que un S. N. pueda ser la expresión clínica de una enfermedad sistemática, incluso en niños 22 • 2 1, la evolución nefrósica de algunas glomerulonefritis y nefritis in-tersticiales 7 • 32 • 03 , los cuadros nefrósicos que pueden aparecer en el curso de tratamientos prolongados con sales de oro y tridiona 7 y sobre todo, la posibilidad de encontrarnos con S. N. resistentes al tratamiento con esteroides en recién nacidos 48 , son argumentos en favor de que la biopsia renal en un elevado número de casos, es absolutamente imprescindible para el diagnóstico de estos enfermos y puede ser de extraordinaria utilidad en el pronóstico y control terapéutico de estos síndromes 6 1 .…”