![](/user_photo/1598_JTFXX.jpg)
Нефротический синдром
.pdf![](/html/1598/365/html_vzbbwY5NV4.8f1e/htmlconvd-arADt311x1.jpg)
During the whole 19th century, thinkinginking waswas dominated by possible relationshipsnships between different forms of Bright’s’s diseasedisease
•Rayer 1840
•Rokintansky 1846
•Johnson 1846-50
•Wilks 1850
•Frerichs 1859
•Traube 1860
•Virchow 1858 etc. etc.
- the advent of effective histology about 1870870 demonstrated glomerular lesions as wellll asas tubular ones (Klebs 1872, Löhlein 1877)) butbut diddid not solve the problem
![](/html/1598/365/html_vzbbwY5NV4.8f1e/htmlconvd-arADt312x1.jpg)
Concepts of renal pathologyogy 18301900
-Congestion
-Degeneration
-Sclerosis
-what was the relation between eachh andand the others ? A chain (Frerichs), or discretediscrete entities (Wilks) ?
1850s onwards:
Contrast made between primary parenchymatous nephritis (Virchoww)) andand primary renal fibrosis (Traube)
![](/html/1598/365/html_vzbbwY5NV4.8f1e/htmlconvd-arADt313x1.jpg)
Nephrosis: an unhelpful conceptcept ??
•Larval nephrosishrosis
-febrile, diabeticiabetic
•Toxic nephrosisrosis
-mercury etcetc..
•Primary nephrosisphrosis
-syphilis,, idiopathicidiopathic
•Nephrotic gl’nephritisl’nephritis
•Amyloid nephrosisphrosis
|
|
|
|
|
|
Fridrich von Müller |
(Fishberg 1934)1934) |
|
|
(1858-1941) |
|
|
|
|
|
![](/html/1598/365/html_vzbbwY5NV4.8f1e/htmlconvd-arADt314x1.jpg)
Fritz Munk: urinary lipid underder polarised light (1911-3)
Munk introduced the term “lipoid nephrosisphrosis”” on the basis of these findingsgs
![](/html/1598/365/html_vzbbwY5NV4.8f1e/htmlconvd-arADt315x1.jpg)
Franz Volhard & Theodor FahrFahr
Volhard
Die Bright’sche Nierenkrankheit. Springer, BerlinBerlin 19141914
![](/html/1598/365/html_vzbbwY5NV4.8f1e/htmlconvd-arADt316x1.jpg)
Too many diverse blinkered viewsviews
•internists vs paediatricians
•pathologists vs clinicians WITH
no one-to-one clin-path correspondencence PLUS
•confusion of different layers of description:cription:
-aetiology (e.g. post streptococcalal nephritis)nephritis)
-pathogenesis (e.g. lipoid nephrosis))
-histopathology (e.g. lobular nephritis)tis)
-clinical picture (e.g. nephotic nephritisritis))
-evolution /outcome (e.g.progressiveve nephritis)nephritis)
![](/html/1598/365/html_vzbbwY5NV4.8f1e/htmlconvd-arADt317x1.jpg)
Where did proteinuria come fromfrom ??
•haematuria had been shown by Bowmanwman in 1840 and many others to be glomerularmerular
•in “pure” nephrosis, in contrast, onn microscopy, glomeruli appeared normalormal
•on the other hand the tubules weree fullfull ofof fat/protein droplets
•only on nephrotic cases of glomeruloulo-- nephritis were there obvious glomerularerular changes
![](/html/1598/365/html_vzbbwY5NV4.8f1e/htmlconvd-arADt318x1.jpg)
A powerful diversion: Albertert Arthur Epstein (1875-1951)51)
Epstein, of the Mt Sinai Hospital in Newew York, believed from the inadequatee technology of the day (1920-30) thatat hehe could show differences between thehe albumin in the urine and in the plasmasma inin nephrosis, and revived the old ideaa ofof anan appropriate rejection of this abnormalrmal substance into the urine through normalnormal glomeruli (“dyscrasic proteiuria”)..
Later he developed the theory that thishis inin turn depended on thyroid dysfunction,tion, again using technology which was flawedflawed
![](/html/1598/365/html_vzbbwY5NV4.8f1e/htmlconvd-arADt319x1.jpg)
Ludwig Aschoff’s supportt ofof aa glomerular origin of proteinuriaia (1917(1917--))
[lipoid dystrophy of the kidney] “is oftenften toto bebe sought in infective-toxic damages off thethe wholewhole organism, which produces a heightenedned permeability of the glomerular capillariesaries toto albumin and lipids, so that reabsorptivetive processes in the tubular system, in thethe sensesense ofof Cushny, are followed at times by albuminoiduminoid deposits of droplets of hyalin, and att othersothers byby fatty deposits in the tubular epitheliumm.. WithoutWithout doubt glomerular changes are presentent inin addition to those in the tubules”
![](/html/1598/365/html_vzbbwY5NV4.8f1e/htmlconvd-arADt320x1.jpg)
Tubular proteinuria in nephrosisrosis:: early dissenting voicess
“ Il est infiniment vraisemblable qu’à l’originee dede lala néphrose lipoïdique existe use cause toxiqueique quiqui lèselèse les capillaires glomerulaires. Selon nous,, enen effeteffet l’accumulation de lipoïdes dans les celluleses tubulairestubulaires représente une infltration de cholestérine queque lesles cellules ont extraient de l’urine glomérulaireire lorslors desdes processus de réabsorption”
(Govaerts P, Cordier MR. Bull Acad Royalyal MedMed
Belgique 1928; 5: 50-548)
- but proof was not evident until the experimentsents ofof Randerath (Erg Pathol 32,91) and his pupilil HainHain (Virchow’s Archiv 301:339) in 1937/8 on salamanders,alamanders, a species Cordier studied first. Until 1950 thethe generalgeneral view was of a tubular origin for nephrotic proteinuraproteinura..