Нефротический синдром

The nephrotic syndrome: a crossroadsossroads

g.n. min. amyloid diabetes change

lupus

profuse proteinuria

hypoalbuminaemia and oedema

thrombosis

infection hyperlipidemia renal failure

Theodor Zwinger III (16488--1724)1724)

Poediatria practica (1722):(1722):

“Anasarca puerorumm.. Edema,Edema, aa general hydrops involvingvolving thethe whole body… from headhead toto footfoot …… with persistent collectionsections ofof lymph. The swellingg isis notnot hardhard oror tense, but is sufficientent thatthat thethe mark of a finger remainsains behindbehind ....

The urine is scanty onon accountaccount ofof the obstruction and compressioncompression of the renal tubules.. WeWe havehave seenseen children … whose eyelidseyelids werewere soso swollen they could scarcelyscarcely openopen their eyes (Latin tr)””

Glomerulonephritis: the historicaltorical background II

- to 1750: “disease” a combination off symptoms,symptoms, humours, and practical treatmentsents-- bone-setting, cutting for stone etcetc..

The enlightenment: introduction of mechanicalechanical and chemical ideas into medicine

1790gases; chemistry on blood and urineurine

1799: Bichat: tissues

1810: Broussais: humoralist theory off diseasedisease

1810: Laennec: physical diagnosis

1830: Dutrochet, Schleiden & Schwannnn:: cellscells

Dropsy was a disease per se inin thethe 18th century

Thus none of his physicians soughtt thethe “cause” of Samuel Johnson’s dropsypsy inin thethe 1780s. Nevertheless, William Heberdenerden (who(who alongside Matthew Baillie introducedced Morgagni’s ideas into England) coulduld write:write:

“a dropsy is very rarely an original distemper,distemper, but is generally a symptom of somee other,other, which is too often incurable”

- and by 1800 its association with liverver diseasedisease and heart failure was recognised

The first description of proteinuricteinuric hypoalbuminemic oedematousatous nephropathy

In 1827,7, BrightBright

and Bostockostock describedribed thethe main featuresfeatures ofof what wewe nownow callcall the nephroticephrotic syndromerome andand relatedd themthem inin aa causativeative chainchain

Why was it so difficult, and tooktook so long, to realise the relationshipnship between dropsy and proteinuriauria ??

•No concept of tissues, cells or evenen individual organ function was evidentdent untiluntil well into the 19th century

•Paradoxically studies of blood chemistry,emistry, by reinforcing humoralism, supportedrted thethe idea that changes in organs were thethe consequence of changes in the blood,lood, andand not vice versa

Glomerulonephritis: the historicaltorical background III

1827 Bright: kidney disease causes albuminuriabuminuria and dropsy (“Bright’s disease”)se”)

1837: improved microscopes, and Valentin’sntin’s double knife for cutting thin sectionstions

1840: Bowman’s description of the glomerulusmerulus

1842: Ludwig’s theory of glomerular filtrationltration

1846: Toynbee (and Bright): glomerularr changeschanges in Bright’s disease on microdissectionissection

1854: carmine - the first histological stainain

1856: Virchow: Cellularpathologie

1869: Klebs: paraffin sectioning

George Johnson (1818 -1896)896)

(Med Chir Trans 1846;29:1-43)

Johnson believed, following Wells, that the albuminuriaalbuminuria inin dropsy came through the glomeruli, since theyey werewere infiltrated with fat as well as the tubules. But hehe illustratedillustrated

only the tubules and urine

Fat and the fatty kidney in thethe early 19th century

•kidneys in dropsy were known to bebe onon occasion greasy (Bright 1827, Rayerer 1840)1840)

•the serum could be latescent from fatfat (Christison 1829)

•histologically, fat globules were evidentvident inin renal tubular cells (Gluge 1839)

•fatty casts and fat bodies were presentsent inin the urine on microscopy (Johnson,, 1846)1846)