Pathologie (Fach) / Niere (Lektion)

6. SJ.

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• Polycystic kidney disease ADPKD: ∼1/1,000; one of the most common inherited kidney diseases in humansARPKD: ∼1/20,000 Autosomal dominant PKD:- Mutation on chromosome 16 (85% of cases) or 4 (15% of cases) with an alteration ...
• Polyzystische Niere 1. Zystisch erweiterte Hohlräume, die von tubulären Epithelzellen ausgekleidet sind 2. Zysten im Kortex und Medulla 3. Vereinzelte eingeschlossene Glomeruli 4. Atrophisches Nierengewebe, teils mit geringer ...
• Multicystic dysplastic kidney Congenital disease where one or both kidneys don't form correctly because the ureteric bud fails to develop properly.- Most commonly unilateral. May be a result of abnormal induction of metanephric blastema ...
• Medullary cystic kidney disease Autosomal dominant disorder that affects the renal tubules and interstitium.- Presents in adulthood Symptoms: Polyuria or polydipsia, family history, salt wasting Diagnosis: Renal biopsy and genetic ...
• Renal cyst disorder - Autosomal dominant polycystic kidney disease - Autosomal recessive polycystic kidney disease - Medullary cystic kidney disease (= autosomal dominant tubulointerstitial kidney disease) - Multicystic ...
• Diabetic nephropathy #1 leading cause of end-stage renal disease. Non-enzymatic glycylation: - Efferent arteriole stiffening (hyaline arteriosclerosis) → Obstruction to blood flow → increased pressure → increase in ...
• Diabetische Glomerulosklerose 1. Hyaline Arteriolosklerose  2. Vermehrung der mesangialen Matrix = Kimmelstiel-Wilson Nodule 3. Verdickte Basalmembran 4. Tubulusatrophie mit interstitieller Fibrose 5. Begleitentzündung
• Niereninfarkt 1. Koagulationsnekrose: Verstärkte Zytoplasmaazidophilie und Verlust der Kernzeichnung bei erhaltener Architektur 2. Hämorrhagischer Randsaum 3. Leukozytärer Randsaum aus neutrophilen Granulozyten
• Amyloidosis Tissue damage from misfolded protein deposits. Amyloid bilds β-sheets that deposit in extracellular space of tissue and cause damage. Systemic amyloidosis:- AL amyloidosis: Light chain is misfolded and ...
• Amyloidnephropathie 1. Homogene, eosinrote Ablagerungen in den Arterienwände 2. Homogene, eosinrote Ablagerungen in den Glomerulusschlingen. 2. Glomeruläre Struktur im Endstadium weitgehend zerstört  Es handelt sich ...
• Hydronephrosis Condition where excessive amount of urine causes the kidney to dilate. Etiology:- Fetus: Congenital ureteropelvic junction obstruction, vesicoureteral reflux, ureterocele, posterior urethral valves- Kidney ...
• Refluxnephropathie 1. Atrophie der Tubuli 2. Pseudostrumiforme Umwandlung 3. Interstitielle Entzündung 4. Arterien mit adaptativer Intimafibrose 5. Sklerosierung der Glomeruli 6. Ektatisch ausgeweitetes Nierenbecken Der ...
• Membranous glomerulonephritis Immune complexes deposit in the glomerular basement membrane which becomes inflammed and damage → nephrotic syndrome.- Proteinuria (>3.5 g/day), hypoalbuminuria (→ edema), hyperlipidemia - Most commonly ...
• Membranoproliferative glomerulonephritis Innume deposits in glomerulus → inflammation → Nephritic syndrome that co-presents with nephrotic syndrome (hematuria, hypertension, oliguria, azotemia) Type I:- Most common form- Circulating immune ...
• Rapidly progressive glomerulonephritis Nephritic syndrome that is characterized by epithelial cell proliferation in to a thick crescent shape.- Usually adults in 50-60s- Poor pronosis if not treated early Type I: - Anti-glomerular basement ...
• Extrakapillär proliferative Glomerulonephritis (Rapidly progressive glomerulonephritis) 1. Extrakapilläre Proliferate (Halbmonde): bestehen aus proliferierten Parietalepithelien und Entzündungszellen (Histiozyten, Neutrophile), Fibrin 2. Fibrinaustritt ...
• Tubulointerstitielles Infiltrat - Differentialdiagnose ... - Chronische Pyelonephritis - Analgetika-Nephropathie - Hydronephrosis - Transplantatabstossung
• Chronic pyelonephritis Where the kidney develops permanent scarring as a result of recurrent acute pyelonephritis. Etiology: 1. Vesicoureteral reflux (eg, failure of vesicoureteral orfice, bladder outlet obstruction)2. Chronic ...
• Akute Pyelonephritis 1. Destruktion der Nierentubuli durch neutrophile Granulozyten 2. Streifenförmige Entzündungsherde vom Mark bis in die Rinde (aszendierende Pathogenese) 3. Hyperämie des angrenzenden Parenchyms
• Transplantatabstossung 1. Intimaödem mit Einengung der Gefässlichtung (akut) 2. Entzündliche Infiltration des Zwischengewebes, Untergang von Tubulusepithelien (akut) 3. Fibrotische Verlegung der Lichtung einer Nierenarterie ...
• Renal cell carcinoma Most common type of kidney cancer in adults.- Form from epithelial cells in the proximal convoluted convule of the kidney in the cortex.- Mutation in VHL gene on chromosome 3 - M > F- Peak incidence: ...
• Klarzelliges Nierenzellkarzinom 1. Nekrotischer Tumor mit Blutungsherden 2. Hellzellige Tumorzellen, welche Fett und Glykogen enthalten 3. Kapillarreiches, stromaarmes Interstitium 4. Tumor bricht oft in Venen ein 5. Pseudokapsel
• Renal cell carcinoma - Subtypes Clear cell RCC (~80%)- Polygonal cells with a clear, glycogen and/or lipid-filled cytoplasm that are arranged as cords or tubules (non-papillary growth)- Unifocal, unilateral growth Papillary RCC (~10%)- ...
• Renal mass - Differential Benign masses- Angiomyolipoma- Oncocytoma- Metanephric adenoma- Renal abscess- Granulomatous renal disease (e.g., renal tuberculosis, xanthogranulomatous pyelonephritis)- Renal cysts (e.g., polycystic ...
• Nephroblastom (Wilms Tumor) 1. Hohlräume ausgekleidet von einem kubischen Epithel 2. Triphasisches Muster: blastemale, epitheliale und stromale Komponente- Blastemzellen: klein, zytoplasmaarm- Epitheliale Komponente: primitive ...
• Wilms' tumor (nephroblastoma) Kidney tumor composed of metanephric blastema cells (give rise to stromal and epithelial cells).- Most common malignant kidney tumor in children.- Peak incidence: 2-5 years Sporadic or due to mutation ...
• Urothelial cancer Urothelial cell carinoma that can affect the renal pelvis, ureter, bladder and urethra. - Most commony in the urothelium of the bladder.- M > F- Peak incidence: 65 years- Cancer sites:→ Bladder (90%)→ ...
• Bladder cancer Urothelial cell carcinoma (= Transitional cell carcinomas) Non-urothelial cell carinoma:- Squamous cell carcinoma (arise from squamous cell metaplasia)→ Typically form in multiple locations→ Keratinization→ ...
• Renal cortical necrosis Irreversible type of prerenal acute renal injury caused by a sudden drop of blood perfusion to the renal cortex.- Cortical radial arteries are end arteries and therefore more susceptible to ischemia. ...
• Oncocytoma Benign tumor arising from the intercalated tubular cells.An oncocytoma is not confined to the kidneys and may develop in the thyroid gland, pancreas, or the pituitary gland. Pathology- Macroscopy: brown ...
• Angiomyolipoma Benign renal tumors that arise from perivascular epithelioid cells and consist of blood vessels, smooth muscle, and mature fat cells. - Mean age of onset: 43 years- Sex: F > M (4:1)- More often on right ...
• Acute tubular necrosis Causes ∼85% of intrinsic AKIs. - The straight segment of the proximal tubule and the thick ascending limb are especially susceptible to ischemic damage in acute tubular necrosis. Etiology:- Ischemic: ...
• Acute interstitial nephritis (tubulointerstitial nephritis) ... Kidney injury most commonly caused by a hypersensitivity reaction to drugs (allergic interstitial nephritis). Some drugs may also lead to crystal-induced acute kidney injury. Allergic interstitial nephritis:- ...
• Pyelonephritis Infection of the renal pelvis and parenchyma that is usually associated with an ascending bacterial infection of the bladder. Pathogens:- Escherichia coli (∼75-90% of cases) - Pseudomonas aeruginosa- ...
• Microscopic polyangiitis Necrotizing vasculitis of small vessels, typically with renal and pulmonary involvement; however, multiple organ systems may be affected. Clinical features: - Similar to that of granulomatosis with polyangiitis, ...

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