Nefrittisk syndrom - Amboss

Question 1

Hva er definsjonen på nefrittisk syndrom?

Answer 1

Azotemia; An increase in the level of nitrogenous waste (both urea and creatinine) due to renal insufficiency.

Question 2

Hvilke underliggende sykdommer er assosiert med nefrittisk syndrom?

Answer 2

Poststreptokokk glomerulonefritt

IgA nefropati (Berger disease)

Småkarsvaskulitt:
- Granulomatøs polyangiit (GPA)
- Mikroskopisk polyangiit (MPA)
- Churg-Strauss syndrom

Anti-GBM nefritt

Thin basement membrane nephropaty (benign familial hematuria)

Alport syndrom

Diffus proliferativ glomerulonefritt

Rapid progressive glomerulonefritt

Membranoproliferativ glomerulonefritt

Question 3

Ved poststreptokokk glomerulonefritt, hvordan er hhv.:
- Epidemiologien
- Klinikk
- Diagnostikk

Answer 3

Question 4

Ved IgA nefropati, hvordan er hhv.:
- Epidemiologien
- Klinikk
- Diagnostikk

Answer 4

Renal histologic findings are identical to those in renal manifestation of IgA vasculitis, indicating a common pathogenesis.

Question 5

Ved granulomatøs polyangiit (GPA), hva er hhv.:
- Epidemiologien
- Klinikk
- Diagnostikk

Answer 5

Question 6

Ved mikroskopisk polyangiit (MPA), hva er hhv.:
- Epidemiologien
- Klinikk
- Diagnostikk

Answer 6

Pausi-immune glomerulonephritis; A condition of glomerulonephritis without associated immune complex or antibody deposits seen on histopathologic examination (e.g., immunofluorescence, electron microscopy) of glomerular tissue. Examples include granulomatosis with polyangiitis and microscopic polyangiitis.

Question 7

Ved eosinofil granulomatose med polyangiit (Churg-Strauss syndrom), hva er hhv.:
- Epidemiologien
- Klinikk
- Diagnostikk

Answer 7

Question 8

Ved anti-GBM antibody disease, hvordan er hhv.:
- Epidemiologien
- Klinikken
- Diagnostikken

Answer 8

Question 9

Ved “thin basement membrane nephropathy” (benign familial hematuria), hva er hhv.:
- Epidemiologien
- Klinikken
- Diagnostikken

Answer 9

Question 10

Ved Alport syndrom, hva er hhv.:
- Epidemiologien
- Klinikken
- Diagnostikken

Answer 10

Question 11

Ved diffus proliferativ glomerulonefritt (DPGN), hva er hhv.:
- Epidemiologien
- Klinikken
- Diagnostikken

Answer 11

Question 12

Ved “Rapidly progressive glomerulonephritis” (RPGN), hva er hhv.:
- Epidemiologien
- Klinikken
- Diagnostikken

Answer 12

Question 13

Ved membranproliferativ glomerulonefritt (MPGN), hva er hhv.:
- Epidemiologien
- Klinikken
- Diagnostikken

Answer 13

Question 14

Hva viser bildet?

Answer 14

Postinfectious glomerulonephritis; Microscopic image of a kidney biopsy (PAS stain, high magnification): A renal corpuscle surrounded by numerous tubules can be seen in the center of the image. Because the renal corpuscle is markedly enlarged, the space between the glomerular capillaries and the parietal layer of the Bowman capsule (black dashed outline) is narrowed. Within the glomerulus, there is hypercellularity due to proliferation of endothelial cells (yellow arrowheads) and mesangial cells (black arrowheads). Inflammatory infiltrate, predominantly neutrophils (blue arrowheads) and monocytes (white arrowheads), is also present. As a result, the lumina of the capillaries are not clearly identifiable. This is the histopathologic appearance of postinfectious glomerulonephritis.

Question 15

Hva er en type III hypersensivitetsreaksjon?

Answer 15

Type III hypersensitivity reaction: Immune complexes (antigen-antibody complexes) are formed in the circulation and get deposited in tissues, preferably within the vascular endothelium, certain organs (e.g., kidney, lung), and the serosal membranes (e.g., synovium, pleura, pericardium). The deposited immune complexes activate the complement cascade, resulting in chemotaxis (recruitment of neutrophils). The antigen-bound IgG bind to the Fc receptors (FcR) on the surface of neutrophils that then release lysosomal enzymes, which damage the local tissues. The site of immune complex deposition determines the clinical features of a type III hypersensitivity reaction (e.g., Arthus reaction, vasculitis, glomerulonephritis, synovitis, pneumonitis).

Question 16

Hva viser bildet?

Answer 16

C3 deposits in poststreptococcal glomerulonephritis; Immunofluorescence photomicrograph of a renal biopsy specimen (very high magnification): Subepithelial deposits of C3 are seen distributed within the capillary loops. The deposits are visible as bright green fluorescence in an irregular granular pattern. This finding is typical of poststreptococcal glomerulonephritis.

Question 17

Answer 17

Anterior lenticonus in Alport syndrome; Slit-lamp photograph of the right eye (mydriatic pupil): The light of the slit-lamp produces an optical cross section of the cornea (left optical cross section) and the lens (right optical cross section). Compared to the cornea, the anterior surface of the lens appears conical (normal physiological shape is indicated by the dashed line). This protrusion of the anterior cortex of the lens is referred to as “anterior lenticonus” and it is associated with Alport syndrome.

Question 18

Hva viser bildet?

Answer 18

Lupus nephritis; Photomicrograph of a renal cortex biopsy (PAS stain; high magnification): The mesangial and endothelial proliferation of the glomerulus has resulted in the dislocation of the capillary lumen and thickening of the capillary wall, creating the appearance of “wire loops.” This is the typical appearance of proliferative glomerulonephritis.

Question 19

Hva viser bildet?

Answer 19

Crescent formation in rapidly progressive glomerulonephritis (RPGN); Photomicrograph of kidney biopsy tissue (H&E stain; high magnification). A hypercellular, crescent-shaped zone with an inflammatory infiltrate (yellow overlay) is visible within the glomerulus. Glomerular crescent formation is a characteristic histological finding in RPGN.

Question 20

Hva viser bildet?

Answer 20

Rapidly progressive glomerulonephritis (RPGN); Photomicrograph of a kidney biopsy sample (PAS stain; very high magnification): The glomerulus shows a hypercellular, crescent-shaped zone (green overlay) with an inflammatory infiltrate (examples of macrophages indicated by arrowheads). Glomerular crescent formation is a characteristic histological finding in RPGN.

Question 21

Hva viser bildet?

Answer 21

Tram-track appearance in membranoproliferative glomerulonephritis; Photomicrograph of a kidney biopsy specimen (PAS stain; high magnification). The glomerulus shows slightly increased mesangial cellularity (examples indicated by circles) and duplication of the glomerular basement membrane (GBM). The latter displays in the form of a double contour of the GBM (tram-track appearance; examples indicated by yellow lines). As a secondary finding, there is an Angiostrongylus larva within the glomerulus (blue overlay). Duplication of the GBM is a typical finding in membranoproliferative glomerulonephritis.

Question 22

Hva er den generelle patofysiologien ved nefrittisk syndrom?

Answer 22

The mechanisms that induce salt retention in nephritic syndrome are not clear.

Question 23

Hvordan er patofysiologien ved membranoproliverativ glomerulonefritt (MPGN)?

Answer 23

Type 3 disease is similarly caused by immune complex deposits.

Question 24

Hva er kliniske kjennetegn på nefrittisk syndrom?

Answer 24

Edema is typically milder than in nephrotic syndrome.

Question 25

Hvordan kan man diagnostisere pas. med mistenkt nefrittisk syndrom?

Answer 25

Urinalyse

Blodprøver

Biopsi

Question 26

Hva kan man finne ved urinalyse ved nefrittisk syndrom?

Answer 26

Glomerular hematuria is a typical finding in nephritic syndrome. It is characterized by acanthocytes, RBC casts, and mild to moderate proteinuria. Nonglomerular hematuria is characterized by bright red or pink urine, the occurrence of blood clots, normal RBC morphology, and the absence of RBC casts.

The irregular cell surfaces of the acanthocytes (Mickey Mouse ears) are the result of osmotic stress inside the tubules. The amount of protein and the number of RBCs in the urine is usually low because of diminished glomerular perfusion and compromised functionality. If a greater number of glomeruli are affected, the glomerular filtration rate will decrease and BUN and creatinine will rise.

Question 27

Hva kan blodprøvene vise ved nefrittisk syndrom?

Answer 27

The BUN:creatinine ratio will be > 15 because tubular function is still intact.

Question 28

Når er det indisert med en nyrebiopsi ved nefrittisk syndrom?

Answer 28

Sometimes indicated in patients with a nonspecific disease pattern to confirm diagnosis

Question 29

Hva viser bildet?

Answer 29

Acanthocytes; Photomicrograph of a peripheral blood smear (Wright-Giemsa stain): Numerous red blood cells are present. Some of them have irregular protrusions at the surface (yellow overlay). This is a typical finding of acanthocytes.

Question 30

Hva er det som gir akantocyttformasjonen i tubuli ved nefrittisk syndrom?

Answer 30

Acanthocyte formation: The irregular cell surfaces of the acanthocytes ("Mickey Mouse ears") are the result of osmotic stress experienced by the red blood cells inside the tubules.

Question 31

Hva viser bildet?

Answer 31

Red blood cell cast; Photomicrograph of urine sediment (high magnification): A cast consisting of multiple red blood cells is visible. RBC casts are typically seen in patients with glomerular damage (e.g., glomerulonephritis).

Question 32

Hva viser bildet?

Answer 32

Red blood cell (RBC) casts in urine sediment; Photomicrograph of urine sediment (phase-contrast microscopy): A tubular cast (blue overlay) is visible in the center of the image. The cast is composed of numerous RBCs, identifiable by their characteristic biconvex shape (examples outlined in yellow), in a matrix of fibrin and plasma proteins. Several epithelial cells surrounding the RBC cast are also visible. RBC casts in urinary sediment are typically seen in glomerulonephritis.

Question 33

Hva slags forskjeller er det mellom presentasjonene til nefrittisk og nefrotisk syndrom?

Answer 33

Question 34

Hva er forskjeller i patofysiologien til nefrittisk og nefrotisk syndrom?

Answer 34

Question 35

Hva er årsaker til nefrittisk og nefrotisk syndrom?

Answer 35

Question 36

Fyll inn figuren

Answer 36

Question 37

Hvilken støttende behandling kan man gi ved nefrittisk syndrom?

Answer 37

Question 38

Hvilken medisinsk behandling kan man gi ved nefrittisk syndrom?

Answer 38

Question 39

Hva slags behandling vil man gi ved hhv.:
- Nyresvikt
- MPGN type 1/2

Answer 39