Nefrotisk syndrom - Amboss

Question 1

Hva er definisjonen på nefrotisk syndrom?

Answer 1

Urine protein quantification cutoffs for nephrotic-range proteinuria are somewhat arbitrarily defined (cutoffs used in the literature usually vary from 3.0–3.5 g/24 hours or spot urine protein/creatinine ratio 3.0–3.5 g/g). Some patients with nephrotic syndrome may present with proteinuria slightly below traditional cutoff values.

Question 2

Hvilke diff.diagnoser har man ved nefrotisk syndrom?

Answer 2

Lipoid nefrose:
- “Minimal change disease”

Fokal segmental glomerulosklerose

Membranøs nefropati

Diabetes nefropati

Amyloid nefropati

Membranoproliferativ glomerulonefritt

Question 3

Hva kjennetegner nefrotisk symptom ved lipoid nefrose?

Answer 3

Question 4

Hva kjennetegner nefrotisk syndrom ved fokal segmental glomerulosklerose?

Answer 4

Question 5

Hva kjennetegner nefrotisk syndrom ved membranøs nefropati?

Answer 5

Between basal membrane and podocytes.

Question 6

Hva kjennetegner nefrotisk syndrom ved diabetes nefropati?

Answer 6

Question 7

Hva kjennetegner nefrotisk syndrom ved amyloid nefropati?

Answer 7

Question 8

Hva kjennetegner nefrotisk syndrom ved membranoproliverativ glomerulonefritt?

Answer 8

Question 9

Hvilken primær etiologi har nefrotisk syndrom?

Answer 9

Nephrotic syndrome may be caused by primary glomerular disorders (80–90% of cases).

Question 10

Hvilke sekundære årsaker kan føre til nefrotisk syndrom?

Answer 10

Nephrotic syndrome may be caused by primary glomerular disorders (80–90% of cases) and/or systemic diseases and toxic exposures (10–20% of cases).

Question 11

Hva er patofysiologien til “Minimal change disease”?

Answer 11

Although the pathophysiology of minimal change disease is not fully understood, it is most likely due to circulating cytokines following a T-cell response.

Question 12

Hva er patofysiologien ved fokal segemental glomerulosklerose?

Answer 12

FSGS is classically not associated with immune complex deposition.

Question 13

Hva er patofysiologien bak membranøs nefropati?

Answer 13

Question 14

Hva er patofysiologien ved amyloid nefropati?

Answer 14

The glomeruli are most commonly affected by amyloid deposition. Can have a similar appearance to diabetic nephropathy.

Question 15

Hvilke sekveler oppstår på grunn av skadene på glomerulus ved nefrotisk syndrom?

Answer 15

Normally, the glomeruli are relatively impermeable to proteins (especially negatively charged ones), blood, and cells. Because the damaged glomeruli continue to operate at the normal filtration rate, the renal function parameters remain largely unchanged while proteins are still released into the urine. Although the cause of lipiduria is not entirely understood, it is thought to involve increased permeability to lipoproteins. Shift of water into interstitium → hypovolemia and stimulation of renin-angiotensin-aldosterone system (RAAS) and ADH secretion → ↑ renal water reabsorption → edema formation. Additionally, activation of epithelial sodium channels in the cortical collecting duct → sodium retention → ↑ edema formation. As a result of vitamin D deficiency, intestinal calcium absorption is reduced, leading to hypocalcemia.

Question 16

Fyll inn figuren

Answer 16

Cross section of renal corpuscle; The renal corpuscle consists of the glomerulus, the glomerular (Bowman) capsule, and the juxtaglomerular apparatus. Plasma components are filtered from the fenestrated glomerular capillaries across the glomerular filtration barrier into the urinary space within the Bowman capsule. The juxtaglomerular apparatus consists of the macula densa, the extraglomerular mesangial cells, and the juxtaglomerular cells. It maintains systemic arterial blood pressure.

Question 17

Hva er klassiske manifestasjoner på nefrotisk syndrom?

Answer 17

Unlike edema related to heart failure, the edema in hypoalbuminemia does not follow gravity. Instead, it appears throughout the entire body. The area around the eyes is usually the first clinically apparent localization of edema because connective tissue in this area is particularly loose, making edema very noticeable. Anasarca; A condition characterized by severe, generalized edema. Common causes include right heart failure, renal failure, and liver cirrhosis.

Question 18

Hvilke symptomer oppstår ved nefrotisk syndrom?

Ikke de klassiske manifestasjonene

Answer 18

Question 19

Hvordan starter man diagnostikken ved nefrotisk syndrom?

Answer 19

Question 20

Hvordan kan man stadfeste proteinurien ved nefrotisk syndrom?

Answer 20

Dipsticks have an increased sensitivity to albumin and the test may be falsely negative if proteinuria is mainly due to excess immunoglobulins (e.g., Bence-Jones proteins in multiple myeloma). False-positive proteinuria may occur in the setting of concentrated urine (as indicated by high specific gravity). Test of choice for establishing a quantitative proteinuria baseline and monitoring treatment. 24-hour urine collection more accurately quantifies protein excretion than the spot urine protein/creatinine ratio, especially at very high levels of proteinuria and when proteinuria is subnephrotic. Spot urine protein/creatinine ratio is usually more practical than 24-hour urine collection in the clinical setting because it is less cumbersome, less prone to collection errors, and provides quicker results. It is thus considered an acceptable alternative for confirming nephrotic-range proteinuria (but not for monitoring treatment).

Question 21

Hva kan man se ved urinsedimentmikroskopi ved nefrotisk syndrom?

Answer 21

Microscopic hematuria may also be seen in nephrotic glomerulopathies. It is more commonly seen in FSGS and membranous nephropathy and usually absent or mild in MCD (Minimal change disease).

Question 22

Hva viser bildet?

Answer 22

Maltese cross-nephrotic syndrome This picture demonstrates a polarized specimen of urine with a cast filled with free cholesterol, which produces Maltese crosses. Fatty casts, oval fat bodies and Maltese crosses are all features of nephrotic syndrome.

https://usmlepathslides.tumblr.com/post/19095023151/maltese-cross-nephrotic-syndrome-this-slide

Question 23

Hvilke blodprøver bør man ta ved nefrotisk syndrom?

Answer 23

Findings such as anemia, hemolysis, thrombocytopenia, leukocytosis, and/or thrombocytosis may suggest a secondary cause of nephrotic syndrome (e.g., SLE, other inflammatory conditions, or malignancy). Reflecting hypervolemic hyponatremia due to increased ADH secretion and water retention. Due to urinary losses. Due to urinary losses. Thought to be due to increased synthesis of fibrinogen in the liver. Due to increased synthesis in the liver. Due to urinary losses.

Question 24

Hvilken bildediagnostisk metode er akt. ved nefrotisk syndrom?

Answer 24

Renal ultrasound:

To assess kidney size and shape and rule out obstruction.

Question 25

Hvilke tester bør man ta hvis man mistenker at årsaken til nefrotisk syndrom skyldes hhv.:
- Diabetes nefropati
- Membranøs nefropati
- Lupus nefritt

Answer 25

In primary membranous nephropathy.

Question 26

Hvilke tester bør man ta hvis man tror at årsaken til nefrotisk syndrom skyldes:
- Myelom eller andre plasmacelle dyskrasier
- Kroniske, virale sykdommer
- Syfilis
- Cryoglobulinemi
- Kongenitale nefrotiske syndromer

Answer 26

The finding of a monoclonal protein indicates a plasma cell dyscrasia. Urine Bence-Jones proteins may be seen in amyloid nephropathy secondary to multiple myeloma.

Question 27

Hvordan vil serum elektroforesen se ut ved nefrotisk syndrom?

Answer 27

Serum protein electrophoresis in nephrotic syndrome.

Question 28

Når er det indikasjon for en renal biopsi ved nefrotisk syndrom?

Answer 28

Deferred; Utsatt. E.g., in patients with long-standing uncontrolled diabetes who have other evidence of microvascular disease and a bland urinary sediment or in children under 6 years of age with a clinical presentation suggesting minimal change disease. The indications for renal biopsy in the evaluation of nephrotic syndrome are not specifically defined in current guidelines. Biopsy is usually pursued to define renal pathology when the etiology of nephrotic syndrome is uncertain, when it is felt to be needed to guide management and provide prognostic information, and in cases of relapse or lack of response to treatment.

Question 29

Basert på patologi, hvordan klassifiserer man det nefrotiske syndromet?

Answer 29

Classification of nephrotic syndrome is based on the pattern of injury as seen on light microscopy (LM) of a renal biopsy specimen.

For a complete assessment, all biopsy specimens should be analyzed using LM, immunofluorescence microscopy (IM), and electron microscopy (EM).

Question 30

Hva ser man på biopsien ved Minimal change disease?

Answer 30

EM: Effacement (injured podocytes undergo effacement whereby they lose their structure and spread out, leading to a reduction in filtration barrier function) of the foot processes of podocytes.

LM: No changes in glomeruli (possibly fat bodies in some proximal tubular cells).

Question 31

Hva viser biopsien ved fokal segmental glomerulosklerose?

Answer 31

Damage to podocytes.

EM: effacement of the foot processes (similar to minimal change disease).

LM: segmental sclerosis and hyalinosis and loss of podocytes.

IM: rarely, focal deposits of IgM, C1, and C3 inside sclerotic lesion.

Question 32

Hva viser biopsien ved membranøs nefropati?

Answer 32

Deposition of antibodies between podocytes and the basal membrane.

EM: subepithelial dense deposits (IgG and C3) with a spike and dome appearance.

LM: diffuse thickening of glomerular capillary loops and basal membrane; located between basal membrane and podocytes.

IM: granular subepithelial deposits of immune complexes and complement.

Question 33

Hva viser biopsien ved diabetisk glomerulonefropati?

Answer 33

Light microscopy shows mesangial matrix expansion, thickening of glomerular membrane, and/or nodular eosinophilic glomerulosclerosis (Kimmelstiel-Wilson lesions).

Question 34

Hva viser biopsien ved lupus nefritt?

Answer 34

Light microscopy shows mesangial proliferation, subendothelial and/or subepithelial immune complex deposition, and thickening of the capillary walls (appear as wire loops).

Question 35

Hva viser biopsien ved amyloid nefropati?

Answer 35

EM: amyloid fibrils

LM: Nodular glomerulosclerosis and apple-green birefringence (dobbeltbrytning) (mesangial amyloid deposition) with Congo red stain under polarized light.

Question 36

Hva viser bildet?

Answer 36

Minimal change disease; Photomicrograph of tissue obtained from renal biopsy (H&E stain; 200x magnification). The basement membrane of the glomerulus are intact, the mesangium does not show proliferation. The capillary lumens are visible and there are no vascular changes. This is a typical appearance of a glomerulus in minimal change disease.

Question 37

Hva viser bildet?

Answer 37

Podocyte foot processes fusion in minimal change disease; Electron micrograph of kidney tissue. The effacement of the podocytes foot processes is visible (examples indicated by green outline) as well as swelling of podocyte bodies (example indicated by red overlay). This finding, together with unremarkable light microscopy, is typical of minimal change disease.

Question 38

Hva viser bildet?

Answer 38

Focal segmental glomerulosclerosis (FSGS); Photomicrograph of a kidney biopsy specimen (PAS stain; very high magnification). A cross-section of a glomerulus is visible at the center of the image. The perihilar region is hypercellular and contains hyaline deposits (examples indicated by blue overlay). Loss of the capillary lumens and diffuse sclerosis is also present. These are the typical features of focal segmental glomerulosclerosis (perihilar type).

Question 39

Hva viser bildet?

Answer 39

Focal segmental glomerulosclerosis; Electron micrograph. Ultrastructural findings of focal segmental glomerulosclerosis showing podocyte foot process effacement (thin black arrows) and protein reabsorption droplet (white arrow). Endothelial cells show tubuloreticular inclusions in the upper right-hand corner (star) Effacement of podocyte foot processes is commonly seen in electron microscopy samples of focal segmental glomerulosclerosis.

Question 40

Hva viser bildet?

Answer 40

Membranous nephropathy; Photomicrograph of a kidney biopsy sample (PAS stain; 550× magnification). There is diffuse thickening of the glomerular basement membrane (blue overlay) and capillaries. These are typical microscopic findings of membranous glomerulopathy (most common glomerulonephritis in adults).

Question 41

Hva viser bildet?

Answer 41

Membranous nephropathy; Photomicrograph of a kidney biopsy specimen (Jones silver stain; very high magnification). Thickening of the basement membrane (indicated by yellow outlines) with spike formation (examples indicated by blue arrowheads) can be seen. This histopathologic appearance is characteristic of membranous nephropathy.

Question 42

Hva viser bildet?

Answer 42

Diabetic nephropathy; Photomicrograph of kidney tissue (PAS stain; high magnification). A cross-section of a glomerulus can be seen (dashed outline), featuring a thickened glomerular basement membrane and containing abundant pink, hyaline depositions of PAS-positive material (Kimmelstiel-Wilson nodules or nodular glomerulosclerosis; examples indicated by yellow overlay). Various tubules and vessels surround the glomerulus. An arteriole with distinct hyaline deposits is located above the glomerulus (hyaline arteriolosclerosis; blue overlay). Nodular glomerulosclerosis combined with hyaline arteriolosclerosis is a typical feature seen in diabetic glomerulopathy.

Question 43

Hva viser bildet?

Answer 43

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 44

Hva viser bildet?

Answer 44

Kidney involvement in amyloidosis; Polarized light photomicrograph of the kidney (Congo red stain; 300x magnification). The sediments in the area of the glomeruli and renal interstitium (white arrow; white arrowheads) appear birefringent in the polarized light and bright green in color (characteristic of amyloids). These are both key diagnostic features of amyloid sediments.

Question 45

Hvordan behandler man ødemet ved nefrotisk syndrom?

Answer 45

Over-aggressive diuresis may lead to AKI (due to intravascular volume depletion, especially in children), electrolyte disturbances, and/or thromboembolism (due to hemoconcentration). Most common diuretics are highly protein-bound and act on the luminal renal tubular membrane. Mechanisms of diuretic resistance in nephrotic syndrome include poor oral bioavailability from gut edema, high volume of distribution in hypoalbuminemia (leading to decreased drug delivery to the kidneys), binding of the drug in the tubular lumen by filtered protein, compensatory sodium retention, and other adaptive mechanisms. Titrate dosages as needed. Albumin is not routinely recommended because there is extremely limited evidence supporting its use in this setting.

Question 46

Hvordan kan man behandle proteinurien ved nefrotisk syndrom?

Answer 46

Elimination or reduction of proteinuria is a major treatment goal for nephrotic syndrome and can lead to increased serum albumin, decreased edema, attenuation of the metabolic effects of heavy proteinuria (e.g., hyperlipidemia), reduction in risk of thromboembolism and infection, and slowing of the progression of chronic kidney disease.

Initiation of specific antiproteinuric therapy may be delayed when immune suppression is expected to lead to rapid and complete remission of nephrotic syndrome (e.g., patients with MCD or another steroid-sensitive nephrotic syndrome). Combined treatment is more effective than either medication class alone but may increase the risk of renal dysfunction and/or hyperkalemia. There is conflicting evidence regarding the risk-benefit ratio of combination therapy. Combination therapy may be considered with caution in patients with inadequate response to therapy with a single class of medication. Patients with an abrupt onset of nephrotic syndrome (often seen in MCD) are at high risk of AKI. Patients with nephrotic syndrome are at risk of protein malnutrition. While lowering dietary protein intake can lead to a reduction in proteinuria, very low-protein diets should be avoided because of the increased risk of malnutrition. High protein intake may help offset urinary protein loss, but very high-protein diets are discouraged because of the risk of worsening proteinuria.

Question 47

Hvordan kan man behandle dyslipidemien ved nefrotisk syndrom?

Answer 47

Nephrotic dyslipidemia usually improves or resolves with treatment of the underlying cause of nephrotic syndrome and reduction of proteinuria. There is a lack of evidence on the effects of specific lipid-lowering therapy on patient outcomes (e.g., cardiovascular events) in nephrotic syndrome. Ezetimibe and/or fibrates should not be used as monotherapy because of limited evidence in nephrotic syndrome. Caution is advised when combining statins with calcineurin inhibitors because of the increased risk of rhabdomyolysis.

Question 48

Hvilken risiko har pas. med nefrotisk syndrom for å få en tromboembolisme?

Answer 48

All patients with nephrotic syndrome are at increased risk of thromboembolism, and this risk becomes progressively higher as serum albumin drops below 3.0 g/L.

No randomized controlled trials are available to guide decisions on prophylaxis and treatment of thromboembolism in nephrotic syndrome. Recommendations are based on observational data and expert opinion. Specialist consultation is advised.

Question 49

Hvilke parametre indikerer at en pas. med nefrotisk syndrom bør få profylaktisk antikoagulasjon?

Answer 49

https://www.med.unc.edu/gntools/bleedrisk.html

Question 50

Hvilke medikamentvalg har man hvis man velger å gi profylaktisk antikoagulantia til pas. med nefrotisk syndrom?

Answer 50

In patients with nephrotic syndrome, there is a theoretical risk of heparin resistance as a result of urinary loss of AT III. However, heparin has been used successfully in practice for both prophylaxis and treatment of VTE in patients with nephrotic syndrome. Warfarin should always be preceded by bridging anticoagulation with IV heparin or low molecular weight heparin.

Question 51

Når er det indikasjon for å gi en pas. med nefrotisk syndrom terapeutisk antikoagulasjon?

Answer 51

In patients with nephrotic syndrome, there is a theoretical risk of heparin resistance as a result of urinary loss of AT III. However, heparin has been used successfully in practice for both prophylaxis and treatment of VTE in patients with nephrotic syndrome. Warfarin should always be preceded by bridging anticoagulation with IV unfractionated heparin or low molecular weight heparin.

Question 52

Hvordan behandler man pas. med nefrotisk syndrom for å minimere infeksjonsrisikoen?

Answer 52

No randomized controlled trials are available to guide specific interventions for the screening and prevention of infections in adults with nephrotic syndrome. Recommendations are based on observational data and expert opinion. Screening and preventive measures should be undertaken prior to or concomitant with immunosuppressive therapy for the underlying cause of nephrotic syndrome.

With heptavalent conjugate vaccine (7vPCV) and the 23-valent polysaccharide vaccine (23vPPV).

Question 53

Hvordan behandler man den underliggende sykdommen ved nefrotisk syndrom?

Answer 53

Depending on the histological diagnosis, patient factors, and individualized assessment of risks and benefits. 80–90% of children presenting with nephrotic syndrome respond to steroids within 4 weeks. Renal biopsy is typically only undertaken for nonresponders. E.g., tumor resection in malignancy-associated membranous nephropathy.

Question 54

Hvordan behandler man den primære membranøse nefropatien ved nefrotisk syndrom?

Answer 54

30–35% of patients undergo spontaneous partial or complete remission with conservative management alone. Associated with partial or complete remission in approx. 60–90% of cases. The rate of ESRD among nonresponders is high (50%).

Question 55

Hvordan behandler man den primære (idiopatiske) fokale segmentale glomerulosklerosen ved nefrotisk syndrom?

Answer 55

Immunosuppressive therapy is not recommended for FSGS manifesting with proteinuria but without nephrotic syndrome. Responses are varied: Rates of complete remission are reported in 28–74% of cases and there and the rate of ESRD among nonresponders is high (> 50%).

Question 56

Hvordan vil man behandle den primære (idiopatiske) minimal change disease ved nefrotisk syndrom?

Answer 56

Early spontaneous remission is rarely seen with MCD. Immunosuppressive therapy is therefore recommended for all patients, given the significant morbidity associated with untreated nephrotic syndrome. Supportive treatment with RAAS inhibitors is usually deferred in children but may be considered in adults, for whom response to glucocorticoids is often slower. Most patients respond well to prednisone (approx. 75% achieve complete remission) but up to a third of adult patients overall subsequently develop frequent relapses or steroid dependence. However, progressive CKD is not part of the natural history of MCD and suggests underlying FSGS.

Question 57

Hvordan vil man behandle diabetes nefropatien ved nefrotisk syndrom?

Answer 57

Question 58

Hvordan vil man behandle amyloid nefropati ved nefrotisk syndrom?

Answer 58

Question 59

Hvilke komplikasjoner kan oppstå pga. nefrotisk syndrom?

Answer 59

Trombotiske komplikasjoner

Atherosklerotiske komplikasjoner

Kronisk nyresvikt

Økt infeksjonsrisiko

Underernæring av proteiner

Vitamin D svikt

Anemi

Question 60

Hva kjennetegner de tromboliske komplikasjonene ved nefrotisk syndrom?

Answer 60

Renal vein thrombosis is most frequently associated with membranous nephropathy.

Question 61

Hvorfor er atherosklerose en komplikasjon ved nefrotisk syndrom?

Answer 61

Question 62

Hvilke bakenforliggende sykdommer har størst risiko for å gi KNS ved nefrotisk syndrom?

Answer 62

FSGS and membranous nephropathy in particular may progress to chronic kidney disease and ESRD (end-stage kidney disease).

Question 63

Hva er det fører til den økte infeksjonsrisikoen ved nefrotisk syndrom?

Answer 63

Question 64

Hva er det som gir underernæring av proteiner ved nefrotisk syndrom?

Answer 64

Loss in lean body mass due to proteinuria may be masked by weight gain caused by concurrent edema.

Question 65

Hva er det som gir vitamin D mangel ved nefrotisk syndrom?

Answer 65

Due to urinary loss of vitamin D binding protein (DBP) and bound 25-hydroxyvitamin D.

Can cause hypocalcemia → ↑ serum parathyroid hormone (PTH) → bone disease.

Question 66

Hva er det som fører til anemien ved nefrotisk syndrom?

Answer 66

Due to urinary loss or impaired synthesis of transferrin (causing hypochromic microcytic anemia), transcobalamin (causing megaloblastic anemia), copper (causing sideroblastic anemia), erythropoietin, and iron.

Question 67

Hvordan er prognosen ved nefrotisk syndrom?

Answer 67

The recovery rate is approximately 90% with glucocorticoid treatment.