3 Nephritis and nephrotic syndrome Flashcards Preview

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Flashcards in 3 Nephritis and nephrotic syndrome Deck (22)
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1

what is nephrotic syndrome characterised by ?

maassive proteinurea >3.5g a day
and hypoalbuminemia
and edema

2

what is the pathophysiology of nephritic syndrome

loss of negative charge in glomerular basement membrane = loss of selective barrier esp negatively charged albumin

podocyte damage and fusion - non selective porteinurea - except in minimal change disease manifest with selective glomerular proteinuria

3

what is a compensatory mechanism to the protein loss we see in nephrotic syndrome and also useful for the diagnosis of nephrotic syndrome

plasma levels of cholesterol , LDL , triglycerides and lipoproteins increase

4

what are the typical clinical features of nephritic syndrome ?

decrease in osmotic pressure - leading to PITTING edema
starts in the periorbital
lung
pleural edema
ascitis


weight gain , lower metabolic rate , muscle stiffness = loss of thyroglobulin transport protein leading to thyroxine deficient

vit D deficicny = hypocalcemia = tetany , paresthesia and muscle spasms

symptoms of underlying disease

frothy urine - protein high

lipid urea - fatty casts

5

what do nephrotic syndrome people have a higher risk for ? and why ?

higher risk for hypercogulability

because anti thrombin 3 , protein C and S = will cause hypercoaguble site

= pulmonary embolism = dyspnea

rena vein thrombosis
=======

higher risk for infection because loss of immunoglobulins

6

what are the initial evaluation we do for nephrotic syndrome ?

CONFIRM NEPHROTIC RANGE PROTEINUREA

qualitative
urine dipstick

hematurea indicate concomitant glomerulonephritis

quantitative
24hr urine protein
pot urin protein / creatnine ratio

===========
Urine sediment microscopy

Nephrotic sediment :
Lipiduria, fatty casts with Maltese cross appearance under polarized light

Renal tubular epithelial cell casts

microscopic Hematuria with acanthocytes
and/or
RBC casts may indicate concomitant glomerulonephritis

===========

additional lab
hb/ hct high - indicate hemoconcentration

creatinine high

SODIUM COMMONLY LOW (RASS AND ADH)- HYPERVOLEMIC HYPONATREMIA
= sometimes lead to hypertension

coagulation factors - fibrinogen and d dimer is high

vit D levels

=========
renal ultrasound

7

what diseases are associated with nephrotic syndrome ?

minimal change disease / lipid nephrosis

focal segmental glomerulosclerosis

membranous glomerulonephritis

diabetic nephropathy = secondary

amyloid nephropathy = secondary

lupus nephritis = secondary

membranoproliferative glomerulonephritis

8

what is the definition of nephritic syndrome ?

inflammatory process that is defined as the presence of one or more of the following

macroscopic hematuria with acanthocytes

RBC casts in urine

proteinurea but less than 3.5g a day

hypertension

mild to moderate edema

sterile pyuria

oliguria

azotemia

9

what is the pathophysiology of nephritis syndrome ?

usually due to inflammation of the glomerulus

1) inflammation leading to cytokine release - white blood cells accumulate in the bowman capsule

the glomerular basement membrane is also porous - leaking protein and RBC

oliguria is because the inflammatory infiltrates in the bowman capsule and the capillaries red fluid across the membrane reduction in GFR

there is SALT retention - leading to intravascular volume expansion = hypertension and edema

2) membranoproliferazive glomerulonephritis

depostion of antibodies between the podocytes and basement membrane

type 1 : sub endothelial immune complex deposition

type 2 (dense deposit disease) : intramembranous deposition of complement C3
C3 nephritic factor - autoantibody which stabilises C3 converts - leading to continuous activation and depletion of C3

10

what are the clinical features of nephritic syndrome ?

intermittent hematurea - red/ brown coloured urine

hypertension

pitting edema

decrease gfr and oligourea leading to uremic symptoms :
= fatigue , weakness , loss of appetite ,
uremic fector
pruritus
uremic pericarditis
encephalopathy - seizures and coma
parethesia

11

how do we diagnose nephritic syndrome ?

hematuria
ancanthocytes
RBC casts
mild to moderate porteinurea
sterile pyuria - sometimes abc casts

blood test
creatinine high
azotemia with BUN high
ANA , ANCA and anti-GBM pantibodies

renal biopsy

12

how is non glomerular hematurea characterised ?

BRIGHT RED
occurrence of blood clots
normal RBC morphology
ABCENCE OF RBC CASTS

13

what are the diseases associated with nephritic syndrome ?

post strep glomerulonephritis

IgA nephropathy - berger disease

small vessel vasculitis
-wegner granulomatosis
-microscopic polyangitis
-eosinophilic granulomatosis with polyangitis
(charge strauss syndrome)

good pasture syndrome

thin basement membrane hematurea

alport syndrome

diffusive proliferative glomerulonephritis

rapidly progressive glomerulonephritis

membranoproliferazive glomerulonephritis

14

what is the etiology of ALPORT SYNDROME ?

x linked recessive inheritance = therefore more severe in males

sometimes autosomal recessive

15

what is the pathophysiology of aport syndrome ?

genetic defect in type 4 collagen - which is a component of the basement membrane of the kidneys , eyes , and chochlea

16

what are the clinical features of aport syndrome ?

age of onset day from infancy to late adulthood

often asymtomatic

intermittent macroscopic hematurea

SENSORINEURAL HEARING LOSS - high freq

ocular 0 anterior lenticonus , retinopathy

as glomerular damage progresses - nephritic syndrome and chronic kidney disease and end stage renal disease

(cant pee , cant see , cant hear a bee)

17

what is the confirmatory test of aport syndrome other than nephritic syndrome diagnostics ?

skin biopsy - absence if collagen 4 alpha 5 chants

======

kidney biopsy
LM = mesangial cell proliferation and sclerosis

EM = splitting and thickening of the glomerular basement membrane - basket weave appearance

immunostaining - absence of type 4 collagen in basement membrane

======

molecular genetic testing

18

what is the treatment for aport syndrome ?

proteinurea - ACE inhibitors / arb

chronic kidney disease treatment )sodium restriction and diuretics)

hearing aid

ocular surgery

the only definitive treatment is kidney transplant

19

what is a complication of aport syndrome after kidney transplant ?

good pasture disease can occur due to newly developed collage type 4 antigens

20

what is the treatment of nephrotic syndrome ?

Edema

Dietary sodium restriction

Fluid restriction: < 1.5 liters/day

Diuretic therapy

High diuretic doses often required because of diuretic resistance.

First-line: oral loop diuretic

Second-line:
Add oral thiazide
OR switch to IV loop diuretic

Consider adjunctive IV albumin 30–60 minutes prior to intravenous loop diuretic.

================

Proteinuria
Elimination or reduction of proteinuria is a major treatment goal for nephrotic syndrome and lead to increased serum albumin, decreased edema, hyperlipidemis, reduction in risk of thromboembolism and infection, and slowing of the progression of chronic kidney disease.


RAAS inhibitor: ACEI or ARB are commonly used.

Effects
Reduces proteinuria
Treats hypertension

Avoid in patients with AKI, hyperkalemia, or abrupt onset of nephrotic syndrome.


Other measures that may be beneficial in combination with an ACEI or ARB:

Thiazide diuretic (e.g., hydrochlorothiazide )

Mineralocorticoid receptor antagonist (e.g., spironolactone )

Dietary protein: avoid very high-protein diet but ensure adequate protein intake (0.8–1.0 g/kg daily).

=============

Dyslipidemia

atorvastatin

========

Hypercoagulability

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.


The following parameters should be taken into account to decide whether prophylactic anticoagulation is indicated:

Serum albumin level: when serum albumin is < 2–2.5 g/dL.

Histological diagnosis: VTE risk is higher in primary membranous nephropathy

Immobility due to edema, obesity, malignancy,

thrombophilic state

Previous history of thromboembolic events

Proteinuria > 10 g/day

Body mass index > 35 kg/m2

Recent abdominal or orthopedic surgery

New York Heart Association class III to IV congestive heart failure


Options
Unfractionated heparin

Low molecular weight heparin

Oral warfarin (target INR 2.0–3.0)

=============

Infectious risk

Screening: Consider screening for hepatitis B,
HIV,
latent TB,
syphilis

Preventive measures :
Pneumococcal vaccination
Annual vaccination for influenza

Consider monthly IV immunoglobulin in patients with repeated bacterial infections and serum IgG < 600 mg/dL.

Consider pneumocystis pneumonia prophylaxis (e.g., trimethoprim-sulfamethoxazole ) in patients receiving high-dose steroids and/or other immunosuppressive therapy

21

what re the complications of nephrotic syndrome ?

Venous thromboembolism deep vein thrombosis, pulmonary embolism

Arterial thromboembolism

Renal vein thrombosis:
Cause: hypercoagulable state (e.g., malignancies, antiphospholipid syndrome, nephrotic syndrome)

Manifestations: flank pain, anuria


Atherosclerotic complications


Chronic kidney disease: FSGS and membranous nephropathy in particular may progress to chronic kidney disease and ESRD.

22

treatment of nephritic syndrome :

Supportive therapy
Low-sodium diet
Water restriction

Medical therapy
If proteinuria and/or hypertension: angiotensin-converting enzyme inhibitors or angiotensin-receptor blockers

If severe hypertension and/or edema: diuretics