29. Chronic glomerulonephritis. Nephrotic syndrome. Flashcards
what are the chronic glomerulonephritis in children
IgA glomerulonephritis - berger disease - iga nephropathy - MOST COMMON and chronic GN in children
(can be nephritic)
membranoproliferative glomerulonephritis
(can be nephritic)
membranous nephropathy
(Most common cause of nephrotic syndrome in adults of Europea)
minimal change disease - MOST COMMON CAUSE OF NEPHROTIC SYNDROME IN CHILDREN!!
foal segmental glomerulosclerosis
(Most common cause of nephrotic syndrome in adults, especially in African American )
idiopathic nephrotic syndrome
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Secondary: Diabetic nephropathy ,
lupus ,
vasculitis(henoch-schonlein purpura)
what is the definition of nephrotic syndrome ?
proteinurea 40 mg/m2/hr
PLUS HYPOALBUMINEMIA AND EDEMA
what is the pathophysiology of nephrotic syndrome ?
loss of negative charge in glomerular basement membrane
podocyte damage and fusion - non selective porteinurea - except in minimal change disease manifest with selective glomerular proteinuria
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
what are the typical clinical features of nephritic syndrome ?
decrease in osmotic pressure - leading to PITTING edema
starts in the periorbital area/ scrotum , labia
lung
pleural edema
ascitis
(face = can be mistaken for allergy)
weight gain , lower metabolic rate , muscle stiffness = loss of thyroglobulin transport protein leading to thyroxine deficient - hypothyroidism
vit D deficicny = hypocalcemia = tetany , paresthesia and muscle spasms
Hypercoagulable state with increased risk of thrombosis and embolic events (e.g., pulmonary embolism, renal vein thrombosis)
Increased susceptibility to infection
Hypertension in some cases
frothy urine - protein high
lipid urea - fatty casts
diagnosis of nephrotic syndrome
1)nephrotic range proteinurea
40 mg/m2/hr
3 urine dipstick tests
2) hypoalbuminemia - serum albumin
<3.0 g/dL
3) hyperlipidemia
cholesterol >250 mg/dL
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first-morning urine protein/creatinine ratio of 2-3 mg/mg or more indicates nephrotic-range proteinuria
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anuria/oliguria due to ↓GFR → Azotemia
general treatment of nephrotic syndrome
dietary sodium restriction
fluid restriction
diuretic therapy first line - loop diuretics - furosemide second line - oral thiazide can add a potassium sparing diuretic - spironolactone
IV albumin
avoid very high protein intake
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antiproteinuric therapy
RAAS - ACEi - ramipril
ARB - losartan
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lipid lowering therapy
atorvastatin
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hypercogubility
infraction heparin
low molecular heparin
or oral warfarin
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infectious risk - prevention
pneumococcal vaccination
anualvaccination for influenza
complications nephrotic syndrome ?
thrombi - renal vein
chronic kidney disease - especially membranous nephropathy
and focal segmental glomerulosclerosis
atherosclerotic complication - high laid profile
what i the treatment of minimal change disease ?
for nephrotic syndrome treatmnet
Prednisone : oral
60mg/m2/day = 4 weeks
40mg/m2/48 hr = for further weeks
steroids for 12 weeks, but porteinurea should normalise for the first 4 weeks
diagnosis of minimal change disease ?
never leads to renal insuffiency
nephrotic syndrome =SELECTIVE ALBUMINUREA WITHOUT evidence of hypertension and MACROSCOPIC hematuria - the only one with selective
urine dipstick
usually renal biopsy is only taken with there is no response to steroids
renal function is preserved
no changes when viewing kidney biopsy specimens under light microscopy,
Under immunofluorescence, there are no immunoglobulins or complement deposits
= hence minimal change disease
electron microscopy =
hallmarks of the disease were discovered.
diffuse loss of visceral epithelial cells foot processes podocyte effacement,
what is the treatment of minimal change disease ?
for nephrotic syndrome treatmnet
Prednisone : oral
60mg/m2/day = 4 weeks
40mg/m2/48 hr = for further weeks
steroids for 12 weeks, but porteinurea should normalise for the first 4 weeks
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if relapse on prednisolone - cyclophosphamide 168mg/kg
cyclosporin . tacrolimus . mycophenolate
what is the etiology for membranous GN?
immune complex mediated !
primary
anti - PLA2R antibodies
secondary HBV, HCV , MALARIA strep SLE / RA lung or prostate cancer NSAIDS / captopril
diagnosis of membranous GN?
serum c3 are usually low esp in case of viral hep c
LM LM :
1) diffuse thickening of basement membrane
GBM appears to be spiked and holey appearance appearance - Swiss cheese).
em : spike and dome pattern of basement membrane
IF - Granular subepithelial deposits of IgG and C3 (dense deposits)
spike and dome
what is the treatment for membranous nephropathy
Children with MN can be divided into two groups based on their clinical presentation
Children with asymptomatic, nonnephrotic proteinuria. These children typically present with no edema, normal serum albumin, and a urine protein to creatinine ratio between 0.2 and 2
managed conservatively with ACEI and ARB
lipid-lowering agents, particularly statins
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otherwise
prednisone 4-8 weeks
if steroid resistant - which most are they do not respond well
cyclophosphamide 2mg/kg/day 12 weeks alternate with steroids
if refectory - considerr calcinurinen inhibitors such as cyclosporin and tacrlimus
Mycophenolate mofetil
Rituximab
what is the etiology of focal segmental sclerosis ?
idiopathic
Circulating Permeability Factor-
FSCG rapidly occurs in graft of renal transplant , however if plasmaphoresis is done before transplant the disease is limited
herion
HIV / CMV/ hep C
sickle cell
congenital malformation - charcot marie tooth - hereditary motor sensory neuropathy - distal leg weakness, foot deformities, and sensory deficits
