Nephrotic Syndrome Flashcards
Nephrotic syndrome can be a mode of presentation of _______ diseases.
glomerular
Nephrotic syndrome
A Clinical syndrome that arises when the ___________ is sufficient to produce __________
urinary protein excretion
hypoproteinaemic oedema.
Functional anatomy of the kidney
A (Simple or complex?)
(Unilateral or bilateral?)
____peritoneal organ
Complex
bilateral
retro
Functional anatomy of the kidney
involved in:
______ of water & solute
Regulation of —— and ____
concentration
Maintenance of ______ balance
Serve as ________ organ
Excretion
salt & water
acid base
an endocrine
Each kidney weighs about ________
The functional unit is the ______
150g
nephron
kidney’ nephron is divided into:
The ______
The ______
The ______
The ______
glomerulus
tubules
interstitium
blood vessels
GLOMERULUS
Consists anastomosing network of ______
capillaries
GLOMERULUS
lined by _________ —————
fenestrated endothelium
GLOMERULUS
The _______ epithelium (separated from the endothelial cells by the _______)
visceral
Glomerular basement membrane
GLOMERULUS
The ______ epithelium (situated on the ____________ & lines the _________)
parietal
Bowman’s capsule
urinary space
The filtration barrier consists of:
Thin layer of ______________
___________
_________
fenestrated endothelial cells
Glomerular basement membrane(GBM)
The podocytes
The Glomerular basement membrane(GBM)
lamina rara _____
lamina rara ______
lamina rara ______
densa
interna
externa
This filtration barrier function by discriminating against:
The _____ of the filtrate
The _____
size
Charge
This filtration barrier function by discriminating against:
The charge, accounted for by
__________ of the capillary wall
The _____ and _______ structure of the GBM
The many ________ present within the wall
Complex structure
collagenous porous & charged
anionic moieties
GBM consists of :
________
_______
_________
_________
type IV collagen
laminin
heparan sulphate
fibronectin
Type ___ collagen in ____ forms a network of structures to which _______
IV
GBM
other proteins attach.
________________
Aka heparan sulphate
polyanionic proteoglycan
Filtration barrier
The _____ serve as antigens & their genetic defects underlie some forms of _______.
glycoproteins
nephritis
The filtration barrier
The visceral epithelial cells(_____) are structurally (simple or complex?) with interdigitations embeded on _______
podocytes
Complex
lamina rara externa
The filtration barrier
Adjacent foot processes forms _________ bridged by ____________.
filtration slits
thin diaphragm
Mechanisms of glomerular injury
Pathogenesis linked to:
Genetic mutation –______ type mutation of ______; _____ syndrome (________ mutation)
Systemic _________ and ________
finnish; podocin
Alport; type 4 collagen
hypertension & atherosclerosis
Mechanisms of glomerular injury
Pathogenesis linked to:
Diabetes mellitus
__________ /________ reaction
Local activation of ________, etc
Hypersensitivity/ auto immune
T cell-like receptors
Nephrotic syndrome is a (specific or non-specific?) disorder in which the _____ are damaged, causing them to ________
non-specific
kidneys
leak large amounts of protein.
Nephrotic syndrome is 15 times more common in (children or adults ?) than in (children or adults?)
Children
Adults
Nephrotic syndrome
______ and ______ have higher incidence compared to ______
.
Hispanics and Africans
Caucasians
CRITERIA for nephrotic syndrome
Proteinuria (>___g/day)
Hypoalbuminemia (< ___g/L)
___________
_________
_____________ state
3
25
Hyperlipidemia
Oedema
Hypercoagulation
PATHOPHYSIOLOGY of nephrotic syndrome
Filtration of (low or high ?) molecular weight (cationic or anionic?) plasma proteins across the glomerular basement membrane is normally prevented by a _______ charged ________ of ____________ of _______
Low
anionic
negatively charged filtration barrier
proteoglycan molecules of heparan sulfate.
PROTEINURIA in nephrotic syndrome
____eased charge selective properties of GBM
Altered ______ of foot processes of podocytes
Decr
slit diaphragm
PROTEINURIA in nephrotic syndrome
Index of severity of glomerular lesion
Early (______)
-_______ (65kDa)
-_________
-_________ (80kDa)
Late (_______)
–____ (160kDa)
Selective
Albumin
Alpha 1-antitrypsin
Transferrin
Non-selective; IgG
HYPOALBUMINAEMIA in nephrotic syndrome
___ loss of albumin
increased ______ of albumin
urinary
catabolism
OEDEMA in nephrotic syndrome
Classical, due to reduced ________
Accumulation of ↑____+ in interstitial compartment and urine Na+ output.
oncotic pressure
Na
OEDEMA in nephrotic syndrome
Accumulation of ↑Na+ in interstitial compartment and urine Na+ output. Evidence of this –
(1)__________ observed even before fall in the __________
(2) intravascular volume is _____________ in most patients with nephrotic syndrome
Na+ retention; serum albumin
normal or even increased
Oedema in nephrotic syndrome
Starling forces are _____ in nephrotic syndrome until ____ in the disease because ____________ remains equivalent to _________
unchanged
late
interstitial fluid HP
plasma OP
Oedema in nephrotic syndrome
sites of renal sodium retention are predominantly in the (proximal or distal?) nephron, not in the (proximal or distal?) nephron, as is expected by changes in _______
Distal
Proximal
Starling forces
HYPER-CHOLESTEROLAEMIA in Nephrotic syndrome
____________ stimulating protein synthesis in the liver, including the ______
Diminution of _________ caused by reduced plasma levels of _______
hypoproteinemia
lipoproteins
lipid catabolism
lipoprotein lipase
HYPER-CHOLESTEROLAEMIA in nephrotic syndrome
Mild cases, mostly _________
Severe cases, ↑__________→ ____ plasma
——- casts in urine
Mild
VLDL (TGs) ; turbid
Fatty
Aetiologic classification of nephrotic syndrome
Can either be ______ or _____
Congenital
Acquired
Aetiologic classification of nephrotic syndrome
Congenital:
-Congenital nephrotic syndrome of the _______ type. Autosomal recessive.
-Gene responsible for this disease entity identified (ie, ____).
Finnish; NPHS1
Aetiologic classification of nephrotic syndrome
Acquired:
•Primary Causes: _______ aetiologies
•_____disease (e.g. SLE, rheumatoid arthritis, polyarteritis nodosa)
Post-infectious
Collagen vascular
Histologic Classification of nephrotic syndrome
-__________
_——————
___________
___________
Minimal Change Disease (MCD) I
Membranous Nephropathy (MN)
Focal Segmental Glomerulosclerosis (FSGS)
- Diffuse Mesangial Hypercellularity
Most common histologicak classification of nephrotic disease is ???
Minimal Change Disease (MCD):
Histologic Classification of nephrotic syndrome
- Minimal Change Disease (MCD): ____% of (children or adults ?)
- Membranous Nephropathy (MN)-(children or adults?)
85
children
adults
CLINICAL FEATURES of nephrotic syndrome
- The most common sign is ______
-______ urine, due to a lowering of the _________ by the severe ______
oedema:
Foamy
surface tension
proteinuria
CLINICAL FEATURES of nephrotic syndrome
oedema:
_______ oedema
_———— oedema
______ effusion and/or pulmonary ______.
-________
Orbital
Pitting pedal
Pleural; edema
Ascites
Other clinical features of nephrotic syndrome
•hypertension is common
T/F
F
Rare
Other clinical features of nephrotic syndrome
•_________ and _______ are uncommon and are seen commonly in nephritic syndrome.
Haematuria or oliguria
In nephrotic syndrome , there is _____ease of Protein-bound substances eg calcium, thyroxine, cortisol, iron
Decrease
TREATMENT of nephrotic syndrome
General measures (supportive)
-monitoring __________ and _____ regularly
-fluid _______
-________(IV _______)
fluid input/output
BP
restriction
diuretics; furosemide
TREATMENT of nephrotic syndrome
Monitoring kidney function: -_____ daily and calculating _____
Prevent and treat any complications
EUCs(electrolytes, urea, creatinine)
GFR
TREATMENT of nephrotic syndrome
Albumin infusions are generally not used because ______________
their effect lasts only transiently.
TREATMENT of nephrotic syndrome
Prophylactic ________
Reduce _____ intake to 1000-2000 mg daily
Moderate amount of ___________food
Increase ________ fat intake
anticoagulation
sodium
high protein animal
unsaturated
Specific treatment of underlying cause
__________ for the glomerulonephritides
Steroids: ______
Immunosupression
prednisolone
Specific treatment of underlying cause
Relapses by prednisolone __mg/kg/day till urine is ____tive for protein. Then, ___mg/kg/day for ____
2nd line drugs (frequent relapse): ______,_______, or ______
2
Negative
1.5; 4weeks
cyclophosphamide, nitrogen mustard or cyclosporin
Specific treatment of underlying cause
Stricter ______ control if diabetic.
Blood pressure control: ________
blood glucose
ACE inhibitors
COMPLICATIONS of nephrotic syndrome
Venous thrombosis: due to ____ of _____ and ______ and the simultaneous increase in ______, especially ___,____,___,____
leak
anti- thrombin 3 and plasminogen
clotting factors
I, VII, VIII, X
COMPLICATIONS of nephrotic syndrome
Infections: encapsulated bacteria such as. _______ and ______
__________ failure
Pulmonary ____
Haemophilus influenzae and Streptococcus pneumonia
Acute renal
edema
PROGNOSIS of nephrotic syndrome
Depends on ______
Minimal change disease has (better or worse?) prognosis due to ________
FSGN often leads to _____
Other factors: level of _____, _____ and _______
cause
Better ; response to steroids
ESRD
proteinuria; B.P control ; GFR
COMPLICATIONS OF nephrotic syndrome
Growth retardation: does not occur in ______. Seen in cases of ___________ or __________.
From ______, _____ (reduced protein intake), and ______ therapy (catabolism).
MCNS
relapses or resistance to therapy
proteinuria
anorexia; steroid
COMPLICATIONS OF nephrotic syndrome
Vitamin D–binding protein deficiency causing
(1) malabsorption of ______ and development of _____ disease (eg, __________________)
(2) ________ because of impairment in mineralization
Vitamin D deficiency can occur.
calcium; bone; osteitis fibrosa cystica
osteomalacia
COMPLICATIONS OF nephrotic syndrome
Thyroxine is reduced due to ________
____cytic ______chromic anaemia: Fe-therapy (sensitive or resistant?) .
decreased TBG.
Micro; hypo
resistant
