308 - Glomerular Diseases (nephrotic) Flashcards
MCD= ___
minimal change disease
MCD is usually in children (___%), and sometimes in adults (__%)
70-90
10-15
MCD is usually ____ but can be ___ to Hodgkin’s lymphoma, allergy, NSAID… The pathophysiology has to do with ___ influencing the ___ barrier
primary
secondary
cytokines
glomerular
MCD common clinical presentation include: acute ___, nephrotic syndrome ( with ___ deposition), proteinuria (>__g/24hr), and ___.
edema
acellular
10 g/24hr
hypoalbuminemia
Less common clinical presentation of MCD include: (5)
HTN (more in adults) microscopic hematuria allergic symptoms renal dysfunction IS nephritis
Children with MCD usually have selective ___, mostly ___.
proteinuria
albumin
Diagnosed MCD will require ___ if the patient does not respond to ____
biopsy
steroids
Treatment for MCD includes- 1st line ____, 2nd ____
prednisone
cyclophosphamide/chlorambucil
In MCD primary responders are patients who undergo full remission (___ mg/24hr) after a course of prednisone.
0.2
In MCD steroid dependent patients are those with ___ during the ___ period from prednisone
relapses
tapering
Frequent relapsers MCD patients are those who have had at least ___ relapses in __ months
2
6
Steroid resistant MCD patients are those who do not respond to steroid treatment. Some of those are diagnosed with ___ in follow up biopsies
FSGS (Focal segmental glomerulosclerosis)
90-95% of children with MCD go through ___ remission after ___ weeks of steroid treatment
full
8
80-85% of ADULTS with MCD go through ___ remission after ___ weeks of steroid treatment
full
20-24
FSGS is defined by ____ scarring that does not involve the entire ___, mostly in the ____ junction.
segmental
glomeruli
corticomedullary
When performing FSGS biopsy which is not deep enough, it may not include all of the pathological findings, leading to a false diagnosis of ___
MCD
FSGS may be secondary to ___, ___, ___, ___ (4)
HIV
HBV
SCD
reflux nephropathy
The clinical presentation of FSGS may include: (3)
proteinuria
hematuria
HTN
Treating FSGS include ____ inhibitors, and when patients are not in the nephrotic range- also ___
RAAS
steroids
In secondary FSGS treatment is firstly directed for the ___ disease + ____. ____ should not be used
primary
proteinuria
steroids
Negative prognostic signs in FSGS involve: (3)
nephrotic range proteinuria (>3.5 g/24 hours)
Afro-American
RF
MGN=___
membranous glomerulonephritis
What is the most common cause for nephrotic syndrome in the adult?
MGN- 30%
MGN is more common in ___
men
25-30% of MGN cases are secondary to: (3)
malignancy (lung/breast/colon)
infection (HBV/malaria/schistosome)
rheumatologic (SLE/RA)
MGN biopsy will show uniform ___ of the GBM. We will also find diffuse granular depositions of ___ and ___
thickening
C3
IgG
The mechanism of primary MGN is probably due to the formation of ___ complexes in situ in reaction to ___ on the ___
immune
antigens
podocytes
Clinical presentation of MGN includes: (4)
nephrotic syndrome
nonselective proteinuria
microscopic hematuria
high frequency of thromboembolic complications
It might be beneficial to treat MGN patients with prophylactic ___ treatment due higher risk of ___ events. Remember they must be with prolonged ___ and no risk for ___
anticoagulant
thromboembolic
proteinuria
bleeding
Treating MGN is directed to solve: (3)
Edema
dyslipidemia
HTN
Treating MGN includes the following drugs: (3)
RAAS inhibitors
Immunosuppressive treatment
(steroids/cyclophosphamide/chlorambucil)
prophylactic anticoagulant treatment
In MGN __ of the patients will go through spontaneous remission, ___ will suffer from relapses of ___ with no renal dysfunction, ___ will progress to ___ or ___ due to complications
1/3 1/3 nephrotic syndrome 1/3 RF death
Negative prognostic factors in MGN include (4)
men
old age
HTN
persistence proteinuria
The main reason for diabetic nephropathy is ___ (45%), followed by ___(40%),
chronic renal failure
diabetes
Risk factors for diabetic nephropathy include: (5)
hyperglycemia HTN dyslipidemia smoking family history
The pathophysiology of diabetic nephropathy involves the loss of the ___ of the interglomerular ___, an increased production of ___ and glomerulosclerosis
negative charge
GBM
matrix
In diabetic nephropathy immunofluorescences there is a non specific deposition of ___ and ___ with no sediments in the EM + vascular changes
IgG
complement
How long until pathological signs appear in diabetic nephropathy?
1-2 years
Clinical presentation of diabetic nephropathy include: (3)
microalbuminuria (30-300 mg/24hr) < 5-10 years
albuminuria (>300 mg/24hr) > 5-10 years
retinopathy (Kimmelstiel - Wilson nodules)
Diagnosis of diabetic nephropathy is mostly based on the following: (3)
normal-enlarged kidneys
diabetic retinopathy
lack of clinical/serologic signs for a different diagnosis
Treatment for diabetic nephropathy includes- monitoring ___ levels, controlling ___ (usually > 3 drugs), ___ inhibitors. DB1 will be initially treated with ___, while DB2 with ___ or ___
glucose HTN RAAS ACEi ACEi/ARB
LCDD=___
light chain deposition disease
In LCDD there is an increased secretion of ___ from ___ cells that can lead to deposition in the ___/___ sites.
light chains
plasma cells
tubular
glomerular
LCDD damages the kidney with cast ____- RF without substantial ___, secondary ___.
nephropathy
proteinuria
amyloidosis
LCDD can also lead to ___ syndrome with ___ (__% of cases will advance to ___)
nephrotic
RF
70
dialysis
We can diagnose LCDD with either ___ or ___
IF- light chain Ab deposition
EM- granular deposition
Treatment for LCDD includes treating the ___ disease, and if possible- autologous ____ transplantation
primary
BM
Renal amyloidosis can be __ or ___.
primary- AL
secondary- AA
Primary renal amyloidosis will present with ___ deposition (75% ___ ). In 10% __ will also be present with lytic bone fractures and BM infiltration (>__ plasma cells). 20% will continue to ___.
light chains lambda MM 30% dialysis
Secondary renal amyloidosis common disease include: (5). It is not uncommon to see nephrotic syndrome in theses patients. 50% will reach dialysis
RA (40%) psoriasis SLE FMF TB
In both forms of renal amyloidosis we will have ___, severe___, renal vein ___, heart with reactive ____, PNS with ___ syndrome, and macroglossia.
hepatomegaly proteinuria thrombosis cardiomyopathy carpal tunnel
We can diagnose renal amyloidosis with ___.
biopsy
Treatment for renal amyloidosis includes- primary: ___+___, secondary: treating the ___ disease
melphalan
autologous BM transplantation
primary
Pulmonary renal syndrome is mainly caused by: (5)
Goodpasture's syndrome Wegner's microscopic polyangiitis Churg Strauss HSP
Treatment for pulmonary renal syndrome includes: empiric ___ + ___ - before lab results are back.
plasmapheresis
methyl prednisone
Basement membrane syndrome has to do with collagen type ___, which is found in different tissues such as: (5). Damage to the GBM leads to medium proteinuria, hematuria, and progressive RF
IV GBM lungs testis cochlea eyes
Remember anti GBM disease: we will find Ab against ____ of collagen __. We might see pulmonary renal syndrome (___) together with RPGN
Alpha 3 NC1
IV
Goodpasture’s
Name 4 basement membrane syndrome diseases:
Anti GBM disease
Alport’s syndrome (X-linked)
thin membrane disease
Nail patella syndrome
Glomerular vascular syndrome involves ___ damage to the capillaries of the ___. Most of the processes are part of a systemic damage.
vascular
glomeruli
Name 5 glomerular vascular syndrome disease- (5)
atherosclerosis nephropathy hypertensive nephropathy cholesterol emboli sickle cell disease Thrombotic microangiopathies (HUS/TTP)
