308 - Glomerular Diseases (nephrotic)

Question 1

MCD= ___

Answer 1

minimal change disease

Question 2

MCD is usually in children (___%), and sometimes in adults (__%)

Answer 2

70-90

10-15

Question 3

MCD is usually ____ but can be ___ to Hodgkin’s lymphoma, allergy, NSAID… The pathophysiology has to do with ___ influencing the ___ barrier

Answer 3

primary
secondary
cytokines
glomerular

Question 4

MCD common clinical presentation include: acute ___, nephrotic syndrome ( with ___ deposition), proteinuria (>__g/24hr), and ___.

Answer 4

edema
acellular
10 g/24hr
hypoalbuminemia

Question 5

Less common clinical presentation of MCD include: (5)

Answer 5

HTN (more in adults)
microscopic hematuria
allergic symptoms 
renal dysfunction
IS nephritis

Question 6

Children with MCD usually have selective ___, mostly ___.

Answer 6

proteinuria

albumin

Question 7

Diagnosed MCD will require ___ if the patient does not respond to ____

Answer 7

biopsy

steroids

Question 8

Treatment for MCD includes- 1st line ____, 2nd ____

Answer 8

prednisone

cyclophosphamide/chlorambucil

Question 9

In MCD primary responders are patients who undergo full remission (___ mg/24hr) after a course of prednisone.

Answer 9

0.2

Question 10

In MCD steroid dependent patients are those with ___ during the ___ period from prednisone

Answer 10

relapses

tapering

Question 11

Frequent relapsers MCD patients are those who have had at least ___ relapses in __ months

Answer 11

2

6

Question 12

Steroid resistant MCD patients are those who do not respond to steroid treatment. Some of those are diagnosed with ___ in follow up biopsies

Answer 12

FSGS (Focal segmental glomerulosclerosis)

Question 13

90-95% of children with MCD go through ___ remission after ___ weeks of steroid treatment

Answer 13

full

8

Question 14

80-85% of ADULTS with MCD go through ___ remission after ___ weeks of steroid treatment

Answer 14

full

20-24

Question 15

FSGS is defined by ____ scarring that does not involve the entire ___, mostly in the ____ junction.

Answer 15

segmental
glomeruli
corticomedullary

Question 16

When performing FSGS biopsy which is not deep enough, it may not include all of the pathological findings, leading to a false diagnosis of ___

Answer 16

MCD

Question 17

FSGS may be secondary to ___, ___, ___, ___ (4)

Answer 17

HIV
HBV
SCD
reflux nephropathy

Question 18

The clinical presentation of FSGS may include: (3)

Answer 18

proteinuria
hematuria
HTN

Question 19

Treating FSGS include ____ inhibitors, and when patients are not in the nephrotic range- also ___

Answer 19

RAAS

steroids

Question 20

In secondary FSGS treatment is firstly directed for the ___ disease + ____. ____ should not be used

Answer 20

primary
proteinuria
steroids

Question 21

Negative prognostic signs in FSGS involve: (3)

Answer 21

nephrotic range proteinuria (>3.5 g/24 hours)
Afro-American
RF

Question 22

MGN=___

Answer 22

membranous glomerulonephritis

Question 23

What is the most common cause for nephrotic syndrome in the adult?

Answer 23

MGN- 30%

Question 24

MGN is more common in ___

Answer 24

men

Question 25

25-30% of MGN cases are secondary to: (3)

Answer 25

malignancy (lung/breast/colon) infection (HBV/malaria/schistosome) rheumatologic (SLE/RA)

Question 26

MGN biopsy will show uniform ___ of the GBM. We will also find diffuse granular depositions of ___ and ___

Answer 26

thickening C3 IgG

Question 27

The mechanism of primary MGN is probably due to the formation of ___ complexes in situ in reaction to ___ on the ___

Answer 27

immune antigens podocytes

Question 28

Clinical presentation of MGN includes: (4)

Answer 28

nephrotic syndrome nonselective proteinuria microscopic hematuria high frequency of thromboembolic complications

Question 29

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 ___

Answer 29

anticoagulant thromboembolic proteinuria bleeding

Question 30

Treating MGN is directed to solve: (3)

Answer 30

Edema dyslipidemia HTN

Question 31

Treating MGN includes the following drugs: (3)

Answer 31

RAAS inhibitors Immunosuppressive treatment (steroids/cyclophosphamide/chlorambucil) prophylactic anticoagulant treatment

Question 32

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

Answer 32

``` 1/3 1/3 nephrotic syndrome 1/3 RF death ```

Question 33

Negative prognostic factors in MGN include (4)

Answer 33

men old age HTN persistence proteinuria

Question 34

The main reason for diabetic nephropathy is ___ (45%), followed by ___(40%),

Answer 34

chronic renal failure | diabetes

Question 35

Risk factors for diabetic nephropathy include: (5)

Answer 35

``` hyperglycemia HTN dyslipidemia smoking family history ```

Question 36

The pathophysiology of diabetic nephropathy involves the loss of the ___ of the interglomerular ___, an increased production of ___ and glomerulosclerosis

Answer 36

negative charge GBM matrix

Question 37

In diabetic nephropathy immunofluorescences there is a non specific deposition of ___ and ___ with no sediments in the EM + vascular changes

Answer 37

IgG | complement

Question 38

How long until pathological signs appear in diabetic nephropathy?

Answer 38

1-2 years

Question 39

Clinical presentation of diabetic nephropathy include: (3)

Answer 39

microalbuminuria (30-300 mg/24hr) < 5-10 years albuminuria (>300 mg/24hr) > 5-10 years retinopathy (Kimmelstiel - Wilson nodules)

Question 40

Diagnosis of diabetic nephropathy is mostly based on the following: (3)

Answer 40

normal-enlarged kidneys diabetic retinopathy lack of clinical/serologic signs for a different diagnosis

Question 41

Treatment for diabetic nephropathy includes- monitoring ___ levels, controlling ___ (usually > 3 drugs), ___ inhibitors. DB1 will be initially treated with ___, while DB2 with ___ or ___

Answer 41

``` glucose HTN RAAS ACEi ACEi/ARB ```

Question 42

LCDD=___

Answer 42

light chain deposition disease

Question 43

In LCDD there is an increased secretion of ___ from ___ cells that can lead to deposition in the ___/___ sites.

Answer 43

light chains plasma cells tubular glomerular

Question 44

LCDD damages the kidney with cast ____- RF without substantial ___, secondary ___.

Answer 44

nephropathy proteinuria amyloidosis

Question 45

LCDD can also lead to ___ syndrome with ___ (__% of cases will advance to ___)

Answer 45

nephrotic RF 70 dialysis

Question 46

We can diagnose LCDD with either ___ or ___

Answer 46

IF- light chain Ab deposition | EM- granular deposition

Question 47

Treatment for LCDD includes treating the ___ disease, and if possible- autologous ____ transplantation

Answer 47

primary | BM

Question 48

Renal amyloidosis can be __ or ___.

Answer 48

primary- AL | secondary- AA

Question 49

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 ___.

Answer 49

``` light chains lambda MM 30% dialysis ```

Question 50

Secondary renal amyloidosis common disease include: (5). It is not uncommon to see nephrotic syndrome in theses patients. 50% will reach dialysis

Answer 50

``` RA (40%) psoriasis SLE FMF TB ```

Question 51

In both forms of renal amyloidosis we will have ___, severe___, renal vein ___, heart with reactive ____, PNS with ___ syndrome, and macroglossia.

Answer 51

``` hepatomegaly proteinuria thrombosis cardiomyopathy carpal tunnel ```

Question 52

We can diagnose renal amyloidosis with ___.

Answer 52

biopsy

Question 53

Treatment for renal amyloidosis includes- primary: ___+___, secondary: treating the ___ disease

Answer 53

melphalan autologous BM transplantation primary

Question 54

Pulmonary renal syndrome is mainly caused by: (5)

Answer 54

``` Goodpasture's syndrome Wegner's microscopic polyangiitis Churg Strauss HSP ```

Question 55

Treatment for pulmonary renal syndrome includes: empiric ___ + ___ - before lab results are back.

Answer 55

plasmapheresis | methyl prednisone

Question 56

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

Answer 56

``` IV GBM lungs testis cochlea eyes ```

Question 57

Remember anti GBM disease: we will find Ab against ____ of collagen __. We might see pulmonary renal syndrome (___) together with RPGN

Answer 57

Alpha 3 NC1 IV Goodpasture's

Question 58

Name 4 basement membrane syndrome diseases:

Answer 58

Anti GBM disease Alport's syndrome (X-linked) thin membrane disease Nail patella syndrome

Question 59

Glomerular vascular syndrome involves ___ damage to the capillaries of the ___. Most of the processes are part of a systemic damage.

Answer 59

vascular | glomeruli

Question 60

Name 5 glomerular vascular syndrome disease- (5)

Answer 60

``` atherosclerosis nephropathy hypertensive nephropathy cholesterol emboli sickle cell disease Thrombotic microangiopathies (HUS/TTP) ```