Term
| What are the two major clinical presentations of glomerular disorders? |
|
Definition
1) proteinuria and nephrotic syndrome
2) hematuria and nephritic syndrome |
|
|
Term
| What is nephrotic syndrome characterized by and caused by? nephritic syndrome? |
|
Definition
1) proteinuria, which can range rom asymptomatic to massive with sequela of protein loss; visceral epithelial cell damage
2) hematuria which can range from asymptomatic to massive with sequela of rapid loss of glomerular function; inflammation of glomerulus w/ associated endothelial injury |
|
|
Term
| What protein type is the first to appear in the ultrafiltrate? What type of proteinuria is it with small molecular weight proteins only present? What is proteinuria w/ high molecular weight proteins? |
|
Definition
1) small molecular weight proteins (e.g. albumin)
2) selective proteinuria
3) non-selective proteinuria |
|
|
Term
| What is tubular type proteinuria? |
|
Definition
| 1) tubular damage with small quantities of albumin present in urine |
|
|
Term
| What are some of the mechanisms of injury to the glomerular epithelial cells (podocytes) leading to proteinuric state/ |
|
Definition
- circulating and filtered antibidies
- cytokines
- drugs
- deposition of amyloid
- deposition of collagenous matrix
- deposition of immune complexes
- defects in structural elements in epithelial cell components |
|
|
Term
| What is the most accurate test to screen for urinary protein? most common screening test (limitation?)? Other test? |
|
Definition
1) quantitative 24hr timed urine collection
2) dipstick test (does not detect small quantities of albumin that may be seen in early stages of kidney disease
3) urinary protein to urinary creatinine (close estimate of 24hr urinary protein |
|
|
Term
| What level of proteinuria is suggestive of kidney disease but not specific? diagnostic of glomerular disease? |
|
Definition
|
|
Term
| What are causes of transient proteinuria? |
|
Definition
1) exercise
2) febrile illnesses
3) hypertensive episodes
4) systemic inflammatory conditions |
|
|
Term
| What are the clinical features of nephrotic syndrome? |
|
Definition
1) normal kidney function
2) normal BP
3) urine sediment: noninflammatory |
|
|
Term
| What is the definition of nephrotic syndrome? |
|
Definition
1) proteinuria >3.5g/d
2) hypoalbuminemia w/ serum albumin <3.5g/dL
3) edema
4) hyperlipidemia and lipiduria |
|
|
Term
| What are common causes of morbidity with nephrotic syndrome? |
|
Definition
1) edemas and effusions (respiratory compomise)
2) infections (IgG and complement wasting)
3) thrombotic/thromboembolic events (AIII and plasminogen wasting)
4) endocrine dysfunction ( |
|
|
Term
| What are the most comon causes of primary glomerular NS? |
|
Definition
1) minimal change disease (MCD) - children
2) focal and segmental glomerulosclerosis (FSGS) - adults
3) Membranous nephropathy (MN) |
|
|
Term
Describe minimal change disease:
type
morphologic exam
epidemiology
prognosis w/ Tx
pathogenesis |
|
Definition
1) glomerular d/o w/ nephrotic syndrome
2) unremarkable glomeruli w/ diffuse and global visceral epithelial cell injury
3) 2-6yrso
4) good prognosis w/ steroids and sodium restriction
5) immune phenomena (URI, post-vavvination, atopic, hodgkin disease) |
|
|
Term
Describe focal segmental glomerulosclerosis (FSGS)
type
morphologic exam
epidemiology
prognosis w/ Tx
cause |
|
Definition
1) glomerular d/o w/ nephrotic range proteinuria and normal kidney function
2) segmental sclerosis of tuft
3) obesity; African americans/hispanics/HIV
4) low prognosis; no Tx (not steroid-responsive); recurs in transplanted kidneys
5) caused by primary (unknown) epithelial injury or secondary (known) epithelial injury |
|
|
Term
| Mutations in which podocyte proteins have been implicated in the development of FSGS? |
|
Definition
|
|
Term
Describe membranous nephropathy
type
morphologically
epidemiology
prognosis w/ Tx
cause |
|
Definition
1) glomerular d/o w/ nephrotic range proteinuria and nephrotic syndrome
2) diffuse and global thickening of glomerular capillary wall (immune complexes; no inflammation)
3) most common cause of NS in white older adults
4) chronic disease; no Tx; nonresponsive to steroiids
5) 80% idiopathic; autoimmune/collagen vascular d/o; chronic infections; sarcoidosis; medications |
|
|
Term
| What are the most common causes of NS resulting from secondary glomerular involvement by a systemic d/o? |
|
Definition
1) diabetes
2) amyloidosis
3) systemic lupus erythematosus |
|
|
Term
| What are drugs that commonly cause nephrotic syndrome? |
|
Definition
1) NSAIDs
2) antibiotics
3) interferon
4) lithium |
|
|
Term
| Describe diabetic nephropathy |
|
Definition
- related to hyperglycemia w/ non-enzymatic glycosylation fo glomerular proteins
- changes in biochemical properties and charge of GBM
- increased synthesis of TIV collagen w/ dec synth of heparan sylfate
- affects entire kidney
- progressino from microalbuminuria to significant proteinuria; loss of GFR; dialysis and kidney transplant
- Tx w/ glycemic control and ACE-I |
|
|
Term
|
Definition
- extracellular deposition of protein in beta-pleated sheets
- AL amyloidosis (Ig) - neoplasm; AA amyloidosis (serum amyloid A-protein) - liver inflammation/familial mediterranean fever
- NO TX except underlying condition |
|
|
Term
| What are the three characteristic findings in amyloidosis of the kidney w/ NS? |
|
Definition
1) positive congo red stain
2) apple green birefringence under polarized light
3) evidence of random non-branching fibrils up to 10nm |
|
|
