Pathology: Kidney 3 Flashcards

Question

HTN prevelance in DM pts with DN

Answer 1

In DM pts with DN, HTN is almost always present

Answer 2

* more rapid progression of DN * increased risk of fatal and nonfatal CV events. * antihypertensive therapies improve survival in both type 1 and type 2 diabetics with DN.

Answer 3

is below 140/90 mm Hg | • Was 130/80 before JNC 8

Answer 4

•Studies suggest that with good glycemic control helps • Decrease risk of progression •Results from the Diabetes Control and Complications Trial (DCCT) • reduction in progression from normoalbuminuria to microalbuminuria • Decrease in and other microvascular complications, specifically retinopathy • Decrease in cardiovascular (CV) sequelae, this persisted despite later deterioration of the glycemic control. •Euglycemia that followed isolated transplantation of the pancreas was associated with a regression of the diabetic glomerulosclerosis. (N Engl J Med 1998; 339:69-75)

Answer 5

* Renin-angiotensin-aldosterone system blockade * Renoprotective independent of BP * Reduces proteinuria * May cause up to 30% decline in GFR but renoprotective in the longterm * Works through renal hemodynamic changes and blocking non-hemodyanmic effects of Ang II

Answer 6

``` • Most patients with DN have: – Low HDL – High TGs – Smaller LDL particle • In type 2 diabetic patients with DN, treatment with statins provides substantial CV benefit – Not seen in DN with ESRD • Current guidelines – LDL <70 mg/dl for diabetic patients with CVD ```

Answer 7

• Smoking cessation – Decreases progression of micro to macro albuminuria • weight reduction – Possibly improves renal outcome via reduction in proteinuria

Answer 8

Amyloidosis | Light chain deposition disease

Answer 9

• Amyloidosis is a generic term for a family of diseases defined by morphologic criteria. – Characterized by the deposition in extracellular spaces of a proteinaceous material

Answer 10

– light chains, secreted by a single clone of B cells • 20% of cases associated with multiple myeloma – Usually lambda light chains (AL) – Systemic amyloidosis (AA) results from chronic inflammation • Not discussed

Answer 11

• kidney is often enlarged and hypertension is absent even when renal function is impaired. • Proteinuria, mainly albuminuria, occurs in the absence of microscopic hematuria. • Tubular defects from amyloid deposits – Renal tubular acidosis (mostly as a part of Fanconi syndrome) – polyuria-polydipsia (resulting from urinary concentration defect)

Answer 12

deposits seen

Answer 13

apple green birefringence

Answer 14

staining for light chain

Answer 15

• AL amyloidosis may infiltrate almost any organ other than the brain – Restrictive cardiomyopathy in 1/3 of pts – Gastrointestinal tract • motility disturbances, malabsorption, hemorrhage, or obstruction – Macroglossia – Splenomegaly – Peripheralnerve • sensory polyneuropathy, autonomic neuropathy(orthostatic hypotension, lack of sweating, bladder dysfunction, impotence) – Skin • Purpura (around the eyes) papules, nodules, and plaques, occurring usually on the face and upper trunk. – Joint • Shoulder pain and swelling

Answer 16

• Deposition of excess immunoglobulin light chains in the kidney – usually  – 50% of cases coexist with multiple myeloma

Answer 17

– proteinuria – hematuria – chronic renal insufficiency

Answer 18

nodular glomerulosclerosis

Answer 19

light chain staining | • Kappa staining

Answer 20

granular deposits along GBM

Answer 21

- Alport Syndrome | - Thin Basement Membrane

Answer 22

• Most commonly X-linked recessive (80%) but can be autosomal recessive as well • Mutation of the COL4A5 gene on chromosome Xq22 which encodes the alpha5 chain of type IV collagen – defect in the basement membrane

Answer 23

• Hematuria – Affected males have persistent microscopic hematuria – episodicgrosshematuria,precipitatedbyURI – Present in the first two decades of life. – More than 90% of females with XLAS have persistent or intermittent microscopic hematuria, but about 7% of obligate heterozygotes never manifest hematuria • Proteinuria is absent early but develops eventually in all males with XLAS and in both males and females with ARAS. • Hypertension • ESRD develops in all affected males with XLAS (90% by age 40) –rate determined by the underlying COL4A5 mutation. – Females with XLAS 12% developed ESRD before the age of 40, 30% by age 60 years, 40% by age 80 years

Answer 24

• Cochlear defects – adherence defect of the organ of Corti to the basilar membrane – 80% of males – 20-30% of female • Ocular defects – 30% to 40% of XLAS males – 15% of XLAS females – Anterior lenticonus, pathognomic (15% of males, associated with ESRD by 30 years) – maculopathy, whitish or yellowish flecks or granulations in a perimacular distribution • Leiomyomatosis (less common) – esophagus and tracheobronchial tree

Answer 25

LM: Early in disease, glomeruli may appear normal. Later global and segmental glomerulosclerosis, interstitial fibrosis

Answer 26

IF: Negative or non-specific IgM, C

Answer 27

EM: variable thickening, thinning, basket weaving, and lamellation of the GBM

Answer 28

``` • No disease-specific therapy – RAAS blockade • Renal replacement is eventually necessary • Transplant – 2-3% will get anti-GMB disease ```

Answer 29

• Usually autosomal dominant inheritance • Continuous or intermittent microhematuria, with or without gross hematuria, and generally no renal insufficiency • Previously considered benign – Proteinuria, HTN and ESRD are unusual • Extra-renal features are rare

Answer 30

LM: normal glomeruli

Answer 31

___________???

Answer 32

• Reassurance • Should be followed – Very small but real risk of progression to ESRD – BMP, urinalysis and BP monitored every 1-2 years