CMPP: Misc Renal Diseases

proteinuria, hypoalbuminemia, edema, and hyperlipidemia

--> secondary to inflammation of glomerular wall

nephrotic syndrome is characterized by...

3 gm

nephrotic syndrome is defined as > ___ of proteinuria daily

protein leaks out of the blood --> this decreases serum osmolality compared to interstitum --> water rushes out

why does edema occur in nephrotic syndrome

focal glomerulosclerosis (#1)

minimal-change nephropathy

membranous nephropathy

hereditary nephropathies

primary (renal) causes of nephrotic syndrome

Diabetes mellitus (#1)

SLE

pre-eclampsia

amyloidosis

Viral infections (hepatitis, HIV)

illicit drugs (heroin abuse)

secondary causes of nephrotic syndrome

glomerulus -- through the endothelium and the filtration slits located between the podocytes

--> damage to any component of the filtration barrier may cause proteinuria

where does filtration of plasma water/solute occur?

albumin -- increased glomerular injury eventually leads to leakage of all plasma proteins

in the pathologic protein filtration associated with nephrotic syndrome, what protein tends to leak first?

hyperlipidemia and atherosclerosis

increased risk of infections

hypocalcemia and bone abnormalities

hypercoagulability

hypovolemia

adverse consequences of proteinuria

proteinuria causes the loss of a protein that breaks down lipid -- increased LDL synthesis --> plaque buildup

how does proteinuria cause HLD?

Strep pneumonia, H influenza, E coli

what are the primary bugs that can cause infectious complications in nephrotic syndrome

spontaneous bacterial peritonitis

infectious complications of nephrotic syndrome include an increased incidence of sepsis including _____________.

urinary loss of vitamin D-binding proteins leads to decreased intestinal calcium absorption

prolonged steroid use can also reduce bone mass

why does nephrotic syndrome result in hypocalcemia and bone abnormalities?

anticoagulant proteins are lost in urine

why does nephrotic syndrome result in hypercoagulability

loss of plasma H2O into the interstitum because H2O follows the protein

--> presents with tachycardia, prolonged cap refill, and oliguria

why does nephrotic syndrome result in hypovolemia?

periorbital edema

first sign of nephrotic syndrome in children

dependent edema (adults)

foamy urine

thrombotic complication

secondary cause -- DM, SLE, pre-eclampsia

other historical factors associated with nephrotic syndrome

3+/4+ proteinuria

3 gm/day -- nephrotic range

hypoalbuminemia

hyperlipidemia

what is seen on the lab studies for one with nephrotic syndrome?

confirms 2 kidneys before biopsy

what is the role of an U/S in the dx of nephrotic syndrome?

peds -- congenital, > 8 y/o, steroid resistance/dependency

adults -- no secondary cause

what is the role of a renal biopsy in the dx of nephrotic syndrome?

Prednisone

what is the corticosteroid of choice in treating nephrotic syndrome?

treats PRIMARY disease process -- decreases inflammation and reduces capillary permeability

Prednisone MOA in nephrotic syndrome

Spironolactone and Furosemide

what diuretics are used to treat nephrotic syndrome?

ACEis and ARBs

diabetic nephrotic syndrome is commonly treated with...

ultrasound guided paracentesis

how is SBP treated in children?

IV albumin

how is severe hypoalbuminemia treated in children?

low Na diet

lipid-lowering agents

no activity restrictions

treat underlying disease

what are other treatments for nephrotic syndrome other than steroids and diuretics

Glomerulonephritis

immunologic mechanism triggers inflammation and proliferation of glomerular tissue; results in damage to the glomerular capillary membrane or mesangium

hematuria, RBC casts, proteinuria

glomerulonephritis tends to present with the sudden onset of...

post-infectious (ACUTE POST-STREP GLOMERULONEPHRITIS), primary renal disease, systemic disease, or misc processes

potential causes of glomerulonephritis

diffuse glomerular swelling secondary to deposition of immune complexes

glomerulus infiltrated by neutrophils and complement activation occurs -- thickened/inflamed basement membrane

pathophysiology of glomerulonephritis

decreased GFR and subsequent activation of RAAS

--> RAAS activation results in intravascular volume expansion, edema, and hypertension

glomerular capillary obstruction leads to...

Nephritogenic Group A strep -- most common predisposing infection

--> pharyngitis (winter)

--> impetigo/cellulitis (summer/fall)

--> 1-3 wks later

what are some infectious causes of glomerulonephritis?

Primary renal diseases (Berger disease)

Systemic diseases (SLE, Guillain-Barre)

Misc. diseases

what are some non-infectious causes of glomerulonephritis?

headache, edema, flank pain, hematuria, oliguria, hypertension

presentation of glomerulonephritis

Hematological disorders/BPH

Infections (UTI)

Trauma

Tumor of the urinary tract

Exercise

Renal (glomerulonephritis)

Stones

acronym for ddx of hematuria

oliguria, hypertension, and edema

the most common presentation of GN is a combination of...

blood and RBC casts

what will a UA show in one with glomerulonephritis?

supportive -- diuretics and vasodilators

tx for glomerulonephritis

prevents Na active transport from the ascending loop of henle -- no concentration gradient -- water stays in tubule and gets excreted in the urine

loop diuretic MOA

Furosemide (Lasix)

loop diuretic agent

reduce total peripheral resistance -- decreases BP

vasodilator MOA

Hydralazine

Nitroprusside

--> indicated in malignant HTN

vasodilator agents

adults, infants

ADPKD is seen in ________, while ARPKD is seen in ________.

RRT

PKD is an incurable disease, and all pts will need eventual...

stage 5 CKD

--> all cases lead to severe CKD

ADPKD is the #1 genetic cause of...

cyst formation and renal enlargement

--> may form cysts in additional organs, including the liver, pancreas, and spleen

ADPKD is characterized by...

3rd/4th decades of life

-->20s/30s

ADPKD usually causes symptoms during...

thickening of the renal tubules and neovascularization

in ADPKD, the bilateral, progressive increase in cysts over time, leads to...

decreased urine-concentrating ability (low urine specific gravity gravity in the setting of dehydration)

first manifestation of ADPKD

low urine specific gravity in the setting of dehydration

decreased urine-concentrating ability is characterized by...

spontaneous bleeding, cerebral aneurysms

the neovascularization associated with ADPKD results in __________, which make a patient at increased risk for ______________.

PKD1 -- more common and more severe, CKD usually develops in the early 50s

PKD2

what are the two types of ADPKD and how do they manifest

heterozygous

those with ADPKD are usually ________ for the trait

throughout life stimulants cause the gene to turn on or off

how does stimulant theory lead explain why ADPKD begins to show more symptoms with age?

abdominal, flank, or back pain

what is the #1 initial complaint associated with ADPKD

transient hematuria

nonspecific symptoms

impaired urine concentrating -- polyuria and nocturia

Hypertension

bilateral flank masses as disease progresses

other common symptoms associated with ADPKD

CKD Stage 5 -- #1 complication

cerebral aneurysms

--> <50 y/o with uncontrolled HTN

Nephrolithiasis

UTIs (stasis of fluid within cysts)

liver, splenic, pancreatic cysts

complications associated with ADPKD

ultrasonography

--> diagnostic criteria based on patient's age and number of cysts

what is the study of choice in ADPKD?

magnetic resonance angiography (MRA)

study of choice for screening for intracranial aneurysms in ADPKD patients

new-onset severe headache

CNS symptoms

prior to elective surgery

FH of cerebral aneurysm or hemorrhage

absolute indications for MRA in ADPKD

control abnormalities related to severe CKD

--> volume overload, HTN, hyperkalemia, metabolic acidosis

--> hyperphosphatemia/decreased Vit D leading to hypocalcemia and secondary hyperparathyroidism, anemia

treat UTIs

control abdominal pain

avoid NSAIDs and other nephrotoxins

proactive discussion of RRT options

how to manage ADPKD

Autosomal recessive polycystic kidney disease

infantile PKD; much less common than ADPKD; characterized by diffuse cystic dilatation of the collecting ducts results in an inability to adequately concentrate the urine

1/4, 1/2

if both parents are carriers for ARPKD, each child has a ____ chance of having the disease and a ___ chance of being a carrier

enlarged polycystic kidneys

oligohydramnios --> will lead to pulmonary hypoplasia

ultrasound may reveal what findings in an infant with ARPKD

congenital hepatic fibrosis

all patients with ARPKD have associated...

1/2

in ARPKD, if only one parent is a carrier, each child has a ___ chance of being a carrier

large palpable flank masses (make delivery difficult)

Potter facies

polyuria/polydipsia (but ya cannot really tell)

may have severe HTN

presentation of ARPKD

Prenatal U/S

--> can see enlarged kidneys, oligohydramnios, congenital hepatic fibrosis

what is the most common means of diagnosis for ARPKD

supportive tx

--> BP control (ACEi/ARBs), prevent hyperkalemia, bicarb supplements, phosphate binders and vitamin D analogs

--> EPO and iron, management of hepatic complications

--> will eventually require renal transplant

how to manage ARPKD