Renal Disease - GU Flashcards
Pre-renal vascular compromise
- Renal artery stenosis
- Renal artery thrombosis
- Renal artery aneurysm
- Atheroembolic renal disease
- Renal vein thrombosis
- Generally associated with hypertension
- Decreased blood flow to the kidney(s) as a result of renal vascular disease may cause an excessive amount of renin to be produced
- Renin increases blood pressure
Renal Artery Stenosis
-Stenosis due to atherosclerosis in 80-90% or fibromuscular dysplasia
Clues
- Sudden onset of hypertension < age 50 = fibromuscular dysplasia; > age 50 = atherosclerosis
- Hypertension not responsive to three or more blood pressure medications
- Increased urea
- Unexplained kidney failure
- Sudden kidney failure when first taking an ACE inhibitor
Renal Artery Thrombosis
- Results from trauma, infection, aneurism, inflammatory disease or fibromuscular dysplasia
- Acute complete blockage
- Sudden onset of flank pain and tenderness
- Fever, hematuria
- Nausea and/or vomiting
- Sudden decrease in kidney function
- Hypertension
Renal Artery Aneurism
- Generally asymptomatic
- Hypertension present in 90 percent
- Dissecting aneurysms may cause flank pain and hematuria
Atheroembolic Renal Disease
-Results from surgery, catheter insertion, anticoagulation
Clues
- Skin lesions such as purpura, mottling of the toes and feet
- Kidney failure, acute or chronic
- Abdominal pain, diarrhea
- Confusion, weight loss, fever, muscle aches
Renal Vein Thrombosis
- Results from trauma, compression of a renal vein by an adjacent structure such as a tumor or aneurysm, nephrotic syndrome, pregnancy, steroid use, and OCPs
- Chronic onset most often asymptomatic
- Acute onset
- Persistent severe flank that may be spasmodic at times
- CVA tenderness
- Decreased kidney function
Nephrotic Syndrome
- A Glomerular problem of leaking protein
- Proteinuria > 3 or 3.5 g/day
- Hypoalbuminemia
- Edema
- Hyperlipidema
- Oval fat bodies in the urine
Loss of protein in urine leads to
-Hypoalbuminemia, which causes -> Decreased plasma oncotic pressure, which ->Reduces ECV, thus circulating volume is decreased, so the kidney -> Increases Na and Water reabsorption in the tubules, which leads to -> EDEMA
Loss of protein in urine means:
- Some of those proteins are endogenous immunoglobulins, which leaves the patient prone to: INFECTION
- Some of those proteins are endogenous anticoagulants, which means: Hypercoagulable state, thus - ARTERIAL AND VENOUS THROMBOSIS
-Both hypoalbuminemia and diminished plasma oncotic pressure play a role in ->Increased hepatic production of VLDL, which results in -> HYPERLIPIDEMIA
Primary Nephrotic Syndrome
2/3
- Minimal-change nephropathy
- Focal glomerulosclerosis
- Membranous nephropathy
- Hereditary nephropathies
secondary Nephrotic Syndrome
1/3
- Diabetes mellitus
- Lupus erythematosus
- Amyloidosis and paraproteinemias
- Viral infections (eg, hepatitis B, hepatitis C, HIV)
- Preeclampsia
Presentation - Nephrotic Syndrome
Edema (serum albumin <3 g/dL)
- Dependent areas first, then generalized
- Periorbital
- Pulmonary edema, pleural effusions (dyspnea)
- Ascites (abdominal fullness)
-Infection, venous thrombosis
Labs - Nephrotic Syndrome
UA - 24hr or UPCR
-Proteinuria due to effacement of podocytes and basement membrane damage - causes foamy urine
Blood has:
- Hypoalbuminemia (<6 g/dL)
- Hyperlipidemia (50% early)
- Hypertrigliceridemia
- Elevated ESR, low Vit D, zinc, and copper in some
Generally need Renal Biopsy for specific diagnosis
General Treatment - Nephrotic Syndrome
Protein Loss
- Dietary protein to replace losses (protein restriction questionable)
- ACE inhibitor or ARB to decrease intraglomerluar pressure [monitor serum Cr and K]
Edema
-Salt restriction + diuretics (thiazide and loop, often high doses)
Hyperlipidemia
-Dietary and pharmacologic
Hypercoagulable state
-If albumin <2 g/dL, anticoagulation therapy
REFER ALL TO NEPHROLOGY
Minimal Change Disease - nephrotic syndrome
- most common in children
- No change on biopsy under light microscopy, but podocyte effacement on electron microscopy
- Idiopathic or post-URI, tumor, drugs, or hypersensitivity
- Usually presents full-blown nephrotic
- Rarely causes acute kidney injury
- Treatment: Prednisone - continue for several weeks after resolution of proteinuria
- Steroid-resistant nephrotic syndrome (4 weeks and persistent proteinuria) or relapses may need alternate treatment
Membranous Nephropathy - Nephrotic Syndrome
- 5th-6th decade presentation usually
- Immune-mediated
- 50% progress to ESRD in 3-10 years
- Treatment is generally immunosuppressive
Focal Segmental Glomerulosclerosis - Nephrotic Syndrome
- Idiopathic or secondary to heroin use, morbid obesity, HIV, or chronic urinary reflux
- Decreased renal function common at presentation
- Progress to ESRD in 6-8 years
Diabetic Nephropathy - Nephrotic Syndrome
- Most common cause of Glomerular disease and ESRD in the US (4000/yr)
- Type I 30-40%, Type II 15-20% after 20 years
- Males, Af Am, Nat Am higher risk
- Albuminuria (macro or microscopic) precedes decline in GFR
Progression
- Hyperfiltration (increase GFR)
- Microalbuminuria (30-300 mg/d)
- Albuminuria (>300 mg/dL)
- GFR returns to normal, then decreases
Treatment - begin early
- Aggressive glycemic control
- Treatment of hypertension : Goal 125-130/75-80
- ACE or ARBs slow progression
Acute Glomerulonephritis
-Immune-triggered inflammatory damage to the glomerulus
Nephritic Pattern -Urine RBCs: Some dysmorphic and some acanthocytes which are diagnostic of glomerular hematuria if >= 5% of urinary RBCs -Occasionally WBCs \+/- Red cell or mixed cellular casts -Variable proteinuria
Acute Glomerulonephritis - Presentation
- Sudden onset of hematuria, proteinuria, and red blood cell (RBC) casts
- Often accompanied be hypertension, edema, azotemia (decreased GFR), and renal salt and water retention
- Primary renal disease or secondary to systemic disease
- Immune complexes deposited in or formed in the glomerulus
- Enlarged glomeruli with infiltrating PMNs and Monocytes
- Granular deposits of IgG, IgM, and C3 along GBM
- Kidneys may be enlarged up to 50%
Most common presentation
-Acute facial puffiness with dark, scanty urine
Nonspecific symptoms
-Weakness, fever, abdominal pain and malaise
Acute Glomerulonephritis - Etiology
-Infectious - Post-streptococcal (PSGN) or other
Non-infectious
- Primary Renal - MPGN, Berger (IgA Nephropathy), etc.
- Systemic Diseases - Vasculitis, SLE, PAN, HSP, Goodpasture
- Other - Guillain-Barre, DPT vaccine, serum sickness
Acute Glomerulonephritis - Epidemiology
- Ig A Nephropathy most common worldwide
- PSGN most common in children, incidence falling in Western countries
- Males affected more often 2:1
- No ethnic predominance
- Some (post-infectious) socioeconomic variance
Acute Glomerulonephritis - History
Onset
- Usually abrupt
- Post-infections up to 3 weeks, variable
- PSGN - 1-2 weeks post pharyngitis or 2-4 weeks dermal
- If only 1-4 days post-streptococcal – preexisting renal disease
SXS
- Hematuria - Universal, even if microscopic
- Gross hematuria (30% of pediatric patients) smoky-, coffee-, or cola-colored urine
- Oliguria
- Edema - mild (involving only the face) to severe, bordering on a nephrotic appearance
- Headache - secondary to hypertension
- Shortness of breath or dyspnea on exertion - This may occur secondary to heart failure or pulmonary edema - uncommon
- Possible flank pain secondary to stretching of the renal capsule
- Identify possible etiologic agent
- Streptococcal throat infection or skin infection
- Recent fever, sore throat, joint pains, hepatitis, travel, valve replacement, and/or intravenous drug use
- Assess disease process symptoms
- Loss of appetite, generalized itching, tiredness, listlessness, nausea, easy bruising, nosebleeds, facial swelling, leg edema, and shortness of breath
Acute Glomerulonephritis - Classic presentation and PE
- Triad of sinusitis, pulmonary infiltrates, and nephritis- Wegener granulomatosis
- Nausea and vomiting, abdominal pain, and purpura - Henoch-Schönlein purpura
- Arthralgias - SLE
- Hemoptysis - Goodpasture syndrome or idiopathic progressive glomerulonephritis
- May have normal exam and blood pressure, but most often edema and hypertension (75%)
- Look for fluid overload signs
- Periorbital and/or pedal edema
- Crackles
- Elevated jugular venous pressure
- Ascites and pleural effusion
- Also look for: Rash (vasculitis, HSP, or lupus nephritis), Pallor, CVA fullness or tenderness, Abnormal neurologic examination or altered level of consciousness (from malignant hypertension), Arthritis
Acute Glomerulonephritis -Labs
-UA - look for dark urine, high specific gravity, protein, blood, dysmorphic RBCs, WBCs, RBC Casts
Blood Tests
- Complete blood count (CBC)
- Blood urea nitrogen (BUN), serum creatinine, and serum electrolytes (especially serum potassium)
- Erythrocyte sedimentation rate (ESR)
- Complement levels (C3, C4, CH50)
- Renal US can be helpful to assess size (smaller kidneys are less likely to recover) and r/o obstruction
- Renal Biopsy is not necessary to diagnose PSGN, but helps to guide therapy and prognosis; required for primary renal disease
IgA Nephropathy
-Any age, peak 2nd and 3rd decade
-40-50% present with visible hematuria after URI
+/- flank pain and fever, mimics UTI or urolithiasis
-Recurrences over a few years
-30-40% present with microscopic hematuria and mild proteinuria noted incidentally - 25% will go on to gross hematuria
<10% present with nephrotic syndrome or edema/hypertension/oliguria with hematuria
-Associations - Cirrhosis, Celiac, HIV, AD Familial
-Diagnosis - Renal Biopsy: Generally not done unless more severe signs
-Acute kidney injury rare from tubular injury due to occulsion from hematuria
IgA Nephropathy - Management
- Patients with isolated hematuria, no or minimal proteinuria, normal GFR
- No treatment, monitor every 6-12 mo
- Patients with persistent proteinuria (above 500 - 1 g/day), normal or sl reduced GFR, mild to moderate histologic findings on renal biopsy
- ACE inhibitor or ARB
- Fish oil (3.3 grams/day or more) if protein excretion above 1 g/day despite three to six months of therapy with an ACE inhibitor or ARB
- More severe disease: Anti-inflammatory/immunosuppressive therapy
- Refer all patient with suspected IgA Nephropathy to a Nephrologist
- Usually benign course but ESRD occurs in 15-20% of patients within 10 years of onset and in about 25-30% of patients by 20 years (biopsied patients only)
Post-Streptococcal Glomerulonephritis (PSGN)
- Typically age 2-12; 5% < 2; 10% >40
- Disease severity and mortality much higher in adults
- Presentation - Latent period after strep infection, dark urine, abrupt onset of periorbital edema
- Nonspecific sx - anorexia, malaise, weakness, nausea and vomiting possible
- HTN 60-80%; Oliguria 10-50% (transient); LV Dysfunction possible
Work up
- As previously discussed, plus streptozyme
- Will have elevated BUN and Cr, transient
- Low serum complement
- Proteinuria may be mild or full-blown nephrotic
PSGN - Management
- Symptomatic - control edema and blood pressure
- Limit salt and water
- Diuretics if severe edema or hypertension (Loop diuretics)
- CCB or ACE for refractory HTN
- Dialysis if life-threatening hyperkalemia, clinical uremia
- Restrict activity until feeling well
- Steroids not indicated
- Treat family members for strep (throat culture everyone) with PenG or Erythromycin 7-10 days
Hydronephrosis
- An obstructive problem that may CAUSE tubulointerstitial disease
- Hydronephrosis - distention of the renal calyces and pelvis with urine as a result of obstruction of the outflow of urine distal to the renal pelvis
- Hydroureter is dilation of the ureter
- May be physiologic or pathologic
- Chronic or acute, bilateral or unilateral
Hydronephrosis - Causes
- Anatomic abnormalities - majority of pediatric cases
- Urethral valves or stricture, or stenosis at the ureterovesical or ureteropelvic junction
- Calculi - most common in young adults
- Prostatic hypertrophy or carcinoma, retroperitoneal or pelvic neoplasms, and calculi - primary causes in older patients
- Pregnant women - 80%, R>L, second trimester, persists 6-12 weeks postpartum
- Increased ureteral pressure leads to -> Decline in GFR within hours which persists for weeks after relief of obstruction
- Severe impairment of renal tubular ability to -> Transport sodium, potassium, and protons and Concentrate and dilute the urine
- Brief disruptions - reversible functional disturbance
- Chronic disruptions - profound tubular atrophy and permanent nephron loss
Hydronephrosis - History
-Acute - Pain - distention of bladder, collecting system, or renal capsule
Stone or fluid load exceeding flow rate
-Non-acute - pain minimal or absent
Congenital UPJ obstruction or tumor
- Upper - flank pain; Lower - pain radiates to ipsilateral testicle or labia
- Anuria possible
Flags
- Blood - stone
- Fever or diabetes - urgent
- Solitary kidney - emergent
Hydronephrosis - Exam, labs, Tx
- Kidney may be palpable
- Bilateral hydronephrosis - LE edema common
- CVA tenderness common
- Distended bladder - Lower obstruction
- Digital rectal exam for prostate hypertrophy, masses, etc.
- UA ->Pyuria - infection, Microscopic hematuria - stone or tumor
- CBC - check for leukocytosis/acute infection.
- Chemistries -> Elevated BUN and creatinine, and hyperkalemia all possible
tx
- Directed at relieving the obstruction
- Generally surgical
Polycystic Kidney Disease
- A genetic architectural problem
- Cystic enlargement of BOTH kidneys
- Hypertension
- Hematuria, polyuria
- Flank pain
- Recurrent UTIs
- Heart valve defects (MVP) with infective endocarditis
- Cerebral and aortic aneurysms
- Liver cysts (with impaired function)
- Renal Stones
- Increased risk for renal carcinomas (ALL renal failure patients have a generally elevated cancer risk)
PKD Genetics
- Most forms of PKD are hereditary
- AD (Autosomal dominant) most common
- AR (Autosomal recessive)
- Congenital PKD: 7.4% of dialysis and 9.5% of transplant patients
- “Acquired” PKD - from aging, dialysis, drugs, hormones
Autosomal recessive - PKD
-RARE - 1:20,000 live births
Morphology
-Elongation of renal collecting tubules into multiple cysts
-Often linked with fetal or neonatal death - enlarged kidneys + lung dysgenesis/pulmonary hypoplasia
Autosomal dominant - PKD
- Dominant Mendelian trait 1:800 live births
- about 50,000 cases in US
- MOST COMMON lethal condition inherited as a dominant trait!
- Incomplete penetrance – highly variable expression pattern
- Symptoms usually present in 3rd-4th decade (though cysts present prior to birth)
- Pain is most common presenting symptom
- Ultrasound is imaging of choice
- Management is control of HTN, UTIs, hematuria, and pain
- ESRD - Dialysis and Transplant
Autosomal dominant - PKD - Risk factors
- PKD1 genotype
- Large kidneys
- Several episodes of gross hematuria
- Severe and frequent kidney infections
- Hypertension
- Multiple pregnancies
- Black racial background
- Male sex
AD PKD History
Pain is most common - abdomen, flank, or back - from:
- Enlargement of one or more cysts
- Bleeding into cyst or ruptured w/ gross hematuria
- Urinary tract infection (eg, pyelonephritis, infected cysts, perinephric abscess)
- Nephrolithiasis and renal colic
OR pain may be from an associated condition
Hematuria : Self-limited, lasting 1 week or less
- Polycystic kidneys are prone to injury: 60% have hemorrhages
- Mild trauma - intrarenal hemorrhage or bleeding into the retroperitoneal space (PAIN!)
AD PKD Exam
Hypertension (diastolic) - 50-75% of patients, even w/normal renal function
- More severe early
- Improves as the renal insufficiency progresses
- Palpable, bilateral flank masses occur in patients with advanced ADPKD
- Nodular hepatomegaly occurs in those with severe polycystic liver disease
AD PKD Complications
- End-stage renal disease (ESRD) - frequent
- Polycystic liver disease
- Cysts in liver, pancreas, spleen
- Pain and infection
- Cerebral aneurisms - 4-10%
- Nephrolithiasis - 20-30%, uric acid stones more likely
AD PKD Work up
Who to work up -Family history of PKD -Hypertension + Recurrent UTIs (>2/yr) Gross hematuria and/or polyuria
- Ultrasound (also for screening relatives
- Serum chemistry profile, including calcium and phosphorus
- Complete blood cell count
- Urinalysis
- Urine culture
- Uric acid determination
- Intact parathyroid hormone assay
AD PKD Treatment
- Control blood pressure
- Control abnormalities related to renal failure
- Treat urinary tract infections
- Treat hematuria
- Reduce abdominal pain produced by enlarged kidneys (analgesics, cyst decompression)
- Heart murmurs: AHA antibiotic prophylaxis
- Refer to Nephrology for most up to date management
- Monitor for and treat hyperkalemia, hyperphosphatemia, hypocalcemia, hyperparathyroidism, metabolic acidosis
- Diet and Activity: Low salt if HTN or renal failure, NO CONTACT SPORTS
