Chapter 12 - Renal Flashcards
What is Potter Sequence, and when do you see it?
Lung hypoplasia, flat face with low set ears, and developmental defects of the extremities.
See with bilateral renal agenesis, OR ARPKD…
Dysplastic Kidney Disease (distinguishing features and what to put on Ddx…)
NONINHERITED, congenital malformation leading to cysts and abnormal tissue in kidney parenchyma (eg cartilage).
Usually unilateral, but can be bilateral, in which case you MUST distinguish from PKD.
Symptoms of ARPKD
Worsening renal failure, hypertension. Also Potter sequence, Congenital hepatic fibrosis and hepatic cysts.
Associated symptoms of ADPKD
Berry aneurysm, hepatic cysts, and mitral valve prolapse.
Diagnosis with cysts in kidney medullary collecting ducts resulting in shrunken kidneys and renal failure?
Medullary Cystic Kidney Disease (Autosomal Dominant).
Lab Signs of PreRenal Azotemia
Serum BUN:Cr ratio >15 FENa 500 (Tubules are STILL WORKING)
Symptoms of Acute Renal Failure (all etiologies)
Azotemia (Increased BUN and Creatinine)
Oliguria (low urine production
Lab Signs of Postrenal Azotemia
Early- same as prerenal (tubules still intact and working)
Late - see tubular damage. BUN:Cr ratio 2%, urine osm <500.
Brown, granular casts in urine (“Muddy Brown”) means…?
Acute Tubular Necrosis!
Lab Signs of Acute Tubular Necrosis?
Tubules stop working, so BUN:Cr ratio 2%, urine osm <500
Causes of Nephrotoxic Acute Tubular Necrosis?
Aminoglycosides (most common), heavy metals, myoglobinuria, ethylene glycol (see OXALATE CRYSTALS in urine), radiocontrast dye, and urate (from TUMOR LYSIS SYNDROME)
How to prevent Tumor Lysis Syndrome?
Good hydration and allopurinol (prevent formation of uric acid leading to excess urate)
Clinical features of Acute Renal Tubular Necrosis?
- Oliguria with brown, granular casts
- Elevated BUN and Cr.
- Hyperkalemia (decreased renal excretion) with metabolic acidosis (high anion gap, as decreased excretion of organic acids)
Causes of Acute Interstitial Nephritis?
DRUGS - NSAIDS, penicillins, diuretics, etc.
Key finding in Acute Interstitial Nephritis?
Eosinophils in urine!
Causes of Renal Papillary Necrosis? (4)
- Chronic analgesic abuse
- DM
- Sickle cell trait/disease
- Severe acute pyelonephritis.
Causes of Intrarenal azotemia leading to Acute Renal Failure? (3)
- Acute Tubular Necrosis
- Acute Interstitial Nephritis
- Renal Papillary Necrosis
Key Findings in Nephrotic Syndrome? (5) Increased risk of what two conditions?
- Proteinuria (>3.5g/day)
- Hypoalbuminemia
- Edema
- Hyperlipidemia/hypercholesterolemia
- Hyperlipiduria
See increased risk of infection (due to loss of IgG’s) and hypercoagulability (due to preferential loss of Antithrombin III)
Who gets Minimal Change Disease most often and what can it be associated with?
Children. Associated with Hodgkin Lymphoma.
How to treat Minimal Change Disease?
Steroids! Excellent response.
Who gets FSGS most often and what is it associated with?
Hispanics and African Americans. Associated with HIV, heroin use, and Sickle Cell Disease.
Who gets Membranous Nephropathy most often and what is it associated with?
Caucasian adults. Associated with HBV, HCV, solid tumors, SLE and drugs (NSAIDS/penicillamine).
“Tram-track” appearance on H&E stain = ?
Membranoproliferative Glomerulonephritis!!!
Type I Membranoproliferative Glomerulonephritis - where are the deposits located and what is it associated with?
Subendothelial deposits. Associated with HBV and HCV.
Type II Membranoproliferative Glomerulonephritis - where are the deposits located and what is it associated with?
Intramembranous deposits (within GBM). Associated with C3 Nephritic Factor (autoantibody that stabilizes C3 Convertase –> increased activation of C3 and complement and inflammation).
Kimmelsteil-Wilson nodules = ?
Diabetic Nephropathy. The nodules themselves are sclerosis of mesangium
How to slow the progression of Diabetic Nephropathy?
ACEi, as this helps to dilate efferent arteriole, thus reducing GFR pressure.
What findings do you see with Systemic Amyloidosis Nephropathy with light microscopy?
Apple-green birefringence under polarized light with Congo red Staining.
What type of bugs can cause Post-Streptococcal Glomerulonephritis?
Nephritogenic Strains of Group A b-hemolytic strep (carry M protein virulence factor). Also some nonstreptococcal organisms as well…
What make up the crescents seen on light microscopy of Rapidly Progressive Glomerulonephritis?
Fibrin and Macrophages!
Causes of granular deposit rapidly progressive glomerulonephritis (as opposed to linear)
Post-strep glomerulonephritis OR diffuse glomerulonephritis (see with SLE!!!)
Symptoms of Wegener granulomatosis?
Hemoptisis, hematuria, and nasopharyngeal symptoms. C-ANCA positive!
How to distinguish Microscopic Polyangiitis from Churg-Strauss syndrome causing Rapidly Progressive Glomerulonephritis?
Both are P-ANCA positive. Churg-Strauss will show garnulomatous inflammation, eosinophilia, and asthma….
Symptoms and cause of Alport Syndrome?
Isolated hematuria, sensory hearing loss, and ocular disturbances. Due to inherited defect in type IV collagen.
Cystitis (definition)
Infection of the bladder
Most common causes of Cystitis (UTI’s)
- E coli (80%)
- Staph Saprophyticus
- Klebsiella pneumoniae
- Proteus mirabilis (alkaline urine with NH4 scent)
- Enterococcus faecalis
Findings with Chronic Pylonephritis?
Cortical scarring (upper and lower poles characteristic of vesicoureteral reflex), blunted calyces, and eosinophilic proteinaceous material filling atrophic tubules (Thyroidization)
Where in the kidney is Epo made?
By renal peritubular interstitial cells…
Effects on bone of Chronic Renal Failure?
Renal Osteodystrophy:
Osteitis Fibrosa Cystica (due to secondary hyperparathyroidism), osteomalacia (cannot mineralize osteoid), and osteoporosis…
What is Angiomyolipoma associated with?
Tuberous Sclerosis!!!
How to treat Calcium Nephrolithiasis?
Thiazide diuretics, as they decrease Ca in urine…
Classic finding with Ammonium magnesium or phosphate nephrolithiasis?
Staghorn calculi –> require surgery.
When do you see Ammonium, magnesium, or phosphate nephrolithiasis?
Infection with urease-positive org (Proteus, Klebsiella)…
Common symptoms seen with Renal Cell Carcinoma?
Fever, weight loss, and PARANEOPLASTIC SYNDROMES (lots, including EPO, renin –> HTN, PTHrP, or ACTH –> Cushings).
Also Left-Sided Varicocele (due to spread into renal vein, blocking drainage from left spermatic vein (right drains directly into IVC).
Molecular pathogenesis of Renal Cell Carcinoma?
Loss of VHL tumor suppressor gene on chrom. 3.
Von Hippel-Lindau disease common malignancies?
Hemangioblastoma of cerebellum and RENAL CELL CARCINOMA.
Molecular pathogenesis of Wilms Tumor and who do you see it in most often?
Associated with WT1 mutations. Most common malignant renal tumor in children…
What is WAGR?
WAGR Syndrome: Wilm's Tumor Aniridia Genital abnormalities mental Retardation
What is Beckwith-Wiedemann syndrome?
Wilms tumor, neonatal hypoglycemia, muscular hemihypertrophy, and organomegaly (incl. tongue)
Risk factors for Transitional Cell Carcinoma (Urothelial)?
SMOKING, naphthylamine, azo dyes (in hair dyes), and long-term cyclophosphamide or phenacetin use.
Two pathways to develop Transitional Cell Carcinoma?
Flat- Early p53 mutations, develops as a high-grade flat tumor.
Papillary- develops as a low-grade tumor that progresses to high-grade and invasive. Not associated with early p53 mutations.
Risk factors for Squamous Cell Carcinoma of the lower Urinary tract?
Chronic Cystitis (older women), Schistosoma hematobium (Egyptian male), long-standing nephrolithiasis…
Cause of Adenocarcinoma of lower Urinary tract?
- Arise from Urachal remnant (duct that connects fetal bladder with yold sac. Tumors arise in the DOME of the bladder)
- Cystitis Glandularis (Chronic inflammation of bladder leading to columnar metaplasia)
- Exstrophy (congenital failure to form caudal portion of the anterior abdominal wall –> exposing bladder wall).
