Renal Physio Flashcards
What is the condition called with a WT2 mutation
Beckwith-Wiedemann syndrome
Describe the symptoms of Beckwith-Wiedmann
Age 2-4; Wilm’s tumor, organomegaly, macroglossia, hemihypertrophy
What symptoms occur with WT1 deletion?
WAGR: Wilm’s tumor, Aniridia, Genituorinary complications, Mental Retardation ages 2-4
Which chromosome is mutated in Wilms?
Chromosome 11
What symptoms occur with WT1 mutation?
Denys-Drash: Wilm’s tumor, pseudo-hermaphroditism, early-onset nephrotic syndrome
Which factors place a pt at risk of Transitional Cell carcinoma of the urinary tract system?
Smokers, people who worked in rubber, plastic, aniline dyes, textiles, leather. Peak incidence in 70s-80s
Sterile pyuria and (-) gram cultures suggest UTI by which organisms
N. gonorrhea or Chlamydia trachomatis
List the risk factors of acute pyelonephritis
- Indwelling catheter
- Diabetes mellitus
- Pregnancy
- Ascending UTI
- VUR
On biopsy what does kidney tissue from chronic pyelonephritis resemble
Thyroid tissue: eosinophilic casts present in tubules
List the consequences of renal failure
Metabolic Acidosis
Dyslipidemia (increased triglycerides)
Hyperkalemia (cannot secrete K+ into tubules)
Uremia: increased BUN
Na+/H2O retention (Hypervolemic hyponatremia)
Growth retardation and development delay
Erythropoetin failure (normocytic normochromic anemia)
Renal osteodystrophy: secondary hyperparathyroidism
List the clinical presentation of uremia in chronic kidney failure
increased BUN (blood urea nitrogen.. ammonia) Nausea and anorexia Encephalopathy and neuropathy Asterixis Pericarditis Platelet dysfunction Itchiness
List the Ps that cause Acute Interstitial Nephritis
Pee (diuretics) Pain-free (NSAIDs) Penicillins and cephalosporins Proton Pump Inhibitors rifamPin
Describe SAAD in renal papillary necrosis
Sickle cell disease or trait
Acute pyelonephritis
Analgesics (NSAIDs)
Diabetes mellitus
What is the mutation in ADPKD
PKD1 on chromosome 16 in 85% cases
PDK2 on chromosome 4 in 15% cases
What other complications is ADPKD associated with?
Saccular aneuryms, mitral valve prolapse, benign hepatic cysts
What is the treatment for ADPKD
ACEIS or ARBs
Which part of the kidney does ARPKD affect?
Collecting ducts
How does an infant with ARPKD present?
POTTER sequence (pulmonary hypoplasia, oligohydramnios, twisted face, twisted limbs, ears low set, renal agenesis
What are some complications with ARPKD
Hepatic fibrosis, systemic hypertension, progressive renal insufficiency and portal hypertension
How does medullary cystic disease appear on ultrasound
Shrunken kidneys; poor prognosis
How doe simple cysts appear on ultrasound
Anechoic, typically incidental findings and asymptomatic
Adverse effects of ACEIs (ACEs CATCHH)
Cough Angioedema (due to increased bradykinin) Teratogen (fetal renal malformations) Creatinine increase (decrease GFR) Hyperkalemia Hypotension
When should you use ACEIs cautiously?
Bilateral renal artery stenosis (indicated in patients with atherosclerotic plaques… chronic TIAs, etc)
What is the mechanism of Angiotensin II receptor blockers (ARBs, -artans)?
Selectively block binding of AII to AT1R.
Clinical use of ARBs
Hypertension, HF, proteinuria, diabetic nephropathy with intolerance to ACE inhibitors (cough, angioedema)
Adverse effects of ARBs
Hypotension, Hyperkalemia, decreased GFR, teratogen
What is the function of Aliskiren?
Direct renin inhibitor, blocks conversion of Angiotensinogen to AI
What is Aliskiren used for?
Hypertension
Contraindications for Aliskiren
Patients taking ACEIs or ARBs
Side effects of Aliskiren
Hyperkalemia, decreased GFR, hypotension
Why do you get hyperkalemia with ACEIs and ARBs?
Angiotensin II is an activator of Aldosterone. If AII is blocked, you have lower levels of Aldosterone and decreased K+ secretion at the DCT
Which amino acids are lost in urine in patients with cystinuria?
COLA: Cysteine, Ornithine, Lysine, Arginine
Do patients with defective transporter of COLA amino acids develop deficiencies? What risks do they have?
No, these are absorbed sufficiently as oligopeptides. Can develop cysteine kidney stones
What is the most common cause of unilateral fetal hydronephrosis?
Inadequate canalization of the uteropelvic junction
In the true pelvis, which structure lies posterior to the ureter
internal iliac artery
In the true pelvis, which structure lies anterior to the ureter
Uterine artery
Patients with sporadic and hereditary renal cell carcinoma likely have a deletion in which gene on which chromosome?
VHL on chromosome 3p
Which part of the nephron absorbs the most water regardless of state of hydration?
Proximal tubule (>60%)
What is the rapid plasma reagin test used for?
Detect syphillis
Which condition can cause false positives in rapid plasma reagin tests?
anti-phospholipid antibody syndrome
Which condition is associated with antiphospholipid antibody syndrome?
Systemic Lupus Erythematous
What can post-renal azotemia (pressure hydronephrosis from bladder outflow obstruction) lead to in renal tubular cells
Parenchymal pressure atrophy
What is the peritoneal location of the bladder?
Extraperitoneal (cannula does not enter peritoneum)
Which pH do you need for calcium phosphate crystals to form?
> 7.0
Which pH do you need for magnesium ammonium phosphate (struvite) crystals to form?
> 7.0
Why are patients with chronic diarrhea likely to form uric acid crystals?
Chronic dehydration, and loss of bicarb leads to state of metabolic acidosis. Kidneys compensate by increasing H+ secretion and HCO3- reabsorption
Which cytokine recruits eosinophiles?
IL-5
How to eosinophils attack parasites?
Bind parasites coated with IgG and IgE, release major basic protein (cytotoxic protein) to kill parasite
How to do eosinophils function in Type I hypersensitivity reactions
Synthesize prostaglandins, leukotrienes and cytokines to contribute to inflammmation
In which part of the nephron is urine isotonic?
Proximal tubule
In which part of the nephron is urine most dilute?
Distal convoluted tubule
In which parts of the nephron is urine most hypertonic?
medullary part of LoH and Medullary part of collecting duct
Prolonged use of NSAIDs can lead to which condition?
Chronic interstitial nephritis
How do NSAIDs lead to papillary damage in the nephron?
They concentrate in the papillae, uncouple ox-phos reactions and deplete glutathione with subsequent lipid peroxidation– leading to damaged tubular and vascular endothelial cells
How do NSAIDs cause ischemic papillary necrosis?
Inhibit prostaglandins, which inhibits vasodilation of afferent arteriole, leading to decreased perfusion and thus ischemic papillary necrosis
Which key histologic feature is seen in LM of Diabetic nephropathy?
Kimmmelstiel-Wilson nodules
How do glomeruli appear in membranoproliferative glomerulonephritis?
HYPERCELLULAR with thickening and splitting of GBM due to subendothelial immune complex deposition
In which pts should you suspect membranoproliferative glomerulonephritis?
Patients with Hepatitis C or B
In which patients should you suspect focal segmental glomerulosclerosis?
HIV, heroin abusers, severe obseity
Crescent formation in rapidly progressive glomerulonephritis occurs due to deposition of which substance?
Fibrin
Which molecular components are deposited along the GBM in patients with type 1 RPGN (Goodpasture syndrome)?
IgG and C3
Which electrolyte imbalance can lead to weakness and arrythmias with T wave flattening, ST depression and prominent U waves?
Hypokalemia
What is the ultimate risk of profound hypokalemia?
Ventricular tachycardia/fibrillation
What is the primary treatment for elevated anion gap metabolic acidosis in diabetics?
Insulin and saline
What potential electrolyte abnormalities present with severe hypothyroidism?
Hyponatremia, extracellular volume expansion and hypoglycemia
What are the common symptoms of Goodpasture syndrome?
Shortness of breath, Hemoptysis, proteinuria and hematuria with dysmorphic red blood cells
What causes Goodpasture syndrome?
Autoantibodies against alpha 3 chain of Type IV collagen in glomerular and alveolar basement membranes
Where is the macula densa located?
Distal convoluted tubule
What does the macula densa monitor?
Na+ concentration and tubular flow rate
Where are JG cells located and what is their function?
Modified smooth muscle cells on AA, secrete renin in response to significant renal hypoperfusion
What is the net renal excretion for a substance A
Net excretion = Filtered (A) - Reabsorbed (A)
What is seen on light microscopy in a patient with poststreptococcal glomerulonephritis?
Enlarged and hypercellular glomeruli
What is seen on immunofluorescence in pt with PSGN?
Granular deposits of IgG, IgM and C3 along the basement membrane and in the mesangium of glomeruli (“lumpy-bumpy”)
What is seen on EM in pt with PSGN?
Electron dense immune deposits on epithelial side of basement membrane
Why does nephritis cause hypertension?
Kidney damage reduces sodium handling ability of kidney–> less sodium is filtered, so more water and sodium is retained (increase body volume)
Fibrin deposition along the basement membrane is characteristic of
Rapidly progressive glomerulonephritis
How does Diabetic nephropathy present on LM?
Kimmelstiel-Wilson nodules, hyalin acellular deposits composed of plasma proteins
Lipid droplets in renal tubules is indicative of
Nephrotic syndrome
Why do you get hypocalcemia and secondary hyperaparthyroidism in chronic kidney disease?
Loss of normal renal parenchyma leads to decreased PO4 secretion and decreased Ca+2 reabsorption. Serum PO4 binds Ca+2 and lowers total Ca+2 (free) leading to hypocalcemia. Damaged tubules cannot produce Vitamin D (1,25-OH), further decreasing Ca+2. Decreased serum Ca+2 leads to increased PTH.
Three functions of PTH
Osteoclastic resorption, releasing Ca+2 and PO4 into serum
Increasing renal Ca+2 reabsorption and reducing phosphate reabsorption
Upregulating renal 1-alpha hydroxylase to form active Vitamin D
Function of active vitamin D
increase intestinal Ca+2 and PO4 absorption
The cytoplasm in clear cell carcinoma is abundant in
Glycogen and lipid content
Why do erythrocytosis, polycythemia and hypercalcemia occur in clear cell carcinoma?
Tumor cells produce EPO and PTHrP
Which laboratory study is most useful for diagnosing cause of patient’s metabolic alkalosis?
Urine chloride levels
Which metabolic alkaloses are saline responsive?
Thiazide/loop diuretic use, vomiting/nasogastric suctioning
The ureter runs anterior to
Iliac vessels
Ureter runs posterior to
Uterine artery
Symptoms of recurrent fever and abdominal pain are indicative of
Recurrent pyelonephritis
How does VUR lead to secondary hypertension
Renal scarring of upper and lower poles of kidney causes loss of kidney function and water retention
Presents as bilateral hydronephrosis and calyceal dilation due to obstruction of urine flow in urethra in males due to malformed Wolfffian duct
Posterior urethral valves
Presents as rapid onset vision changes, encephalopathy and renal failure
Malignant hypertension
Adolescent presenting with neuropathic pain (burning sensation in palms and soles), decreased sweat, red macules and papules and renal failure likely has
Fabry disease
IgA-mediated leukocytooclastic (hypersensitivity) small vessel vasculitis that produces colicky abdomoinal pain, hematuria and palpable purpura on buttocks and legs
Henloch-Schonlein purpura
Patients with Henloch-Schonlen purpura have increased risk of
Intussusception
In the true pelvis, the ureters lie medial to ___ and anterior to ___
ovarian vessels; internal iliac artery
Two side effects of erythropoesis stimulating agents
Hypertension and increased risk of thromboembolic events
Which condition appears hypercellullar with thickening and splitting of glomerular basement membrane due to subendothelial immune complex deposition on light microscopy
Membranoproliferative glomerulonephritis (associated with Hep C)
Can develop secondary to HIV infection, heroin abuse and severe obesity
Focal segmental glomerular sclerosis
Capillary wall thickening and subepithelial deposits (“membrane spikes”) associated with
Membranous glomerulonephritis (lung, breast, prostate, colon cancer)
Nephrotic presentation characterized by
Heavy proteinuria and fatty casts
Nephritic syndrome characterized by
Hematuria, red cell casts, variable proteinuria
In a patient with CKD, what causes fatigue, weakness and itching?
Accumulation of uremia toxins
In which two conditions do you typically see hyaline arteriosclerosis
poorly controlled hypertension and diabetes mellitus
Onion skinning, fibrinoid necrosis, localized destruction of vascular wall with circumferential ring of pink, amorphous material surrounding the lumen
Malignant hypertension
What causes foamy urine in nephrotic syndrome?
To compensate for loss of albumin, liver increases lipoprotein synthesis, which escapes in urine and makes it appear foamy
Biopsy showing marked ballooning and vacuolar degeneration of proximal renal tubules likely indicates
Acute tubular necrosis
Ethanol intoxication increases risk of which kidney stones?
Calcium oxalate
Linear deposits of IG along glomerular basement membrane are characteristic of
Anti-glomerular basement membrane (anti-GBM) disease
Pulmonary hemorrhage and shortness of breath in anti-GBM syndrome occur because
Anti-GBM antibodies cross-react with collagen IV in pulmonary avleolar basement membrane
Granular deposits on IF and membrane splitting on light microscopy are indicative of
Membronoproliferative glomerulosclerosis
Blood supply to proximal ureters
Renal arteries
Blood supply to distal ureters
Common iliac, internal iliac and superior vesical arteries
Crescent formation without immunoglobulin or complement deposition on glomerular basement membrane suggest
Pauci-Immune RPGN (antineutrophil cytoplasmic antibodies);
Which conditions is Pauci-immune RPGN associated with?
Granulomatis with polyangiitis or microscopic polyangiitis
New onset left varicocele in old man with flank pain and hematuria raises suspicion of
Renal vein thrombosis
How does infectious endocarditis lead to glomerular nephritis?
Deposition of circulating immune complexes in glomerular capillary wall
Three mechanisms that stimulate renin release from JGA cells
1) Decreased tubular NaCl sensed by macula densa
2) Decreased pressure in afferent arteriole
3) SNS stimulation
Most common causes of nephrotic syndrome in adults. Appears thick BM on Jones methenamine silver stain with subepithilal spikes
Membranous nephropathy
