renal path flashcards
What renal conditions area associated with sickle cell trait
Autosomal dominant Polycystic Kidney Disease (associated with accelerated disease in black patients with sickle cell trait) and Real Medullary Carcinoma
Horse shoe kidney
90% of cases have fusion of upper pole - patients are usually asymptomatic/normally functioning
Presentation of ADPKD
Cysts not present at birth, renal function intact until the 4th or 5th decade when patients become symptomatic. Inscidious onset of hematuria (1st symptom) followed by polyuria and hypertension (Kiddos- HTN and hematuria)
cysts in the liver, spleen and lungs are assocaited with what renal condition
ADPKD
Intracranial berry aneuysms are associated with what renal condition
ADPKD
Genetic component of ADPKD
PKD1 (85%) and PKD2- PKD2 presents later than PKD1
how does the pathology of ADPKD differ from that of ARPKD
ADPKD= dilation of all parts of the nephron ARPKD= dilation of the collecting tubules
ADPKD differential Diagnosis RCC
RCC R/O based on the fact that ADPKD is bilateral rather than unilateral and the cysts are NONuniform
“Tufting of papillary projections”
ADPKD
what renal condition presents with dilated elongated channels at right angles to the cortical surface
ARPKD
Newborn presents with severe respiratory distress and pulmonary hyperplasia - what renal condition
ARPKD. Pulmonary Hyperplasia is secondary to oligohydramnios
Clinical presnetation of ARPKD
Liver and Kidney most often involved (lungs in neonates) , most present before age 20, Hypertension in almost all cases, liver disease predominates in older children and adults (portal hypertension and splenomegaly)
What cystic disorder is associated with portal hypertension (portal fibrosis)
ARPKD
Buzz word: Fetal lobulation
ARPKD
MC cystic disease in children
Multicystic Dysplastic Kidney
what renal condition has a microscopic appearnce discribed as “Disorganized with dilated tubules with cuffs of primative stroma and island of cartilage”
Multicystic Dysplastic Kidney
what cystic condition is confined to the medullary pyramids and papillae
Medullary sponge kidney
How can you differntiate medullary sponge from PKD
Medullary sponge has intersitial fibrosis
Most common genetic cause of ESRD in children and young adults
Nephrophthisis
Genetic mutation in ARPKD
Polycystic Kidney Hepatic Disease gene (PKHD1)
what type of Nephrophthisis is most common
Familial (juvenile) - becomes clinically evident in childhoood or adolescence.
what cystic condition has cysts most prominent at the corticomedullary junction
Nephrophthisis
Polyuria, polydipsia, cardiac, musculoskeletal, and ocular disease
Nephrophthisis
what renal disease causes growth failure in children
nephrophthisis
Presnetation of Adult onset medullary cystic kidney disease
Present in 3rd -4th decade with polyuria and polydipsia, may also have hyperuricemia and gout
“tubular atrophy interspersed with hypertrophied and dilated tubules”
Adult onset medullary cystic disease
What cystic disease was initially descriebed in patients undergoing dialysis but can also be seen in uremic nondialysized patients
Acquired cystic renal disease
cysts filled with straw colored hemorrgagic fluid and often contain calcium oxalate crystals
acquired cystic renal disease
Most common cystic abnormality of the kidney
Simple Cyst
Name the nephritic diseae
Acute proliferative glomerularnephritis, Rapid progressive glomerular nephritis, IgA nephropathy, Alport Syndrome Membranoproliferative Glomerulonephritis
Buzz word: Subepithelial humps
Acute proliferative glomerulonephritis
7 year old Patient presents with painless gross hematuria 3 weeks after having a sore throat
Acute proliferative glomerulonephritis MCC = Group A strep pharyngitis
Biopsy shows hypercellularity due to infiltration and proliferation of endothelial , mesangial, and epithelial cells
Acute proliferative glomerulonephritis
Cresents seen on biopsy
Rapid progressive Glomerular nephritis
RPGN gross morphology
kidney is large and pale with petichial hemorrage
most common type of GN world wide
IgA nephropathy
recurrent symptomatic hematurua after respiratory infection and absence of systemic disease
IgA nephropathy
Buzz word: Synpharyngitic macroscopic hematuria
igA nephropathy
Biopsy shows mesangial exapansion without significant involvement of capillary walls or lumina
iga nephropathy
Renal Disease, Sensorial Hearing loss, Ocular abnormalities
Alport Syndrome “Cant see, cant pee, cant hear a bee”
what differnetiates Aloprt Syndrome from IgA nephropathy
will always have microscopic hematuria with Alport syndrome. Microscopic hematuria will be abscent between illnesses in IgA nephropathy
what renal condition is caused by abnormal type IV collagen
Alport Syndrome
what renal condition is assoaciated wth Hepatitis C
Membranoproliferative Glomerulonephritis
Buzz word: tram tracking
Membranoproliferative Glomerulonephritis
what types of eposits are seen in type I memranoproliferative GN
Subendithelual deposits between duplicated membranes
what type of deposits are seen in type II membranoproliferative GN
Intramembranous dense deposits = Ribbon like deposits
Name the nephrotic diseases
Membranous Nephropathy, Focal Segmental GN, Diabetic nephropathy Minimal change
Most commmon casye of adult nephrotic syndrome in adults and elderly
membranous GN
What glomerular disease is associated with malignacy
membranous GN
Biopsy shows spikes (progression of basement membrane) near subepithelial deposits and no increase in cellularity
membranous GN
Most common GN leading to ESRD
Focal segmental GN
MCC of focal glomerulonephritis
Obesisty and anabolic steroid use
what would you see on biopsy of FSG
Hyalin insudation and lipid vacuoles in sclerotic areas
Biopsy shows hyaline arteriolosclerosis and basement membrane thickening of tubules and glomerular capillaries
Diabetic Glomerulonephropathy
Buzz word: Nodular glomerulosclerosis or Kinnelstiel Wilson Disease
Diabetic Glomerulonephropathy
buzz word: effacement of podocytes, normal appearing glomerular biopsy
Minimal change disease
most common casue of acute renal failure
Acute tubular injury (tubular necrosis)
what sections of the tubules are sensitive to ischemia and toxins
straight portions of the proximal tubule and medullary thick ascending loop of henle
Biopsy shows eosinophilic hyaline casts and pigmented granular casts (muddy brown casts)
Acute Tubular injury (tubular necrosis)
Biopsy of acute tubular injrut from ethylene glycol
ballooning and hydrophobic or vacuolar degeneration of proximal convoluted tubules. Calcium oxide crystals in tubular lumen
what condition causes intertubular aggregates of neutrophils and neuetrophilic tubulitis with neutrophil casts
Acute pyelonephritis
Buzz word: thyroidization
Chronic pyelonephrits
second most common cause of acute kidney injury after pyelonephritis
drug and toxin induced tublointersitial nephritis
Initial phase of Acute tubular injury
lasts 36 hours - period when patient exposed to ischemia/toxin and parenchymal injury is developing but not yet established.
Maintainance phase of acute tubular injury
Uremia, salt and water overload, rising BUN, HYPERkalemia, metabolic acidosis
Recovery phase of acute tubular injury
diuretic phase large amount of salt and water lost, HYPOkalemia becomes a problem
How does the infiltrate differe in acute vs chronic tubular nephritis
Acute: Leukocyte infiltration (mainly neutrophils and eosinophils) Chronic: inflammation mainly monocytes
what is the most common cause of acute pyelonephritis
Ascending infection (usually E.coli)
name the complications of acute pyelonephritis
Papillary Necrosis, Pyonephrosis, Perinephric abscess
what population is papillary necrosis normally seen in
complication of acute pyelonephritis seen in people with diabetes, sickle cell disease, urinary obstruction, also seen in NSAIDs
describe the gross morphology of papillary necrosis
tips or distal 2/3 of the pyramids have areas of gray/white to yellow necrosis
pyonephrosis
suppurative exudatecannot drain (due to obstruction) and fills the renal pelvis, calyces, and ureter with pus
Acute Uric Acid Nephropathy
precipitation of uric acid in renal tubules and development of acute renal failure - seen in leukemic patients undergoing chemotherapy (cancer cell is killed and uric acid us released- tumor lysis syndrome)
Buzz word: tumor lysis syndrome
Acute Uric Acid nephropathy
Chronic urate nephropathy
Gouty nephropathy- monosodium urates deposit in the distal tubules and collecting ducts and interstitium and form birifringent needle like cysts
what renal conditions are associated with calcium phosphate deposition
Medullary Sponge Kidney and Hypercalcemia and Nephrocalcinosis
Buzz word: Bence Jones Protien
Light chain cast nephropathy (myeloma kidney)
biopsy descrived as Angulated and tubular casts surrouned my macrophages (some of them multinucleated)
Light chain cast nephropathy (myeloma kidney)
what is nephrosclerosis
Sclerosis of renal arterioles and small arteries usually in the setting of HTN that results in parenchymal ischemia and glomerulosclerosis that ultimately shrinks the kidney
Buzz word: Cortical surface described as grainy leather
Nephrosclerosis (fine even granularity)
Athromathous plaque vs fibromuscular dysplasia
fibromuscular dysplasia havs the lumen still in the center
inflammation in the setting of cortical infarct is a response to what
Inflammation is in response to the necrosis NOT the cause of the necrosis
Buzz word: Schistocytes
Thrombotic Microangiopathies - result from shearing of red blood cells
Most commin Genitourinary tumor in children
Wims (Nephroblastoma)
name the benign renal tumors found in adults
Renal Papillary Adenoma, Renal oncocytoma, Angiomyolipoma
Name the malignant renal tumors found in adults
Clear cell renal cell carcinoma, Papillary renal cell carcinoma, Chromophobe renal cell carcinoma, Collecting duct (Bellini duct) carcinoma, renal medullary carcinoma, urothelial carcinomal or the renal pelvis
MCC of ischemia
coronary atherosclerosis (90%)
MC Acute coronary syndrome
Angina pectoris
angina pectrois is most common in what population
middle aged men and women after menopause
most common variant of angina
Stable Angina
T/F Stable angina is associated with plaque disruption
False
What type of angina is relieved by rest or vasodilator
Stable angina
what typically causes unstable angina
disruption of a plaque and superimposed partial thrombus, and probably embolis or vasospasm (or both)
Early response to biochemical response
within seconds - lactate levels rise and ATP falls (due to lact of oxygen and cessation of aerobic metabolism)
loss of myocte contractility happens within what time frome
within 60 seconds
early changes are potentially reversible within what time period
20-30 minutes - ischemia > 1 hour causes damage to cardiac microvasculature
Most MI are what type
Transmural
Progression of Myocardial Necrosisi
Necrosis begins in small zone of myocardium beneath the endocardial surface in the center of the ischemic zone. VERY NARROW ZONE OF MYOCARDIUM BENEATH THE ENDOCARDIUM IS SPARED FROM NECROSIS DUE TO DIFFUSION OF OXYGEN FROM THE VENTRICLE
most common cause of death in adults in the US
MI
when do gross findings of MI first appear
12-24 hours: Dark Mottling of infarct and central pallor
infiltrate of abundant neutrophils are seen when (post MI)
1-3 days
well developed phagocytosis of dead myoctes occurs when (post MI)
7-10 days
MI scar begins to form at ____ and is complete at ____
begins at 2 weeks and is complete at 8 weeks
Buzz word: Contraction bands
indicative of reperfusion injury post MI
Creatinkin phosphokinase dimer specific for cardiac muscle
CKMB
when does CKMB appear in the blood, peak, and return to baseline
appears 2-4 hours after MI, peaks at 24 hours and returns to baseline at 36
what lab test is most sensitive and specific for myocardial damage
Troponin (T and I)
when does troponin appear in the blood, peak, and return to baseline
appears 2-4 hours after MI, peaks at 48 hours, and persists for 10-14 days post MI
what morphological features are seen with chronic ischemic heart disease
left ventricular hypertorphy and ventricular dilation
congenital heart disease occurs when
3-8 weeks gestation
major known cause or congenital heart disease
sporadic genetic abnormalities
almost all congenital abnormalities predispose for what condition
bacterial endocarditis - abnormalities cause turbulent flowt that can damage endocardium
Most common congenital heart condition
Ventricular septal defect
what classification of shunt is cyanotic
Right to left
what classification of shunt is acyanotic
left to right
name the L to R disorders
“all have a D in them” ASD, VSD, PDA, AVDS
most common site of ASD
foramen ovale
most common congenital heart condition presenting in adulthood
ASD
name the R to L disorders
“All have a T in them” Tetrology of fallot, transposition of the great vessels, tricupsid atresia, patent truncus arteriosus, total anomolous venous connection/reutrun
what congenital heart disorder is associated with down syndrome
AVSD
4 characteristics of tetrology of fallot
1: VSD 2: Overriding Aorta 3: RVH 4: Pulmonic stenosis
buzz word: boot shaped heart
tetrology of fallot
infant turns blue when crying
tetrology of fallot
child squats during exercise
tetrology of fallot - squatting increases venous return to the heart
what congenital heart disorder is associated with women with turner syndrome
coartation of the aorta
what are the obstructive congenital heart anomalies
congenital aortic stenosis, congenital pulmonic stenosis, coarctation of the aorta
congenital heart disease: bp in upper extremity elevated and low in the lower extreity
coartation of the aorta
hypertrophic osteoarthropathy is associated withw hat
Right to left shunts - clubbing fo fingers (tips of fingers expand and blunt)
