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