Kidney: Anatomy/ Congenital Disorders/ Acquired Cystic Disease Flashcards
“fenestrated epithelial cells”
podocytes
“modified smooth muscle cells”
mesangial cells
What molecules make up the GBM?
Type IV collagen and GAGs
What cells are important in glomerular inflammation and thrombosis?
Glomerular epithelial cells
Where are podocytes located?
rest on the outer aspect of the GMB
What are foot processes?
cytoplasmic projections from podocytes onto the lamina rara externa of the GMB
What are the functions of the mesangium?
5 functions
1- mechanical support
2- endocytosis and processing of plasma protein
3- maintenance of GMB and matrix
4- modulation of glomerular filtration
5- generation of molecular mediators
What comprises the JG apparatus?
Macula densa, mesangial cells, terminal afferent and proximal efferent arterioles
What do cortical interstitial cells secrete?
EPO
“insufficient amniotic fluid, compressed fetus, low ears, small chin, beak node, pilmonary hypoplasia”
Potter sequence
“complete absence of renal tissue”
renal agenesis
Difference between unilateral and bilateral renal agenesis?
unilateral–> asymptomatic and existing kidney undergoes hypertrophy
bilateral–> still born, link with potter sequence
Unilateral renal agenesis is at increased risk for….
Progressive glomerular sclerosis
“few than 6 renal lobes”
Renal hypoplasia
“enlargement of renal lobules when there are too few lobules”
Oligomeganephronia
Most common location of an ectopic kidney?
pelvis
“conjoined kidneys usually in the lower abdomen”
Horseshoe kidney
Most common renal congenital anomaly?
Horseshoe kidney
“undifferentiated tubules and primitive mesenchyme, cysts may be present”
renal dysplasia
What is the most common abd mass in newborns?
Multicystic renal dysplasia
“inherited defect leading to bilateral enlarged kidneys with cysts in the renal cortex and medulla”
PKD
Who gets the AR form of PKD?
infants
How does AR PKD present?
worsening renal failure ad HTN, potter sequence possible
Hepatic cysts/ fibrosis are also possible
Who gets the AD form of PKD?
Adults
How does AD PKD present?
Young adults with HTN, hematuria, worsening renal failure
Berry aneurysms, hepatic cysts, mitral valve prolapse
PKD1/2 gene is involved in…
primary cilia or tubular epithela cell cell adhesion complexes
so without tubules are abnormal and undifferentiated
Arrangement of cysts in AR PKD?
Radial arrangement
“AD inherited defect leading to cysts in the medullary Collecting Ducts”
medullary cystic kidney diseases
Gene in medullary cystic kidney diseases?
MCKD 1/2 mutation
Symptoms of nephrothisis and medullary cystic disease?
polyuria, polydipsia, enuresis, azotemia, renal failure/ ESRD, atrophic tubules thickened and laminated BM, sclerosis fibrosis, and inflammation
“multiple small cysts in the papillae that arise from CDs
medullary sponge disease
How does a person get acquired cystic disease?
long term dialysis
Acquired cystic disease leads to an increased risk of…
Renal cell carcinoma
