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57 Cards in this Set
- Front
- Back
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Genetic association with infantile PKD
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-autosomal recessive
-PKHD1 gene produces fibropolycystin -controls cell regulation and adhesion |
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morphology of kidney in infantile PKD
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-Large, normal shape
-diffuse radially oriented cysts in collecting ducts |
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Infantile PKD death due to?
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Womb: respiratory insufficiency, oligohydramnios
Infancy: renal failure, hepatic fibrosis |
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Organs involved in infantile PKD
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-kidney
-lung hypoplasia -biliary dysgenesis/hepatic fibrosis (progresses w/time) |
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Genetics of adult PKD
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-autosomal dominant
-new mutation in some -PKD1 gene on chromosome 16 -polycystin gene product (cell proliferation, adhesion) |
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How common is adult PKD?
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most common inherited cause of renal failure
-8-10% of pts with ESRF |
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Location of cysts in infantile PKD
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collecting ducts
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Location of cysts in adult PKD?
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anywhere along the nephron
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Renal morphology in adult PKD
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bilateral spherical cysts take over normal shape by 40yrs
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Amount of nephrons affected in adult PKD?
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1-2% of total
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Pathology in adult PKD
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-proliferation of intratubular cells obstructs tubules and creates cysts
-cysts compress adjacent structures -cytokines destroy good portions |
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Other organs involved in adult PKD
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-cysts in liver (30-50%), pancreas, spleen, lung
-liver fibrosis -berry aneurysms in COW (15%) |
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Clinical presentation of adult PKD
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-flank masses/pain
-HTN -hematuria -UTI -renal fail (late) |
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Pathology of medullary sponge kidney
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-Cysts form in the collecting ducts of the medulla
-Contrast shows bottle brush appearance |
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Complications of medullary sponge kidney
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-asymptomatic in young
-infection (flank pain, dysuria, hematuria) |
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Pathology of nephronophthisis/Medullary cystic dz complex
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-Tubular basement membrane defect causes atrophy of tubules
-small cysts -salt wasting, defective urine concentration |
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Genetic association of nephronophthisis
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50% autosomal dominant/recessive
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dialysis kidney
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-cortical and medullary cysts w/papillary hyperplasia in pts on renal dialysis
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concerns with dialysis kidney
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renal carcinoma
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Who gets simple cysts?
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50% of adults over 50yrs
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Morphology/location of simple cysts
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-cortical
-delicate, fluid filled -flattened epithelial lining |
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symptoms of simple cysts
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none
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Complications with simple cysts
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-hemorrhage
-infection -diff to distinguish from cystic renal carcinoma |
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when does mesonephros appear?
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week 4 of development
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when does metanephros appear?
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week 5 of development; functioning 4 wks later
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Derivatives of the mesonephric duct
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-ureteric bud -> definitive kidney
-trigone of bladder -vas deferens, seminal vesicles -Gartner's duct cysts |
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derivatives of the mesonephric tubules
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epididymis
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Formation of the kidney
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1) mesonephric duct -> ureteric bud
2) bud -> metanephric mesoderm 3) condensation of metanephric blastema around bud 4) blastema -> nephrons and stroma 5) bud -> ureter, renal pelvis, calyces, collecting ducts |
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What is formed by the ureteric bud?
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renal pelvis
calyces ureter collecting ducts |
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what is formed by the metanephric blastema?
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nephrons
stroma |
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when are glomeruli produced?
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gestation weeks 8-36
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where are the youngest glomeruli?
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supcapsular, near the cortex
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How many people have urinary tract abnormalities?
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5-10%
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Complications of bilateral renal agenesis
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-fetal death
-oligohydramnios/Potter sequence -lung hypoplasia |
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Complications of ureteral duplication
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-obstruction
-reflux -hydronephrosis -kidney damage -ureterocoele if 2nd ureter enters at an abnormal site |
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Complications of pelvic ectopic kidney
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can obstruct delivery in pregnancy
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crossed renal ectopia with fusion
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Kidneys are fused on 1 side, both ureters enter bladder at normal sites
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horseshoe kidney
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fusion of lower poles @ midline
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complications of horseshoe kidney
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-partial ureter obstruction
-urinary stasis -infection |
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hydronephrosis
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dilated renal pelvis and calyces
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common site of renal obstruction
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junction of the ureter and pelvis
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what is bladder exstrophy?
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-bladder is open w/mucosa exposed to anterior abdominal wall
-assoc w/epispadias or genital tract malformations -painful |
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concerns re: bladder exstrophy
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squamous carcinoma or adenocarcinoma of the bladder surface
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cause of bladder exstrophy
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-failure of mesoderm migration into anterior abdominal wall and ant bladder wall
-rupture and exposure of bladder mucosa |
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patent urachus?
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Patent urachal duct. No formation of the medial umbilical ligament
-urine leaks thru umbilicus |
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epispadias
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no closure of the urethra on the upper surface of the penis/clitoris
-assoc w/bladder exstrophy |
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hypospadias
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-common
-urethra ends in a groove on the underside of the penis -genital tubercles don't copletely close around the urethra |
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posterior urethral valves
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prevent urine flow down the urethra but allow catheter insertion
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complications of posterior urethral valves
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-oligohydramnios in utero
-bladder hypertrophy -hydroureter -hydronephrosis -renal dysplasia |
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Pathology of renal dysplasia
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-random disorganization
-increased connective tissue -decreased nephrons -multiple random cysts w/in tubules -cartilage lobules -unilateral or bilateral |
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complications of renal dysplasia
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-obstruction of urine flow in fetus
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most common cystic dz in kids
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unilateral cystic renal dysplasia
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cause of abdominal mass in neonates
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unilateral cystic dysplasia
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complications of unilateral hydronephrosis
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-increased infection
-developmental dysplasia |
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complications of bilateral renal agenesis
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-oligohydramnios
-Potter sequence (flat nose, eye creases, low set ears, pulm, limb) -club feet/hands -pulmonary hypoplasia |
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oligohydramnios sequence
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-Potter face
-limb deformation -lung hypoplasia |
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Does bilateral renal agenesis cause renal insufficiency in the infant?
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No. Mother's kidney makes up for the deficit
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