Renal. Polycystic Flashcards
PKD. how is characterized?
by the presence of progressive cystic dilation of renal tubules.
PKD. classification - AD ir AR
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PKD. which type most common?
AD PKD.
PKD. ADPKD - symptoms?
usually asymtomatic until age 30 y/o (as cysts gradually enlarge with time)
PKD. ADPKD - age?
symtoms occur after 30 y/o, about 10 proc. present in childhood
PKD. ADPKD in what other organs? 4
liver, pancreas, spleen, epididymis
PKD. ADPKD in what proc and when need dialysis?
in 50proc. of AD PKD patients ERDS to require dialysis by 60 y/o
PKD. ADPKD. what symptoms if not ERDS?
in other 50 proc. have mildly reduced renal function and only require supportive care and BP control
PKD. AR PKD. how common and severe?
less common than AD, but more severe
PKD. usually asymtomatic until age 30 y/o (as cysts gradually enlarge with time)?
AD PKD
PKD. Affect liver, pancreas, spleen, epididymis?
AD PKD
PKD. AR PKD in what population?
infants and young children.
PKD. AR PKD manifestation?
renal failure, liver fibrosis, portal hypertension. (IN CHILDREN)
PKD. AR PKD. in what case can be death?
ARPKD can lead to death in the first few days of life if assoc. with IN UTERO OLIGURIA (oligohydramnios) leading to Potters sequence
PKD. can lead to death in the first few days of life if assoc. with IN UTERO OLIGURIA (oligohydramnios) leading to Potters sequence?
AR PKD
PKD. Which in children?
AR PKD
PKD. which present as renal failure, liver fibrosis, portal hypertension?
AR PKD
PKD. key fact. Patient has PKD and abruptly develops headache. in which type and what suspect?
AD PKD
suspect subarachnoid hemorrhage
PKD. AD PKD. symptoms?
HTN, bilateral palpable masses, flank pain, history of UTI or gross hematuria.
PKD. AD PKD. symptoms - what additional?
polyuria and nocturia
PKD. AD PKD. symptoms - why pain? 3
due to rupture of cyst, infection or passage or stona
PKD. HTN, bilateral palpable masses, flank pain, history of UTI or gross hematuria.?
AD PKD.
PKD. pain due to rupture of cyst, infection or passage or stona
AD PKD.
PKD. polyuria and nocturia
AD PKD.
PKD. AR PKD - symptoms?
HTN, abdominal distension, flank masses - most commonly presenting findings. IDENTIFIED PRENATALLY.
PKD. AR PKD - symptoms - in what age identified?
IDENTIFIED PRENATALLY.
PKD. in both - what symptom?
may have large, palpable kidneys on abdominal examination.
PKD. what finding does not suggest AD or AR PKD?
A single, simple renal cyst
it does not require further evaluation
PKD. Evaluation instrumental?2
UG (most common) or CT scan
PKD. what is seen on instrumental?
multiple bilateral cysts will be present throughout the renal parenchyma and renal enlargement vill be visualised.
PKD. need genetic evaluation?
available but often not necessary.
AD PKD - PKD1 and PKD2 (called polycystins)
AR PKD - PKHD
PKD. screening criteria?
> 18 y/o + family history –> may be offered screening ultrasound
PKD. treatment main idea?
prevent complications and decr. rate of progression to ESRD.
PKD. a) early management of UTI to prevent renal cyst infection.
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PKD. b) BP control (ACEI, ARBs) - to reduce HTN induced renal damage and control proteinuria
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PKD. c) decr. sodium intake
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PKD. d) lipid control with satin - to decr. CV risk.
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PKD. when dialysis?
dialysis ir transplantation in ERDS
PKD. high fluid why give?
High fluid intake needed to prevent kidney stones and may be helpful to prevent cyst development since ADH stimulate cyst growth.
PKD. what medication may be used?
Vassopressin rec. antagonist are also been approved for use.
UW. AD PKD. clinical presentation?
asymtomatic untill 30-40yo
Flank pain, hematuria, HTN, palpable mases bilateral, CKD
UW. AD PKD. extrarenal presentation?
cerebral aneurism, hepatic and renal cysts, mitral valve prolaps, aortic regurgitation, colonic diverticulosis, ventral and inguinal hernias
UW. AD PKD. diagnosis?
UG shows multiple renal cysts
UW. AD PKD. management?
aggressive control of risk factors for CV and CKD
ACEI for HTN preffered
Hemodialysis, renal transplan ESRD
UW. AD PKD. flank pain - why may be?
due to cystic rupture or nephrolithiasis
UW. AD PKD. HTN mechanism?
cyst enlargement –> localized renal ischemia –> incr. secretion of renin
UW. AD PKD. why poliuria and nocturia?
due to cystic damage to nephron distal tubules with impaired sensitivity toward vasopressin signals –> urinary concentrating defect (ie mild nephorgenic DI)
UW. AD PKD. renal dysfunction to CKD - why?
due to cystic progression
UW. AD PKD. cerebral aneurysm. mechanism how occurs?
disruption of polycystins cause impaired vascular integrity, leading to cerebral aneurysm
UW. AD PKD. cerebral aneurysm. what may contribute?
HTN also contributes by facilitating aneurysm progression and rupture