RENAL 4

Question 1

agnesis
hypoplasia
horseshoe kidney

Answer 1

absence of one or both kidneys
small tumour but normal development
fusion at either pole

Question 2

autosomal dom type 1

type 2

Answer 2

chromosome 16 commonest develop ESRF faster

chrom 4

Question 3

symptoms of ad PKD

Answer 3

large kidneys. decreased urine concentration ability, chronic pain, hypertension, haematuria, distortion of reniform shape, cysts in liver, pancreas, lungs and ovaries with no function effect

Question 4

complications of PKD

Answer 4

distended cysts - chronic pain
bleeding into cyst - pain and haematuria
cyst infection, intracranial aneurysms, mitral/aortic valve prolapse, diverticulitis and colonic perf, increased risk of hernias - abdominal and inguinal

Question 5

ix for PKD, treatment

Answer 5

USS first line. Genetics
hypertension - target <130/80
hydration, decrease protein, tolvaplatan, if in renal failure - dialysis/transplant

Question 6

children AD PKD

Answer 6

renla involvement similar

cerebral aneurysms rare

Question 7

autosomal recessive who
genetics
assoc with what
signs

Answer 7

young children/infants
chrom 6
congenital hepatic fibrosis
bilateral symmetrical. urinary tract normal. cysts appear from CD, reniform shape retained, no gross distortion of kidneys, slow decline in GFR, htn

Question 8

ix for AR PKD

Answer 8

USS first line

CT/MRI

Question 9

alports syndrome is what 
genetics 
disorder of what 
symptoms 
ix
rx

Answer 9

hereditary nephritis
x lined recessive
type 4 collagen matrix
haematuria, proteinuria, SNHL, anterior lenticonus, renal failure

renal biopsy: variable thickness GMB. EM: splitting of lamina dennis

decrease BP decrease proteinuria, dialysis, transplant

Question 10

anderson fibres disease is what 
affects what 
ix
presentation 
rx

Answer 10

inborn erros if glycophingueolipid metabolism
x linked liposomal storage diaease

kidneys, lungs, liver, erythrocytes

plasma/leukocyte alpha GAL activity. renal/skin biopsy
renal failure. angiokeratoma, CM, valvular disease, stroke, acroparasthesia, psychiatric

enzyme replacement - fabryzyme. manage cx

Question 11

medullary cystic kidneys genetics
symp
ix
rc

Answer 11

ADom around 28yo
cortex and medulla shrink
FH, CT
renal transplant

Question 12

medullary sponge kidney genetics
in severe
ix
prognosis

Answer 12

sporadic inheritance
medullar looks like a sponge. cysts have calculi
excretion urography to demonstrate calculi
renal failure unusual

Question 13

HLA is what
types in renal transplantation
suitabiliy

Answer 13

human leukocyte antigen matching surface proteins
DR > B > A
reasonable life expectancy

Question 14

renal transplant absolute contraindications

Answer 14

malignancy, untreated TB, severe IHD, severe airways disease, vasculitis, severe PVD

Question 15

renal transplant surgery

Answer 15

exztraperitoneal procedure 3-4hours, wound 15-20 mins long

Question 16

hyper acute rejection time period
due to what
management

Answer 16

mins to hours
due to pre-existent ABs against donor HLA
unsalvageable - remove kidney

Question 17

acute rejection time period
due to what
management

Answer 17

<6 months
T or B cell mediated
immunosuppression

Question 18

chronic rejection time period
due to what
management

Answer 18

> 6 months
immunological and vascular
deterioration of transplant - no management

Question 19

cyclosporin and tacrolimus

Answer 19

renal dysfunction, hypertension, DM, tremors

Question 20

azathioprine and mycophenolate

Answer 20

leucopenia, anaemia, infection, DM

Question 21

steroids

Answer 21

osteoporosis, DM, weight gain, infection

Question 22

what is the leading cause od ESRF

Answer 22

diabetes

Question 23

what formation in DM
ix
rx

Answer 23

nodule formation - kimmel wilson
history of DM, proteinuria, presence of other diabetic complications
glycemic control, ACIE/ARB, statin, KP transplant in DMtype 1, K transplant, dialysis

Question 24

what is renovascular hypertension

Answer 24

secondary form of hypertension caused by RAS which decreased renal perfusion leading to increase in systemic BP

Question 25

fibromuscylar dysplasia in who
familial in how many
assoc with what
can involve what

Answer 25

F 15-50s
10% - both arteries
other hereditary conditions
cerebral arteries

Question 26

atherosclerotic renovascular disease in who
risk factors
symptoms

Answer 26

men 50+
general atherosclerosis
renovascular hypertension, AKI after treatment of hypertension usually with ACIE, CKd, flash pulmonary oedema, haematuria, renal bruit

Question 27

ischaemic nephropathy is what

Answer 27

decreased GFR wit decreased renal blood flow -> renal atrophy and progressive CKD

Question 28

ix for renovascular htn
rx
CX

Answer 28

screening. USS. r artery duplex, CT, MRI, angiography
angioplasty +/- stenting or stent alone
ACEI contraindicated in bilateral RAS

Question 29

myeloma and renal involvement
ix
pathogenesis 3

Answer 29

50% have renal imp at presentation and 10% require dialysis

normocytic anaemia, fouled formation, increased CRP/PV, protein electrophereios, BJP in urine

Aki secondary to hypercalcaemia, monoclonal IG deposition in disease, cast nephropathy

Question 30

primary amyloidosis
secondary
ix

Answer 30

AL - myeloma
AA
positive red congo staining shows apple green birefringence. serum amyloid precursor scan

Question 31

treatment of renal involvement in vasculitis

Answer 31

immunosuppression
plasma exchange
renal support

Question 32

SLE how many will have renal involvement at presentation and how d they present with ti

Answer 32

proteinuria

50%