RENAL 4 Flashcards
agnesis
hypoplasia
horseshoe kidney
absence of one or both kidneys
small tumour but normal development
fusion at either pole
autosomal dom type 1
type 2
chromosome 16 commonest develop ESRF faster
chrom 4
symptoms of ad PKD
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
complications of PKD
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
ix for PKD, treatment
USS first line. Genetics
hypertension - target <130/80
hydration, decrease protein, tolvaplatan, if in renal failure - dialysis/transplant
children AD PKD
renla involvement similar
cerebral aneurysms rare
autosomal recessive who
genetics
assoc with what
signs
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
ix for AR PKD
USS first line
CT/MRI
alports syndrome is what genetics disorder of what symptoms ix rx
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
anderson fibres disease is what affects what ix presentation rx
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
medullary cystic kidneys genetics
symp
ix
rc
ADom around 28yo
cortex and medulla shrink
FH, CT
renal transplant
medullary sponge kidney genetics
in severe
ix
prognosis
sporadic inheritance
medullar looks like a sponge. cysts have calculi
excretion urography to demonstrate calculi
renal failure unusual
HLA is what
types in renal transplantation
suitabiliy
human leukocyte antigen matching surface proteins
DR > B > A
reasonable life expectancy
renal transplant absolute contraindications
malignancy, untreated TB, severe IHD, severe airways disease, vasculitis, severe PVD
renal transplant surgery
exztraperitoneal procedure 3-4hours, wound 15-20 mins long
hyper acute rejection time period
due to what
management
mins to hours
due to pre-existent ABs against donor HLA
unsalvageable - remove kidney
acute rejection time period
due to what
management
<6 months
T or B cell mediated
immunosuppression
chronic rejection time period
due to what
management
> 6 months
immunological and vascular
deterioration of transplant - no management
cyclosporin and tacrolimus
renal dysfunction, hypertension, DM, tremors
azathioprine and mycophenolate
leucopenia, anaemia, infection, DM
steroids
osteoporosis, DM, weight gain, infection
what is the leading cause od ESRF
diabetes
what formation in DM
ix
rx
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
what is renovascular hypertension
secondary form of hypertension caused by RAS which decreased renal perfusion leading to increase in systemic BP
fibromuscylar dysplasia in who
familial in how many
assoc with what
can involve what
F 15-50s
10% - both arteries
other hereditary conditions
cerebral arteries
atherosclerotic renovascular disease in who
risk factors
symptoms
men 50+
general atherosclerosis
renovascular hypertension, AKI after treatment of hypertension usually with ACIE, CKd, flash pulmonary oedema, haematuria, renal bruit
ischaemic nephropathy is what
decreased GFR wit decreased renal blood flow -> renal atrophy and progressive CKD
ix for renovascular htn
rx
CX
screening. USS. r artery duplex, CT, MRI, angiography
angioplasty +/- stenting or stent alone
ACEI contraindicated in bilateral RAS
myeloma and renal involvement
ix
pathogenesis 3
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
primary amyloidosis
secondary
ix
AL - myeloma
AA
positive red congo staining shows apple green birefringence. serum amyloid precursor scan
treatment of renal involvement in vasculitis
immunosuppression
plasma exchange
renal support
SLE how many will have renal involvement at presentation and how d they present with ti
proteinuria
50%
