Renal Flashcards
Adrenal cortex mnemonic?
GFR-ACD
- Zona Glomerulosa (outside) - mineralocorticoids (mainly Aldosterone)
- Zona Fasciculata (middle): glucocorticoids, mainly Cortisol
- Zona Reticularis (on inside): androgens, mainly Dehydroepiandrosterone (DHEA)
Summary of RAAS?
-
Renin
- Released by JGA cells in kidney in response to reduced renal perfusion, low sodium
- Hydrolyses angiotensin to form angiotensin I
- Stimulated by - low BP/low renal perfusion, hyponatraemia, sympathetic nerve stimulation, catecholamines, erect posture
- Reduced by - B-blockers, NSAIDs
-
Angiotensin
- ACE (lung) converts angiotensin I to angiotensin II
- Vasoconstriction –> increased BP
- Stimulates thirst and stimulates aldosterone/ADH release
-
Aldosterone
- Released by zona glomerulosa in response to raised angiotensin II, K+ and ACTH levels
- Causes retention of Na+ in exchange for K+/H+ in distal tubule
Urinalysis patterns?
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Calcium metabolism?
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Renal bone disease summary? And clinical manifestations?
- Osteitis fibrosa cystica
- AKA hyperparathyroid bone disease
- Adynamic
- Reduction in cellular activity (both osteoblasts and osteoclasts) in bone
- May be due to over treatment with vitamin D
- Osteomalacia
- Due to low vitamin D
- Osteosclerosis
- Osteoporosis
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Management of Renal Anaemia?
- Correction of iron with IV if needed
- Ferritin should be > 200 ng/mL before starting EPO
- EPO is used to target Hb 10-12 (>11 or hematocrit >33%) reach the target within 4 months
- Corrected Hb of > 13.5 is associated with HTN crisis
- Hb < 10.5 increased risk of seizures.
Side effects of EPO?
- HTN and HTN crisis, potentially encephalopathy and seizures (BP increased in 25% of patients)
- EPO induced seizures occurs after 90 days fro starting the treatment
- Bone aches
- Flu-like symptoms
- Skin rashes, urticaria
- Pure red cell aplasia* (due to antibodies against erythropoietin)
- Raised packed cell volume (PCV) = HCT –> increased risk of thrombosis (e.g. Fistula)
- Iron deficiency 2nd to increased erythropoiesis
Reasons why people fail to respond to EPO?
- Iron deficiency
- Inadequate dose
- Concurrent infection/inflammation
- Hyperparathyroid bone disease
- Aluminum toxicity
What mediates hyperacute graft rejection?
IgG
Graft survival stats?
Cadaveric
- 1 year = 90%
- 10 years = 60%
Living donor
- 1 year = 95%
- 10 years = 70%
Post-op problems for renal transplant?
Up to 4 months post-op (can cause graft dysfunction)
- Acute rejection: risk is great in 1st 2 weeks occurs in 30-50% of cases
- Ciclosporin toxicity
- ATN of graft
- Vascular thrombosis
- Urine leakage
- UTI
Management of acute (<6 months) graft failure?
Causes of chronic graft failure (>6 months)?
Acute
- Steroids
- If resistant use monoclonal antibodies
Chronic
- Chronic allograft nephrotherapy
- Ureteric obstruction
- Recurrence of original renal disease
Autosomal dominant polycystic kidney disease - types, diagnostic criteria?
Types
Loci = PKD1 and PKD2 - code for polycystin-1 and 2 respectively
Investigation/Criteria
Abdominal ultrasound
- Recommended after 20 years of age (risk of false -ve)
- <30 yrs - 2 cysts, unilateral or bilateral
- 30-59 yrs - 2 cysts in both kidneys
- >60 yrs - 4 cysts in both kidneys
Management
- Analgeisa
- Abx for UTIs
- HTN control
- Transplantation for ESRF
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Conditions associated with ADPKD?
- Colonic diverticula
- Mitral valve prolapse
Autosomal recessive PKD?
- Much less common than ADPKD
- Chromosome 6
- Dx - usually on prenatal USS or early infancy with abdo masses and renal failure
- ESRF in childhood
- Liver involvement
Nephrotic syndrome causes?
-
Glomerulonephritis (80%)
- Minimal change GN (75% children)
- Membranous GN
- Focal segemnental glomerulosclerosis
-
Systemic disease
- Amyloid
- SLE
-
Drugs
- Gold (sodium aurththiomalate), penicillamine
-
Others
- Congenital
- Neoplasia - carcinoma, lymphoma, leukaemia, myeloma
- Infection - bacterial endocarditis, hep B, malaria
- Renal vein thrombosis
Complications of nephrotic syndrome
- Infection due to urinary immunoglobulin loss
- Thromboembolism related to loss of antithrombin III and plasminogen in the urine
- Hyperlipidemia
- Hypocalcemia (vitamin D and binding protein lost in urine)
- Acute renal failure could be due to thrombotic renal veins - loin pain and haematuria.
Types of glomerulonephritis?
-
Membranous GN (Commonest type)
- Proteinuria/nephrotic syndrome/CRF
- Cause - infections, rheumatoid drugs, malignancy, SLE
- Biopsy - sub-epithelial immune complex deposition (IgG, C3)
- 1/3 resolve, 1/3 respond to cytotoxics, 1/3 develop CRF
-
IgA Nephropathy (Commonest worlwide cause of GN)
- Young adult with painless haematuria after UTI
- Associated with HSP, coeliac, dermatitis herpetiformis
- Mesangial hypercellularity, +ve immunofluoresence for IgA and C3
- Good prognosis - frank haematuria
- Poor prognosis - male, proteinuria, HTN, smoking, high lipids
-
Diffuse Proliferative GN
- Post-streptococcal glomerulonephritis in a child
- Nephritic syndrome/ARF
- Most common form of renal disease in SLE
-
Minimal Change Disease
- Child with nephrotic syndrome
- Causes - Hodgkin’s, NSAIDs
- Good response to steroids, next line cyclophosphamide
- Biopsy - podocyte fusion
- Rule of 1/3
-
Focal Segmental GN
- Idiopathic, or 2ndry to HIV/Heroin, Alport’s, Sickle cell
- Proteinuria/nephrotic syndrome/CRF
-
Rapidly Progressive GN (Crescenteric GN)
- Rapid onset –> ARF
- Causes - Goodpasture’s, ANCA +ve vasculitis (Wegener’s)
-
Mesangiocapillary GN (Membranoproliferative)
- Nephrotic syndrome, haematuria, proteinuria
- Poor prognosis
- Type 1 - cryoglobulinaemia, hepatitis C –> low C4
- Type 2 - partial lipodystrophy –> low C3
Alport’s sydnrome?
- X-linked dominant
- Defect in gene coding for type IV collagen –> abnormal GBM
- More severe in males
Presentation
- Childhood
- Microscopic haematuria
- Progressive renal failure
- Bilateral senorineural deafness
- Lenticonus - protrusion of lens into anterior chamber
- Retinitis pigmentosa
Alport’s with failing renal transplant?
- Caused by anti-GBM antibodies
- Leads to Goodpasture’s syndrome picture
Risk factors for renal stones? Urate stones? Drug causes?
Risk Factors
- Dehydration
- Hypercalciuria, hyperparathyroidism, hypercalcemia
- Cystinuria NOT CYSTINOSIS
- High dietary oxalate
- Renal tubular acidosis
- Medullary sponge kidney, polycystic kidney disease
- Beryllium or cadmium exposure
Urate stones
- Gout
- Ileostomy - loss of bicarb and fluid results in acidic urine –> uric acid
Drug Causes
- Loop diuretics, steroids, acetazolamide, theophylline –> calcium stones
- Thiazides can prevent calcium stones (distal tubular calcium resorption)
Types of Renal Stones?
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Stag horn calculi?
- Involve the renal pelvis and extend into at least 2 calyces.
- Develop in alkaline urine and are composed of struvite (ammonium magnesium phosphate, triple phosphate).
- Ureaplasma urealyticum and Proteus infections predispose to their formation
Albumin creatinine ratio?
ACR
- Early morning specimen
- >2.5 = microalbuminaemia
- All diabetic patients should be screened annually
Management of diabetic nephropathy?
- Dietary protein restriction
- Tight glycemic control
- BP control: aim for < 130/80 mmHg
- Benefits independent of blood pressure control have been demonstrated for ACE inhibitors and angiotensin II receptor blockers - these may be used alone or in combination
- Control dyslipidemia e.g. Statins
CKD stages?
- 1 - >90
- 2 - 60-90
- 3a - 45-49
- 3b - 30-44
- 4 - 15-29
- 5 - <15
Causes of transient and persistent microscopic haematuria?
Transient
- Urinary tract infection
- Menstruation
- Vigorous exercise
- Sexual intercourse
Persistent
- Cancer (bladder, renal, prostate)
- Stones
- Benign prostatic hyperplasia, Prostatitis.
- Urethritis e.g. Chlamydia
- Renal causes: IgA nephropathy, thin basement membrane disease
Fanconi syndrome features and causes?
Generalized disorder of renal tubular transport resulting in:
- Type 2 RTA
- Aminoaciduria
- Glycosuria
- Phosphaturia
Causes
- Cystinosis (most common cause in children)
- Sjogren’s syndrome
- Multiple myeloma
- Nephrotic syndrome
- Wilson’s disease
RTA Types?
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Causes of RTA 4?
- Aldosterone deficiency (hypoaldosteronism): Primary vs. hyporeninemic
- Aldosterone resistance:
- Drugs: Amiloride, Spironolactone, Trimethoprim, Pentamidine
- Pseudohypoaldosteronism
- DM
Cause of RTA 3?
Juvenile RTA
Carbonic anhydrase II deficiency
Rarely discussed
Differences between ATN and Prerenal Uraemia? (Causes of ARF)
Prerenal uraemia = kidneys hold onto sodium to preserve volume
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Papillary Necrosis - causes and features?
Causes
- Chronic analgesia use
- Sickle cell disease
- TB
- Acute pyelonephritis
- Diabetes Mellitus
Features
- Fever, loin pain, haematuria
- On urogram/pyelogram - papillary necrosis with renal scarring (‘cup and spill’)
RCC - location? Associations? Features? Management?
Location
- Most commonly proximal renal tubular epithelium
Associations
- Middle aged men
- Smoking
- Von-hippel-lindau syndrome
- Tuberous slcerosis
Features
- Classically - haematuria, loin pain, abdominal mass
- Pyrexia of unknown origin
- Left varicocele (occlusion of left testicular vein)
- Endocrine effects - secretion EPO (polycythaemia), PTH (hypercalcaemia), renin, ACTH
Management
- Radical nephrectomy
- a-interferon and IL-2 can reduce tumour size and treat mets
- Tyrosine kinase inhibitors (nibs)
Key features of HIV-associated nephropathy (HIVAN)?
- Massive proteinuria
- Normal or large kidneys
- FSGN with focal or global capillary collapse on renal biopsy
- Elevated urea and creatinine
- Normotension
Renal Vascular DIsease - presentation, Ix, Mx?
Presentation
- HTN, CKD, flash pulmonar oedema
- U+Es worse on ACE
Ix
- MR angiography/CT angiography
Mx
- Balloon angioplasty
- Hypokalaemia and metabolic alkalosis due to compensatory hyperaldosteronism
Renal complications of SLE - WHO classification? Management?
Classification
- Class I: normal kidney
- Class II: mesangial glomerulonephritis
- Class III: focal (and segmental) proliferative glomerulonephritis
- Class IV: diffuse proliferative glomerulonephritis
- Class V: diffuse membranous glomerulonephritis
- Class VI: sclerosing glomerulonephritis
Management
- Treat HTN
- Steroids
- Immunosuppressants - azathioprine/cyclophosphamide
Goodpasture’s - cause? Features? Investigations? Management?
Cause
- Type II hypersensitvity reaction
- Anti GBM antibodies against type IV collagen
- Goodpasture antigen - found in kidney and lungs
Features
- Bimodal distribution, HLA DR2
- Pulmonary haemorrhage, rapidly progressive glomerulonephritis
- Factors increasing likelihood of pulmonary haemorrhage - young male, smoking, LRTI, pulmonary oedema
Investigations
- Renal biopsy - linear IgG deposits along basement membrane
- Increased TCLO
- Lung biopsy - accumulation of hemosidren laden macrophages within alveoli
Management
- Plasma exchange
- Steroids
- Cyclophosphamide
Haemolytic uraemic syndrome: triad? causes? Ix? Mx?
Triad
- Acute renal failure
- Migroangiopathic haemolytic anaemia
- Thrombocytopenia
Causes
- Post-dysentery - E coli 0157:H7
- Tumours, pregnancy, cyclosporine, COCP, SLE, HIV
Ix
- FBC - anaemia, thrombocytopenia, fragmented blood film
- U+E - renal failure
- Stool culture
Mx
- Supportive management
- No abx
- Plasma exchange in severe cases with no diarrhoea
Phenylketonuria?
General
- Autosomal recessive
- Defect in phenylalanine hydroxylase - converts phenylalanine to tyrosine –> high levels of phenylalanine –> LD and seizures
- Chromosome 12
Features
- Developmental delay
- Fair hair, blue eyes
- LD, seizures, eczema
- Musty odour to urine and sweat
Diagnosis
- Guthrie test
- Hyperphenylalaninaemia
- Phenylpyruvic acid in urine
Mx
- Dietary restrictions in pregnancy. Poor evidence to suggest strict diet prevents LD.
Cystinuria?
General
- Autosomal recessive
- Formation of recurrent renal stones
- Defect in membrane transport of COLA (cystine, ornithine, lysine, arginine)
Features
- Recurrent renal stones - yellow and crystaline - semi-opaque on X ray
Dx
- Cyanide-nitroprusside test
Mx
- Hydration
- D-penicillamine
- Urinary alkalinization
Homocystinuria?
General
- Autosomal recessive
- Deficiency of cystathione b-synthase –> accumulation of homocysteine
Features
- Fine, fair hair
- MSK - similar to Marfan’s - arachnodactyly
- Neuro - LD, seizures
- Ocular - downwards discolouration of lens (opposite to Marfan’s)
- Increased risk of arterial/venous thrombosis (not coronary)
- Malar flush, levido reticularis
Dx
- Cyanide nitroprusside test
Mx
- B6 supplements
Alkaptonuria?
General
- Autosomal recessive
- Phenylalanine and tyrosine metabolism disorder
- Defect in homogentisate 1,2-dioxygenase. Buildup of tyrosine byproduct (alkapton) accumulates, excreted in urine in large amounts –> cartilage damage, valvular issues, kidney stones
- Common in Slovakia and Dominican republic
Features
- Pigmented sclera, darkened skin in sun-exposed areas and around sweat glands
- Low back pain, hip/shoulder pain –> joint replacement
- Calcification of and regurg in aortic and mitral valves –> replacement or CHD
- Ear wax exposed to air turns black or red after several hours
Tx
- Nitisinone - suppressed homogentisic acid production
Adverse effects of BPH treatment?
Alpha Blockers
- Dizziness, postural hypotension
- Dry mouth
- Depression
5 alpha-Reductase Inhibitors
(Block conversion of testosterone to DHT)
- Erectile dysfunction
- Reduced libido, ejaculation problems
- Gynaecomastia
Prostate Ca Mx?
Localised Disease (T1/T2)
- If life expectancy <10 years - watchful waiting
- If life expectancy >10 years - radical prostatectomy and radical radiotherapy
Locally Advanced Disease (T3/T4)
- Beyond prostatic capsule or involving bladder neck or rectum
- Radiotherapy
Disseminated Disease
- Synthetic GnRH agonist (Goserelin AKA Zoladex)
- Provides negative feedback to anterior pituitary
- Cover with anti-androgen to prevent rise in testosterone
- Anti-androgen (Cyporterone acetate)
- Prevents DHT binding from intracytoplasmic protein complexes
- Orchidectomy
Risk factors for bladder Ca?
- Smoking
- Occupational - aniline dyes used in printing/textiles/rubber industry
- Aromatic amines
- Radiation treatment to pelvis
- Schistosomiasis (S.hematobium infection)
- Mutations (17p13.1 –> p53)
- Cyclophosphamide