Renal + urology Flashcards

(83 cards)

1
Q

Gitelman syndrome

A

Mutations in the thiazide-sensitive Na/Cl symporter

Autosomal recessive

Features:
- Normotensive
- Low Cl-, K+, Mg2+, Ca2+
- Metabolic alkalosis
- Raised urinary Na+ and K+

Investigations:
- Bloods
- 24h urinary Na+ and K+

Management:
- K+ and Mg 2+ supplementation
- Trial of aldosterone antagonists

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

Conn syndrome

A

Primary hyperaldosteronism
- Usually due to adrenal adenoma

Features:
- Hypertension
- Normal/raised Na+, low K+

Management:
- Aldosterone antagonists
- Adrenalectomy

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

Liddle syndrome

A

Pseudo-hyperaldosteronism due to mutations in the ENaC
- Autosomal dominant
- Increase ENaC activity = increased Na+ reabsorption

Features:
- HTN
- Hypokalaemia
- Metabolic alkalosis
- Low renin + aldosterone

Management:
- Potassium-sparing diuretics e.g. amiloride

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

Acute interstitial nephritis

A

Inflammation of the renal tubulointerstitium

Aetiology:
- Drug hypersensitivity
- Rifampicin, allopurinol, antibiotics, NSAIDs
- Autoimmune
- SLE, Sjogrens
- Idiopathic (rare)
- Viral
- Hantavirus
- Bacterial
- Leptospirosis, mycobacteria

Features:
- AKI
- Fever
- Eosinophilia
- Sterile pyuria
- Nephrotic syndrome if NSAID-induced

Management:
- Treat cause
- Steroids

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

Renal tubular acidosis

A

Type I - distal
- Failure of distal and collecting tubular cells to secrete H+ and reabsorb K+
- Features:
- Renal stones - due to urinary alkalosis +
increased secretion of Ca2+ in serum
acidosis
- Diabetes insipidus
- Salt wasting
- Severe metabolic acidosis + hypokalaemia

Type II - proximal
- Failure of proximal tubule cells to reabsorb HCO3
- Features:
- Mild metabolic acidosis +/- hypokalaemia
- Osteomalacia
- Rickets

Type III
- Combined type I and type II

Type IV
- Either low serum aldosterone or renal resistance to aldosterone activity
- Features:
- Hyperkalaemia
- CKD

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

Type 1 renal tubular acidosis

A

Failure of distal and collecting tubular cells to secrete H+ and reabsorb K+

Aetiology:
- Idiopathic or genetic
- Autoimmune disease
- Nephrocalcinosis
- Drugs incl. lithium

Features:
- Renal stones
- due to urinary alkalosis + increased secretion
of Ca2+ in serum acidosis
- Diabetes insipidus
- Salt wasting
- Severe metabolic acidosis + hypokalaemia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

Type 2 renal tubular acidosis

A

Failure of proximal tubule cells to reabsorb HCO3

Aetiology:
- Idiopathic
- Fanconi syndrome
- Drugs incl. NSAIDs, heavy metals

Features:
- Mild metabolic acidosis +/- hypokalaemia
- Osteomalacia
- Rickets

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

Fluid requirements

A

Adult:
25-30 ml/kg/day
50-100g glucose

Child:
- 100ml/kg/day for first 10kg, then 50ml/kg/day for next 10kg, then 20ml/kg/day for the remainder

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

Central pontine myelinolysis (osmotic demyelination syndrome)

A

A disorder in which myelin +/- neuronal cells are damaged by rapid correction of hyponatraemia
- A rise of > 1 mmol/hr or >10 mmol/day
- Symptoms start 2-3 days after hyponatraemia

Features:
- Reduced GCS
- Confusion
- Limb weakness, paralysis, paraesthesia
- Dysphagia
- Dysphasia
- Impaired coordination
- Can progress to coma/death

Management:
- Supportive
- May recover over months or be left with permanent disability

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

Functional parts of kidney

A

Glomerulus
- Receives 600ml/min
- Filters (GFR) 120ml/min
- Filtration depends on molecular weight and charge of molecule
- Foot processes and BM are -ve charge
- Molecules > 4nm are completely blocked (e.g. cells, protein), 2-4nm partially blocked.

PCT
- 70% of reabsorption - esp. glucose, amino acids
- Completely permeable to water and glucose
- Secretes HCO3 to allow H+ reabsorption
- Can vary amount of isotonic reabsorption to modulate the ECF
- Contains a number of organic ion transporters which affect excretion of hormones, drugs, etc.

Loop of Henle
- Establishes the medullary concentration gradient to allow for later reabsorption

DCT
- Contains K+/H+ antiporters which help to maintain plasma K+ and pH

Collecting duct
- Site of ADH action -> aquaporin insertion allowing for reabsorption down the medullary concentration gradient

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

Afferent and efferent arterioles

A

Can be modulated by systemic NA/Adr or by local mechanisms to maintain GFR across a range of cardiac outputs

Afferent
- Constriction -> reduced rate filtration, same amount of filtrate
- Myogenic response
- Constricts when stretched due to stretch-
activated calcium channels

Efferent
- Constriction -> increased rate of filtration
- Dilation -> reduced rate of filtration

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

Renin-aldosterone-angiotensin system

A

Renin:
- Proteolytic enzyme
- Secreted by juxtaglomerular cells in the afferent arteriole in response to:
- NA action on beta-1 adrenoceptors
- Fall in afferent arteriole stretch
- Decreased Na+ load at the macula densa
- Catalyses cleavage of angiotensinogen to angiotensin - 1

Angiotensin:
- 2 step production:
- Angiotensinogen + renin -> angiotensin -1
- angiotensin-1 + ACE -> angiotensin-2
- AT1 stimulation
- Vasoconstriction - raises BP and GFR
- Increased Na+ reabsorption
- AT2 stimulation
- Increases thirst + Na+ appetite
- Stimulates aldosterone synthesis

Aldosterone:
- Acts on thick ascending limb + collecting ducts to promote Na+ reabsorption, H+ and K+ secretion
- Slowly increases expression of ENAC

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

Atrial and brain natriuretic peptides (ANP, BNP)

A

ANP
- Stored in atrial myocytes
- Released in response to atrial stretch (aka high BP)
- Stimulates Na+ and water loss, inhibits ADH and renin

BNP
- Cleaved from NT-proBNP
- Binds to ANP receptors although with reduced efficacy
- Found in brain and heart

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

ADH/vasopressin

A

Synthesised in the SON and PVN, stored in nerve terminals in the posterior pituitary
- Release dependent on osmoreceptors

Effects:
- V1
- Vasoconstriction
- V2
- Aquaporin insertion -> increased water
reabsorption

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

Hereditary angioedema

A

Mutation/deficiency in C1INH (C1 esterase inhibitor) leading to excessive bradykinin levels and episodes of painful swelling
- Similar mechanism can lead to angioedema
with ACEi use

Management
- Ecallantide - synthetic C1INH

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

Hypernatraemia

A

Aetiology:
- Diabetes insipidus
- HHS
- Dehydration
- ATN - early polyuric phase
- Diuretics
- Steroid excess - Cushing, Conn
- Salt poisoning - iatrogenic (NaCl, sodium bicarb), drowning in salt water, high sodium feed

Features:
- Mild (140-180)
- Can be asymptomatic
- Excessive thirst, confusion
- Severe (> 180)
- Ataxia, tremor, coma, seizures
- Raised ICP

Management:
- Aim reduction no greater than 10 mmol/day otherwise risk cerebral oedema
- Hypovolaemic
- Fluid resus + 5% dex
- Euvolaemic
- 5% dex
- Hypervolaemic
- 5% dex + loop diuretic

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

Hyponatraemia

A

Hypertonic
- Serum osmolality > 285
- Hyperglycaemia
- Mannitol infusion

Isotonic
- Serum osmolality 280-285
- Hyperlipidaemia or paraproteinaemia

Hypotonic
- Serum osmolality < 280
- Hypovolaemic
- Urine Na < 20
- Vomiting
- Diarrhoea
- 3rd spacing
- Urine Na > 20
- Diuretics
- Addisons’
- Salt wasting nephropathy
- Euvolaemic
- Urine osmolality > 100
- Primary polydipsia
- Beer potomania
- Ecstasy
- Urine osmolality < 100
- SIADH
- Hypothyroidism
- ACTH deficiency
- Hypervolaemic
- Urine Na < 20
- Heart failure
- Liver failure
- Nephrotic syndrome
- Urine Na > 20
- Kidney failure

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

Severe hyponatraemia management

A

aka symptomatic with seizures and drowsiness

Admit to ITU
- Aim initial rise of 1-2 mmol/hr over first 3 hr
- Can raise Na+ > 10mmol/day with care

Calculate sodium deficit:
Fluid [Na] - serum [Na]/1 + total body water

(1L 0.9% NaCl = 154 mmol)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

Hypoalbuminaemia

A

Aetiology:
- Decreased production
- Chronic inflammation
- Severe malnutrition
- Liver cirrhosis
- Increased loss
- Renal - nephrotic syndrome
- GI - erosive (IBD, malignancy, PUD), non-
erosive (coeliac, sprue, SIBO, Whipples),
raised lymphatic pressure (CCF, mesenteric
TB)
- 3rd spacing
- Increased catabolism
- Prolonged severe illness e.g. ITU

Management:
- Mostly supportive + treat underlying cause
- HAS if cirrhosis or haemodynamic instability not responding to crystalloids alone

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

SIADH

A

Aetiology:
- Malignancy
- SCLC, pancreatic, prostate
- Infections
- TB
- Neurological
- Stroke, SAH, abscess
- Drugs
- SSRIs, Indomethacin, TCAs, thiazide diuretics,
antipsychotics, cyclophosphamide, vincristine

Investigations:
- Paired urine + serum osmolality
- Paired urine + serum sodium
- Water deprivation testing

Management:
- Fluid restriction
- Demeclocycline

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

Hyperkalaemia

A

Aetiology:
- Kidney failure
- Rhabdomyolysis
- Tumour lysis
- Metabolic acidosis
- Hypoglycaemia

Features:
- Muscle weakness
- Paraesthesias
- Palpitations
- Arrhythmia

Investigations:
- Bloods
- ECG - tall tented T waves, broad QRS

Management:
- Calcium gluconate 10ml over 10 mins, repeated
- Insulin/dextrose - 10 units in 250ml 10% dextrose
- Salbutamol nebs 5-10mg

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

Hypokalaemia

A

Aetiology
- Vomiting or diarrhoea
- Loop and thiazide diuretics
- Conn’s syndrome
- Laxative abuse
- Metabolic alkalosis
- Insulin therapy
- Refeeding syndrome

Features:
- Muscle weakness + myalgia + cramps
- Tremor
- Palpitations
- Constipation

Investigations:
- Bloods
- ECG - T wave flattening/inversion, U waves, QT prolongation

Management:
- Treat underlying cause
- Sando-K TT TDS
- IV KCl

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
23
Q

Hypercalcaemia

A

Aetiology
- Malignancy
- Hyperparathyroidism
- Multiple myeloma
- Dehydration
- Paget’s disease
- Addison’s disease
- CKD

Features:
- Bone pain
- Fatigue, weight loss
- Constipation
- Kidney stones, kidney impairment
- Depression, psychosis

Investigations
- Routine bloods
- TFTs, PTH level, vitamin D
- ECG - shortened QTc
- Imaging of bones

Management:
- Acute:
- IV fluids
- IV bisphosphonates

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
24
Q

Hypocalcaemia

A

Aetiology:
- Vitamin D deficiency
- Hypoparathyroidism (incl. post-surgical)

Features:
- Muscle cramps, twitching -> tetany
- Paraesthesias
- Carpopedal spasms
- Chvostek’s + Trousseau’s signs
- Risk of laryngeal tetany and asphyxiation

Investigations:
- Routine bloods
- PTH and vitamin D levels
- ECG - prolonged QTc

Management:
- Acute (calcium < 1.9, symptoms)
- IV calcium gluconate
- Chronic:
- Cholecalciferol (if vit D deficient)
- Calcitriol (if PTH deficient)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
25
Calcium homeostasis
Absorption - GIT - Renal reabsorption Storage: - 99% calcium hydroxyapatite in the bones - Remainin 1% in cells + serum - Protein-bound - Chelated - for transport - Ionised - used in cellular signalling PTH: - Secreted in response to hypocalcaemia, from parathyroid glands (x 4) - Stimulates bone resorption - Increases renal calcium reasbsorption - Increases renal vitamin D activation Vitamin D - Increases in response to hypocalcaemia - Metabolised in two steps into 1,25-dihydroxyvitamin D - Increased expression of GIT calcium-binding proteins -> increased absorption - Stimulates bone resorption Calcitonin - Secreted from parafollicular C cells (thyroid) in response to hypercalcaemia - Inhibits bone resorption - Inhibits renal reabsorption of calcium
26
Phosphate
Levels are usually inverse to calcium High: - Aetiology - CKD, acidosis, tissue lysis, tertiary hyperparathyroidism Low: - Aetiology - primary hyperparathyroidism, refeeding syndrome, ETOH, Cushing's, alkalosis - Features - muscle weakness, tremor, infection, confusion, respiratory depression - Management - PO or IV replacement
27
Hypomagnesaemia
Aetiology: - Malnutrition - Medications - diuretics, PPIs, ciclosporin, cisplatin - Osmotic loss - diabetes - Malabsorption - Prolonged GI suction, stoma, fistula etc. Features: - Fatigue - Weakness - Muscle cramps - Spasticity - Seizures Investigations: - U&Es + bone profile - Leads to hypokalaemia - ECG - prolonged QTc, tachy
28
Biochemical patterns: Adrenal insuff (5) Hyperaldo(3) Phaeochromocytoma(1) Sarcoidosis(2) Carcinoid syndrome(3) Refeeding syndrome(4) Sequelae of parenteral nutrition(3) Rhabdomyolysis (5) Tumour lysis syndrome (6) Toxic alcohols (ethylene glycol as eg)(3) Lithium toxicity (2) Salicylate toxicity (3)
Adrenal insufficiency - Hyponatraemia - Hyperkalaemia - Normal anion gap acidosis - Hypoglycaemia - Hypercalcaemia Hyperaldosteronism - Hypernatraemia - Hypokalaemia - Metabolic alkalosis Phaochromocytoma - beta-2 blockade - Hypokalaemia Sarcoidosis - ACE activity - Hypercalcaemia - Hypercalciuria Carcinoid syndrome - secretory diarrhoea - Hypokalaemia - Hypomagnesaemia - Normal anion gap acidosis Refeeding sybdrome - Hypophosphataemia - Hypokalaemia - Hypomagnesaemia - Hyperglycaemia Parenteral nutrition - Hyperglycaemia - Hyperlipidaemia - Normal anion gap acidosis Rhabdomyolysis - Hyperkalaemia - Hyperphosphataemia - Myoglobinuria - Raised CK, LDH Tumour lysis syndrome - Hyperkalaemia - Hyperphosphataemia - Hypocalcaemia - Hyperuricaemia - Raised anion gap acidosis - Raised LDH Toxic alcohols (e.g. ethylene glycol) - Hypocalcaemia - Raised anion gap acidosis - Raised serum osmolar gap Lithium toxicity - Hypernatraemia - Low anion gap acidosis Salicylate toxicity - High anion gap acidosis - Hypokalaemia - Hyperkaluria
29
Nephrotoxic drugs
Membraneous GN - Pencillamine - Gold - Captopril (Acute) interstitial nephritis - Penicillins - Cephalosporins - NSAIDs - Allopurinol - Phenytoin - PPIs Renal tubular damage: - Amphotericin - Heavy metals - incl. mercury, lithium - Cisplatin - Aminoglycosides - Vancomycin - NSAIDs - Aciclovir
30
Acute tubular necrosis
Renal tubular cell damage (+/- death) which initially causes AKI but can lead to strictural injury Aetiology: - Reduce renal perfusion e.g. hypovolaemia, sepsis - Nephrotoxic medications Pathology: - Loss of brush border - Tubular cell vacuolation and sloughing - Typically Na+ wasting Features - three phases: - Initiation - Oliguria - Uraemia - Maintenance - Worsening oliguria and uraemia - Hyperkalaemia - Acidosis - Fluid overload - Recovery - Polyuria - massive loss of Na+ and K+ - Hypokalaemia Investigations: - Urine analysis - Urine Na+ > 40 - Urine osmolality < 350 - Microscopy - brown epithelial casts + free epithelial cells - Fluid balance - Bloods - Renal US normal
31
Acute interstitial nephritis
Aetiology: - Infection - Medications e.g. NSAIDs, PPIs, antibiotics Features: - AKI - HTN - Rash - Eosinophilia - Fever - Flank pain - from swelling and stretch of the renal capsule
32
Chronic interstitial nephritis
Fibrosis and dysfunction secondary to chronic or repeated insults Aetiology: - Heavy metals - Nephrocalcinosis - Chronic hyperkalaemia - Medication e.g. analgesics
33
Urea:creatinine ratio
Both freely filtered by the tubules but urea should be reabsorbed in a regulated fashion whereas creatinine is not Normal ratio 40-110:1 Used to define types of AKI: - > 110:1 = prerenal - 40-110:1 = post-renal - < 40:1 = intrinsic
34
Azotaemia and uraemia
Azotaemia = increased non-nitrogenous waste products in the blood (usually proteinaceous) Uraemia = increased urea in the blood Azotaemia will precede uraemia Features: - Azotaemia - Fatigue - Muscle weakness - Nausea/vomiting - Uraemia - Itching - Pericardial effusion - Encephalopathy - Stomatitis or parotitis - Tremors - Peripheral neuropathy
35
CKD - aetiology and staging
Aetiology: - Diabetic nephropathy (44%) - HTN (27%) - Glomerulonephritis (8%) - Other - Cystic disease - Urological - Infection - malaria, schistosomiasis Classification - eGFR + ACR: G stage: - Stage 1 - Preserved eGFR + abnormal ACR - Stage 2 - eGFR 60-89 + abnormal ACR - Stage 3 - A = eGFR 45-50 - B = eGFR 30-44 - Stage 4 - eGFR 15-29 - Stage 5 - eGFR < 15 or requiring RRT A stage - A1 - ACR < 3 - A2 - ACR 3-30 - A3 - ACR > 30
36
CKD + bone disease
Pathophysiology: - Reduced vitamin D activation + reduced secretion of phosphate leads to hyperphosphataemia - High phosphate leaches calcium out of bones causing osteomalacia - Compensatory response includes PTH secretion Aim of treatment is to treat high PTH and high phosphate - Reduced dietary phosphate - Phosphate binders - Activated vitamin D supplements e.g. alfacalcidol
37
Haemodialysis
Usually 4hr sessions, 3x a week Access: - AV fistula in the non-dominant hand - 6-8 weeks to mature - Ideally formed several (6) months prior to need - Tunneled dialysis line - Only if fistula fails or severe vascular disease Complications - Fistula stenosis or thrombosis - Line infection - Steal syndrome - Redirection of blood leading to hand ischaemia - Hypotension - Arrhythmias - Dialysis disequilibrium syndrome - Cerebral oedema secondary to rapid fall in urea - Headache, confusion, focal neurology - Anaphylaxis - Usually to dialysis membrane - Accelerated cardiovascular disease
38
Peritoneal dialysis
Utilisation of the peritoneum as a semipermeable membrane to dialyse the blood Performed daily but can be done at home - Requires generally good health (no cardiac comorbities) - Preferred if have a residual renal function Complications: - Exit site infection - Peritonitis - Fatigue, headache, cramps, etc.
39
Continuous RRT
A slower type of dialysis which reduces the haemodynamic shifts associated Allows removal of both fluid and solutes Continuous venovenous haemofiltration or haemodialysis - Dialysis = gradients alone, filtration = pressure Indications: - Pulmonary oedema or pericardial effusion resistant to diuresis - pH < 7.2 - Persistent K+ > 6.5 or other severe eletrolyte abnormalities - Symptomatic uraemia
40
Hyperuricaemia aetilogy
- Gout - Lesch-Nyhan syndrome - Congenital disorder of purine metabolism - Tumour lysis syndrome - High purine diet - Hyperparathyroidism - Lead posioning - Downs syndrome - Exercise - Starvation - Medications e.g. thiazides, salicylates, ETOH
41
Hepatorenal syndrome
Includes both AKI and CKD developed on a background of liver cirrhosis with no other cause for renal impairment Aetiology: - Acute - SBP, GI bleed, ETOH, acute liver failure, large volume ascites paracentesis - Chronic - liver decompensation Pathophysiology: - Hypoalbuminaemia + splanchnic vasodilation -> fall in systemic BP -> renal hypoperfusion - RAAS activates and cannot deactivate leading to worsening renal perfusion and impairment Management: - Aim to restore euvolaemia + splachnic vasoconstriction - HAS + terlipressin - TIPS - Liver transplant
42
Metabloc acidosis
High anion gap (CAT MUDPILES) - > 12 - C - CO, CN - A - alcoholic and starvation ketoacidosis - T - toluene - M - metformin, methanol - U - uraemia - D - diabetic ketoacidosis - P - paracetamol, propylene glycol - I - iron, isoniazid, IEM - L - lactic acidosis - E - ethylene glycol - S - salicylates Normal anion gap (CAGE) - NB hypercholraemic acidosis (Cl - replacing HCO3 - ) or HCO3 loss - C- chloride excess - A - acelazolamide, adrenal insufficiency - G - GI e.g. diarrhoea, vomiting, fistulae - E - extras e.g. RTA
43
D-lactic acidosis
A rare cause of raised anion gap acidosis Aetiology: - Short bowel syndrome - SIBO - Jejuno-ileal bypass Pathophysiology: - Malabsorptive state - carbohydrate is taken up and metabolised by abnormal colonic flora, producing D-lactate - D-lactate competes with pyruvate in the heart and brain (although can be used and metabolised in the liver) Features: - Reduced GCS / confusion - Ataxia - Coma - Heart arrhythmias or block Investigations: - Serum D-lactate levels - not seen on standard assays
44
Nephrotic syndrome
Aetiology: - Membraneous glomerulonephritis - Minimal change disease - SLE - FSGS - Mesangiocapillary glomerulonephritis - Amyloidosis - IgA glomerulonephritis Features: - Hypoalbuminaemia (< 35) - Proteinuria (> 3.5g/day) - Oedema - Hypercoagulability - Hyperlipidaemia - High cholesterole + LDL, normal HDL - Immunodeficiency - esp. pneumococcal infections, loss of IgG - Hypothyroidism - loss of thyroid-binding protein - Vitamin D deficiency - loss of vitamin D binding protein - Abdominal pain - from splanchnic ischaemia Investigations: - Urine - Dipstick - Protein-creatinine ratio - 24h protein collection - Bloods: - Routine - Lipid profile - Serology - syphilis, HIV, hepatitis, autoimmune - Myeloma screening - Increased alpha- and beta-globulin fractions - Renal biopsy Management: - Treat underlying cause - Fluid + salt restriction - Diuresis - furosemide, ACEi (anti-proteinuric) - Anticoagulation
45
Membraneous glomerulonephritis
Aetiology: - Idiopathic (up to 85%) - SLE - Infection - malaria, HBV/HCV - Drugs - pencillamine, NSAIDs, gold - Malignancy (10%) Pathology: - Generalised thickening of the glomerular basement membrane - Subepithelial deposition of IgG and C3 Management: - Diuretics + fluid restriction - Anticoagulation + aspirin - ACEi/ARBs - Immunosuppression if no improvement after 6 months - cyclophosphamide, tacrolimus, biologics Prognosis: - 25% spontaneous remission - 25% partial remission - 50% progressive renal impairment
46
Renal vein thrombosis
Aetiology: - Severe dehydration - e.g. neonate in NICU with insufficient feed - Hypercoagulability - Cancer, nephrotic syndrome, polycythaemia, protein C deficiency Features: - Flank pain - Renal enlargement - Haematuria - Worsening renal function Investigations: - Renal USS - CT angio Management: - Hydration - Anticoagulation
47
Nephritic syndrome
A syndrome of worsening renal failure associated with haematuria, non-nephrotic proteinuria, and hypertension Aetiology: - Paeds: - IgA nephropathy - PSGN - HSP - HUS - Adults - Above causes - Autoimmune e.g. SLE, Goodpasture's, vasculitis - RPGN - MPGN - Infective endocarditis - Cryoglobulinaemia Features: - Hypertension - Renal impairment - Haematuria - Flank pain Investigations - Urinalysis - Bloods - Renal biopsy - Commonly crescenteric appearance - Immunofixation
48
IgA nephropathy
The most common type of glomerulonephritis in adults - 20-40% progress to CKD Aetiology: - 1-2 days following URTI, pneumonia, tonsillitis or gastroenteritis - Post-immunisation - Some association with coeliac disease and cirrhosis Pathology: - Hypercellular mesangium + crescenteric appearance - Granular IgA + C3 deposition Features: - HTN - Visible haematuria - May worsen with exercise - AKI or CKD - High serum IgA (in 50%) Management: - Immunosuppression - steroids - Consider tonsillectomy if tonsillitis is a trigger
49
Post-infectious glomerulonephritis
Aetiology: - Most commonly post-streptococcal - Typically children, 1-2 weeks after infection - Can be viral, fungal, etc. Pathology: - Type III hypersensitivity reaction - Hypercellular mesangium - IgG + C3 deposition Features: - Haematuria - AKI - HTN - Can also present with proteinuria + oedema Investigations: - Urine MCS - Haematuria with RBC casts - Streptolysin-O titre - Complement levels - low C3 Management: - Usually self-limiting with only supportive care required
50
Mesangiocapillary glomerulonephritis
Epidemiology: - 8-30 yrs - M > F Aetiology: - Idiopathic - Infection - Viral - HIV, HBV/HCV - Bacterial - TB, infective endocarditis - Autoimmune - any immune complex deposition disease - Cryoglobulinaemia, SLE, scleroderma - Malignancy - Leukaemia and lymphoma Pathology: - BM splitting allows mesangial cell cytoplasm to spread between the endothelium and basement membrane - Appears as mesangial cell proliferation + subendothelial thickening Features: - Can present as nephrotic syndrome, nephritic syndrome, or more subtle progressive renal failure Investigations: - Low complement levels Prognosis - 50% progress to ESRF at 10 yrs - Fluctuant disease severity
51
Minimal change disease
Aetiology: - Associated with atopy, infection, malignancy, and drugs (NSAIDs) Pathology: - No obvious change on standard microscopy - Effacement of the podocyte foot processes Features: - Massive oedema - Foamy urine - Nephrotic syndrome Management: - Steroids should induce rapid remission
52
Focal segmental glomerulosclerosis
Aetiology: - Idiopathic - M > F, African ethinicity - Drugs - Heroin - Lithium, steroids, doxorubicin - Infection - HepB/C, HIV, CMV - Other - Obesity, diabetes Pathology: - Focal and segmental sclerosis - IgM + C3 positive - Foot process effacement Features: - Paeds - nephrotic syndrome - Adults - spectrum of nephrotic and pre-nephrotic syndromes Investigations: - Urine MCS - Casts + protein - Renal biospy Management: - 1st line - steroids - 2nd line - calcineurin, mycophenolate, rituximab Prognosis: - Usually leads to progressive renal dysfunction - Prognosis worse in idiopathic cases
53
Henoch-Schonlein purpura (HSP)
Epidemiology: - Peaks at 4-6yr - 90% of cases are in under-10s - Rare in both adults and infants Pathophysiology - IgA vasculitis, usually triggered by preceding illness or vaccination Features: - Low-grade fever - Lower limb purpura - Abdominal pain - Arthralgia - Bloody diarrhoea - Renal dysfunction (IgA nephropathy, nephrotic picture) Management: - Usually self-limiting, within 4 weeks - Supportive Prognosis: - Rarely leads to permanent kidney damage (<1%)
54
Haemolytic uraemic syndrome
Secondary to shiga toxin or shiga-like toxin, predominantly produced by gastrointestinal pathogens - Usually 5 days after onset of diarrhoea Features: - Preceding profuse diarrhoea, turning bloody after 1-3 days - Haemolytic anaemia - Thrombocytopenia - AKI with uraemia - (Also abdo pain, confusion, lethargy) Investigations: - Blood film - Schistocytes, thrombocytopenia - DAT+ Management: - Supportive care +/- dialysis - Avoid antibiotics where possible Complications: - Abdominal pain - Perforation or strictures - Pancreatitis - Myocarditis or cardiomyopathy - Retinal haemorrhage - Kidney failure
55
Goodpasture's
Anti-GBM disease affecting both kidneys and lungs - antibodies against type IV collagen Risk factors: - HLA-DR15 - Cocaine use - Smoking - Infection (esp. influenza) Pathology: - Crescenteric glomerulonephritis - Linear IgG deposition along basement membrane Features: - Renal disease - Nephritic syndrome with acute renal failure - Pulmonary disease - Haemoptysis + alveolar haemorrhage Management: - Many (esp. younger patients) may present in acute respiratory failure + AKI - Induction = Prednisolone + cyclophosphamide + plasmapheresis
56
Causes of combined renal + neurological disease (11)
Wilson's disease - Fanconi syndrome -> type II RTA - Basal ganglia dysfunction - Peripheral neuropathy Hypertension - CKD - Hypertensive encephalopathy Haemolytic uraemic syndrome - AKI - Uraemic encephalopathy SLE - Lupus nephritis - typically diffuse sclerosis + crescenteric glomerulonephritis - Encephalitis, neuropathies, seizures Alport syndrome - FSGS with micro- and macroscopic haematuria - Sensorineural deafness APCKD - Renal cysts + CKD - Berry aneurysms, SAH Sickle cell anaemia - Nephropathy with varying appearances - renal papillary necrosis, FSGS - Renal medullary carcinoma - Stroke Lead poisoning - Fanconi syndrome -> type II RTA - Raised ICP Tuberous sclerosis - Renal cysts - Angiomyolipoma can cause HTN and AKI - Seizures - Intellectual impairment Neurofibromatosis - Renal artery stenosis - Learning difficulties Posterior reversible encephalopathy syndrome (PRES) - HTN with AKI/CKD - Seizures, delirium, visual changes
57
Haematuria
Aetiology: - Kidney - e.g. cancer, stone, infarction, cysts, GN - Bladder - e.g. cancer, cystitis, stones, tear - often terminal +/- pain - Prostate - e.g. BPH, varices, post-RT - painless, can be initial or terminal - Urethra - dribbling Also several transient causes - UTI, period, beetroot, exercise, rifampicin, anticoagulants Investigations: - Urine dip +/- MCS - Bloods incl. clotting - Renal USS or CT renal w/ contrast - Flexi-cystoscopy Management: - Exclude transient causes - 2ww - if > 45 with VH or > 60 with non-VH
58
Renal stones
Risk factors: - Male - testosterone increases liver oxalate production - women have higher urinary citrate - Family history - Dehydration - esp. urate and cysteine stones - High oxalate diet - meat, spinach - Hypercalciuria - RTA, hyperparathyroidism Types: - 80-85% calcium oxalate - 10% mixed calcium oxalate/phosphate - 5-10% urate - 2-20% struvite - 1% cysteine Investigations: - Urine dip - Microscopic haematuria - r/o possible UTI -> struvite stones - CT KUB - Radio-opaque: Ca phos > ca oxalate > cysteine > struvite > uric acid Management: - Analgesia - PR diclofenac, tamsulosin - < 5mm - Watch and wait - < 2cm - Lithotripsy, or ureteroscopy if pregnant - > 2cm, obstructive, staghorn - Percutaneous nephrolithotomy +/- nephrostomy
59
Cystinuria
Autosomal recessive disorder - Impaired renal and GI transport of dibasic amino acids (cysteine, ornithine, arginine, lysine) Features: - Up to 3% will produce cysteine renal stones (radio-translucent) - Yellow/brown hexagonal crystals on MCS - Postive urinary cyanide nitroprusside test Management: - High fluid intake - Urinary alkalisation - bicarbonate/citrate
60
ADPCKD
Epidemiology: - Autosomal dominant mutations in PKD 1/2 - Presents between 30-50 yr Pathophysiology: - Polycystin-1/2 mutations lead to dysfunction of cilia and cell junctions - Form large fluid-filled cysts which cause ischaemic atrophy of surrounding parenchyma as well as obstruction of tubules Features: - Polycystic kidneys - Prone to infection, haemorrhage, etc - Progressive renal dysfunction - HTN, oedema, stones - Liver cysts (33%) - Berry aneurysms (30%) - Aortic root dilatation - With associated MR, TR, etc. - Increased risk of renal adenomas Investigations: - Urinalysis + bloods - Imaging - Renal US, CT, MRI - Genetic testing Management: - Early - Monitoring - Antihypertensives - Hydration - Late - Requires RRT - dialysis or transplant
61
Sterile pyuria - aetiology
Infectious: - Renal TB - Perinephric abscesses - Chronic prostatitis - Fungal UTIs Non-infectious: - Drugs - lithium, heavy metal toxicity - Renal stones - Sarcoidosis - Interstitial cystitis - Polycystic kidney disease - Urinary tract malignancies - Renal transplant rejection
62
Urinary casts
Hyaline - Not pathological if occurring in isolation Red cell casts - Always pathological, usually an underlying glomerulonephritis White cell casts - Acute pyelonephritis or interstitial nephritis Granular casts - Tubular or interstitial disease, although finely granular casts can be normal in paeds
63
Renal tumours
Benign: - Adenoma - symptomless Malignant: - Renal cell carcinoma (80% of adult) - M > F - Asytmpomatic, haematuria (50%), loin ache (40%), loin mass (25%), unilateral varicocele (1%) - Paraneoplastic manifestations possible - HTN (renin), hypercalcaemia (PTH), polycythaemia (EPO) - Nephroblastoma - Most common paediatric renal cancer Metastases: - Generally rare but can be from breast, lung, or haematological malignancies
64
Bladder tumours
Epidemiology: - 90% urothelial carcinoma - Middle-aged - Risks - smoking, long-term catheterisation, beta-naphthylamine exposure - Squamous cell carcinoma - Associated with schistosomiasis Features: - Painless haematuria - LUTS - frequency, urgency, dysuria - Possible spread to other pelvic viscera Investigations: - Urine dip - Bloods - Flexi-cystoscopy +/- CT KUB for staging Management: - Superficial = resection + BCG + mitomycin C - Muscle-invasive = platinum chemotherapy + cystectomy -
65
Bladder stones
Similar types and causes as renal stones, many will be formed in kidney and pass into bladder Bladder-unique causes include foreign body (catheter) or stasis (stricture, BPH, atony) Features: - Pain - Suprapubic, perineal, or tip of penis - Worse at end of micturition (bladder contracted) - Urinary frequency - Worse during the day as upright position allows stone to irritate the trigone - Terminal haematuria
66
Pneumaturia
Air or bubbles in the urine, or a history of sputturing passage of urine Aetiology: - Colovesical fistula - Diverticular disease (80%), IBD, colorectal or bladder cancer - Often also have faecuria - Air-forming UTIs Investigations: - Urine dip + culture - Renal US - CT +/- PR contrast - Colonoscopy
67
Testicular torsion
Epidemiology: - Mostly boys aged 10-15 yr - Uncommon in over-30s Features: - Sudden unilateral testicular pain - Although may have non-specific abdo pain - Nausea and vomiting - Tender swollen testis - High in scrotum - Oedematous + erythema Management: - Urgent surgical exploration +/- fixation or orchidectomy - Must happen within 1hr - Ischaemia occurs within 4hr but testis can be salveagable up to 8hr after pain Differentials: - Torted hydatid cyst or epididymal appendix
68
Acute epididymo-orchitis
Aetiology: - Bacterial infection: - Younger men = chlamydia, Older men = E. coli - Viral infection incl. mumps Features: - Gradual onset unilateral pain and swelling - Initially epididymis thickened + tender, then hemiscrotum becomes oedematous and erythematous - Fever - Dysuria - Lymphadenopathy Management: - IV fluids - Antibiotics - 2-6 weeks - May form abscesses requiring IV gentamicin + surgical drainage
68
Fournier's gangrene
Necrotising fasciitis of the scrotum, perineum, and/or perianal region - Often spread from a urinary or anorectal infection Management: - Urgent surgical debirdement - Catheterisation if penile involvement - IV antibiotics
69
Autonomic dysreflexia
Seen in patients with spinal cord injury above the level of T6 Symptoms most commonly triggered by bladder problems e.g. calculi, UTI, instrumentation Symptoms: - Hypertension - Bradycardia - Flushing + diaphoresis - Palpitations - Blurred vision
70
Balanitis
Aetiology: - Infection - Bacterial - STIs - Fungal - candida - Increased risk with diabetes or HIV Features: - Penile soreness + itch - Bleeding + odour from foreskin - Dysuria + dyspareunia - Redness, swelling, and exudate over glans - Tightening or phimosis of the foreskin Investigations: - Urine dip - Foreskin swab - for candida and STIs Management: - Avoid triggers - Clean daily with water - Topical hydrocortisone + imidazole cream - Flucloxacillin if confirmed bacterial infection
71
Prostate cancer
Epidemiology: - Over-50s - Most common cancer in men Investigations: - DRE - hard, craggy surface - PSA - >20 ng/ml suggestive of disseminated disease - Imaging: - Multiparametric MRI - Transrectal USS for biopsy - Bone scan if PSA > 20 or bony pain Gleeson scoring: - Based on the tissue architecture - Scores <= 6 suggest low risk, 7 is intermediate, >= 8 is high risk Management: - Watch and wait - If low risk, well-differentiated, no mets, life- expectancy < 10yr - Radial prostatectomy and/or radiotherapy - removal of prostate, seminal vesicles, pelvic lymph nodes - GnRH antagonists - symptom relief in metastatic disease
72
Testicular cancers
Germ cell tumours - Risk factors - undescended testis, dysgenesis, genetics - Seminoma (50%) - Less aggressive, usually curable with orchidectomy - NSGCT - Teratoma - Yolk sac - Most common testicular cancer in children - Testicular much less aggressive than ovarian - AFP secretion - Choriocarcinoma - Highly aggressive - bHCG secretion - Embryonal Non-germ cell tumours - Lymphoma
73
Nutcracker syndrome
A vascular compression disorder - compression of the L renal vein - Usually between SMA and aorta Can be anatomical but can also be secondary to retroperitoneal or pancreatic cancers or AAA Features: - Renal venous hypertension - Flank pain - Haematuria - Increased risk of renal vein thrombosis Investigations: - Urine dip - CT abdomen
74
Retroperitoneal fibrosis
Aetiology - Idiopathic (70%) - Drugs - methylsergide, etanercept - Malignancy - AAA Features: - Malaise - Back pain - Normocytic anaemia - Uraemia - Raised ESR Investigations: - Bloods - Anaemia, uraemia, raised ESR - CT/MRI - Bilateral ureteric obstruction at level of pelvic brim +/- periaortic mass Management: - Surgery with biopsy + ureteric stenting - Trial of long-term steroids or steroid-sparing agents
75
Chronic reflux disease
Features: - Recurrent UTIs - HTN - Premature renal impairment - Renal atrophy Investigations: - Urinalysis - proteinuria - Imaging - VCUG Management: - Intermittent antibiotic therapy - Surgical correction of any anatomical abnormalities
76
Hyperoxaluria
Aetiology: - Enteric - short bowel syndrome - Dietary - meat, spinach - Male sex - testosterone Features: - Recurrent oxalate stones Management: - Hydration - Calcium supplementation (binds excess free oxalate to enhance excretion) - Low oxalate diet
77
Renal transplant rejection
Hyperacute - Presence of recipient antibodies against the donor kidney - Features: - Within minutes of revascularisation - Kidney swelling + discolouration - RBC clumping + haemorrhage, fibrin deposition - Management: - Transplant nephrectomy Acute - Within 4 weeks of transplant - Either cell-mediated or antibody-mediated - Management: - Increased immunosuppressive therapy Chronic - > 3 months after transplant - Gradual worsening of kidney function - HTN, proteinuria are key markers - Usually due to transplant vasculopathy
78
Xanthogranulomatous pyelonephritis (XGP)
A rare and aggressive form of chronic pyelonephritis Histology: - Enlarged kidney - Destruction and replacement of renal and peri-renal tissue with granulomas and lipid-rich macrophages Features: - Weight loss - Flank pain and mass - Fevers - Anorexia Investigations: - CT abdomen - Replacement of renal parenchyma with rounded, low-density areas surrounded by a ring of enhancement Management: - Antibiotics in acute phase, nephrectomy in chronic phase
79
Lupus nephritis - staging
The spectrum of SLE-associated renal disease characterised by diffuse immune complex deposition - Immune deposits along tubular and glomerular basement membranes - Subendothelial, subepithelial, and mesangial deposits - Tubular reticular structures on EM - Systemic depletion of complement Class I - Minimal mesangial lupus nephritis Class II - Mesangial proliferative lupus nephritis Class III - Focal lupus nephritis Class IV - Diffuse lupus nephritis Class V - Membraneous lupus nephritis Class VI - Advanced sclerotic lupus nephritis
80
Renal artery fibromuscular hyperplasia
A cause of renovascular HTN - 5% of all HTN Features: - Occult HTN with hypertensive eye disease - Periodic pulmonary oedema - Mild hypokalaemia - Worsening renal function post-ACEi - RAAS overactivation Investigations: - Urine - Bloods - Renal US - normal - Renal angiography - arterial stenosis, often in a rosary bead appearance Management - HTN control - ACEi / ARBs - Angioplasty Prognosis: - Variable course and may not progress (vs atherosclerotic disease)
81
Renal tract TB
Initial formation of glomerular granulomas which eventually rupture and lead to medullary disease Features: - Sterile pyuria - Haematuria - Dysuria - Refractory HTN - Renal calcification Management: - Anti-mycobacterial agents
82
Pollakuria
aka benign urinary frequency Aetiology: - Idiopathic - esp paeds - Drugs - risperidone Features: - Increased daytime urinary frequency - Enuresis - Urinary incontinence