All Topics Flashcards
Feature of liddles syndrome
Autosomal disorder = salt-sensitive hypertension characterized by a very high rate of renal sodium uptake. Mutation of ENAC channel. The increased sodium uptake is accompanied by an increased water uptake, leading to an increase in blood volume and secondary hypertension.
Low K+
Metabolic alkalosis
Hypertension
Low renin
low aldosterone
treatment - Amiloride
Features of Gitelman syndrome
Low K +, hypochloremic
Normontensive
Low serum mg2+ but high urine magnesium due to wasting
Low calcium in urine - therefore no stones
Urine prostaglandins = normal
HIGH URINE CL- AND MG2+
Minics thiazide diuretics
How to calculate free water deficit
Serum Na - 140 / 140 x total body water
Which antihypertensives can you give in pregnancy and breast feeding
Pregnancy - Labetolol, Nifedipine, methyldopa
Breast feeding - Same as above - although methyldopa causes risk of post natal depression. You can also use Enalipril
Medications contraindicated in pregnancy
MMF
Cyclophosphamide
Sirolimus
ACEi
Ciprofloxacin
Methotrexate
Criteria for pre-eclampsia
Raised BP (>140/90)
Proteinuria (>3g/L)
>20 weeks
Treatment of pre eclampsia
Aspirin 75mg OD
Labetolol
IV magnesium in eclampsia
Deliver
Pre eclampsia markers
PLGF - Goes down in pre eclampsia
SFT1 - Increased in pre eclampsia
Diagnosis - Increased SFT1: PLGF ratio (>85) or if PLGF <12
Note - Raised Urate in pre-eclampsia
Urea in pregnancy
If urea >17 then start dialysis
If on HD then aim to keep urea <12
Triad of Alports Syndrome
Family history of progressive nephropathy
Sensorineural deafness
Ocular abnormalities
Features of Alports
Anterior lenticonus
X linked recessive so males are affected and female are carriers
Female carriers may not have triad of symptoms but have only haematuria, progressive to ESKD later and often no hearing loss
Presentation tends to worsen with time - starts as heamaturia around age 1o then increasing BP and UPCR with hearing loss and eye issues later
Gene affected in Alports
COL4AE - codes for Type IV collegen
Barters syndrome - features
parelell to Gitlemans
Low BP. Low K+, alkalosis, hypochloremic
HIGH URINE CALCIUM and Chloride
High urine prostoglandins
Rare autosomal disorder
Mutations of the ion transporter or ion channel present in the thick ascending limb of the distal nephron.
Mimics Loop diuretics
Treatment for SIADH
Demeclocycline - Blocks action of ADH at the DCT = Increases free water clearance
What happens in water deprivation test to urine and serum osmolarity in = psychogenic polydipsia, cranial DI and nephrogenic DI
cranial DI the plasma osmolality following the water deprivation test would rise and following administration of desmopressin =increased urine osmolality
Nephrogenic diabetes insipidus = plasma osmolality would rise during the water deprivation test, but the urine would remain dilute, even after desmopressin administration.
psychogenic polydipsia - Serum osmolarity is unchanged but urine osmolarity increases with water deprivation
Types of RTA
Type 1 = distal RTA
Type 2 = Proximal
Type 4 = hyperkalemic distal
Features of Type 1 RTA
Distal RTA
Impaired H+ excretion = causes acidosis
hypokalaemia, Low serum bicarb, high urine bicarb - urine pH >5
low phosphate, high urinary calcium
normal anion gap metabolic acidosis, high urinary anion gap
urinary stone formation (alkaline urine, hypercalciuria)
Caused by Sjorgens, SLE
Tx - potassium citrate and Po bicarb
Features of Type 2 RTA
Proximal RTA = Type 2.
Impaired HCO3- reabsorption = causes acidosis
hyperchloraemic metabolic acidosis
Low K+, low bicarb,
normal urine Ph <5.3
Tx = thiazide and Potassium bicarbonate
Causes - Fanconis, myeloma, drugs’
What are the features of Fanconis syndrome
dysfunction of the renal proximal tubule
Inability to absorb / wasting of = HCO3- and salt as well as volume depletion, and potassium wasting
Urine = increased Glucose, phosphate, uric acid and citrate
Serum = low phosphate , urate and Vit D
Causing bone disease,
Features of Type 4 RTA
Distal
Hyperaldosteronism
Impaired excretion of K+ and H+ into the DCT
Reduced aldosterone leads to increased K +
High urine anion gap
Tx = Fludrocortisone
Causes = DM, Addison’s, NSAIDs
Key differences between RTA types
Type 1 = Urine Ph >5.3 - unable to add acid
Type 2 = Urine Ph <5.3 - still able to acidify the urine
Type 4 = Hyperkalemia
Diagnostic features of Fabrys
Lysosomal storage disorder
X linked so men more affected but women can be carriers
Deficiency of Alpha GLA
Bloods = Reduced alpha GLA, increased GL3 levels
Renal Bx = Zebra bodys, foamy inclusions, podocyte vaculation
Treatment - Apha GLA enzyme replacement
Symptom and time line associated with Fabrys
Child = Neuropathic pain in hands and feet
Adolescents = Heat sensitivity, tinitus, deafness
Adult = ESKD, CVS and CNS involvement
Features of TTP
Fever, Hemolysis, thrombocytopenia, renal and neurological involvement / Confusion
Check ADAMTS 13 (often <50%)
Treatment = PLEX and steroids
Hypercalcemia and malignancy
Myeloma = CRAB
Lymphoma - caused raised Vit D levels (increased 25-OH vit D to 1,25 OH-Vit D
iPTH would be raised in parathyroid cancers
Solid cancers cause bone mets
PTHrp = ectopic production by some solid tumours
Active and inactive Vitamin D
25-OH = In active
1,25 OH-Vit D = active (Calcitrol)
PTH causes activation of 25-OH to 1, 25OH.
1,25 OH causes negative feedback to inhibit conversion
What is hungry bone syndrome
Occurs after parathyroidectomy
Develop hypocalcemia and hypophosphateaemia
The stimulation on osteoclasts is removed so increased osteoblast activity - causing calcium, PO4- and magnesium to be taken up by the bones and reduced in serum
Tx =IV calcitrol (active Vit D - which aims to increase ca absorption from gut and increase osteoclast activity)
BP aims in those with CKD - Proteinuria and no proteinuria
CKD + Protein or those with diabetes= <120-130/80
CKD + NO protein and not diabetic = <140/90
Anaemia aim in CKD
Hb >100
ferritin >200mcg
TSATs >20%
When to start EPO in CKD and key facts
When Hb <100, Iron replete
Starting dose = 30mcg weekly (0.45mcg/kg/week)
Risks = Stroke, raised BP, VTE risk
Note - ACEi reduce EPO production and infection reduces how effective EPO works
Stop when HB >13, target range for Hb 10-12
Increase by 25% each month to achieve Hb. If Hb rises >2 each month reduce dose by 25-50%
How to investigate EPO resistance
Defined as EPO >1.5 mcg/kg/weekly with no improvement
Check reticulocyte count = if Raised = Appropriate marrow response = ?bleeding.
If low then ? issue with RBC production or poor compliance
Causes of EPO resistance = Hyperparathyroidism
If low reticulocyte count - then consider stopping EPO as it can cause pure red cell aplasia - mainly with epoetin Alfa
pathophysiology of iron deficiency in CKD
Hepcidin synthesized by the liver - raised levels cause reduced iron absorption for the gut.
Hepcidin is stimulated by chronic inflammation, CKD or raised iron levels. EPO reduces hepcidin which causes increased reabsorption
In CKD + low levels of EPO = Increased hepcidin = Reduced GI absorption of iron = Anemia
Iron replacement in CKD / HD
HD = increase frequency and reduce dose of Iron
CKD = decrease frequency and increase dose
PO iron first line unless not tolerated
Features of primary, secondary and tertiary hyperparathyroidism and causes
Primary Hyperparathyroidism = HIGH Ca, low PO4-, high PTH, normal/high Vit D - Parathyroid adenoma
Secondary Hyperparathyroidism = low Ca, normal PO4-, high PTH, LOW Vit D - Vit D deficiency or CKD
Tertiary Hyperparathyroidism = high Ca, high PO4-, HIGH PTH, low Vit D - ESKD
Features of Cholesterol Emboli
Livedio reticularis, loin pain, AKI
Bland urine dip
Raised eosinophils, low C3/C4
Features of FMD
Fibromuscular dystrophy
Raised BP, normal renal function
Develop arterial fibroplasia = string of beads of both renal arteries
Tx = Angioplasty
Can also get carotid involvement causing stroke etc
Indications for RAS stenting
Flash pulmonary oedema
Treatment resistant hypertension
Deteriorating renal function
Kidneys need to be >8mm and >70% stenosis to see benefit
Treatment of calciphylaxsis
Stop warfarin, calcium and vitamin D supplements
Hyperbariac oxygen and wound care
cinacalcet and Sodium thiosulphate
NICE guidelines on risk factors for CKD and who to screen
Diabetes
Hypertension
Cardiovascular disease
Structural renal tract pathology
Multisystem disease with potential renal involvement
Opportunistically detected haematuria or proteinuria
A family history of stage 5 chronic kidney disease, or inherited kidney disease.
Features of Sickle cell anaemia and the kidney
Anaemia, raised reticulocyte count and low MCV
Causes papillary necrosis - presents as haematuria
Treatment is conservative, prolonged haematuria may require RBC transfusion
Key Differences between Gittlemans and Barters
Both low K+, normal BP, hypochloremic alkalosis
Barter - High urinary calcium
Gittlemans - low serum magnesium, high urine magnesium, low urine calcium
Features of Papillary Necrosis
AKI and haematuria
The necrosed papilla cause obstruction of the ureter = ureteric colic and AKI
associated with NSAIDs
CT - looks obstructed but no evidence of stone - just some ill defined soft tissue density
Complication of Orlistat treatment
Anti obesity drug
Causes hyperoxaluria
Increases risk of oxalate nephropathy
Management of hypertensive emergencies
1) Oral nifedipine / labetolol /GTN
2) B Blocker
3)ACEi + diuretics
4)Alpha blocker - doxazosin
Features and management of scleroderma renal crisis
BG of scleroderma - Raynards, CREST syndrome, etc
Positive RNA polymerase III antibody
Increased BP + Proteinuria
Renal Bx = onion skin appearance and hypertensive changes
Treatment = ACE inhibitor
Management of HTN in the community
Aged <55 = ACE inhibitor
Aged>55 / black = CCB
2) A+C
3)A+C+D (thiazide)
4) A+C+D +BBlocker or alpha blocker
What is the best BBlocker to use in a hypertensive diabetic patient
Carvedilol
Superior to metoprolol, atenolol and bisoprolol. Doesn’t deteriorate insulin sensitivity
What happens to Urine Na and cl- in vomitting, diarrhoea and diuretics
Vomiting - High urine Na, low urine Cl-
Diarrhoea - Low urine Na, high urine Cl-
Diuretics - High urine Na and Cl-
(Similar ratio of Na and Cl suggests diuretics or tubulopathy)
Effect of thiazides of urinary calcium
low Urine Ca
How to approach bilateral cystic kidney disease
Solid renal mass + renal cell cancer = von hippel lindau syndrome
Solid renal mass no cancer = Tubulosclerosis
Enlarged kidneys + liver fibrosis = AR PKD
Enlarged kidneys no liver fibrosis = AD PKD
Normal sized kidneys / corticomedullary cysts
- Autosomal dominant = Medullary cystic kidney disease (see other card)
-Autosomal recessive = Nephronophthsis - autosomal recessive tubulointerstial disease, early ESKD in childhood, slowly progressive, salt wasting.
Difference between medullary sponge kidney and medullary cystic kidney disease
Medullar sponge kidney =Developmental issue that results in impaired calcium handling and calcium stones.
Medullary cystic kidney disease - Autosomal dominant condition causes corticomedullary cysts
Types of medullary cystic kidney disease
Gout and raised urate = UMOD
Childhood hypertension = REN
Diabetes and issues with urinary tract = MODY 5 gene = HNF1-B
Difference between cystinuria and cystinosis
Cystinuria = excessive excretion of cysteine - causes cysteine stones which are hexagonal and treated with D Penicillinium
Cystinosis - autosomal recessive lysosomal storage disorder - causes growth failure, Fanconi’s, renal impairment, eye and nerve issues
Key features of Alports syndrome
X-linked - men affected, women carriers
CLO4A5 gene = codes for IV collagen which makes up GBM, Cochlea and eyes
Features of thin basement membrane disease
Similar to Alport’s. COL4A3-4 and type 4 collagen disorder
Present with hematuria, no extra renal manifestation
BM <200nm on EM
Nail Patella syndrome
Autosomal dominant
Nails are abnormal or not present, also get knee and elbow issues, patella may dislocate
50% renal involvement - irregular basement membrane which loos ‘ moth eaten’
Which genes are affected in Polycystic kidney disease?
PKD 1 - associated with AR PKD and liver fibrosis, chromosome16
PKD 2 - Milder type, Chromosome 4
Both PKD 1 &2 are involved in signaling proteins to regulate cell proliferation in the kidney - defects mean proliferation becomes out of control - leads to scaring and CKD
Number of cysts and age for PKD diagnosis
Aged <30 = FHx + 3 cysts, no FH then 5+ cysts
Aged 30-60 = FHx + 4 cysts (2 each side), no FHx 5+ cysts
Aged >60 = FHx + 8 cysts (4 each side) , no FHx then 8+ cysts
Between 20-40 yrs a negative US should be followed up by CT scan
On CT scan >10 cysts is diagnostic
Criteria for Tolvapatan, how it works and side effects
> 2.5ml egfr loss a year, for >5yr
5ml loss in one year
Increase kidney size by >5%
Can caused hepatotoxicity and should be discontinued if transaminitis occurs
ACEi is the treatment of choice in PKD patients
Works as a selective inhibition of vasopressin V2 receptor
Additional features associated with polycystic kidney disease
Urate stones
25% have mitral valve prolapse
Raised BP
Treatment of Tubulosclerosis
mTOR inhibitors - Sirolimus or Everolimus
Features of IgG 4 disease
IgG4 can mimic cancer
Pancreatic involvement is common
Renal involvement -causes enlargement with nodules or masses
Low complements, eosinophils, raised IgE
Features of Dents Disease
X-linked recessive condition - affects males
CLCN5 gene
Presents in childhood
Symptoms = nephrocalcinosis / stones, proteinuria and hypercalcemia
Features of HUS
Triad of:
1) Haemolytic anemia
2) AKI
3) Thrombocytopenia
Infection, pregnancy, E.Coli can be a trigger.
Management of Lithium toxicity and indication for RRT
Indications = neurological symptoms or serum lithium level (>3.5 mmol/L).
HD needed rather than CVVH.
Tend to need several sessions as lithium tends to rebound post HD.
Differentiating deranged LFTs and platelet abnormality in pregnancy
TTP - Normal BP, neuro symptoms, ADAMTS13 - Tx= PLEX and steroids
HUS - Occurs post partum = Eculizumab
Acute fatty liver - reduced glucose
HELLP = Hypertension and proteinuria, occurs 3rd trimester
DIC - abnormal clotting
Features of Porphyria
Build up of porphyrins due to liver disorder which affect skin and nervous system
Symptoms are rapid onset and short duration - chest and abdo pain, confusion, fever, raised BP
Causes hyponatremia
Urine goes purple in sunlight
Which HLA antigen is associated with Goodpasture’s
HLA - DR2
Indication for Cinacalcet
treatment of refractory tertiary hyperparathyroidism in patients with ESKD
Those with ‘very uncontrolled’ levels of PTH and high calcium level, and in whom surgical parathyroidectomy is contraindicated
Translocational Hyponatremia
serum sodium is spuriously low
Occurs in context of elevated glucose levels
Serum osmolarity differentiates between true hyponatremia and pseudo-hyponatraemia.
The degree of reduction of serum sodium in relation to blood glucose levels. These changes should resolve with correction of glucose and triglyceride levels.
Features of Lysozyme induced nephropathy
Lysozyme is an enzyme found in macrophages.
In patients with CML large amounts are released into the plasma
The lysozymes get filtered through glomerulus = non albuminic proteinuria
Diagnosis - biopsy - IHC positive for lysosomes in the proximal tubules
Tx = chemo and supportive therapy
Lupus flare in pregnancy
Increased risk of relapse by 40-60%
Gold standard for measuring GFR in pregnancy
24hour urine collection for creatinine clearance
Most tools unhelp for measuring GFR in pregnancy
Treatment of hypoklaemia in Gitlemans
Amiloride
Indications for Bx during pregnancy
New onset GN/ lupus, severe nephrotic syndrome needing treatment, acute decline in renal function
Bx up to 23 weeks but not thereafter
What mechanism of RRT is best for clearing drugs
Hemodialysis > CVVH
Raised PTH
9x upper limit of normal
ICA in PKD patients - Risk factors and who to treat
Risk factors for ICA = smoking, hypertension, and family history of ICA or SAH.
Screening is only for those with FHx ICA or SAH, employed in high-risk occupations (e.g., airline pilots or bus drivers), or those who request screening.
Patients with FHx as their only risk factor are advised to have MRI/MRA every 5–10 years.
Patients who have aneurysms <7 mm in diameter, engage in high-risk occupations, or are planning conception, transplantation, or other major surgery are advised to have MRI/MRA every 1–5 years.
ICA >10 mm in diameter have a relatively high risk of rupture and should be repaired by surgical clipping or endovascular intervention such as deployment of a coil.
Electrolyte effects with lithium
Causes hypercalcemia due to increased calcium reabsorption in tubules and GI tract. Can also cause raised PTH as well
May need treatment with cinacalcet
Bone pain
Vit D / Osteomalacia = generalized bone pain, muscle weakness
Lytic bone lesions = Myeloma = pain at site of lesion,
Metastatic bone lesions
Renal osteodystrophy = occurs in ESKD, prolonged hyperparathyroidism
Features of renal infarction
Often in context of risk factors = e.g A.F
Presentation - Sudden onset flank pain, N+V, fever, flank tenderness. Hematuria
Bloods = Raised LDH, raised lipase, inflammatory markers etc
Treatment = If >24hrs then likely conservative.
Consider anticoagulation if in AF or other risk factors
How to determine renal infarction from atheroembolism
atheroembolism affects smaller vessels including arterioles, usually resulting in glomerular ischemia or interstitial nephritis from inflammatory cell infiltration rather than large territory infarction.
Atheroembolism, usually results in a subacute but progressive decline in kidney function.
Other features = livedo reticularis, purpura, digital ischemia, bowel ischemia, and pancreatitis.
Eosinophilia and C3 hypocomplementemia may be seen
Test to do in hepatitis B-associated membranous nephropathy
Hepatitis B e-Antigen = HBeAg
Causes secondary MN
Circulating HBeAg is specifically associated with hepatitis B-associated membranous nephropathy, and subepithelial HBeAg deposition is implicated in the pathogenesis of this disease.
Features of salicylate poisoning
Respiratory alkalosis secondary to raised RR
Raised anion gap metabolic acidosis
Neuroglycopenia = normal blood glucose but lack of glucose for the brain
Treatment = Dextrose, HD
Contrast CT of kidneys that shows a ‘rim sign’ and anuria
Suggestive of renal cortical necrosis
Often seen in pregnancy related events or pancreatitis
Drug effects on the kidney
Chemotherapies. HIV treatment etc
Gemcitabine - TMA
Cisplatin and Ifosamide = ATN
Ipilimumab = AIN
Tenofovir - Causes fanconis
-Interferon - Causes FSGS
Differences in PAN versus FMD
FMD = Female >male, String of beads. Treatment angioplasty
PAN = Male>female, Hep B, Raised ESR / CRP. Treatment Tx = steroids, cyclophosphamide
Pheochromocytoma - diagnosis and treatment
urinary markers are the most sensitive
Treatment = Alpha channel blocker and then surgery
GRA - Glucocorticoid‐remediable aldosteronism
Autosomal dominant condition
Ectopic expression of aldosterone synthase
cause early onset hypertension
Similar to conns but have FHx and younger onset
Treatment = Dexamethasone
AME - Apparent mineralocorticoid excess
Autosomal recessive disorder
Presents similar to Liddles - low renin and low aldosterone
Causes low urinary free cortisone compared to cortisol
Treatment = ?Spiro
Liquorice and antifungals creates this effect
Can determine difference between AME and Liddles by urinary cortisol / cortisone. Urine cortisol will be raised in liddles compared to free cortisone.
Gordons syndrome
Causes Pseudohypoaldosteronism
Hypertension, raised K+, acidotic
Low renin, normal aldosterone
Treatment = thiazide
Gene mutations in TBM and Alports
Thin basement membrane disease = COL4A3 and COL4A4 genes
Alport’s = COL4A5
Alternative name for MCD
Lipoid Nephrosis
Causes for Pseudohyperphosphatemia
Hyperglobulinemia secondary to Myeloma
Hyperlipidemia, hyperbilirubinemia
Contamination with heparin
Antifungals
What blood test finding is associated with Post streptococcal GN?
Low CH 50 level
TB drugs and common effects
Rifampicin - CYP inducer, Red secretions
Isoniazid - Neuritis, hepatitis
Pyrazinamide - Causes Gout
Ethambutol - Eye issues - optic neuritis
Urinary losses in nephrotic syndrome
Anti-thrombin III
Thyroxine binding globulins = low thyroxine levels
Immunoglobulins
Features of Milk Alkali syndrome
Hypercalcemia and metabolic alkalosis
The hypercalcemia causes diuresis and dehydration / AKI
Made worse by calcium based phosphate binders and bisphosphonates
treatment =- IV fluids top calcium intake +/- furosemide
4 main plasma cell dyscrasias
1)Myeloma / Cast nephropathy = fractured casts
2)AL amyloid = Associated with Lambda LC
3)Monoclonal immunoglobulin deposition disease = Made up of Light chain deposition disease (Kappa LC) and fibrillary / immunotactoid
4)Cryoglobulinaemia
Treatment of Adynamic Bone Disease
Occurs in chronic HD patients - often due to high doses of calcium which suppresses PTH
Bloods = Raised calcium, low PTH
Risk of fractures and vascular complications are high
Treatment
-Stop Vit D and calcium supplements
-Non calcium phosphate binder
-Low calcium dialyzers
What antihypertensive should be given if history of gout
Losartan
Tubulo-Interstial Nephritis and Uveitis syndrome
More common in young patients
Raised ESR and deranged LFTs, low Hb
Tx = Steroids
Features of AIN caused by checkpoint inhibitors
E.g Ipilimumab
Hyponatremia, hypokalemia, hypophosphateaemia
Recommended protein intake in CKD stage 3-5
0.55-0.6g/Kg day if well and stable or 0.6-0.8kg/day in diabetics
