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1
Q

Feature of liddles syndrome

A

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

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2
Q

Features of Gitelman syndrome

A

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

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3
Q

How to calculate free water deficit

A

Serum Na - 140 / 140 x total body water

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4
Q

Which antihypertensives can you give in pregnancy and breast feeding

A

Pregnancy - Labetolol, Nifedipine, methyldopa
Breast feeding - Same as above - although methyldopa causes risk of post natal depression. You can also use Enalipril

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5
Q

Medications contraindicated in pregnancy

A

MMF
Cyclophosphamide
Sirolimus
ACEi
Ciprofloxacin
Methotrexate

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6
Q

Criteria for pre-eclampsia

A

Raised BP (>140/90)
Proteinuria (>3g/L)
>20 weeks

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7
Q

Treatment of pre eclampsia

A

Aspirin 75mg OD
Labetolol
IV magnesium in eclampsia
Deliver

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8
Q

Pre eclampsia markers

A

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

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9
Q

Urea in pregnancy

A

If urea >17 then start dialysis
If on HD then aim to keep urea <12

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10
Q

Triad of Alports Syndrome

A

Family history of progressive nephropathy
Sensorineural deafness
Ocular abnormalities

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11
Q

Features of Alports

A

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

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12
Q

Gene affected in Alports

A

COL4AE - codes for Type IV collegen

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13
Q

Barters syndrome - features

A

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

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14
Q

Treatment for SIADH

A

Demeclocycline - Blocks action of ADH at the DCT = Increases free water clearance

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15
Q

What happens in water deprivation test to urine and serum osmolarity in = psychogenic polydipsia, cranial DI and nephrogenic DI

A

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

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16
Q

Types of RTA

A

Type 1 = distal RTA
Type 2 = Proximal
Type 4 = hyperkalemic distal

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17
Q

Features of Type 1 RTA

A

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

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18
Q

Features of Type 2 RTA

A

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’

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19
Q

What are the features of Fanconis syndrome

A

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,

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20
Q

Features of Type 4 RTA

A

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

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21
Q

Key differences between RTA types

A

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

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22
Q

Diagnostic features of Fabrys

A

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

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23
Q

Symptom and time line associated with Fabrys

A

Child = Neuropathic pain in hands and feet
Adolescents = Heat sensitivity, tinitus, deafness
Adult = ESKD, CVS and CNS involvement

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24
Q

Features of TTP

A

Fever, Hemolysis, thrombocytopenia, renal and neurological involvement / Confusion
Check ADAMTS 13 (often <50%)
Treatment = PLEX and steroids

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25
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
26
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
27
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)
28
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
29
Anaemia aim in CKD
Hb >100 ferritin >200mcg TSATs >20%
30
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%
31
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
32
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
33
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
34
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
35
Features of Cholesterol Emboli
Livedio reticularis, loin pain, AKI Bland urine dip Raised eosinophils, low C3/C4
36
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
37
Indications for RAS stenting
Flash pulmonary oedema Treatment resistant hypertension Deteriorating renal function Kidneys need to be >8mm and >70% stenosis to see benefit
38
Treatment of calciphylaxsis
Stop warfarin, calcium and vitamin D supplements Hyperbariac oxygen and wound care cinacalcet and Sodium thiosulphate
39
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.
40
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
41
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
42
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
43
Complication of Orlistat treatment
Anti obesity drug Causes hyperoxaluria Increases risk of oxalate nephropathy
44
Management of hypertensive emergencies
1) Oral nifedipine / labetolol /GTN 2) B Blocker 3)ACEi + diuretics 4)Alpha blocker - doxazosin
45
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
46
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
47
What is the best BBlocker to use in a hypertensive diabetic patient
Carvedilol Superior to metoprolol, atenolol and bisoprolol. Doesn't deteriorate insulin sensitivity
48
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)
49
Effect of thiazides of urinary calcium
low Urine Ca
50
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.
51
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
52
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
53
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
54
Key features of Alports syndrome
X-linked - men affected, women carriers CLO4A5 gene = codes for IV collagen which makes up GBM, Cochlea and eyes
55
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
56
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'
57
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
58
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
59
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
60
Additional features associated with polycystic kidney disease
Urate stones 25% have mitral valve prolapse Raised BP
61
Treatment of Tubulosclerosis
mTOR inhibitors - Sirolimus or Everolimus
62
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
63
Features of Dents Disease
X-linked recessive condition - affects males CLCN5 gene Presents in childhood Symptoms = nephrocalcinosis / stones, proteinuria and hypercalcemia
64
Features of HUS
Triad of: 1) Haemolytic anemia 2) AKI 3) Thrombocytopenia Infection, pregnancy, E.Coli can be a trigger.
65
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.
66
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
67
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
68
Which HLA antigen is associated with Goodpasture's
HLA - DR2
69
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
70
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.
71
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
72
Lupus flare in pregnancy
Increased risk of relapse by 40-60%
73
Gold standard for measuring GFR in pregnancy
24hour urine collection for creatinine clearance Most tools unhelp for measuring GFR in pregnancy
74
Treatment of hypoklaemia in Gitlemans
Amiloride
75
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
76
What mechanism of RRT is best for clearing drugs
Hemodialysis > CVVH
77
Raised PTH
9x upper limit of normal
78
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.
79
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
80
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
81
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
82
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
83
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.
84
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
85
Contrast CT of kidneys that shows a 'rim sign' and anuria
Suggestive of renal cortical necrosis Often seen in pregnancy related events or pancreatitis
86
Drug effects on the kidney Chemotherapies. HIV treatment etc
Gemcitabine - TMA Cisplatin and Ifosamide = ATN Ipilimumab = AIN Tenofovir - Causes fanconis -Interferon - Causes FSGS
87
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
88
Pheochromocytoma - diagnosis and treatment
urinary markers are the most sensitive Treatment = Alpha channel blocker and then surgery
89
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
90
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.
91
Gordons syndrome
Causes Pseudohypoaldosteronism Hypertension, raised K+, acidotic Low renin, normal aldosterone Treatment = thiazide
92
Gene mutations in TBM and Alports
Thin basement membrane disease = COL4A3 and COL4A4 genes Alport's = COL4A5
93
Alternative name for MCD
Lipoid Nephrosis
94
Causes for Pseudohyperphosphatemia
Hyperglobulinemia secondary to Myeloma Hyperlipidemia, hyperbilirubinemia Contamination with heparin Antifungals
95
What blood test finding is associated with Post streptococcal GN?
Low CH 50 level
96
TB drugs and common effects
Rifampicin - CYP inducer, Red secretions Isoniazid - Neuritis, hepatitis Pyrazinamide - Causes Gout Ethambutol - Eye issues - optic neuritis
97
Urinary losses in nephrotic syndrome
Anti-thrombin III Thyroxine binding globulins = low thyroxine levels Immunoglobulins
98
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
99
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
100
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
101
What antihypertensive should be given if history of gout
Losartan
102
Tubulo-Interstial Nephritis and Uveitis syndrome
More common in young patients Raised ESR and deranged LFTs, low Hb Tx = Steroids
103
Features of AIN caused by checkpoint inhibitors
E.g Ipilimumab Hyponatremia, hypokalemia, hypophosphateaemia
104
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

Renal SCE (7 decks)

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