Nephrology Flashcards
DIDMOAD for Wolfram Syndrome
Causes of Central and Nephrogenic DI
DI
Diabetes Mellitus
Optical Atrophy
Deafness
How to conduct a Fluid Deprivation Test and Interpret it
Whats the treatment for Central and Nephrogenic DI and why
Investigations and Management
Central
Infection (Meningitis)
Infiltrative (Sarcoidosis)
Trauma
Wolfram Syndrome (DIDOAD)
Histiocytosis X and Sarcoidosis
Vascular (Sickle Cell Crisis)
BINGE DRINKING (inhibit Posterior Pituitary)
Congenital nephrogenic DI (AVPR2 gene) - Mutation in Aquaporin Channel
Nephrogenic
CKD
Post Obstructive Uropathy
Intrinsic Kidney Disease - (Casts . Hematuria)
HyperCal , HypoK
DM
Fluid Depriviation Test (2 micorgram IM)
MRI Head + Posterior Pituitary Function
Serum ACE
bHCG (Pineal Germinomas
Stop Offending Drugs
Desmopressin (Central) and Thiazide / NSAIDs (Nephrogenic)
Thiazide Diuretics –> inhibit Prostaglandin Synthesis which cause Volume Contraction and reduce EGFR so less urine goes to Distal Tubule and more solutes are absorbed in the Proximal hence concentrating Urine
Which Diuretic Acts where ?
Acetazolamide - Proximal - Carbonic Anhydrase Inhibitor
Loop Diuretic - Ascending Loop of Henle by Na/K/2Cl symporter
Thiazide - Distal Na/Cl Symporter
Spironolactone - Cortical Cells in Collecting Duct Na/K ATPase
Amiloride - blocks the epithelial sodium channel in the distal convoluted tubule
Explain Plasma Aldosterone / Renin Ratio combinations
Liddle / Bartter (Asociation ?) / RAS / Primary Hyperaldosteronism / Gitelman (association?)
HYPERtension with Hypokalemia differentials
NORMOtension with Hypokalemia differentials
-BOTH high ? > renal artery stenosis with HYPERTENSION but if NORMOTENSIVE then it Bartter Syndrome
-both low ? > liddle’s syndrome (Liddle’s syndrome = L = LOW for both )
-high aldosterone and renin is low : primary hyperaldosteronism
Primary Hyperaldosteronism > overproduction of aldosterone > excessive sodium and water reabsorption in the kidneys > negative feedback onto renin > reduced Angiotensin I and II > attempts to suppress aldosterone but due to primary hyperaldosteroninsm, keeps secreting inappropriate amounts of aldosterone
Liddle - Gain of function of sodium channel in the DCT (Gitleman is Loss of Function here —> Hypomagnesemia) > excessive sodium reabsorbed, regardless of renin/aldosterone > causes negative feedback suppression of renin and aldosterone > low for both.
Bratters > impaired Na/K/2Cl in thick ascending loop > low sodium low water low bp > stimulates renin > stimulates aldosterone > high renin high aldosterone
Bartter – > Hypercalciuria (Due to Paracellular Transport of Mg and Ca blocked due to a lack of positive charge from K+ being kicked out)–> Nephrolithiasis
Hypertension with hypoKALEMIA :
Better spot a cushy little con
Better = 11 beta hydroxysteroid dehydrogenase deficiency
Cushy= cushing syndrome
Little = Liddle’s
Con = Conn’s
——————————-
Normotension and hypokalaemia:
Bartering gits can die vomiting
Bartering= Bartter’s
Gits= Gittleman’s
Die= Diuretics
Vomiting= Vomiting
Kidney Stone Management
Renal stones
watchful waiting if < 5mm and asymptomatic
5-10mm shockwave lithotripsy
10-20 mm shockwave lithotripsy OR ureteroscopy
> 20 mm percutaneous nephrolithotomy
Uretic stones
< 10mm shockwave lithotripsy +/- alpha blockers
10-20 mm ureteroscopy
Stone Prevention for Calcium Oxalate Stones and Uric Acid Stones
Oxalate stones
cholestyramine
pyridoxine
Uric acid stones
allopurinol
urinary alkalinization e.g. oral bicarbonate
ADPKD management
ACEi / ARB for HTN
Cyst Hemorrage - Nephrectomy
Cyst Infection - Ciprofloaxacin
Symptomatic or >10mm anuerysm - Surgical Intervntion
Tolvaptan - CKD 2/3 & rapidly growing
Which HLA shows high risk of rejection of transplant
HLA DR > B > A
Risk Factors for secondary FSGS
and
Light Microscopy and Electron Microscopy findings
HIV
PVB19 via Hemolytic Anemia’s - Sickle Cell or B Thalassemia
Heroin (THE ADDICT)
SLE
Charcot Marie Tooth Disease - Congential
Focal Segmental Sclerosis + Hyalinosis in Light
Podocyte Effacement in Electron
Risk Factors for Secondary Minimal Change Disease
Recent Infection / Vaccination
Hodgkin Lymphoma
NSAIDs / Rifampicin
Infectious Mononucleosis
Risk Factors for Membranous Nephropathy
Light and Electron Microscopy Findings
Syphillis / SLE (Type 5)
HBV / HCV
Malaria
Schistosomiasis
NON Hodgkin
Cancer
Gold
Light - Thick GBM
Electron - SubEPITHELIUM Spike and Dome pattern due to immune complex deposition
FSGS Selective or Non selective proteinuria ?
Non Selective
Only MCD is SELECTIVE
What antibody is associated with Membranous Nephropathy ?
Anti PLAR2 (Spike and Dome Subepithelial on Electron)
Prognostic Factors for MGN ?
Female Good for MGN
Male Poor for IgA
ADPKD Type 1 and Type 2 Chromosome and Genes ?
ADPKD T1 (Chromosome 16) PKD1 Gene - Severe but 85% common
ADPKD T2 (Chromosome 4)
PKD 2 Gene - Less Severe but 15%
Prognostic Markers for IgA
markers of good prognosis: frank haematuria
markers of poor prognosis: male gender,
proteinuria (especially > 2 g/day),
hypertension,
smoking,
hyperlipidaemia,
ACE genotype DD
Non Seminoma Types
non seminomas (Peak 25)
Cu—choriocarcinomas
Y yolk sac
T teratomassS
E embryonal
Factors that increase risk of Pulmonary Hemorrhage ?
smoking
lower respiratory tract infection
pulmonary oedema
inhalation of hydrocarbons
young males
Nephrogenic DI causing Drugs
DR. FLAG
D - Demeclocycline
R - Rifampicin
F - Foscarnet
L - Lithium
A - Amphotericin B
G - Glibenclamide
Side effects of Erythropoietin
Side effects of Erythropoietin
A- accelerated HTN
B- Bone aches
C-Cellular Aplasia of RBCs
D- Deficiency of Iron
E- Exaggerated Skin symptoms ( Rashes , Urticaria)
F- Fistula Thrombosis due to Raised PCV, Flu like symptoms
Which Kidney Stones are Radio-opaque and which are radio-lucent
all the ones that are OOOOOpaque contain an o (phosphate (incl stag horn), oxalate)
all the ones that are radiolucent don’t (urate and xanthine) - just have to remember that cystine are semi-opaque (the c looks like half an o)
High Anion and Normal Anion Gap Causes ?
Methanol
Uremia
DKA
Paraldehyde
Iron, Isoniazid
Lactic Acidosis
Ethyl Glycol
Salicylate
Carbon Monoxide
Aminoglycosides
Theophylline
Normal Anion Gap MA
FUSEDCARS
Fistula (biliary, pancreatic), Ureterogastric conduit, Saline administration, Endocrine (Addison disease, hyper-PTH), Diarrhea,
Carbonic anhydrase inhibitor,
Ammonium chloride,
Renal tubular acidosis, Spironolactone
What is the diagnostic investigation for Recurrent UTI in Peads ?
MCUG for Reflux Uropathy and DMSA later for Renal Scarring
Fibromuscular Dysplasia
Female
Flash Pulmonary Edema
AKI after ACE
Uncontrollable HTN
ACEI in CKD - a decrease in eGFR of up to 25% or a rise in creatinine of up to 30% is acceptable
ACEI for HTN - a decrease in eGFR of up to 15% or a rise in creatinine of up to 20% is acceptable - if more than that - HOLD drug and suspect Renovascular dx
Glomerulonephritis with complement Levels
PIMpS
Post Streptococcal GN
Infective endocarditis (Subacute)
Mp – Membranoproliferative GN
S – SLE Renal
Electrolyte Abnormality in Plasma exchange
Hypocalcemia
Voiding Symptoms / Overactive Bladder / Nocturia management in Men >50
Pelvic Floor exercises
If moderate or severe risk then Alpha blocker
If Prostomegaly + High Risk of Progression then 5-alpha reductase inhibitor
If Prostomegaly + Moderate to High voiding symptoms then 5-alpha reductase inhibitor + Alpha Blocker
Mixed Voiding and Storage Symptoms not responding to Alpha Blocker then add
Antimuscarinic
Overactive Bladder
Bladder Training
Antimuscarinic
How long does Finasteride take to be effective for BPH
Upto 6 months
BPH treatment ?
Watchful Waiting
IPSS >/= 8 (Mild to Moderate) start Alpha blockers
If Prostomegaly + High Risk of Progression then 5-alpha reductase inhibitor
(might reduce PSA level by 50%)
If Prostomegaly + Moderate to High voiding symptoms then 5-alpha reductase inhibitor + Alpha Blocker
Mixed Voiding and Storage Symptoms not responding to Alpha Blocker then
Antimuscarinic
Surgery (TURP)
BPH investigations
urinary frequency-volume chart (3 days at least)
International Prostate Symptom Score (IPSS)
Score 20-35: severely symptomatic
Score 8-19: moderately symptomatic
Score 0-7: mildly symptomatic
Equations for eGFR estimates
**CrCL Cockroft used :
1- Gender
2- Age
3- Weight
4- Serum Creatinine
** MDRD + CKD-EPI used:
1- Gender
2- Age
3- Race
4- Serum Creatinine
**MDRD with 6 variable :
1- Gender
2- Age
3- Race
4- Serum Creatinine
5- BUN
6- Albumin
Renal Artery Stenosis Gold Standard Investigation
MR Angiography
but sequence will be
Renal doppler ultrasound
CT angiograph of renal arteries
MR angiography - non-invasive gold standard
Invasive renal angiography - invasive gold standard
Urgent Dialysis Indication
Indications for dialysis: AEIOU
A – Acidosis – metabolic acidosis
E – Electrolytes – refractory hyperkalemia or rapidly rising potassium levels
I – Ingested substances *
O – Overload – volume overload refractory to diuresis
U – Uremia – elevated urea with signs or symptoms of uremia (pericarditis, neuropathy, or uremic encephalopathy)
*Use Mnemonic SLIME salicylates
lithium
isopropanol
methanol
ethylene glyco
Factors associated with Retroperitoneal Fibrosis
Riedel’s thyroiditis
previous radiotherapy
sarcoidosis
inflammatory abdominal aortic aneurysm
drugs: methysergide
Normal anion Gap ?
8-14
Urea to Creatnine Ratio elevated in Pre-Renal but Normal in ATN why ?
Given that urea absorption is largely modulated in the proximal tubules, it is not affected by diuretics acting more distally.
DPGN associated with Lupus AND ????
PSGN
Calciphylaxis
Calciphylaxis lesion are intensely painful, purpuric patches with an area of black necrotic tissue that may form bullae, ulcerate, and leave a hard, firm eschar
214 emily
Minimal Change Disease not responding to Prednisolone then ?
Cyclophosphamide
Nephrogenic DI mutations less and most common cause ?
the more common –> vasopression (ADH) receptor
the less common form –> mutation in the gene that encodes the aquaporin 2 channel
Tolvaptan antagonizes V1 V2 or V3 ?
Tolvaptan is a vasopressin receptor 2 antagonist
What cancer is associated with Post Organ Transplant ?
Squamous Cell Carcinoma
Hyperacute rejection (minutes to hours)
due to pre-existing antibodies against ABO or HLA antigens
an. example of a type II hypersensitivity reaction
leads to widespread thrombosis of graft vessels → ischaemia and necrosis of the transplanted organ
no treatment is possible and the graft must be removed
Acute graft failure (< 6 months)
usually due to mismatched HLA. Cell-mediated (cytotoxic T cells)
usually asymptomatic and is picked up by a rising creatinine, pyuria and proteinuria
other causes include cytomegalovirus infection
may be reversible with steroids and immunosuppressants
Causes of chronic graft failure (> 6 months)
both antibody and cell-mediated mechanisms cause fibrosis to the transplanted kidney (chronic allograft nephropathy)
recurrence of original renal disease (MCGN > IgA > FSGS)
You review a 42-year-old woman six weeks following a renal transplant for focal segmental glomerulosclerosis. Following the procedure she was discharged on a combination of tacrolimus, mycophenolate, and prednisolone. She has now presented with a five day history of feeling generally unwell with anorexia, fatigue and arthralgia. On examination her sclera are jaundiced and she has widespread lymphadenopathy with hepatomegaly. What is the most likely diagnosis?
HIV/CMV/HCV/HBV/EBV
CMV is the most
Glomerulonephritis that is most characteristically associated with partial lipodystrophy?
Membranoproliferative glomerulonephritis (mesangiocapillary)
type 1 (Classical Pathway activated) : cryoglobulinaemia, hepatitis C, CLL, Lymphoma
type 2 (Alternative Pathway Activated) : partial lipodystrophy, Factor H Deficiency
C4 Low in type I, normal in type 2
C3 Low in Type 2
Type 1 Membranoproliferative GN vs Type 2 Membranoproliferative GN on Microscopy
Type 1 and 2 on Light Microscopy (PAS Stain) -
- Thickened GBM
- Mesangial proliferation & interposition
- Mesangial hypercellularity
Type 1 MPGN Electron Microscopy -
- Immune complex deposits in subendothelial and mesangium
- Tram Track Appearance
Type 2 MPGN Electron Microscopy -
- Complement deposits in GBM
- Tram Track Appearance
Type 1 MPGN vs Type 2 MPGN treatments
Primary (idiopathic):
- Oral cyclophosphamide
- Oral mycophenolate mofetil (MMF) and/or Pred add on
Secondary MPGN:
- Treat the cause
ACEi/ARB
Statins
Wafarin
Target for Anti GBM in Goodpasture’s Disease
NC1 domain of alpha 3 chain of Type IV collagen
Excess NaCl infusion causes what metabolic picture ?
Hyperchloremic metabolic acidosis
- Excess Na+ and Cl-
- If ongoing Diahorrea - Loss of HCO3-
- Excess Cl- force HCO3- into cells and limit HCO3- available for pH buffer
Iatrogenic Causes of Renal/Uretic Stones
- Calcium Stones = SALAD CT
S - Steroids
A - Antacids
L - Loop diuretics
A - Acetozolamide
D - Vit D
C - Vit C
T - Theophylline/ Aminophylline
- Uric acid stones
Salicylate
Thiazides
- Precipitate into stones: SIT
S - Sulfadiazine
I - Indinavir
T - Triamterene
What type of Catheter to use when there is an URGENT need to Dialyse and no time for an AV fistula to mature ?
Tunneled or Non Tunneled Venous Catheters
Pathologies associated with IgA Nephropathy
HSP (IgA vasculitis)
SLE
Coeliac disease
alcoholic liver disease
Prostate Cancer Management
Localized (T1/T2) -
1. Watchful Waiting
2. radical prostatectomy - Erectile Dysfunction
3. radiotherapy: external beam and brachytherapy - Proctitis & Malignancy
Localised Advanced (T3/T4)
1. Hormonal
2. Same as above
3. Same as above
Hormonal
- GnRH agonist - Goreselin - paradoxical overstimulation but often
cyproterone acetate 3 days before till 3 weeks after to avoid tumour flare
GnRH antagonist
- bicalutamide - Non-steroidal anti-androgen
- cyproterone acetate- Steroidal Anti androgen
- abiraterone - Androgen Synthesis inhibitor - when androgen deprivation therapy has failed and before Chemotherapy with docetaxel
- Bilateral Orchidectomy
Tumour Markers for Seminomas and Non Seminomas
Seminoma –> bHCG
Non Seminoma (embryonal, yolk sac, teratoma and choriocarcinoma) —> AFP and/or bHCG
Teratomas in Twenties and Seminomas in Serties
Renal Papillary Necrosis Associations
chronic analgesia use
sickle cell disease
TB
acute pyelonephritis
diabetes mellitus
IVU - Papillary Necrosis and ‘Cup and Spill’
Casts and Cause of AKI
Hyaline Cast = Pre-renal
Granular = ATN
Muddy Brown
Renal Tubular Cells
Sterile Pyuria = AIN
White Cell Casts
Eosinophilia
Dysmorphic Cells = Glomerulonephritis
RBC Casts
Causes of Intra Renal AKI
- Vascular
Infarct
APLS
Scleroderma Crisis
Dissection
Vasculitis (PAN, Takayasu)
- Interstitial
AIN - NSAIDs, Sulpha Drugs, PPI, Check Point Inhibitors, Abx, Recurrent Pyelonephritis
- Tubular
ATN - Ischemia, Aminoglycosides, Crystalluria (Acyclovir, Cisplatin,MTX, Ciprofloxacin)
MM, TLS, Rhabdomyolysis
- Glomerulus
Urology Urgent and Non Urgent Referral Criteria for Hematuria
URGENT
Aged >= 45 years AND:
unexplained visible haematuria without urinary tract infection,
OR
visible haematuria that persists or recurs after successful treatment of urinary tract infection
Aged >= 60 years
AND
unexplained nonvisible haematuria and either dysuria or a raised white cell count on a blood test
NON URGENT
Aged >/=60 years + recurrent or persistent unexplained urinary tract infection
How to prevent Contrast Induced Nephropathy ?
1m/kg/hour for 12 hours pre and post contrast
Withhold Metformin for 48 hours post procedure
Asymptomatic Microscopic Hematuria + Family H/O = ?
Thin Basement Membrane Disease or Benign Familial Hematuria
Autosomal Dominant
Indications for Plasma Exchange
Guillian’s Good MATCH
Guillain-Barre syndrome
Goodpasture’s syndrome
Myasthenia gravisĺ⁹lìĺ⁸
ANCA positive vasculitis if rapidly progressive renal failure or pulmonary hemorrhage
TTP
Cryoglobulinemia
Hyperviscosity syndrome e.g. secondary to myeloma/ HUS
How does Plasma exchange cause Metabolic Alkalosis ?
Plasma exchange has Citrate
Citrate metabolism releases HCO3-
Renal Dysfunction and so HCO3- elevated
Metabolic Alkalosis
Left sided varicocele in malignancy questions
What Cancer should come to mind ?
Renal
Because left testicular vein drains into the renal vein, whereas the right drains into IVC directly. Any obstuction/mass pressure on left side as in Renal mass can lead to backpressure.
Membranous Glomerulonephritis Treament
ACEi
Secondary Typical HUS Causes
HIV
Pneumococcal Vaccination
Treatment of HUS
Atypical HUS give Plasma Exchange
Eclizumab (C5 Monoclonal Inhibitor) > Plasma Exhange in ADULTS ATYPICAL HUS
Wilms Tumor Associations
Beckwith-Wiedemann syndrome (Macroglossia, Umbilical Hernia. Facial Plexus Nevus and Lateralized Growth)
as part of WAGR syndrome with Aniridia, Genitourinary malformations, mental Retardation
Hemihypertrophy
Loss of Function Mutation in WT1 gene on Chromosome 11
Wilms Tumor Mets Commonly where ?
Lung
Cystinuria
1) How it causes recurrent renal stones
2) Chromosome and Gene associations
3) Diagnostic Test
4) Treatment
1) Defect in Membrane Transport for Cystine, Ornithine, Lysine and Arginine (COLA)
2) Chromosome 2: SLC3A1 gene, chromosome 19: SLC7A9
3) Cyanide Nitroprusside Test
4)
Hydration
D-Penicillamine
Urine Alkalinization
Whats Stauffer Syndrome
Cholestatic Picture in RCC patients
Cause of False Negative PSA
Finasteride
Type 1 and Type 2 MPGN Electron Microscopy findings ?
Type 1 - subendothelial and mesangium immune deposits of electron-dense material resulting in a ‘tram-track’ appearance
Type 2 - intramembranous immune complex deposits with ‘dense deposits’
Autosomal Recessive Kidney Disease Mutation ?
Chromosome 6
Fibrocystin Gene
Found via Prenatal USS scan
Mesangial deposition
- IgA nephropathy
- Class II lupus nephritis
- Diabetic nephropathy
- MPGN
Subendothelial space
- RPGN
- Class IV lupus nephritis
Subepithelial Space
- MPGN
- Class V lupus nephritis
- PSGN
Glomerular hypercellularity
- PSGN
- Lupus nephritis
- MPGN
Cystinosis
1. Inheritance
2. Pathophysiology
Homocystinuria
1. Inheritance
2. Pathophysiology
3. Features
4. Management
Cystinosis - autosomal recessive, mutation on chromosome 17.
Accumulation of AA cystine in lysosome -> leads to Francois syndrome
Homocystinuria - Autosomal recessive, deficiency of cysta-thionine beta-synthase.
Features = tall, long arms, DOWNWARD lens dislocation, learning difficulties, DVT
Management = pyridoxine supplements (vitamin B6)
Francois Syndrome Features
Bird Like Face
Abnormal Teeth
Hypotrichosis
Congenital Cataract
Medications that make Urate Stones ?
Loop Diuretic
Steroid
Theophylline
Acetazolamide
