Renal Flashcards
What are the general features of a RTA
Why are they short
What is the general mainstay of treatment
Metabolic acidosis
Normal anion gap
High serum chloride
Boney phosphate is being used as a buffer
Replace bicarb
Furosemide for type 4
Where is the defect in a type 1 RTA
what will you see in the serum and urine
What is the classical age of presentation and how?
What can cause it
Distal convoluted tubule-impaired h+ secretion
Metabolic acidosis, urine is alkalotic, hypokalaemia, high urine calcium, high serum chloride
Stones, polyuria, rickets and FTT. 1st year of life
Medullary sponge kidney
Where is the defect in type 2 RTA?
What is seen in the serum and urine?
What is the syndrome associated called?
What are the congenital and acquired causes?
What is Lowe syndrome otherwise known as?
How does cystinosis present
Proximal convoluted tubule
Metabolic acidosis, high serum chloride, acidic urine, no stones, glucose, protein and amino acids in urine. Low serum K
Fanconi syndrome
Metabolic disease, cystinosis, heavy metal poisoning, chemo agents, gent, ranitidine, amyloid, myeloma
Occulocerebral renal syndrome
X linked recessive
Cataracts, behaviour change, growth failure
Photophobia and retinal detachment, hypothyroid, rickets
Where is the defect in type 4 RTA?
What is seen in blood and urine
Blocks aldosterone
Metabolic acidosis with acidosis urine hyperkalaemia, High urine sodium
What are general rules of thumb for transporter defects?
Metabolic alkalosis
Hypokalaemia
What diuretics do barters and gitlemans act like
What is another name for Lidle syndrome
Barters- like loop
Gitlemans- like thiazides
Liddle- pseudohyperaldosteronism
Where is the defect in barraters syndrome? What gets activated making things worse
When and how does it normally present?
What is seen in blood and urine
What might you see on renal biopsy?
Which subtype has deafness?
Which diuretic does it mimic? How do you know it’s not diuretic abuse?
How is it treated
Ascending Limb of henle Na-k-cl transporter RAAS
Birth- polyhydramnios, poor growth, polyuria and ? Stones
Infantile- polyuria,ftt
Metabolic alkalosis, high urine calcium, chloride, sodium and potassium
hypokalaemia and low serum chloride
Hyperplasia of the JGA
Type 4
Loop- BP normal
Replace electrolytes and give k sparing diuretics. NSAIDs to reduce prostaglandin
How is baratters syndrome treated (2 things)
Give sodium and potassium supps
Give NSAIDS- switches off the prostaglandin effect
Where is the defect in Gitleman syndrome?
What is seen in blood and urine
When does it present?
What diuretic does it mimic?
What might it overlap with?
DCT
Metabolic alkalosis, low serum magnesium, low urine ca
Adolescence
Thiazides
Type 3 barrters
Liddle syndrome
Where is the defect
What is seen on bloods
What measure is important in diagnosis
Collecting duct
Hypokalaemia, metabolic alkalosis and low RAAS
HYPERTENSION
What is the normal daily requirement for sodium
3mM/kg/day
How do you calculate osmolality
What is normal-
2xna + urea + glucose
280-320
How fast should hypernatraemia be corrected
No faster than 0.5mmol/ hour
How do you distinguish between renal and non renal causes of hypernatraemia
What is the calculation
What is normal
Urinary fractional excretion of sodium
High- renal issue- DI or HONK
Low not renal issue- diarrhoea or fever
Urine sodium x serum creat/ urine create serum Na
1%
What are the features of DI on blood and urine?
What causes DI
Hyperna , high serum osmolality, low urine osmolality
Not enough ADH- water can’t be reabsorbed
In DI what should be seen on a water deprivation test
When should the test be stopped?
Low urine osmolality (<700)
High serum osmolality (>300)
>3% weight loss
Too much weight loss
What is DDAVP?
What should it show?
Synthetic adh
No effect if nephrogenic
If central should increase urine osmolality by 50% or more
What are central causes of DI
Brain tumours or post pit dysfunction
Structural- SOD
What is the most likely genetic cause of nephrogenic DI
How is it treated
X linked- mutated adh receptor
Drink Lots of water, low sodium diet, diuretics
Why is desmopressin given in nocturnal enuresis
Brain hasn’t learned to turn on ADH overnight causing nocturnal enuresis
How do you calculate an anion gap?
What is normal?
What does a normal gap indicate?
What does a high gap indicate?
Na+K - Cl+HCO3
10-12
Loss of bicarb
Addition of h
How is DI treated
Low sodium diet, thiazides diuretics, NSAIDs
Desmopressin if central cause
What are causes of a high anion gap
Methanol Uraemia Dka Paraldehyde Isoniazid Lactic acidosis Ethylene glycol Salicytes
What are causes of a normal anion gap
What are the two most common causes
Addisons
Bicarb loss
Chloride Xs
Diuretics
GI losses or RTA
Excess of which 2 diuretics will cause a metabolic alkalosis
Which 2 will cause a metabolic acidosis
Furosemide and thiazides
Acetazolamide and k sparing
What do you see in the blood with laxative abuse.
How is this different from normal diarrhoea
Hypokalaemia and metabolic alkalosis
Usually also increased Mg
Normally diarrhoea causes a normal anion gap metabolic acidosis
Define elevated blood pressure
What is stage one
What is stage 2
Blood pressure greater than the 90th centile
>95th centile
>95th plus 12
What is the best pharmaceutical treatment for htn in children
What is the best treatment for hypertensive urgency. What classes of drugs are they
“Urgency
Ace inhibitor Oral isradipine (calcium channel blocker) Iv hydralazine (vasodilator) Iv labetalol (beta blocker) or sodium nitroprusside ( vasodilator)
What percentage of PUVs go on to ESRF
What percentage have VUR
How are they managed
30%
50%
Relief of obstruction and monitoring renal function. Laser ablation
Where does UPJ obstruction normally occur
What normally causes it
L sided
Intrinsic compression from an artery
Outline the RAAS system
What is the role of ANP
Renin secreted from JG cells when the macula densa at DCT senses low Na or the efferent arteriole senses low Bp
Stimulates AT1 release from the liver. ACE from lungs causes conversion to AT2- causes vasoconstriction
Stimulates- aldosterone- CD- increased Na in exchange for K.
ADH- increases aquoporins in CD to increase water reabsorb
ANP- released when fluid overloaded- reduces Na so less renin-more naturesis
Which syndrome is a horseshoe kidney associated with
What tumour is it associated with
Turners
Wilms-4x increased risk
AR cystic kidney disease How does it present What is seen on ultrasound What other organ is affected When does end stage renal disease usually start
Bilateral flank masses. Feeding difficulties or breathing issues if severe
Lack of corticomedullary differentiation and bulky kidneys
Liver- fibrosis
12 years
AD PKD
What other organ may have cysts
Brain- Berry aneurysms causing SAH
What is nephronoptosis
What is the usual phenotype
What happens the kidney over time
What is the gene associated
Medullary kidney cysts
Red hair,FTT and fanconi syndrome
Gets smaller causing CKD
NPHP1
What three kidney things are seen with TS
Simple cysts
Angiomyplipomas
Renal cell carcinoma
Outline the 3 stages of AKI
1- creat 2x baseline
2-2-3x baseline, u.o less than half ml/kg over 6-12h
3-3x plus with anuria
What are the features to look for in AIN
Sterile pyuria, global tubular dysfunction and uveitis
What fluid should be replaced in aki
How is insensible loss calculated
Insensible loses with urine output and all other loses
400ml/m2/day
What is the most likely bug in PD peritonitis
What is the first line abx regime
Strep pneumonia and E. coli
Cefazolin and ceftazidime
What are the 2 main side effects of cyclosporine
Hirsuitism
Gingival hypertrophy
What are the 2 main side effects of tacrolimus
Alopecia
Diabetes
Low Mg
What are three main side effects of MMF
Lukopenia, anaemia, GI side effects
What is characteristic about haematuria in alports disease
Genetically what occurs
What are other phenotypically features
Microscopic, becomes macroscopic with infections
X linked, type 4 collagen defect
SN hearing loss and anterior lenticonus
What happens in thin basement membrane disease
Will it progress to ESRF
Persistent microscopic haematuria
No!
What is the classical presentation of HSP
What is it
What is a sign of poor prognosis
How long should urine dip and BP be monitored for
What might be useful to treat it
Swelling and haematuria 1-3 weeks after URTI IgA mediated small vessel vasculitis Nephrotic range protein at diagnosis 12 months Steroids
When after a strep infection is PSGN usually seen
What happens complement levels
What would be seen on biopsy
10d after throat infection, 3 weeks after cellulitis
Low
Neutrophil infiltration
How is igA nephropathy differentiated from post infectious (2 ways)
Haematuria at the time of the infection
Normal complement levels
What are three causes of low complement in GN
PSGN
Mesangiocapillary
SLE
What is cresentic GN also known as
Rapidly progressive
In good pastures what are antibodies formed against
Type 4 collagen in basement membrane
What is the triad of HUS
What happens to haptoglobins
AKI low platelets and microangiopathic haemolytic anaemia
Low!
What infection can cause atypical HUS
What neutrophil count shows poor prognosis
Pneumococcus
Neuts >20
What complement pathway is involved in complement mediated HUS
WHich MAB is useful
Alternative pathway
Ecilizumab
What is AIN
what are the characteristic features
What is TINU
What drugs can cause it
Immune mediated kidney infiltration with inflammatory cells. Causes tubular damage
General tubular dysfunction, sterile pyuria, tired with polyuria
With uveitis
NSAIDs, PPIs, antibiotics and diuretics
Loop diuretics
Give an example
Where do they work
How do they work
What is seen in the blood with excess
What diuretic works in a similar way with similar side effects
Where do they work
Loop of henle and DCT
Block nakcl transporter- keep na and k in the lumen and water follows
Hypo k and na
Metabolic alkalosis
High urine mg and ca
Thiazides-DCT
K sparing diuretics
How do they work
What 2 main things are seen in blood
Block aldosterone
Hyper jalapeños and metabolic acidosis
Nephrotic syndrome
Why is there an associated immunodeficiency?
What infections are most common
Reduced production of immunoglobulins
Encapsulated
Nephrotic syndrome
Why is there an associated hupercoagulability and high cholesterol
Reduced oncotic pressure therefore reduced liver perfusion
Raised VLDL
raised clotting factor production
Nephrotic syndrome
What dose of steroids is given and for how long
What defines a relapse
What defines remission
What defines steroid dependence
What defines steroid resistance- what is the risk to developing CKD
60mg/m2 for 6 weeks
>3+ protein over 3 consecutive days
Zero or trace protein “
Relapse on steroids or less than 2 weeks off
No improvement after 4 weeks- around 40 %
What stain is likely to pick up FSGS
Trichrome staining
Which three renal diseases can recurr in transplant
Which can change to another
FSGS, membranoproliferative, and 1/3 of igA
Alports- becomes anti GBM disease
Congenital nephrotic syndrome
What is the most common genetic cause
What is another cause
NPHS1
WT1
Which type of stones are most common
What happens citrate and Oxalate in the urine
What can cause them- 4 things and 2 meds
Calcium stones- oxalate and phosphate
High oxalate, low citrate
Malabsorption and short gut, RTA, hyper PTH
TOPIRAMATE AND ACETAZOLAMIDE
What causes cysteine stones
Which 4 amino acids leak Into the urine
What causes Uric acid stones
What causes struvite stones
What type of stone is likely to cause a staghorn calculus
Cysteinuria- cystine, ornithine, arginine and lysine
Lesch nyhan syndrome
UTIs
Struvite stones
What is dent disease
What are three main features
Which 2 genes are affected
X linked form of fanconi syndrome
Haematuria, polyuria and stones
OCRL1 CLCN5
What medication is useful for giggle incontinence
Oxybutinin
What does the mesonephros form
“ metanephros
Ureteric bud (pelvis and collecting ducts) Everything else
What does DMSA show
What do mag 3 and DTPA show
Structure and scarring
Function (?obstruction)
What type of drugs are cyclosporine and tacrolimus
Calcineurin inhibitors
