Renal Flashcards
Proximal tubule..
Main site for reabsorbtion
Aldosterone acts on..
Na channel in DCT to reabsorb water and sodium (secrete k and H)
Renal angiomyolipomas in what syndrome
TS
Peritonitis organisms in dialysis
SA 20% CONS 40% strep 20% pseudomonas 5%
Fanconi syndrome renal problem location
Proximal tubule dysfunction (RTA2), not reabsorbing all the good stuff so lose sodiuma nd don’t grow well and have tubular dysfunction so drink and wee lots
Causes: cystinosis and Lowes syndrome
In phaeo with HTN don’t use..
Bets blocker but use a blocker (phentolamine IV or oral phenoxybenzamine)
Persistently low C3 in…
Membranoprolif
RPGN presentation
Nephrotic or nephritic
Crescents on biopsy
Tx steroid
Presents with family history and haematuria
Alports genetic test and biopsy result?
Hereditary nephritis mainly XL (dominant)
Mutation in COL4 gene encoding type IV collagen which affects BM
Macroscopic haematuria
Biopsy variable thickness and splitting of BM - basket weave appearance
SNHL ears
Eyes cataracts, anterior lenticonus, macular abnormalities
ESRD 50% by 25
IgA nephropathy
Mostly boys Recurrent (at time of infection) Macroscopic haematuria Most are ok by 10% to EsRf Biopsy IgA deposits Tx steroids If bad
Three causes of nephrotic syndrome
Minimal change (steroids)
FSGN
Memb snouts nephropathy
Renal stone increased by high ? Diet
Na (decreases calcium absorption)
Present with FTT, recurrent episodes dehydration, hypercalciuria, 20% low serum mg, renal stones
Hypokalaemic metabolic acidosis
Bartter syndrome:
AR dysmorphic
Nephrocalcinosis
Like frusemide abuse
Present with hypokalaemic metabolic alkalosis, some mild growth failure, pr sent later, hypomagnesemia, hypocalciuria
Gitelman syndrome
AR defect na/cl CT
Like thiazide abuse
Prune belly =
Abdo wall defect, undescended testes, UT abnormalities
Granular casts in..
ATN
GFR gold standard
Inulin (gold standard but difficult to measure)
DTPA vs DMSA
Parenchyma defects
DMSA for structure/morphology - scarring, static scan, size and % function
DTPA for transit over time and looks for obstruction and drainage, how whole system is working, dynamic
Haematuria excretion rate increases with
Age and exercise
Proteinuria with fever, exercise, heart failure
Risk of nephritis in ahSP after 2m
2%
6 forms of steroid resistant nephrotic syndrome in childhood
Minimal change Membranoproliferative glomerulosis Membranous nephropathy Secondary (IgA, HSP, Lupus) FSGS Congenital
Syndrome causing childhood HTN
Liddle due to acting like there is high aldosterone (but it’s normal)
Best drug for reduction BP, proteinuria, preservation of renal function
ACEi
MCDK
1/4000, non heritable, unilateral nonfunctional cystic mass
Bilateral fatal often other kidney can make up for it but may have VUR - monitoring important
Campomelic dysplasia
SOX9, dysplasia, hydronephrosis
Agenesis kidney in what syndromes
Fraser
Kallman
Pallister-hall
Rubinstein-taybi
ADPKD
Mutation PKD
1/1000
Wide phenotype
Usually asymptomatic in childhood
ARPKD
Bilateral large echogenic kidneys
Mutation PKHD1
1/20000
With congenital hepatic fibrosis and hepatomegaly with portal HTN
Most frequent genetic cause CKD
Neohropnopthisis (tubuloingerstitial sclerosis - concentrating defect)
Causes of CKD
Hypoplasia/dysplasia
Nephronopthisis (normal kidney size)
Obstructive uropathy
Cystic
FSGN Chronic GN Alpert HUS Cortical necrosis
In chronic tubular defect - concentrating defect causes
Polyuria and polydipsia
Enuresis/nocturia
Electrolyte loss in urine
Causes of AKI
Sepsis HUS GN ATN Drugs Dehydration Interstitial nephritis Rhabdo Oncology Cardiac surgery Liver transplant
Tubular damage from what drugs
Aciclovir
Aminoglycosides
ATN urine results
Casts
Low osmolality
<40 urea/cr ratio
High Na
Two other causes HUS
Atypical in 10%
I.e.
Strep pneumoniae
Low C3
Anti GBM
May have pulmonary haemorrhage
Linear staining
Imaging small vs large echogenic kidney
Small structural
Large acute disease (ARF or GN)
Proteinuria
Sign of CKD and will progress - needs f/u
Orthostatic proteinuria
Normal in morning
Teens
Normalises
Nephrocalcinosis
Generalised echogenic appearance due to calcium deposits
- cortical nephrocalcinosis is often due to cortical necrosis – check renal function, BP etc
- Medullary and cortical – hypercalcaemia ( seen with excess Vit D) oxalosis (esp if calculi)
- Medullary alone – very long list of causes
Plasma osmolality
(2xplasma Na) + glu + urea
Chloride in urine
High in diuretic abuse, Bartters, Gitelmans
Low with vomiting and laxative abuse
Pneumococcal HUS pathophysiology
Neuraminidase uncovere T-cryptantigen which binds T IgM
Rituximab
Monoclonal antibody against CD20 causing rapid depletion of B cell populations
Mycophenolate mofetil mechanism of action and side effects
Inhibits T and B cell proliferation
GI, leukopenia, anaemia (not nephrotoxic)
Six causes of allograft dysfuncTION
DehydraTION MedicaTION InfecTION ObstrucTION RejecTION PrefusION problem
High tacrilomus level causes electrolyte low
MG
Tremour, alopecia
Cyclosporine SE
Hirsuitism and gingival hyperplasia
Post transplant lymohoproliferative disorder
Mononucleosis like lesions
Post transplant infection prophylaxis
CMV 3m valganciclovir
Cotrimox
Candida 1m nilstat (and with antibiotics)
Normal anion gap metabolic acidosis (not due to diarrhoea) causes
RTA
Type 1: unable to excrete H+ (urine mor alkaloid making a higher urine pH) low hCO3 level
Type 2: reduced bicarb reabsorption (assoc w Fanconi syndrome), acidic urine (no bicarb buffer)
Type 4: hyperkalaemia major feature, lose sodium in urine and keep potassium from mineralocorticoid def/insensitivity (hx obstructive uropathy or due to adrenal disease)
Type 3 RTA
Rare
Children have mental retardation, cerebral calcification and osteoporosis (carbonic anhydride deficiency)
Drug contraindicated in renal artery stenosis
ACEi
Renal artery stenosis assoc with what…
FiBromuscular dysplasia
Takayasu arteritis
NF1
Williams
Micrhaematuria resolves after how long with PSGN
2 years
Follow up renal after HSP for
3 months (risk 2% at 2m)
Minimal change presents with nephrotic syndrome and what feat on electromicroscopy
The three hallmarks of minimal change disease: diffuse loss of podocyte foot processes, vacuolation, and the appearance of microvilli.
Treating HTN with Ca Channel Blockers Avoid in…
polycystic kidney disease
Older child polyuria and polydipsia
Ultrasound: bilateral small corticomedullary cysts, tubulointersitial fibrosis. Small or normal sized kidneys.
disruption of TBM, tubulo‐interstitial fibrosis, cyst formation.
Presents with polydipsia and polyuria with failure and small echogenic kidneys and retinitis pigmentosa
AR
Nephropnopthisis
Fanconi syndrome
Growth issues/rickets and concentrating defect
Due to proximal tubule disorder (proximal tubule usually reabsorbs glucose and a.a and bicarb/phosphate causing…
Hyperaminoaciduria
Glycosuria
Hypophosphatemia
Hypokalemia
Metabolic acidosis hyperchloremia
Nephrogenic DI
90% are mutations in the ADH receptor (AVPR2)
gene; X‐linked.
• Over 180 mutations so variable affects.
• Remaining 10% are mutations in the aquaporin 2
(AQP2) channel; autosomal recessive/dominant.
Normal bladder volume
(AGE x 30) X 30 = volume (mls)
330ml in 10 year old
Pale kids with high creatinine and poor growth with polyuria& polydipsia enuresis / nocturia Always thirsty Loss of other electrolytes in the urine Little haematuria or proteinuria Has a...
Concentrating defect from tubular +- interstitial cause (non-glomerular)
Also polyuria and hypovolaemia in acute tubular necrosis or interstial insult
HUS triad
Thrombocytopenia, AKI, haemolytic anaemia
Typical from diarrhoea illness
Atypical - pneumococcal and complement def
Most common cause of fanconi syndrome and has crystal deposits on slit lamp eye
Cystinosis
Presents with obesity, diabetes, retinitis pigmentosa, polydactyly, infertility and renal failure which syndrome
Ciliopathic
Bardet–Biedl syndrome
Which syndrome?
Presents with renal cystic dysplasia, central nervous system malformations (occipital encephalocele/NTD), polydactyly (post axial), hepatic fibrosis
Meckle
Treat peritonitis with…
After sample
IP vanc/cefazolin and ceftazadime (IV if septic)
Antifungsl prophylaxis
Post transplant graft dysfunction and haematuria ?organism
BK
Flank mass, haematuria and decreased platelet in newborn suggests
Renal vein thrombus
High what in urine predisposes to kidney stones
Calcium
IgA deposited where in HSP
Mesangium
Treating enuresis with drugs (first and second line)
First is desmopressin and second oxybutynin
Is serum IgA helpful in IgA nephropathy
No (only up in 15% paed pt)
Mechanism injury in renal HUS e.coli
ENDOtoxin
Hypercalciuria diet advice
Low sodium
Type 2 RTA causes
Cystinosis, Lowe syndrome, galactosemia, Wilson disease, tyrosinaemia, interstitial nephritis, medication (gent, isofosamide, cyclosporine)
Present with nephrotic or nephrotic or mixed
Histology showing deposits (C3) and thick capillary walls (tram tracks present)
Membranoproliferative GN (type 1 and 2 - dense deposits)
Renal biopsy deposit C3 and IgG in?
Deposit all immunoglobulins and C3 in?
psGN
SLE
Large placenta
Genetic defect in nephrin or podocin cause
Congenital nephrotic syndrome
What reduces total body K
Resonium
Main site of K+ reabsorption
Proximal convoluted tubule
Carrier freq PKHD1 gene (fibrocystin) involved with ARPKD
1:70
Anion gap formula
Anion Gap = Na+ – (Cl- + HCO3-)
Urinary = UCl-(UNa+UK)
Cr clearance not accurate when renal disease worsens because
approximately 15% of excreted urine creatinine is derived from proximal tubular secretion. Because of the tubular secretion of creatinine, the creatinine clearance typically overestimates the true GFR by 10% to 15%.
MCU should be considered for
infants under 3 months with US abnormalities after their first febrile UTI and /or atypical UTI
for children under 6 months with recurrent febrile UTI and / or abnormal US
for male infants where there is concern regarding obstructive uropathy on US
Needs three days treatment dose antibiotics starting day before
Thiazides mech of action
inhibit reabsorption of sodium (Na+) and chloride (Cl−) ions from the distal convoluted tubules in the kidneys by blocking the thiazide-sensitive Na+-Cl− symporter.
Fractional excretion sodium =
Fractional excretion sodium = 100 x (sodium urine x Cr blood / sodium blood x Cr urine)
reabsorption of most bicarbonate (HCO3) from the glomerular ultrafiltrate is by: A. diffusion of carbon dioxide (CO2). B. intercellular transport. C. pinocytosis. D. primary active transport. E. transcellular aquapores.
A
Aldosterone and ANP role
ANP stimulates sodium loss
Aldosterone stimulates sodium/H20 retention by increased aquaporins luminal surface DCT and CD (loss of K+ and H+ in exchange)
Multicystic dysplastic kidney risks
Bunch of grapes on USS
Not likely cancer or HTN
Some involute
Other kidney having Reflux (PUJ obstruction)
Salt wasting syndromes/hypokalaemic metabolic alkalosis
- Salt (Na and Cl and K) wasting nephropathy more severe (thick ascending loop) like thiazides diuretic (high calcium and chloride in urine)
- Hypokalaemic, hypomagnesiaemia, hypocalciuria (distal tubule) less severe
Bartter syndrome
Gitelman
NEphronophthisis with coloboma/ cerebella =
Jouberts syndrome
vasodilator in renal afforestation arterioles
Prostacyclin (prostaglandins)
Cresenteric glomerulonephritis with IgG and C3 deposits and lord positive anti-GBM antibodies is
Goodpastures
Schistocyte on bloodfilm (=helmet cell) may indicate
HUS
Gene affected in alports
Col4A5
Wilms tumour, proteinuria (diffuse mesangial sclerosis) and ambiguous genitalia capsule be from
Denys-drash syndrome
Prune belly =
Boys
Absent abdominal wall muscle
Bilateral crypdorchidsim
Gu tract abnormality –> kidney failure
Three central causes of micropenis
Kallman
Prader Willi
Panhypopit
Behaviours to delay voiding such as bounding up and down on soles of feet or squatting with urinary incontinence suggests or urge incontinence
Detrusor instability (can’t confirm without invasive test of muscle)
Daytime wetting with urgency and increased voiding frequency consider
overactive bladder, low awareness bladder sensation, poor attention / concentration
Renal USS all day wetters
Treatment needs to relax pelvic floor - behavioural management and can use oxybutinin (antichol)
IgA levels only elevated in what percent of IgA nephropathy
50%
Cerebral salt wasting ANP directly inhibits ADH so ADH is?
Low
Most common cause of nephrotic syndrome
Minimal change
Most common cause of gross haematuria
IgA nephropathy
Renin measurement in HTN - if low suggests
High means renal cause
Mineralocorticoid excess
Electron microscopy: diffuse thickening, thinning, splitting and layering of
glomerular and tubular basement membranes.
Which condition
Alports
Drug that improves calcium in urine
Thiazides (block cl na reabsorption distal tubule)
Most common genetic cause of kidney failure
nephropnopthisis
Stop correcting for BSA GFR what age
2y
Bright kidney on USS causes
Potential causes include: •Dysplasia/ Glomerulocystic disease •ARPKD ( large echogenic kidneys cysts too small to see) •ADPKD •Congenital nephrotic syndrome •Insult such as ischaemia or infection •Beckwith- Wiedeman syndrome •Tuberous sclerosis
Nephropnopthisis and retinal dystrophy two syndromes
Leber and senior loken syndrome
What are the causes of an increased AG metabolic acidosis?
MUDPILES Methanol, Uremia, DKA, Paraldehyde, Iron or Isoniazid, Lactate, Ethylene Glycol, Salicylates
Low chloride in urine when?
Vomiting and laxative abuse
Beta 2 microglobulin used for
In someone with signs of kidney disease, increased levels of B2M in the blood and low levels in the urine indicate that the disorder is associated with glomeruli dysfunction. If B2M is low in the blood and high in the urine, then it is likely that the person has renal tubule damage or disease.
HSP in Armenian may be early indicator of what?
Armenian children with FMF had higher than expected frequency of vasculitis (4.3%). We suppose, that in children with FMF: 1) HSP and PFM vasculitis might be considered as markers of severe FMF and early disease onset 2) M694V homozygous genotype is a risk factor for the development of PFM. These results are consistent with data on the susceptibility of FMF patients in ethnically matched populations in the development of HSP and PFM [Lange-Sperandio B. et al., 2004].MEFV mutation genetic screening is recommended for Armenian children with HSP, PFM vasculitis for early diagnosis of FMF, treatment and prevention of complications.
Renal cysts and MODY gene linked?
Hepatocyte Nuclear Factor 1β
Instead of creatinine what marker can be better and less influenced by muscle mass, age, illness etc
Cystatin C
Tacrilomus potent anti T cell in renal transplant SE (not to be given with cyclosporine)
Tremors, headaches, diarrhoea, hypertension, nausea, renal impairment.
Mycophenilate motefil renal transplant mechanism and SE
inhibits the de novo pathway of guanosine nucleotide synthesis without incorporation into DNA. B and T lymphocyte proliferation depends on de novo guanosine synthesis whereas other cell types can utilise salvage pathways
Diarrhoea and increased cancer risk (lymphoma)
Basiliximab mechanism and side effects
specifically binds to the CD25 antigen on activated t-cells from starting the rejection process
SE low platelets and high lipids
Prevention CMV in transplant
Valgancyclovir 3-6m
Calcium with diuretics (frusemide vs thiazides)
Calcium excretion is increased by loop diuretics and diminished by thiazide-type diuretics and amiloride
What is characterized by weakness, dizziness, headache, and in severe cases, mental status changes in dialysis patient
Dialysis dysequilibrium syndrome is a common neurologic complication seen in dialysis patients
Pseudohyperaldosteronism syndrome
HTN and low K
Liddle
Hypoaldosteronism cause?
CAH
Paroxysmal nocturnal haemoglobinuria mechanism
Sensitive RBC membrane to lysis by complement
Posterior urethral valves and pelvicoureteric junction obstruction differ on MCU by?
PUV has a trabeculated bladder
Name four Causes of false negatives of protein on urine dipstick
captopril, vitamin C, proteinuria, acidic urine
Podocytes are negatively charged and keep albumin in tight junction gene affected in congenital nephrotic syndrome?
Nephrin (NPHS1)
Podocin (NPHS2)
Deny-Drash (WT1)
Hyaline cast in urine may indicate what?
ATN
Boy with kidney stones and proteinuria with calcium in urine and renal impairment from?
Dents syndrome
OCRL1
XL
Affected endocytotic process in PCT
Cataracts, developmental delay, hypotonia and OCRL1 gene affected
Lowe syndrome
Mineralocorticoid from adrenal called
Aldosterone
Pseudoaldosterone with HTN and increased K in?
WNK1
Upregullates NCC
Gordon’s
Present with kidney stone and low potsssium, chloride and metabolic alkalosis
FTT with tubular dysfunction
Like frusemide abuse (LOH)
What is affected and what syndrome
Bartter
(Bart says F and has 4 spikes on head)
Na-K-2cl cotransporter affected
MG and Ca between cell junctions due to gradient
Giggle incontinece treatment
Methylphenidate because centrally mediated
When wee interrupt flow by wctivatiaphincter against flow called
Dysfunctional voiding
Night wetting treatment
Alarm
Desmopressin for sleep over or impacting life
(Oxybutinin)
Lethal ciliopathy with triad occipital encephalocoelenlarge cystic kidneys and polydactyly cause
Meckle-Gruber
How many renal cysts are you allowed per 10 years
1
Caroline disease is?
Hepatic involvement in ARPCKD
