Nephrology Flashcards
What are the 4 primary causes of Nephrotic Syndrome?
Minimal change disease - most common cause in children
Focal segmental glomerulosclerosis (FSGS)
Membranous Nephropathy
Membranoproliferative Glomerulonephritis
Between which age does minimal change disease tend to present?
2-6 years
What is the pathophysiology of minimal change disease?
Minimal change disease is a disease of T-cells which produce cytokines which destroy the epithelial layer (causes flattening of the podocytes which causes leakage of albumin and protein)
Which cancer is associated with minimal change disease?
Hodgkins Lymphoma
What happens to complement factors in minimal change disease?
Normal C3 and C4
What are the indications for biopsy in minimal change disease?
<1 year or >10 years
Not responsive to steroids (no response to steroids after 6 weeks of Tx w/ high dose steroids)
What are the two main complications of minimal change disease?
- Spontaneous Bacterial Peritonitis (due to strep. pneumoniae, e.coli or group B strep)
- Ascities
What percentage of children will outgrow minimal change disease?
80%
Which nephrotic condition should you consider if minimal change disease is not respondent to steroids?
FSGS (NB: FSGS can progress to end stage renal disease)
Which conditions are commonly associated with FSGS?
Heroin use
HIV
Sickle cell anaemia
Which two conditions cause effacement of the foot processes of the epithelial layer?
Minimal change disease
Focal segmental glomerulosclerosis
Which conditions are associated with membranous nephropathy?
SLE (most common GN in SLE)
Solid tumours
Gold
Penicillamine
Infections (Hep B, syphillis, malaria)
What percentage of children with membranous nephropathy progress to end stage renal disease?
25%
Which nephrotic condition is associated with low C3 levels?
Membranoproliferative GN
Which condition is associated with tram/ train track appearance on H&E staining?
Membranoproliferative GN
What is tram track appearance of H&E staining?
Generalised increase in mesangial cells and matrix, capillary walls appear thickened and contain regions of duplication and splitting
What is the characteristics of Minimal change disease/ FSGS?
Proteinuria
Hypoalbuminaemia
Hyperlipidaemia
Hypercoagulable state
Normal C3
“Spike and dome” appearance is associated with which nephrotic condition?
Membranous nephropathy
What are the diagnostic indicators in Lupus Nephritis?
- Positive ANA and anti-dsDNA antibodies
- Low C3 and C4
- Definitive diagnosis is by renal biopsy
When does congenital nephrotic syndrome present?
Between birth and 3 months of age
What are the associations with congenital nephrotic syndrome?
- Severe intractable oedema
- Iron deficiency and vitamin D deficiency
- Hypothyroidism
- Frequent infections due to loss of IgG
- Clots due to loss of antithrombin III
When does screening for microalbuminuria occur in children with diabetes?
Type 1 diabetes - 5 years after diagnosis
Type 2 diabetes - at the time of diagnosis
How long does it take for eGFR to reach adult levels?
1-2 years
Creatinine levels reflect that of the mother for how long after birth?
48 hours
ADH primarily acts at which site?
Collecting ducts
Renin is produced at which site?
The juxtaglomerular apparatus
What is the typical presentation of Alpert’s Syndrome?
Sensorineural hearing loss
Microscopic heamaturia
Progresses to severe renal disease
What are the characteristics of medullary sponge kidneys?
Presents in middle age
Recurrent nephrolithiasis
Urinary tract infections
What is the commonest cause of hypertension in neurofibromatosis type 1?
Renal artery stenosis or phaeochromocytoma
What are the main complications of nephrotic syndrome?
Infections - including spontaneous bacterial peritonitis
Thrombosis - e.g. DVT, PE, renal vein thrombosis
Hypertension
Which investigation assesses for reflux and the presence of posterior urethral valves?
MCUG
Which investigation assesses for scarring of the kidneys?
DMSA
Bartter syndrome can be associated with which neonatal complication?
Polyhydramnios
Features of Gitelmann syndrome?
Hypokalaemia + hypomagnesaemia + hypocalciuria
Normal blood pressure
What is the pathophysiology of Gitelman syndrome?
Autosomal recessive condition resulting in a defect in the sodium chloride transporter in the distal collecting tubules
Features of cystinosis?
Hypokalaemia + hypophosphataemia + a metabolic acidosis with a high urinary pH
Which glomerulonephritis has normal complement?
Primary GN:
- FSGS
- IgA nephropathy
Secondary GN:
- HSP
Which glomerulonephritis has low complement levels?
Primary GN:
- Acute post-streptococcal GN (low C3, normal C4)
Membranoproliferative GN:
- Low C3, low C4
Secondary GN:
- Lupus nephritis (low C3, low C4)
Which medication is indicated in genetic HUS due to complement dysregulation?
Eculizumab
HUS is a triad of which three conditions?
Microangiopathic haemolytic anaemia
Thrombocytopenia
Acute kidney injury
What are the predictive factors for a poor prognosis in HUS?
- Non Shiga toxin HUS
- Prolonged oliguria and anuria
- Severe hypertension
- Involvement in medium sized arteries
- Severity of CNS symptoms
- Peristent consumption of clotting factors
- Age >5 years
Which receptor is mutated in Bartter’s syndrome?
Na+ K+ Cl- co-transporter in the thick ascending limb of the loop of henle
Features of Bartters syndrome?
Metabolic alkalosis (due to K+ exchange for H+ in the distal convulsed tubules)
Low Na, K+ and Chloride
Hyperplasia of the juxtaglomerular apparatus
Elevated renin and aldosterone
What are the investigations of choice in vesico-ureteric reflux?
MCUG - assess degree of reflux
DMSA - assess degree of scarring
Video urodynamic studies - clarify type and severity of VUR
The presence of bilateral hydronephrosis and oligohydramnios on antenatal screening should raise suspicion of?
VUR
Outline the grading of VUR?
Grade I: Reflux into a non-dilated ureter only
Grade II: Reflux into the pelvis and calyces with no dilatation
Grade III: Reflux into the pelvis and calyces with mild dilatation
Grade IV: Reflux into the pelvis and calyces with moderate ureteral tortuosity
Grade V: Ureter/ renal pelvis/ calyces is grossly distended with loss of papillary impressions
Normal complement - purely renal involvement?
IgA nephropathy
Anti-GBM disease
Anti-ANCA disease
Normal complement with systemic involvement (and renal involvement)?
HSP
Alports syndrome
Low C3 with purely renal involvement?
Post streptococcal GN
Mesangioproliferative GN
C3 glomerulonephropathy
Low C3 with systemic involvement?
SLE
Subacute bacterial endocarditis
What is Goodpasture’s Disease a triad of?
GN + Pulmonary haemorrhage + anti-GBM antibody formation
What findings occur in post-streptococcal GN?
Low C3
Low CH50
What are the three heritable renal cystic diseases?
Cysts associated with multiple malformations
Infantile (autosomal recessive) polycystic disease
Adult (autosomal dominant) polycystic disease
Commonest chromosomal defect in polycystic disease 1?
Chromosome 16
Nephrotic syndrome with partial lipodystrophy is most associated with…?
Mesangiocapillary nephritis with C3 nephritic factor
What triad of symptoms could represent a renal vein thrombosis?
Macroscopic haematuria
Thrombocytopenia
Enlarged kidneys (unilateral/ bilateral) - may present with deranged creatinine/ urea
Which PKD is associated with hepatic involvement?
Autosomal recessive
