Nephrology part 1 Flashcards
What are the functions of the kidneys?
○ Waste handling - Urine - Creatinine ○ Water handling ○ Salt balance - Sodium - Potassium - Calcium - Phosphate ○ Acid base control - Bicarbonate ○ Endocrine - Red cells- Erythropoietin - Blood pressure- renin aldosterone angiotensin system - Bone health- PTH, activation of vitamin D
What are the two main presentations of nephrology?
○ Haematuria
○ Proteinuria
In glomerulonephropathy what does blood and protein dictate?
- clinical presentation
- sugersted diagnosis
What are the two main signs of nephritic syndrome?
- Increasing haematuria
- Intravascular overload
What are the two main signs of nephrotic syndrome?
- Increasing proteinurea
- Intravscular depletion
How common are aquired glomerulopathy and congenital glomerulonephropathy?
Acquired- common
Congential- rare
Which componants are affected in acquired glomerulopathy?
- Epitherlial cell (podocyte)
- Basement membrane
- Endothelial cell
- Mesangial cell
What acquired glomerulopathies cause epithelial cells to be damaged?
□ Minimal change disease
□ FSGS (Focal segmental glomerulosclerosis)
□ Lupus
□ Congenital nephrotic syndrome
What acquired glomerulopathies cause the basement membrane to be damaged?
- membranous glomerulopathy
- MPGN (Membranoproliferative glomerulonephritis )
- post infectious glomerulonephritis (PIGN)
What acquired glomerulopathies cause endothelial cells to be damaged?
□ Infection associated glomerulonephritis (PIGN)
□ Haemolytic Uraemic syndrome (HUS)
□ Membranoproliferative glomerulonephritis (MPGN)
□ Lupus
□ Thin basement membrane
What acquired glomerulopathies cause mesangial cells to be damaged?
□ HSP (Henoch-Schonlein purpura )
□ IgA nephropathy
□ Lupus
What are the layers involved in congenital glomerulonephropathy?
- podocyte cytoskeletal integrity
- Basement membrane proteins
- Endothelial/ microvascular integrity
What is the definition of nephrotic syndrome?
Nephrotic range proteinuria results in hypalbuminaemia which results in oedema
How is proteinurea tested for?
□ Dipstix ® Give concentration ® >/=3+ usually abnormal ® False positives ® False negative □ Protein creatinine ration (practical) ® Early morning urine (best) ◊ Normal Pr:Cr ration <20mg/mmol ◊ Nephrotic range >25mg/mmol □ 24 hour urine collection (gold standard) ® Normal <60mg/m^2/24 hours ® Nephrotic range >1g/m^2/24 hours
How is nephrotic syndrome diagnosed?
- Oedema
- Proteinuria
□ Urine dipstix
® Protein 3+
® Blood 2+ blood (not frank)
□ Protein creatinine ration- 1200mg/mmol creatinine
□ Urine Na- 10 - Bloods
□ Albumin low 12mg/dl
□ Normal creatinine- n the most common type
What are the typical features of minimal change disease?
□ Age 1-10 but most commonly 2-5 years □ Normal blood pressure □ Resolving microscopic haematuria □ Normal renal function □ Steroid responsive
What are the atypical features of minimal change disease?
□ Suggestion of autoimmune disease
□ Abnormal renal function
□ Steroid resistance
□ Only if atypical consider biopsy
What is the treatment of typical minimal change disease?
Prednisolone for 8 weeks
What are the side effects from high dose corticosteroids in children?
□ Varicella status (infection)
□ Pneumococcal vaccination
□ Behaviour
□ Hypertension
What is the pathogenesis of minimal change disease?
- Interactions between lymphocytes (T and B cells) and podocytes
- This is reversable with prednisolone
What are the outcomes of minimal change disease?
- 95% remission in 2-4 weeks
- 80% relapse
□ 50% frequent - 80% long term remission
What are the second line immunosuppressions in minimal change disease?
Levamisole, Cyclosporin, Tacrolimus, mycophenolate mofetil, Rituximab
Give an example of an aquired steroid resistant nephrotic syndrome?
- Focal segmental glomerulosclerosis (FSGS)
□ Podocyte loss
□ Progressive inflammation and sclerosis
Give examples of congenital steroid resistant nephrotic syndrome?
- Infant presentations
- NPHS1- nephrin
- NPHS2- podocin
- Podocyte loss
What is the treatment of steroid resistant nephrotic syndrome?
Levamisole, Cyclosporin, Tacrolimus, mycophenolate mofetil, Rituximab
When should you investigate macroscopic haematuria?
- Always abnormal
- Investigate
When should you investigate microscopic haematuria?
- Investigate
□ >trace on 2 occasions - Haemoglobinuria
□ Stix positive + microscopy negative
What are the common causes of macroscopic haematuria?
□ Glomerulonephritis ® Post infection GN ® IGS/HSP □ UTI (dysuria) □ Trauma □ Stones (pain)
What are the common causes of microscopic haematuria?
□ Glomerulonephritis ® Post infection GN ® IGA/ HSP □ UTI □ Trauma □ Stones/ hypercalciuria
What is nephritic syndrome?
○ Clinical diagnosis - Describes glomerulonephritis ○ Haematuria and proteinuria ○ Reduced GFR - Oliguria - Fluid overload □ Raised JVP □ Oedema - Hypertension - Worsening renal failure= rapidly progressing GN ○ Intrarenal cause of acute kidney injury (AKI)
What are the causes of glomerulonephritis?
○ Post infectious GN ○ HSP/ IgA nephropathy ○ Membranoproliferative GN ○ Lupus nephritis ○ ANCA positive vasculitis
What causes post infectious glomerulonephritis?
○ Age of onset ○ Cause - Usually group A strep - Beta haemolytic - Site □ Throat 7-10 days □ Skin 2-4 weeks
How is post infectious glomerulonephritis diagnosed?
- Bacterial culture
- Positive ASOT (anti streptolysin O titre)
- Low C3 normalises
- Remember
What is the differential diagnosis of post infectious glomerulonephritis?
® IgA
® Lupus
® MPGN
What is the treatment for post infectious glomerulonephritis?
- Self limiting
- Antibiotic: penicillin
- Support renal functions
- Overload/ hypertension
□ Diuretics
What is the outcome of post infectious glomerulonephritis?
Not recurrent?
What is IgA nephropathy?
○ Most common glomerulonephritis
○ 1-2 days after URTI
○ Usually in older children and adults
What is the clinical picture of IgA nephropathy?
> Recurrent macroscopic haematuria
+/- chronic microscopic haematuria
Varying degree of proteinuria
How is IgA npehropathy diagnosed?
- Clinical picture
□ Negative autoimmune workup
□ Normal compliment - Conformational biopsy
What is the treatment of IgA nephropathy?
- Mild disease
□ Proteinuria with ACEi - Moderate to severe disease
□ Immunosuppression (KDIGO)
What is the outcome of IgA nephropathy?
- Variable
- 25% ESFR by 10 years post diagnosis
- Outcome better in children?
What is Henoch Scholein purpura?
○ IgA related vasculitis
○ Age of onset: 5-15 years
○ Non streptococcal post infectious GN
How is Henoch Scholein purpura diagnosed?
○ Clinical diagnosis - Mandatory palpable purpura - One of 4 □ Abdominal pain □ Renal involvement □ Arthritis or arthralgia □ Biopsy ® IgA deposition
What can trigger Henoch Scholein purpura?
- Viral URTI in 70%
- Streptococcus, drugs
When do symtoms start happening in Henoch Scholein purpura?
1-3 days post trigger
How long do symptoms last in Henoch Scholein purpura?
> 4-6 weeks
> 1/3 relapse
What is the treatment of Henoch Scholein purpura?
- Symptomatic □ Joints. Gut - Glucocorticoid therapy □ Not helpful in mild nephritis □ May help with GI involvement - Immunosuppression □ Trial in moderate to severe renal disease - Long term □ Hypertension and proteinuria screening
