Renal Flashcards
(179 cards)
What are the 6 nephrotic syndromes?
- Diabetic nephropathy
- Amyloidosis
- Minimal change disease
- Focal segmented glomerulosclerosis
- Membranous neuropathy
- Membranoproliferative glomerulonephritis
What is amylodiosis?
Amyloidosis refers to the disease state caused by abnormal amyloid protein deposits in organs.
What are the clinical manifestations of amyloidosis?
- Kidney: swelling in the face, ankles, and legs
- Heart: palpitations and shortness of breath
- Intestines: malabsorption and weight loss
- Large tongue: speech and swallowing difficulty, noisy breathing at night
- Nerves: numbness or pain in the fingers and toes, alternating bouts of diarrhoea and constipation, orthostatic hypotension
- CNS: Alzheimer’s memory loss and difficulty learning new things
What are the investigations for amyloidosis?
- Fat pad biopsy: apple-green birefringence under polarised microscopy with Congo red stain.
- SAP scan: to show amount and location of amyloid
What is the management for amyloidosis?
- Organ transplant, if extensive damage.
- AL amyloidosis:
- Optimise nutrition, if malabsorption
- Alkylating agent + corticosteroid: e.g. melphalan and prednisolone
- Stem cell transplant
What is minimal change diease?
Minimal change disease (MCD) is a kidney disease that can cause nephrotic syndrome, a condition in which the kidneys leak large amounts of protein. It is called “minimal change” because the damage to the kidneys cannot be seen under a microscope.
What are the investigations for minimal change disease?
- On light microscopy, the glomeruli look completely normal.
- In some cases, there can be lipids in the proximal tubular cells.
- Immunofluorescence is negative.
- The only changes are seen on electron microscopy, where there’s effacement of podocyte foot processes.
What is the management for minimal change disease?
Idiopathic minimalchange disease is the only nephrotic disease that can be consistently treated with corticosteroids.
What is Focal Segmental Glomerulosclerosis?
Focal Segmental Glomerulosclerosis (FSGS) is a rare disease that affects the kidneys’ glomeruli, which are tiny blood vessels that filter waste and excess fluids from the blood. In FSGS, the glomeruli become scarred and damaged.
What are the investigations for Focal Segmental Glomerulosclerosis?
- On light microscopy, there’s sclerosis and hyalinosis among the glomeruli.
- FSG is segmental - only a part of the glomeruli is affected - and focal - only some of the glomeruli are affected.
- Immunofluorescence is often negative, but can sometimes be positive for deposits of C3, C1 or IgM.
What is the management for Focal Segmental Glomerulosclerosis?
Primary FGS has an inconstant response to corticosteroids and some individuals may progress to CKD
What is Membranous Neuropathy?
Membranous neuropathy is a rare autoimmune disease that affects the nerves that control muscle movement and sensation in the body. It is also known as Membranous nephropathy or MN.
What are the clinical manifestations of membranous neuropathy?
The symptoms of membranous neuropathy can vary from person to person, but the most common symptoms include:
- Muscle weakness
- Tingling and numbness in the extremities
- Loss of reflexes
- Difficulty walking or standing
- Pain in the limbs
- Fatigue
- Loss of sensation in the limbs
What are the investigations for membranous neuropathy?
Biopsy
- Light microscopy - diffuse capillary and glomerular basement membrane thickening
- When the sample is stained with silver methenamine, irregular expansions can be seen
- Immunofluorescence will show that the immune complexes are granular and mostly made of IgG and C3.
- Electron microscopy
- flattening of the podocyte foot processes and sub-epithelial deposits of immune complexes.
What is the management for membranous neuropathy?
Poor response to corticosteroids and individuals may progress to CKD
What is Membranoproliferative Glomerulonephritis?
Three types of MPGN: they all cause proliferation of mesangial and endothelial cells in the glomerulus.
What are the risk factors for Membranoproliferative Glomerulonephritis?
- Age (75% idiopathic)
- Infections - hepatitis B, hepatitis C, HIV and endocarditis
- Chronic lymphocytic leukaemiaand cryoglobulinaemia
- Acquired partial lipodystrophy (ADP)
What are the signs of Membranoproliferative Glomerulonephritis?
- Oedema
- Oliguria
- Haematuria
- Hypertension
What are the symptoms of Membranoproliferative Glomerulonephritis?
- Foamy urine (proteinuria)
- Pink, red or ‘coke’ tinged urine (haematuria)
What are the investigations for Membranoproliferative Glomerulonephritis?
- Urine dipstick
- Urine microscopy and culture
- U&Es
- C3 and C4 levels
- Renal biopsy for definitive diagnosis
- PAS staining on light microscopywill show mesangial cell proliferation and capillary thickening
What is the management for Membranoproliferative Glomerulonephritis?
Primary MPGN
- Oral cyclophosphamide
- Oral mycophenolate mofetil (MMF) and oral corticosteroids
Secondary MPGN
- Treat the cause
General
- ACEi/ARB
- Statin
- Oral warfarin
What are the complications of Membranoproliferative Glomerulonephritis?
- Hypertension
- Infection susceptibility
- Pro-thrombotic state
- Hyeperlipidaemia
- Hyperthyroidism
- Hypocalcaemia
- AKI
- Chronic kidney disease
- Rapidly progressive glomerulonephritis
What is post-streptococcal glomerulonephritis?
Post-streptococcal glomerulonephritis (PSGN) is usually an immunologically-mediated delayed consequence of pharyngitis or skin infections caused by streptococcus pyogenes.
What are the 8 nephritic syndromes?
– Post-streptococcal glomerulonephritis
- IgA neuropathy
- Good pastures syndrome
- SLE
- Diffuse proliferative glomerulonephritis
- Rapidly progressive glomerulonephritis
- Alport syndrome
- Membranoproliferative glomerulonephritis