A 27-year-old man is investigated for haemoptysis. He is a non-smoker and has no respiratory history of note. Whilst awaiting a bronchoscopy he becomes lethargic and anorexic.
Blood tests show the following:
A renal biopsy is performed and shows linear IgG deposits along the basement membrane.
What type of antibodies are most likely to cause this type of presentation?
Correct Answer E:
Goodpasture's syndrome:
The combination of haemoptsis, renal failure and linear IgG deposits points to a diagnosis of Goodpasture's syndrome. The ANCA-related nephropathies are associated with crescentic glomerulonephritis.
Goodpasture's syndrome: Goodpasture's syndrome is rare condition associated with both pulmonary haemorrhage and rapidly progressive glomerulonephritis. It is caused by anti-glomerular basement membrane (anti-GBM) antibodies against type IV collagen. Goodpasture's syndrome is more common in men (sex ratio 2:1) and has a bimodal age distribution (peaks in 20-30 and 60-70 age bracket). It is associated with HLA DR2.
Features:
Factors which increase likelihood of pulmonary haemorrhage:
Investigations:
Management:
A 24-year-old woman is diagnosed as having nephrotic syndrome after being investigated for proteinuria. A diagnosis of minimal change glomerulonephritis is made.
What is the most appropriate initial treatment to reduce proteinuria?
Correct Answer E: Minimal change glomerulonephritis - prednisolone.
Angiotensin-converting-enzyme inhibitors may be used to reduce proteinuria in patients with heavy proteinuria or who have a slow response to prednisolone.
Minimal change glomerulonephritis: Minimal change glomerulonephritis nearly always presents as nephrotic syndrome, accounting for 75% of cases in children and 25% in adults.
The majority of cases are idiopathic, but in around 10-20% a cause is found:
Prognosis is overall good, although relapse is common. Roughly:
*only intermediate-sized proteins such as albumin and transferrin leak through the glomerulus
A 64-year-old female is brought to the Emergency Department by her family, who are concerned about her increasing confusion over the past 2 days. On examination she is found to be pyrexial at 38ºC.
Blood tests reveal:
What is the most likely diagnosis?
Correct Answer B: HUS or TTP? Neuro signs and purpura point towards TTP.
The combination of neurological features, renal failure, pyrexia and thrombocytopaenia point towards a diagnosis of thrombotic thrombocytopenic purpura.
Thrombotic thrombocytopenic purpura:
Pathogenesis of thrombotic thrombocytopenic purpura (TTP) :
Causes:
A 45-year-old female with nephrotic syndrome develops renal vein thrombosis.
What changes in patients with nephrotic syndrome predispose to the development of venous thromboembolism?
Correct Answer B:
Nephrotic syndrome:
Triad of:
Loss of antithrombin-III, proteins C and S and an associated rise in fibrinogen levels predispose to thrombosis.
Loss of thyroxine-binding globulin lowers the total, but not free, thyroxine levels.
A 14-year-old girl is referred to the paediatric unit with reduced urine output and lethargy. She has been passing bloody diarrhoea for the past four days. On admission she appears dehydrated.
Bloods show the following:
Given the likely diagnosis, which one of the following organisms is the most likely cause?
Correct Answer C: Haemolytic uraemic syndrome - classically caused by E coli 0157:H7.
Haemolytic uraemic syndrome:
Haemolytic uraemic syndrome is generally seen in young children and produces a triad of:
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