Which one of the following is least recognized as an indication for plasma exchange?
Correct Answer D: Cerebral malaria is not a standard indication for plasma exchange. Exchange transfusions have been tried but it is generally only justified when peripheral parasitemia is greater than 10% of circulating erythrocytes. The role of blood transfusions remains controversial, as they are both expensive and potentially dangerous in many malaria areas.
Plasma exchange:
Indications for plasma exchange:
A 26-year-old man with loin pain and haematuria is found to have autosomal dominant polycystic kidney disease.
A defect in which one of the following genes is likely to be responsible?
Correct Answer E: Most cases of autosomal dominant polycystic kidney disease (ADPKD) are due to a mutation in the PKD1 gene.
The PKD1 gene encodes for a polycystin-1, a large cell-surface glycoprotein of unknown function.
ADPKD: Autosomal dominant polycystic kidney disease (ADPKD) is the most common inherited cause of kidney disease, affecting 1 in 1,000 Caucasians. Two disease loci have been identified, PKD1 and PKD2, which code for polycystin-1 and polycystin-2 respectively.
The screening investigation for relatives is abdominal ultrasound:
Ultrasound diagnostic criteria (in patients with positive family history)
A 62-year-old man is diagnosed with renal cell cancer.
Which one of the following hormones is least likely to be present in excessive levels?
Correct Answer C:
Renal cell cancer: Renal cell cancer is also known as hypernephroma and accounts for 85% of primary renal neoplasms. It arises from proximal renal tubular epithelium.
Associations*:
Features:
Management:
*Incidence of renal cell cancer is only slightly increased in patients with autosomal dominant polycystic kidney disease.
A 5-year-old boy is seen in the Emergency Department due to lethargy and pallor. There is no recent history of diarrhoea. The following results are obtained:
What is the most appropriate management?
Correct Answer E: There is no role for antibiotics, steroids or immunosuppressants in haemolytic uraemic syndrome (HUS). Plasma exchange may be indicated, particularly in severe cases of HUS not associated with diarrhoea.
Haemolytic uraemic syndrome:
Haemolytic uraemic syndrome is generally seen in young children and produces a triad of:
Causes:
Investigations:
A 33-year-old man with a history of coeliac disease is admitted for investigation of recurrent macroscopic haematuria. His urine is typically brown and there is no history of passing clots.
What is the most likely diagnosis?
Correct Answer B: This man has IgA nephropathy which is associated with coeliac disease.
IgA nephropathy:
Basics:
Differentiating between IgA nephropathy and post-streptococcal glomerulonephritis:
Presentations:
Associated conditions:
Prognosis:
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