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Erschienen in:
01.05.2015 | Clinical Quiz
A very rare cause of acute kidney injury: Answers
Erschienen in: Pediatric Nephrology | Ausgabe 5/2015
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1.
Paroxysmal cold hemoglobinuria (PCH).
2.
Acute kidney injury (AKI) in paroxysmal cold haemoglobinuria results from a toxic effect of the released haemoglobin on renal tubules, mediated by tubular lumen occlusion caused by complexes formed of haeme and Tamm–Horsfall protein. A molecular mechanism for a toxic effect of haeme has also been reported that results from a deficiency of haeme oxygenase which in turn is responsible for haeme degradation. This process leads to increased levels of free iron and subsequent renal failure.
3.
Intravascular haemolysis results from the activation of the complement cascade by cold-reactive, biphasic immunoglobulin G (IgG) antibodies. The direct antiglobulin test becomes positive with the use of anti-C3 serum. Protection from cold and symptomatic treatment are of paramount importance in the management of this condition.