Kidney infarction in Friedreich's ataxia with dilated cardiomyopathy


A 37-year-old man with advanced Friedreich's ataxia was referred to our emergency department with acute exacerbated abdominal pain of unclear aetiology. Laboratory tests showed slightly increased inflammatory parameters, elevated troponin and B-type natriuretic peptide, as well as minimal proteinuria. Transthoracic echocardiography revealed a pre-existing dilated cardiomyopathy. Abdominal sonography showed no pathological alterations. Owing to persistent pain under analgesia, a contrast-enhanced CT-abdomen was performed, which revealed a non-homogeneous perfusion deficit of the right kidney, although neither abdominal vascular alteration, cardiac thrombus, deep vein thrombosis nor a patent foramen ovale could be detected. Taking all clinical and radiological results into consideration, the current incident was diagnosed as a thromboembolic kidney infarction. As a consequence, lifelong oral anticoagulation was initiated.

Hi Alan, thank you for sharing this. Maybe this kidney infarction is somehow related to the dilated cardiomyopathy of this patient, as the kidneys are also organs which can accumulate iron in an unhealthy way. I know this from the iron store problems after blood/erythrocyte infusions. I don t know much about defective iron metabolism in ataxias; such exists in at least one of them, but perhaps it can exist in all of them. So iron can be stored on some spots in an accumulated way, mainly in organs (the heart?, the kidneys? The liver?, the brain?) I can imagine that attempts for iron chelation could help to minimize the risks resulting from iron accumulation. There have been done tests with deferiprone, an oral chelator which chelating properties are especially good for the heart, - but : not successful?

In my case they have found considerably increased iron stores in my liver (did not test the heart or kidneys for this) by MRIs in 2008 and 2011. Hematologists tend to deny that these could be caused (only) by the blood transfusions i got during my chemotherapies and then transplantation in 2007/2008. It is said, that some patients store more, other patients fewer amounts of iron in their bodies. 2011 a bone marrow biopsy found out that i have got a massive ironsiderosis in my bone marrow. Maybe this all has been caused by my still undiagnosed ataxia. My personal strategy consists in growing wheat grass at home and drinking the juice for iron chelation.There have been indian studies that wheatgrass is an effective (and cheap) iron chelator, also good for other health reasons).

This text is actually not for free as a fulltext in our Viennese library, - it will be only in 2 years. It would be on me to study the iron storage andcardio- myopathy problems in ataxias, and for that i would not need this fulltext. There are plenty of other fulltexts available for free. And books. But lifetime, personal discipline and energy are restricted. We have got so much of wisdom in all our books and articles..