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A complex case of fatal calciphylaxis in a female patient with hyperparathyroidism secondary to end stage renal disease of graft and coexistence of haemolytic uremic syndrome
AI. Lo Monte, M. Bellavia, G. Damiano, MC. Gioviale, C. Maione, VD. Palumbo, G. Spinelli, C. Tripodo, F. Cacciabaudo, A. Sammartano, S. Buscemi, S. De Luca, S. Di Ganci, G. Buscemi
Language English Country Czech Republic
Document type Case Reports, Journal Article, Research Support, Non-U.S. Gov't
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- MeSH
- Kidney Failure, Chronic complications MeSH
- Fatal Outcome MeSH
- Hemolytic-Uremic Syndrome complications MeSH
- Calciphylaxis etiology MeSH
- Middle Aged MeSH
- Humans MeSH
- Graft Rejection MeSH
- Hyperparathyroidism, Secondary complications MeSH
- Kidney Transplantation * MeSH
- Check Tag
- Middle Aged MeSH
- Humans MeSH
- Female MeSH
- Publication type
- Journal Article MeSH
- Case Reports MeSH
- Research Support, Non-U.S. Gov't MeSH
BACKGROUND: Calciphylaxis is a potentially fatal complication of persistent secondary hyperparathyroidism; its cause is still not clear. Unfortunately there is no close relation in severity of clinical picture, serological and pathological alteration. For this reason the prognosis is difficult to establish. Administration of sodium thiosulphate may reduce the precipitation of calcium crystals and improve the general clinical conditions before surgical parathyroidectomy, which seems the only therapeutic approach able to reduce the mortality risk in these patients. METHODS AND RESULTS: A 60 year old female patient suffering from End Renal Stage Disease, on haemodialysis from 2001 due to the onset of haemolytic uremic syndrome, underwent a kidney transplant in April 2008. After transplantation there was a recurrence of the haemolytic uremic syndrome, with temporary worsening of the graft. Six months later there was a definite loss of graft and return to dialysis treatment. On April 2010 a severe systemic calciphylaxis related to secondary hyperparathyroidism was diagnosed. The patient underwent parathyroidectomy but, because of the unimproved clinical picture, treatment with sodium thiosulphate was initiated. There was only improvement in cutaneous lesions. The worsening general clinical condition of the patient caused death due to general septic complications. CONCLUSIONS: The coexistence of haemolytic uremic syndrome and secondary hyperpathyroidism makes the prognosis poor and, in this case, therapy, which counteracts calcium crystals precipitation, has no effect. Preventive parathyroidectomy can be considered as the only possible treatment.
Consorzio Interuniversitario dei Trapianti d'Organo Rome
Department of Human Pathology Faculty of Medicine University of Palermo
Department of Surgical and Anaesthetic Sciences University of Bologna
Department of Surgical and Oncological Sciences Faculty of Medicine University of Palermo
References provided by Crossref.org
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- $a Lo Monte, Attilio Ignazio $u Department of Surgical and Oncological Sciences, Faculty of Medicine, University of Palermo; PhD Research in Surgical Biotechnology and Regenerative Medicine, Faculty of Medicine, University of Palermo; P. Giaccone University Hospital, Palermo
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- $a A complex case of fatal calciphylaxis in a female patient with hyperparathyroidism secondary to end stage renal disease of graft and coexistence of haemolytic uremic syndrome / $c AI. Lo Monte, M. Bellavia, G. Damiano, MC. Gioviale, C. Maione, VD. Palumbo, G. Spinelli, C. Tripodo, F. Cacciabaudo, A. Sammartano, S. Buscemi, S. De Luca, S. Di Ganci, G. Buscemi
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- $a BACKGROUND: Calciphylaxis is a potentially fatal complication of persistent secondary hyperparathyroidism; its cause is still not clear. Unfortunately there is no close relation in severity of clinical picture, serological and pathological alteration. For this reason the prognosis is difficult to establish. Administration of sodium thiosulphate may reduce the precipitation of calcium crystals and improve the general clinical conditions before surgical parathyroidectomy, which seems the only therapeutic approach able to reduce the mortality risk in these patients. METHODS AND RESULTS: A 60 year old female patient suffering from End Renal Stage Disease, on haemodialysis from 2001 due to the onset of haemolytic uremic syndrome, underwent a kidney transplant in April 2008. After transplantation there was a recurrence of the haemolytic uremic syndrome, with temporary worsening of the graft. Six months later there was a definite loss of graft and return to dialysis treatment. On April 2010 a severe systemic calciphylaxis related to secondary hyperparathyroidism was diagnosed. The patient underwent parathyroidectomy but, because of the unimproved clinical picture, treatment with sodium thiosulphate was initiated. There was only improvement in cutaneous lesions. The worsening general clinical condition of the patient caused death due to general septic complications. CONCLUSIONS: The coexistence of haemolytic uremic syndrome and secondary hyperpathyroidism makes the prognosis poor and, in this case, therapy, which counteracts calcium crystals precipitation, has no effect. Preventive parathyroidectomy can be considered as the only possible treatment.
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