Biomed Pap Med Fac Univ Palacky Olomouc Czech Repub. 2012, 156(3):262-265 | DOI: 10.5507/bp.2012.018

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

Attilio Ignazio Lo Monte^a,b,d, Maurizio Bellavia^a, Giuseppe Damiano^d, Maria Concetta Gioviale^d, Carolina Maione^d, Vincenzo Davide Palumbo^a,b,d, Gabriele Spinelli^a, Claudio Tripodo^b,c,d, Francesco Cacciabaudo^a,b, Antonino Sammartano^a,b, Salvatore Buscemi^f, Salvatore De Luca^a, Simona Di Ganci^a, Giuseppe Buscemi^a,b,d,e

^a Department of Surgical and Oncological Sciences, Faculty of Medicine, University of Palermo, Italy

^b PhD Research in Surgical Biotechnology and Regenerative Medicine, Faculty of Medicine, University of Palermo

^c Department of Human Pathology, Faculty of Medicine, University of Palermo

^d P. Giaccone University Hospital, Palermo

^e Consorzio Interuniversitario dei Trapianti d'Organo, Rome

^f Department of Surgical and Anaesthetic Sciences, University of Bologna

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.

Keywords: secondary hyperparathyroidism, calciphylaxis, kidney transplant, parathyroidectomy

Received: August 31, 2011; Accepted: January 31, 2012; Prepublished online: April 19, 2012; Published: September 1, 2012  Show citation