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.

Lo Monte, A.I., Bellavia, M., Damiano, G., Gioviale, M.C., Maione, C., Palumbo, V.D., et al. (2012). 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. BIOMEDICAL PAPERS OF THE THE FACULTY OF MEDICINE OF PALACKÝ UNIVERSITY, OLOMOUC CZECH REPUBLIC, 156(3), 262-265 [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

LO MONTE, Attilio Ignazio;BELLAVIA, Maurizio;DAMIANO, Giuseppe;GIOVIALE, Maria Concetta;PALUMBO, Vincenzo Davide;SPINELLI, Gabriele;TRIPODO, Claudio;CACCIABAUDO, Francesco;SAMMARTANO, Antonino;Buscemi, Salvatore;DI GANCI, Simona;BUSCEMI, Giuseppe
2012

Abstract

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.
Settore MED/18 - Chirurgia Generale
Settore MED/14 - Nefrologia
Settore MED/08 - Anatomia Patologica
Lo Monte, A.I., Bellavia, M., Damiano, G., Gioviale, M.C., Maione, C., Palumbo, V.D., et al. (2012). 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. BIOMEDICAL PAPERS OF THE THE FACULTY OF MEDICINE OF PALACKÝ UNIVERSITY, OLOMOUC CZECH REPUBLIC, 156(3), 262-265 [10.5507/bp.2012.018].
File in questo prodotto:
File Dimensione Formato  
A complex case.pdf

accesso aperto

Descrizione: Articolo
Dimensione 250.45 kB
Formato Adobe PDF
250.45 kB Adobe PDF Visualizza/Apri

I documenti in IRIS sono protetti da copyright e tutti i diritti sono riservati, salvo diversa indicazione.

Utilizza questo identificativo per citare o creare un link a questo documento: http://hdl.handle.net/10447/97453
Citazioni
  • ???jsp.display-item.citation.pmc??? 3
  • Scopus 11
  • ???jsp.display-item.citation.isi??? 9
social impact