6533b7d2fe1ef96bd125f519

RESEARCH PRODUCT

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.

Salvatore De LucaCarolina MaioneGiuseppe DamianoGiuseppe BuscemiSalvatore BuscemiAttilio Ignazio Lo MonteFrancesco CacciabaudoClaudio TripodoAntonino SammartanoMaurizio BellaviaVincenzo Davide PalumboMaria Concetta GiovialeSimona Di GanciGabriele Spinelli

subject

ParathyroidectomyGraft Rejectionmedicine.medical_specialtymedicine.medical_treatmentCalciphylaxiSettore MED/08 - Anatomia PatologicaGeneral Biochemistry Genetics and Molecular BiologyEnd stage renal diseaseFatal OutcomeSecondary hyperparathyroidism; Calciphylaxis; Kidney transplant; ParathyroidectomymedicineHumansKidney transplantDialysisParathyroidectomySettore MED/14 - NefrologiaCalciphylaxisHyperparathyroidismbusiness.industryCalciphylaxisMiddle Agedmedicine.diseaseKidney TransplantationSurgeryTransplantationSecondary hyperparathyroidismSettore MED/18 - Chirurgia GeneraleHemolytic-Uremic SyndromeKidney Failure ChronicSecondary hyperparathyroidismFemaleHyperparathyroidism SecondaryComplicationbusiness

description

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.

10.5507/bp.2012.018https://pubmed.ncbi.nlm.nih.gov/22660230