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001-es BibID:BIBFORM061593
Első szerző:Hamrahian, S. Mehrdad (orvos)
Cím:Symmetrical craniofacial hypertrophy in patients with tertiary hyperparathyroidism and high-dose cinacalcet exposure / Hamrahian, S. Mehrdad, Pitman, Karen T., Csongrádi Éva, Bain, Justin H., Kanyicska Béla, Fülöp Tibor
Dátum:2012
ISSN:1492-7535
Megjegyzések:We are reporting on a series of two patients with end-stage renal disease on hemodialysis,presented for surgical parathyroidectomy secondary refractory hyperparathyroidism. Both patientshad failed maximized medical managements, including higher-than-usual doses of the calcimimeticcinacalcet (270 and 180 mg/day, respectively). On physical exam, both patients had markedsymmetrical craniofacial hypertrophy with coarse distortion of facial features, similar in appearanceto past reports of Sagliker syndrome. On X-ray and computed tomographic exam, they had peculiarareas of bone absorption on the skull, imitating the radiologic appearance of multiple myeloma.Bone biopsy of the maxilla, however, did not show the expected brown tumor, but rather describedonly fibrosis and reactive bone formations. This phenotype developed while being on cinacalcet,progressed despite escalation of therapy, and improved only after parathyroidectomy. Both patientsdeveloped massive "hungry bone syndrome" after parathyroidectomy necessitating prolonged IVcalcium infusion. This pattern of severe facial distortion likely represented an adverse consequenceof severe tertiary hyperparathyroidism, along with supraphysiologic dose of cinacalcet administrationand 25-hydroxy vitamin D deficiency in sensitive individuals. The genetic base of this observationremained unexplained.
Tárgyszavak:Orvostudományok Klinikai orvostudományok idegen nyelvű folyóiratközlemény külföldi lapban
Megjelenés:Hemodialysis International 16 : 4 (2012), p. 571-576. -
További szerzők:Pitman, Karen T. (orvos) Csongrádi Éva (1969-) (szakorvos) Bain, Justin H. (orvos) Kanyicska Béla (tudományos segédmunkatárs) Fülöp Tibor (1957-) (kardiológus)
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