title: Total parathyroidectomy with autotransplantation for a rare disease derived from uremic secondary hyperparathyroidism, the uremic leontiasis ossea.
abstract: CONCLUSIONS
  We described six uremic leontiasis ossea (ULO) patients who underwent total parathyroidectomy with autotransplantation. ULO demonstrated more a systemic disease than a simple craniofacial deformation. The surgery seemed an effective treatment to alleviate secondary hyperparathyroidism and to improve patients' quality of life. ULO may have a high postoperative recurrence tendency.
  
  
  BACKGROUND
  ULO is a rare disease derived from uremic secondary hyperparathyroidism (SHPT). Previous studies mostly focused on the craniofacial deformations. This study aims to investigate the systemic features of the disease and the surgical outcomes.
  
  
  METHODS
  The present study retrospectively assessed six ULO patients who underwent total parathyroidectomy (TPTX) with autotransplantation (AT). Follow-up data were recorded. The follow-up status was considered as "effectiveness" if serum intact parathyroid hormone (iPTH) levels were <150 pg/mL in the first 3 days after surgery, or as "recurrence" if serum iPTH gradually increased >300 pg/mL during follow-up in patients whose status was initially considered as "effectiveness".
  
  
  RESULTS
  Craniofacial deformations, short stature, thoracocyllosis, spine malformations, osteodynia, and muscle weakness were observed in all patients. Abnormal pulmonary functions were observed in five patients. After surgery, one patient died from respiratory failure. Surgery was effective in the remaining five patients with relieved osteodynia and stopped craniofacial deformation. A mean follow-up of 7.6 (4 to 12) months was available. Three patients suffered from recurrence of hyperparathyroidism originating from autografts.
  
  
  CONCLUSIONS
  Our data suggests that ULO is not only a simple disease with craniofacial malformations but is a severe systemic disease leading to increased surgical risks. TPTX with AT seems an effective treatment to relieve SHPT and to improve quality of life. ULO may have a high postoperative recurrence tendency.
authors.affiliation: Department of Nephrology, First Affiliated Hospital of Nanjing Medical University, 300# Guangzhou Road, Nanjing, Jiangsu, 210029, China.
authors.forename: G
authors.initials: G
authors.lastname: Yang
authors.forename: B
authors.initials: B
authors.lastname: Zhang
authors.forename: X-M
authors.initials: XM
authors.lastname: Zha
authors.forename: N-N
authors.initials: NN
authors.lastname: Wang
authors.forename: C-Y
authors.initials: CY
authors.lastname: Xing
mesh_terms: D015502:Absorptiometry, Photon / Q000379:methods; D000328:Adult; D015519:Bone Density / Q000502:physiology; D005260:Female; D006801:Humans; D006957:Hyperostosis Frontalis Interna / Q000000981:diagnostic imaging / Q000209:etiology / Q000503:physiopathology / Q000601:surgery*; D006962:Hyperparathyroidism, Secondary / Q000150:complications* / Q000503:physiopathology; D008297:Male; D008875:Middle Aged; D010280:Parathyroid Glands / Q000637:transplantation; D016105:Parathyroidectomy / Q000379:methods*; D019990:Perioperative Care / Q000379:methods; D012189:Retrospective Studies; D014182:Transplantation, Autologous; D016896:Treatment Outcome; D014511:Uremia / Q000150:complications* / Q000503:physiopathology