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Osteoporosis International

Erschienen in:

01.03.2014 | Original Article

Total parathyroidectomy with autotransplantation for a rare disease derived from uremic secondary hyperparathyroidism, the uremic leontiasis ossea

verfasst von: G. Yang, B. Zhang, X-M. Zha, N-N. Wang, C-Y. Xing

Erschienen in: Osteoporosis International | Ausgabe 3/2014

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Abstract

Summary

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.

Introduction

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.

Lee VS, Webb MS, Martinez S et al (1996) Uremic leontiasis ossea: “Bighead” disease in humans? (Radiologic, clinical, and pathologic features). Radiology 199:233–240PubMed

Byers PD, Jones AN (1969) Leontiasis ossea. Br J Surg 56:262–267PubMedCrossRef

Windholz F, Cutting WC (1945) Leontiasis ossea. Stanford Med Bull 3:69–72

Cohen J, Diamond I (1953) Leontiasis ossea, slipped epiphyses, and granulosa cell tumor of testis with renal disease: report of a case with autopsy findings. AMA Arch Pathol 56:488–500PubMed

Chang JI, Som PM, Lawson W (2007) Unique imaging findings in the facial bones of renal osteodystrophy. Am J Neuroradiol 28:608–609PubMed

Reidy JM, Motamedi K, Berens D et al (1998) Renal osteodystrophy with leontiasis ossea deformity. Otolaryngol Head Neck Surg 119:525–527PubMedCrossRef

Aggunlu L, Akpek S, Coskun B (2004) Leontiasis ossea in a patient with hyperparathyroidism secondary to chronic renal failure. Pediatr Radiol 34:630–632PubMedCrossRef

Antonetli JR, Hottel TL (2003) Oral manifestations of renal osteodystrophy: case report and review of the literature. Spec Care Dent 23:28–34CrossRef

Barsić N, Cala K, Pavlović D (2010) Brown tumor—a rare manifestation of renal osteodystrophy and severe secondary hyperparathyroidism: case report. Acta Clin Croat 49:299–304PubMed

10.

Kar DK, Gupta SK, Agarwal A et al (2001) Brown tumor of the palate and mandible in association with primary hyperparathyroidism. J Oral Maxillofac Surg 59:1352–1354PubMedCrossRef

11.

Dantas M, Costa RS, Jorgetti V et al (1991) Facial leontiasis ossea: a rare presentation of hyperparathyroidism secondary to chronic renal insufficiency. Nephron 58:475–478PubMedCrossRef

12.

Zhang L, Yao L, Bian WJ et al (2009) Severe uremic leontiasis ossea ameliorated by total parathyroidectomy. Kidney Int 76:1118PubMedCrossRef

13.

Collum J, Jones RH, Lynham A et al (2013) Leontiasis ossea: a presentation of hyperparathyroidism in an indigenous Australian mansecondary to chronic renal failure. J Oral Maxillofac Surg 71:56–61PubMedCrossRef

14.

Kumar S, Thuraisingham R, Yaqoob M (2006) Big-head disease: uremic leontiasis ossea. Kidney Int 69:1709PubMedCrossRef

15.

Sagliker Y, Balal M, Sagliker Ozkaynak P et al (2004) Sagliker syndrome: uglifying human face appearance in late and severe secondary hyperparathyroidism in chronic renal failure. Semin Nephrol 24:449–455PubMedCrossRef

16.

Yildiz I, Sagliker Y, Demirhan O et al (2012) International evaluation of unrecognizably uglifying human faces in late and severe secondary hyperparathyroidism in chronic kidney disease. Sagliker syndrome. A unique catastrophic entity, cytogenetic studies for chromosomal abnormalities, calcium-sensing receptor gene and GNAS1 mutations. Striking and promising missense mutations on the GNAS1 gene exons 1, 4, 10, 4. J Ren Nutr 22:157–161PubMedCrossRef

17.

Zhang L, Yao L, Hua Z et al (2011) Total parathyroidectomy in treatment of Sagliker syndrome in 10 cases of hemodialysing patients with secondary hyperparathyroidism. Zhonghua Nei Ke Za Zhi 50:562–567PubMed

18.

Hogan DB, Couture RA, Levine DZ (1982) Leontiasis ossea, flail chest and pancytopenia in a patient with renal osteodystrophy. Can Med Assoc J 127:1012–1013PubMedCentralPubMed

19.

Gelman R, Gellad FE (1991) Brown tumor of the facial bones. Am J Neuroradiol 12:1179–1181PubMed

20.

Rao P, Solomon M, Avramides A et al (1978) Brown tumors associated with secondary hyperparathyroidism of chronic renal failure. J Oral Surg 36:154–159PubMed

21.

Maxwell DR, Spolnik KJ, Cockerill EM et al (1985) Roentgenographic manifestations of maxillomandibular renal osteodystrophy. Nephron 41:223–229PubMedCrossRef

22.

Bringhurst FR, Demay MB, Kronenberg HM (2003) Hormones and disorders of mineral metabolism. In: Larsen PR, Kronenberg HM, Melmed S (eds) Williams textbook of endocrinology, 10th edn. Elsevier, Philadelphia, pp 1323–1324

23.

Rosenberg AE (2005) Bones, joints, and soft tissue tumours. In: Kumar V, Fausto AK, Robbins N (eds) Robbins and Cotran pathologic basis of disease, 7th edn. Elsevier, Philadelphia, pp 1287–1288

24.

Lacativa PG, Franco FM, Pimentel JR et al (2009) Prevalence of radiological findings among cases of severe secondary hyperparathyroidism. Sao Paulo Med J 127:71–77PubMed

25.

Pfeifle K, Koch H, Grabensee B et al (1974) Monstrous tumorous changes of the facial bones in secondary hyperparathyroidism. Dtsch Med Wochenschr 99:389–396PubMedCrossRef

26.

Falko JM, Bush CA, Tzagournis M et al (1976) Case report. Congestive heart failure complicating the hungry bone syndrome. Am J Med Sci 271:85–89PubMedCrossRef

27.

Ahuja MM, Chopra IJ (1968) Coexistent hyperthyroidism and hyperparathyroidism. Metabolism 17:854–866PubMedCrossRef

28.

Davies DR, Friedman M (1966) Complications after parathyroidectomy. Fractures from low calcium and magnesium convulsions. J Bone Joint Surg Br 48:117–126PubMed

29.

Varthakavi P, Thayil G, Amin S et al (1985) Hypocalcaemic congestive heart failure—a postparathyroidectomy complication. Indian Heart J 37:311–314PubMed

30.

Hu JM, Wu HF, Wang XY et al (2006) Efficiency of total parathyroidectomy and autotransplantation in 31 patients with uremic hyperparathyroidism. Zhonghua Nei Ke Za Zhi 45:714–716PubMed

31.

Schneider R, Slater EP, Karakas E et al (2012) Initial parathyroid surgery in 606 patients with renal hyperparathyroidism. World J Surg 36:318–326PubMedCrossRef

32.

Puccini M, Carpi A, Cupisti A et al (2010) Total parathyroidectomy without autotransplantation for the treatment of secondary hyperparathyroidism associated with chronic kidney disease: clinical and laboratory long-term follow-up. Biomed Pharmacother 64:359–62PubMedCrossRef

33.

Sadideen HM, Taylor JD, Goldsmith DJ (2012) Total parathyroidectomy without autotransplantation after renal transplantation for tertiary hyperparathyroidism: long-term follow-up. Int Urol Nephrol 44:275–281PubMedCrossRef

Titel: Total parathyroidectomy with autotransplantation for a rare disease derived from uremic secondary hyperparathyroidism, the uremic leontiasis ossea
verfasst von: G. Yang
B. Zhang
X-M. Zha
N-N. Wang
C-Y. Xing
Publikationsdatum: 01.03.2014
Verlag: Springer London
Erschienen in: Osteoporosis International / Ausgabe 3/2014
Print ISSN: 0937-941X
Elektronische ISSN: 1433-2965
DOI: https://doi.org/10.1007/s00198-013-2488-1

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Abstract

Summary

Introduction

Methods

Results

Conclusions

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