SAT-820 - Sagliker Syndrome in A Patient with Secondary Hyperparathyroidism

Background: Secondary hyperparathyroidism is characterized by increased PTH secretion in response to chronic hypocalcemic states such as chronic kidney disease. Sagliker syndrome is a rare manifestation of secondary hyperparathyroidism occurring in 0.5% of patients with chronic kidney disease on hemodialysis. Risk factors include patients with a long duration of dialysis and higher PTH levels.

The

Case: The patient is a 40-year-old Filipino male, known case of CKD from chronic glomerulonephritis on hemodialysis. In his 7th year of hemodialysis, he developed craniofacial deformities including a hard palatal mass, widely-spaced teeth, and enlarging mandibular and maxillary bones; as well as skeletal deformities of the hands (shortening of distal phalanges), and axial skeleton (pectus carinatum, kyphosis). These led to speech problems, dysphagia, bone pain, and difficulty in ambulation. Workup revealed a significantly elevated intact PTH of >5000pg/mL (>76-fold elevated), elevated phosphorus and alkaline phosphatase, but ionized calcium within normal range. He was managed as a case of secondary hyperparathyroidism and initially given cinacalcet and sevelamer. Ultrasonography showed parathyroid nodules and an unremarkable thyroid glan.d. Parathyroid scintigraphy (Tc-99m Sestamibi) revealed delayed washout in three foci in the anterior neck suggestive of parathyroid hyperplasia, without any mediastinal uptake to denote ectopic tissue. Subtotal parathyroidectomy with autotransplantation of a fragment of the right superior parathyroid tissue to the right sternocleidomastoid muscle was done. Post-operatively, PTH levels decreased to 1500pg/mL, with a slight improvement in craniofacial deformities and bone pains. However on follow-up, PTH again increased to 3113pg/mL and repeat parathyroid scintigraphy showed avidity in the left inferior thyroid bed. The patient underwent a completion parathyroidectomy with left thyroid lobectomy and post-operatively, PTH decreased significantly to 87.3pg/mL. At 1-month follow-up, PTH remained within the target range (2.4-fold elevated), with the patient reporting an improvement in well-being and no symptoms of bone pain.

Conclusion: Secondary hyperparathyroidism may arise in chronic kidney disease, therefore screening for disorders of bone and calcium metabolism should be done to prevent further complications. Monitoring serum calcium, phosphate, PTH and alkaline phosphatase should begin in all CKD patients at stage G3A. A combination of ultrasonography and scintigraphy provides adequate localization of the parathyroid glands in suspected cases. If medical therapy fails, parathyroidectomy is recommended.

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