Clinical Pathway: Primary Hyperparathyroidism Becky L. Massey MD Assistant Professor

Division of Head and Neck Oncology & Reconstruction

Guidelines

J Clin Endocrinol Metab 2002 87(12):5353-5361.

J Clin Endocrinol Metab 2009, 94(2):366-372.

Abbreviations • • • • • •

HPT: Hyperparathyroidism pHPT: Primary hyperparathyroidism PTH: Parathyroid hormone FHH: Familial Hypocalciuric Hypercalcemia BMD: Bone mineral density DEXA: Dual-energy X-ray absorptiometry

GOALS FOR TODAY – Diagnosis of Primary Hyperparathyroidism – Development of Guidelines – Indications for surgery – Preoperative work-up for surgery – Surgical issues

Primary HPT: Definition & Epidemiology • Primary HPT: Hypercalcemia due to autonomous overproduction of parathyroid hormone. • Incidence: • ~ 1% of adult population • >2 % of population over 55 years old • 2-3x more common in woman

• Primary HPT

Primary HPT: Pathogenesis

– Single adenoma 85% – Hyperplasia 10% – Double adenoma 4%

• Etiology

– Idiopathic – Deficiency in MEN1 tumor suppressor gene – MEN, Familial HPT, HPT-JT

Primary HPT: Diagnosis

• Elevated Ca, normal or elevated PTH • Symptomatic

Easy: Stones, pathologic fracture Difficult: Depression, lethargy, vague aches & pains, fatigue, neurocognitive symptoms

• Asymptomatic

Historical Background: • Before 1960, 50% with stones or osteitis fibrosa cystica • In 2002, 20% stones and 5% osteitis

NIH Guidelines • In 1990, NIH consensus conference and subsequent guidelines re: need for surgery in primary HPT

Annals Int Med 1991

Symptomatic pHPT • Operative management is the only curative therapy for patients with primary HPT • Cure rates >95% • Low morbidity • ? Need to rule out genetic syndrome

Asymptomatic pHPT • Operative management is the only curative therapy for patients with primary HPT • What is asymptomatic? • Risks of waiting? • Can effects be reversed? • Can we predict who will get complications?

Evolution of Guidelines

Also, if medical follow-up is not desirable or suitable

Are guidelines too conservative? • Known risk of decreased bone density, stones • Low level evidence of improvement in neurocognitive symptoms, cardiovascular risk and survival with parathyroidectomy Decrease in hypersomnolence Perrier et al. Surgery 2009

Does Meeting NIH criteria predict different outcome?

Case A.B. • 52 yo woman – Calcium 10.8 (H) – PTH 150 (H) – No known kidney stones, no prior pathologic fractures

S1. Should this patient be sent for surgical consultation? A. Yes B. No

0% A.

0% B.

STATEMENT 1

ALL PATIENTS WITH PRIMARY HPT SHOULD BE REFERRED TO A PARATHYROID SURGEON

• 2008 Guidelines • Symptomatic patients “referred for surgery” • Asymptomatic patients “referred for consultation”

S2. What additional labs do you want on this patient? A. Ionized Calcium B. PTH C. Vit D D. None

0% A.

0%

0%

B.

C.

0% D.

STATEMENT 2

PRIMARY HPT PATIENTS WITH LOW VIT D 25OH SHOULD BE ON VIT D & CALCIUM REPLACEMENT

• 2008 Guidelines – Check Vit D 25 OH in all PHPT patients – Replace if < 20 mg/ml – Continue calcium replacement – Independent of plan to perform surgery

Who requires more workup? • • • • • •

Family history Age < 30 Other related tumors, symptoms Really high calcium Normocalcemic Primary HPT Really low urine calcium

Case Report AB • 24 hour urine calcium= 350 (H) • What if the urine calcium low?

Exclusion of Nonsurgical Disease: Familial Hypocalciuric Hypercalcemia • Inactivating mutation of the calcium sensing receptor • Urine calcium is low • Calcium-creatinine clearance ratio • 24 H Ca/serum Ca x Serum Cr/24 H Ucr=