Normocalcemic primary hyperparathyroidism: long-term follow-up associated with multiple adenomas

Abstract

SUMMARYNormocalcemic primary hyperparathyroidism (NPHPT) is a condition characterized by elevation of the parathyroid hormone (PTH) in the presence of normal serum calcium and the absence of secondary causes. The case described illustrates the long-term follow-up of a postmenopausal woman with NPHPT patient who progressed with multiple adenomas. This case reports a 77-year-old female who has chronic generalized pain and osteoporosis. Her initial serum PTH was 105 pg/mL, with total serum calcium of 9.6 mg/dL, albumin 4.79 g/dL, phosphorus 2.8 mg/dL, and 25OHD after supplementation was 34.6 ng/mL. The bone densitometry (BMD) results were as follows: lumbar spine: T-score -3.0, femoral neck: T-score -2.6 and distal radius: -4.2. Other causes of secondary hyperparathyroidism were ruled out and cervical ultrasound and Tc-99-Sestamibi scan were negative. She used oral alendronate and three infusions of zoledronic acid for treatment of osteoporosis. In the 10 th year of follow-up, after successive negative cervical imaging, ultrasound showed a nodule suggestive of an enlarged right inferior parathyroid gland. PTH levels in fluid which was obtained during fine-needle aspiration (FNA) were over 5,000 pg/mL and a Sestamibi scan was negative. The patient underwent parathyroidectomy, and a histological examination confirmed parathyroid adenoma. Post-operatively serum PTH remained elevated in the presence of normal serum calcium levels. A follow-up cervical ultrasound showed a new solid nodule suggestive of an enlarged right superior parathyroid gland. PTH levels in the aspiration fluid were remarkably high. A second parathyroidectomy was performed, with the excision of a histologically confirmed parathyroid adenoma. In conclusion, this is an unusual presentation of NPHPT and highlights the long-term complications. Arq Bras Endocrinol Metab. 2014;58(5):583-6 SUMáRioHiperparatiroidismo primário normocalcêmico (NPHPT) caracteriza-se pela elevação do hormô-nio da paratiroide (PTH), na ausência da elevação dos níveis séricos de cálcio e exclusão de causas secundárias. O caso descrito ilustra o seguimento de uma mulher na pós-menopausa com NPHPT que evoluiu com múltiplos adenomas. Este caso relata uma paciente de 77 anos de idade que tem dor generalizada crônica e osteoporose. O PTH inicial foi elevado com níveis séricos de cálcio, albumina, fósforo e 25OH vitamina D normais. A densitometria óssea (DMO) evidenciou um T-SCORE da coluna lombar: -3.0, colo do fêmur: -2.6 e rádio distal: -4.2. Outras causas de hiperparatireoidismo secundário foram descartadas e a ultrassonografia cervical e varredura com Sestamibi foram negativos. Fez uso de alendronato e três infusões de ácido zoledrônico para o tratamento da osteoporose. No décimo ano de seguimento, depois de sucessivas imagens negativas, a ultrassonografia evidenciou um nódulo sugestivo de adenoma de paratireoide inferior direita. A paciente foi submetida à paratireoidectomia, e um exame histológico confirmou a hipótese. A elevação dos níveis séricos de PTH no pós-operatório se ma...

Hyperparathyroidism and Bone Health

“…Normocalcemic primary hyperparathyroidism (NPHPT) is characterized by persistently elevated PTH levels, normal calcium levels, and the absence of identifiable causes of secondary HPT [3,43]. This condition has been increasingly diagnosed, being detected particularly during the evaluation of patients with decreased bone mass.…”

Elevated PTH with normal serum calcium level: a structured approach

“…Primary hyperparathyroidism (PHPT) is defined as hypercalcaemia in the presence of high or inappropriately normal parathyroid hormone; there is increasing recognition that a number of patients have a variant with normocalcaemia that could be considered to be a subclinical phase of PHPT . The challenge for the managing clinician is to identify and confirm the biochemical abnormality, exclude other causes of hyperparathyroidism, which are more common and include vitamin D deficiency and hyperparathyroidism secondary to chronic kidney disease (Table ), and assess the clinical context to make an appropriate management plan for the patient.…”

“…In this brief review we describe the assessment of a patient with normocalcaemic hyperparathyroidism.Primary hyperparathyroidism (PHPT) is defined as hypercalcaemia in the presence of high or inappropriately normal parathyroid hormone 1,2 ; there is increasing recognition that a number of patients have a variant with normocalcaemia that could be considered to be a subclinical phase of PHPT. 1,3,4 The challenge for the managing clinician is to identify and confirm the biochemical abnormality, exclude other causes of hyperparathyroidism, which are more common and include vitamin D deficiency and hyperparathyroidism secondary to chronic kidney disease (Table 1), and assess the clinical context to make an appropriate management plan for the patient.Normocalcaemic hyperparathyroidism may be secondary to vitamin D deficiency. It is unlikely that elevated PTH would be secondary to vitamin D deficiency alone if 25OHD is greater than 50 nmol/l, at which level the bone health requirements of 97Á5%…”

Effects of Parathyroidectomy on Normocalcemic Primary Hyperparathyroidism and the Role of Intraoperative PTH Measurement

“…1 80-85% are due to a solitary parathyroid adenoma. 2 Recently recognized variants include normocalcemic pHPT (NCpHPT) and normohormonal pHPT (NHpHPT) which together account for 5% to 28% of pHPT cases. 3 The pathophysiology and natural history of NCpHPT are not clear.…”