Endocrine Abstracts

Case: A 22 year-old female student presented with a history of recurrent pancreatitis since 2007. The most common causes of pancreatitis, including drugs, gallstones, corticosteroids, excess alcohol and hypertriglyceridaemia were excluded. She was noted to have elevated serum calcium, which was considered to be the cause of her pancreatitis, with a detectable serum PTH level. An initial diagnosis of primary hyperparathyroidism was made. However, two neck explorations failed to reveal a parathyroid adenoma. She was referred to us in December 2010 as her episodes of pancreatitis were becoming more frequent and her calcium levels remained persistently elevated. Her investigations were as follows: elevated adjusted calcium 2.79 mmol/L, PTH 4.2 pmol/L (0.6–6.0), low 24 hour urine calcium 0.3 mmol/L, and urine calcium:creatinine ratio <0.003. A repeat ultrasound neck and sestaMIBI scan was negative for a parathyroid adenoma. A clinical diagnosis of familial hypocalciuric hypercalcaemia (FHH) was made, and confirmed on genetic testing which showed a c.1703G>A mutation in the calcium-sensing receptor gene. She was started on cinacalcet 30 mg daily, but had a further episode of pancreatitis with calcium levels ranging between 2.53–2.66 mmol/L. Her cinacalcet was increased to 30 mg twice daily, maintaining her calcium levels between 2.15–2.20 mmol/L. She has not had a further episode of pancreatitis.

Discussion: FHH is usually a benign condition with minimal sequelae from hypercalcaemia. Pancreatitis has been reported rarely, and no clear management strategy has been defined in these cases. Although we have been able to avoid recurrence of pancreatitis by maintaining calcium levels at a low-normal range with cinacalcet, whether this can be an effective long-term treatment remains to be seen.

Conclusion: Cinacalcet was successfully used in treating recurrent pancreatitis in a patient with FHH by maintaining calcium levels in the lower part of the reference range.

Declaration of interest: There is no conflict of interest that could be perceived as prejudicing the impartiality of the research reported.

Funding: No specific grant from any funding agency in the public, commercial or not-for-profit sector.