Correspondence

Case 16-2008: A Woman with Bone Pain

N Engl J Med 2008; 359:1183-1184September 11, 2008

Article

To the Editor:

The diagnosis of secondary hyperparathyroidism in the case discussed by Demay and colleagues (May 22 issue)1 is doubtful. Primary hyperparathyroidism with coexisting vitamin D deficiency presents with normocalcemia and hypophosphatemia.2 Hence, a normal calcium level with a low phosphorus level is not diagnostic of secondary hyperparathyroidism. The features of bone pain and fatigue, brown tumor, lytic bone lesions, and a single parathyroid adenoma are all suggestive of primary hyperparathyroidism. The markedly elevated level of parathyroid hormone suggests a secondary cause but does not rule out primary hyperparathyroidism3; a markedly decreased level of the calcium-sensing receptor is seen in secondary hyperparathyroidism but not in sporadic adenomas.4 We believe that detection of calcium-receptor protein or technetium-99m–labeled sestamibi imaging would help in establishing the underlying diagnosis.5

There is a higher prevalence of primary hyperparathyroidism than secondary hyperparathyroidism among patients with celiac disease. This condition usually presents with normocalcemic hyperparathyroidism, as in the described case.6

Hari K.V.S. Kumar, M.D.
Muthukrishnan Jayaraman, M.D.
Kirtikumar D. Modi, M.D., D.M.
Medwin Hospitals, Hyderabad 500001, India
hariendo@rediffmail.com

6 References

1. 1

   Case Records of the Massachusetts General Hospital (Case 16-2008). N Engl J Med 2008;358:2266-2274
   Full Text | Web of Science | Medline

2. 2

   Silverberg SJ. Vitamin D deficiency and primary hyperparathyroidism. J Bone Miner Res 2007;22:Suppl 2:V100-V104
   CrossRef | Web of Science | Medline

3. 3

   Alzahrani AS, Al Sheef M. Severe primary hyperparathyroidism masked by asymptomatic celiac disease. Endocr Pract 2008;14:347-350
   Medline

4. 4

   Yano S, Sugimoto T, Tsukamoto T, et al. Association of decreased calcium-sensing receptor expression with proliferation of parathyroid cells in secondary hyperparathyroidism. Kidney Int 2000;58:1980-1986
   CrossRef | Web of Science | Medline

5. 5

   Kasai ET, da Silva JW, Mandarim de Lacerda CA, Boasquevisque E. Parathyroid glands: combination of sestamibi-(99m)Tc scintigraphy and ultrasonography for demonstration of hyperplasic parathyroid glands. Rev Esp Med Nucl 2008;27:8-12
   CrossRef | Web of Science | Medline

6. 6

   Maida MJ, Praveen E, Crimmins SR, Swift GL. Coeliac disease and primary hyperparathyroidism: an association? Postgrad Med J 2006;82:833-835
   CrossRef | Web of Science | Medline

To the Editor:

The discussant in the Case Record of a woman with bone pain thought that the patient had osteitis fibrosa cystica due to secondary hyperparathyroidism. He asserts that vitamin D deficiency may have complicated the case because of malabsorption owing to celiac disease. However, in most people, the main source of vitamin D is sunlight exposure.1,2

Christopher Ish, M.D.
Johns Hopkins Community Physicians, Odenton, MD 21113
cish1@jhmi.edu

2 References

1. 1

   Office of Dietary Supplements. Sources of vitamin D. (Accessed August 22, 2008, at http://dietary-supplements.info.nih.gov/factsheets/vitamind.asp#h3.)
   

2. 2

   Cannell JJ, Hollis BW, Zasloff M, Heaney RP. Diagnosis and treatment of vitamin D deficiency. Expert Opin Pharmacother 2008;9:107-118
   CrossRef | Web of Science | Medline

Author/Editor Response

These letters raise the question of whether the patient described in the Case Record had secondary or primary hyperparathyroidism. I agree that primary hyperparathyroidism with coexisting vitamin D deficiency can present with normocalcemia and hypophosphatemia. However, under these circumstances, the diagnosis of autonomous parathyroid hyperfunction, which characterizes primary hyperparathyroidism, cannot be made until underlying disorders that cause secondary hyperparathyroidism have been treated or until hypercalcemia with an inappropriately elevated level of parathyroid hormone is detected.

The treatment of vitamin D deficiency may reveal parathyroid autonomy, as indicated in the Discussion of Management section of the case. Unfortunately, since the patient was lost to follow-up, it could not be determined whether she had underlying parathyroid autonomy in addition to secondary hyperparathyroidism from vitamin D deficiency. Kumar and colleagues cite the report by Kasai et al. on the use of technetium-99m–labeled sestamibi imaging for the evaluation of secondary hyperparathyroidism in a population of patients with chronic kidney disease. This type of imaging has not been validated in patients with parathyroid hyperplasia who have normal renal function.

Ish points out that an important source of vitamin D is sun exposure. Although intentional exposure to ultraviolet radiation has been used to treat nutritional rickets, a recent meta-analysis showed widespread vitamin D insufficiency in the general population.1 Thus, baseline levels of sun exposure are insufficient to maintain normal vitamin D status; this underscores the critical importance of dietary sources of vitamin D.

Marie B. Demay, M.D.
Massachusetts General Hospital, Boston, MA 02114

1 References

1. 1

   Hagenau T, Vest R, Gissel TN, et al. Global vitamin D levels in relation to age, gender, skin pigmentation and latitude: an ecologic meta-regression analysis. Osteporos Int 2008 May 6 (Epub ahead of print).
   

Citing Articles (1)

Citing Articles

1. 1

   Sheng Chin Wu, Sergio Caravita, Maria Beatrice Secchi. (2011) Hyperparathyroidism in celiac disease: always secondary?. Internal and Emergency Medicine
   CrossRef