Author’s Response: The Apparent Paradox of Tall Stature with Hypopituitarism: New Insights from an Old Story

D. T. den Ouden, M. Kroon, P. H. Hoogland, P. H. L. M. Geelhoed-Duijvestijn and J. M. Wit

Leiden University Hospital, Department of Internal Medicine, Leiden, The Netherlands 2300 RC

Address correspondence to: Dr. Danielle T. den Ouden, Leiden University Hospital, Department of Internal Medicine, C1/R P.O. Box 9600, Leiden, The Netherlands 2300 RC. E-mail: denouden99{at}hotmail.com.

To the editor:

The letter of Dr. Faustini-Fustini and colleagues (1) in response to our recent article (2) in JCEM makes three interesting points. First, some additional cases with similar phenotypes are mentioned, which illustrates that the pattern of continuing growth in adulthood—in some cases resulting in gigantism—in panhypopituitary patients may be not as rare as has been previously believed. Second, an additional hypothesis is put forward to explain why some untreated patients with multiple pituitary deficiency continue to grow, whereas others do not. In addition to variability in insulin secretion and sensitivity, and sex steroid sensitivity, the authors suggest that there may also be an interindividual variation of local aromatase activity and, thus, local availability of estrogen. We agree that, indeed, this is a plausible hypothesis; however, it will be virtually impossible to prove or disprove any of these hypotheses. The third point is the unexpected finding of a low bone mineral density, despite the known effects of sex steroid deficiency on bone mineral density. We believe that the patient’s recent humerus fracture and the thoracic kyphoscoliosis indicate a decreased bone strength, whereas the normal bone mineral density indicates a normal amount of calcified bone. We hypothesize that this apparent contradiction can be explained by a slow bone turnover and remodeling that has resulted in a bone with an apparently normal bone mineral density but with a decreased strength. This is consistent with the observations that, in patients with GH deficiency, bone mineral density is not far below the mean for age when corrected for body size (3), while both osteoclastic and osteoblastic activity are decreased (4, 5). The findings in our patient may suggest that sex hormone deficiency may only result in low bone density in the presence of a normal GH secretion.

Received May 13, 2003.

References

  1. Faustini-Fustini M, Balestrieri A, Rochira V, Carani C 2003 The apparent paradox of tall stature with hypopituitarism: new insights from an old story. J Clin Endocrinol Metab 88:4002 (Letters)[Free Full Text]
  2. Den Ouden DT, Kroon M, Hoogland PH, Geelhoed-Duijvestijn PHLM, Wit JM 2002 A 43-year-old male with untreated panhypopituitarism due to absence of the pituitary stalk: from dwarf to giant. J Clin Endocrinol Metab 87:5430–5434[Abstract/Free Full Text]
  3. de Boer H, Blok GJ, van Lingen A, Teule GJ, Lips P, van der Veen EA 1994 Consequences of childhood-onset growth hormone deficiency for adult bone mass. J Bone Miner Res 9:1319–26[Medline]
  4. Bravenboer N, Holzmann P, de Boer H, Blok GJ, Lips P 1996 Histomorphometric analysis of bone mass and bone metabolism in growth hormone deficient adult men. Bone 18:551–557[CrossRef][Medline]
  5. Bravenboer N, Holzmann P, de Boer H, Roos JC, van der Veen EA, Lips P 1997 The effect of growth hormone (GH) on histomorphometric indices of bone structure and bone turnover in GH-deficient men. J Clin Endocrinol Metab 82:1818–22[Abstract/Free Full Text]




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