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Adrenal Tumor Complicating Untreated 21 -Hydroxylase Deficiency in a 5½-Year-Old Boy-Reply

Vijayalakshmi Bhatia, MBBS; Saroj Kant Mishra, MBBS, MS, DNB; Rakesh Kumar Gupta, MBBS, MD
Arch Pediatr Adolesc Med. 1994;148(9):994-995. doi:10.1001/archpedi.1994.02170090108028.
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We thank Khardori for his interest in our article.1 We agree that surgery for tumors complicating CAH should, as a rule, be reserved for those tumors that are biochemically nonsuppressible using steroid treatment or those documented with radiologic imaging to be enlarging. The latter was the case in our patient.

Second, since size has been strongly correlated with malignancy in adrenal tumors, documentation of a lack of increase in tumor size during follow-up might be of importance in avoiding unnecessary surgery for patients with tumors larger than 6 cm. Since previous studies have reported greater frequency and size of adrenal tumors in untreated patients with CAH compared with treated patients with CAH,2,3 perhaps recommendations for routine imaging may be different in treated patients with classic CAH than in patients who received late treatment.

Third, suppression of testosterone levels has been documented, as mentioned in our report, in addition

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