365- How to differentiate between TART and Leydig cell tumor?
TART and Leydig cell tumor mimics each other, and failure to consider a diag-
nosis of TART in a male with CAH having testicular mass may result in inad-
vertent orchidectomy. The important differences between these disorders are
summarized in the table given below.Parameters TA RT Leydig cell tumor
Presentation Infertility Gynecomastia
Cell of origin Adrenal rest cell located in testes Leydig cells
Laterality Bilateral >80 % Bilateral in only 3 %
Reinke crystalloids Absent Present in 35–40 %
Malignant potential Absent Present (10 %)
Treatment Glucocorticoids in initial stages Surgery
Surgery in later stages- What are the causes of decreased growth velocity in a child with CAH on
treatment?
Untreated children with CAH initially have accelerated growth velocity
and subsequently premature epiphyseal closure results in short final adult
height. Optimal therapy with glucocorticoids results in suppression of
ACTH- mediated adrenal androgen production and, consequently, normal-
ization of growth velocity. The most common cause of reduced growth
velocity in a child with CAH on therapy is overzealous treatment with glu-
cocorticoids. This commonly occurs when therapy is aimed to maintain
serum 17(OH)P in the normal range rather than in the recommended range
(4–12 ng/ml). This is because the dose of glucocorticoids required to main-
tain serum 17(OH)P in the recommended range is lower than the dose
which have adverse effects on epiphyseal growth plate. In addition, aggres-
sive treatment with GnRH analogue in patients of CAH with GDPP can
also result in suboptimal growth velocity. Further, children with CAH with
markedly advanced bone age at presentation also have decreased growth
velocity despite optimal treatment.- How to optimize the growth potential in a child with classical CAH?
To maximize fi nal adult height in a child with classical CAH, optimal therapy
with glucocorticoids and mineralocorticoids should be ensured targeting 17(OH)
P, androstenedione, and testosterone in the defi ned range. However, even with
optimal therapy, fi nal adult height is compromised by −1 to −2SD as these chil-
dren experience phases of hyperandrogenemia and glucocorticoid excess. The
various strategies which have been tried to promote height potential in these
children include the use of recombinant growth hormone (rhGH), the combined
use of GnRH agonists with rhGH, or a combination of fl utamide and testolac-10 Congenital Adrenal Hyperplasia