How nonclassic congenital adrenal hyperplasia can be cured?
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Many patients with NCCAH are asymptomatic and current recommendations argue against treatment for those without symptoms. In symptomatic patients, the signs and symptoms of NCCAH vary with age. As stated, in the newborn period, female infants with NCCAH are not typically born with ambiguity of the external genitalia. Premature development of pubic hair has been reported as early as 6 months and in as many as 60% of persons with NCCAH. Of interest is that in children with premature adrenarche, the incidence of NCCAH is reported to be from 5–30%. It is not clear which patients with NCCAH will advance to have frank hirsutism and acne or have additional signs of androgen excess, such as rapid growth and bone age advancement which is associated with premature adrenarche itself. Advancement of bone age and tall stature in childhood can result in truncated final height due to rapid fusion of epiphyses. Although studies have shown this in both classic CAH and NCCAH,most NCCAH children have normal height for their families and those with truncated final height have had inappropriate early onset or rapid progression of pubarche or bone age. Issues of final height are also related to suppressive effects of glucocorticoid treatment. Adult height approaching expected target height has been reported in patients who adhere to strict monitoring. The current guidelines on CAH suggest treating children with NCCAH who have inappropriately early onset or rapid progression of pubarche or bone age. Further, treatment with GnRH analogs and GH prescription if begun before age 12 has resulted in attainment of target height, as demonstrated by Lin-Su et al. However, such therapy is not routinely recommended by the new CAH guidelines except if the predicted height SD is minus 2.25 or below their target height. Further, if used, these therapies should be considered as experimental treatment approaches and part of formally approved clinical trials.
Happy to help!
Regards,
Adira.
Many patients with NCCAH are asymptomatic and current recommendations argue against treatment for those without symptoms. In symptomatic patients, the signs and symptoms of NCCAH vary with age. As stated, in the newborn period, female infants with NCCAH are not typically born with ambiguity of the external genitalia. Premature development of pubic hair has been reported as early as 6 months and in as many as 60% of persons with NCCAH. Of interest is that in children with premature adrenarche, the incidence of NCCAH is reported to be from 5–30%. It is not clear which patients with NCCAH will advance to have frank hirsutism and acne or have additional signs of androgen excess, such as rapid growth and bone age advancement which is associated with premature adrenarche itself. Advancement of bone age and tall stature in childhood can result in truncated final height due to rapid fusion of epiphyses. Although studies have shown this in both classic CAH and NCCAH,most NCCAH children have normal height for their families and those with truncated final height have had inappropriate early onset or rapid progression of pubarche or bone age. Issues of final height are also related to suppressive effects of glucocorticoid treatment. Adult height approaching expected target height has been reported in patients who adhere to strict monitoring. The current guidelines on CAH suggest treating children with NCCAH who have inappropriately early onset or rapid progression of pubarche or bone age. Further, treatment with GnRH analogs and GH prescription if begun before age 12 has resulted in attainment of target height, as demonstrated by Lin-Su et al. However, such therapy is not routinely recommended by the new CAH guidelines except if the predicted height SD is minus 2.25 or below their target height. Further, if used, these therapies should be considered as experimental treatment approaches and part of formally approved clinical trials.
Happy to help!
Regards,
Adira.
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