What is the best approach to the teenage patient presenting with nonclassical Congenital adrenal hyperplasia: should we always treat with glucocorticoids?

@article{Matthews2013WhatIT,
  title={What is the best approach to the teenage patient presenting with nonclassical Congenital adrenal hyperplasia: should we always treat with glucocorticoids?},
  author={Deborah Matthews and Tim D. Cheetham},
  journal={Clinical Endocrinology},
  year={2013},
  volume={78}
}
Patients with Congenital adrenal hyperplasia due to partial deficiency in the enzyme 21‐hydroxlyase can present in childhood or adolescence with signs of adrenal androgen excess. Strategies to reduce the impact of androgen excess in females include cosmetic measures as well as antiandrogens and agents such as the combined oral contraceptive pill. Glucocorticoid may not be appropriate straightaway but can be introduced if other measures are ineffective or when pregnancy is planned. 
Congenital Adrenal Hyperplasia Due to Steroid 21-Hydroxylase Deficiency: An Endocrine Society Clinical Practice Guideline.
Objective To update the congenital adrenal hyperplasia due to steroid 21-hydroxylase deficiency clinical practice guideline published by the Endocrine Society in 2010. Conclusions The writing
Substitution therapy in adult patients with congenital adrenal hyperplasia.
  • N. Reisch
  • Medicine, Biology
    Best practice & research. Clinical endocrinology & metabolism
  • 2015
Diagnosis and Treatment of Congenital Adrenal Cortex Hyperplasia
TLDR
The etiology, clinical manifestation, diagnosis, treatment and monitoring indicators ofCongenital adrenal hyperplasia are summarized in order to improve the clinical physicians’ awareness of the disease, reduce misdiagnosis, and improve the prognosis.
Endocrine disorders and hormonal therapy for adolescent acne
TLDR
Hormonal therapies are effective and well tolerated options for the treatment of acne vulgaris in adolescents with and without endocrine disorders and can be used as monotherapy or in conjunction with benzoyl peroxide, topical retinoic acid, or antibiotics.
17-Hydroxyprogesterone in children, adolescents and adults
  • J. Honour
  • Medicine, Biology
    Annals of clinical biochemistry
  • 2014
TLDR
17-OHP is a diagnostic marker for CAH but opinions differ on the role of 17OHP or androstenedione in monitoring treatment with renin in the salt losing form and the utility of 17- OHP measurements in children, adolescents and adults is considered.
Ultralow-dose Dexamethasone to Preserve Endogenous Cortisol Stress Response in Nonclassical Congenital Adrenal Hyperplasia: A New Promising Treatment
TLDR
It seems that ultralow-dose dexamethasone in treatment of nonclassical CAH would be a promising novel treatment strategy that adverse effects of hyperandrogenism can be reversed while preserving the endogenous cortisol stress response.
Ultralow-dose Dexamethasone to Preserve Endogenous Cortisol Stress Response in Nonclassical Congenital Adrenal Hyperplasia: A New Promising Treatment.
TLDR
It seems that ultralow-dose dexamethasone in treatment of nonclassical CAH would be a promising novel treatment strategy that adverse effects of hyperandrogenism can be reversed while preserving the endogenous cortisol stress response.
Inadequate Cortisol Response to the Tetracosactide (Synacthen®) Test in Non-Classic Congenital Adrenal Hyperplasia: An Exception to the Rule?
TLDR
The cortisol response to tetracosactide was inadequate in 60% of patients with NCCAH, suggesting hydrocortisone therapy may deserve consideration when major stress (surgery, trauma, childbirth) or objectively documented fatigue occurs in NCC AH patients with inadequate cortisol response.
Nichtklassisches adrenogenitales Syndrom
TLDR
Adrenogenital syndrome is an etiologically and clinically heterogeneous pattern of symptoms, which have a disorder in the biosynthesis of cortisol in common, the most commonly occurring disorder is a defect in 21-hydroxylase.
...
...

References

SHOWING 1-10 OF 29 REFERENCES
Approach to the adult with congenital adrenal hyperplasia due to 21-hydroxylase deficiency.
  • D. Merke
  • Medicine, Biology
    The Journal of clinical endocrinology and metabolism
  • 2008
TLDR
Treatment of CAH, especially in the adult patient, remains controversial given the lack of prospective randomized controlled trials comparing treatment regimens, Nevertheless, patients benefit from careful individualized therapy with avoidance of Cushingoid side effects and optimization of reproductive, sexual, and bone health.
Hirsutism of adrenal origin in adolescents: consequences in adults.
  • F. Keleştimur
  • Medicine, Biology
    Journal of pediatric endocrinology & metabolism : JPEM
  • 2001
TLDR
The most common adrenal cause of hirsutism is non-classical congenital adrenal hyperplasia (NC-CAH), the pathophysiology of the hirsUTism in NC- CAH, and the treatment of hirutism in these patients are discussed.
Fertility in women with nonclassical congenital adrenal hyperplasia due to 21-hydroxylase deficiency.
TLDR
Subfertility is mild in NC-CAH, however, the rate of miscarriages is lower in pregnancies occurring with glucocorticoid treatment and argues for treating NC- CAH women wanting pregnancy.
Future Directions in the Study and Management of Congenital Adrenal Hyperplasia due to 21-Hydroxylase Deficiency
TLDR
Congenital adrenal hyperplasia due to 21-hydroxylase deficiency is one of the most common known autosomal recessive disorders and has a high frequency, a variable presentation in children and adults, and potential complications, a thorough understanding of the disorder is of great importance to clinicians working in internal medicine, reproductive medicine, and pediatrics.
Precocious pubarche: distinguishing late-onset congenital adrenal hyperplasia from premature adrenarche.
TLDR
A selective strategy based on a 2-ng/ml basal 17-OHP plasma level threshold would have safely avoided 99% of the unnecessary ACTH tests among patients presenting with PP.
Absence of Clinically Relevant Growth Acceleration in Untreated Children with Non-Classical Congenital Adrenal Hyperplasia
TLDR
In untreated NC-CAH children, growth acceleration is small and generally not visible on their growth charts, therefore, the absence of an increase in growth velocity does not exclude the diagnosis of NC- CAH.
Endocrinology and auxology of sibships with non-classical congenital adrenal hyperplasia.
TLDR
Patients with NCCAH do not appear to be at risk of short adult stature despite increased bone age in childhood and should have stimulated 17-OHP levels measured to exclude the disease, according to their unaffected siblings.
Health Status of Adults with Congenital Adrenal Hyperplasia: A Cohort Study of 203 Patients
TLDR
In the patients studied, glucocorticoid replacement was generally nonphysiological, and androgen levels were poorly controlled, associated with an adverse metabolic profile and impaired fertility and quality of life.
Clinical and molecular characterization of a cohort of 161 unrelated women with nonclassical congenital adrenal hyperplasia due to 21-hydroxylase deficiency and 330 family members.
TLDR
The study of family members underlines the variable expression of NC-CAH even within a family, suggesting that modifier factors may modulate phenotype expression.
Incidence and clinical features of congenital adrenal hyperplasia in Great Britain
TLDR
One child in every 18 000 born in Great Britain has CAH, with similar numbers of boys and girls present clinically in the first year of life, but boys present with more severe manifestations, such as salt-wasting crises.
...
...