Final Height in Patients With CAH

Brief Title

Final Height in Patients With CAH

Official Title

Final Height in Patients With Congenital Adrenal Hyperplasia

Brief Summary

      Congenital adrenal hyperplasia (CAH) is the most common inherited disorder in the adrenal
      gland in children. Growth is usually affected in CAH patients either due to the disease
      itself or treatment consequences.
    

Detailed Description

      CAH comprises a group of autosomal recessive disorders caused by a deficiency of one of five
      enzymes needed for the synthesis of cortisol leading to defect in cortisol synthesis with or
      without aldosterone deficiency and an increase in the production of adrenocorticotropic
      hormone through negative feedback.

      The most common form is 21-hydroxylase deficiency (21OHD), which forms more than 90 % of the
      cases.

      In classic CAH, 75% of the patients have the salt wasting (SW) and 25% have the non
      salt-wasting phenotype (NSW).There are no clinical signs at birth in male infants and in
      female patients, CAH is suspected shortly after birth if there is genital ambiguity, ranging
      from slight clitromegaly to complete masculinization with acceleration of growth and pubertal
      development.

      The non-classic (late onset) form of CAH is a less severe form of 21OHD, and is diagnosed
      later in life.

      Final height in early and late onset patients has been reported as diminished (Hauffa et al,
      1997).This could be attributed to androgen excess or treatment with steroids. Androgen excess
      can occur at any age leading to accelerated growth, early epiphyseal closure and compromised
      final adult height.

      Despite that all forms of CAH differ in their degree of enzymatic deficiency, they all
      represent a therapeutic challenge to pediatric endocrinologists attempting to optimize
      growth.
    


Study Type

Observational


Primary Outcome

Final height in patients with congenital adrenal hyperplasia


Condition

Congenital Adrenal Hyperplasia



Publications

* Includes publications given by the data provider as well as publications identified by National Clinical Trials Identifier (NCT ID) in Medline.

Recruitment Information



Estimated Enrollment

30

Start Date

March 11, 2020

Completion Date

September 11, 2020

Primary Completion Date

August 11, 2020

Eligibility Criteria

        Inclusion Criteria:

          -  Patients having a documented history of classical CAH.

        Exclusion Criteria:

          -  Patients with non classical CAH.

          -  Patients treated with growth hormone.

          -  Chronic use of medications unrelated to CAH which may affect growth like
             immunosuppressive drugs like azathioprine and drugs that affect growth hormone release
             like octreotide, pegvisomant, bromocriptine and cabergoline.

          -  Other chronic diseases that may affect growth like heart disease, inflammatory bowel
             disease and renal disease.

          -  Other causes of adrenal insufficiency.
      

Gender

All

Ages

N/A - 18 Years


Contacts

Rana Ahmed, +2001285819500, [email protected]



Administrative Informations


NCT ID

NCT04293133

Organization ID

CAH


Responsible Party

Principal Investigator

Study Sponsor

Ain Shams University


Study Sponsor

Rana Ahmed, Principal Investigator, Ain Shams University


Verification Date

March 2020