Home | About us | Editorial board | Search | Ahead of print | Current issue | Archives | Submit article | Instructions | Subscribe | Contacts | Advertise | Login 
 
Search Article 
  
Advanced search 
  Users Online: 470 Home Print this page Email this page Small font sizeDefault font sizeIncrease font size  

 
Table of Contents
LETTERS TO THE EDITOR
Year : 2018  |  Volume : 22  |  Issue : 6  |  Page : 858-859

Central precocious puberty complicating congenital adrenal hyperplasia: North Indian experience


1 Department of Pediatrics, Pediatric Endocrinology Unit, Postgraduate Institute of Medical Education and Research, Chandigarh, India
2 Department of Endocrinology, Postgraduate Institute of Medical Education and Research, Chandigarh, India

Date of Web Publication4-Dec-2018

Correspondence Address:
Devi Dayal
Department of Pediatrics, Pediatric Endocrinology Unit, Advanced Pediatrics Center, Postgraduate Institute of Medical Education and Research, Chandigarh - 160 012
India
Login to access the Email id

Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijem.IJEM_254_18

Rights and Permissions

How to cite this article:
Dayal D, Aggarwal A, Seetharaman K, Muthuvel B. Central precocious puberty complicating congenital adrenal hyperplasia: North Indian experience. Indian J Endocr Metab 2018;22:858-9

How to cite this URL:
Dayal D, Aggarwal A, Seetharaman K, Muthuvel B. Central precocious puberty complicating congenital adrenal hyperplasia: North Indian experience. Indian J Endocr Metab [serial online] 2018 [cited 2018 Dec 19];22:858-9. Available from: http://www.ijem.in/text.asp?2018/22/6/858/246834



Sir,

Increased androgen production often leads to peripheral precocious puberty (PPP) in congenital adrenal hyperplasia (CAH).[1] The chronic hyperandrogenemia may activate hypothalamic-pituitary axis and cause central precocious puberty (CPP) with advancement of bone age (BA) that compromises stature.[1] Short stature is indeed common in adults with CAH in less developed countries.[2] Gonadotropin-releasing hormone analogs (GnRHa) reduce the rate of linear growth and BA advancement and are considered an effective adjunct to steroids in the treatment of CPP complicating CAH.[3] However, data on GnRHa use are scarce from resource poor settings.[2],[3]

Of the 55 patients diagnosed as precocious puberty (PP), five (four girls and one boy) treated as CPP complicating CAH between 2007 and 2016 were retrospectively reviewed. Four had simple virilizing CAH whereas one had classic salt-wasting form. The diagnosis of CPP was based on either basal serum LH of ≥ 0.3 IU/L or peak stimulated LH ≥5.0 IU/L. The presenting features were premature breast or pubic hair development in girls and testicular enlargement in boy. The mean chronological age (CA) at onset of PP and at start of treatment was 3.6 ± 0.74 years (range 2.7–4.3 years) and 4.7 ± 1.2 years (range 3.5–6.5 years), respectively. All had an advanced BA (mean 8.0 ± 2.4 years, range 6–12 years) at diagnosis. The mean BA advancement at diagnosis and at 3 years of treatment was 3.2 ± 1.3 years (range 2.4–5.5 years) and 2.0 ± 0.35 years (range 1.5–2.5 years), respectively; the slowing of BA advancement was statistically insignificant (P-value 0.08). Four underwent GnRH stimulation test. The mean baseline and stimulated peak LH values were 0.4 ± 0.55 IU/L (range 0.1–1.23 IU/L) and 5.6 ± 0.52 IU/L (range 5.0–6.2 IU/L), respectively. All children received oral hydrocortisone (8–15 mg/m2) and fludrocortisone. Depot leuprolide (initiated at 3.75 mg per month or 11.25 mg 3 monthly) was titrated according to LH values in follow-up. All children were followed up at 6 monthly intervals for at least 3 years. The mean height velocity (HV) and the mean unstimulated serum LH concentrations before and during 1st, 2nd, and 3rd years of therapy were 8.2 ± 0.5, 6.9 ± 1.7, 7.7 ± 1.0, and 7.6 ± 1.7 cm, and 1.4 ± 2.4, 0.21 ± 0.1, 1.4 ± 2.3, and 0.41 ± 0.1 IU/L, respectively. The improvement in mean predicted adult height (PAH) from the start to completion of 3 years of therapy was statistically significant (142.2 ± 6.3 cm vs 150.4 ± 4.2 cm, P value 0.04). Regression in testicular size and breast size was observed. The mean duration of follow up was 4.2 ± 1.6 years.

GnRHa therapy suppresses gonadotropin levels, stabilizes secondary sexual characteristics, and decreases rate of linear growth and BA maturation.[4] These effects were observed in our patients also. However, the treatment outcomes appear to be different as compared to patients treated in the developed countries.[4],[5] Although our patients showed slowing down of BA advancement, the HV did not decrease significantly after GnRHa therapy in contrast with data from developed countries.[4],[5] Additionally, the improvement in PAH barely reached statistical significance after 3 years of therapy. The lack of significant benefits of GnRHa therapy can be attributed to several factors peculiar to our setup such as delays in diagnosis of CAH due to lack of newborn screening, poor metabolic control, and late presentations with CPP.[2] The mean delay in seeking treatment exceeded 1 year in our patients that probably caused significant BA advancement. It is well-known that BA at initiation of GnRHa therapy is the most important factor that affects HV.[5] Delays in diagnosis and initiation of GnRHa therapy adversely affect height outcomes in CAH complicated by CPP in resource-limited setups.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
   References Top

1.
Witchel SF. Congenital Adrenal Hyperplasia. J PediatrAdolescGynecol 2017;30:520-34.  Back to cited text no. 1
    
2.
Sharma R, Seth A. Congenital adrenal hyperplasia: Issues in diagnosis and treatment in children. Indian J Pediatr 2014;81:178-85.  Back to cited text no. 2
    
3.
Kumar M, Mukhopadhyay S, Dutta D. Challenges and controversies in diagnosis and management of gonadotropin dependent precocious puberty: An Indian perspective. Indian J EndocrMetab 2015;19:228-35.  Back to cited text no. 3
    
4.
Pescovitz OH, Comite F, Hench K, Barnes K, McNemar A, Foster C, et al. The NIH experience with precocious puberty: Diagnostic subgroups and response to short-term luteinizing hormone releasing hormone analogue therapy. J Pediatr 1986;108:47-54.  Back to cited text no. 4
    
5.
Güven A, NurcanCebeci A, Hancili S. Gonadotropin releasing hormone analog treatment in children with congenital adrenal hyperplasia complicated by central precocious puberty. Hormones (Athens) 2015;14:265-71.  Back to cited text no. 5
    




 

Top
 
  Search
 
    Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
    Access Statistics
    Email Alert *
    Add to My List *
* Registration required (free)  

 
  In this article
 References

 Article Access Statistics
    Viewed67    
    Printed0    
    Emailed0    
    PDF Downloaded21    
    Comments [Add]    

Recommend this journal