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An Unusual case of Precocious puberty (20 Nov 2008)
Dr. Gajendra Yadav .

Patient A-T, Consulted me at the age of 3 years 2 months

With Complaints of

  • Breast Development
  • Irritability.
  • Rapid height gain
  • Vaginal Discharge – Whitish.

 

Her height was 94 cm. Weight was 15.4 Kg.,

Mid Parental height 150 cm. (Father 163 cm. Mother 150 cm.)

 

  • Pubertal status A-1, P-2, B-2
  • Hypertricosis present.
  • No goiter.
  • No café au-lait-spots
  • Fundus was Normal.

 

Investigations revealed following:

      Observed Value                                         (Normal Value)           

                      

    FT-4                      0.91 ng/dl.                                    0.7 – 1.85

    TSH                2.28 uIU/ml.                              0.3- 5.0

    LH                  0.20 miu/ml.                            ---

    FSH                0.30 mIU/ml.                            ---

    E 2                    34 pg/ml.                              ---                    

    Prolactin       14.40 ng/ml.                              2-25 (Female)               

 

Synactin Test.

   0 hr              17 OHP       0.30 ng/ml.

   60 min.         17 OHP       0.38 ng/ml.

 

Bone Age       6 years.

 

USG  - 

  • Utero-cervical length 3.7 cm
  • Ovaries small
  • No Supra renal mass.

 

MRI –

  • Normal   pituitary Hypothalamic region
  • Low lying cerebellar _____ (Tonsiler ectopia).
  • Intra medullary csf Intensity cavity – Extending from C5 to D2 vertibral level    s/o syringomyelia

 

Diagnosis –

  • Precocious puberty
  • Cerebellar Tonsillar ectopia with syringomyelia

 

Problem –

  • Source of sex steroid causing precocious puberty could not be detected so following addition tests were performed at age of 3 year 6 months.

 

 

 

 

 

 

 

 

Additional Tests.

                       

                  Observed Value                                         (Normal Value)           

  1. S. Cortisole – AM                      218                  1.10–200 ng/dl  
  2. S. Androstenedione                    0.27                  0.47 – 2.68
  3. DHEAS                                     <300                  114 – 286 ng/ml
  4. IGF-1 level                                
  5. Free Testosterone                      0.21                 0.3 – 3.6 pg/ml.
  6. Progesterone                             0.83                 2.5- 25 ng/ml.
  7. GH- level          
  8. Repeat USG abdomen-Uterus   3.3X1.6X2.3 cm.                         

 

 

On Follow up investigation were performed on follow Up visit at periodic intervals

 

Age               3yr8mth      4yrs       5yr     5yrs6mth     6yrs                7yrs.

 

LH                   0.02          Ud            -            -               -                             7.0

 

FSH                 0.03          ud             -            -                                  3.6

 

E2                   16.5         22.0         25            -                                   68.          

 

Prolactin          16.1         25.0          -          93.52         41.76           9.35

 

Bone Age      8yrs4m     10yr4m   12yr7m      ----          13yrs3m        13yrs4m 

 

USG Pelvis                                                                    Utero cervicle

Length

4.0cm.

transverse

2.3 cm.

A-P 1.7 cm.

Overies

Normal

in Size

Rt- 1.7 ml.

Lt.-2.1 ml.                    

 

 

Had menstrual cycle at age of 6 yrs.

 


 
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