Van Wyk–Grumbach syndrome: A case series and review of literature

ADLYNE REENA ASIRVATHAM; SHRIRAAM MAHADEVAN; KARTHIK BALACHANDRAN; SATISH KUMAR BALASUBRAMANIAM

Volume : VII, Issue : II, February - 2018

Van Wyk–Grumbach syndrome: A case series and review of literature

Adlyne Reena Asirvatham, Shriraam Mahadevan, Karthik Balachandran, Satish Kumar Balasubramaniam

Abstract :

Classic presentation of longstanding hypothyroidism is delayed growth and delayed puberty. However, rarely it may present with paradoxical precocious puberty. Thus awareness about this condition among clinicians is essential to diagnose this not so common syndrome.

Aim:

We aimed to study the characteristics of children who presented with Van Wyk–Grumbach syndrome in the last 10 years. We also intended to determine the duration of resolution of symptoms following thyroxine therapy.

Materials & Methods:

Three children with classic presentations of Van Wyk–Grumbach syndrome were included in this study. Prompt treatment with thyroxine was initiated and the subjects were followed up.

Results:

All three children showed significantly elevated TSH with very low T4 levels. High FSH and suppressed LH were observed in all. Clinical, biochemical and radiological resolution occurred at 8, 9 and 12 months in cases 1, 2 and 3 respectively.

Conclusion:

Van Wyk–Grumbach syndrome is quite rare and needs to be suspected for prompt treatment and complete resolution.