Van Wyk and Grumbach Syndrome: An Unusual Presentation of Acquired Hypothyroidism
Journal Title: Journal of Endocrinology and Diabetes - Year 2017, Vol 4, Issue 3
Abstract
The association in young females of isosexual precocious puberty and/or polycystic ovaries, delayed bone age and hypothyroidism is known as the Van Wyk and Grumbach syndrome (VWGS). A 9-year-old girl presented with breast development, premature menarch and growth delay. An X-ray of the wrist showed a delayed bone age and laboratory data revealed high circulating levels of TSH with prepubertal LH level. The VWGS was diagnosed and thyroid hormone replacement was started with resolution of the symptoms. Clinically this syndrome is a diagnostic challenge because hypothyroidism usually leads to pubertal and growth delay, whereas in case of VWGS hypothyroidism leads to growth delay and precocious puberty. The pathophysiology of VWGS is not yet clear, but the most accepted theory states that the high concentrations of TSH act directly on FSH receptors and produce gonadal enlargement. Thyroid hormone replacement therapy results in a resolution of all signs and symptoms. Our case is unique as this girl did not have multi cystic ovaries.
Authors and Affiliations
Mouna Ammar, Faten Hadj Kacem, Mahdi Kalthoum, Wajdi Sefi, Mohamed Abid
Keywords
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- EP ID EP333795
- DOI 10.15226/2374-6890/4/3/00179
- Views 97
- Downloads 0
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