Dr Om Kumari, Dr Neetu Sharma, Dr Megha Jindal, Dr Kanak Dubey, Dr Latika Chawla
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"Bridging the gap between hypothyroidism and precocious puberty: A case report of Von Wyk Grumbach Syndrome".
Introduction: Von Wyk Grumbach syndrome (VWGS) is a rare clinical entity that presents with the triad of precocious puberty, polycystic ovaries, and severe hypothyroidism. VWGS is an uncommon cause of precocious puberty characterized by dissociation of gonadarche and adrenarche. Unlike other aetiologies of precocious puberty, VWGS is associated with absent growth acceleration and delayed bone maturation due to prolonged, untreated hypothyroidism. The diagnosis is often delayed or overlooked, as the overt or unusual pubertal manifestations may overshadow the subtle signs of hypothyroidism. Failure to identify hypothyroidism as cause of ovarian cyst and precocious puberty may lead to catastrophic ovarian resection and oophorectomy in prepubertal girls and adolescents.
Case presentation: We present a case of VWGS in a 07 years old girl presenting with painful abdomen with precocious puberty and menarche .Relevant review of the literature on this uncommon clinical entity is also presented.
Conclusion: This study underscores the importance of recognizing the atypical presentation of prolonged hypothyroidism with precocious puberty in young children. It emphasizes the need for clinicians to consider the rare entity of VWGS, which could help prevent delays in appropriate management.
期刊介绍:
Journal of Pediatric and Adolescent Gynecology includes all aspects of clinical and basic science research in pediatric and adolescent gynecology. The Journal draws on expertise from a variety of disciplines including pediatrics, obstetrics and gynecology, reproduction and gynecology, reproductive and pediatric endocrinology, genetics, and molecular biology.
The Journal of Pediatric and Adolescent Gynecology features original studies, review articles, book and literature reviews, letters to the editor, and communications in brief. It is an essential resource for the libraries of OB/GYN specialists, as well as pediatricians and primary care physicians.