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Peripheral precocious puberty secondary to severe hypothyroidism.
Melina Gil1, Florencia Martino1, Gonzalo Besseghine2
1Endocrinology Research Center Dr. César Bergadá, National Scientific Research Council and Techniques - FEI, Endocrinology Division; Hospital de General de Niños Ricardo Gutiérrez, City of Buenos Aires, Argentina.
Van Wyk-Grumbach syndrome, a rare condition causing severe hypothyroidism, can lead to genital bleeding and ovarian cysts in children. Prompt thyroid hormone treatment resolves these symptoms and pituitary changes.
Area of Science:
- Pediatric Endocrinology
- Reproductive Endocrinology
- Thyroidology
Background:
- Van Wyk-Grumbach syndrome is a rare clinical manifestation of severe primary hypothyroidism.
- It is characterized by signs of hypothyroidism along with signs of estrogenization, including precocious pseudopuberty.
Observation:
- A 10.9-year-old girl presented with genital bleeding and clinical signs of hypothyroidism.
- Pelvic ultrasound revealed bilateral cystic ovarian masses, and hormonal profiles indicated peripheral precocious puberty secondary to hypothyroidism.
- Pituitary MRI demonstrated significant pituitary elongation due to thyrotropic hyperplasia.
Findings:
- Severe hypothyroidism secondary to chronic lymphocytic thyroiditis was confirmed.
- Treatment with thyroid hormone led to clinical improvement, resolution of adnexal masses, and normalization of pituitary hyperplasia.
Implications:
- This case highlights the importance of recognizing thyroid dysfunction in pediatric patients presenting with unusual symptoms.
- Early diagnosis and prompt referral to pediatric endocrinology are crucial for managing severe hypothyroidism and its associated complications.
- Pediatricians should be vigilant for signs of thyroid dysfunction to prevent severe outcomes like Van Wyk-Grumbach syndrome.
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