VAN WYK GRUMBACH SYNDROME: CASE REPORT
Main Article Content
Abstract
BACKGROUND: Children with primary hypothyroidism usually presents with delay in linear growth and pubertal development, but in rare instances they can present with precocious puberty. In 1960, presentation of primary hypothyroidism with precocious puberty was first reported and labelled as Van Wyk Grumbach syndrome (VWGS). We report a case of VWGS in a six years old girl.
CASE DESCRIPTION: A six years old girl presented with precocious puberty accompanied by short stature and delayed bone age due to untreated hypothyroidism. On examination she had Tanner’s stage B3 (breast development) and P2 (pubic hair). She had normal external genitalia. On workup her Thyroid Stimulating Hormone was 100 mIU/ml, Free-T4 0.7 was ηg/dl, Follicle Stimulating Hormone 8.1 mIU/ml, Luteinizing Hormone 0.12 mIU/ml, estradiol 58 ρg/ml and prolactin 177 ηg/ml. Pelvic ultrasound revealed a uterine size of 5.4 × 3.2 × 3.6 cm and enlarged ovaries with multi cystic appearance. She was started on 50 µg of Levo-thyroxine per day and then was reassessed after 9 weeks of treatment which showed improvement in her Tanner’s Stage and hormonal profile.
CONCLUSION: The girl was diagnosed as VWGS and responded to the treatment which was evident by improvement in her physical and biochemical assessment.
Article Details
Work published in KMUJ is licensed under a
Creative Commons Attribution 4.0 License
Authors are permitted and encouraged to post their work online (e.g., in institutional repositories or on their website) prior to and during the submission process, as it can lead to productive exchanges, as well as earlier and greater citation of published work.
References
Gupta J, Lin-Su K. Van Wyk-Grumbach syndrome in a female pediatric patient with trisomy 21: a case report. Int J Pediatr Endocrinol 2020;2020:2. https://doi.org/10.1186/s13633-020-0072-y
Van Wyk JJ, Grumbach MM. Syndrome of precocious menstruation and galactorrhea in juvenile hypothyroidism: an example of hormonal overlap in pituitary feedback. J Pediatr 1960;57(3):416-35.
Baranowski E, Högler W. An unusual presentation of acquired hypothyroidism: the Van Wyk-Grumbach syndrome. Eur J Endocrinol 2012;166(3):537-42. https://doi.org/10.1530/EJE-11-0494
Reddy P, Tiwari K, Kulkarni A, Parikh K, Khubchandani R. Van Wyk Grumbach Syndrome: A Rare Consequence of Hypothyroidism. Indian J Pediatr 2018;85(11):1028-30. https://doi.org/10.1007/s12098-018-2704-2
Riaz M, Ibrahim MN, Laghari TM, Hanif MI, Raza J. Van Wyk Grumbach Syndrome. J Coll Physicians Surg Pak 2020;30(12):1332-4. https://doi.org/10.29271/jcpsp.2020.12.1332
Biswas M, Sinha MK, Das MK, Sarkar S. Van Wyk-Grumbach syndrome with hemangioma in an infant. J Pediatr Endocrinol Metab 2018;31(9):1057-60. https://doi.org/10.1515/jpem-2018-0049
Marr A, Hardy K, Curtis J. A 14-year-old girl with short stature, incomplete puberty and severe menstrual bleeding. Paediatr Child Health 2018;23(2):85-8. https://doi.org/10.1093/pch/pxx113
Razi SM, Gupta AK, Gupta DC, Gutch M, Gupta KK, Usman SI. Van Wyk-Grumbach Syndrome with Kocher-Debré-Sémélaigne Syndrome: Case Report of a Rare Association. Eur Thyroid J 2017;6(1):47-51. https://doi.org/10.1159/000448993
Egodawaththe NS, Seneviratne SN, Gunasekara S, Amarasekara SM, Weerasekara K. Van Wyk-Grumbach syndrome and oligosyndactyly in a 6-year-old girl: a case report. J Med Case Rep 2020;14(1):166. https://doi.org/10.1186/s13256-020-02472-z