{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,7]],"date-time":"2026-07-07T18:17:19Z","timestamp":1783448239762,"version":"3.55.0"},"reference-count":11,"publisher":"Bioscientifica","issue":"1","license":[{"start":{"date-parts":[[2026,2,17]],"date-time":"2026-02-17T00:00:00Z","timestamp":1771286400000},"content-version":"vor","delay-in-days":3423,"URL":"https:\/\/www.karger.com\/Services\/SiteLicenses"}],"content-domain":{"domain":["journals.bioscientifica.com"],"crossmark-restriction":true},"short-container-title":[],"published-print":{"date-parts":[[2017,2,1]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>Background: Van Wyk-Grumbach syndrome (VWGS) is a rare presentation of juvenile hypothyroidism which manifests in females as chronic autoimmune hypothyroidism, isosexual pseudoprecocious puberty, and multicystic ovaries. It uniquely presents with short stature and delayed bone age unlike other causes of precocious puberty. Kocher-Debr\u00e9-S\u00e9m\u00e9laigne (KDSS) is a rare presentation of juvenile hypothyroidism manifesting as calf muscle pseudohypertrophy, delayed contraction and relaxation of reflexes, and percussion myxedema. Objectives: To diagnose the rare association of VWGS and KDSS and to conduct a follow-up of the patient on replacement therapy. Methods: We present a case of a 9-year-old female child who presented to the endocrine department with complaints of intermittent vaginal bleeding, short stature, and difficulty in walking. On evaluation she was found to be having autoimmune hypothyroidism, FSH-dominated isosexual pseudoprecocious puberty, delayed bone age, secondary pituitary macroadenoma, delayed relaxation of deep tendon reflexes, and pseudohypertrophy of calf muscles. The diagnosis of VWGS associated with KDSS was made. The patient was initially put on 25 \u03bcg thyroxine replacement, which was titrated accordingly, and was followed up after 6 months and 1 year. Results: All the features of the syndrome improved after 12 months of adequate thyroxine replacement. Conclusions: VWGS and KDSS are rare presentations of juvenile hypothyroidism, and their association is even rarer. Early diagnosis and prompt replacement therapy can avoid unnecessary investigations and surgical interventions.<\/jats:p>","DOI":"10.1159\/000448993","type":"journal-article","created":{"date-parts":[[2016,10,3]],"date-time":"2016-10-03T17:06:18Z","timestamp":1475514378000},"page":"47-51","update-policy":"https:\/\/doi.org\/10.1530\/crossmarkpolicy-17","source":"Crossref","is-referenced-by-count":7,"title":["Van Wyk-Grumbach Syndrome with Kocher-Debr\u00e9-S\u00e9m\u00e9laigne Syndrome: Case Report of a Rare Association"],"prefix":"10.1530","volume":"6","author":[{"given":"Syed Mohd.","family":"Razi","sequence":"first","affiliation":[{"name":"1"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Abhinav Kumar","family":"Gupta","sequence":"additional","affiliation":[{"name":"1"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Deepak Chand","family":"Gupta","sequence":"additional","affiliation":[{"name":"1"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Manish","family":"Gutch","sequence":"additional","affiliation":[{"name":"2"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Keshav Kumar","family":"Gupta","sequence":"additional","affiliation":[{"name":"1"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Syeda Iqra","family":"Usman","sequence":"additional","affiliation":[{"name":"3"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"416","published-online":{"date-parts":[[2016,10,4]]},"reference":[{"key":"2026070713082830100_ref1","unstructured":"Wiersinga WM: Thyroid Disease Manager. Adult Hypothyroidism. http:\/\/www.thyroidmanager.org\/chapter\/adult-hypothyroidism (accessed January 12, 2012)."},{"key":"2026070713082830100_ref2","doi-asserted-by":"crossref","unstructured":"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;57:416-435.","DOI":"10.1016\/S0022-3476(60)80250-8"},{"key":"2026070713082830100_ref3","doi-asserted-by":"crossref","unstructured":"Baranowski E, Hoegler W: An unusual presentation of acquired hypothyroidism: the Van Wyk-Grumbach syndrome. Eur J Endocrinol 2012;166:537-542.","DOI":"10.1530\/EJE-11-0494"},{"key":"2026070713082830100_ref4","doi-asserted-by":"crossref","unstructured":"Rajvanshi S, Philip R, Rai GK, Gupta KK: Kocher-Debr\u00e9-S\u00e9m\u00e9laigne syndrome. Thyroid Res Pract 2012;9:53.","DOI":"10.4103\/0973-0354.96047"},{"key":"2026070713082830100_ref5","doi-asserted-by":"crossref","unstructured":"Rastogi A, Bhadada SK, Bhansali A: An unusual presentation of a usual disorder: Van Wyk-Grumbach syndrome. Indian J Endocrinol Metab 2011;15:141.","DOI":"10.4103\/2230-8210.83356"},{"key":"2026070713082830100_ref6","doi-asserted-by":"crossref","unstructured":"Wormsbecker A, Clarson C: Acquired primary hypothyroidism: vaginal bleeding in a quiet child. CMAJ 2010;182:588-590.","DOI":"10.1503\/cmaj.090883"},{"key":"2026070713082830100_ref7","doi-asserted-by":"crossref","unstructured":"Advis JP, Richards JS, Ojeda SR: Hyperprolactinaemia-induced precocious puberty: studies on the mechanism(s) by which prolactin enhances progesterone responsiveness to gonadotropins in prepubertal rats. Endocrinology 1981;108:1333-1342.","DOI":"10.1210\/endo-108-4-1333"},{"key":"2026070713082830100_ref8","doi-asserted-by":"crossref","unstructured":"Anasti JN, Flack MR, Froehlich J, Nelson LM, Nisula BC: A potential novel mechanism for precocious puberty in juvenile hypothyroidism. J Clin Endocrinol Metab 1995;80:276-279.","DOI":"10.1210\/jcem.80.1.7829625"},{"key":"2026070713082830100_ref9","doi-asserted-by":"crossref","unstructured":"Shaw C, Shaw P: Kocher-Debr\u00e9-S\u00e9m\u00e9laigne syndrome: hypothyroid muscular pseudohypertrophy - a rare report of two cases. Case Rep Endocrinol 2012;2012:153143.","DOI":"10.1155\/2012\/153143"},{"key":"2026070713082830100_ref10","doi-asserted-by":"crossref","unstructured":"Agrawal S, Thakur P: Kocher-Debr\u00e9-S\u00e9m\u00e9laigne syndrome. BMJ Case Rep 2010;2010:bcr0420102877.","DOI":"10.1136\/bcr.04.2010.2877"},{"key":"2026070713082830100_ref11","doi-asserted-by":"crossref","unstructured":"Akman AO, Tayfun M, Demirel F, Ucakturk SA, Gungor A: Association of Van Wyk-Grumbach and Debr\u00e9-S\u00e9m\u00e9laigne syndromes with severe hypothyroidism. J Pediatr Adolesc Gynecol 2015;28:e161-e163.","DOI":"10.1016\/j.jpag.2015.03.004"}],"container-title":["European Thyroid Journal"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.karger.com\/Article\/Pdf\/448993","content-type":"unspecified","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/journals.bioscientifica.com\/etj\/article-pdf\/6\/1\/47\/35873\/etj448993.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"syndication"},{"URL":"https:\/\/journals.bioscientifica.com\/etj\/article-pdf\/6\/1\/47\/35873\/etj448993.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,7,7]],"date-time":"2026-07-07T17:47:32Z","timestamp":1783446452000},"score":1,"resource":{"primary":{"URL":"https:\/\/journals.bioscientifica.com\/etj\/article\/6\/1\/47\/4898\/Van-Wyk-Grumbach-Syndrome-with-Kocher-Debre"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2016,10,4]]},"references-count":11,"journal-issue":{"issue":"1","published-print":{"date-parts":[[2017,2,1]]}},"URL":"https:\/\/doi.org\/10.1159\/000448993","relation":{},"ISSN":["2235-0802","2235-0640"],"issn-type":[{"value":"2235-0802","type":"electronic"},{"value":"2235-0640","type":"print"}],"subject":[],"published":{"date-parts":[[2016,10,4]]}}}