{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,2,21]],"date-time":"2025-02-21T21:32:41Z","timestamp":1740173561848,"version":"3.37.3"},"reference-count":34,"publisher":"Endocrinology Research Centre","issue":"2","license":[{"start":{"date-parts":[[2021,12,12]],"date-time":"2021-12-12T00:00:00Z","timestamp":1639267200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/www.probl-endojournals.ru\/jour\/about\/editorialPolicies#openAccessPolicy"}],"content-domain":{"domain":["www.probl-endojournals.ru"],"crossmark-restriction":true},"short-container-title":["Probl Endokrinol (Mosk)"],"abstract":"<jats:p>McCune\u2013Albright\u2013Braitsev Syndrome (MAB syndrome) is a very rare multisystem disease manifested by fibrous bone dysplasia, coffee-and-milk colored spots, hyperfunction of various endocrine glands and a number of pathologies of\u00a0other body systems. We present a description of a clinical case of a severe progressive course of MAB Syndrome in a\u00a0nine-year-old girl. With this diagnosis, the girl is observation of the girl began when she was 2.5 years old, when spots of coffee-and-milk, polyosseous fibrous dysplasia, peripheral premature sexual development against a backdrop of estrogen-\u00adsecreting ovarian cysts, multinodular goiter were detected. In the process of dynamic observation, it was noted that the child\u2019s active growth stopped against a backdrop of deformities of the skeletal system with multiple repeated fractures of the\u00a0extremities; progression of skull deformity with stenosis of the optic nerve canals and deterioration of visual acuity; development of STH hypersecretion, hypophosphatemic rickets, tachycardia. Appropriate suppressive \/ replacement therapy was prescribed for each of the endocrine dysfunctions. The article presents algorithms for examining a girl in dynamics, criteria for choosing a component-wise management tactics and a discussion of the features of the course of all manifestations of the Syndrome.<\/jats:p>","DOI":"10.14341\/probl12847","type":"journal-article","created":{"date-parts":[[2022,4,30]],"date-time":"2022-04-30T08:23:05Z","timestamp":1651306985000},"page":"72-89","update-policy":"https:\/\/doi.org\/10.14341\/crossmarkpolicy2018","source":"Crossref","is-referenced-by-count":0,"title":["Early manifestation and progressive multicomponent current of McCune\u2013Albright-Braitsev syndrome in a girl 9 years old: a clinical case and literature review"],"prefix":"10.14341","volume":"68","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-0283-2428","authenticated-orcid":true,"given":"Ya. V.","family":"Girsh","sequence":"first","affiliation":[{"name":"Surgut State University"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1320-6561","authenticated-orcid":true,"given":"M. A.","family":"Kareva","sequence":"additional","affiliation":[{"name":"Endocrinology Research Centre"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"N. V.","family":"Makazan","sequence":"additional","affiliation":[{"name":"Endocrinology Research Centre"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0438-3582","authenticated-orcid":true,"given":"E. N.","family":"Davygora","sequence":"additional","affiliation":[{"name":"Surgut State University"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"5458","published-online":{"date-parts":[[2021,12,12]]},"reference":[{"key":"ref1","doi-asserted-by":"crossref","unstructured":"McCune DJ, Bruch H. 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