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Association of Turner's syndrome and hypopituitarism: a patient report.
Gallicchio, Carla T; Alves, Solange T F; Ramos, Hilda I; Llerena, Juan C; Guimarães, Marília M.
Afiliação
  • Gallicchio CT; Department of Pediatric Endocrinology, Martagão Gesleira Institute, Clementino Fraga Filho Hospital, Federal University of Rio de Janeiro, Rio de Janeiro, Brazil. cgalichio@ig.com.br
J Pediatr Endocrinol Metab ; 16(6): 901-5, 2003.
Article em En | MEDLINE | ID: mdl-12948305
Turner's syndrome (TS) is associated with a wide spectrum of clinical features, such as short stature and gonadal dysgenesis. While it is a common chromosomal abnormality, the association of Turner's syndrome and hypopituitarism is an uncommon finding. We describe here a girl with concomitant pituitary insufficiency and gonadal dysgenesis. When she was 7 years old, her mother reported that she suffered from frontal headache, asthenia and delayed growth. Basal laboratory thyroid evaluation suggested hypothyroidism, with no evidence of autoimmune disease association. She began taking L-thyroxine. At age 11 years, short stature and complaints of frontal headache still persisted. She was still prepubertal and her bone age was delayed by 2.2 years. Her karyotype was compatible with 45,X/46,XX (100 cells analyzed by FISH) and a CT scan showed empty sella. At 12 years of age, an anterior pituitary stimulation test with insulin, gonadotropin-releasing hormone (GnRH) and thyrotropin-releasing hormone (TRH) showed gonadotropin, thyrotropin (TSH) and growth hormone (GH) deficiency. Replacement therapy with GH was begun and she grew 12 cm during the first year of treatment. This report illustrates that, despite the high incidence of sinusitis, short stature and primary hypothyroidism in TS, we should consider the presence of hypopituitarism when the patient presents low levels of TSH with negative thyroid antibodies and inappropriately low levels of gonadotropins for patients with gonadal dysgenesis.
Assuntos
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Coleções: 01-internacional Base de dados: MEDLINE Assunto principal: Síndrome de Turner / Hipopituitarismo Tipo de estudo: Etiology_studies / Risk_factors_studies Limite: Adolescent / Female / Humans Idioma: En Revista: J Pediatr Endocrinol Metab Assunto da revista: ENDOCRINOLOGIA / PEDIATRIA Ano de publicação: 2003 Tipo de documento: Article País de afiliação: Brasil País de publicação: Alemanha
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Coleções: 01-internacional Base de dados: MEDLINE Assunto principal: Síndrome de Turner / Hipopituitarismo Tipo de estudo: Etiology_studies / Risk_factors_studies Limite: Adolescent / Female / Humans Idioma: En Revista: J Pediatr Endocrinol Metab Assunto da revista: ENDOCRINOLOGIA / PEDIATRIA Ano de publicação: 2003 Tipo de documento: Article País de afiliação: Brasil País de publicação: Alemanha