Hiperplasia congênita da suprarrenal versus puberdade precoce verdadeira: uma visão panorâmica na infância
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2019-09-28
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Universidade Federal de Goiás
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Introduction: Introduction: Early puberty is the onset of sexual development before age eight in girls and before age nine in boys. Congenital adrenal hyperplasia (HSCR) is a familial, autosomal recessive disease caused by a hereditary deficiency of any of the enzymes required for cortisol synthesis, with over 90% of cases due to a 21-hydroxylase deficit. . Objectives: To analyze hormonal dysfunction in childhood with development of precocious puberty. Methodology: The first article is an integrative literature review study, which sought to describe the congenital adrenal hyperplasia, fetal, neonatal, pubertal and adult repercussions. The second article is a case-control study, nested in a prospective and longitudinal cohort, at Dona Iris Hospital and Maternity (HMDI) from October 2014 to April 2019, with female children with early puberty. Results: First article: This is a familial, autosomal recessive disease caused by a hereditary deficiency of cortisol synthesis-related enzymes. Second article: 32 patients were treated between 2014 and 2019, but when applying the inclusion and exclusion criteria, 22 patients remained. The true precocious puberty profile was for girls aged 5-7 years (70%), with onset of complaints between 5 and 7 years (60%), and the first symptoms were pubic hair or breast enlargement or only one of the options with (50%), initial height between 1.17-1.38 m (60%), with target height between 1.40 to 1.55 m (60%), with 80% reaching the target height and final height between 1.56 to 1.66m (70%), follow-up time of 5-6 years (80%). The profile of patients with congenital adrenal hyperplasia was girls aged 8-10 years (58.3%), with onset of complaints between 5 and 7 years (83.3%), and the first symptoms were pubic hair or breast enlargement. or only one of the options (50%), initial height between 1.17-1.38 m in 83.3%, with target height between 1.40 to 1.55 m (66.7%), and 50% achieved target height and 50% did not reach. The final height was between 1.33 to 1.55 m (66.7%), follow-up time of 5-6 years (75%). No significant difference in Fischer test. Conclusion: The article concludes: Early diagnosis and appropriate treatment aim to prevent virilization of the external genitals, prevent dehydration due to salt loss, control hyperandrogenism without affecting the growth rate, preserve the individual's gonadal function, fertility and final height. It concludes in article 2: For true precocious puberty the profile was girls between 5-7 years with onset of complaints between 5 and 7 years and the first symptoms were increased pubic hair and breast enlargement or just one of the options. Initial height ranged from 1.17 to 1.38 m with target height from 1.40 to 1.55. With GnRh (Goserelin) analogue treatment they reached the target height and the final height was between 1.56 and 1.66 m, being followed between 5-6 years. In congenital adrenal hyperplasia the profile was girls between 8-10 years and onset of complaints between 5 and 7 years the first symptoms were pubic hair and breast enlargement or just one of the options. Initial height between 1.17-1.38 m with target height between 1.40 and 1.55 m and with treatment with dexamethasone half reached the target height and half did not reach. Final height was between 1.33 to 1.55 m and were followed between 5-6 years. Using GnRh (Goserelin) analogues for true precocious puberty and dexamethasone for congenital adrenal hyperplasia, no significant difference in final height was observed between the two groups.
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HANNA, Carolina Macedo Saidah. Hiperplasia congênita da suprarrenal versus puberdade precoce verdadeira: uma visão panorâmica na infância. 2019. 57 f. Dissertação (Mestrado em Ciências da Saúde) - Universidade Federal de Goiás, Goiânia, 2019.