O pediatra frente a uma criança com ambigüidade genital

Abstract

Objective:To present the diagnostic criteria of genital ambiguity, the initial medical management and the attitude expected of pediatricians. Sources:Review of the scientific literature in the form of articles indexed on MEDLINE, in English and Portuguese, published between 1990 and 2007 and dealing with the pediatric age group. Summary of the findings:Pediatricians have a fundamental role to play in the assessment of genital ambiguity, the purpose of which is to arrive at an etiologic diagnosis in the shortest possible time in order to define the patient's sex and plan treatment. There are specific diagnostic criteria, but, in general, genitalia are ambiguous whenever there is difficulty in attributing gender to a child. The pediatrician should inform the patient's family that assignment of their child's sex will depend upon detailed laboratory investigations, preferably carried out by a multidisciplinary team at a tertiary service. The 46,XX or 46,XY karyotypes are not alone sufficient to define the gender of rearing, although the test is fundamental to guide the investigation. When there are no palpable gonads, the first hypothesis should be congenital adrenal hyperplasia. Other causes included partial androgen insensitivity, 5α-reductase deficiency, partial gonadal dysgenesis and hermaphroditism. The family should be provided with support and information throughout the assessment process, and their participation is fundamental in the decision of which gender to rear the child in.Conclusions: Although cases of genital ambiguity are relatively rare for pediatricians, they should be wellinformed on the subject and the correct management of these conditions, since they will often be responsible for the initial guidance that families receive and for maintaining contact between them and the multidisciplinary team.J Pediatr (Rio J). 2007;83(5 Suppl):S184-191: Ambiguity, determination, differentiation, genitalia, intersex.

408 Cases of Genital Ambiguity Followed by Single Multidisciplinary Team during 23 Years: Etiologic Diagnosis and Sex of Rearing

“…Sex assignments before referral to our service and before etiological investigation, in addition to older age at the first visit to our hospital (average of 31.7 months), show that pediatricians need to understand better the concept of DSD and its etiologies as well as clinical and psychosocial implications [45]. The average age at first visit was significantly lower in the 46,XX ovarian DSD group, which may be explained by the large number of cases of 21 α -hydroxylase deficiency in the salt-wasting form, for whom the risk of death is high if it is not early diagnosed.…”

Análise Da Potabilidade De Curso D’água Com Trecho No Instituto Federal Do Paraná - Campus Palmas

“…The popular knowledge acquired by JVC students with their families about medicinal plants can be used as a tool for the learning teaching process and thus integrate popular O estigma em relação às pessoas com intersexo é um assunto complexo (Lima, Canguçu-Campinho, Souza, 2015). O tratamento médico pode vir a se prolongar, em algumas circunstâncias, durante a existência da pessoa, com a necessidade de realização de exames, da utilização de medicamentos e, em alguns casos, da realização de cirurgias corretivas (Guerra-Junior, Maciel-Guerra, 2007).…”

“…A pessoa com intersexo ainda tem que enfrentar o preconceito social e cultural perante a sua situação, assim como a ignorância e invisibilidade, que ainda pairam sobre o assunto no meio acadêmico e científico (Guerra-Junior, Maciel-Guerra, 2007…”

Diagnostic and therapeutic approach in newborns with ambiguous genitale with disorder of sex development: consensus report of Turkish Neonatal and Pediatric Endocrinology and Diabetes Societies

“…Here, the process of informing the family is very important; statements including “no sex” or “hermaphrodite” should be avoided, standard information should be given to families and contact with similar families should be targeted (8). Neonatologists and pediatricians are responsible for establishing coordination together with diagnostic assessment, accurate explanation of the medical condition to the family, stating that the final decision would be made after detailed assessment, and providing communication between the family and other physicians in the team (9, 10). This initial explanation is very important and it will be more difficult for the family to accept the situation if an erroneous statement is used.…”