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Endocrinology and gonads
Published in Jagdish M. Gupta, John Beveridge, MCQs in Paediatrics, 2020
Jagdish M. Gupta, John Beveridge
11.11. Congenital adrenal hyperplasia (21-hydroxylase deficiency)is associated with high plasma 17-hydroxyprogesterone levels.is a common cause of ambiguous genitalia in newborn girls.may be associated with salt wasting.is associated with high plasma Cortisol levels.may be complicated by infertility.
Endocrinology
Published in Stephan Strobel, Lewis Spitz, Stephen D. Marks, Great Ormond Street Handbook of Paediatrics, 2019
Mehul Dattani, Catherine Peters
Ambiguous genitalia are usually present at birth. The gender of the child should not be assigned without confirmation. If possible, referral to an expert Disorders of Sex Development (DSD) team should be made. Note should be made of presence or absence of palpable gonads and their position if felt. External genitalia can be described as Prader staging. The disorder may be associated with salt loss in salt-losing congenital adrenal hyperplasia (see pages 403–4). There may be associated dysmorphic features.
Major neonatal conditions
Published in Spencer W. Beasley, John Hutson, Mark Stringer, Sebastian K. King, Warwick J. Teague, Paediatric Surgical Diagnosis, 2018
Spencer W. Beasley, John Hutson, Mark Stringer, Sebastian K. King, Warwick J. Teague
When a newborn has ambiguous genitalia, clinical examination includes assessment of the external genitalia to determine:The size of the phallus.The degree of fusion of the inner genital folds to form a urethra.The degree of outer genital fold fusion to form a scrotum, with its characteristically wrinkled skin.
Ovotestis at 18 years: an accidental discovery in an internally displaced persons’ camp in North-Eastern Nigeria
Published in Journal of Obstetrics and Gynaecology, 2019
Hadiza Abdullahi Usman, Bala Mohammed Audu, Mohammed Bukar, Ahmed A. Mayun
The therapeutic goals in the management of DSD are centred on ensuring an individual with a sound psychosocial and sexual fulfilment in life. The patient and her parents had counselling and psychosocial support by experts, including religious clerics. This is an important goal in the care of DSD to minimise the risk of creating a social outcast (Nthumba et al. 2008). This patient had dominant clinical and functional feminine features and therefore had genitoplasty with the resection of the right ovotesticular tissue, which is discordant with her sex of rearing. The gonadectomy would reduce the risk of a gonadal malignancy (Liu et al. 2014). The option of the Modified Randolph–Hung reduction clitoroplasty offered to this patient would ensure that the orgasmic function and the erectile sensation of the clitoris are maintained. This is superior to a clitoridectomy, as it preserves the glans and its neurovascular bundle. The management of OT-DSD in the adolescent and the adult is quite challenging and associated with psychological trauma. The need for a thorough examination of the external genitalia of new-borns and the appropriate referral of those with ambiguous genitalia early in life will minimise the psychological trauma associated with the management of these cases later in life. This patient benefited from the volunteer service scheme of the Society of Gynaecology and Obstetrics of Nigeria (SOGON), which extended its services to the IDP camp.
The XY Female: Exploring Care for Adolescent Girls with Complete Androgen Insensitivity Syndrome
Published in Comprehensive Child and Adolescent Nursing, 2020
Diagnostic pathways (Ahmed et al., 2015) can advise on the investigations and examinations that should be undertaken when a team is presented with an infant, child or adolescent with a DSD. Specific care pathways are suggested and implemented for the management of infants with ambiguous genitalia (Davies, 2019), including exploring the clinical status of the baby, their history, maternal and family history, and family knowledge and expectations, followed by a detailed assessment, resulting in an ultimate diagnosis and plan of care. Conversely, however, a small group of DSD can present in adolescence, encompassing girls presenting with primary amenorrhea (where menses have not yet commenced), girls with virilization, such as hirsutism or clitoromegaly, or boys with delayed puberty.
Cancer Care Considerations for Sexual and Gender Minority Patients
Published in Oncology Issues, 2019
Mandi L. Pratt-Chapman, Jennifer Potter
Invisibility of intersex people in mainstream society (approximately 1.7 percent of the population)3 is even greater.18 Research to optimize healthcare of intersex people is severely lacking, and education on what is known about intersex medical management is sparse in current medical school curricula.9 Though only a portion of intersex people have ambiguous genitalia, it is vital to note that the longstanding practice of surgical intervention for ambiguous genitalia is not medically indicated and leads to unnecessary and painful lifelong repercussions—including high potential for additional surgeries and infections. In 2017 three former U.S. surgeons general called for a moratorium on intersex infant genital surgeries.19