Ambiguous genitalia: an overview of 17 years' experience

Göllü, Gülnur; Yildiz, Rahşan Vargün; Bingöl-Koloğlu, Meltem; Yağmurlu, Aydın; Şenyücel, Mine; Aktuğ, Tanju; Gökçora, I. Haluk; Dindar, Hüseyin

doi:10.1016/j.jpedsurg.2006.12.036

Cited by 48 publications

(30 citation statements)

References 25 publications

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“…In genitalia apparently female in appearance clitoral diameter greater than 6 mm, palpable gonad within labioscrotal fold, posterior labial fusion, an inguinal mass which could correspond to testicles signifies ambiguity. In this study as in mostother studies, [9,14,15,18] the commonest presentations of ambiguous external genitalia are clitoral hypertrophy, significant micropenis, fusion of labioscrotal fold, hypospadias, absent urethral opening and non-palpable gonads. In this study, all patients had clitoral hypertrophy.…”

Section: Discussionsupporting

confidence: 72%

“…In this study, all patients had clitoral hypertrophy. Clitoral hypertrophy was recorded in 92.9% and 77.2% of cases in a Saudi Arabian and Australian study respectively [9,18]. Clitoral hypertrophy is also the commonest finding in patients with 46XX DSD [9,15].…”

Section: Discussionmentioning

confidence: 99%

“…Laparoscopy/minilaparatomy in the evaluation of children with ambiguous external genitalia is very important especially in developing countries, [12,18] this is because there is usually a challenge in determining genetic Sex of the children especially in developing countries where Karyotyping and sophisticated imaging facilities are not available or require highly trained personnel. In our study, only two patients could do Barr body estimation which was unreliable in determining definitive sex.…”

Section: Discussionmentioning

confidence: 99%

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Ambiguous External Genitalia in Childhood in Port Harcourt, Nigeria

Jaja¹,

Anochie²,

Paediatrician³

2011

Pediatr Therapeut

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Ambiguous external genitalia are a major cause of parental anxiety and a challenge to paediatricians in developing countries. Aims and objectives:This study aims to highlight the pattern of presentation and challenges in the management of ambiguous external genitalia in a developing country. Patients and methods:A prospective study of all patients with ambiguous external genitalia managed in the paediatric endocrine unit of the University of Port Harcourt Teaching Hospital between January 2008 and Dec 2010 was done. Biodata, clinical presentation, management and outcome were documented.Results: Six patients were seen with ambiguous external genitalia during the study period. Age ranged from birth to 16 years at presentation. The commonest presentation was clitoral hypertrophy in all patients. The commonest initial diagnosis was CAH.Definitive confirmation of cause of ambiguous external genitalia with a reconstructive surgery was done in only one patient due to non-availability and high cost of investigation. Three (50%) patients were lost to follow up of which 2(66.7%) have resorted to spiritual treatment. One patient who presented at 16 years of age died from an associated obstructive uropathy with chronic renal failure. Conclusion:The challenges in managing children with ambiguous external genitalia in developing countries include late presentation, high cost andnon-availability of investigations for making definitive diagnosis.

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Section: Discussionsupporting

confidence: 72%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

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Ambiguous External Genitalia in Childhood in Port Harcourt, Nigeria

Jaja¹,

Anochie²,

Paediatrician³

2011

Pediatr Therapeut

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“…Reports from other cultures, such as India [11][12][13], Turkey [14], Saudi Arabia [15], Malaysia [16] and Thailand [17] indicate a strong tradition for Asian and Middle Eastern parents to select male gender for their children born with ambiguous genitalia. In a country like India or Pakistan, DSD patients raised male, even if they are infertile, are more likely to achieve economic independence than patients raised female who are infertile.…”

Section: Rumour and Discriminationmentioning

confidence: 99%

Disorders of sex development (DSDs), their presentation and management in different cultures

Warne

Raza

2008

Rev Endocr Metab Disord

115

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The way disorders of sex development (DSD) are viewed and managed in different cultures varies widely. They are complex conditions and even well-educated lay people find them difficult to understand, but when families are very poor and lacking in basic education, and the health system is starved of resources, traditional beliefs, folk remedies and prejudice combine to make the lives of children and adults with DSD extremely difficult and sad. Rumour and discrimination isolate them from their communities and they become devalued. People with DSDs desire the same things in life as everyone else-to find someone who will love them, to be valued as human beings, to feel at home in their own bodies, to be able to have satisfactory sexual relations should these be desired, to be able to trust their medical advisers and to be integrated into the general community. Long term outcome studies have been published from many countries, but these studies have not necessarily been critical of the values that underpinned the type of treatment given to the patients. There is a need for standardized instruments that would allow a true comparison of the quality of outcomes from the patients' perspective. Much could be done to improve equity between rich and poor countries for the benefit of people with DSDs. A focus on developing cheap, robust diagnostic tests, making essential medicines available for all, training surgeons to do better operations, educating health professionals, families and the general community in order to break down prejudice against people with DSDs, and training mental health workers in this specialized field, would do much to alleviate the burden of the condition.

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“…The general surgical rule is to make the surgical treatment as soon as possible and to choose the gender, which is easier to be achieved with reconstructive treatment [85] but also in accordance to the intrauterine hormonal exposure. The goal is to complete the surgical correction till the age of 2 [86]. It is also important to evaluate, in the presence of nonfunctional/dysgenetic gonads, to evaluate the malignancy potential and to make the right treatment choices [87].…”

Section: Hypogonadism Typesmentioning

confidence: 99%

Hypogonadism in Male Sexual Dysfunction

Stoian¹,

Mozoş²,

Craina³

et al. 2017

Sexual Dysfunction

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Normal testosterone level is influencing all the steps of the male psychosexual development: intrauterine neonatal and final psychosexual development.. At pubertal stage, the quality of testosterone secretion is conditioning the development of the mature male phenotype. In adult life, eugonadism sustains desire, arousal, determines spontaneous erections, facilitates stimulated erection, influencing the response rate to medication. Moreover, eugonadism sustain daydreaming and phantasies, both needed for a normal sexual life. The pathogenic mechanism of all these actions is presented. Talking about hypogonadism means not only the classical types of hypogonadism: due to classical testicular disease of central, hypothalamic and hypophysis disease, but also the partial testosterone deficiency induces by aging (late onset hypogonadism), weight increase (up to 30% of males with metabolic syndrome and 50% of males with diabetes) or secondary hypogonadism described in chronic use of steroids or after long exposure to stress, especially in young males. All these types of hypogonadism, that affect young, middle aged or old males will be presented separately. A therapeutic approach that is individualized for each type of hypogonadism, should consider positive and possible negative effects and all alternatives will be presented: life style changes, sustained weight loss, increase exercise, supplemental therapy, pro fertility treatment.

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Ambiguous genitalia: an overview of 17 years' experience

Cited by 48 publications

References 25 publications

Ambiguous External Genitalia in Childhood in Port Harcourt, Nigeria

Ambiguous External Genitalia in Childhood in Port Harcourt, Nigeria

Disorders of sex development (DSDs), their presentation and management in different cultures

Hypogonadism in Male Sexual Dysfunction

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