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Female Pseudohermaphroditism

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61. Teratology and Drug Use During Pregnancy (Overview)

of these medications. [ ] Thus, the 5-letter system is being phased out over the next 3 years in favor of a more comprehensive system with a narrative summary of the risks posed by drugs. The FDA, the government agency that oversees the safety of drugs, provides the most widely used system to grade the teratogenic effects of medications. Each drug summary will have three sections: pregnancy, lactation, and females and males of reproductive potential. “Pregnancy” merges previous categories of “pregnancy” and “labor (...) and delivery.” “Lactation” replaces “breastfeeding mothers” and all medications will be required to have this section. [ ] “Females and males of reproductive potential” is a new category to include information about pregnancy testing, contraception requirements, and effects on fertility before, during, and after drug therapy. An additional requirement with the new FDA classification system, is the inclusion of information about pregnancy exposure registries under the “pregnancy” category. Contact

2014 eMedicine.com

62. Urethral Diverticulum (Overview)

on the incidence of urethral diverticulum. This statement reflects both the difficulty of the diagnosis and the varying levels of awareness of the disorder throughout the medical community. Recently, increased awareness and discussion of urethral diverticula have allowed the diagnosis to be made more frequently and with less delay than in the past. Next: History of the Procedure In 1805, William Hey first described a female with suburethral diverticulum in the medical literature; however, he claimed to have (...) first observed and treated this lesion about 20 years earlier. In the first half of the 20th century, only 17 cases were reported, and, as a result, the condition was assumed to be quite rare. Reports of cases, including a large series from Johns Hopkins Hospital, dating from 1950-1970 have revealed that suburethral diverticula in fact are not rare. Increased awareness of the diagnosis and increased interest in female urology and urogynecology have led to the publication of many reports and review

2014 eMedicine.com

63. Adrenal Disease and Pregnancy (Overview)

with classic 21-hydroxylase deficiency—3 of whom were female pseudohermaphrodites with the salt-wasting form—following treatment with glucocorticoids. The patients gave birth to 4 females, all healthy and with normal female external genitalia. None of the neonates was affected with 21-hydroxylase deficiency. The placental aromatase activity was sufficient to prevent masculinization of the fetuses' external genitalia in all of these patients. [ ] The authors recommended treatment with glucocorticoids (...) be considered in patients who have had reconstructive genital surgery. Evaluation of the infant . Female pseudohermaphroditism may be either a consequence of maternal hyperandrogenism or, if the father is a carrier, of fetal 21-hydroxylase deficiency. (Male infants may have enlarged external genitalia.) If the external genitalia are ambiguous, appropriate laboratory studies should be performed on the infant to exclude 21-hydroxylase deficiency. Fetal diagnosis and treatment of CAH Diagnosis CAH caused

2014 eMedicine.com

64. Prostate Hyperplasia, Benign (Follow-up)

, and/or digital rectal examination. [ ] Unlike alpha-blockers, which work by reducing smooth muscle tone, 5-ARIs improve LUTS by reducing prostate volume. Thus, patients with larger prostates may achieve a greater benefit. Maximal reduction in prostate volume requires 6 months of therapy. The role of 5-ARIs in medical management of BPH emerged from the discovery of a congenital form of pseudohermaphroditism secondary to dihydrotestosterone (DHT) deficiency, which results from a lack of 5-alpha-reductase (...) to be investigated. [ ] Because 5-ARIs interfere with the metabolism of testosterone, pregnant females or those who are considering conception should not handle crushed or broken tablets because of the potential for absorption and subsequent potential risk to a male fetus. 5-alpha reductase inhibitors and prostate cancer On June 9, 2011, the US Food and Drug Administration (FDA) announced revisions to the prescribing information for 5-ARIs. The FDA advised that prior to initiating therapy with 5-ARIs, clinicians

2014 eMedicine.com

65. Nonneoplastic Epithelial Disorders of the Vulva (Follow-up)

growths of vestibular mucosa are normal variants of female anatomy. Their origin is unknown. No association with human papillomavirus infection has been detected so far. Congenital malformations Ambiguous external genitalia is a term used to describe situations in which the external genital organs are not clearly female or male at birth. Abnormalities of the external genitalia occur in 1 in 4500 births. [ ] The 3 main etiologic categories include (1) female pseudohermaphroditism, accounting for 80 (...) % of ambiguous genitalia; (2) male pseudohermaphroditism, occurring in approximately 15% of cases; and (3) disorders of differentiation. The preponderance of female pseudohermaphroditism is due to a recessive congenital enzymatic defect of adrenal steroid biosynthesis (most commonly, 21-hydroxylase deficiency), resulting in androgen overproduction that virilizes the external genitalia of a female 46,XX fetus with normal ovaries. Although rare, maternal factors can also virilize a female fetus. In male

2014 eMedicine.com

66. Ovotestis (Follow-up)

intelligence and have a normal life expectancy. Fertility potential does exist in people with ovotesticular disorder of sexual development who are given a female sexual assignment. Ovulation can occur, and several pregnancies have been reported in this group. To date, all documented offspring have been male. One report describes a 46,XX/46,XY infertile chimeric male who fathered a child with sperm obtained from his testicular tissue through intracytoplasmic sperm injection. [ , , ] People (...) with a small portion being sexually dissatisfied. Those raised as males may complain of an inability to have and/or maintain erections, while females may complain of vaginal stenosis, recurrent cystitis, and hot flushes. [ ] Previous Next: Patient Education Once the diagnosis of genital ambiguity is made, ongoing psychological support for the patient, parents, and other family members is critical. [ ] Psychological counseling is perhaps even more important for patients with a diagnosis delayed until

2014 eMedicine.com

67. Teratology and Drug Use During Pregnancy (Follow-up)

of these medications. [ ] Thus, the 5-letter system is being phased out over the next 3 years in favor of a more comprehensive system with a narrative summary of the risks posed by drugs. The FDA, the government agency that oversees the safety of drugs, provides the most widely used system to grade the teratogenic effects of medications. Each drug summary will have three sections: pregnancy, lactation, and females and males of reproductive potential. “Pregnancy” merges previous categories of “pregnancy” and “labor (...) and delivery.” “Lactation” replaces “breastfeeding mothers” and all medications will be required to have this section. [ ] “Females and males of reproductive potential” is a new category to include information about pregnancy testing, contraception requirements, and effects on fertility before, during, and after drug therapy. An additional requirement with the new FDA classification system, is the inclusion of information about pregnancy exposure registries under the “pregnancy” category. Contact

2014 eMedicine.com

68. Sexuality: Gender Identity (Diagnosis)

, the prenatal exposure to androgens results in virilization of female genitalia, in what has been called female pseudohermaphroditism. At times, virilization is complete, with substantial clitoromegaly. In such cases, the genitalia are so masculine that male sex is mistakenly assigned to the newborn at birth. Soon, clinical findings and symptomatology reveal the defect in the adrenal gland and the true female genotype of the child. Serious salt-losing nephropathy may be the presenting problem in certain (...) =aHR0cHM6Ly9lbWVkaWNpbmUubWVkc2NhcGUuY29tL2FydGljbGUvOTE3OTkwLW92ZXJ2aWV3 processing > Gender Identity Updated: Mar 16, 2015 Author: Shuvo Ghosh, MD; Chief Editor: Caroly Pataki, MD Share Email Print Feedback Close Sections Sections Gender Identity Definitions Gender identity and gender role Gender identity is defined as a personal conception of oneself as male or female (or rarely, both or neither). This concept is intimately related to the concept of gender role, which is defined as the outward manifestations of personality that reflect the gender identity. Gender

2014 eMedicine Pediatrics

69. Primary Generalized Glucocorticoid Resistance (Overview)

cortisol resistance. Clin Endocrinol (Oxf) . 2001 Sep. 55(3):363-71. . Mendonca BB, Leite MV, de Castro M, et al. Female pseudohermaphroditism caused by a novel homozygous missense mutation of the GR gene. J Clin Endocrinol Metab . 2002 Apr. 87(4):1805-9. . Vottero A, Kino T, Combe H, Lecomte P, Chrousos GP. A novel, C-terminal dominant negative mutation of the GR causes familial glucocorticoid resistance through abnormal interactions with p160 steroid receptor coactivators. J Clin Endocrinol Metab (...) seizures during the course of a febrile illness [ ] ; in a newborn baby with severe hypoglycemia, excessive fatigability with feeding, increased susceptibility to infections, and concurrent growth hormone deficiency [ ] ; and in several adult patients with chronic fatigue. [ , , ] Clinical manifestations of mineralocorticoid excess include hypertension and hypokalemic alkalosis. Clinical manifestations of androgen excess include ambiguous genitalia in a karyotypic female at birth and gonadotropin

2014 eMedicine Pediatrics

70. Primary Generalized Glucocorticoid Resistance (Diagnosis)

cortisol resistance. Clin Endocrinol (Oxf) . 2001 Sep. 55(3):363-71. . Mendonca BB, Leite MV, de Castro M, et al. Female pseudohermaphroditism caused by a novel homozygous missense mutation of the GR gene. J Clin Endocrinol Metab . 2002 Apr. 87(4):1805-9. . Vottero A, Kino T, Combe H, Lecomte P, Chrousos GP. A novel, C-terminal dominant negative mutation of the GR causes familial glucocorticoid resistance through abnormal interactions with p160 steroid receptor coactivators. J Clin Endocrinol Metab (...) seizures during the course of a febrile illness [ ] ; in a newborn baby with severe hypoglycemia, excessive fatigability with feeding, increased susceptibility to infections, and concurrent growth hormone deficiency [ ] ; and in several adult patients with chronic fatigue. [ , , ] Clinical manifestations of mineralocorticoid excess include hypertension and hypokalemic alkalosis. Clinical manifestations of androgen excess include ambiguous genitalia in a karyotypic female at birth and gonadotropin

2014 eMedicine Pediatrics

71. 5-Alpha-Reductase Deficiency (Treatment)

, a third gender is not accepted, suggesting that a decision for gender assignment should occur before the patient is able to make an individual choice. Although gender assignment in the newborn period may not be ideal, this well-intended, and perhaps justifiable approach is undertaken due to social as well as developmental concerns because children typically develop a sense of gender identity by age 3 years. Proponents of early surgery for female gender assignment suggest that early gonadectomy (...) than ensuring that the surgery be performed by a surgeon with extensive experience in caring for patients with DSD. [ ] Medical care Medical care is dictated by gender assignment and surgical intervention Hormone replacement therapy should be considered in patients raised in the male gender and is required in patients raised in the female gender that have undergone gonadectomy In patients with 5-alpha-reductase deficiency who are raised as male, testosterone or dihydrotestosterone (DHT) therapy may

2014 eMedicine Pediatrics

72. Sexuality: Gender Identity (Overview)

, the prenatal exposure to androgens results in virilization of female genitalia, in what has been called female pseudohermaphroditism. At times, virilization is complete, with substantial clitoromegaly. In such cases, the genitalia are so masculine that male sex is mistakenly assigned to the newborn at birth. Soon, clinical findings and symptomatology reveal the defect in the adrenal gland and the true female genotype of the child. Serious salt-losing nephropathy may be the presenting problem in certain (...) =aHR0cHM6Ly9lbWVkaWNpbmUubWVkc2NhcGUuY29tL2FydGljbGUvOTE3OTkwLW92ZXJ2aWV3 processing > Gender Identity Updated: Mar 16, 2015 Author: Shuvo Ghosh, MD; Chief Editor: Caroly Pataki, MD Share Email Print Feedback Close Sections Sections Gender Identity Definitions Gender identity and gender role Gender identity is defined as a personal conception of oneself as male or female (or rarely, both or neither). This concept is intimately related to the concept of gender role, which is defined as the outward manifestations of personality that reflect the gender identity. Gender

2014 eMedicine Pediatrics

73. Pediatric Urologic Gynecology (Diagnosis)

and gynecologists must be familiar with the more common disorders whereby gender assignment and rearing is along female lines. [ ] These conditions include normal-appearing girls who are genetically male (male pseudohermaphroditism; 46,XY DSD) and genetic females appearing as males (female pseudohermaphroditism; 46,XX DSD). Female pseudohermaphrodites are genetic females (ie, XX karyotype) but may appear as males without testes. The most common cause of female pseudohermaphroditism is (21-hydroxylase deficiency (...) in >90%). In this disorder, the absence of 21-hydroxylase results in the overproduction of androgens and results in a wide spectrum of genital abnormalities, ranging from mild masculinization of the clitoris (clitoromegaly; see the image below) to complete masculinization. Female pseudohermaphroditism. Note the clitoromegaly and small introitus. The labioscrotal folds are rugous and hyperpigmented, producing the physical appearance of severe hypospadias with ; however, in all cases, the internal

2014 eMedicine Pediatrics

74. Panhypopituitarism (Diagnosis)

shorter and more overweight, had less education, were less likely to be married, and had a higher unemployment rate, lower incomes, and fewer children. They also scored lower on the Female Sexual Function Index and Male Sexual Quotient. [ ] Previous References Kim SY. Diagnosis and Treatment of Hypopituitarism. Endocrinol Metab (Seoul) . 2015 Dec. 30 (4):443-55. . . Gounden V, Jialal I. Hypopituitarism (Panhypopituitarism). 2018 Jan. . . Migliaretti G, Aimaretti G, Borraccino A, et al. Incidence (...) Onset Hypopituitarism. Horm Res Paediatr . 2015. 84 (2):94-101. . Matthai SM, Smith CS. Pituitary hypoplasia associated with a single central maxillary incisor. J Pediatr Endocrinol Metab . 1996 Sep-Oct. 9(5):543-4. . Willnow S, Kiess W, Butenandt O, et al. Endocrine disorders in septo-optic dysplasia (De Morsier syndrome)--evaluation and follow up of 18 patients. Eur J Pediatr . 1996 Mar. 155(3):179-84. . Burgner DP, Kinmond S, Wallace AM, et al. Male pseudohermaphroditism secondary

2014 eMedicine Pediatrics

75. Adrenal Disease and Pregnancy (Diagnosis)

with classic 21-hydroxylase deficiency—3 of whom were female pseudohermaphrodites with the salt-wasting form—following treatment with glucocorticoids. The patients gave birth to 4 females, all healthy and with normal female external genitalia. None of the neonates was affected with 21-hydroxylase deficiency. The placental aromatase activity was sufficient to prevent masculinization of the fetuses' external genitalia in all of these patients. [ ] The authors recommended treatment with glucocorticoids (...) be considered in patients who have had reconstructive genital surgery. Evaluation of the infant . Female pseudohermaphroditism may be either a consequence of maternal hyperandrogenism or, if the father is a carrier, of fetal 21-hydroxylase deficiency. (Male infants may have enlarged external genitalia.) If the external genitalia are ambiguous, appropriate laboratory studies should be performed on the infant to exclude 21-hydroxylase deficiency. Fetal diagnosis and treatment of CAH Diagnosis CAH caused

2014 eMedicine.com

76. 5-Alpha-Reductase Deficiency (Follow-up)

in newborns. Sex Dev . 2007. 1(3):147-51. . Skordis N, Shammas C, Efstathiou E, Sertedaki A, Neocleous V, Phylactou L. Late diagnosis of 5alpha steroid-reductase deficiency due to IVS12A>G mutation of the SRD5a2 gene in an adolescent girl presented with primary amenorrhea. Hormones (Athens) . 2011 Jul-Sep. 10(3):230-5. . Fénichel P, Paris F, Philibert P, Hiéronimus S, Gaspari L, Kurzenne JY. Molecular diagnosis of 5a-reductase deficiency in 4 elite young female athletes through hormonal screening (...) , Khurana ML, et al. Gender identity of children and young adults with 5alpha-reductase deficiency. J Pediatr Endocrinol Metab . 2008 Feb. 21(2):173-9. . Maimoun L, Philibert P, Cammas B, Audran F, Bouchard P, Fenichel P. Phenotypical, biological, and molecular heterogeneity of 5a-reductase deficiency: an extensive international experience of 55 patients. J Clin Endocrinol Metab . 2011 Feb. 96(2):296-307. . Katz MD, Cai LQ, Zhu YS, et al. The biochemical and phenotypic characterization of females

2014 eMedicine Pediatrics

77. Ambiguous Genitalia and Intersexuality (Follow-up)

Terminology and Revised Nomenclature of Disorders of Sexual Development Previous Revised Female pseudohermaphrodite 46,XX DSD Male pseudohermaphrodite 46,XY DSD True hermaphrodite Ovotesticular DSD XX male 46,XX testicular DSD XY sex reversal 46,XY complete gonadal dysgenesis Contributor Information and Disclosures Author Joel Hutcheson, MD Consulting Staff, Departments of Surgery and Urology, Pediatric Surgical Associates Joel Hutcheson, MD is a member of the following medical societies: , Disclosure (...) undergo hormonal imprinting in utero. Various activists and some health care professionals have called for a moratorium on gender reassignment and genital surgery until studies have been completed on the long-term effects of such surgery. Several long-term follow-up studies are being conducted, including a study by the North American Task Force on Intersexuality. Many health care professionals oppose the proposed moratorium. In a virilized female, the surgical procedure is termed feminizing

2014 eMedicine Pediatrics

78. Teratomas and Other Germ Cell Tumors (Diagnosis)

is difficult to generalize. [ ] When platinum-based chemotherapy–resistant tumors are evaluated, between one third and one half of tumors exhibit microsatellite instability. Previous Next: Etiology With sacrococcygeal teratomas, no causative agents are known. With respect to ovarian germ cell tumors, a familial predilection may be present. Cases in seven families have been reported in which female first-degree relatives had germ cell tumors. In an additional seven families, males had germ cell tumors (...) . Children with DSDs are typically male pseudohermaphrodites with antigen insensitivity or 5-alpha reductase deficiency. These patients with testicular feminization are sometimes discovered serendipitously during a hernia repair. Optimal timing for gonadal resection in these situations is a matter of debate. Gonadal estrogen production may benefit the patient in terms of growth and development. However, gonadoblastoma has been observed in patients as young as 2 months, and frank tumors have been observed

2014 eMedicine Pediatrics

79. Nephrotic Syndrome (Diagnosis)

brucei rhodesiense infection). [ ] Another nonmuscle myosin gene, MYO1E , was recently reported to be associated with FSGS in children. Mutation of the MYO1E gene led to disruption of the podocyte cytoskeleton. [ ] Other genetic forms of nephrotic syndrome continue to shed light on the pathogenesis of INS. Mutations in the developmental regulatory gene WT1 are associated with forms of congenital nephrotic syndrome associated with male pseudohermaphroditism, ( ), and (Frasier syndrome). Mutations (...) is reported in Asian children, (6 times the rate seen in European children). An increased incidence of INS is also seen in Indian, Japanese, and Southwest Asian children. Primary, SSNS is rare in Africa, where nephrotic syndrome is more likely to be secondary or steroid-resistant. These variations in ethnic and geographic distribution of INS underscore the genetic and environmental influences in the development of PNS. [ ] In children younger than 8 years at onset, the ratio of males to females varies

2014 eMedicine Pediatrics

80. Microphallus (Diagnosis)

defects and defects in testosterone biosynthesis (e.g. 17-beta hydroxysteroid dehydrogenase deficiency). [ ] The genitalia of individuals with LH-receptor defects vary from normal female-appearing to male-appearing with micropenis. Individuals with 17-beta hydroxysteroid dehydrogenase deficiency most often have female-appearing genitalia and, less often, ambiguous genitalia. [ ] Defects in peripheral androgen action include (failure of conversion of testosterone to DHT) and partial (PAIS) due (...) to an androgen receptor defect. However, most children with these conditions have varying degrees of incomplete labioscrotal fusion, resulting in hypospadias and genital ambiguity. [ , ] Lastly, genetic syndromes in which micropenis may be a feature include , , and , among others (see Causes). [ , ] Previous Next: Epidemiology Sex By definition, microphallus is an exclusively male condition. However, distinguishing between a male with micropenis and bilateral cryptorchidism and a female with clitoromegaly

2014 eMedicine Pediatrics

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