Congenital adrenal hyperplasia: Results of medical therapy on appearance of external genitalia

Kulshreshtha, Bindu; Khadgawat, Rajesh; Eunice, Marumudi; Ammini, AC

doi:10.1016/j.jpurol.2010.01.001

Cited by 13 publications

(5 citation statements)

References 19 publications

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“…precocious puberty. Similarly, a 2010 study in India used a case study approach to report the effect of steroid therapy during infancy and early childhood on the appearance of the external genitalia of girls with CAH (Kulshreshtha et al, 2010). It was unclear if the three girls in the study had a definitive diagnosis of CAH according to the standards used for diagnosis in the United States, as steroids were not initiated until after two months of age.…”

Section: Resultsmentioning

confidence: 99%

Management of Childhood Congenital Adrenal Hyperplasia—An Integrative Review of the Literature

Fleming¹,

Riper²,

Knafl³

2017

Journal of Pediatric Health Care

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Congenital adrenal hyperplasia (CAH) is an endocrine condition that requires parents to administer steroids up to three times daily, supplementing maintenance doses with oral or injectable doses during times of illness. The purpose of this integrative review was to explore the management, care and associated health issues for children with CAH as well as the family response to the condition. Four literature indexes were searched with 39 articles included. Four themes emerged: 1) health and development related issues; 2) effects of excess androgens; 3) life experience of CAH; and 4) managing and averting adrenal crisis. Families having a child with CAH face complex concerns related their child’s growth, the fear of adrenal crisis events, and the consequences of atypical genitalia for affected girls. Future studies should focus on interventions that provide guidelines to increase parental preparedness in managing adrenal crises as well as creating support systems for affected girls.

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Section: Resultsmentioning

confidence: 99%

Management of Childhood Congenital Adrenal Hyperplasia—An Integrative Review of the Literature

Fleming¹,

Riper²,

Knafl³

2017

Journal of Pediatric Health Care

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“…clitoroplasty, labioplasty and vaginoplasty. Postnatal steroid therapy seems to be associated with an improvement in the external appearance of genitalia in patients with less severe clitoromegaly [17] . Thus clitoroplasty may only be used in severe cases (Prader III–V) and be done at the same time as the common UGS repair.…”

Section: Surgical Managementmentioning

confidence: 96%

Disorders of sexual differentiation: II. Diagnosis and treatment

El‐Sherbiny

2013

Arab Journal of Urology

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ObjectivesTo provide a review and summary of recent advances in the diagnosis and management of disorder(s) of sexual differentiation (DSD), an area that has developed over recent years with implications for the management of children with DSD; and to assess the refinements in the surgical techniques used for genital reconstruction.MethodsRecent publications (in the previous 10 years) were identified using PubMed, as were relevant previous studies, using following keywords; ‘diagnosis and management’, ‘ambiguous genitalia’, ‘intersex’, ‘disorders of sexual differentiation’, ‘genitogram’, ‘endocrine assessment’, ‘gender assignment’, ‘genitoplasty’, and ‘urogenital sinus’. The findings were reviewed.ResultsArbitrary criteria have been developed to select patients likely to have DSD. Unnecessary tests, especially those that require anaesthesia or are associated with radiation exposure, should be limited to situations where a specific question needs to be answered. Laparoscopy is an important diagnostic tool in selected patients. The routine use of multidisciplinary diagnostic and expert surgical teams has become standard. Full disclosure of different therapeutic approaches and their timing is recommended.ConclusionsDiagnostic tests should be tailored according to the available information. Parents and/or patients should be made aware of the paucity of well-designed studies, as these conditions are rare. Unnecessary irreversible surgery should be postponed until a multidisciplinary experienced team, with the parents’ and or patients’ approval, can make a well-judged decision.

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“…A recent small study from French surgeons has suggested that prenatal DEX therapy could potentially be limited to the period of the partitioning window, during the time of urogenital cleavage, which would both reduce total fetal exposure to DEX, yet still facilitate easier surgical correction (99). Enlargement of the genital tubercle continues to occur in late pregnancy without ongoing antenatal treatment, but it is generally responsive to postnatal treatment (100,101).…”

Section: Prenatal Management Of Cahmentioning

confidence: 99%

MANAGEMENT OF ENDOCRINE DISEASE: Congenital adrenal hyperplasia due to 21-hydroxylase deficiency: update on the management of adult patients and prenatal treatment

Bachelot

Grouthier

Courtillot

et al. 2017

European Journal of Endocrinology

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Congenital adrenal hyperplasia (CAH) due to 21-hydroxylase deficiency is characterized by cortisol and in some cases aldosterone deficiency associated with androgen excess. Goals of treatment are to replace deficient hormones and control androgen excess, while avoiding the adverse effects of exogenous glucocorticoid. Over the last 5 years, cohorts of adults with CAH due to 21-hydroxylase deficiency from Europe and the United States have been described, allowing us to have a better knowledge of long-term complications of the disease and its treatment. Patients with CAH have increased mortality, morbidity and risk for infertility and metabolic disorders. These comorbidities are due in part to the drawbacks of the currently available glucocorticoid therapy. Consequently, novel therapies are being developed and studied in an attempt to improve patient outcomes. New management strategies in the care of pregnancies at risk for congenital adrenal hyperplasia using fetal sex determination and dexamethasone have also been described, but remain a subject of debate. We focused the present overview on the data published in the last 5 years, concentrating on studies dealing with cardiovascular risk, fertility, treatment and prenatal management in adults with classic CAH to provide the reader with an updated review on this rapidly evolving field of knowledge.

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Congenital adrenal hyperplasia: Results of medical therapy on appearance of external genitalia

Cited by 13 publications

References 19 publications

Management of Childhood Congenital Adrenal Hyperplasia—An Integrative Review of the Literature

Management of Childhood Congenital Adrenal Hyperplasia—An Integrative Review of the Literature

Disorders of sexual differentiation: II. Diagnosis and treatment

MANAGEMENT OF ENDOCRINE DISEASE: Congenital adrenal hyperplasia due to 21-hydroxylase deficiency: update on the management of adult patients and prenatal treatment

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