Behavioral assessment of females with congenital adrenal hyperplasia

Hamed, Sherifa A.; Attiah, Fadia Ahmed; Elaal, Refaat Fathy Abd; Fawzy, Mohamed

doi:10.1007/s42000-020-00232-8

Cited by 7 publications

(3 citation statements)

References 30 publications

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“…As previously mentioned, nearly half of our CAH participants were found to be in good adrenal control. Androgens play a role in human behavior ( 26 ) and elevated androgens have been associated with an increase in severe behavioral symptoms in girls with CAH ( 27 ). In our cohort of females with classical CAH, 69% (n=11) underwent genitoplasty and 6% (n=1) were planning to undergo later in the year.…”

Section: Discussionmentioning

confidence: 99%

Screening for Anxiety and Depression in Children with Congenital Adrenal Hyperplasia

Jacob

Lin‐Su

Catarozoli

et al. 2023

Jcrpe

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Objective: Congenital adrenal hyperplasia (CAH) is an inherited condition in which individuals require multiple daily doses of medication and are at risk for life-threatening adrenal crisis. The chronic nature and severity of CAH place children at risk for psychiatric morbidity. The aim was to assess the degree of anxiety and depressive symptoms in children with CAH. Methods: A cross-sectional cohort study of children (7-17 years) with CAH and their caregivers were recruited between May and December 2021. Children with hypothyroidism (HT) and their caregivers served as unaffected controls. Validated mental health questionnaires [Children’s Depression Inventory 2 Self Report-Short (CDI-2), Screen for Child Anxiety Related Disorders (SCARED), Patient Health Questionnaire modified for Adolescents (PHQ-A); self and proxy] were completed by participants at one clinic visit. Higher scores indicated greater symptoms of anxiety and depression. Results: A total of 60 children and 56 parents participated. Among the children 34 had CAH (68% female, mean age 11.41±2.5, CAH duration 8.5±4.1) and 26 had HT (73% female, mean age 12.7±2.9 years, HT duration 6.0±4.2 years). There was no increase in anxiety and depression symptoms in children with CAH compared to controls. In sub-analyses, children with CAH and controls reported a greater number of anxiety and depression symptoms than their caregivers on the SCARED and CDI-2, respectively. There was no association between adrenal control and the degree of anxiety or depression symptoms. Conclusion: Children with CAH do not have more symptoms of anxiety or depression compared to controls. Child and caregiver-proxy responses lack agreement, suggesting that children with CAH may continue to benefit from routine mental health evaluation, regardless of voiced caregiver concern.

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Section: Discussionmentioning

confidence: 99%

Screening for Anxiety and Depression in Children with Congenital Adrenal Hyperplasia

Jacob

Lin‐Su

Catarozoli

et al. 2023

Jcrpe

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“…Bone age and 17-IHP, testosterone, and androstenedione levels were independently associated with behavioral issues. Behavioral issues in these females were due to inadequate steroids and androgens (16).…”

Section: Discussionmentioning

confidence: 99%

Treatment of Hypertension in a Child With 11Beta-Hydroxylase Deficiency: A Case Report

Saffari

Arad

2022

JPR

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Introduction: Deficiency of 11-hydroxylase is clinically presented by external genitalia virilization in girls and precocious puberty in boys. Low renin hypertension occurs in both sexes. Early diagnosis and treatment of hypertension can prevent complications. Case presentation: We described a 4.5 years old girl of 46.XX, who presented with ambiguous genitalia at birth and hypertension later in follow-up. The patient had received the appropriate dosage of hydrocortisone and the level of 17-hydroxy progesterone was within the acceptable range but the hypokalemia persisted. Both hypertension and hypokalemia were normalized when spironolactone was added. Conclusion: Intermittent measurement of blood pressure is necessary for patients with 11β hydroxylase deficiency. In these patients, spironolactone is effective in treating mineralocorticoid-mediated hypertension and hypokalemia by blocking mineralocorticoid receptor.

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“…16 However, these children may prefer boys as playmates and exhibit aggressive behaviour. 17 The steroid dose needs to be balanced between adequate androgen suppression and drug toxicity. The consensus among most expert groups is a one or two-stage approach in infancy or delayed until puberty in female children with considerable virilization.…”

Section: Gender Reassignment and Gender Dysphoriamentioning

confidence: 99%

Perspectives on early sex assignment and communication with parents in children with disorders of sexual development

et al. 2022

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Disorders of sexual development are a heterogeneous group of disorders in which chromosomal, gonadal or anatomical sex development is atypical. The majority of these children are recognized at birth by ambiguous genitalia. Legal and societal pressures require the physician and parents to assign sex rapidly. Though sex assignment is undebated in several disorders of sexual development, many others need an individualized approach to gender-related concerns. Gender dysphoria is prevalent in disorders of sexual development, and early gender-defining surgeries have potentially lifelong consequences. We use two cases, one of partial androgen insensitivity and another of simple virilizing congenital adrenal hyperplasia to illustrate that in disorders of sexual development, the ethical management principles remain the same at their core despite the vast differences in phenotypes. Sex assignment should maximize functional, psychological and sexual needs. Sex assigned should coincide with gender identity. We propose that we manage such children with the mutual participation of the physician, parents and the child. Though the parents and the physician have the child's best interests when making a decision, the child's developing autonomy should be protected. The communication of the health provider to the parent must be honest if early sex assignment is difficult.

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Behavioral assessment of females with congenital adrenal hyperplasia

Cited by 7 publications

References 30 publications

Screening for Anxiety and Depression in Children with Congenital Adrenal Hyperplasia

Screening for Anxiety and Depression in Children with Congenital Adrenal Hyperplasia

Treatment of Hypertension in a Child With 11Beta-Hydroxylase Deficiency: A Case Report

Perspectives on early sex assignment and communication with parents in children with disorders of sexual development

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