Emotional and behavioral adjustment in 4 to 11-year-old boys and girls with classic congenital adrenal hyperplasia and unaffected siblings

Abstract

It has been suggested that atypical hormone environments during early development may contribute to subsequent development of psychopathology. Also, it has been suggested that individuals with the autosomal recessive genetic variant, classic congenital adrenal hyperplasia (CAH), might be at increased risk of psychopathology. The present study examined emotional and behavioral adjustment in young children with CAH and their unaffected siblings in the United Kingdom. The parent-reported version of the Strengths and Difficulties Questionnaire (SDQ) was employed to assess adjustment in children aged 4 to 11 years. There were 38 boys with CAH, 43 girls with CAH, 23 unaffected brothers, and 31 unaffected sisters. No differences in emotional or behavioral problems were found between boys or girls with CAH and unaffected same-sex siblings. In addition, affected and unaffected boys in the current sample generally did not differ from boys in the general population. However, compared with girls in the general population, girls with CAH had more difficulties related to conduct problems, hyperactivity/ inattention, and prosocial behavior, and unaffected sisters had more difficulties related to peer problems, conduct problems, and prosocial behavior. These findings suggest that both girls with CAH and unaffected sisters of girls or boys with CAH may be at increased risk of developing behavioral problems. Potential influences related to the early hormone environment, familial process, and social stigma are considered.

Sibling Adjustment to Childhood Chronic Illness: An Integrative Review

“…Alderfer et al (2015) noted that siblings of children with cancer had higher prosocial functioning when compared with siblings of healthy children. Contrarily, other research reports that HS had lower prosocial behavior (Kung et al, 2018) and lower social competence in school, extracurricular activities, and social relationships, due in part by daily anxieties concerning their ill sibling (Gan et al, 2018; Labay & Walco, 2004; Malcolm, 2013). Levels of social functioning differed according to gender and age, such that females and siblings who were younger than the ill child experienced greater prosocial adjustment and fewer social problems, as compared with males or siblings who were older than the ill child (Alderfer et al, 2015; Houtzager et al, 2005; Labay & Walco, 2004; Taylor et al, 2001).…”

“…Some research suggests the behavior of HS is often typical with no differences between them and their peers with a healthy sibling (Brennan et al, 2013; Houtzager, Grootenhuis, et al, 2004; Stephenson et al, 2017), whereas other studies found significant differences in behavioral scores of HS versus their peers without a chronically ill sibling. For example, a relatively common finding is that HS had elevated internalizing and externalizing behavioral issues with many of the scores being in the clinical range, as compared with siblings of healthy children (Gold, Mahrer, et al, 2008; Hollidge, 2000; Kung et al, 2018; Lobato et al, 2005; Wood et al, 2008). Similarly, Alderfer and colleagues (2020) reported one third of siblings had moderate to severe cancer-related traumatic stress.…”

Attention-Deficit/Hyperactivity Disorder Among US Children and Adolescents With Congenital Adrenal Hyperplasia

“…There is limited prior research evaluating ADHD symptoms or diagnoses among patients with CAH. Four studies have used small cohorts of pediatric patients with CAH identified in clinical settings [ 26 , 30 , 45 , 46 ]. One study of 54 pediatric patients in the United States, who were recruited as part of natural history study of patients with excess androgen at the National Institutes of Health Clinical Center, used a semistructured clinical interview to characterize mental disorders [ 46 ].…”

“…They reported that children in the CAH group did not score higher on the attention subscale compared with a mixed control group of unaffected relatives and individuals enlisted through a population registry [ 30 ]. Finally, a fourth study of 82 younger patients (ages 4-11 years) with classic 21OHD in the United Kingdom used the Strengths and Difficulties Questionnaire to assess subjects’ emotional and behavioral adjustment [ 26 ]. They found that girls with CAH, but not boys, had higher scores on the Hyperactivity/Inattention subscale compared with the general pediatric population, but not compared with unaffected sisters and female first cousins.…”

A Universally Accepted Definition of Gender Will Positively Impact Societal Understanding, Acceptance, and Appropriateness of Health Care

“…31 These data imply that circulating sex hormones influence toy selection and, by extension, may influence gender expression independently of societal influences. 32 Prevalence of People With Gender Incongruence Society is changing and there are now increasing numbers of people who selfidentify as gender nonbinary, gender nonconforming, and transgender individuals. In the transgender population, there are approximately 1.4 million people in the United States, or about 0.6% percent of the population, who self-identify as transgender people.…”