We report a case of a fetus with a prenatal diagnosis of classical congenital adrenal hyperplasia (CAH) due to 21-hydroxylase deficiency. Although CAH is typically assessed postnatally, this fetal case had multiple prenatal clinical assessments made feasible by an interdisciplinary CAH center. The approach facilitated the development and delivery of comprehensive and earlier care for the fetus, and the family living with this complex, congenital condition, with perinatology, endocrinology, genetic counseling, psychology, and urology involvement. As well, the addition of fetal MRI to standard ultrasound revealed significant deficits in the biparietal diameter, occipitofrontal diameter, and total intracranial volume of the fetal CAH brain. These early anomalies in the brain suggest that neurological comorbidities observed in older children and adults with CAH should be studied as early as prenatally, with the addition of fetal MRI to ultrasound potentially being useful for identifying and understanding prenatal anomalies in CAH.
Background: Youth with congenital adrenal hyperplasia (CAH) have a higher prevalence of obesity, early adiposity rebound, and increased fat mass. Unsuccessful dietary self-control could contribute to obesity, and understanding food-seeking behavior could therefore guide prevention. Dietary decision-making involves key brain regions such as the limbic system and the prefrontal cortex, which are associated with choice and reward. These regions (i.e., prefrontal cortex, amygdala, hippocampus) can be smaller in volume in CAH patients. However, little is known about dietary decision-making in CAH. We hypothesized that CAH youth would exhibit differences in dietary decision-making and aimed to study food choices in CAH youth compared to controls. Methods: 37 CAH youth (12.2 ± 3.1 y, 60% male, BMIz 1.6 ± 0.8) and 100 controls (11.7 ± 2.4 y, 57% male, BMIz 0.9 ± 1.2) completed a behavioral computer-based food choice task. They rated 30 high- and 30 low-calorie food cues for tastiness, healthiness, and liking. Food pairs discordant for taste and health ratings were generated, and youth were asked to choose the item they wanted to eat. Cursor-trajectory analyses measured area under the curve (AUC) and maximum deviation time (MDT), with successful choice trials evident when the healthier food was chosen. Based on individual ratings for food cues, β-coefficients for ratings predicting food preference were generated. Results: CAH youth and controls did not show differences in food ratings (P > 0.30 for all) or in the percentage of successful trials of total choice trials (P = 0.16). However, CAH youth showed larger mean AUCs compared to controls [T(135) = 2.15; P = 0.03] suggesting that they may experience more conflict and exert more cognitive effort in decision-making. CAH youth also had longer mean MDTs [T(102.3) = 2.59; P = 0.01] in successful choice trials, indicating a later time at which a final decision was made. β-health and β-taste predicting food preference did not differ between groups, and β-health was correlated with successful choice trials in both CAH (r = 0.38, P = 0.02) and controls (r = 0.48, P < 0.01). However, β-taste was negatively correlated with successful choice trials in controls only (r = -0.42, P < 0.01; CAH r = -0.22, P = 0.18). Conclusion: Although youth with and without CAH had similar perceptions of food, CAH youth may exert greater cognitive effort and experience more conflict in dietary decision-making. This could suggest that factors inherent to CAH such as abnormal neural pathways, or disease treatment, could affect the cognitive control of food choices in CAH youth.
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