Behavioral Health Diagnoses in Youth with Differences of Sex Development or Congenital Adrenal Hyperplasia Compared with Controls: A PEDSnet Study.

OBJECTIVE: To evaluate the odds of a behavioral health diagnosis among youth with differences of sex development (DSD) or congenital adrenal hyperplasia (CAH) compared with matched controls in the PEDSnet database. STUDY DESIGN: All youth with a diagnosis of DSD (n = 1216) or CAH (n = 1647) and at least 1 outpatient encounter were extracted from the PEDSnet database and propensity-score matched on 8 variables (1:4) with controls (n = 4864 and 6588, respectively) using multivariable logistic regression. The likelihood of having behavioral health diagnoses was examined using generalized estimating equations. RESULTS: Youth with DSD had higher odds of a behavioral health diagnosis (OR, 1.7; 95% CI, 1.4-2.1; P < .0001) and neurodevelopmental diagnosis (OR, 1.7; 95% CI, 1.4, 2.0; P < .0001) compared with matched controls. Youth with CAH did not have an increased odds of a behavioral health diagnosis (OR, 1.0; 95% CI, 0.9, 1.1; P = .9) compared with matched controls but did have higher odds of developmental delay (OR, 1.8; 95% CI, 1.4, 2.4; P < .0001). CONCLUSIONS: Youth with DSD diagnosis have higher odds of a behavioral health or neurodevelopmental diagnosis compared with matched controls. Youth with CAH have higher odds of developmental delay, highlighting the need for screening in both groups.

Skin-to-Skin Care Is a Safe and Effective Comfort Measure for Infants Before and After Neonatal Cardiac Surgery.

OBJECTIVES: To determine the effect of skin-to-skin care on stress, pain, behavioral organization, and physiologic stability of infants with critical congenital heart disease before and after neonatal cardiac surgery. DESIGN: A baseline response-paired design was used, with infants acting as their own controls before, during, and after skin-to-skin care at two distinct time points: once in the preoperative period (T1) and once in the postoperative period (T2). SETTING: Cardiac ICU and step-down unit in a large metropolitan freestanding children's hospital. SUBJECTS: Convenience sample of 30 infants admitted preoperatively for critical congenital heart disease. INTERVENTIONS: Eligible infants were placed into skin-to-skin care for 1 hour with their biological mothers once each at T1 and T2. MEASUREMENTS AND MAIN RESULTS: Measurements of stress (salivary cortisol), pain and behavior state (COMFORT scale), and physiologic stability (vital signs) were assessed immediately before skin-to-skin care, 30 minutes into skin-to-skin care, and 30 minutes after skin-to-skin care ended.At both T1 and T2, infant pain scores were significantly decreased (p < 0.0001) and infants moved into a calmer behavior state (p < 0.0001) during skin-to-skin care as compared to baseline. At T1, infants also had significantly reduced heart rate (p = 0.002) and respiratory rate (p < 0.0001) and increased systolic blood pressure (p = 0.033) during skin-to-skin care. At both T1 and T2, infant cortisol remained stable and unchanged from pre-skin-to-skin care to during skin-to-skin care (p = 0.096 and p = 0.356, respectively), and significantly increased from during skin-to-skin care to post-skin-to-skin care (p = 0.001 and p = 0.023, respectively). Exploratory analysis revealed differences in cortisol reactivity for infants with higher baseline cortisol (> 0.3 µg/dL) versus lower (≤ 0.3 µg/dL) prior to skin-to-skin care. Infants with higher baseline cortisol at T2 experienced significantly reduced cortisol during skin-to-skin care (p = 0.025). No significant differences in demographics or baseline variables were found between infants in either group. CONCLUSIONS: Skin-to-skin care is a low-cost, low-risk intervention that promotes comfort and supports physiologic stability in infants before and after neonatal cardiac surgery.

Menstrual Bleeding as a Manifestation of Mini-Puberty of Infancy in Severe Prematurity.

We report 2 infants with severe prematurity who presented with uterine bleeding at age 6 months (approximately 2.5 months corrected for gestational age). Mini-puberty of infancy should be considered in the differential diagnosis of girls who present with uterine bleeding during the first 6 months of life.

Cortisol Correlates with Severity of Illness and Poorly Reflects Adrenal Function in Pediatric Acute Respiratory Distress Syndrome.

OBJECTIVE: To test the association between random cortisol and severity of illness in a "real-world" application of current guidelines. STUDY DESIGN: We performed a secondary analysis of a prospective observational cohort of acute respiratory distress syndrome (ARDS). Children with ARDS and vasopressor-dependent shock were identified and random cortisol levels before potential hydrocortisone initiation recorded. The cohort was dichotomized to cortisol < 18 and ≥ 18 µg/dL, and hydrocortisone use and outcomes compared. RESULTS: Of 357 children with ARDS, 155 (15 nonsurvivors; 10%) had vasopressors initiated with cortisol drawn before possible hydrocortisone use. Patients with cortisol < 18 µg/dL had lower severity of illness scores, fewer organ failures, and lower vasopressor scores (all rank-sum P < .05). No benefit was seen with hydrocortisone in either the entire cohort, or when dichotomized by a cortisol cutoff of 18 µg/dL. In patients with cortisol ≥ 18 µg/dL, hydrocortisone was associated with increased mortality after adjustment for either organ dysfunction or vasopressor score. CONCLUSIONS: In children with ARDS with vasopressor-dependent shock, low cortisol correlated with lower severity of illness. Random cortisol was a poor method of diagnosing adrenal insufficiency, and a strategy of hydrocortisone replacement for cortisol < 18 µg/dL did not target a population likely to benefit from hydrocortisone. Future guidelines should reconsider using random cortisol levels alone for assessing adrenal function.

Relationship between chronic transfusion therapy and body composition in subjects with thalassemia.

OBJECTIVE: To measure body composition in patients with thalassemia and explore its relationship to abnormal growth and bone mass. STUDY DESIGN: We conducted a cross-sectional, multicenter study. Fat, lean, and bone mineral density (BMD) were assessed with dual-energy x-ray absorptiometry. Medical history, food frequency, and physical activity questionnaires were conducted in 257 transfused patients with thalassemia (age, 23.7+/-11 years [mean+/-SD]; 51% male) compared with 113 non-transfused patients (21.3+/-13 years; 44% male). RESULTS: Subjects with thalassemia were leaner compared with healthy American subjects from National Health and Nutrition Examination Survey III data. Transfused subjects had a higher percentage of body fat compared with non-transfused subjects after controlling for age, sex, and ethnicity; 11.8% of non-transfused pediatric subjects were considered underweight, significantly lower than National Health and Nutrition Examination Survey data (P=.03). Hemoglobin level was positively related to lean mass (P=.008). Body fat and lean mass were positive predictors for both height and BMD z-scores after adjustment for transfusion status, age, sex, ethnicity, calcium intake, and physical activity (all P<.001). CONCLUSION: Although most adult patients with thalassemia had healthy body composition with rare obesity, young non-transfused patients appear at risk for being underweight. Optimizing physical activity and appropriate use of transfusion therapy may improve growth and bone health in these patients who are at-risk for being underweight.