Normal Adult Height in an Untreated Boy With McCune-Albright Syndrome Presenting With Precocious Puberty.

Background/Objective: We present a boy with McCune-Albright syndrome (MAS)-associated precocious puberty (PP) who achieved normal adult height without treatment. Case Report: The patient presented at 10 years of age with PP and fibrous dysplasia of the right humerus. Examination showed a height 148.7 cm, Tanner 2 pubic hair and 12-15 cc testes. The Bone age (BA) was 13 years with a predicted adult height of 175 cm vs. mid parental target height of 173 cm. Laboratory parameters were as follows: luteinizing hormone (LH) 0.745 mIU/mL (0.2-4.9 mIU/mL), follicle stimulating hormone (FSH) 0.933 mIU/mL (1.8-3.2 mIU/mL), testosterone 42 ng/dL (18-150 ng/dL), inhibin B 436.6 pg/mL (41-238 pg/mL) and AMH 36.1 ng/mL (45.26-191.34 ng/mL). The DNA testing result of tissue from the right humerus was positive for GNAS p. R201C mutation confirming a diagnosis of MAS. Pubertal progression with growth spurt occurred over the next 3 years: growth velocity (GV) 12 cm/y, testosterone 116 ng/dL, LH 0.715 mIU/mL and FSH 1.3 mIU/mL at 10.6 years; GV 10.3 cm/y, BA 13 to 13.6 years, testosterone 450 ng/dL, LH 1.7 mIU/mL and FSH 1.4 mIU/mL at 11.7 years; and GV 3.8 cm/y, BA 17 years, Testosterone 668 ng/dL and LH 4.2 µIU/mL at 13.3 years. Height was 171.2 cm. Discussion: PP is reported in approximately 15% of boys with MAS. PP leads to BA advancement and reduction in final adult height. Our patient achieved normal adult height without treatment in the absence of excess growth hormone. Conclusion: Boys with MAS and PP with slow BA advancement may achieve normal adult height without treatment even in the absence of excess growth hormone.

Effects of a Poetry Intervention on Emotional Wellbeing in Hospitalized Pediatric Patients.

BACKGROUND AND OBJECTIVES: The hospital is often a challenging and unfamiliar environment for families. Hospitalization can increase stress and anxiety among children and caregivers. In this study, we are the first to explore the possible therapeutic effects of poetry on hospitalized pediatric patients' emotional wellbeing. PATIENTS AND METHODS: Patients aged 8 to 17 years old admitted to the inpatient pediatric ward and their parents or guardians were eligible for inclusion. With the validated Pediatric Quality of Life Present Functioning Visual Analogue Scales, 6 items were measured before and after the poetry intervention for each participant: fear, sadness, anger, worry, fatigue, and pain in the present moment. The intervention itself consisted of poetry-based reading and writing exercises. Participants and parents also completed an open-ended qualitative survey on their experience. RESULTS: Data from 44 participants were analyzed. Using the Wilcoxon signed rank test, we showed that the poetry intervention had a statistically significant reduction in 5 of the 6 Pediatric Quality of Life Present Functioning Visual Analogue Scales symptom measures: fear (P = .021), sadness (P = .004), anger (P = .039), worry (P = .041), and fatigue (P < .001). Reduction in pain was not statistically significant (P = .092). Six coded themes emerged from qualitative analysis: the poetry intervention facilitated (1) happiness and (2) family involvement, was viewed as a (3) good distraction and (4) screenless activity, and cultivated (5) creativity and (6) self-reflection. CONCLUSIONS: The poetry intervention led to statistically significant reductions in fear, sadness, anger, worry, and fatigue but not in pain. The study reveals promising results and serves as a starting point for future investigations on the therapeutic impact of poetry on hospitalized pediatric patients.

Factors affecting opioid management for injured children after hospital discharge.

INTRODUCTION: Prescription opioid misuse is a national crisis. Injured children often receive opioid medication at hospital discharge, but the role these prescriptions play in the opioid crisis has not been fully elucidated. Whether these opioids are administered, the duration of severe pain requiring opioids, and what the final disposition of unused opioids is in this population remain unknown. METHODS: A survey of parent/guardian perceptions of their child's pain after injury, duration of opioid administration, opioid storage and disposal, and perceptions of opioid education was designed. During a 12-month period, parents of injured children admitted to an ACS Level 1 Pediatric Trauma Center were prospectively enrolled by convenience sample. Surveys were in two steps with an enrollment survey prior to discharge and a follow-up survey 7-10 days after discharge. RESULTS: Seventy of 114 (61.4%) enrolled parents/guardians completed follow-up survey. Of the 79.1% that reported an opioid prescription for their child, 92.5% filled it. Of those reporting on opioid usage, 10.4% never used the opioid, 75% used opioids <3 days, 12.5% 4-7 days, 2% >7 days. Of those who filled the opioid prescription, 83.7% reported having leftover doses. Reasons for discontinuing opioids included the child no longer had pain (87.2%), the child ran out of medication (5.1%), other (7.7%). Regarding storage, 53.3% reported utilizing an unlocked bathroom cabinet, and 81.3% unlocked kitchen space. Of those reporting unused opioids, 83.3% reported not disposing them, and 38.2% reported no plan for disposal. CONCLUSION: The majority of parents/guardians of injured children report resolution of severe pain requiring opioids within 72 h of hospital discharge, and virtually all by 7 days. The majority of injured children were prescribed a greater number of doses than they needed to treat their pain. Many parents/guardians store opioids in unsecure locations and a significant proportion report no plan to dispose of unused opioid doses. Further investigation is warranted to quantify and address the gap between pain control needs and opioid prescribing practices. The rate of unsecure storage and plan to retain unused opioids are potential targets for discharge opioid education. TYPE OF STUDY: Cross-sectional survey. LEVEL OF EVIDENCE: Level IV.

Severe Dental Disease as a Presenting Sign of Relapsed 6q24-Related Transient Neonatal Diabetes Mellitus.

Transient neonatal diabetes mellitus (TNDM) is a rare form of diabetes that presents in infancy and is characterized by intrauterine growth restriction and hyperglycemia without ketones on urinalysis. Patients are treated with insulin until remission, usually within the first year. Relapse to a permanent state may occur later in life, with a mean age of 14 years. The most common cause of TNDM is a chromosome 6q24 mutation that affects pancreatic ß-cell function. Reports of relapse have been limited. We describe a case of an adolescent female with TNDM due to 6q24 hypomethylation who relapsed at 15 years of age with severe dental disease as the presenting sign.