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Childhood adversity (CA) may alter reactivity to stress throughout life, increasing risk for psychiatric and medical morbidity, yet long-term correlates of milder CA levels among high functioning healthy adolescents are less studied. The current study examined the prevalence and impact of CA exposure among a cohort of healthy motivated elite parachute unit volunteers, prospectively assessed at rest and at the height of an intensive combat-simulation exposure. We found significantly reduced gene expression levels in resting mononuclear cell nuclear receptor, subfamily 3, member 1 (NR3C1), and its transactivator spindle and kinetochore-associated protein 2 (SKA2), that predict blunted cortisol reactivity to combat-simulation stress among CA exposed adolescents. Long-term alterations in endocrine immune indices, subjective distress, and executive functions persist among healthy high functioning adolescents following milder CA exposure, and may promote resilience or vulnerability to later real-life combat exposure.

Background Pediatric surgical procedures involve traumatic stress that may cause psychological distress, leading to decreased adherence to continued surgical follow-up and delayed physical recovery. Risk factors for pediatric medical trauma, however, have not been studied enough. We aim to define the risk factors detectable during hospitalization in pediatric surgery and characterize children at risk of developing PTSD, in order to focus preventive interventions on these children. Methods The participants in this prospective study were parents of 235 children aged 1–13 years hospitalized in a pediatric surgical ward, who form a representative sample of patients of this age in the ward. They completed questionnaires measuring symptoms of psychological distress, 3–5 months after discharge. Results Higher parental stress, parental concerns regarding family social support, and parental concerns regarding sibling problems had a significant positive correlation with the children’s emotional distress measured 3–5 months after hospitalization. Among children aged 1–5 years, emergency (as opposed to elective) operation and a higher number of invasive procedures were also positively correlated with the children’s PTSS. Conclusions There is a need to develop measurements for identifying children at high risk for developing posttraumatic stress following surgical intervention; guidelines for developing such a screening instrument are outlined. Type of study Prognosis study (level of evidence – 1).

Background: Illness, surgery, and surgical hospitalization are significant stressors for children. Children exposed to such medical events may develop post-traumatic medical syndrome (PMTS, pediatric medical traumatic stress) that could slow their physical and emotional recovery. Objective: This study examined the relationship between the level of parental psychological resilience and the development of PMTS in young children. Method: We surveyed 152 parents of children aged 1–6 who were admitted to the pediatric surgery department. Parents completed questionnaires in two phases. In the first phase, one of the parents completed the Acceptance and Action Questionnaire (AAQ-ll) and the Parental Psychological Flexibility (PPF) Questionnaire. In the second phase, about three months after discharge, the same parent completed the Young Child PTSD (Post Traumatic Stress Disorder) Checklist (YCPC) and the UCLA (Los Angeles, CA, USA) PTSD Reaction Index for DSM-5 Parent/Caregiver Version for Children Age 6 Years and Younger Evaluating Post-traumatic Disorder. In addition, the parent completed a Posttraumatic Stress Diagnostic Scale (PDS) questionnaire to assess the existence of post-traumatic symptoms in the parents. Results: The findings indicate that (1) a parent’s psychological flexibility is significantly associated with the level of personal distress (r = −0.45, p &lt, 0.001), (2) a parents’ level of distress is significantly correlated with the child’s level of PTMS, and (3) a parent’s level of psychological flexibility is a significant mediating factor between the level of parental post-traumatic distress and the child’s level of PTMS. Conclusions: A parent’s psychological flexibility may act as a protective factor against the development of the child’s mental distress after hospitalization or surgery.

Individuals with PWS require marked caloric restriction and daily exercise to prevent morbid obesity. Lower energy expenditure, hypotonia, decreased muscle mass, and cognitive impairment make exercise challenging for this population. Exercise guidelines include resistance training as an important component. Myokine responses to resistance exercise may mediate beneficial metabolic effects. We aimed to determine if young PWS adults can perform a resistance exercise program and to measure myokine responses in PWS versus age- and BMI-matched controls. Each group included 11 participants (7M/4F). Ages and BMI for PWS and controls were 30.7 ± 4.6 versus 30.1 ± 4.3 years and 28.3 ± 4.3 versus 28.2 ± 4.2 kg/m2 , respectively. Glucose, creatine kinase (CK), lactate, and myokines were measured before, after, 30, and 60 min after completing eight resistance exercises. Myokines were assayed using a multiplex myokine panel (Merck Millipore). CK was lower in PWS versus controls (62 ± 16 vs.322 ± 100 U/L, p < .04). Peak lactate was 3.7 ± 0.7 in PWS versus 7.3 ± 0.7 mmol/Lin controls (p < .001). The increase in interleukin-6 was similar in PWS and controls (41 ± 16% and 35 ± 10%, respectively). Pre- and post-exercise levels of the six myokines assayed showed no consistent differences between the PWS and control participants. PWS young adults are capable of performing resistance/strength-building exercise. The lower CK and peak lactate levels in PWS may reflect decreased muscle mass in this population. Further studies are needed to determine optimal exercise regimens and assess the role of myokines incontributing to the metabolic phenotype of PWS.

Hyperphagia leading to severe obesity with increased morbidity and mortality is the major manifestation of Prader-Willi syndrome. Caring for these individuals in a home environment is challenging and stressful for caregivers and families. Residential hostels specifically for PWS adults offer programs of diet, exercise, and vocational opportunities, but long-term effects of PWS hostel living have not been reported. We studied long-term changes in body mass index (BMI) for PWS adults living in residential hostels compared with age-matched controls living with families at home. The study included all 34 individuals (18 men) aged >17 years with genetically confirmed PWS living in residential hostels. BMI was recorded at the time of yearly clinic visits and compared to 23 PWS adults (10 men) living at home. BMI on entering the hostel was 36.3 ± 11.0 kg/m2 and decreased to 27.0 ± 5.6 kg/m2 (p

Background Hospitalization and surgery are traumatic experiences that can result after traumatic stress symptoms (PTSS). Surgical interventions for congenital melanocytic nevus (CMN) can be very stressful, but their potential for causing PTSS has not been studied. We aim to determine prospectively whether children undergoing surgery for CMN develop PTSS and what are the specific risk factors for such an event. Objective The authors aim to determine prospectively whether children undergoing surgery for CMN develop PTSS and what the specific risk factors for such an event are. Methods Thirty children who were consecutively hospitalized in a pediatric surgery ward for CMN removal during the study period were recruited voluntarily. About 4 months after discharge from the hospital, the children and their parents were assessed for psychological distress. Results At the assessment 4 months after hospitalization, the children displayed a significant increase in symptoms of distress in comparison with baseline levels. Moreover, 33.3% met full post-traumatic stress disorder (PTSD) diagnostic criteria. The number of invasive procedures, family resources, and parental distress predicted 40% of the variance in PTSS, with parental distress predicting it most significantly. Conclusion The high prevalence of PTSS among children undergoing CMN removal and among their parents emphasizes the importance of actions for prevention and early treatment of psychological distress.

Introduction Surgical procedures involve traumatic stress. Children may develop chronic psychological distress and dysfunction after surgery, with consequent reluctance to comply with medical follow-up care. A literature review of this topic shows that it has been understudied. Our study aims to assess the frequency and characteristics of symptoms of persistent psychological distress in children following surgery, which have not been documented before, in order to promote its awareness and its early identification. Methods Parents of 79 children (aged 1–6) that were hospitalized in a pediatric surgical ward, comprising a representative sample, completed three validated questionnaires assessing their children's psychological symptoms 3–5months after the hospitalization. Results A significant portion of children suffer from psychological distress 3–5months after hospitalization. Moreover, 10.39% of the children exhibited symptoms of PTSD, and 28.6% of parents reported that the child's distress causes dysfunction. Additionally, our findings emphasize the parents' concerns regarding the child's behavior, function, and health following hospitalization. Conclusion Since a significant prevalence of hospitalization-related traumatic stress is documented, the awareness to it has to be improved, in order to reduce its frequency and increase adherence to medical follow-up care. Type of study Prognosis study. Level of evidence 1.

Background: Individuals with PWS require marked caloric restriction and daily exercise to prevent morbid obesity. Lower energy expenditure compared to BMI-matched controls along with hypotonia, decreased muscle mass, and cognitive impairment make exercise particularly challenging for this population. Exercise guidelines include resistance training as an important component. Myokine responses to resistance exercise may have beneficial metabolic effects. Objectives: Determine if young PWS adults can perform a resistance exercise program. Evaluate myokine responses to acute resistance exercise in young adults with PWS and compare responses with age and BMI-matched controls. Methods: Each study group included 11 participants (7M/4F). Mean±SD ages were 30.7±4.6 and 30.1±4.3 years for PWS and controls (NS). BMI values were 28.3±4.3 and 28.2±4.2 kg/m2 for PWS and controls, respectively (NS). Blood samples for glucose, creatine kinase (CK), lactate, hemoglobin A1c (HbA1C) and myokines were obtained before performing a program of eight resistance exercises lasting 45-60 minutes. Additional blood samples were drawn immediately after, and 30 and 60 minutes after completing the exercises. Myokines were assayed using a multiplex myokine panel (Merck Millipore). Paired t-test was used for comparing results for PWS vs controls. The unpaired t-test was used for comparing peak laboratory values with basal levels. Results: Basal levels (mean±SEM) of glucose, hemoglobin A1c, and lactate were similar for PWS and controls but CK was lower in PWS vs controls (62±16 vs 322±100 U/L, p

Introduction Since hospitalization can be a traumatic event for children, many of them may suffer from a cluster of chronic psychological and emotional difficulties called Pediatric Medical Traumatic Stress (PMTS). Although PMTS causes considerable functional impairment and psychological distress and may decrease the children's compliance with post-surgical care, awareness of this condition is low and thus not enough effort is made to prevent it. The objective of this study is to assess prospectively the prevalence and characteristics of PMTS in school-age children following hospitalization in a general pediatric surgery ward and in their parents, which has not been documented before. Materials Patients and Methods We recruited parents of 88 children aged 6 to 13 years old, hospitalized in a pediatric surgery ward and which form a representative sample of the children of this age in the ward. Three to five months after discharge from the hospital, the parents completed questionnaires measuring symptoms of psychological distress. Results About 26.4% of children displayed symptoms of PMTS, and 11.6% of parents suffered from posttraumatic stress disorder following their child's hospitalization. Moreover, we found a medium high positive correlation between the parents' level of distress and that of their child. Conclusion In view of the prevalence of PMTS among school-aged children following surgical intervention, it is necessary to promote increased awareness, preventive interventions, and early identification and treatment of this condition.

Background: As psychiatric consultants to pediatric wards, we are often asked whether to disclose to young children full information about the invasive medical procedures they face. To date, no studies have been published offering an evidence-based answer to this question. This prospective study examined whether sharing medical information with young children regarding invasive interventions correlates with the development of chronic post-traumatic stress three to five months after hospitalization. Method: The participants in this prospective study were parents of 151 children aged 3–13 who were hospitalized in a pediatric surgery ward. The sample was representative of the population hospitalized in this ward during that year. Independent of the study, parents of 104 children chose to share with them information regarding the procedure they were about to undergo, while parents of 47 children chose not to do so. t-Tests were used to assess the correlation between the children's exposure to medical information and their level of long-term post-intervention stress. Results: Findings show an inverse correlation between the children's exposure to medical information and their level of post-traumatic stress several months after their medical episode. The correlation is significant in both preschool children and school-aged children. Conclusions: We suggest the implementation of psychoeducation programs among both medical staff and parents in order to increase awareness of the importance of sharing medical information with young children facing medical challenges. Keywords: Pediatric Medical Traumatic Stress (PMTS), PTSD, Medical information, Surgical procedures

Prader–Willi syndrome (PWS) is a neurodevelopmental disorder characterized by an insatiable appetite, dysmorphic features, cognitive and behavioral difficulties, and hypogonadism. The heterogeneous reproductive hormone profiles indicate that some PWS women may have symptoms of hypoestrogenism, while others may potentially be fertile. We describe our experience in the assessment and treatment of hypogonadism in adolescents and adult females with PWS. The study population consisted of 20 PWS females, age ≥16 years (27.3 ± 7.9 years), followed in our clinic (12 deletion, 7 uniparental disomy, 1 imprinting-center defect). General physical examination, pubertal assessment, body mass index (BMI), gynecological examination, ultrasonography, bone densitometry, and hormonal profiles [FSH, LH, inhibin B, estradiol, prolactin, and TSH] were performed. The relevant assessed factors were: FSH and inhibin B, menstrual cycles (oligo/amenorrhea or irregular bleeding), ultrasound findings (endometrial thickness, uterine/ovarian abnormalities), BMI, bone densitometry, and patient/caregivers attitude. We classified seven women with inhibin B >20 ng/ml as potentially fertile. Following the assessment of the above factors, we recommended the individual-specific treatment; contraceptive pills, intra-uterine device, estrogen/progesterone replacement, and cyclic progesterone, in 3, 1, 4, and 1 patients, respectively. Four patients did not follow our recommendations due to poor compliance or family refusal. We recommended contraception pills for one 26-year-old woman with inhibin B and FSH levels 53 ng/ml and 6.4 IU/L; however, she refused treatment, conceived spontaneously and had an abortion. Guidelines for hormonal replacement therapy in PWS need to be tailored individually depending on physical development, hormonal profiles, bone density, and emotional and social needs of each PWS adolescent and adult. © 2013 Wiley Periodicals, Inc.

ObjectiveThe variable hypogonadism in Prader–Willi syndrome (PWS) has generally been attributed to hypothalamic dysfunction. Recent studies have documented primary testicular dysfunction in PWS males. Our aims were to characterize sexual development and reproductive hormones in PWS females and to investigate the etiology of hypogonadism.DesignA cross-sectional study.MethodsPhysical examination was performed on 45 PWS females (aged 6 weeks to 32 years) and blood samples were obtained for hormonal analyses.ResultsAge of onset and progression of puberty varied; most adults had incomplete sexual development. Spontaneous menarche was reported in four (aged 15–30 years) but all had subsequently developed secondary amenorrhea or oligomennorrhea. Anti-Mullerian hormone levels were within the normal range in all age groups. Inhibin B was consistently low or undetectable; only five women had levels in the low-normal range (20–54 pg/ml). LH was normal in most children, but low (ConclusionsPubertal development in PWS females, as in males, is characterized by normal adrenarche, pubertal arrest, and hypogonadism due to variable combinations of a unique primary gonadal defect and hypothalamic dysfunction.

Background Hypogonadism is a major feature of Prader-Willi syndrome (PWS), but clinical manifestations are variable. Sexual interests and behavior in this population have not been previously described. Objectives We studied PWS adolescents and young adults to assess 1) satisfaction with physical and sexual development, 2) frequency of romantic and sexual experiences, 3) aspirations and expectations regarding marriage, 4) possible relationships between sexual interests and hormone levels, and 5) the desire for hormonal replacement therapy. Methods The study population consisted of 27 individuals (13 males) ages 17-32 (mean 23.5) years with genetically confirmed PWS. Mean intelligence quotient (IQ) was 75 (range 50-100). We conducted structured interviews using questionnaires specifically designed for this study. Results There was a significant negative correlation between IQ and body image in both males and females. IQ showed a positive correlation with interest in dating and romantic activities. Approximately half of PWS males and females reported having been on a date and kissing romantically. All males and 64% of the females wished to be married. Seventy-seven per cent of PWS males wanted hormonal treatment to increase phallic size. We found no correlation between hormone levels and sexual interests. Only 43% of PWS females wanted hormonal medication to achieve regular menstruation. Conclusion Despite documented hypogonadism, PWS young adults are interested in sexual and romantic issues. The range of sexual activities and expectations is variable. Understanding specific sexual characteristics of each individual is important in order to offer proper anticipatory sexual guidance counseling and for appropriate recommendations for hormone replacement.

Recent studies challenge the assumption that hypogonadism in Prader-Willi syndrome (PWS) is due only to hypothalamic dysfunction.The aims of the study were to characterize sexual development and reproductive hormones in PWS males and investigate the etiology of hypogonadism.Physical examination and blood sampling were performed on 37 PWS males, ages 4 months to 32 yr.All had a history of undescended testes; age at orchiopexy ranged from 2 months to 6 yr. Pubertal signs were variable, but none achieved full genital development. Anti-Mullerian hormone (AMH) levels in PWS boys were near the lower limits of normal, decreasing from 44.4 +/- 17.8 ng/ml (mean +/- sd) in young children to 5.9 +/- 4.7 ng/ml in adolescents, similar to normal males. In contrast, inhibin B was consistently low (27.1 +/- 36.1 pg/ml) or undetectable in all age groups. In adult males, FSH levels were high (20.3 +/- 18.3 IU/liter), LH levels were normal (4.2 +/- 4.3 IU/liter), and testosterone levels were low (1.87 +/- 1.17 ng/ml). Only two adults had severe hypogonadotropic hypogonadism with undetectable levels of LH and FSH and high AMH levels (34.9 and 36.7 ng/ml), unlike the other nine adults with AMH levels 2.6 +/- 2.1 ng/ml. Androstenedione (1.06 +/- 0.30 ng/ml) and DHEAS (281.1 +/- 143.6 microg/dl) in adult PWS were normal.Pubertal development in PWS is characterized by normal adrenarche, variable hypothalamic dysfunction, and hypogonadism due to a unique testicular defect. Primary testicular dysfunction is a major component of hypogonadism in PWS.

Background/Aims: To investigate the etiology of hypogonadism in women with Prader-Willi Syndrome (PWS). Methods: Ten women aged 23 ± 5.5 years with PWS and 10 age- and BMI-matched controls were included. Blood samples were drawn and abdominal ultrasounds were performed on days 2–4 of spontaneous cycles or at random from amenorrheic women. Anti-Müllerian hormone (AMH), inhibin B (INB), gonadotropins, sex steroids, TSH, prolactin, ovarian volume and antral follicle count (AFC) in PWS women were compared with results from controls and the reference ranges. Results: Compared to controls, PWS women had lower INB (mean ± SD = 17.6 ± 12.8 pg/ml vs. 110.6 ± 54.5; p = 0.0002) and AMH levels (1.18 ± 0.86 ng/ml vs. 3.53 ± 2.42; p = 0.01). INB levels were exceptionally low in all PWS women, but individual AMH levels overlapped with the levels in the controls. Ovarian volume (mean ± SD = 3.7 ± 2.3 ml vs. 30.5 ± 28.8; p = 0.03) and AFC (6.4 ± 6.9 vs. 14.0 ± 8.2; p = 0.01) were lower in the PWS group compared to the controls. Three PWS patients had abnormally high follicle-stimulating hormone levels, while only 1 had hypogonadotropic hypogonadism. Conclusions: Our results suggest a unique follicular stage-specific insult in women with PWS. Thus, primary ovarian dysfunction is a major component of hypogonadism in PWS.