Personality and Psychopathology in Adults with Noonan Syndrome.

This is the first controlled study regarding personality and psychopathology in adults with Noonan syndrome (NS). Anxiety, depression, alexithymia and symptoms of Attention Deficit-Hyperactivity Disorder and Autism Spectrum Disorder, have been previously described in NS. More information regarding personality and psychopathology in NS could improve mental health care for this population. Therefore, scores on the Minnesota Multiphasic Personality Inventory-2-Restructured Form (MMPI-2-RF), a widely used self-report questionnaire of personality and psychopathology, were compared between patients with NS (n = 18) and matched, healthy controls (n = 18). Furthermore, correlations between MMPI-2-RF scores and alexithymia, measured by the Toronto Alexithymia Scale-20, were investigated. Patients with NS showed significantly higher scores, with medium effect sizes, on MMPI-2-RF scales reflecting infrequent responses (F-r), somatic and cognitive complaints (FBS-r and RBS-r), internalizing problems (EID), demoralization (RCd) and introversion (INTR-r), although the overall profile in both groups was within the non-clinical range. Alexithymia correlated with internalizing problems and negative emotionality in the patient group. In conclusion, patients with NS showed higher levels of introversion, which may predispose them to internalizing problems. These problems were indeed more frequent in patients with NS, especially higher levels of demoralization. Patients may benefit from psychological interventions aimed to decrease internalizing problems, introversion and alexithymia.

Effectiveness of a crossover prosthetic foot in active children with a congenital lower limb deficiency: an explorative study.

BACKGROUND: Children with lower limb prostheses cannot always keep up with their peers during active play. A pediatric crossover foot may be a promising prosthetic alternative for children engaging in high-intensity movements necessary for active play. OBJECTIVES: To compare children's walking performance, running performance, experienced competence, and cosmesis using their prescribed prosthesis compared with the crossover foot. STUDY DESIGN: Pretest-posttest study. METHODS: Children with lower limb amputation or deficiency were recruited. Measurements were taken at baseline with the prescribed prosthesis and 6 weeks later with the crossover foot. Walking speed, energy cost of walking, anaerobic muscle power, stair climbing speed, ankle power, and cosmesis were evaluated. RESULTS: Four children participated in the study. Two children had increased walking speed with the same energy cost, one child had decreased speed with increased energy cost, and one child had the same speed with decreased energy cost. Muscle power increased for three of the four children and ankle power increased for all children while using the crossover foot compared to the prescribed prosthesis. Two children reported knee pain or feeling excessive knee flexion when running with the crossover foot. One child reported negative feelings toward cosmesis of the crossover foot. CONCLUSIONS: This study suggests crossover foot may benefit active children by improving walking and running performance, and decreasing energy cost. However, knee pain reports or negative feelings toward the atypical design suggest the crossover foot may not be ideal for every child. Further research is needed to determine which pediatric users would benefit from this type of prosthetic foot. CLINICAL RELEVANCE: Children with lower limb deficiencies are active prosthetic users who often switch between low- and high-intensity movements in their daily activities. Therefore, they might benefit from a crossover prosthetic design. The preliminary findings of this study suggest the crossover foot (XF) may be a promising foot for active children.