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Macrodystrophia lipomatosa (MDL) is a rare congenital, nonhereditary anomaly characterized by overgrowth of all the mesenchymal elements, predominantly the fibroadipose tissue in a sclerotomal distribution commonly involving the median nerve territory in the upper extremity and plantar nerve territory in the lower extremity. It can be either static or progressive, with the former being the more common. MDL is usually present since birth and the affected digit/region increases in length and girth, and growth ceases after puberty. We discuss a rare case of ulnar nerve territory involvement that progressed to grow even after puberty. [ABSTRACT FROM AUTHOR]

The inclusion of health professionals who have some type of disability brings benefits, such as the increase in empathy that facilitates the relationship with patients. In this work, we present a case study on the inclusion of an undergraduate Dentistry student with upper limb agenesis. In order to improve the quality of life for people with upper limb agenesis, it is urgent to establish inclusion strategies in the educational field. This process included the use of assistive technologies to allow the manipulation of instruments used in the practice of dental clinical activities, enabling future Dentists with upper limb agenesis to have an equal education in relation to their academic peers. The case study presented in this work consists of the follow-up of G., a 23-year-old female Dentistry student. The developed adaptations were made using a low-temperature thermoplastic. The expert team that participated in this process was formed by three occupational therapists and three Dentistry professors. The process included several stages, being the last one the integration, in which training sessions were performed. The process was accessed using two main tools: quantitative questionnaires and qualitative interviews. After the proposed intervention, the volunteer presented a gradual evolution in her occupational performance with the use of most adaptations, promoting her functional training. Most adaptations allowed G. to perform the activities satisfactorily, with the precision inherent to these tasks, even with little training time. This case study demonstrates the high applicability of the proposed technologies. In order to improve the quality of life for people with upper limb agenesis, it is urgent to establish inclusion strategies in the educational field, as assessed in this study case The inclusion process of Dentistry students can include the use of assistive technology to allow the manipulation of instruments for the practice of clinical activities Our study suggests that the assistive adpatations promote a gradual evolution in the students' occupational performance, functional training and motor qualification [ABSTRACT FROM AUTHOR]

Holt-Oram syndrome comprises a rare spectrum of congenital cardiovascular and appendicular skeletal anomalies. However, only a few cases have reported lung involvement in Holt-Oram syndrome. We reported the rare case of a 1-year-old male child patient who presented with upper limb abnormalities and respiratory distress and was diagnosed with pulmonary agenesis and pulmonary arterial hypertension secondary to an atrial septal defect. [ABSTRACT FROM AUTHOR]

To examine whether accelerometry can quantitate asymmetry of upper limb activity in infants aged 3–12 months at risk for developing unilateral spastic cerebral palsy (USCP). A prospective study was performed in 50 infants with unilateral perinatal brain injury at high risk of developing USCP. Triaxial accelerometers were worn on the ipsilateral and contralesional upper limb during the Hand Assessment for Infants (HAI). Infants were grouped in three age intervals (3–5 months, 5–7.5 months and 7.5 until 12 months). Each age interval group was divided in a group with and without asymmetrical hand function based on HAI cutoff values suggestive of USCP. In a total of 82 assessments, the asymmetry index for mean upper limb activity was higher in infants with asymmetrical hand function compared to infants with symmetrical hand function in all three age groups (ranging from 41 to 51% versus − 2–6%, p < 0.01), while the total activity of both upper limbs did not differ. Upper limb accelerometry can identify asymmetrical hand function in the upper limbs in infants with unilateral perinatal brain injury from 3 months onwards and is complementary to the Hand Assessment for Infants. [ABSTRACT FROM AUTHOR]

Introduction: Despite the prevalence of congenital limb deficiencies (CLDs) occurring in around 7.9/10,000 births (Am J Med Genet A. 2011;155A:1225-1235), there is still a gap in the knowledge regarding the etiology of a large proportion of cases, and literature addressing this topic is sparser compared with other conditions. Objective: The aim of this study is to assess the literature surrounding the causes of CLDs and use this to suggest the area in which a potential gap in the information on the causes of CLDs lies. Study Design: This study is a literature review on articles published from the year 2000 onward. Methods: A search was conducted on five databases (CINAHL, Scopus, PubMed, Embase, Cochrane Library) and the ProQuest platform resulting in 18 articles to be discussed, after inclusion and exclusion criteria were applied and critical appraisals were completed. Results and Discussion: The main findings could be separated into four themes including genetics, environment, drugs, and vascular. Comparisons were made among similar literature, although within some topics, this was less effective, given the specificity and reduced volume of research. Positive correlations were seen in all studies; however, the biggest underlying factor for most causes seemed to originate from a vascular disruption. Conclusions: Future studies should focus on an underlying vascular disruption as a base for further research to attempt to find reasoning for the proportion of CLDs with an unknown cause. Further attempts should be made into creating a consensus on classifying and recording CLDs to ensure a more uniform approach to this topic worldwide, allowing comparisons to be drawn more easily. Clinical Relevance: Understanding relevant terminology is vital to ensure that families experiencing CLD receive accurate information about their child's condition. This understanding will also lead to improvements in the quality of recorded data, such as clinical notes and registry inputs. [ABSTRACT FROM AUTHOR]

Producing triggers to switch between modes of myoelectric prosthetic hands has proven to be difficult. We evaluated whether digital training methods were feasible in individuals with an upper limb defect (ULD), whether myosignals in these individuals differ from those of non-impaired individuals and whether acquired skills transfer to prosthesis use. Two groups participated in a 9-day pre-test–post-test design study with seven 45-minute training sessions. One group trained using a serious game, the other with their myosignals digitally displayed. Both groups also trained using a prosthesis. The pre- and post-tests consisted of an adapted Clothespin Relocation Test and the spherical subset of the Southampton Hand Assessment Procedure. After the post-test, the System Usability Scale (SUS) was administered. Clinically relevant performance measures and myosignal features were analysed. Four individuals with a ULD participated. SUS-scores deemed both training methods feasible. Three participants produced only a few correct triggers. Myosignals features indicated larger variability for individuals with a ULD compared to non-impaired individuals (previously published data [1]). Three participants indicated transfer of skill. Even though both training methods were deemed feasible and most participants showed transfer, seven training sessions were insufficient to learn reliable switching behaviour. Trial registration: The study was approved by the medical ethics committee of the University Medical Center Groningen (METc 2018.268). Switching between pre-programmed modes of a myoelectric prosthetic hand can be learned, however it does require training. Serious games can be considered useful training tools for trigger production in early phases of myoelectric prosthesis control training. In order to evoke transfer of skill from training to daily life both task-specificity and focus of attention during training should be taken into account. [ABSTRACT FROM AUTHOR]