Introduction: Pulmonary hypertension is a hemodynamic condition occurring rarely in pediatrics. Nevertheless, it is associated with significant morbidity and mortality. When characterized by progressive pulmonary vascular structural changes, the disease is called pulmonary arterial hypertension (PAH). It results in increased pulmonary vascular resistance and eventual right ventricular failure. In the vast majority of cases, pediatric PAH is idiopathic or associated with congenital heart disease, and, contrary to adult PAH, is rarely associated with connective tissue, portal hypertension, HIV infection or thromboembolic disease.
Areas covered: This article reviews the current drug therapies available for the management of pediatric PAH. These treatments target the recognized pathophysiological pathways of PAH with endothelin-1 receptor antagonists, prostacyclin analogs and PDE type 5 inhibitors. New treatments and explored pathways are briefly discussed.
Expert opinion: Although there is still no cure for PAH, quality of life and survival have been improved significantly with specific drug therapies. Nevertheless, management of pediatric PAH remains challenging, and depends mainly on results from adult clinical trials and pediatric experts. Further research on PAH-specific treatments in the pediatric population and data from international registries are needed to identify optimal therapeutic strategies and treatment goals in the pediatric population. 相似文献
Gastrointestinal stromal tumour (GIST) is a rare tumour of the gastrointestinal tract which does not generally originate in the rectum. The authors describe a case of a 70-year-old man who underwent an anterior resection of the rectum for a low-risk GIST. The patient was not given adjuvant chemotherapy with imatinib and is still disease-free 30 mo after surgery. The authors conclude that although rectal GIST is extremely uncommon, it should be included in differential diagnosis when a tumour in the rectum is detected. Biopsy of the tumour is essential, since this makes it possible to reach a sure preoperative diagnosis based on the immunohistological features of the CDl17 and CD34. Although complete surgical resection with negative tumour margins is the principal curative procedure for primary and nonmetastatic tumours, further studies are still needed for the determination of the most effective treatment strategy for patients with rectal GIST. 相似文献
Background: Children with cerebral palsy (CP) face particular challenges, e.g. daily pain that threaten their participation in school activities. This study focuses on how teachers, personal assistants, and clinicians in two countries with different cultural prerequisites, Sweden and South Africa, manage the pain of children in school settings.Method: Participants’ statements collected in focus groups were analysed using a directed qualitative content analysis framed by a Frequency of attendance-Intensity of involvement model, which was modified into a Knowing-Doing model.Results: Findings indicated that pain management focused more on children’s attendance in the classroom than on their involvement, and a difference between countries in terms of action-versus-reaction approaches. Swedish participants reported action strategies to prevent pain whereas South African participants primarily discussed interventions when observing a child in pain.Conclusion: Differences might be due to school- and healthcare systems. To provide effective support when children with CP are in pain in school settings, an action-and-reaction approach would be optimal and the use of alternative and augmentative communication strategies would help to communicate children’s pain. As prevention of pain is desired, structured surveillance and treatment programs are recommended along with trustful collaboration with parents and access to “hands-on” pain management when needed.
Implications for rehabilitation
??When providing support, hands-on interventions should be supplemented by structured preventive programs and routines for parent collaboration (action-and-reaction approach).
??When regulating support, Sweden and South Africa can learn from each other;
○?In Sweden, the implementation of a prevention program has been successful.
○?In South Africa, the possibilities giving support directly when pain in children is observed have been beneficial.
Purpose: The aim of the present study was to investigate the effectiveness of a prospective memory aid that combines smartphones with Internet-based calendars among community-dwelling patients with traumatic brain injury.
Method: An uncontrolled pre- and post-assessment design was employed to study the use of unmodified, low-cost, off-the-shelf smartphones combined with Internet-calendars as a compensatory memory strategy in community-dwelling patients with traumatic brain injury. Thirteen participants received a 6-week group-based intervention with pre-, post- and 2-month follow-up-assessments by questionnaires and by daily assessment of target behaviors for 2-week periods.
Results: Participants reported significantly fewer retro- and prospective memory problems on questionnaires after the intervention and at follow-up with large effect sizes. The performance of target behaviors, however, improved insignificantly with moderate effect sizes. There were no changes in quality of life or symptoms of emotional distress.
Conclusions: This study adds to a growing body of evidence that smartphones are a useful compensatory aid in rehabilitation of prospective memory that should routinely be considered in rehabilitation of traumatic brain injury patients.
Implication for rehabilitation
Smartphones are easy-to-use and accessible assistive technology for compensatory memory rehabilitation to most traumatic brain injury patients.
By using low-cost, off-the-shelf devices, the technology becomes available to a broader range of patients.
By combining smartphones with Internet-based and cross-platform services (e.g., calendars, contacts) the participants are less device-dependent and less vulnerable to data loss.
Smartphones should routinely be considered as compensatory aid in rehabilitation of prospective memory of traumatic brain injured patients.