What Happens to Our Neuromuscular Patients in Adulthood: Pathway to Independence and Maximal Function.

BACKGROUND: Children with neuromuscular disorders regularly seek care from pediatric orthopaedic surgeons. These conditions can have a significant impact on the growth and development of children and their function and well-being as adults. Questions exist about the long-term outcomes of musculoskeletal interventions performed during childhood. METHODS: A search of recent literature pertaining to the musculoskeletal and functional consequences of cerebral palsy, spina bifida, Duchenne muscular dystrophy, and spinal muscle atrophy was performed. Information from those articles was combined with the experience of the authors and their institutions. RESULTS: Neuromuscular conditions can result in limb and spine deformities that lead to impaired physical function. Orthopaedic interventions during childhood can improve function and well-being and can be durable into adulthood. Unfortunately, many individuals with these conditions transition to adult health care that lacks the informed, collaborative multidisciplinary care they received as children. This can lead to unmet health care needs and a shortage of long-term natural history and outcome studies that would inform the care of children today. CONCLUSIONS: Adults with childhood-onset neuromuscular conditions need, and deserve, dedicated health care systems that include the best aspects of the care they received as children. Pediatric orthopaedic surgeons have a role in promoting the development of such systems and a responsibility to learn from their adult patients. LEVEL OF EVIDENCE: Expert Opinion.

[Clinical guidelines for the diagnosis and treatment of chronic thromboembolic pulmonary hypertension (Part 2)]. / Klinicheskie rekomendatsii po diagnostike i lecheniyu khronicheskoi tromboembolicheskoi legochnoi gipertenzii (II chast').

The paper gives current approaches to treating chronic thromboembolic pulmonary hypertension (CTEPH) from the document «Federal Guidelines for the Diagnosis and Treatment of CTEPH¼ approved at the Third Russian Congress on Pulmonary Hypertension on December 11, 2015. The guidelines had been elaborated to optimize the treatment of patients with CTEPH on the basis of an analysis of the data of the present-day registries and multicenter randomized clinical trials, national and international guidelines and consensus documents, and documents published in recent years. CTEPH is a unique form of pulmonary hypertension since it is potentially curable by surgical treatment. The paper presents indications for and contraindications to pulmonary thromboendartectomy; preparation for surgery; operating room facilities; the specific features of postoperative management and possible complications; and long-term RESULTS: In terms of therapy, in addition to non-pharmacological measures, the authors discuss maintenance and specific treatment options for CTEPH, balloon pulmonary angioplasty, and lung/heart-lung transplantation. In conclusion they propose a management algorithm in patients with CTEPH and requirements for its problem to the center of experts.