Mind-body therapy via videoconferencing in patients with neurofibromatosis: An RCT.

OBJECTIVE: To test, within a single-blind randomized controlled trial, the feasibility, acceptability, efficacy, and durability of a mind-body program (the Relaxation Response Resiliency Program for neurofibromatosis [3RP-NF]) vs an attention placebo control (Health Enhancement Program for NF [HEP-NF]), both delivered via group videoconferencing. METHODS: Sixty-three patients completed baseline assessments and were randomized. Primary outcomes were physical health and psychological quality of life (QoL), measured by the WHOQOL-BREF (World Health Organization QoL abbreviated instrument). Secondary outcomes were social relations and environment QoL, depression, anxiety, pain intensity, and pain interference. RESULTS: Sixty-three participants completed the intervention (100%) and 52 the 6-month follow-up (82.5%). Acceptability was 4.1 (5-point scale). Patients in the 3RP-NF showed greater improvement in physical health QoL (7.69; 95% confidence interval [CI]: 0.29-15.10; p = 0.040), psychological QoL (5.57; 95% CI: 0.17-11.34; p = 0.056), social relations QoL (10.95; 95% CI: 1.57-20.31; p = 0.021), environment QoL (8.02; 95% CI: 2.57-13.48; p = 0.005), and anxiety (-2.32; 95% CI: -3.96 to 0.69; p = 0.006) compared to those in HEP-NF, and gains were maintained at follow-up. Patients in the 3RP-NF did not improve more than those in HEP-NF on depression, with both groups showing improvement. Patients in the 3RP-NF with baseline pain ≥5 of 10 showed improvement in pain intensity from baseline to posttest (1.30; 95% CI: -2.26 to -0.34; p = 0.009) with effects maintained at follow-up; this improvement was not greater than that in HEP-NF. There were more treatment responders in the 3RP-NF group (p < 0.05). CONCLUSIONS: The 3RP-NF delivered via videoconferencing was highly feasible and accepted by patients, and resulted in sustained improvement in QoL. CLASSIFICATION OF EVIDENCE: This study provides Class II evidence that for patients with NF, a mind-body program is superior to an attention placebo control in improving QoL.

Evaluation of a respiratory rehabilitation program in children with scoliosis.

BACKGROUND: Thoracic scoliosis is a lateral curvature of the spine associated with restrictive lung defects, manifested by a decrease in respiratory function tests. We undertook this study to evaluate the effect of a respiratory rehabilitation program over lung function in children with scoliosis. METHODS: We carried out a prospective and deliberate intervention study including 25 consecutive patients, aged 6 to 18 years, diagnosed with thoracic scoliosis. The respiratory rehabilitation program was structured into two phases: institutional and private residence. Statistical analysis was carried out using descriptive parameters and paired t-test and Wilcoxon signed-ranks test. Spearman correlation was used to measure intensity of association among variables. Statistical significance was considered when p <0.05. RESULTS: Idiopathic scoliosis was present in 52% of patients, with right dorsal curvature in 72%. Cobb angle average was 50.6° ± 29.7°. Most importantly, we found a negative correlation between this angle on left curvature and lung function. Initially, the main respiratory symptoms were dyspnea with poor effort tolerance in 52%. After treatment, 88% of patients were asymptomatic and only 4% presented poor effort tolerance. Oxygen saturation and forced vital capacity percentage had a significant increment after the program. CONCLUSION: Respiratory rehabilitation has a positive effect on increasing pulmonary function of children with scoliosis.

Gastrointestinal haemorrhage complicating small bowel neurofibromatosis.

A case of acute gastrointestinal haemorrhage in a patient with systemic neurofibromatosis is presented. The bleeding was localized using mesenteric angiography and small bowel enema to a segment of ileum with multiple smooth muscle neurofibromata. The patient had a complete recovery after the resection and remained asymptomatic at 2 years follow up. Small bowel neurofibromatosis is a rare but important differential in gastrointestinal haemorrhage. Early diagnosis and surgery are vital for patient survival.