The clinical effects of combining postural exercises with chest physiotherapy in cystic fibrosis: A single-blind, randomized-controlled trial.

OBJECTIVES: This study aims to investigate the effects of postural exercises as an adjunct to chest physiotherapy program on respiratory function, exercise tolerance, quality of life (QoL), and postural stability in patients with cystic fibrosis (CF). PATIENTS AND METHODS: In this single-blind, randomized-controlled trial, 19 pediatric CF patients (11 males, 8 females; mean age: 9.36 years; range, 6 to 14 years) were randomly allocated to chest physiotherapy and postural exercise program (Group 1, n=10) or chest physiotherapy program alone (Group 2, n=9) between March 2017 and October 2017. Respiratory functions were assessed with pulmonary function tests, whereas exercise tolerance with the Modified Shuttle Test (MST), quality of life with the Cystic Fibrosis Questionnaire-Revised Child Version (CFQR), and postural stability with the Limits of Stability Test (LOS). All tests were performed before treatment and six weeks, three months, and six months after treatment. RESULTS: Respiratory functions were improved in both groups; however, these changes were not statistically significant. The MST increased after treatment in both groups (p<0.001 and p=0.003 respectively), without a significant difference between the groups. Emotional function and treatment difficulties subdomains in CFQR were significantly increased only in the group with postural exercises (p<0.05). CONCLUSION: The postural exercise program in addition to chest physiotherapy in pediatric CF patients whose postural changes were not taken place did not cause significant changes in respiratory function, exercise tolerance, and postural stability; however, it affected the emotional state well and improved the compliance with the treatment.

The effects of vest type dynamic elastomeric fabric orthosis on sitting balance and gross manual dexterity in children with cerebral palsy: a single-blinded randomised controlled study.

Purpose: To evaluate the effects of vest type dynamic elastomeric fabric orthosis on posture and balance during sitting and gross manual dexterity and to compare the efficacy of daily wearing time of 2 h versus 6 h.Method: Twenty-four children with cerebral palsy (CP) aged 3-9 years with GMFCS levels III and IV were randomised to either of three groups: (i) a control group who received only conventional exercise therapy, (ii) dynamic elastomeric fabric orthosis 2 h group who wore the orthosis for 2 h during therapy and dynamic elastomeric fabric orthosis 6 h group who wore the orthosis for 4 h in addition to the 2 h of wear along with therapy during hospital inpatient stay for 2 weeks. Children continued to use dynamic elastomeric fabric orthosis during the post-discharge period. The primary outcome measure was the Sitting Assessment Scale. The secondary outcome measurements were the sitting dimension of Gross Motor Function Measure, Box and Block Test and Parent Satisfaction Survey. Assessments were made before treatment, at post-treatment, at 1-month post-treatment, and at 3-months post-treatment. Sitting Assessment Scale and Box and Block Test were also assessed when immediately after wearing the orthosis. This trial is registered with Clinicaltrials.gov, under number NCT03191552.Results: All groups showed similar improvements except the control group which showed less improvement in Sitting Assessment Scale scores compared to the dynamic elastomeric fabric orthosis groups. Dynamic elastomeric fabric orthosis groups showed greater improvements compared to the control group in the Sitting Assessment Scale but not in the sitting dimension of Gross Motor Function Measure and Box and Block Test at post-treatment, at 1-month post-treatment and at 3-months post-treatment. When the dynamic elastomeric fabric orthosis groups (2 h versus 6 h) were compared, there were no significant differences in any of the assessments. The Sitting Assessment Scale and Box and Block Test scores also improved immediately after the patients put on the orthosis. At 1-month post-treatment, parents of children in the control group reported less satisfaction than parents of the children in dynamic elastomeric fabric orthosis groups.Conclusions: Dynamic elastomeric fabric orthosis vest has an immediate effect on the sitting balance and gross manual dexterity. It also provides improvements in posture and balance during sitting. Wearing dynamic elastomeric fabric orthosis vest for 2 h during therapy is as much effective as wearing it for 6 h in children with CP in addition to therapy to improve sitting balance.Implications for rehabilitationDynamic elastomeric fabric orthosis vest provides improvements in sitting balance when used in addition to conventional therapy in children with cerebral palsy.Wearing dynamic elastomeric fabric orthosis for 2 h and wearing dynamic elastomeric fabric orthosis vest for 6 h resulted in similar clinical outcomes.Dynamic elastomeric fabric orthosis vest has an immediate effect on sitting balance and gross manual dexterity in children with cerebral palsy.

Does stabilizing input pressure orthosis vest, lycra-based compression orthosis, improve trunk posture and prevent hip lateralization in children with cerebral palsy?

OBJECTIVES: This study aims to investigate whether the use of a lycra-based compression orthosis known as stabilizing input pressure orthosis (SPIO) vest improves trunk posture and hip lateralization in children with cerebral palsy (CP) and to compare the effects of two- and six-hours daily wear of the orthosis. PATIENTS AND METHODS: Between December 2013 and July 2015, a total of 24 children with CP (5 boys, 19 girls; mean age 61.1 months; range 35 to 105 months) with impaired trunk control were included in this single-blind, randomized-controlled study. All were randomized to either of the three groups as the control group (received only conventional exercise therapy), the SPIO 2-hour group (worn orthosis two hours during therapy), and the SPIO 6-hour group (worn orthosis four hours in addition to two hours of wear during therapy). The Sitting Assessment Scale (SAS), Cobb angle, kyphotic angle, and Migration Index were used to evaluate the trunk posture and hip lateralization before treatment and at six months after treatment. RESULTS: The SAS scores improved compared to baseline in all groups. The Cobb angle and kyphotic angle showed a significant decrease at six months after treatment only in the SPIO groups, while intra-group analysis of the Migration Index did not show a statistically significant difference. Changes in the radiographic assessments were similar among the groups, except for the kyphotic angles. The kyphotic angle showed less change in the control group. Comparison of the SPIO groups showed no significant differences in terms of the variables assessed. CONCLUSION: The SPIO vest improves the kyphotic posture, but not scoliosis and hip lateralization in children with CP with impaired trunk control when used in combination with the conventional therapy. Using the SPIO vest for two and six hours also yields similar outcomes.

Multiple sulfatase deficiency: A case series of four children.

Multiple sulfatase deficiency is biochemically characterized by the accumulation of sulfated lipids and acid mucopolysaccharides. The gene sulfatase-modifying factor 1 (SUMF1), recently identified, encodes the enzyme responsible for post-translational modification of a cysteine residue, which is essential for the activity of sulfatases. We describe clinical findings and mutation analysis of four patients. The patients presented with hypotonia, developmental delay, coarse face, ichthyosis, and hepatosplenomegaly. The diagnosis was made through clinical findings, enzymatic assays, and mutation analysis. We were detected to be homozygous for a novel missense mutation c. 739G > C causing a p.G247R amino acid substitution in the SUMF1 protein.

Infantile myofibromatosis in a three-year-old boy presented as subdural hematoma.

Infantile myofibromatosis, mostly developing at birth or in early infancy, is a rare clinical disorder characterized by myofibroblastic lesions. We report clinical, radiological and pathological features of a three-year-old boy who was diagnosed with infantile myofibromatosis originating from the occipital region contrary to radiological findings in advanced diagnostic studies.

Successful treatment of retroperitoneal giant cell-type malignant fibrous histiocytoma in a 5-year-old boy.

Malignant fibrous histiocytoma, usually seen in patients older than 10 years, is an aggressive soft-tissue sarcoma occurring mostly in the extremities and the trunk, but it is extremely rare in children. We report the clinical, radiological and pathologic features of a five-year-old boy who was diagnosed as a retroperitoneally originated malignant fibrous histiocytoma. The patient with unresectable mass was successfully treated with multidisciplinary approach, with chemotherapy, surgery and radiotherapy, by using combined chemotherapy consisting of vincristine, cisplatinum, adriamycin, cyclophosphamide, actinomycin D and dacarbazine.