Pain in Spinal Muscular Atrophy: A Questionnaire Study.

OBJECTIVE: This study aimed to reveal the chronic pain prevalence in spinal muscular atrophy (SMA) patients and identify the clinical characteristics of these patients with chronic pain. The pain status was also investigated in SMA patients with chronic pain. METHODS: This cross-sectional study was conducted between July 2018 and December 2018. SMA type II and type III patients in Japan were mailed a survey questionnaire. The survey items were chronic pain prevalence, clinical characteristics, and motor function. Patients with chronic pain also answered questions on various pain status parameters: pain intensity, frequency, duration, location using body map, and factors that exacerbated and relieved pain. RESULTS: The questionnaire recovery rate was 61.1%. Sixty-four type II (mean age 17.3 ± 11.7 years) and 22 type III (mean age 44.9 ± 21.6 years) patients were eligible for inclusion. The prevalence of chronic pain in type II and III patients was 40.6% and 40.9%, respectively. Type II patients with chronic pain were more likely to report the inability to sit without manual support than those without pain (p = 0.03). Pain intensity in SMA patients was mild, but pain usually occurred daily, for prolonged durations, most often in the neck, back, and lower extremities. Sitting and high physical activity exacerbated pain the most. CONCLUSION: The percentage of patients with SMA with chronic pain was high, at above 40%. Moreover, the pain experienced by patients with SMA was low in intensity but frequent and most common in the lower extremities.

Walking Attainment in Very Low Birth Weight Infants in Japan.

OBJECTIVE: To clarify the corrected age of walking attainment in very low birth weight infants by birth weight and gestational age, and determine perinatal factors affecting the delay in walking attainment. METHOD: This was a longitudinal study. We investigated walking attainment and perinatal factors in 145 very low birth weight infants without neurological abnormalities (mean birth weight 1019.3 ± 299.7 g, gestational age 29.0 ± 2.9 weeks). The study infants were stratified by birth weight (group A: <1,000 g, group B: 1,000 g≤, <1,500 g) and gestational age (group I: <28 weeks, group II: 28 weeks≤, <37 weeks) and were compared using unpaired t-tests. Furthermore, we examined the perinatal factors that affect the delay in walking attainment using multiple regression analysis. RESULTS: Of the walking attainment, infants in Group A were older than those in Group B (50th percentile, 15.8 vs. 14.7 months). Infants in Group I were older than those in Group II (50th percentile, 16.0 vs. 14.8 months). Using multiple regression analysis with walking attainment age as the dependent variable, the duration of mechanical ventilation was found to be significantly related. CONCLUSION: Very low birth weight infants with light weight and short gestational age have delayed walking attainment, and longer duration of mechanical ventilation increases the risk of delay.

Effects of continued positioning pillow use until a corrected age of six months on cranial deformation and neurodevelopment in preterm infants: A prospective case-control study.

BACKGROUND: Preterm infants have a high risk of cranial deformity resulting from external pressures. Such deformity is associated with delayed neurodevelopment. AIMS: We aimed to clarify the effects of continuous use of positioning pillows on cranial deformity and neurodevelopment in preterm infants. METHODS: This prospective case-control study was conducted between November 2018 and August 2019. The continuous use of a pillow was initiated after discharge from the neonatal intensive care unit, up to a corrected age of six months. Preterm infants weighing less than 1800 g without neurological abnormalities were included in the study. Patients were divided into two groups: non-pillow group (NP-group) and pillow group (P-group). The primary outcome was the Bayley Scales of Infant Development III (BSID-III) score. We compared asymmetrical cranial deformity and the BSID-III scores in the two groups at a corrected age of six months using the Fisher's exact test and unpaired t-test, respectively. RESULTS: There were 19 preterm infants (mean gestational age 32.5 ± 1.9 weeks, birth weight 1461.3 ± 244.7) eligible during the study period. The P-group (n = 11) showed asymmetrical cranial deformity at six months less frequently than the NP-group (n = 8) (p = 0.001, Fisher's exact test). Infants in the P-group had significantly higher scores on the BSID-III cognitive scales (95.0 ± 8.4 vs. 86.9 ± 2.6; p = 0.02, unpaired t-test) and fine motor scores on the motor scales (8.6 ± 2.2 vs. 6.6 ± 0.7, p = 0.02, unpaired t-test). CONCLUSIONS: Continuous pillow use in preterm infants is effective in reducing cranial deformity and improved cognitive and fine motor skills. TRIAL REGISTRATION: UMIN Clinical Trials Registry, trial no. UMIN000034400 (http://www.umin.ac.jp/ctr/index.htm).