Joint range of motion limitations in children and young adults with spinal muscular atrophy

Objectives
To elicit descriptive data about limited joint range of motion (ROM) in subjects with type II or III spinal muscular atrophy (SMA) and to examine the relation between the number of motions with limited range and both age and functional ability.
Design
Descriptive cross-sectional study.
Setting
Neurologic pediatric outpatient clinic at a hospital in Taiwan.
Participants
Twenty-seven subjects with SMA type II (mean age, 9.8±6.5y) and 17 with SMA type III (mean age, 12.2±8.7y).
Intervention
Measurement with transparent goniometers of joint ROM bilaterally of the shoulder, elbow, wrist, hip, knee, and ankle.
Main outcome measures
The proportion of participants with each ROM limitation compared with all participants with the same SMA type, age distribution of the participants with each ROM limitation, mean range loss of each motion limitation, and the contracture index (risk index of joint contracture).
Results
Eighty-nine percent of the participants with SMA type II experienced knee extension limitation. Approximately 50% of the participants with both types of SMA had ankle dorsiflexion limitation. The motions of knee and hip extension and ankle dorsiflexion also had a relatively high contracture index. The number of motions with limited range positively correlated (P<.001) with age and upper-extremity functional grade (the higher the functional grade, the poorer the functional ability) for SMA type II.
Conclusions
We found varying degrees of joint ROM limitation. Certain motions were noted to be high risks for the development of contractures. This risk was higher mostly in younger children.

Effect of sodium selenite-enriched reperfusion solutions on rat cardiac ischemia reperfusion injury

Cardiac surgery often generates oxidative stress leading to ischemia reperfusion injury (I-R). Antioxidants have been shown to prevent this injury and have been added to cardioplegic solutions to assist in recovery. In this study, we tested the effectiveness of sodium selenite in protecting against ischemia reperfusion injury and investigated the mechanisms behind this protection. Hearts from male Wistar rats were subjected to ischemia reperfusion using the Langendorf model. Krebs-Henseleit perfusion solutions were supplemented with 0,0.1, 0.5, 1.0, and 10μM sodium selenite. Hearts were perfused for 30 min and then subjected to 22.5 min of global ischemia followed by 45 min reperfusion. Heart rate, ischemic contracture, end diastolic pressure, and developed ventricular pressure were monitored. At the completion of the experiment, hearts were homogenized and tissue extracts were assayed for glutathione peroxidase (GSH-Px) and thioredoxin reductase (Thx-Red) activity. Sodium selenite, at a concentration of 0.5 μM, demonstrated a protective effect on the recovery of cardiac function following I-R, as evidenced by a lower end diastolic pressure and enhanced recovery of rate pressure product. There was no beneficial effect observed in hearts perfused with 0.1 μM sodium selenite-supplemented buffer, whereas poorer functional recovery was observed in hearts perfused with 10 μM sodium selenite-supplemented buffer. The beneficial effect of sodium selenite was not mediated through increased activity of GSH-Px or Thx-Red. This study demonstrates that the addition of sodium selenite to reperfusion solutions, at an optimal concentration of 0.5 μM, assists in cardiac recovery following ischemia reperfusion.

Assessment of acute burn management in 32 low- and middle-income countries

BACKGROUND: The purpose of this study is to ascertain whether acute burn management (ABM) is available at health facilities in low- and middle-income countries (LMICs). METHOD: The study used the World Health Organization situational analysis tool (SAT) which is designed to assess emergency and essential surgical care and includes data points relevant to the acute management of burns. The SAT was available for 1413 health facilities in 59 countries. RESULTS: A majority (1036, 77.5 %) of the health facilities are able to perform ABM. The main reasons for the referral of ABM are lack of skills (53.4 %) and non-functioning equipment (52.2 %). Considering health centres and district/rural/community hospitals that referred due to lack of supplies/drugs and/or non-functioning equipment, almost half of the facilities were not able to provide continuous and consistent access to the equipment required either for resuscitation or to perform burn wound debridement. Out of the facilities that performed ABM, 379 (36.6 %) are capable of carrying out skin grafts and contracture release, which is indicative of their ability to manage full thickness burns. However the magnitude of full thickness burns managed was limited in half of these facilities, as they did not have access to a blood bank. CONCLUSION: The initial management of acute burns is generally available in LMICs, however it is constrained by the inability to perform resuscitation (19 %) and/or burn wound debridement (10 %). For more severe burns, an inability to perform skin grafting or contracture release limits definitive management of full thickness burns, whilst lack of availability to blood further compromises the treatment of major burns.