We report the case of a 44-year-old woman with a partial 6-pyruvoyl tetrahydropterin synthase (6-PTS) deficiency, whose predominant clinical symptom was generalized dystonia with marked diurnal fluctuation. Dystonia was present in the eyelids, oromandibular region, trunk, and extremities (Meige syndrome plus double hemiplegia-like dystonia). A marked and sustained positive response to levodopa was observed. A molecular genetic study revealed a homozygous mutation (I114V) in the 6-PTS gene. This study indicates that genetic abnormality in the 6-PTS gene may be a hereditary dystonia disorder. We speculate that our patient has residual 6-PTS activity in the central nervous system, such as in the liver, and we suggest that residual, but insufficient production of tetrahydrobiopterin may play an important role in causing diurnal fluctuation of symptoms.
Context:There have been few reports investigating the effects of air transportation on patients with decompression illness (DCI).Aims:To investigate the influence of air transportation on patients with DCI transported via physician-staffed emergency helicopters (HEMS: Emergency medical system of physician-staffed emergency helicopters).Settings and Design:A retrospective medical chart review in a single hospital.Materials and Methods:A medical chart review was retrospectively performed in all patients with DCI transported via HEMS between July 2009 and June 2013. The exclusion criteria included cardiopulmonary arrest on surfacing. Statistical analysis used: The paired Student's t-test.Results:A total of 28 patients were treated as subjects. Male and middle-aged subjects were predominant. The number of patients who suddenly surfaced was 15/28. All patients underwent oxygen therapy during flight, and all but one patient received the administration of lactate Ringer fluid. The subjective symptoms of eight of 28 subjects improved after the flight. The range of all flights under 300 m above sea level. There were no significant differences between the values obtained before and after the flight for Glasgow coma scale, blood pressure, and heart rate. Concerning the SpO2, statistically significant improvements were noted after the flight (96.2 ± 0.9% versus 97.3 ± 0.7%). There were no relationships between an improvement in subjective symptoms and the SpO2.Conclusion:Improvements in the subjective symptoms and/or SpO2 of patients with DCI may be observed when the patient is transported via HEMS under flights less than 300 m in height with the administration of oxygen and fluids.
A 39-year-old female recreational diver, who developed cardiopulmonary arrest after diving, was transferred to our department 5 hours after the arrest by a helicopter emergency medical service. The diver performed two repetitive dives to a depth of 27 metres; 50 minutes bottom time for each dive. She had omitted the usual surface interval (resting near the surface) between the dives; the dive profile was otherwise unremarkable. On examination, she was in deep coma with dilative non-reactive pupil, hypotension supported by continous infusion of catecholamine, apnea sup-porting by mechanical ventilation and a widespread marbling rash (cutis marmorata) on her body and extremities (Picture). Whole body computed tomography revealed marked diffuse brain swelling and bilateral severe lung edema without sign of gas. She was diagnosed as clinical brain death due to Type II Decompression Sickness and died on the same day.Cutis marmorata is a distinct cutaneous manifestation of decompression sickness. It is easily recognized by its typical mottled, marbling violaceous appearance. It may start as an intense multifocal itching, followed by a generalized hyperaemia which in turn progresses to irregular dark violet or purple patches. The cutis marmorata is thought to be caused by vascular congestion triggered by vascular inflammation secondary to the development of intravascular gas bubbles. Cutis marmorata is usually transient and does not require any means of treatment. However, it is a warning sign of a more severe manifestation of decompression sickness so that careful follow-up is required.
Abstract:The Disaster Imagination Game (DIG) is a newly developed method for disaster drills based on the knowledge of the Commanding Post Exercises of the Japan Self Defense Force, which uses maps and transparent overlay. The Izunokuni City office held a local liaison meeting for disaster medical care. The related organizations shared all information and confirmed the cooperating system for the huge disaster. In addition to providing information of various hazards created by the huge Nankai trough earthquake, the DIG was performed by the participants. The worst case scenario for such a huge Nankai Trough earthquake would be for a magnitude 9-class earthquake to hit the central and western parts of Japan. After this procedure, the local citizen recognized the importance of self-help and mutual assistance. In addition, vitalization of self or public transportations was an increasingly important task because multiple roads could not be utilized due to quake liquefaction damage or landslides. After exchanging ideas and participating in the DIG, the participants deepened their understanding on relief activities. In addition, the DIG resulted in understanding between each participant and building face-to-face relationships, and the most important factor for disaster relief was accomplished.
Attending an MCLS course may help to improve outcomes in the face of an actual mass casualty incident. (Disaster Med Public Health Preparedness. 2018;12:437-440).
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