[in Japanese]

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[in Japanese]

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We measured the bony landmark distance between the xiphoid process of the sternum and the left and right lateral anterior superior iliac spines on the body surface in 55 individuals with severe motor and intellectual disabilities (SMID), in order to determine the possibility of estimating the Cobb angle that shows the extent of single curve scoliosis from the left/right ratio. We also examined intra- and inter-tester reliability in bony landmark distance measurement with 20 individuals with SMID. A significant regression equation was obtained with regression analysis (p<0.0005). This suggests that the Cobb angle can be estimated from the left/right ratio in bony landmark distance. Both intra-tester reliability (ICC: left and right: >0.995) and inter-tester reliability (ICC: right: 0.98, left: 0.99) were high for measurement of bony landmark distance. These results indicate that easy and non-invasive assessment of the extent of scoliosis is possible in environments that do not allow for use of X-rays.

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Family empowerment has been considered as a crucial index in family support for severely disabled children. Semi-structured interviews were performed with 18 fathers who take care of severely disabled children at home, and it was revealed that there were 7 categories of fathers' roles in family empowerment: "role sharing with the child's mother in his/her cares and housework", "adjustment of work", "being an adviser for the child's mother", "coordinating a family trip, etc.", "communicating with service representatives", "interacting with administrative organizations", and "being involved in judicial (court) issues". The fathers had been sharing the care with mothers. They had been trying to finish their work on time or to change their jobs in order to play their roles. Sometimes they had been listening to the mothers' sufferings and discussing care plans with them. They had been also coordinating family trips, etc. in order to relieve stress of the entire family and make them feel refreshed. Outside the family, they had been exchanging opinions regarding services with service providers (professionals) or dealing with procedures for administrative or judicial issues. The results of this study suggested that in future there will be a need to provide supports for fathers to play their roles and to clarify their roles in various aspects.

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We examined 47 patients with severe motor and intellectual disabilities (SMID) using noninvasive positive pressure ventilation (NPPV) in the chronic phase during the eight-year period from April 2006 to March 2014. We compared associations with age and generation at the onset of treatment, circumstances leading up to treatment, duration of treatment, duration of use of a breathing apparatus, and life prognosis. Statistically significant differences were seen in the discontinuation rate that was higher in those who began treatment in early childhood and in life prognosis that was poorer in those who began treatment in the acute exacerbation phase. This suggests the need to consider that starting NPPV treatment in the chronic phase before acute exacerbation may improve life prognosis and that starting treatment when young may enable discontinuation in the future, in patients with SMID.

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To promote adequate support for people with severe motor disabilities who depend on medical care (Iryo-teki care), we investigated 1,259 such cases in Hyogo Prefecture and made a comparison with data obtained in 2007 by the Ethics Committee of the Japan Pediatric Society. Data were collected from 10 secondary medical care zones within Hyogo Prefecture from June to September, 2014. Medical interventions upon which participants depended included nasal feeding, gastrostomy, suction of nose and mouth, tracheostomy, oxygen inhalation, and artificial ventilation; these were identical with interventions identified in an investigation conducted by the Ministry of Education, Culture, Sports, Science and Technology. The total number of participants dependent on medical care was 1,259, including 933 who received home care and 326 who were hospitalized. The number of participants who were less than 20 years old increased about sixfold from 2007 to 2014, and there were even some cases in underpopulated areas. The number of cases with home oxygen therapy increased eightfold compared with that reported in 2007. Respirator-dependent cases totaled 299 in Hyogo Prefecture, where the incidence ratio of 0.05 out of 1,000 was higher than that in other areas of Japan.

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We examined eight cases of juvenile dentatorubropallidoluysian atrophy (juvenile DRPLA). Seven of the cases were familial and one had no family history and was suspected of being sporadic. All were childhood-onset and accompanied by intellectual impairment, epilepsy, and myoclonus as major symptoms as the disease progressed. All subjects developed severe motor and intellectual disabilities (SMID) by adulthood requiring intensive medical care. We separated the eight cases into onset at infancy, early childhood, or school-age and examined the clinical course including the period of no longer being able to walk independently or being bedridden, the period of epilepsy exacerbation, the period of requiring full tube feeding or laryngotracheal separation surgery, and the period of requiring an artificial ventilator due to exacerbation of breathing symptoms. The speed of progression of the clinical course in individuals diagnosed with juvenile DRPLA tended to differ with the age of onset. It may therefore be important to provide epilepsy management and medical care accordingly at the correct period as required based on close examination of each stage from childhood to adulthood.

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We report the case of a female patient with duodenal ulcer associated with ruptured pseudoaneurysm of the gastroduodenal artery and compression of the esophagus by the spine and aorta. A 42-year-old woman with congenital myopathy was under respiratory support, although self-feeding was possible. During hospitalization for pneumonia, life-threatening hemorrhagic shock occurred after a large amount of tarry stool was observed. The site of bleeding was not detected on gastroduodenal fiberscopy. The patient underwent a computed tomography (CT) angiogram, which identified the source of bleeding as a giant pseudoaneurysm of the gastroduodenal artery (GDA). In addition, rupture of the GDA pseudoaneurysm and extravasation of blood into the duodenal lumen were revealed. Transcatheter arterial microcoil embolization was performed immediately. After surgery, a proton-pump inhibitor was administered and total parenteral nutrition was initiated. Oral intake was gradually advanced, although much food residue was sucked into the nasal cavity. Chest CT and upper endoscopy showed that the upper esophagus was significantly compressed by the aorta and the scoliotic spine, leading to esophageal stenosis. After fasting, endoscopic gastrostomy was performed. When patients with severe motor and intellectual disabilities (SMID) develop a duodenal ulcer, the possibility of a GDA pseudoaneurysm caused by the ulcer must be considered. In addition, since there is a possibility in patients with untreated scoliosis that long-term bed rest and malnutrition exacerbates compression of the esophagus induced by the aorta and spine, posture and acute nutritional management of patients with SMID is crucial.

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The present study aimed to determine stress/eustress changes in developmental intervention of a female infant with a profound brain disorder who required daily medical care. Her level of consciousness was low and reading her emotions was difficult. Therefore, salivary amylase activity (SAA), which reflects stress/eustress changes in humans, was examined. The mean SAA was compared before, during, and after the session. The mean SAA during the session was lower than that before and after the session. The patient was in eustress during the session. SAA included outliers, which were shown by the boxplot method. The outliers were thought to be affected by environmental factors, except for developmental intervention. Therefore, the outliers were excluded and SAA was compared again. When outliers were excluded, the difference in mean of SAA became more obvious. In conclusion, outliers should be considered when analyzing SAA results.

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Special First Aid Trainings (IJLS: Immediate Jushin Life Support) were designed in order to handle emergency situations occurred at institutions for children or adults with severe motor and intellectual disabilities (SMID), whose underlying diseases are likely to be different from those observed at general hospitals. For children or adults with SMID, there is a high risk of developing emergency conditions with respiratory disorders. Hence, a respiratory support system using Jackson-Rees technique was incorporated in the training system as a standard method in order to perform effective resuscitation. In addition, insertion of a cuffed cannula was also included in the training system to deal with cannula-free patients or those with tracheo-brachiocephalic artery fistula. For removal of aspirated foreign bodies, back tapotement was selected as the standard method as many of the patients have deformed body trunks. Three hypothetical conditions: (1) cardiopulmonary arrest, (2) cyanosis in tracheostomy patients, (3) aspiration were established, and the trainings were performed in these conditions repeatedly until these are completely acquired. Furthermore, staff members were able to understand significance of the trainings by teaching newcomers. It can be expected that the first aid trainings will remarkably improve actions of staff members when patients' conditions change abruptly at institutions for children or adults with SMID.

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The objective of this study was to reveal how the degree of windswept hip deformity affect the difficulty assisting in diaper change in 59 children (adults) with severe motor and intellectual disabilities. In order to evaluate quantitatively, Goldsmith Index was used to measure the degree of windswept hip deformity. For evaluation of difficulty assisting in diaper change, a questionnaire survey was conducted on daily diaper change among staff members in charge of each subject to reveal whether or not they had felt difficulty assisting in diaper change, associated with joint mobility in lower limbs. According to the statistical analysis, it was suggested that the percentage of subjects with difficulty in diaper change associated with joint mobility in lower limbs would increase along with the increase in Goldsmith Index scores. The reference point where difficulty assisting in diaper change associated with joint mobility in lower limbs will be seen was considered as "Goldsmith Index Score of 15 or above". This suggests that Goldsmith Index could be an indicator to evaluate how development or progress of windswept hip deformity would affect the assistance in diaper change.

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