[in Japanese]

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[in Japanese]

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[in Japanese]

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[in Japanese]

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[in Japanese]

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[in Japanese]

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[in Japanese]

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[in Japanese]

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[in Japanese]

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[in Japanese]

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[in Japanese]

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[in Japanese]

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[in Japanese]

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[in Japanese]

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[in Japanese]

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[in Japanese]

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Hyponatremia is a common clinical condition in patients with severe motor and intellectual disabilities. In the 104 institutionalized patients at our center, there were 11 patients (10.6%) with chronic hyponatoremia. This study analyzed the hormone secretion, water and electrolyte balance, and underlying diseases in 9 patients whose detailed data were available. Seven out of 9 subjects showed the syndrome of inappropriate antidiuretic hormone secretion (SIADH), but only one subject presented with a serum ADH level above the measurement sensitivity level. All subjects took carbamazepine (CBZ), which could be responsible for the occurrence of SIADH without any excess ADH secretion, and it could also be involved in these hormone, water and electrolyte conditions. Regarding the underlying disease in these subjects, there were 4 subjects with hypothyroidism. However, no subject demonstrated cerebral salt wasting (CSW). CBZ is one of the underlying diseases most commonly associated with chronic hyponatremia in patients with severe motor and intellectual disabilities, and hypothyroidism could thus be related to its development.

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The present study aimed to clarify the motor functions/activities and sensory functions of patients admitted to Japanese public and private institutions for children with severe motor and intellectual disabilities (SMID). Data were obtained from a survey in the residential institutions over 22 years, beginning in 1988. The survey of motor functions/activities comprised the following four items: deformity/contracture, muscle tone, posture and mobility. Seeing and hearing functions were examined as typical sensory functions. Statistical analysis was performed on a categorized group of patients with SMID according to Oshima’s classification: <1 to 4>. The SMID group demonstrated a greater proportion of patients with severe motor impairments and activity limitations, and this showed a tendency to increase over time. A similar patient distribution was determined for sensory impairment. These results appear to reflect a recent advance in aging and severity from the aspect of motor functions/activities and sensory functions. Thus, careful observation is important and appropriate medical intervention and rehabilitation should be provided to prevent from progressive impairment and activity limitation.

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A vision specialist worked together with an occupational therapist to assess the objective refraction, visual acuity, and visual response of 50 institutionalized patients with severe motor and intellectual disabilities using the conventional methods. The results were compared with the Yokochi classification (revised Oshima classification) that classifies severity. Objective refraction tests revealed that 46% of the subjects had 2D or higher astigmatism. Quantitative visual acuity assessment could be performed on 30 subjects, for whom the mean decimal visual acuity was 0.088. Decimal visual acuity of less than 0.05 was only present in subjects with an intelligence level of A or B. Visual response was observed in 18 subjects that showed either optokinetic nystagmus, reflexive startle eyeblink or reactions induced by light perception, while 2 subjects did not exhibit any reactions induced by light perception. For both intelligence level and locomotive function level, ability to perform quantitative evaluation tended to increase as severity decreased. However, a relatively good grating acuity of 0.32 was seen in one subject with an intelligence level of A, the most severe level. This may be accounted for by differences in evaluation method, as assessment of intellectual disabilities is based on language comprehension, while grating ability is assessed by eye movement from visual perception. Cooperating with a specialist to evaluate visual functioning and exchange information may enable more accurate assessment of latent abilities that can then be reflected in severity classification and daily rehabilitation activities.

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The purpose of this study was to develop a scale for evaluating shared decision making regarding medical treatment by parents of children with severe motor and intellectual disabilities (SMID). In addition, this study evaluated the reliability and validity of the developed scale. A temporary scale consisting of 36 items was prepared and distributed to 811 parents. Among the 410 scales returned, valid responses were received from 262 parents of children under the age of 18 with SMID who had received respirator support, tracheotomy or tube-feeding in the past 5 years. As a result of exploratory factor analysis, 6 factors (“consideration about medical treatment from children’s perspective”, “circumstance facilitating participation in decision making”, “respectful attitude of medical staff for feelings and opinions”, “sharing values with each other”, “support in understanding professional opinion and information” and “support to be proactive about physical changes of children”) consisting of 22 items were extracted. Results of confirmatory factor analysis and the known-group technique indicated that the scale has adequate construct validity. Criterion-related validity was confirmed by the observation of significant correlations between all 6 subscales and the 4 subscales of the Decisional Conflict Scale. The Cronbach’s α for all 22 items was 0.946, with α values for each individual factor ranging from 0.81 to 0.89. Based on these findings, the scale for evaluating shared decision making regarding medical treatment by parents of children with SMID was confirmed to be reliable and valid.

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In patients with severe motor and intellectual disabilities, tube feeding with nasogastric intubation is usually selected if they are unable to take anything by mouth because of feeding disorder and swallowing disturbance. Complications that can arise from nasogastric intubation include incorrect insertion into the trachea, insertion into the esophagus and gastrointestinal tract ulceration. We recently encountered a patient with Fukuyama type congenital muscular dystrophy (FCMD) with gastric wall perforation associated with nasogastric intubation. Gastric wall perforation is a rare in complication arising from nasogastric intubation, but we consider that this patient’s underlying disease was FCMD, so dilatation of the stomach as a result of air swallowing may have induced gastric wall perforation.

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Patients with severe motor and intellectual disabilities (herein referred to as "SMID") often spend long periods in bed, and have a high risk of developing kidney stones. At this time, among children with SMID who have stayed at this hospital for an extended time, two cases of repeated urinary-tract infection and recognized kidney stones were targeted to consider the efficacy of cranberry juice (hereinafter referred as CBJ), which has been reported as being effective in preventing urinary-tract infection. Regarding treatment method, Cranberry UR50® was administered at room temperature through a gastronomy tube at 15:00 pm every day. As a result, in Case A, kidney stones observed on both sides had completely shrunk and disappeared 15 months after administration started, and in Case B, kidney stones observed on the right side had completely disappeared 7 months after administration started. It was thought that quinic acid, which is contained in large amounts in CBJ, changes into hippuric acid in the body and is released through the urine. As alkaline approaches to normal pH, and calcium phosphate, which is the main component of kidney stones, is released from the body, and it becomes more difficult to form kidney stones. In conclusion, CBJ is indicated to show a some efficacy in improving nephrolithiasis.

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A case of a 41 year old man with severe Lesch-Nyhan syndrome suffering from kidney stones, and continued bacteriuria and pyuria, is reported. The patient experienced weight loss due to repeated pyelonephritis since the age of 36 years, and was bedridden. Cranberry juice is reported to have urinary infection prevention effect, but there are conflicting opinions regarding the effect on persons with severe motor and intellectual disabilities. In this case, the effect of drinking 300ml/day of 100% cranberry juice was examined. As a result, the frequency of pyelonephritis significantly decreased. The rate of high fever accompanying pyelonephritis decreased, and the period from the start of antibiotic treatment until the fever declined also was shorter. The patient could also get up easier as the frequency of pyelonephritis decreased. The frequency of participation in nursing event increased, leading to improved QOL.

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Orbital cellulitis comprises acute, spreading pyogenic inflammation of the orbita, and may cause serious life-threatening complications including acute visual loss, cavernous sinus thrombosis, endophthalmitis,and brain abscesses. Sinusitis causes 80% to 90% of all cases of orbital cellulitis. On the other hand, patients with severe motor and intellectual disabilities (SMID) are susceptible to infections due to underlying diseases and the infections have a tendency to be prolonged and critical. We have reported orbital cellulitis complicated endophthalmitis in a patient with SMID, who had undergone an operation for an encephalomeningocele, and with severe mental retardation, disturbing behavioral conditions and hyperthyroidism. The laboratory findings indicated severe inflammation (white blood cell counts, 16400/mm3. CRP, 28.9 mg/dl ). In a mucus culture of the eye, G-streptococcus 1+ and multi-resistant staphylococcus aureus 3 colonies were detected. No sinusitis was observed in brain computed tomography. For medication, we performed intravenous drip infusion of antibiotics and gave antibacterial eye-drops, and improved the inflammatory data and ophthalmic symptoms gradually, although it was difficult because of the baseline diseases. Orbital cellulitis is a condition involving acute and serious inflammation of tissues of the orbit, therefore, in patients with SMID, great care should be taken regarding orbital infections such as in this case, in addition to respiratory infections due to swallowing dysfunction.

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The Higashinagano Hospital residency program for the doctor who is going to be concerned with severe motor and intellectual disability (SMID) in the future is based on the National SMID conference “Clinical Practice Program”. Our Hospital has accepted junior residents since 2005 and since, then, continues to offer both one-day or one-week courses. The hospital holds pre-program meetings in which our doctors, nurses, physical therapists, dietitians, welfare staff, school teachers and general affairs staff meet and decide on general program content then assign specific responsibilities for lectures, observations, skill acquisition projects, and so on. Past and present daily resident reports are also carefully reviewed and considered for improvements to current and future training. Use of the program has resulted in the development of a rich curriculum that allows our hospital, which has a limited number of doctors, to effectively manage clinical practice. Many department staffs have significant contributions to the program in order to advance promising doctors for SMID. We hope that more residents will participate in this program of SMID clinical practice in the future.

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[in Japanese]

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