OBJECTIVE: To investigate whether patients with severe motor and intellectual disability (SMID) have nutritional vitamin D and K insufficiencies and clarify the required vitamin supplementation.
METHODS: This prospective observational study enrolled Japanese adults with SMID receiving institutionalized care who underwent blood sampling between February 2020 and February 2022 during annual medical checkups. Serum vitamin K1 and 25-hydroxy vitamin D (25(OH)D) levels were measured to determine their relationship with serum uncarboxylated osteocalcin (ucOC) levels. Vitamin D and K intake was compared among tube-fed and oral-intake patients with SMID and control participants using corresponding serum levels.
RESULTS: The study included 124 patients with SMID (56 men and 68 women; mean age: 53.0 years) and 20 control participants. Serum 25(OH)D levels were significantly higher in the SMID group than in the control group and the oral intake SMID group than in the tube-fed SMID group. In the tube-fed SMID group, vitamin D intake was lower than the daily recommended intake and correlated with serum 25(OH)D levels. Daily vitamin K intake in the tube-fed group was lower than recommended but not correlated with serum vitamin K levels. Serum ucOC levels were significantly higher in the SMID group than in the control group. Tube feeding was significantly and positively correlated with serum 25(OH)D levels. Serum 25(OH)D levels were not correlated with serum vitamin K1 levels.
CONCLUSIONS: The SMID group had higher ucOC levels than the control group, possibly owing to daily vitamin K and D deficiencies. Vitamin D supplementation is recommended to decrease ucOC levels.

The purpose of this study was to investigate the psychological impact of various positionings in subjects with cerebral palsy (CP). The participants were 17 individuals with severe motor and intellectual disability due to CP. They began in a sitting position in their wheelchair, and were placed consecutively in prone or supine positions, with no intervals between placements. Physiological observations were made in each position, and included salivary α-amylase activity, pulse, percutaneous oxygen saturation, respiratory rate, learance or not of airway secretions, and occurrence or not of adverse events. Salivary α-amylase activity values were higher in the prone position than in the baseline and supine positions (p<0.05). Clearance of airway secretions was significantly more prevalent in the prone position than in the baseline and supine positions (p <0.05). The participants\' pulse was significantly lower in the supine and prone positions than in the baseline position (p<0.05). Greater prevalence of airway secretion clearance and significantly higher stress levels as indicated by saliva amylase were observed in the prone position than in the other two positions. Therefore, when such patients are placed in a prone position, close attention to airway management and the potential for psychological stress may be necessary.

Children with severe motor and intellectual disabilities (SMIDs) frequently and continuously receive enteral nutrition and medications and lack adequate exercise, which may lead to dysbiosis, an imbalance in the composition of the gut microbiota. However, studies on the composition of gut microbiota in children with SMIDs are limited. Therefore, we aimed to examine the characteristics of the gut microbiota in children with SMIDs.
16S rRNA gene sequencing was performed using fecal samples of 10 children with SMIDs, who received enteral nutrition through a gastric fistula or gastric tube (SMID group: median age, 10.0 years), and 19 healthy children (healthy control [HC] group: median age, 9.0 years). Microbial diversity, microbial composition, and abundance of butyric acid-producing bacteria were compared between the groups. Daily dietary fiber intake in the SMID group was evaluated using questionnaires.
The Shannon and Simpson indices (alpha diversity indices) were significantly lower in the SMID group than those in the HC group. Beta diversity analysis identified different clusters. Compared with the HC group, Clostridiales and butyric acid-producing bacteria were less abundant and Bacteroidales were more abundant in the SMID group. Dietary fiber intake in the SMID group was approximately two-thirds of the estimated average requirement for healthy Japanese children.
Children with SMIDs showed dysbiosis with alteration in the microbial diversity, which could partly be attributed to their low dietary fiber intake. Further studies, with the intervention of prebiotics, probiotics, and synbiotics, are warranted to improve dysbiosis in children with SMIDs.

OBJECTIVE: Social supports are critical to alleviate the psychological and physical burden of primary caregivers of children with disabilities. This study aims to (1) clarify how cerebral palsy in children affects caregiving burden of the mother, and (2) identify the social supports that can effectively reduce that burden.
METHODS: This is a cross-sectional study in which mothers of children with cerebral palsy completed questionnaires and provided data regarding their child\'s condition, family support, social support usage, degree of satisfaction with supports, and caregiving burden.
RESULTS: We analyzed responses from 1190 mothers. Support usage, particularly of home-visit nursing, home care, home-visit rehabilitation, and mobility support, was higher in severely burdened groups. However, the proportion of satisfaction with social support in groups with light or no burden were higher, particularly in home care, home-visit rehabilitation, training/treatment, and short stays. Mothers whose children have an intellectual disability and gross exercise ≥1 in addition to tube feeding or intravenous nutrition especially felt a strong sense of burden. The most effective measure in reducing mother\'s sense of burden was short stays.
CONCLUSIONS: Mothers with children who can move and have an intellectual disability felt more burden compared with mothers of bedridden children. The findings clarify that supports, such as home care and short stays, have a significant impact on reducing the mother\'s sense of burden.
CONCLUSIONS: Due to the large sample size, we believe that the results can inform efforts to increase social support for caregivers.

Nutritional metabolism is complex in pediatric patients with severe motor and intellectual disability (SMID), and therefore, appropriate estimation of the energy requirements is difficult. Focusing on ghrelin\'s role in energy metabolism regulation, we investigated plasma ghrelin levels in pediatric SMID patients and analyzed its nutritional significance as a regulatory marker of energy reserve.
Fasting plasma total, acyl, and des-acyl ghrelin levels in 40 patients with SMID, including cerebral palsy (CP) (n = 20) and muscular disease (MD) (n = 8), and healthy controls (n = 13) were investigated. The correlations of plasma ghrelin levels with anthropometry, blood nutritional markers, energy intake, and resting energy expenditure (REE) measured with indirect calorimetry were analyzed. A p value < 0.05 was considered significant.
SMID patients had significantly higher acyl ghrelin, and lower body mass index (BMI), z-scores of body weight (BW), body height and BMI, and albumin than controls. CP patients had significantly higher total and acyl ghrelin, z-score of the mid-upper arm circumference (MUAC), retinol-binding protein, transthyretin, creatinine, and glucose than MD patients. Total and acyl ghrelin in CP patients and des-acyl ghrelin in MD patients had significant negative correlations with MUAC and upper arm fat area. In CP patients, total and acyl ghrelin had significant positive correlations with REE/BW (kcal/kg), and total ghrelin was predictive of REE/BW (r2 = 0.625, p < 0.0001).
An increase in acyl ghrelin observed in SMID patients possibly indicates energy reserve deficiency. In CP patients, total and acyl ghrelin inversely reflected total body fat mass, resulting in strongly positive correlations with REE/BW. The measurement of plasma ghrelin may be useful to assess nutritional metabolism and energy reserve in pediatric SMID patients, such as CP and MD patients.

暂无摘要。

BACKGROUND: Few studies have investigated the hospital-based care utilization of children with medical complexity (CMC) in Japan. This study examined the frequency and differences in hospital-based care utilization for CMC according to the level of medical complexity (moderate and severe).
METHODS: Medical records of three pediatric tertiary hospitals in one prefecture were examined in 2014. We examined the number of outpatient visits and of admissions to the hospital for CMC in the 5 years after the introduction of home medical care.
RESULTS: Of 92 CMC, 55 had medical complexity that was moderate (CMC-moderate) and 37 had medical complexity that was severe (CMC-severe). The number of CMC who had medical care introduced at home had increased year by year, especially that of CMC <2 years old; the number of older CMC (i.e. 7-17 years old) had also increased in 2010-2014. The median total outpatient visits was 20 (IQR, 13-29 visits) for CMC-moderate and 20 (IQR, 17-26 visits) for CMC-severe in the first year. CMC-severe had significantly longer length of admissions in the 5 years than CMC-moderate. The number of total visits and admissions during the subsequent 4 years (from the second to the fifth year) was slightly decreased compared with the first year, but this was not significantly different.
CONCLUSIONS: CMC had high utilization of hospital-based care, and consistently utilized hospital-based care in the 5 years after the introduction of home medical care. Further study is needed to examine both hospital-based and home/community-based services use.

BACKGROUND: Severe motor and intellectual disability (SMID) patients cannot express their feelings with language. Understanding what they are thinking about or how they feel is thus difficult. This study focused on brain responses to hearing their own names to clarify the situation in these patients.
METHODS: We performed and analyzed electroencephalography (EEG) for six patients with SMID and eleven healthy subjects. All subjects were presented with auditory stimuli including calling the subject\'s own name (SON) and reading words. EEG was analyzed by time-frequency analysis, event-related spectral perturbation (ERSP) to detect EEG power changes caused by EEG amplitude, and inter-trial coherence (ITC) to investigate phase-locked changes.
RESULTS: ERSP results from healthy subjects showed significant theta power increases as a specific response to SON. While we could not identify a similar pattern in the responses of patients with SMID, analysis of ITC revealed that theta phase-locked activity increased in response to SON not only in all healthy subjects, but also in four patients.
CONCLUSIONS: These results indicate that theta phase-locked activity in some patients with SMID was strongly associated with SON, as in healthy subjects. Our study suggests the existence of specific neural markers that signal an attentional shift in patients upon hearing SON.

OBJECTIVE: Tracheo-innominate artery fistula (TIF) is a rare but life-threatening complication following tracheostomy or tracheoesophageal diversion (TED). Although successful surgical intervention for TIF has been reported, few studies have been performed in patients with severe motor and intellectual disability (SMID). Therefore, we aimed to analyze TIF in patients with SMID to clarify the clinical variables predicting the occurrence and adequate management for lifesaving of TIF.
METHODS: We retrospectively reviewed the records of patients with SMID undergoing surgical tracheostomy and TED between 2006 and 2012 and identified those with TIF. When TIF occurred, we obtained the clinical status and emergency management.
RESULTS: Of 70 patients who underwent tracheostomy or TED during the study period, three patients had TIFs; in one case, TIF was avoided by ligation of the innominate artery before TED. The incidence of TIF in those undergoing tracheostomy and TED was 2.3% and 7.4%, respectively. The interval between tracheostomy and TIF was 14-50 months.
CONCLUSIONS: Patients with SMID may have an increased risk of TIF. Prompt diagnosis and surgical intervention to control the bleeding is the only effective management at present.