Severe Motor And Intellectual Disabilities Research Articles

Alfacalcidol-Induced Kidney Injury in Patients with Severe Motor and Intellectual Disabilities.

Patients with severe motor and intellectual disabilities (SMID) often experience insufficient physical activity, leading to osteoporosis. The active form of vitamin D is commonly prescribed for the prevention or treatment of osteoporosis. We observed four cases of kidney injury believed to be associated with the administration of 1α-OH vitamin D (alfacalcidol) preparations. This study employed a case series design to investigate change in kidney function in SMID patients following administration or discontinuation of alfacalcidol. We retrospectively analyzed data of 23 SMID patients (sex: 10 males, 13 females; age range: 27 to 74 y), and assessed kidney function, serum calcium, and albumin levels. Data was grouped into A: 16 cases collected both before starting alfacalcidol administration and during alfacalcidol administration; and into B: 11 cases collected during alfacalcidol administration and after discontinuation of alfacalcidol administration. Of the 23 patients, four were assigned into both group A and group B. Of the 16 cases in group A, six showed ≥30% decreased kidney function. Of the 11 cases in group B, the median values of modified Cr-eGFR were 43.0 and 65.1 mL/min/1.73 m2, respectively (p=0.008), indicating a significant improvement in kidney function. It is essential for practitioners to understand that osteoporosis may ordinarily occur in SMID patients due to reduced bone stimulation. Thus caution must be exercised when administering active vitamin D preparations to this population, as they carry a risk of kidney organ damage despite having no direct effect on bone health.

Association Between Gut Microbiome Composition and Physical Characteristics in Patients with Severe Motor and Intellectual Disabilities: Perspectives from Microbial Diversity.

The human gut environment undergoes substantial changes as a host ages. This investigation centered on the gut microbiome diversity among patients with severe motor and intellectual disabilities (SMID), examining the association between the gut microbiome composition and physical characteristics with varying levels of diversity. Fourteen subjects were investigated, with physical and defecation status, blood biochemical test, gut microbiome profiling, and fecal metabolites used to divide the patients into a high-diversity group (HD, eight patients) and a low-diversity group (LD, six patients). Findings indicated that the microbiome of the LD group showed delayed maturation reminiscent of neonates and lactating infants. Analysis of the fecal bile acids (BAs) revealed a markedly diminished proportion of deoxycholic acid in the secondary BAs in the LD group, suggestive of inadequate conversion from primary to secondary BAs. Furthermore, the LD group presented with loose stools. The LD group exhibited a higher degree of physical severity, with all patients bedridden and fed via gastrostomy with only enteral formula received. The composition of the gut microbiome and BAs in the LD group was found to differ from those of healthy individuals and the HD group, indicating a potentially immature gut environment for these individuals.

Children and adolescents with severe motor and intellectual disabilities who underwent kidney transplantation.

Kidney transplantation (KT) in children and adolescents with severe motor and intellectual disabilities (SMID) has been a topic of controversy. A multicenter study in Japan showed that KT was not contraindicated for children with multiple handicaps, but no consensus has been reached on KT for patients with SMID. This study aimed to determine whether KT is a viable treatment option for children and adolescents with SMID. A single-center, retrospective study was conducted on children and adolescents with SMID who underwent KT. SMID was defined based on Oshima's classification. Clinical information was collected through a review of medical records. Of 453 children and adolescents who underwent KT between 1983 and 2023 in our institution, six (1.3%) patients with SMID were identified. One patient received KT twice. All patients underwent living KT. Five patients used medical devices, including gastrostomy and a ventriculoperitoneal shunt, prior to KT. Perioperative complications, including hemothorax related to central venous catheter insertion, ventilator-associated pneumonia, and common iliac artery thrombosis requiring graftectomy, occurred in three patients. One patient required vesicostomy owing to refractory urinary tract infection. There was no significant difference in the graft survival rate between patients with SMID and those without SMID. One patient developed graft failure and died after selecting conservative kidney management. Our study showed a favorable graft survival in children and adolescents with SMID who underwent KT. Although careful perioperative management and continued medical care are required, KT may be a viable option for these patients.

Assessing the Subjective Happiness of Parents of Children With Severe Motor and Intellectual Disabilities Receiving Home Care.

Home care for children with severe motor and intellectual disabilities (SMID) is challenging for parents because it is highly intensive and long-lasting. The pursuit of happiness is an essential goal for everyone. However, only a few studies have focused on the happiness of families with such children. The study aimed to examine the subjective happiness of parents of children with SMID receiving home care and identify the factors associated with their happiness. We conducted a cross-sectional online questionnaire-based survey of 23 parents of children with SMID and nurses with children without disabilities as controls at Tottori University Hospital,Yonago, Japan from July 1 to August 31, 2023. We set the subjective happiness scale (SHS) scores as the outcomes. We used the Mann-Whitney U test to compare the SHS scores between the two groups. Moreover, we extracted the clinical and demographic factors affecting the SHS scores of parents of children with SMID using univariate linear regression analysis. We obtained responses from 12 parents with SMID and 105 controls. The average SHS scores of parents with SMID and controls were 4.8 and 4.7, respectively, and both groups did not differ significantly. Univariate analysis showed that parental male sex and the presence of a tracheostomy were negatively associated with the SHS scores of parents. The SHS scores did not differ significantly between parents with SMID and controls. However, more attention seemed necessary for fathers and parents of children who have undergone tracheostomies. Given the exploratory nature of this study and its small sample size, larger-scale investigations are warranted. Additionally, qualitative research conducted after establishing trustful relationships could provide further insights.

Radiofrequency echographic multi-spectrometry-based measurement of bone mineral density in patients with severe motor and intellectual disability: An opportunity for patients with severe scoliosis and hip dislocation

PurposeRadiofrequency echographic multi-spectrometry (REMS) is an ultrasound technology currently used for the densitometric evaluation of osteoporosis and has been validated against dual-energy X-ray absorptiometry. However, the use of REMS for bone densitometry in patients with severe motor and intellectual disabilities (SMID) remains to be reported. This study aimed to investigate whether REMS technology can be used for densitometric evaluation of osteoporosis in patients with SMID with hip dislocation and severe scoliosis. MethodsSixty-five patients with SMID, who resided in a long-term care facility and received comprehensive medical and rehabilitation care, underwent REMS scans of the femoral neck and/or lumbar spine. Data regarding anthropometric parameters (height and weight), bone mineral density (BMD), clinical diagnostic classification, physical ability, presence of scoliosis and hip dislocation, and frontal radiographs of both hip joints were obtained. ResultsWe included 29 men and 34 women (mean age: 52.6 years). All patients underwent successful scanning at either the femoral neck (82.5 %) or lumbar spine (95.2 %). BMD measurements obtained using REMS revealed low values, with a mean BMD, T-score, and Z-score of 0.67 g/cm2, −2.39 standard deviation (SD), and − 1.38 SD, respectively, at the femoral neck and 0.66 g/cm2, −2.70 SD, and − 1.87 SD, respectively, at the lumbar spine. The average Cobb angle of the lumbar spine was 34.0°; furthermore, dislocation rates did not significantly differ between those with and without successful BMD measurements (p = 0.073). Lumbar BMD T-scores were significantly correlated with femoral neck BMD T-scores (p < 0.001, r = 0.530). ConclusionAll patients with SMID were able to undergo measurements of either spinal or femoral neck BMD; furthermore, 77.7 % of the patients underwent measurements at both the lumbar spine and femur. Our data suggest that REMS is useful for measuring BMD in patients with SMID who are residing in institutions.

Social support needs of caregivers rearing children with severe motor and intellectual disabilities at home in Japan.

In Japan, recently, the number of children with severe motor and intellectual disabilities (SMID) is steadily increasing. Caregivers such as parents and family members are struggling with how to live with their children at home and in the community after discharge. The current study aimed to explore the social support needs faced by caregivers while rearing children with SMID in order to identify effective means of social support in Japan. We conducted a cross-sectional survey of the primary caregivers of children with SMID at home through special-needs elementary, junior high, and senior high schools nationwide, using a self-administered, anonymous questionnaire to investigate the actual social support needs of the caregivers. All statements of social support need were coded using Krippendorff content analysis. Questionnaire returns were obtained from 1,176 families, and the descriptions of 1,173 families were included in the analysis. The results of the analysis showed that the needs of the caregivers consisted of seven categories. The social support needs expressed by the caregivers are necessary findings for Japan today, both for the soft side, such as the development of local systems and regulations to support these families, and for the hard side, such as the increase and improvement of facilities and equipment.

Vitamin D Supplementation at a Dose of 10 µg/Day in Institutionalized Children with Severe Motor and Intellectual Disabilities.

Vitamin D (VD) deficiency can lead to health-related consequences. This study determined the effects of VD administration in VD-deficient children with severe motor and intellectual disabilities (SMID). Twenty-eight subjects were included. Among them, 25 subjects with parental consent for VD administration were given 10 µg/day (400 IU/day) of VD in April 2021. Serum 25-hydroxyvitamin D [25(OH)D] levels were measured at least 30 days after the start of VD administration. The total VD intake, serum 25(OH)D levels, and ultraviolet (UV) exposure before the blood tests were investigated. The results showed that the median serum 25(OH)D levels were 8.7 ng/mL (4.3-17.2) and 24.0 ng/mL (7.8-39 ng/mL) from March to May in 2020 and 2021, respectively. Among the 25 subjects, 22 with UV exposure had >20 ng/mL serum 25(OH)D level, and 2 without UV exposure had <20 ng/mL serum 25(OH)D level. Three subjects who did not receive VD supplementation had <20 ng/mL serum 25(OH)D level. Taken together, VD supplementation (10 µg/day) is effective in children with SMID in institutional care. Moreover, it may be sufficient for children with UV exposure, but not for those without.

The Effect of Vitamin K2 Supplementation on PIVKA-II Levels in Patients with Severe Motor and Intellectual Disabilities Undergoing Long-Term Tube Feeding.

Nutritional support is essential for patients with severe motor and intellectual disabilities (SMID) to ensure the smooth provision of medical care. These patients often require long-term tube feeding with enteral formulas, potentially leading to deficiencies in vitamins and trace elements. Additionally, frequent antibiotic use for infections often disrupts gut microbiota, inhibiting vitamin K2 production by intestinal bacteria. We assessed the serum protein induced by vitamin K absence or antagonists-II (PIVKA-II) and undercarboxylated osteocalcin (ucOC) levels to assess the vitamin K status in 20 patients with SMID (median age: 44.1 years, 11 men and 9 women) undergoing long-term tube feeding for durations ranging from 3 to 31 years. Thirteen (65%) and nine (45%) patients had elevated PIVKA-II (<40 mAU/mL) and serum ucOC levels (reference value < 4.50 ng/mL), respectively. Dietary vitamin K1 intake did not differ between patients with and without elevated PIVKA-II levels. Vitamin K2 supplementation for 3 months decreased serum PIVKA-II levels near those within the reference range. Approximately half of the patients with SMID on tube feeding had subclinical vitamin K deficiency. Further studies are needed to ascertain if long-term vitamin K2 supplementation effectively prevents vitamin K deficiency-induced hypercoagulation, osteoporosis, and vascular calcification in patients with SMID.

Changes in Fecal Gut Microbiome of Home Healthcare Patients with Disabilities through Consumption of Malted Rice Amazake.

The aim of this study was to investigate changes in gut microbiome both during and after the consumption of malted rice amazake (MR-Amazake), a fermented food from Japan, in home healthcare patients with disabilities including patients with severe motor and intellectual disabilities (SMID). We monitored 12 patients who consumed MR-Amazake for six weeks, investigating them before and after the intervention as well as six weeks after the end of intake to compare their physical condition, diet, the type of their medication, Constipation Assessment Scale (CAS), and an analysis of their comprehensive fecal microbiome using 16S rRNA sequencing. Their constipation symptom significantly alleviated and principal coordinates analysis revealed that 30% of patients showed significant changes in gut microbiome after MR-Amazake ingestion. Furthermore, Bifidobacterium was strongly associated with these changes. These changes were observed only during MR-Amazake intake; the original gut microbiome was restored when MR-Amazake intake was discontinued. These results suggest that six weeks is a reasonable period of time for MR-Amazake to change human gut microbiome and that continuous consumption of MR-Amazake is required to sustain such changes.

Relationship between energy intake and changes in BMI over time in persons with severe motor and intellectual disabilities(SMID)

Relationship between energy intake and changes in BMI over time in persons with severe motor and intellectual disabilities(SMID)

Surgery for acute abdomen in adult patients with severe motor and intellectual disabilities.

The present study aimed to examine the characteristics, outcomes, and problems related to surgery for acute abdomen in adult patients with severe motor and intellectual disabilities (MID). The clinical records of 35 adult patients with severe MID who received emergency surgery for acute abdomen between 2011 and 2020 were reviewed. The median duration from onset to surgery was 48 hours. There were 2 cases of in-hospital mortality (5.7%), and all the patients underwent surgery more than 72 hours after onset. The in-hospital mortality rate was significantly higher in patients who received surgery later than 72 hours after onset. Bowel obstruction was the most common disease among the acute abdomen cases (71.4%) and most often involved volvulus of the small bowel and cecum. Of the patients with bowel obstruction with severe MID, 72.0% had abdominal distention, 16.0% had abdominal pain, and 4.0% had vomiting. The median duration from onset to surgery was significantly longer in the patients with bowel obstruction with severe MID than in those without severe MID (24 hours vs. 16 hours). Acute abdomen in patients with severe MID was often due to bowel obstruction caused by volvulus. Because patients with severe MID have few symptoms, they are susceptible to adverse surgical outcomes associated with a prolonged duration from onset to surgery.

Consideration for Positive and Negative Effect of Multi-Sensory Environment Interventions on Disabled Patients through Electrocardiography

Many studies have supported the efficacy of multi-sensory environment (MSE) interventions in reducing behavioral and psychological symptoms and improving the quality of life for disabled patients. However, it is difficult to identify the groups that are helped and those who are harmed. This study verified the effect of multi-sensory environment interventions on disabled patients by using thermal images, then addressed the precaution using electrocardiography (ECG). Twenty disabled patients participated in experiments for 12 min: ten with muscular dystrophy (MD) and ten with severe motor and intellectual disabilities (SMID). The continuous measurement of nasal temperature evaluated the emotional arousal after facial detection. The QT-RR relation was used to assess the risk degree. It was found that the continuous measurement of nasal temperature enabled us to evaluate the emotional arousal of disabled patients in MSE with the comparison of ECG. Through the QT-RR relation, it was found that the risk assessment for the patient with SMID was 11 times higher than those with MD because the QT was below 300 ms. Therefore, it was concluded that the specification for the risky group was related to the kind of prescribed medication through continuous measurement.

Tendency and risk factors of acute pancreatitis in children with severe motor and intellectual disabilities: A single-center study

ObjectiveAcute pancreatitis (AP) is a life-threatening disease in children with severe motor and intellectual disabilities (SMID). This study aimed to determine the clinical characteristics and causes of AP in children with SMID. MethodsWe enrolled 29 children with AP admitted to our hospital. Patients were divided into children with SMID (AP with SMID group) and the remaining patients (AP without SMID group). All patients in the AP without SMID group had no pre-existing neurological disabilities. We obtained data on the clinical background of patients, severity of AP, treatments, and outcomes. We enrolled 34 children with SMID without a history of AP (SMID control group) to compare their clinical characteristics with the AP with SMID group. We statistically compared the data between the AP with SMID and AP without SMID groups and between the AP with SMID and SMID control groups. ResultCompared to the AP without SMID group, the AP with SMID group showed significantly more severe pancreatitis (50% vs 12%) and recurrent pancreatitis (58% vs 18%). Moreover, compared with the SMID control group, the AP with SMID group showed significantly more frequent absence of voluntary movement (83% vs 26%), requirement of respiratory devices (75% vs 35%), panhypopituitarism (33% vs 6%), thermoregulatory dysfunction (58% vs 18%), and low serum albumin levels (3.6 vs 4.1 g/dL). ConclusionAP in children with SMID tended to be associated with severe and recurrent pancreatitis. Children with SMID who had a history of AP had more severe neurological impairment among children with SMID. Hypothalamic-pituitary system dysfunction caused by severe cerebral damage may contribute to the development of AP.

Dysbiosis of the gut microbiota in children with severe motor and intellectual disabilities receiving enteral nutrition: A pilot study.

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.

Healthcare workers’ presenteeism causing an outbreak of respiratory infections in a facility for patients with severe motor and intellectual disabilities

BackgroundThis study aimed to identify the source of infection and medical costs for a respiratory infection outbreak in a facility for patients with severe motor and intellectual disabilities (SMID). Presenteeism refers to a situation wherein a person continues going to work despite being ill. MethodsThe cohort included 1 healthcare worker and 17 patients who developed a fever of ≥37.5°C with respiratory symptoms for nearly a month. An outbreak investigation was conducted, which determined the initial case of the outbreak to be a single healthcare worker. We performed a univariate analysis to determine the association of the healthcare worker. From the medical records, we evaluated the costs of addition treatment and laboratory tests for the respiratory infection. ResultsThe source of infection was a healthcare worker at the facility (Odds ratio, 17.5; 95% confidential interval, 3.0–101.8). The total medical cost for hospitalized patients due to this outbreak was $12,324. DiscussionThe source of a respiratory infection outbreak in a facility for SMID was suggested to be a healthcare worker's presenteeism. ConclusionsThe cause of this outbreak was healthcare workers’ presenteeism. To prevent outbreaks, such facilities should address the causative factors.

Clinical Usefulness of Blenderized Food for Selenium Deficiency in Pediatric Patients with Severe Motor and Intellectual Disabilities

Introduction: This study aimed to assess the preventive or therapeutic effects of blenderized food (BF) on selenium deficiency in pediatric patients with severe motor and intellectual disabilities (SMID). Methods: The medical records of all 40 consecutive pediatric patients with SMID who underwent nutritional assessment were retrospectively reviewed and compared between two groups: the enteral formula (EF) group and the BF group fed with BF providing more than 10% of total caloric intake. Next, for the selenium-deficient patients who were newly started on blenderized tube feeds after the first nutritional assessment, improvement of selenium deficiency and change of dietary contents were assessed. Results: The BF group patients had a significantly lower prevalence of selenium deficiency and higher serum selenium levels than the EF group patients. In all 7 selenium-deficient patients who started blenderized tube feeds after the first nutritional assessment, serum selenium levels were significantly increased at the second nutritional assessment, even though total selenium intake, selenium intake by EF, and total caloric intake did not differ significantly, and, in fact, caloric intake was significantly decreased by EF. Conclusion: Combined feeding of BFs can be useful for prevention and therapy of selenium deficiency in pediatric SMID patients.

Tumor Screening, Incidence, and Treatment for Patients with Severe Motor and Intellectual Disabilities.

The increasing age of patients with severe motor and intellectual disabilities (SMID) has become a serious concern. Few studies have investigated tumor treatment in this population. Tumor treatments for 12 SMID patients were examined. Blood tests and ultrasonography were useful for screening. With regard to treatment, surgery for SMID patients was performed in the same manner as for patients without SMID, and the results were generally satisfactory, without major complications. Typically, cancer was diagnosed at an advanced stage, and many metachronous double cancers were observed. Treatment yielded satisfactory results for patients with SMID and their families. Future studies should examine the clinical significance of screening and tumor operative method for patients with SMID.

Gallstones in patients with severe motor and intellectual disability.

Asymptomatic gallbladder stones may be detected with ultrasound; some gallstones produce symptomatic diseases, such as cholecystitis, cholangitis, or pancreatitis. Identifying the clinical features of symptomatic gallstones may help prevent severe complications by providing intervention for asymptomatic gallstones. We aimed to investigate risk factors associated with developing symptomatic disease in pediatric patients with severe motor and intellectual disabilities (SMID). This retrospective study enrolled 30 patients with SMID who were treated at the Nara Medical University between March 2016 and March 2019. We examined the prevalence of gallstones, and the rate at which associated symptoms of gallstones were observed in patients. Furthermore, we compared the clinical features of patients with and without gallstones. Among 30 patients with SMID, 7 (23%) had gallstones, with 6 (86%) of them being symptomatic: 3 patients had acute pancreatitis and 3 had cholecystitis or cholangitis. Among 23 patients without gallstones, 2 had acute pancreatitis and 2 had biliary sludge. Patients with gallstones had significantly lower daily calorie intake than those without gallstones (P = 0.042). Furthermore, the incidence of gallstones was higher in patients who received total parenteral nutrition than in those who did not (P = 0.031). Comparative analysis between symptomatic and asymptomatic patients was not performed because almost all cases were symptomatic. Gallstones were detected in 23% of patients with SMID. The gallstones were symptomatic at a very high rate. Considering this fact, patients with SMID should be surveyed for gallstones; careful management may be needed in such patients.

Oral support for patients with severe motor and intellectual disabilities.

Oral support during feeding was clinically provided to patients with severe motor and intellectual disabilities (SMID) for the prevention of dysphasia. The present study investigated the advantages of oral support anatomically and functionally. A videofluoroscopic swallowing study was conducted on nine patients with SMID (age = 5-41 years; mean age = 15.0; four males, five females) and 24 healthy adults (age = 26-67 years; mean age = 44.3; 16 males, eight females). The movements of the hyoid bone and mandible during pharyngeal swallowing were tracked, and the pharyngeal residues were evaluated. The temporal and spatial features of the movements were compared between patients with and without oral support as well as healthy adults. The mandible moved downward earlier and showed larger displacement in the patients with SMID. The patients also had insufficient anterior displacement of the hyoid, which was associated with the pharyngeal residue. This displacement was enhanced, and the pharyngeal residue decreased with oral support. Oral support to hold the mandible successfully improved hyoid excursion and reduced pharyngeal residue during swallowing, which is a simple and reliable strategy for the prevention of dysphasia in patients with SMID.

Systemic corticosteroid as an adjunctive treatment for lower respiratory tract infection in children with severe motor and intellectual disabilities

IntroductionChildren with severe motor and intellectual disabilities (SMID) are susceptible to severe lower respiratory tract infection (LTRI). As SMID patients are prone to develop recurrent wheezing and are often diagnosed with bronchial asthma, they frequently receive systemic corticosteroids as an adjunctive treatment for LRTIs. However, the efficacy of corticosteroid therapy for LTRIs in SMID children is unclear. We investigated whether or not corticosteroid therapy was associated with better clinical outcomes for SMID children with LRTIs. MethodsOur retrospective study enrolled 217 SMID children 1–15 years old hospitalized for LTRIs. We compared the clinical characteristics and outcomes between patients with and without corticosteroid therapy. ResultsOf the 217 patients, 29 (13.3%) received corticosteroid therapy. The proportion of patients with a history of bronchial asthma was higher and LRTI was more severe in patients with corticosteroid therapy than in those without the therapy. The length of hospital stay (LOHS) was significantly longer in patients with corticosteroid therapy (median 13 days) than in those without corticosteroid therapy (median 9 days) (P = 0.02). The same tendency was shown for the LOHS in patients with severe or moderate LRTI, although not to a significant extent. ConclusionSystemic corticosteroid therapy was not associated with better clinical outcomes in SMID children with LRTIs, even if the patients suffer from severe LRTIs. Corticosteroids should be used cautiously for LRTIs in SMID children because bronchial asthma is likely to be overdiagnosed in these children.