Differing clinical features between Japanese siblings with cerebrotendinous xanthomatosis with a novel compound heterozygous CYP27A1 mutation: a case report.

BACKGROUND: Cerebrotendinous xanthomatosis (CTX) is an autosomal-recessive lipid storage disorder caused by mutations in the CYP27A1 gene encoding the key enzyme in the bile acid synthesis, sterol 27-hydroxylase. Here, we report two Japanese CTX siblings with a novel compound heterozygous CYP27A1 mutation, showing different clinical phenotypes and responses to chenodeoxycholic acid (CDCA) therapy. CASE PRESENTATION: The proband, a 32-year-old man, who had chronic diarrhea, bilateral cataracts, and xanthomas, demonstrated progressive neurological manifestations including ataxia, and spastic paraplegia during a 5-year follow-up period despite normalization of serum cholestanol after initiation of CDCA treatment. He also exhibited cognitive decline although improvement had been observed at the beginning of treatment. Follow-up brain magnetic resonance imaging (MRI) revealed pronounced progressive atrophy in the cerebellum, in addition to expanding hyperintense lesions in the dentate nuclei, posterior limb of the internal capsule, cerebral peduncles, and inferior olives on T2-weighted images. In contrast, the two-year-younger sister of the proband presented with chronic diarrhea, cataracts, xanthomas, and intellectual disability but no other neurological symptoms at the time of diagnosis. CDCA treatment lead to improvement of cognitive function and there were no characteristic CTX-related MRI features during the follow-up period. The siblings shared a paternally inherited c.1420C > T mutation (p.Arg474Trp) and a maternally inherited novel c.1176_1177delGA mutation, predicting p.(Glu392Asp*20). CONCLUSIONS: Our cases suggest that early diagnosis and subsequent initiation of CDCA treatment are crucial before the appearance of characteristic MRI findings and severe neurological manifestations related to CTX. Further studies are required to elucidate mechanisms responsible for the clinical diversity of CTX and prognostic factors for long-term outcomes following initiation of CDCA treatment.

Valosin-containing protein Asp395Gly mutation in a patient with frontotemporal dementia: a case report.

BACKGROUND: Variants in the valosin-containing protein (VCP) gene were identified as one of the causes for inclusion body myopathy associated with Paget disease of the bone and frontotemporal dementia (FTD). Previously identified pathogenic variants in VCP are associated with frontotemporal lobar degeneration with TDP-43 inclusions (FTLD-TDP) pathologically, but p.Asp395Gly VCP was recently reported to cause familial FTD with tauopathy characterized by neurofibrillary tau tangles (NFT) and not FTLD-TDP. We describe the clinical and genetic findings of a patient with p.Asp395Gly valosin-containing protein (VCP), who was diagnosed with FTD without a family history and in the absence of muscle or bone disease comorbidity. CASE PRESENTATION: The patient was a 62-year-old man, who developed atypical depression at the age of 37 years. Subsequently, he presented with self-centered behavior at the age of 45 years. The self-centered behavior intensified from around the age of 50 years, which was accompanied by the development of executive dysfunction; therefore, he visited our hospital at 52 years of age. Magnetic resonance imaging revealed bilateral frontal lobe atrophy. Brain perfusion single-photon emission computed tomography revealed bilateral frontal lobe hypoperfusion. The patient fulfilled the diagnostic criteria for behavioral variant of FTD. Ten years after the diagnosis, computed tomography of the trunk and limbs, muscle biopsy, and bone scintigraphy revealed the absence of concomitant muscle and bone disease. The concentrations of cerebrospinal fluid (CSF) total tau and phosphorylated tau proteins were 389 pg/mL and 53.2 pg/mL (cut-off: 50 pg/mL), respectively. Genetic analyses were performed using the whole-exome and Sanger sequencing methods. We identified p.Asp395Gly VCP in this patient with pure FTD. CONCLUSIONS: p.Asp395Gly VCP was identified in a patient with likely sporadic FTD without concomitant muscle and bone disease. The CSF analysis suggested that our patient may have FTD due to NFT accumulation similar to the familial FTD patients with p.Asp395Gly VCP recently reported. Our findings suggest that a genetic search for the pathogenic variants of VCP should be considered not only for familial FTD, but also for patients with sporadic FTD, even in the absence of comorbid muscle or bone disease.

Prevalence and development of idiopathic normal pressure hydrocephalus: A 16-year longitudinal study in Japan.

OBJECTIVE: We previously investigated the preclinical state of idiopathic normal pressure hydrocephalus (iNPH): asymptomatic ventriculomegaly with features of iNPH on magnetic resonance imaging (AVIM) found in community inhabitants. The aim of the study was to determine how iNPH develops longitudinally. MATERIALS AND METHODS: A previous longitudinal prospective community-based cohort study was initiated in 2000. The 271 70 year-old participants were followed up in 2016 at the age of 86 years. At this time, 104 participants could be reached for clinical examinations and brain magnetic resonance imaging (MRI). iNPH in this study was diagnosed if the participant had more than one symptom in the clinical triad and disproportionately enlarged subarachnoid space hydrocephalus (DESH) on MRI, fulfilling at least an Evans index >0.3 (ventricular enlargement, VE) and a narrowing of the subarachnoid space at the high convexity (tight high convexity, THC). Asymptomatic VE (AVE) plus THC were considered AVIM. RESULTS: Longitudinally throughout 16 years, 11 patients with iNPH were found. The hospital consultation rate was only 9%. Five of the eight patients with AVIM (62.5%) and six of 30 with AVE (20.0%) developed iNPH. Cross-sectionally, eight patients had iNPH (8/104, 7.7% prevalence at the age of 86) in 2016. Disease development was classified into THC-preceding and VE-preceding iNPH. One VE-preceding iNPH case was considered a comorbidity of Alzheimer's dementia. CONCLUSION: Idiopathic normal pressure hydrocephalus had a high prevalence among octogenarians in the evaluated community. iNPH developed not only via AVIM but also via AVE, the latter was also frequent in the elderly.

What is the optimal nutritional assessment tool for predicting decline in the activity of daily living among older patients with heart failure?

The clinical importance of nutritional management in activities of daily living (ADL) among older inpatients with heart failure (HF) is greatly increasing. We determined the optimal nutritional assessment tool that can predict ADL decline among older inpatients with HF. We prospectively investigated 91 inpatients aged ≥ 65 years with HF in an acute hospital. We measured their nutritional status at admission using nutrition indices: the controlling nutritional status (CONUT), the geriatric nutritional risk index, the prognostic nutritional index, and the mini nutritional assessment. Logistic regression analysis was used to estimate the odds ratios (ORs) and 95% confidence intervals (CIs) of the relationships between the malnutrition status assessed by each nutritional index category and the ADL decline measured by the Barthel index (BI) in the univariate and multivariate analyses. Among the participants, 28.6% (n = 26; median age 81.5 years; 69.2% men) of the participants were included in the Reduced BI group and 71.4% (n = 65; median age 79.0 years; 67.7% men) in the Maintained BI group. The Reduced BI group showed a significantly higher CONUT value than the Maintained BI group, but there were no significant differences in other nutritional indices. In the multivariate logistic regression analysis, a higher CONUT score was associated with a significantly elevated risk of Reduced BI (adjusted OR 0.24; 95%CI 0.08-0.75; p = 0.014). We found that CONUT is an appropriate nutritional assessment tool for predicting ADL decline among older inpatients with HF in the early phase of hospitalization.

The use of viabahn VBX stent-grafts for the treatment of extrahepatic portal vein hemorrhage.

Percutaneous transhepatic stent-graft placement usually requires more than a 12 F sheath, which has a higher potential risk of peritoneal hemorrhage. A case of postoperative portal vein hemorrhage after pancreaticoduodenectomy treated using a Viabahn VBX balloon expanding stent-graft is described in this report. The stent-graft was delivered using an 8 F sheath through a transhepatic approach and deployed from the superior mesenteric vein to the main portal vein. Hemostasis was achieved and graft patency was confirmed one year after the procedure.

Physical Exercise Improves Cognitive Function in Older Adults with Stage 3-4 Chronic Kidney Disease: A Randomized Controlled Trial.

INTRODUCTION: Patients with chronic kidney disease (CKD) exhibit a higher probability of having cognitive impairment or dementia than those without CKD. The beneficial effects of physical exercise on cognitive function are known in the general older population, but more research is required in older adults with CKD. METHODS: Eighty-one outpatients (aged ≥65 years) with CKD stage G3-G4 were assessed for eligibility. Among them, 60 were randomized (single-center, unblinded, and stratified) and 53 received the allocated intervention (exercise n = 27, control n = 26). Patients in the exercise group undertook group-exercise training at our facility once weekly and independent exercises at home twice weekly or more, for 24 weeks. Patients in the control group received general care. General and specific cognitive functions (memory, attention, executive, and verbal) were measured, and differences in their scores at baseline and at the 24-week follow-up visit were assessed between the 2 groups. RESULTS: Forty-four patients completed the follow-up at 24 weeks (exercise n = 23, control n = 21). Patients in the exercise group showed significantly greater changes in Wechsler Memory Scale-Revised Logical Memory delayed recall (exercise effect: 2.82, 95% CI: 0.46-5.19, p = 0.03), and immediate and delayed recall (exercise effect: 5.97, 95% CI: 1.13-10.81, p = 0.02) scores than those in the control group. CONCLUSIONS: The 24-week exercise intervention significantly improved the memory function in older adults with pre-dialysis CKD. This randomized controlled trial suggests that physical exercise is a useful nonpharmacological strategy for preventing cognitive decline in these patients.

Relationship between tongue muscle quality and swallowing speed in community-dwelling older women.

OBJECTIVE: The aim of this study was to investigate the relationship between tongue muscle quality index, which was represented as tongue muscle pressure divided by tongue muscle mass, and swallowing speed in community-dwelling older women. METHODS: The inclusion criteria for this cross-sectional study were that participants be community-dwelling older women aged 65 years and above without dysphagia. The exclusion criteria were stroke and Parkinson's disease that directly cause dysphagia. We measured tongue muscle thickness and maximum tongue pressure and the tongue muscle quality index, which was defined as the maximum tongue pressure divided by tongue muscle thickness. We investigated swallowing speed via a 100 ml water swallowing test. To assess the relationship between tongue muscle characteristics and swallowing speed, we performed stepwise multiple regression analysis. RESULTS: Ninety-three participants were enrolled in this study (mean age: 84.2 ± 4.7 years). A stepwise multiple regression analysis showed that age (ß = - 0.292, p < 0.01) and tongue muscle quality index (ß = 0.267, p < 0.01) were related to swallowing speed. CONCLUSION: We found that tongue muscle quality index was related to swallowing speed in community-dwelling older women. According to our findings, it is possible that the tongue muscle quality index is a useful parameter for assessing swallowing speed in older women without dysphagia.

[Relevance of the five-repetition sit-to-stand test for diagnosing sarcopenia in elderly inpatients with cardiac disease].

PURPOSE: The aim of this study was to determine the reference values for diagnosing sarcopenia using the five-repetition sit-to-stand test in elderly inpatients with cardiac disease. METHODS: We studied 71 inpatients with cardiac disease ≥65 years of age (mean age 78.0±7.9 years, 42.3% women) who were admitted between April 2015 and March 2016. Patients were assessed for sarcopenia, and we performed the five-repetition sit-to-stand test. We defined sarcopenia using the Asian Working Group for Sarcopenia-suggested diagnostic algorithm. A logistic regression analysis was performed to estimate the odds ratios (ORs) and 95% confidence intervals (CIs) of the relationship between sarcopenia and the five-repetition sit-to-stand test. A multivariate analysis showed that the age, admission diagnosis, the New York Heart Association classification, the Charlson comorbidity index, and the ratio of extracellular to total body water were relevant covariates. The cut-off value of the five-repetition sit-to-stand test to diagnose sarcopenia was determined using a receiver operating characteristic curve. RESULTS: Sarcopenia was diagnosed in 25 patients (35.2%). A multivariate logistic regression analysis showed that the five-repetition sit-to-stand test was significantly associated with sarcopenia (p=0.024), and the OR (95% CI) was 1.31 (1.04-1.65). The cut-off value of the five-repetition sit-to-stand test to diagnose sarcopenia was 10.9 s (sensitivity 80.0%, specificity 70.0%, area under the curve 0.83). CONCLUSIONS: The five-repetition sit-to-stand test is a useful screening tool for sarcopenia in elderly inpatients with cardiac disease. The cut-off value to diagnose sarcopenia was 10.9 s in this study.

Nationwide survey on cerebrotendinous xanthomatosis in Japan.

Cerebrotendinous xanthomatosis (CTX) is likely to be underdiagnosed and precise epidemiological characteristics of CTX are largely unknown as knowledge on the disorder is based mainly on case reports. We conducted a nationwide survey on CTX to elucidate the frequency, clinical picture, and molecular biological background of Japanese CTX patients. In this first Japanese nationwide survey on CTX, 2541 questionnaires were sent to clinical departments across Japan. A total of 1032 (40.6%) responses were returned completed for further analysis. Forty patients with CTX (50.0% male) were identified between September 2012 and August 2015. The mean age of onset was 24.5 ± 13.6 years, mean age at diagnosis was 41.0 ± 11.6 years, and corresponding mean duration of illness from onset to diagnosis was 16.5 ± 13.5 years. The most common initial symptom was tendon xanthoma, followed next by spastic paraplegia, cognitive dysfunction, cataract, ataxia, and epilepsy. The most predominant mutations in the CYP27A1 gene were c.1214G> A (p.R405Q, 31.6%), c.1421G> A (p.R474Q, 26.3%), and c.435G> T (p.G145=, 15.8%). Therapeutic interventions that included chenodeoxycholic acid, HMG-CoA reductase inhibitor, and LDL apheresis reduced serum cholestanol level in all patients and improved clinical symptoms in 40.5% of patients. Although CTX is a treatable neurodegenerative disorder, our nationwide survey revealed an average 16.5-year diagnostic delay. CTX may be underdiagnosed in Japan, especially during childhood. Early diagnosis and treatment are essential to improve the prognosis of CTX.

[The isometric knee extension strength threshold for maintaining walking speed and step length in older male inpatients].

AIM: To clarify the minimum knee extension muscle strength needed to maintain walking speed and step length in older male inpatients. METHOD: The participants were 786 male inpatients of ≥65 years of age without cerebrovascular disorder, orthopedic disease, malignancy, or dementia. We investigated the participants' isometric knee extension muscle force (kgf/kg), maximum walking speed (m/s) and step length, based on their medical records. The relationship of walking speed and step length to isometric knee extension muscle force was fitted to linear and nonlinear models, and the respective R2 values were compared. Next, the muscle force data were divided into two groups, and two linear functions were calculated. Then, the muscle force value that minimized the sum of the residual sum of squares of the two linear function expressions was obtained. RESULTS: The R2 values of each equation in the nonlinear model were higher than those in the linear model. Among all participants, the muscle force values that minimized the sum of the residual sum of squares for walking speed and step length were 0.33 kgf/kg and 0.43 kgf/kg, respectively. Among participants of ≤74 years of age, the muscle force value that minimized the sum of the residual sum of squares was 0.30 kgf/kg for both walking speed and step length, whereas the values were 0.32 kgf/kg and 0.43 kgf/kg, respectively, in participants of ≥75 years of age. CONCLUSION: Walking speed and step length were significantly decreased in male inpatients of 65-74 years of age when the isometric knee extension force values for both were <0.30 kgf/kg. In contrast, among male inpatients of ≥75 years of age, these values were significantly decreased when the respective isometric knee extension muscle force values were <0.32 kgf/kg and <0.43 kgf/kg.

Whole-exome sequencing and digital PCR identified a novel compound heterozygous mutation in the NPHP1 gene in a case of Joubert syndrome and related disorders.

BACKGROUND: Joubert syndrome and related disorders (JSRD) is a clinically and genetically heterogeneous condition with autosomal recessive or X-linked inheritance, which share a distinctive neuroradiological hallmark, the so-called molar tooth sign. JSRD is classified into six clinical subtypes based on associated variable multiorgan involvement. To date, 21 causative genes have been identified in JSRD, which makes genetic diagnosis difficult. CASE PRESENTATION: We report here a case of a 28-year-old Japanese woman diagnosed with JS with oculorenal defects with a novel compound heterozygous mutation (p.Ser219*/deletion) in the NPHP1 gene. Whole-exome sequencing (WES) of the patient identified the novel nonsense mutation in an apparently homozygous state. However, it was absent in her mother and heterozygous in her father. A read depth-based copy number variation (CNV) detection algorithm using WES data of the family predicted a large heterozygous deletion mutation in the patient and her mother, which was validated by digital polymerase chain reaction, indicating that the patient was compound heterozygous for the paternal nonsense mutation and the maternal deletion mutation spanning the site of the single nucleotide change. CONCLUSION: It should be noted that analytical pipelines that focus purely on sequence information cannot distinguish homozygosity from hemizygosity because of its inability to detect large deletions. The ability to detect CNVs in addition to single nucleotide variants and small insertion/deletions makes WES an attractive diagnostic tool for genetically heterogeneous disorders.

Paraneoplastic Cerebellar Degeneration Accompanied by Seropositivity for Anti-GAD65, Anti-SOX-1 and Anti-VGCC Antibodies Due to Small-cell Lung Cancer.

Paraneoplastic cerebellar degeneration (PCD) is a paraneoplastic neurological syndrome that is rarely accompanied by seropositivity with a combination of multiple antibodies. We herein report a 50-year-old man with PCD accompanied by small-cell lung cancer (SCLC). This patient was seropositive for anti-glutamic acid decarboxylase 65, anti-SRY-related HMG-box gene 1 and anti-voltage-gated calcium channel antibodies. After chemoradiation therapy without immunotherapy, cerebellar ataxia of the trunk and limbs markedly improved, along with a notable amelioration of SCLC. This case suggests that tumor therapy should be started immediately and that a panel of anti-neuronal antibodies should be evaluated when PCD with SCLC is suspected.

Systemic Capillary Responses to Acute Exercise in Hypertensive Seniors: Insights from a Single-Center Pilot Study.

Objective: The aim of this study was to investigate nailfold capillary parameters in community-dwelling individuals aged over 60 years who have hypertension and do not exercise regularly. Furthermore, the study examined the correlations between capillary function and other health-related indicators. DESIGN: This study was a single- center pilot trial. SETTING: The study took place in the Faculty of Health, Tsukuba University of Technology, Japan. PARTICIPANTS: Hypertensive community-dwelling elderly people took part in the study. INTERVENTION: Microcirculation was observed before and 1 min after an arm-curl exercise by means of capillary microscopy of the non-exercised limb. Additionally, we examined other health-related indicators. Methods: We measured the acute effects of reperfusion on nailfold density, flow, and diameters. Secondary outcomes included the correlations between microvascular parameters and other health-related indicators. We hypothesized that brief exercise could enhance microcirculation reperfusion and correlate with other health-related parameters. Results: There were 20 participants with a mean (SD) age of 67.1 (5.8) years. The capillary flow rate changed from 2.3 ± 6.7 to 2.7 ± 0.2 log µm/s (p < 0.01), and the capillary density changed from 0.8 ± 0.2 to 0.9 ± 0.1 log/mm (p < 0.01), which included a significant increase in the non-exercising limb. Significant correlations were observed between the nailfold capillary diameter and body fat mass, the capillary diameter and physical activity, and the capillary density and bone mineral density. Conclusions: The acute effects of exercise on high-risk elderly individuals can be safe, and even 1 of min exercise can potentially improve their nailfold capillary function, despite the brief time, compared to no exercise. The results indicate that capillaries have an impact on the function of the whole body. Thus, they may be a useful diagnostic tool for assessing nailfold capillaries.

Chronological Course and Clinical Features after Denver Peritoneovenous Shunt Placement in Decompensated Liver Cirrhosis.

BACKGROUND: Refractory ascites affects the prognosis and quality of life in patients with liver cirrhosis. Peritoneovenous shunt (PVS) is a treatment procedure of palliative interventional radiology for refractory ascites. Although it is reportedly associated with serious complications (e.g., heart failure, thrombotic disease), the clinical course of PVS has not been thoroughly evaluated. OBJECTIVES: To evaluate the relationship between chronological course and complications after PVS for refractory ascites in liver cirrhosis patients. MATERIALS AND METHODS: This was a retrospective study of 14 patients with refractory ascites associated with decompensated cirrhosis who underwent PVS placement between June 2011 and June 2023. The clinical characteristics, changes in cardiothoracic ratio (CTR), and laboratory data (i.e., brain natriuretic peptide (BNP), D-dimer, platelet) were evaluated. Follow-up CT images in eight patients were also evaluated for ascites and complications. RESULTS: No serious complication associated with the procedure occurred in any case. Transient increases in BNP and D-dimer levels, decreased platelet counts, and the worsening of CTR were observed in the 2 days after PVS; however, they were improved in 7 days in all cases except one. In the follow-up CT, the amount of ascites decreased in all patients, but one patient with a continuous increase in D-dimer 2 and 7 days after PVS had thrombotic disease (renal and splenic infarction). The mean PVS patency was 345.4 days, and the median survival after PVS placement was 474.4 days. CONCLUSIONS: PVS placement for refractory ascites is a technically feasible palliative therapy. The combined evaluation of chronological changes in BNP, D-dimer, platelet count and CTR, and follow-up CT images may be useful for the early prediction of the efficacy and complications of PVS.

Gender-specific Characteristics of Social Factors Related to Frequency of Daily Conversation Among Community-dwelling Older Adults: A Cross-sectional Observational Study.

Objectives: This study examined the social factors affecting the frequency of going out, and social isolation related to the frequency of daily conversation, stratified by gender. Method: The participants comprised 1,016 community-dwelling older Japanese adults, who were categorized into four groups: those who are (1) not isolated socially and go out every day, (2) not isolated socially and do not go out every day, (3) socially isolated and go out every day, and (4) socially isolated and do not go out every day. We performed a logistic regression analysis to assess the relationship between the frequency of daily conversation and the four groups. Results: Our multivariable logistic regression analysis (reference, group 1) showed that the coexistence of poor social factors significantly affected men (group 2: odds ratio [OR] 2.13 [1.10 to 4.12]; group 3: OR 2.92 [1.70 to 5.00]; and group 4: OR 4.28 [2.43 to 7.54]). For women, the frequency of going out was related to daily conversation only with social isolation group (group 2: OR 1.51 [0.77 to 2.98]; group 3: OR 2.42 [1.25 to 4.68]; and group 4: OR 3.81 [2.08 to 7.00]). Conclusion: Our findings suggest that promoting daily conversations of community-dwelling older adults can help prevent related health adversities.

Associations between caregiver burden and care receivers' sharing ratio of household tasks among spousal caregivers.

AIM: We assessed care receivers' sharing ratios of household tasks before they required care and investigated their relationship with caregiver burden. METHODS: A cross-sectional, online survey was completed by 272 spousal caregivers in Japan. Caregiver burden was assessed using the Zarit Burden Interview. Low and moderate-to-high caregiver burdens were defined as those with scores ≤40 and ≥41, respectively. Additionally, caregiver responses to, "How much did your partner contribute to household tasks before the onset of their condition that needed care?" were scored on a 10-point Likert scale ranging from 1 to 10. We defined answers that scored 1-4 as a low sharing ratio and those that scored 5-10 as a high sharing ratio of household tasks of care receivers before developing a condition that required care. RESULTS: Among the husband and wife caregivers, moderate-to-high caregiver burden in 33 (25.0%) and 48 (34.3%) caregivers, respectively. Multivariate logistic regression analysis showed that among the husband caregivers, the high sharing ratio of household tasks of their wives before developing a condition that required care was significantly associated with their caregiver burden (OR 4.55, 95% CI 1.20-17.27); however, no such association was observed among the wife caregivers (OR 0.85, 95% CI 0.37-2.29). CONCLUSIONS: Among husband, but not wife caregivers, the high sharing ratio of household tasks of their wives before they required care was significantly associated with their caregiver burden. Geriatr Gerontol Int 2023; 23: 616-621.

Combination of low muscle strength and malnutrition is associated with longer length of hospital stay among older patients with heart failure.

BACKGROUND: Muscle strength and nutritional status are associated with length of hospital stay (LOHS) in older patients with heart failure (HF). OBJECTIVES: The purpose of the study was to examine the association of the combination of muscle strength and nutritional status on LOHS in older patients with HF. METHODS: This retrospective cohort study included 414 older inpatients with HF (men, 57.2%; median age, 81 years; interquartile range, 75-86 years). Patients were categorized into four groups according to their muscle strength and nutritional status: group 1, high muscle strength and normal nutritional status; group 2, low muscle strength and normal nutritional status; group 3, high muscle strength and malnutrition; and group 4, low muscle strength and malnutrition. The outcome variable was the LOHS, and an LOHS of >16 days was defined as long LOHS. RESULTS: Multivariate logistic regression analysis adjusted for baseline characteristics (reference, group 1) showed that group 4 was associated with a more significant risk of long LOHS (odds ratio [OR], 3.54 [95% confidence interval, 1.85-6.78]). In the subgroup analysis, this relationship was maintained for the first admission HF group (OR, 4.65 [2.07-10.45]) but not for the HF readmission group (OR, 2.80 [0.72-10.90]). CONCLUSIONS: Our results suggest that the long LOHS for older patients with HF at first admission was associated with a combination of low muscle strength and malnutrition but not by either factor individually.

Impact of the quantity and quality of the skeletal muscle on survival among patients undergoing allogeneic hematopoietic stem cell transplantation.

INTRODUCTION: Poor skeletal muscle function is relatively high in patients undergoing allogeneic hematopoietic stem cell transplantation (allo-HSCT), leading to various negative health outcomes. This study aimed to investigate the relationship between the quantity and quality of skeletal muscle before transplantation and the short-to long-term survival after transplantation in patients undergoing allo-HSCT. METHODS: This retrospective cohort study included 156 patients undergoing allo-HSCT (men, 67.3 %; median age, 53 years; interquartile range, 42-61 years). The quantity and quality of the skeletal muscle were measured at the psoas major at the level of the third lumbar vertebrae using a computed tomography (CT) and were defined as psoas muscle index (PMI) and CT values (CTV), respectively. The outcome measure of this study was overall survival (OS) after allo-HSCT, and we examined the relationship between survival at three time points (6, 12, and 24 months) after transplantation, PMI, and CTV. RESULTS: PMI was significantly associated with survival at all time points in the crude model (P < 0.001), and a significant association was observed in the fully adjusted model (P < 0.01). CTV was significantly associated with survival at all time points in the crude model (P < 0.05), but not in the fully adjusted model (P > 0.05). CONCLUSIONS: We found that the quantity and quality of the skeletal muscle before transplantation were significantly associated with OS at 6, 12, and 24 months after transplantation, showing a particularly robust association with quantity.