PURPOSE: The purpose of this cross-sectional study was to examine the relationship between characteristics of severe ALS patient-caregiver couples and health related quality of life (HRQoL) in family caregivers. METHODS: The participants in this study were 89 pairs of ALS patients using ventilators and a family caregiver. The characteristics of the ALS patients and caregivers, Korean-Amyotrophic Lateral Sclerosis Functional Rating Scale-Revised, Zarit Burden Interview and SF-36 were measured in this study. The data were collected from August 2008 to April 2009. Descriptive statistics, Pearson correlation coefficients, and canonical correlation were used for data analysis. RESULTS: The physical component summary and mental component summary of the HRQoL score for family caregivers were 147.49+/-31.63 and 129.09+/-35.83, respectively. HRQoL for caregivers was related to characteristics of the ALS patient-caregiver couples, such as patient's gender, caregiver's age, gender, marital status, daily time spent in caregiving and burden with one significant canonical variable. The significant variate showed that the lower the age, the time spent in caregiving and the burden of caregivers, the higher the HRQoL of caregivers. CONCLUSION: The support systems for caregivers considering caregiver characteristics such as demographics and burden should be implemented to improve the HRQoL of caregivers.
Adult ; Aged ; Amyotrophic Lateral Sclerosis/*psychology ; Caregivers/*psychology ; Cross-Sectional Studies ; Dependency (Psychology) ; Female ; Humans ; Interviews as Topic ; Male ; Middle Aged ; *Quality of Life ; Questionnaires ; Ventilators, Mechanical

Hyperlipidemia has been reported in children with acute lymphoblastic leukemiawho have been treated with L-asparaginase alone or in combination with a steroid. Hyperlipidemia caused by L-asparaginase is usuallyreversible after drug discontinuation, and is rarely associated with severe complications. However, the risk of acute pancreatitis is increased if there is a large increase in triglyceride levels. Prompt correction of hyperlipidemia should be considered to prevent this severe complication. We report a case of successful correction of L-asparaginase-associated severe relapsed hyperlipidemia using post-centrifugal plasma filtration (PCPF), a type of selective plasmapheresis. After 3 sessions of PCPF, the serum concentrations of lipids were markedly reduced. The serum concentration for triglyceridewas reduced to 475 mg/dL from 6,298 mg/dL, the total cholesterol level was reduced to 176 mg/dL from 946 mg/dL, and the level of low-density lipoprotein was reduced to 4 mg/dL from 403 mg/dL. One month later, as determined at the outpatient clinic, the lipid levels were normalized. PCPF was effective for clearing severely increased plasma lipids.
Ambulatory Care Facilities ; Child ; Cholesterol ; Filtration* ; Humans ; Hyperlipidemias* ; Hypertriglyceridemia ; Lipoproteins ; Pancreatitis ; Plasma* ; Plasmapheresis ; Triglycerides

Rhabdomyolysis is defined as necrosis of the skeletal muscle fibers with release of the intracellular muscle constituents, including myoglobins,into the blood and urine. Severe myoglobinemia causes obstruction and necrosis of the renal tubule, resulting in acute renal failure (ARF). In this case, a patient with rhabdomyolysis-induced ARF was in recovery phase after treatment with fluid replacement and continuous renal replacement therapy (CRRT). However, a sudden relapse of severe myoglobinemia occurred during CRRT. To remove myoglobinas rapidly as possible, we applied two sessions of plasmapheresis (total plasma exchange, TPE) in addition to CRRT for two days. The myoglobin level of the patient successfully decreased by 91%, and clinical symptoms and laboratory-measuredabnormalities subsequently improved. If severe myoglobinemia is persistent, or if relapse in spite of CRRT occurs, it would be reasonable to consider TPEas an additional therapy.
Acute Kidney Injury ; Humans ; Muscle Fibers, Skeletal ; Myoglobin* ; Necrosis ; Plasma Exchange ; Plasmapheresis* ; Recurrence ; Renal Replacement Therapy ; Rhabdomyolysis*

This article briefly reviews the research findings on post-stroke rehabilitative therapy mainly published in 2011. Topics on approaches for motor function recovery include the use of body-weight-supported treadmill, robotic training, virtual reality, functional electrical stimulation, intensive treatment and motor imagery. Rehabilitative strategies to improve functional recovery such as comprehensive cardiac rehabilitation, repetitive transcranial magnetic stimulation and transcranial direct current stimulation. Also, pharmacotherapy using noradrenaline agonist reboxetine and botulinum toxin injection are discussed in this review.
Botulinum Toxins ; Electric Stimulation ; Morpholines ; Norepinephrine ; Recovery of Function ; Stroke ; Transcranial Magnetic Stimulation

OBJECTIVE: To investigate the current status of respiratory care in community-dwelling amyotrophic lateral sclerosis (ALS) patients using non-invasive ventilatory support. METHOD: Trained investigators visited patients' homes in order to conduct the survey. Questions regarding the time since diagnosis and ventilatory support, department and type of ventilator were asked. The parameters of mechanical ventilation were noted. The presence of respiratory symptoms, frequency of oxygen saturation monitoring, maneuvers for sputum clearance and frequency of air stacking exercise were also investigated. RESULTS: Data from 169 individuals were analyzed. The mean age was 59.1+/-12.5 years, time since diagnosis was 48.0+/-42.8 months and duration of ventilatory support was 25.7+/-20.8 months. The types of ventilator used were significantly different according to the medical departments where they had been prescribed. More than 50% of the subjects had sleep awakening, shortness of breath or daytime drowsiness despite ventilatory support. Air stacking exercises were performed in 8.8%. Sputum clearing maneuvers such as manually assisted cough, mechanical in/ex-sufflation or postural drainage were used by 13.6% of the individuals. Only 16.0% of the patients checked their oxygen saturation level more than once a day. CONCLUSION: Current respiratory care is inappropriate in ALS patients using non-invasive ventilatory support at home. The current system for ventilator prescription and monitoring needs modification to improve the respiratory care status.
Amyotrophic Lateral Sclerosis ; Cough ; Drainage, Postural ; Dyspnea ; Exercise ; Humans ; Oxygen ; Positive-Pressure Respiration ; Prescriptions ; Research Personnel ; Respiration, Artificial ; Respiratory Therapy ; Sleep Stages ; Sputum ; Ventilators, Mechanical

OBJECTIVE: To describe the quality of life (QOL) and care burden of caregivers of ventilator-dependent amyotrophic lateral sclerosis (ALS) patients and to compare the QOL of ALS caregivers with that of dementia caregivers. METHOD: Ninety-one pairs of ALS patients and their caregivers were interviewed. Patients were asked to provide their age, sex, time since diagnosis, and length of ventilator use, as well as complete the ALS functional rating scale-revised (ALSFRS-R). Caregivers were asked to provide baseline demographic data including age, sex, education level, marital status, link with the patient, occupation, care time, substitute caregiver, and personal caregiver. The short form-36 (SF-36) and burden interview (BI) were also administered to evaluate caregivers' QOL and care burden. T-tests, ANOVA, and Pearson correlation coefficients were used for data analysis. RESULTS: Ninety-one pairs of patients (men 69.2%, women 30.8%) and caregivers (men 24.2%, women 73.6%) completed the study. The mean SF-36 physical component summary (PCS), mental component summary (MCS), and total scores of caregivers were 131.5+/-13.2, 114.3+/-17.6, 245.8+/-28.2, respectively, which showed that the QOL of ventilator-dependent ALS patients was decreased. The BI score was 52.8+/-17.8, which meant that caregivers were heavily burdened. The SF-36 total and MCS were correlated with the BI. Care time was an important factor that influenced QOL and care burden. QOL was significantly lower for ventilator-dependent ALS caregivers than for dementia caregivers. CONCLUSION: This survey revealed the poor QOL and heavy burden of ventilator-dependent ALS caregivers, which necessitates social interventions including strategies about care time.
Aluminum Hydroxide ; Amyotrophic Lateral Sclerosis ; Carbonates ; Caregivers ; Cost of Illness ; Dementia ; Female ; Humans ; Marital Status ; Occupations ; Quality of Life ; Statistics as Topic ; Ventilators, Mechanical

OBJECTIVE: To evaluate the accuracy of the swallowing kinematic analysis. METHODS: To evaluate the accuracy at various velocities of movement, we developed an instrumental model of linear and rotational movement, representing the physiologic movement of the hyoid and epiglottis, respectively. A still image of 8 objects was also used for measuring the length of the objects as a basic screening, and 18 movie files of the instrumental model, taken from videofluoroscopy with different velocities. The images and movie files were digitized and analyzed by an experienced examiner, who was blinded to the study. RESULTS: The Pearson correlation coefficients between the measured and instrumental reference values were over 0.99 (p<0.001) for all of the analyses. Bland-Altman plots showed narrow ranges of the 95% confidence interval of agreement between the measured and reference values as follows: 0.14 to 0.94 mm for distances in a still image, -0.14 to 1.09 mm/s for linear velocities, and -1.02 to 3.81 degree/s for angular velocities. CONCLUSION: Our findings demonstrate that the distance and velocity measurements obtained by swallowing kinematic analysis are highly valid in a wide range of movement velocity.
Biomechanics ; Deglutition ; Epiglottis ; Mass Screening ; Reference Values ; Reproducibility of Results

OBJECTIVE: To measure the intra- and inter-rater reliability of a simple sensorimotor performance test for rats, and to evaluate the learning efficiency of a novice rater for the test. METHOD: Middle cerebral arteries were occluded by intraluminal sutures in 25 male Sprague-Dawley rats (10~12 weeks old). The sensorimotor performance test was performed by a novice and an experienced rater, with each rater performing the test twice each week for 3 consecutive weeks. A ten-minute standardized video about the rating method was shown to the novice rater after the second test each week. RESULTS: The intra- and inter-rater agreement was determined using Cohen's weighted kappa coefficient. The intra-rater reliability was initially poor for the novice (κ [95% confidence interval], 0.31[-0.02, 0.64]), but it improved significantly after 3-week self education using the standardized video (0.81 [0.69, 0.93], showing almost perfect agreement. The reliability of the experienced researcher was good at all times (κ = 0.64, 0.76, 0.71, for week 1, 2, 3, respectively), indicating substantial agreement. The inter-rater reliability showed clear improvement after self-education (κ = 0.44, 0.69, 0.69, for week 1, 2, 3, respectively). Although the total sum score was highly reliable, some of the individual items showed lower intra-and inter-rater agreement. However, each rater showed greater within-rater variability for different subtests. CONCLUSION: The simple sensorimotor performance test showed high degree of intra- and inter-rater agreement when performed by experienced or properly educated raters. The inaccuracy of the novice was rectified by 3-week self-education using a video.
Animals ; Behavioral Research ; Education ; Humans ; Learning Curve* ; Learning* ; Male ; Middle Cerebral Artery ; Motor Activity ; Rats* ; Rats, Sprague-Dawley ; Reproducibility of Results ; Sutures

High-dose methotrexate (MTX) is frequently used for the treatment for various malignancies. The primary route of MTX excretion is through the kidneys, and so it may cause toxicities in patients with renal insufficiency. Prolonged high levels of serum MTX can result in renal dysfunction, pancytopenia and mucositis, but the strategies used for MTX removal have not been universally accepted. We report here on a case of a 55-year-old man with NK cell lymphoma and who was treated with high-dose MTX. He had been receiving hemodialysis due to acute renal failure that was induced by previous chemotherapy. After 24, 48, and 72 hours of MTX infusion, the serum MTX levels were markedly increased to 146.07micromol/L, 111.30micromol/L and 94.37micromol/L, respectively, and so leucovorin rescue was intensified. Therapeutic plasma exchange (TPE) was started on post-MTX day 4, which was after the day of the peak MTX concentration, and this was continued on days 5 and 7 to rapidly reduce the MTX level. The serum MTX level decreased to the normal range without any rebound phenomenon after 2 weeks. However, MTX-induced pancytopenia occurred and the patient then died of septic shock. It is suggested that if the MTX level is very high in spite of conventional treatments, then immediate TPE should be started to avoid MTX toxicities.
Acute Kidney Injury ; Humans ; Kidney ; Killer Cells, Natural ; Leucovorin ; Lymphoma ; Methotrexate ; Middle Aged ; Mucositis ; Pancytopenia ; Plasma ; Plasma Exchange ; Reference Values ; Renal Dialysis ; Renal Insufficiency ; Shock, Septic

Background: The efficacy and safety of denosumab have been established in a phase 3, randomized, placebo-controlled trial in Korean postmenopausal women with osteoporosis. This postmarketing surveillance study was aimed to investigate the safety and effectiveness of denosumab in Korean real-world clinical practice. Methods: Patients with osteoporosis who had received denosumab per the Korean approved indications in the postmarketing setting between September 2014 and September 2019 were enrolled. The primary endpoint was the incidence of adverse events (AEs) and adverse drug reactions (ADRs). The secondary endpoint was the percent change from baseline in bone mineral density (BMD) of the lumbar spine, total hip, and femoral neck. Results: Of the 3,221 patients enrolled, 3,185 were included in the safety analysis set; 2,973 (93.3%) were female, and the mean± standard deviation (SD) age was 68.9±9.9 years. The mean±SD study period was 350.0±71.4 days. AEs, fatal AEs, and ADRs occurred in 19.3%, 0.8%, and 1.6%, respectively. The most frequent AEs, occurring in >0.5% of patients, were dizziness (0.7%), arthralgia (0.7%), back pain (0.6%), and myalgia (0.6%). Hypocalcemia occurred in 0.3% of patients. There were no cases of osteonecrosis of the jaw and atypical femoral fracture. Mean±SD percent change from baseline in BMD of the lumbar spine, total hip, and femoral neck was 7.3%±23.6%, 3.6%±31.4%, and 3.2%±10.7%, respectively. Conclusion The safety and effectiveness of denosumab in Korean patients with osteoporosis in this study were comparable with those in the Korean randomized controlled trial, with no new safety findings.