Prevalence and clinical characteristics of Sarcopenia in older adult patients with stable chronic obstructive pulmonary disease: a cross-sectional and follow-up study.

BACKGROUND: The relationship between sarcopenia and chronic obstructive pulmonary disease (COPD) has been increasingly reported, and there is some overlap regarding their clinical features and pulmonary rehabilitation (PR) strategies. No Korean study has reported the actual prevalence of sarcopenia in patients with stable COPD who are recommended for pulmonary rehabilitation. This study evaluated the prevalence and clinical features of sarcopenia in older adult outpatients with stable COPD and the changes after 6 months. METHODS: In this cross-sectional and 6-month follow-up study, we recruited 63 males aged ≥ 65 diagnosed with stable COPD. Sarcopenia was diagnosed using the AWGS 2019 criteria, which included hand grip strength testing, bioelectrical impedance analysis, Short Physical Performance Battery administration, and Strength, Assistance with walking, Rising from a chair, Climbing stairs, and Falling screening tool administration. A 6-minute walk test (6 MWT) was conducted, forced vital capacity (FVC), forced expiratory volume in 1 s (FEV1), maximal inspiratory and expiratory pressures (MIP and MEP, respectively) and peak expiratory flow (PEF) were assessed, and patient-reported questionnaires were administered. RESULTS: At baseline, 14 (22%) patients were diagnosed with possible sarcopenia, and eight (12.6%) were diagnosed with sarcopenia. There were significant differences in the age; body mass index; Body mass index, airflow Obstruction, Dyspnea, and Exercise index; modified Medical Research Council dyspnea scores; and International Physical Activity Questionnaire scores between the normal and sarcopenia groups. Whole-body phase angle, MIP, MEP, PEF, and 6-minute walk distance (6 MWD) also showed significant differences. Over 6 months, the proportion of patients with a reduced FEV1 increased; however, the proportion of patients with sarcopenia did not increase. CONCLUSION: A relatively low prevalence of sarcopenia was observed in older adult outpatients with stable COPD. No significant change in the prevalence of sarcopenia was found during the 6-month follow-up period. TRIAL REGISTRATION: The study was registered with the Clinical Research Information Service (KCT0006720). Registration date: 30/07/2021.

Erratum: Correction of Tables in the Article "Reference Respiratory Muscle Strength Values and a Prediction Equation Using Physical Functions for Pulmonary Rehabilitation in Korea".

This corrects the article on p. e325 in vol. 38, PMID: 37846788.

Reference Respiratory Muscle Strength Values and a Prediction Equation Using Physical Functions for Pulmonary Rehabilitation in Korea.

BACKGROUND: In Korea, tests for evaluating respiratory muscle strength are based on other countries' clinical experience or standards, which can lead to subjective evaluations. When evaluating respiratory function based on the standards of other countries, several variables, such as the race and cultures of different countries, make it difficult to apply these standards. The purpose of this study was to propose objective respiratory muscle strength standards and predicted values for healthy Korean adults based on age, height, weight, and muscle strength, by measuring maximal inspiratory pressure (MIP), maximal expiratory pressure (MEP), and peak cough flow (PCF). METHODS: This cross-sectional study analyzed MIP, MEP, and PCF in 360 people, each group comprising 30 adult men and women aged 20-70, diagnosed as healthy after undergoing medical check-ups at a general hospital. Hand grip strength (HGS) and the five times sit-to-stand test (FTSST) results were also recorded. Correlations among respiratory muscle strength, participant demographics, and overall muscle strength were evaluated using Pearson's correlation analysis. The predicted values of respiratory muscle strength were calculated using multiple regression analysis. RESULTS: Respiratory muscle strength differed from the values reported in studies from other countries. In the entire samples, both MIP and MEP had the highest correlations with peak HGS (r = 0.643, r = 0.693; P < 0.05), while PCF had the highest correlation with forced expiratory volume in 1 s (r = 0.753; P < 0.05). Age, body mass index, peak HGS, and FTSST results were independent variables affecting respiratory muscle strength. A predictive equation for respiratory muscle strength was developed using the multiple regression equation developed in this study. CONCLUSION: Respiratory muscle strength index may differ by country. For more accurate diagnoses, standard values for each country are required. This study presents reference values for Korea, and a formula for estimation is proposed when no respiratory muscle strength measurement equipment is available. TRIAL REGISTRATION: Clinical Research Information Service Identifier: KCT0006778.

Epidemiology of Congenital Bleeding Disorders: a Nationwide Population-based Korean Study.

BACKGROUND: Except for data in the Korea Hemophilia Foundation Registry, little is known of the epidemiology of congenital bleeding disorders in Korea. METHODS: Data were obtained from the Korean Health Insurance Review and Assessment Service (HIRA) database. RESULTS: From 2010 to 2015, there were 2,029 patients with congenital bleeding disorders in the Korean HIRA database: 38% (n = 775) of these patients had hemophilia A (HA), 25% (n = 517) had von Willebrand disease (vWD), 7% (n = 132) had hemophilia B (HB), and 25% (n = 513) had less common factor deficiencies. The estimated age-standardized incidence rate (ASR) of HA and HB was 1.78-3.15/100,000 and 0.31-0.51/100,000, respectively. That of vWD was 1.38-1.95/100,000. The estimated ASR of HA showed increase over time though the number of new patients did not increase. Most patients with congenital bleeding disorders were younger than 19 years old (47.8%), and most were registered in Gyeonggi (22.1%) and Seoul (19.2%). CONCLUSION: This is the first nationwide population-based study of congenital bleeding disorders in Korea. This study provides data that will enable more accurate estimations of patients with vWD. This information will help advance the comprehensive care of congenital bleeding disorders. We need to continue to obtain more detailed information on patients to improve the management of these diseases.

Hemoglobin Concentration Reference Interval Revisited: a Nationwide Study from Korea.

BACKGROUND: Anemia is a common cause among the elderly for increased mortality. Hemoglobin concentration can be affected by many factors, but the reference interval defined by the World Health Organization has not been adjusted for the previous half century. METHODS: Through using the dataset generated by the National Health Insurance (NHI) health screening program of Republic of Korea, here we attempt to present a close to actual hemoglobin concentration of the Korean population. Between January 2009 and December 2013, a total of 57,409,872 health screening events were registered in the NHI database. Following the exclusion criteria, 6,759,566 participants were enrolled for analyses. RESULTS: Significant portion of the study population was considered 'anemic', while the mean value (2.5% ~ 97.5%) of hemoglobin concentration from the study was 14.8 (12.5 ~ 16.8) g/dL in men and 12.8 (10.6 ~ 14.7) g/ dL in women. Concordant results of hemoglobin concentration declining with age were observed as previous studies have described, supporting the need for separate, possibly lower cutoff in the elderly. CONCLUSIONS: A considerable portion of the participants being categorized as anemia contests the accuracy of the current lower cutoff for anemia. From a large representative dataset, the need for adjustment to the lower cutoff for anemia is suggested.

Real world epidemiology of myeloproliferative neoplasms: a population based study in Korea 2004-2013.

Myeloproliferative neoplasms (MPNs), with an expected increment in number, impose substantial economic and social burdens. To this end, we conducted a nationwide population-based descriptive epidemiology study. We also investigated medical cost associated with MPNs. Prevalence was the highest for essential thrombocythemia (ET) (range 4.1-9.0 per 100,000), followed by polycythemia vera (PV) (range 2.8-5.4 per 100,000) and primary myelofibrosis (PMF) (range 0.5-0.9 per 100,000). ET incurred the highest cumulative total cost at US$35 million and the most frequent hospital visits, while PMF incurred the highest average cost per person at US$5000. The mean hemoglobin level was 16.9 ± 2.2 g/dL for PV males and 15.5 ± 2.7 g/dL for PV females. Further analyses on hemoglobin levels showed the true positive rate of PV from the significantly elevated hemoglobin group (defined as >18.5 g/dL for men and >16.5 g/dL for women) was 3.01% and that of MPNs was 3.1%. Here, we provide the biggest population-based report on MPN epidemiology that can readily be used as a representative Asian data.

Pure Red Cell Aplasia Associated with Monoclonal Gammopathy of Undetermined Significance and Literature Review.

BACKGROUND: Pure red cell aplasia (PRCA) is an uncommon disease which involves an almost complete absence of the erythroid lineage in bone marrow (BM) and causes severe anemia. Cases due to monoclonal gammopathy occurring in plasma cell disorder have been infrequently reported. Here we report a case of PRCA associated plasma cell disorder, especially monoclonal gammopathy of undetermined significance (MGUS). METHODS: A 55-year-old male visited the ER due to general weakness. At his initial visit he exhibited severe anemia. Mild intravascular hemolysis was suspected. For anemia evaluation, BM examination was performed. In BM aspiration, almost no erythroid precursor cells were observed. Also, plasma cells were relatively elevated, at 7.2%. Serum electrophoresis and immunofixation revealed paraproteinemia of 5.1 g/L (IgG and lambda). No hypercalcemia, renal insufficiency or lytic bone lesions were found. This unusual case showed MGUS accompanied by PCRA. We were also able to assume the erythroid cell-specific restriction due to paraprotein, because we ruled out possible causes of PRCA. RESULTS: We discovered several reported cases associated with plasma cell dyscrasia. However, most of these cases involved plasma cell myeloma, characterized by high immunoglobulin burden. Our case demonstrates that PRCA is also observed in cases with MGUS, where immunoglobulin burden is low. CONCLUSIONS: It is not yet accurately known, what parts of erythroid precursors are targeted by M-protein nor what the mechanism is. Therefore, additional research into this matter is necessary.

Cytogenetic profiles of 2806 patients with acute myeloid leukemia-a retrospective multicenter nationwide study.

The cytogenetic and molecular data is recognized as the most valuable prognostic factor in acute myeloid leukemia (AML). Our aim was to systemically analyze the cytogenetics of Korean AML patients and to compare the cytogenetic profiles of various races to identify possible geographic heterogeneity. We retrospectively reviewed medical records of 2806 AML patients diagnosed at 11 tertiary teaching hospitals in Korea between January 2007 and December 2011. The most common recurrent chromosomal abnormality was t(8;21) (8.8 %, 238/2717), but t(15;17) showed an almost same number (8.6 %,235/2717). Among de novo AML, the most frequent aberrations were t(15;17), observed in 229 (10.7 %). The most common French-American-British (FAB) classification type was M2 (32.2 %), and recurrent cytogenetic abnormalities correlated with the FAB subtypes. Among 283 secondary AML cases, myelodysplastic syndrome was the most common predisposing factor. About 67.1 % of the secondary AML cases were associated with chromosomal aberrations, and chromosome 7 abnormalities (n = 45, 15.9 %) were most common. The incidence of FLT3 internal tandem duplication mutation was relatively low at 15 %. Our study reports certain similarities and differences in comparison to previous reports. Such discrepancies call for extensive epidemiological studies to clarify the role of genetic as well as geographic heterogeneity in the pathogenesis of AML.

Familial aplasia cutis congenita associated with mega-cisterna magna.

Aplasia cutis congenita (ACC; MIM 107600) is a congenital skin disorder that manifests as localized absence of skin. Here we report a case of familial ACC and mega-cisterna magna. A female neonate was born with skin defects on the scalp. Brain magnetic resonance imaging demonstrated retrocerebellar space widening suggesting mega-cisterna magna. Her father also had a skin defect on the scalp at birth, and brain computed tomography of the father showed a cystic lesion over the right occipital lobe, similar to the patient's brain imaging. Karyotype 46,XX, t(6;18)(q23.2;q11.2) was identified on G-banded karyotype analysis of the patient and her father, after which whole exome sequencing was carried out, but this was thought to be a coincidental finding. This indicates that ACC may be associated with brain anomaly, although it is very rare.

Mean platelet volume in patients with increased γ-glutamyl transferase.

Mean platelet volume (MPV) is the commonly measured platelet index for platelet size and surrogate marker of platelet activation. Changes of MPV in hepatic disease have been evaluated in inflammatory conditions, fibrosis and malignancy. We also had reported previous studies to analyze clinical meaning of MPV in patients with hepatocellular carcinoma and chronic hepatitis B. However, data about investigation of MPV in cholestatic conditions are not sufficient so far. Recently, it has been reported that MPV was increased in intrahepatic cholestasis of pregnancy. In this study, we planned to investigate the relationship between MPV and glutamyltransferase which is well-established laboratory marker for cholestasis, in various disease conditions.

Changes in cervical muscle activity according to the traction force of an air-inflatable neck traction device.

[Purpose] The purpose of this study was to analyze cervical muscle activity at different traction forces of an air-inflatable neck traction device. [Subjects] Eighteen males participated in this study. [Methods] The subjects put on an air-inflatable neck traction device and the traction forces administered were 40, 80, and 120 mmHg. The electromyography (EMG) signals of the splenius capitis, and upper trapezius were measured to assess the muscle activity. [Results] The muscle activity of the splenius capitis was significantly higher at 80, and 120 mmHg compared to 40 mmHg. The muscle activity of the upper trapezius did not show significant differences among the traction forces. [Conclusion] Our research result showed that the air-inflatable home neck traction device did not meet the condition of muscle relaxation.

De novo hairy cell leukemia with a major BCR/ABL1 rearrangement: a case report with a literature review.

Hairy cell leukemia (HCL) is a very rare mature B-cell neoplasm and its simultaneous occurrence with chronic myeloid leukemia has been reported in only three cases. The pathogenesis and relationship of the two diseases are not clear. Here we report a case of HCL expressing a BCR/ABL1 clone, which showed molecular remission of the fusion clones and achieved partial remission over nine months of cladribine therapy. After a thorough analysis of previous studies and the results of this patient, we speculate that a subclone evolved to have an additional genetic BCR/ABL1 rearrangement. We also review all published literature on HCL with BCR/ABL1 rearrangement and discuss the pathophysiology of these unusual cases.

Mean platelet volume and mean platelet volume/platelet count ratio in infective endocarditis.

Infective endocarditis (IE), an infection of the endocardial surface, frequently leads to life-threatening complications, such as thromboembolism due to platelet activation. We investigated the mean platelet volume (MPV) in Korean patients with IE and the serial changes thereof, in comparison with other laboratory parameters. We analyzed 248 MPV results from 22 patients diagnosed with IE in our hospital between January 2011 and April 2012. MPV was measured with an Advia 2120 (Siemens Healthcare Diagnostics, Tarrytown, NY) using EDTA-containing tubes. The mean MPV differed significantly between the patient and control groups, 8.74 vs. 7.96 fl, respectively. In addition, the platelet count and MPV/platelet count ratio were significantly decreased in the patient group. The total platelet mass and platelet size in IE might be increased. Further studies should examine more patients to verify the changes in the MPV and MPV/platelet count ratio in IE and assess in greater detail the relationship between MPV and thrombotic complications caused by platelet activation.

Masked monoclonal gammopathy in capillary electrophoresis.

BACKGROUND: Although the method of choice to detect M-protein is electrophoresis on an agarose gel, such gel electrophoresis (GE) is labor-intensive, time-consuming, and not standardized. In contrast to GE, capillary electrophoresis (CE) has some merits because it is automated, fast, and highly reproducible. However, CE results occasionally make the interpretation difficult and require additional confirmatory tests like GE. METHODS: In order to assist a correct reporting of CE results and compatible interpretations between two different electrophoresis methods, we report here two unusual cases of monoclonal gammopathy by a pattern of polyclonal gammopathy upon CE interpretation in patients with end stage renal disease and multiple myeloma. RESULTS: In these cases, serum CE showed the broad bumpy peak in the gamma region. This bumpy peak does not drop completely flat after the reaction with anti-FLC. CONCLUSIONS: Because the plasma cell is a B-cell lineage and plays an important role in adaptive immunity, MG accompanying with PG is not rarely found in plasma cell dyscrasia. If the broad bumpy peak is observed in CE, careful examinations must be done to rule out the hidden M-peak. In our cases, a parallel use of gel-based methods was very helpful as it revealed monoclonal bands.

Free light chain levels in pediatric patients with inflammatory conditions.

BACKGROUND: Because free light chain assays measure polyclonal as well as monoclonal free light chain components, some previous studies focused on the potential utility of the free light chain assay for detecting chronic immune stimulation, which occurs in autoimmune diseases and allergies. However, most of these trials have been limited to adult patients. In this study, we evaluated the paired serum and urinary free light chain levels of pediatric patients with various inflammatory conditions to investigate the clinical significance of free light chain measurement in pediatrics. METHODS: The study included 227 paired serum and urine specimens from 134 pediatric patients at our hospital between January and February of 2012. Serum and urinary FLC levels were measured using a Freelight Kit (The Binding Site, Ltd., Birmingham, UK). RESULTS: The serum lambda and urine kappa and lambda components were significantly increased only in the renal impairment group, not in the mild inflammatory group. FLC ratios were not significantly different among these groups. CONCLUSIONS: In serum, only the L components were significantly increased. This result may indicate the presence of a dimeric L structure, in contrast with monomeric K. FLC levels might also be influenced by renal conditions other than mild inflammation. Therefore, as shown in previous studies of adult patients, renal reference ranges might be needed to interpret FLC results, especially for dimeric L components.

Comprehensive Assessment of Lower Limb Function and Muscle Strength in Sarcopenia: Insights from the Sit-to-Stand Test.

The sit-to-stand test is an essential tool used to assess lower limb function and muscle strength in older adults and various patient populations, and also plays a role in sarcopenia screening. Among its forms, the five-time sit-to-stand test (FTSST) is widely used, with previous studies suggesting cutoff values of >10 seconds and >11 seconds for the sitting-to-standing and standing-to-sitting transitions, respectively. The 30-second and 1-minute sit-to-stand tests (30STS and 1MSTS, respectively) also provide comprehensive assessments. While much of the current research on sarcopenia focuses on the FTSST, there is a burgeoning need for an in-depth exploration of the 30STS and 1MSTS. Studies on these tests are vital to refine the criteria for sarcopenia, establish accurate cutoff values, and enhance diagnostic precision and treatment effectiveness. This need highlights the importance of further research into the 30STS and 1MSTS for refining the diagnostic criteria for sarcopenia.

Differences in muscle activity during squat exercises according to the knee angle and standing angle of a sliding tilt table: A comparative study.

BACKGROUND AND PURPOSE: Weight-bearing exercises can help improve physical function; however, they may be difficult to perform in patients with neurological issues or lower limb fractures. The purpose of this study was to evaluate the activity of the leg muscles during squatting when using a new exercise aid designed to help patients with difficulty in performing squats, termed the sliding tilt table, at different knee angles and to estimate the squat level for each angle. METHODS: This study was a cross-sectional, observational study performed in the general community. Participants comprised 30 healthy adult men. Squats were performed by setting the knee joints at 30, 60, and 90° and the tilt table angles to 15, 30, and 45°. The muscle activity of the vastus medialis, tibialis anterior, and gastrocnemius was measured using surface electromyography. RESULTS: As the tilt table and knee joint angle increased, the %maximal voluntary isometric contraction increased. Similar patterns were found in both legs. CONCLUSIONS: Weight-bearing exercises, such as squats, are important exercise elements in patients who require lower limb rehabilitation. Therefore, it is necessary to provide efficient lower limb rehabilitation exercises by adjusting the squat level according to the patient's ability, which can be achieved using a sliding tilt table.

Newborn brachial plexus palsy: evaluation of severity using quantitative ultrasound of muscle.

INTRODUCTION: We studied ultrasound features of muscle after nerve injury. METHODS: We evaluated ultrasound measurements of muscle thickness and backscatter in injured and contralateral uninjured elbow flexors of 51 children with newborn brachial plexus palsy (NBPP) and compared the results to elbow flexor function (Active Movement Scale), defined as normal, moderate, or severe. RESULTS: Compared with uninjured limbs, muscle in injured arms was 15% thinner with severe impairment, 17% thicker with moderate impairment, and no different with normal function. Relative to uninjured limbs, moderately impaired muscle was thicker than both severely impaired and normal strength muscle. Backscatter was higher in injured than in uninjured limbs regardless of function. In 17 patients with sequential measures, muscle thickness, but not backscatter, increased with function over time. CONCLUSIONS: Muscle thickness differentiates moderate from severe impairment after NBPP and increases with recovery over time. Muscle backscatter identifies prior injury regardless of function.

Increased mean platelet volume and mean platelet volume/platelet count ratio in Korean patients with deep vein thrombosis.

The mean platelet volume (MPV) is a laboratory marker associated with platelet function and activity. Increased MPV in thromboembolic disease is considered an important risk factor. The aim of this study was to compare the MPV and MPV/platelet count (MPV/P) ratio between deep vein thrombosis (DVT) and control subjects. We retrospectively reviewed the medical records of patients (n = 91) admitted due to newly diagnosed DVT from December 2010 to March 2012. The control group (n = 311) underwent health screening at our Hospital. Median MPV was higher in DVT patients compared to controls (8.6 fl vs. 7.9 fl, p < 0.0001). The DVT patients also had a higher MPV/P ratio compared to the control group (0.0388 fl/(10(9)/l) vs. 0.0308 fl/(10(9)/l), p < 0.0001). MPV was inversely correlated with platelet count in DVT patients (correlation coefficient = -0.33, p = 0.001). Receiver operator characteristic analysis revealed that an MPV cutoff value of 8.2 fl provided 70.3% sensitivity and 72.7% specificity. An MPV/P cutoff value of 0.0363 fl/(10(9)/l) showed 60% sensitivity and 73% specificity. MPV and MPV/P ratio could be considered meaningful laboratory markers for the risk of DVT.

Mean platelet volume/platelet count ratio in hepatocellular carcinoma.

Mean platelet volume (MPV) has been actively investigated in liver disease such as steatosis, cirrhosis and hepatitis. Recently, MPV/platelet count (PC) ratio has been proposed as a predictor of long-term mortality after myocardial infarction. As PC is known to be decreased in various liver diseases such as cirrhosis, hepatosplenomegaly and malignancy, we planned to evaluate MPV/PC ratio in patients with hepatocellular carcinoma (HCC) in this study. Mean of MPV levels showed significant difference, which were 8.69 fl (range 6.7-12.2 fl) in patients group and 8.02 fl in control group (range 6.7-11.0 fl). In receiver operating characteristic (ROC) curve analysis, the MPV/PC ratio (fl/(10(9)/l)) presented 74.5% of sensitivity and 96.5% of specificity at the criterion > 0.0491 (area under the curve (AUC) = 0.884), while MPV alone showed 57.4% of sensitivity and 81.4% of specificity at the criterion > 8.4 fl. Further studies should evaluate underlying pathogenic mechanisms of MPV/PC ratio difference and various possibilities of this ratio as an indicator of presence of a tumor in HCC.