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Objectives We evaluated the familial risk of seropositive rheumatoid arthritis (RA) and examined interactions between family history and smoking. Methods Using the National Health Insurance and Health Screening Program databases, which include information on familial relationships and lifestyle factors, we identified 5 524 403 individuals with first-degree relatives (FDRs) from 2002–2018. We calculated familial risk using hazard ratios (HRs) with 95% CIs which compare the risk of individuals with and without affected FDRs. Interactions between smoking and family history were assessed on an additive scale using the relative excess risk due to interaction (RERI). Results Individuals with affected FDR had 4.52-fold (95% CI 3.98, 5.12) increased risk of disease compared with those with unaffected FDR. Familial risk adjusted for lifestyle factors decreased slightly (HR 4.49), suggesting that a genetic contribution is the predominant driver in the familial aggregation of RA. Smoking was associated with an increased risk of disease that was more pronounced among heavy (HR 1.92 95% CI 1.70, 2.18) compared with moderate (HR 1.15 95% CI 1.04, 1.28) smoking. In the interaction analysis, the risk associated with the combined effect of smoking and family history was higher than the sum of their individual effects, though statistically non-significant (RERI 1.30 95% CI ‒0.92, 3.51). Heavy smokers with a positive family history showed a prominent interaction (RERI 4.13 95% CI ‒0.88, 9.13) which exceeded moderate smokers (RERI 0.61 95% CI ‒1.90, 3.13), suggesting a dose-response interaction pattern. Conclusion Our findings indicate the possibility of an interaction between RA-associated genes and smoking. [ABSTRACT FROM AUTHOR]

Objective Although air stacking is a widely used lung expansion therapy essential for restrictive lung diseases, important details such as peak insufflation pressure (PIP) and number of squeezes have not been investigated. The purpose of this study was to standardize a method of air stacking to minimize problems with its application by identifying the optimal pressure and number of squeezes performed by professional physicians and investigating the current status of routine air stacking implementation in patients. Methods This prospective cross-sectional test–retest study involved individuals who had neuromuscular disorders and had performed air stacking exercise for longer than 1 year. PIP and number of squeezes were measured to identify the differences between caregivers and physicians. Cases of incorrectly performed air stacking were investigated and categorized. The problems associated with air stacking were evaluated. Results A total of 45 participants were included. PIP was 41.4 (SD = 4.2; range = 34.8–50.0) cm H2O, and optimal number of squeezes was 3.1 (SD = 0.5; range = 2–4). When the air stacking methods used by caregivers were evaluated, 19 of 45 caregivers (42.2%) used methods inappropriately. Higher PIP and larger number of squeezes were observed with caregiver implementation. Thirty caregivers (66.7%) experienced finger or wrist pain; this problem was observed especially in female caregivers, who tended to incorrectly perform air stacking. Conclusions This pilot study showed that the optimal pressure of air stacking was 35 to 50 cm H2O. Caregivers often perform air stacking inappropriately, leading to complications without achieving the purpose of air stacking. The introduction of a new method, such as manometry, can be helpful for achieving optimal air stacking. Impact Optimal pressure of air stacking can be measured with inexpensive, simple, and commercially available digital manometry. This approach enables air stacking to be performed and taught more accurately and efficiently and reduces complications in both patients and caregivers. [ABSTRACT FROM AUTHOR]

Objectives To assess the efficacy of an endothelin receptor antagonist (ERA) and phosphodiesterase type5 inhibitors (PDE5is) for treating SSc-related digital ulcers (DUs). Methods This prospective, multicentre, observational cohort study recruited patients with active SSc-related DUs from 13 medical centres in South Korea. The primary outcome was time to cardinal ulcer (CU) healing. A secondary outcome was time to new DU occurrence. Patients were followed up 4, 8, 12 and 24 weeks after treatment initiation. Results Sixty-three patients were analysed. Their mean age was 49.9 years (s. d. 11.4) and 49 were female. Twenty-eight had limited SSc. Forty-nine patients received ERA, 11 received a PDE5i (9 sildenafil, 1 udenafil and 1 tadalafil) and 3 received other medication. The hazard ratio (HR) for time to CU healing in the ERA group vs the PDE5i group was 0.75 (95% CI 0.35, 1.64; P  = 0.47) in an unadjusted model and 0.80 (95% CI 0.36, 1.78; P  = 0.59) in a model adjusted for age, sex, use of calcium channel blockers (CCBs), total DU number and initial CU area. The HR for new DU development in the ERA group vs the PDE5i group was 0.39 (95% CI 0.16, 0.93; P  = 0.03) in an unadjusted model and 0.32 (95% CI 0.13, 0.81; P  = 0.02) in an adjusted model. No patients receiving CCBs developed new DUs at 24 weeks. Conclusion Time to CU healing is comparable for ERA and PDE5i. ERAs are more effective in reducing new DU occurrence than PDE5is. CCBs may be effective as a background medication. [ABSTRACT FROM AUTHOR]

Background: With the advancement of cardiorespiratory interventions, the survival rate among patients with Duchenne muscular dystrophy (DMD) has increased. Subsequently, pneumothorax has become a significant problem in patients with prolonged ventilatory support.Research Question: What are the frequency, recurrence rate, risk factors, and prognosis of pneumothorax in patients with DMD requiring noninvasive ventilation (NIV)? Also, are there known risk factors of pneumothorax on chest CT scans?Study Design and Methods: This retrospective longitudinal cohort study included 176 patients treated between 2006 and 2019. We collected information regarding location, severity, treatment methods, recurrence frequency, abnormal findings on CT scanning, and date of death. We compared the pneumothorax and nonpneumothorax groups. We calculated the estimated survival probabilities from the age at NIV application according to pneumothorax occurrence.Results: Sixteen of the 176 patients (9.0%) experienced pneumothorax (median age at diagnosis, 24.6 years; range, 20.7-33.7 years). Among the 16 patients, 15 demonstrated pneumothorax after NIV application (median time between diagnosis and initial NIV application, 5.6 years; range, 3 days-9.6 years). Sixteen patients experienced 31 episodes of pneumothoraces (range, one-five episodes); among them, seven episodes (22.6%) were asymptomatic. Known risk factors not clearly visible by radiography scans were found in chest CT scan in 11 patients (68.8%). Seven of 16 patients (43.8%) eventually sustained severe lung damage with pulmonary fibrosis. No significant between-group differences were found in body weight, BMI, and age at NIV application; however, the pneumothorax group showed a significantly higher mortality rate after NIV application.Interpretation: On pneumothorax occurrence in patients with DMD, recurrences and severe lung damage are common; moreover, these patients show higher mortality rates than patients without pneumothorax. Chest CT scans should be performed to identify risk factors, and treatment should be initiated accordingly. In addition, physicians should consider chest CT scanning in the case of suspected pneumothorax, even if no radiographic abnormality is found. [ABSTRACT FROM AUTHOR]

Objectives: We performed preoperative ultrasonography (US) to detect the anatomic course of the superficial radial nerve (SRN) and dominant pathologic tendon of the first extensor compartment in de Quervain tenosynovitis. Methods: We prospectively studied 27 patients (29 wrists) with de Quervain tenosynovitis who underwent surgical release of the first extensor compartment. Preoperatively, US was performed to evaluate the presence of the dominant pathologic tendon and the septum in the subcompartment, number of SRNs in the area of the surgical incision, and anatomic running course of the SRN. These variables were also checked intraoperatively. Cohen κ statistics were calculated to investigate agreement between US and surgical field findings. Results: There were 7 men and 20 women (mean age, 47.8 years; range, 26–67 years). For the dominant pathologic tendon, there were 2 cases (6.9%) of an abductor pollicis longus, 11 cases (37.9%) of an extensor pollicis brevis, and 16 cases (55.2 %) of a nondominant tendon (κ = 0.94). For the subcompartment, there were 10 cases (34.5%) without a septum, 8 (27.6%) with an incomplete septum, and 11 (37.9%) with a complete septum (κ = 0.95). Most SRNs crossed over the first extensor compartment (κ = 0.78). Conclusions: Preoperative US can be useful in detecting the anatomic running course of the SRN and dominant pathologic tendon before surgery for de Quervain tenosynovitis. Classifying the anatomic course of the SRN could be essential to planning surgery, and it could be helpful to prevent injury of the SRN during surgery. [ABSTRACT FROM AUTHOR]

Background: Cervical spondylotic myelopathy (CSM) is the most common cause of cord dysfunction in older individuals. CSM involves either the upper motor neuron or lower motor neuron (LMN) in the cervical spine. The neurologic level of the LMN lesion does not usually correspond with the structural level detected via magnetic resonance imaging (MRI).Objective: To examine the relationship between the structural level of stenosis and the neurologic level of LMN involvement in patients with CSM.Design: Retrospective descriptive study.Setting: Tertiary hospital.Patients: Patients with cervical spondylotic myelopathy, aged 41-79 years.Methods: We reviewed electromyography (EMG) and magnetic resonance imaging (MRI) findings from 17 patients with CSM to objectively show the relation between the structural level of stenosis and the neurologic level with LMN involvement.Main Outcome Measurements: The structural level was defined by spinal canal stenosis with definite cord compression on MRI. The neurologic level was determined by myotomes with abnormal spontaneous activity on EMG.Results: In all patients but one, myotomes with abnormal spontaneous EMG activity were one to 4 levels lower than the stenotic canal shown on MRI.Conclusions: LMN involvement in CSM is usually not concordant with the structural lesion. For accurate diagnosis and treatment, physicians should recognize that myotomal involvement in CSM is often due to canal stenosis one to 4 levels above the lesion.Level Of Evidence: III. [ABSTRACT FROM AUTHOR]

Objectives To serially test the International Classification of Headache Disorders (ICHD) 3rd edition beta version criteria for 6.7.3.1 probable reversible cerebral vasoconstriction syndrome (probable RCVS) in patients with thunderclap headache. Methods We prospectively screened consecutive patients with thunderclap headache who visited the Samsung Medical Center between October 2015 and March 2017. Patients were included in the analysis if they a) visited our hospital within 1 month after onset, b) completed a diagnostic work-up, and c) had no secondary causes other than RCVS. The ICHD-3 beta 6.7.3.1 criteria were evaluated serially during the first visit (visit 1), 2 weeks after the first visit (visit 2), and 1-3 months after onset (visit 3). Results A total of 99 patients completed this study. Based on the first clinical and radiological evaluation, 63 (63.6%) were diagnosed with angiogram-proven RCVS, whilst 36 (36.4%) showed normal neuroimaging. The ICHD-3 beta 6.7.3.1 criteria were fulfilled in 76.2% of patients with angiogram-proven RCVS. In patients with normal angiograms, the ICHD diagnosis of probable RCVS changed over time: 25.0%, 47.2%, and 38.9% at visits 1, 2, and 3, respectively. The sensitivity and specificity of the criteria at visit 1 were 72.4% and 95.5%, respectively, for the prediction of a final diagnosis of overall (angiogram-proven + probable) RCVS. Conclusions The ICHD-3 beta criteria for probable RCVS are applicable for patients with thunderclap headaches at the acute stage, with the exception of criterion C3. Criteria C1 and C2 are less reliable when applied at the first visit. Repeated evaluation is necessary to enhance diagnostic sensitivity. [ABSTRACT FROM AUTHOR]

Introduction: We aimed to analyze the clinical and genetic characteristics of collagen VI-related myopathy.Methods: We analyzed the clinical course and mutation spectrum in patients with collagen VI gene mutations among our congenital muscular dystrophy cohort.Results: Among 24 patients with mutations in collagen VI coding genes, 13 (54.2%) were categorized as Ullrich type, and 11 (45.8%) as non-Ullrich type. Congenital orthopedic problems were similarly observed in both types, yet multiple joint contractures were found only in the Ullrich type. Clinical courses and pathology findings varied between patients. Mutations in COL6A1, COL6A2, and COL6A3 were found in 15 (65%), 3 (13%), and 5 (22%) patients, respectively, without genotype-phenotype association. Five novel variants were detected.Discussion: We verified clinical heterogeneity of collagen VI-related myopathy, which emphasizes the importance of genetic testing. Genotype-phenotype association or early predictors for progression were not identified. Multiple joint contractures predict rapid deterioration. Muscle Nerve 58: 381-388, 2018. [ABSTRACT FROM AUTHOR]

Objective To determine the natural course of cluster headache. Methods We screened patients with cluster headache who were diagnosed at Samsung Medical Center and lost to follow-up for ≥5 years. Eligible patients were interviewed by phone about the longitudinal changes in headache characteristics and disease course. Remission was defined as symptom-free 1) for longer than twice the longest between-bout period and 2) for ≥5 years. Results Forty-two patients lost to follow-up for mean 7.5 (range, 5.0-15.7) years were included. The length of the last bout did not differ from the first one, while the last between-bout period was longer than the first one ( p = 0.012). Characteristics of cluster headache decreased over time: Side-locked unilaterality (from 92.9% to 78.9%), seasonal and circadian rhythmicity (from 63.9% to 60.9% and from 62.2 to 40.5%, respectively), and autonomic symptoms (from 95.2% to 75.0%). Remission occurred in 14 (33.3%) patients at a mean age of 42.3 (range, 27-65) years, which was not different from the age of last bouts in active patients ( p = 0.623). There was a trend for more seasonal and circadian predilection at baseline in the active group ( p = 0.056 and 0.063, respectively) and fewer lifetime bouts and shorter disease duration in patients in remission ( p = 0.063 and 0.090). Conclusions This study first shows the natural courses of cluster headache. Features of cluster headache become less prominent over time. Remission occurred regardless of age. Although no single predictor of remission was found, our data suggest that remission of cluster headache might not be a consequence of more advanced age, longer duration of disease, or accumulation of lifetime bouts. [ABSTRACT FROM AUTHOR]

Background: Sedentary lifestyle, a prevalent finding in modern society, can lead to weakness of the lumbar trunk musculature. Electrical stimulation (ES), in addition to lumbar strengthening exercises, has been shown to increase muscle strength.Objective: To assess the strengthening effect of ES on the lumbar paraspinal muscles during prolonged sitting.Design: Randomized, controlled, single-blind study.Setting: Tertiary hospital.Participants: Thirty healthy subjects without low back pain.Methods: Thirty participants were randomly assigned to the ES group (n = 15) or the control group (n = 15). ES was administered over the lumbar paraspinal muscles, while subjects maintained a seated posture, at a maximal tolerable intensity for 60 minutes per day, three times per week, for 4 consecutive weeks. An identical protocol was used in the control group with muscle stimulation provided at the minimal sensory threshold intensity.Main Outcome Measurements: The isokinetic strength of the lumbar paraspinal muscles was evaluated at baseline, 4 weeks after the initiation of ES, and 8 weeks after the initiation of ES with an isokinetic dynamometer (Biodex Medical Dynamometer System-4) at 60°/s and 120°/s angular velocities.Results: The extension and flexion peak torque at 60°/s showed no significant interaction between the time and intervention or between the interventions. However, after 4 weeks of ES in the sitting position, the isokinetic extensor trunk muscle strength measured at 120°/s angular velocity significantly increased in the ES group (P < .05) compared with that in the control group. This effect was not maintained when measured at 4 weeks after the cessation of ES.Conclusions: ES in a sitting position is a potentially effective and accessible rehabilitation treatment, which can lead to short-term improvement in the extensor trunk muscle strength of the lumbar paraspinal muscles.Level Of Evidence: I. [ABSTRACT FROM AUTHOR]

Objective: The purpose of this study was to assess the T2 values of the glenohumeral joint cartilage in healthy asymptomatic individuals at 3.0 T and to analyze the T2 profile of the humeral cartilage.Materials and Methods: This prospective study was approved by our institutional review board and written informed consent was obtained. Thirteen subjects (mean age, 28.6 years; age range, 24-33 years) were included and underwent multiecho spin-echo T2-weighted MR imaging and T2 mapping was acquired. Regions of interest were placed on the humeral cartilage and glenoid cartilage on oblique coronal images. T2 profiles of humeral cartilage were measured from the bone-cartilage interface to the articular surface. Intra-observer agreement was analyzed using intraclass correlation coefficient (ICC).Results: All 13 joints showed normal appearance on conventional T2-weighted images. The mean T2 values of humeral and glenoid cartilage were 50.5 ± 12.1 and 49.0 ± 9.9 ms, respectively. Intra-observer agreement was good, as determined by ICC (0.736). Longer T2 values were observed at the articular surface with a tendency to decrease toward the bone-cartilage interface. The mean cartilage T2 value was 69.03 ± 21.2 ms at the articular surface and 46.99 ± 19.6 ms at the bone-cartilage interface.Conclusion: T2 values of the glenohumeral joint cartilage were similar to reported values of cartilage in the knee. The T2 profile of normal humeral cartilage showed a spatial variation with an increase in T2 values from the subchondral bone to the articular surface. [ABSTRACT FROM AUTHOR]

Introduction: A change in vital capacity (VC) from standing to supine can be an index of diaphragm paralysis if it exceeds 25%. We aimed to verify whether the postural VC difference increases with age and reflects diaphragm weakness in DMD.Methods: VCs were measured in DMD. Postural VC difference and percentage were calculated from the VC data. Maximal inspiratory pressure (MIP) and MIP percentage were measured as an indirect index of diaphragm weakness.Results: A total of 220 patients and 544 measurements were collected. MIP and MIP percentage decreased significantly with age (P < 0.001 for both). Estimated postural VC difference and percentage also decreased (P < 0.001, P = 0.006, respectively). Age group comparisons showed a significant decrease in younger, but not older subjects.Conclusions: This study shows that the postural VC difference tended to decrease with age and might not reflect diaphragmatic weakness in DMD; however, this should be interpreted cautiously. [ABSTRACT FROM AUTHOR]

Background: Glenohumeral (GH) joint steroid injection is one of the most well-known treatments for frozen shoulder. However, the low accuracy of GH joint injections and the improvement of symptoms after subacromial (SA) steroid injections led us to design a study that compares the efficacy of a steroid injection for primary frozen shoulder according to the injection site. Materials and methods: Patients with primary frozen shoulder were randomly divided into 2 groups according to the location of the injection: a GH group of 37 for the glenohumeral joint and an SA group of 34 for the subacromial space. Injections were completed using ultrasonographic guidance. Evaluations using a visual analog scale (VAS) for pain, the Constant score, and passive range of motion (ROM) were completed at 3, 6, and 12 weeks after the injection. Results: The GH group showed lower pain VAS at 3 weeks, but no statistical difference was found between the 2 groups at 6 and 12 weeks. Improvement in pain was evident at every follow-up visit compared with the preinjection evaluation. There was no significant difference between the 2 groups with respect to the Constant score or ROM at serial follow-up. Conclusions: The GH steroid injection was not superior to a SA injection for patients with primary frozen shoulder even though injection at the GH joint led to earlier pain relief compared with the SA injection. SA steroid injection along with a GH injection is an alternative modality, and the treatment should be individualized and tailored appropriately. [ABSTRACT FROM AUTHOR]

Dynamic arterial elastance (Eadyn) has been suggested as a functional measure of arterial load. We aimed to evaluate whether pre-induction Eadyn can predict post-induction hypotension. Prospective observational study. Adult patients undergoing general anesthesia with invasive and non-invasive arterial pressure monitoring systems. We collected invasive and non-invasive Eadyns (n = 38 in each), respectively. In both invasive and non-invasive Eadyns, pre-induction Eadyns were obtained during one-minute tidal and deep breathing in each patient before anesthetic induction. Post-induction hypotension was defined as a decrease of >30% in mean blood pressure from the baseline value or any absolute mean blood pressure value of <65 mmHg for 10 min after anesthetic induction. The predictabilities of Eadyns for the development of post-induction hypotension were tested using receiver-operating characteristic curve analysis. Invasive Eadyn during deep breathing showed significant predictability with an area under the curve (AUC) of 0.78 (95% Confidence interval [CI], 0.61–0.90, P = 0.001). But non-invasive Eadyn during tidal breathing (AUC = 0.66, 95% CI, 0.49–0.81, P = 0.096) and deep breathing (AUC = 0.53, 95% CI, 0.36–0.70, P = 0.75), and invasive Eadyn during tidal breathing (AUC = 0.66, 95% CI, 0.41–0.74, P = 0.095) failed to predict post-induction hypotension. In our study, invasive pre-induction Eadyn during deep breathing -could predict post-induction hypotension. Despite its invasiveness, future studies will be needed to evaluate the usefulness of Eadyn as a predictor of post-induction hypotension because it is an adjustable parameter. • Post-induction hypotension develops frequently, with an incidence as high as 36.5%. • Although, many studies have identified the predictors of post-induction hypotension, most predictors are not changeable. • Dynamic arterial elastance measured invasively during deep breathing can predict post-induction hypotension. • Despite its invasiveness, dynamic arterial elastance is useful, because it is an adjustable parameter. [ABSTRACT FROM AUTHOR]