High Creatine Kinase Levels Research Articles

Anti-Mi2 Antibody Positive Dermatomyositis With Hyper-Elevated Creatine Kinase: A Case Report

Dermatomyositis (DM) is a critical disease that affects the quality of life of middle-aged and older patients. The clinical findings of DM can be determined by serological profiles of autoantibodies specific to DM. We report the case of a 65-year-old female patient with anti-Mi-2 antibody-positive DM and severe muscular findings. She endured muscular symptoms for three months without appropriate treatment. The patient was successfully treated with prednisolone, azathioprine, and intravenous immunoglobulins. This case highlights the importance of intensive treatment of DM with extremely high creatine kinase levels with steroids, immunosuppressants, and immunoglobulin treatments and the necessity of education on help-seeking behaviors in dealing with symptoms among rural older people to prevent the progression of autoimmune diseases and treat them at an early stage.

Delayed diagnosis of complex glycerol kinase deficiency in a Chinese male infant: a case report

BackgroundXp21 contiguous gene deletion syndrome is a rare genetic metabolic disorder with poor prognosis in infants, involving deletions of one or more genes in Xp21. When deletions of adrenal hypoplasia (AHC), Duchenne muscular dystrophy (DMD), and chronic granulomatosis (CGD) loci are included, complex glycerol kinase deficiency (CGKD) can be diagnosed. We present a case of CGKD that was initially misdiagnosed and died during treatment in our hospital in terms of improving our understanding of the clinical features and diagnosis of this disease, as well as highlighting the need for more precise dosing of corticosteroid replacement therapy.Case presentationA 48-day-old full-term male infant was transferred to our medical center with global growth delay and persistent vomiting. Routine laboratory tests revealed hyperkalemia, hyponatremia, and a high level of creatine kinase. The initial diagnosis was adrenal cortical hyperplasia (ACH), then revised to adrenocortical insufficiency with a normal level of ACTH detected. After supplementing the routine lipid test and urinary glycerol test, CGKD was diagnosed clinically due to positive triglyceridemia and urinary glycerol, and the follow-up gene screening further confirmed the diagnosis. The boy kept thriving after corticosteroid replacement and salt supplementation. While levels of serum ACTH and cortisol decreased and remained low after corticosteroid replacement was administered. The patient died of acute type 2 respiratory failure and hypoglycemia after an acute upper respiratory tract infection, which may be the result of adrenal crisis after infection. Infants with CGKD have a poor prognosis, so physicians should administer regular follow-ups, and parents counseling during treatment to improve the survival of patients.ConclusionsOverall, CGKD, although rare, cannot be easily excluded in children with persistent vomiting. Extensive blood tests can help to detect abnormal indicators. Adrenal crisis needs to be avoided as much as possible during corticosteroid replacement therapy.

Antisense Morpholino-Based In Vitro Correction of a Pseudoexon-Generating Variant in the SGCB Gene.

Limb-girdle muscular dystrophies (LGMD) are clinically and genetically heterogenous presentations displaying predominantly proximal muscle weakness due to the loss of skeletal muscle fibers. Beta-sarcoglycanopathy (LGMDR4) results from biallelic molecular defects in SGCB and features pediatric onset with limb-girdle involvement, often complicated by respiratory and heart dysfunction. Here we describe a patient who presented at the age of 12 years reporting high creatine kinase levels and onset of cramps after strenuous exercise. Instrumental investigations, including a muscle biopsy, pointed towards a diagnosis of beta-sarcoglycanopathy. NGS panel sequencing identified two variants in the SGCB gene, one of which (c.243+1548T>C) was found to promote the inclusion of a pseudoexon between exons 2 and 3 in the SGCB transcript. Interestingly, we detected the same genotype in a previously reported LGMDR4 patient, deceased more than twenty years ago, who had escaped molecular diagnosis so far. After the delivery of morpholino oligomers targeting the pseudoexon in patient-specific induced pluripotent stem cells, we observed the correction of the physiological splicing and partial restoration of protein levels. Our findings prompt the analysis of the c.243+1548T>C variant in suspected LGMDR4 patients, especially those harbouring monoallelic SGCB variants, and provide a further example of the efficacy of antisense technology for the correction of molecular defects resulting in splicing abnormalities.

Anthropometric parameters of obesity can be alternative biomarkers for the potential cardiac dysfunction in obese children.

Childhood obesity, as one of the potential risk factors of cardiovascular diseases, is closely associated with the incidence of cardiovascular disease at a younger age and has become a public health concern worldwide. However, its potential effects on the cardiovascular system have still remained elusive. In this study, we systematically evaluated the cardiovascular characteristics of 79 obese children and 161 normal weight children in Guangzhou (China) using the potential biomarkers for cardiovascular disease. Compared with normal weight children, obese children not only exhibited significantly higher levels of creatine kinase (CK), lactate dehydrogenase (LHD), soluble fms-like tyrosine kinase-1 (s-Flt-1), high-sensitivity C-reactive protein (hs-CRP), and uric acid (UA) (p = 0.0062, 0.0012, 0.0013, 0.0225, and <0.0001, respectively) but also significantly higher diastolic blood pressure (p = 0.0074) and the heart rate (p = 0.0049) were found in obese children. Of 79 obese children, cardiac functions of 40 cases were further assessed by color Doppler echocardiography. The results showed that there were significant differences between the obesity group and the healthy weight group in terms of interventricular septal wall thickness at end-diastolic (IVSd), the left ventricular posterior wall thickness at end-diastolic (LVPWD), and aortic annulus (AO) (p < 0.0001, 0.0003, and p < 0.0001, respectively). Besides, the left and/or right ventricular functions were declined in 52.4% of obese children. Correlation analysis revealed that the anthropometric parameters of obesity were not only significantly correlated with a blood lipid profile but also exhibited a more significant correlation with most of the parameters of cardiac dysfunction than a blood lipid profile. Therefore, our study indicated that obese children in Guangzhou suffered from functional damages related to cardiovascular events, which were characterized by cardiac dysfunction, and the anthropometric parameters of obesity could be economically alternative biomarkers for monitoring of cardiac dysfunction in obese children.

Time-dependent changes in RPILD and mortality risk in anti-MDA5+ DM patients: a cohort study of 272 cases in China.

Anti-melanoma differentiation-associated gene 5 positive (anti-MDA5+)DM has a close relationship with rapidly progressive interstitial lung disease (RPILD) and is associated with high mortality. However, data regarding the time-dependent risk of RPILD and deaths during disease progression are limited. We conducted this study to investigate whether the risk of RPILD and death were time-dependent or not in anti-MDA5+ DM. We assessed a cohort of 272 patients with anti-MDA5+ DM. The clinical characteristics of patients with anti-MDA5+ were collected, and COX regression was used to analyse independent risk factors for RPILD and death. We also described changes in risk of RPILD and death over time and their potential clinical implications. There were 272 anti-MDA5+ DM patients enrolled in this study. According to the multivariate cox regression analysis, short disease course, high CRP level, anti-Ro52 positive and anti-MDA5 titre (++∼+++) were independent risk factors of RPILD. High creatine kinase level, high CRP level and RPILD were independent risk factors for death, and >90% RPILD and 84% mortality occurred in the first 6 months after disease onset. Notably, the first 3 months is a particularly high-risk period, with 50% of RPILD and 46% of deaths occurring. Hazards regarding RPILD and mortality diminished over time during a median follow-up of 12 months. These results suggest significant, time-dependent changes in RPILD and mortality risk in anti-MDA5+ DM patients, providing a cut-off time window to estimate disease progression and poor prognosis.

Comparison of Emergence Agitation Scale Scores and Creatine Kinase Levels After General Anesthesia in Children: A Prospective Cohort Study.

IntroductionA simple indicator of muscle damage is creatine kinase (CK). Although CK elevation is informative for malignant hyperthermia, no study has examined the relationship between the anesthetically awake state and CK in children. We aimed to prospectively examine the relationship between the awakening state and CK on the day after surgery in children who have undergone anesthesia with volatile inhalation anesthetics.MethodsThe study included 119 patients aged 0-15 years and scheduled to undergo general anesthesia for cleft lip and palate-related surgery. Emergence agitation (EA) was assessed after completion of general anesthesia using the five-point EA scale, and patients were divided into the following five groups according to the EA score: EA1, EA2, EA3, EA4, and EA5. The primary outcome was ΔCK (comparison of CK values one week prior to surgery to CK values on the day after surgery) in each EA group. The secondary outcome was ΔCK when the EA score was divided into the following two groups: EA ≤2 (EA score of 1 or 2) and EA ≥3 (EA score of 3, 4, or 5).ResultsThe median ΔCK values in the EA1 to EA5 groups were 3 (quartile −19~9), 5 (−32~88), 99.5 (−18~190.5), 121 (29~219.5), and 144 (41~340.5), respectively, indicating a statistically significant difference overall. Statistically significant differences were also observed between the EA1 and EA4 groups and between the EA2 and EA4 groups. The median ΔCK values in the EA ≤2 and EA ≥3 groups were 3 (quartile −27~85) and 108 (23.5~206.7), respectively, indicating a statistically significant difference.ConclusionThe results of this study revealed that a higher EA score at the time of anesthesia awakening is associated with a larger ΔCK, indicating that a high CK level on the day after surgery is highly related to the state of the patient upon awakening.

The Risk of Ventricular Tachyarrhythmias in Patients with Antimitochondrial Antibodies-Related Noncardiac Diseases

Antimitochondrial antibodies (AMA) are serum autoantibodies specific to primary biliary cholangitis and are linked to myopathy and myocardial damage; however, the presence of AMA as a risk factor for ventricular tachyarrhythmias (VTs) has remained unknown. This study aimed to elucidate whether the presence of AMA-related noncardiac diseases indicates VTs risk.This cohort study enrolled 1,613 patients (883 females) who underwent AMA testing to assess noncardiac diseases. The incidence of VTs and supraventricular tachyarrhythmias (SVTs) from a year before the AMA testing to the last visit of the follow-up were retrospectively investigated as primary and secondary objectives. Using propensity score matching, we extracted AMA-negative patients whose covariates were matched to those of 152 AMA-positive patients. In this propensity score-matched cohort, the incidence of VTs and SVTs in the AMA-positive patients were compared with that in AMA-negative patients.The AMA-positive patients had higher estimated cumulative incidence (log-rank, P = 0.013) and prevalence (5.9% versus 0.7%, P = 0.020) of VTs than the AMA-negative patients. The presence of AMA was an independent risk factor for VTs (hazard ratio, 4.02; 95% CI, 1.44-20.01; P = 0.005). Meanwhile, AMA were associated with atrial flutter and atrial tachycardia development. In AMA-positive patients, VTs were associated with male sex, underlying myopathy, high creatine kinase levels, presence of chronic heart failure or ischemic heart disease, left ventricular dysfunction, presence of SVTs, and the electrocardiographic parameters indicating atrial disorders.The presence of AMA-related noncardiac diseases is an independent risk factor for VTs.

Creatine kinase and prognosis in amyotrophic lateral sclerosis: a literature review and multi-centre cohort analysis

BackgroundAmyotrophic lateral sclerosis (ALS) is a prognostically heterogeneous neurodegenerative disease. Blood creatine kinase (CK) level has been inconsistently reported as a prognostic biomarker and raised levels in some ALS patients have been presumed to reflect muscle wasting, which is also variable.MethodsMEDLINE was systematically searched for papers related to CK in ALS and the relevant studies were reviewed. Using data from 222 ALS patients in a multi-centre, prospective, longitudinal cohort, survival analyses using Kaplan–Meier and Cox proportional hazards models were undertaken in relation to CK and other prognostic factors.ResultsTwenty-five studies investigating CK in ALS were identified, of which 10 specifically studied the link between CK and survival. Five studies observed no association, four found that higher CK levels were associated with longer survival and one, the opposite. In our cohort (n = 222), 39% of patients had a CK level above the laboratory reference range. Levels were higher in males compared to females (p < 0.001), in patients with limb versus bulbar onset of symptoms (p < 0.001) and in patients with higher lower motor neuron burden (p < 0.001). There was no significant trend in longitudinal CK values. Although a higher standardised log (CK) at first visit was associated with longer survival in univariate analysis (hazard ratio 0.75, p = 0.003), there was no significant association after adjusting for other prognostic covariates.ConclusionWhile raised CK levels in ALS do reflect lower motor neuron denervation to a large extent, they are not independently associated with survival when measured in the symptomatic phase of the disease.

Serum creatine kinase levels are not associated with an increased need for continuous renal replacement therapy in patients with acute kidney injury following rhabdomyolysis

Severe rhabdomyolysis can lead to acute kidney injury (AKI). Previous studies have reported a benefit from continuous renal replacement therapy (CRRT) for rhabdomyolysis-associated AKI. Here, we investigated the potential for serum creatine kinase (CK) levels to be used as a marker for CRRT termination in patients with AKI following rhabdomyolysis. We compared different CK levels in patients after CRRT termination and observed their clinical outcomes. We retrospectively collected 86 cases with confirmed rhabdomyolysis-associated AKI, who were receiving CRRT in Tongji Hospital. Patients’ renal functions were assessed within 24 h of intermission, patients with urine output ≥ 1,000 mL and serum creatinine ≤ 265 umol/L were considered for CRRT termination. After termination, 33 patients with a CK > 5,000 U/L were included in an experimental group, and 53 patients with a CK < 5,000 U/L were included in a control group. Clinical outcomes were compared between the two groups. Higher CK levels, as well as worse renal functions, predicted the necessity of CRRT. After CRRT termination, the in-hospital mortality (p = 0.389) and Multiple Organ Dysfunction Syndrome (MODS) incidence (p = 0.064) were similar between the two groups, while the experimental group showed a significantly shorter in-hospital length of stay (p = 0.026) and Intensive Care Unit (ICU) length of stay (p = 0.038). CRRT termination may be independent of CK levels for patients with rhabdomyolysis-associated AKI, and this is contingent on their renal functions having recovered to an appropriate level.

HP42: EMG is a potential biomarker for myositis in patients with antisynthetase syndrome without muscle symptoms

Anti-aminoacyl-tRNA synthetase (ARS) antibodies positive patients sometimes present interstitial lung disease (ILD) without muscle symptoms. We investigated needle electromyography (nEMG) to explore whether there are some myogenic changes or not in patients with antisynthetase syndrome without muscle symptoms. Method: We enrolled three patients who fulfilled anti ARS antibodies positive and ILD of the Connor's criteria for antisynthetase syndrome, and not fulfill the PM/DM criteria. We investigated the nEMG, and magnetic resonance imaging (MRI) of muscles to explore whether there are some myogenic changes or not. Serum creatine kinase (CK), manual muscle testing of 8 muscle groups (MMT8), Inclusion Body Myositis-Functional Rating Scale (IBM-FRS) were measured. This study was approved by the ethics committee of the Nara Medical University School of Medicine. Results: All subjects were females and their ages at the examination were 54 years old (P1), 44(P2) and 69(P3). P1 and P2 had already initiated oral steroid therapy. Although all three patients had no high serum CK level nor decline in IBM-FRS, nEMG revealed that abnormally short durations of the motor unit potential (MUP) patterns in the deltoid and biceps. P3 presented the same MUP pattern in vastus lateralis. MRI revealed inflammatory changes in P2 at left triceps and P3 at anterolateral femurs and posterolateral lower legs. Conclusion: nEMG is usually performed when patients exhibit muscle weakness, but this examination tool can be applicated to detect muscle damage in patients with anti-ARS antibody-associated ILD, prior to muscle weakness, myalgia, and declines in ADLs.

Urine proteomics as a non-invasive approach to monitor exertional rhabdomyolysis during military training

Exertional rhabdomyolysis (ERM), a condition often associated with strenuous exercise, a common practice in the military activities, can be defined as the process of injury and rupture of muscle cell membranes, with leakage of its components into the bloodstream. Creatine kinase (CK) has been used for ERM diagnosis, albeit several studies reported the discrepancy between CK levels and clinical signs or symptoms. In this study, we analyzed the biochemical profile of the blood, and the urinary proteome of ten marine soldiers in a special training course. The samples were collected in two periods, M1 and M2, which correspond to the lowest and highest CK levels during training, respectively. Quantitative urinary proteome profile of M1 and M2 showed changes in proteins involved in immune system and cell adhesion-related pathways after strenuous physical exercise. Changes in the abundance of several proteins was observed in individuals carrying genetic polymorphisms related to greater risk for muscle damage. A panel of proteins (CTSH, PIK3IP1, DEFB1, ITGB1, BCAN, and TNFRSF10C) presented high correlation with classical blood biochemical markers of ERM and AGT MET235Thr and ACE I/D polymorphisms. These proteins represent potential urine markers of muscle damage due to intense physical conditions such as military training activities. SignificanceThis study analyzed the blood and urine of a cohort of marine soldiers enrolled in a special training program including missions with low and high exposure to strenuous exercise. The biochemical blood profile, polymorphisms mapping and mass spectrometry-based analyses of the urinary proteome was evaluated in such a controlled samples. A total of 226 urinary proteins associated to immune system, cell adhesion and redox homeostasis were significantly changes during ERM shedding lights on the disease pathogenesis. In particular, a panel of six proteins were associated to classical ERM markers and could be used as early non invasive biomarkers.

A novel 1-bp deletion variant in DAG1 in Japanese familial asymptomatic hyper-CK-emia

Asymptomatic hyper-CK-emia (ASCK) is characterized by persistent elevation of creatine kinase (CK) in serum without any neurological symptoms. We ascertained a two-generation family of ASCK patients without clear neurological abnormalities except for the high levels of serum CK (810.5 ± 522.4 U/L). We identified a novel 1-bp deletion variant in the DAG1 gene shared by the patients in the family (NM_001177639: exon 3: c.930delC:p.R311Gfs*70). The variant causes premature termination of translation at codon 477, resulting in a protein product completely devoid of the essential DAG1 domain. Since ASCK has been associated with DAG1 in only one case carrying compound heterozygous missense variants, our new finding of a novel 1-bp deletion revealed the previously unknown dominant effect of DAG1 on ASCK.

Abstract 12946: Identification of a LMNA Synonymous Variant Associated With Severe Dilated Cardiomyopathy

Dilated cardiomyopathy (DCM) is one of the most common cardiac phenotypes caused by mutations of lamin A/C (LMNA) gene in humans. Almost 50% of LMNA variants reported in ClinVar as pathogenic or likely pathogenic are due to frameshift, nonsense, or splicing variants, predictive of haploinsufficiency for LMNA protein. In contrast, synonymous variants that do not immediately suggest a haploinsufficiency mechanism are much harder to interpret. We evaluated a cohort of 57 patients who underwent heart transplant for dilated cardiomyopathy for LMNA variants and identified a novel synonymous variant c.936G&gt;A in the last nucleotide of exon 5 of LMNA in a DCM family. Clinically, LMNA c.936G&gt;A carriers presented with severe DCM, conduction disease and high creatine-kinase levels. Sanger sequencing showed the presence of LMNA c.936G&gt;A in the genomic DNA but not in the cDNA derived from one family member’s heart tissue. RT-PCR using RNA from the patient’s heart tissue was performed with primers that amplified LMNA exons 4-6. DNA fragments amplified by RT-PCR were then cloned into pcDNAII vector and 40 clones were picked and sequenced. An aberrantly spliced mRNA containing the variant and a premature stop codon was identified in 1 of the 40 clones. Real-time quantitative PCR showed significantly lower LMNA mRNA levels in the patient’s heart compared to the controls. Our findings suggest that the c.936G&gt;A LMNA variant resulted in aberrant RNA splicing, which possibly triggers nonsense-mediated RNA decay, leading to reduced mRNA and likely protein expression. These results expand the understanding on the association between synonymous LMNA variants and the molecular pathogenesis of LMNA-DCM.

A high prevalence of low bone mineral density in children with glycogen storage disease type III

IntroductionGlycogen storage disease III (GSD III) is an inborn error of carbohydrate metabolism that involves mainly the liver and skeletal muscles with rare potential systemic complications as osteoporosis. The aim of work of the current study was to screen children with GSD III for osteoporosis using Dual-emission-X-ray absorptiometry (DXA) scan and to measure associated parathormone (PTH) and vitamin D. Our secondary objective is to correlate between the degree of osteoporosis and the muscle state as well as the metabolic control.Material and methodsThis cross sectional study included 25 GSD III pediatric cases. The liver biochemical profile, creatine kinase (CK), vitamin D2, PTH levels were tested in addition to electromyogram (EMG), echocardiography and DXA. Twenty-five age and sex matched normal healthy controls were subjected to vitamin D2 analysis and compared to cases.ResultsThe mean ±SD age of the patients was 10.52 ± 3.1 years ranging between 5-18 years. Twenty-one patients (84%) had elevated CK levels, 14 patients (56%) had myopathy and three (12%) had sensory polyneuropathy and almost half of the patients had a mild degree of cardiac muscle hypertrophy. Ten patients (40%) had elevated PTH levels. Twenty-one patients (84%) and 96% of the controls had vitamin D2 deficiency. Thirteen patients (52%) had low BMD; two of them had osteoporosis. Patients with high CK levels significantly had low BMD (P= 0.04).ConclusionsPediatric GSD III patients have a significant prevalence of developing low BMD. It is strongly associated with myopathic changes but not significantly related to metabolic control.

Prognostic and predictive impact of creatine kinase level in non-small cell lung cancer treated with tyrosine kinase inhibitors.

BackgroundThe use of tyrosine kinase inhibitors (TKIs) is associated with incident creatine kinase (CK) elevation in the treatment of advanced non-small cell lung cancer (NSCLC) patients. However, whether higher CK levels are associated with better antitumor responses or survival remains to be explored. We intend to investigate the clinical correlation between CK levels and TKI efficacy in advanced NSCLC.MethodsIn this retrospective study, we enrolled 135 patients with stage IV NSCLC receiving TKI-based therapy in our center between June 2012 to July 2020. CK levels were monitored from the initiation of TKI medication and during the administration period. An X-tile analysis provided the optimal cutoff point for higher baseline CK. Patients were identified and grouped according to their baseline CK level and fold changes during TKI therapy. The primary endpoints included progression-free survival (PFS) and overall survival (OS), and the objective response rate (ORR) was calculated as the secondary endpoint.ResultsAmong the 135 patients included in our study, those with higher baseline CK levels (≥70 U/L) had favorable PFS (15.2 vs. 8.8 months; P=0.028), while patients with significantly elevated CK (the highest CK value/baseline CK value ≥2 times) appeared to gain better PFS (14.6 vs. 10.0 months; P=0.139). The overall ORR was 67.4%. Patients with higher baseline CK levels had numerically higher ORR (74.6% vs. 60.3%; P=0.076). Similarly, patients with significant CK elevation had a superior 4-month PFS rate (77.6% vs. 59.7%; P=0.029). Results from the subgroup analyses were identical to the overall ones. For patients with higher baseline CK levels, those experiencing significant CK elevation had prolonged PFS (17.2 vs. 14.2 months; P=0.038); a same trend was obtained from the lower baseline CK group (<70 U/L) (9.4 vs. 7.9 months; P=0.038). In multivariable analysis, higher baseline CK level and significant CK elevation remained statistically associated with PFS, with hazard ratios of 0.48 and 0.59, respectively.ConclusionsBoth higher baseline CK levels and significant CK elevation after treatment were correlated with prolonged PFS in NSCLC treated with TKIs, suggesting the potential prognostic and predictive impact of CK level on these patients.

Low CD4 T cell count predicts radiological progression in severe and critically ill COVID-19 patients: a case control study.

BackgroundNovel coronavirus disease (COVID-19) has spread globally and caused over 3 million deaths, posing great challenge on public health and medical systems. Limited data are available predictive factors for disease progression. We aim to assess clinical and radiological predictors for pulmonary aggravation in severe and critically ill COVID-19 patients.MethodsPatients with confirmed COVID-19 in Renmin Hospital of Wuhan University, China, between Feb. 6th, 2020 and Feb. 21st, 2020 were retrospectively collected. Enrolled patients were divided into non-progression group and progression group based on initial and follow-up chest CTs. Clinical, laboratory, and radiological variables were analyzed.ResultsDuring the study period, 162 patients were identified and a total of 126 patients, including 97 (77.0%) severe cases and 29 (23.0%) critically ill cases were included in the final analysis. Median age was 66.0 (IQR, 56.0–71.3) years. Median time from onset to initial chest CT was 15.0 (IQR, 12.0–20.0) days and median interval to follow-up was 7.0 (IQR, 5.0–7.0) days. Compared with those who did not progress (n=111, 88.1%), patients in the progression group (n=15, 11.9%) had significantly higher percentage of peak body temperature >38 °C (P=0.002), lower platelet count (P=0.011), lower CD4 T cell count (P=0.002), lower CD8 count (P=0.011), higher creatine kinase level (P=0.002), and lower glomerular filtration rate (P=0.018). On both univariate and multivariable analysis, only CD4 T cell count <200/µL was significant (OR, 6.804; 95% CI, 1.450–31.934; P=0.015) for predicting pulmonary progression.ConclusionsLow CD4 T cell count predicts progression of pulmonary change in severe and critically ill patients with COVID-19.

Recovery-Stress Response of Blood-Based Biomarkers.

The purpose of this study was to investigate blood-based biomarkers and their regulation with regard to different recovery-stress states. A total of 35 male elite athletes (13 badminton, 22 soccer players) were recruited, and two venous blood samples were taken: one in a ‘recovered’ state (REC) after a minimum of one-day rest from exercise and another one in a ‘non-recovered’ state (NOR) after a habitual loading microcycle. Overall, 23 blood-based biomarkers of different physiologic domains, which address inflammation, muscle damage, and tissue repair, were analyzed by Luminex assays. Across all athletes, only creatine kinase (CK), interleukin (IL-) 6, and IL-17A showed higher concentrations at NOR compared to REC time points. In badminton players, higher levels of CK and IL-17A at NOR were found. In contrast, a higher value for S100 calcium-binding protein A8 (S100A8) at REC was found in badminton players. Similar differences were found for BDNF in soccer players. Soccer players also showed increased levels of CK, and IL-6 at NOR compared to REC state. Several molecular markers were shown to be responsive to differing recovery-stress states, but their suitability as biomarkers in training must be further validated.

Underlying diseases in sporadic presentation of high creatine kinase levels in girls

BackgroundPersistent creatine kinase (CK) elevation can occur due to various conditions. Identifying the causes of hyperCKemia is crucial for enabling appropriate follow-up and care. Girls with elevated CK levels may be carriers of Duchenne/Becker muscular dystrophy (DMD/BMD), making diagnosis more difficult than that in boys. This study aimed to elucidate the underlying causes of high CK levels in girls. MethodsFourteen girls (seven symptomatic, seven asymptomatic) with persistently elevated CK levels but without a family history of muscle diseases were referred to our hospital between April 2014 and August 2018. Muscle biopsy and/or genetic analysis were conducted for diagnoses. ResultsAmong the symptomatic girls, six (85.7%) had muscular dystrophy (five DMD/BMD carriers, and one sarcoglycanopathy [limb-girdle muscular dystrophy: LGMDR4]), and one had dermatomyositis. Among the asymptomatic girls, four (57.1%) had muscular dystrophy (three DMD/BMD carriers, and one calpainopathy [LGMDR1]), and three were undiagnosed. ConclusionOur results indicate that muscular dystrophy, including DMD/BMD carriers, must be considered in girls with highperCKemia regardless of symptoms presentation, and in symptomatic girls with dermatomyositis. Investigations in girls with hyperCKemia should be performed under proper ethical considerations. Further research is necessary to develop a diagnostic strategy for girls with hyperCKemia.

A Patient With Late-onset Limb-girdle Muscular Dystrophy Type 2B Mimicking Dermatomyositis: A Case Report and Review.

Limb-Girdle Muscular Dystrophy 2B (LGMD2B) presents with proximal and/or distal muscle weakness and markedly high creatine kinase level. It is caused by the loss of dysferlin due to mutations in the DYSF gene. Due to its similar clinical features as inflammatory myopathy, it is often difficult to distinguish between the two. We present a case of a 48-year-old male who developed progressive proximal muscle weakness, papulosquamous lesions on the knuckles, elevated levels of muscle enzymes, and electromyogram abnormalities. Based on the clinical presentation, the initial impression was dermatomyositis, yet it was refractory to immunosuppressive therapy. Subsequently, dysferlin immunostaining and genetic analysis led to the final diagnosis of LGMD2B. This case shows that LGMD2B can present with extramuscular symptoms mimicking inflammatory myopathy in later stages of life. Dysferlin immunostaining and/or genetic analysis of the DYSF gene are essential for its diagnosis.

A Case of Myotonic Dystrophy Associated with High Level of Creatine Kinase and Cupping Therapy Management Outcome

A Case of Myotonic Dystrophy Associated with High Level of Creatine Kinase and Cupping Therapy Management Outcome