BACKGROUND We report a rare case of primary clinical presentation featuring elevated creatine kinase(CK)levels in a neonate,which is associated with the LAMA2 gene.In this case,a heterozygous mutation in exon5 of the ...BACKGROUND We report a rare case of primary clinical presentation featuring elevated creatine kinase(CK)levels in a neonate,which is associated with the LAMA2 gene.In this case,a heterozygous mutation in exon5 of the LAMA2 gene,c.715C>G(resulting in a change of nucleotide number 715 in the coding region from cytosine to gua-nine),induced an amino acid alteration p.R239G(No.239)in the patient,repre-senting a missense mutation.This observation may be elucidated by the neonatal creatine monitoring mechanism,a phenomenon not previously reported.CASE SUMMARY We analysed the case of a neonate presenting solely with elevated CK levels who was eventually discharged after supportive treatment.The chief complaint was identification of increased CK levels for 15 d and higher CK values for 1 d.Ad-mission occurred at 18 d of age,and despite prolonged treatment with creatine and vitamin C,the elevated CK levels showed limited improvement.Whole exo-me sequencing revealed the presence of a c.715C>G mutation in LAMA2 in the newborn,correlating with a clinical phenotype.However,the available informa-tion offers insufficient evidence for clinical pathogenicity.CONCLUSION Mutations in LAMA2 are associated with the clinical phenotype of increased neonatal CK levels,for which no specific treatment exists.Whole genome sequen-cing facilitates early diagnosis.展开更多
Creatine transporter(CrT)-deficiency,the most common form of the cerebral creatine deficiency syndromes,causes cognition impairments and severe reduction of the brain creatine(Cr)and phosphocreatine(PCr)levels,and res...Creatine transporter(CrT)-deficiency,the most common form of the cerebral creatine deficiency syndromes,causes cognition impairments and severe reduction of the brain creatine(Cr)and phosphocreatine(PCr)levels,and responds poorly to oral Cr supplement as a treatment option.The ca uses of cognitive impairments in CrT-deficient children remain unclear.We recently use gene-targeting to create a mouse model of CrT-deficiency to assess the impacts of Cr/PCr deficiency on brain energetics and stress-adaptation responses(Chen et al.,2021).展开更多
A specific, precise and accurate ion-pair HPLC-UV method has been developed and validated for simultaneous determination of phosphocreatine (PCr), and its metabolite creatine (Cr) as well as related ATP in plasma and ...A specific, precise and accurate ion-pair HPLC-UV method has been developed and validated for simultaneous determination of phosphocreatine (PCr), and its metabolite creatine (Cr) as well as related ATP in plasma and red blood cell (RBC) of rabbits. After addition of TMP as IS, the samples were deproteinized with 6% PCA. The analytes were separated on a Kromasil C18 column using a tertiary gradient mobile phase composed of buffer A (0.2% KH2PO4 + 0.08% tetrabutyl ammonium hydrogen sulphate, pH 3.0), buffer B (buffer A adjusted to pH 7.5 with 1 mol/L NaOH) and MeOH. Detection wavelengths were set at 210 nm for PCr and Cr and 260 nm for ATP and TMP. Some blank samples were initially run for baseline subtraction. The linear detection responses were obtained for PCr concentration over a range of 10 - 7500 mg/ml (plasma) and 5 - 2500 mg/ml (RBC) and for both Cr and ATP concentrations of 10 - 1500 mg/ml (plasma) and 5 - 750 mg/ml (RBC) (r > 0.99). The QC samples of 3 analytes showed intra-day and inter-day precisions (RSD) of - 107%. The method was successfully used to simultaneously determine plasma and RBC concentrations of the 3 analytes and to study pharmacokinetics after iv administration of PCr to rabbits.展开更多
BACKGROUND:The early diagnosis of acute myocardial infarction(AMI)remains challenging,especially for institutions without the high-sensitive cardiac troponin(hs-c Tn)assay.Herein,we aim to assess the value of creatine...BACKGROUND:The early diagnosis of acute myocardial infarction(AMI)remains challenging,especially for institutions without the high-sensitive cardiac troponin(hs-c Tn)assay.Herein,we aim to assess the value of creatine kinase-myocardial band isoenzyme(CK-MB)combined with different cardiac troponin(c Tn)assays in AMI diagnosis.METHODS:This multicenter,observational study included 3,706 patients with acute chest pain from September 1,2015,to September 30,2017.We classified the participants into three groups according to the c Tn assays:the point-of-care c Tn(POC-c Tn)group,the contemporary c Tn(c-c Tn)group,and hs-c Tn group.The diagnostic value was quantified using sensitivity and the area under the curve(AUC).RESULTS:Compared to the single POC-c Tn/c-c Tn assays,combining CK-MB and POC-c Tn/c-c Tn increased the diagnostic sensitivity of AMI(56.1%vs.63.9%,P<0.001;82.7%vs.84.3%,P=0.025).In contrast,combining CK-MB and hs-c Tn did not change the sensitivity compared with hs-c Tn alone(95.0%vs.95.0%,P>0.999).In the subgroup analysis,the sensitivity of combining CKMB and c-c Tn increased with time from symptom onset<6 h compared with c-c Tn alone(72.8%vs.75.0%,P=0.046),while the sensitivity did not increase with time from symptom onset>6 h(97.5%vs.98.3%,P=0.317).The AUC of the combination of CK-MB and POC-c Tn significantly increased compared to the single POC-c Tn assay(0.776 vs.0.750,P=0.002).The AUC of the combined CKMB and c-c Tn/hs-c Tn assays did not significantly decrease compared with that of the single c-c Tn/hs-c Tn assays within 6 h.CONCLUSIONS:The combination of CK-MB and POC-c Tn or c-c Tn may be valuable for the early diagnosis of AMI,especially when hs-c Tn is not available.展开更多
Creatine is one of the most popular forms of protein supplements and is known to improve performance in healthy athletic populations via enhanced muscle mass and adenosine triphosphate energy regeneration.Clinical use...Creatine is one of the most popular forms of protein supplements and is known to improve performance in healthy athletic populations via enhanced muscle mass and adenosine triphosphate energy regeneration.Clinical use of creatine may similarly benefit patients with rheumatoid arthritis(RA), an inflammatory condition characterised by generalised muscle loss termed"rheumatoid cachexia". The adverse consequences of rheumatoid cachexia include reduced strength, physical function and, as a consequence, quality of life.Whilst regular high-intensity exercise training has been shown to increase muscle mass and restore function in RA patients, this form of therapy has very low uptake amongst RA patients. Thus, acceptable alternatives are required. The aim of this review is to consider the potential efficacy of creatine as an anabolic and ergonomic therapy for RA patients. To date, only one study has supplemented RA patients with creatine, and the findings from this investigation were inconclusive. However, trials in populations with similar losses of muscle mass and function as RA, including older adults andthose with other muscle wasting conditions, indicate that creatine is an efficacious way of improving muscle mass, strength and physical function, and may offer an easy, safe and cheap means of treating rheumatoid cachexia and its consequences.展开更多
Creatine kinase is a muscle enzyme that has been reported at various levels in different studies involving patients with amyotrophic lateral sclerosis.In the present retrospective case-control study,we included 582 pa...Creatine kinase is a muscle enzyme that has been reported at various levels in different studies involving patients with amyotrophic lateral sclerosis.In the present retrospective case-control study,we included 582 patients with amyotrophic lateral sclerosis and 582 age-and sexmatched healthy controls.All amyotrophic lateral sclerosis participants received treatment in the Department of Neurology,West China Hospital,China,between May 2008 and December 2018.Serum creatine kinase levels in patients with amyotrophic lateral sclerosis were significantly higher than those in healthy controls.Subgroup analysis revealed that serum creatine kinase levels in men were higher than those in women in both amyotrophic lateral sclerosis patients and healthy controls.Compared with patients with bulbar-onset amyotrophic lateral sclerosis,patients with limb-onset amyotrophic lateral sclerosis had higher creatine kinase levels.Spearman's correlation analysis revealed that serum creatine kinase levels were not correlated with body mass index,Amyotrophic lateral Sclerosis Functional Rating ScaleRevised score,or progression rate.After adjusting for prognostic covariates,higher log creatine kinase values were correlated with higher overall survival in the amyotrophic lateral sclerosis patients.We also investigated the longitudinal changes in serum creatine kinase levels in 81 amyotrophic lateral sclerosis patients;serum creatine kinase levels were decreased at the second blood test,which was sampled at least 6 months after the first blood test.Together,our results suggest that serum creatine kinase levels can be used as an independent factor for predicting the prognosis of amyotrophic lateral sclerosis patients.This study received ethical approval from the Ethics Committee of West China Hospital,China(approval No.2015(236))on December 23,2015.展开更多
KEY POINTS Because so much of the running in soccer is at less than maximal sprinting speed, creatine supplementation likely provides no benefit to match performance.Overwhelming evidence proves that a diet rich in ca...KEY POINTS Because so much of the running in soccer is at less than maximal sprinting speed, creatine supplementation likely provides no benefit to match performance.Overwhelming evidence proves that a diet rich in carbohydrates can fill muscles with glycogen, and glycogen is critical to optimal performance in soccer. Soccer players’ diets, especially in the days before hard training or competition, should include 8-10 grams of carbohydrate per kilogram of body weight (3.5-4.5 g/lb). Cereals, fruits, vegetables, breads, and pastas are good sources ofcarbohydrates. Refueling of muscle with carbohydrates should begin as soon as possible following a match or a strenuous training session. Inadequate replacement of fluids lost in sweat can lead to poor soccer performance and heat illness. Players should aim to drink enough during training sessions and matches so that their body weights after play are within about 1 kg (2.2 lb) of their starting weights. For a light workout or an easy match, especially when the weather is cool, water can be an adequate fluid replacement, if enough is ingested. But when play is strenuous and the weather is hot, carbohydrate-electrolyte sports drinks do a better job of maintaining body fluids.展开更多
Background: Creatine supplementation is a subject that is very well studied. New forms of creatine are suggesting improvements in this supplement performance. Creatine HCl is supposed to have better solubility and abs...Background: Creatine supplementation is a subject that is very well studied. New forms of creatine are suggesting improvements in this supplement performance. Creatine HCl is supposed to have better solubility and absorption than creatine. The aim of this study was to compare the effects of two different doses of creatine HCl with creatine monohydrate on the strength and body composition in recreational weightlifters and to verify the relationship between strength and body composition. Methods: 40 subjects were divided in four groups: Creatine Monohydrate (CMG) 5 g/daily;Creatine HCl-1 (HCl-1) 5 g/daily, Creatine HCl-2 (HCl-2) 1.5 g/daily and Control group (CG) = 5 g of resistant starch/daily. All groups performed a resistance training program during 4 weeks. Body composition and strength were evaluated pre and post intervention. Results: The 1 RM at the Leg press was increased significantly in all groups (CMG: pre = 264.4 ± 83.8 × post = 298.1 ± 90.9;HCl-1: pre = 295.0 ± 88.3 × post = 338.3 ± 86.8 and HCl-2: pre = 274.3 ± 57.1 × post = 305.7 ± 59.4;p p = 0.003), however, there was no statistically significant difference between groups. Fatmass was significantly decreased in HCl-1 (pre = 14.5 ± 8.0 × post = 13.3 ± 8.3;p = 0.034) and HCl-2 (pre = 13.8 ± 5.8 × post = 12.7 ± 5.6;p = 0.005) but fat-free mass was increased only in HCl-1 (pre = 52.2 ± 8.9 × post = 53.8 ± 8.9;p = 0.031), with no differences between groups again. We observed strong correlations between upper limb strength and fat mass (r = 0.93, p p < 0.05) only in HCl-1 group. Conclusions: We concluded that creatine HCl and creatine Monohydrate improve performance but only creatine HCl induces changes on body composition in recreational weightlifters with differences between creatine HCl doses.展开更多
Objective To study the differential diagnosis on cerebral concussion and mild cerebral contusion value of the brain type creatine kinase isoenzyme(CK BB),and evaluate the seriousness of brain damage and prognosis of t...Objective To study the differential diagnosis on cerebral concussion and mild cerebral contusion value of the brain type creatine kinase isoenzyme(CK BB),and evaluate the seriousness of brain damage and prognosis of the patients with acute head injury.Methods Chromatographic separating and fluorometric quantifying technique was used to detect the CK BB activity in the cerebrospinal fluid(CSF) of 117 patients with acute head injury and 12 patients with increased intracranial pressure and 20 normal people.Results The CSF CK BB activity of the patients with acute head injury was remarkably higher than that of the normal people and the CSF CK BB activity increased with the seriousness of brain damage.There was a close relationship between CSF CK BB activity and prognosis,and higher activity of CSF CK BB indicated poor prognosis.Conclusion CSF CK BB activity could be used as a new index to diagnose brain damage and evaluate the seriousness of brain damage and prognosis.展开更多
Objective To observe the relationship between serum creatine kinase isoenzyme MM sub-bands (CKMM3/MM1 ratio) and the gradation of coronary stenosis and provide a simple, reliable, and economical method for identifying...Objective To observe the relationship between serum creatine kinase isoenzyme MM sub-bands (CKMM3/MM1 ratio) and the gradation of coronary stenosis and provide a simple, reliable, and economical method for identifying high-risk unstable angina pectoris (UAP). Mehtods Blood samples were drawn at different time after onset of chest pain in 21 patients with UAP and only once in 20 each volunteers for control. CKMM3/MM1 ratio was detected by nonserial buffer agarose gel electrophoresis. CKMB and CK were observed by velocity method. An emergent coronary arteriography was performed as soon as patients were admitted into hospital. Results Patients with UAP were divided into two subgroups: patients with elevated serum enzyme [P( + )] and patients with normal serum enzyme [P( - ) ] according to CKMM3/MM1 ratio 【 0. 5. Patients with UAP( + ) had higher serum CKMM3/MM1 ratios from 0.5 to 12hrs and serum CKMB from 2 to 12 hrs than those with UAP( - ) and control ( P 【 0. 05) . Serum enzyme concentrations of展开更多
Objective: To observe the influence of nicorandil combined with creatine phosphate sodium on serum BNP, CK-MB, cTnl, SOD, MMP-9 and cardiac function in patients with STEMI. Methods: A total of 60 patients with STEMI w...Objective: To observe the influence of nicorandil combined with creatine phosphate sodium on serum BNP, CK-MB, cTnl, SOD, MMP-9 and cardiac function in patients with STEMI. Methods: A total of 60 patients with STEMI were randomly divided into observation group (30 cases) and control group (30 cases). Observation group: nicorandil combined with creatine phosphate sodium;control group: patients were treated only by nicorandil. Recording and comparing the levels of BNP, CK-MB, cTnl, SOD, MMP-9 and cardiac function before and after treatment. Results: (1) Before treatment, there was no statistically significant difference in the serum BNP, CK-MB, cTnl, SOD, MMP-9 levels between the two groups. After treatment, compared with the same group before treatment, the serum BNP, CK-MB, cTnl, MMP-9 levels of the two groups were significantly lower, the serum SOD level of the two groups were significantly higher, and those levels of observation group were significantly better than the control group, there was significant difference between the two groups. (2) Before treatment, there was no statistically significant difference in LVEF, WMSI, CI between the two groups. After treatment, compared with the same group before treatment, the WMSI of the two groups were significantly lower, the LVEF and CI of the two groups were significantly higher, and those levels of observation group were significantly better than the control group, there was significant difference between the two groups. Conclusion: Nicorandil combined with creatine phosphate sodium for patients with STEMI can perfect the levels of serum BNP, CK-MB, cTnl, SOD, MMP-9 and be beneficial to protect their cardiac function.展开更多
Objectives: Exploring the long-term consequences of elevated postoperative creatine phosphokinase MB as a surrogate measure of cardiac tissue damage subsequent to coronary artery bypass graft surgery (CABG). Methods: ...Objectives: Exploring the long-term consequences of elevated postoperative creatine phosphokinase MB as a surrogate measure of cardiac tissue damage subsequent to coronary artery bypass graft surgery (CABG). Methods: Prospective cohort study including 414 patients subjected to solitary CABG at Odense University Hospital from September 30, 2007, and backwards in time. According to individual postoperative peak creatine phosphokinase MB, patients were assigned to five subgroups. Kaplan Meier survival analysis and Cox proportional-hazard regression evaluated the predictive value of creatine phosphokinase MB on late death from cardiac cause and diagnosis of paroxysmal or persistent atrial fibrillation within 10-years after CABG. Results: There was a general numeric decrease in time to cardiac death with increasing postoperative creatine phosphokinase MB (Log-Rank 0.285). Creatine phosphokinase MB p p p = 0.002) increase in paroxysmal or persistent atrial fibrillation in patients with postoperative atrial fibrillation compared to patients in postoperative sinus rhythm. Conclusions: Postoperative creatine phosphokinase MB was not found predictive of late cardiac death or diagnosis of paroxysmal or persistent atrial fibrillation within 10 years after CABG. However, patients with postoperative atrial fibrillation had increased risk of later developing paroxysmal or persistent atrial fibrillation compared to patients in postoperative sinus rhythm.展开更多
Objective:To study the cardioprotective effectof creatine phosphate sodium by measuring perioperative myocardial enzymes of allogeneic liver transplant patients.Methods:The 26 cases of orthotopic liver transplant pati...Objective:To study the cardioprotective effectof creatine phosphate sodium by measuring perioperative myocardial enzymes of allogeneic liver transplant patients.Methods:The 26 cases of orthotopic liver transplant patients were randomly divided into control group and creatine phosphate sodium group,13 cases in each group.The creatine phosphate sodium group were given creatine phosphate sodium 30 mg/kg at the time of induction.The control group were given equal amount of N.S.All patients at six-phase of before surgery,after anesthesia,before anhepatic phase,anhepatic phase,new liver and the 12 hours after surgery extraction radial artery.The CK,CK-MB and cTnI activity was measured.Results:To compared with control group,creatine phosphate sodium group’s plasma CK,CK-MB and cTnI concentrations in before anhepatic phase,anhepatic phase,new liver and the 12 hours after surgery 4 phase are significantly lower(p<0.05).Conclusions:The creatine phosphate sodium has a protective effect on liver transplantation in patients with myocardial damage.展开更多
Purpose The aim was to explore five established SNPs(rs1815739,rs1805086,rs2700352,rs28497577,and rs28357094)that are known to modulate skeletal muscle protein kinetics in response to creatine supplementation.Methods ...Purpose The aim was to explore five established SNPs(rs1815739,rs1805086,rs2700352,rs28497577,and rs28357094)that are known to modulate skeletal muscle protein kinetics in response to creatine supplementation.Methods A randomized,placebo-controlled,repeated measures design was used.Participants(n=152)were rand-omized divided into one of two groups:CREA(20 g/day creatine monohydrate)or PLAC:(dextrose)for 7 days.SNP were assessed,and participants were classified accordingly.Before and after supplementation,anthropometrics(height and body mass)and performance measures(vertical jump,countermovement vertical jump,squat jump,abdominal crunches,and maximum push-ups)were assessed.Results CREA gained more body mass than PLAC(CREA:Δ0.864±0.06 kg;PLAC:Δ0.154±0.07 kg,P<0.001).In the CREA group,the presence of an A allele for the MYLK1 polymorphism was related to changes in countermovement jump height(P=0.027;effect size[d]=0.41)and leg power(P=0.040,effect size[d]=0.18).The total number of abdominal crunches after supplementation was influenced by treatments and SPP1 gene(P=0.041).A higher number of abdominal crunches was associated with the G allele in the CREA group and the TT genotype in the PLAC group(effect size[d]=0.04).Conclusion Collectively,short-term creatine supplementation increased body mass but was unable to alter muscle perfor-mance.However,following creatine supplementation,participants expressing A alleles in the MYLK1 polymorphism had a greater increase in jump height and leg power and participants expressing G alleles in the SPP1 gene had greater improve-ments in abdominal crunch performance.展开更多
Background: The exRML (exertional rhabdomyolysis) is a pathophysiologic condition of skeletal muscle cell damage and breakdown associated with high intensity or prolonged exercise, normal exercise under extreme circum...Background: The exRML (exertional rhabdomyolysis) is a pathophysiologic condition of skeletal muscle cell damage and breakdown associated with high intensity or prolonged exercise, normal exercise under extreme circumstances, or sudden and excessive skeletal muscle contraction. It may manifest from the increase in CK (creatine kinase) or MYO (myoglobin), a protein that can cause life-threatening injury to the kidney (AKI, acute kidney injury), and may or may not be associated with myoglobinuria. Here, we presented a case of exRML with AKI, and then reviewed the related reports. Vigorous hydration, sodium bicarbonate and furosemide are key treatments. Aim: To examine an elderly patient with exRML induced AKI and the key treatment process. Case summary: A 61-year-old man left our hospital without permission after his admission and has been walking for almost 30 kms with no water and food intake, then was diagnosed exRML and exRML induced AKI with an obvious elevation of CK, MYO and decrease of eGFR (estimated glomerular filtration rate) after coming back, and was treated with vigorous hydration, loop diuresis, sodium bicarbonate, prostaglandin and Shenkang injection. After vigorous resuscitation, the patient’s renal function, CK and MYO returned normal. Conclusions: The exRML can cause serious complications such as AKI and death. Delayed diagnosis can be critical;therefore, manner of time should be taken to achieve a favorable prognosis.展开更多
We studied the relations of intracranial pressure (ICP),creatine kinase (CK) and bralnstem auditory evoked potential (BAEP) in 44 patients with traumatic brain edema who were admitted to our hospital from June 1990 to...We studied the relations of intracranial pressure (ICP),creatine kinase (CK) and bralnstem auditory evoked potential (BAEP) in 44 patients with traumatic brain edema who were admitted to our hospital from June 1990 to February 1991. There were 30 males and 14 females, with age range from 9 to 67 years. The results showed that the abnormal BAEP could reflect the severity of cerebral edema in acute head injury and was related to ICP and serum CK levels. When ICP】30 mmHg (4kPa), the abnormality of BAEP was more obvious than that of the control group (P【0.05); the serum CK levels were also elevated markedly. In patients with ICP over and below 4kPa, the rate of abnormal BAEP was 38.46% and 77.78% respectively (P【0.05). The serum CK level in the normal group or in the group with moderate abnormality of BAEP was significantly different from that in the group with severe abnormality or lack of BAEP (274.8± 98.24 U/L vs 705.3± 364.27 U/L; P【0.001). After treatment, the ICP returned to normal, and the BAEP展开更多
Carbon quantum dot/carbon dot(CD)exhibiting sustained photoluminescence at longer wavelengths in aqueous solution is difficult to prepare,but has enormous potential in biomedical applications.For the first time,we rep...Carbon quantum dot/carbon dot(CD)exhibiting sustained photoluminescence at longer wavelengths in aqueous solution is difficult to prepare,but has enormous potential in biomedical applications.For the first time,we report the magnesium(II)selective fluorescence enhancement of a red-light emitting anthrarufin and boric acid-derived CD in aqueous solution for direct evaluation of creatine kinase(CK)enzyme activity.The CD displayed visually detectable,intense red fluorescence only in the presence of magnesium ion(Mg^2+)at physiological pH value when irradiated with an ultraviolet(UV)source.Concurrently,a significant increase in steady-state fluorescence intensity and fluorescence lifetime was documented.A time-dependent density functional theory(TD-DFT)analysis displayed a bathochromic shift in UV–visible(vis)absorption,and increased oscillator strength of transition resulting from the selective chelation of Mg^2+withβ-hydroxy keto functionality on the surface of the CD.The CD–Mg^2+assembly was subsequently used to conceptualize the detection of CK directly through the exploration of the differential binding affinity of Mg2+with adenosine triphosphate(ATP),adenosine diphophate(ADP),and CD that is otherwise not possible with commercially available kits as of today.Thus,the report delineated here usher grandeur potential of CD for biological explorations related to Mg2+or ATP sensing and monitoring of Mg^2+-dependent enzymatic activity through a clear understanding of the chemistry.展开更多
It has been previously reported that during the denaturation of creatine kinase (ATP: creatine phosphotransferase EC 2.7.3.2) by guanidine hydrochloride, urea or SDS, inactivation occurs before noticeable conformation...It has been previously reported that during the denaturation of creatine kinase (ATP: creatine phosphotransferase EC 2.7.3.2) by guanidine hydrochloride, urea or SDS, inactivation occurs before noticeable conformational change of the enzyme molecule as a whole can be detected, and the inactivation rate constants of the enzyme in the above denaturant solutions are several orders of magnitude faster than those of展开更多
Myoglobin, myosin creatine kinase MM (CK-MM),creatine kinase BB(CK-BB) in cardiac muscle and H chain ofmyosin in atrial and ventricular muscle were studied in speci-mens from 8 patients who died of sudden nocturnal de...Myoglobin, myosin creatine kinase MM (CK-MM),creatine kinase BB(CK-BB) in cardiac muscle and H chain ofmyosin in atrial and ventricular muscle were studied in speci-mens from 8 patients who died of sudden nocturnal deathsyndrome(SNDS) by avidin-biotin complex (ABC) method toinvestigate the possible early or very early myocardial ischemiain the syndrome. Hematoxylin-eosin staining was conductedfor comparison. The results showed evident loss of CK-MM,CK-BB, myoglobin and myosin from cardiac muscle cells, indi-caring that occurrence of SNDS is closely associated withacute myocardial ischemia.展开更多
基金Supported by The Suzhou Science and Technology Development Plan Guiding Project,No.SZSYYXH-2023-YB5The Suzhou Science and Technology Development Plan Project,No.SKY2023002The Suzhou Key Laboratory of Children's Structural Deformities,No.SZS2022018.
文摘BACKGROUND We report a rare case of primary clinical presentation featuring elevated creatine kinase(CK)levels in a neonate,which is associated with the LAMA2 gene.In this case,a heterozygous mutation in exon5 of the LAMA2 gene,c.715C>G(resulting in a change of nucleotide number 715 in the coding region from cytosine to gua-nine),induced an amino acid alteration p.R239G(No.239)in the patient,repre-senting a missense mutation.This observation may be elucidated by the neonatal creatine monitoring mechanism,a phenomenon not previously reported.CASE SUMMARY We analysed the case of a neonate presenting solely with elevated CK levels who was eventually discharged after supportive treatment.The chief complaint was identification of increased CK levels for 15 d and higher CK values for 1 d.Ad-mission occurred at 18 d of age,and despite prolonged treatment with creatine and vitamin C,the elevated CK levels showed limited improvement.Whole exo-me sequencing revealed the presence of a c.715C>G mutation in LAMA2 in the newborn,correlating with a clinical phenotype.However,the available informa-tion offers insufficient evidence for clinical pathogenicity.CONCLUSION Mutations in LAMA2 are associated with the clinical phenotype of increased neonatal CK levels,for which no specific treatment exists.Whole genome sequen-cing facilitates early diagnosis.
文摘Creatine transporter(CrT)-deficiency,the most common form of the cerebral creatine deficiency syndromes,causes cognition impairments and severe reduction of the brain creatine(Cr)and phosphocreatine(PCr)levels,and responds poorly to oral Cr supplement as a treatment option.The ca uses of cognitive impairments in CrT-deficient children remain unclear.We recently use gene-targeting to create a mouse model of CrT-deficiency to assess the impacts of Cr/PCr deficiency on brain energetics and stress-adaptation responses(Chen et al.,2021).
文摘A specific, precise and accurate ion-pair HPLC-UV method has been developed and validated for simultaneous determination of phosphocreatine (PCr), and its metabolite creatine (Cr) as well as related ATP in plasma and red blood cell (RBC) of rabbits. After addition of TMP as IS, the samples were deproteinized with 6% PCA. The analytes were separated on a Kromasil C18 column using a tertiary gradient mobile phase composed of buffer A (0.2% KH2PO4 + 0.08% tetrabutyl ammonium hydrogen sulphate, pH 3.0), buffer B (buffer A adjusted to pH 7.5 with 1 mol/L NaOH) and MeOH. Detection wavelengths were set at 210 nm for PCr and Cr and 260 nm for ATP and TMP. Some blank samples were initially run for baseline subtraction. The linear detection responses were obtained for PCr concentration over a range of 10 - 7500 mg/ml (plasma) and 5 - 2500 mg/ml (RBC) and for both Cr and ATP concentrations of 10 - 1500 mg/ml (plasma) and 5 - 750 mg/ml (RBC) (r > 0.99). The QC samples of 3 analytes showed intra-day and inter-day precisions (RSD) of - 107%. The method was successfully used to simultaneously determine plasma and RBC concentrations of the 3 analytes and to study pharmacokinetics after iv administration of PCr to rabbits.
基金This study was supported by grants from the National Key R&D Program of China (2017YFC0908700, 2017YFC0908703)National S&T Fundamental Resources Investigation Project (2018FY100600, 2018FY100602)+2 种基金Taishan Pandeng Scholar Program of Shandong Province (tspd20181220)Taishan Young Scholar Program of Shandong Province (tsqn20161065, tsqn201812129)Key R&D Program of Shandong Province (2020SFXGFY03, 2019GSF108073)
文摘BACKGROUND:The early diagnosis of acute myocardial infarction(AMI)remains challenging,especially for institutions without the high-sensitive cardiac troponin(hs-c Tn)assay.Herein,we aim to assess the value of creatine kinase-myocardial band isoenzyme(CK-MB)combined with different cardiac troponin(c Tn)assays in AMI diagnosis.METHODS:This multicenter,observational study included 3,706 patients with acute chest pain from September 1,2015,to September 30,2017.We classified the participants into three groups according to the c Tn assays:the point-of-care c Tn(POC-c Tn)group,the contemporary c Tn(c-c Tn)group,and hs-c Tn group.The diagnostic value was quantified using sensitivity and the area under the curve(AUC).RESULTS:Compared to the single POC-c Tn/c-c Tn assays,combining CK-MB and POC-c Tn/c-c Tn increased the diagnostic sensitivity of AMI(56.1%vs.63.9%,P<0.001;82.7%vs.84.3%,P=0.025).In contrast,combining CK-MB and hs-c Tn did not change the sensitivity compared with hs-c Tn alone(95.0%vs.95.0%,P>0.999).In the subgroup analysis,the sensitivity of combining CKMB and c-c Tn increased with time from symptom onset<6 h compared with c-c Tn alone(72.8%vs.75.0%,P=0.046),while the sensitivity did not increase with time from symptom onset>6 h(97.5%vs.98.3%,P=0.317).The AUC of the combination of CK-MB and POC-c Tn significantly increased compared to the single POC-c Tn assay(0.776 vs.0.750,P=0.002).The AUC of the combined CKMB and c-c Tn/hs-c Tn assays did not significantly decrease compared with that of the single c-c Tn/hs-c Tn assays within 6 h.CONCLUSIONS:The combination of CK-MB and POC-c Tn or c-c Tn may be valuable for the early diagnosis of AMI,especially when hs-c Tn is not available.
文摘Creatine is one of the most popular forms of protein supplements and is known to improve performance in healthy athletic populations via enhanced muscle mass and adenosine triphosphate energy regeneration.Clinical use of creatine may similarly benefit patients with rheumatoid arthritis(RA), an inflammatory condition characterised by generalised muscle loss termed"rheumatoid cachexia". The adverse consequences of rheumatoid cachexia include reduced strength, physical function and, as a consequence, quality of life.Whilst regular high-intensity exercise training has been shown to increase muscle mass and restore function in RA patients, this form of therapy has very low uptake amongst RA patients. Thus, acceptable alternatives are required. The aim of this review is to consider the potential efficacy of creatine as an anabolic and ergonomic therapy for RA patients. To date, only one study has supplemented RA patients with creatine, and the findings from this investigation were inconclusive. However, trials in populations with similar losses of muscle mass and function as RA, including older adults andthose with other muscle wasting conditions, indicate that creatine is an efficacious way of improving muscle mass, strength and physical function, and may offer an easy, safe and cheap means of treating rheumatoid cachexia and its consequences.
基金supported by the National Natural Science Foundation of China,No.81301093(to XPC)。
文摘Creatine kinase is a muscle enzyme that has been reported at various levels in different studies involving patients with amyotrophic lateral sclerosis.In the present retrospective case-control study,we included 582 patients with amyotrophic lateral sclerosis and 582 age-and sexmatched healthy controls.All amyotrophic lateral sclerosis participants received treatment in the Department of Neurology,West China Hospital,China,between May 2008 and December 2018.Serum creatine kinase levels in patients with amyotrophic lateral sclerosis were significantly higher than those in healthy controls.Subgroup analysis revealed that serum creatine kinase levels in men were higher than those in women in both amyotrophic lateral sclerosis patients and healthy controls.Compared with patients with bulbar-onset amyotrophic lateral sclerosis,patients with limb-onset amyotrophic lateral sclerosis had higher creatine kinase levels.Spearman's correlation analysis revealed that serum creatine kinase levels were not correlated with body mass index,Amyotrophic lateral Sclerosis Functional Rating ScaleRevised score,or progression rate.After adjusting for prognostic covariates,higher log creatine kinase values were correlated with higher overall survival in the amyotrophic lateral sclerosis patients.We also investigated the longitudinal changes in serum creatine kinase levels in 81 amyotrophic lateral sclerosis patients;serum creatine kinase levels were decreased at the second blood test,which was sampled at least 6 months after the first blood test.Together,our results suggest that serum creatine kinase levels can be used as an independent factor for predicting the prognosis of amyotrophic lateral sclerosis patients.This study received ethical approval from the Ethics Committee of West China Hospital,China(approval No.2015(236))on December 23,2015.
文摘KEY POINTS Because so much of the running in soccer is at less than maximal sprinting speed, creatine supplementation likely provides no benefit to match performance.Overwhelming evidence proves that a diet rich in carbohydrates can fill muscles with glycogen, and glycogen is critical to optimal performance in soccer. Soccer players’ diets, especially in the days before hard training or competition, should include 8-10 grams of carbohydrate per kilogram of body weight (3.5-4.5 g/lb). Cereals, fruits, vegetables, breads, and pastas are good sources ofcarbohydrates. Refueling of muscle with carbohydrates should begin as soon as possible following a match or a strenuous training session. Inadequate replacement of fluids lost in sweat can lead to poor soccer performance and heat illness. Players should aim to drink enough during training sessions and matches so that their body weights after play are within about 1 kg (2.2 lb) of their starting weights. For a light workout or an easy match, especially when the weather is cool, water can be an adequate fluid replacement, if enough is ingested. But when play is strenuous and the weather is hot, carbohydrate-electrolyte sports drinks do a better job of maintaining body fluids.
文摘Background: Creatine supplementation is a subject that is very well studied. New forms of creatine are suggesting improvements in this supplement performance. Creatine HCl is supposed to have better solubility and absorption than creatine. The aim of this study was to compare the effects of two different doses of creatine HCl with creatine monohydrate on the strength and body composition in recreational weightlifters and to verify the relationship between strength and body composition. Methods: 40 subjects were divided in four groups: Creatine Monohydrate (CMG) 5 g/daily;Creatine HCl-1 (HCl-1) 5 g/daily, Creatine HCl-2 (HCl-2) 1.5 g/daily and Control group (CG) = 5 g of resistant starch/daily. All groups performed a resistance training program during 4 weeks. Body composition and strength were evaluated pre and post intervention. Results: The 1 RM at the Leg press was increased significantly in all groups (CMG: pre = 264.4 ± 83.8 × post = 298.1 ± 90.9;HCl-1: pre = 295.0 ± 88.3 × post = 338.3 ± 86.8 and HCl-2: pre = 274.3 ± 57.1 × post = 305.7 ± 59.4;p p = 0.003), however, there was no statistically significant difference between groups. Fatmass was significantly decreased in HCl-1 (pre = 14.5 ± 8.0 × post = 13.3 ± 8.3;p = 0.034) and HCl-2 (pre = 13.8 ± 5.8 × post = 12.7 ± 5.6;p = 0.005) but fat-free mass was increased only in HCl-1 (pre = 52.2 ± 8.9 × post = 53.8 ± 8.9;p = 0.031), with no differences between groups again. We observed strong correlations between upper limb strength and fat mass (r = 0.93, p p < 0.05) only in HCl-1 group. Conclusions: We concluded that creatine HCl and creatine Monohydrate improve performance but only creatine HCl induces changes on body composition in recreational weightlifters with differences between creatine HCl doses.
文摘Objective To study the differential diagnosis on cerebral concussion and mild cerebral contusion value of the brain type creatine kinase isoenzyme(CK BB),and evaluate the seriousness of brain damage and prognosis of the patients with acute head injury.Methods Chromatographic separating and fluorometric quantifying technique was used to detect the CK BB activity in the cerebrospinal fluid(CSF) of 117 patients with acute head injury and 12 patients with increased intracranial pressure and 20 normal people.Results The CSF CK BB activity of the patients with acute head injury was remarkably higher than that of the normal people and the CSF CK BB activity increased with the seriousness of brain damage.There was a close relationship between CSF CK BB activity and prognosis,and higher activity of CSF CK BB indicated poor prognosis.Conclusion CSF CK BB activity could be used as a new index to diagnose brain damage and evaluate the seriousness of brain damage and prognosis.
文摘Objective To observe the relationship between serum creatine kinase isoenzyme MM sub-bands (CKMM3/MM1 ratio) and the gradation of coronary stenosis and provide a simple, reliable, and economical method for identifying high-risk unstable angina pectoris (UAP). Mehtods Blood samples were drawn at different time after onset of chest pain in 21 patients with UAP and only once in 20 each volunteers for control. CKMM3/MM1 ratio was detected by nonserial buffer agarose gel electrophoresis. CKMB and CK were observed by velocity method. An emergent coronary arteriography was performed as soon as patients were admitted into hospital. Results Patients with UAP were divided into two subgroups: patients with elevated serum enzyme [P( + )] and patients with normal serum enzyme [P( - ) ] according to CKMM3/MM1 ratio 【 0. 5. Patients with UAP( + ) had higher serum CKMM3/MM1 ratios from 0.5 to 12hrs and serum CKMB from 2 to 12 hrs than those with UAP( - ) and control ( P 【 0. 05) . Serum enzyme concentrations of
文摘Objective: To observe the influence of nicorandil combined with creatine phosphate sodium on serum BNP, CK-MB, cTnl, SOD, MMP-9 and cardiac function in patients with STEMI. Methods: A total of 60 patients with STEMI were randomly divided into observation group (30 cases) and control group (30 cases). Observation group: nicorandil combined with creatine phosphate sodium;control group: patients were treated only by nicorandil. Recording and comparing the levels of BNP, CK-MB, cTnl, SOD, MMP-9 and cardiac function before and after treatment. Results: (1) Before treatment, there was no statistically significant difference in the serum BNP, CK-MB, cTnl, SOD, MMP-9 levels between the two groups. After treatment, compared with the same group before treatment, the serum BNP, CK-MB, cTnl, MMP-9 levels of the two groups were significantly lower, the serum SOD level of the two groups were significantly higher, and those levels of observation group were significantly better than the control group, there was significant difference between the two groups. (2) Before treatment, there was no statistically significant difference in LVEF, WMSI, CI between the two groups. After treatment, compared with the same group before treatment, the WMSI of the two groups were significantly lower, the LVEF and CI of the two groups were significantly higher, and those levels of observation group were significantly better than the control group, there was significant difference between the two groups. Conclusion: Nicorandil combined with creatine phosphate sodium for patients with STEMI can perfect the levels of serum BNP, CK-MB, cTnl, SOD, MMP-9 and be beneficial to protect their cardiac function.
文摘Objectives: Exploring the long-term consequences of elevated postoperative creatine phosphokinase MB as a surrogate measure of cardiac tissue damage subsequent to coronary artery bypass graft surgery (CABG). Methods: Prospective cohort study including 414 patients subjected to solitary CABG at Odense University Hospital from September 30, 2007, and backwards in time. According to individual postoperative peak creatine phosphokinase MB, patients were assigned to five subgroups. Kaplan Meier survival analysis and Cox proportional-hazard regression evaluated the predictive value of creatine phosphokinase MB on late death from cardiac cause and diagnosis of paroxysmal or persistent atrial fibrillation within 10-years after CABG. Results: There was a general numeric decrease in time to cardiac death with increasing postoperative creatine phosphokinase MB (Log-Rank 0.285). Creatine phosphokinase MB p p p = 0.002) increase in paroxysmal or persistent atrial fibrillation in patients with postoperative atrial fibrillation compared to patients in postoperative sinus rhythm. Conclusions: Postoperative creatine phosphokinase MB was not found predictive of late cardiac death or diagnosis of paroxysmal or persistent atrial fibrillation within 10 years after CABG. However, patients with postoperative atrial fibrillation had increased risk of later developing paroxysmal or persistent atrial fibrillation compared to patients in postoperative sinus rhythm.
文摘Objective:To study the cardioprotective effectof creatine phosphate sodium by measuring perioperative myocardial enzymes of allogeneic liver transplant patients.Methods:The 26 cases of orthotopic liver transplant patients were randomly divided into control group and creatine phosphate sodium group,13 cases in each group.The creatine phosphate sodium group were given creatine phosphate sodium 30 mg/kg at the time of induction.The control group were given equal amount of N.S.All patients at six-phase of before surgery,after anesthesia,before anhepatic phase,anhepatic phase,new liver and the 12 hours after surgery extraction radial artery.The CK,CK-MB and cTnI activity was measured.Results:To compared with control group,creatine phosphate sodium group’s plasma CK,CK-MB and cTnI concentrations in before anhepatic phase,anhepatic phase,new liver and the 12 hours after surgery 4 phase are significantly lower(p<0.05).Conclusions:The creatine phosphate sodium has a protective effect on liver transplantation in patients with myocardial damage.
文摘Purpose The aim was to explore five established SNPs(rs1815739,rs1805086,rs2700352,rs28497577,and rs28357094)that are known to modulate skeletal muscle protein kinetics in response to creatine supplementation.Methods A randomized,placebo-controlled,repeated measures design was used.Participants(n=152)were rand-omized divided into one of two groups:CREA(20 g/day creatine monohydrate)or PLAC:(dextrose)for 7 days.SNP were assessed,and participants were classified accordingly.Before and after supplementation,anthropometrics(height and body mass)and performance measures(vertical jump,countermovement vertical jump,squat jump,abdominal crunches,and maximum push-ups)were assessed.Results CREA gained more body mass than PLAC(CREA:Δ0.864±0.06 kg;PLAC:Δ0.154±0.07 kg,P<0.001).In the CREA group,the presence of an A allele for the MYLK1 polymorphism was related to changes in countermovement jump height(P=0.027;effect size[d]=0.41)and leg power(P=0.040,effect size[d]=0.18).The total number of abdominal crunches after supplementation was influenced by treatments and SPP1 gene(P=0.041).A higher number of abdominal crunches was associated with the G allele in the CREA group and the TT genotype in the PLAC group(effect size[d]=0.04).Conclusion Collectively,short-term creatine supplementation increased body mass but was unable to alter muscle perfor-mance.However,following creatine supplementation,participants expressing A alleles in the MYLK1 polymorphism had a greater increase in jump height and leg power and participants expressing G alleles in the SPP1 gene had greater improve-ments in abdominal crunch performance.
文摘Background: The exRML (exertional rhabdomyolysis) is a pathophysiologic condition of skeletal muscle cell damage and breakdown associated with high intensity or prolonged exercise, normal exercise under extreme circumstances, or sudden and excessive skeletal muscle contraction. It may manifest from the increase in CK (creatine kinase) or MYO (myoglobin), a protein that can cause life-threatening injury to the kidney (AKI, acute kidney injury), and may or may not be associated with myoglobinuria. Here, we presented a case of exRML with AKI, and then reviewed the related reports. Vigorous hydration, sodium bicarbonate and furosemide are key treatments. Aim: To examine an elderly patient with exRML induced AKI and the key treatment process. Case summary: A 61-year-old man left our hospital without permission after his admission and has been walking for almost 30 kms with no water and food intake, then was diagnosed exRML and exRML induced AKI with an obvious elevation of CK, MYO and decrease of eGFR (estimated glomerular filtration rate) after coming back, and was treated with vigorous hydration, loop diuresis, sodium bicarbonate, prostaglandin and Shenkang injection. After vigorous resuscitation, the patient’s renal function, CK and MYO returned normal. Conclusions: The exRML can cause serious complications such as AKI and death. Delayed diagnosis can be critical;therefore, manner of time should be taken to achieve a favorable prognosis.
文摘We studied the relations of intracranial pressure (ICP),creatine kinase (CK) and bralnstem auditory evoked potential (BAEP) in 44 patients with traumatic brain edema who were admitted to our hospital from June 1990 to February 1991. There were 30 males and 14 females, with age range from 9 to 67 years. The results showed that the abnormal BAEP could reflect the severity of cerebral edema in acute head injury and was related to ICP and serum CK levels. When ICP】30 mmHg (4kPa), the abnormality of BAEP was more obvious than that of the control group (P【0.05); the serum CK levels were also elevated markedly. In patients with ICP over and below 4kPa, the rate of abnormal BAEP was 38.46% and 77.78% respectively (P【0.05). The serum CK level in the normal group or in the group with moderate abnormality of BAEP was significantly different from that in the group with severe abnormality or lack of BAEP (274.8± 98.24 U/L vs 705.3± 364.27 U/L; P【0.001). After treatment, the ICP returned to normal, and the BAEP
文摘Carbon quantum dot/carbon dot(CD)exhibiting sustained photoluminescence at longer wavelengths in aqueous solution is difficult to prepare,but has enormous potential in biomedical applications.For the first time,we report the magnesium(II)selective fluorescence enhancement of a red-light emitting anthrarufin and boric acid-derived CD in aqueous solution for direct evaluation of creatine kinase(CK)enzyme activity.The CD displayed visually detectable,intense red fluorescence only in the presence of magnesium ion(Mg^2+)at physiological pH value when irradiated with an ultraviolet(UV)source.Concurrently,a significant increase in steady-state fluorescence intensity and fluorescence lifetime was documented.A time-dependent density functional theory(TD-DFT)analysis displayed a bathochromic shift in UV–visible(vis)absorption,and increased oscillator strength of transition resulting from the selective chelation of Mg^2+withβ-hydroxy keto functionality on the surface of the CD.The CD–Mg^2+assembly was subsequently used to conceptualize the detection of CK directly through the exploration of the differential binding affinity of Mg2+with adenosine triphosphate(ATP),adenosine diphophate(ADP),and CD that is otherwise not possible with commercially available kits as of today.Thus,the report delineated here usher grandeur potential of CD for biological explorations related to Mg2+or ATP sensing and monitoring of Mg^2+-dependent enzymatic activity through a clear understanding of the chemistry.
文摘It has been previously reported that during the denaturation of creatine kinase (ATP: creatine phosphotransferase EC 2.7.3.2) by guanidine hydrochloride, urea or SDS, inactivation occurs before noticeable conformational change of the enzyme molecule as a whole can be detected, and the inactivation rate constants of the enzyme in the above denaturant solutions are several orders of magnitude faster than those of
文摘Myoglobin, myosin creatine kinase MM (CK-MM),creatine kinase BB(CK-BB) in cardiac muscle and H chain ofmyosin in atrial and ventricular muscle were studied in speci-mens from 8 patients who died of sudden nocturnal deathsyndrome(SNDS) by avidin-biotin complex (ABC) method toinvestigate the possible early or very early myocardial ischemiain the syndrome. Hematoxylin-eosin staining was conductedfor comparison. The results showed evident loss of CK-MM,CK-BB, myoglobin and myosin from cardiac muscle cells, indi-caring that occurrence of SNDS is closely associated withacute myocardial ischemia.
