Serum Creatinine Kinase Levels Research Articles

A comparative analysis of neuroendoscopic foramen magnum decompression versus traditional open surgery for Chiari Malformation Type I.

Chiari Malformation Type I (CM1) is characterized by the downward displacement of the cerebellar tonsils below the foramen magnum. The standard surgical treatment for CM1 is foramen magnum decompression and atlas laminectomy (FMD-AL). However, there is a growing interest in exploring minimally invasive techniques, such as neuroendoscopically assisted FMD-AL, to optimize surgical outcomes. The aim is to present the results of the less invasive neuroendoscopic-assisted system application as an alternative to decompression surgery in patients with CM-1 with/without syringomyelia. A retrospective analysis was conducted on 76 patients with CMI who underwent either neuroendoscopic-assisted FMD-AL (n = 23) or open surgery (n = 53). Preoperative and postoperative assessments were performed, including pain levels, functional assessment, outcome and serum creatinine kinase (CK) levels. Surgical parameters and radiological imaging were also evaluated and compared. Both surgical groups showed improvements in pain levels and increase in postoperative CK levels. There were no statistically significant differences between the groups in terms of postoperative JOA scores, VAS scores, CCOS, or syrinx resolution. However, the neuroendoscopic group had significantly lower CK levels, shorter hospital stays, less blood loss, and shorter operation times compared to the open surgery group, indicating reduced muscle damage and potential benefits of the neuroendoscopic assisted approach. Both neuroendoscopy and open surgery groups can effectively alleviate symptoms and improve outcomes in patients with CM1. The neuroendoscopic assisted technique offers the advantage of reduced muscle damage and shorter hospital stays. The choice of surgical technique should be based on individual patient characteristics and preferences. 3 (Retrospective case-control study) according to using the Oxford Centre for Evidence-Based Medicine (CEBM) Table.

Immune-mediated Necrotizing Myopathy in a Patient with Microscopic Polyangiitis: A Case Report.

We herein report a case of immune-mediated necrotizing myopathy (IMNM) in a patient with microscopic polyangiitis (MPA). A 77-year-old Japanese woman presented with a 2-day history of proximal muscle weakness and myalgia, with elevated serum creatinine kinase (CK) levels. Findings of a muscle biopsy were compatible with IMNM; however, anti-SRP and anti-HMGCR antibodies were negative. She also had peripheral neuropathy with elevated serum MPO-ANCA titers, leading to a diagnosis of MPA. IMNM can be a pathological result of MPA muscle involvement.

Quadriceps recovery and pain relief in knee osteoarthritis rats by cog polydioxanone filament insertion.

Quadriceps muscles play a pivotal role in knee osteoarthritis (OA) progression and symptom manifestation, particularly pain. This research investigates the therapeutic effectiveness of muscle enhancement and support therapy (MEST), a recently developed device intended for intramuscular insertion of cog polydioxanone filaments, in quadriceps restoration to alleviate OA pain. Knee OA was induced in Sprague Dawley rats via monoiodoacetate injections. MEST or sham treatment was performed in OA or Naive rat quadriceps. Pain was assessed using paw withdrawal threshold and weight bearing. Quadriceps injury and recovery via MEST were evaluated using biomarkers, tissue morphology, muscle mass, contractile force and hindlimb torque. Satellite cell and macrophage activation, along with their activators, were also assessed. Data were compared at 1- and 3-weeks post-MEST treatment (M-W1 and M-W3). MEST treatment in OA rats caused muscle injury, indicated by elevated serum aspartate transferase and creatinine kinase levels, and local β-actin changes at M-W1. This injury triggered pro-inflammatory macrophage and satellite cell activation, accompanied by heightened interleukin-6 and insulin-like growth factor-1 levels. However, by M-W3, these processes gradually shifted toward inflammation resolution and muscle restoration. This was seen in anti-inflammatory macrophage phenotypes, sustained satellite cell activation and injury markers regressing to baseline. Quadriceps recovery in mass and strength from atrophy correlated with substantial OA pain reduction at M-W3. This study suggests that MEST-induced minor muscle injury triggers macrophage and satellite cell activation, leading to recovery of atrophied quadriceps and pain relief in OA rats.

Paraspinal myositis in a patient with COVID-19 infection: a case report

Myalgia is one of the most common symptoms of COVID-19 infection. With the progression of the COVID-19 pandemic, emerging cases of COVID-related myositis have been reported. However, COVID-19-related myositis of the paraspinal muscle is uncommon. We present a 66-year-old female who was admitted to the hospital for hemorrhagic shock secondary to gastrointestinal bleeding. She developed COVID-19 respiratory tract infection symptoms on the second day of admission with reported severe lower back pain 3 days later. Serum creatinine kinase level was slightly elevated, and magnetic resonance imaging (MRI) revealed paraspinal myositis.

Phenotypic features of dystrophin gene knockout pigs harboring a human artificial chromosome containing the entire dystrophin gene

Mammalian artificial chromosomes have enabled the introduction of extremely large amounts of genetic information into animal cells in an autonomously replicating, nonintegrating format. However, the evaluation of human artificial chromosomes (HACs) as novel tools for curing intractable hereditary disorders has been hindered by the limited efficacy of the delivery system. We generated dystrophin gene knockout (DMD-KO) pigs harboring the HAC bearing the entire human DMD via asomatic cell cloning procedure (DYS-HAC-cloned pig). Restored human dystrophin expression was confirmed by immunofluorescence staining in the skeletal muscle of the DYS-HAC-cloned pigs. Viability at the first month postpartum of the DYS-HAC-cloned pigs, including motor function in the hind leg and serum creatinine kinase level, was improved significantly when compared with that in the original DMD-KO pigs. However, decrease in systemic retention of the DYS-HAC vector and limited production of the DMD protein might have caused severe respiratory impairment with general prostration by 3months postpartum. The results demonstrate that the use of transchromosomic cloned pigs permitted a straightforward estimation of the efficacy of the DYS-HAC carried in affected tissues/organs in a large-animal disease model, providing novel insights into the therapeutic application of exogenous mammalian artificial chromosomes.

Inclusion body myositis triggerred with long-term imatinib use.

Imatinib is an orally administered tyrosine kinase inhibitor with wide clinical use in different indications from solid tumors to hematologic malignancies. Inclusion body myositis (IBM) is an acquired myopathy of both inflammatory and degenerative nature. We present an 81 years old male with a history of gastrointestinal stromal tumor (GIST) operated 8 years ago and was evaluated for the progressive loss of weight and muscle strength leading to total immobilization in 6 months. He was under imatinib for 8 years despite the remission of GIST. Physical examination disclosed diffuse loss of muscle strength, most prominently involvement of distal upper and proximal lower extremity in an asymmetrical pattern with normal serum creatinine kinase level (CK). Further investigations including bilateral thigh MRI, electromyography (EMG), and PET/CT suggested myositis and degenerative myopathy and ruled out any malignancy. Quadriceps femoris biopsy proved the diagnosis of IBM and no trigger except for imatinib was displayed. Clinical improvement in terms of weight loss and muscle weakness was achieved after the discontinuation of imatinib. This is the first case of IBM associated with prolonged use of imatinib not reported in the literature so far. Since imatinib is widely used in different conditions, it is important to be aware of even its rare adverse effects. Poor response of IBM to conventional immunosuppressive agents enhances the value of etiology identification to relieve symptoms in addition to supportive care.

Chlamydia psittaci pneumonia: a clinical analysis of 12 patients.

We conducted a retrospective analysis to explore the clinical characteristics, laboratory examination, imaging features, treatment outcomes, and prognosis of the Chlamydia psittaci (C. psittaci) pneumonia, aiming to improve early diagnosis and treatment. The clinical data of 12 patients with C. psittaci pneumonia diagnosed by metagenomic next-generation sequencing (mNGS) in our hospital were retrospectively analyzed. These data included baseline information, epidemiological history, clinical symptoms and signs, laboratory and chest computed tomography (CT) examination findings, treatment schemes, and prognosis. The average age of the 12 patients was 58.25±13.27 years, and there were 7 (58.3%) males and 5 (41.7%) females in this cohort. Five patients had clear exposure to poultry or birds. The main clinical manifestations included fever (12/12, 100.0%), cough (12/12, 100.0%), expectoration (10/12, 83.3%), and dyspnea (10/12, 83.3%). Laboratory examination showed marked elevation of the total white blood cell (WBC) count, neutrophil (NEUT) count, C-reactive protein (CRP), procalcitonin (PCT), D-dimer, aspartate aminotransferase (AST), alanine aminotransferase (ALT), serum creatinine, and creatine kinase (CK) levels; as well as decreased hemoglobin (HGB), blood platelet (PLT), and albumin (ALB) levels. Arterial blood gas analysis showed that the average value of the oxygenation index (PO2/FiO2) was 290.9±83.1, which was less than 300 in 6 cases (50.0%). The main chest CT features were patchy or consolidation in the bilateral or unilateral lungs, and the boundary was not clear but showed a bronchial inflation sign. Also, some of the cases were accompanied by pleural effusion. Once the etiology was obtained, the patients were quickly treated with doxycycline combined with other antibiotics. All 12 patients improved and were discharged from the hospital. However, two severe patients were admitted to the intensive care unit (ICU) and received ventilation and monitoring treatment. There were no deaths. C. psittaci pneumonia is an atypical community-acquired pneumonia (CAP) caused by C. psittaci infection, with its own laboratory and imaging characteristics. In this study, diagnosis was established based on the application of mNGS owing to the absence of easily available conventional pathogenic evidence. In addition, an aggressive and precise treatment strategy can help achieve a favorable prognosis for patients.

Rhabdomyolysis in Children Under Treatment for Diabetic Ketoacidosis at National Institute of Child Health

Objectives: To determine the frequency of rhabdomyolysis in children under treatment for Diabetic Ketoacidosis (DKA) at National Institute of Child Health (NICH). Materials and Methods: Total 100 patients who were suffering from DKA were enrolled. Blood and urine samples were collected from of each child in aseptic conditions in sterilized container and sent for base line labs, urine ketone and serum creatinine kinase (CK) levels. All the demographic details and duration of diabetes mellitus and DKA and its treatment were recorded on a predesigned data collection proforma. Results: Out of 100 patients, 43 were male and 57 were female with mean age of 9.79±3.26 years. Seventy percent of the patients were aged 7.1-14 years, followed by 25.0% patients were aged 1-7 years and only 5.0% patients were aged of 14.1-18 years. The Mean duration of DM and DKA were 3.89±2.75 years and 9.72±4.65 hours respectively. Mean RBS, Urea and Creatinine level were 404.9± 99.1, 35.70±27.5 mg/dl and 0.73±0.75 mg/dl respectively. Mean hemoglobin and WBC level were 13.61±14.3 g/l and 16.33±11.8 respectively. The mean Na level was 137.79±5.64 mmol/l, mean K level was 4.01±0.89 mmol/l, mean Phosphorus level was 27.6±42.0 mg/dl and mean Cl level was 106.8±6.3 mmol/l. The mean PH was found 7.1±6.49 and mean Bicarbonate was 7.56±3.01 mmol/l. Fifty two percent patients were treated for more than 24 hours and 48.0% patients were treated for less than 24 hours. 7% patients under treatment for DKA were suffered from Rhabdomyolysis and expiry was 5%. Practical implication: In this study we find out that there is very little chance of causing rhabdomyolysis under treatment for DKA but we should take precautionary measures to prevent its progression towards its complications i.e., renal failure and cardiac Arrest which can lead to mortality. This study will lead a positive role in early cure of the DKA patients to avoid further complication. Conclusion: It is concluded that in DKA patients Rhabdomyolysis is of clinical concern because it can result in significant morbidity and mortality by causing Acute Renal failure, DIC, Cardiac Arrest, Arrythmias, significant electrolyte imbalance, but there is very little chance of causing rhabdomyolysis under treatment for DKA in our setup and it too can be avoided by early and aggressive treatment of DKA. Keywords: Rhabdomyolysis, Diabetic Ketoacidosis, Children, Hemoglobin , Creatinine

The characteristics and costs of severe theophylline toxicity in a tertiary critical care unit in Eastern Cape Province, South Africa.

Severe theophylline toxicityrequiring haemodialysisaccounts for approximately one-third of drug toxicity cases admittedto theLivingstone Tertiary Hospital (LTH) intensive care unit (ICU) in Gqeberha, South Africa, imposing a significant resource burden. To investigate the characteristics and burden of severe theophylline toxicity in an Eastern Cape Province tertiaryhospital adult ICU. A retrospective review of all severe theophylline toxicity admissions to the ICU from 1 January 2013 to 31 December 2018 was conducted. Demographic and clinical data were captured and analysed. The National Department of Health 2019 fees schedule was used to calculate costs based on duration of ICU stay and number of haemodialysis sessions received. Of the 57 patients included in the study, 84% were cases of deliberate self-harm. The majority were aged <40 years (77%) and female (79%). The mean (standard deviation (SD)) initial serum theophylline level was 612 (269) µmol/L. Complications included convulsions (n=12; 21%), arrhythmias (n=9; 16%), need for mechanical ventilation (n=7; 12%) and death (n=4; 7%). The main risk factors for these complications were age ≥30 years, an inappropriately normal or elevated initial serum potassium level, an elevated serum creatinine kinase level and an elevated initial serum theophylline level. Receiver operator characteristic curve analysis assessing the initial serum theophylline level as a discriminator for life-threatening complications produced an area under the curve of 0.71 for serum theophylline >400 µmol/L (sensitivity 88%, specificity 12%). All the 4 patients who died had an initial serum theophylline level >1 000 µmol/L. The mean (SD) cost per admission amounted to ZAR16 897 (10 718), with a mean of one 4-hour dialysis session per admission. Severe theophylline toxicity, usually in the context of deliberate self-harm, is a preventable yet life-threatening toxicity encountered at LTH. Demographic risk factors include young females from certain areas in and around Gqeberha. Risk factors for complications include olderage, paradoxically normal or elevatedserumpotassiumlevels, elevatedserumcreatinine kinaselevelsandan initial serum theophylline level >400 µmol/L.Patients with these clinical features should be closely monitored and treated timeously at an appropriate level of care.The need for ICU admission and dialysis, both limited resources, makes the treatment of severe theophylline toxicity costly. Further studies of the underlying psychosocial drivers, local prescribing practicesand preventive interventionsrelated to severe theophylline toxicityare required.

An Ultra-Rare Manifestation of an X-Linked Recessive Disorder: Duchenne Muscular Dystrophy in a Female Patient

Duchenne muscular dystrophy (DMD) is the most common inherited muscle dystrophy. Patients are characterized by muscle weakness, gross motor delay, and elevated serum creatinine kinase (CK) levels. The disease is caused by mutations in the DMD gene located on the X chromosome. Due to the X-linked recessive inheritance pattern, DMD most commonly affects males, who are generally diagnosed between the age of 3-5 years. Here we present an ultra-rare manifestation of DMD in a female patient. Cytogenetic examination showed that she has a t(X;10)(p21.1;p12.1) translocation, which turned out to affect the DMD gene with one of the breakpoints located in exon 54 (detected by genome sequencing). The X-inactivation test revealed skewed X-inactivation (ratio 99:1). Muscle histology and dystrophin immunohistochemistry showed severe dystrophic changes and highly reduced dystrophin expression, respectively. These results, in accordance with the clinical picture and a highly elevated serum CK, led to the diagnosis of DMD. In conclusion, although in very rare cases, DMD can manifest in female patients as well. In this case, a balanced X-autosome reciprocal translocation disrupts the DMD gene and skewed X-inactivation leads to the manifestation of the DMD phenotype.

PET-MRI in idiopathic inflammatory myositis: a comparative study of clinical and immunological markers with imaging findings

BackgroundWe sought to determine the utility of PET-MRI in diagnosing Idiopathic Inflammatory Myositis (IIM), and look for association between FDG uptake and clinical, pathological and laboratory parameters.MethodsA retrospective, observational study was conducted on IIM patients having positive serum autoantibodies and who underwent PET-MRI (3-Tesla SIEMENS Biograph MR scanner) between 2017 and 2021. Thirty patients who underwent PET-MRI to detect systemic metastasis without muscle involvement formed the control group.ResultsIn the IIM cohort, female: male sex ratio was 1.73, mean age at diagnosis was 40.33 years, and the mean duration of illness was 7 months. 33.33% of patients had severe limb weakness. Mi2B (43.33%), Mi2A (43.33%), PL-7(10%), PL-12(6.67%), SRP (16.67%), Tif1gamma (3.33%), NxP2 (3.33%), Ro-52(40%), PM-Scl, U1-RNP, ANA (26.67%) were the serum autoantibodies identified. Using SUV max Ratio to quantify FDG uptake, PET-MRI showed a sensitivity of 100% with 93.3% specificity in diagnosing IIM.FDG uptake was maximum in proximal lower limb region followed by proximal upper limb. Multivariate regression analysis showed that the severity of muscle weakness, serum Mi2B antibody positivity and serum creatinine kinase levels had a significant positive correlation with FDG uptake (value of 0.005, 0.043, 0.042, respectively for whole-body FDG uptake). FDG uptake also showed good correlation with histopathological features and muscle MRI, but there was no significant association with treatment response. Three female patients in our cohort had primary malignancy involving the breast, uterus, and cervix.ConclusionsPET-MRI is a promising diagnostic modality for IIM. PET-MRI reflects the severity of muscle inflammation, showing good association with various clinical/laboratory parameters, histopathology, and muscle MRI. Parameters associated with severe muscle inflammation in PET-MRI—clinical severity of muscle weakness, Mi2B positivity, and serum creatine kinase levels—may be used as clinical/laboratory markers of disease severity in IIM. PET-MRI has the added advantage of detection of systemic malignancy.

Exon skipping induces uniform dystrophin rescue with dose-dependent restoration of serum miRNA biomarkers and muscle biophysical properties

Therapies that restore dystrophin expression are presumed to correct Duchenne muscular dystrophy (DMD), with antisense-mediated exon skipping being the leading approach. Here we aimed to determine whether exon skipping using a peptide-phosphorodiamidate morpholino oligonucleotide (PPMO) conjugate results in dose-dependent restoration of uniform dystrophin localization, together with correction of putative DMD serum and muscle biomarkers. Dystrophin-deficient mdx mice were treated with a PPMO (Pip9b2-PMO) designed to induce Dmd exon 23 skipping at single, ascending intravenous doses (3, 6, or 12 mg/kg) and sacrificed 2 weeks later. Dose-dependent exon skipping and dystrophin protein restoration were observed, with dystrophin uniformly distributed at the sarcolemma of corrected myofibers at all doses. Serum microRNA biomarkers (i.e., miR-1a-3p, miR-133a-3p, miR-206-3p, miR-483-3p) and creatinine kinase levels were restored toward wild-type levels after treatment in a dose-dependent manner. All biomarkers were strongly anti-correlated with both exon skipping level and dystrophin expression. Dystrophin rescue was also strongly positively correlated with muscle stiffness (i.e., Young’s modulus) as determined by atomic force microscopy (AFM) nanoindentation assay. These data demonstrate that PPMO-mediated exon skipping generates myofibers with uniform dystrophin expression and that both serum microRNA biomarkers and muscle AFM have potential utility as pharmacodynamic biomarkers of dystrophin restoration therapy in DMD.

Profiles of anemia in adolescent students with sports club membership in an outpatient clinic setting: a retrospective study.

BackgroundAnemia is a common health issue among adolescents. Anemic conditions could affect physical performance; however, the actual profiles of anemia in adolescent students in sports clubs have not been well documented.MethodsWe conducted a retrospective chart review of individuals aged 13–22 years who belonged to sports clubs in schools and visited an outpatient clinic between August 1, 2016, and August 31, 2020. The medical and laboratory records, including serum levels of ferritin, folate, vitamin B12, and creatinine kinase at their first visit were assessed.ResultsA total of 485 individuals (231 male (48%) and 254 female (52%) patients) were eligible for the study. The most common club activity was track and field (n = 171 (35%)). The overall prevalence of the World Health Organization-defined anemia was 16.5% (95% CI [13.1–20.4]; 9.0% [5.4–13.8] and 23.1% [17.8–29.2] in males and females, respectively) after excluding pre-treated individuals. Hypoferritinemia and elevation of serum creatinine kinase levels were identified as independent contributors to anemia in both sexes (odds ratios: 13.2 (95% CI [4.2–41.1]), p < 0.001 and 14.7 (95% CI [1.8–118.4]), p = 0.012, respectively for males; odds ratios: 6.6 (95% CI [1.3–13.9]), p < 0.001 and 2.7 (95% CI [1.4–5.5]), p = 0.004, respectively for females).DiscussionAnemia is prevalent in both male and female adolescent students in sports clubs. Iron deficiency and excessive training indicated by elevated creatinine kinase levels may contribute to the risk of anemia. Physicians should assess the amount of exercise, and not merely iron storage, in clinical practice.

Analysis of neurology consultations in hospitalized patients with COVID-19.

ObjectiveThe aim of this study was to evaluate patients who were hospitalized with a diagnosis of COVID-19 and were consulted by neurology during their hospital stay.MethodsAll files of patients with COVID-19 who were admitted to Cerrahpasa Medical Faculty Hospital between March 11th and December 31st, 2020 were retrospectively reviewed, and files of patients who consulted by neurology during their stay were included. Demographic and clinical characteristics, neurologic diagnosis, outcome and related laboratory data were extracted from electronic medical records and analyzed. Patients were categorized into the first wave and second wave according to the date of hospitalization.ResultsA total of 2257 patients were hospitalized for COVID-19; among them, 127 were consulted by a neurologist during their hospital stay. Fifteen patients received a consultation for possible drug interactions. Among the remaining 112 patients, the reason for neurology consultation was i. exacerbation of a neurological comorbidity vs ii. new-onset neurological manifestations. The median age was 68.5 ± 14.2 years, and 60.7% were men. Dementia and stroke were the leading neurological comorbidities. COVID-19 disease was more severe in the patients with the new-onset neurological comorbidity than in patients with exacerbation of a neurological comorbidity (p = 0.07). Serum creatinine kinase levels were higher in the new-onset patient group (p < 0.05). Exacerbation of previous neurological disease or new neurological impairment were jointly and severely related to high mortality (overall 35/112 vs 275/2145, p < 0.001; exacerbation 12/45 vs 275/2145 p < 0.01; new-onset 23/67 vs 275/2145, p < 0.001).ConclusionSerious neurological involvement is relatively uncommon in hospitalized patients with COVID-19 and is associated with increased mortality.

Objective predictors of delayed neurological sequelae in patients with altered mental status after carbon monoxide poisoning

Background and Purpose: Following carbon monoxide (CO) poisoning, altered mental status is an important predictor of poor neurological prognosis, including delayed neurological sequelae (DNS). However, it is difficult to interview CO-poisoned patients accurately about exposure intervals and loss of consciousness (LOC). Thus, we investigated whether DNS can be predicted using objective factors such as laboratory results and brain imaging in patients suffering CO poisoning with altered mental status. Methods: This was a prospective observational study involving all CO-poisoned patients who visited the university hospital emergency department (ED) in Bucheon, South Korea, between January 2019 and April 2020. All were registered in the CO registry. We excluded patients who were under 18 years of age, had no change in mental status, were lost to follow-up, had neurological deficits persisting at discharge from the ED, and/or were transferred from another hospital 24 hours after exposure. Results: A total of 21 (25.3%) of 82 patients had DNS with a median onset of 21 (12 to 30) days. Creatinine kinase (CK) (odds ratio 1.0002, 95% confidence interval 2.734-105.231) and brain imaging (odds ratio 3.206, 95% confidence interval 1.008-10.199) were independent prognostic factors of DNS. Conclusions: A high level of serum CK and abnormal brain-imaging results were significant predictors of the occurrence of DNS in CO-poisoned patients with altered mental status. Critically, these are objective rather than subjective factors such as CO exposure interval.

Freeman-Sheldon Syndrome with Stiff Knee Gait - A Case Report.

Introduction:Freeman-Sheldon syndrome (FSS), also known as the distal arthrogryposis (DA) type 2A, is a rare congenital anomaly. We report a unique case of the DA type 2A with mixed clinical features and the unusual presentation of bilateral congenital dislocation of the knee but had unassisted stiff knee gait.Case Report:A 5-year-old female child presented to the clinic with the complaint of inability to bend both knees since birth. She had an unassisted bipedal gait, but could not squat, cross-leg sit, run, and climb stairs without assistance. Her youngest brother had a similar presentation but succumbed to death at the age of 5 months due to respiratory distress. Clinical features were in the favor of FSS. Her serum creatinine kinase level was normal and the electromyography of bilateral tibialis anterior and abductor pollicis brevis was not suggestive of the myotonia. Radiograph of the skull showed cooper beaten skull appearance whereas bilateral pelvis with the hip showed following changes in the right hip; decrease femoral epiphysis height, horizontal proximal femoral physis, and the coxa brevia. She was initially managed conservatively by weekly stretching, manipulation, and casting. As a result, she could flex her knee up to 20°. Although the quadricepsplasty might be helpful for the persistent extension deformity, there was marked quadriceps weakness which could make it harder for the child to stand and walk. In addition, the abnormal muscle physiology in FSS may result in unfavorable outcomes after the surgery. Moreover, a consideration of the surgical aspect is not free of risks which include difficult endotracheal intubation, vein access, and malignant hyperthermia.Conclusion:FSS is a rare congenital anomaly that should be differentiated from another syndrome of the close resemblance, Sheldon Hall syndrome and Schwartz Jampel syndrome which are other rare autosomal recessive disorders characterized by myotonia and the chondrodysplasia. Conservative management has still a role in bilateral knee involvement especially if the patient is an independent walker.

The Effect of Angiotensin-Converting Enzyme Inhibitor and Angiotensin Receptor Blocker on the Outcome of Hospitalization of Patients With Hypertension and COVID-19

Abstract Background To study the effect of angiotensin-converting enzyme inhibitor (ACEI) and angiotensin receptor blocker (ARB) on the outcome of hospitalization in patients with hypertension and novel coronavirus disease 2019 (COVID-19). Methods A retrospective analysis was performed in 202 COVID-19 patients who were hospitalized in Thunder-God Hospital, Wuhan from 12 February 2020 to 30 March 2020. According to whether taking ACEI or ARB, 67 (33.0%) patients with hypertension were divided into 3 groups: A, patients received ACEI or ARB after admission (n = 22); B, patients received ACEI or ARB before admission but interrupted after admission (n = 24); and C, patients were not treated with ACEI or ARB before or after admission (n = 21). Changes of therapeutic indicators in all groups of patients and their application relationship with ACEI/ARB were compared and analyzed. Results There were no significant differences in age, gender, blood pressure, underlying disease severity, or serum biochemical indicators (ALT, LDH, creatinine, and creatine kinase levels) at admission among 3 groups (all P &amp;gt; 0.05). During hospitalization, there were no significant differences in COVID-19-related treatment, oxygen use, hospital mortality, recovery and discharge rate, or days of throat swab nucleic acid turning negative among 3 groups (all P &amp;gt; 0.05). The proportion of calcium channel blocker in groups B and C was higher than group A (95.8% and 85.7% vs. 40.9%, P &amp;lt; 0.01), but there was no significant difference in blood pressure or compliance rates at discharge (P &amp;gt; 0.05). Group A, B, and C patients were hospitalized for 27.4 ± 6.4, 30.0 ± 8.8, and 30.1 ± 9.3 days, respectively (all P &amp;gt; 0.05). Compared with admission values, there were no significant differences in serum ALT, LDH, creatinine, or creatine kinase levels among all 3 groups during hospitalization (all P &amp;gt; 0.05). Conclusions ACEI or ARB has no significant effect on the outcome of hospitalization in patients with hypertension and COVID-19.

A clinically relevant combination treatment with doxorubicin and cyclophosphamide does not induce hepatotoxicity in C57BL/6J mice

Background and aimsChronic exposure to chemotherapeutics can lead to severe adverse events including hepatotoxicity. A combination chemotherapy regimen of doxorubicin (DOX) and cyclophosphamide (CPS) is employed in treatment of several cancers such as leukemia, lymphoma, and breast cancer. It is not well understood whether a combination therapy with DOX and CPS can induce hepatotoxicity. We therefore sought to determine whether co-administration of DOX and CPS at their clinically relevant doses and frequency results in hepatotoxicity. MethodsMale C57BL/6J mice received one intraperitoneal injection of saline or DOX-2 mg/kg and CPS-50 mg/kg once a week for 4 weeks. After the treatment period, liver histology and various serum biomarkers of hepatotoxicity were assessed. ResultsCo-treatment with DOX and CPS did not alter the serum levels of alanine aminotransferase (ALT), alkaline phosphatase (ALP), bilirubin, albumin, globulin, or total protein. Similarly, co-administration of DOX and CPS did not result in a noticeable change in liver histology. However, it was notable that the concomitant treatment with DOX and CPS resulted in a significant increase in serum levels of aspartate aminotransferase (AST). Elevated serum AST levels were also associated with increased serum creatinine kinase (CK) levels, suggesting that the elevated serum AST levels are likely due to muscle injury following the co-administration of DOX and CPS. ConclusionsTaken together, our results, for the first time, suggest that co-administration of DOX and CPS, at their clinically relevant doses and frequency does not induce a significant hepatotoxicity in the mice.

A clinically relevant combination treatment of doxorubicin and cyclophosphamide does not induce hepatotoxicity in C57BL/6J mice

Chronic exposure to chemotherapeutics can lead to severe adverse events including hepatotoxicity. A combination chemotherapy regimen of doxorubicin (DOX) and cyclophosphamide (CPS) is employed in treatment of several cancers such as leukemia, lymphoma, and breast cancer. It is currently not well understood whether a combination therapy of DOX and CPS can induce hepatotoxicity. We therefore sought to determine whether co-administration of DOX and CPS at their clinically relevant doses and frequency results in hepatotoxicity. Male C57BL/6J mice received one intraperitoneal injection of saline or DOX-2mg /kg and CPS-50mg/kg once a week for 4 weeks. After the treatment period, liver histology and various serum biomarkers of hepatotoxicity were assessed. Co-treatment of DOX and CPS did not alter the serum levels of alanine aminotransferase (ALT), alkaline phosphatase (ALP), bilirubin, albumin, globulin, or total protein. Similarly, co-administration of DOX and CPS did not result in a noticeable change in liver histology. However, it was notable that the concomitant treatment with DOX and CPS resulted in a significant increase in serum levels of aspartate aminotransferase (AST). Elevated serum AST levels were also associated with increased serum creatinine kinase (CK) levels, suggesting that the elevated serum AST levels are likely due to muscle injury following the co-administration of DOX and CPS. Taken together, our results, for the first time, suggest that co-administration of DOX and CPS, at their clinically relevant doses and frequency, does not induce a significant hepatotoxicity in the mice.

Health-related quality of life and work impairment in idiopathic inflammatory myopathies in South Australia.

The idiopathic inflammatory myopathies (IIM) are rare autoimmune diseases that are usually chronic and often present with skeletal muscle inflammation and weakness. We sought to examine the impact of IIM in a cohort of 50 South Australian patients on health-related quality of life (HRQOL) and work productivity (WP). We uniquely categorized patients across gender, IIM subtypes, employment status, and also whether there was extramuscular involvement from IIM. Multiple modalities were used, as recommended by the International Myositis Assessment and Clinical Studies Group (IMACS), to assess the impact of IIM, including manual muscle strength testing (MMT-8), the Physician and Patient Global Activity Assessments (PHGAA, PTGAA), Myositis Disease Activity Assessment Tool (MDAAT), and serum creatinine kinase (CK) levels. The impacts of IIM on HRQOL and WP were analyzed using the Medical Outcomes Study 36-items Short Form (SF-36) and Work Productivity and Activity Impairment (WPAI) questionnaires, respectively. We found significantly lower HRQOL outcome scores in most of the SF-36 domains when compared to the most recent population norms (P≤.01). Physical health was predominantly affected with relative preservation of emotional health. There were also significant associations between MMT-8, PHGAA and PTGAA scores and HRQOL and WP. Our findings highlight the significant impact of IIM on HRQOL and WP in a well-characterized cohort of patients with IIM within Australia, and therefore the importance of a holistic approach to the management of these patients.