With the development of critical medical emergency technology, the success rate of sepsis treatment has been significantly improved, and the improvement of the long-term quality of life of sepsis survivors has also attracted more and more attention. ICU-acquired weakness (ICU-AW) refers to a group of syndromes with systemic and symmetrical muscle weakness during the intensive care unit (ICU) hospitalization and cannot be explained by the patient's own disease, which often involve diaphragm and skeletal muscle, resulting in difficulty in weaning and nosocomial infection. The incidence of ICU-AW in sepsis patients is over 50%, making it an important factor affecting the prognosis of these patients. The occurrence of sepsis ICU-AW is related to many factors, which can be summarized into two categories, including sepsis-related factors such as sepsis-associated inflammatory response, sepsis-associated encephalopathy (SAE), and treatment-related factors such as physical immobilization and insufficient nutritional support. The current ICU-AW risk assessment tools are mainly on subjective assessment scales, but there are some limitations in clinical application, and objective assessment tools including predictive model and imaging assessment, which are still in the research stage. "ABCDEF bundle strategy" is an important measure to prevent ICU-AW, in which early rehabilitation is the core element. This review of the literature from the risk factors, risk assessment and early rehabilitation of ICU-AW, and focuses on the timing, content, method and safety assessment of early rehabilitation, aims to improve the understanding of ICU-AW, strengthen the prevention of sepsis with ICU-AW, and improve the prognosis of sepsis patients, not only survive, but also live better.
Humans ; Sepsis/complications* ; Muscle Weakness/etiology* ; Intensive Care Units ; Prognosis ; Quality of Life

Sepsis-acquired weakness (SAW) is a common complication in critically ill patients, yet significant gaps remain in both mechanistic understanding and therapeutic interventions for this condition. SAW not only prolongs the duration of mechanical ventilation and hospitalization but is also closely associated with increased mortality. Even if these SAW patients survive, they often experience long-term physical dysfunction after hospital discharge, leading to diminished quality of life. Emerging evidence suggests that sustained mitochondrial dysfunction may constitute a pivotal pathophysiological basis for the development and progression of SAW, primarily encompassing five key aspects: dysregulated mitochondrial quality control (MtQC), impaired oxidative phosphorylation (OXPHOS), exacerbated oxidative stress, disrupted Ca²⁺; homeostasis, and their mediation of diverse myofiber injuries. This article systematically elucidates the central role of mitochondrial dysfunction in the pathogenesis of SAW. Furthermore, we explore potential therapeutic strategies targeting mitochondrial function, including mitigating mitochondrial oxidative stress, optimizing nutritional support, and supplementing with muscle-derived mesenchymal stem cells. These insights provide a critical theoretical framework for understanding SAW mechanisms and developing clinical interventions, with particular emphasis on the translational value of mitochondrial-targeted therapies in improving outcomes for septic patients.
Humans ; Sepsis/metabolism* ; Mitochondria/metabolism* ; Muscle Weakness/etiology* ; Oxidative Stress ; Oxidative Phosphorylation

Intensive care unit acquired muscle weakness (ICU-AW) is a neuromuscular complication secondary to severe illness. The essence for this disease is skeletal muscle dysfunction. With the development of medical technology, the survival rate for severe patients has been significantly improved. The long term complications for the severe patients with ICU-AW are getting more and more common, and they seriously affect the quality of life and prognosis of patients. However, the current treatment is ineffective. Establishment of ICU-AW animal model is an important way to study the pathogenesis and intervention targets for this disease. There are many risk factors for this disease, and the principles for ICU-AW animal models are not the same at home and abroad, and the methods of preparation are different. The choice of a reasonable animal model is important for the reliability of the results.
Animals ; Critical Illness ; Disease Models, Animal ; Humans ; Intensive Care Units ; Muscle Weakness ; etiology ; mortality ; Muscle, Skeletal ; physiopathology ; Prognosis ; Quality of Life ; Reproducibility of Results ; Survival Rate

A boy aged 11 years was admitted due to intermittent weakness and difficulty in walking for 6 years, and hepatomegaly, glycopenia and unconsciousness for 4 years. The laboratory examinations showed severe metabolic acidosis, hypoglycemia, and abnormal liver function. CT scan showed marked liver enlargement with fat density shadow. The boy was given fluid infusion, correction of acidosis, intravenous injection of glucose, L-carnitine, compound vitamin B, and coenzyme Q10, but he was in a persistent coma and it was difficult to correct refractory metabolic acidosis and hypoglycemia. The boy died. Blood and urinary organic acid screening and gene detection confirmed that the boy had late-onset glutaric aciduria type II (GAIIc) caused by electron-transferring-flavoprotein dehydrogenase (ETFDH) gene defect. GAIIc is an inherited metabolic disease with a low incidence, resulting in a high misdiagnosis rate. GAIIc should be considered for children with recurrent weakness or reduced activity endurance, hypoglycemia, and marked liver enlargement with abnormal liver function. Urinary organic acid analysis and blood tandem mass spectrometry can help with the early diagnosis of GAIIc, and ETFDH gene analysis helps to make a confirmed diagnosis.
Child ; Hepatomegaly ; etiology ; Humans ; Hypoglycemia ; etiology ; Male ; Multiple Acyl Coenzyme A Dehydrogenase Deficiency ; diagnosis ; Muscle Weakness ; etiology

Abducens Nerve Diseases ; etiology ; Brain ; diagnostic imaging ; Cognitive Dysfunction ; etiology ; Epilepsies, Partial ; etiology ; Humans ; Magnetic Resonance Imaging ; Male ; Middle Aged ; Multiple Sclerosis, Chronic Progressive ; cerebrospinal fluid ; complications ; diagnostic imaging ; Muscle Weakness ; etiology ; Oligoclonal Bands ; cerebrospinal fluid ; Paresis ; etiology ; Paresthesia ; etiology ; Seizures ; etiology

OBJECTIVETo analyze clinical characteristics of a combination of dystrophinopathies and Klinefelter's syndrome (karyotype 47, XXY) in one patient.

METHODThe patient was diagnosed as Duchenne muscular dystrophy (DMD) and Klinefelter's syndrome in Beijing Children's Hospital in March, 2013. The clinical manifestations, physical examinations and laboratory test results were analyzed respectively. The clinical characteristics of four cases reported previously were analyzed as well.

RESULTThe 8.5 years old boy presented with symptoms of walking disorder and developmental delay. The patient had facial dysmorphism, waddling gait, Gower's manoeuvre and enlarged calves.Serum creatine kinase level was 21 040 U/L, and he had mild intellectual impairment. Deletions of exons 49-54 of the dystrophin gene were found.Gene dosage analysis revealed a heterozygous deletion in his mother. Five cases have been reported till now, their age ranged from 3.5 to 18 years; 3 of them were DMD, while the other 2 cases were Becker muscular dystrophy (BMD). One of them, detected in pedigree study, whose weakness was minimal in contrast to the proband. The others came to the hospital because of walking disorder or developmental delay. All the patients had enlarged calves, some of them also had Gower's manoeuvre and waddling gait. The patients' height was between 3 rd and 50 th percentile, while 2 of them had facial dysmorphism.Some degree of mental impairment is usual. Their serum creatine kinase were 2 469-24 750 U/L.One of them was detected in pedigree study. Three of them were diagnosed by muscle biopsy, while in the other one mutation analysis was used.

CONCLUSIONThe combination of dystrophinopathies and Klinefelter's syndrome is quite rare, and has clinical features of these two diseases. Mutation analysis (or muscle biopsy) and karyotype analysis can finally diagnose the syndrome.

Child ; Creatine Kinase ; blood ; DNA Mutational Analysis ; Dystrophin ; genetics ; metabolism ; Exons ; genetics ; Gene Deletion ; Heterozygote ; Humans ; Intellectual Disability ; Klinefelter Syndrome ; complications ; diagnosis ; genetics ; Male ; Muscle Weakness ; etiology ; Muscular Dystrophy, Duchenne ; complications ; diagnosis ; genetics ; Mutation ; Pedigree

A young woman with severe vitamin D deficiency presented with proximal muscle weakness, fragility fracture and pseudoarthrosis. On evaluation, she was found to have hypercalcaemia, a single parathyroid adenoma and an undetectable 25-hydroxyvitamin D level. She received parenteral cholecalciferol and subsequently underwent curative parathyroidectomy. Postoperatively, she had hungry bone syndrome, which she gradually recovered from with calcium and calcitriol replacement. Notably, her calcium levels were in the lower limit of normal range and associated with elevated alkaline phosphatase levels at postoperative Day 14. Follow-up for the next four years showed that the patient had remarkable symptomatic and radiological improvements. In this report, we discuss the pathophysiological interactions between vitamin D deficiency and associated primary hyperparathyroidism.
Adenoma ; diagnosis ; diagnostic imaging ; surgery ; Adult ; Female ; Follow-Up Studies ; Fractures, Spontaneous ; diagnostic imaging ; etiology ; surgery ; Humans ; Hyperparathyroidism, Primary ; complications ; diagnosis ; surgery ; Low Back Pain ; diagnosis ; etiology ; Muscle Weakness ; diagnosis ; etiology ; Parathyroid Neoplasms ; diagnosis ; diagnostic imaging ; surgery ; Parathyroidectomy ; methods ; Pseudarthrosis ; diagnostic imaging ; etiology ; physiopathology ; Radiography ; Severity of Illness Index ; Singapore ; Treatment Outcome ; Vitamin D Deficiency ; complications ; diagnosis

OBJECTIVETo evaluate the therapeutic efficacy of hemoperfusion in the treatment of intermediate myasthenia syndrome (IMS) following acute organophosphate poisoning (AOPP).

METHODSEighty cases of IMS following AOPP, who were admitted to the Emergency Department of our hospital from 2006 to 2011 and had complete clinical records, were divided into HP treatment group (n = 36) and non-HP (NHP) treatment group (n = 44). The therapeutic efficacy of HP was evaluated by comparing the clinical data of the two groups.

RESULTSThe HP treatment group showed significantly increased serum cholinesterase activity at 24h and 72 h after admission (P < 0.05), while the NHP treatment group showed significantly increased serum cholinesterase activity at 72 h after admission (P < 0.05). The serum cholinesterase activity in the HP treatment group was significantly higher than that in the NHP treatment group at 24 h after admission (P < 0.05). Compared with the NHP treatment group, the HP treatment group had significantly decreased total atropine dose, time of ventilatory assistance, length of ICU stay, recovery time from coma, incidence of pulmonary infection, and mortality due to respiratory failure (P < 0.05). There were no significant differences in the incidence of upper gastrointestinal hemorrhage and total mortality between the two groups (P > 0.05).

CONCLUSIONHemoperfusion is an effective therapy for improving clinical symptoms, shorten the course of disease, reducing complications, and decreasing the mortality due to respiratory failure in the patients with IMS following AOPP.

Cholinesterases ; blood ; Female ; Hemoperfusion ; Humans ; Male ; Muscle Weakness ; etiology ; therapy ; Organophosphate Poisoning ; therapy ; Syndrome ; Treatment Outcome

We investigated the persistence of viable Orientia tsutsugamushi in patients who had recovered from scrub typhus. Blood specimens were available from six patients with scrub typhus who were at 1 to 18 months after the onset of the illness. The EDTA-treated blood specimens were inoculated into ECV304 cells, and cultures were maintained for 7 months. Sequencing of the 56-kDa type-specific antigen gene of O. tsutsugamushi was performed to ascertain the homology of isolates. O. tsutsugamushi was isolated from all six patients, and nucleotide sequences of isolates serially collected from each patient were identical in all five patients in whom nucleotide sequences were compared. One patient relapsed 2 days after completion of antibiotic therapy; two patients complained of weakness for 1 to 2.5 months after the illness; one patient underwent coronary angioplasty 6 months later; and one patient suffered from a transient ischemic attack 8 months later. This finding suggests that O. tsutsugamushi causes chronic latent infection, which may be associated with certain clinical illnesses, preceded by scrub typhus. Antibiotic therapy abates the symptoms of scrub typhus, but does not eradicate O. tsutsugamushi from the human body.
Adult ; Aged ; Aged, 80 and over ; Antigens, Bacterial/genetics ; Bacterial Proteins/genetics ; Base Sequence ; Case-Control Studies ; Chronic Disease ; Coronary Artery Disease/etiology ; DNA, Bacterial/genetics/isolation & purification ; Female ; Genes, Bacterial ; Humans ; Ischemic Attack, Transient/etiology ; Male ; Membrane Proteins/genetics ; Middle Aged ; Muscle Weakness/etiology ; Orientia tsutsugamushi/genetics/immunology/*isolation & purification ; Recurrence ; Scrub Typhus/complications/drug therapy/*microbiology ; Time Factors

INTRODUCTIONThe number of cases of lead poisoning (LP), a widely known disease with various aetiologies, being reported globally has decreased over the years due to both limited domestic applications of lead and enforcement of stringent safety measures. However, a new presentation of lead poisoning, lead-contaminated opium (LCO), is gradually emerging in our region. This study aimed to determine the prevalence and clinical effects of lead toxicity associated with opium use.

METHODSBetween November 2006 and December 2007, all patients diagnosed with LP at a central laboratory in Tehran, Iran, were assessed for potential causes of poisoning. Patients with a history of LCO abuse were evaluated and recruited for the study.

RESULTSOverall, there were 240 patients with LP, and poisoning from LCO was diagnosed in 25 patients. The duration of addiction was between three months and 40 years, and the duration of symptoms was 28.1 ± 17.7 days. Mean blood lead levels of the patients were 145 ± 61 (range 61-323) μg/dL. The average creatinine and haemoglobin levels were 77.4 ± 8.1 μmol/L and 105 ± 25 g/L, respectively. The association between the duration of addiction and levels of lead in blood was not statistically significant (r = -0.142, p = 0.54). The most common symptoms were gastrointestinal complaints, followed by musculoskeletal complaints with muscle weakness (92%). Anorexia was also a leading complaint.

CONCLUSIONThe results of our study suggest that the possibility of LP should be considered with high suspicion among opium users presenting with acute abdominal symptoms.

Abdomen, Acute ; etiology ; Adult ; Creatinine ; blood ; Cross-Sectional Studies ; Female ; Hemoglobins ; biosynthesis ; Humans ; Iran ; Lead ; blood ; Lead Poisoning ; complications ; diagnosis ; etiology ; Male ; Middle Aged ; Muscle Weakness ; etiology ; Opioid-Related Disorders ; blood ; complications ; Opium ; administration & dosage ; Pain ; etiology ; Prevalence ; Time Factors