OBJECTIVE: To observe the incidence of intensive care unit-acquired weakness (ICU-AW) of mechanically ventilated patients, and to identify the relevant risk factors. METHODS: A prospective cohort study was conducted. The patients admitted to intensive care unit (ICU) of Fuxing Hospital, Capital Medical University, aged 18 years old or older, with the duration of mechanical ventilation ≥ 24 hours and expected to stay in ICU for ≥ 7 days from May 2015 to January 2016 were enrolled. From the 7th day after ICU admission, the patients were evaluated for consciousness every day. If the patient was awake and could cooperate with muscle strength measurement, the day was recorded as T1, and the patient's muscle strength was measured using the Medical Research Council scale (MRC) and recorded, then all patients were divided into two groups according to MRC score, ICU-AW group (MRC score < 48) and non-ICU-AW group (MRC score ≥ 48). The death, transfer or the 28th day of ICU admission were regarded as the end of observation. The data from the first day of ICU admission to T1 (before T1), including metabolic factors (the lowest value of blood sodium, blood potassium, blood calcium, albumin, and the highest value of blood glucose), mechanical ventilation factors (mode and duration of mechanical ventilation), organ dysfunction factors [occurrence and duration of sepsis, multiple organ dysfunction syndrome (MODS)], and drug factors (whether the patients used aminoglycoside, sedative, muscle relaxant or glucocorticoids, etc., the time of these drugs usage and the cumulative dose) of the patients were observed, recorded and analyzed, as well as the data from T1 to the end of the observation period, including the duration of mechanical ventilation, incidence of ventilator associated pneumonia (VAP), 28-day mortality, the length of ICU stay, and the cost of ICU and hospitalization. The relevant factors with statistical significance in univariate analysis were enrolled in multivariate analysis, and Logistic regression equation was established to screen the independent risk factors that might lead to ICU-AW. RESULTS: 486 patients with mechanical ventilation were enrolled in this study, and 37 patients were enrolled according to the inclusion and exclusion criteria, including 15 patients with ICU-AW (with ICU-AW incidence of 40.5%) and 22 patients without ICU-AW. In the univariate analysis, ICU-AW group patients showed statistical differences in following factors as compared with the non-ICU-AW group: age, and the duration of invasive ventilation and the total duration of mechanical ventilation, braking time, sepsis, MODS and duration of them, the usage days and dosage of sedative and glucocorticoid before T1. The total duration of mechanical ventilation from T1 to the end of the observation period, total duration of mechanical ventilation during the observation period, and length of ICU stay of the ICU-AW group were significantly longer than those of the non-ICU-AW group [hours: 190 (110, 274) vs. 4 (0, 57), hours: 337 (237, 477) vs. 78 (43, 170), days: 20±7 vs. 14±7, all P < 0.05], the incidence of VAP, 28-day mortality and total hospitalization expenses were significantly higher than those of the non-ICU-AW group [26.7% (4/15) vs. 0% (0/22), 53.3% (8/15) vs. 9.1% (2/22), 10 thousands Yuan: 18.7±7.7 vs. 12.1±10.8, all P < 0.05]. Multivariate Logistic regression analysis showed that the total duration of mechanical ventilation between the patient awake and ICU admission [odds ratio (OR) = 1.03, 95% confidence interval (95%CI) was 1.01-1.05, P = 0.011] and the duration of MODS (OR = 1.79, 95%CI was 1.17-2.72, P = 0.007) were the independent risk factors for ICU-AW in mechanically ventilated patients. CONCLUSIONS ICU-AW is quite common in patients with mechanical ventilation and has a higher incidence. The risk factors associated with ICU-AW were the total duration of mechanical ventilation and duration of MODS.
Adult ; Humans ; Intensive Care Units ; Muscle Weakness/epidemiology* ; Prevalence ; Prospective Studies ; Respiration, Artificial ; Risk Factors

Acute Disease ; Adolescent ; Adult ; Aged ; Aged, 80 and over ; Child ; Child, Preschool ; Female ; Humans ; Logistic Models ; Male ; Middle Aged ; Muscle Weakness ; chemically induced ; epidemiology ; Organophosphate Poisoning ; Pesticides ; poisoning ; Respiratory Insufficiency ; chemically induced ; epidemiology ; Risk Factors

INTRODUCTIONDermatomyositis (DM) is a multisystem inflammatory disease with a strong association with malignancy. We aimed to describe a series of Asian patients with DM and identify any significant clinical factors associated with malignancy.

MATERIALS AND METHODSThis was a retrospective review of a multi-racial cohort of 69 Asian patients diagnosed with DM over an 11-year period from 1996 to 2006.

RESULTSMalignancy was detected in 15 out of 68 patients (22%), the most common of which was nasopharyngeal carcinoma (7 cases). Compared to the non-malignancy group, the malignancy-associated group was older and had more male patients. There were no statistically significant clinical, serological or laboratory factors associated with a higher risk of malignancy.

CONCLUSIONThis study highlights the importance of ongoing malignancy screening especially for nasopharyngeal carcinoma in Asian patients with DM.

Adolescent ; Adult ; Aged ; Aged, 80 and over ; Carcinoma ; Confidence Intervals ; Dermatomyositis ; complications ; epidemiology ; immunology ; pathology ; Female ; Humans ; Logistic Models ; Male ; Middle Aged ; Muscle Weakness ; Nasopharyngeal Neoplasms ; epidemiology ; immunology ; pathology ; Odds Ratio ; Paraneoplastic Syndromes ; complications ; epidemiology ; immunology ; pathology ; Retrospective Studies ; Risk Factors ; Singapore ; epidemiology ; Young Adult

OBJECTIVETo analyze and summarize the characteristics of glycogen storage disease type II (Pompe disease) patients according to the clinical description and prognosis.

METHODSeventeen Chinese patients diagnosed by acid alpha-glucosidase (GAA) enzyme activity test were reviewed. Clinical data tables were designed. Interviews were made via phone calls. Information was collected to reach the objective.

RESULTFour of 17 patients diagnosed by acid alpha-glucosidase are infantile-onset, symptoms started between 2 to 6 months after birth with increased serum creatine kinase and cardiac problems, with or without respiratory concerns. Other 13 patients were later-onset cases, and their symptoms started between 2 to 22 years of age with increased serum creatine kinase. Eleven later-onset patients started with muscle weakness, 2 patients developed respiratory insufficiency, 2 patients showed scoliosis, and 1 patient expressed increased serum creatine kinase with abnormal liver function. Just 3 of the later-onset patients were treated with mechanical ventilator and adjuvant therapy, others were not. All patients' acid alpha-glucosidase (GAA) enzyme activity analysis showed lower than 10% of normal. Fourteen patients were tested by muscle biopsy pathology, and 9 of them progressed to glycogen storage disease type II; 10 patients received genetic analysis, and 6 of them had two mutations which cause the disorder. Twelve of the 17 patients were interviewed successfully. In 3 of the infant-onset patients the disease resulted in death from respiratory failure, and 1 is still alive at the age of 1 year and 7 months. In 4 of 8 later-onset patients the disease resulted in death from respiratory failure between 3 to 5 years after onset of symptoms. Three of 4 survivors had increased muscle weakness, and 1 patient kept alive with ventilator without any changes. Seven of 12 interviewed patients died, the mortality rate was 58.3%.

CONCLUSIONGlycogen storage disease type II (Pompe disease) present differently in the clinic. Infant-onset Pompe disease is mainly characterized by generalized muscle weakness and obvious cardiac involvement. It's a dangerous disease, with high mortality rate. Later-onset Pompe disease is characterized by chronic proximal muscle weakness and respiratory insufficiency. GAA enzyme activity analysis, muscle biopsy and genetic analysis used to support the diagnosis of Pompe disease. Prognosis of the disease depends on age of onset and respiratory muscle involvement.

Adolescent ; Biopsy ; Cardiomyopathies ; epidemiology ; etiology ; Child ; Child, Preschool ; Clinical Enzyme Tests ; Creatine Kinase ; blood ; Female ; Follow-Up Studies ; Glucan 1,4-alpha-Glucosidase ; genetics ; metabolism ; Glycogen Storage Disease Type II ; diagnosis ; genetics ; pathology ; Humans ; Infant ; Male ; Muscle Weakness ; epidemiology ; etiology ; Prognosis ; Respiratory Insufficiency ; epidemiology ; Retrospective Studies ; Young Adult

OBJECTIVETo explore the clinical and laboratory features and the prognosis of juvenile dermatomyositis (JDM) complicated with interstitial lung disease (ILD).

METHODData of 39 cases of JDM complicated with ILD hospitalized in Beijing Children's Hospital from January 2005 to December 2011 were collected. The clinical features, laboratory data and prognosis of these children were analyzed.

RESULTOf the 39 cases studied, 16 were boys, and 23 girls. The average age of onset was 5.6 years, and 61.5% of the patients' age of onset (24 cases) was under 6 years. Rashes (17 cases, 43.6%), simultaneous eruption of rashes and muscle weakness (14 cases, 35.9%), fever (4 cases, 10.1%), or muscle weakness (3 cases, 7.7%) were common initial symptoms of the disease. Only 51.3% of the patients (20 cases) had the symptoms of respiratory system, but (24 cases) 61.5% were complicated with that of the gastrointestinal system; (27 cases) 69.2% had at the same time electrocardiographic and echocardiographic abnormalities. The chest high resolution computed tomography (HRCT) showed cord or band-like shadows in their lungs of more than half of the cases (25 cases, 64.1%), and other changes included ground glass-like shadow (10 cases, 25.6%), net and lineation-like shadow (9 cases, 23.1%), nodular change (5 cases, 12.8%). The patients complicated with lung essential infiltration accounted for as high as 71.8% (28 cases). These imaging changes were largely seen on both dorsal sides of their lungs. Severe patients also had mediastinal emphysema, pneumothorax, pneumorrhagia or aerodermectasia. Twenty-four patients underwent pulmonary function examination, and 62.5% of the patients' pulmonary function (15 cases) was abnormal. The fatality rate of the cases studied was 10.1%.

CONCLUSIONThe imaging changes of patients suffering from JDM with ILD were often more severe as compared to the clinical symptoms, and were often complicated with damages to other systems and organs. The prognosis of those patients was poorer than others. Patients with JDM especially at a younger age of onset and with various organ damages should be examined with chest HRCT examinations as early as possible.

Child ; Child, Preschool ; Dermatomyositis ; complications ; diagnosis ; drug therapy ; Female ; Glucocorticoids ; administration & dosage ; therapeutic use ; Humans ; Immunosuppressive Agents ; administration & dosage ; therapeutic use ; Lung ; diagnostic imaging ; pathology ; Lung Diseases, Interstitial ; diagnosis ; drug therapy ; etiology ; Male ; Methotrexate ; administration & dosage ; therapeutic use ; Muscle Weakness ; diagnosis ; epidemiology ; etiology ; Prognosis ; Respiratory Function Tests ; Retrospective Studies ; Tomography, X-Ray Computed