BackgroundSevere congenital neutropenia (SCN) is an immunodeficiency disease characterized low blood neutrophil counts, early bacterial infections, and risk of leukaemia development. Heterozygous mutations in the ELANE gene coding neutrophil elastase are associated with SCN. Patients with SCN suffer from recurrent bacterial infections and often succumb them. To our knowledge, this is the first report of SCN from Vietnam.Case presentationA 6-year-old boy was admitted due to severe bacterial infection and severe neutropenia. He had recurrent infections from 8 months of age, and was misdiagnosed with tuberculosis and and autoimmune neutropenia in infancy at 21 and 41 months of age, respectively. His medical report has showed severe neutropenia for many times. In direct DNA sequencing analysis, we found an ELANE gene mutation (R81P), which had been confirmed to cause SCN.ConclusionThe missed and delayed diagnosis may be attributable to insufficient awareness of this rare disease on the background of frequent infections even in the immunocompetent pediatric population in Vietnam. Our results indicate further evidence for the role of ELANE in SCN.
Background: Bungarus candidus is a common accident, leading to respiratory failure due to respiratory muscle paralysis. Artificial ventilation is an essential intervention to cure. Objective: Assess results of artificial ventilation in patients bitten by Bungarus candidus. Subjects and Methods: The ventilation method is volume control with 2 different Vt levels: Vt 8-10ml/kg with PEEP 5cmH2O versus Vt 12-15ml/kg with out PEEP. To describe figures of ventilation, complication and microbiology causes of pneumonia. Results: 64 patients were put on ventilation. Average time of artificial ventilation was 9.8±7.1 days. Complications were due to ventilator-associated pneumonia (59.4%), the most common is Acinetobater baumanii 45%, followed by P. aeruginosa, K. pneumoniae, S. and Candida albicans. Complete recovery is 94%, sequelae is 3% and mortality is 3%. Sequelae and mortality mainly is due to respiratory failure and complications associated with mechanical ventilation. Conclusion: High Vt 12-15ml/kg mode is less atalectasis than Vt 8-10ml/kg with PEEP 5cmH2O. Complications mainly related to ventilator-associated pneumonia, the most common etiology is A.baumanii. Key words: Bungarus candidus, mechanical ventilation, respiratory muscle paralysis, complications.
ventricular (RV) hypertrophy showed spontaneous contractile activity and asynchronous Ca 2+ release during inotropic interventions [1,2]. Our aim was to investigate whether these changes also occurred at the single cell level. Male Wistar rats were injected with 60 mg kg-1 of monocrotaline (MCT, n=3) or saline as control (CON, n=4). Four weeks post injection, hearts were enzymatically digested to yield isolated cardiomyocytes. RV myocytes were collected and measurements of cell shortening and intracellular Ca 2+ (fura-2AM, 340/380 ratio) were made in response to inotropic interventions. Application of 20mM caffeine revealed Ca 2+ content of the sarcoplasmic reticulum (SR). MCT myocytes had the largest cell area (43386292mm 2) versus CON (31706262mm 2 , p=0.01), although no difference in cell shortening was observed. MCT myocytes had larger Ca 2+ transients at 1Hz stimulation (p=0.004), with higher susceptibility to spontaneous activity during ß-adrenergic stimulation and low caffeine (1mM) application. There was no difference between groups in Ca 2+ store content, although the time constant of caffeine transient decay was prolonged in MCT (17.361.5s) versus CON (13.760.6s, p=0.05). Although there was no difference in shortening or SR Ca 2+ content, there was evidence of changes in Ca 2+ handling in MCT myocytes during inotropic stimulation. Furthermore, hypertrophic MCT myocytes exhibited slower trans-sarcolemmal Ca 2+ removal during 20mM caffeine, potentially as a result of disorganized T-tubular arrangement [2].
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