BACKGROUND Mitochondrial myopathies are characterized by primary dysfunction of the mitochondrial respiratory chain;they typically present as chronic muscle weak-ness.Clinically visible acute respiratory dysfunctions ...BACKGROUND Mitochondrial myopathies are characterized by primary dysfunction of the mitochondrial respiratory chain;they typically present as chronic muscle weak-ness.Clinically visible acute respiratory dysfunctions associated with mito-chondrial myopathies occur rarely.CASE SUMMARY In this report,we present the case of a patient who developed postoperative hypoventilation after undergoing an uneventful administration of general anesthesia.A 34-year-old woman with no family history of myopathy underwent laparoscopic removal of a right-sided ureteric stone.Two days postoperatively,her oxygen saturation decreased rapidly,and blood gas analysis revealed hypercapnia.We promptly intubated and initiated the patient and initiated her on mechanical ventilation as she remained awake.Clinical examination findings were unremarkable;the results of laboratory investigations,including those for thyroid,hepatic,renal,and neuromuscular functions,were within normal limits.Muscle biopsy revealed muscle fibers of varying sizes as well as several dege-nerating and regenerating myofibers.Modified Gomori trichrome staining of the cross-sections revealed ragged red fibers.Based on these findings,we diagnosed the patient with mitochondrial myopathy.The patient’s condition gradually improved,and she was discharged on a home ventilator 73 days postoperatively.CONCLUSION Our case highlights that mitochondrial myopathy should be considered in the differential diagnosis of patients with postoperative respiratory failure.展开更多
Background Kawasaki disease (KD) is known as systemic vasculitis, and more than half of the patients with KD have myo-carditis, which can induce ventricular dysfunction. In this study, we evaluate left ventricular (LV...Background Kawasaki disease (KD) is known as systemic vasculitis, and more than half of the patients with KD have myo-carditis, which can induce ventricular dysfunction. In this study, we evaluate left ventricular (LV) dysfunction in patients with KD based on the myocardial performance index (MPI) using pulse Doppler (PD) and tissue Doppler imaging (TDI), from the acute to convalescent phases. Methods We retrospectively studied 89 children diagnosed with KD from January 2010 to August 2012. We assessed the presence of coronary artery lesions (CALs) and the LV ejection fraction, PD-MPI, and TDI-MPI at diagnosis, and 2, 14, and 56 days after intravenous immunoglobulin (IVIG) treatment. We enrolled 70 healthy children as a control group. Results The ejection fraction in patients with KD at diagnosis (67.3 ± 0.9%) was lower than that in the control group (69.8 ± 0.8%,P = 0.035), and the LV TDI-MPIs for patients with KD at diagnosis (0.49 ± 0.01) and 2 days after IVIG treatment (0.48 ± 0.01) were higher than those in the control group (0.45 ± 0.01,P = 0.002,P = 0.033, respectively). No signifi cant diff erences were found in the LV dysfunction between the patients with complete and incomplete KD. Septal TDI-MPIs in patients with KD with CAL at diagnosis (0.52 ± 0.02) were higher than those in patients with KD without CAL (0.47 ± 0.01,P = 0.019). Conclusions Transient LV dysfunction occurred in patients with complete and incomplete KD in the acute stage. In patients with KD with CAL at diagnosis, the LV dysfunction was more prominent. The PD-MPI and TDI-MPI are useful parameters for assessing LV function in patients with KD.展开更多
文摘BACKGROUND Mitochondrial myopathies are characterized by primary dysfunction of the mitochondrial respiratory chain;they typically present as chronic muscle weak-ness.Clinically visible acute respiratory dysfunctions associated with mito-chondrial myopathies occur rarely.CASE SUMMARY In this report,we present the case of a patient who developed postoperative hypoventilation after undergoing an uneventful administration of general anesthesia.A 34-year-old woman with no family history of myopathy underwent laparoscopic removal of a right-sided ureteric stone.Two days postoperatively,her oxygen saturation decreased rapidly,and blood gas analysis revealed hypercapnia.We promptly intubated and initiated the patient and initiated her on mechanical ventilation as she remained awake.Clinical examination findings were unremarkable;the results of laboratory investigations,including those for thyroid,hepatic,renal,and neuromuscular functions,were within normal limits.Muscle biopsy revealed muscle fibers of varying sizes as well as several dege-nerating and regenerating myofibers.Modified Gomori trichrome staining of the cross-sections revealed ragged red fibers.Based on these findings,we diagnosed the patient with mitochondrial myopathy.The patient’s condition gradually improved,and she was discharged on a home ventilator 73 days postoperatively.CONCLUSION Our case highlights that mitochondrial myopathy should be considered in the differential diagnosis of patients with postoperative respiratory failure.
文摘Background Kawasaki disease (KD) is known as systemic vasculitis, and more than half of the patients with KD have myo-carditis, which can induce ventricular dysfunction. In this study, we evaluate left ventricular (LV) dysfunction in patients with KD based on the myocardial performance index (MPI) using pulse Doppler (PD) and tissue Doppler imaging (TDI), from the acute to convalescent phases. Methods We retrospectively studied 89 children diagnosed with KD from January 2010 to August 2012. We assessed the presence of coronary artery lesions (CALs) and the LV ejection fraction, PD-MPI, and TDI-MPI at diagnosis, and 2, 14, and 56 days after intravenous immunoglobulin (IVIG) treatment. We enrolled 70 healthy children as a control group. Results The ejection fraction in patients with KD at diagnosis (67.3 ± 0.9%) was lower than that in the control group (69.8 ± 0.8%,P = 0.035), and the LV TDI-MPIs for patients with KD at diagnosis (0.49 ± 0.01) and 2 days after IVIG treatment (0.48 ± 0.01) were higher than those in the control group (0.45 ± 0.01,P = 0.002,P = 0.033, respectively). No signifi cant diff erences were found in the LV dysfunction between the patients with complete and incomplete KD. Septal TDI-MPIs in patients with KD with CAL at diagnosis (0.52 ± 0.02) were higher than those in patients with KD without CAL (0.47 ± 0.01,P = 0.019). Conclusions Transient LV dysfunction occurred in patients with complete and incomplete KD in the acute stage. In patients with KD with CAL at diagnosis, the LV dysfunction was more prominent. The PD-MPI and TDI-MPI are useful parameters for assessing LV function in patients with KD.
