Objective To examine the correlation between the health-related quality of life measured by the St.George's Respiratory Questionnaire(SGRQ) and the commonly used physiological measures in lymphangioleiomyomatosis(...Objective To examine the correlation between the health-related quality of life measured by the St.George's Respiratory Questionnaire(SGRQ) and the commonly used physiological measures in lymphangioleiomyomatosis(LAM).Methods This study retrospectively analyzed the SGRQ scores and other measures(the Borg scale of breathlessness at rest,6-minute walking distance,blood oxygen levels,and pulmonary function) of patients diagnosed and confirmed with LAM.Altogether 38 patients between June 2007 and November 2009 were included.Results The mean values of the SGRQ three components(symptoms,activity,and impacts) and total scores in the LAM patients were 46.95±28.90,58.47±25.41,47.89±29.66,and 51.11±26.35,respectively.The SGRQ total or component scores were correlated well with the Borg scale of breathlessness,6-minute walking distance,partial pressure of oxygen in arterial blood,spirometry and diffusion capacity of lung.There were poor correlations between SGRQ score and residual volume or total lung capacity.In our preliminary observation,sirolimus improved the SGRQ total and three component scores and the Borg scale of breathlessness significantly after 101-200 days of treatment(n=6).Conclusions The SGRQ score in LAM is correlated well with physiological measures(Borg scale of breathlessness,6-minute walking distance,blood oxygen levels,and pulmonary function tests).The SGRQ could therefore be recommended in baseline and follow-up evaluation of patients with LAM.Treatment with sirolimus,an inhibitor of mammalian target of rapamycin,may improve the quality of life and patient's perception of breathlessness in LAM.展开更多
Objective BAG3-related myopathy is a rare condition so far reported in twenty patients worldwide.The purpose of this study was to draw attention to this rare disease and to the fact that BAG3-related myopathy should b...Objective BAG3-related myopathy is a rare condition so far reported in twenty patients worldwide.The purpose of this study was to draw attention to this rare disease and to the fact that BAG3-related myopathy should be considered as a rare differential diagnosis of hypercapnia.Methods We report a sporadic case of a 14-year-old Chinese girl with a de novo p.Pro209 Leu mutation in BAG3 and reviewed the literatures for reported cases related to this mutation.Results We described a 14-year-old Chinese girl who presented with gradually appearing symptoms of hypercapnia that required assisted ventilation.The muscle biopsy and the blood whole-exome sequencing results confirmed the diagnosis of myofibrillar myopathy with a de novo p.Pro209 Leu mutation in BAG3.Totally twentyone patients from twenty families with a confirmed diagnosis of BAG3-related myopathy were reported to date,including this patient and literature review.The male to female ratio was 11:10 and most showed initial symptoms in the first decade of life.Most patients presented toe/clumsy walking or running as the onset symptom,followed by muscle weakness or atrophy.Creatine kinase levels were elevated in fourteen patients and were normal in three.Eighteen patients developed respiratory insufficiency during the disease course and thirteen(one could not tolerate non-invasive assisted ventilation)required non-invasive assisted ventilation for treatment.Except for one not reported,heart involvement was found in seventeen patients during the disease course and seven underwent heart transplantation.Z-disk streaming and aggregation could be observed in most of the patients’muscle histology.In the long-term follow-up,five patients died of cardiac or respiratory failure.Conclusion BAG3-associated myopathy is a rare type of myofibrillar myopathy.It should be considered as a rare differential diagnosis of hypercapnia.展开更多
Although respiratory failure commonly occurs during the course of myasthenia gravis (MG),it is rarely described as the first presentation in patients with previously unrecognized MG.Here we reported one case of MG in ...Although respiratory failure commonly occurs during the course of myasthenia gravis (MG),it is rarely described as the first presentation in patients with previously unrecognized MG.Here we reported one case of MG in a 76-year-old man with the isolated respiratory failure as the first symptom.As illustrated by this case,it is important to consider neuromuscular disorders in cases of unexplained respiratory failure.展开更多
Objective:Robot-assisted radical prostatectomy(RARP)requires pneumoperitoneum(Pnp)and a steep head-down position that may disturb respiratory system compliance(Crs)during surgery.Our aim was to compare the effects of ...Objective:Robot-assisted radical prostatectomy(RARP)requires pneumoperitoneum(Pnp)and a steep head-down position that may disturb respiratory system compliance(Crs)during surgery.Our aim was to compare the effects of different degrees of neuromuscular block(NMB)on Crs with the same Pnp pressure during RARP.Methods:One hundred patients who underwent RARP were enrolled and randomly allocated to a deep or moderate NMB group with 50 patients in each group.Rocuronium was administered to both groups:in the moderate NMB group to maintain 1–2 responses to train-of-four(TOF)stimulation;and in the deep NMB group to maintain no response to TOF stimulation and 1–2 responses in the post-tetanic count.Pnp pressure in both groups was 10 mm Hg(1 mm Hg=133.3 Pa).Peak inspiratory pressure(Ppeak),mean pressure(Pmean),Crs,and airway resistance(Raw)were recorded after anesthesia induction and at 0,30,60,and 90 min of Pnp and post-Pnp.Surgical space conditions were evaluated after the procedure on a 4-point scale.Results:Immediately after the Pnp,Ppeak,Pmean,and Raw significantly increased,while Crs decreased and persisted during Pnp in both groups.The results did not significantly differ between the two groups at any of the time points.There was no difference in surgical space conditions between groups.Body movements occurred in 14 cases in the moderate NMB group and in one case in the deep NMB group,and all occurred during obturator lymphadenectomy.A significant difference between the two groups was observed.Conclusions:Under the same Pnp pressure in RARP,deep and moderate NMBs resulted in similar changes in Crs,and in other respiratory mechanics and surgical space conditions.However,deep NMB significantly reduced body movements during surgery.展开更多
基金Supported by National Basic Research Program of China (973 Program) (2009CB522106)
文摘Objective To examine the correlation between the health-related quality of life measured by the St.George's Respiratory Questionnaire(SGRQ) and the commonly used physiological measures in lymphangioleiomyomatosis(LAM).Methods This study retrospectively analyzed the SGRQ scores and other measures(the Borg scale of breathlessness at rest,6-minute walking distance,blood oxygen levels,and pulmonary function) of patients diagnosed and confirmed with LAM.Altogether 38 patients between June 2007 and November 2009 were included.Results The mean values of the SGRQ three components(symptoms,activity,and impacts) and total scores in the LAM patients were 46.95±28.90,58.47±25.41,47.89±29.66,and 51.11±26.35,respectively.The SGRQ total or component scores were correlated well with the Borg scale of breathlessness,6-minute walking distance,partial pressure of oxygen in arterial blood,spirometry and diffusion capacity of lung.There were poor correlations between SGRQ score and residual volume or total lung capacity.In our preliminary observation,sirolimus improved the SGRQ total and three component scores and the Borg scale of breathlessness significantly after 101-200 days of treatment(n=6).Conclusions The SGRQ score in LAM is correlated well with physiological measures(Borg scale of breathlessness,6-minute walking distance,blood oxygen levels,and pulmonary function tests).The SGRQ could therefore be recommended in baseline and follow-up evaluation of patients with LAM.Treatment with sirolimus,an inhibitor of mammalian target of rapamycin,may improve the quality of life and patient's perception of breathlessness in LAM.
基金supported by the Youth Program of National Natural Science Foundation of China(Grant No.82003309)the National Key Research and Development Program of China(Grant 2020YFB1313700)。
文摘Objective BAG3-related myopathy is a rare condition so far reported in twenty patients worldwide.The purpose of this study was to draw attention to this rare disease and to the fact that BAG3-related myopathy should be considered as a rare differential diagnosis of hypercapnia.Methods We report a sporadic case of a 14-year-old Chinese girl with a de novo p.Pro209 Leu mutation in BAG3 and reviewed the literatures for reported cases related to this mutation.Results We described a 14-year-old Chinese girl who presented with gradually appearing symptoms of hypercapnia that required assisted ventilation.The muscle biopsy and the blood whole-exome sequencing results confirmed the diagnosis of myofibrillar myopathy with a de novo p.Pro209 Leu mutation in BAG3.Totally twentyone patients from twenty families with a confirmed diagnosis of BAG3-related myopathy were reported to date,including this patient and literature review.The male to female ratio was 11:10 and most showed initial symptoms in the first decade of life.Most patients presented toe/clumsy walking or running as the onset symptom,followed by muscle weakness or atrophy.Creatine kinase levels were elevated in fourteen patients and were normal in three.Eighteen patients developed respiratory insufficiency during the disease course and thirteen(one could not tolerate non-invasive assisted ventilation)required non-invasive assisted ventilation for treatment.Except for one not reported,heart involvement was found in seventeen patients during the disease course and seven underwent heart transplantation.Z-disk streaming and aggregation could be observed in most of the patients’muscle histology.In the long-term follow-up,five patients died of cardiac or respiratory failure.Conclusion BAG3-associated myopathy is a rare type of myofibrillar myopathy.It should be considered as a rare differential diagnosis of hypercapnia.
文摘Although respiratory failure commonly occurs during the course of myasthenia gravis (MG),it is rarely described as the first presentation in patients with previously unrecognized MG.Here we reported one case of MG in a 76-year-old man with the isolated respiratory failure as the first symptom.As illustrated by this case,it is important to consider neuromuscular disorders in cases of unexplained respiratory failure.
基金Project supported by the Zhejiang Province Public Welfare Technology Application Research Project(No.LGF20H010006),China。
文摘Objective:Robot-assisted radical prostatectomy(RARP)requires pneumoperitoneum(Pnp)and a steep head-down position that may disturb respiratory system compliance(Crs)during surgery.Our aim was to compare the effects of different degrees of neuromuscular block(NMB)on Crs with the same Pnp pressure during RARP.Methods:One hundred patients who underwent RARP were enrolled and randomly allocated to a deep or moderate NMB group with 50 patients in each group.Rocuronium was administered to both groups:in the moderate NMB group to maintain 1–2 responses to train-of-four(TOF)stimulation;and in the deep NMB group to maintain no response to TOF stimulation and 1–2 responses in the post-tetanic count.Pnp pressure in both groups was 10 mm Hg(1 mm Hg=133.3 Pa).Peak inspiratory pressure(Ppeak),mean pressure(Pmean),Crs,and airway resistance(Raw)were recorded after anesthesia induction and at 0,30,60,and 90 min of Pnp and post-Pnp.Surgical space conditions were evaluated after the procedure on a 4-point scale.Results:Immediately after the Pnp,Ppeak,Pmean,and Raw significantly increased,while Crs decreased and persisted during Pnp in both groups.The results did not significantly differ between the two groups at any of the time points.There was no difference in surgical space conditions between groups.Body movements occurred in 14 cases in the moderate NMB group and in one case in the deep NMB group,and all occurred during obturator lymphadenectomy.A significant difference between the two groups was observed.Conclusions:Under the same Pnp pressure in RARP,deep and moderate NMBs resulted in similar changes in Crs,and in other respiratory mechanics and surgical space conditions.However,deep NMB significantly reduced body movements during surgery.
