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Objective:To study the characteristics of clinical, laboratory, imaging, genetic and differential diagnosis of McLeod syndrome.Methods:The clinical characteristics of 2 cases of McLeod syndrome confirmed by gene detection in Qilu Hospital (Qingdao) on June 27, 2018 and in Qilu Hospital of Shandong University on September 11, 2019 were analyzed retrospectively. And the characteristics of patients of McLeod syndrome reported in China were analyzed in combination with literature review.Results:Both of the 2 patients were adult male, aged 57 and 61 years, respectively, with a slowly progressive course, beginning with gradually involuntary movement of trunk and extremities, involving involuntary biting of the tongue and dysphagia. Two patients had mild cognitive impairment; one patient had emotional agitation. Imaging study showed atrophy of caput nuclei caudate. Neuroelectrophysiological examination of case 1 showed sensory axon neuropathy in both upper limbs with severe damage to the left ulnar nerve. Creatine kinase (CK) was mildly elevated in 2 patients. The peripheral blood smear of 1 patient showed increased acanthocytes, accounting for 13%, the other patient showed no increased acanthocyte. McLeod syndrome related gene was tested in the 2 patients, case 1 with deletion mutation of exon 2 of XK gene, and case 2 with hemizygotic mutation of XK gene c.898delC p.L300 *. Conclusions:The clinical manifestations of McLeod syndrome are various and the differential diagnosis is crucial. For elderly male with cephalic facial chorea, elevated CK level and neuromuscular diseases, the possibility of McLeod syndrome should be screened.
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Objective To explore the learning curve characteristics of Mckeown-type minimally invasive esophagectomy and effects of the para-recurrent laryngeal nerve lymphadenectomy on efficacy.Methods The retrospective cohort study was conducted.The clinicopathological data of 163 patients with esophageal squamous cell carcinoma (ESCC) who underwent Mckeown-type minimally invasive esophagectomy in the Affiliated Tumor Hospital of Xinjiang Medical University between January 2011 and December 2015 were collected.According to the para-recurrent laryngeal nerve lymphadenectomy in the different learning curve stages (early,medium and later stages),49 patients who didn't undergo right para-recurrent laryngeal nerve lymphadenectomy were allocated into the group A,65 who underwent para-recurrent laryngeal nerve lymphadenectomy were allocated into the group B,and 49 underwent bilateral para-recurrent laryngeal nerve lymphadenectomy were allocated into the group C.Observation indicators:(1) comparisons of intra-and post-operative recovery among groups;(2) comparisons of follow-up and survival among groups;(3) correlation analysis between operation time or volume of intraoperative blood loss and cases of learning curve of Mckeown-type minimally invasive esophagectomy.Follow-up using outpatient examination and telephone interview was performed to detect postoperative survival up to April 2017.Measurement data with normal distribution were represented as-x±s.Comparison among groups was analyzed using the ANOVA,and pairwise comparison was done using the independent-sample t test.Measurement data with skewed distribution were described as M (range),and comparison of count data was done using the chi-square test.The survival time was calculated by the Kaplan-Meier method,and Log-rank test was used for survival analysis.Correlation analysis was done by Spearman rank correlation.Results (1) Comparisons of intra-and post-operative recovery among groups:patients in the 3 groups underwent successful Mckeown-type minimally invasive esophagectomy of ESCC,without conversion to open surgery.The operation time,total number of lymph node dissected,number of thoracic lymph node dissected and volume of intraoperative blood loss were respectively (395±94) minutes,14.7±6.9,9.6±5.4,(175± 100) mL in the group A and (329±67) minutes,20.4±9.1,11.4±7.3,(117±49) mL in the group B and (301±51)minutes,25.8±11.0,14.8±10.1,(115±50) mL in the group C,with statistically significant differences in above indicators among groups (F=21.962,1.992,5.775,12.744,P＜0.05),between group A and group B (t =3.135,3.741,4.324,4.375,P＜0.05) and between group A and group C (t=5.120,3.415,5.712,6.130,P＜0.05).There was no statistically significant difference in operation time and volume of intraoperative blood loss between group B and group C (t =2.325,2.459,P＞0.05).There were statistically significant differences in total number of lymph node dissected and number of thoracic lymph node dissected between group B and group C (t =2.751,3.245,P＜0.05).Cases with unilateral recurrent laryngeal nerve injury,anastomotic leakage and pneumonia were respectively 7,8,7 in the group A and 17,19,10 in the group B and 11,15,10 in the group C,with no statistically significant differences (x2 =0.968,3.292,0.773,P＞0.05).Number of lymph node dissected at right and left para-recurrent laryngeal nerve were respectively 0,0 in the group A and 1.9±1.8,0 in the group B and 2.6±2.1,1.1±0.8 in the group C.Of 35patients with unilateral recurrent laryngeal nerve were treated with symptomatic and supportive treatment of neuro nutrition,18 encountered permanent hoarseness and 17 recovered well.Patients with anastomotic fistula and pneumonia were improved by sufficient drainage and antibiotic therapy.(2) Comparisons of follow-up and survival among groups:149 of 163 patients were followed up for 17-65 months,with a median time of 32 months,including 43 in the group A,61 in the group B and 45 in the group C.Survival time of patients who received follow-up was recpectively (31.3±2.6) months,(32.2± i.6) months and (25.5±2.5) months in group A,B and C,with no statistically significant differences (x2=4.412,P＞0.05).(3) Correlation analysis between operation time or volume of intraoperative blood loss and cases of learning curve of Mckeown-type minimally invasive esophagectomy:results of correlation analysis showed that there was a significant negative correlation between operation time or volume of intraoperative blood loss and cases of learning curve of Mckeown-type minimally invasive esophagectomy (r=-0.632,-0.451,P＜0.05),showing a decreasing trend in operation time and volume of intraoperative blood loss with increasing surgical cases.Conclusions The operation time and volume of intraoperative blood loss are gradually declining with learning curve process of Mckeown-type minimally invasive esophagectomy.Para-recurrent laryngeal nerve lymphadenectomy cannot increase the incidence of recurrent laryngeal nerve injury,with more completely lymphadenectomy.
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Objective To explore the feasibility and safety of Mckeown-type minimally invasive esophagectomy in para-recurrent laryngeal nerve lymphadenectomy. Methods A total of 163 cases underwent minimally invasive McKeown resection for esophageal carcinoma in the Affiliated Tumor Hospital of Xinjiang Medical University were retrospectively analyzed. Patients were divided into routine treatment group (n=63), right recurrent nerve lymph node dissection group (right group, n=53) and bilateral recurrent laryngeal nerve lymph node dissection group (bilateral group, n=47) according to the operation modes. The postoperative pathology, operation time, intraoperative bleeding volume, postoperative hospitalization time, number of lymph nodes and pneumonia, anastomotic fistula, laryngeal nerve injury and other complications were compared between three groups of patients. Pathological conditions were consisted of the location of tumor, the degree of differentiation, T stage and pathological stage. Postoperative complications were followed up for 6 months. Results There were no significant differences in tumor location, pathological differentiation degree, T stage and pathologic stage between three groups. The amount of bleeding was more in the routine group than that of the right group and the bilateral group (P<0.05). There was no significant difference in the amount of bleeding between the right group and the bilateral group. The total lymph nodes and thoracic lymph nodes were increased in order in routine group, the right group and the bilateral group, and there was significant difference between three groups(P<0.05). There were no significant differences in other operation data and complications between three groups. Conclusion McKeown-type minimally invasive esophagectomy shows good feasibility and safety for para-recurrent laryngeal nerve lymphadenectomy .