Volume 100 ·  Issue 14 ·  2020

Porphyria is a kind of metabolic disease caused by the abnormal increase of the concentration of porphyrin or its precursors [such as delta-aminolevulinic acid (delta-ALA) and porphobilinogen (PBG)] due to the lack of enzyme activity in the heme biosynthesis pathway, which accumulates in tissues and causes cell damage. Porphyria is a rare disease, and the incidence of different types of porphyria varies. In adults, porphyria cutanea tarda (PCT), acute intermittent porphyria (AIP) and erythropoietic protoporphyria (EPP) are the most common. Symptomatic PCT prevalence was 40 per million (US)^[1]。 The prevalence rate of AIP in Europe is about 5.4/million, the annual incidence rate of symptomatic AIP is about 0.13/million, and the incidence rate of symptomatic AIP, porphyria variante (VP), and hereditary faecal porphyria (HCP) is 1.00:0.62:0.15^[2]。 EPP prevalence ranged from 5.0/million (UK) to 13.3/million (Netherlands)^[3]。 There is no epidemiological data in China. In order to standardize the diagnosis and treatment of porphyria in China, experts from the Red Blood Cell Disease (Anemia) Group of Hematology Branch of Chinese Medical Association discussed and formulated this consensus.

To evaluate the accuracy of STOP-Bang (SBQ) questionnaire in the screening of patients with obstructive sleep apnea (OSA).

The data were collected from September, 2011 to May, 2012 at the Sleep Medicine Center of West China Hospital, Sichuan University. A total of 350 consecutive patients (302 males and 48 females, 19-68 years old, average (42.9±11.0) years old) with suspected OSA who underwent an overnight polysomnography (PSG) were recruited into this study. SBQ questionnaire was completed. Based on the severity of OSA which were determined by apnea-hypopnea index (AHI), the patients were classified into four groups of primary snoring (<5 events/h), mild (5-14 events/h), moderate (15-29 events/h) and severe OSA (≥30 events/h). To evaluate the accuracy of SBQ, sensitivity, specificity, positive predictive values, negative predictive values and the area under the receiver operating characteristics curve of SBQ questionnaire were calculated.

Of the subjects, 322 (92.0%) were classified as high risk based on the questionnaire. With mild+moderate+severe OSA (AHI≥5 events/h), moderate+severe OSA (AHI≥15 events/h), and severe OSA as cutoffs, the sensitivity of SBQ questionnaire were 94.0%, 94.1%, and 95.3%; the specificity were 25.7%, 15.2%, and 13.2%; the positive predictive values were 91.9%, 79.2%, and 63.4%; the negative predictive values were 32.1%, 42.9%, and 64.3%, respectively. When variable BMI and age cutoffs (BMI≥24 kg/m² and age>42 years old), the questionnaire had the highest sensitivity (98.4%, 98.2%, and 99.5%, respectively) and the lowest specificity (8.6%, 3.8%, and 5.2%, respectively).

When BMI and/or age cutoff values gradually decline, the sensitivity of SBQ questionnaire are increased, but the specificity and positive predictive values are declined. Lowering BMI and/or age cutoffs possibly does not improve questionnaire accuracy.

To evaluate the diagnostic efficacy of Mini Nutritional Assessment Short-form (MNA-SF) in predicting acute exacerbation of old chronic obstructive pulmonary disease (COPD) patients.

The clinical data and Nutritional assessment of 202 outpatients who were given treatment in the Second Affiliated Hospital of Soochow University from January 2017 to May 2019 were analyzed. According to the frequency of acute exacerbation in the last year, patients were divided into high-risk group and low-risk group. The dependent variable was the risk of the acute exacerbation of COPD while the predicting model was established by using multivariate Logistic regression. Finally, the accuracy, sensitivity and specificity of the model was evaluated by the receiver operating characteristic (ROC) curve.

Among the 202 COPD patients, 131 patients (64.9%) were brought into the high-risk group and 71 patients (35.1%) were brought into the low-risk group. MNA-SF scores in high-risk group were significant lower than those in low-risk group [(9.4±2.1) vs (11.6±1.9), P<0.001]. The logistic regression analysis showed that MNA-SF score [OR=0.556(95%CI: 0.445-0.695), P<0.05] was an independent factor of acute exacerbation. The obtained model was Logit(P)=4.413-0.586×MNA-SF scores. The accuracy of model for the risk of the acute exacerbation of COPD was 77.4%, with a sensitivity of 79.7%, a specificity of 72.1%.

MNA-SF is qualified for predicting the acute exacerbation of COPD patients in stable stage.

To determine the status of bone metastasis (BM) and prognosis factors of patients with renal cell carcinoma (RCC) in our center.

The clinical and medical records of RCC patients with BM, who were admitted to the Department of Urology, Bone Oncology and Spine Surgery, Beijing Jishuitan Hospital from August 2009 to August 2017 were collected. The gender, age, time of BM, location of BM, numbers of BM, presence or absence of visceral metastasis, pathological types of BM were investigated. The patients were followed up regularly, and the survival curves were analyzed by Kaplan-Meier method. Cox proportional hazard regression model was used to estimate the prognostic factors.

A total of 51 RCC patients with bone metastasis were collected. The age of patients ranged from 38 to 76 (58.6±8.2) years old, including 39 males (76.5%) and 12 females (23.5%). The ratio of male to female was 3.25∶1. The patients were followed up for 8 to 109 months, with a median follow-up time of 30 months. The follow-up rate was 90.2%. Thirty-one (60.8%) patients died at the last follow-up, with a median overall survival (OS) time of 25 months. The median OS was 38 months and 20 months in the solitary BM group (26 cases, 51.0%) and BM ≥ 2 group (25 cases, 49.0%), respectively. The difference between the two groups was statistically significant (P=0.021). The median OS was 30 months, 69 months and 17 months in the axis BM group (22 cases, 43.1%), appendicular BM group (19 cases, 37.3%) and both the axis and appendicular BM group (10 cases, 19.6%), respectively. The difference between the groups was statistically significant (P=0.012). The median OS was 22 months and 38 months in the patients with (15 cases, 29.4%) and without (36 cases, 70.6%) visceral metastases groups, respectively. The difference between the two groups was statistically significant (P=0.007). Univariate and multivariate Cox regression analysis showed that the numbers of BM (HR=3.130, 95%CI: 1.502-6.520, P=0.035) and visceral metastasis (HR=4.699, 95%CI: 1.810-9.545, P=0.001) were independent prognostic factors for RCC with BM.

Solitary BM, no visceral metastasis are good prognostic factors for RCC with BM. For these patients, radical resection of BM is feasible to improve survival rate.

To analyze the clinical pathological characteristics and incidence of thyroid cancer.

The clinical and pathological data of 21 980 thyroid cancer patients who underwent surgery in the Department of Thyroid Surgery of the First Affiliated Hospital of Zhengzhou University from January 2012 to December 2018, including the gender, age, pathological type, tumor size, tumor number, central and lateral lymph node metastasis, was retrospectively analyzed.

There were 16 895 females and 5 085 males (gender ratio: 3.3 to 1), aged 4 to 95 (47.6±11.8) years old. Except for 2012, the average onset age of females was higher than that of males, and both genders showed a trend of early onset over time (females: Z=-2.703, P=0.007; males: Z=-3.004, P=0.003). The proportion of female aged 25 to 39 and male aged 20 to 39 was increasing, but the proportion of both genders aged over 60 was decreasing (all P<0.05). With the increase of tumor length and diameter, the positive rate of central lymph nodes metastasis (Z=-2.205, P=0.027) and lateral lymph node metastasis (Z=-2.205, P=0.027) gradually increased.

The onset age of thyroid cancer exhibited a much younger trend, with an increasing proportion of women aged 25-39 and men aged 20-39. Therefore, it should be suggested to strengthen the screening of people in the corresponding age range. The newly diagnosed thyroid cancer was mainly thyroid micropapillary carcinoma, with a high proportion of lymph node metastasis and multiple foci, and thus the optimal treatment methods need to be carefully considered.

To investigate the clinical efficacy of Laparoscopic cornuotomy on Interstitial Tubal Pregnancy (IP) which diameter was shorter than 3 cm.

The women who had IP under 3 cm diameter were selected from January, 2016 to December, 2018 at the Department of Gynecology, JiaXing Maternity and Child Health care Hospital. A total of 32 IP patients were divided into two groups. They were all treated with laparoscopic surgery. 17 patients were in study group, conducted by a cornuotomy and suturing the cornual.15 patients were in control group, conducted by a cornual resection and suturing the cornual. Patients′ genenral conditions were not significantly different(all P values>0.05). The peri-surgical data and the related clinical data were compared in the two groups.

All the 32 patients were successfully treated by laparoscopic surgery. The mean operating time was significantly shorter for cornuotomy than for cornual resection[ (33±6) min vs (53±9) min, P<0.05].Changes in blood loss[(45±5) ml vs (50±7) ml]、the total hospital stays[ (4.4±1.4) d vs (4.6±1.4) d] and the recovery time of HCG[(16±5) d vs (15±5) d] were not significantly different between the two groups (allP values>0.05) . There were no persistent ectopic pregnancy and uterine rupture happened in two groups. Compared with the control group,the interval time to pregnancy was shorter[ (8±3) m vs (16±4) m,P<0.05] and the number of full-term pregnancy cases were more (9 vs 3, P<0.05).

The Laparoscopic cornuotomy was feasible and safe on interstitial tubal pregnancy which diameter was shorter than 3cm. It deserved popularization in the clinical work and regarded as one of selection operative treatment on interstitial heterotopic pregnancy.

Investigating the diagnostic value of MRI for cervical cancer, including preoperative staging, vagina involvement and lymph node metastasis is the aim of this pape.

Select 116 patients with UCC of the second hospital of JiLin University from October 2016 to April 2019. All patients accept MRI examination. Ultimately, all patients accept surgical treatment. Use SPSS19.0 software to analyze MRI results of all the patients.Using the postoperative pathological results as the golden standard in the diagnosis of cervicalcancer diagnosis. The dates from the MRI preoperative staging, preoperative clinical staging and the postoperative pathologic staging were compared through chi-square test. And the dates of preoperative MRI in cervical cancer vagina involvement and lymph node metastasis diagnosis and postoperative pathological results were compared through Mcnemar chi-square test. The difference was statistically significant(P<0.05).

1, The differences between MRI staging and the postoperative pathologic staging have no statistical significance (P>0.05), whilethe difference between MRI staging and preoperative clinical staging during was statistically significant (P<0.05). Using the postoperative pathological staging as the examination standards, the accuracy of preoperative clinical staging is only 67.5%, and cervical cancer overall preoperative MRI staging accuracy was 95%; 2, Preoperative MRI diagnosis and postoperative pathologic results in cervical vaginal involvement and lymph node metastasis has highsensitivity and specificity, were 97.0%, 96.2%, 93.2%, 97.8%. Checked by Mcnemar chi-square test, the differences between themhave no statisticalsignificance (P>0.05), namely the preoperative MRI diagnosis and postoperative pathological results have consistency in clinical.

The combination of MRI and FIGO clinical stage can impro the accuracy of clinical staging of cervical cancer. MRI can be used as the important tool to assess cervical cancer preoperative staging, and to choose and formulate reasonable cervical cancer treatment plan.

To compare the composition of DJ tube encrustation and original stone, and to discuss the causes and prevention of encrustations.

Twenty-five patients with DJ tube encrustation (1 case of INLAY OPTIMA^® tube, Bard Medical, 16 cases of Universa^® Firm tube, Cook Medical, 8 cases of Polaris™ Loop tube, Boston Scientific) in our institution from Januarry 2018 to April 2019 wereretrospective analyzed. Stones and encrustations were analyzed using the Fourier transform infrared spectroscopy analysis system to compare the differences in composition.

76% (19/25) patients had the exact same DJ tube encrustations composition as the original stone component; 24% (6/25) patients had DJ tube encrustation and original stone composition inconsistent (5 cases of calcium oxalate stone, 1 case of apatite carbonate.) It maybe related to the control of urinary tract infection after ureteral obstruction. The earliest DJ tube with encrustation was a pre-expansion tube before flexible-ureteroscopy, and the indwelling time was only 18 days.

DJ tubes of different brands and models have the risk of formation of encrustation. Most of the encrustation has the same composition as the original stone. The prevention of DJ tube scorpion can be started from: (1) removing the DJ tube within 6 weeks or replace it regularly when necessary; (2) selecting the appropriate tube including diameter and model; (3) actively controlling urinary tract infection; (4) treating the metabolic diseases, such as hypercalcemia, hyperuricemia, et al, and providing personalized guidance for each patient.

To investigate the goal-oriented retroperitoneoscopic adrenalectomy and report the initial experiment.

A total of 102 patients were selected to our clinic experiment, and performed retroperitoneoscopic adrenalectomy with the new method. including adrenal cortex adenoma 76 cases, phaochromocytoma 12 cases, adrenal cyst 6 cases, myelolipoma 4 cases, gangliocytoma 1 case and corticohyperplassia 3 cases. The mean diameter of the tumors was 2.8 cm (0.5-5.8 cm). The operative procedure was briefly described as such, with ultrasound guiding, a needle was punched percutaneously up to the adrenal mass or the renal upper pole from lateral to posterior axillary line just below the inferior border of the 12th rib. labeled the pathway of the needle with methylene blue. Along the way of the needle, a 12 mm port was introduced into the retroperitoneal space with closed method, and the laparoscope with a working tunnel was introduced to make a tunnel along the label up to the adrenal for finally removing it. Additional port should be used when it was needed in the procedure.

The procedures of all patients were successful, and 10 patients were performed with only one port, 81 patients with two ports, 11 patients with three ports. The operative duration was 49 (31-115) min, the average blood loss was 38 (0-260) ml. There was no transition to open surgery and no perioperative complications. The length of postoperative hospital stay was 4.1 d (2-7 d). 98 patients were available for follow-up of 16.5 months (1-38 months), no complication was found.

The new method of retroperitoneoscopic adrenalectomy is feasible and safe for renal masses, and compared to the conventional method, it may be less trauma to the abdominal wall and retropertoneal tissue, and it was also better on cosmetics.

To investigate the offline clinical effect of external diaphragm pacemaker (EDP) combination with High-flow nasal cannula oxygen therapy in postoperative severe cerebral hemorrhage patients.

A total of 123 severe postoperative severe cerebral hemorrhage patients with tracheotomy and mechanical ventilation (MV) were selected, who were admitted in NICU of PLA Rocket Force Characteristic Medical Center from October 2016 to December 2019. These patients were randomly divided into the High-flow nasal cannula (HFNC) group (n=63) and HFNC+EDP group (n=60). In the HFNC group, the HFNC was given to the patients who were off ventilator. The HFNC+EDP group was added on the basis of treatment in the HFNC group, once a day and 30 minutes each time.

Compared to the HFNC group, the oxygenation index (OI) of the HFNC+EDP group was higher in the next 1, 12, 24 and 48 hour [(209±15) mmHg vs (218±18) mmHg, (215±14) mmHg vs (222±17) mmHg, (223±13) mmHg vs (235±15) mmHg, (236±7) mmHg vs (257±12) mmHg,P<0.05]. The offline time was shorter [(13±3) d vs (12±3) d,P<0.05]. The excursion of diaphragm was higher [(1.94±0.08) cm vs (2.91±0.11) cm,P<0.05]. The length of stay in NICU was shorter [(33±14) d vs (28±9) d,P<0.05]. The Glasgow Coma Scale (GCS) was higher when being discharged from the NICU (5.9±2.1 vs 7.8±0.4, P<0.05) and the hospital (9.9±2.1 vs 11.0±2.0, P<0.05).

EDP combination with HFNC can increase the excursion of diaphragm, shorten the offline time and length of stay in the NICU, thereby enhancing the early recovery and improving the prognosis.

To investigate the role of nitric oxide (NO) in hydrogen sulfide (H₂S)-induced inhibition upon colonic smooth muscle contraction.

Immunohistochemistry was applied to observe the distribution of H₂S-producing enzymes CBS and CSE in adult male Wistar rats. Organ bath system was used to observe the spontaneous contraction of colonic smooth muscle. Patch clamp technique was applied to record currents of L-type calcium channel (I_Ca,L) in smooth muscle cells.

Specific immunoreactivity for CSE and CBS was observed in mucosa, smooth muscle and enteric plexus of rat proximal colon. NaHS elicited relaxation in a concentration-dependent manner upon muscle contraction in the presence of tetrodotoxin. The NaHS IC₅₀ of LM was 917.6 μmol/L (95% CI: 776.3-1 085 μmol/L, n=6) and the NaHS IC₅₀ of CM was 730.4 μmol/L (95% CI: 592.2-900.8 μmol/L, n=6). The SNP-induced relaxation in muscle strips was partially reversed by NaHS (P<0.05). Instead, the relaxation caused by NaHS was decreased by the sGC inhibitor ODQ but affected neither by NO precursor L-arginine, the NO inhibitor L-NNA nor the competitive cGMP antagonist PET-cGMP. NaHS (100 μmol/L) increased I _Ca,L while NaHS (300 μmol/L) decreased the peak I _Ca,L with modifying the ion channel characteristics (P<0.05).

Exogenous hydrogen sulfide might have a dual effect on colonic motility and its inhibitory effect might be independent of NO signaling system. L-type calcium channel may play an important role during the process of H₂S modulating colonic contraction.

To explore the effect of CtBP-Interacting protein (CtIP) on oxidative damage of cerebral endothelia cells and its mechanism.

Cerebral endothelia cells were stimulated by TBHP to induce oxidative damage. The cell line of CtIP gene were prepared by over-expression and interfering lentivirus technology. Cell damage was detected by immunofluorescence assay of cysteinyl aspartate specific proteinase-3 (Caspase-3). The expression of CtIP and Caspase-3 protein was detected by Western blotting, and the related genes of CtIP signaling pathway were detected by Realtime RT-PCR.

The results of immunofluorescence and Western blotting showed that overexpression of CtIP inhibited the Caspase-3 expression reducing to 1/3 level compared with normal cultured cerebral endothelia cells. Interfering with CtIP expression resulted in the Caspase-3 expression increased significantly to 4/5 level compared with normal cultured cerebral endothelia cells and cerebral endothelia cells were damaged more severely. Realtime RT-PCR data showed that expression of CtIP significantly increased the expression of BRCA1 and ZBRK1 genes, but inhibited the expression of p21 gene.

It is confirmed that CtIP gene has the significantly inhibitory effect on injured cerebral endothelia cells, and the regulatory relationship between CtIP gene and BRCA1, ZBRK1 and p21 genes in the process of injury are determined.

To investigate the clinical characteristics and risk factors of severe bronchial asthma in Chinese people over 14 years old.

According to the multi-stage random cluster sampling methods, a total of 164 215 subjects were visited by a questionnaire in the epidemiology survey from eight provinces (Beijing, Shanghai, Guangdong, Liaoning, Henan, Shanxi, Jiangsu, Sichuan provinces) located in seven regions (north, northeast, east, central China, south, southwest and northwest) of China from February 2010 to August 2012. A total of 2 034 were diagnosed as asthma. The clinical characteristics and related risk factors of patients with severe asthma in China were analyzed.

Among all asthma patients, 560 were newly diagnosed, accounting for 27.5% (560/2 034) and the percentage of previously confirmed patients was 72.5% (1 474/2 034). A total of 145 were eligible for severe asthma, accounting for 9.8% (145/1 474) of previously confirmed asthmatics and 7.1% (145/2 034) of all asthmatics. 83.5% (121/145) severe asthmatics had at least one trigger factor. Correlation analysis showed that the risk factors of severe asthma were: smoking (OR=1.543, 95%CI: 1.250-1.814), obesity (OR=2.186, 95%CI: 1.972-2.354), petting (OR=2.135, 95%CI: 1.904-2.283), combined with allergic rhinitis (OR=3.456, 95%CI: 2.721-4.326), gastroesophageal reflux disease (OR=1.842, 95%CI: 1.682-2.140), bronchiectasis (OR=1.665, 95%CI: 1.347-1.912) or chronic obstructive pulmonary disease (OR=1.312, 95%CI: 1.171-1.694).

The most common comorbidities in severe asthmatics in China are allergic rhinitis and gastroesophageal reflux disease. The risk factors of severe asthma include obesity, allergic rhinitis, gastroesophageal reflux disease, chronic obstructive pulmonary disease, bronchiectasis, smoking and petting.