Objective To evaluate the surgical treatment for renal cell carcinoma with inferior vena cava tumor thrombus and the clinical significance of multidisciplinary treatment. Methods Two cases of renal cell carcinoma with inferior vena cava thrombus diagnosed by Doppler ultrasonography and CT were included in this retrospective analysis. The tumor thrombus was in level Ⅱ in one case and in level Ⅳ in the other. Coagulation test and complete blood count were done again before surgery. Human albumin, fibrinogen, prothrombin complex, plasma, platelet, UW and irrigating solution were prepared before the operation.Under general anesthesia, surgery was performed using abdomen inverted Y shaped incision. Right radical nephrectomy was finished by the urological surgeon; the vena cava was completely dissected from the renal vein level to the secondary porta of the liver by the hepatobiliary surgeon, the vena cava and the surrounding branch vein were blocked in the upper and lower vena cava tumor thrombus; tumor thrombus was removed completely by the vascular surgeon. In one case (patient with level Ⅳ thrombus ) where the tumour thrombus invaded the wall of the vena cava, the thrombus was found to be extending to the cavo-atrial junction but not into the right atrium. The left femoral venous-right atrial bypass was established, the cardiopulmonary bypass lasted for 241 mia, and the aorta was blocked for 18 min. Salvage autotransfusion was used during surgery, and the hepatic vein of the secondary liver porta was anastomosed to artificial vascular graft.The data for surgical indication, operation time, operative blood loss and postoperative hospital stay were analyzed. Results Right radical nephrectomy and inferior vena cava thrombectomy were performed successfully, and the two patients were discharged on the 15th and 27th day after surgery, respectively. The two patients were followed up for 1 and 16 months after surgery, respectively, and both survived without local recurrence and distant metastasis. Conclusion Radical nephrectomy and inferior vena cava thrombectomy is the preferred method for patients without metastasis, and multidisciplinary cooperation could shorten the operation time, reduce the tumor recurrence and increase the survival rate of patients.

The clinical and genetic data were retrospectively analyzed in a pedigree with pseudohypoparathyroidism type Ⅰ a.Clinically typical Albright hereditary osteodystrophy (AHO),hypocalcemia,hyperphosphatemia,and PTH- and TSH-resistance were manifested in the proband,but not in his brother and parents.The proband's symptom of epilepsy was alleviated by treatment with calcium and vitamin D,which was of no avail in regard to AHO.After GNAS1 genes were sequenced and compared with the GenBank data among the family members,a deletion of c.1107_1108 ( p.Glu370ArgfsX11 ) in exon l3 of GNAS1 gene leading to a frameshift mutation was found in the proband and his mother.It suggested that the GNAS1 gene mutation might be related to the pathogenesis of the disease.

OBJECTIVETo analyze CYP21A2 gene mutation in two families with 21-hydroxylase deficiency (21-OHD) and to explore the correlation between genotype and clinical phenotype.

METHODSTwo patients with 21-OHD and their families were investigated. CYP21A2 gene mutation was analyzed by PCR and direct sequencing.

RESULTSThe probands from family 1 and 2 have been respectively diagnosed with simple virilizing and non-classical 21-OHD. Both showed increased baseline serum 17hydroxyprogesterone, testosterone and adrenocorticotropic hormone (ACTH), but had no evidence of salt loss. Computer tomography revealed bilateral adrenal hyperplasia in both patients. After 1 year treatment, both had conceived successfully. DNA sequencing revealed that the proband of family 1 had compound heterozygous mutations for IVS2 13 A>G and Ile172Asn. Her father was heterozygous for Ile172Asn, whilst her mother and brother were heterozygous for IVS213A/C>G. In family 2, the proband was heterozygous for Arg341Trp and Gln318X. Her father, sister and nephew were heterozygous for Arg341Trp, whilst her mother was heterozygous for Gln318X. her brother and niece were non-affected. Carriers of single heterozygous mutations in both families had no clinical sign.

CONCLUSIONIn both families, the disease has been caused by compound heterozygous mutations, for which there has been a good genotype-phenotype agreement. Screening of CYP21A2 gene can facilitate both diagnosis and genetic counseling.

Adrenal Hyperplasia, Congenital ; blood ; enzymology ; genetics ; Adrenocorticotropic Hormone ; blood ; Adult ; Base Sequence ; Child ; Female ; Genotype ; Humans ; Male ; Molecular Sequence Data ; Mutation, Missense ; Pedigree ; Phenotype ; Steroid 21-Hydroxylase ; genetics ; metabolism ; Testosterone ; blood ; Young Adult

Objective:To survey the knowledge, attitudes, and practice about chronic obstructive pulmonary disease (COPD) among general practitioners in Sichuan province.Methods:From October to November 2020, a questionnaire survey on knowledge, attitudes and practice (KAP) among 104 general practitioners who participated in the training of the comprehensive prevention and control of COPD organized by Sichuan Provincial Health Commission. The content of the questionnaire included the basic information and the KAP status of COPD. The descriptive analysis, Mann-Whitney U test, and Kruskal-Wallis H were used for statistical analysis. Results:A total of 104 questionnaires were distributed and 102 were returned with a response rate of 98.1%. General practitioners with different ages, professional titles, visits of COPD patients each month, and whether or not to refer COPD patients to higher-level hospitals were not significantly different in the scores of knowledge( U=1 276.00, H=0.78, U=1 074.00, U=589.00), attitude( U=1 141.50, H=1.75, U=1 090.00, U=585.00), and practice( U=1 221.00, H=0.31, U=1 163.00, U=499.50) (all P>0.05). In knowledge part the highest correctness rate was "quitting smoking is the most powerful interventions affecting the natural disease course of COPD" (100.0%, 102/102); the lowest was that"the main objective examination for judging the airflow limitation of COPD patients is the lung function test"(5.9%, 6/102). In the part of the attitude, the highest positive rate was "educating patients to quit smoking and avoid exposure to second-hand smoke" (65.7%,67/102); the lowest positive rate was "provide exercise guidance to patients "(43.1%, 44/102) and " be responsible for long-term treatment follow-up" (43.1%, 44/102). In the part of practice, the highest behavioral rate was "when receiving patients with COPD, actively pay attention to the treatment of comorbid diseases" (95.1%, 97/102); the lowest behavioral rate was "when patients with COPD were seen at the clinic, and the patients were advised of the best time to use the inhaler" (65.7%, 67/102). Conclusions:The study indicates that the relevant departments need to increase the intensity of knowledge training for general practitioners in Sichuan province. General practitioners should continuously improve their education, prevention, and treatment ability and implement the primary responsibilities for COPD management.

Objective:To compare the clinical outcome of transepithelial photorefractive keratectomy (TransPRK) using 1 050 Hz ablation frequency and intelligent pulse technique (SPT) and small incision lenticule extraction (SMILE) for myopia and astigmatism.Methods:A cohort study was performed.Eighty-five eyes of 43 patients who received TransPRK for myopia and 85 eyes of 46 patients who received SMILE for myopia in the Ineye Hospital of Chengdu University of TCM were enrolled from August 2017 to April 2018.The follow-up duration was 6 months.The changes of visual acuity and diopter were observed and compared before and after operation, and the predictability, stability, safety, effectiveness and long-term vision were compared between the different surgeries.This study complied with the Declaration of Helsinki and the study protocol was approved by the Ethics Committee of Ineye Hospital of Chengdu University of TCM.Results:The refractive power tended to be emmetropic and relatively stable in the TransPRK group, and the refraction varied from mild hyperopia to emmetropic gradually during 6 months after SMILE.There was no significant difference in the spherical equivalent (SE) between the two groups before and after operation (all at P>0.05). No significant difference was found in mean validity index between the two groups at 6 months after surgery (1.189±0.248 vs.1.120±0.205; t=1.862, P=0.065). The uncorrected visual acuity (UCVA) in the SMILE group was significantly higher than that in the TransPRK group at 7 days and 1 month after surgery ( P<0.05), and there was no significant difference in UCVA between the two groups at 3 months and 6 months after surgery ( P>0.05). The safety index at 6 months after surgery in the TransPRK group was 1.209±0.222, which was significantly higher than 1.143±0.178 in the SMILE group, with a significant difference between the two groups ( t=2.024, P=0.045). Conclusions:The predictability, stability, safety, effectiveness and long-term vision are good after TransPRK with SPT and SMILE for myopia and astigmatism.The safety index is better in TransPRK compared with SMILE, and the restoration of vision is faster after SMILE than that after TransPRK.

【Objective】 To analyze the effect of extensively hydrolyzed formula(eHF) in the treatment of feeding intolerance in preterm infants and the effect on hospital infection, in order to provide reference for the clinical treatment of feeding intolerance in preterm infants. 【Methods】 A total of 208 cases of preterm infants with feeding intolerance diagnosed and treated in Shandong Heze Municipal Hospital from April 2017 to February 2020 were selected into the clinical trial for eligibility assessment, then were randomly assigned into study group(n=100) and control group(n=100) after screening and exclusion. Children in the control group were fed with standard preterm formula, while children in the study group were fed with eHF. Feeding tolerance indicators, including daily milk intake, time to meconium evacuation, time to full gastrointestinal nutrition, total gastric residual counts(GRV1) in the 7-d period after resumption of breastfeeding, ratio of all-day gastric residual counts/all-day estimated milk intake after resumption of breastfeeding(GRV2) were compared between the two groups, and growth indicators(body weight growth rate, head dimension growth rate), complication incidence [necrotizing enterocolitis(NEC), pathological jaundice, positive fecal occult blood or blood in stool] and incidence of hospital-acquired infections. 【Results】 The daily milk intake(t=5.037) of the study group was higher than that of the control group, and the time of foetal excretion(t=9.217), the time to reach full gastrointestinal nutrition(t=15.833), GRV1(t=6.737), GRV2(t=9.956) were lower than those of the control group, and the differences were all statistically significant(P<0.05). The rate of weight gain(t=2.454) and head dimension growth(t=5.469) in the study group was significantly higher than those of the control group(P<0.05). The incidence of the three complications of NEC, pathological jaundice and positive fecal occult blood or blood in stool(χ²=4.310) and the incidence of hospital infections(χ²=4.688) were significantly lower in the study group than in the control group(P<0.05). 【Conclusions】 Compared with the standard formula milk for preterm infants, eHF can significantly improve the feeding intolerance of preterm infants, promote growth and development, and reduce the occurrence of hospital-acquired infections. Therefore, eHF can be widely used in clinic for preterm infants with feeding intolerance.

Objective:To observe the corneal morphology and visual quality after transepithelial photorefractive keratectomy (Trans-PRK) with smart pulse technique (SPT) and 1 050 Hz cutting frequency in the correction of myopia and astigmatism.Methods:A self-controlled case series study was conducted.Sixty five eyes of 33 patients who underwent Trans-PRK surgery in Ineye Hospital of Chengdu University of TCM from July 2017 to June 2018 were followed up for 6 months.The uncorrected visual acuity (UCVA) converted to logarithm of the minimum angle of resolution (LogMAR) unit, best corrected visual acuity (BCVA) (LogMAR), and spherical equivalent (SE) of the subjects were recorded.The anterior corneal surface symmetry index (SI), the anterior corneal surface Q value in the range of 6, 7, 8, and 9 mm diameter, the spherical aberration, coma, trefoil and total higher-order aberration of the anterior corneal surface, the strehl ratio (SR), and the modulation transfer function (MTF) of 10, 20, 30, and 40 c/d in the horizontal and vertical meridian directions before and after surgery were measured with Sirius corneal topography analyzer.The differences of each index among different time points were compared, and the correlation between indexes was analyzed by Pearson correlation analysis.This study followed the Declaration of Helsinki.The study protocol was approved by the Medical Ethics Committee of Ineye Hospital of Chengdu University of TCM (No.2020yh-004). All patients signed the informed consent form before surgery.Results:The average preoperative BCVA and SE were -0.09±0.06 and (-4.24±1.24)D.The mean UCVA and SE at 7 days, 1, 3 and 6 months postoperatively were -0.10±0.08 and (0.03±0.63)D, -0.12±0.06 and (0.08±0.53)D, 1.16±0.06 and (0.02±0.79)D, -0.18±0.05 and (0.08±0.37)D, respectively.The SI at different time points after the surgery were significantly higher than that before operation (all at P<0.05). At 1, 3 and 6 months after surgery, the Q value of anterior corneal surface in different diameter ranges increased from negative to positive, showing statistically significant differences (all at P<0.05). At each time point after surgery, the trefoil and total higher-order aberrations of the anterior corneal surface increased to varying degrees.Coma at 7 days and 6 months after surgery were significantly higher than that before surgery, and spherical aberration at 3 and 6 months after surgery were significantly higher than that before surgery (all at P<0.05). The SR values at 3 and 6 months after operation were significantly higher than that before operation (all at P<0.05). At 6 months after operation, the MTF values at different spatial frequencies of the horizontal meridian and the MTF values at 30 and 40c/d spatial frequencies of the vertical meridian were lower than those before operation, and the differences were statistically significant (all at P<0.05). The correlation analysis showed that the Q value of different diameter ranges was positively correlated with spherical aberration ( r=0.798-0.925, P<0.05), total higher-order aberration ( r=0.596-0.630, P<0.05), SI ( r=0.235-0.303, P<0.05) and corneal ablation depth ( r=0.583-0.659, P<0.05) at 6 months after surgery.SI was positively correlated with spherical aberration ( r=0.307, P<0.05), coma ( r=0.424, P<0.05), total higher-order aberration ( r=0.300, P<0.05), corneal ablation depth ( r=0.227, P<0.05), and eccentric cutting amount ( r=0.281, P<0.05). There was no correlation between SR and aberration, corneal ablation depth, eccentric cutting amount, etc.(all at P≥0.05). Conclusions:Trans-PRK using SPT to correct myopic astigmatism can improve vision, stabilize diopter, enhance retinal imaging quality, increase the asymmetry of the anterior corneal surface, and introduce different degrees of higher-order aberrations.

Objective To investigate the features of Langerhans cell histiocytosis (LCH). Method Skin lesions,systemic involvement,imaging characteristics,laboratory tests,immunophenotying,treatment response,and survival of 122 LCH patients treated at our center from February 1983 to August 2013 were retrospectively analyzed. Results LCH was associated with diverse skin lesions. Lung was the most involved organ,followed by bone,skin,lymph nodes,liver,spleen,oral cavity,and thyroid. Multisystem LCH was more common than single-system LCH. Single-system LCH was mostly treated by surgery,whereas multisystem LCH by combined chemotherapy. Conclusion LCH has diverse clinical manifestations,with lungs being the most often involved organ. Surgery or chemotherapy is the mainstream treatment.