Clinical and gene mutation characteristics of patients with hereditary ellipsocytosis: nine cases report and literature review / 中华血液学杂志

Objective: To report gene mutations in nine patients with hereditary elliptocytosis (HE) and analyze the characteristics of pathogenic gene mutations in HE. Methods: The clinical and gene mutations of nine patients clinically diagnosed with HE at Institute of Hematology & Blood Diseases Hospital from June 2018 to February 2022 were reported and verified by next-generation sequencing to analyze the relationship between gene mutations and clinical phenotypes. Results: Erythrocyte membrane protein gene mutations were detected among nine patients with HE, including six with SPTA1 mutation, one with SPTB mutation, one with EPB41 mutation, and one with chromosome 20 copy deletion. A total of 11 gene mutation sites were involved, including 6 known mutations and 5 novel mutations. The five novel mutations included SPTA1: c.1247A>C (p. K416T) in exon 9, c.1891delG (p. A631fs*17) in exon 15, E6-E12 Del; SPTB: c.154C>T (p. R52W) ; and EPB41: c.1636A>G (p. I546V) . Three of the six patients with the SPTA1 mutation were SPTA1 exon 9 mutation. Conclusion: SPTA1 is the most common mutant gene in patients with HE.

Caring Ethics of Long-term Care for the Elderly / 中国医学伦理学

With the continuous increase of the elderly in China, the proportion of elderly with disabilities and dementia remains high. Long-term care, as a care system for elderly with disabilities and dementia, has some problems that misunderstanding of the concept of long-term care, the de-emotionalization of care behaviour, and the impact on family ethics. The care ethics grounds the universal caring experience, and provides an ethical norm with relationship as a pivot and emotion as a connection. Its characteristics of emphasis on contextual, relational and emotional have constructive implications for improving long-term care system. To realize care, it is significant to clarify the concept and improve the system in the ethical context, focus on both sides of the relationship based on demand, let care practice infiltrate into life through turning emotion into action, and build a long-term care system with a temperature to improve the overall quality of life of the elderly.

Exploratory study on noninvasive evaluation of renal histopathology by ultrasonic shear wave elastography / 中华肾脏病杂志

Objective:To determine a relationship between ultrasound shear wave elastography (SWE) and pathological lessions of renal tissues in children with chronic kidney disease (CKD).Methods:It was a cross-sectional observational study, involving children admitted to the Department of Pediatrics of Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology from January to December 2021 with definite pathological diagnosis through kidney biopsy. The SWE was used to determine the Young's modulus (elastic modulus) of the cortex and medulla of the upper, middle, and lower poles of the kidney. The renal histopathology was classified or graded. The statistical method was used to analyze the relationship between Young's modulus of the inferior polar cortex (YM cor) and medulla (YM med) of the right kidney and renal pathology. Results:The study included 110 children with definite pathological diagnosis through renal biopsy, aged (10.1±3.4) years old (2-17 years old), with 55 males (50.0%). The body mass index was (20.6±2.4) kg/m 2, and mean arterial pressure was (95±24) mmHg. There were 94 patients (85.4%) with CKD stage 1, 8 patients (7.3%) with CKD stage 2, and 8 patients (7.3%) with CKD stage 3. There was no significant difference of YM cor and YM med in the upper and middle poles of the right kidneys, and YM med in the lower poles of right kidneys in CKD patients with different stages (all P>0.05). Both YM cor [(15.75±3.36) kPa] and YM med [(13.50±2.43) kPa] of CKD stage 3 patients were significantly higher than those of CKD stage 1 patients [(12.94±2.45) kPa, (11.88±2.23) kPa](both P<0.05). There was no significant difference of YM cor and YM med in the lower poles of right kidneys between stage 1 and stage 2 CKD patients (both P>0.05). YM cor[(17.93±3.23) kPa] and YM med [(15.50±1.48) kPa] in patients with crescentic glomerulonephritis were higher than those in patients with focal segmental glomerulosclerosis [(12.71±2.42) kPa, (11.57±2.63) kPa] and mesangial proliferative glomerulonephritis [(12.73±2.04) kPa, (11.48±2.10) kPa](all P<0.05). There was no significant difference of YM cor and YM med between focal segmental glomerulosclerosis and mesangial proliferative glomerulonephritis (both P>0.05). YM cor [(16.30±2.63) kPa] and YM med [(15.54±1.59) kPa] of Lee's Ⅳ grade of IgA nephropathy were higher than those of Lee's Ⅲ grade [(13.32±2.70) kPa, (12.57±2.50) kPa](both P<0.05), while the International Study of Kidney Disease in Children grade of purpura nephritis had no significant correlation with YM cor and YM med (both P>0.05). YM cor [(15.41±2.37) kPa] and YM med [(13.82±2.59) kPa] of interstitial fibrosis/tubular atrophy (T1/T2) group of IgA nephropathy mixed with purpura nephritis were significantly higher than those of T0 group's [(12.99±2.40) kPa, (11.79±2.05) kPa] (both P<0.05). Moreover, crescent formation (C1) group had a higher YM cor [(14.21±2.77) kPa] and YM med [(12.80±2.47) kPa] than those in C0 group [(12.73±2.15) kPa, (11.59±1.97) kPa] (both P<0.05), while YM cor and YM med were unrelated to the mesangial hypercellularity (M), endocapillary cellularity (E), segmental sclerosis or adhesion (S) indicators (all P>0.05). In lupus nephritis patients, YM cor ( r=0.744, P=0.035) and YM med ( r=0.728, P=0.009) were favorably linked with the chronic index, but not with the activity index (both P>0.05). Conclusions:Renal interstitial fibrosis/tubular atrophy and crescentic development are connected with YM cor and YM med at the lower pole of the kidney as measured by SWE. SWE can be used to assess the chronic renal lesions in children with CKD in the early and middle stages. It may develop into a new noninvasive way to assess renal pathology.

Distribution characteristics of special types of diabetes mellitus in Chinese population: A literature-based analysis from 2011 to 2021 / 中华内分泌代谢杂志

Objective:To analyze the distribution characteristics of special types of diabetes in China, in order to provide a theoretical basis for the diagnosis and treatment of special types of diabetes.Methods:Pubmed, CNKI, and WanFang Data were searched for the case reports and clinical studies of special types of diabetes in China from 2011 to 2021. After independent literature screening by 2 researchers according to the inclusion and exclusion criteria, diseases and the number of corresponding cases included were extracted for statistics. The etiological composition and disease characteristics of three subtypes of special type diabetes were analyzed.Results:A total of 613 articles(7 377 patients)were included and roughly divided into eight subtypes of special type diabetes according to etiological classification for disease composition analysis. The results by ratio in descending order were as follows: mono-genetic gene defects in islet β-cell function, pancreatogenic diabetes, diabetes induced by drugs or chemicals, endocrine disease, mono-genetic gene defects in insulin action, other genetic syndromes associated with diabetes, infection, and uncommon immune-mediated diabetes. The disease composition of the three subtypes of special types of diabetes that we focused on were mono-genetic gene defects in islet β-cell function(50.21%), pancreatogenic diabetes(35.65%), and mono-genetic gene defects in insulin action(1.56%). The composition analysis of the special types of diabetes in each subtype showed that neonatal diabetes mellitus(NDM, n=1 749, 23.71%)and maturity onset diabetes in young(MODY, n=1 554, 21.07%)accounted for the largest proportions. According to the composition analysis of each subtype of MODY patients, the top three subtypes were MODY2(50.89%), MODY3(16.03%), and MODYX(8.91%). In addition, taking MODY as an example, patients with de novo mutations(DNMs)and(or)new mutation sites were summarized and analyzed. The results revealed 31 MODY patients with DNMs(1.99%) and 339 MODY patients with new mutation sites(21.81%). Conclusions:According to the literature analysis, NDM and MODY represent the largest proportion of patients with special type diabetes in China. MODY2 patients make up the largest proportion of MODY patients. In addition, diabetic patients carrying DNMs and(or)new mutation sites should be taken seriously.

Summary of best evidence and practice recommendations for nonpharmacological interventions of urinary incontinence in elderly women / 中华健康管理学杂志

Objective:To integrate the best evidence of non-drug intervention of urinary incontinence in elderly women and to formulate practical recommendations.Methods:In this systematic review study, using “elderly woman”,“urinary incontinence”,“bladder training”,“pelvic floor muscle training”,“enuresis”,“leakage of urine” as the key words, the 6S evidence resource pyramid model was used to search in British Medical Journal best practice, Uptodate, World Health Organization, Guidelines International Network, National Institute for Health and Care Excellence, Chinese Medical Association, Scottish Intercollegiate Guideline Network, Registered Nurses Association of Ontario, Cochrane Library, The Joanna Briggs Institute (JBI), New Zealand Guidelines Group, Polish Society of Gynecologists and Obstetricians, PubMed, Embase, Medline, Web of Science, SinoMed, China National Knowledge Infrastructure, WanFang Data, etc. The evidence retrieved included evidence-based knowledge base resources, clinical practice guidelines, expert consensus, systematic review, etc. Data were retrieved from January 1, 2017 to May 1, 2022, and collated from May 2, 2022 to May 25, 2022. Two researchers independently evaluated the quality of literature and extracted data using the AGREE Ⅱ and JBI evidence-based health care center assessment tools. The JBI evidence-based health care center′s evidence pre-rating system and evidence recommendation rating system were applied to rank the evidence; and under the guidance of the evidence structure of JBI, the strength of evidence recommendation was determined and the best evidence was extracted and summarized in combination with the study group discussion and expert opinion.Results:A total of 9 articles were retrieved, including 7 guidelines and 2 systematic reviews; and 6 guidelines were classified as Grade A and 1 as grade B; both 2 systematic reviews were rated as Grade A; 84% (27/32) of the items were evaluated as “Yes”. Evidence were summarized as 34 pieces of best evidence from 6 dimensions, including “overall recommendation, evaluation of type and degree of urinary incontinence, lifestyle change, behavioral therapy, prevention of precipitating factors, intervention in special population”; the flow chart of screening, evaluation, special symptoms, life style and behavior therapy was combed, and the practical suggestions were formed.Conclusions:The overall quality of the literature on non-drug intervention of urinary incontinence in elderly women is high, and the level of evidence is high. Early identification of urinary incontinence types and assessment of disease severity, lifestyle changes, avoidance of predisposing factors and behavioral therapy are the key to non-drug treatment of urinary incontinence in those patients.

Observational study on perioperative outcomes of pelvic exenteration / 中华胃肠外科杂志

Objective: To investigate the surgical indications and perioperative clinical outcomes of pelvic exenteration (PE) for locally advanced, recurrent pelvic malignancies and complex pelvic fistulas. Methods: This was a descriptive study.The indications for performing PE were: (1) locally advanced, recurrent pelvic malignancy or complex pelvic fistula diagnosed preoperatively by imaging and pathological examination of a biopsy; (2)preoperative agreement by a multi-disciplinary team that non-surgical and conventional surgical treatment had failed and PE was required; and (3) findings on intraoperative exploration confirming this conclusion.Contraindications to this surgical procedure comprised cardiac and respiratory dysfunction, poor nutritional status,and mental state too poor to tolerate the procedure.Clinical data of 141 patients who met the above criteria, had undergone PE in the Sixth Affiliated Hospital of Sun Yat-sen University from January 2018 to September 2022, had complete perioperative clinical data, and had given written informed consent to the procedure were collected,and the operation,relevant perioperative variables, postoperative pathological findings (curative resection), and early postoperative complications were analyzed. Results: Of the 141 included patients, 43 (30.5%) had primary malignancies, 61 (43.3%) recurrent malignancies, 28 (19.9%) complex fistulas after radical resection of malignancies,and nine (6.4%)complex fistulas caused by benign disease. There were 79 cases (56.0%) of gastrointestinal tumors, 30 cases (21.3%) of reproductive tumors, 16 cases (11.3%) of urinary tumors, and 7 cases (5.0%) of other tumors such mesenchymal tissue tumors. Among the 104 patients with primary and recurrent malignancies, 15 patients with severe complications of pelvic perineum of advanced tumors were planned to undergo palliative PE surgery for symptom relief after preoperative assessment of multidisciplinary team; the other 89 patients were evaluated for radical PE surgery. All surgeries were successfully completed. Total PE was performed on 73 patients (51.8%),anterior PE on 22 (15.6%),and posterior PE in 46 (32.6%). The median operative time was 576 (453,679) minutes, median intraoperative blood loss 500 (200, 1 200) ml, and median hospital stay 17 (13.0,30.5)days.There were no intraoperative deaths. Of the 89 patients evaluated for radical PE surgery, the radical R0 resection was achieved in 64 (71.9%) of them, R1 resection in 23 (25.8%), and R2 resection in two (2.2%). One or more postoperative complications occurred in 85 cases (60.3%), 32 (22.7%)of which were Clavien-Dindo grade III and above.One patient (0.7%)died during the perioperative period. Conclusion: PE is a valid option for treating locally advanced or recurrent pelvic malignancies and complex pelvic fistulas.

Consensus on laboratory diagnosis of congenital adrenal hyperplasia due to 21 hydroxylase deficiency / 中华医学遗传学杂志

21 hydroxylase deficiency (21-OHD), the most common form of congenital adrenal hyperplasia, is caused by defects in CYP21A2 gene, which encodes the cytochrome P450 oxidase (P450C21) involved in glucocorticoid and mineralocorticoid synthesis. The diagnosis of 21-OHD is based on the comprehensive evaluation of clinical manifestation, biochemical alteration and molecular genetics results. Due to the complex structure of CYP21A2, special techniques are required to perform delicate analysis to avoid the interference of its pseudogene. Recently, the state-of-the-art diagnostic methods were applied to the clinic gradually, including the steroid hormone profiling and third generation sequencing. To standardize the laboratory diagnosis of 21-OHD, this consensus was drafted on the basis of the extensive knowledge, the updated progress and the published consensuses and guidelines worldwide by expert discussion organized by Rare Diseases Group of Pediatric Branch of Chinese Medical Association, Medical Genetics Branch of Chinese Medical Doctor Association, Birth Defect Prevention and Molecular Genetics Branch of China Maternal and Child Health Association. and Molecular Diagnosis Branch of Shanghai Medical Association.

The prescription rule analysis of the New Theory on Spleen Dampness Syndrome by Academician TONG Xiaolin / 数字中医药（英文）

@#Objective To analyze the basic characteristics, drug features, prescription rules, and drug-symptom relationships of patients in the splenic deficiency and impairment stage, by data mining of medical records under the New Theory on Spleen Dampness Syndrome (Pi Dan Xin Lun, 《脾瘅新论》). Methods Medical records listed in the “New Theory on Spleen Dampness Syndrome – Understanding and Treatment of Metabolic Syndrome from the Perspective of Traditional Chinese Medicine”, and which were diagnosed with the spleen dampness syndrome at the splenic deficiency and impairment stage, during January 2004 and December 2016 were selected. These patients’ data, including basic information, clinical symptoms, laboratory examination results, traditional Chinese medicine (TCM) and western medicine diagnoses, treatment methods, prescriptions, etc., were collected. The collected data were subsequently compiled into a medical record database using the Epidata 3.1 data management software, followed by the use of Apriori algorithm provided in the SPSS Modeler 14.2 statistical software to investigate the association rules between drug-drug, drug-symptom, and drug-western medicine indices. Results (i) A total of 51 medical records were included, involving 17 types of syndromes. Among them, the top three with frequency ≥ 3 included “Phlegm and blood stasis, and thoracic obstruction” “Deficiency-weakness of the spleen Qi, and static blood blocking collaterals”, and “Deficiency-weakness of the spleen Qi, and static blood blocking collaterals”. Alternatively, of the 14 treatment methods, the top three treatments with frequency of ≥ 3 included “Activating Yang and eliminating turbidity, and removing phlegm and dredging channel blockage” “Strengthening the spleen and benefiting Qi, and eliminating phlegm to activate the channels”, and “Warming Yang and benefiting Qi, and expelling cold to remove obstructions”. Among the 15 prescriptions, the top three used with frequency ≥ 3 included Huangqi Guizhi Wuwu Tang (黄芪桂枝五物汤), Gualou Xiebai Banxia Tang (瓜蒌薤白半夏汤), and Ganjiang Huangqin Huanglian Renshen Tang (干姜黄芩黄连人参汤). Lastly, of the 83 drugs used for a total of 476 times, those with frequency ≥ 15 included Huanglian (Coptidis Rhizoma), Huangqi (Astragali Radix), Jiudahuang (Wine-processed Rhei Radix et Rhizoma), Jixueteng (Spatholobi Caulis), Shengjiang (Zingiberis Rhizoma Recens), Huangqin (Scutellariae Radix), and Guizhi (Cinnamomi Ramulus). (ii) For the drug-drug associations, under the criteria of support ≥ 15% and confidence = 100%, seven second-order association rules, seven third-order rules, and six fourth-order roles were identified. The top-ranking rule of each was “Huangqin (Scutellariae Radix) → Huanglian (Coptidis Rhizoma)” “Ganjiang (Zingiberis Rhizoma) + Huangqin (Scutellariae Radix) → Huanglian (Coptidis Rhizoma)”, and “Baishao (Paeoniae Radix Alba) + Guizhi (Cinnamomi Ramulus) + Jixueteng (Spatholobi Caulis) → Huangqin (Scutellariae Radix)”, respectively. Alternatively, the drug-symptom associations were analyzed under the criteria of support ≥ 5% and confidence = 100%, which derived eight second-order association rules, 31 third-order rules, and 30 fourth-order rules. The top-ranking association rule of each order was “Huangqi (Astragali Radix) → Limb edema” “Guizhi (Cinnamomi Ramulus) + Jixueteng (Spatholobi Caulis) → Limb numbness and pain”, and “Guizhi (Cinnamomi Ramulus) + Jixueteng (Spatholobi Caulis) + Huangqi (Astragali Radix) → Limb numbness and pain”, respectively. Similarly, the drug-western medicine index associations were investigated under the criteria of support ≥ 5% and confidence = 100%, and five second-order association rules, 16 third-order rules, and 16 fourth-order rules were identified. In this category, the top-ranking association rule of each order was “Qinpi (Fraxini Cortex) → Uric acid” “Huanglian (Coptidis Rhizoma) + Ganjiang (Zingiberis Rhizoma) → Glycated hemoglobin”, and “Huanglian (Coptidis Rhizoma) + Ganjiang (Zingiberis Rhizoma) + Huangqin (Scutellariae Radix) → Glycated hemoglobin”, respectively. Conclusion Through association rule mining, this study objectively and quantitatively demonstrated the drug-drug, drug-symptom, and drug-physicochemical index associations of patients with the spleen dampness syndrome at the splenic deficiency and impairment stage treated by Academician TONG Xiaolin. The results indicated that treatment for these patients adopted the “state-target” syndrome differentiation method. The drug combination was characterized by “small prescriptions”, targeting both the patient’s symptoms and signs (syndrome target) and western medicine indices (treatment target). This study could provide references for future research on the academic thoughts and medical experience of Academician TONG Xiaolin.

Pharmacokinetic study of anti-human T-cell porcine immunoglobulin combined with cyclosporine A immunosuppressive therapy in patients with severe aplastic anemia / 中华血液学杂志

Objective: To study the metabolic characteristics of anti-human T-cell porcine immunoglobulin (p-ATG) in patients with severe aplastic anemia (SAA) . Methods: For patients with SAA treated with p-ATG combined cyclosporine A (CsA) immunosuppressants between February 2017 and December 2017, the p-ATG dose was 20 mg·kg(-1)·d(-1) over 12 h of intravenous administration for 5 consecutive days. The blood concentration of p-ATG was detected by the three-antibody sandwich ELISA method, the pharmacokinetic analysis software was fitted, and the second-chamber model method was used to calculate the pharmacokinetic parameters and plot the pharmacokinetic curve. Adverse events were recorded and the hematologic reactions were determined at 6 months after treatment. Results: Sixteen patients with SAA treated with p-ATG were enrolled, including 8 females and 8 males, with a median age of 22 years (range, 12 to 49 years) and a median weight of 62.5 kg (range, 37.5 to 82.0 kg) . The pharmacokinetics of p-ATG could be evaluated in 14 cases. p-ATG is distributed in vivo as a two-chamber model, with an average drug concentration peak (T(max)) of (5.786±2.486) days, a peak concentration (C(max)) of (616±452) mg/L, and a half-life of (10.479±8.242) days. The area under the drug time curve (AUC) was (5.807±3.236) mg/L·d. Six months after treatment, 8 of 14 patients received a hematologic response; the AUC (0-t) of the effective group and ineffective groups was (7.50±3.26) mg/L·d vs (4.50±2.18) mg/L·d, and the C(max) was (627±476) mg/L vs (584±382) mg/L, respectively. Conclusion: The plasma concentration of p-ATG reached a peak after 5 days of continuous infusion, and then decreased slowly, with a half-life of 10.479 days, and the residual drug concentration was detected in the body 60 days after administration. A relationship between drug metabolism and efficacy and adverse reactions could not be determined.

Reassessing the six months prognosis of patients with severe or very severe aplastic anemia without hematological responses at three months after immunosuppressive therapy / 中华血液学杂志

Objective: To reassess the predictors for response at 6 months in patients with severe or very severe aplastic anemia (SAA/VSAA) who failed to respond to immunosuppressive therapy (IST) at 3 months. Methods: We retrospectively analyzed the clinical data of 173 patients with SAA/VSAA from 2017 to 2018 who received IST and were classified as nonresponders at 3 months. Univariate and multivariate logistic regression analysis were used to evaluate factors that could predict the response at 6 months. Results: Univariate analysis showed that the 3-month hemoglobin (HGB) level (P=0.017) , platelet (PLT) level (P=0.005) , absolute reticulocyte count (ARC) (P<0.001) , trough cyclosporine concentration (CsA-C0) (P=0.042) , soluble transferrin receptor (sTfR) level (P=0.003) , improved value of reticulocyte count (ARC(△)) (P<0.001) , and improved value of soluble transferrin receptor (sTfR(△)) level (P<0.001) were related to the 6-month response. The results of the multivariate analysis showed that the PLT level (P=0.020) and ARC(△) (P<0.001) were independent prognostic factors for response at 6 months. If the ARC(△) was less than 6.9×10(9)/L, the 6-month hematological response rate was low, regardless of the patient's PLT count. Survival analysis showed that both the 3-year overall survival (OS) [ (80.1±3.9) % vs (97.6±2.6) %, P=0.002] and 3-year event-free survival (EFS) [ (31.4±4.5) % vs (86.5±5.3) %, P<0.001] of the nonresponders at 6 months were significantly lower than those of the response group. Conclusion: Residual hematopoietic indicators at 3 months after IST are prognostic parameters. The improved value of the reticulocyte count could reflect whether the bone marrow hematopoiesis is recovering and the degree of recovery. A second treatment could be performed sooner for patients with a very low ARC(△).

Characteristics of bone marrow compensatory erythropoiesis in hereditary spherocytosis / 中华血液学杂志

Objective: To reveal the compensatory features of bone marrow (BM) erythropoiesis in hereditary spherocytosis (HS) and to explore the effect of diferent hemoglobin levels on this compensation. Methods: Clinical and laboratory data of patients with HS were collected, and the peripheral blood absolute reticulocytes counts value was taken as the surrogate parameter to evaluate the ability of erythropoiesis compensation. BM erythropoiesis compensation in HS with diferent degrees of anemia were evaluated. Results: ①Three hundred and two patients were enrolled, including 115 with compensated hemolytic disease, 74 with mild anemia, 90 with moderate anemia, and 23 with severe anemia. ②Hemoglobin (HGB) was negatively correlated with serum erythropoietin in the decompensated hemolytic anemia group (EPO; rs=-0.585, P<0.001) . ③The median absolute reticulocyte count (ARC) of HS patients was 0.34 (0.27, 0.44) ×10(12)/L, up to 4.25 times that of normal people. The maximum ARC was 0.81×10(12)/L, about 10 times that of normal people. The median ARC of patients with compensated hemolytic disease was 0.29 (0.22, 0.38) ×10(12)/L, up to 3.63 times that of normal people. The median ARC of patients with hemolytic anemia was 0.38 (0.30, 0.46) ×10(12)/L, which was significantly higher than the patients with compensated hemolytic disease, up to 4.75 times that of normal people (z=4.999, P=0.003) . ④ ARC was negatively correlated with HGB in the compensated hemolytic disease group (rs=-0.177, P=0.002) and positively correlated with HGB in the decompensated hemolytic anemia group (rs=0.191, P=0.009) . There was no significant difference in the ARC among patients with mild, moderate, and severe anemia (χ(2)=4.588, P=0.101) . ⑤The median immature reticulocyte production index of the mild, moderate, and severe anemia groups was 13.1% (9.1%, 18.4%) , 17.0% (13.4%, 20.8%) , and 17.8% (14.6%, 21.8%) , respectively; the mild anemia group had lower index values than the moderate and severe anemia groups (P(adj) values were both<0.05) , but there was no significant difference between the latter groups (P(adj)=1.000) . The median immature reticulocyte count of patients in the mild, moderate, and severe groups was 5.09 (2.60, 7.74) ×10(10)/L, 6.24 (4.34, 8.83) ×10(10)/L, and 7.00 (3.07, 8.22) ×10(10)/L, respectively; there was no significant difference among the groups (χ(2)=3.081, P=0.214) . Conclusion: HGB can be maintained at a normal level through bone marrow erythropoiesis, while red blood cells are reduced in HS. However, once anemia develops, the bone marrow exerts its maximum erythropoiesis capacity and does not increase, regardless of anemia aggravation or serum EPO increase.

Efficacy of transcatheter arterial embolization and laparotomy in the treatment of severe liver injury: a comparative study / 中华创伤杂志

Objective:To compare the efficacy of transcatheter arterial embolization (TAE) with laparotomy in the treatment of severe liver injury.Methods:A retrospective cohort study was conducted to analyze the clinical data of 48 patients with severe liver injury admitted to 909th Hospital of Joint Logistics Support Force (Affiliated Dongnan Hospital of Xianmen University Medical College) from December 2013 to June 2020, including 28 males and 20 females; aged 16-75 years [(45.7±6.2)years]. There were 25 patients with grade III, 15 grade IV and 8 grade V according to the American Association for the Surgery of Trauma (AAST) classification. After general treatments such as infusion and hemostasis, TAE was performed in 26 patients (TAE group) and laparotomy in 22 patients (laparotomy group). The operation time and length of hospital stay were compared between the two groups. Erythrocyte, hemoglobin and serum creatinine were compared before operation and at postoperative 1 day. Alanine aminotransferase (ALT) and aspartate aminotransferase (AST) were analyzed before operation and at postoperative 1, 3, 7 days. Complications were observed.Results:All patients were followed up for 12-60 months [(17.1±9.1)months]. The operation time and length of hospital stay were (65.7±9.2)minutes and (21.6±6.6)days in TAE group, significantly shorter than (162.5±28.1)minutes and (31.5±7.4)days in laparotomy group ( P<0.05 or 0.01). There was no significant difference between the two groups referring to erythrocyte, hemoglobin and serum creatinine before operation and at postoperative 1 day (all P>0.05). There was no significant difference in ALT and AST between the two groups before operation (all P>0.05). TAE group showed ALT level of 1 154(884, 1 698)U/L, (975.3±400.9)U/L and (403.4±232.9)U/L at postoperative 1, 3, 7 days, significantly lower than 2 053(1 965, 2 132)U/L, (1 604.1±188.2)U/L and (915.3±160.5)U/L in laparotomy group (all P<0.05). TAE group showed AST level of (1 313.2±542.0)U/L, 525(302, 971)U/L and 174(84, 324)U/L at postoperative 1, 3, 7 days, significantly lower than (1 962.9±245.4)U/L, 1 478(1 089, 1 677)U/L and 837(674, 1 006)U/L in laparotomy group ( P<0.05 or 0.01). The complication rate was 26.9% (7/26) in TAE group, significantly lower than 59.1% (13/22) in laparotomy group ( P<0.05). Conclusion:For severe liver injury, TAE can significantly shorten operation time and length of hospital stay, accelerate the recovery of liver function and reduce the complication rate in comparison with laparotomy.

External validation and comparison of different prediction models of postoperative nausea and vomiting in elderly patients with thoracoscopic partial pneumonectomy / 中国实用护理杂志

Objective:To investigate and compare the predictive effects of two postoperative nausea and vomiting (PONV) prediction models in elderly patients with thoracoscopic partial pneumonectomy.Methods:The total of 227 elderly patients who underwent thoracoscopic partial pneumonectomy in Brain Hospital Affiliated to Nanjing Medical University from October 2021 to January 2022 were collected. Apfel risk score and Koivuranta risk score were used to predict the risk of PONV in each patient. The area under the ROC curve was used to evaluate the discrimination ability of the two prediction models; Hosmer-Lemeshow goodness of fit test was used to evaluate the calibration of the two prediction models.Results:Among 227 patients, 74 had PONV, and the incidence of PONV was 32.6%. The area under the ROC curve of Apfel and Koivuranta risk scores in the risk prediction of PONV in elderly patients with thoracoscopic partial pneumonectomy were 0.640 and 0.683 respectively. There was no significant difference between the two ( Z=1.54, P>0.05). The Hosmer-Lemeshow goodness of fit test showed that the accuracy of the two models in predicting PONV risk in elderly patients with thoracoscopic partial pneumonectomy was better ( P>0.05). Conclusions:The difference between the two models in PONV risk prediction in elderly patients with thoracoscopic partial pneumonectomy is general, Koivuranta model is more suitable for predicting PONV in elderly patients with thoracoscopic partial pneumonectomy, but its applicability is still insufficient.

Clinical study of warm needling moxibustion plus intra-articular injection of sodium hyaluronate for hip involvement in ankylosing spondylitis / 针灸推拿医学（英文版）

Objective: To observe the efficacy of warm needling moxibustion plus intra-articular injection of sodium hyaluronate for hip involvement in ankylosing spondylitis (AS). Methods: A total of 60 patients with hip involvement in AS were randomly divided into a control group and an observation group, with 30 cases in each group. The patients in the control group were given an intra-articular injection of sodium hyaluronate, once a week. The patients in the observation group were given additional warm needling moxibustion, once a day, with a 2-day interval after five consecutive days of treatment. After 5 weeks, changes in such indicators as visual analog scale (VAS) score, Harris score, Bath ankylosing spondylitis disease activity index (BASDAI), Bath ankylosing spondylitis functional index (BASFI), serum cartilage oligomeric matrix protein (COMP), interleukin (IL)-17 were observed, and the efficacy was evaluated. Six months after treatment, Bath ankylosing spondylitis radiology index-hip (BASRI-hip) was evaluated. Results: After treatment, the total effective rate in the observation group was higher than that in the control group (P<0.05). After treatment, the VAS scores in both groups were lower than those before treatment (P<0.05), and the score of the observation group was lower than that of the control group (P<0.05). After treatment, the Harris scores of both groups were higher than those before treatment (P<0.05), and the score of the observation group was higher than that of the control group (P<0.05). Six months after treatment, the BASRI-hip score of the control group was higher than that before treatment (P<0.05), while the score of the observation group was not significantly different from that before treatment (P>0.05) and was lower than that of the control group (P<0.05). After treatment, the scores of BASDAI and BASFI of both groups were lower than those before treatment (P<0.05), and the scores of the observation group were lower than those of the control group (P<0.05). After treatment, the levels of serum COMP and IL-17 of both groups were lower than those before treatment (P<0.05), and the levels of the observation group were lower than those of the control group (P<0.05). Conclusion: The efficacy of warm needling moxibustion plus intra-articular injection of sodium hyaluronate for hip involvement in AS is better than the intra-articular injection of sodium hyaluronate alone. This combined approach can alleviate hip pain, improve hip functions, delay the destruction of the hip, prevent AS development, and reduce the levels of serum COMP and IL-17.

Main Factors Influencing the Platelet Spreading / 中国实验血液学杂志

OBJECTIVE@#To explore the main factors of platelet spreading and provide the foundation for related research.@*METHODS@#Platelets (2×107/ml) were draw from C57BL/6J mouse and kept at 22 ℃ for 1-2 hours. Platelets (2×107/ml) were were allowed to adhere and spread on the fibrinogen-coated slides, after staining F-actin in platelets, the platelets were observed with the confocal microscopy. The effects of different concentrations of fibrinogen (10 μg/ml, 30 μg/ml, 100 μg/ml) and kinds of agonists ［thrombin(0.01,0.05,0.1 U/ml), ADP（5,10,20 μmol/L）, U46619（0.125,0.25,0.5 μmol/L）］ on platelets were analyzed. The platelet spreading was successful if the spreading rate was higher after treated with agonists.@*RESULTS@#Compared to the group which coated with 10 μg/ml and 100 μg/ml fibrinogen, the platelet density is optimal when coated with 30 μg/ml fibrinogen. In addition, under the stimulation of thrombin, ADP and U46619, the spreading rate of platelets showed a certain concentration-dependent increasing.@*CONCLUSION@#The platelet spreading is easily influenced by various factors, the platelet spreading can be induced successfully at 0.1 U/ml thrombin, 20 μmol/L ADP and 0.5 μmol/L U46619 on the slide coated with 30 μg/ml fibrinogen.

Integrative analysis of prognostic long non-coding RNAs with copy number variation in bladder cancer / 浙江大学学报（英文版）（B辑：生物医学和生物技术）

Copy number variations (CNVs), which can affect the role of long non-coding RNAs (lncRNAs), are important genetic changes seen in some malignant tumors. We analyzed lncRNAs with CNV to explore the relationship between lncRNAs and prognosis in bladder cancer (BLCA). Messenger RNA (mRNA) expression levels, DNA methylation, and DNA copy number data of 408 BLCA patients were subjected to integrative bioinformatics analysis. Cluster analysis was performed to obtain different subtypes and differently expressed lncRNAs and coding genes. Weighted gene co-expression network analysis (WGCNA) was performed to identify the co-expression gene and lncRNA modules. CNV-associated lncRNA data and their influence on cancer prognosis were assessed with Kaplan-Meier survival curve. Multi-omics integration analysis revealed five prognostic lncRNAs with CNV, namely

Relationship between Changes of Lymphocyte Subsets and Early Hematologic Response in Immunosuppressive Therapy of Severe Aplastic Anemia Patients / 中国实验血液学杂志

OBJECTIVE@#To explore the relationship between the change of lymphocyte subsets before and after immunosuppressive therapy (IST) with disease severity of severe aplastic anemia (SAA) and hematologic response to IST.@*METHODS@#The clinical data of 94 patients with SAA/VSAA treated by r-ATG and CsA in our hospital from December 2009 to October 2011 was analyzed retrospectively. Among them, 26 patients who had sequential data of lymphocyte subsets and cytokines before and after treatment were enrolled. The relationship between lymphocyte subsets, cytokine level before IST and disease severity, as well as the relationship between changes if lymphocyte subsets, changes of cytokine and the HR after IST for 6 months was analyzed.@*RESULTS@#There were no statistical differences in the ratio and absolute count of lymphocyte, the ratio and absolute count of each lymphocyte subsets, including CD3@*CONCLUSION@#The hematopoietic recovery and early hematologic remission may be affected by the intensity of immune suppression reflected from the changes of lymphocyte subsets and the immune reconstruction reflected from the recovery of lymphocyte subsets. The immune reconstruction is most significant within 3 months after IST.

Generalized lipodystrophy type 1 due to compound heterozygous mutation of AGPAT2 gene: One case report and literature review / 中华内分泌代谢杂志

Congenital generalized lipodystrophy type 1 (CGL1) is an autosomal recessive genetic disease caused by mutations in AGPAT2 gene. The main clinical mainifestations include body subcutaneous fat loss, muscle hypertrophy, obvious subcutaneous veins, pseudoacromegaly, hirsutism, and acanthosis nigricans. What′s more, CGL1 is always accompanied by metabolic diseases. Therefore, it is easily misdiagnosed as metabolic syndrome, type 2 diabetes, polycystic ovary syndrome, acromegaly, or Cushing′s syndrome. Meanwhile, it is difficult to distinguish it from partial lipoatrophy syndrome. In this article, we present clinical and molecular characteristics of a patient with CGL1 and review mutations reported in literature to replenish current knowledge about this orphan disease.

Research progress on influencing factors and nursing intervention of thirst in critical patients / 中国实用护理杂志

Thirst is one of the common complaints of critically ill patients, persistent and intense thirst is closely related to a variety of negative emotions, such as anxiety, depression, etc., which can cause restlessness, sleep disorders, and reduce the quality of life; at the same time, it can cause bacterial growth in the patient′s oral cavity, increasing the risk of secondary infection, thereby delaying the recovery of the disease and prolonging the length of ICU hospitalization. Therefore, this article reviews the current status, risk factors, and intervention strategies of thirst in critically ill patients in order to provide a reference and basis for medical staff to carry out thirst intervention measures in critically ill patients.

The role of blood ammonia in the prognosis evaluation of septic patients in the emergency department / 中华急诊医学杂志

Objective:To investigate the role of blood ammonia in the evaluation of the prognosis of septic patients in the emergency department and to compare its value with mortality in emergency department sepsis (MEDS) score.Methods:A retrospective clinical study was conducted to septic patients who were diagnosed in the Emergency Department of West China Hospital of Sichuan University from June 2017 to May 2018, and met the diagnostic criteria established by the diagnostic criteria of the American College of Chest Physicians/Society of Critical Care Medicine in 2001. The subjects who had other diseases that affected blood ammonia level and were lost to follow-up were excluded. MEDS scores were collected, and the survival status of patients was followed up by telephone. The independent samples t test was used to compare the differences between the two groups, receiver operating characteristic (ROC) curve was used to assess the accuracy of the prediction of sepsis mortality, and the logistic regression model was used to explore the value of the combined use of blood ammonia and MEDS score.Results:Eighty subjects were finally included in the study and divided into the 1-week survival group ( n=52), 1-week death group ( n=28); 4-week survival group ( n=37), 4-week death group ( n=43); 12-week survival group ( n=33), 12-week death group ( n=47); 1-year survival group ( n=32), and 1-year death group ( n=48). There was no statistical difference in the demographic characteristics of subjects between the groups. The average blood ammonia level of all the subjects who died was higher than that of the patients who survived in the same period [(116.57 ± 85.33) μmol/L vs (77.63 ± 35.82) μmol/L, (108.53 ± 73.00) μmol/L vs (71.19 ± 32.53) μmol/L, (106.74 ± 71.59) μmol/L vs (69.21 ± 28.84) μmol/L, (105.77 ± 71.14) μmol/L vs (69.50 ± 29.25) μmol/L, P<0.05]. Based on death after one week, four weeks, twelve weeks and one year, the area under ROC curve (AUC) of blood ammonia was 0.668 (95% CI: 0.542-0.793, P=0.014), 0.706 (95% CI: 0.593-0.819, P=0.002), 0.705 (95% CI: 0.592-0.818, P=0.002), and 0.697 (95% CI: 0.582-0.811, P=0.003), respectively. Compared with the use of blood ammonia, lactic acid or MEDS score alone, the combined use of blood ammonia and MEDS score increased the accuracy of prognosis evaluation in sepstic patients ( P<0.05). Conclusions:Blood ammonia has a high value in predicting the short-term and 1-year prognosis of septic patients in the emergency department. The combined use of blood ammonia and MEDS score can further improve its predictive value.