Objective: To understand the current status of diagnosis and treatment of chronic lymphocytic leukemia (CLL) /small lymphocytic lymphoma (SLL) among hematologists, oncologists, and lymphoma physicians from hospitals of different levels in China. Methods: This multicenter questionnaire survey was conducted from March 2021 to July 2021 and included 1,000 eligible physicians. A combination of face-to-face interviews and online questionnaire surveys was used. A standardized questionnaire regarding the composition of patients treated for CLL/SLL, disease diagnosis and prognosis evaluation, concomitant diseases, organ function evaluation, treatment selection, and Bruton tyrosine kinase (BTK) inhibitor was used. Results: ①The interviewed physicians stated that the proportion of male patients treated for CLL/SLL is higher than that of females, and the age is mainly concentrated in 61-70 years old. ②Most of the interviewed physicians conducted tests, such as bone marrow biopsies and immunohistochemistry, for patient diagnosis, in addition to the blood test. ③Only 13.7% of the interviewed physicians fully grasped the initial treatment indications recommended by the existing guidelines. ④In terms of cognition of high-risk prognostic factors, physicians' knowledge of unmutated immunoglobulin heavy-chain variable and 11q- is far inferior to that of TP53 mutation and complex karyotype, which are two high-risk prognostic factors, and only 17.1% of the interviewed physicians fully mastered CLL International Prognostic Index scoring system. ⑤Among the first-line treatment strategy, BTK inhibitors are used for different types of patients, and physicians have formed a certain understanding that BTK inhibitors should be preferentially used in patients with high-risk factors and elderly patients, but the actual use of BTK inhibitors in different types of patients is not high (31.6%-46.0%). ⑥BTK inhibitors at a reduced dose in actual clinical treatment were used by 69.0% of the physicians, and 66.8% of the physicians had interrupted the BTK inhibitor for >12 days in actual clinical treatment. The use of BTK inhibitors is reduced or interrupted mainly because of adverse reactions, such as atrial fibrillation, severe bone marrow suppression, hemorrhage, and pulmonary infection, as well as patients' payment capacity and effective disease progression control. ⑦Some differences were found in the perceptions and behaviors of hematologists and oncologists regarding the prognostic assessment of CLL/SLL, the choice of treatment options, the clinical use of BTK inhibitors, etc. Conclusion: At present, a gap remains between the diagnosis and treatment of CLL/SLL among Chinese physicians compared with the recommendations in the guidelines regarding the diagnostic criteria, treatment indications, prognosis assessment, accompanying disease assessment, treatment strategy selection, and rational BTK inhibitor use, especially the proportion of dose reduction or BTK inhibitor discontinuation due to high adverse events.
Female ; Humans ; Male ; Aged ; Middle Aged ; Leukemia, Lymphocytic, Chronic, B-Cell/drug therapy* ; Prognosis ; Lymphoma, B-Cell ; Immunohistochemistry ; Immunoglobulin Heavy Chains/therapeutic use*

Humans ; Asthma/drug therapy* ; Biological Therapy

OBJECTIVES: To investigate the incidence of extrauterine growth retardation (EUGR) and its risk factors in very preterm infants (VPIs) during hospitalization in China. METHODS: A prospective multicenter study was performed on the medical data of 2 514 VPIs who were hospitalized in the department of neonatology in 28 hospitals from 7 areas of China between September 2019 and December 2020. According to the presence or absence of EUGR based on the evaluation of body weight at the corrected gestational age of 36 weeks or at discharge, the VPIs were classified to two groups: EUGR group (n=1 189) and non-EUGR (n=1 325). The clinical features were compared between the two groups, and the incidence of EUGR and risk factors for EUGR were examined. RESULTS: The incidence of EUGR was 47.30% (1 189/2 514) evaluated by weight. The multivariate logistic regression analysis showed that higher weight growth velocity after regaining birth weight and higher cumulative calorie intake during the first week of hospitalization were protective factors against EUGR (P<0.05), while small-for-gestational-age birth, prolonged time to the initiation of total enteral feeding, prolonged cumulative fasting time, lower breast milk intake before starting human milk fortifiers, prolonged time to the initiation of full fortified feeding, and moderate-to-severe bronchopulmonary dysplasia were risk factors for EUGR (P<0.05). CONCLUSIONS It is crucial to reduce the incidence of EUGR by achieving total enteral feeding as early as possible, strengthening breastfeeding, increasing calorie intake in the first week after birth, improving the velocity of weight gain, and preventing moderate-severe bronchopulmonary dysplasia in VPIs.
Female ; Fetal Growth Retardation ; Gestational Age ; Hospitalization ; Humans ; Incidence ; Infant ; Infant, Newborn ; Infant, Premature ; Infant, Very Low Birth Weight ; Prospective Studies ; Risk Factors

Objective:To analyze the risk factors of bronchopulmonary dysplasia(BPD)in very preterm infants(VPI), and to provide scientific basis for the prevention and treatment of BPD in VPI.Methods:A prospective multicenter study was designed to collect the clinical data of VPI in department of neonatology of 28 hospitals in 7 regions from September 2019 to December 2020.According to the continuous oxygen dependence at 28 days after birth, VPI were divided into non BPD group and BPD group, and the risk factors of BPD in VPI were analyzed.Results:A total of 2 514 cases of VPI including 1 364 cases without BPD and 1 150 cases with BPD were enrolled.The incidence of BPD was 45.7%.The smaller the gestational age and weight, the higher the incidence of BPD( P<0.001). Compared with non BPD group, the average birth age, weight and cesarean section rate in BPD group were lower, and the incidence of male infants, small for gestational age and 5-minute apgar score≤7 were higher( P<0.01). In BPD group, the incidences of neonatal respiratory distress syndrome(NRDS), hemodynamically significant patent ductus arteriosus, retinopathy of prematurity, feeding intolerance, extrauterine growth restriction, grade Ⅲ~Ⅳ intracranial hemorrhage, anemia, early-onset and late-onset sepsis, nosocomial infection, parenteral nutrition-associated cholestasis were higher( P<0.05), the use of pulmonary surfactant(PS), postnatal hormone exposure, anemia and blood transfusion were also higher, and the time of invasive and non-invasive mechanical ventilation, oxygen use and total hospital stay were longer( P<0.001). The time of starting enteral nutrition, cumulative fasting days, days of reaching total enteral nutrition, days of continuous parenteral nutrition, days of reaching 110 kcal/(kg·d) total calorie, days of reaching 110 kcal/(kg·d) oral calorie were longer and the breastfeeding rate was lower in BPD group than those in non BPD group( P<0.001). The cumulative doses of amino acid and fat emulsion during the first week of hospitalization were higher in BPD group( P<0.001). Multivariate Logistic regression analysis showed that NRDS, invasive mechanical ventilation, age of reaching total enteral nutrition, anemia and blood transfusion were the independent risk factors for BPD in VPI, and older gestational age was the protective factor for BPD. Conclusion:Strengthening perinatal management, avoiding premature delivery and severe NRDS, shortening the time of invasive mechanical ventilation, paying attention to enteral nutrition management, reaching whole intestinal feeding as soon as possible, and strictly mastering the indications of blood transfusion are very important to reduce the incidence of BPD in VPI.

Objective: To explore the heterogeneity and correlation of clinical phenotypes and genotypes in children with disorders of sex development (DSD). Methods: A retrospective study of 1 235 patients with clinically proposed DSD in 36 pediatric medical institutions across the country from January 2017 to May 2021. After capturing 277 DSD-related candidate genes, second-generation sequencing was performed to analyzed the heterogeneity and correlation combined with clinical phenotypes. Results: Among 1 235 children with clinically proposed DSD, 980 were males and 255 were females of social gender at the time of initial diagnosis with the age ranged from 1 day of age to 17.92 years. A total of 443 children with pathogenic variants were detected through molecular genetic studies, with a positive detection rate of 35.9%. The most common clinical phenotypes were micropenis (455 cases), hypospadias (321 cases), and cryptorchidism (172 cases) and common mutations detected were in SRD5A2 gene (80 cases), AR gene (53 cases) and CYP21A2 gene (44 cases). Among them, the SRD5A2 mutation is the most common in children with simple micropenis and simple hypospadias, while the AMH mutation is the most common in children with simple cryptorchidism. Conclusions: The SRD5A2 mutation is the most common genetic variant in Chinese children with DSD, and micropenis, cryptorchidism, and hypospadias are the most common clinical phenotypes. Molecular diagnosis can provide clues about the biological basis of DSD, and can also guide clinicians to perform specific clinical examinations. Target sequence capture probes and next-generation sequencing technology can provide effective and economical genetic diagnosis for children with DSD.
3-Oxo-5-alpha-Steroid 4-Dehydrogenase/genetics* ; Child ; China/epidemiology* ; Cryptorchidism/genetics* ; Disorders of Sex Development/genetics* ; Female ; Genital Diseases, Male ; Genotype ; Humans ; Hypospadias/genetics* ; Male ; Membrane Proteins/genetics* ; Penis/abnormalities* ; Phenotype ; Retrospective Studies ; Steroid 21-Hydroxylase/genetics*

OBJECTIVE: To investigate the incidence rate and risk factors for metabolic bone disease of prematurity (MBDP) in very low birth weight/extremely low birth weight (VLBW/ELBW) infants. METHODS: The medical data of 61 786 neonates from multiple centers of China between September 1, 2013 and August 31, 2016 were retrospectively investigated, including 504 VLBW/ELBW preterm infants who met the inclusion criteria. Among the 504 infants, 108 infants diagnosed with MBDP were enrolled as the MBDP group and the remaining 396 infants were enrolled as the non-MBDP group. The two groups were compared in terms of general information of mothers and preterm infants, major diseases during hospitalization, nutritional support strategies, and other treatment conditions. The multivariate logistic regression analysis was used to investigate the risk factors for MBDP. RESULTS: The incidence rate of MBDP was 19.4% (88/452) in VLBW preterm infants and 38.5% (20/52) in ELBW preterm infants. The incidence rate of MBDP was 21.7% in preterm infants with a gestational age of < 32 weeks and 45.5% in those with a gestational age of < 28 weeks. The univariate analysis showed that compared with the non-MBDP group, the MBDP group had significantly lower gestational age and birth weight, a significantly longer length of hospital stay, and a significantly higher incidence rate of extrauterine growth retardation ( CONCLUSIONS A lower gestational age, hypocalcemia, extrauterine growth retardation at discharge, and neonatal sepsis may be associated an increased risk of MBDP in VLBW/ELBW preterm infants. It is necessary to strengthen perinatal healthcare, avoid premature delivery, improve the awareness of the prevention and treatment of MBDP among neonatal pediatricians, and adopt positive and reasonable nutrition strategies and comprehensive management measures for preterm infants.
Birth Weight ; Bone Diseases, Metabolic/etiology* ; China/epidemiology* ; Female ; Humans ; Infant ; Infant, Extremely Low Birth Weight ; Infant, Newborn ; Infant, Premature ; Infant, Very Low Birth Weight ; Pregnancy ; Retrospective Studies ; Risk Factors

Objective: To explore the influencing factors for serum potassium >4.4 mmol/L in the morning of parathyroidectomy in hemodialysis patients with secondary hyperparathyroidism (SHPT). Methods: The clinical data of 72 patients with SHPT who received regular hemodialysis and underwent parathyroidectomy in Guangdong Provincial People's Hospital from January 2012 to December 2018 were analyzed retrospectively. There were 37 males and 35 females, aged from 25 to 69 years, and the dialysis timespan was from 0.5 to 11 years. The levels of parathyroid hormone, serum potassium and serum calcium before hemodialysis were examined one day before operation, and hemodialysis time and dewatering volume after hemodialysis without heparin were recorded, and also the level of serum potassium in the morning of parathyroidectomy was detected. The occurrences of hyperkalemia during and after operation were studied. The factors related to hyperkalemia in the morning of parathyroidectomy were evaluated by Pearson or Spearman correlation analysis, and the cut-off values of risk factors were calculated by receiver operating characteristic (ROC) curve. Results: Serum potassium >4.4 mmol/L in the morning of parathyroidectomy existed in 23 of 72 patients. Correlation analysis showed that serum potassium one day before operation ((4.93±0.56)mmol/L, r=0.656, P<0.001) and dehydration volume ((2.37±0.75)L, r=0.261, P=0.027) were positively correlated with serum potassium in the morning of parathyroidectomy((4.16±0.54)mmol/L). Serum potassium before hemodialysis one day before operation was a main predictor for serum potassium in the morning of parathyroidectomy (AUC=0.791, P<0.001). The cut-off value of serum potassium before hemodialysis one day before operation was 5.0 mmol/L. Conclusion: Serum potassium before hemodialysis one day before operation in patients with SHPT can predict serum potassium in the morning of parathyroidectomy, offering imformation for the safety of operation.
Calcium ; Female ; Humans ; Hyperkalemia/etiology* ; Hyperparathyroidism, Secondary/surgery* ; Male ; Parathyroid Hormone ; Parathyroidectomy ; Renal Dialysis ; Retrospective Studies

Objective:To investigate the clinical efficacy of ex vivo liver resection and autotransplantation (ELRA) for liver complex space-occupying lesions.Methods:The retrospective and descriptive study was conducted. The clinicopathological data of 50 patients with liver complex space-occupying lesions who underwent ELRA in the First Hospital Affiliated to Army Medical University between June 2009 and May 2017 were collected. There were 36 males and 14 females, aged from 13 to 69 years, with a median age of 51 years. All patients underwent ELRA. Observation indicators: (1) surgical situations; (2) postoperative situations; (3) follow-up. Follow-up was conducted by outpatient examination or telephone interview. Patients were followed up according to the individual follow-up plan in the first 6 months after discharge, and then once every 3 to 6 months to detect tumor recurrence and survival up to May 2019. Measurement data with normal distribution were represented as Mean± SD. Measurement data with skewed distribution were represented as M (range). Count data were expressed as absolute numbers or percentages. The Kaplan-Meier method was used to calculate survival rates and draw survival curves, and Log-rank test was used for survival analysis. Results:(1) Surgical situations: all the 50 patients underwent ELRA successfully, and postoperative pathological examination showed the R 0 resection rate was 100%(50/50). The operation time of the 50 patients were (630±186)minutes, of which 9 patients with liver benign occupation had the operation time of (684±168)minutes and 41 patients with liver malignant tumor had the operation time of (618±190)minutes. The operation time of temporary reconstruction of inferior vena cava and portacaval shunt, time of anhepatic phase, volume of intraoperative blood loss of the 50 patients were (35±9)minutes, (256±71)minutes, 2 000 mL(range, 400-10 000 mL), respectively. The remnant liver mass to standard liver mass ratio of the 50 patients was 65%±16%, of which 9 patients with liver benign occupation had the remnant liver mass to standard liver mass ratio of 63%±14% and 41 patients with liver malignant tumor had the remnant liver mass to standard liver mass ratio of 65%±17%. Of the 50 patients, 35 had vascular invasion (7 cases with liver benign occupation, 28 cases with liver malignant tumor), of which 24 (6 cases with liver benign occupation, 18 cases with liver malignant tumor) underwent in vitro vascular reconstruction, 12 (5 cases with liver benign occupation, 7 cases with liver malignant tumor) had bile duct invasion and underwent choledochojejunostomy due to the inability of the common bile duct to anastomose the ends. Two cases with liver metastasis of gastric cancer, one case with liver metastasis of colon cancer and one case with liver metastasis of pancreatic cancer underwent radical gastrectomy, radical resection of colon cancer, and pancreaticoduodenectomy, respectively. (2) Postoperative situations: the duration of postoperative hospital stay of the 50 patients were 25 days (range, 11-169 days). Of the 50 patients, 12 had pleural effusion who were treated with pleural puncture drainage, 10 had bile leakage who were treated with abdominal puncture drainage, 3 had bile duct anastomotic leakage who were treated with endoscopic nasobiliary drainage or biliary stent implantation, 6 underwent reoperation among which 4 underwent exploratory laparotomy due to abdominal hemorrhage, 1 underwent portal vein reconstruction due to abdominal hemorrhage combined with portal vein thrombosis, and 1 underwent salvage liver transplantation due to liver failure. Nine of the 50 patients died within 90 days after surgery, all of whom had liver malignant tumor. Among them, 3 died of multi-organ dysfunction syndrome caused by severe infection, 3 died of acute liver failure, 2 died of abdominal hemorrhage and 1 died pulmonary embolism. (3) Follow-up: all the 50 patients were followed up for 1 to 119 months. The overall survival time, 1-, 3-, 5-year overall and tumor-free survival rates of the 50 patients after operation were 17 months (range, 1-119 months), 68.0%, 45.9%, 41.1% and 41.9%, 33.4%, 30.8%, respectively. The overall survival time, 1-, 3-, 5-year overall and tumor-free survival rates of the 9 patients who with liver benign occupation after operation were 68 months (range, 10-114 months), 88.9%, 88.9%, 88.9% and 88.9%, 88.9%, 88.9%, respectively. The overall survival time, 1-, 3-, 5-year overall and tumor-free survival rates of the 41 patients who with liver malignant tumor after operation were 15 months (range, 1-119 months), 63.4%, 36.6%, 31.0% and 31.5%, 21.0%, 18.0%, respectively. There were significant differences in the overall and tumor-free survival rates between patients who with liver benign occupation and patients who with liver malignant tumor ( χ2=7.626, 11.766, P<0.05). Conclusions:ELRA can be applied in the treatment of liver complex space-occupying lesions. The selection criteria of patients with liver malignant tumor should be more rigorous to reduce perioperative mortality.

OBJECTIVE: To evaluate the protective effects of salvianolate on percutaneous coronary intervention (PCI) related myocardial injury or myocardial infarction after elective PCI in non-ST-segment elevation acute coronary syndrome (NSTE-ACS) patients. METHODS: A total of 149 patients with NSTE-ACS who underwent elective PCI were enrolled. The patients were randomly allocated in a 1:1 ratio to the salvianolate group (74 cases) or the control group (75 cases). After exclusion criteria of coronary angiography, 60 patients with PCI therapy remained in the salvianolate group and 68 in the control group. The incidence and the severity of PCI related myocardial injury or myocardial infarction, in addition to major adverse cardiac events (MACEs) during 1 year follow-up after PCI were studied between the two groups. Multivariate logistic regression analysis was used to determine the independent factors for PCI related myocardial injury or myocardial infarction after elective PCI. RESULTS: Compared with the control group, salvianolate treatment reduced the incidence of PCI related severe myocardial injury or myocardial infarction (11.7% vs. 26.5%, P=0.035). The rate of MACEs or all-cause death within 1 month or 1 year after the procedure was not significantly different between the two groups. CONCLUSIONS Periprocedural treatment with salvianolate reduces the incidence of PCI related severe myocardial injury or myocardial infarction, although it does not influence clinical prognosis. [Chinese clinical trial registry: ChiCTR1800016992].