Objective To explore the impact of pH value of the reaction system on the properties of bovine hemoglobin modified with aldehydeated polyethylene glycol(PEG-bHb).Methods PEG-bHb conjugates were synthesized under varying pH conditions(6.0,6.5,7.4 and 8.0)of the reaction system while consistent molar ratios,temperature,and reaction time were maintained.The structural and functional attributes of PEG-bHb were characterized.Results The proportion of methemoglobin decreased with an increase in pH.In a weakly acidic reaction environment,the PEG-bHb was found to be relatively highly modified.At pH 6.5,the average number of PEG chains attached to the bHb surface was 6.86±0.38.Selective PEG modification of the N-terminal α-NH2 groups was more pronounced under weakly acidic conditions.Specifically,at pH 6.5,the modification efficiency of the N-terminal α-NH2 groups of bHb by aldehyde-activated PEG reached 95.4％for the α-chains and 99.3％for the β-chains.The PEG modification influenced the heme region microenvi-ronment of bHb,with minimal structural impact observed at pH 6.5.After modification,the oxygen affinity of PEG-bHb was enhanced,the Hill coefficient was reduced,and there were significant increases in colloid osmotic pressure,viscosity,and particle size,all of which differed markedly from the unmodified bHb group(P＜0.001).Conclusion The synthesis of PEG-bHb under weakly acidic conditions can result in a high degree of selective modification of the N-terminal α-NH2 groups and an overall high degree of modification.

Patients with diabetic retinopathy often experience diabetes distress when facing the challenges of the disease, which has adverse effects on their health outcomes. This paper summarizes the current situation of diabetes distress in patients with diabetic retinopathy, and the assessment tools, influencing factors, and intervention measures, aiming to provide a reference for healthcare professionals to conduct intervention studies.

Objective:To summarize the clinical features of 8 cases with intramycardial hematoma(IMH) following congenital cardiac surgery in children.Methods:We retrospectly searched 8 patients with intramycardial hematoma after congenital cardiac surgery in Guangzhou Women and Children’s Medical Center from 2008 to 2024.Results:Mean age and mean weight at surgery were(13±15) months and(7.8±3.5)kg respectively. 6 of 8 cases were interventricular septal hematoma. In the other 2 patients, intramycardial hematoma was located in left ventrical free wall. All IMH were postoperatively detected by transthoracic echocardiaography. Two patients were managed with ECMO intraoperatively. Finally, all patients were discharged successfully with good clinical results. Mean time to IMH resolution in six patients was(33.5±4.6) days and mean left ventricular ejection fraction(LVEF) was 0.60±0.09. Another patient was followed up for 3 months and the IMH was not absorbed. One patient was lost to follow-up.Conclusion:IMH is a rare complication after congenital heart disease. The absorption of hematoma is a dynamic process and mean time to IMH resolution is about 1month. In IMH patients with hemodynamic instability, ECMO can be a good treatment to create opportunities for hematoma absorbtion.

OBJECTIVE: To explore the clinical characteristics and genetic etiology of a consanguineous Chinese pedigree affected with Congenital coagulation factor XII (XII) deficiency. METHODS: Members of the pedigree who had visited Ruian People's Hospital on July 12, 2021 were selected as the study subjects. Clinical data of the pedigree were reviewed. Peripheral venous blood samples were taken from the subjects. Blood coagulation index and genetic testing were carried out. Candidate variant was verified by Sanger sequencing and bioinformatic analysis. RESULTS: This pedigree has comprised 6 individuals from 3 generations, including the proband, his father, mother, wife, sister and son. The proband was a 51-year-old male with kidney stones. Blood coagulation test showed that his activated partial thromboplastin time (APTT) was significantly prolonged, whilst the FXII activity (FXII:C) and FXII antigen (FXII:Ag) were extremely reduced. The FXII:C and FXII:Ag of proband's father, mother, sister and son have all reduced to about half of the lower limit of reference range. Genetic testing revealed that the proband has harbored homozygous missense variant of c.1A>G (p.Arg2Tyr) of the start codon in exon 1 of the F12 gene. Sanger sequencing confirmed that his father, mother, sister and son were all heterozygous for the variant, whilst his wife was of the wild type. By bioinformatic analysis, the variant has not been included in the HGMD database. Prediction with SIFT online software suggested the variant is harmful. Simulation with Swiss-Pbd Viewer v4.0.1 software suggested that the variant has a great impact on the structure of FXII protein. Based on the Standards and Guidelines for the Interpretation of Sequence Variants: A Joint Consensus Recommendation of the American College of Medical Genetics and Genomics (ACMG), the variant was rated as likely pathogenic. CONCLUSION The c.1A>G (p.Arg2Tyr) variant of the F12 gene probably underlay the Congenital FXII deficiency in this pedigree. Above finding has further expanded the spectrum of F12 gene variants and provided a reference for clinical diagnosis and genetic counseling for this pedigree.
Male ; Female ; Humans ; Middle Aged ; Factor XII/genetics* ; Pedigree ; Codon, Initiator ; East Asian People ; Mothers ; Factor XII Deficiency/genetics* ; Mutation

Objective To construct an intervention program for diabetic retinopathy(DR)patients undergoing surgery based on the theory of'Timing It Right'.Methods Guided by the theory of'Timing It Right',based on literature review and semi-structured interview,the first draft of the intervention program was formulated.From December 2022 to February 2023,15 medical and nursing experts in ophthalmology and endocrinology from 5 provinces(cities)in Zhejiang,Sichuan,Shanxi,Chongqing and Shanghai were interviewed by Delphi expert for 2 rounds to seek their advice and revision,and the final draft was formed.Results The effective recovery rate of 2 rounds of expert consultations was 93.75％and 100％.The authority coefficients were 0.87.The importance and feasibility of Kendall'W coordination coefficients were 0.325,0.138 and 0.193,0.141 in 2 rounds,respectively(P＜0.001).The finalized intervention program consisted of 6 months,which included 5 first-level items,18 second-level items and 43 third-level items.Conclusion The intervention program for DR patients undergoing surgery based on the theory of'Timing It Right'is reliable,scientific,feasible and practical,and it can meet the needs of such patients,providing a reference for improving their self-management ability,improving their negative emotions and delaying disease progression.

Objective:To examine the early and midterm surgical outcome of pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries (PA/VSD/MAPCA) using revised surgical strategies.Methods:A retrospective analysis of clinical data, surgical methods, and follow-up results was performed of 104 cases of PA/VSD/MAPCA in Department of Cardiovascular Surgery, Guangzhou Women and Children′s Medical Center from January 2017 to September 2022. There were 55 males and 49 females, aged ( M(IQR)) 33.9(84.0) months (range: 0.5 to 209.6 months) at the first surgical procedures. The anatomical classification included 89 cases of type B and 15 cases of type C. The number of major aortopulmonary collateral arteries was 4.2 (3.0) (range: 1 to 8). The Kaplan-Meier method was used for survival estimation. Results:In the first stage of surgery, 50 patients underwent a complete primary repair, 12 patients underwent partial repair, 32 patients underwent palliative right ventricular-pulmonary artery connection, and only 10 patients chose the Blalock-Taussig shunt. There were 10 cases of early death. In the second stage, 14 patients underwent complete repair and 4 patients underwent partial repair with no early death. The interval between the two surgeries was 19 (10) months (range: 9 to 48 months). Finally, during the 40 (34) months follow-up period, a total of 64 patients were complete repair and the right/left ventricular pressure ratio after complete repair was 0.63±0.16 (range: 0.36 to 1.00). Survival analysis showed that survival rates at 1 and 5 years after first-stage surgery were both 89.4% (95% CI: 83.5% to 95.3%). At 28 (34) months (range: 1 to 67 months) of follow-up after complete repair, the survival analysis showed that the survival rates at 1 and 5 years were both 95.2% (95% CI: 89.9% to 100%). Conclusions:Using combined approaches tailored to individual patients and optimized unifocalization strategy, the complete repair rate at one stage and the cumulative complete repair rate at 5 years improved significantly with a lower right/left ventricular pressure ratio and satisfactory early and intermediate survival.

Objective:To examine the early and midterm surgical outcome of pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries (PA/VSD/MAPCA) using revised surgical strategies.Methods:A retrospective analysis of clinical data, surgical methods, and follow-up results was performed of 104 cases of PA/VSD/MAPCA in Department of Cardiovascular Surgery, Guangzhou Women and Children′s Medical Center from January 2017 to September 2022. There were 55 males and 49 females, aged ( M(IQR)) 33.9(84.0) months (range: 0.5 to 209.6 months) at the first surgical procedures. The anatomical classification included 89 cases of type B and 15 cases of type C. The number of major aortopulmonary collateral arteries was 4.2 (3.0) (range: 1 to 8). The Kaplan-Meier method was used for survival estimation. Results:In the first stage of surgery, 50 patients underwent a complete primary repair, 12 patients underwent partial repair, 32 patients underwent palliative right ventricular-pulmonary artery connection, and only 10 patients chose the Blalock-Taussig shunt. There were 10 cases of early death. In the second stage, 14 patients underwent complete repair and 4 patients underwent partial repair with no early death. The interval between the two surgeries was 19 (10) months (range: 9 to 48 months). Finally, during the 40 (34) months follow-up period, a total of 64 patients were complete repair and the right/left ventricular pressure ratio after complete repair was 0.63±0.16 (range: 0.36 to 1.00). Survival analysis showed that survival rates at 1 and 5 years after first-stage surgery were both 89.4% (95% CI: 83.5% to 95.3%). At 28 (34) months (range: 1 to 67 months) of follow-up after complete repair, the survival analysis showed that the survival rates at 1 and 5 years were both 95.2% (95% CI: 89.9% to 100%). Conclusions:Using combined approaches tailored to individual patients and optimized unifocalization strategy, the complete repair rate at one stage and the cumulative complete repair rate at 5 years improved significantly with a lower right/left ventricular pressure ratio and satisfactory early and intermediate survival.

Bilateral pulmonary artery banding was reported to be used for truncas in 1976. With conjunctive duct stenting or Prostaglandin infusion it can improve the surgical outcomes of neonates with several complex congenital heart diseases. This review introduces scenarios that bilateral pulmonary artery banding could be used, how to band the pulmonary artery and complications, prospecting for providing suggestion for surgical treatments of neonatal complex congenital heart diseases.

Objective    To summarize the results of surgical treatment for 79 patients with coarctation of the aorta (CoA) combined with complex anomalies (CA) in recent years. Methods    The data from 79 patients with CoA combined with CA admitted to Guangzhou Women and Children's Medical Center between January 19, 2010 and September 7, 2017 were collected and analyzed. There were 52 males and 27 females. The median age was 71 days, and the median weight was 4.3 kg. There were 26 patients combined with tracheostenosis and 7 patients with preoperative tracheal intubation. Extended end-to-side anastomosis was used to correct the CoA, and the associated cardiac abnormalities were treated simultaneously by an incision through median sternotomy. All operations were performed by the same group of surgeons. Results    The median deep hypothermic circulatory arrest was 18 (13-28) minutes, the median aorta cross-clamp time was 62 (15-199) minutes, the median cardiopulmonary bypass time was 145 (71-674) minutes, the median ventilation time was 72 (9-960) hours, the median length of ICU stay was 144 (12-1 944) hours, and the median length of hospital stay was 24 (2-93) days. Early death occurred in 9 patients and late death occurred in 5 patients. Reoperation occurred in 28 patients and recoarctation developed in 10 patients. After operation, transcoarctation gradient was reduced, and the transcutaneous oxygen saturation, C-reactive protein (CRP), lactate and creatinine were increased. Compared to the survival group, both preoperative and postoperative transcutaneous oxygen saturation were worse and the postoperative levels of plasma brain natriuretic peptide (BNP) and CRP were higher in the death group. Conclusion    Correcting the CoA and the associated CA simultaneously with extended end-to-side anastomosis by an incision through median sternotomy is effective and safe, and the outcomes of immediate and medial-term are satisfactory. Improving oxygenation and limiting elevation of BNP and CRP levels may reduce the death rate.

Objective:To summarize the early and middle terms of the revascularization of remnant pulmonary artery in unilateral absent intrapericardial pulmonary artery.Methods:We retrospectively analyzed the medical records of 14 patients(7 males and 7 females) with unilateral absent pulmonary artery, in which 10 were right and 4 were left, the median age at surgery was 5 months. The patients received operation from January 2009 to December 2020. 14 patients, 2 cases associated with tetralogy of Fallot, and 1 case with aortopulmonary window. The diagnosis was made by enhanced CT scan or pulmonary vein wedge angiography. The median diameter of the affected hilar pulmonary artery remnants was(3.20±0.94)mm, and the Z value was -3.92±1.64. All the patients received single-stage revascularization: group A: tube graft interposition in 3 patients, autologous pericardial roll in 5; group B: direct anastomosis in 2, unifocalization in one and main pulmonary artery flap angioplasty in the rest 3.Results:No hospital death occurred. There were no difference finds in the age and weight at operation, the cardiopulmonary bypass time, the mechanical ventilation time, and the length of intensive care unit stay between the two groups. All the patients took aspirin for anticoagulation for 6 months after the operation. The follow-up period was 1 month to 68 months. Because the neo-PA stenosis at the anastomosis was found in one patient in group B, transcatheter balloon angioplasty was performed at 41 months after surgery. Nonetheless, the results were encouraging, symptoms have improved in all patients. The diameter and Z value of the latest ipsilateral pulmonary artery was(6.25±0.99)mm and -2.34±1.18 respectively, significantly improved when compared to the preoperative value. Residual pulmonary artery hypertension was not found. The Z value of the affected side of the pulmonary artery in group B was significantly improved than that in group A.Conclusion:Early and aggressive pulmonary artery revascularization is effective at restoring normal antegrade flow to the affected lung, resulting in improved diameter of the PA, and UAPA patient’s symptoms. The use of autologous pulmonary artery tissue angioplasty may reach a more satisfying result. However, transcatheter intervention may diminish the new pulmonary artery stenosis temporarily, reoperation is still needed in the long-term follow-up.