Chipaozi, the dried mature fruit of Thladiantha dubia Bge. family Cucurbitaceae, hasbeen found to be used in mixture with Wanggua, the fruit of Trichosanthes cucumeroides (Ser. ) Maxim.of the same family.A comparative study on the morphological and characteristics of these two drugs were carried out and reported.

Objective To evaluate the effect of hepatic vein occlusion and stent replacement in treatment for Budd-Chiari syndrome(BCS).Methods Forty three patients with BCS were underwent percutanous puncture,radiography,transjugular angioplasty,balloon dilation and stent placement for hepatic vein under Doppller ultrasounographic guidance from July 2001 to September 2006. Results Technical success was 100%with no complications.The medium vein pressure was reduced from 32.5 tO 20 cm H2O(1 cm H2O-0.098 kPa)after stents replacement(P＜0.01).The hepatic vein angioplasty revealed that all stents were patent and branches were disappeared.The symptoms in 38 patients were disappeared immediately,and improved in 5 patients.All patients were followed up of 32 months(ranged 1-62).Except one patient died of severe gastric bleeding,the 42 patients were survived with symptoms free.Conclusion Hepatic vein occlusion and stent replacement are safe and effective in treatment of BCS.

Objective: To investigate the safety and efficacy of pingyangmycin (bleomycin A5) sclerotherapy for early peripheral arteriovenous malformations (AVM). Methods: Thirteen cases of early-stage [Schobinger clinical stage (Ⅰ/Ⅱ)] peripheral AVM patients (11 stage Ⅰ and 2 stage Ⅱ patients) aged between 3 months and 51 years were selected between January 2012 and May 2015. Pingyangmycin sclerotherapy injections were administered with B-scan ultrasonography or digital subtraction angiography positioning. All patients underwent relevant supplementary examinations before and after the procedure, and clinical evaluation was performed based on the improvement of the clinical symptoms of the patient and re-examination of lesion by imaging. Results: A total of 88 injections were administered to the 13 patients with an average of 6.7 injections per patient, 3-6 years of follow-up. Based on clinical evaluation, 7 patients were generally cured, 3 patients had significant improvement, 2 patients had partial improvement, and 1 patient had no improvement. Seven patients had different levels of postoperative swelling that resolved on its own. No serious complications occurred. Conclusions Pingyangmycin sclerotherapy for treatment of early peripheral AVM was effective and had few complications. It can control further progression of pathological changes.

Objective    To analyze the clinical efficacy of transthoracic occlusion via a right subaxillary incision and conventional surgery in the treatment of ventricular septal defect (VSD). Methods    The clinical data of patients with congenital VSD undergoing right subaxillary incision surgery in our hospital from January 2017 to January 2020 were retrospectively analyzed. According to the surgical methods, the patients were divided into two groups: a conventional surgery group (conventional group) and a transthoracic occlusion group (occlusion group). There were 221 patients in the conventional group, including 97 males and 124 females, with an average age of 2.6±2.2 years and an average weight of 13.4±6.2 kg; there were 185 patients in the occlusion group, including 90 males and 95 females, with an average age of 3.2±2.6 years and an average weight of 14.7±6.6 kg. The clinical effectiveness was compared. Results    The success rate of surgery was 100% in both groups. The intraoperative blood loss was less in the occlusion group (P<0.05). The incision length, operation time, postoperative mechanical ventilation time, retention time in the intensive care unit, the time to resume normal diet and normal activities after operation were all shorter than those in the conventional group (P all <0.05). The total cost during hospitalization of the conventional group was less than that of the occlusion group (P<0.001). There was no statistical difference in the incidence rate of perioperative complications between the two groups (P>0.05). During the follow-up (15.8±8.8 months), the incidence of complications in the conventional group was higher than that in  the occlusion group with a statistical difference (P<0.001). Conclusion    Compared with conventional surgery, transthoracic occlusion for VSD via right subaxillary incision has the advantages of smaller incision, shorter operation time, less blood loss, shorter postoperative recovery time and less long-term complications. However, the total hospitalization cost is relatively high, mainly because of the high consumables cost, and the long-term effects still need further comparative observation.

Objective    To reveal the risk factors for delayed recovery and complications in infants with weight≤ 5.0 kg after surgical ventricular septal defect (VSD) closure. Methods    We retrospectively reviewed a consecutive series of 86 patients with weight≤5.0 kg who were admitted to our institution for surgical VSD closure between January 2016 and July 2019, including 31 males and 55 females with an age of 17-266 (80.3±40.4) d and a weight of 2.5-5.0 (4.4±0.6) kg. The VSDs were divided into perimembranous (n=65, 75.6%), subaortic (n=17, 19.8%) and subaortic combined muscular types (n=4, 4.7%). Mechanical ventilation (MV) time≥24 h or ICU stay≥72 h were defined as delayed recovery. Death, sudden circulatory arrest, complete heart block requiring a permanent or temporary pacemaker implantation, neurological complications, reoperation (for residue shunt or valvular regurgitation), reintubation and diaphragmatic paralysis were considered as significant major adverse events. Results    There was no death, reoperation due to residual VSD or neurological complication. Totally 51 (59.3%) patients had MV timec≥24 h and 51 (59.3%) patients stayed in the ICU≥ 72 h. Two (2.3%) patients required temporary pacemaker and six (7.0%) patients required reintubation. During the follow-up of 3-36 (15.8±8.8) months, 1 patient died of pneumonia after discharge, 5 patients suffered mild tricuspid valve regurgitation and 1 patient suffered decreased left ventricular systolic function in the follow-up. No aortic valve injuries occurred. Conclusion    For patients whose weight≤5.0 kg, short-term results of surgical VSD closure are excellent. Low weight and age may prolong MV time; low birth weight and pulmonary hypertension may prolong ICU stay, but are not independent risk factors.

The Ehlers-Danlos syndromes (EDS) are a group of rare hereditary connective tissue disorders characterized by joint hypermobility, skin hyperextensibility, and tissue fragility. The clinical and genetic hetero- geneity of EDS frequently leads to underdiagnosis and misdiagnosis. Genetic testing is an essential approach to clarify the underlying diagnosis. Recent research has preliminarily established genotype-phenotype correlations and introduced the novel concept of " disease spectrum" in some subtypes. These studies deepen our understanding of EDS etiology and provide important insights into clinical management. Published in 2023, the Chinese Guidelines for Diagnosis and Treatment of the Ehlers-Danlos Syndromes(the Guidelines) recommend performing genetic testing with deep phenotyping for patients who meet the clinical diagnostic criteria or are suspected of having EDS. However, it should be noted that the clinical diagnosis might differ from the molecular diagnosis. Furthermore, cutting-edge approaches such as periodic data reanalysis, integration of RNA sequencing into family-based whole-genome sequencing, and third-generation sequencing may facilitate the reclassification of variants of uncertain significance or resolve undiagnosed cases. This article summarizes recent progress in the genetics research of EDS, with the hope of offering a valuable resource for clinical diagnosis, treatment and scientific research to optimize the quality of life of patients with EDS.