Objective To investigate the clinical feature and misdiagnosis of 128 patients with Wilson’s disease and proposed the points of diagnosis for this disease. MethodsOne hundred and twenty eight patients with Wilson’s disease from Jan. 1983 to Apr. 2000 of our hospital were reviewed. Their clinical feature and diagnosis condition, such as the first onset symptoms, misdiagnosis conditions and hypersplenism were studied. Results①The first onset symptoms: 64 showed liver dysfunction and 52 presented manifestation of central nerves system (CNS) (Pgt;0.05). ②The age of liver dysfunction was significantly younger (13 years) than that of CNS involved patients (23 years, P＜0.05). ③Patients with the liver damage had enlarged liver (3 cm below the right rib) and hypersplenism and 12 patients had to receive splenectomy. ④Gastroscopy or barium meal showed that esophageal varicose rate was 30.8%（12/39） and 7 patients had large upper gastrointestinal bleeding. Conclusion The age of liver involved patients is significantly younger than that of the CNS involved patients. The liver cirrhosis, hypersplenism and portal hypertension are very common complications of Wilson’s disease and some prevention and cure measures should be taken for these complications.
Objective To evaluate the effect of laparoscopic splenectomy （LS） and open splenectomy （OS） forhypersplenism secondary to liver cirrhosis with portal hypertension. Methods Cochrane Central Register of Controlled Trials， Cochrane Database of Systematic Reviews， Medline， Embase， CINAHL， CBM， CNKI， VIP and WanFang database were searched for randomized control trials or retrospective studies related to the effect of LS and OS for hyper-splenism secondary to liver cirrhosis with portal hypertension. Then studies were screened according to the inclusion and exclusion criterias， data were collected， and quality of studies were evaluated. RevMan 5.1.0 software was used for meta-analysis. Results Seventeen retrospective studies and one randomized control trial were enrolled. The results of meta analysis showed that， the estimated blood loss， postoperative hospital stay， rate of postoperative complication， and WBC level on 1 day after operation of LS group were significantly lower or shorter than those of OS group （P＜0.05）. However， there were no significant differences on operative time， WBC level on 7 days after operation， postoperative platelets （1 day and 7 days after operation）， alanine aminotransferase （1 day and 7 days after operation）， and total bilirubin （1 day and 7 days after operation） between LS group and OS group （P＞0.05）. Conclusions LS may be more effective to reduce blood loss， postoperative hospital stay， and rate of postoperative complication in patients with hypersplenism secondary to liver cirrhosis with portal hypertension in comparison to OS. However， the effectiveness of LS on liver function is not clear.
ObjectiveTo establish a model of portal hypertension with hypersplenism in SD rats by portal vein binding combined with splenic vein ligation. MethodsSixty healthy male SD rats were randomly divided into three groups: sham operation group （only laparotomy, n=20）, portal vein binding group (only binding, n=20), and portal vein binding combined with splenic vein ligation group (combined operation group, n=20). The counts of platelet, erythrocyte, and leukocyte were examined just before operation and once a week after operation for 7 weeks. Portal pressure, shortaxis, and longaxis diameter of spleen were examined just before operation and seven weeks after operation. At the seventh week, all the animals were sacrificed, spleen index and pathology changes of each group were examined. ResultsErythrocyte and platelet counts in combined operation group were significantly lower than those in the other two groups on the third week （Plt;0.05）, and there was no significant difference in leukocyte count among three groups （Pgt;0.05）. Compared with the preoperative value, portal pressure increased significantly on the seventh week in both portal vein binding group and combined operation group, and was higher than that in the sham operation group （Plt;0.05）. The two diameters of spleen also increased significantly in combined operation group on the seventh week （Plt;0.05）, and were larger than those in the other two groups （Plt;0.05）. The same result was found in spleen index （Plt;0.05）. Typical pathological changes of hypersplenism presented only in combined operation group on the seventh week after operation. ConclusionsPortal vein binding combined with splenic vein ligation can induce experimental secondary hypersplenism successfully. This procedure is simple and stable, and helpful to the scientific research.
【Abstract】ObjectiveTo explore the appropriate surgical management of the primary hepatocellular carcinoma with hypersplenism. MethodsOf 67 patients who has primary hepatocellular carcinoma with hypersplenism, 17 cases had hepatectomy combined with splenectomy, 7 cases had hepatectomy only, and the other 43 patients were treated with hepatic artery embolization and splenic artery embolization. ResultsThe symptoms of hypersplenism disappeared and the hemogram became normal 30 d after operation in 17 patients who had hepatectomy combined with splenectomy, but worsened in 7 patients who only had simple hepatectomy and 6 cases of those patients were treated with splenic artery embolization 3-7 months after operation. In 43 patients treated with hepatic artery embolization and splenic artery embolization, 79%（34/43）had improved hypersplenism symptoms and the hemogram became normal. ConclusionThe treatment of primary hepatocellular carcinoma with hypersplenism should be strived for hepatectomy combined with splenectomy. If the liver mass cannot be resected, hepatic artery embolization and splenic artery embolization should be chosen.
【Abstract】Objective To investigate the influence of hepatectomy combined with splenectomy on curative effect of primary hepatocellular carcinoma patients associated with hypersplenism.Methods Twenty three cases of primary hepatocellular carcinoma associated with hypersplenism were analyzed retrospectively and divided into hepatectomy combined with splenectomy group (n=10) and hepatectomy combined with ligation of splenic artery (n=13). Peripheral blood samples were collected 1 week before operation and 3 monthes after operation respectively. The levels of CD4, CD8, CD16, CD4/CD8, WBC and PLT in the blood were detected. Survival rate between the two groups was compared. Results There were not significant differences in the expressional levels of CD4, CD8, CD16, CD4/CD8,WBC and PLT before operation, bleeding quantity during the operation and rate of severe complications after operation in the two groups. The expressional levels of CD4, CD16, CD4/CD8, WBC and PLT of hepatectomy combined with splenectomy group were much higher in 3 months after operation than those in 1 week before operation and in hepatectomy combined with ligation of splenic artery group (P＜0.01) and the latter with no distinct changes of the above indexes. There was no difference of survival rate between the two groups. But in the hepatectomy combined with ligation of splenic artery group, 7 patients of 13 patients were compelled to terminate chemotherapy because of their low WBC while not a single patient was forced to do so in the hepatectomy combined with splenectomy group. Conclusion Hepatectomy combined with splenectomy is useful for patients with hepatocellular carcinoma associated with hypersplenism to restore the immunological function and the application of chemotherapy after operation.
Objective To defect the level of platelet antibody-IgG (PA-IgG) in patients with congestive splenomegaly and hypersplenism and the change of PA-IgG level after splenectomy and subtotal splenectomy. Methods Twenty four cases of congestive splenomegaly and hypersplenism were investigated. Results The level of PA-IgG in 24 cases were higher than normal range (Ｐ＜0.01), while the platelet count were lower than normal range and there was a significant negative correlation between the level of PA-IgG and platelet count (r＝-0.4747, Ｐ＜0.05). After subtotal splenectomy or splenectomy, the level of PA-IgG descended, the platelet count raised and the negative correlation between the level of PA-IgG and platelet count disappeared. Conclusion The results suggest that there is a immunoregulation between PA-IgG and platelet. Perhaps spleen has some relation with the immunoregulation.
目的探讨肝细胞癌合并脾功能亢进患者同期行肝癌切除和脾切除的安全性及可行性。 方法回顾性分析2001年11月至2012年4月期间笔者所在医院收治的52例肝细胞癌合并脾功能亢进同期施行肝癌切除和脾切除患者的临床资料。 结果肝癌切除联合脾切除19例，肝癌切除联合脾切除加贲门周围血管离断术33例。手术时间（249.63±40.90）min（182～340 min），术中出血量（580.77±260.31）mL（200～1 700）mL。全组无死亡病例，术后并发症包括：胸腔积液11例，肺内感染3例，肝断面感染3例，胆汁漏1例，切口感染2例，高胆红素血症3例，门静脉系统血栓形成22例，均经保守治疗后好转。术后第14天，患者的白细胞和血小板计数分别由术前的（3.19±1.59）×109/L和（53.96±18.94）×109/L升至（8.86±5.06）×109/L和（464.90±189.27）×109/L（P＜0.05）；术后红细胞计数变化不明显，甚至有轻度下降。 结论对于肝细胞癌合并脾功能亢进患者，选择合适的病例同期行肝癌切除和脾切除是安全可行的，而且脾切除有助于缓解脾功能亢进。
ObjectiveTo summarize the recent development of surgical treatment for portal hypertension with hypersplenism. MethodsThe related literatures on various operation treatment of hypertension with hypersplenism at home and abroad in recent years were collected and reviewed. ResultsThere are many operation treatment methods of hypertension with hypersplenism, includes the pericardial devascularization, subtotal splenectomy, partial splenic embolization, spleen radiofrequency ablation, splenic artery ligation, distal splenorenal shunt, and so on. The different operation methods each has its advantages and disadvantages, but there are a certain percentage of the incidence of complications. At the same time, due to the limited understanding of the function of the spleen in portal hypertension, the treatment of "resecting" or "reserving" spleen has always been the hot spot of the academic dispute. ConclusionFor what kind of operation method is the most suitable for the treatment of hypertension with hypersplenism is no fixed conclusion.
ObjectiveTo systematically evaluate the efficacy and safety of transcatheter arterial chemoembolization (TACE) combined with partial splenic embolization (PSE) for hepatocellular carcinoma (HCC) with hypersplenism. MethodWe searched The Cochrane Library (Issue 11, 2015), PubMed, EMbase, CBM, VIP, CNKI and WanFang Data databases from inception to November 1st 2015, to collect randomized controlled trials (RCTs) about TACE combined with PSE in treating HCC with hypersplenism. Two reviewers independently screened literature, extracted data, and assessed the risk of bias of included studies. Then meta-analysis was performed using RevMan 5.3 software. ResultsA total of 11 RCTs involving 708 patients were included. The results of meta-analysis showed that: Compared with TACE alone, TACE combined with PSE could significantly improve postoperative CD4 count (MD=6.99, 95%CI 4.60 to 9.38, P<0.00001), CD4/CD8 ratio (MD=0.64, 95% 0.45 to 0.84, P<0.00001), and the rate of half year survival (RR=1.16, 95%CI 1.02 to 1.32, P=0.02), decrease the incidences of spontaneous peritonitis (RR=0.20, 95%CI 0.05 to 0.48, P=0.03) and varices bleeding (RR=0.17, 95%CI 0.04 to 0.68, P=0.01). The two groups had similar incidence of post-embolization syndrome (RR=1.17, 95%CI 0.79 to 1.75, P=0.44). ConclusionTACE combined with PSE is more safe and effective than TACE alone in unresectable HCC with hypersplenism. Due to limited quantity and quality of the included studies, the above conclusion should be further verified by conducting more high quality, large scale RCTs.
ObjectiveTo explore the effect of partial splenic embolization on splenectomy plus devascularization of esophageal and gastric vein. MethodsTwenty three cirrhosis patients with portal hypertension combined the hypersplenism (partial splenic embolization group), who received partial splenic embolization in our hospital from June 2010 to June 2015, as well as 30 cirrhosis patients with portal hypertension combined the hypersplenism without undergoing partial splenic embolization in the same period (non-partial splenic embolization group), were collected retrospectively. All patients underwent splenectomy plus devascularization of esophageal and gastric vein. Comparison of operation time, intraoperative blood loss, intraoperative blood transfusion volume, postoperative total flow of abdominal drainage tube, postoperative gastrointestinal function recovery time, hospital stay, and the incidence of complication was performed. ResultsThe operation time[(3.56±0.70) h vs. (1.78±0.28) h], intraoperative blood loss (900 mL vs. 250 mL), intraoperative blood transfusion volume (800 mL vs. 200 mL), postoperative total flow of abdominal drainage tube (450 mL vs. 150 mL), postoperative gastrointestinal function recovery time[(43.38±18.68) h vs. (27.60±12.39) h], hospital stay (12 d vs. 7 d), and incidence of incision infection[34.8% (8/23) vs. 10.0% (3/30)] of partial splenic embolization group were all higher or longer than those corresponding indexes of non-partial splenic embolization group (P < 0.05). All patients of 2 groups were followed up by telephone visit for 6-58 months, and the median was 28-month. There was no recurrence of gastrointestinal hemorrhage during the follow-up period. ConclusionsSplenectomy is more difficult, and maybe has more intraoperative blood loss and complications for cirrhosis patients with portal hypertension combined the hypersplenism, who received partial splenic embolization ever. For these patents, the recovery time is longer. We should make choice of partial splenic embolization or splenectomy directly according to the patients' situation, to implement individualized treatment, so we can make the biggest benefit for patients.