原发性淀粉样变五例临床分析

目的：提高对原发性淀粉样样变的认识,方法：分析5例原发性淀粉样变的临床表现和实验室检查,结果：5例肾脏受累,4例心脏受累,3例肝肿大。结论：原发性淀粉样变临床表现多样化,大多为非特异性,易误诊,确诊靠病理活检,遇到肝脾肿大及肾功能不全等多系统损害的病人,应想到原发性淀粉样变。     

脾大部切除术治疗肝硬化脾大脾亢的疗效分析

目的 探讨脾大部切除术治疗肝硬化脾大脾亢的疗效。方法 对２６例肝硬化脾大脾亢病人行脾大部分切除加断流术,术后１年内随访,观测门静脉压力和直径、外周血细胞计数、骨髓细胞学、血小板相关ＩｇＧ（ＰＡＩｇＧ）及残腺大小的变化。结果 脾大部切除加断流术后,门静脉压力下降,门静脉直径缩小,残脾显像良好,无明显增大,外周血红细胞、白细胞和血小板计数升高,骨髓像大致正常,ＰＡＩｇＧ下降,随访１年见脾亢明显改善或消     

Effects on splenic, hepatic, hematological, and growth parameters following high-dose poloxamer 407 administration to rats

The nonionic surfactant poloxamer 407, NF (PIuronic ^® F-127, NF) has previously been shown to produce marked hyperlipidemia in rats at a dose of 1.5 g/kg for greater than 96 h following a single intraperitoneal (i.p.) injection (Wout et al. J. Parenter. Sci. Technol., 46 (1992) 192–200). In an effort to characterize any potential toxicity of the polymeric vehicle to various organ systems in the rat following multiple i.p. injections, a dose of 0.33 g/kg per day (10% w/w solution) or 1.0 g/kg per day (30% w/w solution) of poloxamer 407 was administered once daily for 4 consecutive days. When compared to control (non-injected) animals, rats injected with 0.33 g/kg per day of poloxamer 407 did not show a significant (p > 0.05) increase or decrease in spleen, liver, or total body weight. A complete blood count (CBC) with a white blood cell (WBC) differential was performed on blood samples collected on day five from rats injected with poloxamer 407 at both doses. The CBC with WBC differentia] was conducted to assess any changes in the WBC count, percent lymphocytes (LY), percent monocytes (MO), percent granulocytes (GR), red blood cell (RBC) count, hemoglobin (HGB), percent hematocrit (HCT), and the mean corpuscular volume (MCV) following administration of poloxamer 407. Rats injected i.p. with a dose of 0.33 g/kgper day of poloxamer 407 for 4 days demonstrated a significant (p < 0.05) increase in the number of MO when compared to controls. Administration of 1.0 g/kg per day of poloxamer 407 to rats for 4 days demonstrated distinct splenomegaly when compared to non-injected control animals. In addition, a significant (p < 0.05) reduction in body weight and significant (p < 0.05) decrease in the percent LY, RBCs, HGB, and percent HCT were noted. Lastly, a significant (p < 0.05) increase in the number of WBCs and the percent MO was observed in this same group of rats. However, rats administered 1.0 g/kg per day of poloxamer 407 for 4 days were observed to have no detectable changes in the values of the MCV, the percent GR, or liver-to-body weight ratio when compared to control animals. Thus, repetitive i.p. injections of poloxamer 407 to rats at a dose of 1.0 g/kg per day for four days results in splenomegaly and a reduction in total body weight. Splenomegaly in rats administered poloxamer 407 at a dose of 1.0 g/kg per day resulted from red pulp expansion due to infiltration of macrophages which contained phagocytized lipids.     

多普勒超声探测充血性脾大致门静脉血液动力学变化

目的:研究口服葡萄糖负荷试验,是否能提高多普勒超声对充血性脾大的诊断水平。方法:82例被观察者,按脾大是否存在及脾大的原因分成3组,然后在多普勒超声下采用口服葡萄糖溶液(25g,250ml)的方法来测定门静脉主干的血流速度及直径,并在口服前、口服30分钟后进行比较。结果:充血性脾大组的36例肝硬化患者(包括在试验前门静脉血流速度接近正常的6例患者),其门静脉的血流速度与口服葡萄糖前相比,未发生明显变化(<6cm/s)P>0.05。而对照组与非充血性脾大组(包括试验前门静脉血流速度低于正常的2例患者),则出现了血流速度明显加快的变化(>6cm/s)P<0.01。结论:葡萄糖负荷试验根据门脉压的不同,可使门静脉的血流速度发生相应的变化,这样为充血性脾大的鉴别诊断提供了新的检测方法。     

彩色多普勒超声对肝炎后肝硬化脾肿大脾内血流的研究

目的　应用彩色多普勒检测脾内动、静脉血流参数 ,为肝硬化门静脉高压症的诊断与治疗提供依据。方法　肝硬化患者和正常对照组各 81例 ,检测脾动、静脉及其脾内分支。结果　正常对照组脾动脉及其脾内分支越接近末梢血流阻力指数越低 ,而肝硬化各组结果与其相反。肝硬化各组脾充血指数与正常对照组相比较有极显著性差异。结论　应用彩色多普勒检测脾动、静脉及其脾内分支血流参数对肝硬化门静脉高压症的诊断与治疗有重要的临床意义     

A case of splenomegaly in CBL syndrome

IntroductionWe present a child with unexplained splenomegaly to highlight this feature as a presenting sign of the RASopathy CBL syndrome and to draw attention to the power and utility of next generation genomic sequencing for providing rapid diagnosis and critical information to guide care in the pediatric clinical setting.Clinical reportA 7-year-old boy presented with unexplained splenomegaly, attention deficit hyperactivity disorder, mild learning difficulties, easy bruising, mild thrombocytopenia, and subtle dysmorphic features. Extensive haematological testing including a bone marrow biopsy showed mild megaloblastoid erythropoiesis and borderline fibrosis. There were no haematological cytogenetic anomalies or other haematological pathology to explain the splenomegaly. Metabolic testing and chromosomal microarray were unremarkable. Trio whole-exome sequencing (WES) identified a pathogenic de novo heterozygous germline CBL variant (c.1111T > C, p.Y371H), previously reported to cause CBL syndrome and implicated in development of juvenile myelomonocytic leukemia (JMML).DiscussionCBL syndrome (more formally known as “Noonan-syndrome-like disorder with or without juvenile myelomonocytic leukemia”) has overlapping features to Noonan syndrome with significant variability. CBL syndrome and other RASopathy disorders—including Noonan syndrome, neurofibromatosis 1, and Costello syndrome—are important to recognize as these are associated with a cancer-predisposition. CBL syndrome carries a very high risk for JMML, thus accurate diagnosis is of utmost importance. The diagnosis of CBL syndrome in this patient would not have been possible based on clinical features alone. Through WES, a specific genetic diagnosis was made, allowing for an optimized management and surveillance plan, illustrating the power of genomics in clinical practice.     

Simultaneous partial splenectomy during liver transplantation for advanced cirrhosis patients combined with severe splenomegaly and hypersplenism

BACKGROUND The most effective treatment for advanced cirrhosis and portal hypertension is liver transplantation(LT).However,splenomegaly and hypersplenism can persist even after LT in patients with massive splenomegaly.AIM To examine the feasibility of performing partial splenectomy during LT in patients with advanced cirrhosis combined with severe splenomegaly and hypersplenism.METHODS Between October 2015 and February 2019,762 orthotopic LTs were performed for patients with end-stage liver diseases in Tianjin First Center Hospital.Eighty-four cases had advanced cirrhosis combined with severe splenomegaly and hypersplenism.Among these patients,41 received partial splenectomy during LT(PSLT group),and 43 received only LT(LT group).Patient characteristics,intraoperative parameters,and postoperative outcomes were retrospectively analyzed and compared between the two groups.RESULTS The incidence of postoperative hypersplenism(2/41,4.8%)and recurrent ascites(1/41,2.4%)in the PSLT group was significantly lower than that in the LT group(22/43,51.2%;8/43,18.6%,respectively).Seventeen patients(17/43,39.5%)in the LT group required two-stage splenic embolization,and further splenectomy was required in 6 of them.The operation time and intraoperative blood loss in the PSLT group(8.6±1.3 h;640.8±347.3 mL)were relatively increased compared with the LT group(6.8±0.9 h;349.4±116.1 mL).The incidence of postoperative bleeding,pulmonary infection,thrombosis and splenic arterial steal syndrome in the PSLT group was not different to that in the LT group,respectively.CONCLUSION Simultaneous PSLT is an effective treatment and should be performed in patients with advanced cirrhosis combined with severe splenomegaly and hypersplenism to prevent postoperative persistent hypersplenism.     

比较腹腔镜与开放手术在小儿巨脾症的应用

目的：对比腹腔镜脾切除术(LS)和传统开放脾切除术(OS)治疗小儿巨脾的临床疗效,探讨腹腔镜切除小儿巨脾的可行性及安全性。方法：选取32例小儿巨脾的病例资料,根据手术方法不同,将其中16例LS组与16例OS组进行对比分析。结果：两组均实施巨脾切除术;两组相比,LS组手术时间长,术中出血量少,术后肛门排气早,术后住院时间短,副脾发现例数多;术后并发症发生率、长期随访率、有效率差异没有统计学意义(P>0.05)。结论：腹腔镜小儿巨脾切除术是一种安全、有效、可行的手术方法,能减少并发症,具有创伤小、恢复快、美容的临床疗效。     

EB病毒阳性老年人弥漫大B细胞淋巴瘤二例并文献复习

 目的　分析我国老年人EB病毒（EBV）阳性弥漫大B细胞淋巴瘤（DLBCL）的临床特点,提高对该病的认识。方法　报道2例老年人EBV阳性DLBCL患者临床、实验室资料及治疗经过。结果　老年人EBV阳性DLBCL高发年龄70～79岁,患者主要表现为全身淋巴结肿大,可伴结外器官受累,可出现巨脾和免疫性溶血性贫血,多伴有发热、体质量减轻等B症状,病理表现为多形性大细胞浸润,伴不同程度的反应性细胞,特别是T细胞增殖。结论　老年EBV阳性DLBCL患者肿瘤细胞CD20或CD79a阳性,EBV 编码的小RNA（EBER）阳性。疾病进展快,对标准化疗反应差。利妥昔单抗对CD20阳性病例短期有效,但疗效有限。患者的生存期短。死亡原因主要是感染所致的呼吸衰竭。