Diagnostic challenge of hyper-IgD syndrome in four children with inflammatory gastrointestinal complaints

Objective. Hyper-IgD syndrome (HIDS) is a rare autosomal recessive disease characterized by recurrent fever, lymphadenopathy, diarrhoea, abdominal pain, headache, arthralgia and skin rash. Abdominal symptomatology may mimic inflammatory bowel disease. We report on four patients with HIDS who had been previously investigated for Crohn's disease (CD). The levels of IgD were measured in a series of patients with CD to evaluate the specificity of this assay in the differential diagnosis between the two conditions. Material and methods. Diagnosis of HIDS was based on clinical criteria as well as immunological or genetic data. IgD levels were measured in the four subjects affected by HIDS, in 59 patients with CD and in a group of 160 healthy controls. Result. All patients underwent a variety of gastroenterological investigations because inflammatory bowel disease was suspected. Ultrasonography was pathologic in all the patients, showing enlargement of mesenteric lymph nodes. Abdominal leucocyte scintigraphy displayed diffuse signals of mild to moderate degree. IgD and IgA levels were elevated in three out of four patients. No difference in IgD values was found in CD patients as compared to the control group. Conclusions. Gastrointestinal complaints associated with recurrent fever and mesenteric adenopathy warrant genetic investigation for HIDS, in order to avoid unnecessary invasive investigations and treatment.     

Intravenous immunoglobulin increases survival time in the acute phase of experimental Chagas disease

Chagas disease induced by Trypanosoma cruzi (Tc) infection is an important cause of mortality and morbidity affecting the cardiovascular system for which presently available therapies are insufficient and largely inadequate. Intravenous immunoglobulin (IVIg) is a therapeutic preparation containing normal polyspecific IgG obtained from plasma pools of several thousand healthy donors and is used in several autoimmune, inflammatory and infectious diseases. In the study of heart from mice chronically infected with Tc, we observed that IVIg restores type 1 atrioventricular block or bradycardia. In the present study, we investigated the effects of IVIg in acute Tc infection. Intravenous immunoglobulin administration after the first week of infection was associated with an increase in survival time. Taken together, results observed in the chronic and in the acute phase associate IVIg treatment with a favourable outcome in T. cruzi infection.     

蛛网膜下腔出血急性炎症反应综合征与预后的关系

目的　探讨蛛网膜下腔出血 (SAH)急性炎症反应综合征 (AIRS)与预后的关系。方法　 15 4例 SAH患者 ,以下标准≥ 2项者诊断为 AIRS:T<36℃或 >38℃ ,心率 >90次 /分钟 ,呼吸 >2 0次 /分钟 ,血 WBC<4 0 0 0 /mm3或 >12 0 0 0 / mm3。根据 Glasgow评分对 AIRS标准和其它预后因素进行评估。结果　 AIRS与 Hunt- Hess和Hess临床分级评分差、CT表现出血量大和血糖增高明显相关。 AIRS患者病死率高于非 AIRS患者。在 AIRS标准中 ,心率、呼吸、WBC是影响预后的独立因素。结论　伴有 AIRS的 SAH患者脑组织广泛损害、临床症状重、预后差。     

曲美他嗪对急性冠脉综合征患者血清炎性因子水平的影响

目的探讨曲美他嗪治疗急性冠脉综合征（ACS）的机制。方法将同期收治的100例ACS患者随机分为观察组和对照组各50例,两组均予ACS常规治疗,在此基础上观察组口服曲美他嗪,连续4周。分别测定两组治疗前后血清C反应蛋白（CRP）、肿瘤坏死因子-α（TNF-α）、白介素-6（IL-6）水平。结果治疗后观察组血清CRP、TNF-α、IL-6水平显著低于治疗前及对照组（P〈0．01）。结论曲美他嗪可通过抗炎作用治疗ACS。     

瑞舒伐他汀对急性冠脉综合征血浆细胞因子水平的影响及临床价值分析

目的探讨瑞舒伐他汀对急性冠脉综合征(ACS)血浆细胞因子的影响。方法 87例ACS患者分为瑞舒伐他汀组44例和阿托伐他汀组43例,其中根据瑞舒伐他汀剂量又分为瑞舒伐他汀20 mg组22例和瑞舒伐他汀10 mg组22例,治疗6月;另选35例在我院体检中心体检的健康人群作为对照组。对比各组血浆炎症细胞因子水平、检测斑块的形状和回声情况、大小、数量和厚度,同时测量分叉内膜厚度(IMT)。结果瑞舒伐他汀20 mg组、瑞舒伐他汀10 mg组在血清肿瘤坏死因子(TNF-α)、白细胞介素6(IL-6)、基质金属蛋白酶(MMP-9)和超敏C-反应蛋白(hs-CRP)水平明显低于阿托伐他汀组(P0.05)。瑞舒伐他汀20 mg组、瑞舒伐他汀10 mg组在斑块厚度、IMT减少方面,明显优于阿托伐他汀组,但瑞舒伐他汀20 mg组与瑞舒伐他汀10 mg组比较未见明显差异。结论 ACS患者早期服用小剂量瑞舒伐他汀可降低血浆细胞因子水平,减轻反应炎症,稳定动脉粥样斑块,从而降低严重心血管病发生率,改善ACS患者的预后。     

Effect of etanercept and anakinra on inflammatory attacks in the hyper-IgD syndrome: introducing a vaccination provocation model

BACKGROUND: Hyper-IgD and periodic fever syndrome (HIDS) is an hereditary autoinflammatory syndrome, characterised by recurrent inflammatory attacks. Treatment of HIDS is difficult, although simvastatin is beneficial and etanercept might be effective. Studying the treatment of a rare periodic syndrome is complicated by the varying frequency and severity of symptoms and low prevalence. Our aim was to develop a system of clinical observations to evaluate effectiveness of treatment-on-demand. METHODS: Seven fever episodes in three HIDS patients were monitored, with and without administration of etanercept or anakinra. We developed a clinical score, which includes 12 symptoms. In one patient, inflammatory attacks were provoked by vaccination. RESULTS AND CONCLUSIONS: At the onset of an attack, all patients reported a clinical score between 20 and 25. The score was used to quantify severity and define the end of an attack. Reproducible monitoring of inflammatory episodes was difficult, even in this pilot study. The effect of early administration of etanercept was variable. In one patient, a fever episode could be readily provoked within 12 to 24 hours by vaccination. In this patient, the IL-1ra analogue anakinra was more successful in aborting the inflammatory attack than etanercept. We propose that this vaccination model will allow evaluation of treatment-on-demand in a controlled setting.     

The expression of toll-like receptor 2, 4 of livers in mice with systemic inflammatory response syndrome

BACKGROUND: The complicated pathogenesis of systemic inflammatory response syndrome (SIRS) is a hot topic in critical care medicine. In this study we explored the expression of toll-like receptor (TLR) 2, 4 of livers in SIRS mice and evaluated the role of TLR2, 4 in the pathogenesis of SIRS. METHODS: Forty BABL/C mice were randomly divided into 2 groups: control (n=20) and SIRS (n=20). SIRS model was induced by severe acute pancreatitis. Blood routine, blood amylase, glutamic pyruvic transaminase and temperature were measured. Histological changes of pancreases and livers were observed microscopically. The mRNA expressions of TLR2, 4 were detected by real-time polymerase chain reaction (PCR). The protein expressions of TLR2, 4 were examined by Western blot. RESULTS: Marked edema, inflammatory cell infiltration, hemorrhage, and necrosis were observed in the pancreases and livers of SIRS mice. The concentrations of amylase and glutamic pyruvic transaminase were increased significantly. Body temperature and white blood cell count were decreased. The mRNA and protein expressions of TLR2, 4 increased markedly in SIRS mice. Significant difference was observed between SIRS and control mice (P<0.01). CONCLUSION: The expressions of TLR2, 4 of livers were increased markedly in SIRS mice, indicating that TLR might play an important role in the pathogenesis of SIRS.     

Effects of high-dose intraperitoneal aprotinin treatment on complement activation and acute phase response in acute severe pancreatitis

Forty-eight patients with severe acute pancreatitis were treated with intraperitoneal lavage in a double-blind randomized multi-center trial. One group (aprotinin group,n=22) was also treated intraperitoneally with high doses of the protease inhibitor aprotinin. In the group not treated with aprotinin (control group), 6 patients were operated on because of pancreatic necrosis, compared with none in the treated group. Complement activation and the acute phase response were studied with measurements of anaphylatoxin C3a, C1 inhibitor (C1 Inh), interleukin 6 (IL-6), and C-reactive protein (CRP). The control group had higher plasma levels of C3a and lower levels of C1 Inh compared with the aprotinin group. The differences were statistically significant for C3a but not for C1 Inh. Both groups had high plasma levels of IL-6 and CRP. There were no differences between the groups in CRP levels, but the control group had higher IL-6 levels (not statistically significant) than the aprotinin group. This was caused by very high levels in the 6 patients operated on because of pancreatic necrosis, indicating that IL-6 could be a good plasma marker of pancreatic necrosis. The results also show that massive antiprotease treatment reduces complement activation, as illustrated by the lower C3a levels in the aprotinin group. The lower C1 Inh levels in the control group could have been caused by an increased consumption of the inhibitor.     

他汀序贯疗法对急性冠状动脉综合征合并2型糖尿病患者炎性因子和脂联素影响的研究

目的探讨他汀序贯疗法对急性冠状动脉综合征(ACS)合并2型糖尿病患者炎性因子和脂联素的影响。方法选择ACS患者119例,将合并糖尿病患者61例分为对照1组32例和序贯治疗1组29例,ACS未合并糖尿病患者58例分为对照2组28例和序贯治疗2组30例;另选糖尿病患者31例为糖尿病对照组,正常体检者26例为正常对照组。比较各组脂联素、可溶性血管细胞黏附因子1(sVCAM-1)、脂蛋白磷脂酶A2(Lp-PLA2)、妊娠相关蛋白A(PAPP-A)水平。结果与正常对照组比较,糖尿病对照组脂联素明显降低,sVCAM-1、Lp-PLA2、PAPP-A明显升高(P<0.01)。与对照1组和对照2组比较,序贯治疗1组和序贯治疗2组脂联素水平明显升高,sVCAM-1、Lp-PLA2、PAPP-A明显降低(P<0.01)。结论他汀序贯疗法可明显提高合并或未合并糖尿病ACS患者的脂联素水平,降低其炎症标记物水平。ACS合并或未合并糖尿病患者应用他汀序贯疗法后,对脂联素和血清炎症标记物的影响无明显差异。     

老年急腹症患者全身炎症反应综合征和多器官功能不全综合 …

目的 分析全身性炎症反应综合征（ＳＩＲＳ）向多脏器功能不全综合征（ＭＯＤＳ）的发展过程,探索ＭＯＤＳ的防治策略。方法 回顾性分析２８０例老年急腹症患者的ＳＩＲＳ和ＭＯＤＳ的临床资料。结果 老年急腹症患者入院时,ＳＩＲＳ的发生率４１．４％,其中ＭＯＤＳ发生率１２．９％,病死率１０．３％;经治疗（包括手术和保守治疗）４８小时后,仍伴有ＳＩＲＳ的患者３９例中,１５例发展为ＭＯＤＳ。２８０例老年急腹症患者     

Effect of somatostatin on immune inflammatory response in patients with severe acute pancreatitis

OBJECTIVE: Somatostatin regulates immune inflammatory response via apoptosis and adhesion of leukocytes in many diseases. This article reported a study that aimed to observe the mechanism and effect of somatostatin on the immune inflammatory response through apoptosis and adhesion of leukocytes in severe acute pancreatitis. METHODS: Thirty‐eight patients with severe acute pancreatitis, that fulfilled the guidelines for the treatment of severe acute pancreatitis of China and Balthazar computed tomography severity index (≥5) were enrolled consecutively. Nineteen of these patients received our routine treatment and 19 received additional somatostatin. In all patients the expressions of CD₄, CD₈, CD₉₅/CD₉₅ ligand and CD₁₈/CD₆₂ ligand on leukocytes were determined by flow cytometry, both upon admission and on the fourth day. Thirty healthy volunteers constituted the normal healthy group. RESULTS: In the treatment group, CD₄, CD₄ : CD₈ ratio and CD₆₂ ligand on leukocytes increased from 11.4 ± 8.2, 0.47 ± 0.10 and 25.5 ± 9.2 to 22.1 ± 9.7, 0.68 ± 0.11 and 36.2 ± 11.7 (P < 0.05) respectively, while CD₉₅ ligand on both lymphocyte and polymorphonuclear cells increased from 0.65 ± 0.21 and 0.76 ± 0.29 to 1.18 ± 0.32 and 1.58 ± 0.43 after treatment with somatostatin (P < 0.05). Furthermore, lactate dehydrogenase, aspartate aminotransferase, amylase, C reactive protein and acute physiology and chronic healthy evaluation (APACHE II) score in the treatment group reduced faster than those in the control group (P < 0.05), though there was no difference in mortality (15.7%vs 5.3%) between the two patient groups (P > 0.05). CONCLUSION: Somatostatin can modulate the immune inflammatory response and the severity of severe acute pancreatitis through apoptosis and adhesion of leukocytes, but this modulatory effect by itself is not strong enough to improve the final.     

Effects of glutamine supplementation on gut barrier, glutathione content and acute phase response in malnourished rats during inflammatory shock

AIM: To evaluate the effect of glutamine on intestinal mucosa integrity,glutathione stores and acute phase response in protein-depleted rats during an inflammatory shock. METHODS: Plasma acute phase proteins (APP),jejunal APP mRNA levels,liver and jejunal glutathione concentrations were measured before and one,three and seven days after turpentine injection in 4 groups of control,protein-restricted,protein-restricted rats supplemented with glutamine or protein powder. Bacterial translocation in mesenteric lymph nodes and intestinal morphology were also assessed. RESULTS: Protein deprivation and turpentine injection significantly reduced jejunal villus height,and crypt depths. Mucosal glutathione concentration significantly decreased in protein-restricted rats. Before turpentine oil,glutamine supplementation restored villus heights and glutathione concentration (3.24 ± 1.05 vs 1.72 ± 0.46 μmol/g tissue,P < 0.05) in the jejunum,whereas in the liver glutathione remained low. Glutamine markedly increased jejunal α1-acid glycoprotein mRNA level after turpentine oil but did not affect its plasma concentration. Bacterial translocation in protein-restricted rats was not prevented by glutamine or protein powder supplementation. CONCLUSION: Glutamine restored gut glutathione stores and villus heights in malnourished rats but had no preventive effect on bacterial translocation in our model.     

ALI/ARDS患者呼出气冷凝液和血清中PCT检测及其临床意义

目的研究ALI/ARDS患者呼出气冷凝液(EBC)和血清中降钙素原(PCT)的变化规律,探讨PCT在ALI/ARDS诊断、病情评估、预后判断中的临床意义。方法选择行机械通气的ALI/ARDS患者33例;同时选择20例健康体检者为健康对照组。观察ALI/ARDS患者与健康对照组之间血清和EBC中PCT的差异,分析临床指标和各种指标之间的相关性。结果 1ALI/ARDS组EBC和血清中PCT高于健康对照组。2ARDS组患者第七天EBC和血清中PCT中PCT高于ALI组。3存活组患者治疗后EBC和血清中PCT水平下降,病死组患者EBC和血清中PCT持续升高。4EBC和血清中PCT与Pa O2/Fi O2、APACHEⅡ有相关性。结论 ALI/ARDS患者EBC和血清中PCT的检测,有助于ALI/ARDS的诊断、病情评估和判断预后。     

The modification of serum lipids after acute coronary syndrome and importance in clinical practice

Atherosclerosis is a pathology characterized by low-grade vascular inflammation rather than a mere accumulation of lipids. Inflammation is central at all stages of atherosclerosis. Acute coronary syndrome significantly affects the concentration and composition of the lipids and lipoproteins in plasma. Plasma triglyceride and very low density lipoprotein levels increase, while high density lipoprotein, low density lipoprotein and total cholesterol levels decrease. Early treatment of hyperlipidemia provides potential benefits. However, post-event changes in lipid and lipoproteins lead to delays in the choice of the treatment. This review focuses on the mechanism and the clinical importance of the relevant changes.     

急性缺血性脑卒中患者血小板活化水平与全身炎症反应综合征的相关性

目的 动态观察缺血性脑卒中患者外周血血小板活化水平,分析血小板活化与伴发全身炎症反应综合征(SIRS)的关系. 方法 动态监测缺血性脑卒中患者外周血血小板平均内含物浓度(MPC)和白细胞计数及发病早期血浆纤维蛋白原(FIB)和血小板聚集率等指标,比较SIRS组与无SIRS组患者的各项参数,对发病早期MPC水平与SIRS评分以及当日白细胞计数、FIB和血小板聚集率等进行相关性分析. 结果 MPC水平在发病第1天略有下降,从第2天至病程第45天一直保持较低水平,均值在229～242 g/L之间波动.SIRS组与无SIRS组比较发病早期FIB差异无统计学意义(t=1.835,P=0.07),其余各指标差异有统计学意义(均为P＜0.01).发病前3 d MPC水平与SIRS评分呈显著负相关(相关系数分别为-0.392、-0.376、-0.341,t值分别为3.484、3.405、3.125,均为P＜0.01),而与当日白细胞计数、FIB及血小板聚集率无相关性(P＞0.05). 结论 缺血性脑卒中患者整个病程期间血小板一直保持较活化的状态,伴发SIRS的患者发病早期血小板活化水平、白细胞计数和血小板聚集率均高于未伴发SIRS的患者.早期的血小板活化水平提示SIRS的严重程度,血小板活化可能是SIRS的独立危险因素.     

中性粒细胞VCS参数在全身炎症反应综合征患者中的变化及意义

目的：探讨全身炎症反应综合征（SIRS）患者中性粒细胞VCS参数的变化及意义。方法：利用Beckman-Coulter Gen．S自动血细胞分析仪的体积、高频传导、激光散射（VCS）技术,测定45例健康体检者（对照组）和121例重症监护病房（ICU）的患者（非SIRS组47例、非感染性SIRS组34例、感染性SIRS组40例）的白细胞总数（WBC）、中性粒细胞百分数（NE％）和NE的体积（NEV）、浆核比（NE-C）、胞浆颗粒特性（NE-S）及它们各自的平均分布宽度（SD）,并用ROC曲线评价各参数监测ICU的患者SIRS病情的敏感性和特异性。结果：ICU的患者WBC、NE％、NE—V、NEV-SD、NEC-SD、NES-SD明显高于对照组,而NE—C、NE-S明显下降（P〈0．01）;SIRS组第2、4、6天的WBC与NE％和第4、6天的NEVSD及第6天的NE—V均高于非SIRS组相应的时间点,而NE-S明显降低（P〈0．01或P〈0．05）;SIRS组的患者在进人ICU的第2天与第4、6天NE—V、NES-SD差异有统计学意义;非感染性SIRS组的NEV—SD、NEC—SD、NES-SD、NE—S和感染性SIRS组除NE-C外的NE-VCS参数与非SIRS组均差异有统计学意义（P％0．01或P〈0．05）,且以感染性SIRS组的WBC、NE—V、NE—S、NEV-SD、NEC—SD的变化最显著;评价患者非SIRS与SIRS的病情,NE-VCS参数的ROC曲线下面积明显高于WBC和NE％。结论：中性粒细胞VCS参数在ICU的患者不同病情中可发生明显的变化,其变化随着病情的加重而更加显著;NE—VCS参数对评价SIRS病情的敏感性和特异性优于WBC和NE％。     

长正五聚蛋白-3与高敏C反应蛋白在急性冠状动脉综合征的变化及意义

目的:探讨长正五聚蛋白-3(PTX-3),高敏C反应蛋白(hsCRP)在急性冠状动脉综合征中的变化及意义.方法:入选经冠状动脉造影患者170例.用ELISA法测定血浆PTX-3浓度,比较各组间差异.并与hsCRP比较.结果:①急性心肌梗死患者PTX-3浓度显著高于不稳定型心绞痛组[(7.20±4.44)μg/L,(6....     

Effects of nutritional support combined with insulin therapy on serum proteins,inflammatory factors,pentraxin-3, and serum amylase levels in patients with diabetic ketoacidosis complicated with acute pancreatitis

To explore the effects of nutritional support combined with insulin therapy on serum protein, procalcitonin (PCT), C-reactive protein (CRP), tumor necrosis factor-α (TNF-α), pentraxin-3 (PTX-3), and serum amylase (AMS) levels in patients with diabetic ketoacidosis complicated with acute pancreatitis.A total of 64 patients with diabetic ketoacidosis complicated with acute pancreatitis admitted to our hospital from January 2018 to February 2019 were enrolled in this prospective study. They were divided into the study group and the control group according to the random number table method, with 32 patients in each group. Patients in the study group were given nutritional support combined with insulin therapy, and patients in the control group were given insulin therapy.There were no significant differences in general data including age, gender, body mass index, course and type of diabetes, acute physiology and chronic health evaluation II, RANSON, CT grades between the 2 groups before treatment (all P > .05). After 7 days of treatment, the clinical efficacy of the study group was significantly higher than that of the control group (study group vs control group, 94.44% vs 75.00%, P < .05). After 7 days of treatment, the levels of prealbumin and albumin in the study group were significantly higher than those in the control group (P < .05). After 7 days of treatment, the levels of PCT, CRP, TNF-α, PTX-3, and AMS in the 2 groups were significantly lower than those before treatment (P < .05), and the levels of PCT, CRP, TNF-α, PTX-3, and AMS in the study group were significantly lower than those in the control group. After 7 days of treatment, the levels of IgG, IgM, and IgA in the 2 groups were significantly higher than those before treatment, and the levels of IgG, IgM, and IgA in the study group were significantly higher than those in the control group (P < .05).Nutritional support combined with insulin is obviously effective in the treatment of diabetic ketoacidosis complicated with acute pancreatitis, which can improve serum protein levels, reduce inflammatory response, improve immune function, and is worthy of clinical application.     

远程数据传输在慢快综合征患者术后急性期随访中的应用

目的 了解远程数据传输对慢快综合征患者起搏器术后急性期随访中的应用价值.方法 纳入2009年8月至2012年8月明确诊断为慢快综合征并植入永久性起搏器的患者120例,按植入起搏器是否具有远程数据传输功能将患者分为对照组（n=68）和远程监测组（n=52）.按照随访方案进行随访,分析两组有意义随访[随访时进行了起搏参数调整和（或）药物的调整]及非常规随访（患者因不适、担心等任何原因在随访方案以外的时间主动到医院进行的随访）率的差别及非常规随访的原因.结果 在起搏器植入术后3个月内,对照组有20例（29.4%）就诊,远程监测组有30例（57.7%）得到医生的复诊建议,其中29例到医院就诊,两组非常规随访率有显著统计学差异（P＜0.01）.对照组中18例（26.4%）患者进行了有意义随访,远程监测组27例患者进行了有意义的随访（51.9%）,两组有意义随访率有显著统计学差异（P＜0.01）.对照组首次随访40%是由于心慌不适感觉就诊.术后3个月内收到的监测组患者报警信息主要是因为高心室率（59.6%）和频繁的模式转换（51.9%）.结论 起搏器远程数据传输可及时发现慢快综合征患者起搏术后起搏参数及药物调整需求,指导患者到医院进行有意义随访.