Pediatric Churg-Strauss syndrome in Mexico

Churg-Strauss syndrome (CSS) is one of the rarest forms of vasculitis, and very rarely presents in the pediatric population. We present two cases of childhood CSS, both with hepatic and cardiac involvement. To our knowledge, these are the first two cases of CSS in the pediatric population described in Mexico.     

Surgical results of 40 patients with malignant tracheobronchial lesions

Abstract The objective of this study was to evaluate the results of tracheobronchoplasty performed on a variety of malignant diseases which involved the tracheobronchus. Between July 1988 and March 1996 tracheobronchial surgery was performed on 40 patients who had a variety of malignant diseases. The primary diseases were bronchogenic carcinoma ( n = 26), tracheobronchial tumour ( n = 5), thyroid cancer ( n = 6), and oesophageal cancer ( n = 3). Operative procedures that were performed on the tracheobronchus were sleeve lobectomy ( n = 22) or bilobectomies ( n = 5), sleeve pneumonectomy ( n = 3), sleeve resection of trachea ( n = 7) and bronchus ( n = 3). There was one postoperative death with a mortality rate of 2.5%. However, there were no significant postoperative complications apart from the one postoperative death; one patient developed a bronchopleural fistula and empyema. In lung cancer patients, the 2 year survival rate was 47.3%, and one (3.8%) local tumour recurrence. Four of five patients who had tracheobronchial tumours were alive and free from disease during 2–6 year follow-up period. One patient who had malignant fibrous histiocytoma died of brain metastasis 6 months after the operation. Among six patients whose thyroid cancer involved the trachea, one patient survived for 7 years, the other five patients were still alive and free from disease 2–5 years after the operation. Of the three patients whose oesophageal carcinoma involved the tracheobronchus, there was one operative death and the others died of tumour recurrence 1 and 2 years, respectively. We suggest that tracheobronchoplasty is a safe procedure with low morbidity and mortality rates in carefully selected patients with malignant diseases.     

Churg-Strauss syndrome revealed by granulomatous acute pericarditis: two case reports and a review of the literature

BACKGROUND: Churg-Strauss syndrome (CSS) is a necrotizing systemic vasculitis with extravascular granulomas and eosinophilic infiltrates of small vessels. CSS is usually revealed by nonspecific signs of necrotizing vasculitis in a context of late-onset asthma and blood eosinophilia. It is considered a systemic vasculitis with the highest prevalence of cardiac involvement and can lead to rapid-onset heart failure due to specific cardiomyopathy. Pericardial effusion may also occur during CSS and is usually well tolerated. OBJECTIVE: The objective of these case reports was to indicate that CSS may present as tamponade, with or without other visceral involvement. METHODS: Among CSS patients treated during the past 10 years at 2 French university hospitals, we have identified and described 2 cases revealed by tamponade with pericardial biopsy-proven granulomatous vasculitis. We have also reviewed the international medical literature in PubMed on cardiac involvement in CSS. RESULTS: The first case report describes a 66-year-old man who had an isolated cardiac tamponade with both inflammatory syndrome and eosinophilia. Long-term remission was obtained with corticosteroids. The second case report describes a 46-year-old woman whose CSS presented with tamponade and associated central nervous system and myocardial involvement. Remission was obtained with corticosteroids and cyclophosphamide. In both cases, CSS was assessed by histological analysis of a pericardial sample. CONCLUSIONS: CSS may present as isolated cardiac tamponade. Whereas pericarditis with myocardial injury warrants immunosuppressive therapy, isolated pericarditis without other visceral involvement of poor prognosis only requires corticosteroid therapy.     

内镜下黏膜切除术治疗消化道平坦型病变103例分析

目的回顾分析内镜下黏膜切除术（EMR）对消化道平坦型病变的治疗效果，并讨论其并发症和操作方法。方法103例共114处消化道平坦型病变，以注射法或透明帽辅助法行黏膜切除治疗，记录其操作方法、并发症、术后病珲类型及随访情况。结果113处病变经EMR或EPMR完整切除，治愈率99．12％。并发症：术中出血7例（6．80％），以APC止血成功。术后迟发出血1例（0．97％），内科保守治疗成功，无穿孑L发生。术后病理：良性腺瘤73处，黏膜肌层平滑肌瘤5处，乳头状瘤2处，增生性息肉11处，炎性息肉17处，黏膜内癌（m癌）3处，黏膜下层癌（sml癌）2处，同有肌层癌（nap癌）1处。sml癌追加外科肠段切除，手术标本未见肿瘤残存或浸润。mp癌行黏膜下注射后抬举征可疑阳性，术后追加外科肠段切除，可见肠旁淋巴结转移（1／6）。癌变病例随访3～9月，未见病变残留或复发。结论镜下黏膜切除术可安全有效切除大多数消化道平坦型病变。〉10min病变术后应常规作病理检查。采用纯切模式，较大功率有助于保持标本完整性。     

Multiple colonic ulcers caused by Churg-Strauss syndrome in a 15-year-old girl

Churg–Strauss syndrome (CSS), or allergic granulomatous angiitis, is an uncommon vasculitic syndrome that is found mainly in middle-aged adults. We describe a 15-year-old girl with CSS, diagnosed by histological findings and characteristic clinical features. The patient experienced two episodes of catastrophic gastrointestinal vasculitis, resulting in resection of 150 cm of small intestine and right hemicolectomy. Colonoscopic examination showed multiple colonic ulcers with active bleeding. The clinical course of the patient was grave and refractory to the therapy of steroid and cytotoxic drugs. In the world literature only two patients with multiple colonic ulcers caused by CSS have been reported, and very rare cases of childhood-onset CSS have been published. We reviewed CSS in children and found that the prognosis was poorer than that in adults. Received: 4 December 2000 / Accepted: 9 March 2001     

三叶因子2在胃黏膜病变中的表达

[目的]观察三叶因子2(TFF2)在胃黏膜病变中的表达及临床意义。[方法]在浅表性胃炎、糜烂性胃炎及胃溃疡3种轻、中、重病变的胃病患者胃黏膜中测定TFF2。观察TFF2在这三个阶段的变化。[结果]TFF2的表达在浅表性胃炎中低、在糜烂性胃炎中次低,在胃溃疡中最低,病情轻重程度与TFF2的表达呈负相关。[结论]TFF2具有保护胃黏膜免受致病因素攻击的作用。     

The mechanics of left ventricular dysfunction in patients with Churg-Strauss syndrome

Background: Heart is frequently involved in Churg‐Strauss syndrome (CSS). However, the mechanics of left ventricular (LV) dysfunction in CSS has not been studied. Objective: To assess the mechanics of LV function and to characterize the contribution of longitudinal, circumferential and rotational deformation to LV dysfunction in CSS. Methods: We enrolled 22 CSS patients (eight males, mean age 43.2 ± 9.5 years) in remission of their disease and 22 sex‐ and age‐matched healthy subjects. All patients underwent conventional and two‐dimensional speckle‐tracking echocardiography. Global longitudinal, circumferential and rotational deformation parameters were calculated. Results: CSS subjects demonstrated lower LV ejection fraction (EF) than controls (56.6 ± 15.0% vs 63.8 ± 3.4%; P < 0.05). When compared to those with LVEF ≥ 50% (n = 14), CSS patients with LVEF < 50% (n = 7) had decreased global peak‐systolic longitudinal and circumferential strain/strain rate (all P < 0.001) and tended to have lower global peak‐systolic radial strain (P = 0.05). There were no differences between these two subgroups in global peak‐systolic radial strain rate and LV twist/torsion. When comparing individual systolic and diastolic parameters early diastolic longitudinal and circumferential strain rate demonstrated the highest correlation with corresponding global longitudinal and circumferential peak‐systolic strain/strain rate (r < ?0.80, P < 0.001 for all correlations). Conclusions: In CSS LV systolic dysfunction strongly correlates with longitudinal and circumferential, but not radial or rotational systolic components, indicating that impaired LV systolic function may result predominantly from impaired contraction of inner and middle, but not outer myocardial fiber layers. The spatial correspondence between systolic and diastolic deformation parameters suggests the similar impact of pathologic process on systolic and diastolic function in CSS. (Echocardiography 2012;29:568‐578)     

潘托拉唑对胃黏膜损伤保护作用及其机制

目的:探讨潘托拉唑对大鼠胃黏膜损伤保护的作用及其机制.方法:在乙醇诱导大鼠胃黏膜损伤前,预先给予(iv)潘托拉唑(20mg/kg)、L-硝基-精氨酸甲酯(L-NAME,4mg/kg)及L-精氨酸(250mg/kg).采用激光多普勒血流计(LDF)测定胃黏膜血流量(GMBF),采用镉粒还原和比色法测定胃黏膜和血浆NO-2/NO-3含量,并观察了胃黏膜损伤指数(ulcerindex,UI)、溃疡坏死组织和中性粒细胞浸润严重程度的变化.结果:与模型损伤组比,潘托拉唑组大鼠UI明显降低(5.7±2.1vs25.4±2.5,P<0.01),溃疡坏死组织和中性粒细胞浸润程度明显减轻.预先用L-NAME处理后,潘托拉唑保护胃黏膜损伤作用明显减弱;L-NAME抑制作用可被L-精氨酸拮抗.iv潘托拉唑,可增加GMBF、胃黏膜和血浆NO-2/NO-3,L-NAME可逆转这种作用,但对潘托拉唑抑制酸分泌作用无明显影响.结论:潘托拉唑通过一氧化氮介导对大鼠胃黏膜损伤有重要的保护作用,而与潘托拉唑抑制酸分泌作用无关.     

Churg–Strauss syndrome: a retrospective study of 11 cases from a single center in Japan

Objective: To investigate the clinical characteristics of patients with Churg–Strauss syndrome (CSS), including symptoms, blood chemistry and immunological findings. Patients and methods: We retrospectively investigated the records of 11 patients (six female and five male) with CSS admitted to our hospital from September 2003 to October 2009. Results: Eight patients had preceding symptoms including bronchial asthma and allergic rhinitis. Seven patients showed eosinophilia. Nine patients had mononeuritis multiplex. Positive findings of myeloperoxidase‐antineutrophil cytoplasmic antibody (MPO‐ANCA) were found in five patients. Neither clinical manifestations nor laboratory findings were correlated with positivity for MPO‐ANCA. However, the MPO‐ANCA‐positive group showed a higher level of blood urea nitrogen and proteinuria than those negative for MPO‐ANCA. Ten patients recovered after starting steroid or immunosuppressive therapy, although one patient died of unknown etiology. Conclusion: Although general assessments based on various factors such as medical history, clinical manifestation and laboratory studies are indispensable in CSS, MPO‐ANCA might be useful as a predictor of renal dysfunction in patients with CSS.     

一氧化氮在雷贝拉唑对大鼠胃黏膜损伤保护中的作用

目的:探讨一氧化氮(NO)在雷贝拉唑对大鼠胃黏膜损伤保护中的作用．方法:在乙醇诱导大鼠胃黏膜损伤前,预先给予雷贝拉唑(20 mg/kg)灌胃,1-硝基-精氨酸甲酯(1-NAME,4 mg／kg)、1-精氨酸(250 mg／kg)及d-精氨酸(250 mg／kg)iv．采用激光多普勒血流计(LDF)测定胃黏膜血流量(GMBF),采用镉粒还原和比色法测定胃黏膜和血浆NO-2／NO-3含量,并观察胃黏膜损伤指数(UI)、溃疡坏死组织和中性粒细胞浸润严重程度的变化．结果:与模型损伤组比,雷贝拉唑组大鼠UI明显降低(5．5±0．5 vs 25．2±2.3,P<0．01),溃疡坏死组织和中性粒细胞浸润程度明显减轻(坏死物质 → ／≤ :1／9 vs 8／2,P<0．01;中性粒细胞 → ／≤ :3／7 vs 9／1,P<0．01)．预先用1-NAME处理后,雷贝拉唑保护胃黏膜损伤作用明显减弱;1-NAME抑制作用可被1-精氨酸拮抗,而不被d-精氨酸拮抗．向胃内灌注雷贝拉唑,可增加GMBF、胃黏膜和血浆NO-2／NO-3,1-NAME可逆转这种作用,但对雷贝拉唑抑制酸分泌作用无明显影响．结论:雷贝拉唑对大鼠胃黏膜损伤保护作用与NO有关,而与雷贝拉唑抑制酸分泌作用无关．     

内镜下黏膜切除术治疗Peutz-Jeghers巨大十二指肠息肉71例临床研究北大核心CSCD

目的评估内镜下黏膜切除术(EMR)治疗Peutz-Jeghers综合征(PJS)巨大十二指肠息肉的安全性及有效性。方法收集2013年2月至2020年8月在空军特色医学中心确诊为PJS十二指肠息肉并经EMR治疗的病例资料,统计EMR治疗PJS十二指肠巨大息肉的完整切除率、并发症发生率。比较巨大息肉组(直径≥3 cm)与普通息肉组(直径<3 cm)患者EMR手术完整切除率和并发症发生情况,并分析EMR治疗PJS十二指肠息肉手术并发症发生的影响因素。结果共71例患者纳入研究,男44例,女27例,中位年龄为26岁(5~58岁)。内镜下切除息肉最大中位直径为2.0 cm(0.6~13.0 cm),所有患者均成功实施EMR手术,63例患者EMR治疗PJS十二指肠息肉实现完整切除(63/71,88.7%),巨大息肉组EMR手术完整切除率低于普通息肉组(77.4%比97.5%),差异有统计学意义(P=0.023)。EMR手术相关并发症总发生率5.6%(4/71),1例患者同时存在术中创面渗血和术后胰腺炎。巨大息肉组与普通息肉组间并发症发生率(9.7%比2.5%)差异无统计学意义(P>0.05)。内镜下EMR切除十二指肠息肉有无并发症发生在患者性别、年龄、有无PJS家族史、手术史、息肉数量、切除方式上的差异均无统计学意义(P值均>0.05),而息肉位于乳头部位者并发症发生率(50%,3/6)显著高于非乳头部位者(1.5%,1/65),差异有统计学意义(P=0.001)。结论EMR治疗PJS巨大十二指肠息肉总体安全有效的,可作为PJS十二指肠息肉的首选治疗方案。息肉部位是EMR手术相关并发症发生的重要影响因素。     

Large-vessel arteritis and myelodysplastic syndrome: report of two cases

Myelodysplastic syndrome (MDS) is frequently associated with autoimmune diseases such as polymyalgia, arthritis, and rarely, with systemic vasculitis. The pathogenesis of these autoimmune complications remains unknown, but there is increasing evidence of profound immune dysregulation in MDS. In the few cases reported so far, vasculitides associated with MDS affected mainly cutaneous vessels. Here we describe two cases of acute large-vessel vasculitis in association with MDS. The first patient is a 67-yr-old male presenting with a massive large-vessel arteritis as primary manifestation of refractory anemia with excess of blasts type 1 (RAEB-1). The second patient is a 60-yr-old male, who presented with acute thoracic aortitis after a 2-yr history of refractory anemia with ringed sideroblasts (RARS). Both patients received immunosuppressive treatment with steroids, leading to rapid improvement of systemic inflammatory symptoms, vessel wall injury and peripheral blood counts. Whereas the first patient displayed sustained favorable hematologic responses under long-term steroid therapy, there was a rapid transformation into secondary acute myeloid leukemia in the second patient. We conclude that large-vessel vasculitis should be added to the list of potential autoimmune complications in MDS. In this clinical setting, steroid therapy may alleviate inflammatory symptoms and result in beneficial hematologic responses.     

Two cases of necrotizing crescentic glomerulonephritis with skin lesions

Two cases of rapidly progressive glomerulonephritis (RPGN) with skin vasculitis-associated anti-neutrophil cytoplasmic antibody (ANCA) are examined. Both cases showed purpura on the lower legs on admission and subsequently revealed renal or pulmonary vasculitis. One case responded positively to oral prednisolone alone, but the other case showed high ANCA titer and renal—pulmonary vasculitis. We discuss the diversity of clinical symptoms and the titer of antibody-associated vasculitis.     

Varioliform mucosal polypoid lesions in intestinal tract in a patient with adult T-cell leukemia

We describe a case of adult T-cell leukemia (ATL) with intestinal infiltration. In the early clinical stage, the endoscopic findings for the intestine were similar to those of amebic enterocolitis, i.e., varioliform mucosal polypoid lesions, and amebic cyst was detected with stool examination. Although no specific pathological factor could be identified on biopsy, the patient was treated for amebiasis as a diagnostic therapy. the findings of varioliform mucosal polypoid lesions were detected in the duodenum on endoscopic examination, but the lesions eventually disappeared during the treatment for amebiasis. We then suspected lymphoma partially masked by, the amebiasis. Immunological staining of a specimen of the colonic mucosa revealed T cell invasion and Southern blotting demonstrated adult T-cell leukemia provirus invasion. Thus, ATL cell infiltration of the intestinal tract was confirmed It is suggested that systemic disease should also be considered when varioliform mucosal polypoid lesions are found on colonoscopic examination.     

CASE REPORT: Endoscopic ultrasonographic findings of mucosal prolapse syndrome

A 41-year-old man with mucosal prolapse syndrome received endoscopic ultrasonography. The lesion was mildly hypoechoic and irregular, with extension into the muscularis propria and disappearance of the normal mucosal and submucosal layers. In addition, an enlargement of the muscularis propria, bridging towards the mucosal layer with merging of the two layers, was observed. These findings suggest the usefulness of endoscopic ultrasonography in the diagnosis of mucosal prolapse syndrome.     

Familial Churg-Strauss syndrome in two sisters

Churg-Strauss syndrome (CSS) is an uncommon systemic vasculitis with an increase in the number of eosinophils in the peripheral blood and tissues. Its pathogenesis is unknown, and there is no evidence that genetic factors influence susceptibility to this disease. We present a case of familial CSS in two sisters with atopic-type bronchial asthma and negative perinuclear anti-neutrophil cytoplasmic antibody results. We investigated the human leukocyte antigen typing of the sisters and their six living siblings but found no evidence for heritability of CSS. To our knowledge, this is the first report of familial CSS.     

Cardiac involvement in Churg-Strauss syndrome: a follow-up of three cases

Three cases of Churg-Strauss syndrome with myocardial involvementare reported. Cardiac disease is known to be the major causeof death in Churg-Strauss syndrome. Aggressive therapy (steroidsand cyclophosphamide) may cure the myocardial dysfunction associatedwith the disease.     

热休克预处理对大鼠烧伤后急性胃黏膜损伤的保护作用

目的观察热休克预处理对严重烧伤大鼠胃黏膜的影响。方法热休克预处理诱导热休克蛋白产生,然后制作烧伤大鼠急性胃黏膜损伤模型,进行胃黏膜损伤指数评分。运用免疫组化方法,检测胃黏膜热休克蛋白的表达,并进行图像分析。结果热休克预处理组胃黏膜损伤积分显著低于对照组(P<0.01)。免疫组化分析显示其胃黏膜热休克蛋白表达较对照组明显增强(P<0.01)。结论热休克预处理对烧伤大鼠胃黏膜具有保护作用,其保护机制可能与热休克蛋白诱导表达增强有关。     

内镜分片黏膜切除术治疗巨大结直肠平坦型病变的临床评价及复发率随访

目的评价内镜分片黏膜切除术(endoscopic piecemeal mucosal resection,EPMR)治疗巨大结直肠平坦型病变的疗效、安全性及应用价值,并随访观察其复发率。方法经术前内镜检查评估及病理证实,符合内镜切除适应证,采用EPMR切除46例直径≥30 mm结直肠平坦型病变,均为侧向发育型肿瘤(LST),术后标本全部行精确的病理学评价。结果 46例结直肠LST患者中,病灶位于直肠38例,占82.6%,完全切除率93.5%(43/46),切除治愈率97.8%(45/46)。术后出血发生率为4.3%,均经内镜止血成功,无1例发生穿孔。术后病理均为腺瘤,伴低级别上皮内瘤变30例,伴高级别上皮内瘤变10例,黏膜内癌6例。全组患者随访3~96个月,仅2例复发,复发率4.3%,再次内镜处理获愈。结论 EPMR是治疗巨大结直肠平坦型病变安全、有效的方法,掌握切除方法和技巧,可以完全切除病变,减少残留及复发率。     

Role of mucosal microcirculation in gastric lesions induced by lateral hypothalamic lesions in rats

To evaluate the pathophysiology underlying gastric mucosal lesions induced by lateral hypothalamic (LH) lesions, we investigated the changes in acid secretion, gastric mucosal blood flow, gastric mucus and mucosal integrity in the corpus during the 4 h period and 48 h after the production of bilateral electrolytic LH lesions in male Sprague-Dawley rats. Gastric mucosal lesions were macroscopically produced 24 h (63%) and 48 h (83%) after LH lesions, although there were no visible lesions at 7 h. Gastric acid secretion was significantly increased 48 h after LH lesions, compared with that in the control group. Gastric mucosal blood flow and transmucosal potential difference (PD) in the LH lesion group immediately decreased after LH lesions and did not recover during 4 h and at 48 h. On the contrary, in the control group, gastric mucosal blood flow decreased after the brain surgery but soon recovered, and there was no significant change in PD. LH lesions resulted in the reduction of intramucosal mucus to 50% 3 h after LH lesions. Moreover, we exposed the stomach to 10 mmol/L taurocholic acid (TCA) 3 h after LH lesions to examine the disruption in gastric mucosal defensive function in rats with LH lesions. The recovery of the reduced PD by TCA was slow and gastric mucosal lesions were easily formed in the LH lesion group. These results suggest that gastric mucosal ischaemia after lesioning of LH immediately results in the disruption of mucosal defensive function before the formation of visible gastric lesions, and predisposes to the formation of gastric mucosal lesions by a delayed increase in acid secretion.