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171.
目的 通过测定肠粘膜和血中一氧化氮的改变,探讨先天性巨结肠病理生理变化和先天性巨结肠合上肠结膜炎的原因。方法 比色法测定25例HD患者肠粘膜中一氧化氮合酶和手术前后血中NO含量。结果 (1)HD粘膜层中NOS含量狭窄段组扩张段组低,2组间差异有显著性。(2)HD狭窄段粘膜层中NOS含量较非HD粘膜层中NOS含量低,2组间差异有显著性。(3)HD术前组较非HD组血中NO含量明显减低,2组间差异有显著 相似文献
172.
Cynthia Fernández-Lainez Isabel Ibarra-González Miguel Ángel Alcántara-Ortigoza Liliana Fernández-Hernández Sergio Enríquez-Flores Ariadna González-del Ángel Nenad Blau Beat Thöny Sara Guillén-López Leticia Belmont-Martínez Matilde Ruiz-García Marcela Vela-Amieva 《Brain & development》2018,40(7):530-536
Background
Tetrahydrobiopterin (BH4) is the cofactor for 6-pyruvoyl-tetrahydropterin synthase (PTPS); it is involved in BH4 biosynthesis and is encoded by PTS gene. Its deficiency (PTPSD) is characterized by hyperphenylalaninemia (HPA) and deficit in central monoamine neurotransmitters. We describe the clinical and mutational spectrum of five patients with PTPSD, from four unrelated Mexican families. All patients had symptomatic diagnosis and presented severe early neurological manifestations and HPA.Methods
Clinical and biochemical data from studied patients were recorded. Responsible PTPSD genotypes was determined by direct and bidirectional Sanger DNA sequencing of the six PTS coding exons and their exon-intron borders, and these were directly searched in the available relatives. The novel PTS missense variant [NM_3000317.2:331G?>?T, p.(Ala111Ser)] was subjected to in silico, to predict a possible deleterious effect.Results
Diminished fetal movements were perceived as a uniform characteristic in the studied group. DNA sequencing showed two known p.(Arg251) and p.(Val132TyrFs119) and the novel missense p.(Ala111Ser) PTS variants, the latter representing potentially a frequent PTPSD-responsible allele (50%, 4/8) in Mexican patients. In silico protein modeling analysis of the p.(Ala111Ser) variant revealed loss of hydrophobic interactions between the alanine and neighboring valines, suggesting that these changes in polarity may be detrimental for enzyme function, structure and/or stability.Conclusions
This work contributes to the knowledge of PTPS molecular spectrum. The delayed diagnosis of these patients emphasizes the importance of considering BH4 metabolism defects in the differential diagnosis of HPA, especially for countries that are beginning their HPA newborn screening programs. 相似文献173.
目的 分析隐源性多灶性溃疡狭窄性小肠炎(CMUSE)临床特征,为临床诊治提供参考。方法 结合首都医科大学附属北京世纪坛医院2012年2月收治的1例CMUSE病例,检索中英文数据库,收集确诊或疑诊为CMUSE的个案报道,并对病例的一般资料、临床表现、实验室检查、影像学检查、内镜检查、病理检查、治疗方法与转归进行总结分析。结果 共收集22例患者,确诊18例,疑诊4例;男10例,女12例;中位年龄为32岁,自发病至确诊的中位时间为3年。常见的临床表现有腹痛(20例),腹胀(10例),恶心、呕吐(8例),便血、黑便(7例)等;实验室检查低清蛋白血症12例,便隐血试验阳性11例;11例行小肠镜检查,9例行胶囊内镜检查,8例行小肠气钡双重对比造影,均可见阳性改变;12例行手术治疗,7例术后复发;12例行糖皮质激素治疗,8例病情缓解,4例无效;1例英夫利昔单抗治疗有效。结论 CMUSE是一种罕见的、病因不明的疾病,小肠多发浅表溃疡和肠腔狭窄是其典型表现。胶囊内镜、双气囊小肠镜对该疾病的诊断有重要意义,激素为其一线治疗,内镜下球囊扩张术开始用于治疗,其他治疗有营养支持、外科手术等。 相似文献
174.
细菌性痢疾是由志贺菌属引起的以发热、腹痛、腹泻、排黏液脓血便、里急后重等为主要症状的急性肠道传染病,以喹诺酮类、氨基糖苷类及β-内酰胺类抗生素治疗为主。伪膜性肠炎是抗生素相关性腹泻的一种特殊类型,主要表现为腹泻、腹胀、腹痛和发热。两者临床表现非常相似,临床上易误诊或漏诊,如不尽早诊断并及时治疗,后果严重。现将我院腹泻门诊收集的5例细菌性痢疾并发伪膜性肠炎报道如下。 相似文献
175.
目的系统评价新生儿坏死性小肠结肠炎的危险因素,为制订预防措施提供循证依据。方法电子检索MEDLINE(1980年第1期至2009年第7期)、EMBASE(1980年第1期至2009年第7期)、Cochrane图书馆(2009年第3期)和中国期刊全文数据库(1994年第1期至2009年第7期)、中文科技期刊全文数据库(1989年第1期至2009年第7期)、中国生物医学文献数据库(1978年第1期至2009年第7期),并手工检索相关杂志。纳入相关的病例对照研究,采用RevMan5.0软件和Stata软件进行Meta分析。结果共纳入14篇相关文献,均为病例对照研究,其中中文1篇,英文13篇,累计病例624例,对照2340例。结论本研究显示预防新生儿呼吸窘迫综合征和败血症有助于降低新生儿坏死性小肠结肠炎的发生率。 相似文献
176.
目的 探讨老年患者使用广谱抗生素诱发难辨梭菌性结肠炎的临床特点、内镜下改变及防治措施。 方法 分析8例老年难辨梭菌性结肠炎患者的临床资料,对难辨梭菌分离、鉴定,并行细胞毒素测定。 结果 所用抗生素的种类繁多,在使用抗生素后3 天至2周内发生腹泻,重者伴腹膜炎,4 例行纤维结肠镜检查均见有小片至大斑片伪膜附着;治疗措施为停用原抗菌药物,应用万古霉素和(或)甲硝唑及加强支持疗法。8 例中7例治愈,1例死亡。 结论 老年人特别是术后使用广谱抗生素者易诱发难辨梭菌性结肠炎 相似文献
177.
Objective To study the effects of ITF on PI3K (phosphatidylinositol 3-kinase) and caspases-3/9 (cysteinyl aspartate-specific protea-ses) in neonatal rat NEC model. Methods NEC model of neonatal rats was established. Asphyxia stress was accomplished by exposure to l00% nitrogen for 60s, followed by exposure to coldness (4 ℃ ) for 10 min twice daily. Neonatal rats were fed formula (200 kcal/kg/day) every 3 h via an gavage tube. The feeding volume began at 0. 1 cc every 3 h and was increased incrementally. This procedure is done once everyday and continued for 3 days. On the 4th day,all the subjects were sacrificed. Fifty neonatal rats were randomized into five groups: A) NEC + NS0. 2mi, (B) NEC + ITF 0. 2 mg, (C) NEC(Wortmannin (0. 1 mg/kg) (D) NEC + ITF 0. 2 mg + Wortmannin (0. 1 mg/kg), (E) control. The intestinal tissue located at the boundary of ileum and cecum was sampled for histology. The remaining intestinal tissue was homogenized. After the homogenate was centrifuged,supernatants were used to assay PI3K and Caspase-3 and caspase-9. Results The pathological lesions showed that intestinal necrosis was severe in group A、C and D, which was graded as 3points. They were significantly decreased in group B,which was graded 1 point. The level of PI3K(pg/ml)in Group A was higher than those in group E (P<0. 05), the latter had difference with those in group D(P>0. 05). The level of PI3K(pg/ml) were the strongest in group B and were lowest in group C. The activity of Caspase-3 and Caspase-9 were significantly increased in group A compared to those in group B and E (P<0. 01 ),and was significantly increased in Group C(P<0. 01 ). Caspase-3 and Caspase-9 levels of group A and D,group B and E showed no significant difference(P>0. 05). Conclusions Intestinal inflammation was ameliorated by intraperitoneal ITF. ITF may provide a new therapy for NEC; PI3K/Akt signal pathways might play important roles in signal transduction during NEC;ITF may protect the intestinal injury of neonatal Wistar rat by activation of PI3K/Akt signal transduction pathway, and down regulation of caspase-9. 相似文献
178.
Cannioto Z Berti I Martelossi S Bruno I Giurici N Crovella S Ventura A 《European journal of pediatrics》2009,168(2):149-155
Inflammatory bowel disease (IBD) is uncommon in children younger than 2 years of age. The criteria for differentiating IBD
from other diseases with similar clinical presentation is unclear. We describe 16 patients who, between 1984 and 2004, received
a histological diagnosis of IBD during the first two years of life. Six patients presented with histological Crohn’s disease,
eight with ulcerative colitis and two with indeterminate colitis. The median age at diagnosis was 125 days (range 1 day to
18 months) and the medium follow up was 89 months (range 65 days to 20 years). The disease appeared to be very severe: four
children (25%) underwent total parenteral nutrition (TPN), two received colectomy (12.5%) and three patients died. Many of
the patients required an aggressive, multidrug, immunosuppressive approach (azathioprine [AZA], Infliximab, thalidomide, cyclosporine
A). One child presented with a hypogammaglobulinaemia without any specific immunodeficiency, while in the other patients,
Wiskott-Aldrich syndrome (WAS) (4 cases) and chronic granulomatous disease (CGD) (2 cases) were identified. In 6/16 cases,
allergic colitis was first considered; these cases initially underwent cow’s milk protein-free diet as the only therapy before
IBD was finally diagnosed. In conclusion, early IBD has a severe prognosis and often needs an aggressive therapeutic approach.
Furthermore, an improper diagnosis of allergic colitis might cause an important diagnostic delay. Some severe immunodeficiencies,
such as WAS and CGD, may represent a problem in terms of differential diagnosis and might be wrongly diagnosed as very early
onset IBD. 相似文献
179.
Nathan Selsky Faripour Forouhar George Y Wu 《World journal of gastroenterology : WJG》2013,19(37):6296-6298
A 49 years old Vietnamese male with a history of thalassemia,presented with gastrointestinal symptoms and signs of hemolysis.He was diagnosed with yersinia enterocolitis.Yersinia is a gram-negative rod that most frequently occurs in children especially during the winter months.In the current case,the bone marrow biopsy showed hemophagocytosis along with positive cultures for Yersinia.The microorganism likely triggered hemophagocytosis.This syndrome,also known as,hemophagocytic lymphohistiocytosis,is defined by fever for more than 7 d,cytopenia of two or more cell lines,hemophagocytosis,hepatitis,serum ferritin greater than500,jaundice,lymphadenopathy,and hepatosplenomegaly.This disorder can be either familial or secondary to a strong immunologic activation.Both have an overwhelming activation of T-cells and macrophages. 相似文献
180.
新生儿坏死性小肠结肠炎(NEC)是导致早产儿死亡的主要疾病之一。目前临床一致认为,早产儿的喂养方式,包括乳类选择、开奶时间、增加奶量方式等,对新生儿NEC发病及复发影响较大,但临床对于预防新生儿NEC发生的标准喂养方式,迄今尚未达成共识。笔者拟就预防新生儿NEC喂养方式的研究进展进行综述,为进一步研究预防新生儿NEC的喂养方式提供参考依据。 相似文献