TSLP在溃疡性结肠炎患者结肠黏膜的表达及临床意义

目的探讨胸腺基质淋巴细胞生成素(TSLP)在溃疡性结肠炎患者结肠黏膜上皮内的表达及其临床意义。方法分别采集溃疡性结肠炎患者结肠黏膜组织22例,健康人群结肠黏膜组织20例,将溃疡性结肠炎患者进行Mayo评分及分期,分别测出其血清C反应蛋白(C-reactive protein,CRP)、红细胞沉降率(erythrocyte sedimentation rate,ESR)水平,通过免疫荧光法及免疫印迹法(Western blot,WB)检测溃疡性结肠炎患者结肠黏膜TSLP蛋白的表达,比较免疫印迹结果与CRP、ESR水平及Mayo评分的相关性。结果溃疡性结肠炎患者TSLP表达水平明显增加,主要表达于肠上皮腺体及基质内炎性细胞中;UC患者肠上皮黏膜内TSLP相对表达为1.076±0.091,健康对照组0.604±0.098,且与CRP、ESR水平呈正相关,与Mayo评分即疾病的活动度呈正相关。结论 TSLP的过表达提示其可能参与UC的发病机制。     

溃疡性结肠炎患者血清25(OH)维生素D水平与疾病活动性的相关性

目的检测溃疡性结肠炎患者血清25(OH)维生素D水平,并分析25(OH)维生素D水平与疾病活动性的关系,探讨其临床意义。方法选择40例溃疡性结肠炎患者和30例健康对照者为研究对象,采用ELISA法检测溃疡性结肠炎患者和健康体检者血清中25(OH)维生素D水平,应用Mayo评分法对UC病人的疾病活动度进行分级,将维生素D水平与UC患者临床资料以及疾病活动度进行相关性分析。结果溃疡性结肠炎患者血清25(OH)维生素D水平(15.8±12.3)ng/ml明显低于健康对照组(42.5±26.7)ng/ml,p0.05,血清25(OH)维生素D水平与患者年龄、性别、病程、是否吸烟、抗生素的应用、5-氨基水杨酸的应用及非甾体类抗炎药的应用无相关性,但与皮质类固醇激素的应用及溃疡性结肠炎的活动性相关。结论溃疡性结肠炎患者低储备维生素D水平与疾病活动性及皮质类固醇激素的应用相关。     

血清UCH-L1与NGAL对重度脑损伤患者的病情及短期预后的评估价值

目的分析血清泛素羧基末端水解酶L1(UCH-L1)与中性粒细胞明胶酶相关脂质运载蛋白(NGAL)水平对评估重度脑损伤(SBI)患者病情与预后结局的临床价值。方法选择我院就诊的88例脑损伤患者作为研究对象,根据格拉斯哥昏迷评分(GCS)水平分为:轻度组(49例,GCS评分≥9)和重度组(39例,GCS评分<9)。另选择同时期健康体检者50例为对照组。检测并比较三组入院时血清UCH-L1与NGAL水平的差异。采用Pearson相关分析评估血清UCH-L1与NGAL水平分别与GCS评分的相关性。随访6月,依据患者预后情况分为预后不良组和预后良好组,比较两组血清UCH-L1、NGAL水平以及GCS评分的差异。应用多元Logistic回归分析与SBI患者短期预后相关危险因素。应用受试者工作特征曲线(ROC)评估血清NGAL与UCH-L1水平对SBI患者随访6月预后生存的诊断性能。结果重度组血清UCH-L1与NGAL水平显著高于轻度组,而轻度组血清UCH-L1与NGAL水平显著高于对照组(UCH-L1水平分别为28.7±5.2、15.4±3.6、10.5±2.3ng/mL,NGAL水平分别为253.8±33.4、161.5±27.3、136.6±25.1ng/mL,均P<0.05)。Pearson相关分析显示脑损伤患者血清UCH-L1与NGAL水平均分别与GCS评分呈线性负相关(r=-0.713,P=0.023、r=-0.852,P=0.011)。随访6月,预后不良组血清UCH-L1及NGAL水平均高于预后良好组(UCH-L1水平分别为36.2±3.9、11.6±2.1ng/mL,NGAL水平分别为285.8±24.6、114.3±10.2ng/mL,均P<0.05),而GCS评分低于预后良好组(GCS评分分别为5.2±1.2、13.8±1.1,P<0.05)。多元Logistic回归分析显示,血清UCH-L1及NGAL水平为随访6月SBI患者生存相关的危险因素(OR=2.765,P=0.029、OR=2.965,P=0.024)。ROC曲线显示,血清UCH-L1截断值为19.5ng/mL,预测SBI患者随访6月预后不良的AUC为0.826,灵敏度为89.4%,特异性为80.1%。而血清NGAL截断值为182.5ng/mL,预测的AUC为0.895,灵敏度为91.2%,特异性为85.7%。两者联合预测的AUC为0.927,灵敏度为93.2%,特异性为88.9%。结论血清UCH-L1与NGAL水平能有效评估颅脑损伤程度,对短期预后判断有较高价值。     

血清降钙素原及C反应蛋白检测在老年肺炎抗菌药物治疗中的价值

目的:探讨血清降钙素原(PCT)、C-反应蛋白(CRP)检测在指导老年肺炎抗菌治疗中的作用。方法:2015-05—2016-03本院呼吸内科收治的老年肺炎患者91例,入院时PCT0.50ng/ml,CRP6.00ng/ml,随机分为观察组(n=44)和对照组(n=47),两组患者均根据抗菌药物的临床应用原则进行治疗。治疗过程中,对照组依据患者体征、临床表现等变化,调整或停止抗生素的应用;观察组患者则根据患者血清PCT和CRP的水平并结合临床表现调整或停止抗生素的治疗。比较两组患者住院时间、住院费用和抗生素使用时间的差异。结果:治疗前两组患者PCT、CRP、白细胞计数(WBC)及血沉(ESR)水平差异无统计学意义,治疗后,观察组患者PCT、CRP、WBC和ESR均明显低于对照组(P0.05);观察组患者抗生素使用时间明显短于对照组(P0.05);观察组老年肺炎患者PCT、CRP、WBC与抗生素使用时间长短均呈显著正相关(r=0.684、0.720、0.430,P0.01)。结论:血清PCT、CRP检测指导老年肺炎抗菌治疗可缩短抗生素使用时间,值得临床推广应用。     

溃疡性结肠炎患者外周血Tim-4 mRNA表达水平的变化及意义

目的 探究溃疡性结肠炎患者外周血Tim-4 mRNA的表达变化及临床意义。方法 选取2015年2月~2019年4月我院收治的溃疡性结肠炎患者38例作为观察组,另选取同期来我院体检的健康者30名作为对照组,采用荧光定量PCR测定外周血Tim-4 mRNA表达水平,比较两组Tim-4 mRNA、C反应蛋白（CRP）、红细胞沉降率（ESR）表达水平及观察组Tim-4 mRNA与CRP和ESR的相关性。结果 观察组Tim-4 mRNA、CRP、ESR表达均高于对照组[（0.79±0.37）vs（0.33±0.20）]、[（6.08±3.21）mg/L vs（0.99±0.87）mg/L]、[（19.12±12.13）mm/h vs（5.58±4.10）mm/h],差异有统计学意义（P＜0.05）。观察组外周血单个核细胞Tim-4 mRNA表达水平与CRP呈正相关（r=0.376,P＜0.05）,而与ESR无相关性（r=0.077,P＞0.05）。结论 溃疡性结肠炎患者Tim-4 mRNA异常上调,并与CRP密切相关,可能参与溃疡性结肠炎的致病过程。     

血清基质金属蛋白酶-3与类风湿关节炎的相关性研究

目的 探讨血清基质金属蛋白酶-3（matrix metalloproteinases-3,MMP-3）与类风湿关节炎（rheumatoid arthritis,RA）及其炎症反应的相关性.方法 用免疫比浊法（immunoturbidimetric assays）检测146例RA患者（RA组）及232例非RA人群（对照组）血清MMP-3水平;对两组MMP-3结果进行ROC曲线分析;分析RA患者血清MMP-3与反映RA患者炎症活动程度临床指标的关系.结果 RA组血清MMP-3浓度为170.00±19.36ng/mL,明显高于对照组的47.92±1.98ng/mL,差异有统计学意义（P＜0.001）.血清MMP-3诊断RA的ROC曲线下面积为0.731,取诊断界限值为71.65 ng/mL时,敏感度为58.9％,特异度为88.79％.RA组患者血清MMP-3水平与其他RA诊断以及反应RA炎症反应的指标,如C反应蛋白（C-reaction protein,CRP）、前白蛋白（prealbumin,PA）、血沉（erythrocyte sedimentation rate,ESR）、血小板（platelet）以及白细胞计数（WBC）均具有显著相关性,P值均＜0.001.结论 RA患者血清MMP-3水平显著高于正常入,其水平与反映RA患者炎症活动程度临床指标CRP、PA、ESR、PLT以及WBC具有显著相关性.类风湿性关节炎患者血清MMP-3水平与类风湿性关节炎的炎症活动性有关.     

溃疡性结肠炎相关癌的回顾性探讨

目的 通过对565例溃疡性结肠炎患者进行癌变风险分析,为溃疡性结肠炎相关癌（UC-CRC）的早期筛查及预防提供依据.方法 对收治的565例溃疡性结肠炎患者进行分组,其中15例UC-CRC组患者,随机抽取28例溃疡性结肠炎组患者,对两组临床相关资料进行回顾性分析.结果 在565例溃疡性结肠炎患者中,UC-CRC组患者有15例,总体风险为3.77％.两组患者在年龄、病变内镜活检、疾病病程、血清CRP水平等方面差异有统计学意义（P ＜0.05,P＜0.01）.高危风险因素显示：疾病病程、病变范围、血清CRP水平、病理诊断为UC-CRC的高危因素.结论 溃疡性结肠炎患者中有较高的UC-CRC癌变风险,溃疡性结肠炎的疾病病程、血清CRP水平、内镜活检为不典型增生及程度与UC-CRC的发生呈正相关.     

血清25(OH)D与克罗恩病患者病变部位及疾病严重程度的相关性研究

目的 探讨克罗恩病(Crohn’s disease, CD)患者血清25(OH)D水平与病变部位、疾病活动性、肠道炎症程度以及激素治疗等因素的相关性。方法 收集2020年1月至2022年12月在南京鼓楼医院就诊的CD患者97例，及同期在体检中心进行健康体检者79例。比较两组人群25(OH)D水平的差异。记录CD患者就诊时的一般资料、临床表现、肠镜检查结果、白细胞计数(WBC)、血红蛋白(Hb)、C反应蛋白(CRP)、血沉(ESR)、血清白蛋白(ALB)水平及用药情况，并分析25(OH)D水平与其相关性。结果 CD组的25(OH)D水平显著低于健康对照组[12.87(3.00～33.84)ng/mL vs 19.97(16.28～39.61)ng/mL](Z=-8.518,P<0.001),回结肠病变的CD患者25(OH)D水平显著低于回肠病变者[11.07(3.00～33.84)ng/mL vs 14.68(3.00～27.59)ng/mL](Z=-2.081,P=0.037),回结肠病变的CD患者25(OH)D水平明显低于结肠病变者[11.07(3.00～33.84)ng/mL...     

类风湿关节炎患者血清HMGB1与疾病活动性、炎性因子及临床指标的相关性研究

为研究RA患者血清高迁移率族蛋白1(high mobility group box protein 1,HMGB1)与RA活动性、炎性因子及临床指标的相关性,选取RA患者60例(活动期30例、非活动期30例)和健康对照者30例纳入研究。应用双抗体夹心ELISA测定血清HMGB1、IL-6、IL-8、基质金属蛋白酶3(matrix metalloproteinase 3,MMP-3)的表达水平,红外线障碍法检测ESR,免疫比浊法检测CRP及RF水平,并收集同期临床资料。结果显示,活动期组RA患者血清HMGB1水平[(10.13±1.45)ng/mL]显著高于非活动期组[(8.00±1.46)ng/mL]和健康对照组[(7.68±1.61)ng/mL](均P0.01);非活动期组RA患者HMGB1水平略高于健康对照组,但组间差异无统计学意义(P0.05)。RA患者血清HMGB1表达水平与疾病活动性呈正相关(r=0.547,P0.05)。活动期组和非活动期组RA患者血清IL-6、IL-8、MMP-3、ESR、CRP及RF水平均高于健康对照组(均P0.05),且活动期组高于非活动期组(均P0.05)。RA患者血清IL-6、IL-8、MMP-3、ESR、CRP及RF水平与疾病活动性呈正相关(r值分别为0.938、0.939、0.916、0.915、0.907、0.927,均P0.05),与血清HMGB1水平呈正相关(r值分别为0.539、0.558、0.550、0.515、0.509、0.518,均P0.05)。该研究提示,HMGB1可能参与了RA病情的发展演变过程,与RA活动性及炎性因子、临床指标的异常表达具有显著相关性,可以作为RA临床诊断的辅助指标。     

卡文治疗溃疡性结肠炎患者的疗效分析与临床意义

目的 探讨卡文治疗溃疡性结肠炎患者的疗效与临床意义.方法 选取2016年10月至2017年10月本院收治的溃疡性结肠炎患者140例,随机分为观察组(n=70)和对照组(n=70),对照组患者给予常规治疗和护理干预,观察组患者在此基础上给予卡文治疗,观察患者治疗疗效;检测患者治疗前后血清中肿瘤坏死因子-α(tumor necrosis factor alpha,TNF-α)、干扰素-γ(interferon-γ,IFN-γ)和白细胞介素-10(interleukin-10,IL-10)的水平.结果 与对照组相比,观察组患者应用卡文治疗溃疡性结肠炎后,临床治疗的有效率显著提高,临床不良反应发生率显著下降,具有统计学差异(P＜0.05).与对照组治疗后相比,观察组患者应用卡文治疗溃疡性结肠炎后,患者体内TNF-α和IFN-γ水平显著降低,IL-10水平显著升高,差异具有统计学意义(P＜0.05).溃疡性结肠炎患者体内TNF-α和IFN-γ水平与疾病程度存在显著的正性相关关系(P＜0.01);溃疡性结肠炎患者体内IL-10水平与疾病程度存在显著的负性相关关系(P＜0.01).结论 卡文辅助治疗溃疡性结肠炎具有较好的临床疗效,可有效缓解患者血清炎症因子的表达情况.     

Schizophrenia in a North Swedish geographical isolate, 1900–1977. Epidemiology, genetics and biochemistry

Over 200 schizophrenic patients belonging to three major and interrelated pedigree complexes have been investigated over the past 30 years in a North Swedish geographically isolated population, presently numbering about 6,000. An intensive investigation of a number of biochemical correlates and genetic markers in a few selected families belonging to one of the major pedigrees has indicated new strategies for the current research program.
Schizophrenia, as defined operationally, is significantly associated with decreased activities of two enzymes (1) blood platelet monoamine oxidase, (2) plasma dopamine-β-hydroxylase, and (3) with the genetic marker Gc² (group specific antigen). Both enzymes are subject to genetic variation. A positive score for linkage between schizophrenia and low plasma DBH activity has been calculated, but, so far, available data are insufficient for discrimination between linkage and partial contribution of genetically controlled low plasma DBH to the pathogenesis of the disease. Alternatively, both mechanisms could be involved.
As a model for continued research, schizophrenia is explained as based on a double dominant-recessive genotype (Aabb), representing a vulnerability which in about 50 % of cases develops into clinical schizophrenia. It is suggested that the dominant mutation (A) operates on or affects MAO activity, and that the recessive genotype (bb) is instrumental in low variates of DBH activity and very likely such variates within the normal range of physiological variation. Moreover, it is suggested that the combined effects of MAO- and DBH-reduced efficiency on the metabolism of e.g. dopamine could be an essential pathogenic mechanism for the schizophrenic illness which is segregating in this population.     

The universal muscular chain reaction,muscle spasm,Torsions, ruptures and extravasations. Chameleons of pathology and some manifestations of simple muscular disorders

Most bodily functions require the coordinated actions of complementary and supplementary paired muscle groups. Where this essential muscular cooperation is lacking, hollow organs may burst and others become literally screwed up, giving rise to many similar spastic diseases such as Torticollis, Twisted ovarian cyst, Torsion of the Testis, Volvulus of the intestines, Varicose Veins, Megacolon, Aortamegaly, Scoliosis, Erb's Palsy, Peyronie's Disease, Main-en-Griffe, Undescended Foot (Pes Cavus), Talipes, Strabismus. Spasm is “panenepidemic” and unclassified examples of Torsion Dystonia and Dyskinesia really are as common as debt and taxes.     

Hypo- und Hypermagnesämie aus kardiologischer Sicht

Zusammenfassung Eine Reihe pathologischer Zustände bedingen Magnesiummangel. Zustände mit Hypermagnesämie sind ebenfalls bekannt, doch wesentlich seltener. Für den Kardiologen beachtenswert ist, daß unter Therapie mit bestimmten Diuretica bei Herzinsuffizienz, bei Herzinfarkt, Kardiomyopathie, Digitalisintoxikation und bestimmten Herzrhythmusstörungen Hypomagnesämie beobachtet wurde. Leider kann in der klinischen Routine nur ein extracelluläres Magnesiumdefizit durch Serumbestimmungen gemessen werden; über Magnesiummangel einzelner Organe kann nichts ausgesagt werden. Hinweise für Magnesiummangel geben aber neben der Messung des Serumspiegels Anamnese, klinischer Befund, bestimmte EKG-Veränderungen wie auch evtl. Hypokalämie, ein Zustand, bei dem sich oft — besonders bei Aldosteronismus — parallele Veränderungen zeigten.Tierexperimente deuten darauf hin, daß infarktähnliche Läsionen unter Magnesiummangel entstehen, doch ob Herzinfarkt beim Menschen durch Magnesiummangel ausgelöst werden kann, ist noch ungeklärt. In Leichenherzen zeigte sich im Infarktgebiet neben Calciumakkumulation signifikanter Magnesiumverlust, wobei unklar blieb, ob sich Ursache oder Folge des Infarktes widerspiegelten. Falls ein ursächlicher Zusammenhang besteht, ist er im Myokardstoffwechsel selbst zu suchen, wie bei der Alkoholkardiomyopathie, wo myokardialer Magnesiummangel zumindest als pathogenetischer Teilfaktor anerkannt wird. Andererseits versucht man aber auch Beziehungen zwischen Atherosklerose, Blutgerinnung und Hypomagnesämie herzustellen, in der Meinung, daß Magnesiummangel auch über den coronaren Pathomechanismus des Herzinfarktes wirken könnte. Sicher scheint, daß gewisse EKG-Veränderungen und Herzrhythmusstörungen durch einen irritierten Magnesiumhaushalt bedingt sein können, da sie bei Gabe bzw. Entzug von Magnesium verschwinden. Daß Magnesiummangel die Glykosidtoleranz verringert, wird tierexperimentell bestätigt. Unter Hypomagnesämie bewirkt Acetylstrophanthidin eher und länger Rhythmusstörungen als ohne, außerdem lassen diese sich durch Magnesiumgaben eliminieren. Da in gewissen Fällen spontane und digitalisinduzierte Herzrythmusstörungen durch Magnesiuminjektionen beseitigt wurden, scheint Magnesium als Therapeuticum angebracht. Einsatz verschiedener Magnesiumsalze bei Angina pectoris, degenerativen Herzerkrankungen und Herzinsuffizienz ohne geprüften und offensichtlich gestörten Magnesiumhaushalt ist fragwürdig, weil keine eindeutigen klinischen Erfolgsbeweise vorliegen. Immerhin mag es aber larvierte, durch Serumbestimmungen nicht erfaßbare Mangelzustände geben. Allgemein erscheint es aus kardiologischer Sicht ratsam, den Magnesiumhaushalt zu überwachen und in entsprechenden Fällen auszugleichen, um möglichen Myokardläsionen oder fatalen Herzrhythmusstörungen entgegenzuwirken.     

Environmental risk factors and their role in the management of atopic dermatitis

Introduction: The etiology of atopic dermatitis (AD) is multifactorial with interaction between genetics, immune and environmental factors.

Areas covered: We review the role of prenatal exposures, irritants and pruritogens, pathogens, climate factors, including temperature, humidity, ultraviolet radiation, outdoor and indoor air pollutants, tobacco smoke exposure, water hardness, urban vs. rural living, diet, breastfeeding, probiotics and prebiotics on AD.

Expert commentary: The increased global prevalence of AD cannot be attributed to genetics alone, suggesting that evolving environmental exposures may trigger and/or flare disease in predisposed individuals. There is a complex interplay between different environmental factors, including individual use of personal care products and exposure to climate, pollution, food and other exogenous factors. Understanding these complex risk factors is crucial to developing targeted interventions to prevent the disease in millions. Moreover, patients require counseling on optimal regimens for minimization of exposure to irritants and pruritogens and other harmful exposures.     

HLA genes in Amerindians from Mexico San Vicente Tancuayalab Teenek/Huastecos

Huastecos or Teenek Amerindians are presently living at North East Mexico (San Luis Potosi State). They have probably one of the most ancient culture of Mexico and Central America together with Mayas and Olmec groups with which also show close relationships. Proximity to Atlantic Ocean/Mexican Gulf originated that Spaniards had very early contact with them at about 1519 CE or before. In the present paper we have aimed to study HLA gene profile which may be useful for HLA and disease epidemiology and transplant programs in Teeneks. HLA-DRB1*04:07, -DRB1*14:06 and -DRB1*04:11 have been found in high frequency like in other Amerindian groups. High frequency typical Amerindians HLA extended haplotypes have been found, such as A*02-B*35-DRB1*04:07-DQB1*03:02; A*68-B*39-DRB1*04:07-DQB1*03:02 and A*02-B*39-DRB1*04:07-DQB1*03:02; also new haplotypes have been described, like A*02-B*52-DRB1*04:11-DQB1*03:02, A*68-B*35-DRB1*14:02-DQB1*03:01 and A*68-B*40-DRB1*16:02-DQB1*03:01. Genetic proximity is observed not only to linguistically close Mayans, but also to Mazatecans, Mixtecans and Zapotecans, who speak an altogether different languages; it shows once more that genes and languages do not correlate. This population was greatly diminished after European contact between 1500 and 1600 years CE; in fact, North and South America First Inhabitants population was brought from 80 down to 8 million people because of diseases (i.e.: measles, smallpox or influenza), slavery and war.     

État de l’art : nouveaux anticoagulants et transfusion

Direct oral anticoagulants (DOAC) are indicated for stroke prevention in atrial fibrillation and for the prevention and treatment of venous thromboembolism. As any anticoagulant, they are associated with a bleeding risk. Management of DOAC-induced bleeding is challenging. Idarucizumab, antidote for dabigatran, is currently available and is part of the therapeutic strategy, whereas antidotes for anti-Xa agents are under development. Activated or non-activated prothrombin concentrates are proposed, although their efficacy to reverse DOAC is uncertain. We propose an update on DOAC-associated bleeding management, integrating the availability of idarucizumab and the critical place of DOAC concentration measurements.     

HLA in North Colombia Chimila Amerindians

HLA-A,-B,-C,-DRB1 and -DQB1 alleles have been studied in Chimila Amerindians from Sabana de San Angel (North Colombian Coast) by using high resolution molecular typing. A frequent extended haplotype was found:HLA-A*24:02-B*51:10-C*15:02-BRB1*04:07-DQB1*03:02 (28.7%) which has also been described in Amerinndian Mayos Mexican population (Mexico, California Gulf, Pacific Ocean). Other haplotypes had already been found in Amerindians from Mexico (Pacific and Atlantic Coast), Peru (highlands and Amazon Basin), Bolivia and North USA. A geographic pattern according to HLA allele or haplotype frequencies is lacking in Amerindians, as already known. Also, five new extended haplotypes were found in Chimila Amerindians. Their HLA-A*24:02 high frequencies characteristic is shared with aboriginal populations of Taiwan; also, HLA-C*01:02 high frequencies are found in New Zealand Maoris, New Caledonians and Kimberly Aborigines from Australia. Finally, this study may show a model of evolutionary factors acting and rising one HLA allele frequency (-A*24:02), but not in others that belong to the same or different HLA loci.     

The universal muscular chamber. A basic unit of the genitourinary, cardiovascular, gastro-intestinal, skeletal, cutaneous, respiratory and other systems, endocameral hypertension; and spasm, the key to their idiopathic diseases and arthropathies

Starting with the integument, we see many organs are contractile sacs or multiples thereof, which tubes or bags constitute the major part of the entire body. Recognition of this basic unit and its characteristics sheds new light, individually and collectively, on many disorders previously considered unrelated. Muscular tears and perforations develop in the walls of these chambers, being no way peculiar to those organs, wherein, hydrochloric acid occurs. So, it is not necessary to explain the absence of excessive acid from patients who exhibit holes in the gastric, uterine, aortic, duodenal, rectal, pulmonary, retina, and other walls. Muscle, not acid is the great common factor relating idiopathic disorders in the gastrointestinal tract to each other and to similar diseases in other systems. When the units are linked together, the lesions tend to appear as arthropathies, i.e. at the joints. Rephrasing common-place observations, frees us from conventional, conceptual cul-de-sacs. An observation is only as good as its interpretation, so all possibilities must be considered, otherwise, we will remain blinded by our misconceptions.     

Renal dysplasia and asplenia in two sibs

A family is reported in which two sibs, one male and the other female, both died within 24 hours of birth with enlarged polycystic kidneys. Postmortem histology in the second child showed gross renal dysplasia. In both children the pancreas was enlarged, nodular and cystic but the liver appeared macroscopically normal. In the second child, histological examination confirmed pancreatic fibrosis with cystic dilation of ducts, but showed portal fibrosis with bile duct proliferation in the liver.
This combination of findings is very reminiscent of those in a girl and her brother reported by Ivemark et al. (1959). The children reported here also showed absence or hypoplasia of the spleen, cardiac anomalies and other features of the Ivemark syndrome (Ivemark 1955), a quite different, usually sporadic, congenital disorder. It is suggested that the children described here have a distinct lethal congenital disorder, probably inherited in an autosomal recessive manner.