Peking Union Medical College Hospital

About: Peking Union Medical College Hospital is a healthcare organization based out in Beijing, China. It is known for research contribution in the topics: Medicine & Population. The organization has 15996 authors who have published 16018 publications receiving 226505 citations.

γ-Secretase Gene Mutations in Familial Acne Inversa

Abstract: Acne inversa (AI), also known as hidradenitis suppurativa, is a chronic, recurrent, inflammatory disease of hair follicles that often runs in families We studied six Chinese families with features of AI as well as additional skin lesions on back, face, nape, and waist and found independent loss-of-function mutations in PSENEN, PSEN1, or NCSTN, the genes encoding essential components of the γ-secretase multiprotein complex Our results identify the γ-secretase component genes as the culprits for a subset of familial AI, implicate the γ-secretase-Notch pathway in the molecular pathogenesis of AI, and demonstrate that familial AI can be an allelic disorder of early-onset familial Alzheimer's disease

Bacillary dysentery as a causative factor of irritable bowel syndrome and its pathogenesis

Abstract: Background and aims: The incidence of irritable bowel syndrome (IBS) or functional bowel disorders (FBD) after bacillary dysentery (BD) has not been extensively evaluated, and little is known of the pathogenesis of post-infective (PI) IBS. Therefore, we investigated the incidence of IBS and FBD in a Chinese patient population who had recovered from BD. To further elucidate its pathogenesis, neuroimmunological changes, including interleukins (IL), mast cells, neuropeptides, and the relationship between mast cells and intestinal nerves, were investigated. Methods: A cohort study of 295 patients who had recovered from BD (shigella identified from stool in 71.4%) and 243 control subjects consisting of patient siblings or spouses who had not been infected with BD were included in the study. All subjects were followed up using questionnaires for 1–2 years to explore the incidence of FBD and IBS, as defined by the Rome II criteria. In 56 cases of IBS (PI and non-PI) from another source, the number of mast cells in biopsy specimens from the intestinal mucosa were stained with antitryptase antibody and counted under light microscopy. Also, the relationship of mast cells to neurone specific enolase (NSE), substance P (SP), 5-hydroxytryptamine (5-HT), or calcitonin gene related peptide positive nerve fibres was observed using double staining with alcian blue and neuropeptide antibodies. In 30 cases of IBS (PI-IBS, n = 15) taken at random from the 56 cases, expression of interleukin (IL)-1α, IL-1β, and IL-1 receptor antagonist (IL-1ra) mRNAs in intestinal mucosa were identified using reverse transcription-polymerase chain reaction. The above results were compared with 12 non-IBS controls. Results: In the BD infected cohort, the incidences of FBD and IBS were 22.4% and 8.1% (in total)–10.2% (among those in who shigella were identified) respectively, which were significantly higher (p Conclusions: BD is a causative factor in PI-IBS. The immune and nervous system may both play important roles in the pathogenesis of PI-IBS.

Resuscitation fluid use in critically ill adults: an international cross-sectional study in 391 intensive care units

Abstract: Recent evidence suggests that choice of fluid used for resuscitation may influence mortality in critically ill patients. We conducted a cross-sectional study in 391 intensive care units across 25 countries to describe the types of fluids administered during resuscitation episodes. We used generalized estimating equations to examine the association between patient, prescriber and geographic factors and the type of fluid administered (classified as crystalloid, colloid or blood products). During the 24-hour study period, 1,955 of 5,274 (37.1%) patients received resuscitation fluid during 4,488 resuscitation episodes. The main indications for administering crystalloid or colloid were impaired perfusion (1,526/3,419 (44.6%) of episodes), or to correct abnormal vital signs (1,189/3,419 (34.8%)). Overall, colloid was administered to more patients (1,234 (23.4%) versus 782 (14.8%)) and during more episodes (2,173 (48.4%) versus 1,468 (32.7%)) than crystalloid. After adjusting for patient and prescriber characteristics, practice varied significantly between countries with country being a strong independent determinant of the type of fluid prescribed. Compared to Canada where crystalloid, colloid and blood products were administered in 35.5%, 40.6% and 28.3% of resuscitation episodes respectively, odds ratios for the prescription of crystalloid in China, Great Britain and New Zealand were 0.46 (95% confidence interval (CI) 0.30 to 0.69), 0.18 (0.10 to 0.32) and 3.43 (1.71 to 6.84) respectively; odds ratios for the prescription of colloid in China, Great Britain and New Zealand were 1.72 (1.20 to 2.47), 4.72 (2.99 to 7.44) and 0.39 (0.21 to 0.74) respectively. In contrast, choice of fluid was not influenced by measures of illness severity (for example, Acute Physiology and Chronic Health Evaluation (APACHE) II score). Administration of resuscitation fluid is a common intervention in intensive care units and choice of fluid varies markedly between countries. Although colloid solutions are more expensive and may possibly be harmful in some patients, they were administered to more patients and during more resuscitation episodes than crystalloids were.

The cytokine storm and factors determining the sequence and severity of organ dysfunction in multiple organ dysfunction syndrome.

Abstract: Multiple organ dysfunction syndrome (MODS) is a major cause of morbidity and mortality in intensive care units. It is being encountered frequently in critically ill patients owing to advancements in organ-specific supportive technologies to survive the acute phase of severe sepsis and shock. It is now believed that MODS is the result of an inappropriate generalized inflammatory response of the host to a variety of acute insults. The pathologic mechanisms of MODS were reviewed, and factors determining the sequence and severity of organ dysfunction were discussed in depth. In the early phase of MODS, circulating cytokines cause universal endothelium injury in organs. In the later phase of MODS, overexpression of inflammatory mediators in the interstitial space of various organs is considered a main mechanism of parenchyma injury. The difference in constitutive expression and the upregulation of adhesion molecules in vascular beds and the density and potency of intrinsic inflammatory cells in different organs are the key factors determining the sequence and severity of organ dysfunction. By activating the intrinsic inflammatory cell in a distant organ, organ dysfunctions are linked in a positive feedback loop through circulating inflammatory mediators. Antagonists targeted at adhesion molecules may alleviate the severity of endothelial damage. And nonsteroidial anti-inflammatory drugs or steroids administered judiciously in the early phase of MODS may retard the progress of multiple organ failure.