Ambiguous marks were typically single, short scars usually located on

only 1 body region and could not be reliably attributed to lions. Because 9.5% (n = 67) of individuals were not photographed on both sides, we applied a correction factor to take into account the probability that some individuals may have predation marks on the unphotographed side of the body. The location of each mark on the body was recorded, including body side and region (Fig. 3). To supplement predation-mark data, we examined the season and age of death of 52 giraffe carcasses presumed killed by lions between 1966 and 2011. The data are from a continuous long-term study of lions in the central woodlands and south-eastern plains of Serengeti. Means are reported as ±sd and significance was GSK-3 inhibition α = 0.05. Results presented here focus on marks convincingly attributable buy Ridaforolimus to lions. An estimated 10.6% of giraffes (13.1% of giraffes >1

year old) show evidence of surviving at least 1 lion attack. The estimated prevalence of claw marks was significantly higher among adults (17.6%) than subadults (3.7%) [χ2 = 21.34, degrees of freedom (d.f.) = 1, P < 0.0001]. Of the 7 subadults observed with claw marks, 1 was a yearling, 1 was a 2-year-old and 5 were estimated to be between ages 3 and 5 years at first sighting. No claw marks were observed on calves. We found a highly significant relationship between sex and claw-mark prevalence, with estimated prevalence higher among females (14.1%, n = 379) than males (6.5%, n = 323) (χ2 = 10.69, d.f. = 1, P = 0.001). This result is caused by sex differences among adults [adult females (22.0%) vs. adult males (12.0%), χ2 = 5.83, d.f. = 1, P = 0.016; subadult females (5.4%) vs. subadult males (2.1%), P = 0.27, 2-sided Fisher's exact test]. Predation-mark prevalence for each study area is presented in Table 1. Across age–sex classes, claw-mark prevalence was found to be lower in Kirawira than in Seronera, the 2 well-sampled areas. For giraffes of both sexes >1 year old, estimated claw-mark prevalence was 1.3% for

Kirawira (n = 177) compared with 18.3% for Seronera (n = 311) Amylase (χ2 = 29.14, d.f. = 1, P < 0.0001). This result is attributable to the difference in claw-mark prevalence among adults. Figure 4 summarizes age–sex trends in claw-mark prevalence for Kirawira and Seronera. We observed fresh claw marks, evidenced by dried blood, on 1 subadult female. All other claw marks appeared healed or were too superficial to cause bleeding (Fig. 2). No injuries appeared severe, but subcutaneous damage could not be assessed. Giraffes with hind leg marks did not have any visible reduction in leg motion. We observed no instances of hamstringing. Claw marks were most frequently detected on the rump, followed by the hind leg and flank (Fig. 3). Hind leg marks occurred both above and below the hock. We observed partially amputated tails on 6.8% (n = 5) of individuals with claw marks (n = 74) (Fig. 2d).

15 In this issue of HEPATOLOGY, two selleck compound elegant studies from the laboratories of Jacob Nattermann and Hugo Rosen give important new insights into the biological role of NKp46 in HCV infection.16, 17 Indeed, by using different experimental models and different study cohorts, both studies come to similar conclusions. This itself is a remarkable finding in a field where studies examining the phenotype and function of NK cells have often yielded diverging data. The first important finding of these studies is that high expression of NKp46 (NKp46high) defines a specific human NK-cell subset. Indeed, in comparison to NKp46dim cells, NKp46high

NK cells are characterized by a higher expression of immature differentiation markers, such as CD127, CD62L, and CD27,17 a higher functional ability (e.g., a higher target cell cytotoxicity) and a higher IFN-γ production after stimulation with IL-12 and IL-1516, 17 as well as a stronger up-regulation of genes involved in cytotoxicicty after stimulation with Toll-like receptor ligands.16 Although the majority of the NKp46high NK-cell subset is also CD56bright, differences in functional

and phenotypical properties indicate that NKp46 expression defines a unique NK-cell subset. buy Selumetinib This is further supported by microarray analysis that showed a differential regulation of more than 800 genes in NKp46high versus CD56bright NK cells.17 Importantly, by using NK cells from chronically HCV-infected patients17 or from healthy donors16 and by using the replicon system17 or the Huh7.5 Japanese fulmanant hepatitis type 1 in vitro infection system16 as a readout, both studies show that NKp46high cells have an

increased anti-HCV activity. Most likely, combined noncytolytic and cytolytic effector functions contribute to the antiviral activity of NKp46high NK cells. Indeed, Krämer et al. provide evidence that soluble factors, specifically IFN-γ, contribute to the antiviral effect, Branched chain aminotransferase because incubation of HCV-replicating Huh7 cells with supernatants from NKp46high cells led to a significant inhibition of HCV replication and because this inhibition could be effectively blocked by the addition of anti-IFN-γ.17 This is in agreement with previous studies that have shown a control of HCV replication by NK-cell IFN-γ secretion in vitro18, 19 and after adoptive transfer of NK/NK T cells after liver transplantation in vivo.20 The contribution of cytolytic effector mechanisms in NKp46-mediated antiviral activity is supported by studies showing that cytotoxicity is the major mechanism involved in NK-cell-mediated elimination of HCV-infected hepatocytes21 and that NK cells can kill HCV-infected hepatocytes by perforin/granzyme and TNF-related apoptosis-inducing ligand–mediated mechanisms.

Both DKO and RBP KO mice demonstrate elevation in alanine aminotransferase levels compared to that of

control and HNF-6 KO mice (Table 1), indicative of hepatocellular injury. However, DKO check details mice also demonstrate extensive hepatic necrosis (Fig. 2D, arrowhead; Supporting Fig. 1), as well as increased collagen deposition with areas of bridging fibrosis between portal tracts developing by age P60 (Fig. 2D, arrow). Isolated loss of either HNF-6 or RBP-J alone failed to show significant necrosis or collagen deposition compared to control at age P60 (Fig. 2A-C). With the observed elevation in total bilirubin and alkaline phosphatase demonstrating significant cholestasis, these data show that loss of HNF-6 in the setting of Notch signaling loss leads to enhanced cholestatic liver injury characterized by bridging hepatic fibrosis. To determine the intrahepatic ductal histopathology of mice with loss of HNF-6 alone and within the background of Notch signaling loss, we performed staining with Adriamycin supplier wsCK as a marker of BECs. Mice with isolated loss of HNF-6 showed no detectable phenotypic difference in IHBD wsCK staining compared to control (Fig. 3A,B,E,F,I,J). At age E16.5, RBP KO and DKO mice demonstrate hilar ductal plate formation of similar appearance to control mice (Fig. 3A-D). This data agrees with previously published

data, because mice with Alb-Cre or alpha-fetoprotein enhancer and albumin promoter Cre recombinase (AFP-Cre)-mediated loss of RBP-J demonstrate ductal plate formation of normal appearance at age E16.5, but subsequently Etofibrate show a significant decrease in postnatal cytokeratin-positive BECs and formed IHBDs.11, 12 Consistent with this, at P3 there

were visibly fewer wsCK-positive (+) cells associated with ductal plates and tubular structures in RBP KO mice (Fig. 3G). DKO mice also demonstrate a visible decrease in the number of wsCK+ cells at age P3 (Fig. 3H). At P15, a complete loss of all peripheral wsCK+ cells compared to control is observed (Fig. 3I,L). Cytokeratin-positive bile ducts in P15 DKO mice were only observed centrally within the hepatic lobe and costained positive with Dolichos biflorus agglutinin (DBA) (Supporting Fig. 2). This was consistent among DKO mice examined at age P15 (n = 5). To investigate the etiology of BEC paucity in DKO mice at P3 and P15, we analyzed both apoptosis and proliferation within BECs of DKO compared to age-matched controls. In DKO mice, there was no visible difference in apoptosis by TUNEL method within the wsCK+ BEC population compared to control at P3 (data not shown). Proliferation analysis performed by costaining with cytokeratin-19 (CK19) and Ki67 (Supporting Fig. 3A) showed no difference in the ratio of proliferative BECs in DKO mice at P3 and P15 when compared to age-matched controls (Supporting Fig. 3B).

We obtained genotyping data for 67 single nucleotide polymorphisms (SNPs) across 31 candidate genes and evaluated statistical associations of these variants with total anti-HAV seropositivity (as an indication of prior infection) using DNA samples from 6,779 participants in the Third National Health and Nutrition Examination Survey (NHANES III).17 anti-HAV, antibody to HAV; CDC, Centers for Disease Control and Prevention; CI, confidence interval; CYP, cytochrome P450 enzyme;

FDR, false discovery rate; FDR-P, P value adjusted for false discovery rate; HAV, hepatitis A virus; NCHS, National Center MK-8669 manufacturer for Health Statistics; NHANES III, Third National Health and Nutrition Examination Survey; OR, odds ratio; SNP, single nucleotide polymorphism. All procedures were approved by the Centers for Disease Control and Prevention (CDC) Ethics Review Board, and written informed consent was obtained from all participants. In 2001, the CDC and the National Center for Health Statistics (NCHS) Ethics Review Board approved a revised plan that allows linkage of genetic data to NHANES information through NCHS Research Data Centers to ensure confidentiality of the study participants’ identities.

More information on the DNA bank is available online.18 The current study was approved by the CDC Ethics Review Board. NHANES III was conducted between 1988 and 1994, and the survey design, population, and DNA bank have been described elsewhere.19, 20 Briefly, NHANES III used a stratified, multistage probability PD98059 cell line design to provide nationally representative estimates of the health and nutritional status of the civilian, noninstitutionalized population aged ≥2 months in the United States. NHANES III oversampled minorities (non-Hispanic blacks, Mexican Americans), the young, and the elderly. Data were collected from household interviews and physical examinations. A

DNA bank was created from blood samples collected during the second phase (1991-1994) from 7,159 participants (-)-p-Bromotetramisole Oxalate aged ≥12 years, 62% of whom originated from households containing multiple family members (mean, 1.59 members per household; range, 1-11 members per household). Genetic data were combined with behavioral, environmental, and clinical information available in NHANES III. Analyses included only those who were tested for anti-HAV antibodies and who self-reported as non-Hispanic white (n = 2,619), non-Hispanic black (n = 2,095), or Mexican American (excluding Hispanic persons of other origin [n = 2,065]). Persons who did not self-report as one of these three groups were classified as “other” (n = 348) and were excluded because of concerns about their small numbers and a mix of race/ethnicities not fitting the three main race/ethnicities that were categorized. Serum specimens were stored at −20°C prior to serologic testing.

We aimed to clarify the clinical usefulness of TUS for improving the safety of capsule endoscopy (CE) in patients with Crohn’s disease (CD).

AZD8055 mw Methods: Subjects were 76 patients with CD who underwent double-balloon endoscopy (DBE) and/or patency capsule (PC) examination and/or CE before TUS. The TUS findings were classified as intestinal narrowing and distension at the oral side (Type A), extensive bowel wall thickening (Type B), focal bowel wall thickening (Type C), or no abnormality (Type D). We compared TUS findings to DBE, PC, and CE findings with respect to small-bowel stricture, defined as failure of the enteroscope to pass through the small bowel or capsule retention. Results: Small-bowel stricture was discovered in 50% (38/76) of patients. One or more learn more strictures were detected by TUS in 95% (36/38) of these patients and corresponded to one of the

three TUS abnormality types. One (3%) of the small-bowel strictures was proximal, 4 (11%) were deep, and 33 (86%) were distal. Identified strictures corresponded to TUS findings as follows: 100% (17/17) to Type A, 58% (11/19) to Type B, 33% (8/24) to Type C, and 10% (2/20) to Type D. The two strictures showing no TUS abnormality (Type D) were located deep in the small bowel. Conclusion: When TUS reveals Type A or Type B findings in patients with CD, CE or PC should not be attempted. If TUS shows Type C or Type D findings,

PC examination should be performed before CE. Key Word(s): 1. Transabdominal ultrasonography capsule endoscopy Crohn’s disease Presenting Author: OSAMU NAKASHIMA Additional Authors: MINORU MURAYAMA, KATSUO YAMAZAKI, KAZUO KOIZUMI Corresponding Author: OSAMU NAKASHIMA Affiliations: Izumi Memorial Hospital, Izumi Memorial Hospital, mafosfamide Izumi Memorial Hospital Objective: One of the common symptoms of colorectal cancer is obstruction, which usually occurs in advanced. Therefore, palliation is the aim of therapy in most of these patients. The most important feature of palliative therapy in patients with obstructive unresectable colorectal cancer is to achieve colonic decompression to eliminate the obstructive symptoms and to avoid bowel perforation. Surgical therapy has been the standard therapy for this problem for many years, which usually affords a temporary or permanent colostomy.However, although surgical therapy is an effective solution of colorectal obstruction, it is often a heavy burden to the patient, because the patient is usually in a poor clinical condition.

Efficacy results are summarized in the table. Durable viral suppression was maintained, and 7 additional patients (5 HBeAg+ and 2 HBeAg- ) experienced loss of HBsAg (5 patients with seroconversion to anti-HBs) between Years 5-8. No resistance to TDF was detected through Year 8. Through Year 8, a confirmed renal event (either ≥0.5 mg/dL increase in serum creatinine, or serum phosphorus <2 mg/dL, or creatinine clearance <50 mL/min) was observed in 2.2% of patients, and BMD (T scores) of hip and spine were stable between Years 4-8. Conclusions: PXD101 concentration Long term results from these two studies show TDF to be safe and effective with no evidence of resistance

through 8 years. aMissing=Failure (LTE-TDF analysis); bMissing=excluded

Selleck Staurosporine (On-treatment analysis); cNA=not applicable; dKaplan-Meier-ITT% Disclosures: Patrick Marcellin – Consulting: Roche, Gilead, BMS, Vertex, Novartis, Janssen, MSD, Abbvie, Alios BioPharma, Idenix, Akron; Grant/Research Support: Roche, Gilead, BMS, Novartis, Janssen, MSD, Alios BioPharma; Speaking and Teaching: Roche, Gilead, BMS, Vertex, Novartis, Janssen, MSD, Boehringer, Pfizer, Abbvie Edward J. Gane – Advisory Committees or Review Panels: Novira, AbbVie, Novartis, Gilead Sciences, Janssen Cilag, Vertex, Achillion, Tekmira, Merck, Idenix; Speaking and Teaching: AbbVie, Novartis, Gilead Sciences, Janssen Cilag Robert Flisiak – Advisory Committees or Review Panels: Gilead, Merck, Roche, Bristol Myers Squibb, Janssen, Novartis, Abbvie; Grant/Research

Support: Roche, Bristol Myers Squibb, Janssen, Novartis, Gilead, Vertex, Merck; Speaking and Teaching: Janssen, Merck, Roche, Bristol Myers Squibb, Gilead, Abbvie Huy N. Trinh – Advisory Committees or Review Panels: BMS, Gilead; Grant/ Research Support: BMS, Gilead; Speaking and Teaching: BMS, Gilead, vertex; Stock Shareholder: Gilead Joerg Petersen – Advisory Committees or Review Panels: Bristol-Myers Squibb, Gilead, Novartis, Merck, Bristol-Myers Squibb, Erlotinib purchase Gilead, Novartis, Merck; Grant/ Research Support: Roche, GlaxoSmithKline, Roche, GlaxoSmithKline; Speaking and Teaching: Abbott, Tibotec, Merck, Abbott, Tibotec, Merck Selim Gurel – Speaking and Teaching: Glead, BMS, Roche, MSD, Glead, BMS, Roche, MSD, Janssen Kelly D. Kaita – Advisory Committees or Review Panels: Gilead, Merck, Roche, Janssen, Boehringer, BMS, GSK, Vertex, Abbvie; Grant/Research Support: Gilead, Merck, Roche Naoky Tsai – Advisory Committees or Review Panels: BMS, Gilead, AbbVie; Grant/Research Support: BMS, Gilead, AbbVie, Janssen, Beckman; Speaking and Teaching: BMS, Gilead, AbbVie, Janssen, Roche, Merck John F. Flaherty – Employment: Gilead Sciences; Stock Shareholder: Gilead Sciences Raul E. Aguilar Schall – Employment: Gilead Sciences, Inc. Kathryn M. Kitrinos – Employment: Gilead Sciences, Gilead Sciences; Stock Shareholder: Gilead Sciences, Gilead Sciences Mani Subramanian – Employment: Gilead Sciences John G.

2C) and supported the augmentation of HCV PF-01367338 datasheet replication by STAT3. Furthermore, activation of STAT3 by way of exogenous cytokine treatment with LIF, a known activator of STAT3, resulted in a significant increase in STAT3 phosphorylation at Y705, as expected (Fig. 2D), while pretreatment with LIF for 24 hours prior to infection with JFH-1, resulted in a marked 2-fold increase in HCV RNA levels (Fig. 2E). These

results indicate that activated STAT3 acts to either directly assist HCV replication or potentially induce the expression of specific STAT3-dependent genes that are in turn able to create an environment that is favorable for HCV replication. Collectively, the above experiments show that activation of STAT3 results in enhanced HCV replication. To extend these observations,

selleck the converse sets of experiments were performed using both an siRNA knockdown approach and a panel of chemical inhibitors that block STAT3 activation. To validate our knockdown approach STAT3 siRNA and a control siRNA were transfected into Huh-7 cells and total STAT3 determined by immunoblot. Despite numerous attempts, we were only able to reduce STAT3 expression by ∼50% (Fig. 3Ai). To determine the effect of STAT3 siRNA knockdown on HCV replication, Huh-7.5 cells were transfected with STAT3 siRNA or a control scrambled siRNA, and infected with HCV JFH-1. The knockdown of STAT3 with siRNA significantly decreased HCV RNA levels by ∼50% (Fig. 3Aii). These results confirm previous findings in the literature, where a genome-wide siRNA screen of Huh-7 cells infected with HCV JFH-1 revealed STAT3 as a candidate host factor involved in HCV replication.[1] Next we used a number of commercial STAT3 inhibitors: (1) AG490 is a JAK-2 protein tyrosine kinase (PTK) inhibitor that indirectly inhibits Y705 phosphorylation of STAT3; (2) STA-21 is a novel selective small molecule inhibitor of STAT3, which binds to the SH-2 domain of STAT3 and specifically prevents dimerization

of STAT3 and DNA binding[18]; and (3) S31-201 is a cell-permeable inhibitor of STAT3 that targets oxyclozanide the STAT3-SH2 domain and blocks STAT3 dependent transcription.[19] Supporting Fig. 2 outlines the STAT3 signaling cascade and demonstrates the specific points where these inhibitors exert their function. The effects of STA-21-mediated inhibition of STAT3 on HCV replication were first investigated in an established HCV infection. HCV genomic replicon cells and JFH-1-infected Huh-7.5 cells treated with STA-21 (10 μM) for 24 hours demonstrated an approximate decrease in HCV RNA of 50% (Fig. 3B) and 70% (Fig. 3C), respectively. Given these findings, it appears that STAT3 activation, or STAT3-dependent gene expression, are involved in augmenting HCV replication at the RNA level.

We propose a model of migraine as a dysfunction of a “neurolimbic” pain network. The influence between brainstem and cortical centers is bidirectional, reflecting the bidirectional interaction of pain and mood. Neurolimbic dysfunction may increase this website as migraine becomes more chronic or refractory. The neurolimbic model expands the model of migraine as a dysfunction of brainstem nuclei. A neurolimbic model may help bridge a gap in understanding the migraine attack,

the interictal dysfunctions of episodic migraine, the progression to chronic migraine, and the common comorbidities with other disorders (such as fibromyalgia, irritable bowel syndrome, and mood and anxiety disorders), which may also be considered neurolimbic. A neurolimbic model of migraine may be a useful heuristic that would impact both clinical treatment and research agendas, as well as education of physicians and patients. “
“Giant cell arteritis (GCA) should be considered in the differential

diagnosis of any new onset headache occurring in individuals over the age of 50 years. Headache is the most common complaint in GCA patients but the clinical characteristics of the headache itself does not help in making a diagnosis as the headache can occur anywhere on the head, not just the temples, GDC0449 be mild to severe in intensity and be dull to throbbing in quality. As other things can cause new onset headache in older individuals, additional clinical symptoms or signs that may suggest GCA as a diagnosis would be useful to clinicians. Two cases are presented that suggests that new onset stabbing headache associated with a new daily persistent headache is a possible diagnostic sign for a diagnosis of GCA. Nothing in the literature to date has mentioned new onset stabbing headache as part of the presenting however symptom complex for GCA. “
“We report a SPECT and PET voxel-based analysis of cerebral

blood flow and metabolic rate for glucose in a 23-year-old woman with familial hemiplegic migraine (FHM) caused by ATP1A2 gene mutation. In comparison with healthy subjects, a PET scan showed brain glucose hypometabolism, controlaterally to the hemiplegia, in the perisylvian area early in the attack (Day 1), without any SPECT perfusion abnormalities. Decrease in metabolic rate was only partially reversible at Day 78, concordant at this time with a remaining hemisensory loss. These findings provide further evidence for a primary cortical metabolic dysfunction in FHM. “
“(Headache 2011;51:73-84) Objective.— To evaluate the long-term tolerability of telcagepant for acute treatment of intermittent migraine attacks. Background.— Telcagepant is a calcitonin gene-related peptide (CGRP) receptor antagonist being investigated for the acute treatment of migraine. Methods.

Urinary tract infections and spontaneous bacterial peritonitis were the most frequent infections. Model for End-Stage Liver Disease (MELD) score and nosocomial first infection were predictive of I-ACLF. The 30-day mortality reached 23%. MELD score, I-ACLF, white

blood cell count, and second infection were predictive of mortality. As already reported by the Chronic Liver Failure (CLIF) Consortium, the higher the number of organ failures, the worse the prognosis. (Hepatology 2014;60:250-256.) To maintain cellular energy levels, cells can break down their own components in a complex catabolic process called autophagy. Autophagy protein 5 (ATG5) is an E3 ubiquitin ligase that is important for the formation of the autophagosome. APO866 in vivo In a previous Highlights article, we commented on ATG5 mediating the caffeine-induced reduction in intracellular lipids. In this issue of Hepatology, the work

of Toshima et al. is reported on, whereby they used mice lacking hepatocellular ATG5 to investigate the role of autophagy during liver regeneration. They found that liver regeneration activates autophagy, and that autophagy is necessary to maintain β-oxidation and adenosine triphosphate production in mitochondria. Absence of ATG5 did not compromise the increase in liver weight after partial hepatectomy; on the contrary, it was higher in genetically modified Proteasome inhibition mice. However, the absence of ATG5 impaired the postoperative mitotic response of hepatocytes, which became senescent and hypertrophic. Amisulpride This work identifies autophagy as an important recycling source of energy for normal liver regeneration. (Hepatology

2014;60:290-300.) Evaluation of elevated bilirubin levels in a patient treated in the intensive care unit (ICU) is a classic consultation for hepatologists. Invariably, not one cause, but several potential causes are found, for example, sepsis, transfusions, and drugs. The role of parenteral nutrition is often debated. Vanwijngaerden et al. used the data of the randomized, controlled EPaNIC trial, which was designed to test the effect of early (within 48 hours) versus late (after day 8) parenteral nutrition on the outcome of critical illness. They report that circulating levels of total bilirubin were higher in the ICU patients randomized to receive late parenteral nutrition during the week without this support. The values became identical in the two groups when both received parenteral nutrition. In contrast, levels of alanine aminotransferase (ALT), alkaline phosphatase, and gamma-glutamyltranspeptidase (GGT) were lower in the late parenteral nutrition group, and fewer patients developed sludge in this group. These data confirm that hyperbilirubinemia in ICU patients is not necessarily a result of cholestasis, but instead suggest that it can be related to caloric deficit resulting from withholding parenteral nutrition. (Hepatology 2014;60:202-210.

Currently, the data available for DDAVP use in pregnancy are from a number of small trials and cases studies, so any conclusions drawn are from limited Everolimus cell line evidence which illustrates the need for further

research in the role of DDAVP in the management of pregnancies complicated by bleeding disorders. The authors stated that they had no interests which might be perceived as posting a conflict or bias. “
“Summary.  Haemophilia A (HA) is an X-linked recessive bleeding disorder caused by a lack or decrease of coagulation factor VIII activity. The molecular diagnosis of HA is challenging and a variety of different mutations have been identified throughout the F8 gene. Our aim was to detect the causative mutation in 266 HA patients from Emilia-Romagna region (Italy) and in all suspected carriers. see more Molecular analysis of F8 in 201 HA patients (152 index cases) was performed with a combination of several indirect and direct molecular approaches, such as long distance polymerase chain reaction, multiplex ligation-dependent probe amplification, denaturing high performance liquid chromatography and direct sequencing. The analysis revealed 78 different mutations, 23 of which were novel, not having been reported in national or international databases. The detection rate was

100%, 86% and 89% in patients with severe, moderate and mild HA, respectively. The information provided by this registry will be helpful for monitoring the treatment of HA patients in Emilia-Romagna

and also for reliable genetic Morin Hydrate counselling of affected families in the future. “
“Summary.  Recently, the United Kingdom Haemophilia Centre Doctors Organisation published recommendations for the standard of care for assessment and treatment of patients with bleeding disorders in the emergency department (A&E). An audit was undertaken to compare the level of care to the acceptable standards in a tertiary hospital A&E, attached to a haemophilia comprehensive care centre. A&E attendances were found by cross referencing all patients with known bleeding disorders against the EDMS attendance system. Visits from the past 3 years were identified to produce sufficient data and electronic notes from these visits were then accessed, and marked against the proforma. Data were available from 45 of a total of 54 patients, who had a total of 75 emergency visits documented. In all aspects of care, the standards were not adequately met including the average length of time between booking and clinical assessment, early initiation of specific haemostatic treatment, seeking haematology advice and arrangement of follow-up. Also no specialist clotting investigations were done with only 9/11 patients admitted having their haematological diagnosis recorded. In addition, only very few patients had the severity of bleeding disorder noted and less than half their first line treatment documented.