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Background Autoimmune polyendocrine syndrome type 1 (APS1) is a rare inherited autoimmune disease, characterized by primary adrenocortical insufficiency and hypoparathyroidism. Pathologic mutations in the autoimmune regulator (AIRE) gene, which participates in the regulation of T-cell self-tolerance can lead to APS-1. Defective function of AIRE encourages production of multiple anti-cytokine and organ-specific autoantibodies, leading to severe autoimmune disease across multiple organs. Autoimmune enteropathy is one of the autoimmune conditions associated with APS-1. Symptoms include chronic diarrhoea and weight loss. Extraintestinal manifestations can include haemolytic anaemia and hepatitis. Purpose To report on a patient with APS-1 who exhibited exacerbations of acute-on-chronic diarrhoea and transaminitis. Method We describe the case of a 14-year-old female, the first child of healthy, first-cousin consanguineous parents, with a history of APS type 1 with a homozygous disease-causing variant in the AIRE gene (c.415C>T, p.139*), hypoparathyroidism and Addison’s disease who presented with worsening diarrhoea and unintentional weight loss (15kg) in the past year. Upper and lower endoscopies performed 10 months and 2 months prior to this admission revealed chronic gastritis and patchy active colitis, without features of a specific etiology. Enteroendocrine cells were present. Celiac disease, inflammatory bowel disease, eosinophilic gastroenteritis, and IPEX syndrome were clinically and histologically ruled out. Result(s) On admission, the patient was having five watery stools per day with associated hypocalcemia and hypokalemia which were felt to be more resistant to treatment than should be explained by her Addison’s disease alone. Albumin was preserved (46 g/L). A stool PCR test for Adenovirus was positive, though serum adenovirus PCR was negative. ALT was 231 U/L and AST was 283 U/L. IgG level was elevated (17.7 g/L), anti-nuclear antibody (ANA) was positive (1:320), and anti-smooth muscle antibody (ASMA) was also positive (1:160). It was therefore suspected that he had autoimmune enteropathy and autoimmune hepatitis, and a liver biopsy was performed as well as upper and lower endoscopies. Liver biopsy demonstrated only minimal macrosteatosis (less than 5%) but no evidence of hepatitis. No abnormal endoscopic findings were observed. Histology showed no typical features of autoimmune enteropathy. Enteroendocrine cells were noted in both small and large intestinal biopsies. Her elevated liver enzymes and diarrhea were resolved spontaneously over a 2-week period. Conclusion(s) We present a patient with APS-1 with acute-on-chronic diarrhoea and transaminitis with elevated IgG and positive ANA and ASMA. Endoscopy and liver biopsy did not reveal evidence of autoimmune enteropathy or autoimmune hepatitis. This case raises the importance of maintaining a broad differential diagnosis when diarrhoea is encountered in APS-1 as this may occur for reasons outside of autoimmune enteroendocrine cell loss. Please acknowledge all funding agencies by checking the applicable boxes below None Disclosure of Interest None Declared ENDOSCOPY, TECHNOLOGY & IMAGING

BACKGROUND: Exocrine pancreatic insufficiency is a prominent feature of cystic fibrosis (CF). Trypsin is a measure of pancreatic function in CF patients. It was previously thought that the pancreas was irreversibly damaged in patients with CF by a certain age. However, newer modulator therapies for CF, namely ivacaftor, have shown the potential to improve pancreatic function (as demonstrated in both the KIWI and ARRIVAL study cohorts). Ivacaftor is a CFTR modulator that improves the opening of the defective CFTR channel. Another CF modulator, lumacaftor/ivacaftor combination therpay, improves trafficking of the defective CFTR channel to the membrane. Lumacaftor/ivacaftor combination therapy has not been able to demonstrate a benefit on pancreatic function in previously published work. AIMS: To describe a case of acute pancreatitis in a patient with previous pancreatic insufficient CF after lumacaftor/ivacaftor combination therapy. METHODS: We present the case of a 16-year-old female with CF (genotype: delF508/delF508) treated with lumacaftor/ivacaftor. RESULTS: Prior to initiation of lumacaftor/ivacaftor therapy this patient had severe pancreatic insufficiency with a serum trypsin of 3X the upper limit of normal and compatible ultrasound findings are consistent with a diagnosis of acute pancreatitis. This is the first reported case presentation of acute pancreatitis in a previously pancreatic insufficient CF patient occurring after treatment with lumacaftor/ivacaftor combination therapy. This case raises the question of whether her pancreatic function has normalized with lumacaftor/ivacaftor therapy or whether we are seeing the emergence of a new CF phenotype where there is some improvement in the exocrine function of the pancreas enough to cause pancreatitis. FUNDING AGENCIES: None

There is an ever increasing need to monitor and reduce the levels of particulates in semiconductor ultrapure water. This paper reviews the source and significance of one class of particulates, the lipopolysaccharide (LPS) derived from gram negative bacteria and its detection and quantitaton by Limulus amebocyte lysate (LAL) test.

An extensive hazard division (HD) 1.2 open air testing program has now been completed. A test program investigating in-structure HD 1.2 effects is currently underway. The results of these programs and a literature survey form the basis of a database of MD 1.2 effects. Currently, for quantity distance purposes, HD 1.2 ammunition is treated differently than HD 1.1 items. The database of HD 1.2 effects suggests that this difference is not appropriate. Based on this database, proposed changes to the quantity distance criteria for HD 1.2 ammunition have been developed. This paper will describe those proposed changes. It will then compare the proposed changes with both the current United States and NATO/United Kingdom criteria.

With the early generations of computed tomographic (CT) scanners, interpretation of abdominal and pelvic scans focused on the solid organs, hollow viscera, and retroperitoneum. Attention to blood vessels generally was given only to the aorta and inferior vena cava and their larger branches. The newer generations of scanners allow rapid acquisition of high-resolution images during the vascular phase of mechanical bolus injection of intravenous contrast material. Visualization of second-order vascular branches has thus become routine. Recent improvements in software allow real-time reconstruction of data in multiple planes, which enables demonstration of long segments of vessels within a single image. Approximately 7,000 abdominal and pelvic CT scans were reviewed with attention to vascular detail. Cases are presented that illustrate peripancreatic, perigastric, parietal, and hypogastric vessels; fetal remnants and structures that may be mistaken for vessels; and collateral pathways of both arterial and venous flow. With increasing use of helical CT scanning, smaller vessels can be identified with greater confidence. Knowledge of normal CT vascular anatomy facilitates understanding of collateral pathways when vessel engorgement is perceived.

The assembly and integration of the SSF elements and systems throughout the build-up sequence are addressed focusing on ECLSS hardware interfaces and stage performance. The ECLSS encompasses six subsystems which provide temperature and humidity control, atmospheric control and supply, atmospheric revitalization, water recovery and management, waste management, and the fire detection and protection.

Limbic evoked potentials (LEPs) were recorded from the hippocampi of three epilepsy surgery patients with unilateral medial temporal lobe seizure onset. In each patient, stable large amplitude LEPs which displayed polarity inversion across successive levels of hippocampus were present unilaterally, but were absent or rudimentary in the hippocampus corresponding to the electrographically proven seizure onset. After administration of scopolamine, LEPs in the "normal" hippocampi were markedly altered with slowing of the individual components and increased trial to trial variability. In addition, two patients developed reliable, high amplitude, polarity inverting LEPs on the side of seizure onset which had either absent or rudimentary LEPs in the baseline state. The results suggest that LEPs are under cholinergic modulation.

We have previously reported the detection of endotoxin in the amniotic fluid of patients with gram-negative intraamniotic infection. Endotoxin or lipopolysaccharide is a potent biologic product capable of inducing prostaglandin release from several cell types and, therefore, may be involved in the onset of human parturition in the presence of intraamniotic infection. This article describes a technique for the quantification of endotoxin in amniotic fluid. The method uses a computer-assisted quantification of the turbidimetric reaction between the Limulus amebocyte lysate and endotoxin. Serial dilutions of Escherichia coli endotoxin in culture-negative amniotic fluid were prepared, and the samples were run in the assay. Amniotic fluid was found to enhance the reaction, and a dilution of 1:20 was required for this biologic fluid to behave similarly to pyrogen-free water. The sensitivity of this kinetic turbidimetric technique in the detection of endotoxin in amniotic fluid was 40 pg/ml. This method was applied to the quantification of endotoxin concentration in amniotic fluid in 26 patients with intraamniotic infection and premature rupture of membranes. Patients in active labor had higher concentrations of endotoxin (median = 47,514 pg/ml) than nonlaboring patients (median = 635 pg/ml) (p less than 0.025). Therefore, women with preterm labor had a higher median concentration of endotoxin in amniotic fluid than patients who were not in labor.