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Introduction: Erdheim-Chester disease (ECD) is a rare and difficult-to-treat non-Langerhans cell histiocytosis characterized by the excessive production and accumulation of histiocytes. This study reports a case of ECD, emphasizing both its diagnosis, assessment and treatment of the pain associated with the disease. Case Report: Six years ago, a 39-year-old male patient presented with generalized pain of moderate intensity in the lower limbs that involved periods of greater intensity associated with ambulation. The diagnosis of histiocytosis associated with panhypopituitarism and adrenal insufficiency was proposed. For a specific diagnosis, a bone lesion biopsy was performed, revealing the presence of histiocytic proliferation that was CD1 negative, S100 protein positive, and CD68 negative. Therefore, the diagnosis of non-Langerhans histiocytosis known as ECD was confirmed. During the two years that followed, the patient presented with severe bone pain, particularly in the lower limbs and cranial vault, and the pain subsided to a certain extent with the use of tramadol and paracetamol. Because of the pain, the patient was unable to walk and became bedridden As the patient remained in severe pain, even after the administration of morphine, the opioid was changed from morphine (60mg/day) to oxycodone (30mg/day) for a convenient dosing schedule; furthermore, the oxycodone dosage was scheduled to increase to 40mg/day that same week. The patient experienced significant pain reduction, requiring rescue analgesia only once or twice a week. Conclusion: To the best of our knowledge, this is the first case report on the characterization and treatment of pain specific to ECD, and we highlight that the patient had a good response to treatment.

Background: This article reviews current knowledge of Gaucher’s disease (GD) and liver involvement and reports our experience: how many patients with chronic liver disease of unknown origin could be affected by Gaucher’s disease. Patients and methods: Over 24 months, we tested 75 sine causa chronic liver disease patients (30 women and 45 men, mean age 55 years, range 15 to 77). Results: None of the 75 patients was affected by Gaucher’s disease. Conclusion: We believe that the chronic liver disease patient is unlikely to be affected by Gaucher’s disease. Probably this disease is to be found in cases of coexistence of hepatic disease and other symptoms of Gaucher’s disease (bone, neurological, bone marrow involvement).

Introduction: Fluid management is the cornerstone of treatment for acute pancreatitis (AP), but the proper rate and volume is still controversial. We aim to evaluate the role of aggressive hydration in AP patients. Methods: We retrospectively reviewed and analyzed 279 hospitalized patients of AP. Severity was determined by the Revised Atlanta classification; validated clinical scores were also calculated based on clinical information upon presentation. We extracted amount of fluid received by at 6, 12, 24 and 48 hours after presentation. Aggressive hydration was defined as amount higher than 10 ml/kg bolus followed by infusion at 1.5 ml/kg/h. After direct comparison between aggressive versus non-aggressive hydration groups, propensity-score match was performed to control severity, APACHE II and BISAP score. Post-match comparison as well as a subgroup comparison were conducted. Results: At 24 hours, 125 (44.8%) patients received aggressive hydration averaged at 5.1 L (2-18 L), while 154 (55.2%) patients received non-aggressive hydration averaged at 2.5 L. Post-match comparison showed that aggressive hydration group had longer hospital stay (MAP: 5.3 vs 4.5, p = 0.145, MSAP/SAP: 8.3 vs 4.8 d, p = 0.007), and higher rate of intensive care unit admission (mild: 12.9% vs 4.4%, p = 0.042, moderately severe or severe: 36.8% vs 3.1%, p = 0.001), while showed no difference in rate of mortality or re-admission by 1 year. In patients who presented without organ failure, aggressive hydration did not change the rate of development of organ failure (14.1% vs 12.5%, p = 0.731), but the aggressive hydration group had a trend towards longer hospital stay (5.5 vs 4.6 d, p = 0.083) and higher rate of MICU admission (12.1% vs 4.8%, p = 0.051)

Heterotopic gastric mucosa (HGM) is an islet of gastric mucosa within the esophageal mucosa. These lesions can sit throughout the digestive tract and rarely in the upper third of the esophagus. The pathophysiology of HGM remains poorly understood. Our study aims to estimate the prevalence of HGM, clinical signs, endoscopic, microscopic aspects and different epidemiological factors associated. All patients from a single endoscopy center with HGM of the upper third of the esophagus were included over a 5-month evaluation period. All lesions seen in endoscopy were confirmed by histological analysis. The prevalence was 1.3% with a clear male predominance. 80% of patients were symptomatic and received medical treatment, clinical evolution was good. No case of dysplasia was identified and no complication was observed. Due to insufficient data in the evolutionary literature, the management of HGM remains debated and could resemble that of Barett’s esophagus for monitoring and therapeutic management, particularly in the event of symptoms or dysplasia.

Introduction: Pancreatic pseudocysts (PPs) are mostly delayed complications of acute or chronic pancreatitis and trauma. Pancreatic pseudocysts are usually managed by supportive medical treatment without surgical procedure. All the surgical interventions (percutaneous, endoscopic or surgical approaches) are based on the location, size, symptoms, complications of the pancreatic pseudocyst and medical condition of the patients. Recently, laparoscopic cystogastrostomy has become most appropriate approach especially for retrogastric pancreatic pseudocysts. In this study, we would like to report results of laparoscopic anterior transgastric cystogastrostomy by using linear articulated endo GIA stapler (Covidien medium thick purple) and versa-lifter (versa lifter®, laparoscopic retractor, manufactured by protomedlabs, France) in 14 pancreatic pseudocysts patients. Methods: We retrospectively analyzed data of patients with pancreatic pseudocysts treated by laparoscopic anterior transgastric cystogastrostomy from September 2010 to October 2014. All of the patients were controlled for the recurrence of pancreatic pseudocysts in February 2017. Results: 14 patients with pancreatic pseudocysts were managed by laparoscopic anterior transgastric cysto-gastrostomy. Conversion was performed in only one patient (7%). There were no symptoms and signs of recurrence of pancreatic pseudocyst during on average 43.6 months follow up time. Conclusion: Laparoscopic cystogastrostomy by using articulated linear endo-GIA stapler and versa-lifter is a safe and effective method for management of appropriate retro-gastric pancreatic pseudocysts.

Familial adenomatous polyposis is an autosomal dominant syndrome of variable penetration and constitutes the second frequent inherited syndrome enunciating the emergence of a colorectal carcinoma. The syndrome is accompanied by exemplification of defective adenomatous polyposis coli (APC) gene located upon chromosome 5q21 with a prototypic denomination of colonic adenomatous polyps usually exceeding a > 100. Incriminated individuals develop innumerable colonic and rectal polyps, particularly during early teenage years and are accompanied by an almost 100% possible emergence of colorectal carcinoma within 40 years in untreated subjects [1]. Prophylactic colectomy is advisable to substantially reduce possible occurrence of colorectal carcinoma. Familial adenomatous polyposis is concurrent with associated neoplasms such as gastric or duodenal cancer, hepatoblastoma or desmoid tumour along with a probable emergence of extra-colonic carcinomas [1,2].

There is growing evidence that gastroesophageal disease is influenced by the esophageal microbiome, and that commensal bacteria of the oropharynx, stomach, and colon are thought to have a role in modulatiing pathogenesis. These emerging hypotheses are based on observed changes in the composition of the esophageal flora, notably, repeated observations: 1. There is an abundance of gram-positive bBacteria in the healthy esophagus. are more gram positive prevalent 2. The esophageal bacterial population becomes increasingly gram negative with disease progression. Associated with this shift to a more gram negative prevalence is an increase in the potential for the presence of antigenic lipopolysaccharide (LPS). The immunoreactivity of LPS endotoxin thought to promote susceptibility to inflammation and disease. The pathogenesis of the more common diseases of the esophagus e.g. gastroesophageal reflux disease (GERD), esophageal dysmotility (achalasia), eosinophilic esophagitis (EoE), Barrett’s esophagus (BE), and esophageal cancer, are well-established. Emerging data suggest however, that these are all characterized by an immune-mediated inflammatory cascade, propogated by a dysbiotic state. Thereby, the ability of the healthy “normative state” to protect against foreign bacteria is compromised. This dysbiosis thereby can create adverse inflammatory or immunoregulatory responses with progression of disease. In the normal healthy state, the esophageal microbiome is constituted in-part, by a multitude of gram positive bacteria, many of which produce antibacterial peptides called bacteriocins. Bacteriocins are selective and used to maintain population integrity by killing off foreign bacteria. When the “normative biome” is interrupted (e.g. antibiotics, medications, diet, environmental factors), the constitutional changes may allow a more hospitable imbalance favoring the proliferation of opportunistic pathogens. Therefore it seems rational that defining, perhaps that defining, perhaps cultivating, a protective bacterial community that could help prevent or mitigate inflammatory diseases of the esophagus. Furthermore, in conjunction with evidence demonstrating that some bacteriocins are cytotoxic or antiproliferative toward cancer cell lines, further exploration might provide a rich source of effective peptide-based drug targets. Therapeutic options targeting the microbiome, including prebiotics, probiotics, antibiotics and bacteriocins, have been studied, albeit the attributable effects on the esophagus for the most part, have been unrecognized by clinicians. This review focuses on the current knowledge of the involvement of the microbiome in esophageal diseases (most notably GERD/Barrett’s esophagus/esophageal cancer) and identifies emerging new concepts for treatment.

Acute pancreatitis is inflammation of the pancreas that may be accompanied by a systemic inflammatory response which results in impairment of the functioning of various organs, systems. Pancreatitis associated vascular complications very often cause morbidity and mortality. There are various cardiovascular complications like shock, hypovolemia, pericardial effusion, and sometimes ST–T changes in the electrocardiogram (ECG) presenting as acute myocardial infarction (AMI). Acute myocardial infarction complicating acute pancreatitis has rarely been studied and the exact process of myocardial injury still remains unclear. We here report a case of Acute Pancreatitis associated with acute myocardial Infarction.

Hepatocellular carcinoma (HCC) is characterized by high morbidity, high recurrence, and high mortality rates. In China, the morbidity of HCC is fifth among all malignant tumors and HCC is the third most common cause of cancer-related deaths. Most HCC patients also have liver cirrhosis. Surgery is the sole curative method for HCC; however, many patients are diagnosed with HCC during its advanced stages so radical resection can no longer be performed. Therefore, the proportion of patients who undergo radical hepatectomy is less than 30%. Patients with mildly advanced HCC cannot undergo hepatectomy and thus transcatheter arterial chemoembolization (TACE) and/or biological targeted therapy are alternative options. However, data on the effects of TACE therapy or biological targeted therapy are limited. Therefore, an investigation of multimodal and individualized treatments is critical to ensure the best treatment. In June 2018, we treated an advanced HCC patient with multiple metastases and right portal vein tumor thrombus. The patient exhibited partial remission after undergoing treatment with TACE and crizotinib capsules for 1 month. The case and a literature review are reported here.

Background: Like other viruses, the SARS-CoV-2 (severe acute respiratory syndrome coronavirus 2) appears to be responsible for several autoimmune complications. The occurrence of autoimmune hemolytic anemia has been described in several case reports. This AIHA was also noticeable by the important number of blood transfusions required for COVID-19 (coronavirus disease 2019) patients. By investigating RBC coating autoantibodies, this article attempts to clarify the autoimmune aspect of the anemia in the context of SARS-CoV-2 infection. Results: A large population of COVID-19 patients selected at Saint-Luc University Hospital showed an average of 44% DAT positivity. In this population, the intensive care patients were more prone to DAT positivity than the general ward patients (statistically significant result). The positive DAT appeared « transmissible » to other RBCs via COVID-19 DAT-positive patient’s plasma. Conclusion: The strongest hypothesis explaining this observation is the targeting of cryptic antigens by autoantibodies in COVID-19 patients.

Background: Leukemia is frequently associated with fundoscopic abnormalities. However, no organized effort has been made for analyzing leukemic retinopathy in our country. This study was done to observe the demographic profile and correlation between fundoscopic findings of retinal exudates and hematological parameters in leukemic patients. Materials and Methods: The study was a hospital-based descriptive cross-sectional study among 50 leukemic patients in Medicine and Oncology departments of Bangladesh Medical College and Hospital (BMCH) from May, 2020 to October, 2020. Fundoscopic examination was done which was reviewed by an ophthalmologist. Collected data was analyzed statistically by using SPSS-17 (Chicago, Illinois). Results: Among 50 leukemic patients’, the fundal lesion was detected in 32 patients (64%), retinal exudates are rare. Only 3 patients (6%) had exudates while others (29 patients, 58%) have retinal hemorrhages. Exudates did not show any statistically significant relationship with types of leukemia (p value = 0.53). There was no statistically significant association between fundal exudates and high white cell count (p = 0.56) or low hemoglobin level (p = 0.11) or low platelet level (p = 0.11). Conclusion: This study has identified retinopathy occurring frequently in leukemic patients. Therefore, an adequate attention should be paid at fundoscopic evaluation while treating leukemic patients. 

Introduction: Forty-six per cent children have allergic rhinoconjunctivitis (Allergologica 2005). Working hypothesis: Ocular topical cyclosporine improves the quality of life for these patients. Material, methods, design: 2-year prospective study (2015-16), 40 patients with topical corticosteroids without improvement, followed 20 and 20 switched to corticosteroids cyclosporine 0.05%. Interview with Quality of Life Questionnaire in Children with rhinoconjunctivitis (PRQLQ) before and at the end of treatment. Mean age of 10.3 years with 60% male-40% female. Treatments were applied from January to March. There were 15 questions divided into two blocks. Children responded using a card with responses rated from zero (not bothered at all) to 6 (quite upset). Results: Before the 100% reported that, the itching was very bothersome. In the group of 40 children, 80% showed symptoms of epiphora and 60% showed symptoms of ocular inflammation. 100% complained of significant discomfort in rubbing their eyes, 30% did not like to take medications. Headaches affected 20%. 100% stated that they cannot play normally. 80% showed decreased concentration in class. Continuing with corticosteroids did not show statistically significant changes. Patients with cyclosporine improved the results by 3 points, with decreased itching, tearing, swelling, pain, eye rubbing, medication and headaches. In the 2nd questionnaire there was limited variation in the results related to fatigue, malaise, and irritability but with substantially improved balance of sleep, insomnia and concentration at school. Conclusion: Cyclosporine A is a cyclic polypeptide calcineurin inhibitor developed from the fungus Tolypocladium inflatum. The first dilution was 2% but it is currently used at a dilution of 0.05% and recent publications suggest nanosuspensions. Our study showed improvement in parameters related to symptoms, especially itching and lower improvement of psychological aspects, this achieves a better quality of life for children and more willingness to adhere to the treatment.

The widespread availability of non-traditional dietary supplements and pharmacologically active substances via the Internet continues to introduce mechanisms for inadvertent toxidromes not commonly seen. Consumers are virtually unrestricted in their ability to acquire products purporting augmentation of normal physiology for the purposes of enhancement, recreation, and/or potential abuse. The safety profiles at standard or toxic doses remain largely unknown for many agents that can be purchased electronically. We report a case of mixed toxicity related to phenibut and fosaracetam, both of which are readily available for consumer purchase from online retailers. Written and verbal consent was obtained for this case presentation.

Inflammatory Bowel Disease (IBD)-associated arthritis is called Enteropathic Arthritis (EA) which is classified among the group of Spondyloarthritis (SpA), because its presentation is variable. The current trend is to classify them as autoinflammatory rather than autoimmune diseases, since no antibodies have yet been identified. The study of biomarkers (BM) will help us with early identification and hence, to provide treatment in the early stages, prior to radiographic progression, which will enable prompt identification of the disease phenotype. 42 patients diagnosed with IBD were included, of which 48% were females; the mean age of the study group was 48.12 ± 5.02 (95% CI). The average time of evolution of disease was 37.57 ± 14.28 months; most patients referred to the rheumatologist had a diagnosis of ulcerative colitis (83%). According to our analysis, we were able to determine that the three most significant variables influencing the development of sacroiliitis were: Lactoferrin, ANCA and HLA B27 (p < 0.5). The variable that can be ruled out because of its almost neglectable contribution was fecal calprotectin.

Purpose: Percutaneous abscess drainage (PAD) is the first-line approach for abscess in Crohn’s disease (CD) since it procrastinates or avoids surgery especially in postoperative abscesses [within 30 days post-operative (p.o.)]. We retrospectively evaluated the effectiveness, complications and outcome after PAD in postoperative and spontaneous abscesses and factors influencing the outcomes. Methods: We performed PAD in 91 abscesses, 45 (49,5%) postoperative and 46 (50,5%) spontaneous. We defined the overall success (OS) as clinical (CS) and technical success (TS) when imaging documented the resolution of the abscess with no surgery within 30 days. Conversely, patients without abscess at the time of surgery, were considered as TS but clinical failure (CF). We also analyzed the overall failure (OF) defined as CF with or without technical failure (TF). Overall technical success (OTS) was OS plus TS. Complications were classified as major and minor according to the Interventional Radiology Criteria. Results: In postoperative abscesses we found 91% OS, 9% OF, no TF and 100% OTS. In spontaneous abscesses we found 33% OS, 67% OF, 6.4% TF, 95,6% OTS. A total abscess resolution was achieved in 97,8% of patients. No major complication occurred; only 1 case of minor complication. Factors statistically influencing the outcome were postoperative vs spontaneous collections (OF: 9% vs. 67%, p < 0.0001), multiloculated vs uniloculated collections (OF: 38% vs. 1%, p < 0.0001) and upper abdominal vs lower location (OF: 13% vs. 25%, p <0.05). Conclusion: Our data confirms the safety and effectiveness of PAD even in cases needing surgery within 30 days; most remarkable, PAD allows avoidance of early reoperation in almost all the patients with postoperative abscess.

Background:While recognition and documentation of true drug allergy is critically important, most physicians acknowledge that its prevalence is likely overestimated, often on the basis of historical, sometimes anecdotal evidence. Correct or not, once applied, drug allergy labels may result in altered, potentially inferior therapy, increased costs and prolonged hospitalisation. Objective:Estimate the point prevalence, accuracy and symptomatology of self-reported drug allergy in a typical, large NHS Acute Trust adult inpatient population. In the subset with penicillin allergy (PA), estimate additional management costs from the use of alternative antibiotics and readmission rates in the previous 5 years. Methods:Data on self-reported drug allergies were extracted from 440 adult inpatient prescription charts over a 4 month period. Where penicillin allergy (PA) was reported, alternative antibiotic regimens were recorded and their additional costs calculated. Hospital electronic records were used to assess readmission rates of PA patients. Results:194/440 inpatients (44.5%) reported at least one drug allergy. Antibiotic allergy was most commonly reported (51%), followed by analgesic (23%) and antiemetic (12%) allergy. PA accounted for 76% of reported antibiotic allergy. The commonest reported symptoms were cutaneous (42%) and gastrointestinal (18%). Where antibiotic therapy was required for patients with PA to manage acute infections, Ciprofloxacin, Clarithromycin, Teicoplanin, Clindamycin and Cefuroxime were the most commonly employed alternatives. Extrapolation of these figures to include the entire Trust inpatient population suggested that the use of alternative antibiotics in PA patients incurred additional annual expenditure of £268,000. Further, 87% of PA patients had been admitted more than once in the preceding 5 years, with 74% requiring further courses of antibiotics during these admissions. Conclusion:Self-reported drug allergy, and in particular PA, is common in hospital inpatient populations and, in addition to the potentially unnecessary hazards to individual patients resulting from the use of alternative antibiotics, results in a considerable additional financial burden to the healthcare system. This problem could be eliminated by the provision of a nationwide and equitable tertiary Allergy service.

Acute liver failure (ALF) in children is a severe disease with a high mortality rate. The current treatment strategies are still defective, with many cases die when liver transplantation is unavailable. The current protocol of steroids therapy improved the survival rate of hepatitis A virus (HAV)-related ALF. However, there is still a high mortality for non-HAV cases. Stem cell therapy (SCT) has been tried in experimental animals with ALF and in few adult studies with acute-on-chronic liver failure. No previous trials of SCT have been tested in children with ALF. The absence of SCT application in ALF in children could be due to some issues. These could be related to safety, sources, administration route, optimum dosage, efficacy, and survival. It is proposed that could be the future therapy if these obstacles have been well studied and solved.

Seizure is clinical manifestation of sudden disruption of the normal electrical activity of cortical neurons. The brain electrical activity is periodically disturbed, alteration in neural cell integrity, increase in firing impulses and spread to adjacent normal neurons result in temporary brain dysfunction with alterations in consciousness, behavior or motor function. It may be triggered by illness, infection, stress, stroke, brain tumor, or the underlying cause may not completely understand. Status epilepticus (SE) is a medical emergency and requires prompt diagnosis and treatment. Treatment includes general support measures, drugs to suppress epileptic activity and relieving the underlying condition. Refractory SE requires admission to an intensive care unit (ICU) to allow adequate monitoring and support of respiratory, metabolic and hemodynamic functions and cerebral electrical activity. For SE treatment, benzodiazepines are the first line antiepileptic agents, and if benzodiazepines fail to control seizures, Phenytoin is usually indicated; Phenobarbital or Valproate may also be considered. For refractory SE, Propofol and Thiopental represent first line agents after careful assessment of potential risks. In refractory SE, general anesthesia may be required. There is currently no unique consensus for definite treatment option of RSE. In this review, the management protocol of seizure, assessment, monitoring, and different alternative therapy would be discussed.

Chest compression is the fundamental technique in cardiopulmonary resuscitation (CPR) in patients with cardiac arrest [1]. The quality and the early implementation of CPR are essential to improve the prognosis and the chances of restoring spontaneous circulation. In the literature, there are some articles about the poor quality of chest compression [2-4]. Therefore chest compression is as crucial as alerting the emergency services or early defibrillation in the survival chain. In accordance with the guidelines, chest compressions have to be performed continuously to improve the outcome [5]. However, the efficacy of manual chest compression diminishes over time with the fatigue of the provider (which appears within minutes of starting the procedure), and is impaired during transportation manoeuvres, which expose patients to unforeseen interruptions and a deterioration in the quality du massage in terms of power and rhythm. The efficacy of manual chest compression has been reported to fall by 20% per minute [6,7]. Mechanical chest compression overcome this problem of operator fatigue by ensuring constant efficacy in terms of both quality and quantity. Even though current data show no difference between manual chest compression and automated systems in terms of survival, haemodynamic studies in animal models have shown that mechanical techniques are more effective [8].

This review examines of the concept of a defective chloride channel in epithelial cells being a major cause of cystic fibrotic pathophysiology. The central concept of the defective chloride ion channel paradigm is that faulty CFTR protein or failed delivery of CFTR protein to the mucosal membrane of epithelial cells is the basis of cystic fibrosis. Defective placement or function of CFTR prevents hydration of bronchial mucus that is normally caused by epithelial cells; these are capable through chloride ion secretion of transporting fluid to the mucosal surface. This concept relies heavily on a paradigm taken from intestinal physiology-namely that the intestinal epithelial cell secretes chloride ion and fluid and that this has conferred heterozygote selective advantage in carriers of the cystic fibrosis gene. This present review examines the evidence for that hypothesis and assembles evidence from past studies that it is the smooth muscle cell that is of greater relevance. This review does not aim to provide an overview of current research into cystic fibrosis. The intention is to provide an overview of past research that led to the concept of a failure of epithelial cells to hydrate bronchial mucus because of compromised CFTR function. It is important to present all past evidence for aspects of the chloride secretion hypothesis and its associated heterozygote advantage concept so that the important evidential milestones can be re-assessed.