Articles

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)

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).

Hepatitis C Virus (HCV) infection is usually treated with direct acting antivirals (DAAs) for 12 weeks. In treatment naive patients with genotype (GT) 1 infection without cirrhosis and baseline viral load < 6 million, 8 weeks of Ledipasvir/Sofosbuvir (LDV/SOF) is an option. Eight weeks with Glecaprevir/Pibrentasvir (GLE/PIB) is an option for patients with GT 1 through 6 without cirrhosis. Our objective was to evaluate achievement of Sustained Virologic Response (SVR) after 8 weeks of LDV/SOF or GLE/PIB in our HCV-infected veterans. Patients with HCV infection that received GLE/PIB or LDV/SOF for a planned 8 weeks of therapy in the past four years were reviewed (January 2015-September 2018). Treatment outcomes were evaluated through medical record review. Two hundred sixty-five veterans were initiated on 8 weeks of therapy with either GLE/PIB or LDV/SOF. Of these, 231 (87%) were initiated on 8 weeks of LDV/SOF and 34 (13%) were initiated on 8 weeks of GLE/PIB. The majority of patients had GT 1 (93%) infection. One hundred and ninety-five veterans who completed 8 weeks of LDV/SOF and 30 veterans on GLE/PIB had follow-up viral loads. The overall SVR was 95%. Treatment with GLE/PIB resulted in a higher SVR rate (100%) compared to LDV/SOF (95%). Elderly patients had similar SVR rates. Treatment with 8 weeks of DAA is effective in our veteran population and showed an SVR rate similar to literature reports. The SVR for patients treated with 8 weeks LDV/SOF was slightly lower than the SVR for GLE/PIB; however, the GLE/PIB population was smaller

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.

Essential Thrombocythemia (ET) is currently classified as a Philadelphia negative myeloproliferative neoplasm (MPN) together with polycythemia vera (PV) and primary myelofibrosis (PMF); the latter can be further divided in pre-fibrotic primary myelofibrosis (pre-PMF) and overt myelofibrosis, as listed in the revised 2016 World Health Organization classification of myeloid malignancies (WHO 2016). Overall, respect to the others MPNs, ET is characterized by favorable prognosis, lower life expectancy if compared to the control population, increased risk of thrombohemorrhagic complications along with possible evolution in myelofibrosis and leukemic transformation. In this review the authors will review current knowledge on biology, clinical aspects, prognosis and stratification of thrombotic risk, therapeutic options and outcome in ET patients.

In 1901, ABO system of blood groups was determined by Karl Landsteiner. It is present in different organisms like rodents, primates that includes chimpanzees, bonobos etc. The blood groups type in this system depends on some genes that are specific ABO gene. Arachnophobia is the dread of creepy crawlies and different creature like spiders. Individuals suffering from arachnophobia generally feels uneasy in any place where they accept that they could harbour arachnids or their existence for example, webs. 100 samples of blood from volunteers were used for Blood group test. The Blood groups were tested and results were recorded after the test all the used kits were discarded. The results shows that there is no clear cut difference between the arachnophobic males and non-arachnophobic male’s percentages, so no relation found in males. Similarly, in females both phobic and non-phobic ladies have no differences in their result values so, no relation was found. Whereas, in case of arachnophobic males and females comparison no relation was found. So, there is no relationship between ABO blood group system and arachnophobia and there may be a relation between non-phobia and AB+, B+ blood group in males whereas, in females only B+ blood group have relation with non-phobia.

Varicella zoster virus behaves differently from other herpes viruses as it differs from them in many aspects. Recently, there has been growing evidence on the beneficial effects of the virus in immune compromised hosts and these effects are translated into prolongation of survival. The reported beneficial effects of the virus include: (1) stimulation of bone marrow activity in patients with hematologic malignancies and bone marrow failure syndromes, (2) antitumor effects in various hematologic malignancies and solid tumors, and (3) association with graft versus host disease which has anticancer effects. Additionally, there are several reports on the safety of the live-attenuated even in severely immune suppressed individuals and on the emerging role of the virus in cancer immunotherapy. In this review, the following aspects of the virus will be thoroughly discussed: (1) new data on the genetic background, pathogenesis, vaccination, and new therapeutic modalities; (2) bone marrow microenvironment and hematopoiesis; (3) cells involved in the pathogenesis of the virus such as: mesenchymal stem cells, dendritic cells, natural killer cells, T-cells and mononuclear cells; (4) cellular proteins such as open reading frames, glycoproteins, promyelocytic leukemia protein, chaperons, and SUMOs; (5) extracellular vesicles, exosomes, and micro-RNAs; and (6) signaling pathways, cytokines, and interferons.

Varicella zoster virus (VZV), a double-stranded DNA virus, is a highly contagious human neurotropic virus that belongs to the alpha group of herpes viruses [1-4]. Primary VZV infection (chickenpox) occurs in childhood then the virus becomes latent in the nerve ganglia [1,5-7]. Reactivation of the virus may occur decades later and cause herpes zoster (HZ) which is manifested by a typical painful skin eruption that has characteristic dermatomal distribution [1,5]. Reactivation of VZV is usually predisposed to: old age; comorbid medical conditions such as diabetes mellitus, chronic obstructive airway disease, and end-stage renal disease; and immunosuppression due to malignancy, autoimmune disorders, immunosuppressive therapies, trauma, cytotoxic chemotherapy, hematopoietic stem cell transplantation (HSCT), and solid organ transplantation (SOT) [1,5-7].

Background: Pure red cell aplasia is characterized by anemia, reticulocytopenia and diminished bone marrow erythroid precursors. It has multifactorial etiology and consequently several therapeutic interventions. Case: In August 2017, a young patient was diagnosed to have pure red cell aplasia. She was given immunosuppressive therapy for approximately two months but this treatment was stopped due to intolerance. Later on she developed herpes zoster infection that was treated with valacyclovir. Subsequently, it was noted that the patient became blood transfusion independent due to normalization of her hemoglobin and regeneration of the erythroid precursors in the bone marrow. Discussion: Varicella zoster virus behaves differently from other members of the herpes group of viruses such as cytomegalovirus and Epstein-Barr virus. Two retrospective studies, performed in patients with malignant hematological disorders and bone marrow failure, have shown that infection with the virus may cause stimulation of the three cell lines in the bone marrow and superior overall survival. Conclusion: The outcome of the patient presented confirms the findings of the two studies showing long-term beneficial effects of varicella zoster virus infections in immunocompromised individuals.

Background: Invasive fungal infections cause significant morbidity and mortality in patients with hematologic malignancies and in recipients of hematopoietic stem cell transplantation. Case: We report a patient with relapsed acute myeloid leukemia who developed disseminated Fusarium infection during the neutropenic period following the salvage cycle of chemotherapy given at King Fahad specialist Hospital in Dammam, Saudi Arabia. The invasive fungal infection was successfully managed with a combination of voriconazole and liposomal amphotericin-B. Discussion: Fusarium species can cause invasive infections that may become disseminated and life-threatening in patients with acute myeloid leukemia. Conclusion: Combined antifungal therapy and recovery of neutrophil count are essential to control invasive Fusarium infections