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J Hepatobiliary Pancreat Sci 2021 Jun 11. Epub 2021 Jun 11.

Department of General and Pancreatic Surgery, The Pancreas Institute, University of Verona Hospital Trust, Verona, Italy.

Background: The Japanese difficulty scoring system (DSS) was developed to assess the difficulty of laparoscopic distal pancreatectomy (LDP). The study aimed to validate a modified DSS (mDSS) in a European high-volume center.

Methods: Patients' clinical data underwent LDP for benign and malignant pancreatic lesion between September 2013 and February 2020 were reviewed. Read More

Front Immunol 2021 25;12:675909. Epub 2021 May 25.

Division of Molecular Psychoimmunology, Institute for Genetic Medicine, Graduate School of Medicine, Hokkaido University, Sapporo, Japan.

The lipopolysaccharides (LPSs) of are reported to be TLR4 antagonists. Accordingly, the extract of (RAP99) is used as a health supplement for humans and animals in Japan to regulate immune responses . We previously analyzed the LPS structure of RAP99 (RAP99-LPS) and found it is different from that of -LPS but similar to lipid A from (RSLA), a known antagonist of TLR4, with both having three C14 fatty acyl groups, two C10 fatty acyl groups, and two phosphates. Read More

Clin Lymphoma Myeloma Leuk 2021 May 11. Epub 2021 May 11.

Clinical Pathology and Oncologic Laboratory Medicine Department, National Cancer Institute, Cairo University, Egypt.

Background: FMS-like tyrosine kinase 3 internal tandem duplication (FLT3-ITD) is a molecular genetic alteration significantly affecting the clinical outcome in patients with acute myeloid leukemia (AML). FLT3-ITD mutations are characterized by variable mutant-to-wild allelic ratios (ARs) and sizes of the duplicated sequences. The size of the inserted sequence may vary from a few to hundreds of nucleotides. Read More

Ann Palliat Med 2021 May;10(5):5694-5705

Department of Emergency Medicine, Affiliated Hospital of Nantong University, Nantong, China.

Background: The present study set out to investigate the epidemiological and clinical characteristics of 65 patients with tsutsugamushi disease.

Methods: The clinical data of 65 patients with tsutsugamushi disease, who were admitted to the Affiliated Hospital of Nantong University were retrospectively analyzed. The clinical symptoms, laboratory examination results, clinical treatment plans, treatments, and outcomes of the patients were analyzed. Read More

Transplant Rev (Orlando) 2021 May 29;35(4):100630. Epub 2021 May 29.

Division of Pediatric Gastroenterology and Hepatology, Department of Pediatrics, University Medical Center Groningen, University of Groningen, Groningen, the Netherlands. Electronic address:

Introduction: Portal vein obstruction (PVO) is a significant vascular complication after liver transplantation (LT) in pediatric patients. Current treatment strategies include percutaneous transluminal angioplasty (PTA), with or without stent placement, mesorex bypass (MRB), splenorenal shunt, mesocaval shunt, endovascular recanalization (EVR), splenic artery embolization and splenectomy. However, specific characteristics of patients undergoing intervention and selection of individual treatment and its efficacy have remained unclear. Read More

J Infect Dev Ctries 2021 05 31;15(5):625-629. Epub 2021 May 31.

Department of Internal Medicine, School of Medicine and Health Sciences, Atma Jaya Catholic University of Indonesia, Penjaringan, Kota Jakarta Utara, Daerah Khusus Ibukota Jakarta, Indonesia.

In tropical countries, endemic diseases such as malaria can be challenging to distinguish from COVID-19 because of the similarities in presenting symptoms. Here we reported a case of a young soldier with fever and myalgia six days before admission, with non-productive cough, chills, nausea and vomiting, dizziness, and headache for two days. Previously, he had experienced four times of malaria infection. Read More

Turk Arch Pediatr 2021 1;56(3):245-248. Epub 2021 May 1.

Pediatric Hematology and Oncology Clinic, Kanuni Sultan Süleyman Training and Research Hospital, Istanbul, Turkey.

Objective: This study aims to evaluate the demographic information, clinical and laboratory findings of patients with glucose 6 phosphate dehydrogenase deficiency.

Material And Methods: We collected data by reviewing files and electronic records of 65 patients with glucose 6 phosphate dehydrogenase deficiency under the age of 18 years who were followed up in our clinic between 2007 and 2019. Demographic, clinical, and laboratory features, family history, complications of the disease, and history of splenectomy and cholecystectomy were evaluated. Read More

J Med Case Rep 2021 Jun 8;15(1):316. Epub 2021 Jun 8.

Faculty of Medicine- Physiology Department, The University of Medicine and Pharmacy Craiova, Craiova, Romania.

Background: Left ventricular noncompaction is a rare cardiomyopathy characterized by a thin, compacted epicardial layer and a noncompacted endocardial layer, with trabeculations and recesses that communicate with the left ventricular cavity. In the advanced stage of the disease, the classical triad of heart failure, ventricular arrhythmia, and systemic embolization is common. Segments involved are the apex and mid inferior and lateral walls. Read More

Cureus 2021 May 29;13(5):e15321. Epub 2021 May 29.

Department of Hematology and Oncology, Bronx Lebanon Hospital, New York, USA.

Intravesical administration of Bacillus Calmette-Guerin (BCG) has been utilized for the treatment of superficial bladder cancer for the past several decades. Though this treatment is well tolerated in general, both local and systematic side effects have been reported. We present a case of a patient who presented with systemic symptoms of fever with chills associated with leucopenia, thrombocytopenia, abnormal liver function tests (LFTs), and splenomegaly a few weeks after an episode of traumatic instillation of intravesical BCG. Read More

Front Immunol 2021 20;12:667781. Epub 2021 May 20.

College of Fishery, Guangdong Ocean University, Guangdong Provincial Key Laboratory of Pathogenic Biology and Epidemiology for Aquatic Economic Animal, Key Laboratory of Control for Disease of Aquatic Animals of Guangdong Higher Education Institutes, Southern Marine Science and Engineering Guangdong Laboratory, Zhanjiang, China.

Leukocyte cell-derived chemotaxin 2 (LECT2) is a multifunctional cytokine that especially plays an important role in innate immune. However, the roles of LECT2 in the immune response of the economically important fish Nile tilapia () against bacterial infection remains unclear. In this study, a gene from Nile tilapia () was identified, and its roles in the fish's immune response against bacterial infection were determined and characterised. Read More

Malar J 2021 Jun 6;20(1):249. Epub 2021 Jun 6.

Tang Center for Herbal Medicine Research, Institute of Chinese Materia Medica, China Academy of Traditional Chinese Medical Sciences, No. 16 Dongzhimen Nanxiaojie, Dongcheng District, Beijing, 100700, China.

Background: Malaria is a fatal disease that presents clinically as a continuum of symptoms and severity, which are determined by complex host-parasite interactions. Clearance of infection is believed to be accomplished by the spleen and mononuclear phagocytic system (MPS), independent of artemisinin treatment. The spleen filters infected red blood cells (RBCs) from circulation through immune-mediated recognition of the infected RBCs followed by phagocytosis. Read More

Wiad Lek 2021 ;74(5):1180-1188

SUMY STATE UNIVERSITY, SUMY, UKRAINE.

Objective: The aim: To establish the dependence of the concentration of galectin-9(CGal-9) in the serum of patients with chronic viral hepatitis C (CVHC) on the type of antiviral therapy (AVT), clinical-laboratory, autoimmune and integrative parameters, non-invasive methods of assessing the degree of fibrosis.

Patients And Methods: Materials and methods: CGal-9 in serum were determined in 68 patients with CVHC and 20 healthy individuals, and clinical-laboratory and integrative parameters, noninvasive methods for assessing the degree of fibrosis were studied.

Results: Results:There were three groups: baseline (I), pegylated interferon (PEG-IFN) with ribavirin (II), velpatasvir with sofosbuvir (III). Read More

Phytother Res 2021 Jun 5. Epub 2021 Jun 5.

Núcleo de Pesquisa em Doenças Negligenciadas, Universidade Guarulhos, Guarulhos, São Paulo, Brazil.

Schistosomiasis is a widespread human parasitic disease currently affecting over 200 million people, particularly in poor communities. Chemotherapy for schistosomiasis relies exclusively on praziquantel (PZQ). Previous studies have shown that licarin A (LIC-A), a dihydrobenzofuran neolignan, exhibited in vitro antiparasitic activity against Schistosoma mansoni adult worms. Read More

Ann Hematol 2021 Jun 5. Epub 2021 Jun 5.

Peking University People's Hospital, Peking University Institute of Hematology, National Clinical Research Center for Hematologic Disease, Beijing Key Laboratory of Hematopoietic Stem Cell Transplantation, Beijing, China.

To explore the differences in the clinical features, treatment responses, and outcomes among children, adolescents, and adults with chronic myeloid leukemia in the chronic phase (CML-CP) receiving imatinib as first-line therapy. Data from children (0-8 years for girls and 0-10 years for boys), adolescents (9-19 years for girls and 11-19 years for boys), and adults (age ≥ 20 years) with newly diagnosed CML-CP receiving imatinib as first-line therapy between 2006 and 2019 were retrospectively reviewed. In total, 135 children (cohort 1), 189 adolescents (cohort 2), and 658 adults (cohort 3: age 20-39 years, n = 305; cohort 4: age 40-59 years, n = 270; and cohort 5: age 60-83 years, n = 83) were included in this study. Read More

J Allergy Clin Immunol 2021 Jun 1. Epub 2021 Jun 1.

Liver Diseases Branch, National Institute of Diabetes & Digestive & Kidney Diseases, NIH, Bethesda, MD, USA. Electronic address:

Background: Late-onset complications in X-linked agammaglobulinemia (XLA) are increasingly recognized. Nodular regenerative hyperplasia (NRH) has been reported in primary immunodeficiency but data in XLA are limited.

Objective: To describe NRH prevalence, associated features and impact in patients with XLA. Read More

PLoS One 2021 4;16(6):e0252825. Epub 2021 Jun 4.

Department of Ophthalmology, University Medical Center Mainz, Mainz, Germany.

Introduction: Niemann-Pick type C (NPC) is a lysosomal storage disease that is progressive and life-limiting, with an estimated incidence of 1:120,000 live births. In addition to systemic manifestation with (hepato-)splenomegaly, there are a number of neurological manifestations (ataxia, dysarthria, dementia, cataplexy, epileptic seizures, and psychiatric disorders). Characteristic is vertical supranuclear gaze palsy, which is often overlooked. Read More

Hematol Transfus Cell Ther 2021 May 18. Epub 2021 May 18.

Hospital Israelita Albert Einstein (HIAE) São Paulo, SP, Brazil. Electronic address:

Objective: Describe the clinical and laboratory characteristics and the transfusion strategy of patients at Hospital Israelita Albert Einstein with platelet refractoriness and identify their etiological characteristics. Standardize the platelet immunofluorescence technique by flow cytometry as a test for platelet compatibility in immune platelet refractoriness in transfusion support.

Methods: Review of medical records of refractory platelet patients followed at HIAE from January 2011 to May 2017. Read More

Orphanet J Rare Dis 2021 Jun 3;16(1):255. Epub 2021 Jun 3.

, Roboleo & Co, Leeds, UK.

Background: Progressive familial intrahepatic cholestasis is a rare, heterogeneous group of liver disorders of autosomal recessive inheritance, characterised by an early onset of cholestasis with pruritus and malabsorption, which rapidly progresses, eventually culminating in liver failure. For children and their parents, PFIC is an extremely distressing disease. Significant pruritus can lead to severe cutaneous mutilation and may affect many activities of daily living through loss of sleep, irritability, poor attention, and impaired school performance. Read More

Int J Mol Sci 2021 May 23;22(11). Epub 2021 May 23.

Department of Clinical Genetics, Medical College, University of Rzeszów, 35-959 Rzeszów, Poland.

Mucopolysaccharidosis type II (MPS II) is a lysosomal storage disorder based on a mutation in the gene that encodes iduronate 2-sulphatase. As a result, there is an accumulation of glycosaminoglycans-heparan sulphate and dermatan sulphate-in almost all body tissues, which leads to their dysfunction. Currently, the primary treatment is enzyme replacement therapy, which improves the course of the disease by reducing somatic symptoms, including hepatomegaly and splenomegaly. Read More

Animals (Basel) 2021 May 17;11(5). Epub 2021 May 17.

Department of Veterinary Sciences, University of Messina, 98168 Messina, Italy.

Canine leishmaniosis (CanL) is responsible for splenic pathological changes. The main features detectable from ultrasound examination are splenomegaly and diffuse alterations of the echostructure. The study aimed to highlight whether these ultrasound changes are related to the severity of the disease or to a modification of splenic microvascularization that can be detected in vivo through contrast-enhanced ultrasonography (CEUS). Read More

Int J Mol Sci 2021 May 21;22(11). Epub 2021 May 21.

Department of Pharmacology and Toxicology, Institute of Pharmacy, University of Regensburg, 93040 Regensburg, Germany.

Inositol 1,4,5-triphosphate receptor-associated cGMP kinase substrate 1 (IRAG1) is a substrate protein of the NO/cGMP-signaling pathway and forms a ternary complex with the cGMP-dependent protein kinase Iβ (PKGIβ) and the inositol triphosphate receptor I (IPR-I). Functional studies about IRAG1 exhibited that IRAG1 is specifically phosphorylated by the PKGIβ, regulating cGMP-mediated IP-dependent Ca-release. IRAG1 is widely distributed in murine tissues, e. Read More

Viruses 2021 May 21;13(6). Epub 2021 May 21.

Department of Infectious Diseases, Division of Viral Gastroenteritis and Hepatitis Pathogens and Enteroviruses, Robert Koch Institute, 13353 Berlin, Germany.

Avian hepatitis E virus (aHEV) is the major etiological agent of hepatitis-splenomegaly syndrome (HSS), big liver and spleen disease (BLSD), and hepatic rupture hemorrhage syndrome (HRHS) in chickens. Infections with aHEV cause a significant decrease in egg production and increased mortality in chickens worldwide. However, studies on the prevalence of aHEV in Nigeria are scarce. Read More

Br J Haematol 2021 Jun 1. Epub 2021 Jun 1.

Haematology and Oncology, Medical Faculty Mannheim, Heidelberg University, Mannheim, Germany.

We report on 45 patients with myeloid neoplasms and concurrent Janus kinase 2 (JAK2) V617F and KIT proto-oncogene, receptor tyrosine kinase (KIT) D816V (JAK2 /KIT ) mutations, which are individually identified in >60% of patients with classical myeloproliferative neoplasms (MPN) and >90% of patients with systemic mastocytosis (SM) respectively. In SM, the concurrent presence of a clonal non-mast cell neoplasm [SM with associated haematological neoplasm (SM-AHN)] usually constitutes a distinct subtype associated with poor survival. All 45 patients presented with a heterogeneous combination of clinical/morphological features typical of the individual disorders (e. Read More

Trop Doct 2021 May 31:494755211020610. Epub 2021 May 31.

Department of Hematology, 28730All India Institute of Medical Sciences, New Delhi, India.

Long-standing moderate to marked splenomegaly suggests several differential diagnoses, both haematological and infectious, particularly leishmaniasis and malaria in endemic areas. Non-infectious causes may be missed in these regions, especially if pitfalls of serological testing are not considered. Careful patient evaluation is necessary to arrive at the correct diagnosis. Read More

J Pediatr Hematol Oncol 2021 May 31. Epub 2021 May 31.

Department of Pediatrics Division of Pediatric Hematology and Oncology, Kanuni Sultan Süleyman Training and Research Hospital Division of Pediatric Hematology and Oncology, Istinye University Medical Faculty, Istanbul, Turkey.

Objective: The aim of this study was to evaluate the demographics, clinical, and laboratory findings and treatment responses of patients with hereditary spherocytosis (HS).

Materials And Methods: Data of children with HS were examined. Diagnosis was based on clinical history, physical examination, family history, presence of spherocytes on peripheral blood smear, and osmotic fragility test. Read More

ANZ J Surg 2021 May 28. Epub 2021 May 28.

Department of Surgery, St George Private Hospital, Sydney, New South Wales, Australia.

J Inflamm Res 2021 20;14:2045-2060. Epub 2021 May 20.

Department of Rheumatology and Immunology, Second Affiliated Hospital of Chongqing Medical University, Chongqing, People's Republic of China.

Purpose: Angiotensin 1-7 [Ang-(1-7)] has been identified as an important anti-inflammatory and anti-fibrotic factor. This study determined how the ACE2-Ang-(1-7)-Mas axis affected M1/M2 macrophage polarization and thus contributed to anti-inflammatory processes in the cecal ligation and puncture (CLP)-induced inflammation model.

Materials And Methods: ELISA, western blotting, and qRT-PCR were used to verify that Ang-(1-7) decreased the expression of pro-inflammatory cytokines and increased anti-inflammatory cytokines. Read More

Front Immunol 2021 10;12:682934. Epub 2021 May 10.

Department of Hematology and Oncology, Knappschaftskrankenhaus, Ruhr-University Bochum, Bochum, Germany.

Hemophagocytic lymphohistiocytosis (HLH) is a disorder of uncontrolled immune activation with distinct clinical features including fever, cytopenia, splenomegaly, and sepsis-like symptoms. In a young adolescent patient a novel germline variant (NM_032638.5 (GATA2): c. Read More

Genet Med 2021 May 26. Epub 2021 May 26.

Department of Dermatology, University Hospital Münster, Münster, Germany.

Purpose: Prolidase deficiency is a rare inborn error of metabolism causing ulcers and other skin disorders, splenomegaly, developmental delay, and recurrent infections. Most of the literature is constituted of isolated case reports. We aim to provide a quantitative description of the natural history of the condition by describing 19 affected individuals and reviewing the literature. Read More

Leukemia 2021 May 26. Epub 2021 May 26.

CHU de Lille, Univ Lille, INSERM U1286, Infinite, Lille, France.

Allogeneic haematopoietic cell transplantation (allo-HCT) remains the only curative approach in myelofibrosis (MF). Despite advances over recent decades, relapse and non-relapse mortality rates remain significant. Relapse rates vary between 15 and 25% across retrospective studies and management strategies vary widely, ranging from palliation to adoptive immunotherapy and, in some cases, a second allo-HCT. Read More