19,533 results match your criteria Splenomegaly


[Clinical characteristics and genetic analysis of hereditary spherocytosis caused by mutations of ANK1 and SPTB genes].

Zhongguo Dang Dai Er Ke Za Zhi 2019 Apr;21(4):370-374

Department of Hematology and Oncology, Pediatric Medical Center, Hunan Provincial People's Hospital, Changsha 410005, China.

This study analyzed the clinical features of 5 children with hereditary spherocytosis (HS) and the characteristics of ANK1 and SPTB gene mutations. All 5 children were confirmed with HS by peripheral blood genetic detection. Anemia, jaundice and splenomegaly were observed in all 5 children. Read More

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April 2019
1 Read

Selenium supplementation suppresses immunological and serological features of lupus in B6.Sle1b mice.

Autoimmunity 2019 Apr 22:1-12. Epub 2019 Apr 22.

b Department of Biochemistry and Molecular Biology , Pennsylvania State University College of Medicine , Hershey , PA , USA.

Systemic lupus erythematosus (SLE) is a debilitating multi-factorial immunological disorder characterized by increased inflammation and development of anti-nuclear autoantibodies. Selenium (Se) is an essential trace element with beneficial anti-cancer and anti-inflammatory immunological functions. In our previous proteomics study, analysis of Se-responsive markers in the circulation of Se-supplemented healthy men showed a significant increase in complement proteins. Read More

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https://www.tandfonline.com/doi/full/10.1080/08916934.2019.1
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http://dx.doi.org/10.1080/08916934.2019.1603297DOI Listing
April 2019
1 Read

Pregnancy in a Woman with Latent Myeloproliferative Neoplasm Induced Chronic Portal Vein Thrombosis, Portal Cavernoma, and Gastric Varices.

Case Rep Obstet Gynecol 2019 14;2019:5702983. Epub 2019 Mar 14.

Department of General Internal Medicine, Faculty of Medicine, University of Sherbrooke, Quebec, Canada.

Extra-hepatic portal vein thrombosis (EHPVO) represents the obstruction of the portal vein outside the liver and is not related to chronic liver disease or neoplasia. In chronic EHPVO, collateral veins and portal hypertension develop, resulting in splenomegaly and variceal formation. Myeloproliferative neoplasms (MPN) are the most frequent acquired etiology of EHPVO. Read More

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http://dx.doi.org/10.1155/2019/5702983DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6437724PMC

"Honeycomb" pattern of gallbladder wall thickening - A forward step in early diagnosis of "Severe Dengue Fever".

Indian J Radiol Imaging 2019 Jan-Mar;29(1):14-18

Department of Radiology, Apollo Hospitals International Limited, Gandhinagar, India.

Aims And Objectives: To study "Honeycomb" pattern of gallbladder wall thickening (GBWT) in dengue fever (DF) and to assess its clinical significance in early diagnosis of severe DF.

Materials And Methods: A total 244 patients of DF were studied, 84 patients were classified as severe DF, 61 patients as DF with warning signs, and 99 patients as DF without warning signs. Abdominal ultrasound was performed for assessment of GBWT patterns, hepatomegaly, splenomegaly, pancreatic enlargement, ascites, pleural effusion, and other additional findings were recorded in severe DF cases. Read More

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http://dx.doi.org/10.4103/ijri.IJRI_363_18DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6467033PMC

[E-cadherin Expression in Children with Acute Leukemia and Its Clinical Significance].

Zhongguo Shi Yan Xue Ye Xue Za Zhi 2019 Apr;27(2):339-347

Department of Hematology & Oncology, Childhren's Hospital Affiliated to Chongqing Medical University; Key Laboratory of Child Development and Disorders of Ministry of Education; China International Science and Technology Cooperation Base of Child Development and Critical Disorders; Chongqing Key Laboratory of Pediatrics, Chongqing 400014, China,E-mail:

Objective: To investigate the correlation of E-cadherin expression level with the clinical characterastics in children with acute leukemia (AL), and to explore the possible regulatory mechanism.

Methods: Real-time quantitative RT-PCR was applied to detect the expression level of E-cadherin in bone marrow samples from 135 child patients diagnosed as AL, and its relevance with clinical indicators was statistically analyzed. The expression levels of E-cadherin, β-catenin, and Akt/p-Akt were detected by using Western blot. Read More

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http://dx.doi.org/10.19746/j.cnki.issn.1009-2137.2019.02.005DOI Listing

The impact of JAK/STAT inhibitor ruxolitinib on the genesis of lymphoproliferative diseases

Turk J Med Sci 2019 Apr 18;49(2):661-674. Epub 2019 Apr 18.

Background/aim: Ruxolitinib, a JAK/STAT signaling pathway inhibitor targeted drug, has been approved for the controlling of disease symptoms and splenomegaly in patients with myeloproliferative neoplastic diseases. Recently, it has been proposed that ruxolitinib-induced JAK/STAT pathway inhibition in myelofibrosis is associated with an elevated frequency of aggressive B-cell lymphomas. However, the biological basis and significance of this pharmacobiological adverse event is unknown. Read More

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http://online.journals.tubitak.gov.tr/openDoiPdf.htm?mKodu=s
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http://dx.doi.org/10.3906/sag-1807-152DOI Listing
April 2019
1 Read

Adding hydroxyurea in combination with ruxolitinib improves clinical responses in hyperproliferative forms of myelofibrosis.

Cancer Med 2019 Apr 17. Epub 2019 Apr 17.

Department of Medicine and Surgery, Hematology and Hematopoietic Stem Cell Transplant Center, University of Naples Federico II, Naples, Italy.

Ruxolitinib, an orally bioavailable and selective inhibitor of Janus kinase 1 (JAK1) and JAK2, significantly reduces splenomegaly and disease-related symptoms in patients with myelofibrosis (MF). However, no clear survival benefit has been demonstrated, which may in part reflect suboptimal drug exposure related to lower dosages needed to minimize hematological toxicity, specifically cytopenias. Furthermore, the optimal management of specific conditions such as leukocytosis or thrombocytosis in patients under ruxolitinib therapy is still undefined. Read More

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http://dx.doi.org/10.1002/cam4.2147DOI Listing
April 2019
1 Read

Chronic low-level cadmium exposure in rats affects cytokine production by activated T cells.

Toxicol Res (Camb) 2019 Mar 4;8(2):227-237. Epub 2019 Jan 4.

Department of Pharmacology & Toxicology , Michigan State University , East Lansing , MI 48824 , USA . Email: ; Tel: +517-884-6692.

Cadmium is a toxic metal and common environmental contaminant. Chronic cadmium exposure results in kidney, bone, reproductive, and immune toxicity as well as cancer. Cadmium induces splenomegaly and affects the adaptive immune system, but specific effects vary depending on the dose, model, and endpoint. Read More

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http://xlink.rsc.org/?DOI=C8TX00194D
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http://dx.doi.org/10.1039/c8tx00194dDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6425995PMC
March 2019
1 Read

Clinical, immunologic, and molecular spectrum of patients with LPS-responsive beige-like anchor protein (LRBA) deficiency: a systematic review.

J Allergy Clin Immunol Pract 2019 Apr 14. Epub 2019 Apr 14.

Non-communicable Diseases Research Center, Alborz University of Medical Sciences, Karaj, Iran. Electronic address:

Background: LPS-responsive beige-like anchor protein (LRBA) deficiency is a primary immunodeficiency and immune dysregulation syndrome caused by biallelic mutations in the LRBA gene. These mutations usually abrogate the protein expression of LRBA, leading to a broad spectrum of clinical phenotypes including autoimmunity, chronic diarrhea, hypogammaglobulinemia and recurrent infections.

Objective: Our aim was to systematically collect all studies reporting on the clinical manifestations, molecular and laboratory findings, and management of patients with LRBA deficiency. Read More

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http://dx.doi.org/10.1016/j.jaip.2019.04.011DOI Listing

Brucellosis in pregnancy: results of multicenter ID-IRI study.

Eur J Clin Microbiol Infect Dis 2019 Apr 15. Epub 2019 Apr 15.

Clinical Sciences, Liverpool School of Tropical Medicine, Liverpool, UK.

Brucellosis in pregnant women is reported to be associated with obstetric complications (OCs), and adequate data for human brucellosis during pregnancy are largely lacking. We performed this multicenter retrospective cross-sectional study to evaluate the epidemiology, clinical course, treatment responses, and outcomes of brucellosis among pregnant women. The study period comprised a 14-year period from January 2002 to December 2015. Read More

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http://link.springer.com/10.1007/s10096-019-03540-z
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http://dx.doi.org/10.1007/s10096-019-03540-zDOI Listing
April 2019
4 Reads

Clinical Aspects, Immunophenotypic Analysis and Survival Rate of Chronic Lymphocytic Leukaemia Patients in Erbil City, Iraq.

Authors:
Kawa M Hasan

Sultan Qaboos Univ Med J 2018 Nov 28;18(4):e461-e467. Epub 2019 Mar 28.

Department of Medicine, College of Medicine, Hawler Medical University, Erbil, Iraq.

Objectives: Chronic lymphocytic leukaemia (CLL) is characterised by an accumulation of clonal B cells in the blood, bone marrow and lymphatic tissue. This study aimed to evaluate the clinical and immunophenotypic characteristics and survival rate of CLL patients.

Methods: This retrospective study was conducted at the Nanakaly Hospital for Blood Diseases & Oncology in Erbil, Iraq, between January 2011 and December 2017. Read More

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http://dx.doi.org/10.18295/squmj.2018.18.04.006DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6443287PMC
November 2018
1 Read

18F-FDG PET/CT for Staging and Evaluation of Therapy in a Patient With Unusual Hairy Cell Leukemia Presentation.

Clin Nucl Med 2019 Apr 12. Epub 2019 Apr 12.

Department of Hematology, University Medical Centre Ljubljana, Slovenia.

Hairy cell leukemia is a rare hematologic malignancy characterized by splenomegaly, pancytopenia, and susceptibility to infections. We report a case of a 66-year-old man, diagnosed with hairy cell leukemia, without severe cytopenias and splenomegaly, but with an extensive pathological retroperitoneal mass and infiltration of the spleen and skeletal involvement. All findings were highly avid on pretreatment F-FDG PET/CT scan. Read More

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http://dx.doi.org/10.1097/RLU.0000000000002557DOI Listing
April 2019
1 Read

Cyanobacterial bioactive compound EMTAHDCA recovers splenomegaly, affects protein profile of E. coli and spleen of lymphoma bearing mice.

Mol Biol Rep 2019 Apr 12. Epub 2019 Apr 12.

Laboratory of Microbial Genetics, Department of Botany, Banaras Hindu University, Varanasi, 221005, India.

The antibacterial and anticancerous properties of EMTAHDCA have already been reported in our previous study. However, mode of action of EMTAHDCA is still elusive. The present study was aimed to investigate the molecular targets in Escherichia coli and spleen of lymphoma-bearing mice in response to cyanocompound 9-ethyliminomethyl-12 (morpholin-4-ylmethoxy)-5, 8, 13, 16-tetraaza -hexacene-2, 3- dicarboxylic acid (EMTAHDCA) isolated from fresh water cyanobacterium Nostoc sp. Read More

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http://link.springer.com/10.1007/s11033-019-04659-0
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http://dx.doi.org/10.1007/s11033-019-04659-0DOI Listing
April 2019
3 Reads

Splenectomy following JAK1/JAK2 inhibitor therapy in patients with myelofibrosis undergoing allogeneic stem cell transplantation.

Hematol Oncol Stem Cell Ther 2019 Apr 6. Epub 2019 Apr 6.

Raisa Gorbacheva Memorial Research Institute of Children Oncology, Hematology and Transplantation, Department of Hematology, Transfusiology, Transplantation, Faculty of Postgraduate Education, Pavlov First Saint Petersburg State Medical University, Saint-Petersburg, Russia.

Background: Allogeneic hematopoietic stem cell transplantation (alloHSCT) is the only treatment option with curative potential in patients with myelofibrosis (MF). The aim of our study was to evaluate the safety of splenectomy before alloHSCT in MF patients who failed to achieve significant spleen response after ruxolitinib therapy.

Methods: Splenectomy was performed in 12 patients for alloHSCT with myelofibrosis-primary (6 patients), post-polycythemia vera (3 patients). Read More

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https://linkinghub.elsevier.com/retrieve/pii/S16583876193002
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http://dx.doi.org/10.1016/j.hemonc.2019.03.001DOI Listing
April 2019
4 Reads

Composite Lymphoma Containing Mantle Cell and Peripheral T-cell Lymphoma, Not Otherwise Specified: A Report of 2 Cases Treated With Up-front Autologous Stem Cell Transplantation.

Appl Immunohistochem Mol Morphol 2019 Apr 9. Epub 2019 Apr 9.

Departments of Hematology.

We report 2 cases of composite lymphoma comprising mantle cell lymphoma and peripheral T-cell lymphoma, not otherwise specified, a rare association that has only been reported twice in the literature. In case 1, a 64-year-old woman presented with massive splenomegaly and lymphadenopathy. Immunohistochemical studies of the lymph node biopsy suggested the presence of 2 lymphomas, a predominant component of a peripheral T-cell lymphoma, not otherwise specified and an in situ mantle cell neoplasia. Read More

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http://dx.doi.org/10.1097/PAI.0000000000000769DOI Listing
April 2019
1 Read

Pelvic lipomatosis associated with portal vein thrombosis and hydronephrosis: a case report.

Authors:
Özkacmaz Sercan

J Int Med Res 2019 Apr 10:300060519840912. Epub 2019 Apr 10.

Kırşehir Ahi Evran University, Kırşehir, Turkey.

Pelvic lipomatosis is an uncommon disease with no clear etiology and it occurs secondary to deposition of a large amount of fatty tissue in the pelvis. This deposition causes compression to the rectum, bladder, and venous structures. Because of this compression, various symptoms, such as recurrent urinary infections, dysuria, tenesmus, and constipation, have mostly been reported. Read More

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http://dx.doi.org/10.1177/0300060519840912DOI Listing
April 2019
3 Reads

ALPK1 missense pathogenic variant in five families leads to ROSAH syndrome, an ocular multisystem autosomal dominant disorder.

Genet Med 2019 Apr 10. Epub 2019 Apr 10.

Department of Ophthalmology and Visual Sciences, John A Moran Eye Center, University of Utah School of Medicine, Salt Lake City, UT, USA.

Purpose: To identify the molecular cause in five unrelated families with a distinct autosomal dominant ocular systemic disorder we called ROSAH syndrome due to clinical features of retinal dystrophy, optic nerve edema, splenomegaly, anhidrosis, and migraine headache.

Methods: Independent discovery exome and genome sequencing in families 1, 2, and 3, and confirmation in families 4 and 5. Expression of wild-type messenger RNA and protein in human and mouse tissues and cell lines. Read More

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http://www.nature.com/articles/s41436-019-0476-3
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http://dx.doi.org/10.1038/s41436-019-0476-3DOI Listing
April 2019
4 Reads
7.329 Impact Factor

Osteolytic Lesions in Primary Myelofibrosis and Effect of Ruxolitinib Therapy: Report of a Case and Literature Review.

Chemotherapy 2019 Apr 9;63(6):340-344. Epub 2019 Apr 9.

Hematology Division, Foundation IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy,

Here, we report the case of a young female affected by primary myelofibrosis (PMF) who developed an osteolytic lesion of the humerus during the follow-up, and the possible efficacy of ruxolitinib in controlling this rare event. After 26 years of follow-up, the patient reported onset of acute pain at the proximal region of the left upper limb. An X-ray revealed an osteolytic bone lesion in the proximal third of the humeral shaft, which was then confirmed by magnetic resonance imaging. Read More

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http://dx.doi.org/10.1159/000497246DOI Listing
April 2019
3 Reads

An extremely severe case of Aicardi-Goutières syndrome 7 with a novel variant in IFIH1.

Eur J Med Genet 2019 Apr 6. Epub 2019 Apr 6.

Division of Neonatology, Center for Maternal-Fetal, Neonatal, and Reproductive Medicine, National Center for Child Health and Development, Tokyo, Japan.

We describe herein an extremely severe case of Aicardi-Goutières syndrome 7 (AGS7). The female patient was the daughter of nonconsanguineous parents and developed cardiomegaly, pericardial effusion, splenomegaly, and intracranial calcification during the fetal period. Because her cardiotocogram showed a non-reassuring fetal status, she was delivered at 29 weeks and 4 days of gestation by an emergency cesarean section. Read More

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http://dx.doi.org/10.1016/j.ejmg.2019.04.003DOI Listing
April 2019
1 Read

Rbpj expression in regulatory T cells is critical for restraining T2 responses.

Nat Commun 2019 04 8;10(1):1621. Epub 2019 Apr 8.

Chair for Immunology, University Regensburg and University Hospital Regensburg, Franz-Josef-Strauss-Allee 11, 93053, Regensburg, Germany.

The transcriptional regulator Rbpj is involved in T-helper (T) subset polarization, but its function in T cells remains unclear. Here we show that T-specific Rbpj deletion leads to splenomegaly and lymphadenopathy despite increased numbers of T cells with a polyclonal TCR repertoire. A specific defect of Rbpj-deficient T cells in controlling T2 polarization and B cell responses is observed, leading to the spontaneous formation of germinal centers and a T2-associated immunoglobulin class switch. Read More

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http://www.nature.com/articles/s41467-019-09276-w
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http://dx.doi.org/10.1038/s41467-019-09276-wDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6453958PMC
April 2019
5 Reads

Autoimmune Lymphoproliferative Syndrome: An Overview.

Arch Pathol Lab Med 2019 Apr 8. Epub 2019 Apr 8.

From the Department of Pathology and Laboratory Medicine, University of Wisconsin, Madison.

Autoimmune lymphoproliferative syndrome (ALPS) is an inherited nonmalignant lymphoproliferative disorder characterized by heterozygous mutations within the first apoptosis signal receptor (FAS) signaling pathway. Defects in FAS-mediated apoptosis cause an expansion and accumulation of autoreactive CD4 and CD8 (double-negative) T cells, leading to cytopenias, splenomegaly, lymphadenopathy, autoimmune disorders, and a greatly increased lifetime risk of lymphoma. The differential diagnosis of ALPS includes infection, other inherited immunodeficiency disorders, primary and secondary autoimmune syndromes, and lymphoma. Read More

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http://dx.doi.org/10.5858/arpa.2018-0190-RSDOI Listing
April 2019
4 Reads

[THE FEATURES OF THE COURSE OF INFECTIOUS MONONUKLEOSIS OF DIFFERENT ETIOLOGY IN CHILDREN].

Georgian Med News 2019 Feb(287):51-56

Odessa National Medical University, Ukraine.

Aim - to study the effect of different pathogens (EBV, CMV, HHV-6, and MIXT) on the severity of clinical-paraclinical manifestations of infectious mononucleosis in children. The clinical and laboratory study performed for 410 children aged from 10 months up to 12 years with infectious mononucleosis. The association of herpes viruses, mainly EBV, CMV and HHV type 6, takes part in the formation of the clinical picture of IM in (52,9%) of cases. Read More

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February 2019
3 Reads

Rigosertib ameliorates the effects of oncogenic KRAS signaling in a murine model of myeloproliferative neoplasia.

Oncotarget 2019 Mar 8;10(20):1932-1942. Epub 2019 Mar 8.

Department of Oncological Sciences, Icahn School of Medicine at Mount Sinai, New York, NY 10029, USA.

Aberrant signaling triggered by oncogenic or hyperactive RAS proteins contributes to the malignant phenotypes in a significant percentage of myeloid malignancies. Of these, juvenile myelomonocytic leukemia (JMML), an aggressive childhood cancer, is largely driven by mutations in genes and those that encode regulators of these proteins. The mouse model mirrors several key features of this disease and has been used extensively to determine the utility and mechanism of small molecule therapeutics in the context of RAS-driven myeloproliferative disorders. Read More

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http://dx.doi.org/10.18632/oncotarget.26735DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6443005PMC
March 2019
1 Read

Asrij/OCIAD1 suppresses CSN5-mediated p53 degradation and maintains mouse hematopoietic stem cell quiescence.

Blood 2019 Apr 5. Epub 2019 Apr 5.

Molecular Biology and Genetics Unit, Jawaharlal Nehru Centre for Advanced Scientific Research, India

Inactivation of the tumor suppressor p53 is essential for unrestrained growth of cancers. However, only 11% of hematological malignancies have mutant p53. Mechanisms that cause wild type p53 dysfunction and promote leukemia are inadequately deciphered. Read More

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http://dx.doi.org/10.1182/blood.2019000530DOI Listing
April 2019
6 Reads

Concomitant necrobiosis lipoidica and splenic abscess.

Authors:
Enver H Fekaj

J Surg Case Rep 2019 Mar 29;2019(3):rjz088. Epub 2019 Mar 29.

Department of Abdominal Surgery, University Clinical Center of Kosovo, Str. 'Bulevardi i Deshmoreve', Prishtina, Republic of Kosovo.

Necrobiosis lipoidica is an idiopathic dermatosis of unknown origin, occurring mainly in patients with diabetes. Splenic abscesses are rare entities. We report a case with concomitant necrobiosis lipoidica and splenic abscess. Read More

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http://dx.doi.org/10.1093/jscr/rjz088DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6439504PMC
March 2019
5 Reads

Splenic rupture secondary to amyloid light-chain (AL) amyloidosis associated with multiple myeloma.

J Surg Case Rep 2019 Mar 29;2019(3):rjz021. Epub 2019 Mar 29.

Trauma Surgery and Surgical Critical Care, Geisinger Wyoming Valley Hospital, Wilkes-Barre, PA 18711, USA.

Splenic rupture in the absence of major trauma is a rare occurrence, which may occur by idiopathic means or a specific pathologic process. One such condition, amyloidosis, involves the extracellular deposition of abnormally folded 'amyloid' protein, which can affect the spleen. Protein infiltration in the organ may cause splenomegaly and potentially capsular rupture in advanced cases. Read More

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http://dx.doi.org/10.1093/jscr/rjz021DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6439512PMC
March 2019
1 Read

Efficacy and safety of apixaban in a patient with systemic venous thromboembolism associated with hereditary antithrombin deficiency.

J Cardiol Cases 2019 Mar 20;19(3):106-109. Epub 2018 Dec 20.

Department of Cardiology, Nishinomiya Municipal Central Hospital, Nishinomiya, Japan.

A 40-year-old man with progressively worsening dyspnea was admitted to our hospital. On physical examination, pulse oximetry results demonstrated 80% oxygen saturation in room air. The lungs sounded clear, and both extremities appeared normal, without pitting edema. Read More

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http://dx.doi.org/10.1016/j.jccase.2018.12.007DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6429534PMC
March 2019
1 Read

Focused ultrasound to diagnose HIV-associated tuberculosis (FASH) in the extremely resource-limited setting of South Sudan: a cross-sectional study.

BMJ Open 2019 Apr 2;9(4):e027179. Epub 2019 Apr 2.

Doctors With Africa CUAMM, Yirol, South Sudan.

Objective: Our cross-sectional study aimed at evaluating the diagnostic performance of Focused Assessment with Sonography for HIV-associated tuberculosis (FASH) to detect extrapulmonary tuberculosis in extremely resource-limited settings, with visceral leishmaniasis as a differential diagnosis with overlapping sonographic feature.

Design: Cross-sectional study.

Setting: Voluntary Counselling and Testing Centre (VCT) of Yirol Hospital, South Sudan. Read More

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http://dx.doi.org/10.1136/bmjopen-2018-027179DOI Listing

NUP98-HBO1-fusion generates phenotypically and genetically relevant chronic myelomonocytic leukemia pathogenesis.

Blood Adv 2019 Apr;3(7):1047-1060

Laboratory of Oncology, School of Life Sciences, Tokyo University of Pharmacy and Life Sciences, Tokyo, Japan.

Chronic myelomonocytic leukemia (CMML) constitutes a hematopoietic stem cell (HSC) disorder characterized by prominent monocytosis and myelodysplasia. Although genome sequencing has revealed the CMML mutation profile, the mechanism of disease development remains unclear. Here we show that aberrant histone acetylation by nucleoporin-98 (NUP98)-HBO1, a newly identified fusion in a patient with CMML, is sufficient to generate clinically relevant CMML pathogenesis. Read More

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http://dx.doi.org/10.1182/bloodadvances.2018025007DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6457235PMC
April 2019
5 Reads

Characteristics and Outcomes of Young Adults with Philadelphia negative Myeloproliferative Neoplasms.

Eur J Haematol 2019 Apr 2. Epub 2019 Apr 2.

Rappaport faculty of medicine, Technion, Haifa, Israel.

Objective: Approximately 10% of Philadelphia (Ph) negative myeloproliferative neoplasms (NPM) are diagnosed at young adulthood. We aim to define the features of this group.

Methods: A multicenter retrospective study, including patients 18-45 years of age, diagnosed with Ph negative MPN between 1985-2017. Read More

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http://dx.doi.org/10.1111/ejh.13232DOI Listing
April 2019
6 Reads

Clinical presentation and hematological profile among young and old chronic lymphocytic leukemia patients in Sudan.

BMC Res Notes 2019 Apr 2;12(1):202. Epub 2019 Apr 2.

Department of Hematology, Faculty of Medical Laboratory Sciences, Al Neelain University, Khartoum, Sudan.

Objective: To assess the clinical presentation and hematological profile among young (≤ 55 years) and old (> 55 years) chronic lymphocytic leukemia patients in Sudan.

Result: In the present cross-sectional descriptive study, out of 110 cases studied, among them 31 (28.2%) were young (≤ 55 years) patients with mean age 48 years, and 79 (71. Read More

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http://dx.doi.org/10.1186/s13104-019-4239-7DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6446286PMC
April 2019
3 Reads

NOD- Mouse Model is suitable to Study Osteoarticular Brucellosis and Vaccine Safety.

Infect Immun 2019 Apr 1. Epub 2019 Apr 1.

Department of Veterinary Pathobiology, College of Veterinary Medicine & Biomedical Sciences, Texas A&M University, College Station, Texas, USA.

Osteoarticular brucellosis is the most common complication in -infected humans regardless of age, sex, or immune status. The mechanism of bone destruction caused by species remained partially unknown due to the lack of a suitable animal model. Here, to study this complication, we explored the suitability of the use of the NOD- mouse to study osteoarticular brucellosis and examined the potential use of this strain to evaluate the safety of live attenuated vaccine candidates. Read More

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http://dx.doi.org/10.1128/IAI.00901-18DOI Listing
April 2019
3 Reads

Protective effects of ambroxol in psoriasis like skin inflammation: Exploration of possible mechanisms.

Int Immunopharmacol 2019 Mar 29;71:301-312. Epub 2019 Mar 29.

Department of Regulatory Toxicology, National Institute of Pharmaceutical Education and Research (NIPER), Balanagar, Hyderabad, Telangana 500037, India. Electronic address:

The purpose of this study was to investigate the protective effects of ambroxol in psoriasis-like skin inflammation both in vitro and in vivo and delineate the molecular mechanism of ambroxol. Our data demonstrated that ambroxol has an imperative role in inhibiting the lipopolysaccharide (LPS) stimulated nitrite levels, total cellular and mitochondrial reactive oxygen species level which was determined by Griess assay, DCFDA, and MitoSOX Red staining, respectively. We found that ambroxol remarkably reduced imiquimod (IMQ) induced epidermal hyperplasia, psoriasis area and severity index (PASI) scoring, splenomegaly, skin, and ear fold thickness. Read More

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http://dx.doi.org/10.1016/j.intimp.2019.03.035DOI Listing
March 2019
2 Reads

Notch Signaling in Nestin-Expressing Cells in the Bone Marrow Maintains Erythropoiesis via Macrophage Integrity.

Stem Cells 2019 Apr 1. Epub 2019 Apr 1.

Department of Hematology, Faculty of Medicine, University of Tsukuba, Tsukuba, Japan.

Notch signaling plays pivotal roles in both hematopoietic stem/progenitor and their niche cells. Myeloproliferative phenotypes are induced by disruption of Notch signaling in nonhematopoietic bone marrow (BM) cells. Nestin-expressing cells in the BM reportedly represent a component of the hematopoietic stem cell niche. Read More

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http://dx.doi.org/10.1002/stem.3011DOI Listing
April 2019
3 Reads

Etiology, presenting features and outcome of children with non-cirrhotic portal vein thrombosis: A multicentre national study.

Dig Liver Dis 2019 Mar 6. Epub 2019 Mar 6.

Paediatric Liver, GI and Transplantation, Hospital Papa Giovanni XXIII Bergamo, Bergamo, Italy. Electronic address:

Objectives: Non-cirrhotic portal vein thrombosis (PVT) is a main cause of portal hypertension in children. We describe the characteristics at presentation and outcome of a cohort of patients with PVT to determine clinical features and predictors of outcome.

Methods: We recorded: (1) Associated factors: prematurity, congenital malformations, neonatal illnesses, umbilical vein catheterization (UVC), deep infections, surgery; (2) congenital and acquired prothrombotic disorders; (3) features at last follow up including survival rate and need for surgery. Read More

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http://dx.doi.org/10.1016/j.dld.2019.02.014DOI Listing
March 2019
2 Reads
2.963 Impact Factor

Mice with Calr mutations homologous to human CALR mutations only exhibit mild thrombocytosis.

Blood Cancer J 2019 Mar 29;9(4):42. Epub 2019 Mar 29.

Department of Gastroenterology and Hematology, Faculty of Medicine, University of Miyazaki, Miyazaki, Japan.

Calreticulin (CALR) exon 9 frameshift mutations, commonly detected in essential thrombocythemia (ET) and primary myelofibrosis patients, activate signal transducer and activator of transcription (STAT) proteins in the presence of Myeloproliferative Leukemia Virus (MPL) and induce ET in vivo. Loss of the KDEL motif, an endoplasmic reticulum retention signal, and generation of many positively charged amino acids (AAs) in the mutated C-terminus are thought to be important for disease induction. To test this hypothesis, we generated mice harboring a Calr frameshift mutation using the CRISPR/Cas9 system. Read More

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http://dx.doi.org/10.1038/s41408-019-0202-zDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6440999PMC
March 2019
2 Reads

[ELABORATION AND TREATMENT DECISION IN INCIDENTAL SPLANCHNIC VEIN THROMBOSIS].

Harefuah 2019 Mar;158(3):176-179

Liver Unit of Clalit Medical Services - Haifa and Western, Galilee region.

Introduction: In the present article, a patient with incidental findings in computerized tomography (CT) of cavernous transformation of the splanchnic veins, thrombosis of the splenic and portal veins, esophagus and gastric varicose veins and splenomegaly is presented. The CT was performed due to mild chronic normocytic anemia known for two years and the elevated level of LDH (Lactic dehydrogenase). Although usually such incidental findings without cirrhosis do not necessitate anticoagulation therapy according to the literature, in cases of myeloproliferative diseases, anticoagulation is required in order to prevent thrombus propagation. Read More

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March 2019
1 Read

[A Case of Stage ⅣRectal Cancer with Dyskeratosis Congenita].

Gan To Kagaku Ryoho 2019 Mar;46(3):515-517

Dept. of Gastroenterological Surgery, Kanazawa University.

A 27-year-old man was diagnosed with dyskeratosis congenita from DKC1 gene mutation at 9 years of age and had been followed-up regularly.An upper gastrointestinal endoscopy performed for vomiting revealed gastric varices.Further examination resulted in a diagnosis of Stage Ⅳrectal cancer with portal hypertension, splenomegaly, liver, and lung metastasis and he was referred to our department. Read More

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March 2019
2 Reads

Chronic alcohol consumption exacerbates murine cytomegalovirus infection via impairing nonspecific and specific NK activation in mice.

FASEB Bioadv 2019 Jan 14;1(1):18-31. Epub 2018 Sep 14.

Department of Pharmaceutical Sciences, College of Pharmacy and Pharmaceutical Sciences, Washington State University, Spokane, WA 99210.

Chronic alcohol consumption increases the susceptibility to infectious diseases by compromising immune system. Cytomegalovirus infection is common in human and usually is asymptomatic in immunocompetent people. However, it can induce life-threatening medical complications in immunocompromised individuals such as alcoholics. Read More

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http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6430117PMC
January 2019
1 Read

Radiation: a poly-traumatic hit leading to multi-organ injury.

Cell Biosci 2019 12;9:25. Epub 2019 Mar 12.

1Radiation Combined Injury Program, Armed Forces Radiobiology Research Institute, Bethesda, MD 20889 USA.

The range of radiation threats we face today includes everything from individual radiation exposures to mass casualties resulting from a terrorist incident, and many of these exposure scenarios include the likelihood of additional traumatic injury as well. Radiation injury is defined as an ionizing radiation exposure inducing a series of organ injury within a specified time. Severity of organ injury depends on the radiation dose and the duration of radiation exposure. Read More

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http://dx.doi.org/10.1186/s13578-019-0286-yDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6417034PMC
March 2019
2 Reads

A 30-Year-Old Carrier of Gaucher Disease with Multiple Myeloma.

Case Rep Oncol Med 2019 13;2019:6469196. Epub 2019 Feb 13.

Lenox Hill Hospital, Northwell Health, New York, NY, USA.

We are reporting a case of a 30-year-old male with no past medical history who presented with new onset of renal failure, anemia, and splenomegaly and was diagnosed with multiple myeloma. Given the splenomegaly and the patient's Jewish heritage, blood tests were done and the patient was found to be a Gaucher disease carrier. The association of Gaucher disease and multiple myeloma has previously been reported; however, we want to describe the case of a young Gaucher disease carrier who developed multiple myeloma and provide a review of the literature. Read More

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http://dx.doi.org/10.1155/2019/6469196DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6393880PMC
February 2019
1 Read

Castleman Disease in a Patient with Common Variable Immunodeficiency.

Case Reports Immunol 2019 14;2019:5476383. Epub 2019 Feb 14.

Peritoneal Surface Malignancy and Soft Tissue Sarcoma Program, Messina University Medical School Hospital, Messina, Italy.

Common variable immunodeficiency (CVID) is a primary immunodeficiency due to a disorder of the adaptive immune system which causes hypogammaglobulinemia and therefore an increased susceptibility to infection; noninfectious, inflammatory conditions including systemic autoimmunity and lymphoproliferative complications are also commonly associated with CVID. Castleman disease (CD) is a systemic disease clinically characterized by diffuse lymphadenopathy, splenomegaly, anemia, and systemic inflammatory symptoms. This makes CD a great mimicker of more common benign and malignant masses in the neck, chest, abdomen, and pelvis. Read More

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http://dx.doi.org/10.1155/2019/5476383DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6393918PMC
February 2019
2 Reads

Unusual first presentation of a metabolic disorder.

BMJ Case Rep 2019 Mar 22;12(3). Epub 2019 Mar 22.

Paediatric Emergency Department, North Middlesex University Hospital NHS Trust, London, UK.

An 8-month-old boy presented to hospital with a fever, irritability and 'back arching'. On examination, he demonstrated profound opisthotonic posturing and had tonsillitis. He had a full septic screen and was treated with broad spectrum antibiotics. Read More

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http://dx.doi.org/10.1136/bcr-2018-226716DOI Listing
March 2019
2 Reads

Old age increases microglial senescence, exacerbates secondary neuroinflammation, and worsens neurological outcomes after acute traumatic brain injury in mice.

Neurobiol Aging 2019 Feb 20;77:194-206. Epub 2019 Feb 20.

Department of Anesthesiology and Center for Shock, Trauma and Anesthesiology Research (STAR), University of Maryland School of Medicine, Baltimore, MD, USA. Electronic address:

After traumatic brain injury (TBI), individuals aged over 65 years show increased mortality and worse functional outcomes compared with younger persons. As neuroinflammation is a key pathobiological mechanism of secondary injury after TBI, we examined how aging affects post-traumatic microglial responses and functional outcomes. Young (3-month-old) and aged (18-month-old) male C57Bl/6 mice were subjected to moderate-level controlled cortical impact or sham surgery, and neurological function was evaluated. Read More

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http://dx.doi.org/10.1016/j.neurobiolaging.2019.02.010DOI Listing
February 2019
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Hereditary spherocytosis caused by copy number variation in SPTB gene identified through targeted next-generation sequencing.

Int J Hematol 2019 Mar 21. Epub 2019 Mar 21.

Department of Laboratory Medicine, College of Medicine, Seoul St. Mary's Hospital, The Catholic University of Korea, Seoul, Republic of Korea.

Hereditary spherocytosis (HS) is a heterogeneous genetic disorder characterized by spherocytosis on peripheral blood smear with hemolytic anemia, accompanied by signs of hemolysis. Herein, we report a 5-month-old Korean girl with HS resulting from a de novo 271 Kb microdeletion of 14q23.3. Read More

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http://dx.doi.org/10.1007/s12185-019-02630-0DOI Listing
March 2019
3 Reads
1.679 Impact Factor

Ultrasound Elastography to Quantify Liver Disease Severity in Autosomal Recessive Polycystic Kidney Disease.

J Pediatr 2019 Mar 19. Epub 2019 Mar 19.

Department of Radiology, Children's Hospital of Philadelphia, Philadelphia, PA; Department of Radiology, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, PA.

Objectives: To evaluate the diagnostic accuracy of ultrasound elastography with acoustic radiation force impulse (ARFI) to detect congenital hepatic fibrosis and portal hypertension in children with autosomal recessive polycystic kidney disease (ARPKD).

Study Design: Cross-sectional study of 25 children with ARPKD and 24 healthy controls. Ultrasound ARFI elastography (Acuson S3000, Siemens Medical Solutions USA, Inc, Malvern, Pennsylvania) was performed to measure shear wave speed (SWS) in the right and left liver lobes and the spleen. Read More

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http://dx.doi.org/10.1016/j.jpeds.2019.01.055DOI Listing
March 2019
2 Reads

Neutrophilic eccrine hidradenitis secondary to pegfilgrastim in a patient with synovial sarcoma.

Clin Case Rep 2019 Mar 7;7(3):533-536. Epub 2019 Feb 7.

University of Kansas Hospital Kansas City Kansas.

Here, we report a case of neutrophilic eccrine hidradenitis (NEH) in a teenage male with synovial sarcoma associated with extracutaneous manifestations including myositis and splenomegaly secondary to pegfilgrastim. To the best of our knowledge, NEH has not been previously reported to occur in association with extracutaneous manifestations. Read More

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http://dx.doi.org/10.1002/ccr3.1932DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6406152PMC
March 2019
2 Reads

New Noonan syndrome model mice with RIT1 mutation exhibit cardiac hypertrophy and susceptibility to β-adrenergic stimulation-induced cardiac fibrosis.

EBioMedicine 2019 Mar 18. Epub 2019 Mar 18.

Department of Medical Genetics, Tohoku University Graduate School of Medicine, Sendai, Japan. Electronic address:

Background: Noonan syndrome (NS) is a genetic disorder characterized by short stature, a distinctive facial appearance, and heart defects. We recently discovered a novel NS gene, RIT1, which is a member of the RAS subfamily of small GTPases. NS patients with RIT1 mutations have a high incidence of hypertrophic cardiomyopathy and edematous phenotype, but the specific role of RIT1 remains unclear. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S23523964193015
Publisher Site
http://dx.doi.org/10.1016/j.ebiom.2019.03.014DOI Listing
March 2019
11 Reads

Indigofera oblongifolia protects against trypanosomiasis-induced spleen injury.

J Infect Public Health 2019 Mar 18. Epub 2019 Mar 18.

Department of Zoology, College of Science, King Saud University, Riyadh, Saudi Arabia.

Background: Trypanosomiasis is a neglected tropical disease, transmitted by blood-sucking insects and can affect humans and animals, depending on the species of Trypanosoma parasite. Trypanosoma has acquired resistance to the majority of drugs used; hence, alternative medicines are required. Indigofera oblongifolia leaf extract (IOE) has been shown to treat blood stage malaria. Read More

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http://dx.doi.org/10.1016/j.jiph.2019.03.005DOI Listing
March 2019
2 Reads