19,420 results match your criteria Splenomegaly


Prevalence of MPL (W515K/L) Mutations in Patients with Negative-JAK2 (V617F) Myeloproliferative Neoplasm in North-East of Iran.

Iran J Pathol 2018 25;13(4):397-402. Epub 2018 Sep 25.

Cellular and Molcular Research Center, Birjand University of Medical Sciences, Birjand, Iran.

Background And Objective: Janus kinase 2 (JAK2) and Myeloproliferative Leukemia (MPL) mutations are confirmatory indicators for Myeloproliferative Neoplasm (MPN). The current study was performed to determine the frequency of MPL mutation in MPN patients without JAK2 mutation, in order to assign MPL mutation frequency in North-East of Iran.

Methods: Total of 105 negative JAK2 cases including 5 Myeloproliferative Disorders (MPD), 15 Polycytemia Vera (PV) and 15 Essential Thrombocytosis (ET) who referred to Qaem Medical Center were assigned to this study. Read More

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September 2018

Clinical characteristics of extranodal NK/T-cell lymphoma-associated hemophagocytic lymphohistiocytosis.

Cancer Manag Res 2019 23;11:997-1002. Epub 2019 Jan 23.

Department of Medical Oncology, Fudan University Shanghai Cancer Center, Shanghai 200032, China,

Background: Extranodal natural killer (NK) / T cell lymphoma is a subtype of non-Hodgkin's lymphoma (NHL) that usually has an aggressive clinical course. It is the predominant trigger of lymphoma-associated hemophagocytic syndrome (LAHS), which is highly lethal and with extremely poor prognosis. This study is aiming to characterize the associated clinical features and prognostic factors of the disease. Read More

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https://www.dovepress.com/clinical-characteristics-of-extran
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http://dx.doi.org/10.2147/CMAR.S183784DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6350644PMC
January 2019
4 Reads

Visceral leishmaniasis with haemophagocytic lymphohistiocytosis.

BMJ Case Rep 2019 Feb 13;12(2). Epub 2019 Feb 13.

Department of General Medicine, Christian Medical College and Hospital, Vellore, Tamil Nadu, India.

A 27-year-old man presented with high-grade intermittent fever for 4 months, generalised fatigue for 2 months, intermittent gum bleeds for 1 month and loss of weight of 15 kg. He appeared cachectic with generalised wasting, had pallor and features of reticuloendothelial system proliferation. His liver span was 17 cm. Read More

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http://dx.doi.org/10.1136/bcr-2018-226361DOI Listing
February 2019

Gaucher disease: single gene molecular characterization of one-hundred Indian patients reveals novel variants and the most prevalent mutation.

BMC Med Genet 2019 Feb 14;20(1):31. Epub 2019 Feb 14.

FRIGE's Institute of Human Genetics, FRIGE House, Jodhpur Gam Road, Satellite, Ahmedabad, Gujarat, 380015, India.

Background: Gaucher disease is a rare pan-ethnic, lysosomal storage disorder resulting due to beta-Glucosidase (GBA1) gene defect. This leads to the glucocerebrosidase enzyme deficiency and an increased accumulation of undegraded glycolipid glucocerebroside inside the cells' lysosomes. To date, nearly 460 mutations have been described in the GBA1 gene. Read More

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http://dx.doi.org/10.1186/s12881-019-0759-1DOI Listing
February 2019

Fever of Unknown Origin: Don't Forget Kala-Azar Even in Europe.

Eur J Case Rep Intern Med 2018 22;5(2):000772. Epub 2018 Feb 22.

Haematology Department, Hospital Universitario Miguel Servet, Zaragoza, Spain.

We present the case of a 70-year-old male patient with fever of unknown origin after a long period of convalescence from a previous admission to a chronic care hospital. During the admission, multiple combinations of antibiotic and antifungal treatments were prescribed, but with persistent fever and, eventually, neutropenia (200 lymphocytes, 0 neutrophils). Given the suspicion of infection at bone marrow level, a biopsy was performed as was serology of , both diagnostic determinations. Read More

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http://dx.doi.org/10.12890/2017_000772DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6346938PMC
February 2018

Haemophagocytic Lymphohistiocytosis in a 16-Year-Old Boy.

Mymensingh Med J 2019 Jan;28(1):241-244

Dr Md Atikur Rahman, Phase-B Resident, Department of Internal Medicine, Bangabandhu Sheikh Mujib Medical University (BSMMU), Dhaka, Bangladesh; E-mail:

Haemophagocytosis encompasses phagocytosis of erythrocytes, leukocytes, platelets and their precursors by macrophages in bone marrow and other tissues. Haemophagocytic lymphohistiocytosis (HLH) usually presents with high fever, pancytopenia, splenomegaly, lymphadenopathy, haemophagocytosis in bone marrow, liver, lymphnodes or CSF. We report coagulase negative Staphylococcus induced HLH in a 16 year old boy presenting with high grade fever, lymphadenopathy, hepatosplenomegaly, pancytopenia with neutropenic sepsis in the department of Medicine, Bangabandhu Sheikh Mujib Medical University (BSMMU), Dhaka, Bangladesh on 28th November 2016. Read More

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January 2019

Risk-factors Associated with Poor Outcomes in VEO-IBD Secondary to XIAP Deficiency: A Case Report and Literature Review.

J Pediatr Gastroenterol Nutr 2019 Feb 7. Epub 2019 Feb 7.

Division of Gastroenterology, Hepatology and Nutrition, Boston Children's Hospital, Boston, MA.

Very early onset inflammatory bowel disease (VEO-IBD) represents a diagnostic and treatment challenge. Here we present a case of VEO-IBD secondary to a mutation in BIRC4 gene, which encodes X-linked inhibitor of apoptosis protein (XIAP), in a 17 month-old male with severe failure to thrive, intractable diarrhea and hepatosplenomegaly. Endoscopy and histology identified only mild duodenitis and ileitis, but severe pancolitis with crypt abscesses and epithelium apoptosis. Read More

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http://Insights.ovid.com/crossref?an=00005176-900000000-9653
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http://dx.doi.org/10.1097/MPG.0000000000002297DOI Listing
February 2019
6 Reads

Roux-en-Y Gastric Bypass Reversal for Severe Malnutrition and Cirrhosis.

Obes Surg 2019 Feb 11. Epub 2019 Feb 11.

Assistance Publique-Hôpitaux de Paris (AP-HP), Department of Digestive and Metabolic Surgery, Avicenne University Hospital, Centre intégré Nord Francilien de la prise en charge de l'Obésité (CINFO), Université Paris XIII-UFR SMBH « Léonard de Vinci », 125 route de Stalingrad, 93000, Bobigny, France.

Background: Severe post-operative malnutrition (SM) is a dreaded complication after gastric bypass often related to the short bowel syndrome consecutive limb length mismeasurement or intestinal resections. Patients with rapid weight loss or malnutrition can experience liver failure with cirrhosis and require liver transplantation (LT). Malnutrition can constitute a contraindication to LT since it negatively impacts on postoperative morbidity. Read More

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http://dx.doi.org/10.1007/s11695-019-03770-2DOI Listing
February 2019

Epigenetic modification in 4T1 mouse breast cancer model by artificial light at night and melatonin - the role of DNA-methyltransferase.

Chronobiol Int 2019 Feb 12:1-15. Epub 2019 Feb 12.

b The Israeli Center for Interdisciplinary Research in Chronobiology , University of Haifa , Haifa , Israel.

Currently, one of the most disputed hypotheses regarding breast cancer (BC) development is exposure to short wavelength artificial light at night (ALAN) as multiple studies suggest a possible link between them. This link is suggested to be mediated by nocturnal melatonin suppression that plays an integral role in circadian regulations including cell division. The objective of the research was to evaluate effects of 1 × 30 min/midnight ALAN (134 µ Wcm, 460 nm) with or without nocturnal melatonin supplement on tumor development and epigenetic responses in 4T1 tumor-bearing BALB/c mice. Read More

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http://dx.doi.org/10.1080/07420528.2019.1574265DOI Listing
February 2019
1 Read

Hand-assisted laparoscopic splenectomy is a useful surgical treatment method for patients with excessive splenomegaly: A meta-analysis.

World J Clin Cases 2019 Feb;7(3):320-334

Department of General Surgery, Hepato-Biliary-Pancreatic Surgery Division, The Second Affiliated Hospital of Nanchang University, Nanchang 330006, Jiangxi Province, China.

Background: Hand-assisted laparoscopic splenectomy (HALS) can help overcome the drawbacks of laparoscopic splenectomy (LS) while maintaining its advantages.

Aim: To evaluate the efficacy and advantages of HALS for splenomegaly.

Methods: The relevant literature was reviewed using the PubMed, EMBASE, Cochrane, Ovid Medline, and Wanfang databases to compare the clinical outcomes of HALS and LS. Read More

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http://dx.doi.org/10.12998/wjcc.v7.i3.320DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6369387PMC
February 2019
1 Read

[Recurrence of malignant lymphoma immediately after treatment for hepatitis C virus using direct-acting antivirals].

Nihon Shokakibyo Gakkai Zasshi 2019 ;116(2):177-183

Department of Anatomic Pathology, Kurashiki Central Hospital.

A Japanese male in his 70s with chronic hepatitis C was diagnosed with diffuse large B-cell lymphoma and achieved and maintained complete remission following treatment with eight cycles of R-CHOP (rituximab, cyclophosphamide, doxorubicin hydrochloride, vincristine sulfate, and prednisolone). Seven years later, he received the direct-acting antivirals (DAAs) sofosbuvir/ledipasvir for hepatitis C virus (HCV) genotype 1b. Although the patient achieved sustained virological response immediately after the initial treatment period, laboratory data showed elevation of LD and soluble IL-2R. Read More

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https://www.jstage.jst.go.jp/article/nisshoshi/116/2/116_177
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http://dx.doi.org/10.11405/nisshoshi.116.177DOI Listing
January 2019
3 Reads

[Clinical Significance of Detecting the Erythrocyte Life Expectancy in Patients with Malignant Lymphoma Complicated with Anemia].

Zhongguo Shi Yan Xue Ye Xue Za Zhi 2019 Feb;27(1):80-85

Department of Hematology, Peking Union Hospital, Peking Union Medical College, Chinese Academy of Medical Sciences, Beijing 100730,

Objective: To investigate the clinical value of erythrocyte life span in the diagnosis and treatment of lymphoma patients with anemia.

Methods: The clinical data of 45 patients with lymphoma diagnosed by Department of Hematology in Beijing Union Hospital from January 2017 to June 2017 were collected. The life of erythrocytes was detected by alveolar gas CO test. Read More

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http://dx.doi.org/10.7534/j.issn.1009-2137.2019.01.013DOI Listing
February 2019
1 Read

EDA fibronectin-TLR4 axis sustains megakaryocyte expansion and inflammation in bone marrow fibrosis.

J Exp Med 2019 Feb 7. Epub 2019 Feb 7.

Department of Molecular Medicine, University of Pavia, Pavia, Italy

The fibronectin EDA isoform (EDA FN) is instrumental in fibrogenesis but, to date, its expression and function in bone marrow (BM) fibrosis have not been explored. We found that mice constitutively expressing the EDA domain (EIIIA), but not EDA knockout mice, are more prone to develop BM fibrosis upon treatment with the thrombopoietin (TPO) mimetic romiplostim (TPO). Mechanistically, EDA FN binds to TLR4 and sustains progenitor cell proliferation and megakaryopoiesis in a TPO-independent fashion, inducing LPS-like responses, such as NF-κB activation and release of profibrotic IL-6. Read More

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http://dx.doi.org/10.1084/jem.20181074DOI Listing
February 2019
1 Read

The Schistosomiasis SpleenOME: Unveiling the Proteomic Landscape of Splenomegaly Using Label-Free Mass Spectrometry.

Front Immunol 2018 22;9:3137. Epub 2019 Jan 22.

Departamento de Ciências Biológicas, Núcleo de Pesquisas em Ciências Biológicas, Universidade Federal de Ouro Preto, Ouro Preto, Brazil.

Schistosomiasis is a neglected parasitic disease that affects millions of people worldwide and is caused by helminth parasites from the genus . When caused by , it is associated with the development of a hepatosplenic disease caused by an intense immune response to the important antigenic contribution of adult worms and to the presence of eggs trapped in liver tissue. Although the importance of the spleen for the establishment of immune pathology is widely accepted, it has received little attention in terms of the molecular mechanisms operating in response to the infection. Read More

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https://www.frontiersin.org/article/10.3389/fimmu.2018.03137
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http://dx.doi.org/10.3389/fimmu.2018.03137DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6352917PMC
January 2019
4 Reads

[Histoplasmosis in AIDS patients without tegumentary manifestations].

Rev Chilena Infectol 2018 ;35(5):560-565

Unidad de Micología, Hospital de Enfermedades Infecciosas, Buenos Aires, Argentina.

Background: Histoplasmosis is a mycosis with a high prevalence in HIV/AIDS patients. Clinical presentation includes a wide spectrum of manifestations and diagnosis usually takes up to several weeks in patients who do not present cutaneous lesions.

Aim: To determine the clinical and microbiological characteristics as well as some biochemical parameters in patients with AIDS-associated histoplasmosis without tegumentary lesions, in order to develop a guideline which enables an early empiric treatment in cases of difficult diagnosis. Read More

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http://dx.doi.org/10.4067/s0716-10182018000500560DOI Listing
January 2018
1 Read

Hand-assisted Laparoscopic Splenectomy Advantages Over Complete Laparoscopic Splenectomy For Splenomegaly.

Surg Laparosc Endosc Percutan Tech 2019 Feb 1. Epub 2019 Feb 1.

Department of General Surgery, The Second Affiliated Hospital of Nanchang University, Nanchang, China.

Background: Laparoscopic splenectomy (LS) is generally applied for splenectomy, However, it also brings some technical challenges, especially for splenomegaly. Hand-assisted laparoscopic splenectomy (HALS) can help to overcome these drawbacks while maintaining the superiority of LS. This study was aimed to evaluate the efficacy and advantage of HALS for splenomegaly. Read More

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http://dx.doi.org/10.1097/SLE.0000000000000640DOI Listing
February 2019
1 Read

Laparoscopic Splenectomy Versus Open Splenectomy In Massive and Giant Spleens: Should we Update the 2008 EAES Guidelines?

Surg Laparosc Endosc Percutan Tech 2019 Feb 1. Epub 2019 Feb 1.

Division of Hematology 1, IRCCS San Martino, University Hospital, National Institute for Cancer Research, Genoa, Italy.

The objective of this study was to derive some useful parameters to define the feasibility of laparoscopic splenectomy (LS) in massive [spleen longitudinal diameter (SLD)>20 cm] and giant spleens (SLD>25 cm). Between December 1996 and May 2017, 175 patients underwent an elective splenectomy. A laparoscopic approach was used in 133 (76%) patients. Read More

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http://dx.doi.org/10.1097/SLE.0000000000000637DOI Listing
February 2019
1 Read

Lymphoid Interstitial Pneumonia in Common Variable Immune Deficiency - Case Report With Disease Monitoring in Various Therapeutic Options: Pleiotropic Effects of Rituximab Regimens.

Front Pharmacol 2018 18;9:1559. Epub 2019 Jan 18.

Department of Immunology of Infectious Diseases, Ludwik Hirszfeld Institute of Immunology and Experimental Therapy, Polish Academy of Sciences, Wrocław, Poland.

Lymphoid interstitial pneumonia (LIP) is a rare lymphoproliferative disease. LIP in common variable immunodeficiency (CVID) was observed in a patient during immunomodulatory therapy after progression of the disease (i.e. Read More

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http://dx.doi.org/10.3389/fphar.2018.01559DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6346143PMC
January 2019
1 Read

A Rare Case of Spontaneous Tumor Lysis Syndrome in Idiopathic Primary Myelofibrosis.

Am J Case Rep 2019 Feb 3;20:146-150. Epub 2019 Feb 3.

Department of Hemato-Oncology, Chosun University Hospital, Gwangju, South Korea.

BACKGROUND Tumor lysis syndrome (TLS) is an oncologic emergency resulting from the massive destruction of tumor cells after cytotoxic chemotherapy for chemosensitive malignancies with a high tumor burden. Its clinical manifestations include severe electrolyte disturbances, metabolic acidosis, acute renal failure secondary to urate deposition in the kidney, heart, and skeletal muscle, and nervous system dysfunction. We report an extremely rare case of spontaneous TLS (STLS) in idiopathic primary myelofibrosis (PMF). Read More

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http://dx.doi.org/10.12659/AJCR.912682DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6369657PMC
February 2019
2 Reads

Refugee children with beta-thalassemia in Turkey: Overview of demographic, socioeconomic, and medical characteristics.

Pediatr Blood Cancer 2019 Feb 1:e27636. Epub 2019 Feb 1.

Pediatric Hematology and Oncology, University of Health Sciences, Ankara Child Health and Diseases Hematology Oncology Training and Research Hospital, Ankara, Turkey.

Aim: Since the beginning of the Syrian civil war, more than 3.5 million Syrians have been under temporary protection status in Turkey. Because beta-thalassemia (BT) is a prevalent disorder in the Mediterranean countries, we decided to estimate the prevalence of and make an overview of the demographic, socioeconomic, medical characteristics, and healthcare problems of refugee children with BT. Read More

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http://dx.doi.org/10.1002/pbc.27636DOI Listing
February 2019
1 Read

Diagnosis of Mucopolysaccharidosis Based on History and Clinical Features: Evidence from the Bajio Region of Mexico.

Cureus 2018 Nov 20;10(11):e3617. Epub 2018 Nov 20.

Pediatrics, Hospital General De San Luis De La Paz, San Luis de La Paz, MEX.

Introduction Mucopolysaccharidosis (MPS) are infrequent deposit diseases; generally, the diagnosis is delayed until symptoms appear. Age or presentation is related to the severity of the disease. A substantial number of patients are misdiagnosed since they describe nonspecific initial symptoms and signs in common. Read More

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http://dx.doi.org/10.7759/cureus.3617DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6349567PMC
November 2018
1 Read

[Risk factors for recurrent thrombosis in patients with polycythemia vera and essential thrombocythemia].

Zhonghua Xue Ye Xue Za Zhi 2019 Jan;40(1):17-23

Department of Hematology, Beijing Anzhen Hospital, Capital Medical University, Beijing 100029, China.

To analyze the clinical characteristics of recurrent thrombosis in patients with polycythemia vera (PV) and essential thrombocythemia (ET) to probe the risk factors for recurrent thrombosis in patients with ET and PV. The clinical data of 104 ET and PV patients with thrombosis in Beijing Anzhen Hospital from February 2001 to November 2016 were retrospectively analyzed. Thrombosis reoccurred in 38 patients. Read More

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http://dx.doi.org/10.3760/cma.j.issn.0253-2727.2019.01.004DOI Listing
January 2019
2 Reads

[A study of clinical characteristics and prognosis of primary myelofibrosis patients with thrombocytopenia in varied degrees].

Zhonghua Xue Ye Xue Za Zhi 2019 Jan;40(1):12-16

Institute of Hematology and Blood Diseases Hospital, CAMS & PUMC, The State Key Laboratory of Experimental Hematology, Tianjin 300020, China.

To evaluate clinical characteristics and prognosis of primary myelofibrosis (PMF) patients with thrombocytopenia in varied degrees. Clinical features and survival data of 1 305 Chinese patients with PMF were retrospectively analyzed. The prognostic value of thrombocytopenia in patients with PMF was evaluated. Read More

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http://dx.doi.org/10.3760/cma.j.issn.0253-2727.2019.01.003DOI Listing
January 2019
1 Read

Abscess of the spleen in a patient with infectious endocarditis.

Ter Arkh 2018 Nov;90(11):98-101

I.M. Sechenov First Moscow State Medical University of the Ministry of Health of the Russian Federation, Moscow, Russia.

A patient with infective endocarditis (IE), complicated by the development of the abscess of the spleen, is described. The diagnosis of IE was verified several months after the onset of clinical symptoms (fever, hemorrhagic skin rashes, splenomegaly).The patient suspected hemorrhagic vasculitis and lymphoma of the spleen, which were not confirmed. Read More

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http://dx.doi.org/10.26442/terarkh2018901198-101DOI Listing
November 2018
2 Reads

Blood transfusion therapy for β-thalassemia major and hemoglobin E β-thalassemia: Adequacy, trends, and determinants in Sri Lanka.

Pediatr Blood Cancer 2019 Jan 29:e27643. Epub 2019 Jan 29.

Colombo North Teaching Hospital, Ragama, Sri Lanka.

Background: Regular blood transfusion therapy still remains the cornerstone in the management of β-thalassemia. Although recommendations are clear for patients with β-thalassemia major, uniform transfusion guidelines are lacking for patients with hemoglobin E β-thalassemia. In this study, we aim to describe the adequacy, trends, and determinants of blood transfusion therapy in a large cohort of pediatric patients with β-thalassemia major and hemoglobin E β-thalassemia. Read More

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http://dx.doi.org/10.1002/pbc.27643DOI Listing
January 2019
1 Read
2.562 Impact Factor

The effect of splenectomy on complement regulatory proteins in erythrocytes in β-thalassemia major.

Arch Med Sci 2019 Jan 30;15(1):191-195. Epub 2018 Dec 30.

Department of Medical Oncology, Antalya Education and Research Hospital, Antalya, Turkey.

Introduction: Hemolysis due to ineffective erythropoiesis is a serious problem β-thalassemia major (β-TM) patients. The role of complement system in the etiopathogenesis of hemolysis observed in β-TM were released. Hemolysis induced by activation of complement system is prevented by complement regulatory proteins. Read More

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http://dx.doi.org/10.5114/aoms.2018.81036DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6348341PMC
January 2019
1 Read

SMCR8 negatively regulates AKT and MTORC1 signaling to modulate lysosome biogenesis and tissue homeostasis.

Autophagy 2019 Jan 29:1-15. Epub 2019 Jan 29.

a Department of Molecular Biology and Genetics, Weill Institute for Cell and Molecular Biology , Cornell University , Ithaca , NY , USA.

The intronic hexanucleotide expansion in the C9orf72 gene is one of the leading causes of frontotemporal lobar degeneration (FTLD) and amyotrophic lateral sclerosis (ALS), two devastating neurodegenerative diseases. C9orf72 forms a heterodimer with SMCR8 (Smith-Magenis syndrome chromosome region, candidate 8) protein. However, the physiological function of SMCR8 remains to be characterized. Read More

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http://dx.doi.org/10.1080/15548627.2019.1569914DOI Listing
January 2019
1 Read

Disseminated Blastomycosis in a Teenager Presenting with Pleural Effusion and Splenomegaly.

J Emerg Med 2019 Jan 25. Epub 2019 Jan 25.

Medical College of Wisconsin, Milwaukee, Wisconsin; Children's Hospital of Wisconsin, Milwaukee, Wisconsin.

Background: Blastomycosis is caused by a fungus endemic to states and providences bordering the Lawrence Rivers and the Great Lakes. It can lead to significant pathology in both immunocompetent and immunocompromised hosts. This case report describes disseminated blastomycosis in an otherwise healthy 16-year-old patient. Read More

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http://dx.doi.org/10.1016/j.jemermed.2018.12.035DOI Listing
January 2019
1 Read

Chronic Basophilic Leukaemia in a Dog.

J Comp Pathol 2019 Jan 27;166:5-8. Epub 2018 Nov 27.

Department of Veterinary Pathology, School of Veterinary Medicine, Nippon Veterinary and Life Science University, Tokyo, Japan. Electronic address:

A 13-year-old neutered female mixed-breed dog with a clinical history of emaciation, inappetence and vomiting for 2 months was presented. Blood tests showed marked leucocytosis with increased neutrophil and basophil count, mild thrombocytosis and anaemia. Seven days after the initial visit, the dog died and was submitted for necropsy examination. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S00219975183003
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http://dx.doi.org/10.1016/j.jcpa.2018.10.170DOI Listing
January 2019
6 Reads

Whole exome sequencing identified a novel mutation (p.Ala1884Pro) of β-spectrin in a Chinese family with hereditary spherocytosis.

J Gene Med 2019 Jan 28:e3073. Epub 2019 Jan 28.

Department of Cell Biology, The School of Life Sciences, Central South University, Changsha, China.

Background: Hereditary spherocytosis (HS) is an inherited disorder of erythrocyte. The typical feature of HS is the presence of spherical-shaped erythrocytes on the peripheral blood smear. According to previous studies, more than five candidate genes, such as ANK1, SPTB, SPTA1, SLC4A1 and EPB42 have been identified in HS patients. Read More

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http://dx.doi.org/10.1002/jgm.3073DOI Listing
January 2019
2 Reads

Liver histopathological findings in advanced heart failure: a reappraisal of cardiac cirrhosis concept.

Intern Emerg Med 2019 Jan 28. Epub 2019 Jan 28.

Internal Medicine, University of Campania 'L. Vanvitelli', Naples, Italy.

Cardiogenic liver disease is a common yet poorly characterized complication of advanced heart failure (HF), and may impact clinical management in the setting of heart transplant evaluation. In this retrospective study, we describe clinical and histopathological features of liver injury in advanced HF, with a focus on the role of liver biopsy. Included were 45 HF patients, assessed for possible heart transplant, who underwent liver biopsy for suspected liver disease. Read More

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http://dx.doi.org/10.1007/s11739-019-02033-3DOI Listing
January 2019
1 Read

Transient Elastography for Detection of Liver Fibrosis in Children With Autosomal Recessive Polycystic Kidney Disease.

Front Pediatr 2018 11;6:422. Epub 2019 Jan 11.

Department of Gastroenterology, Hepatology, Feeding Disorders and Pediatrics, Children's Memorial Health Institute, Warsaw, Poland.

Congenital hepatic fibrosis (CHF) is invariably present in all patients with autosomal recessive polycystic kidney disease (ARPKD) but is usually clinically asymptomatic. The portal hypertension in the course of CHF develops and progresses over time, so an early detection of liver fibrosis remains crucial. The aim of the study was to evaluate a predictive value of transient elastography for evaluating liver disease progress in pediatric ARPKD patients. Read More

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http://dx.doi.org/10.3389/fped.2018.00422DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6336693PMC
January 2019
1 Read

Dysregulated TRAF3 and BCL2 Expression Promotes Multiple Classes of Mature Non-hodgkin B Cell Lymphoma in Mice.

Front Immunol 2018 11;9:3114. Epub 2019 Jan 11.

Instituto de Investigaciones Biomédicas "Alberto Sols", CSIC-UAM, Madrid, Spain.

TNF-Receptor Associated Factor (TRAF)-3 is a master regulator of B cell homeostasis and function. TRAF3 has been shown to bind and regulate various proteins involved in the control of innate and adaptive immune responses. Previous studies showed that TRAF3 overexpression renders B cells hyper-reactive to antigens and Toll-like receptor (TLR) agonists, while TRAF3 deficiency has been implicated in the development of a variety of B cell neoplasms. Read More

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http://dx.doi.org/10.3389/fimmu.2018.03114DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6338067PMC
January 2019
2 Reads

Septic Shock Predisposed by an Underlying Chronic Neutrophilic Leukemia with an Atypical Presentation; A Case Report.

Case Rep Oncol 2018 Sep-Dec;11(3):871-879. Epub 2018 Dec 20.

Department of Hematology & Oncology, University of Texas Medical Branch, Galveston, Texas, USA.

Background: Chronic neutrophilic leukemia (CNL) is an extremely rare myeloproliferative neoplasm (MPN). Due to the difficulty in its diagnosis, the diagnostic criterion was just recently revised in 2016. CNL is defined as: A clonal disorder with sustained primary neutrophilia, with normal neutrophil maturation, that does not meet other MPN criteria, as well as no identifiable mutations of the PDGFRA, PDGFRB or FGFR1 or PCM1-JAK2 genes, and, either, the presence of a CSF3R mutation, or if absent, the presence of sustained neutrophilia (> 3 months), splenomegaly and no other identifiable cause of reactive neutrophilia including the absence of a plasma cell neoplasm, or, if present, demonstration of myeloid cell clonality by cytogenetics. Read More

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http://dx.doi.org/10.1159/000495458DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6341362PMC
December 2018
2 Reads

Differential hepatic features presenting in Wilson disease-associated cirrhosis and hepatitis B-associated cirrhosis.

World J Gastroenterol 2019 Jan;25(3):378-387

Department of Gastroenterology, The First Affiliated Hospital of Guangdong Pharmaceutical University, Guangzhou 510000, Guangdong Province, China.

Background: Cirrhosis is a chronic late stage liver disease associated with hepatitis viruses, alcoholism, and metabolic disorders, such as Wilson disease (WD). There are no clear markers or clinical features that define cirrhosis originating from these disparate origins. We hypothesized that cirrhosis is not one disease and cirrhosis of different etiology may have differential clinical hepatic features. Read More

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https://www.wjgnet.com/1007-9327/full/v25/i3/378.htm
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http://dx.doi.org/10.3748/wjg.v25.i3.378DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6343092PMC
January 2019
2 Reads

PLD4 is a genetic determinant to systemic lupus erythematosus and involved in murine autoimmune phenotypes.

Ann Rheum Dis 2019 Jan 24. Epub 2019 Jan 24.

Department of Rheumatology and Clinical Immunology, Graduate School of Medicine, Kyoto University, Kyoto, Japan

Objectives: Systemic lupus erythematosus (SLE) is an autoimmune disease that is characterised by autoantibody production and widespread inflammation damaging many organs. Previous genome-wide association studies (GWASs) have revealed over 80 genetic determinants of SLE, but they collectively explain a fraction of the heritability, and only a few were proven in vivo for the involvement in SLE. We conducted a meta-analysis of SLE GWAS in the Japanese population, followed by functional analyses of a susceptibility gene with use of mutant mice. Read More

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http://dx.doi.org/10.1136/annrheumdis-2018-214116DOI Listing
January 2019
2 Reads

CRISPR/Cas9-mediated knockout of causes hemolytic anemia with splenomegaly in C57BL/6 mice.

Lab Anim Res 2018 Dec 31;34(4):302-310. Epub 2018 Dec 31.

Graduate School of Translational Medicine, Seoul National University College of Medicine, Seoul, Korea.

CD47 (integrin-associated protein), a multi-spanning transmembrane protein expressed in all cells including red blood cells (RBCs) and leukocytes, interacts with signal regulatory protein α (SIRPα) on macrophages and thereby inhibits phagocytosis of RBCs. Recently, we generated a novel C57BL/6J knockout ( hereafter) mouse line by employing a CRISPR/Cas9 system at Center for Mouse Models of Human Disease, and here report their hematological phenotypes. On monitoring their birth and development, mice were born viable with a natural male-to-female sex ratio and normally developed from birth through puberty to adulthood without noticeable changes in growth, food/water intake compared to their age and sex-matched wild-type littermates up to 26 weeks. Read More

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http://dx.doi.org/10.5625/lar.2018.34.4.302DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6333621PMC
December 2018
1 Read

Role of mast cells in the generation of a T-helper type 2 dominated anti-helminthic immune response.

Biosci Rep 2019 Feb 15;39(2). Epub 2019 Feb 15.

Department of Pathology, College of Medicine, Ohio State University Wexner Medical Center, Columbus, OH, U.S.A.

Mast cells are long-lived, innate immune cells of the myeloid lineage which are found in peripheral tissues located throughout the body, and positioned at the interface between the host and the environment. Mast cells are found in high concentrations during helminth infection. Using mast cell deficient mice, a recently published study in by Gonzalez et al. Read More

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http://bioscirep.org/lookup/doi/10.1042/BSR20181771
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http://dx.doi.org/10.1042/BSR20181771DOI Listing
February 2019
4 Reads

Long non-coding RNAs MALAT1, MIAT and ANRIL gene expression profiles in beta-thalassemia patients: a cross-sectional analysis.

Hematology 2019 Dec;24(1):308-317

e Department of Biochemistry, Faculty of Medicine , Northern Border University , Arar , Saudi Arabia.

Objectives: Beta-thalassemia (β-thal) is one of the most common genetic disorders worldwide. Multiple genetic and epigenetic mechanisms could be implicated in the pathogenesis and/or phenotype variations. We sought to explore the serum expression profile of three disease-related long non-coding RNAs (lncRNAs) in a sample of Egyptian β-thal patients with correlation to the patients' clinicolaboratory data. Read More

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http://dx.doi.org/10.1080/16078454.2019.1570616DOI Listing
December 2019
3 Reads
1.189 Impact Factor

Surgical management of hereditary spherocytosis Current strategies.

Ann Ital Chir 2018 ;89:473-478

Background: Hereditary spherocytosis is a benign hematologic disease, which needs surgical treatment when medical therapy fails. Currently, the surgical strategies consist mainly in total or partial splenectomy, which can be performed either in open or in laparoscopic fashion. In this study, we analyzed our series of splenectomies for hereditary spherocytosis and we discuss about the surgical management, reviewing the Literature. Read More

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January 2018
15 Reads

Various initial presentations of Epstein-Barr virus infection-associated post-transplant lymphoproliferative disorder in pediatric liver transplantation recipients: Case series and literature review.

Pediatr Transplant 2019 Jan 20:e13357. Epub 2019 Jan 20.

Faculty of Medicine Ramathibodi Hospital, Department of Pediatrics, Division of Gastroenterology, Mahidol University, Bangkok, Thailand.

PTLD is a rare but potentially life-threatening condition, which shows a higher prevalence in children than in adults. From 129 children who underwent LT, we reported 5 cases with biopsy-proven PTLD at a single teaching hospital. Four patients had shared clinical presentations including fever, lymphadenopathy, and splenomegaly. Read More

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http://doi.wiley.com/10.1111/petr.13357
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http://dx.doi.org/10.1111/petr.13357DOI Listing
January 2019
6 Reads

Cold type autoimmune hemolytic anemia- a rare manifestation of infectious mononucleosis; serum ferritin as an important biomarker.

BMC Infect Dis 2019 Jan 18;19(1):68. Epub 2019 Jan 18.

Sri Jayawardanapura General Hospital, Thalapathpitiya, Nugegoda, Western province, Sri Lanka.

Background: Infectious mononucleosis is one of the main manifestations of Epstein - Barr virus, which is characterized by fever, tonsillar-pharyngitis, lymphadenopathy and atypical lymphocytes. Although 60% of patients with IMN develop cold type antibodies, clinically significant hemolytic anemia with a high ferritin level is very rare and validity of serum ferritin as an important biomarker has not been used frequently.

Case Presentation: 18-year-old girl presented with fever, malaise and sore throat with asymptomatic anemia, generalized lymphadenopathy, splenomegaly and mild hepatitis. Read More

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http://dx.doi.org/10.1186/s12879-019-3722-zDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6339345PMC
January 2019
3 Reads

Autoimmune hepatitis in 847 childhood-onset systemic lupus erythematosus population: a multicentric cohort study.

Adv Rheumatol 2018 Dec 13;58(1):43. Epub 2018 Dec 13.

Pediatric Rheumatology Unit, Children's Institute, Hospital das Clinicas HCFMUSP, Faculdade de Medicina, Universidade de Sao Paulo, Sao Paulo, SP, Brazil.

Objective: To evaluate autoimmune hepatitis (AIH) in a multicenter cohort of childhood-onset systemic lupus erythematosus (cSLE) patients.

Methods: This retrospective multicenter study included 847 patients with cSLE, performed in 10 Pediatric Rheumatology services of São Paulo state, Brazil. AIH was defined according to the International Autoimmune Hepatitis Group criteria (IAHGC). Read More

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http://dx.doi.org/10.1186/s42358-018-0043-7DOI Listing
December 2018
3 Reads

Canakinumab for the treatment of hyperimmunoglobulin D syndrome.

Expert Rev Clin Immunol 2019 Mar 26;15(3):215-220. Epub 2019 Jan 26.

b Pediatric Rheumatology Unit, Pediatrics Department, Hospital Sant Joan de Déu , Universitat de Barcelona , Barcelona , Spain.

Introduction: Mevalonate Kinase Deficiency (MKD) is a rare monogenic autoinflammatory disorder (AID) with autosomal recessive inheritance caused by mutations in the MVK gene. It includes hyperimmunoglobulinemia D syndrome (HIDS) and mevalonic aciduria (a severe form). Patients have recurrent inflammatory attacks with high fever, gastrointestinal symptoms, lymphadenopathy, splenomegaly, arthralgia, rash, pharyngitis, aphtosis and constitutional complaints. Read More

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https://www.tandfonline.com/doi/full/10.1080/1744666X.2019.1
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http://dx.doi.org/10.1080/1744666X.2019.1571410DOI Listing
March 2019
6 Reads

Splenectomy in patients older than 65 years - a single-center experience.

Rozhl Chir 2018 ;97(12):546-550

Introduction: Despite current efforts at the most conservative approach possible to splenic injury, splenectomy remains a relatively common surgical procedure. Indications for splenectomy, however, are not limited to spleen injury. In senior patients in particular, non-traumatic reasons for splenectomy becomemore frequent. Read More

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January 2018
1 Read

Prediction of the presence of esophageal varices using spleen stiffness measurement by transient elastography in cirrhotic patients.

Acta Gastroenterol Belg 2018 Oct-Dec;81(4):496-501

Hospital U. Ramón y Cajal, Carretera Colmenar Viejo km 9,1, Madrid, España.

Background And Study Aims: Upper gastrointestinal endoscopy (UGE) is currently recommended in cirrhotic patients to detect the presence of esophageal varices (EV). Spleen stiffness measurement (SSM) with FibroScan has been used for this purpose, showing variable sensitivity (S) and specificity (Sp). The aim of this study was to evaluate the capability of SSM to detect the presence and size of EV in cirrhotic patients in comparison to other noninvasive modalities. Read More

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January 2019
3 Reads

[Clinicopathological characteristics of adult T cell leukemia/lymphoma].

Zhonghua Bing Li Xue Za Zhi 2019 Jan;48(1):11-16

Department of Pathology, Fujian Cancer Hospital, Fujian Medical University Cancer Hospital, Fuzhou 350014, China.

To investigate the clinical presentation, clinicopathologic features, diagnosis and differential diagnosis of adult T cell leukemia/lymphoma (ATLL). Four cases of ATLL from Fujian Cancer Hospital between October 2017 and May 2018 were analyzed using hematoxylin-eosin and immunohistochemical stains and polymerase chain reaction (PCR) for HTLV-1 provirus genes. The relevant literature was reviewed. Read More

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http://dx.doi.org/10.3760/cma.j.issn.0529-5807.2019.01.003DOI Listing
January 2019
2 Reads

[Gaucher disease: A review].

Rev Med Interne 2019 Jan 10. Epub 2019 Jan 10.

Service de médecine interne, hôpitaux universitaires Paris Nord-Val-de-Seine, hôpital Beaujon, Assistance publique-Hôpitaux de Paris (AP-HP), 100, boulevard du Général-Leclerc, 92110 Clichy, France; Centre de référence des maladies lysosomales, hôpitaux universitaires Paris Nord-Val-de-Seine, hôpital Beaujon, Assistance publique-Hôpitaux de Paris (AP-HP), 100, boulevard du Général-Leclerc, 92110 Clichy, France.

Gaucher disease is a rare autosomal recessive genetic disease, caused by a deficiency of the lysosomal enzyme, glucocerebrosidase that leads to the accumulation of its substrate (glucosylceramide) in lysosomal macrophages. In the general population, its incidence varies between 0.4 and 5. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S02488663183118
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http://dx.doi.org/10.1016/j.revmed.2018.11.012DOI Listing
January 2019
7 Reads

Chronic active EBV infection associated with NK cell lymphoma and hemophagocytic lymphohistiocytosis in a 27-year-old woman: A case report.

Medicine (Baltimore) 2019 Jan;98(2):e14032

Department of Infectious Diseases, The Third Affiliated Hospital of Hebei Medical University, Shijiazhuang.

Rationale: Chronic active Epstein-Barr virus infection (CAEBV) is a common infectious disease that often affects multiple organs or systems. However, it is liable to be neglected and misdiagnosed owing to its insidious onset, lack of specific findings in the early phase, and a general lack of awareness among clinicians. PATIENT CONCERNS:: a 27-year-old woman case has been described who was initially misdiagnosed as drug-induced liver injury due to onset presentation of mild splenomegaly, recurrent liver dysfunction, and disputable pathological evidence of liver biopsy. Read More

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http://dx.doi.org/10.1097/MD.0000000000014032DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6336602PMC
January 2019
5 Reads
5.723 Impact Factor