19,334 results match your criteria Splenomegaly
Clin Imaging 2018 Nov 27;54:71-77. Epub 2018 Nov 27.
Department of Radiology, Division of Vascular and Interventional Radiology, Columbia University Medical Center, 177 Fort Washington Ave, Milstein Hospital - 4 Hudson North, New York, NY 10032, United States of America.
Purpose: To report the effect of partial splenic embolization (PSE) on hematological indices and the procedure's safety in pre- and post-liver transplant (LT) patients.
Materials And Methods: A single-center retrospective study evaluating all patients who underwent PSE over a 16-year period was performed. Inclusion criteria were splenomegaly confirmed by imaging and at least one of the following cytopenias: hemoglobin ≤10 g/dL, WBC count ≤1500 μL, or platelet count ≤100,000 μL. Read More
Klin Lab Diagn 2018 ;63(1):51-54
The state budget institution of health care "The clinical center of prevention and struggle with AIDS" of the Minzdrav of the Krasnodar kraii, 350015, Krasnodar, Russia.
The purpose of study is the clinical laboratory characteristic of acquired acute cytomegalovirus infection in nonimmunocompromised adult patients. The sampling included 75 patients with acquired acute cytomegalovirus infection admitted to the specialized clinical infection hospital of the Minzdrav of the Krasnodar kraii. The verifcation of diagnosis was implemented using serological tests and polymerase chain reaction. Read More
J Ultrasound Med 2018 Dec 14. Epub 2018 Dec 14.
Infectious Diseases, Federal University of Triângulo Mineiro, Uberaba, Brazil.
Objectives: To evaluate whether abdominal ultrasound (US) with a gallbladder (GB) contractility study or motor function test can be used as a diagnostic tool in patients with dengue and warning signs in acute and recovery phases.
Methods: Fifty-one individuals in the acute phase of dengue presenting with warning signs (dengue group) and 49 healthy individuals without a history of dengue or hepatobiliary disease (control group) were studied with abdominal US and a GB contractility study.
Results: Statistical differences in US measurements of the liver (right lobe, P = . Read More
Scand J Immunol 2018 Dec 13:e12742. Epub 2018 Dec 13.
Pharmicell Co, Ltd, 484, Dunchon-daero, Jungwon-gu, Seongnam, Gyeonggi-do, Korea.
Myeloid-derived suppressor cells (MDSCs) play an important role in controlling the immune response against cancer and in suppression of autoimmunity and allergic inflammation. However, the beneficial effects of MDSCs on the experimental mouse model of psoriasis have not been reported. Therefore, we investigated the anti-psoriatic effect of MDSCs on IMQ-induced skin inflammation in mice and explored the mechanisms involved. Read More
J Card Surg 2018 Dec 12. Epub 2018 Dec 12.
Department of Surgical Intensive Care Unit, The First Affiliated Hospital of Zhejiang University School of Medicine, Hangzhou, China.
Cancer Sci 2018 Dec 12. Epub 2018 Dec 12.
United Graduate School of Drug Discovery and Medical Information Sciences, Gifu University, 1-1 Yanagido, Gifu, 501-1194, Japan.
Therapy based on targeted inhibition of BCR-ABL tyrosine kinase has greatly improved the prognosis for patients with Philadelphia chromosome (Ph)-positive leukemia and tyrosine kinase inhibitors (TKIs) have become the standard therapy. However, some patients acquire resistance to TKIs that is frequently associated with point mutations in the BCR-ABL. We previously reported that a medium-chain fatty-acid derivative AIC-47 induced transcriptional suppression of BCR-ABL and perturbation of the Warburg effect, leading to growth inhibition in Ph-positive leukemia cells. Read More
J Natl Compr Canc Netw 2018 Dec;16(12):1539-1545
Department of Medicine, and Division of Hematology and Oncology, Northwestern University Feinberg School of Medicine; and Robert H. Lurie Comprehensive Cancer Center of Northwestern University, Chicago, Illinois.
Polycythemia vera (PV) and essential thrombocythemia (ET) are Philadelphia chromosome-negative chronic myeloproliferative neoplasms (MPNs), characterized by expansion of normal blood counts, bleeding, thrombosis, and the potential for transformation to myelofibrosis (MF) or acute myeloid leukemia (AML). The primary goals of treatment for MPNs are to reduce the risk of thrombosis, alleviate systemic symptom burden (eg, fatigue, pruritus, microvascular symptoms, and symptomatic splenomegaly), and to prevent transformation to MF/AML. Preventing transformation is clearly important, but not expected with current therapies. Read More
MMWR Morb Mortal Wkly Rep 2018 12 14;67(49):1358-1362. Epub 2018 Dec 14.
In 2014, panel physicians from the International Organization for Migration (IOM), who conduct Department of State-required predeparture examinations for U.S.-bound refugees at resettlement sites in Uganda, noticed an unusually high number of Congolese refugees with enlarged spleens, or splenomegaly. Read More
Zhonghua Lao Dong Wei Sheng Zhi Ye Bing Za Zhi 2018 Oct;36(10):755-758
HulunBuir People's Hospital, Department of Brucellosis, HulunBuir Clinical Medical College, Inner Mongolia University for Nationalitie, HulunBuir 021008, China.
To analyse epidemiological, clinical characteristics and laboratory examination results of 245 occupational brucellosis form 2008 to 2018, which providing theoretical basis for prevention and control of occupational brucellosis. Based on the China Center for Disease Control and Prevention Information System, a database of occupational brucellosis cases in HunlunBuir from January 2008 to July 2018 was established. The Epidemiological characteristics, Clinical manifestation, laboratory examination of 245 occupational brucellosis and 359 without occupational brucellosis were comparatively analyzed about the same period. Read More
Wideochir Inne Tech Maloinwazyjne 2018 Dec 24;13(4):454-459. Epub 2018 Jul 24.
Department of Hepatobiliary Pancreatic Surgery, People's Hospital of Zhengzhou University, Henan Provincial People's Hospital, Zhengzhou, China.
Introduction: Laparoscopic splenectomy for massive splenomegaly secondary to liver cirrhosis and portal hypertension in patients with an extremely low platelet count (< 1 × 10/l) presents several challenges. The posterolateral laparoscopic splenectomy approach may be a feasible and safe technique for these patients.
Aim: To evaluate the feasibility and safety of the posterolateral laparoscopic splenectomy approach in patients with platelet counts < 1 × 10/l secondary to liver cirrhosis and portal hypertension. Read More
Rev Gastroenterol Peru 2018 Jul-Sep;38(3):280-284
Facultad de Medicina Humana, Universidad Nacional San Luis Gonzaga. Ica, Perú; Sociedad Científica de Estudiantes de Medicina de Ica (SOCEMI). Ica, Perú.
Gaucher disease is an autosomal recessive lysosomal storage disorder characterized by deficiency of beta-glucosidase that would lead to the accumulation of glucosylceramide mainly in cells of the mononuclear phagocytic system causing systemic effectations. We present a patient of twenty years who is suffering from chronic pain in the left hypochondrium with episodes of bleeding for 3 years and sensation of thermal rise, physical examination revealed jaundice and massive splenomegaly, without neurological involvement. Severe osteoporosis, pancytopenia, and the presence of portal vein thrombosis with cavernomatous transformation complicated by portal biliopathy simulating a klatskin tumor, marrow and enzymatic studies were compatible with Gaucher disease, were shown as unexpected findings. Read More
Case Rep Med 2018 5;2018:4628439. Epub 2018 Nov 5.
Texas A&M University, College Station, TX, USA.
Sarcoidosis is a systemic granulomatous disease of unknown etiology characterized by presence of noncaseating granulomas in the involved organs. The pulmonary interstitium is most commonly affected but extrapulmonary involvement can occur in almost any other organ system. Such an involvement can occur with or without the presence of pulmonary involvement, but isolated extrapulmonary involvement has been noted only in around 10% of cases. Read More
Am J Physiol Gastrointest Liver Physiol 2018 Dec 6. Epub 2018 Dec 6.
Murdoch Childrens Research Institute, Australia.
Expression of the cytokine IL-11 is elevated in human Helicobacter pylori infection and progressively increases with worsening gastric pathology. Additionally, IL-11 is required for tumour development in STAT3 dependent murine models of gastric cancer (GC) and, when administered acutely, causes resolving atrophic gastritis. However, it is unclear whether locally elevated IL-11 ligand expression can, in isolation from oncogenic gp130-JAK-STAT pathway mutations, initiate GC pathogenesis. Read More
BMC Complement Altern Med 2018 Dec 5;18(1):320. Epub 2018 Dec 5.
Herbal Medicine Research Center, Institute for Medical Research, Kuala Lumpur, Malaysia.
Background: Carica papaya leaf juice (CPLJ) was well known for its thrombocytosis activity in rodents and dengue patients. However, the effect of CPLJ treatment on other parameters that could contribute to dengue pathogenesis such as nonstructural protein 1 (NS1) production and viremia level have never been highlighted in any clinical and in vivo studies. The aim of this study is to investigate the effect of freeze-dried CPLJ treatment on NS1 and viremia levels of dengue fever mouse model. Read More
Comput Biol Chem 2018 Oct 29;78:133-143. Epub 2018 Oct 29.
Tianjin Key Laboratory on Technologies Enabling Development of Clinical Therapeutics and Diagnostics (Theranostics), School of Pharmacy, Tianjin Medical University, Tianjin, 300070, China. Electronic address:
Juvenile myelomonocytic leukaemia, an aggressive myeloproliferative neoplasm, is characterized by thrombocytopenia, splenomegaly, fever and excess myelomonocytic cells. Approximately 35% of patients with JMML occur D61Y mutation in PTPN11, and it increases the activity of the protein. However, the effect of the D61Y mutation on SHP2 conformations in molecular basis is poorly understood. Read More
Ann Med Surg (Lond) 2018 Dec 16;36:199-202. Epub 2018 Nov 16.
Tecnologico de Monterrey, Escuela de Medicina y Ciencias de la Salud, Dr. Ignacio Morones Prieto O 3000, Monterrey, 64710, Mexico.
Introduction: Splenic hamartoma is a primary benign tumor of the spleen, with approximately 150 cases documented in the literature to date, with only a few cases associated with symptoms and hematologic disorders.
Presentation Of Case: A 49-year-old female with no past medical history, presented to the emergency department complaining of a three-month history of intermittent abdominal pain and 12 kg of weight loss. Physical examination revealed abdominal distension and a big palpable and painless mass on the left side of her abdomen measuring 14 cm. Read More
Can J Gastroenterol Hepatol 2018 1;2018:1275720. Epub 2018 Nov 1.
Department of General, Visceral, and Transplantation Surgery, University of Heidelberg, Heidelberg, Germany.
Background: It is a novel idea that platelet counts may be associated with postoperative outcome following liver surgery. This may help in planning an extended hepatectomy (EH), which is a surgical procedure with high morbidity and mortality.
Aim: The aim of this study was to evaluate the predictive potential of platelet counts on the outcome of EH in patients without portal hypertension, splenomegaly, or cirrhosis. Read More
Turk J Pediatr 2018 ;60(3):264-269
Department of Pediatric Hematology, University of Health Sciences, Ankara Child Health and Diseases Hematology Oncology Training and Research Hospital, Ankara, Turkey.
Güngör A, Yaralı N, Fettah A, Ok-Bozkaya İ, Özbek N, Kara A. Hereditary spherocytosis: Retrospective evaluation of 65 children. Turk J Pediatr 2018; 60: 264-269. Read More
Caspian J Intern Med 2018 ;9(4):334-340
Department of General Medicine, Yashoda Hospital, Hyderabad, India.
Background: Dengue is endemic and prevalent in tropical and sub-tropical countries including India and can cause significant mortality and morbidity. There are limited studies available on factors associated with severe dengue from India, to investigate the predictors of severe dengue in south Indian patients.
Methods: We recruited 334 patients with dengue admitted in Yashoda Hospital, Hyderabad. Read More
Pol J Pathol 2018 ;69(3):314-318
A 56-year-old female presented with popliteal venous thrombosis, splenomegaly, and sclerotic bone lesions. Bone marrow biopsy showed fibrosis, proliferation of abnormal blood vessels and lymphatics, bone remodelling, and no significant changes in haematopoietic elements. Following a relatively indolent initial clinical course, one year later she rapidly deteriorated and died of respiratory failure associated with widespread disease. Read More
Hemoglobin 2018 Nov 29:1-7. Epub 2018 Nov 29.
e Department of Haematogenetics , National Institute of Immuno-Haematology , Parel, Mumbai , India.
Hemoglobinopathies and thalassemias are the most commonly encountered monogenic disorders of blood in humans, posing a major genetic and public health problem round the globe. Hb S (HBB: c.20A>T)-β-thalassemia (β-thal) is a compound aberrant heterozygosity with inconsistent phenotypic expression, which are poorly described and clinically mapped. Read More
Endocrinol Diabetes Metab Case Rep 2018 Nov 22;2018. Epub 2018 Nov 22.
Endocrinology, Diabetes and Metabolism Department, Centro Hospitalar e Universitario de Coimbra EPE, Coimbra, Portugal.
Addison's disease (AD) is the most common endocrine manifestation of antiphospholipid syndrome (APS), but it remains a very rare complication of the syndrome. It is caused by adrenal venous thrombosis and consequent hemorrhagic infarction or by spontaneous (without thrombosis) adrenal hemorrhage, usually occurring after surgery or anticoagulant therapy. We present a clinical case of a 36-year-old female patient with a previous diagnosis of APS. Read More
J Gynecol Obstet Hum Reprod 2018 Nov 19. Epub 2018 Nov 19.
Fetal Medicine unit, Université Claude Bernard, Lyon 1, Hospices Civils de Lyon, Hôpital Femme Mère Enfant, 59 boulevard Pinel, 69500, Bron, Lyon, France. Electronic address:
We report prenatal imaging features of four cases of neonatal hemochromatosis due to an alloimmune disease. All cases exhibited intra uterine growth restriction (IUGR) without arguments for a vascular etiology, associated with oligohydramnios. Placental hydrops was present in 75% of cases. Read More
J Gastroenterol Hepatol 2018 Nov 21. Epub 2018 Nov 21.
Department of General Surgery, China-Japan Friendship Hospital, Beijing, China.
Background And Aim: Idiopathic portal hypertension (IPH) refers to a relatively rare condition characterized by intrahepatic portal hypertension in the absence of underlying disease such as liver cirrhosis.
Methods: We retrospectively reviewed 338 patients with IPH that were diagnosed at the pathological consultation center of our hospital.
Results: The ratio of male to female patients was 1:1. Read More
Turk J Gastroenterol 2018 Nov 16. Epub 2018 Nov 16.
Department of Medical Genetics, Marmara University School of Medicine, İstanbul, Turkey.
Chanarin Dorfman syndrome is a multisystem, very rare, autosomal recessive lipid storage disorder, characterized by the accumulation of lipid vacuoles in neutrophils, and was first described by Dorfman in 1974. Due to a mutation in the ABHD5 gene of the short arm of chromosome 3, lipid is stored in the granulocytes at various sites in the human body, such as the muscle, liver, eye, ear, central nervous system, and bone marrow. Clinically, the disease is presented with ichthyosis, hearing loss, hepatomegaly, splenomegaly, cirrhosis, cataract, keratopathy, myopathy, and mental retardation. Read More
Parasitol Res 2018 Nov 19. Epub 2018 Nov 19.
Departamento de Patologia Clínica Veterinária, Setor de Patologia Veterinária, Faculdade de Veterinária, Universidade Federal do Rio Grande do Sul (UFRGS), Av. Bento Gonçalves 9090, Prédio 42505, Porto Alegre, RS, 91540-000, Brazil.
Avian malaria is a mosquito-borne disease that affects multiple avian species and is caused by protozoans of the genus Plasmodium. An avian malaria infection caused by Plasmodium sp. in Magellanic penguins (Spheniscus magellanicus) with high mortality is described in a zoo in Southern Brazil. Read More
Respir Med Case Rep 2019 4;26:11-13. Epub 2018 Nov 4.
Internal Medicine Department, Hurley Medical Center/ Michigan State University, Flint, MI, USA.
A 61-year-old male presented with worsening dyspnoea and constitutional symptoms for few weeks followed by bloody diarrhoea and loss and fever. Physical exam revealed tachycardia, respiratory distress, and splenomegaly without lymphadenopathy. Work up showed pancytopenia, hypofibrinogenemia, acute kidney injury requiring haemodialysis, high ferritin level, positive IgG and IgM for EBV and positive soluble CD25. Read More
Contemp Oncol (Pozn) 2018 30;22(3):178-183. Epub 2018 Sep 30.
Department of Radiology, Acibadem Adana Hospital, Acibadem University, Adana, Turkey.
Aim Of The Study: To evaluate computed tomography (CT) findings of gastrointestinal graft-versus-host disease (GI-GVHD) occurring in children after haematopoietic stem-cell transplantation (HSCT).
Material And Methods: From February 2013 to May 2018, 225 paediatric patients underwent HSCT. Sixty-eight patients (30%) presented with clinical diagnosis of acute GI-GVHD in the first 100 days after HSCT. Read More
Am Surg 2018 Jun;84(6):e201-e203
Curr Opin Hematol 2019 Jan;26(1):16-21
Department of Pediatrics, Glycogen Storage Disease Program, Connecticut Children's Medical Center, Hartford, Connecticut, USA.
Purpose Of Review: Glycogen storage disease Ib (GSD Ib) is characterized by hepatomegaly, hypoglycemia, neutropenia, enterocolitis and recurrent bacterial infections. It is attributable to mutations in G6PT1, the gene for the glucose-6-phosphate transporter responsible for transport of glucose into the endoplasmic reticulum. Neutropenia in GSD Ib is now frequently treated with granulocyte colony-stimulating factor (G-CSF). Read More
Vaccine 2018 Dec 16;36(52):8148-8157. Epub 2018 Nov 16.
Department of Immunology, College of Basic Medical Sciences, Jilin University, Changchun, Jilin, China. Electronic address:
The adjuvant effects of flagellin on regulation of immune response have been proved; whether flagellin could assist tumor cell lysate (TCL) to enhance anti-glioma immunity remains to be investigated. This study tests a hypothesis that therapeuticly intracranial administration with flagellin plus TCL enhances the effects of specific immunotherapy on glioma in mice. In this study, GL261 cells were transferred into C57BL/6 mice and the GL261-bearing mice were subcutaneously or intracranially inoculated with flagellin plus TCL, flagellin, TCL or saline. Read More
J Ultrasound 2018 Nov 16. Epub 2018 Nov 16.
Paediatric Radiology Department, "Federico II" University Hospital, Naples, Italy.
In infants and children, the spleen is involved in many pathological processes, whether those processes are isolated or related to systemic diseases. Pathology of the pediatric spleen includes congenital anomalies, splenomegaly, trauma, focal lesions, infarction, and tumors. Ultrasonography (US) is a widely available, fast, noninvasive imaging technique to assess the size, shape, and position of the spleen, as well as to define splenic echotexture. Read More
Proc Natl Acad Sci U S A 2018 Dec 15;115(49):E11523-E11531. Epub 2018 Nov 15.
Center for the Genetics of Host Defense, University of Texas Southwestern Medical Center, Dallas, TX 75390-8505;
The SMCR8-WDR41-C9ORF72 complex is a regulator of autophagy and lysosomal function. Autoimmunity and inflammatory disease have been ascribed to loss-of-function mutations of or in mice. In humans, autoimmunity has been reported to precede amyotrophic lateral sclerosis caused by mutations of However, the cellular and molecular mechanisms underlying autoimmunity and inflammation caused by C9ORF72 or SMCR8 deficiencies remain unknown. Read More
IEEE Trans Med Imaging 2018 Nov 13. Epub 2018 Nov 13.
The findings of splenomegaly, abnormal enlargement of the spleen, is a non-invasive clinical biomarker for liver and spleen disease. Automated segmentation methods are essential to efficiently quantify splenomegaly from clinically acquired abdominal magnetic resonance imaging (MRI) scans. However, the task is challenging due to (1) large anatomical and spatial variations of splenomegaly, (2) large inter- and intra-scan intensity variations on multi-modal MRI, and (3) limited numbers of labeled splenomegaly scans. Read More
Acta Clin Croat 2018 Jun;57(2):362-365
Department of Hematology, Sestre milosrdnice University Hospital Centre, Zagreb, Croatia.
T-cell large granular lymphocytic leukemia (T-LGLL) is an uncommon but probably underdiagnosed disease caused by clonal proliferation of large granular lymphocytes. Diagnosis is typically based on the high number of morphologically characteristic lymphoid cells and finding of an abnormal immunophenotype by flow cytometry. Because of its relatively indolent clinical behavior, observation is often an appropriate therapy. Read More
Medicine (Baltimore) 2018 Nov;97(46):e13220
Department of Hematology, Union Hospital, Fujian Medical University, Fujian Institute of Hematology, Fujian Provincial Key Laboratory of Hematology, Fuzhou, China.
Rationale: The risk of leukemic transformation in myeloproliferative neoplasm (MPN) has been increasing with time. Partial Tandem Duplications of the MLL gene (MLL-PTD) has been reported in de novo acute myeloid leukemia (AML), but not in MPN blast phase. The post-MPN AML developed adverse clinical outcomes, which showed no noticeable improvement over the past 15 years. Read More
Tunis Med 2018 Jul;96(7):458-461
Hemophagocyticlymphohistocytosis (HLH) is a proliferation of histiocytes with importanthemophagocytosisoccurring in different organs such as the spleen and the bone marrow. HLH is now increasingly diagnosed in the context of infections, malignancies and connective tissue diseases. Although brucellosis is an endemic infection in Tunisia, its association with HLH is a very rare condition which should be considered in patients with splenomegaly and cytopenia. Read More
World J Clin Cases 2018 Nov;6(13):688-693
Department of Rheumatology and Immunology, The First Affiliated Hospital of Chengdu Medical College, Chengdu 610000, Sichuan Province, China.
A 48 year-old Chinese woman suffering from polyarthritis, irregular fever and trichomadesis was admitted to the hospital. A diagnosis of systemic lupus erythematosus (SLE) was made based on polyarthritis, pancytopenia, reduced complement 3, multiple positive autoantibodies, a positive Coomb's test and protein in her urine. In addition, splenomegaly was detected during physical examination and confirmed by abdominal ultrasonography and magnetic resonance imaging, indicating that the patient had SLE and portal hypertension. Read More
Digestion 2018 Nov 13:1-6. Epub 2018 Nov 13.
Interdisziplinäre Ultraschallzentrum, Universitätsklinikum Gießen und Marburg, Philipps Universität Marburg, Marburg, Germany.
Purpose: In contrast to splenomegaly, the clinical value of a small spleen (hyposplenia) is unclear. Contrast-enhanced ultrasound (CEUS) has not been investigated systematically in hyposplenia.
Methods: Between February 2005 and January 2017, 43 patients with hyposplenia (< 7 × 3 cm) were examined by B-mode ultrasound (US) and CEUS. Read More
QJM 2018 Nov 13. Epub 2018 Nov 13.
Department of Medicine, All India Institute of Medical Sciences, New Delhi. Address: Department of Medicine, 3rd floor teaching block, AIIMS, New Delhi-29.
Wilson's Disease is an inborn error of metabolism associated with accumulation of copper in the body manifesting as hepatic, neurological and behavioural symptoms. Almost 20% patients initially present with behavioural symptoms such as depression, personality changes or even mania. Schizophrenia and catatonia are less common. Read More
Curr Pediatr Rev 2018 Nov 12. Epub 2018 Nov 12.
Division of Pediatric Gastroenterology, Hepatology and Nutrition MUSC Children's Hospital, South Carolina. United States.
IMPORTANCE Alpha1-antitrypsin (AAT) deficiency is a common, but underdiagnosed genetic condition, affecting 1 in 1500 individuals, which can present insidiously with liver disease in children. Although clinical practice guidelines exist for the management of AAT deficiency, especially with regards to pulmonary involvement, there are no published recommendations that specifically relate to the management of the liver disease and monitoring for lung disease associated with this condition, particularly in children. OBJECTIVE To review the literature on the management of AAT deficiency-associated liver disease in adults and children. Read More
J Obstet Gynaecol India 2018 Dec 5;68(6):447-451. Epub 2017 Jun 5.
Department of Obstetric Medicine, Fernandez Hospital, Unit 2, 3-6-282, opp: Old MLA Quarters, Hyderguda, Hyderabad, Telangana 500029 India.
Background: The course of pregnancy in a woman with portal hypertension is a difficult one as it is associated with complications like variceal bleeding, splenic artery rupture and coagulopathy. All these pose a threat to a woman's life. Although this condition is rare, every obstetrician should have a high index of suspicion when an antenatal mother presents with splenomegaly, thrombocytopenia or hematemesis. Read More
Intern Med J 2018 Nov 10. Epub 2018 Nov 10.
Shaare Zedek Medical Center and Hadassah Medical School, Jerusalem, Israel.
Background: Gaucher disease (GD) presents with a range of signs and symptoms. Physicians can fail to recognize the early stages of GD owing to a lack of disease awareness, which can lead to significant diagnostic delays and sometimes irreversible but avoidable morbidities.
Aims: The Gaucher Earlier Diagnosis Consensus (GED-C) initiative aimed to identify signs and co-variables considered most indicative of early type 1 and type 3 GD, to help non-specialists identify 'at-risk' patients who may benefit from diagnostic testing. Read More
PLoS One 2018 7;13(11):e0206947. Epub 2018 Nov 7.
Public Health Bureau, Tainan City Government, Tainan, Taiwan.
Background And Aim: The role of non-invasive methods to evaluate fibrosis severity of chronic hepatitis C (CHC) subjects in community needs to be explored. This study investigated FIB-4 and transient elastography (TE) in staging liver fibrosis of CHC subjects in community.
Methods: A total of 905 subjects who were positive for anti-HCV antibody from five districts of Tainan City of Taiwan were invited to participate in surveillance activities for CHC. Read More
Transplant Proc 2018 Nov 15;50(9):2718-2722. Epub 2018 Mar 15.
Department of Transplant Surgery, Jichi Medical University, Tochigi, Japan.
Purpose: After undergoing the Kasai procedure for biliary atresia (BA), most patients develop severe splenomegaly that tends to be improved by liver transplantation. However, fluctuations in splenic volume long after transplantation remain to be elucidated.
Patients And Methods: Seventy-one consecutive patients who had undergone pediatric living donor liver transplantation (LDLT) for BA were followed up in our outpatient clinic for 5 years. Read More
Transplant Proc 2018 Nov 19;50(9):2611-2613. Epub 2018 Mar 19.
Department of Surgery, Tohoku University Graduate School of Medicine, Sendai, Japan.
Objectives: In adults undergoing living donor liver transplantation (LDLT), the transplanted livers are partial grafts, and the portal venous pressure is higher than that observed with whole liver grafts. In patients undergoing LDLT concomitant with splenomegaly, portal venous flow is often diverted to collateral vessels, leading to a high risk of portal vein thrombosis. In such cases, occlusion of the collateral veins is important; however, complete occlusion of all collaterals without blocking the blood flow through the splenic artery causes portal hypertension and liver failure. Read More
Eur J Radiol 2018 Nov 19;108:133-139. Epub 2018 Sep 19.
Radiology Department, Angers University Hospital, Angers, France; HIFIH Laboratory, UPRES 3859, SFR 4208, Bretagne Loire University, Angers, France.
Objectives: Advanced chronic liver disease is frequent yet largely underdiagnosed. Doppler-US is a common examination and we recently identified three simple Doppler-US signs associated with severe liver fibrosis. Recent Doppler-US devices include elastography modules, allowing for liver stiffness measurement (LSM). Read More
Zhonghua Nei Ke Za Zhi 2018 Nov;57(11):811-815
Department of General Internal Medicine, Peking Union Medical College Hospital, Peking Union Medical College, Chinese Academy of Medical Sciences, Beijing 100730, China.
To investigate the clinical features of adult-onset chronic active Epstein-Barr virus infection (CAEBV). A total of 21 adult patients with CAEBV who were admitted to the department of General Internal Medicine at Peking Union Medical College Hospital from January 2006 to January 2016 were retrospectively analyzed. Demographic data, disease duration, clinical manifestations, laboratory findings, treatments and prognosis were reviewed. Read More
Pediatrics 2018 Nov;142(5)
Departments of Pediatric Rheumatology.
Deficiency of adenosine deaminase 2 (DADA2) is a rare autoinflammatory disease that was firstly described in patients with early-onset strokes, livedo reticularis, and periodic fever resembling polyarteritis nodosa. In reported case series, researchers described highly variable manifestations, including autoimmunity, immunodeficiency, hepatosplenomegaly, pancytopenia, ichthyosiform rash, and arthritis, in patients with DADA2. A thirteen-year-old female patient who was born to consanguineous parents was admitted to our hospital with generalized edema and leg pain. Read More
Blood Adv 2018 Nov;2(21):2814-2828
Cancer Research Center (IBMCC, USAL-CSIC), Department of Medicine and Cytometry Service (NUCLEUS), CIBERONC, University of Salamanca, Salamanca, Spain.
Systemic mastocytosis (SM) is a highly heterogeneous disease with indolent and aggressive forms, with the mechanisms leading to malignant transformation still remaining to be elucidated. Here, we investigated the presence and frequency of genetic variants in 34 SM patients with multilineal D816V mutations. Initial screening was performed by targeted sequencing of 410 genes in DNA extracted from purified bone marrow cells and hair from 12 patients with nonadvanced SM and 8 patients with advanced SM, followed by whole-genome sequencing (WGS) in 4 cases. Read More