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https://www.readbyqxmd.com/read/28631052/anatomic-variations-of-the-spleen-current-state-of-terminology-classification-and-embryological-background
#1
REVIEW
Ivan Varga, Jozef Babala, David Kachlik
A thorough understanding of the anatomy, physiology, and development of the spleen is essential for determining the pathophysiological mechanisms underpinning splenic diseases and congenital variations. The aim of this review is to briefly summarize current knowledge regarding the normal development of the spleen, and to provide an overview of clinically relevant congenital splenic variations. These include such variations as asplenia, polysplenia, hyposplenia, lobulation of spleen, accessory spleens, accessory splenic nodules, wandering spleen, splenogonadal and splenopancreatic fusion, splenic cysts, and cavernous haemangioma of the spleen...
June 19, 2017: Surgical and Radiologic Anatomy: SRA
https://www.readbyqxmd.com/read/28624464/complex-phenotypes-associated-with-stim1-mutations-in-both-coiled-coil-and-ef-hand-domains
#2
Elizabeth Harris, Umar Burki, Chiara Marini-Bettolo, Marcella Neri, Chiara Scotton, Judith Hudson, Marta Bertoli, Teresinha Evangelista, Bas Vroling, Tuomo Polvikoski, Mark Roberts, Ana Töpf, Kate Bushby, Daniel McArthur, Hanns Lochmüller, Alessandra Ferlini, Volker Straub, Rita Barresi
Dominant mutations in STIM1 are a cause of three allelic conditions: tubular aggregate myopathy, Stormorken syndrome (a complex phenotype including myopathy, hyposplenism, hypocalcaemia and bleeding diathesis), and a platelet dysfunction disorder, York platelet syndrome. Previous reports have suggested a genotype-phenotype correlation with mutations in the N-terminal EF-hand domain associated with tubular aggregate myopathy, and a common mutation at p.R304W in a coiled coil domain associated with Stormorken syndrome...
May 4, 2017: Neuromuscular Disorders: NMD
https://www.readbyqxmd.com/read/28473921/incidentally-detected-asplenia-in-a-healthy-64-year-old-female-live-kidney-donor
#3
Leoniek D Wolff, Mijntje N Nijboer, Jacqueline Vd Wetering, Frank J M F Dor
Heterotaxia syndromes are rare birth defects which can result in developmental malformations. A 64-year-old woman presented to the hospital for preoperative screening for kidney donation; during which she was found to have no gallbladder and no spleen, without any signs of surgical removal. This could be a new description of a heterotaxia syndrome.
April 2017: Oxford Medical Case Reports
https://www.readbyqxmd.com/read/28422834/feasibility-and-safety-of-emergency-laparoscopic-partial-splenectomy-a-retrospective-analysis
#4
Hongyu Li, Yonggang Wei, Bing Peng, Bo Li, Fei Liu
The increased awareness of asplenia-related life-threatening complications has led to the development of parenchyma sparing splenic resections in past few years. The aim of this study is to retrospectively analyze the feasibility and safety of laparoscopic partial splenectomy (LPS) in selected emergency patients.From January 2013 to December 2015, there were 46 emergency patients, diagnosed with splenic rupture, admitted in our department. Selection criteria for LPS: (1) Preoperative CT scan revealed single pole rupture without spleen pedicle injury; (2) BP>90/60 mm Hg and heart rates <120 bpm; (3) No sigh of multiple organ injury...
April 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28410813/post-hoc-analysis-of-a-randomized-controlled-trial-diabetes-mellitus-modifies-the-efficacy-of-the-13-valent-pneumococcal-conjugate-vaccine-in-elderly
#5
Susanne M Huijts, Cornelis H van Werkhoven, Marieke Bolkenbaas, Diederick E Grobbee, Marc J M Bonten
BACKGROUND: The 13-valent pneumococcal conjugate-vaccine (PCV13) was effective in preventing vaccine-type Community-Acquired Pneumonia (VT-CAP) and Invasive Pneumococcal Disease (VT-IPD) in elderly subjects, but vaccine efficacy (VE) in patients with comorbidities at time of vaccination is unknown. METHODS: This is a post hoc analysis of the CAPiTA study, a double blind, randomized controlled trial with 84,496 immunocompetent participants aged ⩾65years, receiving PCV13 or placebo vaccination...
April 12, 2017: Vaccine
https://www.readbyqxmd.com/read/28329282/possible-transfusion-transmitted-babesia-divergens-like-mo-1-infection-in-an-arkansas-patient
#6
Mary J Burgess, Eric R Rosenbaum, Bobbi S Pritt, Dirk T Haselow, Katie M Ferren, Bashar N Alzghoul, Juan Carlos Rico, Lynne M Sloan, Poornima Ramanan, Raghunandan Purushothaman, Robert W Bradsher
A patient with asplenia and multiple red blood cell transfusions acquired babesiosis infection with Babesia divergens-like/MO-1 organisms and not Babesia microti, the common United States species. He had no known tick exposure. This is believed to be the first transfusion-transmitted case and the fifth documented case of B. divergens-like/MO-1 infection.
June 1, 2017: Clinical Infectious Diseases: An Official Publication of the Infectious Diseases Society of America
https://www.readbyqxmd.com/read/28213046/splenectomy-as-a-destination-improving-quality-of-care-among-asplenic-veterans-through-a-travel-clinic
#7
Aaron P Mitchell, Joel C Boggan, Karen Lau, David L Simel
BACKGROUND: Asplenic patients are at risk for severe infections, but adherence to recommended preventive education and vaccination is poor. The goal of this study was to demonstrate that a targeted intervention can improve vaccination rates in a population of asplenic veterans. METHODS: Surgically asplenic patients actively receiving care in our health care system were identified via a database search. Patients were contacted via mailed letters and encouraged to attend an existing travel clinic with a new process designed for asplenic patients...
February 14, 2017: American Journal of Medicine
https://www.readbyqxmd.com/read/28069783/secondary-asplenia-as-a-complication-of-severe-meningococcal-sepsis
#8
Lourdes Castillo-Campos, Luis Bachiller-Carnicero, María José Megía-Sevilla, María Isabel González-Tomé
We report the case of an 11-month-old infant who had a medical history of meningococcal septicaemia 6 months prior to this admission and presented to hospital with an episode of fever due to Streptococcus pneumoniae bacteraemia. During this second admission, an abdominal ultrasound scan revealed secondary asplenia.
January 9, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28032338/sf1-and-spleen-development-new-heterozygous-mutation-literature-review-and-consequences-for-nr5a1-mutated-patient-s-management
#9
C Colson, E Aubry, M Cartigny, A-A Rémy, H Franquet, X Leroy, G Kéchid, C Lefèvre, R Besson, M Cools, A F Spinoit, C Sultan, S Manouvrier, P Philibert, J Ghoumid
Steroidogenic factor 1 (encoded by SF1/NR5A1) is a transcription factor with multiple target genes involved in the development and function of multiple steroidogenic and non-steroidogenic tissues. NR5A1 mutations lead to several phenotypes, including sex reversal, spermatogenesis failure, premature ovarian failure and adrenocortical insufficiency. The implication of NR5A1 mutations in spleen development anomalies was recently highlighted. We provide new evidence of this involvement, describing a novel heterozygous non-sense NR5A1 mutation in a 46,XY-DSD with polysplenia female proband and her father, who had hypospadias and asplenia...
December 29, 2016: Clinical Genetics
https://www.readbyqxmd.com/read/28008743/fatal-cytomegalovirus-infection-following-cardiac-surgery-in-a-neonate-with-asplenia
#10
Tomomi Hasegawa, Yoshihiro Oshima, Mayuko Yamamoto, Yumi Sato
No abstract text is available yet for this article.
December 2016: Pediatrics International: Official Journal of the Japan Pediatric Society
https://www.readbyqxmd.com/read/27925427/spleen-australia-guidelines-for-the-prevention-of-sepsis-in-patients-with-asplenia-and-hyposplenism-in-australia-and-new-zealand
#11
REVIEW
Kudzai Kanhutu, Penelope Jones, Allen C Cheng, Louise Grannell, Emma Best, Denis Spelman
People with asplenia or hyposplenism are at increased risk of fulminant sepsis which carries a high mortality rate. A range of preventive measures are recommended although there is ongoing evidence that knowledge of and adherence to these strategies is poor. There have been significant changes in recommended vaccinations since the previously published recommendations in 2008. We provide current recommendations to help Australian and New Zealand clinicians in the prevention of sepsis in patients with asplenia and hyposplenia...
December 7, 2016: Internal Medicine Journal
https://www.readbyqxmd.com/read/27876257/tubular-aggregate-myopathy-with-features-of-stormorken-disease-due-to-a-new-stim1-mutation
#12
Jean-Baptiste Noury, Johann Böhm, Georges Arielle Peche, Lucie Guyant-Marechal, Anne-Laure Bedat-Millet, Léa Chiche, Robert-Yves Carlier, Edoardo Malfatti, Norma B Romero, Tanya Stojkovic
STIM1 is a reticular Ca(2+) sensor composed of a luminal and a cytosolic domain. Missense mutations in the luminal domain have been associated with tubular aggregate myopathy (TAM), while cytosolic mutations can cause Stormorken syndrome, a multisystemic disease associating TAM with asplenia, thrombocytopenia, miosis, ichthyosis, short stature and dyslexia. Here we present the case of a 41-year-old female complaining of exercise intolerance. Clinical examination showed short stature, scoliosis, proximal muscle weakness with lower limb predominance, and ophthalmoplegia...
October 14, 2016: Neuromuscular Disorders: NMD
https://www.readbyqxmd.com/read/27869440/immunology-update-new-vaccines
#13
S Paul Starr
A new 9-valent human papillomavirus (HPV) vaccine is effective against more cancer-causing HPV types than previous vaccines. HPV vaccine series started with previous vaccines can be completed with the 9-valent vaccine. Two new influenza vaccines are available for adults 65 years and older: a high-dose vaccine and an enhanced adjuvant vaccine. These elicit stronger antibody responses than standard-dose vaccines. Current guidelines specify no preference for the new versus standard-dose vaccines. Two new group B meningococcal vaccines are intended for use during outbreaks and for patients with asplenia, complement deficiencies, frequent occupational meningococcus exposure, or for patients who desire protection from type B meningococcus...
November 2016: FP Essentials
https://www.readbyqxmd.com/read/27864025/heterotaxy-syndrome-with-and-without-spleen-different-infection-risk-and-management
#14
Eva Piano Mortari, Anwar Baban, Nicoletta Cantarutti, Chiara Bocci, Rachele Adorisio, Rita Carsetti
No abstract text is available yet for this article.
June 2017: Journal of Allergy and Clinical Immunology
https://www.readbyqxmd.com/read/27833056/splenic-rupture-in-community-acquired-pneumonia-a-case-report
#15
Stephen Guy, Stefaan De Clercq
INTRODUCTION: Splenic rupture is a rare but potentially lethal complication of community acquired pneumonia. PRESENTATION OF CASE: We present an unusual case of haemorrhagic shock following splenic rupture requiring emergency splenectomy in a 49 year old female with community acquired pneumonia. DISCUSSION: The epidemiology, aetiology, pathogenesis, clinical features, investigation, management and outcomes of atraumatic splenic rupture are discussed...
2016: International Journal of Surgery Case Reports
https://www.readbyqxmd.com/read/27830065/streptococcus-pneumoniae-serotype-19a-meningitis-in-well-vaccinated-immunocompetent-13-month-old-child-a-case-of-pcv13-failure
#16
Simon Kayemba-Kay's, Abdul Monem Badran, Cécile Lagneaux, Tamas Kovacs, Anne Heron
We report a 13-month-old immune-competent male child who was diagnosed with pneumococcal serotype 19A meningitis despite having received three PCV13 injections. Clinicians are reminded that bacterial meningitis can still occur, even in correctly vaccinated children. Investigations should include immune system screening along with abdominal ultrasound to exclude asplenia.
November 2016: Clinical Case Reports
https://www.readbyqxmd.com/read/27811836/recommendations-for-use-of-meningococcal-conjugate-vaccines-in-hiv-infected-persons-advisory-committee-on-immunization-practices-2016
#17
Jessica R MacNeil, Lorry G Rubin, Monica Patton, Ismael R Ortega-Sanchez, Stacey W Martin
At its June 2016 meeting, the Advisory Committee on Immunization Practices (ACIP) recommended routine use of meningococcal conjugate vaccine (serogroups A, C, W, and Y; including MenACWY-D [Menactra, Sanofi Pasteur] or MenACWY-CRM [Menveo, GlaxoSmithKline]) for persons aged ≥2 months with human immunodeficiency virus (HIV) infection. ACIP has previously recommended routine vaccination of persons aged ≥2 months who have certain medical conditions that increase risk for meningococcal disease (1), including persons who have persistent (e...
November 4, 2016: MMWR. Morbidity and Mortality Weekly Report
https://www.readbyqxmd.com/read/27776576/overwhelming-post-splenectomy-sepsis-in-patients-with-asplenia-and-hyposplenia-a-retrospective-cohort-study
#18
J Chong, P Jones, D Spelman, K Leder, A C Cheng
Overwhelming post-splenectomy infection (OPSI) is a serious complication of asplenia and is associated with encapsulated organisms, most commonly Streptococcus pneumoniae, but also Haemophilus influenzae and Neisseria meningitidis. We aimed to estimate the risk of infection in this patient group. We reviewed data collected by the Victorian Spleen Registry in Australia. On registration, all patients are asked about significant infections requiring admission to hospital for intravenous antibiotics; those requiring admission to ICU were defined as OPSI...
January 2017: Epidemiology and Infection
https://www.readbyqxmd.com/read/27765456/-pneumococcal-disease-in-adults-risk-levels-and-vaccine-recommendations
#19
Angel Vila-Córcoles, Olga Ochoa-Gondar
There are currently two anti-pneumococcal vaccines available for use in adults: the classical 23-valent polysaccharide pneumococcal vaccine (PPV23) and the new 13-valent pneumococcal conjugate vaccine (PCV13). The main advantage of the PCV13 is the potentially better immunogenicity, with its major disadvantages being the higher cost and the lower serotype-coverage than the PPV23. The currently available scientific evidence supports the following basic recommendations: (i)among adults with greatest risk (basically asplenia and immunocompromised), a dual vaccination (PCV13+PPV23) is recommended; (ii)among adults with increased risk (basically persons >65years-old and patients 15-64years with chronic pulmonary or heart disease, diabetes and/or alcoholism), a single vaccination with PPV23 is recommended (single dose in primo-vaccinated >65years; re-vaccination at 5-10years in those primo-vaccinated <65years-old); and (iii) in the rest of adults (risk normal/low) vaccination is not recommended...
October 17, 2016: Atencion Primaria
https://www.readbyqxmd.com/read/27735157/ivemark-syndrome
#20
Pushpa Masiwal, K S Chenthil, B Priyadarsini, J Gnanaprakasam, Iyengaran Srihari
Ivemark syndrome or right atrial isomerism is a rare syndrome of asplenia / hyposplenia with malformation of heart and abnormal arrangement of internal organs of chest and abdomen and is classified under heterotaxy disorder. We describe here the case of a 14 year old boy diagnosed with asplenia, dextrocardia with double outlet right ventricle and midline liver.
May 2016: Journal of the Association of Physicians of India
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