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https://www.readbyqxmd.com/read/28069783/secondary-asplenia-as-a-complication-of-severe-meningococcal-sepsis
#1
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/short-report-sf1-and-spleen-development-new-heterozygous-mutation-literature-review-and-consequences-for-nr5a1-mutated-patient-s-management
#2
Cindy Colson, Estelle Aubry, Maryse Cartigny, Amélie-Anne Rémy, Hélène Franquet, Xavier Leroy, Géraldine Kéchid, Christine Lefèvre, Rémi Besson, Martine Cools, Anne Françoise Spinoit, Charles Sultan, Sylvie Manouvrier, Pascal Philibert, Jamal 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 nonsense 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
#3
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
#4
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
#5
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
#6
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
#7
Eva Piano Mortari, Anwar Baban, Nicoletta Cantarutti, Chiara Bocci, Rachele Adorisio, Rita Carsetti
No abstract text is available yet for this article.
November 15, 2016: Journal of Allergy and Clinical Immunology
https://www.readbyqxmd.com/read/27833056/splenic-rupture-in-community-acquired-pneumonia-a-case-report
#8
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
#9
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
#10
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
#11
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
#12
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
#13
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
https://www.readbyqxmd.com/read/27650028/-prevention-of-severe-infections-in-asplenic-patients
#14
Th J M Verheij
The lifetime risk of dying from sepsis is approximately 2-3% in persons with (functional) asplenia. Both patients and their physicians are not always fully aware of these risks and the implementation of preventive measures is insufficient. Physicians should inform both patients and colleagues on the consequences of lost splenic function. Primary care physicians should screen their lists for patients with (functional) asplenia and offer these patient adequate preventive measures when needed.
2016: Nederlands Tijdschrift Voor Geneeskunde
https://www.readbyqxmd.com/read/27641144/high-burden-of-genetic-conditions-diagnosed-in-a-cardiac-neurodevelopmental-clinic
#15
Paula C Goldenberg, Betsy J Adler, Ashley Parrott, Julia Anixt, Karen Mason, Jannel Phillips, David S Cooper, Stephanie M Ware, Bradley S Marino
BACKGROUND: There is a known high prevalence of genetic and clinical syndrome diagnoses in the paediatric cardiac population. These disorders often have multisystem effects, which may have an important impact on neurodevelopmental outcomes. Taken together, these facts suggest that patients and families may benefit from consultation by genetic specialists in a cardiac neurodevelopmental clinic. OBJECTIVE: This study assessed the burden of genetic disorders and utility of genetics evaluation in a cardiac neurodevelopmental clinic...
September 19, 2016: Cardiology in the Young
https://www.readbyqxmd.com/read/27610253/plesiomonas-shigelloides-septic-shock-leading-to-death-of-postsplenectomy-patient-with-pyruvate-kinase-deficiency-and-hemochromatosis
#16
Mohammed Samannodi, Andrew Zhao, Yaser Nemshah, Kevin Shiley
Although Plesiomonas shigelloides, a water-borne bacterium of the Enterobacteriaceae family, usually causes self-limiting gastroenteritis with diarrhea, several cases of sepsis have been reported. We report the case of a 43-year-old male patient with hemochromatosis, pyruvate kinase deficiency, and asplenia via splenectomy who developed septic shock caused by P. shigelloides complicated by respiratory failure, renal failure, liver failure, and disseminated intravascular coagulation. Early aggressive antimicrobial therapy and resuscitation measures were unsuccessful and the patient passed away...
2016: Case Reports in Infectious Diseases
https://www.readbyqxmd.com/read/27606905/surgical-management-of-hiatal-hernia-in-children-with-asplenia-syndrome
#17
Hiromu Miyake, Koji Fukumoto, Masaya Yamoto, Hiroshi Nouso, Masakatsu Kaneshiro, Mariko Koyama, Naoto Urushihara
Purpose Patients with asplenia syndrome (AS) are likely to have upper gastrointestinal tract malformations such as hiatal hernia. This report discusses the treatment of such conditions. Methods Seventy-five patients with AS underwent initial palliation in our institution between 1997 and 2013. Of these, 10 patients had hiatal hernia. Of the patients with hiatal hernia, 6 had brachyesophagus and 7 had microgastria. Results Of the 10 patients with hiatal hernia, 9 underwent surgery in infancy (7 before Glenn operation, 2 after Glenn operation)...
September 8, 2016: European Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/27600384/ct-abdominal-imaging-findings-in-patients-with-sickle-cell-disease-acute-vaso-occlusive-crisis-complications-and-chronic-sequelae
#18
REVIEW
Carly S Gardner, Daniel T Boll, Priya Bhosale, Tracy A Jaffe
Sickle cell disease (SCD) is the most prevalent hemoglobinopathy. Survival in patients with SCD has improved over the past few decades. These patients experience a lifetime of repeated acute pain crises, which are thought to result from sickling and microvascular occlusions; acute abdominal pain is common. Moreover, repeated crises often lead to organ dysfunction, such as asplenia, hepatic failure, and renal failure. The spleen, liver, biliary system, kidneys, and gastrointestinal tract can all be affected...
December 2016: Abdominal Radiology
https://www.readbyqxmd.com/read/27573083/recommendations-for-serogroup-b-meningococcal-vaccine-for-persons-10-years-and-older
#19
(no author information available yet)
This policy statement provides recommendations for the prevention of serogroup B meningococcal disease through the use of 2 newly licensed serogroup B meningococcal vaccines: MenB-FHbp (Trumenba; Wyeth Pharmaceuticals, a subsidiary of Pfizer, Philadelphia, PA) and MenB-4C (Bexsero; Novartis Vaccines, Siena, Italy). Both vaccines are approved for use in persons 10 through 25 years of age. MenB-FHbp is licensed as a 2- or 3-dose series, and MenB-4C is licensed as a 2-dose series for all groups. Either vaccine is recommended for routine use in persons 10 years and older who are at increased risk of serogroup B meningococcal disease (category A recommendation)...
September 2016: Pediatrics
https://www.readbyqxmd.com/read/27516778/bone-and-joint-infections-due-to-haemophilus-parainfluenzae-case-report-and-review-of-the-literature
#20
Conar R O'Neil, Evan Wilson, Bayan Missaghi
Haemophilus parainfluenzae is a normal inhabitant of the human respiratory tract. However it is an increasingly recognized pathogen in invasive infections, particularly in the immunocompromised host and where there is disruption of the normal skin or mucosal barriers. We present a case of a 56-year-old female with a history of asplenia who developed H. parainfluenzae septic arthritis of the hip following an intra-articular steroid injection. We also summarize previously reported cases of bone and joint infections caused by H...
2016: Canadian Journal of Infectious Diseases & Medical Microbiology
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