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Jakob Pugi, Manuel Carcao, Luke J Drury, Jacob C Langer
BACKGROUND: Laparoscopic partial splenectomy (LPS) theoretically maintains long-term splenic immune function for children with hereditary spherocytosis (HS). Our goal was to review our results after LPS and to determine if specific genetic mutations influence outcome. METHODS: All children with HS undergoing LPS between 2005 and 2016 were reviewed. RESULTS: Thirty-one children underwent LPS (16 male) at a median age of 9 (range 2-18) years...
February 8, 2018: Journal of Pediatric Surgery
Hirofumi Obinata, Shinichi Nishibe, Yoko Ishihara
Background: Heterotaxy syndrome (HS) is characterized by a wide variety of cardiac and extra-cardiac malformations, including pulmonary valve stenosis, interruption of the inferior vena cava, total anomalous pulmonary venous connection (TAPVC), asplenia, polysplenia, intestinal malrotation, and preduodenal portal vein (PDPV). We report the case of a heterotaxic infant with an infracardiac TAPVC and preduodenal portal vein who experienced repetitive hemodynamic instability during urgent laparotomy for duodenal obstruction...
2018: JA Clin Rep
Nancy L Van Buren, Jed B Gorlin, Robyn C Reed, Jerome L Gottschall, Stephen C Nelson
BACKGROUND: Acute splenic sequestration crisis is a complication of sickle cell disease (SCD) occurring when intrasplenic red blood cell (RBC) sickling prevents blood from leaving the spleen, causing acute splenic enlargement. Although typically seen in young children, it has been reported in older children with hemoglobin (Hb)SC disease, eventually resulting in functional asplenia. Ceftriaxone is a frequently used antibiotic of choice for children with SCD, because of its efficacy against invasive pneumococcal disease...
February 22, 2018: Transfusion
Alicia Arnott, Penelope Jones, Lucinda J Franklin, Denis Spelman, Karin Leder, Allen C Cheng
Background: Overwhelming post-splenectomy infection (OPSI) is a serious complication of asplenia. Clinical guidelines recommend a number of measures to reduce the risk of OPSI, but awareness and adherence to preventative measures is generally poor. We aimed to determine whether a registry for asplenic/hyposplenic patients was associated with a reduction in the incidence of infection with encapsulated bacteria. Methods: We performed a retrospective cohort study of asplenic/hyposplenic patients in the state of Victoria, Australia who registered with Spleen Australia from 2003 - 2014...
February 17, 2018: Clinical Infectious Diseases: An Official Publication of the Infectious Diseases Society of America
Hafsa Abbas, Angelica CiFuentes Kottkamp, Naeem Abbas, Richard Cindrich, Manisha Singh
BACKGROUND Cryptococcal meningitis in patients who are seronegative for the human immunodeficiency virus (HIV) and in patients who are splenectomized is rare. This report is an unusual case of meningeal and cerebellar infection due to the encapsulated yeast, Cryptococcus neoformans, which has not previously been associated with asplenia. CASE REPORT A 65-year-old HIV-negative patient with a previous splenectomy, presented with a three-day history of fever, vomiting, and headache. His symptoms progressed to generalized body aches, persistent fever, and neck stiffness...
February 19, 2018: American Journal of Case Reports
Deane Yim, Hazumu Nagata, Christopher Z Lam, Lars Grosse-Wortmann, Mike Seed, Edgar Jaeggi, Shi-Joon Yoo
BACKGROUND: It is advocated that heterotaxy should be segregated into right or left isomerism according to atrial appendage morphology. However, atrial situs determination is often based on the pattern of associated findings rather than on atrial morphology itself, which can be difficult to define. The objective was to assess how often concordant patterns of isomerism classified by atrial appendage morphology, bronchopulmonary pattern, and splenic status are breached using cardiac magnetic resonance or computed tomography...
February 2018: Circulation. Cardiovascular Imaging
Yoichi Robertus Fujii
MicroRNA (miRNA) is the noncoding gene: therefore, the miRNA gene inheritably controls protein gene expression through transcriptional and post-transcriptional levels. Aberrant expression of miRNA genes causes various human diseases, especially cancers. Although cancer is a complex disease, cancer/miRNA implication has yet been grasped from the perspective of miRNA profile in bed side. Since miRNA is the mobile genetic element, the clinical verification of miRNA in microvesicle of blood is too much straggle to predict potential cancer/miRNA associations without bioinformatical computing...
2018: Methods in Molecular Biology
Christopher A Febres-Aldana, Lydia Hernandez Howard
Sickle cell disease (SCD) manifests itself with vaso-occlusive episodes leading to infarction. Placement of intravascular catheters provides a useful route for management of pain crises as well as other complications. However, catheter misuse is a commonly unrecognized problem, which can have lethal consequences. We present a case of fatal splenic sequestration/hyperhemolysis secondary to foreign body pulmonary and systemic embolization due to intravenous administration of hydromorphone pills in a young woman with SCD...
January 18, 2018: American Journal of Forensic Medicine and Pathology
Mohammad Faizan Zahid, Ashish Pal Singh Bains
No abstract text is available yet for this article.
December 28, 2017: Blood
Chamath Premawardena, Donald Bowden, Zane Kaplan, Claire Dendle, Ian John Woolley
OBJECTIVES: Asplenia and hyposplenism carry a significant risk of ongoing morbidity and mortality which can be reduced by education, vaccination and antibiotic use. We aimed to assess education and other methods of prevention in a cohort of patients with haemoglobinopathy in a tertiary referral centre, which also had access to a post-splenectomy registry created to reduce post-splenectomy infection risk. METHODS: A standardized questionnaire was used on patients who attended the service for regular therapy...
December 13, 2017: Hematology (Amsterdam, Netherlands)
Döndü Üsküdar Cansu, Hava Üsküdar Teke, Ahmet Musmul, Cengiz Korkmaz
In systemic lupus erythematosus (SLE), the most commonly encountered finding related to platelets is thrombocytopenia whereas thrombocytosis is rarely reported. Our aim here was to reveal the type and the frequency of thrombocytosis in SLE patients along with its causes. Data of patients were evaluated retrospectively. Patients who had a platelet count of > 450,000/mm3 (> 450 × 109/L) in at least two subsequent counts and lasting more than 6 months during the follow-up were considered to have "persistent thrombocytosis"...
November 8, 2017: Rheumatology International
Jennifer Teichman, Ahmed Taher, Abdulaziz Hashi, Akshay Bagai, Michelle Sholzberg
More recent immune thrombocytopenia (ITP) treatment strategies enhance platelet production with the use of thrombopoietin receptor agonists (TPO-RA) such as eltrombopag. Patients receiving TPO-RA agents may be at an increased risk of thromboembolism, however the pathophysiology and common underlying risk factors are not well understood. We present the case of a young asplenic woman on eltrombopag for chronic ITP with acute myocardial infarction involving the right coronary artery. Past medical history was significant for remote mediastinal radiation for lymphoma and splenectomy for ITP...
November 3, 2017: Journal of Thrombosis and Thrombolysis
Jelena Z Arnautovic, Areej Mazhar, Stela Tereziu, Kashvi Gupta
BACKGROUND Isolated congenital asplenia is a poorly understood and rare form of primary immunodeficiency, often associated with life-threatening infections. CASE REPORT We encountered a unique case of a 22-year-old asplenic male who presented with severe iron-deficiency anemia secondary to occult gastrointestinal bleeding since age 15. Our extensive work-up confirmed jejunal arteriovenous malformations as the source of the bleed. Six months after the treatment, the patient has reported no further episodes of gastrointestinal bleeding and his hemoglobin has remained stable...
October 19, 2017: American Journal of Case Reports
Daisuke Kondoh, Tomomi Kawano, Tomoaki Kikuchi, Kaoru Hatate, Kenichi Watanabe, Motoki Sasaki, Norio Yamagishi, Hisashi Inokuma, Nobuo Kitamura
BACKGROUND: Laterality disorders of the abdominal organs include situs inversus totalis that mirrors the arrangements of all internal organs and heterotaxy syndrome (situs ambiguus) in which the thoracic or abdominal organs are abnormally arranged. Heterotaxy is often accompanied by multiple congenital malformations, and it generally comprises asplenia and polysplenia syndromes. To our knowledge, polysplenia syndrome has been reported in only three cattle, and computerized tomographic (CT) images of these animals were not obtained...
September 29, 2017: BMC Veterinary Research
Sandra A Farrell, Sandi Sodhi, Christian R Marshall, Andrea Guerin, Anne Slavotinek, Tara Paton, Karen Chong, Wilma L Sirkin, Stephen W Scherer, Félix-Antoine Bérubé-Simard, Nicolas Pilon
Isolated congenital diaphragmatic hernia is often a sporadic event with a low recurrence risk. However, underlying genetic etiologies, such as chromosome anomalies or single gene disorders, are identified in a small number of individuals. We describe two fetuses with a unique pattern of multiple congenital anomalies, including diaphragmatic hernia, short bowel and asplenia, born to first-cousin parents. Whole exome sequencing showed that both were homozygous for a missense variant, c.950A>C, predicting p...
September 12, 2017: American Journal of Medical Genetics. Part A
L Szenborn, I V Osipova, H Czajka, S M Kharit, T Jackowska, N François, M A Habib, D Borys
BACKGROUND: Immunization with pneumococcal vaccines is an important prophylactic strategy for children with asplenia or splenic dysfunction, who are at high risk of bacterial infections (including S. pneumoniae). This study aimed to assess immunogenicity and safety of pneumococcal non-typeable Haemophilus influenzae protein D conjugate vaccine (PHiD-CV, GSK) in this at-risk population. METHODS: This phase III, multi-centre, open-label, controlled study, in which at-risk children with asplenia or splenic dysfunction were enrolled (age strata: 2-4, 5-10 and 11-17years), was conducted in Poland and the Russian Federation...
September 25, 2017: Vaccine
Andrew Headrick, Erinn O Schmit, David W Kimberlin
The post Hib-vaccine era has resulted in increased cases of invasive, non-Hib, Haemophilus influenzae infections. One of the encapsulated species, type a, displays the most similarity in virulence to type b. Our patient, a fully-vaccinated four-year-old female, with newly diagnosed asplenia in the setting of heterotaxy, had a prolonged hospital course with disseminated Haemophilus influenzae type a infection.
August 26, 2017: Pediatric Infectious Disease Journal
Pradeep Bangalore Prakash, Sook-Kyung C Kwon, Aditya Badheka, Veerajalandhar Allareddy
No abstract text is available yet for this article.
August 1, 2017: Clinical Pediatrics
L Yeo, R Romero
OBJECTIVE: To evaluate the performance of color and bidirectional power Doppler ultrasound combined with Fetal Intelligent Navigation Echocardiography (FINE) in examining the fetal heart. METHODS: A prospective cohort study was conducted of fetuses in the second and third trimesters with a normal heart or with congenital heart disease (CHD). One or more spatiotemporal image correlation (STIC) volume datasets, combined with color or bidirectional power Doppler (S-flow) imaging, were acquired in the apical four-chamber view...
October 2017: Ultrasound in Obstetrics & Gynecology
Jonathan N Johnson, Cindy S Barrett, Wayne H Franklin, Eric M Graham, Nancy J Halnon, Brandy A Hattendorf, Catherine D Krawczeski, James J McGovern, Matthew J O'Connor, Amy H Schultz, Jeffrey M Vinocur, Devyani Chowdhury, Jeffrey B Anderson
INTRODUCTION: In 2012, the American College of Cardiology's (ACC) Adult Congenital and Pediatric Cardiology Council established a program to develop quality metrics to guide ambulatory practices for pediatric cardiology. The council chose five areas on which to focus their efforts; chest pain, Kawasaki Disease, tetralogy of Fallot, transposition of the great arteries after arterial switch, and infection prevention. Here, we sought to describe the process, evaluation, and results of the Infection Prevention Committee's metric design process...
December 2017: Congenital Heart Disease
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