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About 24,082 results
Grifols announces FDA approval of Xembify®, 20% subcutaneous immunoglobulin for primary immunodeficiencies
https://www.grifols.com/en/view-news/-/news/grifols-announces-fda-approval-of-xembify-20-subcutaneous-immunoglobulin-for-primary-immunodeficiencies

Jul 4th, 2019 - Grifols (MCE:GRF, MCE:GRF.P NASDAQ:GRFS), a leading global producer of plasma-derived medicines, announced today that Xembify®, its new 20% subcutaneous immunoglobulin, has been approved by the U.S. Food and Drug Administration (FDA). Xembify® is used to treat primary immunodeficiencies.

Head‐to‐head oral prophylactic antibiotic therapy for chronic obstructive pulmonary disease
https://www.cochranelibrary.com/cdsr/doi/10.1002/14651858.CD013024.pub2/full
The Cochrane Database of Systematic Reviews; Threapleton, C. et. al.

May 23rd, 2019 - Chronic obstructive pulmonary disease (COPD; including chronic bronchitis and emphysema) is a chronic respiratory condition characterised by shortness of breath, cough and recurrent exacerbations. Long‐term antibiotic use may reduce both bacterial load and inflammation in the airways. Studies have shown a reduction of exacerbations with antibiotics in comparison to placebo in people with COPD, ...

Antimicrobial Prophylaxis for Adult Patients With Cancer-Related Immunosuppression: ASCO and IDSA Clinical Practice Guideline Update
http://ascopubs.org/doi/full/10.1200/JCO.18.00374
Taplitz, R. et. al.

Sep 3rd, 2018 - Antibacterial and antifungal prophylaxis is recommended for patients who are at high risk of infection, including patients who are expected to have profound, protracted neutropenia, which is defined as < 100 neutrophils/µL for > 7 days or other risk factors. Herpes simplex virus–seropositive patients undergoing allogeneic hematopoietic stem-cell transplantation or leukemia induction therapy sho...

Optimisation of empirical antimicrobial therapy in patients with haematological maligna...
https://doi.org/10.1016/S2352-3026(17)30211-9
The Lancet. Haematology; Aguilar-Guisado M, Espigado I et. al.

Nov 20th, 2017 - Continuation of empirical antimicrobial therapy (EAT) for febrile neutropenia in patients with haematological malignancies until neutrophil recovery could prolong the therapy unnecessarily. We aimed to establish whether EAT discontinuation driven by a clinical approach regardless of neutrophil recovery would optimise the duration of therapy. We did an investigator-driven, superiority, open-labe...

Application of the MASCC and CISNE Risk-Stratification Scores to Identify Low-Risk Febr...
https://doi.org/10.1016/j.annemergmed.2016.11.007
Annals of Emergency Medicine; Coyne CJ, Le V et. al.

Jan 3rd, 2017 - Although validated risk-stratification tools have been used to send low-risk febrile neutropenic patients home from clinic and inpatient settings, there is a dearth of research evaluating these scores in the emergency department (ED). We compare the predictive accuracy of the Multinational Association for Supportive Care in Cancer (MASCC) and Clinical Index of Stable Febrile Neutropenia (CISNE)...

Efficacy and safety of benralizumab for patients with severe asthma uncontrolled with h...
https://doi.org/10.1016/S0140-6736(16)31324-1
Lancet (London, England); Bleecker ER, FitzGerald JM et. al.

Sep 9th, 2016 - Eosinophilia is associated with worsening asthma severity and decreased lung function, with increased exacerbation frequency. We assessed the safety and efficacy of benralizumab, a monoclonal antibody against interleukin-5 receptor α that depletes eosinophils by antibody-dependent cell-mediated cytotoxicity, for patients with severe, uncontrolled asthma with eosinophilia. We did a randomised, d...

Severe chronic primary neutropenia in adults: report on a series of 108 patients.
https://doi.org/10.1182/blood-2015-03-634493
Blood Sicre de Fontbrune F, Moignet A et. al.

Aug 11th, 2015 - Severe chronic primary neutropenia (CPN) is a rare entity, and long-term outcome and risk factors for infections in severe CPN adults have not been described to date. We report the characteristics and outcomes of 108 severe adult CPN patients enrolled in a multi-institutional observational study. Severe CPN adults were mostly female (78%), and median age at diagnosis was 28.3 years. Diagnosis w...

Effect of oral eliglustat on splenomegaly in patients with Gaucher disease type 1: the ...
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4962880
JAMA Mistry PK, Lukina E et. al.

Feb 17th, 2015 - Gaucher disease type 1 is characterized by hepatosplenomegaly, anemia, thrombocytopenia, and skeletal disease. A safe, effective oral therapy is needed. To determine whether eliglustat, a novel oral substrate reduction therapy, safely reverses clinical manifestations in untreated adults with Gaucher disease type 1. Phase 3, randomized, double-blind, placebo-controlled trial conducted at 18 site...

Ruxolitinib versus standard therapy for the treatment of polycythemia vera.
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4358820
The New England Journal of Medicine; Vannucchi AM, Kiladjian JJ et. al.

Jan 29th, 2015 - Ruxolitinib, a Janus kinase (JAK) 1 and 2 inhibitor, was shown to have a clinical benefit in patients with polycythemia vera in a phase 2 study. We conducted a phase 3 open-label study to evaluate the efficacy and safety of ruxolitinib versus standard therapy in patients with polycythemia vera who had an inadequate response to or had unacceptable side effects from hydroxyurea. We randomly assig...

The lymphoid variant of hypereosinophilic syndrome: study of 21 patients with CD3-CD4+ ...
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4602413
Medicine Lefèvre G, Copin MC et. al.

Nov 14th, 2014 - The CD3-CD4+ aberrant T-cell phenotype is the most described in the lymphoid variant of hypereosinophilic syndrome (L-HES), a rare form of HES. Only a few cases have been reported, and data for these patients are scarce. To describe characteristics and outcome of CD3-CD4+ L-HES patients, we conducted a national multicentric retrospective study in the French Eosinophil Network. All patients who ...

Genetic variants associated with phenytoin-related severe cutaneous adverse reactions.
https://doi.org/10.1001/jama.2014.7859
JAMA Chung WH, Chang WC et. al.

Aug 6th, 2014 - The antiepileptic drug phenytoin can cause cutaneous adverse reactions, ranging from maculopapular exanthema to severe cutaneous adverse reactions, which include drug reactions with eosinophilia and systemic symptoms, Stevens-Johnson syndrome, and toxic epidermal necrolysis. The pharmacogenomic basis of phenytoin-related severe cutaneous adverse reactions remains unknown. To investigate the gen...

Transplantation outcomes for severe combined immunodeficiency, 2000-2009.
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4183064
The New England Journal of Medicine; Pai SY, Logan BR et. al.

Jul 30th, 2014 - The Primary Immune Deficiency Treatment Consortium was formed to analyze the results of hematopoietic-cell transplantation in children with severe combined immunodeficiency (SCID) and other primary immunodeficiencies. Factors associated with a good transplantation outcome need to be identified in order to design safer and more effective curative therapy, particularly for children with SCID diag...

Antibiotic prescribing for adults with acute bronchitis in the United States, 1996-2010.
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4529023
JAMA Barnett ML, Linder JA

May 22nd, 2014 - Antibiotic prescribing for adults with acute bronchitis in the United States, 1996-2010.|2014|Barnett ML,Linder JA,|therapeutic use,drug therapy,statistics & numerical data,

Drug reaction with eosinophilia and systemic symptoms, or virus reactivation with eosin...
https://doi.org/10.1111/bjd.13079
The British Journal of Dermatology; Almudimeegh A, Rioux C et. al.

Apr 29th, 2014 - We report a case of drug reaction with eosinophilia and systemic symptoms (DRESS) in a patient with HIV receiving antitoxoplasmic drugs (adiazine and pyrimethamine) and levetiracetam along with highly active antiretroviral therapy (tenofovir-emtricitabine, darunavir and ritonavir). Cytomegalovirus (CMV) infection was reactivated in the 7 weeks before the development of DRESS but was successfull...

Clinical picture and treatment of 2212 patients with common variable immunodeficiency.
https://doi.org/10.1016/j.jaci.2013.12.1077
The Journal of Allergy and Clinical Immunology; Gathmann B, Mahlaoui N et. al.

Mar 3rd, 2014 - Common variable immunodeficiency (CVID) is an antibody deficiency with an equal sex distribution and a high variability in clinical presentation. The main features include respiratory tract infections and their associated complications, enteropathy, autoimmunity, and lymphoproliferative disorders. This study analyzes the clinical presentation, association between clinical features, and differen...

The optimal number of biopsy fragments to establish a morphologic diagnosis of eosinoph...
https://doi.org/10.1038/ajg.2013.463
The American Journal of Gastroenterology; Nielsen JA, Lager DJ et. al.

Jan 21st, 2014 - Eosinophilic esophagitis (EoE) is characterized clinically by dysphagia, chest pain, and food impaction, and morphologically by increased numbers of intraepithelial eosinophils and marked basal hyperplasia of the squamous mucosa. The consensus criteria for a diagnosis of EoE include the presence of ≥15 eosinophils/HPF in biopsies from both proximal and distal esophagus in the absence of other c...

Association of Nonobstructive Chronic Bronchitis With Respiratory Health Outcomes in Ad...
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7052787
JAMA Internal Medicine; Balte PP, Chaves PHM et. al.

Mar 2nd, 2020 - Chronic bronchitis has been associated with cigarette smoking as well as with e-cigarette use among young adults, but the association of chronic bronchitis in persons without airflow obstruction or clinical asthma, described as nonobstructive chronic bronchitis, with respiratory health outcomes remains uncertain. To assess whether nonobstructive chronic bronchitis is associated with adverse res...

Long-term Sequelae of Nonobstructive Chronic Bronchitis-Is Airflow Obstruction Important?
https://doi.org/10.1001/jamainternmed.2019.7280
JAMA Internal Medicine; Woodruff PG, Lazarus SC

Mar 1st, 2020 - Long-term Sequelae of Nonobstructive Chronic Bronchitis-Is Airflow Obstruction Important?|2020|Woodruff PG,Lazarus SC,|

Age-related prevalence and clinical significance of neutropenia - isolated or combined ...
https://doi.org/10.1002/ajh.25756
American Journal of Hematology REFERENCES; Palmblad J, Siersma V et. al.

Feb 13th, 2020 - Neutropenia (NP), that is, an absolute blood neutrophil count (ANC) <1.5 g/L, accompanies various diseases. However, the clinical significance of NP, detected in routine complete blood cell counts (CBC) in primary care, is poorly characterized. Here, from a primary care resource with ANCs from >370 000 individuals, we identified and followed neutropenic subjects for the next 4 years for novel I...

Infectious vaccine-derived rubella viruses emerge, persist, and evolve in cutaneous gra...
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6837625
PLoS Pathogens; Perelygina L, Chen MH et. al.

Oct 28th, 2019 - Rubella viruses (RV) have been found in an association with granulomas in children with primary immune deficiencies (PID). Here, we report the recovery and characterization of infectious immunodeficiency-related vaccine-derived rubella viruses (iVDRV) from diagnostic skin biopsies of four patients. Sequence evolution within PID hosts was studied by comparison of the complete genomic sequences o...

Benralizumab for PDGFRA-Negative Hypereosinophilic Syndrome.
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6557265
The New England Journal of Medicine; Kuang FL, Legrand F et. al.

Apr 3rd, 2019 - Hypereosinophilic syndrome is a group of diseases defined by marked eosinophilia in blood or tissue and eosinophil-related clinical manifestations. Benralizumab is a monoclonal antibody against interleukin-5 receptor α, which is expressed on human eosinophils. In this randomized, double-blind, placebo-controlled, phase 2 trial, we administered a series of three monthly subcutaneous injections o...

Safety of endoscopy in cancer patients with thrombocytopenia and neutropenia.
https://doi.org/10.1016/j.gie.2018.12.004
Gastrointestinal Endoscopy; Abu-Sbeih H, Ali FS et. al.

Dec 14th, 2018 - Cancer patients are prone to thrombocytopenia and neutropenia, which increase the risk of bleeding and infection. We assessed the safety of endoscopic procedures in cancer patients with thrombocytopenia and/or neutropenia. We studied consecutive cancer patients with thrombocytopenia and/or neutropenia who underwent endoscopic procedures from 2010 through 2015. Neutropenia was defined as an abso...

Lymphopenia and risk of infection and infection-related death in 98,344 individuals fro...
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6211632
PLoS Medicine; Warny M, Helby J et. al.

Nov 1st, 2018 - Neutropenia increases the risk of infection, but it is unknown if this also applies to lymphopenia. We therefore tested the hypotheses that lymphopenia is associated with increased risk of infection and infection-related death in the general population. Of the invited 220,424 individuals, 99,191 attended examination. We analyzed 98,344 individuals from the Copenhagen General Population Study (D...

Meta-analysis examining the epidemiology of clozapine-associated neutropenia.
https://doi.org/10.1111/acps.12898
Acta Psychiatrica Scandinavica; Myles N, Myles H et. al.

May 22nd, 2018 - Clozapine is associated with life-threatening neutropenia. There are no previous meta-analyses of the epidemiology of clozapine-associated neutropenia. To determine the cumulative incidence of mild, moderate and severe neutropenia, incidence of death related to severe neutropenia, case fatality rate of neutropenia and the longitudinal incidence of neutropenia following exposure to clozapine. A ...

Dexpramipexole as an oral steroid-sparing agent in hypereosinophilic syndromes.
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6073324
Blood Panch SR, Bozik ME et. al.

May 9th, 2018 - Hypereosinophilic syndromes (HESs) are a heterogeneous group of disorders characterized by peripheral eosinophilia and eosinophil-related end organ damage. Whereas most patients respond to glucocorticoid (GC) therapy, high doses are often necessary, and side effects are common. Dexpramipexole (KNS-760704), an orally bioavailable synthetic aminobenzothiazole, showed an excellent safety profile a...

Phase I/II trial of the oral regimen ixazomib, pomalidomide, and dexamethasone in relap...
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6005710
Leukemia Krishnan A, Kapoor P et. al.

Dec 18th, 2017 - In this phase I/II trial, a triplet regimen of ixazomib (Ixa: 3 or 4 mg), pomalidomide (Pom: 4 mg), and dexamethasone (Dex: 40 mg) was administered to 32 lenalidomide-refractory multiple myeloma (MM) patients; 31 were evaluable for response and toxicity. At dose level 1 (DL1, 3 mg Ixa), 1/3 patients experienced grade 3 fatigue, grade 3 lung infection, grade 4 neutropenia, and grade 4 thrombocyt...

Peripheral Eosinophilia in Patients With Inflammatory Bowel Disease Defines an Aggressi...
https://doi.org/10.1038/ajg.2017.402
The American Journal of Gastroenterology; Click B, Anderson AM et. al.

Nov 7th, 2017 - Peripheral blood eosinophilia (PBE) in inflammatory bowel disease (IBD) is associated with ulcerative colitis (UC) and active disease. Little data exist on the long-term impact of PBE on disease course. We aimed to investigate the multi-year patterns of PBE and its impact on disease severity in a large IBD cohort. We performed a registry analysis of a consented, prospective, natural history IBD...

Diagnosis of primary antibody and complement deficiencies in young adults after a first...
https://doi.org/10.1016/j.cmi.2017.02.005
Clinical Microbiology and Infection : the Official Publication of the European Society of Clinical Microbiology and Infectious Diseases; Sanges S, Wallet F et. al.

Feb 13th, 2017 - Screening for primary immunodeficiencies (PIDs) in adults is recommended after two severe bacterial infections. We aimed to evaluate if screening should be performed after the first invasive infection in young adults. Eligible patients were retrospectively identified using hospital discharge and bacteriology databases in three centres during a 3-year period. Eighteen to 40-year-old patients wer...

Plasma presepsin level is an early diagnostic marker of severe febrile neutropenia in h...
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5217328
BMC Infectious Diseases; Koizumi Y, Shimizu K et. al.

Jan 6th, 2017 - Febrile neutropenia (FN) is a common infectious complication in chemotherapy. The mortality of FN is higher in hematologic malignancy patients, and early diagnostic marker is needed. Presepsin is a prompt and specific marker for bacterial sepsis, but its efficacy in severe febrile neutropenia (FN) is not well confirmed. We tried to clarify whether it is a useful maker for early diagnosis of FN ...

Emergency Department Management of Patients With Febrile Neutropenia: Guideline Concord...
https://doi.org/10.1111/acem.13079
Academic Emergency Medicine : Official Journal of the Society for Academic Emergency Medicine; Baugh CW, Wang TJ et. al.

Sep 9th, 2016 - The Infectious Diseases Society of America and the American Society of Clinical Oncology recommend risk stratification of patients with febrile neutropenia (FN) and discharge with oral antibiotics for low-risk patients. We studied guideline concordance and clinical outcomes of FN management in our emergency department (ED). Our urban, tertiary care teaching hospital provides all emergency and i...

The GerdQ questionnaire and high resolution manometry support the hypothesis that proto...
https://doi.org/10.1111/apt.13718
Alimentary Pharmacology & Therapeutics; Savarino EV, Tolone S et. al.

Jul 4th, 2016 - Little is known about the relationship between proton pump inhibitor-responsive oesophageal eosinophilia (PPI-REE), eosinophilic esophagitis (EoE) and gastro-oesophageal reflux disease (GERD). To compare high resolution manometry features and symptom profiles of patients with EoE, PPI-REE and GERD. Consecutive patients diagnosed with EoE or PPI-REE according to international criteria (presence ...

The efficacy of step-down therapy in adult patients with proton pump inhibitor-responsi...
https://doi.org/10.1111/apt.13496
Alimentary Pharmacology & Therapeutics; Gómez-Torrijos E, García-Rodríguez R et. al.

Dec 14th, 2015 - Proton pump inhibitor-responsive oesophageal eosinophilia (PPI-REE) is common in patients with suspected eosinophilic oesophagitis (EoE). However, the long-term efficacy of PPIs and the best maintenance doses are yet to be defined. To evaluate the durability of the response to PPI therapy after tapering PPI doses in PPI-REE patients. Prospective study conducted on PPI-REE patients. Upon complet...

Intravenous immune globulin and thromboembolic adverse events in patients with hematolo...
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4713161
Blood Ammann EM, Jones MP et. al.

Oct 7th, 2015 - In patients with hypogammaglobulinemia secondary to chronic lymphocytic leukemia (CLL) or multiple myeloma (MM), intravenous immune globulin (IVIg) may be administered to reduce the risk of infection. Since 2013, IVIg products have carried a boxed safety warning about the risk of thromboembolic events (TEEs), with TEEs reported in 0.5% to 15% of patients treated with IVIg. In this retrospective...

Peripheral blood eosinophilia and hypersensitivity reactions among patients receiving o...
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4640981
The Journal of Allergy and Clinical Immunology; Blumenthal KG, Youngster I et. al.

May 19th, 2015 - Although drug-induced peripheral eosinophilia complicates antimicrobial therapy, little is known about its frequency and implications. We aimed to determine the frequency and predictors of antibiotic-induced eosinophilia and subsequent hypersensitivity reactions (HSRs). We evaluated a prospective cohort of former inpatients receiving intravenous antibiotic therapy as outpatients with at least 1...

Partial splenic embolization in the treatment of prolonged thrombocytopenia due to hype...
https://doi.org/10.1007/s00520-018-4192-3
Supportive Care in Cancer : Official Journal of the Multinational Association of Supportive Care in Cancer; Passhak M, Shachar SS et. al.

Apr 26th, 2018 - Hypersplenism-related thrombocytopenia (HST) may delay or preclude chemotherapy. Partial splenic embolization (PSE) has been used at our center to overcome prolonged HST. Between November 2012 and April 2015, 11 PSE procedures were performed in 10 patients; 9 had metastatic colorectal cancer and 1 had widespread pancreatic cancer. PSE was performed by selective catheterization of the splenic ar...

Sirolimus as an alternative treatment in patients with granulomatous-lymphocytic lung d...
https://doi.org/10.1111/pai.12890
Pediatric Allergy and Immunology : Official Publication of the European Society of Pediatric Allergy and Immunology; Deyà-Martínez A, Esteve-Solé A et. al.

Mar 13th, 2018 - One of the most frequent non-infectious complications of humoral immunodeficiencies with a CVID-like pattern is a particular form of inflammatory lung disease which is called granulomatous-lymphocytic interstitial lung disease (GLILD). Its development worsens patient prognosis, with a significant decrease in survival. Currently, there are no unified guidelines regarding its management, and diff...

Incidence and prognostic impact of cytogenetic aberrations in patients with systemic ma...
https://doi.org/10.1002/gcc.22526
Genes, Chromosomes & Cancer; Naumann N, Jawhar M et. al.

Jan 17th, 2018 - The clinical behavior of systemic mastocytosis (SM) is strongly associated with activating mutations in KIT (D816V in >80% of cases), with the severity of the phenotype influenced by additional somatic mutations, for example, in SRSF2, ASXL1, or RUNX1. Complex molecular profiles are frequently associated with the presence of an associated hematologic neoplasm (AHN) and an unfavorable clinical o...

Cardiopulmonary anomalies in incontinentia pigmenti patients.
https://doi.org/10.1111/ijd.13835
International Journal of Dermatology; Onnis G, Diociaiuti A et. al.

Dec 2nd, 2017 - Incontinentia pigmenti (IP) is a rare inherited genodermatosis that usually involves the skin, and also teeth, oral cavity, central nervous system, eyes, blood with eosinophilia, and rarely skeletal system, breast, heart, and lungs. Skin lesions usually appear early, at birth or within the first 2 weeks of life, with four different phases tending to follow Blaschko lines that may overlap. We re...

Prevalence and burden of chronic bronchitis symptoms: results from the BOLD study.
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5699921
The European Respiratory Journal; Mejza F, Gnatiuc L et. al.

Nov 23rd, 2017 - We studied the prevalence, burden and potential risk factors for chronic bronchitis symptoms in the Burden of Obstructive Lung Disease study.Representative population-based samples of adults aged ≥40 years were selected in participating sites. Participants completed questionnaires and spirometry. Chronic bronchitis symptoms were defined as chronic cough and phlegm on most days for ≥3 months eac...

Vaccination with a human parainfluenza virus type 3 chimeric FHN glycoprotein formulate...
https://doi.org/10.1016/j.vaccine.2017.10.095
Vaccine Garg R, Brownlie R et. al.

Nov 20th, 2017 - Human parainfluenza virus type 3 (PIV3) is a major cause of lower respiratory disease i.e. bronchitis, bronchiolitis or pneumonia, in infants and young children. Presently there is no licensed vaccine against PIV3. To produce an effective subunit vaccine, a chimeric FHN glycoprotein consisting of the N-terminal ectodomain of the fusion (F) protein linked to the haemagglutinin-neuraminidase (HN)...

World Health Organization-defined eosinophilic disorders: 2017 update on diagnosis, ris...
https://doi.org/10.1002/ajh.24880
American Journal of Hematology; Gotlib J

Oct 18th, 2017 - The eosinophilias encompass a broad range of nonhematologic (secondary or reactive) and hematologic (primary, clonal) disorders with potential for end-organ damage. Hypereosinophilia has generally been defined as a peripheral blood eosinophil count greater than 1500/mm3 and may be associated with tissue damage. After exclusion of secondary causes of eosinophilia, diagnostic evaluation of primar...

Congenital neutropenia in the era of genomics: classification, diagnosis, and natural h...
https://doi.org/10.1111/bjh.14887
British Journal of Haematology; Donadieu J, Beaupain B et. al.

Sep 6th, 2017 - This review focuses on the classification, diagnosis and natural history of congenital neutropenia (CN). CN encompasses a number of genetic disorders with chronic neutropenia and, for some, affecting other organ systems, such as the pancreas, central nervous system, heart, bone and skin. To date, 24 distinct genes have been associated with CN. The number of genes involved makes gene screening d...

ERS statement on protracted bacterial bronchitis in children.
https://doi.org/10.1183/13993003.02139-2016
The European Respiratory Journal; Kantar A, Chang AB et. al.

Aug 25th, 2017 - This European Respiratory Society statement provides a comprehensive overview on protracted bacterial bronchitis (PBB) in children. A task force of experts, consisting of clinicians from Europe and Australia who manage children with PBB determined the overall scope of this statement through consensus. Systematic reviews addressing key questions were undertaken, diagrams in accordance with the P...

Imported Infections with Mansonella perstans Nematodes, Italy.
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5572860
Emerging Infectious Diseases; Gobbi F, Beltrame A et. al.

Aug 18th, 2017 - We report 74 patients in Italy infected with Mansonella perstans nematodes, a poorly described filarial parasite. M. perstans nematodes should be included in the differential diagnosis for patients with eosinophilia from disease-endemic countries. Serologic analysis is useful for screening, and testing for microfilaremia in peripheral blood should be performed for parasite-positive patients.

Risk factors predisposing to the development of hypogammaglobulinemia and infections po...
https://doi.org/10.1080/08830185.2017.1346092
International Reviews of Immunology; Christou EAA, Giardino G et. al.

Aug 11th, 2017 - Rituximab (RTX) is a monoclonal antibody against CD20, commonly used in the treatment of hematological malignancies and autoimmune diseases. The use of RTX is related to the development of hypogammaglobulinemia and infections. Aim of this review is to summarize the evidence supporting the association of specific risk factors with the development of hypogammaglobulinemia and infections post-RTX....

Peripheral eosinophilia as the first manifestation of B-cell acute lymphoblastic leukem...
https://doi.org/10.1182/blood-2016-12-754812
Blood Toboso DG, Campos CB

Jul 21st, 2017 - Peripheral eosinophilia as the first manifestation of B-cell acute lymphoblastic leukemia with t(5;14)(q31;q32).|2017|Toboso DG,Campos CB,|genetics,ultrastructure,genetics,ultrastructure,metabolism,etiology,physiopathology,genetics,genetics,genetics,etiology,physiopathology,blood,genetics,

Congenital toxoplasmosis in Austria: Prenatal screening for prevention is cost-saving.
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5503164
PLoS Neglected Tropical Diseases; Prusa AR, Kasper DC et. al.

Jul 11th, 2017 - Primary infection of Toxoplasma gondii during pregnancy can be transmitted to the unborn child and may have serious consequences, including retinochoroiditis, hydrocephaly, cerebral calcifications, encephalitis, splenomegaly, hearing loss, blindness, and death. Austria, a country with moderate seroprevalence, instituted mandatory prenatal screening for toxoplasma infection to minimize the effec...

Hematopoietic stem cell transplantation from unrelated donors in children with DOCK8 de...
https://doi.org/10.1111/petr.13015
Pediatric Transplantation; Uygun DFK, Uygun V et. al.

Jun 30th, 2017 - DIDS is a unique form of combined immune deficiency characterized by an unusual susceptibility to cutaneous viral infections, severe allergies with eosinophilia and elevated immunoglobulin E titers, autoimmunity, and cancer. HSCT is considered the standard of care for this deadly disease. We have retrospectively analyzed the outcome of allogeneic HSCT from unrelated donors in patients with DIDS...

Efficacy of β-Lactam/β-Lactamase Inhibitor Combinations for the Treatment of Bloodstrea...
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5527609
Antimicrobial Agents and Chemotherapy; Gudiol C, Royo-Cebrecos C et. al.

Jun 6th, 2017 - β-Lactam/β-lactamase inhibitors (BLBLIs) were compared to carbapenems in two cohorts of hematological neutropenic patients with extended-spectrum-β-lactamase (ESBL) bloodstream infection (BSI): the empirical therapy cohort (174 patients) and the definitive therapy cohort (251 patients). The 30-day case fatality rates and other secondary outcomes were similar in the two therapy groups of the two...

Oral Glucocorticoid-Sparing Effect of Benralizumab in Severe Asthma.
https://doi.org/10.1056/NEJMoa1703501
The New England Journal of Medicine; Nair P, Wenzel S et. al.

May 22nd, 2017 - Many patients with severe asthma rely on oral glucocorticoids to manage their disease. We investigated whether benralizumab, a monoclonal antibody directed against the alpha subunit of the interleukin-5 receptor that significantly reduces the incidence of asthma exacerbations, was also effective as an oral glucocorticoid-sparing therapy in patients relying on oral glucocorticoids to manage seve...

Inflammatory bowel disease caused by primary immunodeficiencies-Clinical presentations,...
https://doi.org/10.1111/pai.12734
Pediatric Allergy and Immunology : Official Publication of the European Society of Pediatric Allergy and Immunology; Tegtmeyer D, Seidl M et. al.

May 17th, 2017 - Inflammatory bowel diseases (IBD) including Crohn's disease (CD) and ulcerative colitis have a multifactorial pathogenesis with complex interactions between polygenetic predispositions and environmental factors. However, IBD can also be caused by monogenic diseases, such as primary immunodeficiencies (PID). Recently, an increasing number of these altogether rare diseases have been described to ...

Risk factors of allopurinol-induced severe cutaneous adverse reactions in a Thai popula...
https://doi.org/10.1097/FPC.0000000000000285
Pharmacogenetics and Genomics; Saksit N, Tassaneeyakul W et. al.

May 16th, 2017 - Allopurinol is one of the most common causes of severe cutaneous adverse drug reactions (SCARs) including drug reactions with eosinophilia and systemic symptoms (DRESS), Stevens-Johnson syndrome (SJS), and toxic epidermal necrolysis (TEN). This study identified the risk factors associated with the development of allopurinol-induced SCARs in a Thai population. Eighty-six allopurinol-induced SCAR...

Tropical pulmonary eosinophilia masquerading as asthma in a 5-year-old girl.
https://doi.org/10.1080/20469047.2017.1319541
Paediatrics and International Child Health; Randev S, Kumar P et. al.

May 10th, 2017 - Tropical pulmonary eosinophilia (TPE) is an exaggerated immunological response to filarial antigens mainly seen in adults in endemic areas and is uncommonly reported in children. The diagnosis is frequently delayed as it mimics asthma clinically, while the chest radiograph might show miliary mottling which is often confused with that in pulmonary tuberculosis. A 5-year-old girl presented with e...

The effectiveness and safety of same-day versus next-day administration of long-acting ...
https://doi.org/10.1007/s00520-017-3703-y
Supportive Care in Cancer : Official Journal of the Multinational Association of Supportive Care in Cancer; Lyman GH, Allcott K et. al.

May 9th, 2017 - Granulocyte colony-stimulating factors (G-CSF) are commonly used in clinical practice to prevent febrile neutropenia (FN). US and EU prescribing information and treatment guidelines from the NCCN, ASCO, and EORTC specify that pegfilgrastim, a long-acting (LA) G-CSF, should be administered at least 24 h after myelosuppressive chemotherapy. Nevertheless, many patients receive LA G-CSFs on the sam...

A Case Report of Hypoglycemia and Hypogammaglobulinemia: DAVID Syndrome in a Patient Wi...
https://doi.org/10.1210/jc.2017-00341
The Journal of Clinical Endocrinology and Metabolism; Lal RA, Bachrach LK et. al.

May 4th, 2017 - Deficient anterior pituitary with variable immune deficiency (DAVID) syndrome is a rare disorder in which children present with symptomatic adrenocorticotropic hormone (ACTH) deficiency preceded by hypogammaglobulinemia from B-cell dysfunction with recurrent infections, called common variable immunodeficiency (CVID). Subsequent whole exome sequencing studies have revealed germline heterozygous ...

Invasive pulmonary mucormycosis: rare presentation with pulmonary eosinophilia.
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5410085
BMC Pulmonary Medicine; Hirano T, Yamada M et. al.

Apr 29th, 2017 - Fungi can cause a variety of infectious diseases, including invasive mycosis and non-invasive mycosis, as well as allergic diseases. The different forms of mycosis usually have been described as mutually exclusive, independent entities, with few descriptions of overlapping cases. Here, we describe the first reported case of a patient with the complication of pulmonary eosinophilia in the course...

Prospective evaluation of the diagnostic value of sensitive KIT D816V mutation analysis...
https://doi.org/10.1111/all.13187
Allergy Kristensen T, Vestergaard H et. al.

Apr 22nd, 2017 - Sensitive KIT D816V mutation analysis of blood has been proposed to guide bone marrow (BM) investigation in suspected systemic mastocytosis (SM). The aim of this prospective study was for the first time to compare the D816V status of the "screening blood sample" used to guide BM biopsy in suspected SM to the outcome of the subsequent BM investigation. Fifty-eight adult patients with suspected S...

Response and progression on midostaurin in advanced systemic mastocytosis: KIT D816V an...
https://doi.org/10.1182/blood-2017-01-764423
Blood Jawhar M, Schwaab J et. al.

Apr 20th, 2017 - In advanced systemic mastocytosis (advSM), disease evolution is often triggered by KIT mutations (D816V in >80% of cases) and by additional mutations (eg, in SRSF2, ASXL1, and/or RUNX1 [S/A/Rpos in >60% of cases]). In a recently reported phase 2 study, midostaurin, a multikinase/KIT inhibitor, demonstrated an overall response rate (ORR) of 60% in advSM but biomarkers predictive of response are ...

Prevalence of Atypical Pathogens in Patients With Cough and Community-Acquired Pneumoni...
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5389400
Annals of Family Medicine; Marchello C, Dale AP et. al.

Apr 4th, 2017 - Community-acquired pneumonia (CAP), acute cough, bronchitis, and lower respiratory tract infections (LRTI) are often caused by infections with viruses or Streptococcus pneumoniae. The prevalence of atypical pathogens Mycoplasma pneumoniae, Chlamydophila pneumoniae, Legionella pneumophila, and Bordetella pertussis among patients with these illnesses in the ambulatory setting has not been previou...

Association of the HLA-B*53:01 Allele With Drug Reaction With Eosinophilia and Systemic...
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5399947
Clinical Infectious Diseases : an Official Publication of the Infectious Diseases Society of America; Thomas M, Hopkins C et. al.

Apr 3rd, 2017 - Drug reaction with eosinophilia and systemic symptoms (DRESS) syndrome is a rare, severe adverse event during treatment with raltegravir. The occurrence of DRESS syndrome during treatment with other drugs is strongly associated with particular HLA alleles. We performed HLA testing in 3 of the 5 patients previously reported to have developed raltegravir-induced DRESS syndrome and in 1 previously...

Immunodeficiencies Associated with Abnormal Newborn Screening for T Cell and B Cell Lym...
https://doi.org/10.1007/s10875-017-0388-4
Journal of Clinical Immunology; Jyonouchi S, Jongco AM et. al.

Mar 29th, 2017 - Newborn screening for SCID has revealed the association of low T cells with a number of unexpected syndromes associated with low T cells, some of which were not appreciated to have this feature. This review will discuss diagnostic approaches and the features of some of the syndromes likely to be encountered following newborn screening for immune deficiencies.

Fibromyalgia prevalence and associated factors in primary Sjögren's syndrome patients i...
https://www.ncbi.nlm.nih.gov/pubmed/28240585
Clinical and Experimental Rheumatology; Torrente-Segarra V, Corominas H et. al.

Feb 27th, 2017 - To assess fibromyalgia (FM) prevalence in a large cohort of primary Sjögren's syndrome patients (pSS) from a National Database. Data included in the national retrospective register of pSS patients of the Spanish Society of Rheumatology (SJOGRENSER) were analysed. 437 pSS patients were included and a 14.6% of FM prevalence was found. FM-pSS patients significantly showed more constitutional, fati...

Gastrointestinal Manifestations in X-linked Agammaglobulinemia.
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5414010
Journal of Clinical Immunology; Barmettler S, Otani IM et. al.

Feb 26th, 2017 - X-linked agammaglobulinemia is a primary humoral immunodeficiency characterized by hypogammaglobulinemia and increased susceptibility to infection. Although there is increased awareness of autoimmune and inflammatory complications in X-linked agammaglobulinemia (XLA), the spectrum of gastrointestinal manifestations has not previously been fully explored. We present a case report of a family wit...

A Review of Clinical and Imaging Findings in Eosinophilic Lung Diseases.
https://doi.org/10.2214/AJR.16.17315
AJR. American Journal of Roentgenology; Bernheim A, McLoud T

Feb 22nd, 2017 - The purpose of this article is to review the clinical and imaging findings associated with eosinophilic lung diseases. The spectrum of eosinophilic lung diseases comprises a diverse group of pulmonary disorders that have an association with tissue or peripheral eosinophilia. These diseases have varied clinical presentations and may be associated with several other abnormalities. Characteristic ...

Acute Liver Failure/Injury Related to Drug Reaction With Eosinophilia and Systemic Symp...
https://doi.org/10.1097/TP.0000000000001655
Transplantation Ichai P, Laurent-Bellue A et. al.

Feb 16th, 2017 - Drug reaction with eosinophilia and systemic symptoms (DRESS) is a rare severe adverse drug-induced reaction with multiorgan involvement. The outcome and prediction of those patients who develop severe acute liver injury (sALI) or acute liver failure (ALF) remain little known. A multicenter retrospective study of patients admitted with a diagnosis of DRESS-related sALI or ALF. Histological revi...

Autoimmune and inflammatory manifestations occur frequently in patients with primary im...
https://doi.org/10.1016/j.jaci.2016.12.978
The Journal of Allergy and Clinical Immunology; Fischer A, Provot J et. al.

Feb 13th, 2017 - Primary immunodeficiencies (PIDs) are inherited diseases associated with a considerable increase in susceptibility to infections. It is known that PIDs can also predispose to cancer and immune diseases, including allergy, autoimmunity, and inflammation. We aimed at determining the incidence of autoimmunity and inflammation in patients with PIDs. We have retrospectively screened 2183 consecutive...

Eosinophilic Meningitis Due to Infection With Paragonimus kellicotti.
https://doi.org/10.1093/cid/cix102
Clinical Infectious Diseases : an Official Publication of the Infectious Diseases Society of America; Bahr NC, Trotman RL et. al.

Feb 5th, 2017 - Paragonimus kellicotti is an emerging pathogen in the United States with 19 previously reported cases, most in Missouri. Pulmonary symptoms with eosinophilia are most common, though 1 case did involve the central nervous system with few symptoms. We describe the first 2 cases of eosinophilic meningitis due to Paragonimus kellicotti.

Management of Children With Chronic Wet Cough and Protracted Bacterial Bronchitis: CHES...
https://doi.org/10.1016/j.chest.2017.01.025
Chest Chang AB, Oppenheimer JJ et. al.

Feb 1st, 2017 - Wet or productive cough is common in children with chronic cough. We formulated recommendations based on systematic reviews related to the management of chronic wet cough in children (aged ≤ 14 years) based on two key questions: (1) how effective are antibiotics in improving the resolution of cough? If so, what antibiotic should be used and for how long? and (2) when should children be referred...

Incidence of Severe Neutropenia in HIV-Infected People Starting Antiretroviral Therapy ...
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5266303
PloS One; Leroi C, Balestre E et. al.

Jan 25th, 2017 - In sub-Saharan Africa, antiretroviral therapy (ART) including drugs with potential toxicity such as Zidovudine (ZDV) are routinely prescribed. This study aimed at estimating the incidence of severe neutropenia and associated factors after ART initiation in five West African countries. A retrospective cohort analysis was conducted within the international epidemiologic database to evaluate AIDS ...

A human immunodeficiency syndrome caused by mutations in CARMIL2.
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5473639
Nature Communications; Schober T, Magg T et. al.

Jan 23rd, 2017 - Human T-cell function is dependent on T-cell antigen receptor (TCR) and co-signalling as evidenced by immunodeficiencies affecting TCR-dependent signalling pathways. Here, we show four human patients with EBV+ disseminated smooth muscle tumours that carry two homozygous loss-of-function mutations in the CARMIL2 (RLTPR) gene encoding the capping protein regulator and myosin 1 linker 2. These pat...

Diagnostic accuracy of fractional exhaled nitric oxide measurement in predicting cough-...
https://doi.org/10.1016/j.jaci.2016.11.037
The Journal of Allergy and Clinical Immunology; Song WJ, Kim HJ et. al.

Jan 15th, 2017 - Individual studies have suggested the utility of fractional exhaled nitric oxide (Feno) measurement in detecting cough-variant asthma (CVA) and eosinophilic bronchitis (EB) in patients with chronic cough. We sought to obtain summary estimates of diagnostic test accuracy of Feno measurement in predicting CVA, EB, or both in adults with chronic cough. Electronic databases were searched for studie...

Masitinib for treatment of severely symptomatic indolent systemic mastocytosis: a rando...
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5985971
Lancet (London, England); Lortholary O, Chandesris MO et. al.

Jan 10th, 2017 - Indolent systemic mastocytosis, including the subvariant of smouldering systemic mastocytosis, is a lifelong condition associated with reduced quality of life. Masitinib inhibits KIT and LYN kinases that are involved in indolent systemic mastocytosis pathogenesis. We aimed to assess safety and efficacy of masitinib versus placebo in severely symptomatic patients who were unresponsive to optimal...

Interventional Treatment of Pulmonary Lymphatic Anomalies.
https://doi.org/10.1053/j.tvir.2016.10.005
Techniques in Vascular and Interventional Radiology; Itkin M

Dec 20th, 2016 - Pulmonary lymphatic diseases have been recognized for many years and have been referred as pulmonary lymphangiectasia, pulmonary lymphangiomatosis, plastic bronchitis, and idiopathic chylothorax or chylopericardium. The lymphatic etiology of these conditions has been determined by detection of cystic lymphatic structures on biopsy or postmortem examination. Development of new imaging techniques...

Acute Bronchitis.
https://www.ncbi.nlm.nih.gov/pubmed/27929206
American Family Physician; Kinkade S, Long NA

Dec 8th, 2016 - Cough is the most common illness-related reason for ambulatory care visits in the United States. Acute bronchitis is a clinical diagnosis characterized by cough due to acute inflammation of the trachea and large airways without evidence of pneumonia. Pneumonia should be suspected in patients with tachypnea, tachycardia, dyspnea, or lung findings suggestive of pneumonia, and radiography is warra...

A potential founder variant in CARMIL2/RLTPR in three Norwegian families with warts, mo...
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5118205
Molecular Genetics & Genomic Medicine; Sorte HS, Osnes LT et. al.

Nov 29th, 2016 - Four patients from three Norwegian families presented with a common skin phenotype of warts, molluscum contagiosum, and dermatitis since early childhood, and various other immunological features. Warts are a common manifestation of human papilloma virus (HPV), but when they are overwhelming, disseminated and/or persistent, and presenting together with other immunological features, a primary imm...

Budesonide Oral Suspension Improves Symptomatic, Endoscopic, and Histologic Parameters ...
https://doi.org/10.1053/j.gastro.2016.11.021
Gastroenterology Dellon ES, Katzka DA et. al.

Nov 27th, 2016 - Pharmacologic treatment of eosinophilic esophagitis (EoE) is limited to off-label use of corticosteroids not optimized for esophageal delivery. We performed a randomized, controlled phase 2 trial to assess the ability of budesonide oral suspension (BOS), a novel muco-adherent topical steroid formulation, to reduce symptoms and esophageal eosinophilia in adolescents and adults with EoE. In this ...

Newborn Screening for Severe Primary Immunodeficiency Diseases in Sweden-a 2-Year Pilot...
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5226987
Journal of Clinical Immunology; Barbaro M, Ohlsson A et. al.

Nov 22nd, 2016 - Newborn screening for severe primary immunodeficiencies (PID), characterized by T and/or B cell lymphopenia, was carried out in a pilot program in the Stockholm County, Sweden, over a 2-year period, encompassing 58,834 children. T cell receptor excision circles (TREC) and kappa-deleting recombination excision circles (KREC) were measured simultaneously using a quantitative PCR-based method on D...

A prospective randomized trial comparing piperacillin/tazobactam with meropenem as empi...
https://doi.org/10.1002/pbc.26360
Pediatric Blood & Cancer; Sano H, Kobayashi R et. al.

Nov 22nd, 2016 - This randomized prospective study was designed to assess whether piperacillin/tazobactam (PIPC/TAZ) is as effective as meropenem (MEPM) as a first-line antibiotic treatment for febrile neutropenia (FN). FN episodes were randomly assigned to receive either PIPC/TAZ (337.5 mg/kg per day in three doses, 1-hr DIV, maximum 13.5 g per day) or MEPM (120 mg/kg per day in three doses, 1-hr DIV, maximum ...

Febuxostat hypersensitivity: another cause of DRESS syndrome in chronic kidney disease?
https://www.ncbi.nlm.nih.gov/pubmed/27852432
European Annals of Allergy and Clinical Immunology; Paschou E, Gavriilaki E et. al.

Nov 17th, 2016 - Febuxostat is a xanthine oxidase inhibitor that during the last years has successfully replaced allopurinol treatment in patients with chronic kidney disease (CKD) and hyperuricemia. Several adverse events have been observed during therapy with febuxostat. DRESS (Drug Reaction with Eosinophilia and Systemic Symptoms) syndrome induced by febuxostat has been poorly described, mainly in patient wi...

Pomalidomide in myeloproliferative neoplasm-associated myelofibrosis.
https://doi.org/10.1038/leu.2016.299
Leukemia Schlenk RF, Stegelmann F et. al.

Oct 24th, 2016 - Myeloproliferative neoplasm (MPN)-associated myelofibrosis is a MPN characterized by bone marrow fibrosis, cytopenias, splenomegaly and constitutional symptoms. Pomalidomide, an immune-modifying drug, is reported to improve anaemia and thrombocytopenia in some patients with MPN-associated myelofibrosis. We designed a phase 2 study of pomalidomide in patients with MPN-associated myelofibrosis an...

Risk of solid cancer, cardiovascular disease, anaphylaxis, osteoporosis and fractures i...
https://doi.org/10.1002/ajh.24490
American Journal of Hematology; Broesby-Olsen S, Farkas DK et. al.

Oct 20th, 2016 - In patients with systemic mastocytosis (SM), several aspects of morbidity remain poorly understood. We assessed the risk of solid cancers, cardiovascular disease, anaphylaxis, osteoporosis, and fractures in SM patients. Using Danish medical registries, we conducted a nationwide population-based cohort study including 687 adult (≥15 years) SM patients diagnosed during 1997-2012. A comparison coh...

Systemic mastocytosis in adults: 2017 update on diagnosis, risk stratification and mana...
https://doi.org/10.1002/ajh.24553
American Journal of Hematology; Pardanani A

Oct 20th, 2016 - Disease overview:Systemic mastocytosis (SM) results from a clonal proliferation of abnormal mast cells (MC) in one or more extra-cutaneous organs. The major criterion is presence of multifocal clusters of morphologically abnormal MC in the bone marrow. Minor diagnostic criteria include elevated serum tryptase level, abnormal MC expression of CD25 and/or CD2, and presence of KITD816V. Risk strat...

Efficacy, safety, tolerability and pharmacokinetics of a novel human immune globulin su...
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5167020
Clinical and Experimental Immunology; Borte M, Kriván G et. al.

Oct 18th, 2016 - A highly concentrated (20%) immunoglobulin (Ig)G preparation for subcutaneous administration (IGSC 20%), would offer a new option for antibody replacement therapy in patients with primary immunodeficiency diseases (PIDD). The efficacy, safety, tolerability and pharmacokinetics of IGSC 20% were evaluated in a prospective trial in Europe in 49 patients with PIDD aged 2-67 years. Over a median of ...

Well-differentiated systemic mastocytosis showed excellent clinical response to imatini...
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5072932
Medicine Huang L, Wang SA et. al.

Oct 15th, 2016 - Well-differentiated systemic mastocytosis (WDSM) is a rare, recently recognized provisional subvariant of systemic mastocytosis (SM). We report a case of WDSM that showed excellent clinical and cutaneous response to imatinib in the absence of known molecular genetic abnormalities. We present a 24-year-old woman with childhood onset of skin manifestations that progressed to mediator-related syst...

Physical health conditions and quality of life in adults with primary immunodeficiency ...
https://doi.org/10.1016/j.jaci.2016.08.027
The Journal of Allergy and Clinical Immunology; Barlogis V, Mahlaoui N et. al.

Oct 4th, 2016 - Most children with primary immunodeficiencies (PIDs) now reach adulthood. However, few studies have evaluated their health status and health-related quality of life (HRQoL). To investigate long-term morbidity, the French Reference Center for PIDs initiated a prospective multicenter cohort: the French Childhood Immune Deficiency Long-term Cohort. The data collected were used to assess the physic...

Evaluation of the effects and adverse drug reactions of low-dose dexamethasone premedic...
https://doi.org/10.1007/s00520-016-3420-y
Supportive Care in Cancer : Official Journal of the Multinational Association of Supportive Care in Cancer; Kang RY, Yoo KS et. al.

Sep 30th, 2016 - A weekly docetaxel regimen had comparable efficacy with a tri-weekly schedule and caused significantly less severe neutropenia and febrile neutropenia. Therefore, a weekly docetaxel regimen has become increasingly common in cancer treatment. Premedication with corticosteroids can effectively prevent or reduce the severity of hypersensitivity and fluid retention. However, no recommended steroid ...

Augmented Renal Clearance in Patients With Febrile Neutropenia is Associated With Incre...
https://doi.org/10.1097/FTD.0000000000000346
Therapeutic Drug Monitoring; Hirai K, Ishii H et. al.

Sep 29th, 2016 - Augmented renal clearance (ARC) has frequently been observed in critically ill patients. The risk factors for ARC in patients, including those in the general ward, and their influences on vancomycin (VCM) treatment remain unclear. The aims of this study were to investigate the risk factors for ARC and to evaluate the influence of ARC on the pharmacokinetic parameters of VCM. This study included...

Antimold Prophylaxis May Reduce the Risk of Invasive Fusariosis in Hematologic Patients...
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5119009
Antimicrobial Agents and Chemotherapy; Varon AG, Nouér SA et. al.

Sep 27th, 2016 - Hematologic patients with superficial skin lesions on admission growing Fusarium spp. are at a high risk for developing invasive fusariosis during neutropenia. We evaluated the impact of primary prophylaxis with a mold-active azole in preventing invasive fusariosis in these patients. Between August 2008 and December 2014, patients with acute leukemia or aplastic anemia and recipients of hematop...

Periodontal and other oral manifestations of immunodeficiency diseases.
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5352551
Oral Diseases; Peacock ME, Arce RM et. al.

Sep 15th, 2016 - The list of immunodeficiency diseases grows each year as novel disorders are discovered, classified, and sometimes reclassified due to our ever-increasing knowledge of immune system function. Although the number of patients with secondary immunodeficiencies (SIDs) greatly exceeds those with primary immunodeficiencies (PIDs), the prevalence of both appears to be on the rise probably because of s...

US-Guided, Direct Puncture Retrograde Thoracic Duct Access, Lymphangiography, and Embol...
https://doi.org/10.1016/j.jvir.2016.06.030
Journal of Vascular and Interventional Radiology : JVIR; Guevara CJ, Rialon KL et. al.

Sep 6th, 2016 - To describe technical details, success rate, and advantages of direct puncture of the thoracic duct (TD) under direct ultrasound (US) guidance at venous insertion in the left neck. All patients who underwent attempted thoracic duct embolization (TDE) via US-guided retrograde TD access in the left neck were retrospectively reviewed. Indications for lymphangiography were iatrogenic chyle leak, pu...

Failing phrenics: an obscure cause of exertional dyspnea: Case report and literature re...
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5265774
Medicine Rafiq A, Ijaz M et. al.

Jul 21st, 2016 - Idiopathic phrenic nerve palsy is a rare cause of exertional dyspnea. We present a case of a patient presenting with worsening dyspnea of an unknown etiology found to be related to bilateral phrenic nerve palsy. Forty-two-year-old man presented to our emergency department with exertional dyspnea, orthopnea, and a left lower lobe consolidation treated initially as bronchitis by his primary physi...

Cyclosporine Treatment of Drug-Induced Hypersensitivity Syndrome.
https://doi.org/10.1001/jamadermatol.2016.2220
JAMA Dermatology; Kirchhof MG, Wong A et. al.

Jul 20th, 2016 - Drug-induced hypersensitivity syndrome (DIHS), also known as drug reaction with eosinophilia and systemic symptoms (DRESS) syndrome, is a potentially life-threatening reaction to medications with a mortality rate up to 10%. Standard therapy involves the use of systemic corticosteroids with tapering doses extending up to 9 months after the initial reaction. Alternative treatments for DIHS are ne...

Deferiprone-induced agranulocytosis: 20 years of clinical observations.
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5129477
American Journal of Hematology; Tricta F, Uetrecht J et. al.

Jul 14th, 2016 - Use of the iron chelator deferiprone for treatment of iron overload in thalassemia patients is associated with concerns over agranulocytosis, which requires weekly absolute neutrophil counts (ANC). Here, we analyze all episodes of agranulocytosis (n = 161) and neutropenia (n = 250) during deferiprone use in clinical trials (CT) and postmarketing surveillance programs (PMSP). Rates of agranulocy...

FDA Approval: Uridine Triacetate for the Treatment of Patients Following Fluorouracil o...
https://doi.org/10.1158/1078-0432.CCR-16-0638
Clinical Cancer Research : an Official Journal of the American Association for Cancer Research; Ison G, Beaver JA et. al.

Jul 12th, 2016 - On December 11, 2015, the FDA approved uridine triacetate (VISTOGARD; Wellstat Therapeutics Corporation) for the emergency treatment of adult and pediatric patients following a fluorouracil or capecitabine overdose regardless of the presence of symptoms, and of those who exhibit early-onset, severe, or life-threatening toxicity affecting the cardiac or central nervous system, and/or early onset...

Immunoglobulin A deficiency following treatment with lamotrigine.
https://doi.org/10.1016/j.braindev.2016.06.006
Brain & Development; Maruyama S, Okamoto Y et. al.

Jul 11th, 2016 - Lamotrigine (LTG) is an anti-epileptic drug and mood-stabilizing agent, whose adverse effects include skin rash and dizziness. Interactions with the immune system are rare, and only a few cases linking hypogammaglobulinemia to LTG treatment have been previously described. In this report, we describe a case in which a patient developed hypogammaglobulinemia, and a subsequent immunoglobulin A (Ig...

Heterozygous Loss-of-Function SEC61A1 Mutations Cause Autosomal-Dominant Tubulo-Interst...
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5005467
American Journal of Human Genetics; Bolar NA, Golzio C et. al.

Jul 8th, 2016 - Autosomal-dominant tubulo-interstitial kidney disease (ADTKD) encompasses a group of disorders characterized by renal tubular and interstitial abnormalities, leading to slow progressive loss of kidney function requiring dialysis and kidney transplantation. Mutations in UMOD, MUC1, and REN are responsible for many, but not all, cases of ADTKD. We report on two families with ADTKD and congenital ...

Expanding the Phenotype of TRNT1-Related Immunodeficiency to Include Childhood Cataract...
https://doi.org/10.1001/jamaophthalmol.2015.5833
JAMA Ophthalmology; Hull S, Malik AN et. al.

Jul 8th, 2016 - A multiorgan syndromic disorder characterized by sideroblastic anemia, immunodeficiency, periodic fever, and developmental delay with an uncharacterized retinal dystrophy is caused by TRNT1. This report of a family with a homozygous mutation in TRNT1 expands the ocular phenotype to include cataract and inner retinal dysfunction and details a mild systemic phenotype. A consanguineous family with...

Drug-specific CD4+ T-cell immune responses are responsible for antituberculosis drug-in...
https://doi.org/10.1111/bjd.14839
The British Journal of Dermatology; Ye YM, Hur GY et. al.

Jul 4th, 2016 - A multidrug regimen including isoniazid, rifampicin, pyrazinamide and ethambutol is commonly used as first-line treatment for tuberculosis. However, this regimen can occasionally result in severe adverse drug reactions, such as drug reaction with eosinophilia and systemic symptoms (DRESS) syndrome and drug-induced liver injury. The culprit drug and mechanistic basis for the hypersensitive react...

Efficacy and Safety of Midostaurin in Advanced Systemic Mastocytosis.
https://doi.org/10.1056/NEJMoa1513098
The New England Journal of Medicine; Gotlib J, Kluin-Nelemans HC et. al.

Jun 29th, 2016 - Advanced systemic mastocytosis comprises rare hematologic neoplasms that are associated with a poor prognosis and lack effective treatment options. The multikinase inhibitor midostaurin inhibits KIT D816V, a primary driver of disease pathogenesis. We conducted an open-label study of oral midostaurin at a dose of 100 mg twice daily in 116 patients, of whom 89 with mastocytosis-related organ dama...

Endocrine manifestations of systemic mastocytosis in bone.
https://doi.org/10.1007/s11154-016-9362-3
Reviews in Endocrine & Metabolic Disorders; Greene LW, Asadipooya K et. al.

May 30th, 2016 - Systemic Mastocytosis (SM) is characterized by accumulation of clonal, neoplastic proliferations of abnormal mast cells (MC) in one or more organ system other than skin. Presence of these multifocal clusters of abnormal mast cells is an essential feature of SM. Frequently associated with D816V (KIT) mutation, the presence of this mutation and elevated serum tryptase are minor criteria for diagn...