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Treatment of CVID children

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https://www.readbyqxmd.com/read/27357411/comprehensive-activities-to-increase-recognition-of-primary-immunodeficiency-and-access-to-immunoglobulin-replacement-therapy-in-poland
#1
Małgorzata Pac, Ewa Bernatowska
UNLABELLED: The study presents an overview on current situation of primary immunodeficiency (PID) patients in Poland and the 2014 annual report of the Polish Working Group for Immunodeficiency (PWGID). The group was set up in 2005 to improve diagnosis, treatment, and care of patients with immunodeficiencies and currently includes 15 pediatric and 13 adult centers. According to PWGID report 4099, PID patients are recognized in Poland, with the prevalence 10.6/100,000. The majority of them (54...
August 2016: European Journal of Pediatrics
https://www.readbyqxmd.com/read/27276370/common-variable-immunodeficiency-immune-thrombocytopenia-rituximab-and-splenectomy-important-considerations
#2
Ruta Arays, Sahil Goyal, Kim M Jordan
Common variable immunodeficiency (CVID) is readily considered in patients presenting with recurrent sino-pulmonary infections, however this disease has a broad range of clinical manifestations and diagnosis can be delayed by several years. We present the case of a 44-year-old postpartum female who presented with nausea, vomiting and abdominal distension. Four years prior, she was hospitalized for treatment of immune thrombocytopenia (ITP) with splenectomy and rituximab followed by two episodes of bacterial meningitis despite immunizations...
August 2016: Postgraduate Medicine
https://www.readbyqxmd.com/read/26868026/common-variable-immunodeficiency
#3
REVIEW
Biman Saikia, Sudhir Gupta
Common variable immunodeficiency (CVID) is the most common primary immunodeficiency of young adolescents and adults which also affects the children. The disease remains largely under-diagnosed in India and Southeast Asian countries. Although in majority of cases it is sporadic, disease may be inherited in a autosomal recessive pattern and rarely, in autosomal dominant pattern. Patients, in addition to frequent sino-pulmonary infections, are also susceptible to various autoimmune diseases and malignancy, predominantly lymphoma and leukemia...
April 2016: Indian Journal of Pediatrics
https://www.readbyqxmd.com/read/26831679/respiratory-manifestations-and-management-in-children-with-common-variable-immunodeficiency
#4
REVIEW
Chetan Pandit, Peter Hsu, Peter van Asperen, Sam Mehr
Common variable immunodeficiency is an antibody deficiency that usually presents in childhood with recurrent sino-pulmonary infections. Diagnostic delay is frequent and thus respiratory morbidity is common, ranging from recurrent suppurative bronchitis to bronchiectasis. Immunoglobulin replacement therapy is the mainstay of treatment, whilst prophylactic antibiotic therapy and muco-ciliary clearance are additional treatment options. This review examines the diagnosis and management of respiratory issues in children with CVID...
June 2016: Paediatric Respiratory Reviews
https://www.readbyqxmd.com/read/26504182/sirolimus-is-effective-in-relapsed-refractory-autoimmune-cytopenias-results-of-a-prospective-multi-institutional-trial
#5
MULTICENTER STUDY
Karen L Bride, Tiffaney Vincent, Kim Smith-Whitley, Michele P Lambert, Jack J Bleesing, Alix E Seif, Catherine S Manno, James Casper, Stephan A Grupp, David T Teachey
Patients with autoimmune multilineage cytopenias are often refractory to standard therapies requiring chronic immunosuppression with medications with limited efficacy and high toxicity. We present data on 30 patients treated on a multicenter prospective clinical trial using sirolimus as monotherapy. All children (N = 12) with autoimmune lymphoproliferative syndrome (ALPS) achieved a durable complete response (CR), including rapid improvement in autoimmune disease, lymphadenopathy, and splenomegaly within 1 to 3 months of starting sirolimus...
January 7, 2016: Blood
https://www.readbyqxmd.com/read/25400867/nodular-lymphoid-hyperplasia-in-the-gastrointestinal-tract-in-adult-patients-a-review
#6
REVIEW
Andreia Albuquerque
Nodular lymphoid hyperplasia of the gastrointestinal tract is characterized by the presence of multiple small nodules, normally between between 2 and 10 mm in diameter, distributed along the small intestine (more often), stomach, large intestine, or rectum. The pathogenesis is largely unknown. It can occur in all age groups, but primarily in children and can affect adults with or without immunodeficiency. Some patients have an associated disease, namely, common variable immunodeficiency, selective IgA deficiency, Giardia infection, or, more rarely, human immunodeficiency virus infection, celiac disease, or Helicobacter pylori infection...
November 16, 2014: World Journal of Gastrointestinal Endoscopy
https://www.readbyqxmd.com/read/25318568/primary-immunodeficiency-in-infection-prone-children-in-southern-sweden-occurrence-clinical-characteristics-and-immunological-findings
#7
Nicholas Brodszki, Göran Jönsson, Lillemor Skattum, Lennart Truedsson
BACKGROUND: Primary immunodeficiency diseases (PIDs) comprise a heterogeneous group of disorders mainly characterized by increased susceptibility to infections. The aims of this study were to estimate the occurrence rate of PID in the paediatric (age ≤ 18 years) population of southern Sweden (approx. 265,000 children) and to describe their demographic, clinical and immunological characteristics. During a period of 4 years, in four paediatric speciality clinics in Skåne County in southern Sweden, children being seen for infections and fulfilling specific criteria were evaluated according to a predefined examination schedule...
2014: BMC Immunology
https://www.readbyqxmd.com/read/23647505/characteristics-of-children-with-non-hodgkin-lymphoma-associated-with-primary-immune-deficiency-diseases-descriptions-of-five-patients
#8
Suna Emir, Emine Vezir, Dilek Azkur, Hacı Ahmet Demir, Ayşe Metin
BACKGROUND: An increased incidence of non-Hodgkin lymphoma (NHL) has been seen in various primary immune deficiency (PID) cases. The present study aimed to evaluate the clinical characteristics and treatment outcomes of five cases with NHL associated with primary immunodeficiency. METHODS: We retrospectively evaluated five patients with primary immunodeficiency who developed NHL. Two patients had ataxia-telangiectasia (A-T), one patient had common variable immunodeficiency (CVID), one patient had Bloom's Syndrome, and one patient had Wiskott-Aldrich syndrome (WAS)...
September 2013: Pediatric Hematology and Oncology
https://www.readbyqxmd.com/read/23233596/the-many-faces-of-common-variable-immunodeficiency
#9
REVIEW
Charlotte Cunningham-Rundles
Common variable immunodeficiency (CVID) is a rare immune deficiency characterized by low levels of serum IgG, IgA, and/or IgM, with a loss of Ab production. The diagnosis is most commonly made in adults between the ages of 20 and 40 years, but both children and much older adults can be found to have this immune defect. The range of clinical manifestations is broad, including acute and chronic infections, inflammatory and autoimmune diseases, and an increased incidence of cancer and lymphoma. For all of these reasons, the disease phenotype is both heterogeneous and complex...
2012: Hematology—the Education Program of the American Society of Hematology
https://www.readbyqxmd.com/read/23054346/primary-immunodeficiency-diseases-in-children-15-year-experience-in-a-tertiary-care-medical-center-in-qatar
#10
Mohammad S Ehlayel, Abdulbari Bener, Mohammad Abu Laban
BACKGROUND: Primary immunodeficiency diseases (PID) are a group of heterogeneous, rare, genetic, mainly childhood disorders that affect specific components of immune system leading to serious complications. OBJECTIVES: This study is aimed at describing the prevalence and the categories of PID, the ages of onset and the diagnosis, the clinical presentations, the treatment modalities and the overall outcome of affected patients. MATERIALS AND METHODS: A retrospective study was conducted on 131 pediatric patients (aged 0-14 years) diagnosed with PID at Hamad General Hospital during a 15-year period (1998-2012)...
February 2013: Journal of Clinical Immunology
https://www.readbyqxmd.com/read/22312934/efficacy-of-intravenous-immunoglobulin-treatment-in-children-with-common-variable-immunodeficiency
#11
S Baris, H Ercan, H Hasret Cagan, A Ozen, E Karakoc-Aydiner, C Ozdemir, N N Bahceciler
BACKGROUND: Children with common variable immunodeficiency (CVID) have increased susceptibility to infections. OBJECTIVE: We evaluated the role of intravenous immunoglobulin (IVIG) replacement therapy on the clinical outcome of patients with CVID. METHODS: We studied children diagnosed with CVID and treated with IVIG (500 mg/kg every 3 weeks). RESULTS: The study population comprised 29 children with CVID (mean [SD] age, 11...
2011: Journal of Investigational Allergology & Clinical Immunology
https://www.readbyqxmd.com/read/22207346/development-of-common-variable-immunodeficiency-in-iga-and-igg2-deficient-patients-with-systemic-lupus-erythematosus
#12
Kazuhide Suyama, Yukihiko Kawasaki, Yusaku Abe, Masahiro Watanabe, Shinichiro Ohara, Tomoko Oikawa, Nobuko Sakai, Kouichi Hashimoto, Mitsuaki Hosoya
BACKGROUND: There have been few reports on children who developed common variable immunodeficiency (CVID) in association with immunoglobulin A (IgA) and IgG2 deficiencies and systemic lupus erythematosus (SLE). CASE-DIAGNOSIS/TREATMENT: Our patient experienced nephrotic syndrome and acute respiratory distress syndrome (ARDS) caused by influenza A/H1N1 virus infection at 5 years of age. A diagnosis of IgA and IgG2 deficiency and SLE was made on the basis of severe proteinuria, hematuria, hypocomplementemia, high anti-DNA antibody and antinuclear antibody (ANA) titers, and malar rash...
March 2012: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/21995182/the-use-of-complementary-and-alternative-medicine-in-patients-with-common-variable-immunodeficiency
#13
Y Karali, H Saglam, Z Karali, S S Kilic
OBJECTIVES: Complementary and alternative medicine (CAM) usage is a reality in patients with chronic diseases, but there are no data on CAM usage in immunodeficiency diseases necessitating intravenous immunoglobulin (IVIG) therapy.The aim of this study was to investigate the rate of CAM usage in patients with common variable immunodeficiency (CVID). METHODS: Forty-three patients (29 boys and 14 girls) with CVID and receiving IVIG every 3 weeks were included. Data were collected through a questionnaire completed by the parents...
2011: Journal of Investigational Allergology & Clinical Immunology
https://www.readbyqxmd.com/read/21981575/efficacy-and-safety-of-rituximab-in-common-variable-immunodeficiency-associated-immune-cytopenias-a-retrospective-multicentre-study-on-33-patients
#14
MULTICENTER STUDY
Delphine Gobert, James B Bussel, Charlotte Cunningham-Rundles, Lionel Galicier, Agnès Dechartres, Alice Berezne, Bernard Bonnotte, Thierry DeRevel, Christophe Auzary, Roland Jaussaud, Claire Larroche, Alain LeQuellec, Marc Ruivard, Pascal Seve, Amar Smail, Jean-François Viallard, Bertrand Godeau, Olivier Hermine, Marc Michel
Patients with common variable immunodeficiency (CVID) are at high risk of developing immune thrombocytopenia (ITP) and/or autoimmune haemolytic anaemia (AHA). Given their underlying immunodeficiency, immunosuppressive treatment of these manifestations may increase the risk of infection. To assess efficacy and safety of rituximab in patients with CVID-associated ITP/AHA, a multicentre retrospective study was performed. Thirty-three patients, 29 adults and four children, were included. Patients received an average of 2·6 treatments prior to rituximab including steroids, intravenous immunoglobulin and splenectomy (21%)...
November 2011: British Journal of Haematology
https://www.readbyqxmd.com/read/21960740/proteomic-analysis-of-sera-from-common-variable-immunodeficiency-patients-undergoing-replacement-intravenous-immunoglobulin-therapy
#15
Giuseppe Spadaro, Concetta D'Orio, Arturo Genovese, Antonella Galeotafiore, Chiara D'Ambrosio, Stefano Di Giovanni, Monica Vitale, Mario Capasso, Vincenzo Lamberti, Andrea Scaloni, Gianni Marone, Nicola Zambrano
Common variable immunodeficiency is the most common form of symptomatic primary antibody failure in adults and children. Replacement immunoglobulin is the standard treatment of these patients. By using a differential proteomic approach based on 2D-DIGE, we examined serum samples from normal donors and from matched, naive, and immunoglobulin-treated patients. The results highlighted regulated expression of serum proteins in naive patients. Among the identified proteins, clusterin/ApoJ serum levels were lower in naive patients, compared to normal subjects...
2011: Journal of Biomedicine & Biotechnology
https://www.readbyqxmd.com/read/21635229/airway-and-interstitial-lung-disease-are-distinct-entities-in-paediatric-common-variable-immunodeficiency
#16
A A J M van de Ven, P A de Jong, D P Hoytema van Konijnenburg, O A M Kessels, M Boes, E A M Sanders, S W J Terheggen-Lagro, J M van Montfrans
Common variable immunodeficiency (CVID) is a common primary immune deficiency, caused by undefined defects in lymphocyte function, and is treated routinely by immunoglobulin substitution. CVID complications include airway disease (AD) and interstitial lung disease (ILD). It was not known if AD and ILD in CVID have a common immunological aetiology and should be considered separate features of the same disease, or as distinct syndromes that require specialized monitoring and treatment. We used high-resolution computed tomography (CT) to diagnose AD or ILD in paediatric CVID patients...
August 2011: Clinical and Experimental Immunology
https://www.readbyqxmd.com/read/21345299/high-prevalence-of-primary-immune-deficiencies-in-children-with-autoimmune-disorders
#17
Julie Barsalou, Claire Saint-Cyr, Eric Drouin, Françoise Le Deist, Elie Haddad
OBJECTIVES: Autoimmunity is a well-recognised manifestation of primary immunodeficiency disorders. However, the prevalence of primary immunodeficiency among children with autoimmune diseases is not well characterised. The objective of this retrospective study was to describe the prevalence of primary immunodeficiency disorders in a paediatric population with autoimmune diseases. METHODS: We retrospectively analysed a cohort of patients investigated for diverse autoimmune conditions from June 1st 2005 to December 31st 2006 in the Rheumatology and Immunology service of a tertiary care paediatric hospital in Canada...
January 2011: Clinical and Experimental Rheumatology
https://www.readbyqxmd.com/read/21276061/diffuse-normolipemic-plane-xanthoma-in-a-child-with-common-variable-immunodeficiency
#18
Stefano Cambiaghi, Vinicio Boneschi, Laura Maffeis, Carlo Gelmetti
Diffuse plane xanthoma is extremely rare in children. Although it may be associated with systemic disorders, its etiology remains obscure in a number of patients. The case of a boy with common variable immunodeficiency and normal serum lipid levels, who developed diffuse plane xanthoma during treatment with intravenous immunoglobulins, is reported.
January 2011: Pediatric Dermatology
https://www.readbyqxmd.com/read/21168377/-spectrum-of-primary-immunodeficiencies-in-a-tertiary-hospital-over-a-period-of-10-years
#19
A Martín-Nalda, P Soler-Palacín, T Español Borén, I Caragol Urgelles, C Díaz de Heredia Rubio, C Figueras Nadal
INTRODUCTION: More than 200 primary immunodeficiencies (PID) have been described and about 60% present during childhood. Early diagnosis and treatment have been shown to improve patient outcome. AIM: Analysis of patients with a PID diagnosed in a paediatric tertiary care hospital-referral centre over a period of 10 years. PATIENTS AND METHODS: Medical records of all paediatric patients followed up in our unit were retrospectively reviewed...
February 2011: Anales de Pediatría: Publicación Oficial de la Asociación Española de Pediatría (A.E.P.)
https://www.readbyqxmd.com/read/21062271/do-ribosomopathies-explain-some-cases-of-common-variable-immunodeficiency
#20
REVIEW
S Khan, J Pereira, P J Darbyshire, S Holding, P C Doré, W A C Sewell, A Huissoon
The considerable clinical heterogeneity of patients with common variable immunodeficiency disorders (CVID) shares some similarity with bone-marrow failure disorders such as Diamond-Blackfan anaemia (DBA) and Shwachman-Diamond syndrome (SDS), now recognized as defects in ribosome biogenesis or ribosomopathies. The recognition of a patient with DBA who subsequently developed CVID lends support to our previous finding of a heterozygous mutation in the SBDS gene of SBDS in another CVID patient, suggesting that ribosome biogenesis defects are responsible for a subset of CVID...
January 2011: Clinical and Experimental Immunology
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