Published in Clin Exp Immunol on September 01, 2009
How I treat common variable immune deficiency. Blood (2010) 1.85
Retracted The European internet-based patient and research database for primary immunodeficiencies: update 2011. Clin Exp Immunol (2012) 1.11
Gene therapy on the move. EMBO Mol Med (2013) 1.10
The United Kingdom Primary Immune Deficiency (UKPID) Registry: report of the first 4 years' activity 2008-2012. Clin Exp Immunol (2014) 1.08
The German national registry for primary immunodeficiencies (PID). Clin Exp Immunol (2013) 1.05
Prevalence of primary immunodeficiency in Korea. J Korean Med Sci (2012) 1.04
Classification of primary immunodeficiency diseases by the International Union of Immunological Societies (IUIS) Expert Committee on Primary Immunodeficiency 2011. Clin Exp Immunol (2012) 1.02
Use of combination chemotherapy for treatment of granulomatous and lymphocytic interstitial lung disease (GLILD) in patients with common variable immunodeficiency (CVID). J Clin Immunol (2012) 1.00
Educational paper: primary antibody deficiencies. Eur J Pediatr (2011) 1.00
Modeling primary immunodeficiency disease epidemiology and its treatment to estimate latent therapeutic demand for immunoglobulin. J Clin Immunol (2013) 0.96
Modeling the prevalence of immunodeficiency-associated long-term vaccine-derived poliovirus excretors and the potential benefits of antiviral drugs. BMC Infect Dis (2015) 0.93
Primary vs. secondary antibody deficiency: clinical features and infection outcomes of immunoglobulin replacement. PLoS One (2014) 0.91
Educational paper: Primary immunodeficiencies in children: a diagnostic challenge. Eur J Pediatr (2010) 0.91
Nationwide survey of patients with primary immunodeficiency diseases in Japan. J Clin Immunol (2011) 0.91
Common variable immunodeficiency and idiopathic primary hypogammaglobulinemia: two different conditions within the same disease spectrum. Haematologica (2013) 0.88
The prevalences [correction] and patient characteristics of primary immunodeficiency diseases in Turkey--two centers study. J Clin Immunol (2012) 0.87
Profile of the patients who present to immunology outpatient clinics because of frequent infections. Turk Pediatri Ars (2014) 0.84
Health-related quality of life in patients with primary immunodeficiency disease. Allergy Asthma Clin Immunol (2015) 0.83
Health-related quality of life, anxiety, and depressive symptoms in children with primary immunodeficiencies. Patient Prefer Adherence (2014) 0.83
Quantification of IgM and IgA anti-pneumococcal capsular polysaccharides by a new ELISA assay: a valuable diagnostic and prognostic tool for common variable immunodeficiency. J Clin Immunol (2012) 0.82
Pseudomonas infection in antibody deficient patients. Eur J Microbiol Immunol (Bp) (2014) 0.81
Prevalence and morbidity of primary immunodeficiency diseases, United States 2001-2007. J Clin Immunol (2014) 0.80
When to initiate immunoglobulin replacement therapy (IGRT) in antibody deficiency: a practical approach. Clin Exp Immunol (2017) 0.80
Primary immunodeficiency in infection-prone children in southern Sweden: occurrence, clinical characteristics and immunological findings. BMC Immunol (2014) 0.80
Comprehensive report of primary immunodeficiency disorders from a tertiary care center in India. J Clin Immunol (2012) 0.80
Home care use of intravenous and subcutaneous immunoglobulin for primary immunodeficiency in the United States. J Clin Immunol (2012) 0.79
Primary immunodeficiency diseases in oman: five years' experience at sultan qaboos university hospital. World Allergy Organ J (2012) 0.79
Primary immunodeficiencies: a 27-year review at a tertiary paediatric hospital in Cape Town, South Africa. J Clin Immunol (2010) 0.78
The PedPAD study: boys predominate in the hypogammaglobulinaemia registry of the ESID online database. Clin Exp Immunol (2014) 0.78
The Incidence and Prevalence of Common Variable Immunodeficiency Disease in Taiwan, A Population-Based Study. PLoS One (2015) 0.78
Otologic evaluation of patients with primary antibody deficiency. Eur Arch Otorhinolaryngol (2016) 0.77
Primary immunodeficiency diseases at reference and high-specialty hospitals in the state of Guanajuato, Mexico. Biomed Res Int (2013) 0.77
Severe combined immunodeficiency in Greek children over a 20-year period: rarity of γc-chain deficiency (X-linked) type. J Clin Immunol (2011) 0.77
The Duesseldorf warning signs for primary immunodeficiency: is it time to change the rules? J Clin Immunol (2015) 0.76
Long-term follow-up on affinity maturation and memory B-cell generation in patients with common variable immunodeficiency. J Clin Immunol (2013) 0.76
Distribution and clinical features of primary immunodeficiency diseases in Chinese children (2004-2009). J Clin Immunol (2011) 0.76
Collaborating to improve quality of life in primary immunodeficiencies: World PI Week, 2013. J Clin Immunol (2013) 0.76
Recurrent respiratory tract infections in children - analysis of immunological examinations. Cent Eur J Immunol (2015) 0.76
CD40 Ligand Deficient C57BL/6 Mouse Is a Potential Surrogate Model of Human X-Linked Hyper IgM (X-HIGM) Syndrome for Characterizing Immune Responses against Pathogens. Biomed Res Int (2015) 0.75
Impact of Site of Care on Infection Rates Among Patients with Primary Immunodeficiency Diseases Receiving Intravenous Immunoglobulin Therapy. J Clin Immunol (2017) 0.75
Does the route of immunoglobin replacement therapy impact quality of life and satisfaction in patients with primary immunodeficiency? Insights from the French cohort "Visages". Orphanet J Rare Dis (2016) 0.75
[Humoral immunodeficiency : awareness for better support]. Pan Afr Med J (2014) 0.75
Comprehensive activities to increase recognition of primary immunodeficiency and access to immunoglobulin replacement therapy in Poland. Eur J Pediatr (2016) 0.75
Somatic Hypermutation Defects in Common Variable Immune Deficiency. Curr Allergy Asthma Rep (2017) 0.75
Primary immunodeficiency diseases: an update from the International Union of Immunological Societies Primary Immunodeficiency Diseases Classification Committee. J Allergy Clin Immunol (2007) 2.98
NIH conference. New insights into common variable immunodeficiency. Ann Intern Med (1993) 2.52
Subcutaneous immunoglobulin replacement in patients with primary antibody deficiencies: safety and costs. Lancet (1995) 2.43
Primary immunodeficiency diseases in Australia and New Zealand. J Clin Immunol (2007) 1.58
Primary immunodeficiency diseases in Norway. J Clin Immunol (2000) 1.48
The European internet-based patient and research database for primary immunodeficiencies: results 2004-06. Clin Exp Immunol (2007) 1.41
Primary immunodeficiency syndrome in Spain: first report of the National Registry in Children and Adults. J Clin Immunol (1997) 1.41
The ESID Online Database network. Bioinformatics (2007) 1.24
Primary immunodeficiency disorders in Kuwait: first report from Kuwait National Primary Immunodeficiency Registry (2004--2006). J Clin Immunol (2007) 1.24
LMO2-associated clonal T cell proliferation in two patients after gene therapy for SCID-X1. Science (2003) 19.77
DPC4, a candidate tumor suppressor gene at human chromosome 18q21.1. Science (1996) 11.13
Gene therapy of human severe combined immunodeficiency (SCID)-X1 disease. Science (2000) 10.40
Activation-induced cytidine deaminase (AID) deficiency causes the autosomal recessive form of the Hyper-IgM syndrome (HIGM2). Cell (2000) 9.99
Somatic SF3B1 mutation in myelodysplasia with ring sideroblasts. N Engl J Med (2011) 7.89
Interferon-gamma-receptor deficiency in an infant with fatal bacille Calmette-Guérin infection. N Engl J Med (1996) 5.36
Mutations in RAB27A cause Griscelli syndrome associated with haemophagocytic syndrome. Nat Genet (2000) 5.11
Lymphoid development in mice with a targeted deletion of the interleukin 2 receptor gamma chain. Proc Natl Acad Sci U S A (1995) 5.06
Perforin gene defects in familial hemophagocytic lymphohistiocytosis. Science (1999) 4.93
Mutations in Fas associated with human lymphoproliferative syndrome and autoimmunity. Science (1995) 4.80
Artemis, a novel DNA double-strand break repair/V(D)J recombination protein, is mutated in human severe combined immune deficiency. Cell (2001) 4.79
Impairment of mycobacterial immunity in human interleukin-12 receptor deficiency. Science (1998) 4.58
CD40 ligand mutations in x-linked immunodeficiency with hyper-IgM. Nature (1993) 4.11
Mutations in TNFRSF13B encoding TACI are associated with common variable immunodeficiency in humans. Nat Genet (2005) 3.97
X-linked anhidrotic ectodermal dysplasia with immunodeficiency is caused by impaired NF-kappaB signaling. Nat Genet (2001) 3.86
Clinical spectrum of X-linked hyper-IgM syndrome. J Pediatr (1997) 3.63
CD40-CD40L independent Ig gene hypermutation suggests a second B cell diversification pathway in humans. Proc Natl Acad Sci U S A (2001) 3.54
Allelotype of pancreatic adenocarcinoma using xenograft enrichment. Cancer Res (1995) 3.33
A human IFNGR1 small deletion hotspot associated with dominant susceptibility to mycobacterial infection. Nat Genet (1999) 3.12
Mitochondrial DNA deletion in Pearson's marrow/pancreas syndrome. Lancet (1989) 2.98
The diagnosis and management of chronic cough. Eur Respir J (2004) 2.96
Griscelli disease maps to chromosome 15q21 and is associated with mutations in the myosin-Va gene. Nat Genet (1997) 2.93
The calcium current activated by T cell receptor and store depletion in human lymphocytes is absent in a primary immunodeficiency. J Biol Chem (1994) 2.92
Oxidative DNA damage and senescence of human diploid fibroblast cells. Proc Natl Acad Sci U S A (1995) 2.89
Partial interferon-gamma receptor 1 deficiency in a child with tuberculoid bacillus Calmette-Guérin infection and a sibling with clinical tuberculosis. J Clin Invest (1997) 2.76
The Arabidopsis thaliana genome contains at least 29 active genes encoding SET domain proteins that can be assigned to four evolutionarily conserved classes. Nucleic Acids Res (2001) 2.70
Attitudes and knowledge relevant to family planning among New Orleans Negro women. Am J Public Health Nations Health (1966) 2.60
Pearson's marrow-pancreas syndrome. A multisystem mitochondrial disorder in infancy. J Clin Invest (1990) 2.46
Autosomal recessive osteopetrosis: variability of findings at diagnosis and during the natural course. Pediatrics (1994) 2.33
Isolation of new lymphotropic retrovirus from two siblings with haemophilia B, one with AIDS. Lancet (1984) 2.32
Inherited interleukin 12 deficiency in a child with bacille Calmette-Guérin and Salmonella enteritidis disseminated infection. J Clin Invest (1998) 2.30
Incidence, severity, and prevention of infections in chronic granulomatous disease. J Pediatr (1989) 2.22
Human recombinant interferon alfa-2a for the treatment of Behçet's disease with sight threatening posterior or panuveitis. Br J Ophthalmol (2003) 2.20
Hemophagocytic lymphohistiocytosis. Report of 122 children from the International Registry. FHL Study Group of the Histiocyte Society. Leukemia (1996) 2.15
Idiopathic disseminated bacillus Calmette-Guérin infection: a French national retrospective study. Pediatrics (1996) 2.14
Mutations in activation-induced cytidine deaminase in patients with hyper IgM syndrome. Clin Immunol (2000) 2.13
Immunological conditions of children with BCG disseminated infection. Lancet (1995) 2.05
Treatment of the immune dysregulation, polyendocrinopathy, enteropathy, X-linked syndrome (IPEX) by allogeneic bone marrow transplantation. N Engl J Med (2001) 2.05
Specific inhibition of in vitro Candida-induced lymphocyte proliferation by polysaccharidic antigens present in the serum of patients with chronic mucocutaneous candidiasis. J Clin Invest (1978) 2.03
Long-term effect of bone-marrow transplantation for childhood-onset cerebral X-linked adrenoleukodystrophy. Lancet (2000) 1.98
Fatal disseminated Mycobacterium smegmatis infection in a child with inherited interferon gamma receptor deficiency. Clin Infect Dis (1997) 1.96
Conditional abatement of tissue fibrosis using nucleoside analogs to selectively corrupt DNA replication in transgenic fibroblasts. Mol Ther (2001) 1.95
Wnt-1 regulation of connexin43 in cardiac myocytes. J Clin Invest (2000) 1.87
Exacerbation of murine ileitis by Toll-like receptor 4 mediated sensing of lipopolysaccharide from commensal Escherichia coli. Gut (2007) 1.85
Severe combined immunodeficiency: a retrospective single-center study of clinical presentation and outcome in 117 patients. J Pediatr (1993) 1.81
CNS involvement at the onset of primary hemophagocytic lymphohistiocytosis. Neurology (2012) 1.80
The sensory and sympathetic innervation of guinea-pig lung and trachea as studied by retrograde neuronal tracing and double-labelling immunohistochemistry. Neuroscience (1992) 1.78
Chemokine-receptor expression on T cells in lung compartments of challenged asthmatic patients. Clin Exp Allergy (2005) 1.76
The mitosome, a novel organelle related to mitochondria in the amitochondrial parasite Entamoeba histolytica. Mol Microbiol (1999) 1.75
An embryonic-like methylation pattern of classical satellite DNA is observed in ICF syndrome. Hum Mol Genet (1993) 1.75
Early and prolonged intravenous immunoglobulin replacement therapy in childhood agammaglobulinemia: a retrospective survey of 31 patients. J Pediatr (1999) 1.73
Atypical X-linked severe combined immunodeficiency due to possible spontaneous reversion of the genetic defect in T cells. N Engl J Med (1996) 1.73
Outcomes of hematopoietic stem cell transplantation for Hurler's syndrome in Europe: a risk factor analysis for graft failure. Bone Marrow Transplant (2007) 1.72
Extracorporeal shock wave therapy of nonunion or delayed osseous union. Clin Orthop Relat Res (2001) 1.67
Conditional lineage ablation to model human diseases. Proc Natl Acad Sci U S A (1998) 1.64
Intestinal peptide transport: ex vivo uptake studies and localization of peptide carrier PEPT1. Am J Physiol Gastrointest Liver Physiol (2001) 1.62
Perioperative management of patients with cardiac implantable electronic devices. Br J Anaesth (2011) 1.61
Mutation of RFXAP, a regulator of MHC class II genes, in primary MHC class II deficiency. N Engl J Med (1997) 1.60
Graphitic tribological layers in metal-on-metal hip replacements. Science (2011) 1.60
Deficient peptide loading and MHC class II endosomal sorting in a human genetic immunodeficiency disease: the Chediak-Higashi syndrome. J Cell Biol (1998) 1.60
Role of prostaglandin E2 in the induction of nonspecific T lymphocyte suppressor activity. J Immunol (1981) 1.55
Two genes are responsible for Griscelli syndrome at the same 15q21 locus. Genomics (2000) 1.54
Treatment of childhood autoimmune haemolytic anaemia with rituximab. Lancet (2001) 1.54
Oscillating expression of c-Hey2 in the presomitic mesoderm suggests that the segmentation clock may use combinatorial signaling through multiple interacting bHLH factors. Dev Biol (2000) 1.53
The complex evolutionary history of gorillas: insights from genomic data. Mol Biol Evol (2006) 1.52
Defect in IgV gene somatic hypermutation in common variable immuno-deficiency syndrome. Proc Natl Acad Sci U S A (1998) 1.51
The chronically symptomatic vulva: aetiology and management. Br J Obstet Gynaecol (1995) 1.50
Frequency and severity of central nervous system lesions in hemophagocytic lymphohistiocytosis. Blood (1997) 1.49
Autoimmune lymphoproliferative syndromes: genetic defects of apoptosis pathways. Cell Death Differ (2003) 1.48
A primary T-cell immunodeficiency associated with defective transmembrane calcium influx. Blood (1995) 1.48
Measles virus induces abnormal differentiation of CD40 ligand-activated human dendritic cells. J Immunol (2000) 1.47
MIXED CULTURES OF PURE STRAINS OF FIBROBLASTS AND EPITHELIAL CELLS. J Exp Med (1922) 1.47
Correlation of granuloma structure with clinical outcome defines two types of idiopathic disseminated BCG infection. J Pathol (1997) 1.47
Expression of nitric oxide synthase in kidney macula densa cells. Kidney Int (1992) 1.46
Emergency stenting for acute occlusion after coronary balloon angioplasty. Circulation (1988) 1.45
Current controversies in colorectal surgery: the way to resolve uncertainty and move forward. Colorectal Dis (2012) 1.45
The interaction between Cdc42 and WASP is required for SDF-1-induced T-lymphocyte chemotaxis. Blood (2001) 1.44
Efficacy and safety of Hizentra(®) in patients with primary immunodeficiency after a dose-equivalent switch from intravenous or subcutaneous replacement therapy. Clin Immunol (2011) 1.44
Dynamics of viral variants in HIV-1 Nef and specific cytotoxic T lymphocytes in vivo. J Immunol (1996) 1.43
Major histocompatibility complex class II deficiency: clinical manifestations, immunologic features, and outcome. J Pediatr (1993) 1.43
Factor VII and activated-factor-VII content of prothrombin complex concentrates. The PCC Study Group. Vox Sang (1997) 1.43
Severe aplastic anemia of neonatal onset: a single-center retrospective study of six children. J Pediatr (1998) 1.43
Human immunodeficiency virus gp120 and derived peptides activate protein tyrosine kinase p56lck in human CD4 T lymphocytes. Eur J Immunol (1993) 1.42
Partial albinism with immunodeficiency (Griscelli syndrome). J Pediatr (1994) 1.42
The European internet-based patient and research database for primary immunodeficiencies: results 2004-06. Clin Exp Immunol (2007) 1.41
Impact of the implantable cardioverter-defibrillator on rehospitalizations. Eur Heart J (1996) 1.40
[Model trial: use and evaluation of a problem-oriented learning program in internal medicine]. Med Klin (Munich) (1999) 1.39
Serotypes, hemagglutinins, siderophore synthesis, and serum resistance of Klebsiella isolates causing human urinary tract infections. J Infect Dis (1993) 1.39
[Modifying therapeutic in immune response]. Arch Fr Pediatr (1992) 1.38
Treatment of juvenile chronic myelomonocytic leukemia by allogeneic bone marrow transplantation. Bone Marrow Transplant (1994) 1.38
Lymphoproliferative syndrome with autoimmunity: A possible genetic basis for dominant expression of the clinical manifestations. Blood (1999) 1.38
Abnormal methylation pattern in constitutive and facultative (X inactive chromosome) heterochromatin of ICF patients. Hum Mol Genet (1994) 1.38
Abundant production of brain-derived neurotrophic factor by adult visceral epithelia. Implications for paracrine and target-derived Neurotrophic functions. Am J Pathol (1999) 1.37
Evaluation of correlation between dose and clinical outcomes in subcutaneous immunoglobulin replacement therapy. Clin Exp Immunol (2012) 1.36