PubRank

Marianne Debré

Author PubWeight™ 34.26‹?›

Top papers

Rank Title Journal Year PubWeight™‹?›
1 Efficacy of gene therapy for X-linked severe combined immunodeficiency. N Engl J Med 2010 3.54
2 Phosphoinositide 3-kinase δ gene mutation predisposes to respiratory infection and airway damage. Science 2013 3.33
3 Allogeneic bone marrow transplantation in mevalonic aciduria. N Engl J Med 2007 3.18
4 Immunodeficiency, autoinflammation and amylopectinosis in humans with inherited HOIL-1 and LUBAC deficiency. Nat Immunol 2012 2.38
5 Efficacy of etanercept for the treatment of juvenile idiopathic arthritis according to the onset type. Arthritis Rheum 2003 2.03
6 Long-term outcome after hematopoietic stem cell transplantation of a single-center cohort of 90 patients with severe combined immunodeficiency. Blood 2009 2.03
7 FAS-L, IL-10, and double-negative CD4- CD8- TCR alpha/beta+ T cells are reliable markers of autoimmune lymphoproliferative syndrome (ALPS) associated with FAS loss of function. Blood 2009 1.91
8 Clinical, immunologic and genetic analysis of 29 patients with autosomal recessive hyper-IgM syndrome due to Activation-Induced Cytidine Deaminase deficiency. Clin Immunol 2004 1.51
9 Activation-induced cytidine deaminase (AID) is required for B-cell tolerance in humans. Proc Natl Acad Sci U S A 2011 1.44
10 Autosomal dominant STAT3 deficiency and hyper-IgE syndrome: molecular, cellular, and clinical features from a French national survey. Medicine (Baltimore) 2012 1.31
11 Natural antibodies sustain differentiation and maturation of human dendritic cells. Proc Natl Acad Sci U S A 2004 1.24
12 Invasive mold infections in chronic granulomatous disease: a 25-year retrospective survey. Clin Infect Dis 2011 1.13
13 Severe cutaneous papillomavirus disease after haemopoietic stem-cell transplantation in patients with severe combined immune deficiency caused by common gammac cytokine receptor subunit or JAK-3 deficiency. Lancet 2004 1.12
14 Expansion of regulatory T cells in patients with Langerhans cell histiocytosis. PLoS Med 2007 1.10
15 Major histocompatibility complex class II expression deficiency caused by a RFXANK founder mutation: a survey of 35 patients. Blood 2011 1.09
16 Prevalence and clinical impact of norovirus fecal shedding in children with inherited immune deficiencies. J Infect Dis 2012 1.07
17 Morbidity and mortality from ataxia-telangiectasia are associated with ATM genotype. J Allergy Clin Immunol 2011 0.97
18 Polymerase ε1 mutation in a human syndrome with facial dysmorphism, immunodeficiency, livedo, and short stature ("FILS syndrome"). J Exp Med 2012 0.94
19 Protective effect of IgM against colonization of the respiratory tract by nontypeable Haemophilus influenzae in patients with hypogammaglobulinemia. J Allergy Clin Immunol 2011 0.92
20 Inherited MST1 deficiency underlies susceptibility to EV-HPV infections. PLoS One 2012 0.91
21 Protein A Sepharose immunoadsorption can restore the efficacy of platelet concentrates in patients with Glanzmann's thrombasthenia and anti-glycoprotein IIb-IIIa antibodies. Br J Haematol 2002 0.87
22 Granulomatous inflammation in cartilage-hair hypoplasia: risks and benefits of anti-TNF-α mAbs. J Allergy Clin Immunol 2011 0.78
23 A randomized controlled trial of genotypic HIV drug resistance testing in HIV-1-infected children: the PERA (PENTA 8) trial. Antivir Ther 2006 0.76