PubRank

Douglas B Kuhns

Author PubWeight™ 49.28‹?›

Top papers

Rank Title Journal Year PubWeight™‹?›
1 STAT3 mutations in the hyper-IgE syndrome. N Engl J Med 2007 8.99
2 Mutations in GATA2 are associated with the autosomal dominant and sporadic monocytopenia and mycobacterial infection (MonoMAC) syndrome. Blood 2011 4.21
3 Autosomal dominant and sporadic monocytopenia with susceptibility to mycobacteria, fungi, papillomaviruses, and myelodysplasia. Blood 2009 3.00
4 Distinct mutations in IRAK-4 confer hyporesponsiveness to lipopolysaccharide and interleukin-1 in a patient with recurrent bacterial infections. J Exp Med 2003 1.83
5 A hypermorphic missense mutation in PLCG2, encoding phospholipase Cγ2, causes a dominantly inherited autoinflammatory disease with immunodeficiency. Am J Hum Genet 2012 1.81
6 Hematologically important mutations: X-linked chronic granulomatous disease (third update). Blood Cells Mol Dis 2010 1.70
7 A phase 1 clinical trial of long-term, low-dose treatment of WHIM syndrome with the CXCR4 antagonist plerixafor. Blood 2014 1.66
8 The CXCR4 antagonist plerixafor corrects panleukopenia in patients with WHIM syndrome. Blood 2011 1.65
9 Impaired priming and activation of the neutrophil NADPH oxidase in patients with IRAK4 or NEMO deficiency. J Immunol 2009 1.52
10 Signal transducer and activator of transcription 1 (STAT1) gain-of-function mutations and disseminated coccidioidomycosis and histoplasmosis. J Allergy Clin Immunol 2013 1.52
11 Hypomorphic Rag mutations can cause destructive midline granulomatous disease. Blood 2010 1.47
12 Inhibition of human neutrophil IL-8 production by hydrogen peroxide and dysregulation in chronic granulomatous disease. J Immunol 2005 1.34
13 Thioglycollate peritonitis in mice lacking C5, 5-lipoxygenase, or p47(phox): complement, leukotrienes, and reactive oxidants in acute inflammation. J Leukoc Biol 2002 1.33
14 Hematologically important mutations: the autosomal recessive forms of chronic granulomatous disease (second update). Blood Cells Mol Dis 2010 1.25
15 Severe congenital neutropenia resulting from G6PC3 deficiency with increased neutrophil CXCR4 expression and myelokathexis. Blood 2010 1.17
16 Diagnostic paradigm for evaluation of male patients with chronic granulomatous disease, based on the dihydrorhodamine 123 assay. J Allergy Clin Immunol 2003 1.15
17 Evaluating the cytotoxicity of innate immune effector cells using the GrB ELISPOT assay. J Transl Med 2004 1.15
18 Interleukin-12 receptor β1 deficiency predisposing to disseminated Coccidioidomycosis. Clin Infect Dis 2011 1.09
19 Loss-of-function of the protein kinase C δ (PKCδ) causes a B-cell lymphoproliferative syndrome in humans. Blood 2013 1.08
20 Tryptophan/kynurenine metabolism in human leukocytes is independent of superoxide and is fully maintained in chronic granulomatous disease. Blood 2010 1.07
21 Cutting edge: expression of IL-1 receptor-associated kinase-4 (IRAK-4) proteins with mutations identified in a patient with recurrent bacterial infections alters normal IRAK-4 interaction with components of the IL-1 receptor complex. J Immunol 2005 1.07
22 Activation of triggering receptor expressed on myeloid cells-1 on human neutrophils by marburg and ebola viruses. J Virol 2006 1.05
23 Brief report: genotype, phenotype, and clinical course in five patients with PAPA syndrome (pyogenic sterile arthritis, pyoderma gangrenosum, and acne). Arthritis Rheum 2011 0.97
24 Intermediate phenotypes in patients with autosomal dominant hyper-IgE syndrome caused by somatic mosaicism. J Allergy Clin Immunol 2013 0.92
25 Abnormal nasal nitric oxide production, ciliary beat frequency, and Toll-like receptor response in pulmonary nontuberculous mycobacterial disease epithelium. Am J Respir Crit Care Med 2013 0.90
26 Intrathoracic nontuberculous mycobacterial infections in otherwise healthy children. Pediatr Pulmonol 2009 0.88
27 Generation of functionally mature neutrophils from induced pluripotent stem cells. Methods Mol Biol 2014 0.85
28 Editorial: will the real neutrophil please stand up? J Leukoc Biol 2011 0.85
29 Serologic reactivity to the emerging pathogen Granulibacter bethesdensis. J Infect Dis 2012 0.83
30 B-cell activating factor (BAFF) is elevated in chronic granulomatous disease. Clin Immunol 2013 0.77
31 Genetic Risk for Inflammatory Bowel Disease Is a Determinant of Crohn's Disease Development in Chronic Granulomatous Disease. Inflamm Bowel Dis 2016 0.75
32 Genetic Risk for Inflammatory Bowel Disease Is a Determinant of Crohn's Disease Development in Chronic Granulomatous Disease. Inflamm Bowel Dis 2016 0.75