PubRank

Philip N Hawkins

Author PubWeight™ 176.28‹?›

Top papers

Rank Title Journal Year PubWeight™‹?›
1 NALP3 forms an IL-1beta-processing inflammasome with increased activity in Muckle-Wells autoinflammatory disorder. Immunity 2004 13.55
2 Neonatal-onset multisystem inflammatory disease responsive to interleukin-1beta inhibition. N Engl J Med 2006 6.17
3 Definition of organ involvement and treatment response in immunoglobulin light chain amyloidosis (AL): a consensus opinion from the 10th International Symposium on Amyloid and Amyloidosis, Tours, France, 18-22 April 2004. Am J Hematol 2005 5.64
4 Use of canakinumab in the cryopyrin-associated periodic syndrome. N Engl J Med 2009 5.19
5 Natural history and outcome in systemic AA amyloidosis. N Engl J Med 2007 4.54
6 Misdiagnosis of hereditary amyloidosis as AL (primary) amyloidosis. N Engl J Med 2002 4.47
7 T1 mapping for myocardial extracellular volume measurement by CMR: bolus only versus primed infusion technique. JACC Cardiovasc Imaging 2013 4.21
8 Targeting C-reactive protein for the treatment of cardiovascular disease. Nature 2006 4.07
9 Safety and efficacy of RNAi therapy for transthyretin amyloidosis. N Engl J Med 2013 3.97
10 Interleukin-1-receptor antagonist in the Muckle-Wells syndrome. N Engl J Med 2003 3.86
11 Noncontrast T1 mapping for the diagnosis of cardiac amyloidosis. JACC Cardiovasc Imaging 2013 3.53
12 Eprodisate for the treatment of renal disease in AA amyloidosis. N Engl J Med 2007 3.38
13 New criteria for response to treatment in immunoglobulin light chain amyloidosis based on free light chain measurement and cardiac biomarkers: impact on survival outcomes. J Clin Oncol 2012 3.35
14 Evaluation and management of the cardiac amyloidosis. J Am Coll Cardiol 2007 3.12
15 Outcome in systemic AL amyloidosis in relation to changes in concentration of circulating free immunoglobulin light chains following chemotherapy. Br J Haematol 2003 2.74
16 Association of mutations in the NALP3/CIAS1/PYPAF1 gene with a broad phenotype including recurrent fever, cold sensitivity, sensorineural deafness, and AA amyloidosis. Arthritis Rheum 2002 2.58
17 A European collaborative study of treatment outcomes in 346 patients with cardiac stage III AL amyloidosis. Blood 2013 2.38
18 In vivo regulation of interleukin 1beta in patients with cryopyrin-associated periodic syndromes. J Exp Med 2009 2.34
19 Infusion of pharmaceutical-grade natural human C-reactive protein is not proinflammatory in healthy adult human volunteers. Circ Res 2013 2.26
20 Updates in cardiac amyloidosis: a review. J Am Heart Assoc 2012 2.21
21 Bortezomib with or without dexamethasone in primary systemic (light chain) amyloidosis. J Clin Oncol 2010 2.20
22 Rapid and complete resolution of proteinuria due to renal amyloidosis in a patient with rheumatoid arthritis treated with infliximab. Arthritis Rheum 2002 2.19
23 Cardiovascular magnetic resonance and prognosis in cardiac amyloidosis. J Cardiovasc Magn Reson 2008 2.01
24 Proinflammatory effects of bacterial recombinant human C-reactive protein are caused by contamination with bacterial products, not by C-reactive protein itself. Circ Res 2005 1.91
25 Therapeutic Clearance of Amyloid by Antibodies to Serum Amyloid P Component. N Engl J Med 2015 1.84
26 Antibodies to human serum amyloid P component eliminate visceral amyloid deposits. Nature 2010 1.81
27 Cyclophosphamide, bortezomib, and dexamethasone therapy in AL amyloidosis is associated with high clonal response rates and prolonged progression-free survival. Blood 2012 1.80
28 Abnormal tumor necrosis factor receptor I cell surface expression and NF-kappaB activation in tumor necrosis factor receptor-associated periodic syndrome. Arthritis Rheum 2008 1.61
29 Cardiovascular magnetic resonance measurement of myocardial extracellular volume in health and disease. Heart 2012 1.56
30 Safety and efficacy of risk-adapted cyclophosphamide, thalidomide, and dexamethasone in systemic AL amyloidosis. Blood 2006 1.54
31 Heterogeneity among patients with tumor necrosis factor receptor-associated periodic syndrome phenotypes. Arthritis Rheum 2003 1.51
32 Inflammatory bowel disease and systemic AA amyloidosis. Dig Dis Sci 2013 1.43
33 Efficacy of bortezomib in systemic AL amyloidosis with relapsed/refractory clonal disease. Haematologica 2008 1.38
34 Cardiac transplantation for amyloid heart disease: the United Kingdom experience. J Heart Lung Transplant 2004 1.36
35 Systemic amyloidosis and the gastrointestinal tract. Nat Rev Gastroenterol Hepatol 2009 1.34
36 Diagnostic performance of 123I-labeled serum amyloid P component scintigraphy in patients with amyloidosis. Am J Med 2006 1.32
37 Human plasma fibrinogen is synthesized in the liver. Blood 2006 1.27
38 Hereditary systemic amyloidosis due to Asp76Asn variant β2-microglobulin. N Engl J Med 2012 1.26
39 Quantification of myocardial extracellular volume fraction in systemic AL amyloidosis: an equilibrium contrast cardiovascular magnetic resonance study. Circ Cardiovasc Imaging 2012 1.25
40 Online registry for mutations in hereditary amyloidosis including nomenclature recommendations. Hum Mutat 2014 1.23
41 Sequential heart and autologous stem cell transplantation for systemic AL amyloidosis. Blood 2005 1.22
42 Outcome in renal Al amyloidosis after chemotherapy. J Clin Oncol 2011 1.21
43 AL amyloidosis associated with IgM paraproteinemia: clinical profile and treatment outcome. Blood 2008 1.20
44 Senile systemic amyloidosis: clinical features at presentation and outcome. J Am Heart Assoc 2013 1.20
45 Perspectives in treatment of AL amyloidosis. Br J Haematol 2007 1.17
46 Outcome of autologous stem cell transplantation for AL amyloidosis in the UK. Br J Haematol 2006 1.13
47 Quantitative high-resolution microradiographic imaging of amyloid deposits in a novel murine model of AA amyloidosis. Amyloid 2005 1.13
48 Phenotype, genotype, and sustained response to anakinra in 22 patients with autoinflammatory disease associated with CIAS-1/NALP3 mutations. Arch Dermatol 2006 1.12
49 Liver transplantation in transthyretin-related familial amyloid polyneuropathy. Curr Opin Neurol 2004 1.11
50 The emerging role of interleukin-1β in autoinflammatory diseases. Arthritis Rheum 2011 1.09
51 Systemic amyloidosis in England: an epidemiological study. Br J Haematol 2013 1.08
52 Diagnosis, pathogenesis, treatment, and prognosis of hereditary fibrinogen A alpha-chain amyloidosis. J Am Soc Nephrol 2008 1.07
53 Hereditary auto-inflammatory disorders and biologics. Springer Semin Immunopathol 2006 1.06
54 Amyloidogenicity and clinical phenotype associated with five novel mutations in apolipoprotein A-I. Am J Pathol 2011 1.04
55 Sustained pharmacological depletion of serum amyloid P component in patients with systemic amyloidosis. Br J Haematol 2010 1.04
56 Translocations of 14q32 and deletions of 13q14 are common chromosomal abnormalities in systemic amyloidosis. Br J Haematol 2002 1.04
57 Structure, folding dynamics, and amyloidogenesis of D76N β2-microglobulin: roles of shear flow, hydrophobic surfaces, and α-crystallin. J Biol Chem 2013 1.04
58 The electrocardiographic features associated with cardiac amyloidosis of variant transthyretin isoleucine 122 type in Afro-Caribbean patients. Am Heart J 2012 1.03
59 Transmission of systemic transthyretin amyloidosis by means of domino liver transplantation. N Engl J Med 2005 1.02
60 Pathogenetic mechanisms of amyloid A amyloidosis. Proc Natl Acad Sci U S A 2013 1.01
61 Guidelines on the diagnosis and investigation of AL amyloidosis. Br J Haematol 2014 1.01
62 Sustained remission of symptoms and improved health-related quality of life in patients with cryopyrin-associated periodic syndrome treated with canakinumab: results of a double-blind placebo-controlled randomized withdrawal study. Arthritis Res Ther 2011 1.00
63 Cardiovascular magnetic resonance for amyloidosis. Heart Fail Rev 2015 1.00
64 Molecular dissection of Alzheimer's disease neuropathology by depletion of serum amyloid P component. Proc Natl Acad Sci U S A 2009 0.99
65 V122I transthyretin variant in elderly black Americans. N Engl J Med 2015 0.99
66 Use of anakinra (Kineret) in the treatment of familial cold autoinflammatory syndrome with a 16-month follow-up. J Cutan Med Surg 2008 0.96
67 AA amyloidosis complicating the hereditary periodic fever syndromes. Arthritis Rheum 2013 0.94
68 The significance of paired MEFV mutations in individuals without symptoms of familial Mediterranean fever. Eur J Hum Genet 2002 0.93
69 Brief Report: whole-exome sequencing revealing somatic NLRP3 mosaicism in a patient with chronic infantile neurologic, cutaneous, articular syndrome. Arthritis Rheumatol 2014 0.93
70 Cardiac transthyretin amyloidosis. Heart 2012 0.93
71 Pathophysiology and treatment of systemic amyloidosis. Nat Rev Nephrol 2013 0.93
72 Developments in the scientific and clinical understanding of autoinflammatory disorders. Arthritis Res Ther 2009 0.91
73 The importance of complete screening for amyloid fibril type and systemic disease in patients with amyloidosis in the respiratory tract. Sarcoidosis Vasc Diffuse Lung Dis 2002 0.91
74 Amyloidosis. Ann Clin Biochem 2012 0.91
75 Guidelines on the management of AL amyloidosis. Br J Haematol 2014 0.87
76 Diagnostic performance of amyloid A protein quantification in fat tissue of patients with clinical AA amyloidosis. Amyloid 2007 0.87
77 Cardiac phenotype and clinical outcome of familial amyloid polyneuropathy associated with transthyretin alanine 60 variant. Eur Heart J 2011 0.87
78 Measurement of Tissue interstitial volume in healthy patients and those with amyloidosis with equilibrium contrast-enhanced MR imaging. Radiology 2013 0.87
79 Systemic amyloidosis. Curr Opin Pharmacol 2006 0.86
80 Involvement of X-box binding protein 1 and reactive oxygen species pathways in the pathogenesis of tumour necrosis factor receptor-associated periodic syndrome. Ann Rheum Dis 2012 0.86
81 Utility of factor X concentrate for the treatment of acquired factor X deficiency in systemic light-chain amyloidosis. Blood 2014 0.86
82 Methodological challenges in monitoring new treatments for rare diseases: lessons from the cryopyrin-associated periodic syndrome registry. Orphanet J Rare Dis 2013 0.85
83 Clinical characteristics in subjects with NLRP3 V198M diagnosed at a single UK center and a review of the literature. Arthritis Res Ther 2013 0.85
84 Proteolytic cleavage of Ser52Pro variant transthyretin triggers its amyloid fibrillogenesis. Proc Natl Acad Sci U S A 2014 0.84
85 Hereditary amyloidosis in early childhood associated with a novel insertion-deletion (indel) in the fibrinogen Aalpha chain gene. Kidney Int 2005 0.83
86 An Israeli Arab patient with a de novo TNFRSF1A mutation causing tumor necrosis factor receptor-associated periodic syndrome. Arthritis Rheum 2002 0.83
87 Cardiac amyloidosis, a monoclonal gammopathy and a potentially misleading mutation. Nat Clin Pract Cardiovasc Med 2008 0.82
88 Lenalidomide and dexamethasone for systemic AL amyloidosis following prior treatment with thalidomide or bortezomib regimens. Br J Haematol 2014 0.82
89 Laryngeal presentation of systemic apolipoprotein A-I-derived amyloidosis. Laryngoscope 2009 0.82
90 From scleredema to AL amyloidosis: disease progression or coincidence? Review of the literature. Clin Rheumatol 2005 0.82
91 Structural and folding dynamic properties of the T70N variant of human lysozyme. J Biol Chem 2003 0.81
92 Familial amyloid polyneuropathy associated with the novel transthyretin variant Arg34Gly. Amyloid 2012 0.81
93 Novel gelsolin variant as the cause of nephrotic syndrome and renal amyloidosis in a large kindred. Amyloid 2014 0.81
94 Isolation and characterization of pharmaceutical grade human pentraxins, serum amyloid P component and C-reactive protein, for clinical use. J Immunol Methods 2012 0.81
95 Differential cytokine secretion results from p65 and c-Rel NF-κB subunit signaling in peripheral blood mononuclear cells of TNF receptor-associated periodic syndrome patients. Cell Immunol 2011 0.80
96 Familial amyloidotic polyneuropathy with severe renal involvement in association with transthyretin Gly47Glu in Dutch, British and American-Finnish families. Amyloid 2004 0.80
97 AA amyloidosis complicating hyperimmunoglobulinemia D with periodic fever syndrome: a report of two cases. Arthritis Rheum 2006 0.80
98 Amyloidosis, not myeloma. Br J Haematol 2005 0.79
99 Abnormal N-terminal fragment of brain natriuretic peptide in patients with light chain amyloidosis without cardiac involvement at presentation is a risk factor for development of cardiac amyloidosis. Haematologica 2011 0.79
100 Acute renal failure and nephrotic range proteinuria due to amyloidosis in an HIV-infected patient. Am J Med Sci 2006 0.78
101 Efficacy and safety of canakinumab therapy in paediatric patients with cryopyrin-associated periodic syndrome: a single-centre, real-world experience. Rheumatology (Oxford) 2013 0.78
102 Clinical characteristics and SAP scintigraphic findings in 10 patients with AGel amyloidosis. Amyloid 2014 0.78
103 Monitoring systemic amyloidosis using MRI measurements of the extracellular volume fraction. Amyloid 2013 0.78
104 Systemic amyloidosis: getting to the heart of the matter. Br J Hosp Med (Lond) 2010 0.78
105 AL amyloidosis: new drugs and tests, but old challenges. Oncology (Williston Park) 2012 0.78
106 Drug Insight: emerging therapies for amyloidosis. Nat Clin Pract Nephrol 2006 0.78
107 Hereditary amyloidosis with progressive peripheral neuropathy associated with apolipoprotein AI Gly26Arg: outcome of hepatorenal transplantation. Liver Transpl 2007 0.77
108 Clinical profile and treatment outcomes of immunoglobulin D associated AL amyloidosis. Br J Haematol 2013 0.77
109 A prospective study of nutritional status in immunoglobulin light chain amyloidosis. Haematologica 2012 0.76
110 Novel pharmacological strategies in amyloidosis. Nephron Clin Pract 2003 0.76
111 Reply to S. Girnius et al. J Clin Oncol 2013 0.75
112 Hereditary fibrinogen A alpha-chain amyloidosis: clinical phenotype and role of liver transplantation. Blood 2010 0.75
113 The role of PBSCT in treatment of AL amyloidosis is far from settled. Blood 2004 0.75
114 Amyloidosis and primary biliary cirrhosis, Rodriguez-Luna et al. Dig Dis Sci 2006 0.75
115 Hereditary amyloidosis caused by R554L fibrinogen Aα-chain mutation in a Spanish family and review of the literature. Amyloid 2013 0.75
116 Images in cardiovascular medicine. Intermittent loss of second heart sound. Circulation 2006 0.75
117 CMR-Verified Regression of Cardiac AL Amyloid After Chemotherapy. JACC Cardiovasc Imaging 2017 0.75
118 A novel transthyretin variant p.H110D (H90D) as a cause of familial amyloid polyneuropathy in a large Irish kindred. Amyloid 2014 0.75
119 Generalized amyloidosis in an Eastern Bongo (Tragelaphus eurycerus isaaci). J Zoo Wildl Med 2011 0.75
120 Biochemical basis of the amyloid diseases. Br J Hosp Med (Lond) 2010 0.75