Syndrome of cyanosis, digital clubbing, and hepatic disease in siblings.

PubWeight™: 1.30‹?› | Rank: Top 10%

🔗 View Article (PMID 4169366)

Published in J Pediatr on January 01, 1968

Authors

A Silverman, M D Cooper, J H Moller, R A Good

Articles by these authors

In vitro bactericidal capacity of human polymorphonuclear leukocytes: diminished activity in chronic granulomatous disease of childhood. J Clin Invest (1967) 10.26

Functional analysis of two human T-cell subpopulations: help and suppression of B-cell responses by T cells bearing receptors for IgM or IgG. J Exp Med (1977) 9.57

Studies of the metabolic activity of leukocytes from patients with a genetic abnormality of phagocytic function. J Clin Invest (1967) 9.01

The Ink4a tumor suppressor gene product, p19Arf, interacts with MDM2 and neutralizes MDM2's inhibition of p53. Cell (1998) 8.33

Evolutionary conservation of surface molecules that distinguish T lymphocyte helper/inducer and cytotoxic/suppressor subpopulations in mouse and man. J Exp Med (1981) 6.82

Suppressor cell activity after concanavalin A treatment of lymphocytes from normal donors. J Exp Med (1976) 5.50

Altered cell differentiation and proliferation in mice lacking p57KIP2 indicates a role in Beckwith-Wiedemann syndrome. Nature (1997) 4.82

Risk factors for arrhythmia and sudden cardiac death late after repair of tetralogy of Fallot: a multicentre study. Lancet (2000) 4.75

Acute poststreptococcal glomerulonephritis: immune deposit disease. J Clin Invest (1966) 4.64

Immunological reconstitution of sex-linked lymphopenic immunological deficiency. Lancet (1968) 4.41

The T cell dependence of B cell differentiation induced by pokeweed mitogen. J Immunol (1976) 4.40

Thymus-dependent membrane antigens in man: inhibition of cell-mediated lympholysis by monoclonal antibodies to TH2 antigen. Proc Natl Acad Sci U S A (1981) 4.38

The functions of the thymus system and the bursa system in the chicken. J Exp Med (1966) 4.07

Immunofluorescent studies of the development of pre-B cells, B lymphocytes and immunoglobulin isotype diversity in humans. Eur J Immunol (1977) 4.00

Fatal granulomatous disease of childhood. An inborn abnormality of phagocytic function. Lancet (1966) 3.97

The IgA system. I. Studies of the transport and immunochemistry of IgA in the saliva. J Exp Med (1966) 3.97

Lymphotoxin alpha/beta and tumor necrosis factor are required for stromal cell expression of homing chemokines in B and T cell areas of the spleen. J Exp Med (1999) 3.78

Primary immunodeficiencies. Report of a World Health Organization Committee. Pediatrics (1971) 3.69

Wiskott-Aldrich syndrome. An immunologic deficiency disease involving the afferent limb of immunity. Am J Med (1968) 3.54

Pinocytosis by epithelium associated with lymphoid follicles in the bursa of Fabricius, appendix, and Peyer's patches. An electron microscopic study. Am J Anat (1973) 3.38

Suppression of immunoglobulin class synthesis in mice. I. Effects of treatment with antibody to -chain. J Exp Med (1972) 3.32

Early production of intracellular IgM by B-lymphocyte precursors in mouse. Nature (1976) 3.29

A map of local adaptation in Arabidopsis thaliana. Science (2011) 3.27

Protection of phagocytized bacteria from the killing action of antibiotics. Nature (1966) 3.12

Human T lymphocyte subpopulations defined by Fc receptors and monoclonal antibodies. A comparison. J Exp Med (1980) 3.03

Suppression of immunoglobulin G synthesis as a result of antibody-mediated suppression of immunoglobulin M synthesis in chickens. Proc Natl Acad Sci U S A (1970) 2.93

Identification of a p69,71 complex expressed on human T cells sharing determinants with B-type chronic lymphatic leukemic cells. J Exp Med (1980) 2.89

Differentiation capacity of cultured B lymphocytes from immunodeficient patients. J Clin Invest (1973) 2.65

Complement activity and inflammatory neutrophil exudation in man. Studies in patients with glomerulonephritis, essential hypocomplementemia and agammaglobulinemia. Int Arch Allergy Appl Immunol (1967) 2.64

Characterization of HNK-1+ (Leu-7) human lymphocytes. I. Two distinct phenotypes of human NK cells with different cytotoxic capability. J Immunol (1982) 2.63

Morphological and histochemical analyses of two human T-cell subpopulations bearing receptors for IgM or IgG. J Exp Med (1978) 2.58

Agammaglobulinaemia with B lymphocytes. Specific defect of plasma-cell differentiation. Lancet (1971) 2.55

The cellular immune defect in chronic mucocutaneous candidiasis. Lancet (1969) 2.53

C2 deficiency. Development of lupus erythematosus. J Clin Invest (1973) 2.52

Mouse pre-B cells synthesize and secrete mu heavy chains but not light chains. Cell (1980) 2.50

A newly defined X-linked trait in man with demonstration of the Lyon effect in carrier females. Lancet (1967) 2.47

Development and distribution of immunoglobulin-containing cells in the chicken. An immunofluorescent analysis using purified antibodies to mu, gamma and light chains. J Immunol (1971) 2.46

Influence of diet on survival of mice. Proc Natl Acad Sci U S A (1976) 2.45

An NK1.1+ CD4+8- single-positive thymocyte subpopulation that expresses a highly skewed T-cell antigen receptor V beta family. Proc Natl Acad Sci U S A (1992) 2.43

A monoclonal antibody (MMA) that identifies a differentiation antigen on human myelomonocytic cells. Clin Immunol Immunopathol (1982) 2.41

Deficient immunologic functions of NZB mice. Proc Soc Exp Biol Med (1968) 2.41

Immunologic abnormalities in pathogen-free cats experimentally infected with feline immunodeficiency virus. J Virol (1990) 2.40

Differences in susceptibility of mature and immature mouse B lymphocytes to anti-immunoglobulin-induced immunoglobulin suppression in vitro. Possible implications for B-cell tolerance to self. J Exp Med (1975) 2.40

The complement profile in acute glomerulonephritis systemic lupus erythematosus and hypocomplementemic chronic glomerulonephritis. Contrasts and experimental correlations. Int Arch Allergy Appl Immunol (1968) 2.38

Effects of protein insufficiency on immune responsiveness. Am J Clin Nutr (1974) 2.37

Localization of immunological complexes fixing beta1C (C3) in germinal centers of lymph nodes. J Exp Med (1969) 2.36

Chemical suppression of adaptive immunity. Adv Immunol (1967) 2.35

Postnatal expansion of the natural killer and keller cell population in humans identified by the monoclonal HNK-1 antibody. J Exp Med (1982) 2.35

Differential activation requirements for virgin and memory T cells. J Immunol (1988) 2.34

Growth of a pure population of mouse mast cells in vitro with conditioned medium derived from concanavalin A-stimulated splenocytes. Proc Natl Acad Sci U S A (1981) 2.33

NK1.1+ CD4+ CD8- thymocytes with specific lymphokine secretion. Eur J Immunol (1993) 2.32

Effect of antibiotics on the bactericidal activity of human leukocytes. J Lab Clin Med (1968) 2.31

Occurrence of malignancy in immunodeficiency diseases. A literature review. Cancer (1971) 2.31

T-cell depletion of HLA-identical transplants in leukemia. Blood (1991) 2.25

Monoclonal antibody analysis of human T lymphocyte subpopulations exhibiting autologous mixed lymphocyte reaction. Proc Natl Acad Sci U S A (1981) 2.16

Suppression of B-cell differentiation by leukocytes from hypogammaglobulinemic patients. J Clin Invest (1976) 2.15

The short arm of chromosome 11 is a "hot spot" for hypermethylation in human neoplasia. Proc Natl Acad Sci U S A (1988) 2.14

Improved tests for the evaluation of neutrophil function in human disease. J Lab Clin Med (1968) 2.11

A mammalian equivalent of the avian bursa of Fabricius. Lancet (1966) 2.11

Selective deposition of immunoglobulin A1 in immunoglobulin A nephropathy, anaphylactoid purpura nephritis, and systemic lupus erythematosus. J Clin Invest (1980) 2.11

A novel pair of immunoglobulin-like receptors expressed by B cells and myeloid cells. Proc Natl Acad Sci U S A (1997) 2.11

Circulating B-cells in patients with immunodeficiency. Am J Pathol (1972) 2.07

Expression of C3d receptors during human B cell differentiation: immunofluorescence analysis with the HB-5 monoclonal antibody. J Immunol (1984) 2.06

Development and function of the immunoglobulin producing system. I. Effect of bursectomy at different stages of development on germinal centers, plasma cells, immunoglobulins and antibody production. Int Arch Allergy Appl Immunol (1969) 2.06

In vitro generation of B lymphocytes in mouse foetal liver, a mammalian 'bursa equivalent'. Nature (1974) 2.05

Pre-B-cell leukemia. A new phenotype of childhood lymphoblastic leukemia. N Engl J Med (1978) 2.05

Polysplenia: a review of 146 cases. Pediatr Cardiol (1983) 2.05

Kaposi's sarcoma: a review and recent developments. CA Cancer J Clin (1981) 2.05

Ubiquitin-dependent degradation of IkappaBalpha is mediated by a ubiquitin ligase Skp1/Cul 1/F-box protein FWD1. Proc Natl Acad Sci U S A (1999) 2.04

Development of "autoimmune processes" in rabbits after neonatal removal of central lymphoid tissue. Lancet (1965) 2.04

Influence of the human T-lymphotropic virus/lymphadenopathy-associated virus on functions of human lymphocytes: evidence for immunosuppressive effects and polyclonal B-cell activation by banded viral preparations. Proc Natl Acad Sci U S A (1985) 2.04

Prevention of type I diabetes in nonobese diabetic mice by allogenic bone marrow transplantation. Proc Natl Acad Sci U S A (1985) 2.03

Effects of ursodeoxycholic acid and aspirin on the formation of lithogenic bile and gallstones during loss of weight. N Engl J Med (1988) 2.02

Identification of the TCL1 gene involved in T-cell malignancies. Proc Natl Acad Sci U S A (1994) 2.02

Restriction of surface immunoglobulin determinants to lymphocytes of the plasma cell line. J Immunol (1971) 2.01

Phytohaemagglutinin unresponsiveness of lymphocytes from babies with congenital rubella. Nature (1967) 1.99

Mechanism of action of migration inhibitory factor (MIF). I. Evidence for a receptor for MIF present on the peritoneal macrophage but not on the alveolar macrophage. J Exp Med (1972) 1.99

Sequential expression of immunoglobulin on developing mouse B lymphocytes: a systematic survey that suggests a model for the generation of immunoglobulin isotype diversity. J Immunol (1978) 1.99

Gene for familial psoriasis susceptibility mapped to the distal end of human chromosome 17q. Science (1994) 1.99

Transplantation for severe combined immunodeficiency with HLA-A,B,D,DR incompatible parental marrow cells fractionated by soybean agglutinin and sheep red blood cells. Blood (1983) 1.98

Risk factors for chronic graft-versus-host disease after HLA-identical sibling bone marrow transplantation. Blood (1990) 1.98

Rationale for bone marrow transplantation in the treatment of autoimmune diseases. Proc Natl Acad Sci U S A (1985) 1.97

Tissue localization and CD8 accessory molecule expression of T gamma delta cells in humans. J Immunol (1989) 1.96

The nephrotic syndrome of childhood: immunologic, clinical, and pathologic correlations. J Clin Invest (1966) 1.96

Congenital cardiac disease associated with polysplenia. A developmental complex of bilateral "left-sidedness". Circulation (1967) 1.96

T cells can bind antigen via cytophilic IgM antibody made by B cells. J Immunol (1973) 1.95

Humoral and cell-mediated immune responses in fully allogeneic bone marrow chimera in mice. J Exp Med (1980) 1.94

Chronic glomerulonephritis associated with low serum complement activity (chronic hypocomplementemic glomerulonephritis). Medicine (Baltimore) (1970) 1.94

Glomerular localization and transport of aggregated proteins in mice. Lab Invest (1967) 1.94

Human B cell differentiation. II. Pokeweed mitogen-responsive B cells belong to a surface immunoglobulin D-negative subpopulation. J Exp Med (1982) 1.92

Passive hemolysis by serum and cobra venom factor: a new mechanism inducing membrane damage by complement. Proc Natl Acad Sci U S A (1969) 1.92

Phorbol myristate acetate: a mitogen selective for a T-lymphocyte subpopulation. J Exp Med (1977) 1.91

Human lymphocyte differentiation antigens HB-10 and HB-11. II. Differential production of B cell growth and differentiation factors by distinct helper T cell subpopulations. J Immunol (1985) 1.91

B lymphocyte precursors in human bone marrow: an analysis of normal individuals and patients with antibody-deficiency states. J Immunol (1978) 1.88

Rosette-formation with mouse erythrocytes. II. A marker for human B and non-T lymphocytes. Clin Exp Immunol (1976) 1.87