Linked Life Data

9414283

Source:http://linkedlifedata.com/resource/pubmed/id/9414283

Statements in which the resource exists as a subject.
PredicateObject
rdf:type
lifeskim:mentions
pubmed:issue
1
pubmed:dateCreated
1998-2-2
pubmed:abstractText
Primary autoimmune neutropenia (AIN) is caused by granulocyte-specific autoantibodies and occurs predominantly in infancy. Clinical presentation and diagnosis have not been well established, resulting in burdening diagnostic investigations and unnecessary treatment with granulocyte colony-stimulating factor (G-CSF). In the present study, clinical, laboratory, and immunologic data of 240 infants with primary AIN were evaluated. Suspected association with parvovirus B19 infection was investigated using serologic and DNA-based methods. Primary AIN was mainly diagnosed at the age of 5 to 15 months but was observed as early as day 33 of life. In 90% of the cases, AIN was associated with benign infections despite severe neutropenia. Spontaneous remission, shown by 95% of the patients, usually occurred within 7 to 24 months. Autoantibodies in the patient's sera were not always present, and screening had to be repeated several times until antibody detection succeeded. About 35% of the autoantibodies showed preferential binding to granulocytes from NA1 and NA2 homozygous donors. Bone marrow was typically normocellular or hypercellular, with a variably diminished number of segmented granulocytes. A significant association with parvovirus B19 infection was not found. Symptomatic treatment with antibiotics was sufficient in most patients. Eighty-nine percent of the patients received antibiotics (cotrimoxazole) for prophylaxis of infections. For severe infections or for surgical preparation, G-CSF, corticosteroids, and intravenous IgG were administered, resulting in increased neutrophil counts in 100%, 75%, and 50% of the patients treated, respectively. In combination with the detection of granulocyte-specific antibodies, the typical clinical picture allowed diagnosis of AIN without burdening investigations. Treatment with G-CSF was found to be a reliable alternative to temporarily increase the neutrophil count.
pubmed:language
eng
pubmed:journal
pubmed:citationSubset
AIM
pubmed:chemical
pubmed:status
MEDLINE
pubmed:month
Jan
pubmed:issn
0006-4971
pubmed:author
pubmed:issnType
Print
pubmed:day
1
pubmed:volume
91
pubmed:owner
NLM
pubmed:authorsComplete
Y
pubmed:pagination
181-6
pubmed:dateRevised
2011-11-17
pubmed:meshHeading
pubmed-meshheading:9414283-Adrenal Cortex Hormones, pubmed-meshheading:9414283-Anti-Bacterial Agents, pubmed-meshheading:9414283-Antibodies, Viral, pubmed-meshheading:9414283-Antibody Specificity, pubmed-meshheading:9414283-Autoantibodies, pubmed-meshheading:9414283-Autoantigens, pubmed-meshheading:9414283-Autoimmune Diseases, pubmed-meshheading:9414283-Bacterial Infections, pubmed-meshheading:9414283-Bone Marrow, pubmed-meshheading:9414283-Child, Preschool, pubmed-meshheading:9414283-DNA, Viral, pubmed-meshheading:9414283-Disease Progression, pubmed-meshheading:9414283-Female, pubmed-meshheading:9414283-Granulocyte Colony-Stimulating Factor, pubmed-meshheading:9414283-Humans, pubmed-meshheading:9414283-Immunoglobulins, Intravenous, pubmed-meshheading:9414283-Immunosuppressive Agents, pubmed-meshheading:9414283-Infant, pubmed-meshheading:9414283-Male, pubmed-meshheading:9414283-Neutropenia, pubmed-meshheading:9414283-Neutrophils, pubmed-meshheading:9414283-Parvoviridae Infections, pubmed-meshheading:9414283-Parvovirus B19, Human, pubmed-meshheading:9414283-Recombinant Proteins, pubmed-meshheading:9414283-Remission, Spontaneous
pubmed:year
1998
pubmed:articleTitle
Diagnosis and clinical course of autoimmune neutropenia in infancy: analysis of 240 cases.
pubmed:affiliation
Institute for Clinical Immunology and Transfusion Medicine, Justus-Liebig University, Giessen, Germany.
pubmed:publicationType
Journal Article, Research Support, Non-U.S. Gov't