High- vs low-dose immunoglobulin therapy in the long-term treatment of X-linked agammaglobulinemia.
The data of 29 patients with X-linked agammaglobulinemia, who received immunoglobulin replacement therapy between 1965 and 1990, were analyzed for dose-dependent long-term results concerning infectious complications. Patients who received high-dose intravenous immunoglobulin replacement (greater than 400 mg/kg every 3 weeks) showed a significant increase in trough serum IgG levels and a significant decrease in the incidence of pneumonias and the number of days spent in the hospital compared with patients receiving intravenous immunoglobulin low-dose (less than 200 mg/kg every 3 weeks) or intramuscular immunoglobulin (less than 100 mg/kg every 3 weeks) treatment. Improvements in therapeutic outcome were particularly evident when high-dose intravenous immunoglobulin replacement therapy was started before the age of 5 years. Bacterial meningitis, chronic pulmonary disease, and bronchiectasis occurred in the intramuscular immunoglobulin group but did not occur in either of the intravenous immunoglobulin groups. High-dose intravenous immunoglobulin therapy may have a positive impact on the clinical course and may prevent severe complications in patients with X-linked agammaglobulinemia.
Liese
J G
JG
Department of Pediatrics, University of Munich, Federal Republic of Germany.
Wintergerst
U
U
Tympner
K D
KD
Belohradsky
B H
BH
D000208:Acute Disease; D000293:Adolescent; D000328:Adult; D000361:Agammaglobulinemia / Q000097:blood / Q000150:complications / Q000235:genetics / Q000628:therapy*; D000367:Age Factors; D002648:Child; D002675:Child, Preschool; D002908:Chronic Disease; D004305:Dose-Response Relationship, Drug; D008040:Genetic Linkage; D006801:Humans; D007116:Immunization, Passive; D007074:Immunoglobulin G / Q000097:blood; D016756:Immunoglobulins, Intravenous / Q000008:administration & dosage* / Q000009:adverse effects / Q000627:therapeutic use; D015994:Incidence; D007223:Infant; D007239:Infections / Q000453:epidemiology / Q000209:etiology; D008297:Male; D012189:Retrospective Studies; D013997:Time Factors; D016896:Treatment Outcome; D014797:Vital Capacity; D014960:X Chromosome
D007074:Immunoglobulin G; D016756:Immunoglobulins, Intravenous