XML Feed for RxPG News   Add RxPG News Headlines to My Yahoo!   Javascript Syndication for RxPG News

Research Health World General
 
  Home
 
 Latest Research
 Cancer
 Psychiatry
 Genetics
 Surgery
 Aging
 Ophthalmology
 Gynaecology
 Neurosciences
 Pharmacology
 Cardiology
 Obstetrics
 Infectious Diseases
 Respiratory Medicine
 Pathology
 Endocrinology
 Immunology
  Autoimmune Diseases
  Immunosupressants
  Monoclonal Antibodies
 Nephrology
 Gastroenterology
 Biotechnology
 Radiology
 Dermatology
 Microbiology
 Haematology
 Dental
 ENT
 Environment
 Embryology
 Orthopedics
 Metabolism
 Anaethesia
 Paediatrics
 Public Health
 Urology
 Musculoskeletal
 Clinical Trials
 Physiology
 Biochemistry
 Cytology
 Traumatology
 Rheumatology
 
 Medical News
 Health
 Opinion
 Healthcare
 Professionals
 Launch
 Awards & Prizes
 
 Careers
 Medical
 Nursing
 Dental
 
 Special Topics
 Euthanasia
 Ethics
 Evolution
 Odd Medical News
 Feature
 
 World News
 Tsunami
 Epidemics
 Climate
 Business
 
 India
Search

Last Updated: Nov 18, 2006 - 12:32:53 PM
Report
Clinical Immunology

Immunology Channel
subscribe to Immunology newsletter

Latest Research : Immunology

   DISCUSS   |   EMAIL   |   PRINT
Report shows significant improvement in survival rates for patients with X-linked agammaglobulinemia
Jan 14, 2006 - 6:42:00 PM, Reviewed by: Dr. Rashmi Yadav

"In the last 20 years the outlook for patients with XLA has significantly improved, thanks to earlier diagnosis and improved gamma globulin therapy."

 
Individuals who have a rare genetic immune system disorder that prevents them from making antibodies nevertheless appear to be moderately healthy and lead productive lives, according to results of a study by investigators at St. Jude Children's Research Hospital. A report on this study appears in the current online edition of Clinical Immunology.

The study of 41 adults with X-linked agammaglobulinemia (XLA) showed that they can function as relatively healthy, productive individuals, even though they remain vulnerable to chronic, low-grade infections. These individuals had a mean age of 4 years (range 1 month to 53 years) when their diseases were diagnosed; and 27 of the patients had family histories of XLA. The study was based on results of a questionnaire filled out by each participant concerning current and past medical problems and quality of life.

"Until we did this study, there was almost nothing in the medical literature about adults with XLA," said Mary Ellen Conley, M.D., a member of the Department of Immunology at St. Jude and senior author of the report. "In fact, old reports we read stated that the vast majority of these patients have chronic lung disease by age 15. We and other physicians were quite surprised at how well these patients are doing with the proper care."

XLA is a rare disease that is inherited through a mutation in the Btk gene on the X-chromosome--one of the two types of sex chromosomes. Males with this disease have very low levels of infection-fighting proteins called antibodies. Treatment includes aggressive use of antibiotics and replacement of the missing antibodies with gamma globulin.

"Almost all of the adults with XLA had chronic medical problems; however, these problems did not interfere with normal daily activities, and the quality of life in this group was equivalent to that of the general male population of the United States," said Vanessa Howard, R.N., M.S.N., a nurse practitioner for the Immunology service at St. Jude and first author of the paper. "In the past, the majority of patients with XLA died of acute or chronic infections in the first two decades of life," Howard noted. "But in the last 20 years the outlook for patients with XLA has significantly improved, thanks to earlier diagnosis and improved gamma globulin therapy. Our study is reassuring and helps to put into perspective the ability of such patients to thrive with proper care, despite this potentially devastating disease."

Forty of the 41 patients were receiving intravenous infusions of gamma globulin every two to four weeks and 16 (39 percent) were taking chronic (long-term) antibiotics in order to prevent infections, the St. Jude team reported.

In addition, 17 patients (41 percent) had not been hospitalized for infection since diagnosis and 28 (68 percent) had not been hospitalized in the past five years. Moreover, 34 (83 percent) of these patients rated their health as good, very good or excellent; 35 (86 percent) of patients missed fewer than 10 days of work or school due to illness in the past year; and 18 (44 percent) reported they had not missed any work or school.

Thirteen of the 41 patients reported that they had chronic lung disease, and 33 indicated that they had one or more episodes of sinusitis in the preceding year. Other diseases that were common but not debilitating among this group were arthritis, diarrhea and skin infections.

Several factors may have contributed to the increased survival of this group of patients. When compared to all patients with XLA, a high percentage of the patients included in this study had mild mutations in Btk. In addition, most had older brothers or cousins with XLA. Thus, they were probably diagnosed earlier and treated more aggressively.

Other authors of the study include Mehmet Kocak (St. Jude); Jeffrey M. Green (Children's Hospital of Philadelphia); Savita Pahwa (University of Miami School of Medicine, Fla.); Jerry A. Winkelstein (The Johns Hopkins University School of Medicine; Baltimore, Md.); and John M. Boyle (SRBI; Silver Spring, Md.).
 

- A report on this study appears in the current online edition of Clinical Immunology.
 

www.stjude.org

 
Subscribe to Immunology Newsletter
E-mail Address:

 

This work was supported in part by the National Institutes of Health, the National Cancer Institute, ALSAC and the Federal Express Chair of Excellence.

St. Jude Children's Research Hospital
St. Jude Children's Research Hospital is internationally recognized for its pioneering work in finding cures and saving children with cancer and other catastrophic diseases. Founded by late entertainer Danny Thomas and based in Memphis, Tenn., St. Jude freely shares its discoveries with scientific and medical communities around the world. No family ever pays for treatments not covered by insurance, and families without insurance are never asked to pay. St. Jude is financially supported by ALSAC, its fund-raising organization. For more information, please visit www.stjude.org.

Contact: Carrie Strehlau
[email protected]
901-495-2295
St. Jude Children's Research Hospital


Related Immunology News

Pregnant women with lupus are at higher risk for complications
Molecular 'signature' protects cells from viruses
Discovery in the evolution of the immune system absorbing cells
Leeds University study shows eculizumab may be an effective therapy for PNH
Research Reveals Inner Workings of Immune System �Thermostat�
CD23 Protein in Stool Samples may Indicate Food Allergy
Molecular signals triggering maturation of natural killer cells uncovered
New method to analyse the Major Histocompatibility Complex (MHC) of the human genome
Front Line Immune Cells Mature in Four Stages - Study
Caspase-12 gene that shuts down immune system is found in 20% of people of African descent


For any corrections of factual information, to contact the editors or to send any medical news or health news press releases, use feedback form

Top of Page

 

© Copyright 2004 onwards by RxPG Medical Solutions Private Limited
Contact Us