Primary Antibody Deficiencies at Queen Rania Children Hospital in Jordan: Single Center Experience

Authors

  • Adel M Wahadneh Immunology, Allergy and Rheumatology Division, Queen Rania Children Hospital, King Hussein Medical Center, Amman, Jordan
  • Mohammad A. Almutereen Immunology, Allergy and Rheumatology Division, Queen Rania Children Hospital, King Hussein Medical Center, Amman, Jordan
  • Mohammad E Abu-Shukair Immunology, Allergy and Rheumatology Division, Queen Rania Children Hospital, King Hussein Medical Center, Amman, Jordan
  • Raed M. Alzyoud Immunology, Allergy and Rheumatology Division, Queen Rania Children Hospital, King Hussein Medical Center, Amman, Jordan
  • Zeyad M.Habahbeh Immunology, Allergy and Rheumatology Division, Queen Rania Children Hospital, King Hussein Medical Center, Amman, Jordan
Abstract:

Background: Primary antibody deficiency, the most common primary immunodeficiency disorder, represents a heterogeneous spectrum of conditions caused by a defect in any critical stage of B cell development and is characterized by impaired production of normal amounts of antigen-specific antibodies. Objective: This retrospective study aimed at description and analysis of demographic, clinical, immunological features and complications of subjects diagnosed with primary antibody deficiency at a referral center in Jordan. Methods: The medical records of pediatric patients who were diagnosed as primary antibody deficiency (PAD) during the period from January 2006 to June 2013 were reviewed. Patients were diagnosed as PADs based on the Pan-American Group for Immunodeficiency (PAGID) and the European Society for Immunodeficiency (ESID) diagnostic criteria. Results: A total number of 53 patients with PAD were identified; 37(70%) males and 16(30%) females, 16(30%) patients with congenital agammaglobulinemia, 16(30%) patients with common variable immunodeficiency, 4(7.5%) patients with IgG subclass deficiency, 10(19%) cases with transient hypogammaglobulinemia of infancy and 7(13.5%) patients as undefined PAD. The most common infection among patients was pneumonia (62%); followed by suppurative otitis media in 49% of patients. Cytopenia was the most noted autoimmune association and was found at prevalence of 22 %, other autoimmune associations (17%) including inflammatory arthritis, discoid lupus, inflammatory bowel disease, vasculitis and celiac disease. The prevalence of long-term complications was 58%, the most frequent ones were; stunted growth in 13%, bronchiectasis and lymphoproliferation in 11% for each. Conclusions: Our results indicated that congenital agammaglobulinemia and common variable immunodeficiency are the most frequent primary antibody deficiency in our patients. The awareness of families, general population as well as primary health physicians is crucial in the establishment of early diagnosis and prompt commencement of appropriate therapy for PADs.

Upgrade to premium to download articles

Sign up to access the full text

Already have an account?login

similar resources

primary antibody deficiencies at queen rania children hospital in jordan: single center experience

background: primary antibody deficiency, the most common primary immunodeficiency disorder, represents a heterogeneous spectrum of conditions caused by a defect in any critical stage of b cell development and is characterized by impaired production of normal amounts of antigen-specific antibodies. objective: this retrospective study aimed at description and analysis of demographic, clinical, im...

full text

Primary antibody deficiencies at Queen Rania Children Hospital in Jordan: single center experience.

BACKGROUND Primary antibody deficiency, the most common primary immunodeficiency disorder, represents a heterogeneous spectrum of conditions caused by a defect in any critical stage of B cell development and is characterized by impaired production of normal amounts of antigen-specific antibodies. OBJECTIVE This retrospective study aimed at description and analysis of demographic, clinical, im...

full text

Antibiotic Prescribing Patterns in Outpatient Emergency Clinics at Queen Rania Al Abdullah II Children's Hospital, Jordan, 2013.

OBJECTIVE To investigate antibiotics prescribing patterns in the outpatient pediatric emergency clinic at Queen Rania Al Abdullah II Children's Hospital at Royal Medical Services in Amman, Jordan. METHODS The data was collected from the emergency pharmacy over the period of a -five consecutive months. The methodology recommended by the World Health Organization for investigating drug use in a...

full text

Genotype Pattern of Pediatric Familial Mediterranean Fever in Jordan: A Single Center Experience

Background Familial Mediterranean fever (FMF) is an autosomal recessive autoinflammatory disorder caused by mutations in the MEFV gene. The disease is especially common among Mediterranean ancestry, mostly Armenian, Turkish, Jewish and Arab populations. We aimed to describe genotype pattern of FMF in the Jordanian children and to compare it with other populations. Materials and Methods A retros...

full text

Impact of delayed diagnosis in children with primary antibody deficiencies.

BACKGROUND Primary antibody deficiencies (PADs) are heterogeneous group of disorders, characterized by hypogammaglobulinemia and increased susceptibility to recurrent infections. To evaluate the diagnostic delay in Iranian PADs in association with their infections, we scored such manifestations to find an association between such delay and the scoring system. METHODS Forty-eight patients with...

full text

My Resources

Save resource for easier access later

Save to my library Already added to my library

{@ msg_add @}


Journal title

volume 11  issue 1

pages  49- 58

publication date 2014-03-01

By following a journal you will be notified via email when a new issue of this journal is published.

Hosted on Doprax cloud platform doprax.com

copyright © 2015-2023