• Users Online: 133
  • Print this page
  • Email this page
ORIGINAL ARTICLE
Year : 2021  |  Volume : 11  |  Issue : 6  |  Page : 262-268

Clinical and laboratory profile of children with primary immunodeficiency - Perspective from a developing country


1 Department of Pediatrics, St. Johns Medical College Hospital, Bengaluru, Karnataka, India
2 Division of Pediatric Hematology and Oncology, St. Johns Medical College Hospital, Bengaluru, Karnataka, India
3 Department of Pediatrics, Aster CMI Hospital, Bengaluru, Karnataka, India

Correspondence Address:
Dr. Ranjini Srinivasan
Department of Pediatrics, St. Johns Medical College Hospital, Bengaluru - 560 034, Karnataka
India
Login to access the Email id

Source of Support: None, Conflict of Interest: None


DOI: 10.4103/cmrp.cmrp_74_21

Rights and Permissions

Aim: The aim of the study was to describe the clinical and laboratory features of children diagnosed with primary immunodeficiency from India. Background: Primary immunodeficiency disorders (PIDs) are a diverse group of conditions with heterogeneous presentation. Materials and Methods: A retrospective chart review of clinical and laboratory data of 40 children with primary immunodeficiency between 1 month and 18 years of age was carried out. A diagnosis of primary immunodeficiency was made based on the standard criteria. Results: Among the 40 children reviewed, males were more affected than females (1.8:1). Seventy per cent of the patients had onset of symptoms before 1 year of age. Recurrent fever (50%) was the most common presentation. Various infections included pneumonia (45%), gastrointestinal infections (18%), oral thrush (15%), recurrent otitis media (7.5%) and recurrent skin infections (5%). Non-infective complications included neurological manifestations such as developmental delay and seizures (10%), growth failure (38%) and cytopenia. Patients were categorised into combined humoral and cellular immunodeficiency (30%), predominant antibody deficiencies (12%), diseases of immune dysregulation (32%), congenital deficiencies in phagocytes (10%) and deficiencies in innate and intrinsic immunity (15%). A genetic confirmation of the immunodeficiency could be obtained only in half of all patients (49%). Conclusions: Children with PIDs can present with typical manifestations such as recurrent life-threatening infections or may have more unusual presentations such as cytopenia or unexplained growth failure.


[FULL TEXT] [PDF]*
Print this article     Email this article
 Next article
 Previous article
 Table of Contents

 Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
 Related articles
 Citation Manager
 Access Statistics
 Reader Comments
 Email Alert *
 Add to My List *
 * Requires registration (Free)
 

 Article Access Statistics
    Viewed628    
    Printed8    
    Emailed0    
    PDF Downloaded77    
    Comments [Add]    

Recommend this journal