[Primary immunodeficiencies in seriously ill children: Report of 3 clinical cases].

Distribution of Primary Immunodeficiency Disorders Diagnosed in a Tertiary Referral Center, Tehran, Iran (2006-2013)

Background: Primary immunodeficiency disorders (PID) are a group of hereditary disorders characterized by an increased susceptibility to severe and recurrent infections, autoimmunity, lymphoproliferative disorders, and malignancy. Objective: To evaluate the demographic and clinical data of PID patients diagnosed in a referral pediatric hospital. Method: All PID cases with a confirmed diagnosis,...

Primary immunodeficiency diseases (PIDs) is a diverse group of diseases, characterized by a defect in the immune system. These patients are susceptible to recurrent respiratory infections, gastrointestinal problems, autoimmune diseases, and malignancies. In most cases, patients with primary immunodeficiency disorders have genetic defects and are monogenic disorders that follow a simple Mendelia...

A Review of Primary Immunodeficiency Diseases with Skin Manifestations

Introduction: Primary immunodeficiencies (PID) are rare heterogeneous disorders with defects in which one or more components of the immune system are malfunctioning. Clinical presentations of the patients according to type of immunodeficiency are variable. The majority of these patients are susceptible to infections depending on the type of disorder. In these patients, one of the most important...

Description of the Clinical Course and Severity Score Progression in Critically Ill Children with Acute Bronchiolitis on High-Flow Nasal Cannula Support

Background and Objective: Bronchiolitis is one of the main causes of morbidity and mortality in children. High-flow nasal cannulas (HFNCs) are an alternative for managing moderate to severe cases. Our aim was to describe the outcomes in critically ill children with bronchiolitis who receive HFNC support. Materials and Methods: This was a retrospective cohort study of critically ill children wh...

Severe axillary lymphadenitis after BCG vaccination: alert for primary immunodeficiencies.

The bacilli Calmette-Guérin (BCG) vaccine is administered to all newborns in countries where tuberculosis is endemic. Immunocompromised hosts, namely patients with human immunodeficiency virus infection or primary immunodeficiencies, are especially prone to serious complications from this vaccine. We report three cases of BCG disease in children with primary immunodeficiencies: one with a parti...