common variable immunodeficiency (cvid) is one of the primary immunodeficiencies, which usually presents with recurrent bacterial infections, particularly in respiratory and gastrointestinal systems and hypogammaglobulinemia. we present here a case of cvid who was suffering from chronic watery diarrhea since 3 months before admission. past medical history was uneventful about recurrent infectio...

Background: Frequent infections is among the most frequent clinical dilemmas for primary care physicians. Immunodeficiency disorders are a heterogeneous group of illnesses that predispose patients to the recurrent infections, autoimmunity and malignancies. The aim of this study was to evaluate the clinical and laboratory data collected for the final diagnosis of patients referred with recurrent...

background: frequent infections is among the most frequent clinical dilemmas for primary care physicians. immunodeficiency disorders are a heterogeneous group of illnesses that predispose patients to the recurrent infections, autoimmunity and malignancies. the aim of this study was to evaluate the clinical and laboratory data collected for the final diagnosis of patients referred with recurrent...

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...

introduction: oral manifestations can be the first signs of human immunodeficiency virus/acquired immunodeficiency syndrome (hiv/aids) and a useful marker for the progression of this disease. the present study aimed to determine the prevalence of oral manifestations and examine their relationship with socio-demographic factors in hiv-positive patients in the health centers affiliated to zahedan...

primary immunodeficiencies constitute a group of highly complex congenital disorders most of which are characterized by a very poor prognosis. allogeneic hematopoietic stem cell transplantation (hsct) has become an established curative treatment approach in many of these disorders, which may be permanently corrected. in this presentation basic and practical aspects of hsct are presented, with a...

how to cite this article: mansouri nejad se, yazdan panah mj, tayyebi meibodi n, ashrafzadeh f, akhondian j, beiraghi toosi m, eslamieh h. griscelli syndrome: a case report. iran j child neurol. 2014 autumn;8(4): 72-75. objective griscelli syndrome (gs) is a rare autosomal recessive immune deficiency disorder that presents with pigmentary dilution of the skin and hair, recurrent skin and pulmon...

patients with organic acidemia are prone to different infections, which lead to acidosis episodes. some studies have evaluated the status of immune system in acidotic phase in these patients, but to the best of our knowledge no study has evaluated the immune system in non-acidotic phase of the disease. in this study, thirty-one patients with organic acidemia were enrolled. for evaluation of hum...

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,...