Impact of delayed diagnosis in children with primary antibody deficiencies.
نویسندگان
چکیده
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 PADs, who were referred to our center during a 25-year period, were enrolled in this study. Each episode of infection, before making the PADs diagnosis, got a score of 5 or 10 based on the severity of infections. RESULTS The diagnosis was made with median delay of 34.5 months, when the patients had mean score of 94.48 ± 52.89. There was a significant direct association between this scoring system and delay diagnosis. The score of 50 was considered as the cutoff point in our patient group. In this score, the suspicions to PADs in more than 90% of patients true positively lead to diagnosis of PADs. CONCLUSION Although diagnosis delay significantly decreased over time, PADs still continue to be diagnosed late. Based on the results of this study, the assessment of immune system should be performed in the patients with 50 total score or about 25 score per year.
منابع مشابه
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...
متن کاملDistribution 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,...
متن کاملبررسی میزان سرمی anti-HBs در کودکانی که در دوره نوزادی با واکسن نوترکیب هپاتیت B واکسینه شده اند
Introduction: Vaccination with the major surface antigen of hepatitis B virus (HBsAg) induces anti-HBs antibody production and level of 10 IU/L is considered protective. It has been shown that the level of anti-HBs antibody does wane after vaccination. The aim of this study was to evaluate the persistence of anti-HBs antibodies in healthy Iranian children 10 years after primary vaccination. Me...
متن کاملX-linked agammaglobulinemia caused by new mutation in BTK gene: a case report.
AIM Primary immunodeficiencies (PID) are becoming a recognized public health problem worldwide. The most important subgroup of these disorders are the antibody deficiencies. X-linked agammaglobulinaemia was the first described entity of this group and is characterised by early onset of recurrent bacterial infections, profound deficiency of all immunoglobulin isotypes and markedly reduced number...
متن کاملLangerhans cell histiocytosis of the clavicle in a 10-years-old girl
Langerhans cell histiocytosis (LCH) is an abnormal proliferation of Langerhans cells in one or more organs; bone is the most frequently involved organ in which the skull is the most frequent .The clavicle is rarely the primary site of the LCH, thereby the timely diagnosis is often delayed and most of the patients are primarily treated for acute or chronic osteomyelitis. Herein we report a 10 ye...
متن کاملذخیره در منابع من
با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید
برای دانلود متن کامل این مقاله و بیش از 32 میلیون مقاله دیگر ابتدا ثبت نام کنید
ثبت ناماگر عضو سایت هستید لطفا وارد حساب کاربری خود شوید
ورودعنوان ژورنال:
- Journal of microbiology, immunology, and infection = Wei mian yu gan ran za zhi
دوره 44 3 شماره
صفحات -
تاریخ انتشار 2011