Search

Although presentation of COVID‐19 in patients with immunodeficiency could be mild, it should not be missed, while early diagnosis and appropriate treatment can survive infected patients. Because even severe infections in PID patients may be presented with few symptoms and signs, this diagnosis should be considered in those immunocompromised patients who have exacerbating preexisting symptoms.

Primary immunodeficiency disorders (PIDs) are often associated with autoimmune disease due to the dysregulation of the immune system and distributed central and peripheral tolerance. In many inborn immune deficiencies, lymphocytes may be present but they are dysfunctional, allowing for the development of excessive autoreactivity skipping negative selection process and resultant autoimmune disease. This chapter summarizes the clinical approaches to the main immune dysregulations including hemophagocytic lymphohistiocytosis syndromes, autoimmunity with lymphoproliferation and autoimmunity without lymphoproliferation.

Background. In the recent coronavirus disease 2019 (COVID-19) pandemic, millions of people have been affected so far. Clinical manifestations and natural history of infections have a very wide range, which is important to know it in various underlying diseases. Although children are less affected than adults, its presentation especially in those with underlying diseases should not be neglected.Case presentation. Herein we report the first case of specific antibody deficiency, who was affected with COVID-19. The patient suffered from rhinorrhea and modestly increased in productive cough, but real-time reverse transcription polymerase chain reaction was positive for COVID-19.Conclusions. Although presentation of COVID-19 in patients with immunodeficiency could be mild, it should not be missed, while early diagnosis and appropriate treatment can survive affected patients.

Background: Cystic fibrosis (CF) is a multi-systemic disorder with an autosomal recessive inheritance. It is characterized by recurrent pulmonary infections and gastrointestinal problems, particularly pancreas insufficiency. Objectives: The aim of this study was to evaluate growth peptides in CF patients. Methods: This cross sectional study was approved by Mashhad University of Medical Sciences ethical committee. Patients were between six months to 18 years old, who were referred to Dr. Sheikh Hospital subspecialty clinic. They were enrolled for the study after obtaining a written constant. Demographic data was recorded. Of all cases, 10-cc blood sample was obtained and growth peptide levels were determined and measured. Data were analyzed by SPSS version 16 and Mann Whitney and χ2 tests were used. Results: Patients’ mean age at onset was 3.4 ± 1.1 months. Overall, 36 (60%) children were male and 24 (40%) were female. Mean body mass index (BMI) was 15.9 ± 3.2 kg/m2. Mean levels of growth hormone was 1.9 ± 0.37 (ng/mL), insulin-like growth factor 1 (IGF1) was 85.8 ± 9.6 microg/L and insulin-like growth factor binding protein 3 (IGFBP3) level was 18538 ± 10643 pg/mL. Conclusions: The mean serum levels of GH, IGF1, and IGFBP3 in CF children with growth failure was lower than children without growth failure. Regarding the relatively high prevalence of growth disorders in children with cystic fibrosis, selected, appropriate, and standard medical treatments could improve the outcome for these children.