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Both the innate and adaptive arms of the immune system are involved in the development of autoimmune diseases. The main mechanism of disease is due to adaptive immune cells that are active against self-antigens. These cells can cause major damage to body tissues. Innate lymphoid cells (ILCs) are an important type of innate immune cell, whose role has been highlighted in recent years. ILCs are responsible for some of the inflammation in the pathogenesis of autoimmune diseases. In this review, we discuss the role of ILCs in the immune response, as well as their involvement in various autoimmune diseases.

Introduction The prevalence of undiagnosed primary immunodeficiency diseases is remarkably high and contributes to increasing the rate of morbidity and mortality among this group of patients. Objective To examine the 10 warning sign scoring system in patients suspected of primary immune deficiency and also estimate the diagnostic delay in patients with proven disease. Methods This descriptive cross-sectional study was carried out during the years 2015–2016 in Ali Asghar (AS) Clinic and Hospital. Two hundred patients with suspected primary immune deficiency disease were eligible for inclusion in the study. Multivariable logistic regression analysis was used to determine the relation between findings. Results In this study, the majority of suspected cases of immunodeficiency were males (57%) with a mean age of 3.33 ± 2.89 years. Twenty-one (10.5%) patients were diagnosed with immunodeficiency disease. The mean diagnostic delay among primary immune deficient patients was 2.05 ± 1.7 years. There was a significant relationship between having parental consanguinity (OR = 2.68, 95% CI: 1.07–6.70), allergies (OR = 5.03, 95% CI: 1.13–22.31), vaccine adverse effects (OR = 9.31, 95% CI: 1.24–69.96) and primary immune deficiency diagnosis. No association was observed between age (OR = 0.98, 95% CI: 0.84–1.14), gender (OR = 0.99, 95% CI: 0.39–2.47), immune deficiency scoring (OR = 0.68, 95% CI: 0.31–1.45) and primary immune deficiency diagnosis. Conclusion Ten warning sign scoring system is of less value to consider a patient suspected of having primary immune deficiency. There is a meaningful delay in diagnosis of primary immune deficiencies especially in antibody deficiency defects which seeks further upgrading of knowledge in physicians.

Cholangiocarcinoma (CCA) is the second most frequent primary liver tumor and defined as the heterogeneous group of tumors derived from cells in the biliary tree. Based on the anatomical locations (intrahepatic, perihilar, and distal), there are various approaches to the diagnosis and treatment of CCA. Imaging modalities, staging classifications, understandings around natural behavior of CCA, and therapeutic strategies have had remarkable progress in recent years. This article reviews and discusses the epidemiology, clinical presentation, diagnosis, and treatment modalities of CCA; determines the appropriate inclusion and exclusion criteria for liver transplantation (LT); and defines the risk of disease progression for patients in the waiting list of LT.

OBJECTIVES Protein tyrosine phosphatase non-receptor type 22 gene (PTPN22) single-nucleotide polymorphisms (SNP) have been associated with a number of different autoimmune diseases. This study aimed to investigate the association of five polymorphisms of PTPN22 gene with susceptibility to ulcerative colitis (UC) in Iran. MATERIALS AND METHODS A total of 67 patients diagnosed with UC (35 female and 32 male all under 18 years) and 93 healthy subjects were selected. The samples were genotyped for the, rs12760457, rs2476601, rs1310182, rs1217414, and rs33996649 in PTPN22 gene using real-time polymerase chain reaction (PCR) allelic discrimination TaqMan genotyping assays. RESULTS The frequencies of the rs1310182 A and G alleles, and also the AA and GG genotypes were significantly different between the patient and the control groups (p

The well-being of the health care workforce is the cornerstone of every well-functioning health system. As a result of the pandemic, medical healthcare providers are under an enormous amount of workload pressure along with increased total health expenditures. The overwhelming burden of COVID-19 illness could lead to caregiver burnout. Direct-to-consumer telemedicine can enable patients to connect with their healthcare provider at a distance. This virtual platform could be used by smartphones or webcam-enabled computers and allows physicians to effectively screen patients with early signs of COVID-19 before they reach to hospital.

The prevalence and mortality rate of colorectal cancer have been dramatically rising globally. Currently, colorectal cancer is emerging as the fourth leading cause of death and the third most common malignancy worldwide. The major drawback in colorectal cancer treatment is related to severe adverse events of both chemotherapy and radiation therapy that lead to toxicity and inflammation. Recently, melatonin as an antioxidant, immune-stimulant, and antimutagenic agent has been noticed. Different studies worked on the molecular role of melatonin on carcinogenesis progression. Overall, the anticancer activity of melatonin, combined with its actions via multiple signaling pathways, is considered hugely exciting to use this drug as a possible treatment strategy to cure cancer. Apart from its anticancer potential, this drug has shown to induce modulation of chemotherapy toxicity and improving its therapeutic efficacy. The present review aimed to discuss the possible role of melatonin usage in management of colorectal cancer.

BACKGROUND: Pediatric-onset systemic lupus erythematosus (pSLE) accounts for about 10%-20% of all patients with SLE. Deficiencies in early complement components of the classical pathway are the strong genetic risk factor for the development of SLE. In this study, clinical and laboratory manifestations of both complement-deficient and normal complement pSLE patients were compared. MATERIALS AND METHODS: To investigate clinical and immunological manifestations of pSLE in Iran, 36 consecutive pSLE patients (onset before 18 years) who were followed up over a period of 2 years, were studied. Complement C1q and C2 levels were measured using radial immunodifusion assay and complement C3 and C4 levels were measured using nephelometry. Medical records were retrospectively evaluated from patient database of Children Medical Center Hospital. Data were assessed through descriptive analysis (confidence interval = 95%), paired t-test, and Pearson correlation test. RESULTS: Twenty-one patients (58%) had at least one component of complement deficiency. Ten patients (27%) had low C1q level, 11 patients (30.5%) had low C2, nine patients (25%) had low C3, and four patients (11%) had low C4 level. Serum level of complement in pSLE was significantly lower than the control group, except C4 (P = 0.005). The low C1q patients had an earlier age of onset of disease (P < 0.0001). The cutaneous manifestations were more frequent and much more severe in pSLE with low complement (100% vs. 73%). The frequency of renal and musculoskeletal symptoms was equal, but renal morbidity was more common in pSLE with low complement. Positivity for anti-ds-DNA was less common in pSLE with low complement (71% vs. 86%). CONCLUSION: In pSLE patients with early disease onset and more aggressive SLE manifestations and negative anti-ds-DNA test, complement deficiency should be considered.

Background: We conducted a meta-analysis to investigate the possible effects of vitamin D deficiency on clinical outcomes of critically-ill children.Methods: We searched Scopus-Embase and PubMed-Medline databases to find eligible observational articles. Odds ratios (ORs) and 95% confidence intervals (CIs) were used to estimate the strength of associations.Results: Seventeen articles (eighteen studies) involving 2987 critically ill patients met our inclusion criteria. Vitamin D deficiency was not associated with increased mortality. A significant association was only observed in very high developed countries between vitamin D deficiency and risk of sepsis [OR (95%CIs): 2.65 (1.30, 5.41)] and ventilation support requirement [OR (95%CIs): 1.35 (1.03, 1.77)].Conclusion: Our findings suggest that vitamin D deficiency is not associated with higher mortality among critically ill children but increases susceptibility to sepsis and the need for ventilator support in critical care settings.

Background: Procalcitonin (PCT) has been shown as a reliable diagnostic biomarker for identifying sepsis and bacterial infection. Objectives: The present study examined the sensitivity and specificity of PCT evaluation in the diagnosis of infectious diseases after liver transplantation. Methods: The present prospective cohort study was conducted on postoperative liver transplant (LT) patients in the liver transplant ward of Imam Khomeini Hospital Complex affiliated to Tehran University of Medical Sciences between January 2014 and March 2015. Serum PCT levels were evaluated before transplantation and one, two, and six or seven days post-operation by semi-quantitative kits with a 30 seconds response. Results: A total number of 28 LT patients were enrolled in this study. The mean patients’ ages were 48.6 ± 10.9 (range 26 - 66) years old. Serum PCT levels in all patients were < 0.5 ng/mL prior to the operation. At first and second days post-operation, PCT levels were more than 2 ng/mL in all patients and decreased to 0.5 ng/mL after 6 or 7 days in 23 patients. Serum PCT level of higher than 5 ng/mL on the first and second days post-surgery with a sensitivity of 77.8% and specificity of 79% had the most accuracy for the infection diagnosis. The PCT level more than 5 ng/mL in the sixth and seventh days had a 100% positive predictive value in the infection prediction. Conclusions: Serial evaluations of serum PCT after liver transplantation had good sensitivity and specificity to predict postoperative infection. Increased serum PCT level within the first days after transplantation and/or failure to decrease after one week, predicts infectious complications and undesirable outcome.

Defects in interleukin-10 (IL10) and interleukin-10 receptors (IL10R) are closely related to very early onset (infantile) inflammatory bowel disease (VEO-IBD). In the present study, we report a novel homozygous null mutation within interleukin-10 receptor B (IL10RB) gene in a child presenting with severe VEO-IBD. In accordance with previous reports, our patient manifested with chronic diarrhea, failure to thrive, intermittent fever and multiple anal ulcers associated with Candidiasis. Homozygous null mutation within IL10RB gene (c.92C > T, p.S31P) affecting the extracellular domain of protein was discovered in this patient. In conclusion, the diagnosis of IL-10R gene mutations should always be considered as a possible cause of refractory diarrhea and failure to thrive. Mutation analysis could help detect the genetic defects associated with these clinical manifestations and to determine the most appropriate treatment option for patients affected by this disease.

[Background] Common variable immunodeficiency (CVID) is a heterogeneous group of disorders, characterized by recurrent upper and lower respiratory tract infections and some noninfectious clinical complications., [Objective] To provide a detailed evaluation of respiratory presentations and complications in a cohort of Iranian patients with CVID., [Methods] A retrospective cohort study was conducted on 245 CVID patients who were recorded in the Iranian primary immunodeficiency disorders registry network. Respiratory manifestations were evaluated by reviewing clinical hospital records, immunologic findings, pulmonary function tests (PFT), and high-resolution computed tomography (HRCT) scans., [Results] Most of the patients (n = 208, 85.2%) had experienced at least 1 episode of acute respiratory manifestation, and pneumonia was observed in 31.6 % (n = 77) of cases as a first disease manifestation. During the follow-up, pneumonia, sinusitis, and otitis media were documented in 166 (68.6%), 125 (51.2%), and 103 (42.6%) cases, respectively. Abnormal PFT measurements were documented in 53.8% of patients. Among these patients, 21.5% showed restrictive changes, whereas 18.4% of patients showed an obstructive pattern. Bronchiectasis was the most frequent radiological finding, confirmed in 27.2% of patients. Patients with bronchiectasis were older at the time of immunodeficiency diagnosis (P < .001) and had longer diagnosis delay (P < .001) when compared with patients without bronchiectasis., [Conclusion] This study highlights the importance of monitoring the respiratory tract system even in asymptomatic patients. Pulmonary function tests and CT scans are the most commonly used techniques aiming to identify these patients early, aiming to reduce the rate of long-term respiratory complications., Funding Sources: This research was supported by the Tehran University of Medical Sciences, Tehran, Iran (grant no. 35741).

Hyper Immunoglobulin M syndrome (HIGM) is a rare primary immunodeficiency disorder characterized by low or absent levels of serum IgG, IgA, IgE and normal or increased levels of serum IgM. Various X-linked and autosomal recessive/dominant mutations have been reported as the underlying cause of the disease. Based on the underlying genetic defect, the affected patients present a variety of clinical manifestations including pulmonary and gastrointestinal complications, autoimmune disorders, hematologic abnormalities, lymphoproliferation and malignancies which could be controlled by multiple relevant therapeutic approaches. Herein, the epidemiology, pathogenesis, clinical manifestations, diagnosis, management, prognosis and treatment in patients with HIGM syndrome have been reviewed.

Introduction Primary immunodeficiency diseases (PID) are a heterogeneous group of inherited disorders, characterised by recurrent severe infections, autoimmunity and lymphoproliferation. Despite impressive progress in identification of novel PID, there is an unfortunate lack of awareness among physicians in identification of patients with PID, especially in non-capital cities of countries worldwide. Result This study was performed in a single-centre paediatric hospital in Northern Iran during a 21-year period (1994–2015). Ninety-four patients were included in this study. The majority of cases had antibody deficiencies (37.23%), followed by well-defined syndromes with immunodeficiency in 16 (17.02%), phagocytic disorders in 15 patients (15.95%), complement deficiencies in 15 patients (15.95%), immunodeficiencies affecting cellular and humoral immunity in nine patients (9.57%), disease of immune dysregulation in three (3.19%), and defects in intrinsic and innate immunity in one (1.06%). Conclusion It seems that there are major variations in frequency of different types of PID in different regions of a country. Therefore, reporting local data could provide better ideas to improve the local health care system strategists and quality of care of PID patients.