Clinical and immunological characteristics of 69 leukocyte adhesion deficiency-I patients.

BACKGROUND: In order to support the comprehensive classification of Leukocyte Adhesion Deficiency-I (LAD-I) severity by simultaneous screening of CD11a/CD18, this study assessed clinical, laboratory, and genetic findings along with outcomes of 69 LAD-I patients during the last 15 years. METHODS: Sixty-nine patients (40 females and 29 males) with a clinical phenotype suspected of LAD-I were referred to Immunology, Asthma, and Allergy research institute, Tehran, Iran between 2007 and 2022 for further advanced immunological screening and genetic evaluations as well as treatment, were enrolled in this study. RESULTS: The diagnosis median age of the patients was 6 months. Delayed umbilical cord separation was found in 25 patients (36.2%). The median diagnostic delay time was 4 months (min-max: 0-82 months). Forty-six patients (66.7%) were categorized as severe (CD18 and/or CD11a: below 2%); while 23 children (33.3%) were in moderate category (CD18 and/or CD11a: 2%-30%). During the follow-ups, 55.1% of children were alive with a mortality rate of 44.9%. Skin ulcers (75.4%), omphalitis (65.2%), and gingivitis (37.7%) were the most frequent complaints. Genetic analysis of the patients revealed 14 previously reported and three novel pathogenic mutations in the ITGB2 gene. The overall survival of patients with and without hematopoietic stem cell transplantation was 79.3% and 55.6%, respectively. CONCLUSION: Physicians' awareness of LAD-I considering delayed separation of umbilical cord marked neutrophilic leukocytosis, and variability in CD11 and CD18 expression levels, and genetic analysis leads to early diagnosis and defining disease severity. Moreover, the prenatal diagnosis would benefit families with a history of LAD-I.

Epidemiology, Sociodemographic Factors and Comorbidity for Allergic Rhinitis, Asthma, and Rhinosinusitis Among 15 to 65-year-Old Iranian Patients.

Background: It is well established that upper and lower airways are often clumped together when diagnosing and treating a disease. This study was designed to determine the prevalence of upper and lower airway diseases and to assess the effect of sociodemographic factors on the prevalence and the comorbidity of these disorders. Methods: This cross-sectional population-based study included patients with ages ranging between 15 to 65 years, who were referred to allergy outpatient clinics in various provinces of Iran from April to September 2020. A modified global Allergy and Asthma European Network (GA2LEN) screening questionnaire was filled out by local allergists of the 12 selected provinces in Iran. Information about the patients and sociodemographic factors was also recorded. Statistical analysis was done by univariate statistical analyses and multiple logistic regressions in SPSS software Version 26. Results: Out of 4988 recruited patients, 1078 (21.6%) had the symptoms of allergic rhinitis (AR) and 285 (5.7%) met the criteria of asthma. The prevalence of acute rhinosinusitis (ARS) and chronic rhinosinusitis (CRS) was 21.6 % and 22%, respectively. The highest prevalence of AR and ARS was in Tehran with the arateof of 33.9% each. Asthma was more prevalent in Khuzestan (14.2%) and CRS in Baluchestan (57.5%). Our analysis showed that the patients with asthma were most likely to have other allergic diseases as well-CRS (OR = 4.8; 95% CI, 2.02- 5.82), AR (OR= 2.5, 95% CI, 2.10-3), ARS (OR = 1.8; 95% CI, 2.10-3), followed by eczema (OR = 1.4; 95% CI, 1.13-1.67).We found that those individuals with CRS were most likely to have painkiller hypersensitivity (OR= 2.1; 95% CI, 1.21-3.83). Furthermore, smoking has been found more than 1.5 folds in patients with ARS. After adjusting variables, there was no correlation between education, occupation, and ethnicity with the studied diseases. Conclusion: Rhinosinusitis is a common condition among Iranian patients. This study confirmed that inflammation of the upper and lower airways can occur simultaneously. Gender, education, occupation, and ethnicity were found to be irrelevant in the development of either AR, asthma, ARS, or CRS.

Adverse reactions in a large cohort of patients with inborn errors of immunity receiving intravenous immunoglobulin.

BACKGROUND: Intravenous immunoglobulins (IVIg) are the major treatment in inborn errors of immunity (IEI) disorders; However, IVIg infusions show some adverse effects. We aimed to assess the adverse reactions of IVIg infusions. METHODS: Data of IVIg infusions in IEI patients were collected from 2011 to 2021. Totally, 363 IEI patients received IVIg regularly in Iran entered the study. The adverse reactions are classified regarding their severity and chronicity. RESULTS: 22,667 IVIg infusions were performed in the study. 157 patients (43.2%) and 1349 (5.9%) infusions were associated with at least one type of adverse reaction. The highest rates of adverse reactions were seen in severe combined immunodeficiency. Myalgia, chills, headache, fever, and hypotension were the most frequent adverse effects of IVIg. CONCLUSION: The reactions affect almost half of the patients mainly in the first infusions which necessitate the close observation of IEI patients receiving IVIg.

Molecular and immunochemical characterization of Pop n 2: A new allergen of Populus nigra pollen.

BACKGROUND: Pollen is one of the most common allergens that cause respiratory allergies worldwide. Pollen grains from poplars have been reported as important sources of pollinosis in many countries. OBJECTIVE: The aim of the present study was to determine the molecular and immunochemical characterization of Pop n 2, a novel allergen of Populus nigra (P nigra) pollen extract. METHODS: In this study, the pollen extract of P nigra was analysed by SDS-PAGE, and the allergenic profile was determined by IgE immunoblotting and specific ELISA using the sera of twenty allergic patients. The coding sequence of Pop n 2 was cloned and expressed in the Escherichia coli BL21 (DE3) using plasmid the pET-21b (+). Finally, the expressed recombinant Pop n 2 was purified by affinity chromatography. RESULTS: Pop n 2 belongs to the profilin family with a molecular weight of approximately 14 kDa. Pop n 2 is the most IgE-reactive protein (about 65%) in the P nigra pollen extract. The cDNA sequencing results indicated an open reading frame 396 bp that encodes 131 amino acid residues. The results of ELISA and Immunoblotting assays showed that recombinant Pop n 2 could react with the IgE antibody in patients' sera, like its natural counterpart. CONCLUSION: Our data revealed that Pop n 2 is a significant allergen in the P nigra pollen extract. Moreover, we observed that the recombinant Pop n 2 produced by the pET-21b (+) vector in the E colisystem acts as its natural counterpart.

Potential of trees leaf/ bark to control atmospheric metals in a gas and petrochemical zone.

Leaf and bark of trees are tools for assessing the effects of the heavy metals pollution and monitoring the environmental air quality. The aim of this study was to evaluate the presence of Ni, Pb, V, and Co metals in four tree/shrub species (Conocarpus erectus, Nerium oleander, Bougainvillea spectabilis willd, and Hibiscus rosa-sinensis) in the heavily industrial zone of Asaloyeh, Iran. Two industrial zones (sites 1 and 2), two urban areas (sites 3 and 4), and two rural areas (sites 5 and 6) in the Asaloyeh industrial zone and an uncontaminated area as a control were selected. Sampling from leaf and bark of trees was carried out in spring 2016. The metals content in the washed and unwashed leaf and bark was investigated. The results showed that four studied metals in N. oleander, C. erectus, and B. spectabilis willd in all case sites were significantly higher than that of in the control site (p < 0.05). The highest concentration of metals was found in sites 3, 4, and 6; this was due to dispersion of the pollutants from industrial environments by dominant winds. The highest comprehensive bio-concentration index (CBCI) was found in leaf (0.37) and bark (0.12) of N. oleander. The maximum metal accumulation index (MAI) in the samples was found in leaf of N. oleander (1.58) and in bark of H. rosa-sinensis (1.95). The maximum bio-concentration factor (BCF) was seen for cobalt metal in the N. oleander leaf (0.89). The nickel concentration in washed-leaf samples of C. erectus was measured to be 49.64% of unwashed one. In general, the N. oleander and C. erectus species were found to have the highest absorption rate from the atmosphere and soil than other studied species, and are very suitable tools for managing air pollution in highly industrialized areas.

Henoch-Schonlein purpura due to sodium valproate: A case report.

We report a 5-year-old girl with a generalized seizure treated with sodium valproate syrup. She returned to the clinic with Henoch-Schonlein purpura after 20 days due to the consumption of sodium valproate. To our knowledge, this is the second case of sodium valproate-induced HSP.

Composite bone substitute materials based on beta-tricalcium phosphate and magnesium-containing sol-gel derived bioactive glass.

In the present study, bioceramic composites with improved mechanical and biological properties were synthesized by sintering mixtures of beta-tricalcium phosphate and SiO(2)-CaO-MgO-P(2)O(5) sol-gel derived bioactive glass at 1000-1200 degrees C. The physical, mechanical, structural and biological properties of the composites were evaluated by appropriate experiments such as microhardness, bending strength, XRD, SEM and MTT. The results showed that 1000 and 1100 degrees C were not appropriate temperatures for sintering the composites and in contrast, the microhardness, bending strength and bulk density significantly increased by increasing in quantity of bioglass phase when the samples were sintered at 1200 degrees C. No significant difference was found between the fracture toughness of the composites and pure beta-tricalcium phosphate. beta-tricalcium phosphate was structurally stable up to 1200 degrees C and did not transform to its alpha form even in the presence of the bioglass phase but migration of magnesium cations from the glass composition into its lattice structure was found by right-shift in XRD patterns, especially when the composite contained higher amount of bioglass component. Calcium silicate was also crystallized in the composition of the composites, which was more detectable in higher sintering temperatures. The results of the MTT test showed that proliferation of human osteosarcoma cells on the composites was considerably better than that of pure beta-TCP.

Sudden unilateral blindness in a girl with Kawasaki disease.

This article describes a 9-year-old girl with clinical and laboratory features of Kawasaki disease. On day 7 of her febrile illness, she developed sudden loss of vision in the right eye. Visual acuity was no light perception. Dilated funduscopy showed diffuse intense retinal whitening, narrowing retinal arterioles, and a pale swollen disk-but no cherry-red spot. These findings suggested ophthalmic artery obstruction. Kawasaki disease can be sight-threatening.

The Results of Autologous Skin Test in Patients with Chronic Urticaria in Hamadan, Iran.

INTRODUCTION: The etiology of chronic urticaria is unknown in many cases. In this study, we demonstrated the presence of autoimmune antibodies in patients with chronic urticaria by using of the Autologous Serum Skin Test (ASST). METHODS: We performed a cross-sectional study to detect the presence of autologous antibodies in the serum of 38 patients (25 females and 13 males) with idiopathic chronic urticaria who were referred to the Hamedan Allergy Clinic in 2014. All of the necessary tests for demonstrating chronic urticaria were performed, including complete blood count (CBC), thyroid and liver functionality tests, and the prick test but they did not confirm the cause of chronic urticaria. We conducted the Autologous Serum Skin Test on the patients and analyzed the results. RESULTS: In 15 patients (39%), the ASST was positive. Of the 15 patients with positive autoimmune chronic urticaria, five patients (33%) were males, and 10 patients (67%) were females. CONCLUSION: We concluded that many patients with chronic urticaria have autoimmune urticaria. It is the reason for the lack of the response to treatment with common medications for urticaria. New ways of treatment must be considered for them.

Demographic and Clinical Findings in Children Undergoing Bronchoscopy for Foreign Body Aspiration.

BACKGROUND: Foreign body aspiration (FBA) is a significant cause of airway distress, mortality, and morbidity in children. Diagnosis of FBA can be challenging and is sometimes delayed for weeks or even months. If not diagnosed and treated promptly, FBA can result in serious consequences. METHODS: For this retrospective study, we investigated the medical records of 89 children who underwent bronchoscopy for suspected FBA and recorded relevant demographic, clinical, and treatment data. RESULTS: Of the 89 patients identified for this study, 51 had a definitive diagnosis of FBA. Among these patients, choking, chronic cough and wheezing, cyanosis, and dyspnea were the most frequent symptoms of FBA. The foreign bodies were located in the left bronchus (45.1%), the right bronchus (35.3%), the trachea (15.7%), and in both the right and left bronchi (3.9%). Seeds were the most prevalent foreign body, found in 39.2% of the patients. CONCLUSION: Lack of complete medical history in patients with suspected FBA is one of the main causes of delayed FBA diagnosis. Bronchoscopy is considered the definitive diagnostic method for FBA and should be conducted in all patients with suspected FBA because of the low risk of complications and reduced probability that FBA diagnosis and treatment will be delayed.

Initial intravenous gamma-globulin treatment failure in Iranian children with Kawasaki disease.

The purpose of this study was to determine the initial rates of intravenous gamma-globulin treatment (IVIG) failure in Kawasaki disease (KD) and their predisposing factors. This study was a retrospective analysis of the initial response to IVIG (2 g/kg), assessed from the medical reports of all patients admitted to Namazee Hospital pediatric ward, from March 1998 to March 2002, and who fulfilled the criteria for KD. Data were available for 64 patients, 58 of whom (90.6%) became afebrile 48 hours after completion of the initial dose of IVIG (Group I) and six (9.4%) who remained febrile (Group II). Two patients had a prompt response to a second dose of IVIG. In Group I, five patients (8.6%) developed coronary artery disease, seen on echocardiography. In Group II, two patients (33.3%) developed coronary artery disease. No significant difference was found in the prevalence of coronary artery disease between the two groups (p = 0.12), or in age or gender. The rate of initial treatment failure was 9.4% in this cohort of patients, which is comparable with previous reports. No predictive factors such as coronary artery disease, age or gender were found for initial treatment failure in KD.

The Incidence of Allergic Disorders in First Degree Relatives of Neonates with Transient Tachypnea of Neonate.

BACKGROUND: The role of maternal allergic disorders to increase the risk of Transient Tachypnea of Neonate (TTN) in neonates remained unclear. We determined the incidence of allergic disorders in first degree relatives of neonates suffered from TTN to clear role of these allergic disorders to predispose TTN in neonates. MATERIALS AND METHODS: In a cross-sectional study carried out at Fatemieh hospital between September 2010 and September 2011, all consecutive neonates with the diagnosis of TTN were included into the study. Those neonates were not treated after 5 days of hospitalization were excluded. Baseline information with regard to the history of allergic diseases among first degree relatives of neonates were charted from family members using a structured questionnaire at enrolment by interviewing and examination if required. RESULTS: The two groups were matched for baseline data including neonate gender, birth weight, and type of delivery. In the TTN group, one of first degree relatives (2.9%) suffered from bronchial asthma and two of them (5.7) had atopic dermatitis. In total, allergic diseases was revealed in 8.6% of first degree relatives of neonates with TTN. Besides, none of the first degree relatives of neonates in healthy neonates group experienced bronchial asthma or atopic dermatitis. Allergic rhinitis was not also found in the relatives of the two study neonates groups. Comparing incidence of allergic diseases in first degree relatives of neonates in TTN and healthy groups showed no significant difference (8.6% in TTN group versus 0.0% in healthy group, p = 0.076). CONCLUSION: Our study showed that the incidence of allergic disorders in first degree relatives of neonates suffered from TTN is higher than healthy newborns, but these differences are not statistically significant.

Development of beta-tricalcium phosphate/sol-gel derived bioactive glass composites: physical, mechanical, and in vitro biological evaluations.

In this study, composites of beta-tricalcium phosphate (beta-TCP) and sol gel derived bioactive glass (10, 25, and 40 wt %) based on the SiO(2)-CaO-MgO-P(2)O(5) system were prepared and sintered at 1000-1200 degrees C. The mechanical properties were investigated by measuring bending strength, Vickers hardness and fracture toughness. Structural properties were evaluated by XRD and SEM analysis, and the biological properties were studied by soaking the samples in simulated body fluid (SBF) and in contact with osteoblastic cell for viability assay. When the samples were sintered at 1200 degrees C, the mechanical strength increased, up to 34%, by increasing the amount of bioactive glass phase. In contrast, it decreased when the samples were sintered at 1000 and 1100 degrees C. The results showed that the strength could be improved up to 56% when more firing period was used. Incorporation of the bioactive glass phase into beta-TCP increased the microhardness but did not significantly change the fracture toughness. Phase analysis revealed that beta-TCP or magnesium-substituted beta-TCP was the main crystalline phase of the composites beside some calcium silicate crystallized in the bioactive glass phase. Plenty precipitation of calcium phosphate layer onto the surfaces of the beta-TCP/bioactive glass composites soaked in SBF indicated superior bioactivity of these materials compared to pure beta-TCP without any precipitation. The ability of beta-TCP/bioactive glass composites to support the growth of human osteoblastic cells was considerably better than that of pure beta-TCP. These results may be used to indicate which compositions and processing conditions can provide appropriate materials for hard tissue regeneration.

Onychomadesis in a patient with immunoglobulin class switch recombination deficiency.

Immunoglobulin class switch recombination deficiencies (Ig CSR deficiencies) or Hyper IgM syndromes (HIGM) are a group of primary immunodeficiency diseases, characterized by defective CD40 signaling of B cells, resulting in reduced CSR and somatic hypermutation. The affected patients are characterized by low serum levels of IgG and IgA, and normal or elevated levels of IgM, which lead to an increased susceptibility to infections. We describe a 3 year-old boy with frequent bacterial infections of the skin and respiratory tract, mucosal ulcers, and diarrhea. He experienced onychomadesis of both fingernails and toenails during a recent bacterial infection. Quantitative immunoglobulin measurements revealed high levels of serum IgM and very low levels of IgG, IgA, and IgE. Clinical and immunologic studies supported the diagnosis of HIGM. Exclusion of CD40L, CD40, AID and UNG genes by molecular analysis in this patient may suggest a new form of selective CSR deficiency.

Hair loss as a sign of Kawasaki disease.

Kawasaki disease is a multi system disorder with varying clinical expressions. This disease is an acute systemic vasculitis of unknown etiology that has recently recognized as a leading cause of acquired heart disease in children of many developed countries. We describe an unusual instance of hair loss in a patient with Kawasaki disease. A 26 months old boy developed prolonged high fever, bilateral conjunctival infection, arthralgia and erythromatosis skin rash. He was admitted to the hospital with the diagnosis of Kawasaki disease. Laboratory results included an erythrocyte sedimentation rate (ESR) above 100 and platelet count > 1000,000. The patient developed acute and unprovoked scalp hair loss. He was treated with intravenous immunoglobulin (IVIG) 2 g/kg and aspirin 100 mg/kg/day with complete improvement of signs and symptoms. This report documents hair loss as an uncommon presentation of Kawasaki disease.

Synergistic effect of intravenous immunoglobulins and iodinated contrast media on renal function.

Acute renal failure is one of the side effects while using intravenous immunoglobulins. This complication is also observed with iodinated contrast media. Herein, we describe a patient with acute renal failure who received intravenous immunoglobulins and iodinated contrast media concomitantly. Both drugs are responsible for osmotic nephrosis. The same effect on renal cells may explain a synergistic effect on renal function.