Evaluation of the frequency and diagnostic delay of primary immunodeficiency disorders among suspected patients based on the 10 warning sign criteria: A cross-sectional study in Iran

BACKGROUND: Common Variable Immunodeficiency (CVID) is characterized by an impaired antibody production and a higher susceptibility to encapsulated bacterial infections. Lung disease is considered to be the most important cause of morbidity and mortality. METHODS: We analyzed clinical, radiological and functional characteristics in 80 patients with CVID assisted in the Unidad Inmunologia e Histocompatibilidad at Durand Hospital from 1982 to 2018. RESULTS: Of the 80 patients, 55 showed pathologic lung Computed Tomography (CT). Twenty of them (36.4%) showed bronchiectasis; 26 (47.3%) interstitial involvement associated with nodules and adenopathies called GLILD (granulomatous-lymphocytic interstitial lung disease); and nine patients (16.3%) showed other lesions. Nine percent of patients with lung disease showed CT progression; none of them had spirometry worsening. GLILD patients had normal and restrictive patterns in lung function tests, in equal proportions. Two patients - one with GLILD and the other one with bronchiectasis - had an increase in spirometric pattern severity without CT progression. Lung biopsy was performed in 19% of GLILD patients, all of whom had histopathologic diagnosis of Lymphoid Interstitial Pneumonia (LIP). CONCLUSIONS: GLILD is the major cause of lung disease in CVID. Computed tomography is useful for diagnosis but not necessary in follow-up, in which functional tests should have better correlation with clinical evolution, reducing radiation exposure. Biopsy should be indicated when the clinical diagnosis is unclear. Treatment should be considered whenever there is clear evidence of disease progression

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Evaluation of the frequency and diagnostic delay of primary immunodeficiency disorders among suspected patients based on the 10 warning sign criteria: A cross-sectional study in Iran.

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.

The Fauna and Active Season of Mosquitoes in West of Fars Province, Southwest of Iran.

Culicidae are highly important for public health as they can be vectors of diseases and are responsible for a wide spectrum of infections. Five collection sites were selected randomly with regards to existing facilities in Firouzabad County. For collecting larvae and total catch for adult mosquitoes, sampling was carried out by dipping technique for collecting larvae and total catch for adult mosquitoes. A total of 689 adults and 1313 larvae of Culicidae were collected, of which 3 genera and 6 species of Culicidae were recognized, namely, Anopheles superpictus, Anopheles d’thali, Culex sinaiticus, Culex theileri, Culex mimeticus, and Culiseta longiareolata. Cx. theileri was the most frequent Culicidae collected at Firouzabad, with a total of 613 and 247 larval and adult specimens, respectively. The highest number of mosquitoes was collected in June (31.1%) and the lowest in May (3.4%). The mean temperatures in June and May were 31.3˚C and 28.2˚C, respectively. We found some vectors that are of medical and veterinary importance; our results could be applied in vector control programs that aim at eradication or control of mosquitoes in this area.

Effect of high energy electron beam (10MeV) on specific heat capacity of low-density polyethylene/hydroxyapatite nano-composite.

In the present work, thermal properties of low density polyethylene (LDPE) and its nano composites are investigated. For this purpose LDPE reinforced with different weight percents of hydroxyapatite (HAP) powder which was synthesized via hydrolysis method are produced. The samples were irradiated with 10MeV electron beam at doses of 75 to 250kGy. Specific heat capacity measurement have been carried out at different temperatures, i.e. 25, 50, 75 and 100°C using modulated temperature differential scanning calorimetry (MTDSC) apparatus and the effect of three parameters include of temperature, irradiation dose and the amount of HAP nano particles as additives on the specific heat capacity of PE/HAP have been investigated precisely. The MTDSC results indicate that the specific heat capacity have decreased by addition of nano sized HAP as reinforcement for LDPE. On the other hand, the effect of radiation dose is reduction in the specific heat capacity in all materials including LDPE and its nano composites. The HAP nano particles along with cross-link junctions due to radiation restrain the movement of the polymer chains in the vicinity of each particle and improve the immobility of polymer chains and consequently lead to reduction in specific heat capacity. Also, the obtained results confirm that the radiation effect on the specific heat capacity is more efficient than the reinforcing effect of nano-sized hydroxyapatite.

BACKGROUND TRACK DENSITY REDUCTION OF 50-HZ-HV ECE-PROCESSED THICK POLYCARBONATE DETECTORS TO IMPROVE LOWER DETECTION LIMIT.

A recent novel development of 50-Hz-HV ECE of 1-mm-thick and 250-µm-thick polycarbonate track detectors (PCTDs) has proved some promising results for some health physics, dosimetry and ion-beam-related applications. The method while proved having some good characteristics for some applications provided a relatively higher background track density (BGTD) in particular when very high voltages are applied to the PCTDs. In order to decrease the minimum detection limit (MDL) of the PCTDs and to further promote its applications for low dose, the BGTD was reduced by applying a layer removal methodology applying ethylendiamine (EDA). The effects of EDA concentrations (50, 60, 65, 70, 75, 80, 85 and 90 %) in water at room temperature (26°C) and soaking durations up to 100 min at different EDA concentration on BGTD reduction were studied. The thickness of the layer removed from the surface of a PCTD highly depends on the soaking time and EDA concentration; it increases as the EDA concentration increases up to for example 700 µm after 2 h of soaking in the EDA solution. After ∼10 min of soaking duration at any of the above-stated concentrations, the BGTD reaches its minimum value, a value which differs from concentration to concentration. An EDA concentration of 85 % in water provided the lowest BGTD of 64.06 ± 3.12 tracks cm- 2; ∼6 times lower than that of its original value. It is shown that the layer removal process does not change the registration characteristics of the PCTD and its appearance significantly. The MDL of the PCTDs depends strongly on the BGTD. The MDL values for a desired confidence level were also studied by three calculation methods. The results of the BGTD and the MDL studies under different conditions applied are presented and discussed.

Investigation of a Case of Genotype 5a Hepatitis C Virus Transmission in a French Hemodialysis Unit Using Epidemiologic Data and Deep Sequencing.

BACKGROUND Hepatitis C virus (HCV) is a major cause of chronic liver disease worldwide. A patient was recently found to be HCV seropositive during hemodialysis follow-up. OBJECTIVE To determine whether nosocomial transmission had occurred and which viral populations were transmitted. DESIGN HCV transmission case. SETTING A dialysis unit in a French hospital. METHODS Molecular and epidemiologic investigations were conducted to determine whether 2 cases were related. Risk analysis and auditing procedures were performed to determine the transmission pathway(s). RESULTS Sequence analyses of the NS5b region revealed a 5a genotype in the newly infected patient. Epidemiologic investigations suggested that a highly viremic genotype 5a HCV-infected patient who underwent dialysis in the same unit was the source of the infection. Phylogenetic analysis of NS5b and hypervariable region-1 sequences revealed a genetically related virus (>99.9% nucleotide identity). Deep sequencing of hypervariable region-1 indicated that HCV quasispecies were found in the source whereas a single hypervariable region-1 HCV variant was found in the newly infected patient, and that this was identical to the major variant identified in the source patient. Risk analysis and auditing procedures were performed to determine the transmission pathway(s). Nosocomial patient-to-patient transmission via healthcare workers' hands was the most likely explanation. In our dialysis unit, this unique incident led to the adjustment of infection control policy. CONCLUSIONS The data support transmission of a unique variant from a source with a high viral load and genetic diversity. This investigation also underlines the need to periodically evaluate prevention and control practices.

Does consumption of polyunsaturated fatty acids influence on neurorehabilitation in traumatic spinal cord-injured individuals? A double-blinded clinical trial.

STUDY DESIGN: A double-blinded randomized clinical trial. OBJECTIVES: The anti-inflammatory and neuroprotective effect of omega-3 fatty acids have been shown so far, but still its influence on clinical measures in spinal cord-injured human models were not known. We tried to investigate changes in disability and dependency scores in chronic traumatic spinal cord-injured patients after 14 months of ω-3 fatty-acid consumption. METHODS: Main inclusion criteria were: traumatic spinal cord injury (SCI) and post injury duration longer than 1 year. Disability and dependency was assessed using U.K Functional Independence Measure and Functional Assessment Measure (FIM+FAM) scale. MorDHA capsules (435 mg of docosahexaenoic acid and 65 mg of eicosapentaenoic acid) were administered in treatment group, whereas control group received placebo capsules for 14 months. U.K. FIM+FAM scale were estimated before intervention and at the end of the trial. RESULTS: Fifty-four patients in treatment group and 50 patients in placebo group completed the trial. Highest scores were detected in cognitive domain in both groups before and after intervention. Most dependency was observed in locomotion subscale and secondly in sphincter control. Scores of none of these components were changed by ω-3 fatty-acid consumption. CONCLUSION: Although omega-3 fatty acids have been shown to have neuroprotective effect in acute phase of SCI, it seems that they have no significant influence in chronic inflammatory state of SCI. The positive effect of ω-3 fatty acid in chronic neurorecovery process, if exists, is weaker to exert any significant improvement in UK FIM+FAM scores in spinal cord-injured individuals.

Management of anticancer treatment in patients under chronic dialysis: results of the multicentric CANDY (CANcer and DialYsis) study.

BACKGROUND: One million people worldwide benefit from chronic dialysis, with an increased rate in Western countries of 5% yearly. Owing to increased incidence of cancer in dialyzed patients, the management of these patients is challenging for oncologists/nephrologists. PATIENTS AND METHODS: The CANcer and DialYsis (CANDY) retrospective multicenter study included patients under chronic dialysis who subsequently had a cancer (T0). Patients were followed up for 2 years after T0. Prescriptions of anticancer drugs were studied with regard to their renal dosage adjustment/dialysability. RESULTS: A total of 178 patients from 12 institutions were included. The mean time between initiation of dialysis and T0 was 30.8 months. Fifty patients had received anticancer drug treatment. Among them, 72% and 82% received at least one drug needing dosage and one drug to be administered after dialysis sessions, respectively. Chemotherapy was omitted or prematurely stopped in many cases where systemic treatment was indicated or was often not adequately prescribed. CONCLUSIONS: Survival in dialysis patients with incident cancer was poor. It is crucial to consider anticancer drug treatment in these patients as for non-dialysis patients and to use current available specific drug management recommendations in order to (i) adjust the dose and (ii) avoid premature elimination of the drug during dialysis sessions.

Molecular analysis of human influenza virus in Tehran, Iran.

BACKGROUND: To date there are no reports of molecular and phylogenetic analyses of human influenza virus in Tehran, Iran. OBJECTIVES: We isolated and characterized circulating influenza viruses in a sample of patients in Tehran. METHODS: Nasal and pharyngeal swabs were collected from 57 individuals who were suspected of having influenza between October 2005 and January 2007. These samples were cultured and subsequently genotyped by RT-PCR and sequencing analyses. RESULTS: Twelve of 57 samples (21%) were positive for human influenza virus. Out of the 12 positive samples, 7 were A/H3N2 (58%), 3 were A/H1N1 (25%) and 2 were B subtypes (17%). The phylogenetic analysis of the hemagglutinin gene showed that the H1N1 isolates were close to the A/New Caledonia/20/99 and the H3N2 isolates were close to the A/Panama/2007/99 and A/Moscow/10/99 vaccine strains. CONCLUSION: In a sample of clinical patients in Tehran, Iran, the predominant subtype of human influenza virus was determined to be A/H3N2, followed by A/H1N1 and B. In addition, phylogenetic analysis on H1 showed some genetic drifts from vaccine strains, but the phylogeny of H3 demonstrated that these isolates were from the previous vaccine strains.

Hemodialysis reduces plasma apolipoprotein C-I concentration making VLDL a better substrate for lipoprotein lipase.

Apolipoprotein Cs (apoC-1, apoC-II, and apoC-III) are lipoprotein components that have regulatory effects on enzymes involved in lipoprotein metabolism. Owing to their low molecular weights, apoCs can adsorb onto and/or pass through dialysis membranes. Our study determines the consequence of hemodialysis (HD) on plasma concentrations of apoCs and on the activities of enzymes modulated by apoCs. Plasma samples were collected from 28 patients with chronic renal failure before and after HD. Plasma apoC-II levels were unchanged, whereas apoC-III levels were slightly decreased in post-dialysis plasmas. The apoC-I content was markedly reduced during HD. This was due to a significant decrease in the apoC-I content of very low-density lipoprotein (VLDL), whereas the apoC-I content of high-density lipoprotein (HDL) was unchanged. Although HDL bound apoC-I is thought to inhibit cholesterol ester transfer protein, no change in the ability of pre- and post-dialysis VLDL to interact with the transfer protein were observed. Complementary experiments confirmed that VLDL-bound apoC-I has no transfer protein inhibitory potential. In contrast, an increase in the ability of post-dialysis apoC-I-poor VLDL to act as substrate for lipoprotein lipase (LPL) was found compared to pre-dialysis VLDL. Our study shows that apoC-I losses during HD might be beneficial by improving the ability of VLDL to be a substrate for LPL thus improving plasma triglyceride metabolism.