Bilateral basal ganglia involvement in a patient with Griscelli syndrome.

We report a 6-year-old Iranian boy with silvery-gray hair, eyelashes and the eyebrows who was admitted because of seizures and subsequent stupor. He had previous history of acute hemiparesis at 1 year of age and hepatitis-like syndrome 3 months ago. Microscopic examination of the patient's hair shaft revealed different sized clumps of melanin seen in the center of the shafts. Bone marrow aspiration revealed erythroid hyperplasia and erythrophagocytic cells. Bilateral frontal cortical and subcortical high signal lesions, dirty white matter, high signal areas in the upper pons and in both caudates and lentiform nuclei in T2 WI were the brain MRI findings of the patient. He died in the accelerated phase of Griscelli Syndrome (GS) type 2. To our knowledge we report the first case of GS from Iran.     

Preparation,characterization, and cytotoxic effects of liposomal nanoparticles containing cisplatin: an in vitro study

Cisplatin is a chemotherapeutic agent used for treating various malignancies. The study aimed to prepare pegylated liposomal cisplatin and evaluate its efficacy against human breast cancer cell line MCF‐7. Drug‐loaded nanoparticles were synthesized by reverse phase evaporation technique. The study is highlighted by extensive characterization of nanoparticles in terms of nanoparticle morphology, type of drug entrapment, cisplatin retention capability, and cytotoxicity effects. The size, size distribution, and zeta potential of nanodrug were estimated 142 nm, 0.33, and ?22 mV, respectively. Drug‐loading efficiency was equal to 48% that occurred physically. Furthermore, high retention capability (39% of drug was released after 72 h) with significantly enhanced cytotoxicity of nanodrug (1.75 times more than the standard drug) confirmed the potency of liposomal nanoparticles as proper cisplatin carrier.     

A comparison between on-demand usage of rFVIIa vs prophylaxis use of emicizumab in high titer inhibitory hemophilia A patients in Iran: A cost–utility analysis

Background:Hemophilia A (HA) is an inherited X-linked bleeding disease with costly treatment, especially for high titer inhibitory patients. Emicizumab, a new humanized bispecific antibody, has been approved for use to prevent or reduce the frequency of bleeding episodes in HA patients with inhibitors. This study evaluated the cost-utility of emicizumab prophylaxis (EP) in comparison with recombinant factor VII activated on-demand treatment in HA patients with inhibitors.Methods:A life-time Markov model with payer and societal perspectives was developed in different age groups with different annual bleeding rates (ABR). Efficacy of treatments were extracted from HAVEN trials. Utilities were retrieved from published evidence. Costs were calculated based on Iran food and drug administration official website, national tariff book for medical services and hospital data. One-way deterministic sensitivity analysis was performed.Results:EP was dominant choice in comparison with on-demand administration of recombinant factor VII activated in all age groups with ABR 20 and 25, and it remained dominant in patients with age 2 and age 12 at start point with ABR 16 and 17. The reported incremental cost-effectiveness ratio for the group with ABR 18 at the age 20, was 12,936 United States Dollars which is lower than the acceptable threshold of cost-effectiveness in Iran (1–3 gross domestic product per capita) and EP can be considered as cost-effective choice in this scenario.Conclusion:EP was found to be a dominant and cost-effective choice for Iranian HA patients with factor VIII inhibitors with ABR 18 and above with considerable cost saving.     

Wavelength and pulse energy optimization for detecting hypoxia in photoacoustic imaging of the neonatal brain: a simulation study

Cerebral hypoxia is a severe injury caused by oxygen deprivation to the brain. Hypoxia in the neonatal period increases the risk for the development of neurological disorders, including hypoxic-ischemic encephalopathy, cerebral palsy, periventricular leukomalacia, and hydrocephalus. It is crucial to recognize hypoxia as soon as possible because early intervention improves outcomes. Photoacoustic imaging, using at least two wavelengths, through a spectroscopic analysis, can measure brain oxygen saturation. Due to the spectral coloring effect arising from the dependency of optical properties of biological tissues to the wavelength of light, choosing the right wavelength-pair for efficient and most accurate oxygen saturation measurement and consequently quantifying hypoxia at a specific depth is critical. Using a realistic neonate head model and Monte Carlo simulations, we found practical wavelength-pairs that quantified regions with hypoxia most accurately at different depths down to 22 mm into the cortex neighboring the lateral ventricle. We also demonstrated, for the first time, that the accuracy of the sO₂ measurement can be increased by adjusting the level of light energy for each wavelength-pair. Considering the growing interest in photoacoustic imaging of the brain, this work will assist in a more accurate use of photoacoustic spectroscopy and help in the clinical translation of this promising imaging modality. Please note that explaining the effect of acoustic aberration of the skull is not in the scope of this study.     

D-WASPAS: Addressing Social Cognition in Uncertain Decision-Making with an Application to a Sustainable Project Portfolio Problem

Decision-making is an interdisciplinary area that has roots in mathematics, economics, and social science. Multiple-criteria group decision-making (MCGDM) is one of the most applicable areas of decision-making. Social cognition is involved in group decision-making. Therefore, it is necessary to address how decision makers (DMs) process and apply judgments and information during the process. In recent years, many approaches have been applied to MCGDM. As an important aspect of this process, uncertainty has led to the application of fuzzy sets. However, utilizing various decision-making approaches can result in different results and confusion among DMs. Moreover, using classic fuzzy sets and expressing degrees of belonging by crisp values has proven to be inadequate for uncertain decision-making environments. This paper presents a novel MCGDM approach, double-weighted aggregated sum product assessment (D-WASPAS), under interval-valued Pythagorean fuzzy (IVPF) uncertainty. The proposed approach applies knowledge measures to address the objective weights of criteria. Then, subjective and objective weights of criteria are aggregated to create a more appropriate weight. This approach considers three decision-making methods. In the first, an IVPF-ARAS (additive ratio assessment) method is extended to rank the alternatives. In the second, an IVPF-EDAS (evaluation based on distance from average solution) method is developed to rank the alternatives. In the third, a novel IVPF-COADAP (complex adequate appraisal) method is utilized for a third ranking. To aggregate the results, two steps are carried out using the WASPAS method. First, the results of the ranking approaches are aggregated. This process starts with computing the objective weights of the ranking approaches and aggregating the outcome with the subjective weights of the approaches. Then, the WASPAS method is applied to aggregate the obtained rankings and obtain a set of rankings for each DM. The second aggregation is utilized to aggregate the results for the DMs and reach a final set of rankings. Similarly, the subjective and objective weights of the DMs are applied in the WASPAS to aggregate the results. It should be noted that since the WASPAS method is utilized twice to aggregate the results, this approach is called D-WASPAS. A case study of the application of the proposed method shows that it is applicable to many multiple-criteria analysis and decision-making processes. Moreover, the results are more reliable because various decision-making methods are taken into consideration, and it is a last-aggregation process. Double-weighted aggregated sum product assessment offers a novel decision-making framework that is applicable in real-world decision-making situations. The proposed method is based on interval-valued Pythagorean fuzzy sets (IVPFSs), which would be especially applicable to uncertain situations. Also, it would enhance calculations of the process by offering more flexibility in dealing with uncertainty. Consequently, introducing this new decision-making framework and applying extended fuzzy sets would make the proposed method more widely applicable. The last-aggregation nature of this method avoids loss of cognitive information and assigning weights to the DMs, and the different ranking methods address the social cognition that leads to the judgments expressed and the final decisions.

     

Association of Serum Vitamin D Concentration With Clinical Symptoms and Quality of Life in Patients With Irritable Bowel Syndrome

Objective: A high prevalence of vitamin D deficiency (VDD) in gastrointestinal (GI) disorders and the role of vitamin D in the function of the gut have been shown previously. Therefore, we aimed to evaluated the VDD and the possible association of the GI symptoms severity and quality of life (QoL) score with the serum levels of vitamin D in irritable bowel syndrome (IBS).

Methods: A total of 90 patients with IBS based on Rome III criteria enrolled in the study from the tertiary referral university hospital. In addition, 90 sex- and age-matched healthy controls (HCs) were recruited. To measure the serum levels of 25(OH)D₃, blood samples were taken from all the participants. Severity of clinical symptoms, IBS quality of life (IBS-QoL), and IBS symptom severity score (IBSSS) were assessed.

Results: In 66.7% of IBS patients, serum 25(OH)D₃ concentrations were <20?ng/mL. The mean serum 25(OH)D₃ of IBS patients was statistically (p?<?0.05) lower vs. HCs. When different subtypes were analyzed, the serum 25(OH)D₃ concentrations in diarrhea-predominant IBS were statistically (p?<?0.05) lower as compared to HCs. Furthermore, the lower serum concentrations of 25(OH)D₃ were associated (p?<?0.05) with higher severity of abdominal pain and distention, flatulence, overall GI symptoms, and IBSSS. However, a direct significant association was seen between IBS-QoL and serum 25(OH)D₃.

Conclusion: Results of this study showed a high prevalence of VDD in patients with IBS. In addition, VDD was associated with a higher severity of clinical symptoms and lower QoL in IBS.     

Enhancement of the Therapeutic Effect of Albendazole on Cystic Echinococcosis using a Herbal Product

Purpose: Albendazole is the drug of choice for chemotherapy of cystic echinococcosis (CE) but 40% of patients do not respond to it satisfactorily. With regard to the previously reported scolicidal activity of Zataria multiflora, this study was done to evaluate the efficacy of the co-administration of albendazole and Z. multiflora aromatic water (AW) on hydatid cysts in a murine model. Materials and Methods: Laboratory mice were infected with 1,500 viable protoscoleces. After development of hydatid cysts, the infected mice were allocated into four treatment and one control group. The mice of groups 1, 2, 3, and 4 were treated orally with albendazole (100 mg/kg), Z. multiform AW (100 ml/l), albendazole (100 mg/kg) – Z. multiform AW and albendazole ((50 mg/kg) – Z. multiform AW respectively. Two months later, all the mice were euthanized and necropsied and the number, size and weight of hydatid cysts were compared between different groups. Results: The size of the largest cysts, as well as the total weight of cysts, were significantly lower (p < .05) in the mice treated with albendazole (50 mg/kg) – Z. multiflora AW in comparison to those treated with albendazole (100 mg/kg) alone. Conclusions: The results of this study revealed the superiority of co-administration of albendazole and Z. multiflora AW to albendazole alone in the treatment of CE in laboratory mice. Since Z. multiflora AW is a safe and useful drink, it may be used successfully to enhance the therapeutic effect of albendazole in the patients infected with hydatid disease.     

Genetic diversity of an avian nasal schistosome causing cercarial dermatitis in the Black Sea-Mediterranean migratory route

Parasitology Research - This study is part of an effort to document the diversity of avian schistosomes in ducks and snails in Northern Iran, a major flyway (Black Sea/Mediterranean) for migratory...     

A probable causative factor for an old problem: selenium and glutathione peroxidase appear to play important roles in epilepsy pathogenesis

PURPOSE: Only recently has it become known that oxidative stress and generation of reactive oxygen species are the cause and the consequence of epileptic seizures. Due to the protective role of selenium (Se) and selenoproteins against oxidative damage and the ability to promote neuronal cell survival, we compared serum selenium level and red blood cell Glutathione peroxidase activity (RBC GPx) between epileptic and healthy children. METHODS: In a case control study, 53 epileptic children were compared with 57 healthy children in the same age and community of residence. Serum Se and RBC GPx activity were measured with an atomic absorption spectrophotometry and Cayman standard glutathione assay kit, respectively. RESULTS: The mean (+/-standard deviation) of serum Se was 72.90 microg/L (+/-22.20) and 86.00 microg/L (+/-15.00) in patient and control groups, respectively. For RBC GPx activity the mean (+/-standard deviation) was 440.57 nmol/min/ml (+/-264.00) and 801.00 nmol/min/ml (+/-267.00) in patient and control groups, respectively. Statistical analysis showed a significant lower means of serum Se and RBC GPx activity in patient group compared to that of healthy control group (p < 0.001). CONCLUSION: Lower serum Se and RBC GPx activity in epileptic patients compared to healthy children may support the proposed crucial role of Se and GPx activity in the pathogenesis of epilepsy. However, RBC GPx activity in the case of selenium deficiency could not be a sensitive and specific indicator of Se status in serum that led us to supplant Se measurement with RBC GPx activity.     

Imported Lymphatic Filariasis in an Indian Immigrant to Iran

Lymphatic filariasis (LF), a nematode disease transmitted by arthropod vectors, is repeatedly reported in immigrant population. This disease is not endemic in Iran; however, different species of mosquitoes, capable of transmission of parasite microfilaria, are distributed in the country. Hereby, incidental detection of an imported case of LF due to Wuchereria bancrofti in an Indian worker in Iran is reported. Identification of the case was performed based on morphological and morphometrical characteristics of microfilaria and PCR sequencing.