Shiraz University of Medical SciencesIranian Journal of Medical Sciences0253-071643220180301Delayed Complications and Long-term Management of Sulfur Mustard Poisoning: Recent Advances by Iranian Researchers (Part І of ІІ)1031244051610.30476/ijms.2018.40516ENEmadodinDarchini-MaraghehMedical Toxicology Research Centre, Faculty of Medicine, Mashhad University of Medical Sciences, Mashhad, IranMahdiBalali-MoodMedical Toxicology Research Centre, Faculty of Medicine, Mashhad University of Medical Sciences, Mashhad, IranJournal Article20151215Chemical warfare agents are the most brutal weapons among the weapons of mass destruction. Sulfur mustard (SM) is a potent toxic alkylating agent known as “the King of the Battle Gases”. SM has been the most widely used chemical weapon during the wars. It was widely used in World War I. Thereafter, it was extensively employed by the Iraqi troops against the Iranian military personnel and even civilians in the border cities of Iran and Iraq in the period between 1983 and 1988. Long-term incapacitating properties, significant environmental persistence, lack of an effective antidote, and relative ease of manufacturing have kept SM a potential agent for both terrorist and military uses. Even 3 decades after SM exposure, numerous delayed complications among Iranian victims are still being reported by researchers. The most common delayed complications have been observed in the respiratory tracts of chemically injured Iranian war veterans. Also, skin lesions and eye disorders have been observed in most Iranian SM-exposed war veterans in the delayed phase of SM intoxication. Thus, extensive research has been conducted on Iranian war veterans during the past decades. Nevertheless, major gaps still continue to exist in the SM literature. Part І of this paper will discuss the delayed complications and manifestations of exposure to SM among Iranian victims of the Iran–Iraq conflict. Part ІІ, which will appear in the next issue of Iran J Med Sci, will discuss the long-term management and therapy of SM-exposed patients.https://ijms.sums.ac.ir/article_40516_c1bba264289e47fdb53f0132eb445e61.pdfShiraz University of Medical SciencesIranian Journal of Medical Sciences0253-071643220180301High Prevalence of Vitamin D Deficiency among Iranian Population: A Systematic Review and Meta-Analysis1251394051910.30476/ijms.2018.40519ENRezaTabriziHealth Policy Research Center, Institute of Health, Student Research Committee, Shiraz University of Medical Sciences, Shiraz, Iran;MahmoodMoosazadehHealth Science Research Center, Addiction Institute, Mazandaran University of Medical Sciences, Sari, Iran0000-0002-5452-514MaryamAkbariHealth Policy Research Center, Institute of Health, Student Research Committee, Shiraz University of Medical Sciences, Shiraz, Iran;0000-0002-9211-6371Mohammad HosseinDabbaghmaneshEndocrine and Metabolism Research Center, Nemazee Hospital, Shiraz University of Medical Sciences, Shiraz, IranMinooMohamadkhaniDepartment of Epidemiology, School of Medicine, Rafsanjan University of Medical Sciences, Rafsanjan, IranZatollahAsemiResearch Center for Biochemistry and Nutrition in Metabolic Diseases, Kashan University of Medical Sciences, Kashan, IranSeyed TaghiHeydariHealth Policy Research Center, Institute of Health, Student Research Committee, Shiraz University of Medical Sciences, Shiraz, Iran;MojtabaAkbariDepartment of Epidemiology, Faculty of Health, Shiraz University of Medical Sciences, Shiraz, IranKamran BLankaraniHealth Policy Research Center, Institute of Health, Student Research Committee, Shiraz University of Medical Sciences, Shiraz, Iran;Journal Article20160724Background: The prevention and correction of vitamin D deficiency requires a precise depiction of the current situation and identification of risk factors in each region. The present study attempted to determine these entities using a systematic review and meta-analysis in Iran.Methods: Articles published online in Persian and English between 2000 and November 1, 2016, were reviewed. This was carried out using national databases such as SID, IranMedex, Magiran, and IranDoc and international databases such as PubMed, Google Scholar, and Scopus. The heterogeneity index among the studies was determined using the Cochran (Q) and I2 test. Based on the heterogeneity results, the random-effect model was applied to estimate the prevalence of vitamin D deficiency. In addition, meta-regression analysis was used to determine heterogeneity-suspected factors, and the Egger test was applied to identify publication bias.Results: The meta-analysis of 48 studies identified 18531 individuals with vitamin D deficiency. According to the random-effect model, the prevalence of vitamin D deficiency among male, female, and pregnant women was estimated to be 45.64% (95% CI: 29.63 to 61.65), 61.90% (95% CI: 48.85 to 74.96), and 60.45% (95% CI: 23.73 to 97.16), respectively. The results of the meta-regression analysis indicated that the prevalence of vitamin D deficiency was significantly different in various geographical regions (β=4.4; P=0.023).Conclusion: The results obtained showed a significant prevalence of vitamin D deficiency among the Iranian population, a condition to be addressed by appropriate planning.https://ijms.sums.ac.ir/article_40519_bddbb66fd289d778d066f4defa465364.pdfShiraz University of Medical SciencesIranian Journal of Medical Sciences0253-071643220180301Prevalence of Multiple Coronary Artery Disease Risk Factors in Kerman: A Population-Based Study in Southeast Iran1401494051710.30476/ijms.2018.40517ENHamidNajafipourCardiovascular Research Center, Institute of Basic and Clinical Physiology Sciences, Kerman University of Medical Sciences, Kerman, Iran0000-0002-8030-8704MahdiAfshariDepartment of Epidemiology, School of Public Health, Zabol University of Medical Sciences, Zabol, Iran0000-0002-3159-8741FarzanehRostamzadehPhysiology Research Center, Institute of Basic and Clinical Physiology Sciences and Department of Physiology, Kerman University of Medical Sciences, Kerman, Iran0000-0002-7879-9938Journal Article20170523Background: The risk of disease with 1 risk factor is increased by the presence of additional risk factors. The goal of this study was to assess the prevalence of multiple coronary artery disease (CAD) risk factors among adults in Kerman, Iran, to identify the population groups most at risk.Methods: The present study included 5900 adults aged between 15 and 75 years in 2011 in Kerman, Iran. They were selected by 1-stage cluster sampling. Blood pressure, fasting blood glucose, lipids, and 6 CAD risk factors were assessed in the study population. Standardized prevalence rates were compared between the genders and age groups using the χ2 test. A Phttps://ijms.sums.ac.ir/article_40517_b34f724ee314b6d33a682a01418076a9.pdfShiraz University of Medical SciencesIranian Journal of Medical Sciences0253-071643220180301From Negligence to Perception of Complexities in Adherence to Treatment Process in People with Diabetes: A Grounded Theory Study1501574052010.30476/ijms.2018.40520ENSeyedeh NarjesMousavizadehNursing and Midwifery School, Shahid Beheshti University of Medical Sciences, Tehran, IranTaherehAshktorabDepartment of Medical-Surgical Nursing, Nursing and Midwifery School, Shahid Beheshti University of Medical Sciences, Tehran, IranFazlollahAhmadiDepartment of Nursing, Faculty of Medical Sciences, Tarbiat Modares University, Tehran, IranMitraZandiDepartment of Medical-Surgical Nursing, Nursing and Midwifery School, Shahid Beheshti University of Medical Sciences, Tehran, IranJournal Article20161215Background: Poor adherence of patients with type 2 diabetes to treatment is one of the most complex and important clinical concerns. It is the main issue of the present decade and acknowledged as a challenge to control and treat diabetes. This study was carried out to explore and understand how adherence to treatment process occurs among Iranian patients with type 2 diabetes.Methods: The present study is qualitative with grounded theory approach. The data were collected from December 2015 to July 2016 in Tehran (Iran) through individual semi-structured in-depth interviews, field notes, and memos from 21 patients with type 2 diabetes; combined with two members of their families and a healthcare professional. The data were analyzed based on Corbin and Strauss constant comparative analysis (2008).Results: Adherence to treatment is a transitional, interactive, and continuous process. For patients with diabetes, this process includes unperceived threat in diagnosis time (poor knowledge and skills, bottleneck of dependencies, superficial understanding of the new situation), bitter belief (downhill quality of life, physical and emotional treatment feedbacks), and adaptation to treatment (self-care dominance, regimen integration in daily activities). The process of adherence to treatment was influenced by knowledge and skill, social support, beliefs and values, psychological characteristics of people, and the nature of diabetes. Conclusion: Adherence to treatment in Iranian people with diabetes depends on the family and social context, which is challenging for the patient and leads to the negligence of health behaviors. It is vital for healthcare providers to identify these factors to encourage patients to adhere and commit to treatment in order to prevent irreversible complications of diabetes.https://ijms.sums.ac.ir/article_40520_4605d0df79437039351724df8363683f.pdfShiraz University of Medical SciencesIranian Journal of Medical Sciences0253-071643220180301The Effects of Size and Type of Vocal Fold Polyp on Some Acoustic Voice Parameters1581634052310.30476/ijms.2018.40523ENElahehAkbariDepartment of Speech and Language Pathology, School of Rehabilitation Sciences, Iran University of Medical Sciences, Tehran, IranSadeghSeifpanahiDepartment of Speech and Language Pathology, School of Rehabilitation Sciences, Hamadan University of Medical Sciences, Hamadan, IranAliGhorbaniDepartment of Speech and Language Pathology, School of Rehabilitation Sciences, Iran University of Medical Sciences, Tehran, Iranhttps://orcid.org/0000-0002-4817-7476FarzadIzadiENT-Head and Neck Research Center, Hazrat Rasoul AKram Hospital, Iran University of Medical Sciences, Tehran, IranFarhadTorabinezhadDepartment of Speech and Language Pathology, School of Rehabilitation Sciences, Iran University of Medical Sciences, Tehran, IranJournal Article20170205Background: Vocal abuse and misuse would result in vocal fold polyp. Certain features define the extent of vocal folds polyp effects on voice acoustic parameters. The present study aimed to define the effects of polyp size on acoustic voice parameters, and compare these parameters in hemorrhagic and non-hemorrhagic polyps.Methods: In the present retrospective study, 28 individuals with hemorrhagic or non-hemorrhagic polyps of the true vocal folds were recruited to investigate acoustic voice parameters of vowel/ æ/ computed by the Praat software. The data were analyzed using the SPSS software, version 17.0. According to the type and size of polyps, mean acoustic differences and correlations were analyzed by the statistical t test and Pearson correlation test, respectively; with significance level below 0.05. Results: The results indicated that jitter and the harmonics-to-noise ratio had a significant positive and negative correlation with the polyp size (P=0.01), respectively. In addition, both mentioned parameters were significantly different between the two types of the investigated polyps.Conclusion: Both the type and size of polyps have effects on acoustic voice characteristics. In the present study, a novel method to measure polyp size was introduced. Further confirmation of this method as a tool to compare polyp sizes requires additional investigations.https://ijms.sums.ac.ir/article_40523_fbfd5a40e45e6f02a0681491de0273f2.pdfShiraz University of Medical SciencesIranian Journal of Medical Sciences0253-071643220180301Hyperuricemia Induces Wnt5a/Ror2 Gene Expression, Epithelial–Mesenchymal Transition, and Kidney Tubular Injury in Mice1641734052510.30476/ijms.2018.40525ENWiwit Ananda WahyuSetyaningsihDepartment of Anatomy Faculty of Medicine, Universitas Gadjah Mada, Yogyakarta, IndonesiaNurArfianDepartment of Anatomy Faculty of
Medicine, Universitas Gadjah Mada,
Yogyakarta, Pharmaco Street,
North Sekip, Yogyakarta 55281,
Indonesia0000-0003-1694-2054EfrayimSuryadiMuhammad MansyurRomiUntungTranggonoDwi Cahyani RatnaSari0000-0002-1126-4939Journal Article20161104Background: Hyperuricemia contributes to kidney injury, characterized by tubular injury with epithelial–mesenchymal transition (EMT). Wnt5a/Ror2 signaling drives EMT in many kidney pathologies. This study sought to evaluate the involvement of Wnt5a/Ror2 in hyperuricemia-induced EMT in kidney tubular injury.Methods: A hyperuricemia model was performed in male Swiss background mice (3 months old, 30–40 g) with daily intraperitoneal injections of 125 mg/kg body weight (BW) of uric acid. The mice were terminated on day 7 (UA7, n=5) and on day 14 (UA14, n=5). Allopurinol groups (UAl7 and UAl14, each n=5) were added with oral 50 mg/kg BW of allopurinol treatment. The serum uric acid level was quantified, and tubular injury was assessed based on PAS staining. Reverse transcriptase-PCR was done to quantify Wnt5a, Ror2, E-cadherin, and vimentin expressions. IHC staining was done for E-cadherin and collagen I. We used the Shapiro–Wilk for normality testing and one-way ANOVA for variance analysis with a Phttps://ijms.sums.ac.ir/article_40525_6d96ac3f398b941dd16064315fdef106.pdfShiraz University of Medical SciencesIranian Journal of Medical Sciences0253-071643220180301Urtica Dioica Distillate Regenerates Pancreatic Beta Cells in Streptozotocin-Induced Diabetic Rats1741834052610.30476/ijms.2018.40526ENAliGohariDepartment of Biochemistry, School of Medicine, Shiraz University of Medical Sciences, Shiraz, IranAliNoorafshanHistomorphometry and Stereology Research Center, Shiraz University of Medical Sciences, Shiraz, Iran0000-0003-1082-011XMasoumehAkmaliDepartment of Biochemistry, School of Medicine, Shiraz University of Medical Sciences, Shiraz, IranFahimehZamani-GarmsiriDepartment of Biochemistry, School of Medicine, Shiraz University of Medical Sciences, Shiraz, IranAtefehSeghatoleslamResearch Center for Traditional Medicine and History of Medicine, Shiraz University of Medical Sciences, Shiraz, IranJournal Article20161005Background: Urtica dioica is known as an anti-hyperglycemic plant. Urtica dioica distillate (UD) is a traditional Iranian drink, locally known as “aragh gazaneh”. In spite of its widespread consumption in Iran, according to traditional Iranian medicine, there is no scientific report on the usefulness of UD for diabetic patients. This survey was designed to evaluate its protective effects for the recovery from diabetes by determining the serum insulin, blood glucose, volume of pancreatic islets, and the number and volume of β-cells in diabetic rats.Methods: A total of 48 Sprague-Dawley male rats (200-250 g) were randomly distributed into 6 groups (n=8), including non-diabetic plus distilled water (DW), non-diabetic plus UD, diabetic plus DW, diabetic plus UD, diabetic plus insulin, and diabetic plus glibenclamide. DW, UD, and glibenclamide were administered via intragastric gavage and insulin was injected subcutaneously. After four weeks of experiments, blood samples were collected for serum insulin and blood glucose assay. Pancreas was also evaluated using stereological method. The SPSS software was used for statistical analysis. Kruskal-Wallis, repeated measurements, and Mann-Whitney U test were applied for comparisons between the groups.Results: The treatment of diabetic rats with UD reduced the blood glucose dramatically (P<0.001) and increased serum insulin levels significantly (P=0.03) in comparison to the diabetic plus DW rats. Treatment with UD did not affect the mean β-cell volumes in the diabetic rats when compared to the diabetic plus DW rats, but the islet volumes and β-cell numbers were significantly recovered.Conclusion: UD treatment in diabetic rats improves hyperglycemia by partially restoring plasma insulin levels. The data suggest that UD prevents islet atrophy and/or regenerate pancreatic β-cells.https://ijms.sums.ac.ir/article_40526_505e88fe91ac7aa2bd4f66c27ec25428.pdfShiraz University of Medical SciencesIranian Journal of Medical Sciences0253-071643220180301The Preventive Role of Pioglitazone in Glycerol-Induced Acute Kidney Injury in Rats during Two Different Treatment Periods1841944052710.30476/ijms.2018.40527ENRamaMouslehDepartmentof Pharmacology and Toxicology, Faculty of Pharmacy, Damascus University, Damascus, SyriaShazaAl LahamDepartmentof Pharmacology and Toxicology, Faculty of Pharmacy, Damascus University, Damascus, SyriaAhmadAl-ManadiliDepartmentof Oral Histopathology, Faculty of Dentistry, Damascus University, Damascus, SyriaJournal Article20161125Background: Acute kidney injury is the most life-threatening complication of rhabdomyolysis. Glycerol is commonly used to induce this injury. The aim was to investigate the renoprotective effects of pioglitazone and the possible advantage of administering the drug for a longer period.Methods: Twenty-four male Albino Wistar rats were randomly divided into 4 groups (n=6/group): (A) control, (B) glycerol (50%, 10 mL/kg intramuscularly), (C) glycerol+pioglitazone (10 mg/kg orally for 3 days), and (D) glycerol+pioglitazone (for 6 days). Serum urea and creatinine levels were measured to assess the renal function. Reduced glutathione (GSH) levels and histological alterations were also measured. Statistical analysis was performed using Prism (version 6). The numerical data were evaluated by ANOVA, followed by the Tukey tests. The categorical data were evaluated by the Mann–Whitney test and the Fisher exact tests. Phttps://ijms.sums.ac.ir/article_40527_65eb06354fb89d5953bfcabcbd754edd.pdfShiraz University of Medical SciencesIranian Journal of Medical Sciences0253-071643220180301Amelioration of Pentylenetetrazole-Induced Seizures by Modulators of Sigma, N-Methyl-D-Aspartate, and Ryanodine Receptors in Mice1952014052810.30476/ijms.2018.40528ENMojtabaKeshavarzDepartment of Pharmacology, Bushehr University of Medical Sciences, Bushehr, Iran; and Shiraz Neuroscience Research Center, Shiraz University of Medical Sciences, Shiraz, Iran;BehdadYekzamanSchool of Medicine, Bushehr University of Medical Sciences, Bushehr, IranJournal Article20161008Background: Sigma receptors, N-methyl-D-aspartate (NMDA) antagonist, and modulators of intracellular calcium may be useful for seizure control. Therefore, we aimed to evaluate the antiepileptic effects of opipramol, a sigma receptor agonist, against pentylenetetrazole (PTZ)-induced seizures in mice and assess ketamine and caffeine interaction with the antiepileptic effects of opipramol.Methods: PTZ (100 mg/kg) was used for the induction of seizure in 72 male albino Swiss strain of mice (n=8). Opipramole (10, 20, and 50 mg/kg), ketamine (50 mg/kg), caffeine (200 mg/kg), opipramole (20 mg/kg) plus ketamine (50 mg/kg), opipramole (20 mg/kg) plus caffeine (200 mg/kg), diazepam (5 mg/kg as a positive control), and the vehicle were administered interaperitoneally 30 minutes before the injection of PTZ. The latency was recorded for the clonic, tonic-clonic seizures, and death of animals after the injection of PTZ. Kruskal-Wallis test followed by Dunn’s test was used for the analysis of data. Statistical analysis was performed with the SPSS software version 23.0 and Phttps://ijms.sums.ac.ir/article_40528_7a29df970b2c2a12996343eaa089bc41.pdfShiraz University of Medical SciencesIranian Journal of Medical Sciences0253-071643220180301First Report on the Percentiles of the Glomerular Filtration Rate in Iranian Children Using the 2009 Schwartz Equations2022074051510.30476/ijms.2018.40515ENPeymanRoomizadehSchool of Medicine, Iran University of Medical Sciences, Tehran, IranAlalehGheissariIsfahan Kidney Diseases Research Center, Isfahan University of Medical Sciences, Isfahan, Iran; and Department of Pediatric Nephrology, Isfahan University of Medical Sciences, Isfahan, IranRoyaKelishadiChild Growth and Development Research Center, Department of Pediatrics, Isfahan University of Medical Sciences, Isfahan, Iran;FarhadMahmoudiMedical Students’ Research Center, Isfahan University of Medical sciences, Isfahan, IranAminAbediniIsfahan Kidney Diseases Research Center, Isfahan University of Medical Sciences, Isfahan, IranJournal Article20170414The glomerular filtration rate (GFR) is widely considered the best overall index of renal function. The Schwartz equations are designed for measuring the GFR in children between 1 and 16 years of age. In the present study, we investigated the percentiles of the GFR in a general population of Iranian children with no known renal disease via the 2009 Schwartz equations (updated and combined equations). Between 2010 and 2011, we selected 687 children aged 7–16 years from the Iranian province of Isfahan. Blood samples were analyzed for blood urea nitrogen, creatinine, and cystatin C. For each child, we calculated the GFR using 2 Schwartz equations. The Wilcoxon test was applied to examine the differences in the estimated GFRs between the equations. To determine the correlation between the GFRs obtained via the updated and combined Schwartz equations in the boys and the girls and also for the correlation between age and the GFR, we performed the Pearson or Spearman correlation coefficients. The statistical analyses were conducted using MedCalc, version 12.1.4.0 (MedCalc Software, Mariakerke, Belgium). The mean GFR was 100.06±19.80 mL/min/1.73 m2 based on the updated equation and 96.10±18.44 mL/min/1.73 m2 according to the combined equation. No significant differences were observed between these equations in estimating the GFRs. The correlation analysis for determining the association between age and the GFR estimated by the updated (r=0.05, P=0.1) and combined (r=0.06, P=0.09) Schwartz equations was not statistically significant. In conclusion, in the current study we showed that the updated and combined Schwartz equations exhibited high concordance in estimating GFR values in this age group.https://ijms.sums.ac.ir/article_40515_0e9a68f0a0f105f967b4816204727554.pdfShiraz University of Medical SciencesIranian Journal of Medical Sciences0253-071643220180301Optimization of the Static Human Osteoblast/Osteoclast Co-culture System2082134052110.30476/ijms.2018.40521ENJames JamJollyDepartments of Pharmacology, Faculty of Medicine, Pusat Perubatan University Kebangsaan Malaysia, Jalan Yaacob Latif, Bandar Tun Razak, 56000 Cheras, Wilayah Persekutuan Kuala Lumpur, MalaysiaKok-YongChinDepartments of Pharmacology, Faculty of Medicine, Pusat Perubatan University Kebangsaan Malaysia, Jalan Yaacob Latif, Bandar Tun Razak, 56000 Cheras, Wilayah Persekutuan Kuala Lumpur, MalaysiaMohd FoziNur FarhanaDepartments of Pharmacology, Faculty of Medicine, Pusat Perubatan University Kebangsaan Malaysia, Jalan Yaacob Latif, Bandar Tun Razak, 56000 Cheras, Wilayah Persekutuan Kuala Lumpur, MalaysiaEkramAliasDepartments of Biochemistry, Faculty
of Medicine, Pusat Perubatan University Kebangsaan Malaysia, Jalan Yaacob Latif, Bandar Tun Razak, 56000 Cheras, Wilayah Persekutuan Kuala Lumpur, MalaysiaKien HuiChuaDepartments of Physiology, Faculty of Medicine, Pusat Perubatan University Kebangsaan Malaysia, Jalan Yaacob Latif, Bandar Tun Razak, 56000 Cheras, Wilayah Persekutuan Kuala Lumpur, MalaysiaWan NurainiWan HasanDepartments of Pharmacology, Faculty of Medicine, Pusat Perubatan University Kebangsaan Malaysia, Jalan Yaacob Latif, Bandar Tun Razak, 56000 Cheras, Wilayah Persekutuan Kuala Lumpur, MalaysiaSoelaimanIma-NirwanaDepartments of Pharmacology, Faculty of Medicine, Pusat Perubatan University Kebangsaan Malaysia, Jalan Yaacob Latif, Bandar Tun Razak, 56000 Cheras, Wilayah Persekutuan Kuala Lumpur, MalaysiaJournal Article20170428Osteoblasts (OBs) and osteoclasts (OCs) are 2 major groups of bone cells. Their cell-to-cell interactions are important to ensure the continuity of the bone-remodeling process. Therefore, the present study was carried out to optimize an OB/OC co-culture system utilizing the human OB cell line hFOB 1.19 and OCs extracted from peripheral blood mononuclear cells (PBMNCs). It was a 2-step procedure, involving the optimization of the OB culture and the co-culture of the OBs with PBMNCs at an optimum ratio. Firstly, pre-OBs were cultured to 90% confluency and the time required for differentiation was determined. OB differentiation was determined using the van Gieson staining to detect the presence of collagen and Alizarin Red for calcium. Secondly, OBs and OCs were co-cultured at the ratios of 1 OC: 1 OB, 1 OC: 4 OBs, 2 OCs: 1 OB, and 1 OC: 2 OBs. Tartrate-resistant acid phosphatase (TRAP) staining was used to detect the differentiation of the OCs. The results showed that collagen was present on day 1, whereas calcium was detected as early as day 3. Based on the result of TRAP staining, 1 OC: 2 OBs was taken as the most appropriate ratio. No macrophage colony-stimulating factor and receptor activator of the nuclear factor-κB ligand were added because they were provided by the OBs. In conclusion, these optimization processes are vital as they ensure the exact time point and ratio of the OB/OC co-culture in order to produce a reliable and reproducible co-culture system. https://ijms.sums.ac.ir/article_40521_06aa8a0a8bb6d72b5c959c44f7edbc09.pdfShiraz University of Medical SciencesIranian Journal of Medical Sciences0253-071643220180301Pulmonary Embolism as a Rare Complication of Liver Hydatid Cyst: Report of Two Cases2142174051410.30476/ijms.2018.40514ENOmarToumiDepartment of General Surgery, Fattouma Bourguiba Hospital, Faculty of Medicine, University of Monastir, Monastir, Tunisia0000-0002-5777-5881RandaSalemDepartment of Radiology, Fattouma Bourguiba Hospital, Faculty of Medicine, University of Monastir, Monastir, TunisiaSadok BenJabraDepartment of General Surgery, Fattouma Bourguiba Hospital, Faculty of Medicine, University of Monastir, Monastir, TunisiaGhediraAbdesslemDepartment of General Surgery, Fattouma Bourguiba Hospital, Faculty of Medicine, University of Monastir, Monastir, TunisiaFaouziNoomenDepartment of General Surgery, Fattouma Bourguiba Hospital, Faculty of Medicine, University of Monastir, Monastir, TunisiaAbdel AzizHamdiDepartment of General Surgery, Fattouma Bourguiba Hospital, Faculty of Medicine, University of Monastir, Monastir, TunisiaJournal Article20161018Hydatid cyst is a helminth infection. The rupture of a hepatic hydatid cyst in inferior vena cava is a rare and lethal complication. Pulmonary embolism is the commonest manifestation. The diagnosis of hydatid cyst is made by histopathological or serological examination, and imaging may suggest the probability of hydatid cyst. Surgical treatment should be performed with caution and always under vascular control. We present two cases of hydatid cyst ruptured into the inferior vena cava. The patients also presented with dyspnea, hemoptysis, and intermittent cough at the time of admission. This presentation will be accompanied by a brief review of the literature. The radiological findings suggested the diagnosis. The first patient had a fatal hydatid pulmonary embolism and death occurred on the second day of hospitalization. The second patient refused surgery. These cases also exemplify the clinical and radiographic findings as well as the management of this complication.https://ijms.sums.ac.ir/article_40514_7b97f1e1b1f7a678dba768809a2f96c7.pdfShiraz University of Medical SciencesIranian Journal of Medical Sciences0253-071643220180301A New Mutation Causing Severe Infantile-Onset Pompe Disease Responsive to Enzyme Replacement Therapy2182224051810.30476/ijms.2018.40518ENHosseinMoravejNeonatal Research Center, Shiraz University of Medical Sciences, Shiraz, Iran; and Department of Pediatrics, School of Medicine, Shiraz University of Medical Sciences, Shiraz, IranAnisAmirhakimiDepartment of Pediatrics, School of Medicine, Shiraz University of Medical Sciences, Shiraz, Iran0000-0002-2365-6286AlirezaShowrakiSchool of Medicine, Shiraz University of Medical Sciences, Shiraz, IranHamidAmoozgarNeonatal Research Center, Shiraz University of Medical Sciences, Shiraz, IranZahraHadipourDepartment of Medical Genetics, Sarem Cell Research Center and Hospital, Tehran, IranGhasemNikfarDepartment of Pediatrics, Fasa University of Medical sciences, Fasa, IranJournal Article20161111Pompe disease (PD), also known as “glycogen storage disease type II (OMIM # 232300)” is a rare autosomal recessive disorder characterized by progressive glycogen accumulation in cellular lysosomes. It ultimately leads to cellular damage. Infantile-onset Pompe disease (IOPD) is the most severe type of this disease and is characterized by severe hypertrophic cardiomyopathy and generalized hypotonia. Mutations in the acid alpha-glucosidase (GAA) gene, located at locus 17q25.3, are responsible for the disease leading to reduced activity of the acid alpha-glucosidase enzyme. To date, approximately 400 pathogenic mutations have been reported in the GAA gene. The aim of this study is to report a novel nonsense mutation in exon 4 of the GAA gene in an Iranian child suffering from IOPD. The patient was a female neonate with hypertrophic cardiomyopathy and a positive family history of IOPD. After definite diagnosis, enzyme-replacement therapy (ERT) was started for the patient, who was 2 months old. Now at the age of 20 months, she has had good growth and development and her echocardiographic parameters are within the normal range. This report shows that IOPD patients with this mutation can be treated with ERT successfully.https://ijms.sums.ac.ir/article_40518_0ea089ce08791952f534e3214b2cb8ea.pdfShiraz University of Medical SciencesIranian Journal of Medical Sciences0253-071643220180301Central Acinic Cell Carcinoma of the Mandible Simulating as Benign Odontogenic Lesion: A Case Report2232264052210.30476/ijms.2018.40522ENManasBajpaiDepartment of Oral and maxillofacial Pathology, NIMS Dental College, Jaipur, IndiaNileshPardheDepartment of Oral and maxillofacial Pathology, NIMS Dental College, Jaipur, IndiaBetinaChandoliaDepartment of Oral and maxillofacial Pathology, NIMS Dental College, Jaipur, IndiaManikaAroraDepartment of Oral and maxillofacial Pathology, NIMS Dental College, Jaipur, IndiaJournal Article20160928Centrally occurring salivary gland tumors are rare. Because of a considerable overlap between the clinical and histopathological features, this group of tumors often produces a diagnostic difficulty to the clinicians and oral pathologists. Acinic cell carcinoma (ACC) is an unusual, low-grade, malignant salivary gland tumor that represents approximately 2% of the salivary gland tumors with almost 90% arising in the parotid gland. The rest involve the submandibular and the minor salivary gland. ACC of the jaw is extremely rare and, to our knowledge, only 8 cases have been reported in the English literature. Herein, a case of primary intraosseous ACC of the mandible in a 31-year-old woman is presented. The present case is unique, as the central ACC has never been reported in a patient in the third decade of life. The complete surgical removal of the tumor was carried out under general anesthesia along with the extraction of teeth #31, #32, #41, and #42. The follow-up period of 1-year was uneventful.https://ijms.sums.ac.ir/article_40522_85c8221443b400c4f6bfdd7ef7c31b81.pdfShiraz University of Medical SciencesIranian Journal of Medical Sciences0253-071643220180301Simultaneous Presence of Macular Corneal Dystrophy and Retinitis Pigmentosa in Three Members of a Family2272304052410.30476/ijms.2018.40524ENFarhadNejatVision Health Research Center, Tehran, Iran0000-0002-9614-0926HosseinAghamollaeiApplied Biotechnology Research Center, Baqiyatallah University of Medical Sciences, Tehran, IranShivaPirhadiDepartment of Biomedical Engineering, Science and Research Branch, Islamic Azad University, Tehran, IranKhosrowJadidiDepartment of Ophthalmology, Baqiyatallah University of Medical Sciences, Tehran, IranMohammad AminNejatDepartment of Biomedical Engineering, Science and Research Branch, Islamic Azad University, Tehran, IranJournal Article20160725Macular corneal dystrophy (MCD) is an autosomal recessive hereditary disease. In most cases, various mutations in carbohydrate sulfotransferase 6 (CHST6) gene are the main cause of MCD. These mutations lead to a defect in keratan sulfate synthesis. Retinitis pigmentosa (RP) is another eye disorder with nyctalopia as its common symptom. It has been shown that more than 65 genes have been implicated in different forms of RP. Herein, we report on a 9-member family with 2 girls and 5 boys. Both parents, one of the girls and one of the boys had normal eye vision and another boy had keratoconus. Other children (1 girl and 2 boys) suffered from both MCD and RP. Corneal transplantation and medical supplements were used for MCD and RP during the follow-up period, respectively. Based on the family tree, it seems that the inheritance of both diseases is autosomal recessive. Based on our search of databases, there is no report on the simultaneous presence of MCD and RP. To the best of our knowledge, the present article is the first case report on this topic. Molecular genetic investigation is needed to clarify the mechanism of concurrent MCD and RP.https://ijms.sums.ac.ir/article_40524_33471711356764d3913fdad5c90781da.pdf