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Background: Cystic fibrosis is a chronic and progressive genetic disease with a worldwide prevalence. As the disease progresses, symptoms develop, and make its management more challenging. Accumulating evidence suggests that early diagnosis of CF can significantly contribute to preventing reported nutritional problems including anemia, vitamin deficiencies, and hypoalbuminemia. This cross-sectional study was conducted to assess disease severity in cystic fibrosis patients using the Shwachman-Kulczycki score, as well as to determine its relation with anemia and vitamin D deficiency. Materials and Methods: Clinical and CF-related laboratory data were collected from the medical records of 57 CF patients with a definitive diagnosis. At the time of diagnosis, physicians performed simultaneous, blood sampling and scoring of patients using the Shwachman scoring system. Results: The mean age of patients was 16.12±6.48 years. Total scores of 86-100, 71-85, 56-70, 41-55, and <40, were reported in 5.4%, 7.1%, 14.3%, 14.3%, and 58.9% of CF patients, respectively. A significant correlation was found between disease severity and patients' age (P=0.02). The analysis also showed that the disease severity was significantly higher in anemic patients when compared to non-anemics (p =0.006). Based on the results, 33 patients with normochromic, 11 patients with microcytic, and 6 patients with macrocytic anemia were diagnosed in this study. We did not find a significant difference between disease severity and vitamin D levels (P=0.150). Conclusion: The scoring system used in the current study could reflect properly the clinical status of CF patients. However, simultaneous use of various methods using a larger sample size for comparison of results is suggested to improve the accuracy of findings. [ABSTRACT FROM AUTHOR]

Streptococcus iniae is among the major pathogens of a large number of fish species cultured in fresh and marine recirculating and net pen production systems. Ten Iranian medicinal plants were assessed for their antimicrobial activity against Streptococcus iniae isolates obtained from diseased Oncorhynchus mykiss (Salmonidae; Walbaum, 1972) collected from fish farms in Iran. The antibacterial activity of ethanol extracts of Punica granatum, Quercus branti, Glycyrrhiza glabra and essential oils of Heracleum lasiopetalum, Satureja bachtiarica, Thymus daenensis, Myrtus communis, Echinophora platyloba, Kelussia odoratissima and Stachys lavandulifolia against Steptococcus iniae was evaluated by disc diffusion and serial dilution assays. Most of the extracts and essential oils showed a relatively high antibacterial activity against Streptococcus iniae. Of the plants studied, the most active extracts were those obtained from the essential oils of Satureja bachtiarica, Echinophora platyloba, Thymus daenensis and the ethanol extract of Quercus branti. Some of the extracts were active against Streptococcus iniae. Two essential oils showed lower MIC values; Heracleum lasiopetalum (78 μg/ml) and Satureja bachtiarica (39 μg/ml). The essential oil of Satureja bachtiarica could be an important source of antibacterial compounds against the Streptococcus iniae isolated from rainbow trout.

Background: Tranexamic acid (TXA) is an inexpensive therapy effective at minimizing perioperative blood loss and transfusion. However, it remains underutilized due to safety concerns. Cleft lip and cleft palate are among the most frequent congenital anomalies, accounting for 65% of head and neck anomalies, and 15000 neonates are born with these anomalies annually. Objectives: This study aimed to investigate the effect of preoperative intravenous TXA on hemorrhage in patients undergoing cleft palate reconstruction surgery in the Khatam Al-Anbia Hospital, Zahedan, Iran. Methods: This clinical trial was conducted on 60 patients undergoing palatoplasty in the Khatam Al-Anbia Hospital. The patients were randomly assigned into two groups: TXA receivers and controls. Data were collected using information forms and then analyzed using SPSS ver. 22. Results: The mean age of the participants was 21.42±9.46 months, and of the 60 patients, 31 were boys, and 29 were girls. The mean bleeding volume was 11.73±4.42 milliliter in the TXA receiving group and 17.36±4.99 milliliters in the control group, and the mean duration of surgery was 41.90±8.00 and 49.93±11.37 minutes in the TXA receiving and control groups, respectively. The mean PT, PTT, and Hb were not significantly different before and after surgery in the two groups. Conclusions: Tranexamic acid causes a significant reduction in the duration of surgery and mean bleeding volume in palatoplasties, but it has no effects on PT, PTT, and Hb levels before and after surgery. [ABSTRACT FROM AUTHOR]

Pearson syndrome is a rare mitochondrial disorder confirmed by mt-DNA deletion which typically occurs in the first two years of life. That is to say children are at high fatal risk, most infants are marked with some common features especially anemia and pancreatitis, which results in death in early childhood. A 6-month-old Iranian female infant was presented with macrocytic anemia, required packed red blood cell transfusions. She also was affected by exocrine pancreatic dysfunction, in which she underwent Creon treatment. By first year of age she had experienced some severe metabolic crises intermittently. After hospitalized for some months she was expired unfortunately. In conclusion, Pearson syndrome, as a rare disease affects many organs, such as liver, kidney, pancreas, bone marrow, which led to anemia, failure to thrive, and multi organ failure. In such cases, a physician must consider and evaluate all possible damages, especially anemia and pancreatitis. We present a case of Pearson syndrome with anemia. [ABSTRACT FROM AUTHOR]

Cerebral ischemic stroke is a common leading cause of disability. Flaxseed is a richest plant-based source of antioxidants. In this study, the effects of flaxseed oil (FSO) pretreatment on functional motor recovery and gene expression and protein content of neurotrophic factors in motor cortex area in rat model of brain ischemia/reperfusion (I/R) were assessed. Transient middle cerebral artery occlusion (tMCAo) in rats was used as model brain I/R. Rats (6 in each group) were randomly divided into four groups of Control (Co+normal saline [NS]), Sham (Sh+NS), tMCAo+NS and tMCAo+FSO. After three weeks of pretreatment with vehicle or FSO (0.2 ml~800 mg/kg body weight), the rats were operated in sham and ischemic groups. Ischemia was induced for 1 h and then reperfused. After 24 h of reperfusion, neurological examination was performed, and animals were sacrificed, and their brains were used for molecular and histopathological studies. FSO significantly improved the functional motor recovery compared with tMCAo+NS group (P<0.05). A significant reduction in brain-derived neurotrophic factor (BDNF) and glial cell-derived neurotrophic factor (GDNF) mRNAs and protein levels were observed in the tMCAo+NS group compared with Co+NS and Sh+NS group (P<0.05). A significant increase of BDNF and GDNF mRNAs and proteins was recorded in the tMCAo+FSO group compared with Co+NS, Sh+NS and tMCAO+NS groups (P<0.05). The results of the current study demonstrated that pretreatment with FSO had neuroprotective effects on motor cortex area following cerebral ischemic stroke by increasing the neurotrophic factors (BDNF, GDNF). [ABSTRACT FROM AUTHOR]

This study investigates the effects of melatonin on the sperm quality and testis weight after the combination of swimming exercise and nandrolone decanoate (DECA). Two groups of male Wistar rats were treated for eight weeks as follows; group A consist of CO (control), Sham, N (DECA), S (swimming) and NS (DECA plus swimming); and group B: Sham M (sham melatonin), M (melatonin), MN (melatonin plus DECA), MS (melatonin plus swimming), MNS (melatonin, DECA plus swimming). The motility of sperm was significantly improved in melatonin groups in comparison to N, S and NS groups (P≤0.05). The left testes weight was decreased in N, NS and MNS groups, and the right testes weight was decreased in N,S,NS, MS and MNS groups in compare with the control group. This study concluded that melatonin probably could improve the sperm motility and sex organs weight after the combination of DECA and exercise. [ABSTRACT FROM AUTHOR]

Introduction: Stroke is one of the most important reasons of death. Hence, trials to prevent or lessen the complications originated by stroke are a goal of public health worldwide. The ischemia-reperfusion causes hypoxia, hypoglycemia and incomplete repel of metabolic waste products and leads to accumulation of free radicals triggering neuronal death. The A1 adenosine receptoras an endogenous ligand of adenosine is known to improve cell resistance to destructive agentsby preventing apoptosis. Vitamin C as a cellular antioxidant is also known as an effective factor to reduce damages initiated by free radicals. We studied the protective effects of A1 receptor agonist in combination with vitamin C against ischemia-reperfusion. Methods: Ischemia was induced by common carotid artery occlusion in bulb-c mice (20-30 gr). Y-Maze was employed to scale the short-term memory and Nissl staining was used to count the cells in hippocampus. Results: We found that concurrent treatment of A1 receptor agonist and vitamin C significantly reduced neuronal death in CA1. The Memory scores were also significantly improved (P<0.05). Discussion: Our data point to the therapeutic effects of CPA/vitamin C co-administration and highlight the beneficial role of A1 adenosine receptor signaling in the context of stroke. [ABSTRACT FROM AUTHOR]

Objective(s) Bone marrow is the traditional source of human multipotent mesenchymal stem cells (MSCs), but adipose tissue appears to be an alternative and more readily available source. In this study, rat adipose-derived stem cells (ADSCs) were induced to differentiate into Schwann-like cells and compared with rat bone marrow stem cells (BMSCs) for their Schwann-like cells differentiation potential. Materials and Methods BMSCs and ADSCs were characterized for expression of MSCs-specific markers, osteogenic and adipogenic differentiation. They were induced to differentiate into Schwann-like cells and analyzed for expression of the Schwann specific markers. The immunocytochemical differentiation markers were S-100 and real time quantitative Real-time polymerase chain reaction (RT-PCR) markers were S100, P75 and glial fibrillary acidic protein (GFAP). 3-(4, 5-Dimethylthiazol- 2-yl)-2, 5-diphenyltetrazolium bromide (MTT) assay and Annexin V-Fluorescein isothiocyanate (FITC)/ Propidium iodide (PI) double labeling method were employed to detect early stage cell apoptosis. Results BMSCs and ADSCs showed similarities in expression of the MSC-specific markers, osteogenic and adipogenic differentiation. Both quantitative RT-PCR and immunocytochemical analysis demonstrated that BMSCs and ADSCs had equal expression of the Schwann-specific markers following Schwann-like cells differentiation. However, gene expression of P75 was higher in BMSCs compared with ADSCs. MTT assay and flow cytometry found that of the total BMSCs and ADSCs in the culture medium, 20% to 30% of the cells died, but the remaining cell population remained strongly attached to the substrate and differentiated. Conclusion Comparative analysis showed that Schwann-like cell differentiation potential of ADSCs was slightly decreased in comparison with BMSCs. Therefore, BMSCs are more favorable choice than ADSCs for tissue engineering. [ABSTRACT FROM AUTHOR]

Background: G6PC3 deficiency is a new neutropenic syndrome, which is characterized by severe persistent neutropenia, early onset infections and additional organ involvement, especially cardiac and urogenital malformations. Case Presentation: In this report, we present the clinical details of a recently known case of severe congenital neutropenia (SCN) with G6PC3 mutation, who experienced the first episode of infections at birth. Repeated absolute neutrophil count of less than 500/μl was detected during work-up of sepsis in the first month of life. SCN was diagnosed and granulocyte colonystimulating factor (GCSF) administration initiated. Bone marrow examination revealed maturation arrest in myeloid series at promyelocyte-myelocyte stage. Diarrhea, bronchiolitis, and urinary tract infection were other infectious complications, while hydronephrosis, atrial septal defect, and patent ductus arteriosus were other manifestations. Conclusion: Prompt and accurate diagnosis of neutropenic patients and appropriate treatment can prevent further complications and improve the quality of life of the affected patients. [ABSTRACT FROM AUTHOR]

Severe congenital neutropenia (SCN) is a rare primary myelopoiesis disorder, characterized by persistent severe neutropenia and early-onset bacterial infections. Herein, we describe an 11-month-old male who was referred with recurrent cutaneous infections and chronic diarrhea. Serial complete blood counts indicated persistent neutropenia. Bone marrow aspiration of the patient demonstrated maturation arrest of myeloid series at promyelocyte-myelocyte stages. W44X mutation in the HAX1 gene confirmed the diagnosis of SCN. The patient was successfully treated with granulocyte colony-stimulating factor. Severe congenital neutropenia should be considered in children with early-onset recurrent infections and neutropenia, since early diagnosis and appropriate treatment can prevent further complications. [ABSTRACT FROM AUTHOR]

Background: Colon tumor is generated and maintained by a small subset of chemo-resistant cancer cells known as Cancer Stem-like Cells (CSCs) that are able to self-renew and differentiate into various cell types within the cancer milieu. CSCs are identified through expression of CD133 that is the most important surface marker of these cells. Epithelial Cell Adhesion Molecule (EpCAM) is another colon CSCs marker. Other markers that are probably involved in colon tumorigenesis are Leucine-rich repeat- containing G-protein-coupled Receptor 5 (LGR5), B cell-specific Moloney murine leukemia virus insertion site 1 (BMI1) and Ten-Eleven Translocations (TETs). Methods: Here, mRNA expression rates of LGR5, BMI1 and TETs were surveyed by real-time PCR. After collection and digestion, colon samples were used to isolate CD133 and EpCAM positive CSCs through evaluation of AC133 EpCAM by Magnetic Activated Cell Sorting (MACS) and flow cytometry. Real-time PCR was carried out for assessing expressions of LGR5, BMI1 and TETs. Results: High expressions for LGR5, BMI1, TET1 and TET2 in the CD133 and EpCAM positive CSCs (p≤0.05 vs. non-CSCs) were found. TET3, however, showed no significant changes for mRNA expression in the CSCs. Conclusion: In conclusion, high mRNA expressions for LGR5, BMI1, TET1 and TET2 in the CD133 and EpCAM positive CSCs may be a useful criterion for better identification of the cells involved in colon cancer in order to specify therapeutic targets against this type of cancer. [ABSTRACT FROM AUTHOR]

Background: Progesterone as a sex steroid hormone is thought to affect and prevent demyelination, but its role in promoting myelin repair is far less investigated. In this study, remyelinating potential of progesterone in corpus callosum was evaluated on an experimental model of MS. Methods: In this experimental study, adult male C57BL/6 mice were fed with 0.2% (w/w) cuprizone in ground breeder chow ad libitum for 6 weeks. At day zero, after cuprizone removal, mice were divided randomly into two groups: (a) placebo group, which received saline pellet implant, (b) progesterone group, which received progesterone pellet implant. Some mice of the same age were fed with their normal diet to serve as the healthy control group. Two weeks after progesterone administration, Myelin content was assessed by Luxol-fast blue staining. The myelin basic protein (MBP) and proteolipid protein (PLP) expression were assessed using Western blot analysis and the changes in the number of oligodendrocytes and oligodendroglial progenitor cells were assessed by immunohistochemistry (IHC) and flow cytometry. Results: Luxol-fast blue staining revealed enhanced remyelination in the progesterone group when compared with the placebo group. Densitometry measurements of immunoblots demonstrated that MBP and PLP proteins contents were significantly increased in the progesterone group compared with the placebo group. Flow cytometry and IHC analysis showed increases in Olig2 and O4 cells in the progesterone group compared with the placebo group. Conclusion: Overall, our results indicate that progesterone treatment can stimulate myelin production and that it may provide a feasible and practical way for remyelination in diseases such as multiple sclerosis. [ABSTRACT FROM AUTHOR]

This article reviews the current knowledges of congenital bleeding disorders (CBD) amid the COVID-19 pandemic. It appears that CBD is not associated with higher risk of getting COVID-19 and so the prevalence of COVID-19 among them seems not higher compared to the general population. In absence of specific therapeutic recommendations, it is essential to make a correct assessment of the risk of haemorrhage/thrombosis. Based on expert opinion, strategies for outpatient management include adherence to prescribed regimens, telemedicine, and communication about COVID-19 in patients with CBD. More data should be also collected to better characterize the impact of COVID-19 on patients with CBD. The current findings encourage further studies to determine the prevalence of SARS-CoV2 infection in CBD patients to understand more fully the burden of this novel pathogen and to develop adequate preventive measures against this infection.

Background: Ischemic stroke is a common leading cause of death and disability with lack of effective therapies. In this study, T3 was intra-ventricularly injected to evaluate gene expression and protein concentration of and brain-derived neurotrophic factor (BDNF) and Glial cell-derived neurotrophic factor (GDNF) in hippocampal CA1 region in rat model of brain ischemia/reperfusion (I/R)., Methods: In this study, transient middle cerebral artery occlusion (tMCAo) was used as model of ischemic brain stroke. Rats were randomly divided in four groups of Co, Sh, tMCAo and tMCAo + T3. Then, a single dose of intra-ventricular T3 was administered via a Hamilton syringe. Passive avoidance test was used as behavioral investigations. After 21 days, the animals were sacrificed and their brains were used for molecular and histopathological studies., Results: T3 significantly improved the learning and memory compared with tMCAo group according to Morris water maze findings (P < 0.05). Step-through latency (STL) significantly decreased in tMCAo group (P < 0.05). There were significant increase in the STL of T3 group compared with tMCAo group (P < 0.05).A significant reduction in BDNF mRNAs and protein levels were observed in the tMCAo compared with Co and Sh group (P < 0.05). A significant increase of BDNF and GDNF mRNAs and proteins was recorded in tMCAo + T3 group compared with Co, Sh and tMCAO groups (P < 0.05)., Conclusions: The results of current study demonstrated that T3 had therapeutic effects on cerebral ischemic stroke by increasing the neurotrophic factors (BDNF, GDNF) in CA1 region of hippocampus. The effects of intracerebroventricular microinjection of T3on memory and learning in rat model of ischemic brain stroke.

There is some evidence that human T-cell lymphotropic virus (HTLV-1) infection has a reverse association with gastric cancer (GC). Data about this association in the Iranian population are scarce. In this study we therefore assessed the frequency of anti-HTLV-1 antibody in GC patients and compare it to antibody presence in healthy individuals in Iranian population. This case control study was performed between 2008-2011 on 201 GC patients and 219 control subjects. HTLV-1 antibodies were assessed by ELISA and the positive results were confirmed by western blotting. Totals of 201 gastric cancer patients and 219 controls were enrolled in this study. The tumors in the majority of patients (45.3%) were in the distal (non-cardia) area. Mean age of patients at the time of diagnosis was 59.2±12.5 and mean age of controls was 57.7±11.3. While only one GC patient (0.5%) was positive for HTLV-1 antibody, there were four individuals (1.89%) from the control group with antibodies. In addition, smoking had statistically significant relationship with cancer (P=0.001). Our study showed that the frequency of HTLV-1 antibody in patients was lower than in controls, similar to the results obtained in Japan. Further investigations with a larger sample size are needed in order to determine the association between GC and HTLV-1 infection in Iran.

Background and Aim: Colon polyps are important lesions and a concern because of the potential for colorectal cancer, one of the most common causes of cancer-related deaths in Iran. The distribution of polyps in the colon may affect the efficacy of screening modalities. The aim of this study was to determine clinical and pathology characteristics of colorectal polyps in the Iranian population., Methods: This cross sectional survey covered 856 polypectomies in 716 patients, with anatomical distribution, size and histopathology of the polyps described in 2004-2009 in the educational hospital of Taleghani in Tehran., Results: Polyps were observed in 437 males and 279 females. The distribution was 3.12 percent located in the rectum, 19.6 percent in the sigmoid colon, 24.4 percent in the descending colon, 13.9 percent in the transverse colon, and 29.6 percent in the cecum and ascending colon. Some 77(9%) were non-neoplastic and 779 (91%) were neoplastic. Adenomas were present in 727 (85%) cases, of these 411 (56%) were left-sided and 316 (44% ) were right-sided. Carcinoma was observed in 52 cases, 18(34.5%) being left sided and 34(65.5% of carcinomas) right sided. Of the total, 354 were advance polyp (>1cm, villous type, high grade dysplasia), 87(34%) being found in patients under 50 years of age and 149 (58.6 %) being right sided., Conclusion: This study showed a significant number of adenomas and carcinomas to lie proximal to the splenic flexure. Thus, it is expected that examination of the colon limited to the splenic flexure would miss 44% of such lesions. The increasing right-sided prevalence of these lesions with age suggests that evaluation of the proximal bowel is particularly important in older people. In addition there were higher stages of dysplasia and malignancy in larger polyps.

Background: A high rate of colorectal cancer occurrence is established in individuals with a positive family history of this type of cancer., Objectives: The aim of this study was to investigate the prevalence of colorectal cancer in first degree and second degree relatives of colorectal cancer patients., Methods: Family medical histories of 489 first degree relatives of colorectal cancer patients were obtained by a questionnaire. 249 average risk patients with no family history of colorectal cancer were included as control patients., Results: In our study from a total of 489 case patients, 153 (31.3%) had at least one close relative affected by colon cancer. Case-control analysis showed an odd ratio of 3.1 (95% CI, 2.07 to 6.27) for one and 5.7 (CI, 2.39 to 13.56) for two affected relatives. Cases with a positive family history had a 3.006 times greater risk in developing colorectal cancer if a first degree relative was affected comparing with a 4.9 times greater risk if a second degree family member was diagnosed with colorectal cancer. Our study indicated a higher risk for developing colorectal cancer in male family relatives 50 years and older. Rectal area was found the most tumor side affected in case and control patients., Conclusion: First-degree relatives of patients with colorectal cancer had an increased risk of developing this type of cancer. The risk was greater when diagnosis was in male, elderly patients and other first-degree relatives were affected.

Objectives: To investigate the prevalence of colorectal adenomas and other advanced lesions in first degree relatives of Iranian patients diagnosed with colorectal cancer by colonoscopy and pathologic evaluation., Methods: Iranian colorectal cancer patients (probands) were identified through pathologic reports of the regional cancer registry. First degree relatives (siblings, parents and offspring) of probands were evaluated for the existence of polyps and precancerous lesions via colonoscopy screening. Control patients were chosen among average risk population with no family history of colorectal cancer who agreed to colonoscopy screening., Results: A total of 184 subjects underwent colonoscopy screening from April 2007 to March 2009. From 90 cases among families of probands, 70 (77.8%) had a normal colonoscopy result, 18 (20%) demonstrated polyps and 2 (2.2%) were diagnosed with colorectal cancer. Colonoscopy screening in 94 average risk control patients discovered polyps in 4 (4.3%) cases and the other 90 (95.7%) patients had normal total colonoscopy results. Mean age of cases with advanced lesions was 48.4∓10.2 and 41.5∓18.4 in control patients with polyps. The incidence of polyps was significantly higher in males, and in family case patients compared to controls., Conclusions: Based on colonoscopy screening, the prevalence of colorectal adenoma and precancerous lesions in first degree relatives of patients diagnosed with colorectal cancer is significantly higher than in the average risk population.