Olmsted syndrome in an Iranian boy with a new de novo mutation in TRPV3.

Olmsted syndrome (OS) is a rare congenital skin disorder characterized by palmoplantar keratoderma, periorificial hyperkeratotic lesions and alopecia. Constriction of digits, onychodystrophy and pruritus may also occur. Recently, pathogenic heterozygous mutations in TRPV3 were identified, with most cases showing de novo dominant inheritance. We present the clinical and molecular features of OS in a 10-year-old Iranian boy. He had mutilating palmoplantar keratoderma, periorificial keratotic plaques, diffuse alopecia and constriction bands (pseudoainhum), which led to autoamputation of two digits. TRPV3 was sequenced and a new de novo heterozygous missense mutation, c.2076G>C (p.Trp692Cys), was identified. This case illustrates the characteristic clinical features and complications that can present in OS, and further expands the molecular basis of this genodermatosis.

A new homozygous nonsense mutation in LAMA3A underlying laryngo-onycho-cutaneous syndrome.

Laryngo-onycho-cutaneous (LOC) syndrome is a subtype of autosomal recessive junctional epidermolysis bullosa in which there is prominent skin and mucosal granulation tissue that can lead to delayed wound healing, laryngeal obstruction and blindness. Thus far, all cases are of Punjabi ancestry and have been shown to result from a founder mutation in the LAMA3 gene, notably involving a single nucleotide insertion mutation in exon 39, which is specific to the LAMA3A (designated exon 1 of LAMA3A) and not the LAMA3B1 or LAMA3B2 isoforms. Here, we describe a new pedigree with LOC syndrome. Affected individuals (from Iran) have the characteristic clinicopathological and molecular features of LOC syndrome: prominent granulation tissue (especially affecting the eyes), normal intensity laminin-332 immunostaining at the dermal-epidermal junction, and autosomal recessive mutations in the LAMA3A-specific exon. The pathogenic mutation is a homozygous nonsense mutation, designated p.Gln57X, which just affects the laminin-α3a transcript. These findings therefore expand the molecular basis of LOC syndrome.

Lupus vulgaris at the site of BCG vaccination: report of three cases.

Lupus vulgaris (LV) is a rare complication of the bacille Calmette-Guérin (BCG) vaccination, and about 65 cases of inoculation tuberculosis resembling LV have been reported in the literature. We report three cases of LV, developing many years later at the inoculation site of BCG vaccine. All three cases had a single BCG vaccination, with a LV lesion at or in the vicinity of the vaccination site, a strong positive Mantoux test, noncaseating granuloma histologically, and two of the patients had a positive PCR result for mycobacterial complex. One of the patients had an unusually delayed appearance of the LV lesion, after an interval of about 17 years, and another case was remarkable because of the large size of the lesion (210 x 110 mm).

Comparison of tea and sesame seed oils as two natural antioxidants in a fish oil model system by radical scavenging activity.

Tea and sesame seed oils, two natural antioxidants at 5% and 10% levels, were added to carp (Cyprinus caprio) and common kilka (Clupeonella cultiventris caspia) oils, and the peroxide value and thiobarbituric acid were evaluated after 13 days at 60 degrees C (oven test). The results showed that the antioxidant effects of tea and sesame seed oils in the two aforementioned fish oils were statistically similar. In addition, the radical scavenging activity (RSA) of tea and sesame seed oils (extracted with two solvents:n-hexane and benzene) and their methanolic extract (methanol-soluble phase), ethanolic extract (ethanol-soluble phase) and lipid fraction (non-soluble in methanol or ethanol) were evaluated by the 2,2-diphenyl-1-picrylhydrazyl radical method. The results revealed that the RSA of sesame seed oil was higher than that of tea seed oil (IC(50) = 52 mg and 45 mg, respectively). In addition, among ethanolic and methanolic extracts, the RSA of the ethanolic extract of sesame seed oil (IC(50) = 65 ml and 68 ml in n-hexane and benzene solvents, respectively) and of the ethanolic extract of tea seed oil in benzene solvent (IC(50) = 73 ml) were the highest. Also, the RSA in the lipid fraction, non-soluble phase of sesame seed oil was the highest (IC(50) = 74 mg and 81 mg in n-hexane and benzene solvents, respectively). Furthermore, after 6 days at 60 degrees C the RSA values of tea and sesame seed oils were the same.

Potential therapeutic roles of stem cells in ischemia-reperfusion injury.

Ischemia-reperfusion injury (I/RI), produced by an initial interruption of organ blood flow and its subsequent restoration, contributes significantly to the pathophysiologies of stroke, myocardial infarction, renal I/RI, intestinal I/RI and liver I/RI, which are major causes of disability (including transplant failure) and even mortality. While the restoration of blood flow is required to restore oxygen and nutrient requirements, reperfusion often triggers local and systemic inflammatory responses and subsequently elevate the ischemic insult where the duration of ischemia determines the magnitude of I/RI damage. I/RI increases vascular leakage, changes transcriptional and cell death programs, drives leukocyte entrapment and inflammation and oxidative stress in tissues. Therapeutic approaches which reduce complications associated with I/RI are desperately needed to address the clinical and economic burden created by I/RI. Stem cells (SC) represent ubiquitous and uncommitted cell populations with the ability to self-renew and differentiate into one or more developmental 'fates'. Like immune cells, stem cells can home to and penetrate I/R-injured tissues, where they can differentiate into target tissues and induce trophic paracrine signaling which suppress injury and maintain tissue functions perturbed by ischemia-reperfusion. This review article summarizes the present use and possible protective mechanisms underlying stem cell protection in diverse forms of ischemia-reperfusion.

Effect of gamma irradiation on the stability of anthocyanins and shelf-life of various pomegranate juices.

Food irradiation is a process which exposing food to ionizing radiations and it can improve the safety of food. The pomegranate juice contained considerable anthocyanins and has become a new functional food available for dieting and health. In the present study, the effects of gamma irradiation (0-10kGy) on the stability of anthocyanins and inhibition of microbial growth in pomegranate juice during storage were investigated. Results indicated that the irradiation at all applied doses, significantly reduced total and individual anthocyanins. Moreover, irradiation with higher dosages (3.5-10kGy) had undesirable effect on the total content of anthocyanins. However, irradiation at 2.0kGy had effectively diminished the total bacteria and fungi count and retarding microbial growth during storage. Based on adverse effect of gamma irradiation on ACs content of studied juices, it is not recommended to irradiate pomegranate juice with dosage higher than 2.0kGy.

Application of Tragacanth gum impregnated with Satureja khuzistanica essential oil as a natural coating for enhancement of postharvest quality and shelf life of button mushroom (Agaricus bisporus).

The effect of Tragacanth gum (TG) coating incorporated with 100, 500 and 1000ppm Satureja khuzistanica essential oil (SEO) on the postharvest quality and shelf life of button mushroom (Agaricus bisporus) stored at 4±1°C for 16days was investigated. Weight loss, firmness, browning index (BI), total phenolics, ascorbic acid, microbial and sensory quality were measured. The results indicated that treatment with TG containing SEO (TGSEO) maintained 92.4% tissue firmness, and reduced microorganism counts, such as yeasts and molds and Pseudomonas, compared to uncoated samples. Furthermore, mushrooms treated with TGSEO coating exhibited up to 57.1% decreased in BI, significantly higher levels of total phenolics (85.6%) and ascorbic acid accumulation (71.8%) than control and its efficiency was better than that TG coating alone. Sensory evaluation demonstrated the capability of TGSEO coating for preserving the quality of mushroom during the storage. The results obtained endorse that application of TGSEO coating might be a simple and effective technique for prolonging their postharvest shelf life of mushroom by up to 16days.

Chitosan-cinnamon essential oil nano-formulation: Application as a novel additive for controlled release and shelf life extension of beef patties.

In this study cinnamon essential oil-incorporated chitosan nanoparticles (CEO-CSNPs) were produced by an emulsion-gelation method. Size, zeta potential and polydispersity index of the formed nanoparticles were 235.6nm, 25.1mV and 0.33, respectively. In vitro release evaluation showed an initial burst effect, followed by a slow CEO release during 104h (at pH∼5). Different formulations of patties including control, samples containing free and encapsulated CEO and ascorbic acid were analyzed for physicochemical characteristics, microbial growth and sensorial attributes during 8days of storage at 4°C. Both free and encapsulated CEO decreased the microbial population of patties compared to the control (p<0.05) throughout the experiment. On the 8th day, the best formulations in TBARS test were AA-0.05 (0.05% of ascorbic acid) and 0.1-EN-CEO (0.1% of encapsulated CEO). During storage, the color parameters of the patties containing encapsulated CEO changed slightly whereas color and metmyoglobin content significantly decreased in samples containing free CEO and control. Free CEO had an unfavorable impact on color and odor but the incorporation of encapsulated CEO improved the consumer acceptability. The principle component analysis clearly distinguished 6 formulation groups based on physicochemical characteristics.

Optimization of ultrasonic assisted continuous production of biodiesel using response surface methodology.

This paper evaluates and optimizes the continuous production of biodiesel from waste cooking oil. In this research work, methanol and potassium hydroxide were used as catalyst engaging response surface methodology. For this purpose, the central composite experimental design (CCED), the effects of various factors such as irradiation distance, probe diameter, ultrasonic amplitude, vibration pulse and material flow into the reactor on reaction yield were studied to optimize the process. The results showed that all of the considered parameters affect the reaction efficiency significantly. The optimum combination of the findings include: irradiation distance which was 75 mm, probe diameter of 28 mm, ultrasonic amplitude of 56%, vibration pulse of 62% and flow rate of 50 ml/min that caused the reaction yield of 91.6% and energy consumption of 102.8 W. To verify this optimized combination, three tests were carried out. The results showed an average efficiency of 91.12% and 102.4 W power consumption which is well matched with the model's predictions.

[Epidemiology of cardiovascular disease in the urban population of the town of Pahlavi (North Iran)]. / Epidemiologie de la maladie cardiovasculaire dans la population urbaine de la ville de Pahlvai (nord de l'Iran)

A survey of cardiovascular diseases was carried out in June-August 1973 in the city of Pahlavi, a port in the Caspian area in Iran. A representative sample was taken from persons aged 35 years and older from both sexes. The prevalence of high blood pressure (WHO definition) is 29,7% in men and 31,0% in women of this age group. ECG tracings were prepared and the prevalence of various abnormalities are presented. A considerable proportion of the population have ST segment and T wave abnormalities. Correlation between ECG abnormalities and blood pressure figures has been studied. This study shows that there is a relationship between ECG abnormalities of group 3, 4 and 5 (Minnesota Coding) and the levels of blood pressure.

Comparison of the prevalence of hypertension in the rural and urban areas of Guilan Province, Northern Iran.

Blood pressure levels of three groups of people 35 years and older in the Guilan province, Northern Iran are compared. One group in the city of Bandar Pahlavi (550) another group in the coastal villages near the city (510) and the third in the mountain villages, less than 100 km far from the city (272). This study shows that in both sexes, both systolic and diastolic blood pressure are lowest in the mountain villages, highest in the city and intermediate in the coastal villages. The possible causes of the observed differences are discussed.

Kinetic-spectrophotometric determination of propylthiouracil based on its inhibitory effect on the reduction of neutral red by hypophosphite.

A new kinetic-spectrophotometric method for determination of propylthiouracil (PTU) is described. The proposed method is simple, rapid, inexpensive and sensitive for the determination of PTU in pure and tablet forms. This method is based on the inhibitory effect of propylthiouracil on the palladium(II)-catalyzed reaction between neutral red and hypophosphite ions. The effect of various parameters such as: dye, hypophosphite, and Pd(II) concentrations, pH, ionic strength, and temperature were optimized. Two distinct linear calibration graphs were observed in the ranges 0.006-0.033 (n = 6, r = 0.9991) and 0.033-0.300 ppm (n = 10, r = 0.9980). The variable time method was used. The limit of detection was 0.004 ppm. The proposed method was applied for the determination of propylthiouracil in pure and tablet forms.

Temperature-dependent chemical components accumulation in Hippodamia variegata (Coleoptera: Coccinellidae) during overwintering.

Hippodamia variegata (Goeze) is a widespread predator of aphid and has been reported as the most important naturally occurring enemy of aphids, in many countries. In this study, fluctuating levels of cryoprotectants in nonoverwintering and overwintering coccinellids were assessed in field and natural overwintering sites in Hamadan, Iran, from September of 2010 to June of 2011. Moreover, accumulations of polyols were investigated in response to subzero temperatures. Results demonstrated that concentrations of cryoprotectants changed during the study period under natural conditions. Large amounts of metabolic reserves, in the form of glycogen, accumulated before overwintering. Glycogen began to breakdown but polyols accumulated when ambient temperature decreased from October to December. Overwintering coccinellids displayed a threefold increase in levels of trehalose and a fivefold increase in levels of glucose and total cryoprotectants, but there was even a 45-fold increase in myo-inositol content when subzero temperatures were experienced. It was suggested that myo-inositol and glucose are the major cryoprotectants components in this species. In tests for cold response, the optimal temperature for polyol synthesis seems to be below -3°C. From November to February, all the collected coccinellids that had been kept at 10°C in the laboratory were dead after 30 d, indicating that high temperatures were not favorable for overwintering of this population. Moreover, exposure to high temperatures resulted in rapid catabolism of sugar alcohols. This result demonstrated significant correlation between ambient temperature decrease and accumulation of polyols, suggesting that low temperatures are responsible for polyols synthesis in this species.

Seasonal patterns of cold hardiness and cryoprotectant profiles in Brevicoryne brassicae (Hemiptera: Aphididae).

The cabbage aphid Brevicoryne brassicae (L.) (Hemiptera: Aphididae), one of the most important pests of cruciferous crops, overwinters as nymph and adult on winter cabbages in Tehran, Iran, and forms large populations on these plants during winter. To determine the cold hardiness of this aphid, adults and first-instar nymphs were collected monthly from ornamental cabbages planted in the field from October of 2009 to May of 2010. Supercooling points and LT(50) values (the temperature at which 50% of population died after 24-h exposure to subzero temperatures) were investigated. To elucidate the relation between cold hardiness and cryoprotectants, sugars and polyols were determined by high performance liquid chromatography. Changes of mean supercooling point were small during sampling dates. LT(50) values of adults decreased gradually from -6.9°C in October to -12.2°C in January, showing the increase in cold hardiness during cold seasons. Moreover, nymphs showed similar trends in LT(50) values. Glucose, mannitol, myo-inositol, and trehalose were identified in overwintering aphids. Total amount of the cryoprotectants increased to the highest (72.8 ± 9.2 µmol/g fresh weight) in January. There was a significant correlation between LT(50) values and the cryoprotectant contents, suggesting their important role in aphid cold hardiness. Results have shown that B. brassicae is sufficiently cold tolerant to survive mild winter temperatures and is able to form large populations on winter cabbages, causing it to be considered a threat to early season crops, especially oilseed rape.

Obstetrical brachial plexus palsy: electrodiagnostical study and functional outcome.

Obstetrical Brachial Plexus Palsy (OBPP) is a complication of difficult delivery and resulted from excessive traction on the brachial plexus during delivery. Erb palsy, klumpke paralysis and panplexus palsy reported in 46, 0.6 and 20% of patients, respectively. Unilateral injury is more common than bilateral injury. Risk factors include macrosomia, multiparity, prior delivery of a child with OBPP, breech delivery shoulder dystocia, vacium and forceps assisted delivery and excessive maternal weight gain. The recovery rate is usually reported to be between 80 and 90%. We evaluated 42 children with OBPP. Out of them, we could follow only 28 cases during two years. Poor to moderate recovery occurred in 13 cases. Good to complete (expected) recovery occurred in 15 cases. Most of the patients were females. Right side palsy was more prevalent than left side palsy. Vaginal delivery without forceps was the most mode of delivery. Vertex was the most common presentation. Most of the patients were term. The mean weight of the birth was 3.8 kg. Erb palsy and pan-plexus palsy consisted of 71.4 and 28.6% of lesions. In patients with Erb palsy, there were preganglionic palsy in 3 (15.8%) and postganglionic palsy in 16 (84.2%) cases, while all the patients with panplexus palsy had postganglionic palsy. All patients with complete recovery (9 of 15) had Erb palsy and postganglionic lesion. Erb palsy was present in 71.4% and panplexus palsy was present in 28.6% of cases. Also, 23.8% of cases had preganglionic and 76.2% of cases had postganglionic injures.

Optimization of the omega-3 extraction as a functional food from flaxseed.

The fatty acid content, total lipid, refractive index, peroxide, iodine, acid and saponification values of Iranian linseed oil (Linum usitatissimum) were studied. For optimization of extraction conditions, this oil was extracted by solvents (petroleum benzene and methanol-water-petroleum benzene) in 1:2, 1:3 and 1:4 ratios at 2, 5 and 8 h. Then its fatty acid content, omega-3 content and extraction yield were determined. According to the statistical analysis, petroleum benzene in a ratio of 1:3 at 5 h was chosen for the higher fatty acid, extraction yield, and economical feasibility. For preservation of omega-3 ingredients, oil with specified characters containing 46.8% omega-3 was kept under a nitrogen atmosphere at -30 degrees C during 0, 7, 30, 60 and 90 days and its peroxide value was determined. Statistical analysis showed a significant difference in the average amount of peroxide value only on the first 7 days of storage, and its increase (8.30%) conformed to the international standard.

Effect of cultivar on chemical composition of some Iranian spinach.

This research was carried out to determine fatty acids, phenolic compounds, oxalic acid, protein, fat, total fiber and mineral elements of Iranian spinach cultivars (Varamin 1, Varamin 2, Varamin 3, Zabol, Kashan, Qom and Zanjan). Results showed that cultivar Zabol had the highest protein (2.59%). The highest fat percentage, phenolic compounds, P, linoleic acid (18:2) and the lowest oxalic acid were found in cultivar Kashan. Cultivar Varamin 2 had the highest total fiber (2.22%). Dry matter, K, Ca, Cu and Zn contents of cultivar Zanjan were higher than the other cultivars. Cultivar Qom had the highest amounts of Mg and Fe. Cultivar Varamin 3 had the highest Na (93.8 mg/100 g fw). Cultivars Qom and Varamin l had the highest percentage of linolenic acid (18:3). Present results for chemical composition of studied cultivars, were similar to values reported by other researchers.

Congenital anomalies in Iran: a cross-sectional study on 1574 cases in the North-West of country.

BACKGROUND: At least one congenital anomaly is present in between 1% and 6% of all infants throughout the world. The aim of this study was to document some epidemiological features of congenital anomalies in the North-West of Iran. METHODS: The study cases (n = 1574) comprised all births registered/notified to three university-hospitals of Tabriz University of Medical Sciences, Iran, from 2000 to 2004. RESULTS: Total prevalence of congenital anomalies was 165.5 per 10 000 births [95% confidence interval (CI): 157-174]. Genito-urinary tract and kidney defects, anomalies of nervous system and limb anomalies accounted proportionally for more than 65% of anomalies in the region. The total prevalence of congenital anomalies in the study area increased from 104.6 per 10 000 births in 2000 (95% CI: 90-119) to 170.1 per 10 000 births in 2004 (95% CI: 152-189). CONCLUSIONS: It is concluded that the data from this cross-sectional study of congenital anomalies in the North-West of Iran may be used as the baseline information to establish a population-based registry of birth defects in the area for health care and research purposes.

Evaluation of rural primary health care services in Iran: report on vital statistics in West Azarbaijan.

Iranian indigenous front-line health workers (FLHW) were locally trained to deliver environmental and primary health care services to mothers and children. After two years, there was a significant decline in crude birth and death rates and in infant and 1-5 year mortality rates in the test area as compared to a control area. One out of four encounters was referred to a health center physician, the remainder being handled by the FLHWs themselves. (Am J Public Health 1981;71:739-742.)

Evaluation of cellular and biochemical parameters of blood and peritoneal fluid following exploratory laparotomy in the goat.

To evaluate the effects of exploratory laparotomy on cellular and biochemical parameters of blood and peritoneal fluid, an experiment was conducted using 10 Iranian cross-bred male goats. Approximately 10 ml of blood and 1-1.5 ml of peritoneal fluid were collected from all animals prior to operation for estimation of control values. Exploratory laparotomy was performed under local analgesia. Blood and peritoneal fluid samples were collected at 24, 48, 72 and 96 h after exploratory laparotomy. The results revealed that after exploratory laparotomy, the number of white blood cells and the percentage and absolute number of neutrophils and band neutrophils significantly increased (P < 0.05). However, the percentage of lymphocytes decreased significantly (P < 0.05). The concentrations of blood urea nitrogen significantly increased (P < 0.05). Furthermore, following the operation, the percentage and absolute number of neutrophils in the peritoneal fluid significantly increased (P < 0.05). In contrast, the percentage of lymphocytes in the peritoneal fluid decreased significantly (P < 0.05). The concentration of protein in the peritoneal fluid increased significantly (P < 0.05).