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Approach to Hereditary Storage Diseases in Patients with Hepatosplenomegaly
Get PDFHow to Cite This Article: Razzaghy Azar M. Approach to Hereditary Storage Diseases in Patients with Hepatosplenomegaly. Iran J Child Neurol. 2015 Autumn;9:4(Suppl.1): 18-19. Pls see pdf.
Assessment of vitamin D status in healthy children and adolescents living in Tehran and its relation to iPTH, gender, weight and height
No full textThis study was conducted to determine the frequency of vitamin D deficiency and its correlation with different factors. Three hundred and thirteen healthy children and adolescents (192 females and 121 males aged 818 years, mean ± SD, 12.7 ± 2.3 years) were enrolled, and measurements of serum 25-hydroxyvitamin D 25(OH)D (using EIA) and intact parathyroid hormone (iPTH) (using immunoradiometric assay (IRMA)) were conducted. The grades of vitamin D status were defined according to blood level of 25(OH)D as follows: severely deficient 75 nmol/L in order to prevent PTH rising. © 2010 Informa UK, Ltd
A novel treatment for height growth in patients with growth hormone insensitivity syndrome by cyproheptadine hydrochloride
No full textBackground: Cyproheptadine HCl (CyproH) is an appetite-stimulating drug and while it was prescribed for a patient with growth hormone insensitivity syndrome (GHIS) for increasing appetite, his height growth was surprisingly increased. Therefore, the aim of this study was to investigate the effect of CyproH on growth parameters of the patients with GHIS. Patients and design: Twenty patients were enrolled in two prospective cohorts at two different times. Fifteen cases were observed for 1.17 ± 1.3 years without treatment (observation period, OP). Then, CyproH was administered for 2.2 ± 2.7 years (treatment period, TP), and growth parameters were compared within these two periods. Five patients who did not receive any treatment for 1-8.24 years (4 ± 2.9) were the control group. Results: Height velocity (HV) increased from 1.88 ± 0.7 to 6.1 ± 0.8 cm/year and HV-SDS reached from �4.5 ± 0.74 to �0.21 ± 1.2 in OP and TP, respectively (P <.001), whereas HV and HV-SDS were 2.2 ± 1.1 cm/yr and �4.2 ± 1.2, respectively, in controls (P <.001). Height SDS was �7.0 ± 1.7 and increased to �6 ± 2.2 after treatment (P =.002). Gain in height was 2.3 ± 0.6 SDS in 5 patients who were treated for 5.4 ± 2.8 years. BMI-SDS was not significantly changed within two time periods and also in cases and controls. Conclusion: CyproH caused height growth in the patients with GHIS, and therefore, this treatment can be considered as an alternative option to IGF-I injection. © 2018 John Wiley & Sons Lt
Pheochromocytoma and renal agenesis
Get PDFPheochromocytomas are cathecolamine producing tumor arising from chromaffin cells of adrenal medulla and extra adrenal tissues. Renal anomalies have been reported rarely in this disease. Here we report a rare case of pheochromocytoma associated with renal agenesis. Patient was a boy presented with headache and hypertension. In radiologic examinations, an adrenal mass was detected in the left side, with no renal tissue on that side. It was reported an adrenal pheochromocytoma in histopathology. Renal agenesis confirmed during surgery. All of the patients symptoms alleviated after the resection of tumor. Considering association of renal abnormalities with pheochromocytoma, it is recommended to perform genetic analysis and kidney evaluation in cases with MEN type 2 or each of its components. © 2008 Tehran University of Medical Sciences. All rights reserved
Going Beyond Counting First Authors in Author Co-citation Analysis
Get PDFThe present study examines one of the fundamental aspects of author co-citation analysis (ACA) - the way co-citation
counts are defined. Co-citation counting provides the data on which all subsequent statistical analyses and mappings
are based, and we compare ACA results based on two different types of co-citation counting - the traditional type that
only counts the first one among a cited work's authors on the one hand and a non-traditional type that takes into
account the first 5 authors of a cited work on the other hand. Results indicate that the picture produced through this non-traditional author co-citation counting contains more coherent author groups and is therefore considerably clearer. However, this picture represents fewer specialties in the research field being studied than that produced through the traditional first-author co-citation counting when the same number of top-ranked authors is selected and analyzed. Reasons for these effects are discussed
Variations on the Author
Get PDF“Variations on the Author” discusses two of Eduardo Coutinho’s recent films (Um Dia na Vida, from 2010, and Últimas Conversas, posthumously released in 2015) and their contribution to the general question of documentary authorship. The director’s filmography is characterized by a consistent yet self-effacing form of authorial self-inscription: Coutinho often features as an interviewer that rather than express opinions propels discourses; an interviewer that is good at listening. This mode of self-inscription characterizes him as an author who is not expressive but who is nonetheless markedly present on the screen. In Um Dia na Vida, however, Coutinho is completely absent form the image, while Últimas Conversas, on the contrary, includes a confessional prologue that moves the director from the margins to the center of his films. This article examines the ways in which these works stand out in the filmography of a director who offers new insights into the notion of cinematic authorship
Appropriate Similarity Measures for Author Cocitation Analysis
Get PDFWe provide a number of new insights into the methodological discussion about author cocitation analysis. We first argue that the use of the Pearson correlation for measuring the similarity between authors’ cocitation profiles is not very satisfactory. We then discuss what kind of similarity measures may be used as an alternative to the Pearson correlation. We consider three similarity measures in particular. One is the well-known cosine. The other two similarity measures have not been used before in the bibliometric literature. Finally, we show by means of an example that our findings have a high practical relevance.information science;Pearson correlation;cosine;similarity measure;author cocitation analysis
Erythrocyte susceptibility to oxidative stress and antioxidant status in patients with type 1 diabetes
Get PDFIn this study, malondialdehyde (MDA) level as an index of erythrocyte susceptibility to oxidative stress and antioxidant defense system (glutathione level (GSH), glutathione peroxidase enzyme activity (GPx) in erythrocytes and ferric reducing ability of plasma (FRAP) as the total plasma antioxidant capacity were measured in 35 patients with type 1 diabetes and 28 age and sex-matched normal subjects. MDA level was significantly elevated in diabetic patients (650.9 ± 144.3 nmol/g versus 476.5 ± 138.5 nmol/g Hb, P < 0.001). The level of MDA was positively correlated with duration of diabetes (r = 0.29, P < 0.05) and HbA1C (r = 0.39, P < 0.05) and negatively with FRAP (r = -0.3, P < 0.05). The level of GSH and FRAP were lower in patients than controls (7.05 ± 1.6 μmol/g versus 8.24 ± 0.9 μmol/g Hb, and 389.05 ± 82.3 μmol/l versus 520.4 ± 124.1 μmol/l, respectively, P < 0.001). GPx activity was not significantly different between the two groups. GSH and FRAP were negatively correlated with HbA1C (r = -0.334, P < 0.01 and r = -0.5, P < 0.01, respectively). In conclusion, there seems to be an increased susceptibility to oxidative stress and decreased antioxidant defense in patients with type 1 diabetes, which may be due to poor glycemic control. © 2007 Elsevier Ireland Ltd. All rights reserved
Visfatin in obese children and adolescents and its association with insulin resistance and metabolic syndrome
No full textBackground: Visfatin, also known as nicotinamide phosphoribosyltransferase, is an adipokine that has been implicated in obesity, insulin resistance (IR) and diabetes mellitus. Since obesity profoundly affects serum lipids, insulin, and glucose metabolism, the aim of this study was to evaluate the relationships between visfatin and metabolic parameters in childhood obesity. Methods: A total of 73 Iranian children and adolescents (31 controls; 42 obese), between the ages of 7 and 16 years, were selected and clinically evaluated. Serum visfatin, leptin, insulin and adiponectin were measured using ELISA, and insulin resistance was calculated by the Homeostasis Model of Assessment of Insulin Resistance (HOMA-IR). Fasting plasma glucose (FPG), triglyceride (TG), total cholesterol (TC), LDL-C and HDL-C were also measured. Metabolic syndrome (MetS) was determined according to IDF criteria. Results: Obese subjects presented significantly higher levels of insulin, LDL-C, HOMA-IR, and leptin and lower levels of adiponectin. Serum Visfatin was higher in obese children than in the control children, and it was significantly higher in obese children with MetS or IR, compared with obese children without MetS or IR. Visfatin levels showed positive correlations with FPG, insulin, and HOMA-IR, in obese subjects and a negative correlation with adiponectin, but no correlation with leptin. Adiponectin levels were correlated with HDL-C and Insulin levels in obese subjects. Leptin levels were correlated with Body mass index (BMI) but not with metabolic parameters. Conclusions: Visfatin is increased in obese children and adolescents, and has a more prominent association with IR and MetS parameters, compared with leptin and adiponectin. © 2015 Informa Healthcare
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