Negar Khalighi; Rahim Vakili; Mohammad Ali kiani; Seyed Ali Jafari; Hamid Reza Kianifar
Abstract
Introduction: Cystic fibrosis is one of the most common autosomal recessive diseases that affects sweat glands and mucosa. CF is a hereditary disease with annual incidence of about 2500 new cases in United Kingdom. Insulin-like growth factor-1 (IGF-1) and insulin-like growth factor binding protein-3 ...
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Introduction: Cystic fibrosis is one of the most common autosomal recessive diseases that affects sweat glands and mucosa. CF is a hereditary disease with annual incidence of about 2500 new cases in United Kingdom. Insulin-like growth factor-1 (IGF-1) and insulin-like growth factor binding protein-3 levels decrease in CF. The aim of this study was to assess the role of growth peptides in patients with CF. Method: We searched PubMed, Google scholar, IranMedex, and Scientific Information Database (SID) in September 2012 to April 2014. We included clinical studies with available abstracts and full texts that were in English or Persian languages. Manual searching was conducted within the reference lists of articles. Two reviewers independently applied eligibility criteria, assessed quality, and extracted data.Result: The earliest study was published in 1997 and the most recent one was in 2014. Study participants were adults in 3 studies (20%) and 12 studies (80%) were conducted in children. Patients with CF have lower levels of IGF-1 and there is a significant correlation between IGF-1 levels and growth index in patients with CF.Conclusions: IGF-1 decreases in children with CF and might be the cause of poor growth and low body mass index in these children.
Leili Zarifmahmoudi; Maryam Khalesi; Ramin Sadeghi; Seyed Ali Jafari; Mohammad Ali Kiani; Hamidreza Kianifar
Abstract
Introduction: Hepatopulmonary syndrome (HPS) is known as a chronic liver disease associated with severe pulmonary deoxygenation due to intrapulmonary vascular vasodilation. Although liver transplantation is accepted as a main treatment of HPS, identifying effective drugs for recovery of HPS can be effective ...
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Introduction: Hepatopulmonary syndrome (HPS) is known as a chronic liver disease associated with severe pulmonary deoxygenation due to intrapulmonary vascular vasodilation. Although liver transplantation is accepted as a main treatment of HPS, identifying effective drugs for recovery of HPS can be effective in postponing the transplantation and decreasing the mortality rate of patients before the transplantation. In this study we briefly reviewed the pathogenesis of HPS and also systematically reviewed the current pharmacological treatment of HPS. Method: Pubmed, Scopus, and Google scholar were searched for the relevant English language clinical and experimental articles about the medications used in the treatment of HPS. Results: A total of 38 articles were included in this study which mostly resulted in decreasing NOS expression, NO production, endothelin-1 activation, intrapulmonary angiogenesis and increasing oxygenation.Conclusion: Various drugs have been proposed in treatment of HPS but more large controlled trial studies, is necessary to determine the exact efficacy of each drugs for HPS recovery.