Unique presentations and chronic complications in adult cystic fibrosis: do they teach us anything about CFTR?
Author(s)Boyle Michael P
Keywordscongenital bilateral absence of the vas deferens
cystic fibrosis transmembrane conductance regulator
Diseases of the respiratory system
Specialties of internal medicine
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Abstract<p>Abstract</p> <p>The increase in numbers of adults with cystic fibrosis (CF) has allowed us to identify previously unrecognized chronic complications of CF, as well as appreciate unique presentations of cystic fibrosis-related diseases. Do these chronic complications and unique presentations provide us with new insight into cystic fibrosis transmembrane conductance regulator (CFTR) function? Current data suggest that the 'chronic complications' reveal mainly the effect of a long-term absence of previously recognized CFTR functions. In contrast, the 'unique presentations' provide new insight into the role of CFTR in different tissues.</p>
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The Effect of Using Sewerage System on Incidence of Acute Diarrhoea on Children under Five Years Old in Bandar-Anzali City in Guilan Province in 2009-Phase IMorteza Fallah-Karkan; Hannan Ebrahimi; Zahra Akbarian; Seyyede Zohreh Banihashemi; Maryam Rostamnejad; Matin Daneshyari; Elahe Sayyarifard2; Kaveh Marzbani; Haleh Ahmadnia; Javad Moazzami-Sahzabi; et al. (Iranian Society for Infectious Diseases and Tropical Medicine, 2013-07-01)Backgroun and objective:The scarce water resources and Poor quality drinking water with the lack of a proper sewage collection and disposal system and increasing management problems due to rapid population growth made Islamic Republic of Iran to pay priority attention to the water and sewerage problems of districts in its five-year development plan. In this regard, water and sewerage project in Bandar Anzali became a priority by Guilan province Urban Water and Sewerage Company with the government partnership and World Bank financial support. The objective of this study is to complete the first phase of a two-phase study to determine the impact of using an urban sewerage system on acute Diarrhoea in children under five years of age in Bandar-Anzali in the Guilan province.Materails and methods: The study is a concurrent control before and after field trial which is carried out in two stages: before (phase I) and after (phase II). Sampling for phase I was performed in the middle month of each season, November and February 2009 and May and August 2010. The incidence of acute Diarrhoea was measured with the participation of 2400 mothers of children under 5 years old in the city of Bandar-Anzali in two groups: the intervention group (inside the sewerage system project perimeters) and the control group (outside the project perimeters). Data were collected by local female general practitioners and medical students in each city, under supervision of the community medicine department and by door-to-door interview with mothers. Data were analyzed using the SPSS 11.5 software package and the Pearson Chi-Square was used to compare qualitative variables between groups, and the t-test and One-Way ANOVA was used to compare quantitative variables.Results: The annual incidence of acute diarrhoea in children under five years old was 11.9%. The seasonal pattern of acut diarrheoa incidence was 12.6% in May, 13.3 in August, 11.5% in November, and 10.3% in February respectively. The highest incidence of acute diarrhoea was seen in children from 2 to 5 years of age.Conclusion: The study showed the incidence of acute diarrhoea on children under five years old in Bandar-Anzali city is moderate and it follows a seasonal pattern. The highest rate was seen in summer and the lowest rate in winter. It is expected to decrease with the initiation of the sewerage system program.
Clinical Features, Presence of Human Herpesvirus-8 and Treatment Results in Classic Kaposi SarcomaÖzlem Su; Nahide Onsun; Hande Arda; Ömer Ümmetoğlu; Ayşe Pekdemir (Galenos Yayincilik, 2008-12-01)Background and Design: Classic Kaposi sarcoma (KS) occurs predominantly among the elderly, with Jews, Italians and Greeks. Classic KS has been seen relatively frequently in Turkey. Our aim was to evaluate the demographic, clinical features of Kaposi sarcoma and etiopathological role of human herpesvirus-8 (HHV-8). Treatment results of 18 classic Kaposi’s sarcoma were also concluded.Material and Method: Eighteen cases of classic Kaposi sarcoma diagnosed as clinically and histopathologically between January 2001 and August 2008 in our dermatology department were taken to this study. Demographic, clinical features and treatment results were reviewed retrospectively in all patients. HHV-8 was investigated in the lesional skin of 7 patients.Results: A male/female ratio of 2/1 was found. Mean age at diagnosis was 67.2 (37-94) years. Bilaterally lower extremities were involved in 15 patients (83.3%), the trunk was involved in 3 patients (16.6%). Plaques and nodules were the common type of lesions (66.6% and 55.5%). Nine patients had no symptoms (50%). Edema was the most common symptom (38.8%). A second primary malignancy was found in 2 patients (11.1%). HHV-8 was detected in 6 of the 7 patients(85.7%). Majority of the patients were treated with interferon alfa (subcutaneously) and cryotherapy as a monotherapy or a combination therapy. Imiquimod was the second agent in combined treatment (27.7%). Conclusion: We suggest that interferon alfa and imiquimod can be used as first line therapy agents with their antiviral and immunmodulatuar features in the treatment of KKS. (Turkderm 2008; 42: 122-6)
Medicina y Seguridad del Trabajo se integra en HINARI Programa de Acceso a la Investigación en Salud de la Organización Mundial de la Salud (OMS) Medicina y Seguridad del Trabajo has been integrated into HINARI Access to Research in Health Programme of the World Health Organization (WHO)Gaby Caro-Salazar; Jorge Veiga-Cabo (Escuela Nacional de Medicina del Trabajo, 2013-03-01)HINARI Programa de Acceso a la Investigación en Salud liderado por la OMS junto con las principales editoriales asociadas facilita acceso a información en ciencias de la salud y biomedicina a más de 100 países en vías de desarrollo de forma gratuita o a bajo costo. Constituye una de las colecciones más extensas de literatura científica que actualmente existen, con acceso a más de 29.000 recursos de información en 30 idiomas diferentes. Además cuenta con programa docente a distancia que permite la capacitación en diferentes herramientas relacionadas con el acceso, utilización y gestión de la información científica. Entre los asociados se cuenta con la participación de instituciones como la Biblioteca Nacional de Medicina de los Estados Unidos, el Centro de Capacitación y Divulgación de Información para África (ITOCA) y las Universidad de Yale y Cornell (EEUU). La revista de Medicina y Seguridad del Trabajo se acaba de incorporar recientemente al programa HINARI de la OMS.<br>HINARI Access to Research in Health Programme led by the World Health Organization enables access to information related to health sciences and biomedicine to over 100 developing countries, free or low cost. It is one of the most extensive collections of current scientific literature, accessing over 29,000 information resources in 30 different languages. It also has distance learning courses that allows training in different tools related to access, use and management of scientific information. Among the associated partners there are institutions like the United States National Library of Medicine, Information Training and Outreach Center for Africa (ITOCA) and Yale and Cornell Universities. Medicina y Seguridad del Trabajo journal has recently joined the HINARI Programme.