The Schnitzler syndrome

The Schnitzler syndrome is a rare and underdiagnosed entity which is considered today as being a paradigm of an acquired/late onset auto-inflammatory disease. It associates a chronic urticarial skin rash, corresponding from the clinico-pathological viewpoint to a neutrophilic urticarial dermatosis, a monoclonal IgM component and at least 2 of the following signs: fever, joint and/or bone pain, enlarged lymph nodes, spleen and/or liver, increased ESR, increased neutrophil count, abnormal bone imaging findings. It is a chronic disease with only one known case of spontaneous remission. Except of the severe alteration of quality of life related mainly to the rash, fever and pain, complications include severe inflammatory anemia and AA amyloidosis. About 20% of patients will develop a lymphoproliferative disorder, mainly Waldenström disease and lymphoma, a percentage close to other patients with IgM MGUS. It was exceedingly difficult to treat patients with this syndrome until the IL-1 receptor antagonist anakinra became available. Anakinra allows a complete control of all signs within hours after the first injection, but patients need continuous treatment with daily injections

Collagen Dysregulation in the Dermis of the Sagg/+ Mouse: A Loose Skin Model

The Sagg/+ mouse is an ethylnitrosourea-derived mutant with a dermal phenotype similar to some of the subtypes of Ehlers-Danlos syndrome (EDS) and cutis laxa. The dermis of the Sagg/+ mouse has less dense and more disorganized collagen fibers compared to controls. The size of extracted Type I dermal collagen was the same as that observed in normal skin; however, more collagen could be extracted from Sagg/ + skin, which also showed decreased collagen content and decreased steady-state levels of α1(I), α2(I), α1(V), and α2(V) procollagen mRNAs. The biomechanical properties of Sagg/+ skin were significantly decreased relative to normal skin. However, there were no significant differences in the quantities of the major collagen cross-links, that is, dehydrohydroxylysinonorleucine and dehydrohistidinohydroxymerodesmosine between Sagg/+ and normal skin. Electron microscopic evaluation of Sagg/+ skin indicated that the mutation interferes with the proper formation of collagen fibrils and the data are consistent with a mutation in Type V collagen leading to haploinsufficiency with the formation of two sub-populations of collagen fibrils, one normal and one with irregular shape and a larger diameter. Further study of this novel mutation will allow the identification of new mechanisms involved in the regulation of normal and pathologic collagen gene expression

Urban vs. rural patients. Differences in stage and overall survival among patients treated surgically for lung cancer

Introduction. Besides the undoubted influence of risk factors on morbidity and survival time, there are also other environmental factors, such as awareness of the prevalence of risk factors and the availability of modern diagnosis and treatment methods. Objective. To evaluate differences in lung cancer 5-year overall survival rates between urban and rural patients hospitalized in the Department of Thoracic Surgery of the Medical University in Lublin, Poland, and possible influence of several risk factors on these rates. Materials and methods. The analysis was based on 125 lung cancer patients who underwent surgical procedures in years 2006-2007 and who agreed to take part in the survey. The study aimed at recognition of the health situation and selected demographic traits of people who had been treated surgically for lung cancer. The differences were evaluated between rural and urban inhabitants in gender, age, lung function, smoking habits, exposure to risk factors at work, family history of cancer, staging of the disease, histological type of cancer, post-surgical treatment, and their possible influence on overall survival. Results. The results showed that the only noted differences between urban and rural population were in tobacco smoking and lung function. Survival rates were very similar and did not differ from the European average. Conclusions. The assumption that Polish rural patients are presenting with later cancer stages at the time of diagnosis, and have worse chances for survival, has become invalid in modern times

Air pollution: how many cigarettes does each Pole ‘smoke’ every year and how does it influence health, with special respect to lung cancer?

Introduction. Air pollution is one of the most important issues of our times. Air quality assessment is based on the measurement of the concentration of substances formed during the combustion process and micro-particles suspended in the air in the form of an aerosol. Microscopic atmospheric particulate matters (PM) 2.5 and 10 are mixtures of organic and inorganic pollutants smaller than 2.5 and 10 μm, respectively. They are the main cause of negative phenomena in the earth’s atmosphere of Earth and human health, especially on the respiratory and cardiovascular systems. Particulates have the ability to cause permanent mutations of tissue, leading to neoplasms and even premature deaths. Nitrogen dioxide (NO2) is one of the main pollutants which arises mainly during the burning of fossil fuels. Based on numerous scientific researches, it has been proved that long-term exposure to NO2 could increase morbidity of cancer due to inflammatory processes increasing abnormal mutations. Materials and method. Data available in the Polish National Cancer Registry, Chief Inspectorate for Environmental Protection and Map of Health Needs in the Field of Oncology for Poland, WHO Air Quality Guidelines 2005 were analyzed. Air pollution was also evaluated: PM2.5, PM10, NO2, and compared with lung cancer morbidity. Results and conclusions. Based on the available data and literature, it can be concluded that in 2009–2017, on average, each Pole smoked ten cigarettes a day +/- 2. Therefore, it can be estimated that after 60 years everyone had 30 package-years of smoking, leading to a high risk of lung cancer and other smoking related diseases. Additionally air quality in Poland is not satisfactory, exceeding the standards presented in the WHO Guidelines 2005. It can be assumed that this may translate into an additional, independent continuous increase in morbidity and mortality dependent on smoking

Abstract quality assessment of articles from the Annales de Dermatologie

Introduction. Article's abstracts are an important part of the publication, widely available in electronic databases. We assessed the quality of abstracts in the Annales de Dermotologie. The main objective was to compare abstract quality in 3 periods in the past decade. in this journal, structured abstracts are required since 1993. The secondary objective was to compare structured and non stuctured abstract quality

Reasons for delay in diagnosis and treatment of lung cancer among patients in Lublin Voivodeship who were consulted in Thoracic Surgery Department

Introduction: Despite the progress which has been made in the diagnosis and treatment of lung cancer, it is still one of the main causes of death in both men and women. The introduction of new therapeutic modalities did not improve the 5-year survival results of lung cancer patients. The Lublin Voivodeship is a sparsely-inhabited area with little urbanization and a population of about 2.2 million people. Only 46.8% of its citizens live in the towns, while the national average is 61.9%. Objectives: The aim of the study was to compare the differences in the periods of time and reasons for delay in diagnosis and initiation of treatment of lung cancer among patients who are inhabitants of the rural and urban regions of Lublin Voivodeship, and who were consulted in Thoracic Surgery Department. Materials and methods: 300 lung cancer patients who were consulted in the Thoracic Surgery Outpatient Clinic or who were hospitalized in the Department of Thoracic Surgery in the period between 2 January 2010 – 7 January 2011 were included in the study. Delays were calculated for two periods of time: 1) time from the first signs of the disease to the first medical examination; 2) the time from the first visit to a doctor to the start of treatment, or disqualification from the causative treatment. The time of the first delay for the urban and rural populations was similar and ranged from 2-37 weeks and 2-23 weeks, respectively. Lack of time and disregard of signs of disease were the most commonly reasons given for the first delay among rural residents. The urban population indicated fear and lack of time as the main reasons of delay. Assessment of the second reason for delay was possible thanks to a specially designed research protocol which gathered the main reasons of delay in several subgroups that enabled their statistical evaluation. The length of second period was similar for both populations. Results: There were no significant differences in the length of the time of delay between the two assessed groups. In both groups, delays dependent on poor healthcare access were similar. Among rural inhabitants, the most often reasons of delay were waiting for hospital admission and re-bronchoscopy. In the urban population, the most common reasons for delay were waiting for hospitalization and CT procedure. Conclusions: The results of the presented research allowed the following conclusions to be drawn: between the two assessed groups there were no differences in the length of the time of delay; 2) delays in diagnosis and treatment were too long for the patients and could affect the severity of the disease and final prognosis; 3) there is a need for intensification of information campaigns on lung cancer in order to reduce the delays dependent on patients, and to improve the cooperation of family doctors, pulmonologists, thoracic surgeons and oncologists

Rural–urban residence and cancer survival in high‐income countries: A systematic review

There is some evidence that place of residence is associated with cancer survival, but the findings are inconsistent, and the underlying mechanisms by which residential location might affect survival are not well understood. We conducted a systematic review of observational studies investigating the association of rural versus urban residence with cancer survival in high-income countries. We searched the Ovid Medline, EMBASE, and CINAHL (Cumulative Index to Nursing and Allied Health Literature) databases up to May 31, 2016. Forty-five studies published between 1984 and 2016 were included. We extracted unadjusted and adjusted relative risk estimates with the corresponding 95% confidence intervals. Most studies reported worse survival for cancer patients living in rural areas than those in urban regions. The most consistent evidence, observed across several studies, was for colorectal, lung, and prostate cancer. Of the included studies, 18 did not account for socio-economic position. Lower survival for more disadvantaged patients is well documented; therefore, it could be beneficial for future research to take socio-economic factors into consideration when assessing rural/urban differences in cancer survival. Some studies cited differential stage at diagnosis and treatment modalities as major contributing factors to regional inequalities in cancer survival. Further research is needed to disentangle the mediating effects of these factors, which may help to establish effective interventions to improve survival for patients living outside major cities