Quality of life assessment in core outcome sets: A position statement of the EADV Task Force on Quality of Life and Patient Oriented Outcomes.

Core outcome sets (COSs) are an agreed standardized collection of outcomes that should be measured and reported in all clinical trials for a specific clinical condition. Tsekhe aim of our position paper by the European Academy of Dermatology and Venereology (EADV) Task Force on Quality of Life and Patient Oriented Outcomes was to identify the challenges and Patient Oriented Outcomes advantages in the development of COSs within dermatological QoL research. Twelve EADV Task Force multidisciplinary team members presented a total of 101 items (51 advantages and 50 disadvantages). All participants considered that COS are beneficial for comparison between different studies, treatments, dermatological diseases, geographical location and ethnicities. In conclusion, the EADV Task Force on Quality of Life and Patient Oriented Outcomes has recognized the primacy of advantages of COS and deliberated that the disadvantages in COS are related to development process and use of COS.

A case-control study of risk factors associated with Zoon balanitis in men.

BACKGROUND: Zoon balanitis (ZB) is a chronic inflammatory benign mucositis. Its etiopathogenesis still remains hypothetical and speculative. OBJECTIVES: To determine risk factors associated with genital ZB in men. METHODS: This is a case-control study including 30 patients diagnosed with ZB and 54 patients with dermatological diseases other than ZB enrolled in the Dermatological Clinic of the University of Padova, Italy, from September 2015 to June 2018. Univariate and multivariate logistic regression analyses were used for analysis of data collected. RESULTS: According to multivariate logistic regression analysis, risk factors for ZB were as follows: the mean daily cigarettes consumption (OR 1.065; 95% CI 1.8-11.4; P = 0.006) and the number of weekly foreskin retractions (OR 0.847; 95% CI 5.5-24.1; P = 0.003). There were no statistically significant differences between cases and controls according to age, presence of circumcision as well in number of sexual partners. CONCLUSIONS: To our knowledge, this is the first case-control study showing that smoking and poor genital hygiene are associated with being affected by ZB.

Simulating the life course of psoriasis patients: the interplay between therapy intervention and marital status.

BACKGROUND: Psoriasis, a chronic relapsing inflammatory disease affecting primarily the skin, shows multiple comorbidities including depression, cardiovascular diseases and other relevant conditions. Psoriasis patients experience social isolation, job loss, financial difficulties and partnership problems. Inversely, psychosocial impairments may negatively influence the disease course. OBJECTIVE: To explore the feasibility of a model describing the interaction of psychosocial and clinical factors over the life course of patients. METHODS: We considered only seven states for members of a hypothetical population: single and healthy, single and having a psoriasis flare, single and 'cured', coupled and healthy, coupled and having a psoriasis flare, coupled and 'cured', and dead. Transition probabilities between states were taken from the Norwegian Population Register for the healthy population and from epidemiological research articles. Clinical experience allowed adjustments on the assumed parameters. RESULTS: Our macromodel, which simulates the effect of therapy intervention on patients' partnership status, yields a description of the transitions between the seven states. Treatment efficacy shows only a negligible effect on the chances of living with a partner. CONCLUSIONS: Mathematical modelling of interactions between social and health variables is in principle feasible. However, complex models, comprising more variables (for instance: employment status, depression level, obesity etc.), are needed for more realistic simulations for the interactions studied. As increasing the number of variables leads to an exponential increase of the model's state space, switching to micromodelling (representing each individual separately) may be necessary.

Respiratory disease in Niemann-Pick type C2 is caused by pulmonary alveolar proteinosis.

Niemann-Pick diseases are hereditary neurovisceral lysosomal lipid storage disorders, of which the rare type C2 almost uniformly presents with respiratory distress in early infancy. In the patient presented here, the NPC2 exon 4 frameshift mutation c.408_409delAA caused reduced NPC2 protein levels in serum and lung lavage fluid and the synthesis of an aberrant, larger sized protein of around 28 kDa. Protein expression was strongly reduced also in alveolar macrophages. The infant developed failure to thrive and tachypnea. Lung lavage, computer tomography, and histology showed typical signs of pulmonary alveolar proteinosis with an abnormal intraalveolar accumulation of surfactant as well as macrophages. An NPC2-hypomorph animal model also showed pulmonary alveolar proteinosis and accumulation of macrophages in the lung, liver, and spleen long before the mice died. Due to the elevation of cholesterol, the surfactant had an abnormal composition and function. Despite the removal of large amounts of surfactant from the lungs by therapeutic lung lavages, this treatment was only temporarily successful and the infant died of respiratory failure. Our data indicate that respiratory distress in NPC2 disease is associated with a loss of normal NPC2 protein expression in alveolar macrophages and the accumulation of functionally inactive surfactant rich in cholesterol.