Perspectives of adult patients with lysosomal storage diseases on the transition from pediatric to adult healthcare in Turkey.

AIM: In many countries, adult clinics specifically dedicated to adult patients with lysosomal storage diseases (LSDs) do not exist. In Turkey, these patients are managed either by pediatric metabolic specialists or adult physicians who do not specifically specialize in LSDs. In this study, we aimed to identify the unmet clinical needs of these adult patients and their suggestions. METHODS: The focus group participants were 24 adult LSD patients. Interviews were conducted in person. RESULTS: A total of 23 LSD patients and parents of a patient with mucopolysaccharidosis type-3b with intellectual deficit were interviewed, with 84.6% of patients diagnosed after the age of 18 years and 18% of patients diagnosed before the age of 18 years desiring management by adult physicians. Patients with particular physical characteristics or severe intellectual deficit declined the transition. Patients reported structural problems in the hospital and social problems associated with pediatric clinics. They made suggestions to facilitate the possible transition. CONCLUSION: With improved care, more patients with LSDs survive into adulthood or receive the diagnosis in adulthood. Children with chronic diseases need to transition to the care of adult physicians when they reach adulthood. Thus, there is an increasing need for adult physicians to manage these patients. In this study, most LSD patients accepted a well-planned and organized transition. Problems were related to stigmatization and social isolation in the pediatric clinic or adult issues with which pediatricians are not familiar. There is a need for adult metabolic physicians. Thus, health authorities should adopt necessary regulations for training of physicians in this field.

The role of C-reactive protein ratio in predicting mortality in patients with Fournier gangrene.

PURPOSE: This study aimed to determine the C-reactive protein (CRP) ratio for the survival of patients with Fournier gangrene (FG). METHODS: Fifty-two patients with FG between January 2011 and September 2018 were retrospectively analyzed. Data on clinical presentation, Fournier Gangrene Severity Index (FGSI), CRP ratio, management, and outcome were analyzed. The CRP ratio was calculated as preoperative CRP/postoperative CRP value that measured 48 hours after surgical intervention. Possible alternative cutoff points for the FGSI and CRP were determined by receiver operating characteristic (ROC) analyses. The risk factors related to the prognosis were evaluated by univariate and multivariable logistic regression analyses. RESULTS: The mean CRP ratios were 6.7±6.6 in the survivor group and 1.2±0.8 in the nonsurvivor group (P=0.001). FGSI was significantly higher in the non-survivor group compared to survivor group (8.5±2.5 vs. 3.5±2.2, P=0.001). There was a negative correlation between FGSI and CRP ratio (r=-0.51). ROC analysis determined the cutoff value as 1.78 for CRP (sensitivity, 86%; specificity, 82%; area under the ROC curve, 0.90) to predict death. The incidence of death for patients with CRP ratio of ≤1.78 increased 26.7 fold for those with CRP ratio of >1.78 (95% confidence interval [CI], 4.8-146.5; P=0.001). In the multivariable logistic regression model, CRP ratio (odds ratio [OR], 10.3; 95% CI, 1.5-72.2; P=0.019) and FGSI (OR, 17.8; 95% CI, 2.6-121.1; P=0.003) were independent risk factors for death. CONCLUSION: The CRP ratio is a simple method to use to predict mortality in FG.

Worse pre-admission quality of life is a strong predictor of mortality in critically ill patients.

Objectives: In this study, we aimed to investigate whether quality of life (QoL) before intensive care unit (ICU) admission could predict ICU mortality in critically ill patients. Patients and methods: Between January 2019 and April 2019, a total of 105 ICU patients (54 males, 51 females; mean age: 58 years; range, 18 to 91 years) from two ICUs of a tertiary care hospital were included in this cross-sectional, prospective study. Pre-admission QoL was measured by the Short Form (SF)-12- Physical Component Scores (PCS) and Mental Component Scores (MCS) and EuroQoL five-dimension, five-level scale (EQ-5D-5L) within 24 h of ICU admission and mortality rates were estimated. Results: The overall mortality rate was 28.5%. Pre-admission QoL was worse in the non-survivors independent from age, sex, socioeconomic and education status, and comorbidities. During the hospitalization, the rate of sepsis and ventilator/hospital-acquired pneumonia were similar among the two groups (p>0.05). Logistic regression analysis adjusted for sex, age, education status, and Acute Physiology and Chronic Health Evaluation II (APACHE II) scores showed that pre-admission functional status as assessed by the SF-12 MCS (odds ratio [OR]: 14,2; 95% confidence interval [CI]: 2.5-79.0), SF-12 PCS (OR: 10.6; 95% CI: 1.8-62.7), and EQ-5D-5L (OR: 8.0; 95% CI: 1.5-44.5) were found to be independently associated with mortality. Conclusion: Worse pre-admission QoL is a strong predictor of mortality in critically ill patients. The SF-12 and EQ-5D-5L scores are both valuable tools for this assessment. Not only the physical status, but also the mental status before ICU admission should be evaluated in terms of QoL to better utilize ICU resources.

Traditional and complementary medicine use and beliefs during COVID-19 outbreak: A cross-sectional survey among the general population in Turkey.

OBJECTIVE: Coronavirus disease 2019 (COVID-19) caused a universal psychosocial impact, with many individuals exposed to threats preferring to try self-care interventions and non-conventional approaches such as traditional and complementary medicine (T&CM) for preventive purposes. This study was conducted to determine the use of and beliefs about T&CM among a subset of the general Turkish population during the COVID-19 outbreak. METHOD: A cross-sectional online survey was carried out among the general population (aged ≥ 18 years) of Adana, Turkey during the strict lockdown period (April 11 to April 30, 2020). The survey instrument included details about sociodemographic characteristics, general information, T&CM use and beliefs. It was distributed among eligible participants via social media channels (Instagram, WhatsApp and Facebook accounts). RESULTS: Out of a total 389 participants, 39.3% (n = 153) used T&CM and 60.7% were non-T&CM users during COVID-19. Of those using T&CM, 61 (39.8%) reported the usage of more than one form of T&CM, mostly herbal medicine (30.8%), followed by nutritional supplements/vitamins (23.8%). 33.9% (n = 52) of participants using T&CM did not report T&CM use to theirmedical physicians. A statistically significant difference was observed between T&CM users and non-T&CM users in gender, age, marital status, level of education, income, and prior use of T&CM (p < 0.05). Social media (n = 204; 52.4%) was the primary source of information for T&CM use. Overall, 33.7%, 54.8% and 39% of participants in this stduy believed that T&CM therapies are effective, have fever side-effects/safe and should be use for COVID-19, respectively. CONCLUSION: During the outbreak of COVID-19, a significant proportion of the population reported the use of T&CM, with different beliefs about T&CM being observed. Better-structured T&CM-specific educational programs, enhanced physician-patient communication and access to reliable information are needed to ensure appropriate T&CM use during pandemics in Turkey.