Renal replacement therapies in the aftermath of the catastrophic Marmara earthquake

Renal replacement therapies in the aftermath of the catastrophic Marmara earthquake.BackgroundRenal replacement therapy is of vital importance in the treatment of crush syndrome victims, who are frequently encountered after catastrophic earthquakes. The Marmara earthquake, which struck Northwestern Turkey in August 1999, was characterized by 477 victims who needed dialysis.MethodWithin the first week of the disaster, questionnaires containing 63 clinical and laboratory variables were sent to 35 reference hospitals that treated the victims. Information considering the features of dialyses obtained through these questionnaires was submitted to analysis.ResultsOverall, 639 casualties with renal complications were registered, 477 of whom (mean age 32.3 ± 13.7 years, 269 male) needed dialysis. Among these, 452 were treated by a single dialysis modality (437 intermittent hemodialysis, 11 continuous renal replacement therapy and 4 peritoneal dialysis), while 25 victims needed more than one type of dialysis. In total, 5137 hemodialysis sessions were performed (mean 11.1 ± 8.0 sessions per patient) and mean duration of hemodialysis support was 13.4 ± 9.0 days; this duration was shorter in the non-survivors (7.0 ± 8.7 vs. 10.0 ± 9.8 days, P = 0.005). Thirty-four victims who underwent continuous renal replacement therapy had higher mortality rates (41.2 vs. 13.7%, P < 0.0001). Only eight victims were treated by peritoneal dialysis, four of whom also required hemodialysis or continuous renal replacement therapy. The mortality rate in the dialyzed victims was 17.2%, a significantly higher figure compared to the mortality rate of the non-dialyzed patients with renal problems (9.3%; P = 0.015).ConclusionSubstantial amounts of dialysis support may be necessary for treating the victims of mass disasters complicated with crush syndrome. Dialyzed patients are characterized by higher rates of morbidity and mortality

Cystic fibrosis in Turkey: First data from the national registry.

Background Cystic fibrosis (CF) care has been implemented in Turkey for a long time; however, there had been no patient registry. For this purpose, the Turkish National CF Registry was established. We present the first results of registry using data collected in 2017. Methods The data were collected using a data-entry software system, which was accessed from the internet. Demographic and annually recorded data consisted of 15 and 79 variables, respectively. Results There were 1170 patients registered from 23 centers; the estimated coverage rate was 30%. The median age at diagnosis was 1.7 years (median current age: 7.3 years); 51 (4.6%) patients were aged over 18 years. Among 293 patients who were under 3 years of age, 240 patients (81.9%) were diagnosed through newborn screening. Meconium ileus was detected in 65 (5.5%) patients. Genotyping was performed in 978 (87.4%) patients and 246 (25.2%) patients' mutations were unidentified. The most common mutation was deltaF508 with an allelic frequency of 28%, followed by N1303K (4.9%). The median FEV1% predicted was 86. Chronic colonization with Pseudomonas aeruginosa was seen in 245 patients. The most common complication was pseudo-Bartter syndrome in 120 patients. The median age of death was 13.5 years in a total of 15 patients. Conclusions Low coverage rate, lack of genotyping, unidentified mutations, and missing data of lung functions are some of our greatest challenges. Including data of all centers and reducing missing data will provide more accurate data and help to improve the CF care in Turkey in the future

Clinical features and accompanying findings of Pseudo-Bartter Syndrome in cystic fibrosis

Background Pseudo-Bartter syndrome (PBS) is a rare complication of cystic fibrosis (CF) and there are limited data in the literature about it. We aimed to compare clinical features and accompanying findings of patients with PBS in a large patient population