Background: Our aim was to investigate the acute effect of inhaled NaCl 5% on spirometry indices in patients aged over six years with cystic fibrosis (CF). Methods: 44 children over 6 years of age with CF took part in this study. Spirometry indices were measured. After receiving two puffs of salbutamol spray, the children were administered with 5 ml NaCl 5% by a medical compressed air system using a nebulizer kit and immediately a spirometric test was taken again. Spirometry results were recorded and the data were analyzed by SPSS v.18. Results:The percentages of the annual decline of forced expiratory volume in one second (FEV1) were 10.12 ± 31.28, 7.26 ± 17.10, and 13.8 ± 21.7 in children aged 6 -8 years, 9 -12 years, and over 13 years, respectively. There were significant differences in FVC, FEV1/FVC, MEF50, MEF75, MMEF25/27 before and after hypertonic NaCl 5% inhalation (P < 0.05). A significant decline in FEV1 was observed only in 2% of the children. Conclusions: Patients with CF exhibited a suitable response to acute inhalation of hypertonic NaCl 5%.

Objectives: The research aimed to evaluate the Pediatric Index of Mortality 3 (PIM-3) for determining the risk of mortality among pediatric intensive care unit patients. Methods: A retrospective analysis was conducted on case records, as well as patient data from all admissions to the PICU of Mofid Children’s Hospital, Tehran, from October 2017 to February 2018. Employing an android calculator application, the PIM-3 score was estimated early within the first PICU admission. Then, the PIM-3 score and mortality rate were analyzed using the Mann-Whitney U test. In addition, calibration and discrimination were assessed by the Hosmer-Lemeshow goodness-of-fit test and a receiver operating characteristic curve method, respectively. Finally, the Standardized Mortality Ratio (SMR) was calculated. Results: In this study, 365 young infants, ranging from 10 to 29-months-old, were included. The overall mortality rate was 10.4%. Further, the patients’ PIM-3 scores ranged from 0.06% to 2.37% (95% confidence interval), with a mean of 1.45% (4.16% in non-survivors and 1.14% in survivors). The SMR was estimated at 7.18, demonstrating the underprediction of the death rate. The AUC of 0.714 (95% CI: 0.626 to 0.801) demonstrated a fair to good discrimination power of PIM-3 as an international standard risk-adjusted mortality indicator. Moreover, this score underpredicted the risk of mortality in young infants admitted to our ICU in 2017. Generally, the prediction was weak among low-risk patients. Therefore, the Pediatric Index of Mortality-3 score has the potential to be implemented in our PICU by modifying the expected probability of death by multiplying the original PIM-3 score by 7.12.

Background: Gram-negative bacteria are a major cause of pulmonary infection in patients with cystic fibrosis (CF). Objectives: The study aimed to conduct the molecular identification of Gram-negative bacterial flora causing pulmonary infection in children with CF. Methods: In this study, sputum samples were taken from 64 CF children undergoing treatment as outpatients or inpatients at a referral children's hospital in Tehran. The PCR technique was used to detect the presence of Gram-negative bacteria, namely Pseudomonas spp., Acinetobacter baumannii, Stenotrophomonas maltophilia, and Burkholderia cepacia. Results: All samples were positive for 16srRNA. Pseudomonas spp. and A. baumannii were detected in 47% and 14% of the studied samples, respectively. Co-colonization by Pseudomonas spp. and A. baumannii was observed in three (5%) samples. Conclusions: According to this survey, Pseudomonas spp. were the most prevalent Gram-negative bacteria isolated from CF patients with pulmonary infection by molecular assays.