Comparison of clinical and laboratory characteristics of COVID-19 and influenza in hospitalized children.

PURPOSE: COVID-19 and influenza infections have similar modes of transmission and clinical symptoms but have different prognoses and treatment methods; therefore, it is important to make a final diagnosis. Herein, we aimed to compare the demographic, clinical, and laboratory differences in hospitalized pediatric patients with COVID-19 and influenza. METHODS: This retrospective study comprised patients with COVID-19 managed between March 2020 to February 2022, and patients with influenza managed between December 2017 to February 2022, at a tertiary care hospital. The clinical data and laboratory parameters were obtained from the medical records of the hospital. Pediatric intensive care unit (PICU) admission, need for oxygen support, and the mortality rates of the patients were recorded and compared statistically. RESULTS: Overall, 107 patients with COVID-19 and 250 patients with influenza were included. Underlying chronic disease (UCD) rates were statistically higher in patients with COVID-19 (p < 0.001). When the symptoms were compared, fever, cough, and runny nose were more common in patients with influenza, and abdominal pain and rash were more common in patients with COVID-19 (p < 0.05). In patients with influenza, white blood cell count and absolute neutrophil count values were lower (p = 0.021 and p = 0.037, respectively), and aspartate aminotransferase and creatinine kinase values were higher (p = 0.007 and p < 0.001, respectively). PICU admission rates and oxygen support needs were similar in both groups (p > 0.05). When the virus was COVID-19, it had 7.8 times higher risk of mortality compared to influenza (p = 0.002). There were statistically significant risk for mortality when the virus was COVID-19, the risk of mortality was 6.9 times higher in those with UCD, 8.5 times higher in those with admission to PICU and 3.8 times higher in those with needing mechanical ventilation (MV) compared to when the virus was influenza (p = 0.004, p = 0.006 and p = 0.049, respectively). The mortality rate was higher in patients with COVID-19 (p = 0.007). CONCLUSION: This study showed that COVID-19 might negatively affect the survival times and increase mortality rates, especially in children with an UCD, admitted to the PICU and in need of MV.

Case presentation: a severe case of cobalamin c deficiency presenting with nephrotic syndrome, malignant hypertension and hemolytic anemia.

BACKGROUND: The etiology of nephrotic syndrome can vary, with underlying metabolic diseases being a potential factor. Cobalamin C (cblC) defect is an autosomal recessive inborn error of metabolism caused by mutations in the MMACHC gene, resulting in impaired vitamin B12 processing. While cblC defect typically manifests with hematological and neurological symptoms, renal involvement is increasingly recognized but remains rare. CASE PRESENTATION: We describe a 7-month-old male patient presenting with fatigue and edema. His first laboratory findings showed anemia, thrombocytopenia, hypoalbuminemia and proteinuria and further examinations reveals hemolysis in peripheric blood smear. During his follow up respiratory distress due to pleural effusion in the right hemithorax was noticed. And fluid leakage to the third spaces supported nephrotic syndrome diagnosis. The patient's condition deteriorated, leading to intensive care admission due to, hypertensive crisis, and respiratory distress. High total plasma homocysteine and low methionine levels raised suspicion of cobalamin metabolism disorders. Genetic testing confirmed biallelic MMACHC gene mutations, establishing the diagnosis of cblC defect. Treatment with hydroxycobalamin, folic acid, and betaine led to remarkable clinical improvement. DISCUSSION/CONCLUSION: This case underscores the significance of recognizing metabolic disorders like cblC defect in atypical presentations of nephrotic syndrome. Early diagnosis and comprehensive management are vital to prevent irreversible renal damage. While cblC defects are more commonly associated with atypical hemolytic uremic syndrome, this case highlights the importance of considering cobalamin defects in the differential diagnosis of nephrotic syndrome, especially when associated with accompanying findings such as hemolysis. Our case, which has one of the highest homocysteine levels reported in the literature, emphasizes this situation again.

Resuscitating the resuscitation: A single-centre experience on extracorporeal cardiopulmonary resuscitation.

AIM: Extracorporeal cardiopulmonary resuscitation (ECPR) is the rapid deployment of venoarterial extracorporeal membrane oxygenation (ECMO) during active cardiopulmonary resuscitation or in patients with intermittent return of spontaneous circulation. This study aimed to describe the demographic characteristics and outcomes of patients undergoing ECPR to identify survival-associated factors. METHODS: The study was conducted in an extracorporeal life support centre of a tertiary hospital in Turkey and included all patients who underwent ECPR for in-hospital cardiac arrest between April 2013 and June 2021. Complications included bleeding, neurological injury, renal failure, hepatic failure, limb ischemia and bloodstream infections. The primary outcomes were survival of ECMO and survival to discharge. Neurological outcomes were assessed using the Pediatric Cerebral Performance Category Scale for children and the Category of Cerebral Performance Scale for adults. RESULTS: The study included 26 patients (24 paediatric, 2 adults), 22 (85%) of them had cardiac pathology. Bleeding was the most common complication. Twelve (46%) patients survived ECMO, 9 (35%) survived to discharge. Sex, age, primary diagnosis, cardiac arrest rhythm and ECMO duration were not significantly associated with the primary outcomes. Bleeding, neurological injury and renal failure were associated with poorer survival to discharge. The neurological outcomes of all survivors to discharge were good. CONCLUSIONS: ECPR is not commonly accessible. Sharing the experience of the few treating centres to date is crucial to accumulating sufficient knowledge about its efficiency and raising clinician awareness. This limited single-centre experience demonstrated the utility of ECPR.

Multisystem inflammatory syndrome in children during severe acute respiratory syndrome coronavirus-2 pandemic in Turkey: A single-centre experience.

AIM: Severe acute respiratory syndrome coronavirus-2 (SARS-CoV-2) infection may result in a life-threatening hyperinflammatory condition named multisystem inflammatory syndrome in children (MIS-C). We aimed to assess demographics, clinical presentations, laboratory characteristics and treatment outcomes of patients with MIS-C. METHODS: We performed a retrospective study of patients with MIS-C managed between August 2020 and March 2021 at Dr. Sami Ulus Maternity Child Health and Diseases Training and Research Hospital in Turkey. RESULTS: A total of 45 patients (23 male, 51%) with a median age of 8.7 years (interquartile range: 5.6-11.7 years) were enrolled to study. The SARS-CoV-2 serology was positive in 43 (95%) patients. Organ-system involvement included the dermatologic in 41 (91%), cardiovascular in 39 (87%), hematologic in 36 (80%) and gastrointestinal in 36 (80%) patients. Acute anterior uveitis was diagnosed in nine (20%) patients. Two patients presented with clinical findings of deep neck infection such as fever, neck pain, trismus, swelling and induration on the cervical lymph node. One patient presented with Henoch-Schonlein purpura-like eruption. Coronary artery dilatation was detected in five (11%) patients. For treatment of MIS-C, intravenous immunoglobulin was used in 44 (98%) patients, methylprednisolone in 27 (60%) and anakinra in 9 (20%) patients. The median duration of hospitalisation was nine days. All patients recovered. CONCLUSIONS: Children with MIS-C might have variable clinical presentations. Acute anterior uveitis might be a prominent presentation of MIS-C and require ophthalmological examination. It is essential to make patient-based decisions and apply a stepwise approach for the treatment of this life-threatening disease.

An Outbreak of Shiga Toxin-Positive Enteroaggregative Escherichia coli 0104:H4 Related Hemolytic Uremic Syndrome in Turkey: A Multicenter Study.

INTRODUCTION: Serious outbreaks related to Shiga toxin-producing Escherichia coli-associated hemolytic uremic syndrome (STEC-HUS) have been reported globally. In 2011, Germany experienced a significant outbreak of HUS caused by enteroaggregative Escherichia coli (EAEC) O104:H4 strain. Since then, no other outbreaks of this strain have been reported. This study aims to evaluate pediatric patients affected by the second documented worldwide outbreak of STEC-HUS (EAEC O104:H4 serotype) contaminating local drinking water. METHODS: Medical records of patients hospitalized in five pediatric intensive care units (PICU) diagnosed with STEC-HUS between July and September 2022 were evaluated retrospectively. RESULTS: Eighteen patients (14 girls, and 4 boys) were enrolled in the study. The median age was 7.4 [Intetquartile range (IQ) 1.3-17] years. Abdominal pain was the most common symptom (100%). The mean duration between symptom onset and development of STEC-HUS was 3 days (IQ 1-9). EAEC O104:H4 serotype was detected in the stool samples of eight patients. Neurological involvement was observed in three patients, cardiac involvement in two patients, and both in one patient. Two patients required respiratory support and dialysis was performed in 16 (88.8%) patients. Plasmapheresis was administered to two patients, and eculizumab was given to four. No mortality was reported during follow-up; the mean durations of PICU and hospital stays were 11.3 and 31.6 days, respectively. CONCLUSION: Outbreaks of HUS can have severe implications on mortality and morbidity. However, timely diagnosis and implementation of appropriate supportive care, including dialysis, respiratory support, and suitable medical treatment for eligible patients, can lead to favorable outcomes.

An exceptional cause of acute respiratory failure in an infant: negative pressure pulmonary edema.

Acute respiratory failure is an important reason for pediatric intensive care admissions. Lung parenchymal disease, airway obstruction, or neuromuscular dysfunction are the most common causes. Negative pressure pulmonary edema, characterized by pulmonary edema associated with upper airway obstruction, can rarely cause sudden onset respiratory failure. Herein, we describe an infant who suffered sudden onset respiratory failure and pulmonary hemorrhage while being held facedown for a bath, was admitted to the pediatric intensive care unit, intubated and mechanically ventilated for three days, and was diagnosed with negative pressure pulmonary edema, and discharged without any sequelae. Negative pressure pulmonary edema is a rare entity. Its true frequency is not known due to the lack of awareness. This report aimed to increase clinician familiarity with negative pressure pulmonary edema in patients with sudden onset respiratory failure and/or pulmonary hemorrhage.

Attention to diseases that re-emerge due to refusal of vitamin K: an infant case.

Intramuscular vitamin K injection is recommended for all newborns to prevent bleeding. However, the number of parents who reject vitamin K is at an increase. We present a 1-month girl who presented with haemorrhagic shock due to extraordinary intra-thoracic bleeding. The patient was treated with thoracentesis and blood transfusion. Parents were informed the about the benefits of Vitamin K and they were convinced to continue a routine immunization programme.

Evaluation of non-infectious complications in children receiving parenteral nutrition.

BACKGROUND: Parenteral nutrition may lead to inevitable complications. AIMS: To determine the indications, metabolic and mechanical complications of parenteral nutrition in children. METHODS: One hundred fifty-eight children (91 males; 57.8%) who received 179 episodes of individualized parenteral nutrition for ≥ 5 days within 2 years were analyzed. Indications and duration of parenteral nutrition, effect on growth, and metabolic and central venous catheter-related non-infectious complications were evaluated. RESULTS: Parenteral nutrition was administered in 179 different episodes (109 males; 60.9%), and the median age during these episodes was 64.0 (14.0-129.0) months. The most common indications were hematological malignancies, gastrointestinal surgery, and hematopoietic stem cell transplantation. Most of the electrolyte imbalances occurred in the first 3 days. Hypophosphatemia (44.7%), hypomagnesemia (43.0%), hypokalemia (43.0%), hyponatremia (40.8%), and hypertriglyceridemia (38.2%) were the most common metabolic complications. Liver transaminases elevated in 32/145 (22.1%) episodes and bilirubin in 30/149 (21.0%). Ursodeoxycholic acid treatment was added to 25 patients with hypertransaminasemia and/or hyperbilirubinemia. Transaminase levels improved in 16 (64%) and bilirubin levels in 15 (60%) patients receiving ursodeoxycholic acid. Catheter thrombosis was seen in 4.5% of the episodes. The targeted energy could be given more efficiently via central catheters rather than peripheral venous accesses. Patients' bodyweights increased in 39.1% of the episodes. CONCLUSIONS: Close monitoring of electrolyte levels, especially in the first 3 days, is crucial to prevent complications of parenteral nutrition. When individualized PN preparations are used for metabolically unstable patients, it can be easier to maintain the blood glucose, lipids, and electrolyte levels within the normal range.

Dyskinesia due to mexiletine overdose: a rare presentation.

BACKGROUND: Mexiletine, a class IB antiarrhythmic, is a structural analog of lidocaine. Our knowledge of mexiletine overdose is based on lidocaine overdose reports. Only a few cases of mexiletine overdose have been reported, including fatal overdoses. Mexiletine toxicity primarily affects the central nervous, cardiovascular, and gastrointestinal systems. CASE: A 16-year-old female was brought to our hospital by ambulance after taking an unknown dose of mexiletine in a suicide attempt. Ventricular fibrillation developed while in the ambulance; cardiopulmonary resuscitation was started and spontaneous circulation returned within 1 min. The patient had been taking oral mexiletine for 1 month to treat primary erythromelalgia. Her vital signs were normal, but she was unconscious. Following gastric lavage she was transferred to the pediatric intensive care unit. Midazolam and levetiracetam were required due to uncontrolled seizures. During the first hour of hospitalization, severe dyskinesia characterized by abnormal involuntary large hyperkinetic movements in all 4 extremities was observed and successfully treated with 2 doses of intravenous biperiden. The patient was discharged on day 6 of hospitalization. CONCLUSIONS: Mexiletine overdose can be life-threatening. In addition to rapid and effective resuscitation, rapid identification and management of cardiovascular and central nervous system manifestations are key to preventing morbidity and mortality. The presented case had severe dyskinesia that was successfully treated with repeated doses of biperiden. Biperiden did not cause arrhythmia. Based on the presented case, we think biperiden should be considered for the treatment of movement disorders in cases of mexiletine overdose.

Evaluation of nutritional status in pediatric intensive care unit patients: the results of a multicenter, prospective study in Turkey.

Introduction: Malnutrition is defined as a pathological condition arising from deficient or imbalanced intake of nutritional elements. Factors such as increasing metabolic demands during the disease course in the hospitalized patients and inadequate calorie intake increase the risk of malnutrition. The aim of the present study is to evaluate nutritional status of patients admitted to pediatric intensive care units (PICU) in Turkey, examine the effect of nutrition on the treatment process and draw attention to the need for regulating nutritional support of patients while continuing existing therapies. Material and Method: In this prospective multicenter study, the data was collected over a period of one month from PICUs participating in the PICU Nutrition Study Group in Turkey. Anthropometric data of the patients, calorie intake, 90-day mortality, need for mechanical ventilation, length of hospital stay and length of stay in intensive care unit were recorded and the relationship between these parameters was examined. Results: Of the 614 patients included in the study, malnutrition was detected in 45.4% of the patients. Enteral feeding was initiated in 40.6% (n = 249) of the patients at day one upon admission to the intensive care unit. In the first 48 h, 86.82% (n = 533) of the patients achieved the target calorie intake, and 81.65% (n = 307) of the 376 patients remaining in the intensive care unit achieved the target calorie intake at the end of one week. The risk of mortality decreased with increasing upper mid-arm circumference and triceps skin fold thickness Z-score (OR = 0.871/0.894; p = 0.027/0.024). The risk of mortality was 2.723 times higher in patients who did not achieve the target calorie intake at first 48 h (p = 0.006) and the risk was 3.829 times higher in patients who did not achieve the target calorie intake at the end of one week (p = 0.001). The risk of mortality decreased with increasing triceps skin fold thickness Z-score (OR = 0.894; p = 0.024). Conclusion: Timely and appropriate nutritional support in critically ill patients favorably affects the clinical course. The results of the present study suggest that mortality rate is higher in patients who fail to achieve the target calorie intake at first 48 h and day seven of admission to the intensive care unit. The risk of mortality decreases with increasing triceps skin fold thickness Z-score.

Surfactant for a Patient with Refractory Pyopneumothorax and Acute Respiratory Distress Syndrome Due to Pneumococcal Necrotizing Pneumonia Complicated by a Bronchopleural Fistula.

Background: Necrotizing pneumonia rarely occurs in children, but when it does it can be complicated by bronchopleural fistula, empyema, pneumothorax, sepsis, and acute respiratory distress syndrome (ARDS). Antimicrobial therapy is the cornerstone of its management; however, surgery is necessary in some cases. Ideally, surgical interventions are kept to a minimum, but this is not always possible if there is a mass effect from air and fluid in the pleural space, pulmonary necrosis leading to massive hemoptysis, uncontrolled sepsis, or difficulties with assisted ventilation. Case Presentation: Herein we present a patient with refractory pyopneumothorax and ARDS due to pneumococcal necrotizing pneumonia complicated by a bronchopleural fistula. The patient's clinical condition deteriorated despite antibiotics, surgical drainage, and assisted ventilation. Owing to pneumothorax with a high percentage of air leakage, bilateral diffuse collapse of the lungs, and insufficient oxygenation, surgical treatment was considered, but because of the patient's lack of tolerance for surgery due to hemodynamic reasons and the complications associated with surgery, medical treatment was determined to be more appropriate. Surfactant treatment was administered to the patient, resulting in significant clinical improvement. Conclusion: To the best of our knowledge, this is the first report of the use of surfactant to treat ARDS due to necrotizing pneumonia. Based on the presented case, we think surfactant can be considered as a salvage treatment for such patients.

COVID-19 Transmission and Clinical Features in Pediatric Intensive Care Health Care Workers.

OBJECTIVE: This study aims to investigate modes of transmission and clinical features of coronavirus disease 2019 in healthcare workers in pediatric intensive care units. MATERIALS AND METHODS: This multicenter descriptive study was conducted between March and November 2020. Patient demographics, clinical characteristics, origin of coronavirus disease 2019, treatment modalities, and loss of workdays were recorded. RESULTS: Seven hundred and sixty-eight healthcare workers from 16 pediatric intensive care units were enrolled and 114 (14.8%) healthcare workers with a mean age of 29.7 ± 6.7 years became coronavirus disease 2019 patients. Seventy-six (66.7%) patients were female. Approximately half (54.3%) of the patients were physicians, 34.2% were nurses, and 11.4% were ancillary staff. Transmission was deemed to occur through patient contact in 54.3% of the patients. Comorbid illness was present 10.5% of the patients. Transmission occurred during endotracheal intubation in 21%, cardiopulmonary resuscitation in 9.6%, and non-invasive ventilation in 12.2% of patients, while transmission was a result of multiple possible procedures in 43.8%. Intensive care admission was needed for 13.1% of the patients. Five patients needed oxygen by cannula, 7 needed oxygen with a non-rebreathing mask, 5 needed high-flow nasal cannula support, 5 needed non-invasive ventilation, and 3 needed invasive mechanical ventilation. Fortunately, no infected healthcare workers died. CONCLUSION: Coronavirus disease 2019 in healthcare workers is a significant problem in pediatric intensive care units. Transmission seems to occur particularly frequently during patient care procedures such as intubation, ventilation and aerosol therapy, which highlights the importance of proper use of full sets of personal protective equipment during all procedures during care of coronavirus disease 2019 patients.

Increased Severe Cases and New-Onset Type 1 Diabetes Among Children Presenting With Diabetic Ketoacidosis During First Year of COVID-19 Pandemic in Turkey.

Introduction: There have been some significant changes regarding healthcare utilization during the COVID-19 pandemic. Majority of the reports about the impact of the COVID-19 pandemic on diabetes care are from the first wave of the pandemic. We aim to evaluate the potential effects of the COVID-19 pandemic on the severity of diabetic ketoacidosis (DKA) and new onset Type 1 diabetes presenting with DKA, and also evaluate children with DKA and acute COVID-19 infection. Methods: This is a retrospective multi-center study among 997 children and adolescents with type 1 diabetes who were admitted with DKA to 27 pediatric intensive care units in Turkey between the first year of pandemic and pre-pandemic year. Results: The percentage of children with new-onset Type 1 diabetes presenting with DKA was higher during the COVID-19 pandemic (p < 0.0001). The incidence of severe DKA was also higher during the COVID-19 pandemic (p < 0.0001) and also higher among children with new onset Type 1 diabetes (p < 0.0001). HbA1c levels, duration of insulin infusion, and length of PICU stay were significantly higher/longer during the pandemic period. Eleven patients tested positive for SARS-CoV-2, eight were positive for new onset Type 1 diabetes, and nine tested positive for severe DKA at admission. Discussion: The frequency of new onset of Type 1 diabetes and severe cases among children with DKA during the first year of the COVID-19 pandemic. Furthermore, the cause of the increased severe presentation might be related to restrictions related to the pandemic; however, need to evaluate the potential effects of SARS-CoV-2 on the increased percentage of new onset Type 1 diabetes.

Peer Education Model for Basic Life Support Training among High School Children: A Randomized Trial.

BACKGROUND AND OBJECTIVES: The aim of the study was to test the effectiveness of the peer education method on the learning and application of Basic Life Support (BLS) in high school students and to test the effectiveness of the peer education model on the BLS instructor training. METHODS: High school grade one students were included in the study. Students were divided in two groups (Group A and Group B). Peer instructors who were trained by health professionals trained students in Group A. Peer instructors who were trained by their peers trained students in Group B. Pre- and post-training awareness and knowledge tests were applied to measure the awareness and knowledge of all students. Students' success in applying BLS steps was evaluated by a practical exam that was coordinated by physicians using a checklist. RESULTS: Result of the pre-post training awareness questionnaire, pre-post training knowledge tests, and practical exam indicated that instructors trained by their peers were as effective as the instructors trained by medical physicians in terms of giving BLS training to high school students. In the 16-step BLS application competence evaluation, the students in Group A applied BLS with a success rate of 90.2% and in Group B with a success rate of 93.4%. CONCLUSION: In the current study, it was shown that the peer education model is effective in BLS training and BLS instructor training in high school students. This novel method of peer education gives an opportunity to overcome the stated shortage in the budget and in trained instructors.

A novel technique with double catheter suctioning for clearing away the left lung atelectases: Selective left bronchial aspiration (SeLBA).

INTRODUCTION: Atelectasis is a common problem in intensive care units. It usually occurs due to airway obstruction caused by pulmonary secretions. As the left main bronchus angulates from carina more sharply, suctioning of the secretions from left side is less effective, causing failure in treatment of left lung atelectases. If chest physiotherapy and medical treatment fail, bronchoscopy should be considered; but it requires experience, time, effort and a higher cost. OBJECTIVES: To evaluate the success of a novel method "selective left bronchial aspiration (SeLBA)" in the treatment of left lung atelectases. METHODS: The study consisted of 10 patients who suffered left lung atelectasis and treated with double catheter suctioning technique, SeLBA in Pediatric Intensive Care Unit of Hacettepe University Ihsan Dogramaci Children's Hospital between June 2017 and February 2019. The outcomes were evaluated by comparing the chest X-rays before and after the procedure. RESULTS: All of the patients' atelectases resolved successfully by SeLBA procedure. CONCLUSION: We introduce this novel technique of reopening the atelectased left lung as a rapid, safe and practical alternative for bronchoscopic clear away.

Long term azithromycin therapy in patients with cystic fibrosis.

Inflammation is a central contributor to the pathogenesis of cystic fibrosis (CF) pulmonary disease; so limiting the excessive production of inflammatory mediators represents a major therapeutic strategy for slowing the decline in lung function and improving survival. The macrolide antibiotic azithromycin (AZM) has anti-inflammatory properties and immunomodulatory effects that may be beneficial in CF. The aim of this study was to document the long term use of AZM effect on pulmonary function, nutritional status and number of pulmonary exacerbations in patients with CF. Twenty four patients with CF aged 4-23 years followed at Hacettepe University Department of Pediatric Pulmonology between May 2007- December 2014 enrolled in the study from 630 CF patients. They received 10 mg/kg/day of AZM three times a week. Pulmonary function parameters, sputum cultures, body mass index (BMI) Z scores and number of pulmonary exacerbations were analyzed at different time intervals (at the visits at months 6, 9 and 12). Median age of the patients was 14.7 (range 4-23 years) years and median treatment duration was 14 months (range 6-60 months). Initially, median FEV1% was found 68% (range 30%-100%), BMI was found 17.05 (range 13.3-26.5) and oxygen saturation was found 95% (range 84%-99%). At the end of the 6th, 9th and 12th months of the AZM therapy; no significant differences in FEV1% and oxygen saturation parameters were found compared to the initial time, however BMI increased significantly (p=0.03), also the number of pulmonary exacerbations (p < 0.001) and severe exacerbations (p < 0.001) needing intravenous antibiotic treatment were significantly reduced at the 6th and 12th month. At the end of the 12th month of AZM; Methicillin sensitive S. aureus (MSSA) colonization was significantly increased (p=0.005) and increased macrolide resistance was detected (p=0.008). Although, this study could not be designed as a placebo controlled study, the results showed that at least 6 months of AZM treatment led to a significiant reduction in the number of pulmonary exacerbations requiring antibiotics and improvement on nutritional status. Despite increased P. aeruginosa antibiotic resistance and MSSA colonization rates, the lower incidence of acute exacerbations in patients receiving AZM is an important and clinically relevant measure of beneficial effect. Therefore, long term use of AZM may be considered to slow pulmonary deterioration in CF patients with P. aeruginosa colonization.