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Background: Cystic fibrosis is a complex progressive disease which assessing its progression and severity is essential. For this purpose there are scoring systems available to evaluate the disease severity. Objectives: The aim of the present study was to determine the clinical status of CF patients using shwachman score system in the pediatric pulmonary ward of Masih Daneshvari Hospital. Patients and Methods: A cross-sectional study was conducted to evaluate the clinical status with shwachman score system. 23 patients ranging from 5 to23 years were enrolled in this study. All data was extracted objectively from Shwachman-Kulczycki scoring system. Results: The overall mean Shwachman-Kulczycki score was 53.48 ± 13.8. Total scores of < 40 (severe), 41-55, 56-70, and 71-85 were detected in 1.7%, 39.1%, 30.4% and 8.7% of patients respectively. None of the patients were categorized in the excellent range. We found no statistically significant correlation between mortality rate and clinical score (P = 0.136). However, the results showed a statistically significant correlation between mortality rate and Shwachman score, (P = 0.02). Conclusions: Shwachman-Kulczycki score is an easily applied scoring system which can reflect the clinical status of patients objectively. However, concurrent use of other evaluating tools such as pulmonary function test (PFT) and Computed tomography scoring systems provide a more robust monitoring and a reliable evaluation tool.

Article type: Original Article Background: Cystic fibrosis (CF) is a genetic disease with an autosomal recessive pattern of inheritance. CF caused by a mutation in the cystic fibrosis transmembrane conductance regulator (CFTR) gene and characterized by impaired transport of chloride ions across the cell membrane. Staphylococcus aureus, Pseudomonas aeruginosa, and Burkholderia cepacia have been identified in the cultures of respiratory secretions of CF patients, and infections of these microorganisms are associated with high rates of morbidity and mortality. In patients with CF, severe inflammation of the airway can cause advanced bronchiectasis, which may result in respiratory failure and death. Objectives: This study aimed at evaluating the clinical findings of laboratory tests, bacterial colonization, and drug resistance in children and young adults with CF who hospitalized at the Pediatric Pulmonary Department of Masih Daneshvari Hospital, Tehran, Iran.

Background: Empyema in pediatric, if not treated adequately, would be an aggressive disease with many complications. Complete treatment of empyema includes broad antibiotics administration during the first week of the disease. However, in some cases, organized fibrinous tissue prevents antibiotic penetration and surgical intervention is required. Objectives: The aim of this study was to compare the duration of admission, duration of intravenous antibiotic therapy, time to response to treatment, and outcomes of medical and surgical interventions. Patients and Methods: In a retrospective, observational study, 60 patients with empyema were included; 35 were treated medically (medical group) and 25 surgically (surgical group). Results: Age and sex were not significantly different between the two groups. Only one of the patients in the medical group and one in the surgical group had immune deficiency. Coughing was presented in 25 patients in medical group and in 17 patients in surgical group as a symptom. Hospitalization days of were significantly lower in the medical group (12.4 ± 5.5) compared with the surgical group (20.4 ± 5.2) (P = 0.041). Duration of intravenous antibiotic therapy was not significantly lower in the medical group compared with the surgical group (P = 0.085). Time to response to treatment (from the admission day to the clinical improvement) was significantly lower in the medical group compared with the surgical one (P = 0.024). Mortality was 0% in both groups. Conclusions: If medical treatment gets started early in the course of empyema, prognosis would be excellent. Besides, there was no significant difference in short- and long-term outcomes between medical or surgical interventions.

Background: Congenital lung malformation (CLM) is a rare condition with high incidence of mortality and morbidity. CLM is often discovered incidentally on routine prenatal ultrasound or other postnatal imaging tests. Depending on the size of lesion and degree of functional impairment, prognosis is different. Today, fetal lung abnormalities are increasingly detected prenatally by ultrasound imaging. Objectives: This study aimed to evaluate the CLM cases in pediatric pulmonary ward of Masih Daneshvari Hospital during an 8-year period from 2002 to 2010. Materials and Methods: In a retrospective study, 21 patients (12 females, nine males), diagnosed with CLM, were enrolled. The mean age of participants was 7.3 years (ranging from 1 month to 16 years). All patients were histopathologically diagnosed with CLM and their demographic data including age, sex, and clinical signs and symptoms were recorded. To confirm the diagnoses and rule out the differentials, patients underwent bronchoscopy, angiography and other proper diagnostic procedures. Afterwards, they were divided into several subgroups based on the malformation and type of involvement, including agenesia, hypoplasia, aplasia, bronchogenic cyst, and sequestration. Results: We reported two agenesis, five hypoplasia, six aplasia, two bronchogenic cyst, and six sequestration cases. All patients were symptomatic at the time of diagnostic workup. The most common clinical signs and symptoms were respiratory distress and recurrent pulmonary infections. Conclusions: Considering the wide range of clinical manifestations from no clinical symptom to severe respiratory distress at birth, imaging including chest X-ray, CT scan with contrast and MRI are of great values for early diagnosis of CLM. Early diagnosis could provide a more appropriate timing of evaluation and treatment options, lower mortality and morbidity, and better prognosis.