Every patient of this group will be educated and instructed about usage, dosing and side effects of the drug.
Dose: azithromycin 500 mg three times weekly for 6 months. added to the conventional treatment.

Every patient of this group will receive the conventional treatment.

Bronchiectasis

administration of azithromycin three times weekly for six months

Macrolides

Macrolides in COPD- Bronchiectasis Overlap

Safety and Efficacy of Long-Term Macrolide Therapy in Patients With COPD-Bronchiectasis Overlap Syndrome

1)-ototoxicity by whispered- voice test before starting treatment and monthly The examiner stands arm's length (0.6 m) behind the seated patient and whispers a combination of numbers and letters (for example, 4-K-2) and then asks the patient to repeat the sequence.
If the patient responds correctly, hearing is considered normal; if the patient responds incorrectly, the test is repeated using a different number/letter combination The examiner always stands behind the patient to prevent lip reading The other ear is assessed similarly with a different combination of numbers and letters
-2)-assessment of hepatotoxicity by measuring liver enzymes (aspartate transaminase AST, Alanine transaminase ALT (Unit/Liter)) before starting treatment and monthly.
3)-assessment of possible prolongation of QT interval (millimeters) by ECG (electrocardiogram) before starting treatment and monthly.

symptoms:
Dyspnea score by mMRC (modified medical research council) score, with strenuous exercise 0 when hurrying or walking up a slight hill +1 Walks slower than people of the same age because of dyspnea +2 Stops for breath after walking 100 yards +3 Too dyspneic to leave house +4
sputum volume by (milliliter),
bronchiectasis health questionnaire (BHS)
exacerbation: Frequency of outpatient visits per month and hospital admission if happened (number\month).
systemic inflammation:
CBC (complete blood count) (White Blood Cells(n/ microliter), differential count (%)
Erythrocyte Sedimentation Rate (millimeter/hour)
C-Reactive Protein (milligram\ liter)
Microbiological:
Change in microbiological picture based on sputum culture and sensitivity
- Functional: Change in FEV1 (forced expiratory volume at one second) (liter/second.) all these will be done before starting treatment and monthly.

Alaa Sayed Ali

Assistant Lecturer

Assiut University

All participants will be receiving the study drug

Tracheostomy Infection

Inhaled antibiotic with anti-pseudomonal properties

Inhaled Aztreonam

Benioff Children's Hospital Oakland

The Use of Inhaled Aztreonam in Children With a Tracheostomy Tube and Pseudomonas

A-PACT: The Use of Inhaled Aztreonam to Eliminate or Decrease the Bacterial Burden of Pseudomonas Aeruginosa in Children With a Tracheostomy Tube.

See if the number of days of systemic antibiotics needed decreases after participant starts using the study drug. (compare 1 year prior to starting study drug to the 1 year while patient is on the study drug)

See if bacterial density of Pseudomonas is decreased or eliminated with the study drug (will compare all cultures from the year prior to starting the trial to the year during the trial)

to see if there is a difference in how effective the study drug is depending on what type of trach tube the subject has. Will assess this by looking at the Pseudomonas bacterial density from the tracheostomy tube cultures from the year prior to starting the study drug and compare to the year while on the study drug.

Can study drug decrease number of hospital admissions form the year prior to starting the study drug compared to the year while taking the study drug

Can the study drug decrease the duration of hospitalizations from the year prior to starting the study drug compared to the year while taking the study drug

Rachna Wadia

Clinical Assistant Professor, Pediatric Pulmonary and Principal Investigator

UCSF Benioff Children’s Hospital Oakland

Gilead Sciences

two-center, prospective, case control clinical trial

Pa negative or concurrently enrolled in the EPIC Clinical Trial

For study purposes, OP swabs or expectorated sputum for bacterial culture will be obtained annually beginning in the calendar year of first isolation of Pa from a respiratory culture at the local site laboratory. A serum sample for serology assessment and banking will be obtained annually in conjunction with a scheduled clinical blood draw whenever possible. Under a separate consent, additional blood (6 ml) will be collected for DNA use and banking for studies of genetic factors that may be associated with CF pathogenesis, disease progression, and clinical outcomes. These studies will test for association between gene variants and various CF-related phenotypes using either a targeted (i.e., candidate gene) approach or by performing a whole-genome scan.

Cystic Fibrosis

The University of Alabama at Birmingham

Children's Hospital of Los Angeles / USC Medical School

Kaiser Permanente Medical Center

Packard Children's Hosp., Stanford University

University of California, San Francisco

Children's Hospital Denver

duPont Hospital for Children

Nemours Children's Clinic

All Children's Hospital CF Center

Emory University, Cystic Fibrosis Center

Medical College of Georgia

Children's Memorial Hospital

Riley Hospital, Indiana University

University of Iowa

University of Kentucky

Maine Medical Center

Children's Hospital, Boston

University of Mass Memorial Health Care

University of Michigan

Children's Hospital of Michigan

Spectrum Health Hospitals - DeVos Children's Hospital

Children's Hospitals & Clinics

University of Mississippi Medical Center

Children's Mercy Hospital

Cardinal Glennon Children's Hospital - St. Louis University

Washington University School of Medicine/St. Louis Children's Hospital

University of Nebraska

Dartmouth-Hitchcock Medical Center

Monmouth Medical Center

Albany Medical College

Women & Children's Hospital of Buffalo

Schneider Children's Hospital

University of Rochester

SUNY Upstate Medical Center

New York Medical College/Westchester Medical Center

University of North Carolina, Chapel Hill

Children's Hospital Medical Center of Akron

Rainbow Babies & Childrens Hospital

Children's Hospital

Children's Medical Center

Oregon Health Sciences University

Penn State Milton S. Hershey Medical Center

St. Christopher's Hospital for Children

Children's Hospital of Pittsburgh

Rhode Island Hospital

LeBonheur Children's Medical Center

Vanderbilt University Medical Center

Cook Children's Medical Center

Texas Children's Hospital

University of Utah

Vermont Children's Hospital at Fletcher Allen Health Care

Children's Hospital & Regional Medical Center

University of Wisconsin Hospital and Clinics

Children's Hospital of Wisconsin

The EPIC Observational Study

Longitudinal Assessment of Risk Factors For and Impact of Pseudomonas Aeruginosa Acquisition and Early Anti-Pseudomonal Treatment in Children With CF

CF Therapeutics Development Network Coordinating Center

Cystic Fibrosis Foundation