borderline secondary right heart dysfunction should be evaluated with an electrocardiogram (EKG), CXR, and cardiology consultation for possible echocardiogram Rib fracture can occur secondary to osteopenia in patients with CF with poor nutrition, or secondary to overly aggressive chest percussion and postural drainage Superficial ecchymoses and point tenderness along the rib margin in the setting of malnutrition and scant subcutaneous fat tissue may suggest the diagnosis of rib fracture; diagnosis can be confirmed radiologically, although findings may not be apparent on CXRs Treatment is complicated by the need to at least temporarily limit airway clearance, which can lead to increasing airway obstruction History of fracture or suspicious behavior should also raise the question of child abuse in young children TABLE 99.5 CHEST PAIN IN CYSTIC FIBROSIS PATIENTS Common Uncommon Costochondritis Rib fracture Pleurisy/pleuritis Pulmonary embolism Pneumonia Pneumothorax Esophagitis/GERD Rare Cardiac disease Respiratory Failure Thickened airway secretions with bacterial infection, mucous hypersecretion, bronchoconstriction, mucosal edema, inflammation, and fibrosis contribute to respiratory muscle fatigue and can lead to the development of respiratory failure in CF The goal of treatment is to optimize gas exchange and acid–base balance, keeping in mind that these patients may have some degree of pulmonary hypertension and cor pulmonale Management includes maintaining adequate oxygenation and ventilation along with intensifying antibacterial treatment and airway clearance Supplemental oxygen should be introduced with caution in patients with chronic CO2 retention to avoid suppressing hypoxic ventilator drive Ventilation support may be necessary, and noninvasive means can be considered including CPAP or BiPAP The patient should also be evaluated for comorbidities (e.g., atypical infections, ABPA, pneumothorax), which can be precipitating events for acute or subacute decompensation Clinical Indications for Discharge or Admission Indications for hospitalization will vary according to the acute conditions diagnosed and underlying severity of disease Consultation with a pulmonologist or the patient’s CF team may provide insight into the need for hospitalization Follow-Up Care The majority of CF patients in the United States are followed in CFF approved and supported centers All centers’ contact information is available at the CFF website (http://www.cff.org ) Close contact between emergency physicians and the CF center team caring for this patient population facilitates acute management and continuity of care PULMONARY HEMORRHAGE CLINICAL PEARLS AND PITFALLS Pulmonary hemorrhage may be seen in acute respiratory illnesses or after thoracic trauma, the initial presentation of a chronic vasculitic condition, or during exacerbation of a chronic condition such as CF Pulmonary hemorrhage can present with significant respiratory distress and hemodynamic compromise Management primarily involves assessment and support of oxygenation, ventilation, and hemodynamics, with consideration of diagnostic testing for the underlying condition Bronchoscopy is generally reserved for patients with persistent bleeding or when diagnostically necessary Blood products including packed red blood cells, platelets, coagulation factors, and fresh-frozen plasma should be considered if signs of significant bleeding and/or shock are present Current Evidence Pulmonary hemorrhage, or bleeding into the lung, most commonly manifests clinically with hemoptysis Although relatively uncommon, pulmonary hemorrhage can be dramatic and life threatening Therefore, early evaluation and treatment is paramount The potential causes vary and include acute infection, exacerbation of chronic pulmonary disease, vascular malformations, and thoracic trauma among others The relative frequency of causative etiologies will vary significantly by the population being evaluated, making estimation of incidence of disease difficult Table 99.6 provides a differential diagnosis for pulmonary hemorrhage by category Goals of Treatment Management for pulmonary hemorrhage involves support of oxygenation and ventilation and attention to hemodynamics, along with appreciation of the underlying disorder for which other specific therapies may be necessary TABLE 99.6 CAUSES OF PULMONARY HEMORRHAGE IN CHILDREN Clinical Considerations Clinical Recognition Pulmonary hemorrhage results from pathology of lung tissue which can occur in the setting of acute infection such as pneumonia, exacerbation of chronic disease such as CF, or acute localized injury after thoracic trauma Hemoptysis is the most common symptom and finding Patients may have significant respiratory distress and may exhibit signs of hemorrhagic shock Triage Children with pulmonary hemorrhage require prompt assessment as they may present with severe respiratory distress and hemorrhagic shock Initial Assessment/H&P Hemoptysis is the most common presentation of pulmonary hemorrhage It may be necessary to distinguish this from hematemesis or blood from the nose, tonsils, pharynx, or upper airway Findings may be mild with blood-streaked sputum, or patients may present with massive blood loss and shock Hypoxia and shortness of breath are most likely with significant hemorrhage or as a result of exacerbation of underlying condition Apprehension is not uncommon in these children as dyspnea is compounded by the visualization of blood loss Children with recurrent intrapulmonary bleeding are more likely to be anemic As a result, they may also present with fatigue and poor weight gain as symptoms Examination findings are often nonspecific and include tachypnea, tachycardia, and hypoxia Crackles may be appreciated over the affected area, although isolating the location of the bleeding by auscultation is difficult For older patients, identifying the affected area may be best accomplished by asking the patient where they feel pain or discomfort Other signs on examination may be helpful in elucidating an underlying diagnosis, such as abnormal cardiac sounds with heart failure, rash or joint involvement with collagen vascular disease, or external signs of thoracic injury in trauma patients Cardiorespiratory decompensation can occur in children with severe anemia or shock from severe hemorrhage Radiographs will vary depending on etiology Alveolar infiltrates may be transient localized processes, or diffuse and chronic In idiopathic pulmonary hemosiderosis, diffuse alveolar changes are usually symmetric and spare the apices and costophrenic angles (See Fig 99.4 ) Because most children swallow their sputum, a presumptive diagnosis can be made by finding hemosiderin-laden macrophages in nasogastric washings; these macrophages will stain blue with Prussian blue reaction More definitive diagnosis, however, requires bronchoscopy and bronchoalveolar lavage (BAL) Finding similar macrophages from BAL is diagnostic, and direct visualization of the airways provides an opportunity to potentially localize the site and assess the activity of bleeding Lung biopsy is required only for patients with recurrent bleeding in whom no diagnosis can be made on a clinical basis and alternative systemic diseases cannot be excluded ... contact information is available at the CFF website (http://www.cff.org ) Close contact between emergency physicians and the CF center team caring for this patient population facilitates acute