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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.