0071643192.pdf

PEDIATRICS

TREATMENT

■ O 2 , inhaled bronchodilators, and corticosteroids

■ Some patients may also respond to diuretics.

■ IV fluids for patients who are dehydrated

■ Admit if hypoxic, there is a concurrent infection (such as RSV or pneumonia),

or if respiratory distress persists despite treatment.

COMPLICATIONS

■ Apnea, hypercarbia, or hypoxemia

■ Dehydration

■ Cor pulmonale

CYSTICFIBROSIS

Cystic fibrosis is the most common lethal congenital condition in the United

States, occurring in roughly 1 in 2500 Caucasians. The incidence in other

ethnic groups is 1 in 17,000. Although there are hundreds of different muta-

tions leading to CF, the most common is DeltaF508. The gene product

affected by all of these mutations is the CFTR protein, which regulates chlo-

ride transport across cell membranes. Patients with CF have a reduction in

chloride transport back into epithelial cells, resulting in thick secretions. As a

result, CF patients develop respiratory disease similar to early-onset COPD,

with additional deficiencies in the pancreatic exocrine function, male infertil-

ity, and abnormally elevated levels of chloride in sweat.

SYMPTOMS

■ Frequent lung and sinus infections

■ Failure to thrive due to malabsorption of fats

■ Increased incidence of rectal prolapse

■ Meconium ileus in infants

■ Fatty stools

EXAM

■ Increased AP diameter of the chest

■ Small size for age

DIAGNOSIS

■ CF is generally not diagnosed in the ED setting.

■ Sweat chloride testing

■ Serum electrolytes reveal low chloride and sodium levels.

TREATMENT

■ Antibiotics

■ Inhaled bronchodilators

■ Steroids

COMPLICATIONS

■ Respiratory failure

■ Hemoptysis

■ Dehydration, particularly during warm weather or exertion

■ Electrolyte abnormalities: Low serum chloride, low serum sodium, elevated

serum bicarbonate