Pediatric Alveolar Proteinosis Follow-up

Updated: Jan 16, 2021
  • Author: Danielle M Goetz, MD; Chief Editor: Girish D Sharma, MD, FCCP, FAAP  more...
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Follow-up

Further Outpatient Care

Monitor for disease progression. Increasing symptoms may warrant further lung lavage. Vigorous lung physiotherapy may be helpful.

The optimal imaging modality for serial follow-up remains unclear. Lee et al (1997) concluded that, although HRCT findings are most closely correlated with pulmonary function, plain chest radiography provides sufficient information in conjunction with clinical findings and PFT. [48]

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Further Inpatient Care

Admit patients for diagnostic workup and lung lavage. For affected neonates, lung transplantation should be considered early.

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Inpatient & Outpatient Medications

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  • Bronchodilators may be used if evidence of airway reactivity is present.

  • Mucolytics, including acetylcysteine, trypsin, and ambroxol, have all been used; their efficacy is unknown.

  • GM-CSF is useful in autoimmune PAP.

  • Although a high-calorie diet is essential, ensure that the carbohydrate load is not excessive because this may exacerbate respiratory difficulties by causing a high respiratory quotient and hence a high CO2 burden.

  • No medications are specifically contraindicated.

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Transfer

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  • Transfer to a tertiary center may be necessary for further diagnostic workup, supportive care ECMO, or lung transplantation.

  • In view of the rarity of this condition, consider early transfer to a tertiary level institution experienced in managing this condition.

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Deterrence/Prevention

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  • Smoking by, or around, the patient must be discouraged.

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Complications

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  • Respiratory failure

  • Multiple procedures for whole lung lavage

  • Death

  • Superinfection

  • Pulmonary fibrosis

  • Secondary amyloidosis

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Prognosis

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  • CAP: Without lung transplantation, mortality is almost inevitable in the neonatal age group. [18]

  • Acquired pulmonary alveolar proteinosis (PAP): Analysis of published data suggested an actuarial 5-year disease-specific survival rate of 88% ±5%. The 5-year survival rate for children younger than 5 years was 14% ±13%, although this rate represented data from 7 children. [18] Many investigators have reported spontaneous remission in a select group of adults with acquired PAP. Seymour and Presneill (2002) reported 24 of 303 patients (7.9%) had spontaneous improvement. [18] Whether this improvement led to true resolution of the disease and restoration of lung function and surfactant clearance was unclear.

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Patient Education

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  • Patients with PAP may benefit from postural drainage, but no data support this hypothesis.

  • Smoking near the patient must be discouraged.

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