Childhood Pulmonary Alveolar Proteinosis (PAP)

Abstract

Pulmonary alveolar proteinosis is a rare disease in childhood, generally found in infants less than 1 year old. Survival time averages less than one year after the onset of symptoms, which consist mainly of cough and dyspnea. Of the 23 patients with pulmonary alveolar proteinosis, seven had thymic alymphoplasia (30%); this association leads to speculation that the immuno-logic system may be incompetent or contribute to the etiology of the disease. The diagnosis of pulmonary alveolar proteinosis is confirmed by biopsy, although chest roentgenograms and sputum staining may be helpful. Therapy consists of the use of steroids, antibiotics, and inhalation therapy; bronchopulmonary lavage has been unsuccessful in children. Si nce the original description of pulmonary alveolar proteinosis by Rosen et al1 in 1958, 139 adult cases have been reported.2 The dis¬ ease is rarer in childhood, and only 22 cases have been reported to date.1-3-12 We present the 23rd case and a review of the previous 22 cases. Report of a CaseA white girl, 7 years 2 months old, was first referred to this clinic when she was 6 years 2 months old because of pneumonia in the middle lobe of the right lung, leuko¬ penia, and fever. At the age of 5 years, she was given griseofulvin for a ring¬ worm-like lesion of the abdomen. At that time, leukopenia was first noted (white blood cell count [WBC] 3,000/cu mm). A biopsy of the lesion reportedly showed nonspecific vasculitis, and the child was treated with steroids for ten days. Dur¬ ing the next year, the lesion disappeared spontaneously, but upper-respiratory tract infection recurred and the leukopenia per¬ sisted.On first admission to our clinic, June 26, 1969, she was in moderate respiratory dis¬ tress and had a temperature of 103 F (39.4 C) and rales in both lung bases. A chest roentgenogram showed a diffuse in¬ terstitial infiltrate (Fig 1, left). Cultures of blood and bronchial washings were negative, and bone marrow examination showed no abnormality. Multiple skin tests for fungi and tubercle bacilli were negative. Tests for rheumatoid factor and lupus erythematosus cells were negative. Serum IgA, IgM, and IgG concentrations were, respectively, 1.45, 1.35, and 14.5 mg/100 ml. The leukopenia persisted (3,000 WBC /cu mm), with a normal differential cell count. Open lung biopsy showed a recent and an old intraalveolar hemorrhage suggestive of pulmonary hemosiderosis, as well as mild subacute and chronic interstitial pneumonitis. Gas¬ tric aspirates were negative for hemosiderin. Treatment consisted of ampicillin and supportive therapy. The fever sub¬ sided, and she was dismissed on July 3, at which time she was receiving 30 nig of prednisone per day. Three episodes of bronchitis, pneu¬ monitis, and fever occurred before her final admission on May 9, 1970, at the age of 7 years. Figure 1, center, shows a chest x-ray film that was taken during one of those episodes. At the final admission, the chief complaints were fever, dyspnea, cyanosis, and cough productive of clear sputum. There was no hemoptysis. ...

Pulmonary Alveolar Proteinosis and tuberculosis in a diabetic patient: a rare or a seldom diagnosed association

“…Almost 80% of the patients are between 20 and 50 years old, although it has also been described in newborns [33,34], school age children [35,36], and in the elderly [37]. The main symptom is exertion dyspnea.…”

Desquamative interstitial pneumonitis and alveolar lipoproteinosis: Diagnostic difficulties and therapy problems with an infant

“…In contrast to our patient, Bhagwat et al's patient benefited from corticoids. ALP is usually unresponsive to steroids and therefore our patient was presumably different from that of Bhagwat et al [3,11]. The only effective treatment of ALP is bronchopulmonary lavage with muco-or proteolytics, a procedure, which has only been used widely to date in adults.…”

“…This disease is named fibrosing alveolitis [6]. Like DIP in childhood, ALP occurs more frequently in infancy with a small peak in the newborn period [3,13,16]. A relationship between both diseases and the transition of DIP to ALP has been described by Bhagwat et al [1] and was also reviewed in the pathology literature [13].…”

“…The only effective treatment of ALP is bronchopulmonary lavage with muco-or proteolytics, a procedure, which has only been used widely to date in adults. The low success rate of lavage in children was attributed to the small size of the bronchial tree and to inadequate lavage volumes [3].…”

“…The fact that Carrington et al [2] have seen many spontaneous improvements in a large group of adults with DIP indicates that the onset of treatment seems to be tess important than the age at which the disease first strikes. The prognosis of ALP in childhood is very poor and lethality is 100% if the disease begins in infancy, unless effective lung layage can be performed [3].…”