Observations on the Relationship of Desquamative Interstitial Pneumonia and Pulmonary Alveolar Proteinosis in Childhood: A Pathologic and Experimental Study

Bhagwat, A G; Wentworth, Paul; Conen, Patrick E.

doi:10.1378/chest.58.4.326

Cited by 35 publications

(12 citation statements)

References 13 publications

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“…and Maygarden et al . also demonstrate lung pathology with features overlapping between DIP and PAP 18,19 . We did not find reports of idiopathic or secondary alveolar lipoproteinosis in adult patients that showed abundant alveolar macrophages admixed with the lipoproteinaceous fluid, and we did not find any prior reports of cases that shared the histopathological, histochemical, and ultrastructural features exhibited by the five present cases.…”

Section: Discussioncontrasting

confidence: 49%

Variant alveolar lipoproteinosis: A syndrome with distinct clinical and pathological features

Nishino

Medoff

Mark³

et al. 2011

Pathology International

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Pulmonary alveolar proteinosis (PAP) is a rare condition in which pulmonary macrophages fail to clear surfactant, resulting in the alveolar accumulation of lipoproteinaceous debris. The histopathology of PAP is typified by diffuse filling of terminal airways with eosinophilic, PAS/diastase (PAS/D)-positive acellular material. We present five patients who showed histopathological changes in the lungs consistent with mild PAP. However, these cases were notable for the abundance of degenerating alveolar macrophages, weak PAS staining of lipoproteinaceous material and paucity of lamellated bodies on ultrastructural examination. Only one patient showed the CT finding of mosaiform 'crazy-paving' and the opalescent bronchoalveolar lavage fluid characteristic of PAP. In one case, therapeutic lung lavage based on a presumptive diagnosis of PAP exacerbated respiratory distress. Three patients showed partial or near-complete resolution of disease in response to high-dose corticosteroid therapy, a treatment approach that is generally ineffective in PAP. We conclude that distinguishing 'variant alveolar lipoproteinosis' from classical PAP is clinically important. Despite the otherwise typical appearance of lipoproteinaceous alveolar material in lung biopsies, the presence of degenerating foamy macrophages and atypical histochemical, ultrastructural and radiographic features suggest a steroid-responsive form of proteinosis that is likely pathogenetically distinct and may not be amenable to whole-lung lavage.

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Section: Discussioncontrasting

confidence: 49%

Variant alveolar lipoproteinosis: A syndrome with distinct clinical and pathological features

Nishino

Medoff

Mark³

et al. 2011

Pathology International

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“…The ratios of protein and phospholipid to DNA in the precipitate fraction were 10.7-fold and 54.5-fold higher than those of rat lung parenchyma. These findings suggest that the major materials accumulating in the alveoli of patients with alveolar proteinosis may be materials normally existing in the alveoli, and may not mainly consist of cell components such as alveolar macrophages, although the materials found in the alveoli of this disease are known to contain several kinds of cells and desquamative materials from the alveolar wall such as type II cells (Kuhn et al 1966;Bhagwat et al 1970).…”

Section: Protein Analysismentioning

confidence: 99%

Biochemical characterization of pulmonary washings of patients with alveolar proteinosis, interstitial pneumonitis and alveolar cell carcinoma.

Onodera

Nakamura

Sato

et al. 1983

Tohoku J. Exp. Med.

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“…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]. In contrast to our patient, Bhagwat et al's patient benefited from corticoids.…”

Section: Discussionmentioning

confidence: 51%

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

et al. 1986

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A desquamative, interstitial pneumonitis was diagnosed histologically in a 9-month-old boy who first became ill at the age of 5 weeks. The desquamative interstitial pneumonitis was associated with an acquired cytomegalovirus (CMV) infection. Despite treatment with corticoids, acyclovir and artificial ventilation, the patient died of pulmonary insufficiency at the age of 15 months. The autopsy revealed an alveolar lipoproteinosis.

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Observations on the Relationship of Desquamative Interstitial Pneumonia and Pulmonary Alveolar Proteinosis in Childhood: A Pathologic and Experimental Study

Cited by 35 publications

References 13 publications

Variant alveolar lipoproteinosis: A syndrome with distinct clinical and pathological features

Variant alveolar lipoproteinosis: A syndrome with distinct clinical and pathological features

Biochemical characterization of pulmonary washings of patients with alveolar proteinosis, interstitial pneumonitis and alveolar cell carcinoma.

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

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