| Capillary apposition and density in the diagnosis of alveolar capillary dysplasia. | |
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MedLine Citation:
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PMID: 18983610 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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AIM: Alveolar capillary dysplasia (ACD) is a rare disorder, typically presenting with persistent pulmonary hypertension of the newborn. The aim was to characterise further the histological features of patients suspected of having ACD and to correlate histopathological features with outcome. METHODS AND RESULTS: Three pathologists retrospectively reviewed 21 surgical lung biopsy specimens (SLBx) where ACD entered the differential diagnosis. Semi-quantitative assessment showed that there was a spectrum of muscular arterial hypertrophy, capillary apposition to epithelium and capillary density within the interstitium, with the latter being more disordered in ACD. Misalignment of pulmonary vessels was also frequently seen. Four of 19 patients survived beyond the neonatal period, these having higher degrees of capillary apposition and density. Associated extrapulmonary abnormalities were common, most frequently with ACD. CONCLUSION: Poor capillary apposition and density, allied with medial arterial hypertrophy and misalignment of pulmonary vessels are the strongest diagnostic features of ACD. Of the four patients alive, all had high capillary apposition and density, suggesting that these features may be of prognostic value. SLBx remains useful in such cases as it may help predict patients who survive the neonatal period and also identify patients with disorders that are not primarily vascular anomalies. |
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Authors:
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L Melly; N J Sebire; M Malone; A G Nicholson |
Publication Detail:
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Type: Journal Article |
Journal Detail:
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Title: Histopathology Volume: 53 ISSN: 1365-2559 ISO Abbreviation: Histopathology Publication Date: 2008 Oct |
Date Detail:
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Created Date: 2008-11-05 Completed Date: 2009-01-29 Revised Date: 2010-05-12 |
Medline Journal Info:
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Nlm Unique ID: 7704136 Medline TA: Histopathology Country: England |
Other Details:
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Languages: eng Pagination: 450-7 Citation Subset: IM |
Affiliation:
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Department of Histopathology, Royal Free Hospital, London, UK. lucy.melly@royalfree.nhs.uk |
Export Citation:
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APA/MLA Format Download EndNote Download BibTex |
| MeSH Terms | |
Descriptor/Qualifier:
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Abnormalities, Multiple
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diagnosis,
pathology* Biopsy Capillaries / abnormalities*, pathology Humans Infant Infant, Newborn Lung / blood supply, pathology Persistent Fetal Circulation Syndrome / pathology* Pulmonary Alveoli / abnormalities*, blood supply*, pathology Pulmonary Artery / abnormalities, pathology Pulmonary Veins / abnormalities, pathology Retrospective Studies |
| Comments/Corrections | |
Comment In:
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Histopathology. 2010 Feb;56(3):401-2; author reply 402-3
[PMID:
20459541
]
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From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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