Document Detail

Capillary apposition and density in the diagnosis of alveolar capillary dysplasia.
MedLine Citation:
PMID:  18983610     Owner:  NLM     Status:  MEDLINE    
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.
L Melly; N J Sebire; M Malone; A G Nicholson
Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  Histopathology     Volume:  53     ISSN:  1365-2559     ISO Abbreviation:  Histopathology     Publication Date:  2008 Oct 
Date Detail:
Created Date:  2008-11-05     Completed Date:  2009-01-29     Revised Date:  2010-05-12    
Medline Journal Info:
Nlm Unique ID:  7704136     Medline TA:  Histopathology     Country:  England    
Other Details:
Languages:  eng     Pagination:  450-7     Citation Subset:  IM    
Department of Histopathology, Royal Free Hospital, London, UK.
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MeSH Terms
Abnormalities, Multiple / diagnosis,  pathology*
Capillaries / abnormalities*,  pathology
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
Comment In:
Histopathology. 2010 Feb;56(3):401-2; author reply 402-3   [PMID:  20459541 ]

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