[Importance of lung function for pulmonary hypertension in sarcoidosis]. / Bedeutung der Lungenfunktion für die pulmonale Hypertonie bei Sarkoidose.

BACKGROUND: The reported prevalence of pulmonary hypertension (PH) in sarcoidosis is variable and depends on the ethnic background, the underlying pathophysiology and the diagnostic methods used. We aimed to analyse the prevalence and the severity of PH and its relationship to ventilation and gas exchange in sarcoidosis. METHODS: We performed a retrospective analysis of echocardiography, pulmonary function tests, blood gases and right heart catheterisation of 123 patients with sarcoidosis. RESULTS: 5.6 % of all patients showed pulmonary hypertension. Parenchymal lung abnormalities (seen in 59.4 % of all patients) had no influence on PH. In patients with PH VC (p < 0.001), FEV1 (p < 0.001), FEV1/VC (p = 0.01), TLCO/VA (p < 0.001), pO2 (p = 0.04) and SO2 (p < 0.01), but not ITGV, TLC and RV were significantly lower. We found significant negative correlations for right ventricular systolic pressure (RVSP) and VC (R = -0.52, p < 0.01), FEV1 (R = -0.53, p < 0.01), DLCO-VA (R = -0.55, p = 0.02), pO2 (R = -0.69, p = 0.04) and SO2 (R = -0.91, p < 0.001). CONCLUSION: Prevalence and severity of PH were similar to published Japanese data, but were less than reported in African-Americans. PH was associated with decreased vital capacity, forced exspiratory volume at one second and oxygenation; however, it was independent from parenchymal abnormalities. The influence of power of breathing on VC and FEV1 and its correlation with RVSP should be evaluated in a prospective trial.

Human bronchus and intestine express the same mucin gene.

The amino acid and sugar composition of mucins from various organs is similar but not identical. This could arise by one or more of the following: organ-specific processing of a single core protein, organ-specific splicing of a single mucin mRNA, or organ-specific expression of various mucin genes. To begin to investigate the source of this variability, we examined (a) immunological cross-reactivity and (b) cDNA cross-hybridization, among several mucin-secreting organs of the human body. Peptide-directed antibodies raised against both nondeglycosylated (LS) and deglycosylated (HFB) intestinal mucin strongly stained mucous cells in the bronchial epithelium and submucosal glands, indicating homology between mucins of the bronchus and intestine at the peptide level. By screening a bronchus cDNA library with an intestinal mucin cDNA, SMUC-41, we isolated a bronchus mucin cDNA, HAM-1. This cDNA is 96% homologous to the first repeat of SMUC-41. HAM-1 hybridized to restriction fragments of human genomic DNA identical to those hybridizing to SMUC-41 on Southern blots. SMUC-41 also hybridized to polydisperse transcripts in the bronchus, cervix, gall bladder, and mammary gland, indicating mucin homology among all these organs at the RNA level. We conclude that the bronchus and intestine express a common mucin gene, which is likely co-expressed by at least several other mucin-secreting organs.

Inhalation of aerosolized vitamin a: reversibility of metaplasia and dysplasia of human respiratory epithelia -- a prospective pilot study.

The objective of this preliminary uncontrolled study was twofold: First, to assess the feasibility of retinyl palmitate inhalation and second, to analyze the changes of metaplastic lesions of the respiratory epithelium (metaplasia or dysplasia) following retinyl palmitate inhalation. The response to a daily dose of 18.000 I.U. retinyl palmitate by inhalation over a period of 3 month was assessed in 11 subjects (9 smokers, 2 ex-smokers). Using white-light bronchoscopy combined with autofluorescence bronchoscopy, bronchial biopsies were taken before and after a 3 month-period. The biopsy samples were evaluated blind by a referee lung pathologist. The overall response rate (remission or partial remission) was 56% (95% CI 0.30 0.79; p<0.05). These data suggest that inhalation of retinyl esters could be a promising therapeutical approach for chemoprevention of lung cancer. Vitamin A; chemoprevention; lung cancer; squamous metaplasia; dysplasia; retinoids