1989
DOI: 10.1177/000331978904000812
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Pulmonary Hypertension in the CREST Syndrome: Variant of Systemic Sclerosis (Scleroderma) — A Case Report

Abstract: The CREST syndrome (calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly, telangiectasia) is a variant but not a benign form of scleroderma. Pulmonary hypertension without pulmonary fibrosis appears to be more prevalent in the CREST than in scleroderma patients. Moreover, pulmonary hypertension in CREST may be rapidly progressive and a cause of sudden death from severe cor pulmonale.

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Cited by 8 publications
(2 citation statements)
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“…BSG 45/70 [10/18] mm n.W., Hb 7,7 g/dl (6,6; nach Gabe von 2 Ery-Konzentraten 9,4) [13, [5][6][7][8][9][10][11][12][13][14][15][16][17], Hb E 19 pg (24) [28][29][30][31][32][33][34], Ery 3,86 Mio./µl [4,[3][4][5]7], Retikulozyten 19/1000 Erys [3][4][5][6][7][8][9][10][11][12][13][14][15], Leuko 5200/µl [3800-10500],Thrombo 182000/µl [140000-345000], AP 418 U/l (319) [65-220], Gamma-GT 255 U/l (121) …”
Section: Labor (Kontrollwerte) [Normwerte]unclassified
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“…BSG 45/70 [10/18] mm n.W., Hb 7,7 g/dl (6,6; nach Gabe von 2 Ery-Konzentraten 9,4) [13, [5][6][7][8][9][10][11][12][13][14][15][16][17], Hb E 19 pg (24) [28][29][30][31][32][33][34], Ery 3,86 Mio./µl [4,[3][4][5]7], Retikulozyten 19/1000 Erys [3][4][5][6][7][8][9][10][11][12][13][14][15], Leuko 5200/µl [3800-10500],Thrombo 182000/µl [140000-345000], AP 418 U/l (319) [65-220], Gamma-GT 255 U/l (121) …”
Section: Labor (Kontrollwerte) [Normwerte]unclassified
“…[5,7,9]. Die gastroskopisch gefundenen Teleangiektasien sind nicht auf Venenkonvolute zurückzuführen, wie sie bei portalen Umgehungskreisläufen vorhanden wären.…”
Section: Endosonographie Des Oberen Gastrointestinaltraktesunclassified