Dtsch Med Wochenschr 2007; 132(3): 103-107
DOI: 10.1055/s-2007-959297
Übersicht | Review article
Kardiologie, Pneumologie
© Georg Thieme Verlag KG Stuttgart · New York

Pulmonale Hypertonie bei Sichelzellkrankheiten

Epidemiologie, Pathogenese, Diagnostik und TherapiePulmonary hypertension in sickle cell diseaseEpidemiology, pathogenesis, diagnosis and treatmentG. Maschmeyer1 , A. Haas1 , R. Dickerhoff2 , F.-X Kleber3
  • 1Klinikum Ernst von Bergmann, Medizinische Klinik, Abt. Hämatologie und Onkologie, Potsdam
  • 2Asklepios Klinik Sankt Augustin GmbH, Abt. f. Allg. Kinder- und Jugendheilkunde, Sankt Augustin
  • 3Unfallkrankenhaus Berlin, Klinik für Innere Medizin, Berlin
Weitere Informationen

Publikationsverlauf

eingereicht: 19.6.2006

akzeptiert: 26.10.2006

Publikationsdatum:
12. Januar 2007 (online)

Literatur

  • 1 Adams R J, Brambilla D. Optimizing Primary Stroke Prevention in Sickle Cell Anemia (STOP 2) Trial Investigators . Discontinuing prophylactic transfusions used to prevent stroke in sickle cell disease.  N Engl J Med. 2005;  353 2769-2778
  • 2 Aidoo M, Terlouw D J, Kolczak M S. et al . Protective effects of the sickle cell gene against malaria morbidity and mortality.  Lancet. 2002;  359 1311-1312
  • 3 ATS Committee on Proficiency Standards for Clinical Pulmonary Function Laboratories . ATS statement: guidelines for the six-minute walk test.  Am J Resp Crit Care Med. 2002;  166 111-117
  • 4 Bachir D. Prise en charge de l’adulte drépanocytaire. Management of adults with sickle cell anemia.  Pathol Biol. 1999;  47 39-45
  • 5 Badesch D B, McLaughlin V V, Delcroix M. et al . Prostanoid therapy for pulmonary arterial hypertension.  J Am Coll Cardiol. 2004;  43 56s-61s
  • 6 Barst R J, Langleben D, Frost A. et al . Sitaxsentan therapy for pulmonary arterial hypertension.  Am J Respir Crit Care Med. 2004;  169 441-447
  • 7 Castro V, Alberto F L, Costa R N. et al . Polymorphism of the human platelet antigen-5 system is a risk factor for occlusive vascular complications in patients with sickle cell anemia.  Vox Sang. 2004;  87 118-123
  • 8 Castro O, Hoque M, Brown B D. Pulmonary hypertension in sickle cell disease: cardiac catheterization results and survival.  Blood. 2003;  101 1257-1261
  • 9 Charache S, Terrin M L, Moore R D. et al . Effect of hydroxyurea on the frequency of painful crises in sickle cell anemia. Investigators of the Multicenter Study of Hydroxyurea in Sickle Cell Anemia.  N Engl J Med. 1995;  332 1317-1322
  • 10 Charache S, Dover G J, Moore R D. et al . Hydroxyurea: effects on hemoglobin F production in patients with sickle cell anemia.  Blood. 1992;  79 2555-2565
  • 11 Deng Z, Morse J H, Slager S L. et al . Familial primary pumonary hypertension (Gene PPH-1) is caused by mutations in the bone morphogenetic protein receptor-II gene.  Am J Hum Genet. 2000;  67 737-744
  • 12 Dickerhoff R, Mahlberg R. Leitlinie Sichelzellkrankheiten der Deutschen Gesellschaft für Hämatologie und Onkologie,. www.dgho.de 2005
  • 13 Gladwin M T, Rodgers G P. Pathogenesis and treatment of acute chest syndrome of sickle-cell anaemia.  Lancet. 2000;  355 1476-1478
  • 14 Gladwin M T, Schechter A N. Nitric oxide in sickle cell disease.  Semin Hematol. 2001;  38 333-342
  • 15 Gladwin M T, Sachdev V, Jison M L. et al . Pulmonary hypertension as a risk factor for death in patients with sickle cell disease.  N Engl J Med. 2004;  350 886-895
  • 16 Haque A K, Gokhale S, Rampy B A, Adeboyega P, Duarte A, Saldana M J. Pulmonary hypertension in sickle cell hemoglobinopathy. A clinicopathologic study of 20 cases.  Hum Pathol. 2002;  33 1037-1043
  • 17 Hess J R, MacDonald V W, Brinkley W W. Systemic and pulmonary hypertension after resuscitation with cell-free hemoglobin.  J Appl Physiol. 1993;  74 1769-1778
  • 18 Houston P E, Rana S, Sekhasaria S, Perlin E, Kim K S, Castro O L. Homocysteine in sickle cell disease: relationship to stroke.  Am J Med. 1997;  103 192-196
  • 19 Lane K B, Machado R D, Pauciulo M W. et al . Heterozygous germline mutations in BMPR2, encoding a TGF-beta receptor, cause familial primary pulmonary hypertension. The International PPH consortium.  Nat Genet. 2000;  26 81-84
  • 20 Machado R F, Gladwin M T. Chronic sickle cell lung disease: new insights into the diagnosis, pathogenesis and treatment of pulmonary hypertension.  Br J Haematol. 2005;  129 449-464
  • 21 Machado R F, Martyr S, Kato G J. et al . Sildenafil therapy in patients with sickle cell disease and pulmonary hypertension.  Br J Haematol. 2005;  130 445-453
  • 22 Minneci P C, Deans K J, Zhi H. et al . Hemolysis-associated endothelial dysfunction mediated by accelerated NO inactivation by decompartmentalized oxyhemoglobin.  J Clin Invest. 2005;  115 3409-3417
  • 23 Morris C R, Kato G J, Poljakovic M. et al . Dysregulated arginine metabolism, hemolysis-associated pulmonary hypertension, and mortality in sickle cell disease.  JAMA. 2005;  294 81-90
  • 24 Nahavandi M, Tavakkoli F, Wyche M Q, Perlin E, Winter W P, Castro O. Nitric oxide and cyclic GMP levels in sickle cell patients receiving hydroxyurea.  Br J Haematol. 2002;  119 855-857
  • 25 Olschewski H, Simonneau G, Galiè N. et al . Inhaled iloprost in severe pulmonary hypertension.  N Engl J Med. 2002;  347 322-327
  • 26 Platt O S, Brambilla D J, Rosse W F. et al . Mortality in sickle cell disease. Life expectancy and risk factors for early death.  N Engl J Med. 1994;  330 1639-1644
  • 27 Reiter C D, Wang X, Tanus-Santos J E. et al . Cell-free hemoglobin limits nitric oxide bioavailability in sickle-cell disease.  Nat Med. 2002;  8 1383-1389
  • 28 Rother R P, Bell L, Hillmen P, Gladwin M T. The clinical sequelae of intravascular hemolysis and extracellular plasma hemoglobin: a novel mechanism of human disease.  JAMA. 2005;  293 1653-1662
  • 29 Schenk P, Madl C, Kramer I. et al . Acute vasodilatator testing in primary and secondary hypertension.  Wien Klin Wochenschr. 2001;  113 76-81
  • 30 Serjeant G R. Sickle-cell disease.  Lancet. 1997;  350 725-730
  • 31 Setty B N, Stuart M J. Vascular cell adhesion molecule-1 is involved in mediating hypoxia-induced sickle red blood cell adherence to endothelium: Potential role in sickle-cell disease.  Blood. 1996;  88 2311-2320
  • 32 Simonneau G, Galie N, Rubin L J. et al . Clinical classification of pulmonary hypertension.  J Am Coll Cardiol. 2004;  (Suppl S) 43 5S-12S
  • 33 Steinberg M H. Management of sickle cell disease.  N Engl J Med. 1999;  340 1021-1030
  • 34 Steinberg M H, Barton F, Castro O. et al . Effect of hydroxyurea on mortality and morbidity in adult sickle cell anemia: risks and benefits up to 9 years of treatment.  JAMA. 2003;  289 1645-1651
  • 35 Stuart M J, Nagel R L. Sickle cell disease.  Lancet. 2004;  364 1343-1360
  • 36 Styles L A, Schalkwijck C G, Aarsmann A J, Vichinsky E P, Lubin B H, Kuypers F A. Phospholipase A2 levels in acute chest syndrome of sickle cell disease.  Blood. 1996;  87 2573-2578
  • 37 Styles L A, Aarsmann A J, Vichinsky E P, Kuypers F A. Secretory phospholipase A(2) predicts impending acute chest syndrome in sickle cell disease.  Blood. 2000;  96 3276-3278
  • 38 Tanoue L T. Pulmonary hypertension in the collagen vascular diseases.  Semin Respir Crit Care Med. 2003;  24 287-296
  • 39 Vichinsky E P, Neumayr L D, Earles A N. et al . Causes and outcomes of the acute chest syndrome in sickle cell disease.  N Engl J Med. 2000;  342 1855-1865
  • 40 Vichinsky E. New therapies in sickle cell disease.  Lancet. 2002;  360 629-631

Prof. Dr. Georg Maschmeyer

Medizinische Klinik, Hämatologie und Onkologie, Klinikum Ernst von Bergmann

Charlottenstraße 72

14467 Potsdam

Telefon: 0331/2416001

Fax: 0331/2416000

eMail: gmaschmeyer@klinikumevb.de