HELLP-SYNDROME
Abstract
The pathophysiology of HELLP syndrome is not well defined. Nowadays endothelial dysfunction if considered the key moment of the development of HELLP-syndrome. Endothelial cell dysfunction results in hypertension, proteinuria, and increased platelet activation and aggregation. Furthermore, activation of the coagulation cascade causes consumption of platelets due to adhesion onto a damaged and activated endothelium, in addition to microangiopathic hemolysis caused by shearing of erythrocytes as they traverse through capillaries laden with platelet-fibrin deposits. Multiorgan microvascular injury and hepatic necrosis causing liver dysfunction contribute to the development of HELLP.
About the Authors
A. D. Makatsariya
First Moscow State Medical Sechenov University of the Ministry of Health Russian Federation
Russian Federation
Russian Federation
V. O. Bitsadze
First Moscow State Medical Sechenov University of the Ministry of Health Russian Federation
Russian Federation
Russian Federation
D. Kh. Khizroeva
First Moscow State Medical Sechenov University of the Ministry of Health Russian Federation
Russian Federation
Russian Federation
References
1. Abramovici D., Friedman S.A., Mercer B.M. et al. Neonatal outcome in severe preeclampsia at 24 to 36 weeks’ gestation: does the HELLP (hemolysis, elevated liver enzymes, and low platelet count) syndrome matter? Am. J. Obstet. Gynecol. 1999; 180: 221-225.
2. Altamura C., Vasapollo B., Tibuzzi F. et al. Postpartum cerebellar infarction and haemolysis, elevated liver enzymes, low platelet (HELLP) syndrome. Z. Neurol. Sci. 2005; 26 (1): 40-2.
3. Barton J.R., Riely С.A., Adamec Т.А. et al. Hepatic histopathologic condition does not correlate with laboratory abnormalities in HELLP syndrome (hemolysis, elevated liver enzymes, and low platelet count. Am. J. Obstet. Gynecol. 1992; 167: 1538-1543.
4. Barton J.R., Sibai B.M. Care of the pregnancy complicated by HELLP syndrome. Obstet. Gynecol. Clin. North. Am. 1991; 18: 165-179.
5. Baxter J.K., Weinstein L. HELLP syndrome: the state of the art. Obstet. Gynecol. Surv. 2004; 59 (12): 838-45.
6. Brandenburg V.M., Frank R.D., Heintz В. et al. HELLP syndrome, multifactorial thrombophilia and postpartum myocardial infarction. J. Perinat. Med., 2004; 32 (2): 181-3.
7. Chames M.C., Haddad B., Barton J.R. et al. Subsequent pregnancy outcome in women with a history of HELLP syndrome at 28 weeks of gestation. Am. J. Obstet. Gynecol. 2003; 188: 1504-1508.
8. Clark S.L., Phelan J.R., Allen S.H. et al. Antepartum reversal of hematologic bnormalities associated with the HELLP syndrome: a report of three cases. J. Reprod. Med. 1986; 31: 70-72.
9. Eeltink C.M., van Lingen R.A., Aarnoudse J.G. et al. Maternal haemolysis, elevated liver enzymes and low platelets syndrome: specific problems in the newborn. Eur. J. Pediatr. 1993; 152: 160-163.
10. Egerman R.S., Sibai B.M. HELLP syndrome. Clin. Obstet. Gynecol. 1999; 42: 381-389.
11. Goodlin R.C., Cotton D.B., Haesslein H.C. Severe edema-proteinuria-hypertension gestosis. Am. J. Obstet. Gynecol. 1978; 32: 595-598.
12. Goodlin R.C. Preeclampsia as the great impostor. Am. J. Obstet. Gynecol. 1991; 164: 1577-1581.
13. Isler C.M., Barrilleaux P.S., Magann E.F. et al. A prospective, randomized trial comparing the efficacy of dexamethasone and betamethasone for the treatment of antepartum HELLP (hemolysis, elevated liver enzymes, and low platelet count syndrome. Am. J. Obstet. Gynecol. 2001; 184: 1332-1339.
14. Katz V.L., Farmer R., Kuler J.A. Preeclampsia into eclampsia: Towards a new paradigm. Am. J. Obstaet. Gynecol. 2000; 182: 1389-1394.
15. Koenig M., Roy M., Baccot S. et al.Thrombotic microangiopathy with liver, gut, and bone infarction (catastrophic antiphospholipid syndrome) associated with HELLP syndrome. Clin. Rheumatol. 2005; 24 (2); 166-8.
16. Krauss T., Augustin H.G., Osmers R. et al. Activated protein resistance and factor V Leiden in patients with hemolysis, elevated liver enzymes, low platelets syndrome. Obstet. Gynecol. 1998; 92: 457-460.
17. Le T.T.D., Tieulie N., Costedoat N. et al. The HELLP syndrome in the antiphospholipid syndrome: retrospective study of 16 cases in 15 women. Ann. Rheum. Dis. 2005; 64: 273-278.
18. Magann E.F., Bass D., Chauhan S.P. et al. Antepartum corticosteroids: disease stabilization in patients with the syndrome of hemolysis, elevated liver enzymes, and low platelets (HELLP). Am. J. Obstet. Gynecol. 1994; 71: 1148-1153.
19. Magann E.F., Perry K.G., Meydrech E.F. et al. Postpartum corticosteroids: accelerated recovery from the syndrome of hemolysis, elevated liver enzymes, and low platelets (HELLP). Am. J. Obstet. Gynecol. 1994; 171: 1154-1158.
20. Martin J.N. Jr., Blake P.G., Perry K.G. et al. The natural history of HELLP syndrome: > patterns of disease progression and regression. Am. J. Obstet. Gynecol. 1991; 164: 1500-1513.
21. Minakami H., Oka N., Sato T. et al. Preeclampsia: a microvesicular fat disease of the liver? Am. J. Obstet. Gynecol. 1988; 159: 1043-1047.
22. Moessmer G., Muller B., Kolben M. et al. HELLP syndrome with fetal growth retardation in a woman homozygous for the prothrombin gene variant 20210A. Thromb. Haemost. 2005; 93 (4): 787-8.
23. O'Brien J.M., Barton J.R. Controversies with the diagnosis and management of HELLP syndrome. Clin. Obstet. Gynecol. 2005; 48 (2): 460-77.
24. Osmanagaoglu M.A., Osmanagaoglu S., Bozkaya H. Systemic lupus erythematosus complicated by HELLP syndrome. Anaesth. Intensive Care. 2004; 32 (4): 569-74.
25. Schlembach D., Beinder E., Zingsem J. et al. Association of maternal and/or fetal factor V Leiden and G20210A prothrombin mutation with HELLP syndrome and intrauterine growth restriction. Clin. Sci (Lond). 2003; 105 (3): 279-85.
26. Sibai B.M., Ramadan M.K., Usta I. et al. Maternal morbidity and mortality in 442 pregnancies with hemolysis, elevated liver enzymes, and low platelets (HELLP syndrome). Am. J. Obstet. Gynecol. 1993: 169: 1000-1006.
27. Sibai B.M., Ramadan M.K., Chari R.S. et al. Pregnancies complicated by HELLP syndrome (hemolysis, elevated liver enzymes, and low platelets): subsequent pregnancy outcome and long-term prognosis. Am. J. Obstet. Gynecol. 1995; 172: 125-129.
28. Sullivan С.A., Magann E.F., Perry K.G. et al. The recurrence risk of the syndrome of hemolysis, elevated liver enzymes, and low platelets (HELLP) in subsequent gestations. Am. J. Obstet. Gynecol. 1994; 171: 940-943.
29. Tanner B. Ohler W.G., Hawighorst S., Schaffer U., Knapstein P.G. Complications in HELLP syndrome due to peripartal hemostatic disorder. Zentralbl. Gynakol. 1996; 118 (4): 213-20.
30. VanPampus M.G., Wolf H., Westenberg S.M. et al. Maternal and perinatal outcome after expectant management of the HELLP syndrome compared with preeclampsia without HELLP syndrome. Eur. J. Obstet. Gynecol. Reprod. Biol. 1998; 76: 31-36.
31. Wiebers D.O. Ischemic cerebrovascular complications of pregnancy. Arch. Neurol. 1985; 2: 1106-1113.
32. Witsenburg C.P., Rosendaal F.R., Middeldorp J.M. et al. Factor VIII levels and the risk of preeclampsia, HELLP syndrome, pregnancy related hypertension and severe intrauterine growth retardation. Thromb. Res. 2005; 115 (5): 387-92.
33. Yalcin О.Т., Sener T., Hassa H. et al. Effects of postpartum corticosteroids in patients with HELLP syndrome. Int. J. Gynaecol. Obstet. 1998; 61: 141-148.
Review
For citations:
Makatsariya A.D., Bitsadze V.O., Khizroeva D.Kh. HELLP-SYNDROME. Obstetrics, Gynecology and Reproduction. 2014;8(2):61-68. (In Russ.)
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