Very early HELLP-Syndrom – vergeblicher Aktionismus oder geordneter Rückzug?

Das „Very early HELLP-Syndrom“ stellt die Geburtsmedizin vor besondere Herausforderungen. Es handelt sich dabei um die für ein HELLP-Syndrom typische auftretende Trias aus Hämolyse, ansteigender Leberwerte und einer Thrombozytopenie zu einem Zeitpunkt, an dem die Lebensfähigkeit des Kindes außerhalb des Mutterleibes noch nicht gegeben ist. Es sind vor allem die mütterlichen Risiken, die zu einer raschen Beendigung der Schwangerschaft drängen und demgegenüber der erklärte Wunsch betroffener Paare, das Ungeborene – unter Ausschöpfung aller medizinischer Möglichkeiten – in ein Gestationsalter zu begleiten, zu dem eine Maximalversorgung außerhalb des Mutterleibes möglich wird. Dabei darf nicht allein auf die Grenze der extrauterinen Lebensfähigkeit geschaut werden, sondern es muss zukunftsweisend abgewogen werden, unter welchen Voraussetzungen der Neonatologe eine Maximaltherapie bzw. Unterstützung des Kindes postpartal beginnen soll.

• Literatur

• 1 Weinstein L. Syndrome of hemolysis, elevated liver enzymes, and low platelet count: a severe consequence of hypertension in pregnancy. Am J Obstet Gynecol 1982; 142: 159-167
• 2 Geary M. The HELLP syndrome. Br J Obstet Gynaecol 1997; 104: 887-891
• 3 Karumanchi SA, Maynard SE, Stillman IE. et al. Preeclampsia: a renal perspective. Kidney Int 2005; 67: 2101-2113
• 4 Magann EF, Martin jr. JN. Twelve steps to optimal management of HELLP syndrome. Clin Obstet Gynecol 1999; 42: 532-550
• 5 Broekhuizen FF, Elejalde R, Hamilton PR. Early-onset preeclampsia, triploidy and fetal hydrops. J Reprod Med 1983; 28: 223-226
• 6 Alsulyman OM, Castro MA, Zuckerman E. et al. Preeclampsia and liver infarction in early pregnancy associated with the antiphospholipid syndrome. Obstet Gynecol 1996; 88 (4 Pt 2): 644-646
• 7 Sherer DM, Dalloul M, Stimphil R. et al. Acute onset of severe hemolysis, elevated liver enzymes, and low platelet count syndrome in a patient with a partial hydatidiform mole at 17 weeks gestation. Am J Perinatol 2006; 23: 163-166
• 8 Tsirigotis P, Mantzios G, Pappa V. et al. Antiphospholipid syndrome: a predisposing factor for early onset HELLP syndrome. Rheumatol Int 2007; 28: 171-174
• 9 Audibert F, Friedman SA, Frangieh AY. et al. Clinical utility of strict diagnostic criteria for the HELLP (hemolysis, elevated liver enzymes, and low platelets) syndrome. Am J Obstet Gynecol 1996; 175: 460-464
• 10 Barton JR, Sibai BM. Diagnosis and management of hemolysis, elevated liver enzymes, and low platelets syndrome. Clin Perinatol 2004; 31: 807-833 vii
• 11 Padden MO. HELLP syndrome: recognition and perinatal management. Am Fam Physician 1999; 60: 829-836 839
• 12 Sibai BM. Diagnosis and management of gestational hypertension and preeclampsia. Obstet Gynecol 2003; 102: 181-192
• 13 Sibai BM. Imitators of severe preeclampsia. Obstet Gynecol 2007; 109: 956-966
• 14 Berry EL, Iqbal SN. HELLP syndrome at 17 weeks gestation: a rare and catastrophic phenomenon. J Clin Gynecol Obstet 2014; 3: 147-150
• 15 Abildgaard U, Heimdal K. Pathogenesis of the syndrome of hemolysis, elevated liver enzymes, and low platelet count (HELLP): a review. Eur J Obstet Gynecol Reprod Biol 2013; 166: 117-123
• 16 Schaarschmidt W, Rana S, Stepan H. The course of angiogenic factors in early- vs. late-onset preeclampsia and HELLP syndrome. J Perinat Med 2013; 41: 511-516
• 17 Várkonyi T, Nagy B, Füle T. et al. Microarray profiling reveals that placental transcriptomes of early-onset HELLP syndrome and preeclampsia are similar. Placenta 2011; 32 (Suppl.) S21-S29
• 18 Haram K, Mortensen JH, Nagy B. Genetic aspects of preeclampsia and the HELLP syndrome. J Pregnancy 2014; 2014: 910751
• 19 Nelson J, Lewis B, Walters B. The HELLP syndrome associated wiht fetal medium-chain acyl-CoA dehydrogenase deficiency. J Inherit Metab Dis 2000; 23: 518-519
• 20 Fang CJ, Richards A, Liszewski MK. et al. Advances in understanding of pathogenesis of aHUS and HELLP. Br J Haematol 2008; 143: 336-348
• 21 Mihu D, Costin N, Mihu CM. et al. HELLP syndrome – a multisystemic disorder. J Gastrointestin Liver Dis 2007; 16: 419-424
• 22 Haram K, Svendsen E, Abildgaard U. The HELLP syndrome: clinical issues and management. A Review. BMC Pregnancy Childbirth 2009; 9: 8
• 23 Verlohren S, Herraiz I, Lapaire O. et al. The sFlt-1/PlGF ratio in different types of hypertensive pregnancy disorders and its prognostic potential in preeclamptic patients. Am J Obstet Gynecol 2012; 206: 58.e1-58.e8
• 24 Ahmed A. Informed decision making and abortion: crisis pregnancy centers, informed consent, and the first amendment. J Law Med Ethics 2015; 43: 51-58
• 25 Glantz LH. Pregnancy and informed consent to research. J Am Med Womens Assoc (1972) 2000; 55: 263-264
• 26 Wallace K, Martin jr. JN, Tam Tam K. et al. Seeking the mechanism(s) of action for corticosteroids in HELLP syndrome: SMASH study. Am J Obstet Gynecol 2013; 208: 380.e1-380.e8
• 27 Martin jr. JN, Rose CH, Briery CM. Understanding and managing HELLP syndrome: the integral role of aggressive glucocorticoids for mother and child. Am J Obstet Gynecol 2006; 195: 914-934
• 28 Martin jr. JN, Thigpen BD, Rose CH. et al. Maternal benefit of high-dose intravenous corticosteroid therapy for HELLP syndrome. Am J Obstet Gynecol 2003; 189: 830-834
• 29 Fonseca JE, Méndez F, Cataño C. et al. Dexamethasone treatment does not improve the outcome of women with HELLP syndrome: a double-blind, placebo-controlled, randomized clinical trial. Am J Obstet Gynecol 2005; 193: 1591-1598
• 30 Sullivan CA, Magann EF, Perry jr. KG. 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
• 31 van Esch JJA, van Heijst AF, de Haan AFJ. et al. Early-onset preeclampsia is associated with perinatal mortality and severe neonatal morbidity. J Matern Fetal Neonatal Med 2017; 30: 2789-2794
• 32 Akolekar R, Syngelaki A, Poon L. et al. Competing risks model in early screening for preeclampsia by biophysical and biochemical markers. Fetal Diagn Ther 2013; 33: 8-15
• 33 van Rijn BB, Hoeks LB, Bots ML. et al. Outcomes of subsequent pregnancy after first pregnancy with early-onset preeclampsia. Am J Obstet Gynecol 2006; 195: 723-728
• 34 Seeho SK, Algert CS, Roberts CL. et al. Early-onset preeclampsia appears to discourage subsequent pregnancy but the risks may be overestimated. Am J Obstet Gynecol 2016; 215: 785.e1-785.e8
• 35 Lemonnier M, Beucher G, Morello R. et al. [Subsequent pregnancy outcomes after first pregnancy with severe preeclampsia and delivery before 34 weeks of gestation]. J Gynecol Obstet Biol Reprod (Paris) 2013; 42: 174-183
• 36 Álvarez-Fernández I, Prieto B, Rodríguez V. et al. New biomarkers in diagnosis of early onset preeclampsia and imminent delivery prognosis. Clin Chem Lab Med 2014; 52: 1159-1168
• 37 Rolnik DL, Wright D, Poon LC. et al. Aspirin versus placebo in pregnancies at high risk for preterm preeclampsia. N Engl J Med 2017; 377: 613-622
• 38 Arain N, Mirza WA, Aslam M. Review-Vitamin D and the prevention of preeclampsia: a systematic review. Pak J Pharm Sci 2015; 28: 1015-1021
• 39 DeSousa J, Tong M, Wei J. et al. The anti-inflammatory effect of calcium for preventing endothelial cell activation in preeclampsia. J Hum Hypertens 2016; 30: 303-308
• 40 Trumbo PR, Ellwood KC. Supplemental calcium and risk reduction of hypertension, pregnancy-induced hypertension, and preeclampsia: an evidence-based review by the US Food and Drug Administration. Nutr Rev 2007; 65: 78-87
• 41 Habli M, Eftekhari N, Wiebracht E. et al. Long-term maternal and subsequent pregnancy outcomes 5 years after hemolysis, elevated liver enzymes, and low platelets (HELLP) syndrome. Am J Obstet Gynecol 2009; 201: 385.e1-385.e5
• 42 Eser B, Guven M, Unal A. et al. The role of plasma exchange in HELLP syndrome. Clin Appl Thromb Hemost 2005; 11: 211-217
• 43 Vafaeimanesh J, Nazari A, Hosseinzadeh F. Plasmapheresis: lifesaving treatment in severe cases of HELLP syndrome. Caspian J Intern Med 2014; 5: 243-247
• 44 Thadhani R, Kisner T, Hagmann H. et al. Pilot study of extracorporeal removal of soluble fms-like tyrosine kinase 1 in preeclampsia. Circulation 2011; 124: 940-950
• 45 Burwick RM, Feinberg BB. Eculizumab for the treatment of preeclampsia/HELLP syndrome. Placenta 2013; 34: 201-203
• 46 Andries G, Karass M, Yandrapalli S. et al. Atypical hemolytic uremic syndrome in first trimester pregnancy successfully treated with eculizumab. Exp Hematol Oncol 2017; 6: 4
• 47 Gately R. et al. Life-threatening pregnancy-associated atypical haemolytic uraemic syndrome and its response to eculizumab. Nephrology (Carlton) 2017; 22 (Suppl. 01) 32-35
• 48 Servais A, Devillard N, Frémeaux-Bacchi V. et al. Atypical haemolytic uraemic syndrome and pregnancy: outcome with ongoing eculizumab. Nephrol Dial Transplant 2016; 31: 2122-2130