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Archives of Gynecology and Obstetrics

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01-06-2014 | Maternal-Fetal Medicine

Neonatal and maternal risk in Hermansky–Pudlak syndrome: peripartum management—brief report and review of literature

Authors: Cornelia Bachmann, Harald Abele, Diethelm Wallwiener, Karl Oliver Kagan

Published in: Archives of Gynecology and Obstetrics | Issue 6/2014

Abstract

Introduction

Challenges of modern medicine are peripartum bleeding complications as one of the most frequent cases of emergency in obstetrics with a prevalence of 0.5–5.0 %, meaning the main cause of maternal morbidity and mortality. In this context, inherited diseases such as Hermansky–Pudlak syndrome (HPS) should be recognized. HPS is a rare disease and belongs to a heterogeneous group of autosomal recessive disorders characterized by the triad of partial oculocutaneous albinism, disorder of “ceroid” metabolism and platelet storage pool deficiency with bleeding disorder.

Materials and methods

We report on a 30-year-old primipara, to show the peripartum obstetrical and anaesthesiological management. The patient presented with contractions in our outpatient department in the 39th gestational week. In previous operations there were bleeding complications due to HPS.

Discussion

Therefore, to minimize bleeding complications we aimed a vaginal birth, advised against the peridural anesthesia and optimized the coagulation parameters. The subsequent delivery was performed as vacuum extraction without complications. Patient and newborn could be discharged from the hospital without complications.

Conclusion

In conclusion, decisive factor for patients with bleeding disorders is a close interdisciplinary cooperation between obstetrician and anesthesiologist.

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Title: Neonatal and maternal risk in Hermansky–Pudlak syndrome: peripartum management—brief report and review of literature
Authors: Cornelia Bachmann
Harald Abele
Diethelm Wallwiener
Karl Oliver Kagan
Publication date: 01-06-2014
Publisher: Springer Berlin Heidelberg
Published in: Archives of Gynecology and Obstetrics / Issue 6/2014
Print ISSN: 0932-0067
Electronic ISSN: 1432-0711
DOI: https://doi.org/10.1007/s00404-013-3110-7

At a glance: The STEP trials

Springer Medicine

Neonatal and maternal risk in Hermansky–Pudlak syndrome: peripartum management—brief report and review of literature

Abstract

Introduction

Materials and methods

Discussion

Conclusion

At a glance: The STEP trials

Springer Medicine

Abstract

Introduction

Materials and methods

Discussion

Conclusion

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