title: Neonatal and maternal risk in Hermansky-Pudlak syndrome: peripartum management-brief report and review of literature.
abstract: BACKGROUND
  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.
  
  
  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.
  
  
  CONCLUSIONS
  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.
  
  
  CONCLUSIONS
  In conclusion, decisive factor for patients with bleeding disorders is a close interdisciplinary cooperation between obstetrician and anesthesiologist.
authors: Department of Gynecology, University Tübingen, Calwer Str. 7, 72070, Tübingen, Germany, cornelia.bachmann@med.uni-tuebingen.de.
authors: Cornelia
authors: C
authors: Bachmann
authors: Harald
authors: H
authors: Abele
authors: Diethelm
authors: D
authors: Wallwiener
authors: Karl Oliver
authors: KO
authors: Kagan
mesh_terms: D003894:Deamino Arginine Vasopressin / Q000627:therapeutic use*; D005260:Female; D006490:Hemostatics / Q000627:therapeutic use*; D022861:Hermanski-Pudlak Syndrome / Q000150:complications*; D006801:Humans; D007231:Infant, Newborn; D008297:Male; D006473:Postpartum Hemorrhage / Q000517:prevention & control*; D011247:Pregnancy; D011248:Pregnancy Complications; D014620:Vacuum Extraction, Obstetrical; D055815:Young Adult
chemical_list: D006490:Hemostatics; D003894:Deamino Arginine Vasopressin