Management of Hermansky-Pudlak syndrome in pregnancy and review of literature

BMJ Case Rep. 2016 Nov 17:2016:bcr2016217719. doi: 10.1136/bcr-2016-217719.

Abstract

We report on the obstetric outcome of a woman aged 27 years with Hermansky-Pudlak syndrome (HPS). She underwent a caesarean section after failed induction of labour. Platelet transfusion was administered in a set schedule for 36 hours, starting 2 hours before delivery. The child had good Apgar scores and there were no significant problems of prolonged bleeding during the procedure. 72 hours postpartum, a haematoma developed at the site of the wound, subsequently complicated by a secondary infection for which she received antibiotics. Wound care was provided in an outpatient setting during 2 weeks, in which the infection stabilised and responded to the treatment. Mother and child could leave the hospital after 6 days.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Anti-Bacterial Agents / therapeutic use
  • Apgar Score*
  • Cesarean Section*
  • Disease Management*
  • Female
  • Hematoma
  • Hermanski-Pudlak Syndrome / complications*
  • Hermanski-Pudlak Syndrome / therapy
  • Humans
  • Infant, Newborn
  • Infections / complications
  • Infections / drug therapy
  • Labor, Induced
  • Platelet Transfusion*
  • Postpartum Period
  • Pregnancy
  • Pregnancy Complications / therapy*
  • Pregnancy Complications, Hematologic / prevention & control
  • Wound Healing*

Substances

  • Anti-Bacterial Agents