Journal Article

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

Jacquemyn, Yves, Muys, Joke, Van Avermaete, Freya

2016

Overview

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.

• Publisher: BMJ Publishing Group Ltd,BMJ Publishing Group LTD,BMJ Publishing Group
• Subject: 19-30 years / Adult / Albinism / Anesthesia / Anti-Bacterial Agents - therapeutic use / Antibiotics / Apgar Score / Blood / Blood platelets / Case reports / Cesarean Section / Childbirth & labor / Disease Management / Epistaxis / Europe (West) / Female / General anesthesia / Hematology / Hematoma / Hemoglobin / Hermanski-Pudlak Syndrome - complications / Hermanski-Pudlak Syndrome - therapy / Hospitals / Humans / Infant, Newborn / Infection - complications / Infection - drug therapy / Infections / Labor, Induced / Laboratories / Medical history / Middle-East / Mortality / Patients / Platelet Transfusion / Postpartum Period / Pregnancy / Pregnancy Complications - therapy / Pregnancy Complications, Hematologic - prevention & control / Pulmonary fibrosis / Rare Disease / Surgery / Vagina / Wound Healing