To interpret HRQoL data precisely, knowledge of the content, scoring, reliability, and validity of a questionnaire is important. Recently, there is a growing interest in the assessment of HRQoL in the field of hemophilia; results are presented for pediatric and adult hemophilia patients. “
“Summary. Pregnancy, labour and delivery present intrinsic haemostatic challenges to women with and carriers of bleeding disorders
and their offspring. Deficiency of fibrinogen and factor XIII are associated with miscarriage, placental abruption and foetal loss. The risk of antenatal complications including antepartum haemorrhage Rapamycin nmr is unknown in women with other bleeding disorders. There is a significant risk of postpartum haemorrhage (primary and secondary) in women with all types of bleeding disorders. This can be serious and life threatening in those with severe defects such as Bernard Soulier syndrome and Glanzmann’s thrombasthenia. Three to four percent of infants with haemophilia experience cranial bleeding that occurs during labour and delivery.
The safest method of delivery for affected babies remains controversial. However, the rate of planned Caesarean section is increasing among known carriers of haemophilia. If vaginal delivery is planned, prolonged labour and difficult delivery especially vacuum extraction are associated with the highest risk of cranial bleeding and should be avoided. The optimal management Obatoclax Mesylate (GX15-070) of pregnancy in women with inherited bleeding disorders requires a multidisciplinary approach and advanced individualized management plan taking selleck chemicals llc into consideration obstetric and bleeding risk factors. Women with mild or moderate bleeding disorders can be managed at their local maternity unit in close collaboration
with a tertiary centre. However, those with severe or rare disorders or carrying an affected infant should be managed in a tertiary centre with an onsite Haemophilia centre. “
“Summary. Factor replacement with BIOSTATE®, a factor VIII (FVIII)/von Willebrand factor concentrate, forms the mainstay of treatment for children with von Willebrand disorder (VWD) in Australia and New Zealand. However, published data on the clinical efficacy and safety of BIOSTATE in the VWD paediatric population are limited. We retrospectively assessed the efficacy and safety of BIOSTATE in 43 children with VWD who received treatment for surgery, non-surgical bleeds or continuous prophylaxis at eight paediatric haemophilia centres in Australia and New Zealand. Data were collected on patient demographics, disease history, treatment history, dosage, administration, adverse reactions, concomitant medications and excessive bleeding events. BIOSTATE provided excellent/good haemostatic efficacy in 90% of surgical procedures (n = 42) with a mean daily FVIII dose of 47 IU FVIII:C kg−1 and a median treatment duration of 3 days.
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