IN THE NAME OF GOD. دکترزهرابدیعی شیراز 28-29 خرداد 93.

IN THE NAME OF GOD

خانم در زایمان و حاملگیاختالالت به مبتال های

دهنده خونریزیدکترزهرابدیعی

93خرداد 29-28شیراز

Preconception counselling

• This is most important for women with severe bleeding disorders or those who could potentially carry a severely affected baby, such as carriers of hemophilia

• It provides adequate information for women and their family about

- genetic implications of their disorder- the available reproductive choices,- and options for prenatal diagnosis.

planning for pregnancy establishing how and where the pregnancy can best

be managed. immunization against hepatitis A and B for those likely

to require blood transfusion general advice such as folic acid supplementation. A DDAVP test dose can also be carried out to assess

response. the opportunity to speak with a pediatric

hematologist regarding the care of a potentially affected child.

Antenatal managementNormal pregnancy is accompanied by Increased concentrations of several coagulation factors

including VIII, VWF, and a pronounced increase in fibrinogen

reduced fibrinolytic activity secondary to increased levels of plasminogen activator inhibitors, especially during the third trimester

All of these changes contribute to the hypercoagulable state of pregnancy and, in women with bleeding disorders, to improved hemostasis

however, women with bleeding disorders often do not achieve the same levels of clotting factors that other women do and, therefore, are still at an increased risk of bleeding complications.

Except :Carriers of Hemophilia AvWD type 1

Baseline factor VIII and von Willebrand factor levels should be checked at some stage early in the pregnancy, such as during the first consultation with an obstetrician and in the third trimester (ideally at around 34 weeks).

The level of vWF may not rise significantly during the first or even second trimester and therefore an early miscarriage may be accompanied by significant bleeding

There is a profound increase in the risk of miscarriage and placental abruption resulting in fetal loss or preterm delivery in women with deficiencies of fibrinogen or factor XIII

Factor replacement is recommended and used to reduce the risk of miscarriage and fetal loss in these women

in women with other bleeding disorders is less clear the risk of:

• miscarriage, • antepartum bleeding,• and adverse outcome

Also consider obstetric causes as a reason for bleeding

labour and delivery

There is no consensus on the factor levels that are safe for regional anesthesia,

but if levels are at least 50% and the rest of the coagulation studies are normal, regional anesthesia may be considered safe.

It is often difficult to obtain factor levels during labour and is therefore acceptable to use third trimester levels to formulate an appropriate plan.

If the factor level is low, intravenous access should be established and prophylactic treatment administered

The management of childbirth will depend on the needs of the mother and her potentially affected infant at the time of delivery

Caesarean section is not routinely indicated merely because of possibility of fetal involvement

DDAVP may be used to raise factor VIII and vWF levels in

carriers of hemophilia A and type I VWD prior to invasive procedures.

It is generally thought to be safe for mother and fetus, but care must be taken in its administration at the time of childbirth.

At the time of childbirth, administration of DDAVP, combined with fluids and oxytocin, may result in life-threatening hyponatremia. Therefore, fluid balance and electrolyte levels should be strictly monitored.

• In pregnancies with potentially affected fetuses should be avoided :

1. Invasive intrapartum monitoring techniques (e.g. fetal scalp electrode,fetal blood sampling)

2. instrumental deliveries (ventouse, midcavity or rotational forceps, as serious head bleeding may result from these procedures.

Normal vaginal delivery is not absolutely contraindicated in these pregnancies,

but prolonged labour should be avoided and delivery achieved by the least traumatic

method.

Although cesarean section may not completely eliminate the risk of serious neonatal bleeding complications ,early recourse to cesarean section should be considered to minimize the risk of neonatal bleeding complications.

Low forceps delivery may be considered less traumatic than cesarean section when the head is deeply engaged in the pelvis and an easy outlet delivery is anticipated

A cord blood sample should be collected from neonates at risk of moderate or severe inherited bleeding disorders to assess coagulation status and clotting factor levels.

If delivery has been traumatic or if there are clinical signs suggestive of head bleeding,a cranial ultrasound should be performed

It is also advisable to consider prophylactic cover in these cases.

Intramuscular injections should be avoided in neonates at risk until the coagulation status is known.

Vitamin K may be given orally routine immunizations given intradermally or

subcutaneously. If vitamin K is given intramuscularly (IM),apply firm

pressure to the site for five to ten minutes. Heel sticks should also have pressure applied for five

minutes and close observation of the site for 24 hours.

Any surgical procedures (e.g. circumcision) should be delayed until the coagulation status of the neonate is known.

assessing neonatal clotting factor levels

It should be appreciated that the levels of vitamin K dependent factors (FII, FVII, FIX, and FX) correlate with gestational age due to liver immaturity and reach adult levels at six months of age.

It is therefore not reliable to diagnose mild forms of inherited bleeding disorders at birth.

It is almost impossible to diagnose the much commoner, milder forms of vWD in a newborn as the level of vWF rises significantly during birth and an apparently normal result may thus mask a mild form of vWD.

Hemophilia A can be diagnosed at birth.

Severe forms of RBDs.

Postpartum management

Postpartum hemorrhage (PPH) is a major cause of maternal morbidity and mortality

an estimated 140,000 maternal deaths each year worldwide

the most common causes of PPH are• uterine atony• retained placenta or placenta pieces,• genital tract trauma, • Coagulation disorders

Postpartum management After the delivery, the elevated coagulation

factors return to pre-pregnancy levels within 14 to 21 days of delivery.

Risk of primary PPH (blood loss of more than 500 mL in the first 24 hours after delivery)

Secondary PPH (excessive bleeding occurring between 24 hours and six weeks post delivery),

especially those with severe disorders

Perineal/vaginal hematoma are rare complications of vaginal birth,

but are also more likely to occur in women with bleeding disorders, especially after operative vaginal deliveries

Reducing the risk of PPH

1. prophylactic replacement therapy is recommended to cover labour, delivery, and the immediate postpartum period (at least three to four days for vaginal delivery and five to seven days for C/S )

2. Active management of the third stage of labour is associated with a significant reduction in blood loss during childbirth.

• It entails the administration of prophylactic uterotonics (agents that increase uterine muscle contractility),

• early cord clamping, • and controlled traction of the umbilical cord.

3- During C/S : meticulous surgical hemostasis should be practiced to minimize blood loss.

4- Care must also be taken to minimize maternal genital and perineal trauma,for prevention of perineal hematoma

Oral tranexamic acid can be used for the prevention and management of secondary PPH.

Combined oral contraceptive pills, if not contraindicated, are an option for preventing excessive bleeding in the late postpartum period.

It is important to keep in mind the obstetric risk factors and causes of PPH in women with inherited bleeding disorders.

Inherited bleedingdisorder

Hemostatic levels,suggested (IU/dL)

Normal range(non-pregnant)

(IU/dL

VWD 50 50 – 175

Carrier of hemophilia A

50 50 – 150

Carrier of hemophilia B

50 50 – 150

Fibrinogen deficiency 1-1.5 gr/dl 2-4 gr/dl To maintain >1.0 g/Lduring pregnancy

FII deficiency 20 – 30 50 – 150

FV deficiency 15 – 25 50 – 150

FVII deficiency 10 – 20 50 – 150

FX deficiency 10 – 20 50 – 150

FXI deficiency 20-70 70 – 150

FXIII deficiency 20 – 30 70 – 150 To maintain >3 IU/dLduring pregnancy

در / 1364متولد • فاکتور سالگی / 21تشخیص 7کمبوددر : , , • خونریزی گاهی اپیستاکسی کبودی ، ندارد جراحی عمل سابقه حال ح شر

و ... پا مچ ، آرنج مفاصل

•3 / اول ماهه سه در سقط FVII=2.8%و PT = 48 نوبتیک • درجه والدین، 1385/8تشخیص •

شدن • 1385/10حاملههفت • فاکتور وجود عدمهفتگی • تزریق حاملگی )FFPتحت هفتم ماه تا گرفت ،(10cc/kgقرارهفته • هر وزن ) 1.2mgبعد با ( 20μ/kgمعادل 57نووسونمجموعا • تزریق با طبیعی ، 4-3زایمان علیرغم mg 1.2ویال زایمان از بعد ، شد انجام

! نداشت هم خونریزی و نکرد تزریق خودش فاکتور مصرف به توصیه

سال سن ( 3 ( 1389مجددا در کرد مراجعه حاملگی با بعد هفته 7سال هفته هر درمان گرفت mg 1.2تحت قرار فاکتور سطح زایمان از 3.9قبل ، عارضه بدون زایمان

: فاکتور / 9تشخیص / 1369متولد کمبود سالگی13

/ :و خونریزی تروما با ناف بند از خونریزی عالیماز / FFPتزریق بعد طبیعی ماهیانه عادت ،

هر کرایو تزریق تحت تهران در یک 4تشخیص هفتهبار

سال هر 1388حاملگی کرایو تزریق یک 2با هفتهتا فاکتور 8بار تهیه از بعد ، دو 500ماهگی هر واحد

وزن ) ، ( 52هفته عارضه بدون طبیعی زایمان فقط زایمان کرد 2برای دریافت فاکتور ویال سال مجدد 1392حاملگی

در / 1365متولد سال ) 23تشخیص شدید ،( 88سالگی ویلبراند فون + : پر محل از خونریزی ، بینی کوتر طفولیت در شدید های اپیستاکسی سابقه

منوراژی + + دست بریدن دندان کردن عمو و پدری مادربزرگ پدر، در عالئم ) (داشت حاملگی قصد : سه طی فاکتورهیومت تزریق به نیاز و آمین ترانس به پاسخ عدم منوراژی کنترل

اول روز کیلو / 48وزن ، فاکتور ویال دو نوبت دو هفته هر حاملگی در شد انجام طبیعی زایمان قطع سوم درهفته و ویال دو میان در روز دوم هفته ، ویال دو روز هر اول هفته

بیمار خود توسط دارو

خونریزی / 25روز با مراجعه ، زایمان از تا بعد مرداد ازبستری 1391آذر نوبت ، چند مختلف هورمونی درمانهای دریافت و

متریال / / گرافی سونو آخرین در فاکتور گاهی کرایو گاهیبررسی ، کورتاژ کاندید ، شد مطرح لخته احتمال و رحم در اکوژن

/ ، کرد دفع نسجی بخود خود منفی های بافت مهارکنندهجفت ) ( باقیمانده دژنره دوآل خونریزی دسی قطع نهایتا و

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