The obstetric and gynaecological management of women with inherited bleeding disorders – review with guidelines produced by a taskforce of UK Haemophilia Centre Doctors’ Organization

Lee, C. A.; Chi, Claudia; Pavord, Sue; Bolton‐Maggs, Paula; Pollard, Debra; Hinchcliffe-Wood, A.; Kadir, Rezan A.

doi:10.1111/j.1365-2516.2006.01314.x

Cited by 253 publications

(345 citation statements)

References 264 publications

(367 reference statements)

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“…Even if somewhat non-specific (anaemia, pallor and apnea) and sometimes more unambiguous (seizures, lethargy and paresis), these recurrent symptoms must be recognized by caregivers in maternity hospitals as possibly because of ICH [12,33,39]. In the absence of high level evidence-based guidelines, questions such as the role of ultrasound scan (screening or targeted imaging) and the treatment approach (prophylactic or early curative), have been discussed and remain under debate [11,16,33,40]. The role of imaging should be considered along with the optimal timing of discharge from hospital, because it has been observed that ICH occur rather early (mean 4-5 days) [12].…”

Section: Prevention and Treatment Of Neonatal Ichmentioning

confidence: 99%

“…Even if some guidelines for haemophilia care include recommendations for the management of carrier women and the mode of delivery, successful prevention of ICH in newborns with haemophilia remains as a well-documented difficult task [40]. Actually, in identified carriers, prevention requires careful management and surveillance of the newborn, which means that the haemophilia specialist should transmit detailed information to obstetricians, anaesthesiologists and paediatricians in a multidisciplinary approach.…”

Section: Prevention and Treatment Of Neonatal Ichmentioning

confidence: 99%

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Haemophilia in the first years of life

et al. 2008

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Summary. Surgery in infants and young children with haemophilia, when preceded by accurate diagnosis and accompanied by safe and effective factor prophylaxis, is not associated with a significant risk of haemorrhage. Haemophilic newborns undergoing circumcision or major surgery prior to diagnosis and in the absence of appropriate haemostatic prophylaxis remain as a concern. Inhibitor development has replaced haemorrhage as the major surgical complication in the developed world, largely because of the intensity of treatment used to secure haemostasis. For that reason only, essential surgery should be performed. Intracranial haemorrhage (ICH) during the neonatal period affects 3.5-4.0% of all haemophilia boys in countries with a good standard of health care, which is considerably (40-80 times) higher than expected in the normal population. Because of the high frequency of sporadic cases, ICH in the neonatal period can only be partially prevented by improved carrier diagnosis and counselling. Infections and thrombosis are the major serious complications of central venous lines. Large differences are seen in the frequency of these complications, the most plausible explanations are probably related to the protocol used for device care, the quality of education and the compliance of the users, an issue addressed in an on-going study.

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Section: Prevention and Treatment Of Neonatal Ichmentioning

confidence: 99%

Section: Prevention and Treatment Of Neonatal Ichmentioning

confidence: 99%

Haemophilia in the first years of life

et al. 2008

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“…32 The validity of PBAC has been questioned because of a wide variation in its specificity and sensitivity for diagnosis of menorrhagia in different studies. 18,[33][34][35] However, it is a simple non-invasive tool that can be helpful in clinical practice for the initial assessment and monitoring of treatment.…”

Section: Menorrhagia and Ibdsmentioning

confidence: 99%

Gynaecological problems and choices for contraception in women with inherited bleeding disorders

Burton¹,

Chi²,

Kadir³

2008

j fam plann reprod health care

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15-20% of cases and is characterised by abnormal structures of VWF that affect interaction between platelets, vessel walls and VWF. Type 3 VWD is the least common but most severe form of the disease. It is an autosomalrecessive disorder characterised by a severe deficiency of VWF and factor VIII.Haemophilia A and B are X-linked IBDs characterised by deficiencies of clotting factor VIII or IX, respectively. Men inherit the condition whilst women are affected as carriers. The incidence of haemophilia A and B in the general population is 1 in 101000 and 1 in 601000, respectively, 6 but the carrier rates in women are unknown. A wide range of factor VIII or IX levels have been reported among carriers of haemophilia, from the upper limit of normal through to very low levels similar to affected males caused by random X-chromosome inactivation (lyonisation). 7 Thus carriers may have an increased bleeding tendency and experience significant bleeding symptoms. In a study by Plug et al. carriers were found to experience more spontaneous and provoked haemorrhage compared to non-carriers in families with haemophilia. 8

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“…There is no apparent increased bleeding risk for neonates with VWD, but for fetuses at risk of type 2 and 3 VWD invasive monitoring techniques or vacuum extraction and forceps use at delivery should be preferably avoided. 68 Prenatal diagnosis is required only in selected families with type 3 VWD.…”

Section: Casementioning

confidence: 99%

How I treat von Willebrand disease

Rodeghiero

Castaman

Tosetto

2009

Blood

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Recent multicenter studies have clarified the molecular basis underlying the different von Willebrand disease (VWD) types, all of which are caused by the deficiency and/or abnormality of von Willebrand factor (VWF). These studies have suggested a unifying pathophysiologic concept. The diagnosis of VWD, remains difficult because its clinical and laboratory phenotype is very heterogeneous and may overlap with normal subjects. Stringent criteria are therefore required for a clinically useful diagnosis. In this paper, we delineate a practical approach to the diagnosis and treatment of VWD. Our approach is based on the critical importance of a standardized bleeding history that has been condensed into a final bleeding score and a few widely available laboratory tests, such as VWF ristocetin cofactor activity, VWF antigen and factor VIII. This approach would help identify those subjects who will probably benefit from a diagnosis of VWD. The next step involves performing a trial infusion with desmopressin in all patients who fail to exhibit an enhanced responsiveness to ristocetin. On the basis of these results and through a series of illustrative examples, the clinician will be able to select the best approach for the optimal management of VWD, according to the patient's characteristics and clinical circumstances. (Blood. 2009;114: 1158-1165)

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The obstetric and gynaecological management of women with inherited bleeding disorders – review with guidelines produced by a taskforce of UK Haemophilia Centre Doctors’ Organization

Cited by 253 publications

References 264 publications

Haemophilia in the first years of life

Haemophilia in the first years of life

Gynaecological problems and choices for contraception in women with inherited bleeding disorders

How I treat von Willebrand disease

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