Pregnancy and Delivery in Bernard-Soulier Syndrome

Bernard Soulier Syndrome (BSS) is an inherited bleeding disorder characterized by macrothrombocytopenia and absence of ristocetin-induced platelet aggregation. Clinical findings vary from person to person. Most of the patients are diagnosed with muco-cutaneous bleeding such as purpura, epistaxis and gingival bleeding in early childhood. Few pregnant women with BSS are described in the literature. Management of thrombocytopenia during pregnancy and delivery requires a multidisciplinary approach. The family should be warned about the potentially life-threatening bleeding during pregnancy and the delivery and the decision about mode of delivery should be individualised, involving discussion with patient and multidisciplinary team.

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Responses of Platelets to Strains of Streptococcus sanguis: Findings in Healthy Subjects, Bernard-Soulier, Glanzmann’s, and Collagen-Unresponsive Patients

Thrombosis and Haemostasis ◽

10.1055/s-0038-1651098 ◽

1987 ◽

Vol 57 (02) ◽

pp. 222-225 ◽

Cited By ~ 19

Author(s):

A H Soberay ◽

M C Herzberg ◽

J D Rudney ◽

H K Nieuwenhuis ◽

J J Sixma ◽

...

Keyword(s):

Platelet Aggregation ◽

Healthy Subjects ◽

Normal Subjects ◽

Membrane Glycoprotein ◽

Response Mechanism ◽

Platelet Response ◽

Induce Platelet Aggregation ◽

Streptococcus Sanguis ◽

Lag Times ◽

Bernard Soulier Syndrome

SummaryThe ability of endocarditis and dental strains of Streptococcus sanguis to induce platelet aggregation in plasma (PRP) from normal subjects were examined and compared to responses of PRP with known platelet membrane glycoprotein (GP) and response defects. S. sanguis strains differed in their ability to induce normal PRPs to aggregate. Strains that induced PRP aggregation in more than 60% of donors were significantly faster agonists (mean lag times to onset of aggregation less than 6 min) than those strains inducing response in PRPs of fewer than 60% of donors.Platelets from patients with Bernard-Soulier syndrome aggregated in response to strains of S. sanguis. In contrast, platelets from patients with Glanzmann’s thrombasthenia and from a patient with a specific defect in response to collagen were unresponsive to S. sanguis. These observations show that GPIb and V are not essential, but GPIIb-IIIa and GPIa are important in the platelet response mechanism to S. sanguis. Indeed, the data suggests that the platelet interaction mechanisms of S. sanguis and collagen may be similar.

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Impaired Prothrombin Consumption in Bernard-Soulier Syndrome Is Corrected In Vitro by Human Factor VIII

Thrombosis and Haemostasis ◽

10.1055/s-0038-1655972 ◽

1997 ◽

Vol 77 (02) ◽

pp. 383-386 ◽

Cited By ~ 8

Author(s):

S Bellucci ◽

J P Girma ◽

M Lozano ◽

D Meyer ◽

J P Caen

Keyword(s):

Factor Viii ◽

Human Factor ◽

Platelet Membrane ◽

Giant Platelets ◽

Prolonged Bleeding Time ◽

Human Factor Viii ◽

Von Willebrand ◽

Willebrand Factor ◽

Bernard Soulier Syndrome

SummaryThe Bernard-Soulier syndrome (BSS) is characterized by thrombocytopenia with giant platelets, a prolonged bleeding time with defective platelet adhesion to the subendothelium related to a defect in platelet membrane glycoprotein lb (GPIb) and a decreased prothrombin consumption. The mechanism of the latter abnormality remains unknown. In this study, we showed that this defect was corrected by the addition of purified human factor VIII (FVIII) to blood from four patients with BSS. The correction of prothrombin consumption was almost complete at concentrations between 1.5 and 3 IU/ml of FVIII procoagulant activity (VIII.'C) and partially abolished by a monoclonal antibody which neutralizes VIII:C. This correction was specific for FVIII and was not observed after addition of purified human FIX. It was obtained, in the same magnitude range, with FVIII complexed to von Willebrand factor (vWF) but not with free vWF. These data provide a new insight into the knowledge of the physiological interaction between the platelet membrane and the vWF-FVIII complex facilitating plasma coagulation activation and may lead to helpful therapeutic advances.

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Relationship Between Mepacrine-Labelled Dense Body Number, Platelet Capacity to Accumulate 14C-5-HT and Platelet Density in the Bernard-Soulier and Hermansky-Pudlak Syndromes

Thrombosis and Haemostasis ◽

10.1055/s-0038-1666908 ◽

1979 ◽

Vol 42 (02) ◽

pp. 694-704 ◽

Cited By ~ 31

Author(s):

F Rendu ◽

A T Nurden ◽

M Lebret ◽

J P Caen

Keyword(s):

Dense Body ◽

Human Subjects ◽

Human Platelets ◽

Dense Bodies ◽

Storage Organelle ◽

Hereditary Disorders ◽

Labelling Procedure ◽

Normal Human ◽

Hermansky Pudlak Syndrome ◽

Bernard Soulier Syndrome

SummaryWe have used the mepacrine-labelling procedure to measure the dense body (serotonin storage organelle) content of the platelets of 2 hereditary disorders where abnormalities in dense body number were suspected. The platelets were incubated with mepacrine and examined by fluorescence microscopy. A mean number of 5.4 ± 0.8 (SD) dense bodies per platelet was calculated from the data obtained using platelets isolated from 40 normal human subjects. In contrast the platelets of 2 patients with the Bernard-Soulier syndrome contained an average of 14 and 17 labelled granules. This increase was associated with a much greater capacity of the platelets to accumulate 14C-5-HT. The opposite result was obtained using the platelets from 2 patients with the Hermansky-Pudlak syndrome which contained few granules labelled by mepacrine and took up less 14C-5-HT than normal human platelets. Centrifugation of the patients’ platelets on discontinuous sucrose gradients showed that the platelets of the 2 Bemard-Soulier patients were much denser than normal whereas a high proportion of low density platelets was observed in the Hermansky-Pudlak syndrome. These results further define the platelet abnormalities in the two syndromes and suggest that dense body number may be one of the factors governing platelet density.

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RARE CONGENITAL THROMBOCYTOPATHIES IN PRACTICE OF PEDIATRIC HEMATOLOGIST: BERNARD–SOULIER SYNDROME, THERAPY PRINCIPLES

PEDIATRIA Journal named after G N SPERANSKY ◽

10.24110/0031-403x-2020-99-4-258-262 ◽

2020 ◽

Vol 99 (4) ◽

pp. 258-262

Author(s):

A.A. Osipova ◽

◽

I.V. Markova ◽

T.A. Bykova ◽

V.N. Ovechkina ◽

...

Keyword(s):

Bernard Soulier Syndrome

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Infeksi Herpes Simpleks Dalam Kehamilan

Jurnal Ilmu Kedokteran ◽

10.26891/jik.v10i1.2016.1-4 ◽

2017 ◽

Vol 10 (1) ◽

pp. 1

Author(s):

Fauzia Andrini Djojosugito

Keyword(s):

Herpes Simplex ◽

Caesarean Section ◽

Sexually Transmitted Diseases ◽

Recurrent Infection ◽

Neonatal Infection ◽

Sexually Transmitted ◽

Caesarean Section Delivery ◽

Common Causes ◽

Pregnancy And Delivery ◽

Simplex Virus

Herpes simplex virus is one the most common causes of sexually transmitted diseases. This infection is common inreproductive age women and can cause severe sequealae for fetus and neonates because this virus can be transmittedto the fetus during pregnancy and the newborn. Infection of Herpes simplex can occurs as first or primary infectionand recurrent infection. Most of the case is asymptomatic. Rarely during intrauterine, the risk of transmission ofmaternal-fetal can be increased during the last trimester of pregnancy and delivery. Based on type of infection,primary or recurrent, performing a treatment with antiviral drugs or caesarean section delivery can decrease the riskof neonatal infection

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COMPORTAMENTO REPRODUTIVO DAS MULHERES RIBEIRINHAS DO AMANÃ

Scientific Magazine UAKARI ◽

10.31420/uakari.v1i1.4 ◽

2008 ◽

Vol 1 (1) ◽

pp. 31-40 ◽

Cited By ~ 2

Author(s):

Edila Arnaud Ferreira Moura

Keyword(s):

Sustainable Development ◽

Reproductive Health ◽

Reproductive Behavior ◽

Reproductive Period ◽

Health Programs ◽

Local Conditions ◽

The Sustainable Development ◽

Rivers And Lakes ◽

Pregnancy And Delivery ◽

The Mean

This work presents the results of a study about the reproductive behavior of riverine women living in the Sustainable Development Reserve Amanã, in the Middle Solimões region, state of Amazonas. The study was done in 2001 with the objective of identify aspects of the reproductive behavior of 83 women, collected through reproductive history methodology. These women live in small localities along the rivers and lakes in the middle of the Amazonian forest. The study identified that these women initialize their reproductive period at the age of 17, in average, and the mean age of their last pregnancy occurs at the age of 42, considering those above 50 years old. 36% of these 83 women had lost one or more of their children before the age of five. Only 16% use contraceptives and 12% are sterilized. They dont have access to the basic health assistance in a regular basis and the local midwives are extremely important in their assistance during pregnancy and delivery. This study em phasizes the importance of reproductive health programs including those social and cultural local conditions.

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