Successful pregnancy and delivery management in a patient with Bernard Soulier Syndrome

2022 ◽  
pp. 1753495X2110671
Author(s):  
Ufuk Demirci ◽  
Esra Altan Erbilen ◽  
Elif Gülsüm Ümit ◽  
Cihan İnan ◽  
N. Cenk Sayın ◽  
...  

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.

1987 ◽  
Vol 57 (02) ◽  
pp. 222-225 ◽  
Author(s):  
A H Soberay ◽  
M C Herzberg ◽  
J D Rudney ◽  
H K Nieuwenhuis ◽  
J J Sixma ◽  
...  

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.


Author(s):  
Enrico Ferrazzi ◽  
Luigi Frigerio ◽  
Valeria Savasi ◽  
Patrizia Vergani ◽  
Federico Prefumo ◽  
...  

2012 ◽  
Vol 25 (3) ◽  
pp. e69-e71 ◽  
Author(s):  
Fatma Devran Bildircin ◽  
Halil Suat Ayyildiz ◽  
Migraci Tosun ◽  
Erdal Malatyalioglu ◽  
Ender Ariturk ◽  
...  

1984 ◽  
Vol 63 (2) ◽  
pp. 185-186 ◽  
Author(s):  
S. Michalas ◽  
A. Malamitsi-Puchner ◽  
H. Tsevrenis

2021 ◽  
Vol 6 (1) ◽  
pp. 12
Author(s):  
Hisham A Imad ◽  
Juthamas Phadungsombat ◽  
Emi E Nakayama ◽  
Sajikapon Kludkleeb ◽  
Wasin Matsee ◽  
...  

Chikungunya virus is an Alphavirus belonging to the family Togaviridae that is transmitted to humans by an infected Aedes mosquito. Patients develop fever, inflammatory arthritis, and rash during the acute stage of infection. Although the illness is self-limiting, atypical and severe cases are not uncommon, and 60% may develop chronic symptoms that persist for months or even for longer durations. Having a distinct periodical epidemiologic outbreak pattern, chikungunya virus reappeared in Thailand in December 2018. Here, we describe a cohort of acute chikungunya patients who had presented to the Bangkok Hospital for Tropical Diseases during October 2019. Infection was detected by a novel antigen kit and subsequently confirmed by real-time RT-PCR using serum collected at presentation to the Fever Clinic. Other possible acute febrile illnesses such as influenza, dengue, and malaria were excluded. We explored the sequence of clinical manifestations at presentation during the acute phase and associated the viral load with the clinical findings. Most of the patients were healthy individuals in their forties. Fever and arthralgia were the predominant clinical manifestations found in this patient cohort, with a small proportion of patients with systemic symptoms. Higher viral loads were associated with arthralgia, and arthralgia with the involvement of the large joints was more common in female patients.


2021 ◽  
Vol 14 (3) ◽  
pp. e234181
Author(s):  
Daisy Khera ◽  
Saurabh Agarwal ◽  
Prawin Kumar ◽  
Kuldeep Singh

A 2-month-old boy presented to us with bilateral microtia, left lower motor neuron facial palsy, micrognathia, hemivertebra, bifid rib, bifid thumb and absent/hypoplastic right-sided depressor anguli oris. He had bilateral external auditory canal atresia, although response to loud sound was present. Brain stem evoked response audiometry (BERA) was advised at 3 months of age. Karyotype was normal. We diagnosed him as a case of oculo-auriculo-vertebral spectrum. Child was discharged on request by the family with the plan for bone-anchored hearing aid after BERA and plan for pinna and ear canal reconstruction at a later age but child did not come for any follow-up visit. On telephonic enquiry, it was found that he is thriving well but has developmental delay including speech delay. We conclude that children presenting with external ear abnormalities should be screened for multiple congenital anomalies so that a multidisciplinary approach to management can be planned.


2021 ◽  
pp. 153857442110462
Author(s):  
Ahmed A. Sorour ◽  
Levester Kirksey ◽  
Sarah Keller ◽  
Michael S. O’Connor ◽  
Sean P. Lyden

Catastrophic antiphospholipid syndrome (CAPS) is a rare life threatening presentation of antiphospholipid syndrome. Surgery has been proposed as one of the triggering factors for this life threatening entity. There are no detailed published reports in the current literature describing CAPS as a complication after surgery. We report a case of a 21 year old that developed CAPS postoperatively and discuss the multidisciplinary approach for diagnosis and management.


2021 ◽  
Vol 14 (5) ◽  
pp. e240448
Author(s):  
Kim Pramanik ◽  
Philip Webb ◽  
RanaShoaib Hamid

We present to you a case of life-threatening haemoptysis secondary to non-cystic fibrosis bronchiectasis complicated by bronchial artery pseudoaneurysms. We discuss this patient’s emergency medical management using intravenous tranexamic acid, which resulted in successful resuscitation and eventual survival, and evaluate the need for urgent anaesthetic and interventional radiology input in such a case.


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