An unusual manifestation of bleeding diathesis in a patient with osteogenesis imperfecta

Edge, G.; Okafor, B.; Fennelly, M.; Ransford, A. O.

doi:10.1046/j.1365-2346.1997.00115.x

Cited by 21 publications

(21 citation statements)

References 11 publications

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“…Five patients reported haemorrhages during surgery requiring blood transfusion and other medical interventions, such as a repeat surgery. These types of events have previously been reported in the literature in OI patients (Mayer et al , ; Edge et al , ; Kastrup et al , ; Mondai et al , ; Faqeih et al , ; Paterson & Monk, ). Interestingly, 18% of the OI patients appeared to have an elevated BS but laboratory testing did not identify a coagulopathy.…”

Section: Discussionsupporting

confidence: 73%

“…Five of these six case‐reports described a single patient and one reported on 20 infants aged between 1 and 6 months old. (Mayer et al , ; Edge et al , ; Kastrup et al , ; Mondai et al , ; Faqeih et al , ; Paterson & Monk, ) To our knowledge, no recent studies have been performed in OI patients to identify or exclude a haematological cause. The aim of this study was firstly to investigate whether a bleeding tendency is present and, secondly, to perform haematological studies to identify or exclude coagulation disorders.…”

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confidence: 99%

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Bleeding and bruising in Osteogenesis Imperfecta: International Society on Thrombosis and Haemostasis bleeding assessment tool and haemostasis laboratory assessment in 22 individuals

et al. 2019

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Osteogenesis imperfecta (OI) is characterized by susceptibility to bone fractures. Other symptoms, such as easy bruising and bleeding complications during surgery necessitating transfusions, have also been reported. The aim of the cross-sectional pilot study was to assess the bleeding and bruising tendency in OI patients and to screen for possible underlying haematological disorders. Bleeding tendency was investigated using the International Society on Thrombosis and Haemostasis bleeding assessment tool (ISTH-BAT) in 22 adult OI patients. Laboratory testing was performed to investigate for bleeding disorders or abnormal coagulation. Four patients [OI type 1(n = 3), OI type 4(n = 1)] had a bleeding score (BS) fitting with a bleeding tendency, but without test results pointing to a coagulopathy. Two patients [OI type 1(n = 1), OI type 3 (n = 1)] without a bleeding tendency according to the BS had increased fibrinolysis. This is the second largest study to date addressing bleeding tendency in OI and the first study to use ISTH-BAT and elaborate laboratory testing for coagulopathies. Four patients had an increased bleeding tendency. However, laboratory testing demonstrated no bleeding disorder or abnormal coagulation. Increased fibrinolysis was demonstrated in two patients without bleeding tendency on BS. Vascular fragility as a cause of bleeding tendency in OI has been suggested earlier. Further research on bleeding tendency in OI is important.

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Section: Discussionsupporting

confidence: 73%

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confidence: 99%

Bleeding and bruising in Osteogenesis Imperfecta: International Society on Thrombosis and Haemostasis bleeding assessment tool and haemostasis laboratory assessment in 22 individuals

et al. 2019

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“…Additional typical clinical features include blue sclera, DI, hyperlaxity of ligaments and skin, hearing impairment, short stature and bone deformities (1). OI presents a complex clinical challenge; it results in abnormal blood coagulation and airway obstruction, cardiovascular anomalies and delayed wound healing (5,6). It is a rare disorder, with an incidence rate of ~1 in 10,000–20,000 births (1).…”

Section: Discussionmentioning

confidence: 99%

Osteogenesis imperfecta type I: A case report

Ren

Jian

et al. 2014

Experimental and Therapeutic Medicine

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A 15-year-old male patient was admitted to hospital having experienced repeated fractures over the previous three years, predominantly due to falling down or overexertion. The clinical signs and radiological features, such as recurrent fractures, blue sclera and low bone mineral density (BMD) level, all led to the diagnosis of a mild form of osteogenesis imperfecta (OI) type I. The patient began treatment with a regular intake of calcium (1,000 mg/day), an adequate intake of vitamin D (800 U/day) and intravenous pamidronate (60 mg). Following four months of treatment, the symptoms and quality of life of the patient improved. This patient appears to be a rare case of OI type I.

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“…[8] Coagulopathy with a sudden development of widespread petechiae has also been reported. [8] Therefore, due precautions regarding any unexpected bleeding were taken in the form of availability of adequate blood, fresh frozen plasma and platelet concentrates.…”

Section: Discussionmentioning

confidence: 99%

A rare presentation of a child with osteogenesis imperfecta and congenital laryngomalacia for herniotomy

et al. 2011

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Sometimes anaesthesiologists come across rare congenital anomalies in their practice. The inherent complications associated with the disorder necessitate tailor-made approaches for providing anaesthesia to even seemingly simple surgical interventions. Here, we share our experience of anaesthesia management of an infant with congenital laryngomalacia and recently diagnosed osteogenesis imperfecta type 1 who had presented to us with an acute abdomen for a semi-emergency herniotomy.

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An unusual manifestation of bleeding diathesis in a patient with osteogenesis imperfecta

Cited by 21 publications

References 11 publications

Bleeding and bruising in Osteogenesis Imperfecta: International Society on Thrombosis and Haemostasis bleeding assessment tool and haemostasis laboratory assessment in 22 individuals

Bleeding and bruising in Osteogenesis Imperfecta: International Society on Thrombosis and Haemostasis bleeding assessment tool and haemostasis laboratory assessment in 22 individuals

Osteogenesis imperfecta type I: A case report

A rare presentation of a child with osteogenesis imperfecta and congenital laryngomalacia for herniotomy

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