Spontaneous intracerebral hematoma in an adolescent with factor XIII deficiency. Case report

Giraldi, C; Creta, S.; Martini, Afif; Ioppoli, C.; Valleriani, A.M.; Muratorio, A

doi:10.1007/bf02333957

Cited by 3 publications

(2 citation statements)

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“…Other manifestations included cephalohematoma and intraperitoneal bleeding. Intracranial hemorrhage has been observed in other case studies of congenital FXIII deficiency 41‐44 . The bleeding manifestations of congenital FXIII deficiency are substantial and the risk of fatal intracranial hemorrhage or joint bleeding leads to substantial morbidity and mortality unless factor replacement therapy is provided.…”

Section: Clinical Manifestations Of Fxiii Deficiencymentioning

confidence: 97%

Biology of Factor XIII and clinical manifestations of Factor XIII deficiency

Levy

Greenberg

2012

Transfusion

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Section: Clinical Manifestations Of Fxiii Deficiencymentioning

confidence: 97%

Biology of Factor XIII and clinical manifestations of Factor XIII deficiency

Levy

Greenberg

2012

Transfusion

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“…There is no exact information regarding the prevalence of ICH in neonates because frequency of this feature is dependent on various determining factors including severity of disease, prenatal diagnosis, obstetric issues, and management of the newborn. 4,[13][14][15][16] However, based on recent studies, ICH affects 3.5 to 4% of all hemophilic children in the neonatal period, which is much higher than normal population. 4,16 The frequency of ICH in hemophilic patients after the neonatal period is 290 to 796 per 10 6 patients in a year (prevalence of 2.7-12%) which, compared with the nonhemophilic population, is $20 to 50 times higher.…”

Section: Intracranial Hemorrhage In Congenital Bleeding Disordersmentioning

confidence: 99%

Intracranial Hemorrhage: A Devastating Outcome of Congenital Bleeding Disorders—Prevalence, Diagnosis, and Management, with a Special Focus on Congenital Factor XIII Deficiency

Alavi

Jalalvand

Assadollahi

et al. 2017

Semin Thromb Hemost

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Intracranial hemorrhage (ICH) is a medical emergency. In congenital bleeding disorders, ICH is a devastating presentation accompanied with a high rate of morbidity and mortality. The prevalence of ICH is highly variable among congenital bleeding disorders, with the highest incidence observed in factor (F) XIII deficiency (FXIIID) (∼30%). This life-threatening presentation is less common in afibrinogenemia, FVIII, FIX, FVII, and FX deficiencies, and is rare in severe FV and FII deficiencies, type 3 von Willebrand disease and inherited platelet function disorders (IPFDs). In FXIIID, this diathesis most often occurs after trauma in children, whereas spontaneous ICH is more frequent in adults. About 15% of patients with FXIIID and ICH die; the bleeding causes 80% of deaths in this coagulopathy. Although in FXIIID, the bleed most commonly is intraparenchymal (> 90%), epidural, subdural, and subarachnoid hemorrhages also have been reported, albeit rarely. As this life-threatening bleeding causes neurological complications, early diagnosis can prevent further expansion of the hematoma and secondary damage. Neuroimaging plays a crucial role in the diagnosis of ICH, but signs and symptoms in patients with severe FXIIID should trigger replacement therapy even before establishment of the diagnosis. Although a high dose of FXIII concentrate can reduce the rate of morbidity and mortality of ICH in FXIIID, it may occasionally trigger inhibitor development, thus complicating ICH management and future prophylaxis. Nevertheless, replacement therapy is the mainstay of treatment for ICH in FXIIID. Neurosurgery is performed in patients with FXIIID and epidural hematoma and a hemorrhage diameter exceeding 2 cm or a volume of ICH is more than 30 cm. Contact sports are not recommended in people with FXIIID as they can elicit ICH. However, a considerable number of safe sports and activities have been suggested to have more benefits than dangers for patients with congenital bleeding disorders, and are hence suitable for these patients.

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