Intracranial Haemorrhage In Haemophilia Research Articles

Incidence and mortality rates of intracranial hemorrhage in hemophilia: a systematic review and meta-analysis

AbstractIntracranial hemorrhage (ICH) is a severe complication that is relatively common among patients with hemophilia. This systematic review aimed to obtain more precise estimates of ICH incidence and mortality in hemophilia, which may be important for patients, caregivers, researchers, and health policy makers. PubMed and EMBASE were systematically searched using terms related to “hemophilia” and “intracranial hemorrhage” or “mortality.” Studies that allowed calculation of ICH incidence or mortality rates in a hemophilia population ≥50 patients were included. We summarized evidence on ICH incidence and calculated pooled ICH incidence and mortality in 3 age groups: persons of all ages with hemophilia, children and young adults younger than age 25 years with hemophilia, and neonates with hemophilia. Incidence and mortality were pooled with a Poisson-Normal model or a Binomial-Normal model. We included 45 studies that represented 54 470 patients, 809 151 person-years, and 5326 live births of patients with hemophilia. In persons of all ages, the pooled ICH incidence and mortality rates were 2.3 (95% confidence interval [CI], 1.2-4.8) and 0.8 (95% CI 0.5-1.2) per 1000 person-years, respectively. In children and young adults, the pooled ICH incidence and mortality rates were 7.4 (95% CI, 4.9-11.1) and 0.5 (95% CI, 0.3-0.9) per 1000 person-years, respectively. In neonates, the pooled cumulative ICH incidence was 2.1% (95% CI, 1.5-2.8) per 100 live births. ICH was classified as spontaneous in 35% to 58% of cases. Our findings suggest that ICH is an important problem in hemophilia that occurs among all ages, requiring adequate preventive strategies.

Management of intracranial hemorrhage in hemophilia A patients

Intracranial hemorrhage due to head trauma in patients with hemophilia is a major cause of mortality and morbidity. The incidence of central nervous system bleeding in these patients is between 3 and 8%. In this study, we shared our experience on the treatment and follow-up of seven pediatric patients with hemophilia A who had intracranial bleeding due to trauma. Between 2010 and 2019, the clinical and radiological findings of seven pediatric patients with hemophilia A with intracranial hemorrhage were retrospectively evaluated in our clinic. One patient underwent operation owing to intracranial hemorrhage, and the other six underwent conservative treatment. Hemoglobin, aPTT (activated partial thromboplastin time), and factor VIII levels were measured at regular intervals. For intracranial hemorrhage follow-up, regular computed tomography (CT) was performed. All patients visited the emergency department with initial neurological complaints. Further, two of the seven patients died, one was treated with sequelae (cerebral palsy), and the other four were treated without sequelae. Early diagnosis and treatment of intracranial hemorrhage is very important in patients with bleeding disorders. Factor VIII replacement should be performed in such patients prior to radiological examinations and consultations. The main objective should be to bring factor VIII levels to normal limits during their treatment and follow-up.

Characteristics of Intracranial Haemorrhage in Haemophilic Children-Report in Single Center in China

Objectives: Intracranial haemorrhage (ICH) is the most severe complication for patients with haemophilia. Our study objectives were to analyze the characteristics of Intracranial haemorrhage in haemophilia children.Methods: Intracranial haemorrhage cases in haemophilic children were retrospective analyzed from Jan. 2008 to Dec. 2013. Patients with sever trauma inhospitalized in the surgical department were excluded.Results: Of 439 severe or intermediate haemophilic children 29(6.6%) experienced 32 events of ICH. The 29 children included 20 haemophilia A boys and 9 heamophilia B boys. The age of onset of ICH from 1 month to 17 years old (mediate age 3 years old). Head trauma histories was found in 79.3% (23/29) children 1 day to 1 week before ICH. Of 29 patients 15 (51.7%) had the presentation of convulsion or coma. The haemorrhage site included 2 events in extradural, 2 events in subdural, 6 events in brain parenchyma, 1 event in subarachnoid space, 21 events had multiple sites ICH. The ICH mortality was 6.9% (2/29). Only two children had done prophylaxis treatment with low dose (10-15u/kg, 2/w) before ICH. Inhibitor were only tested in 8 children who were all positive when ICH occurred.Conclusion: The most significant risk factors for ICH included the presence of an inhibitor and reported head trauma. Our patients with ICH had low mortality maybe because most patients had no inhibitor who could have good response to factor treatment, and these patients had excluded patients with sever trauma in the surgical department. DisclosuresNo relevant conflicts of interest to declare.

Effect of intracranial bleeds on the neurocognitive, academic, behavioural and adaptive functioning of boys with haemophilia

Brain insults are a risk factor for neuropsychological and academic deficits across several paediatric conditions. However, little is known about the specific effects of intracranial haemorrhage (ICH) in boys with haemophilia. The study compared neurocognitive, academic and socio-emotional/behavioural outcomes of boys with haemophilia with and without a history of ICH. Of 172 consecutive patients seen at a Pediatric Comprehensive Care Hemophila Centre, 18 had a history of ICH. Sixteen boys between the ages of 3 and 17 years were available for study and were matched to controls with haemophilia of the same age and disease severity and on the basis of maternal education. Groups were compared on neuropsychological and academic outcomes. Attention, socio-emotional function and executive skills were compared using data from parent questionnaires. Differences were found in intellectual function, visual-spatial skill, fine motor dexterity and particularly language-related skills, including vocabulary, word reading and applied math problem solving. Despite these group differences, outcomes were within the average range for most boys with ICH. No group differences were found in behavioural and socio-emotional functioning. Although ICH in haemophilia is not benign, it was not associated with significant cognitive and academic consequences for most boys. Early neuropsychological assessment may be indicated when there is a history of ICH. Investigation of age at ICH and quantitative measures of brain in relation to neurocognitive outcomes in larger groups of boys with ICH would be useful.

Intracranial haemorrhage in haemophilia A and B

In countries with a good standard of health care, intracranial haemorrhage (ICH) during the neonatal period affects 3.5-4.0% of all haemophilia boys, which is considerably (40-80 times) higher than expected in the normal population. ICHs are also frequent after the neonatal period, affecting 3-10% of the haemophilia population who are mainly treated on demand. The risk is higher in inhibitor patients. Spontaneous haemorrhage is reported more frequently than trauma-induced haemorrhage in most studies. The prevalence of ICH in patients treated with a prophylactic regimen is not known. Although more frequent in younger patients, a substantial proportion of ICH occur in adults, suggesting that general risk factors because of age, such as hypertension, are increasingly important as the haemophiliac gets older. Some studies have reported a substantial proportion of ICH affecting patients with milder forms of haemophilia. The risk of ICH has to be considered when discussing treatment strategies for haemophilia patients.

P-2-170 - Intracranial hemorrhage in hemophilia

P-2-170 - Intracranial hemorrhage in hemophilia

Recombinant activated factor VII (rFVIIa) therapy for intracranial hemorrhage in hemophilia A patients with inhibitors.

We report the use of recombinant VIIa (rFVIIa) in the treatment of five ICHs in two factor VIII-deficient patients with inhibitors. In four of five ICHs, rFVIIa was the only factor replacement used at doses of 60-135 micrograms/kg every 2-4 hr for 12-14 days. Hemostasis at the primary site of bleeding was achieved in all cases, and all patients survived with no permanent neurologic deficits. However, the patient who received the highest dose of rFVIIa during the first 4 days of therapy developed clinical symptoms consistent with a cerebral vascular accident of the brainstem characterized by acute onset of truncal ataxia and upward-gaze nystagmus on day 8 of rFVIIa therapy. While receiving rFVIIa therapy for treatment of these five ICHs, four treatment courses were complicated by bleeding at sites other than the primary site, including two episodes of localized oozing at central line insertion sites, two episodes of hemarthrosis, and two episodes of epistaxis. Antifibrinolytic therapy with tranexamic acid was effective in two of these episodes. Laboratory evaluation revealed shortening of the PT, variable shortening without normalization of the APTT, peak factor VII activity levels 7-30-fold higher than normal baseline values, and normal antithrombin III (ATIII) and alpha 2-antiplasmin levels. In four of five ICHs, there was a 20-40% decrease in fibrinogen levels from baseline. The decrease in fibrinogen was accompanied by an increase in fibrin degradation products in 3/5 episodes and a 15-35% decrease in plasminogen activity levels in 2/5 episodes. Tissue factor pathway inhibitor (TFPI) levels remained stable and in the normal range. Although rFVIIa is an effective new therapy for the treatment of ICH in hemophilia patients with inhibitors, its optimal use with respect to safety and efficacy requires further clinical study.

Intracranial haemorrhage in haemophilia in Nigerians

This paper reports the occurrence of intracranial haemorrhage in 3 Nigerians with classical haemophilia. As far as we are aware no instance of intracranial haemorrhage was mentioned in previous reports on haemophilia from other parts of Africa south of the Sahara.

Intracranial Hemorrhage In Hemophilia

To the Editor.— Despite Hattersley's strong exception ( 216 :2144, 1971) to my statement that "... intracranial hemorrhage in hemophilia is rare," I agree fully with his view and with his suggestion on the handling of head injuries. Fortunately, spontaneous intracranial bleeding is rare but head injuries will continue to occur. The following case illustrates how rewarding prompt and proper treatment can be. Report of a Case.— The patient was born in 1949 and the diagnosis of hemophilia A was made at the age of 11 months on the basis of a clotting time of 60 minutes and a prothrombin consumption time of nine seconds. He had the typical episodes of bleeding which responded well to local treatment of cold and pressure and to transfusions of fresh-frozen plasma. On July 4, 1965, he fell from his bicycle and bruised his forehead above the left eye. Two days later, he received 250 ml

Intracranial hemorrhage in hemophilia

Intracranial hemorrhage in hemophilia

Intracranial Hemorrhage In Hemophilia

ABSTRACT To the Editor.— In a recent LETTER TO THE EDITOR (216:518, 1971), Quick made a statement to which I must take strong exception. He said: ... intracranial hemorrhage in hemophilia is rare.I am of course aware of Dr. Quick's many contributions in the field of the coagulopathies. And, of course he is literally right in his statement. Intracranial bleeding in the hemophiliac is rare, compared with hemarthrosis, intramuscular hematoma, or renal bleeding. On the other hand, when it occurs, it is not infrequently the cause of death.I feel that a known hemophiliac who has received trauma to the head should be observed closely for evidences of bleeding, and should be treated vigorously on the first appearance of such signs. I have on several occasions treated such patients preventively with infusions of factor VIII.

Intracranial hemorrhage in hemophilia

Intracranial hemorrhage in hemophilia

Management of intracranial haemorrhage in haemophilia.

Management of intracranial haemorrhage in haemophilia.

Management of intracranial haemorrhage in hemophilia.

Management of intracranial haemorrhage in hemophilia.

Intracranial Haemorrhage in Haemophilia

Intracranial Haemorrhage in Haemophilia

INTRACRANIAL HAEMORRHAGE IN HAEMOPHILIA.

INTRACRANIAL HAEMORRHAGE IN HAEMOPHILIA.