hemophilia a/crise épileptique

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Convulsions and respiratory arrest in association with desmopressin administration for the treatment of a bleeding tonsil in a child with borderline haemophilia.

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Desmopressin (DDAVP) may be used to augment the action of factor VIII in mild haemophilia. Its use has been associated with serious adverse effects. We report a case of a three-year-old child with a family history of haemophilia who suffered complications due to severe acute hyponatraemia following

Unusual presentation of haemophilia in two paediatric patients.

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Haemophilia A is a rare X-linked recessive bleeding disorder caused by deficiency or functional defects in coagulation factor VIII (FVIII). Here, we report two cases of challenging diagnosis of haemophilia A because of unusual presentation. The first case is a 10-month-old female, admitted to our

A Retrospective Analysis of Intracranial Hemorrhage in Children with Hemophilia A.

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To investigate the incidence, risk factors, clinical manifestations and prognosis of intracranial hemorrhage (ICH) in children with hemophilia A in a center of China, we conducted a retrospective analysis of 126 children with hemophilia A at our hospital in recent 4 years. Thirty-six children with

Surgical management of intractable epilepsy in children with hemophilia.

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Intracranial hemorrhage occurs in 2-8% of patients with hemophilia and can result in neurologic sequelae including seizure disorders. There is a paucity of data concerning the surgical treatment of epilepsy in children with hemophilia. We review our experience with 2 children who developed medically

Hemophilia in the newborn without family history--pattern of clinical presentation of three patients.

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BACKGROUND Hemophilia is the most frequently diagnosed inborn clotting factor deficiency in the newborn. In about half of the cases diagnosis is made during neonatal period. However, due to different clinical presentation comparing to older children, hemophilia in the newborn could be misdiagnosed,

[Haemophilia A and intracranial bleedings in infants].

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OBJECTIVE Haemophilia is a well-known X-linked recessive bleeding disorder related to a deficiency of factor VIII. The aim of the paper is to point out the role of an early diagnosis in cases of intracranial bleeding, especially in neonates and to highlight the bad prognosis. METHODS Three patients

Posterior Fossa Hemorrhage in a Term Neonate with Hemophilia A.

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Posterior fossa hemorrhage is rare in term baby and difficult to assess. The clinical signs are nonspecific and usually delay the diagnosis. We present a 5-day-old male neonate of posterior fossa hemorrhage with the initial presentations of fever and seizure and early deduced by cranial

Congenital Hemophilia A Presenting With Hashimoto's Encephalopathy and Myocarditis: The First Reported Case.

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Here, we report a case of hemophilia A with myocarditis, encephalopathy, and spontaneous intramedullary hemorrhage. A 14-month-old male infant presented with loss of consciousness, generalized tonic-clonic convulsions, and cardiac failure. The neurological examination was normal. Myocarditis was

Hyponatremia and seizures in young children given DDAVP.

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Desmopressin (DDAVP), a synthetic vasopressin, temporarily corrects bleeding abnormalities associated with mild hemophilia A, von Willebrand disease, and disorders of platelet function. The side effects of DDAVP are considered benign although most of its use has been in adults and older children. We

Acute Life-Threatening Hemorrhage in Neonates With Severe Hemophilia A: A Report of 3 Cases.

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Hemorrhagic shock is a rare, emergent condition that is often fatal in newborns. In this article, we report cases of 3 neonates presenting with acute, life-threatening hemorrhage who were subsequently diagnosed with severe hemophilia (<1% factor VIII). The first infant was tachycardic, pale, and had

Cerebral infarct associated with factor V Leiden mutation in a boy with hemophilia A.

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An 11-year-old boy with mild hemophilia A was admitted to our hospital because of focal convulsions. Magnetic resonance imaging showed an old occipital infarct. Protein C, S, antithrombin III, anticardiolipin antibodies and fibrinogen were normal. Heterozygosity for factor V Leiden mutation was

Acquired hemophilia, meningioma, and diphenylhydantoin therapy.

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A patient is reported in whom a meningioma of the lateral one-third of the sphenoid ridge was completely removed and long-term prophylaxis for seizures with diphenylhydantoin was prescribed. One and a half years later, a powerful inhibitor developed that specifically neutralized Factor VIII, the

Munchausen syndrome presenting as hemophilia: a convenient and economical "steal" of disease and treatment.

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Munchausen's syndrome and Munchausen's syndrome by proxy (MSBP) can cause many conditions, including bleeding problems, seizures, failure to thrive and others. We report here an unusual case in which a mother presented to the hospital for her hemophiliac son's failure to thrive, subsequently for her

Atypical indications for OtoScan laser-assisted myringotomy.

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OBJECTIVE To describe the role of OtoScan laser-assisted myringotomy (OtoLAM) for indications other than chronic otitis media or recurrent acute otitis media. METHODS Cross-sectional review. METHODS Twenty-seven office-based OtoLAM procedures were performed in 21 patients for "atypical" reasons. The

[Intracranial hemorrhage in hemophiliacs. Study of 10 episodes].

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The intracranial haemorrhages presented within a group of 64 haemophiliacs along 25 years were revised. During this period, 10 such episodes were seen in 8 patients with haemophilia A; six of them appeared in children under 10 years of age. In five instances there were traumatic antecedents, whereas

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