Factor products

Barton, Cassie A.; Bierman, Jesse

doi:10.1002/jac5.1069

Cited by 2 publications

(2 citation statements)

References 42 publications

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“…19 Manifestasi klinis pasien dengan penghambat secara umum ditandai dengan makin Tabel 6. Produk faktor IX rekombinan 7,15…”

Section: Analisisunclassified

Hemofilia dan Perkembangan Terapinya

Lawrenti

2021

CDK

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Hemofilia A dan B adalah gangguan perdarahan yang diturunkan yang ditandai dengan defisiensi protein koagulasi faktor VIII dan IX. Terapi pengganti dengan faktor pembekuan (konsentrat faktor VIII atau IX dari plasma atau rekombinan) masih merupakan terapi hemofilia baik saat perdarahan maupun sebagai profilaksis. Terapi profilaksis merupakan terapi standar untuk pasien hemofilia berat karena dapat mencegah kerusakan sendi, menurunkan frekuensi perdarahan sendi dan lainnya, serta memperbaiki kualitas hidup. Keputusan memilih terapi harus berdasarkan pertimbangan medis dan klinis, dan bersifat individual.Hemophilia A and B are inherited bleeding disorders characterised by deficiency of coagulation protein factor VIII and IX, respectively. Replacement therapy with clotting factors (factor VIII or IX through plasma-derived or recombinant concentrate) is the mainstay of treatment at time of bleeding or as prophylactic based on individual needs. Prophylaxis treatment is standard treatment for patients with severe hemophilia as it prevents joint damage, reduces frequency of joint and other bleeding and improves quality of life. Choice of therapy should be based solely on medical and clinical considerations and individualized.

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“…19 Manifestasi klinis pasien dengan penghambat secara umum ditandai dengan makin Tabel 6. Produk faktor IX rekombinan 7,15…”

Section: Analisisunclassified

Hemofilia dan Perkembangan Terapinya

Lawrenti

2021

CDK

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“…The following formulas [ 19 ] can be used for the replacement of FVIII and FIX in acute medical situations:…”

Section: Reviewmentioning

confidence: 99%

Skewed Inactivation of X Chromosome: A Cause of Hemophilia Manifestation in Carrier Females

Shoukat

Ghous

Tarar

et al. 2020

Cureus

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Hemophilia is an X-linked recessive hereditary disorder that classically affects males due to the presence of only one X chromosome in males. Females are usually carriers due to the presence of counterpart X chromosome, but many times manifestations of hemophilia are seen in heterozygous carrier females. This is a result of skewed lionization, in which more normal X chromosomes are converted to bar body, and more abnormal chromosomes remain active in body cells, causing the dominant manifestation of the disease. The severity of manifestations is directly proportional to the level of the clotting factor in the blood. The disease can be severe enough to cause life-threatening bleeding, especially during delivery. Physicians usually reluctant to assume hemophilia in the differential diagnosis of the bleeding disorders in women but manifesting carrier females with hemophilia are not uncommon. Our review of the literature will give an opportunity to understand this issue more precisely as well as will discuss the disease manifestations and its updated management.

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Factor products

Cited by 2 publications

References 42 publications

Hemofilia dan Perkembangan Terapinya

Hemofilia dan Perkembangan Terapinya

Skewed Inactivation of X Chromosome: A Cause of Hemophilia Manifestation in Carrier Females

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