Hemophilia B (PTC Deficiency, or Christmas Disease)

Quick, Armand J.; Hussey, Clara V.

doi:10.1001/archinte.1959.00270050084014

Cited by 36 publications

(25 citation statements)

References 28 publications

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“…1 This dramatic brief T-cell lymphopenia has previously been described in both cancer and HIV clinical trials of rhIL-7. 2,3 Tissue redistribution of T lymphocytes had been invoked to explain this unusual occurrence but there was no data supporting this hypothesis until now.…”

Section: Irini Sereti National Institutes Of Healthmentioning

confidence: 83%

“…1 Quick's suggestion has been supported by some more recent data. In a preliminary Canadian report, adult severe hemophilia B patients were found to have had 35% fewer bleeds than severe hemophilia A patients, 2 while a second Canadian study, on virtually the whole of the Canadian hemophilia population, reported that significantly more severe hemophilia A patients used prophylaxis (69%) compared with severe hemophilia B patients (32%).…”

mentioning

confidence: 74%

“…uPAR Ko mice are deficient in a series of important functions (inflammation, bone homeostasis, kidney and hematopoietic stem cells mobilization and homing). [1][2][3][4] Some of these have been linked to human pathology.…”

Section: Acknowledgment: This Work Was Supported By the Intramural Rementioning

confidence: 99%

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Where have all the T cells gone?

Sereti¹

2009

Blood

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Section: Irini Sereti National Institutes Of Healthmentioning

confidence: 83%

mentioning

confidence: 74%

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Where have all the T cells gone?

Sereti¹

2009

Blood

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“…physical activity, trauma, the state of the underlying joint and how the patientÕs underlying haemostatic system responds to replacement therapy. There is also debate as to whether the same level of FVIII or FIX has an identical effect on the haemostatic system [32][33][34][35], and it is possible that adequate trough levels for prophylaxis may differ between the two disorders.…”

Section: Pharmacokinetic Parameters and Break Through Bleeds During Pmentioning

confidence: 99%

Implications of coagulation factor VIII and IX pharmacokinetics in the prophylactic treatment of haemophilia

Fischer²,

et al. 2010

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Summary. The pharmacokinetic (PK) response to factor VIII (FVIII) and factor IX varies between patients and this has important clinical implications for treatment. Although PK is affected by patient characteristics, this relationship is too weak to infer a result for an individual and, if required, PK must be measured. An important determinant of the efficacy of prophylaxis is the length of time an individual spends with a low level of coagulation factor. This time is more dependent on the patientÕs coagulation factor half-life and the frequency of dosing than in vivo recovery and dose infused. Improved understanding of the effect of PK and dose frequency on factor levels in patients on prophylaxis will help tailor regimens to individuals better and allow more cost effective use of coagulation factor concentrates. Calculations suggest that adults need less FVIII per kg body weight than children. The effect of half-life on trough levels questions the logic of Monday, Wednesday, Friday dosing and suggests a role for innovative regimens including low-dose daily treatment which leads to either higher trough levels or decreased FVIII requirement. This may expand access to prophylaxis in healthcare systems with limited resources and potentially improve patient outcomes. The ideal trough level will vary between individuals and at different times of their lives and may be <1 IU dL )1. If PK is to be used in routine clinical practice, a simplified method for its measurement is required and this methodology is becoming available.

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“…More than 50 years ago, prior to the availability of clotting factor concentrates, Quick et al noticed that severe HB was less handicapping than HA. 6 More recently, many studies demonstrated a higher use of continuous prophylaxis and greater factor consumption in severe HA patients compared with those with severe HB. [7][8][9][10] Moreover, Tagariello et al, in a retrospective survey of joint arthroplasty in the frame of the Italian Hemophilia Center Association, showed that patients with HA had a 3-fold higher risk of undergoing orthopedic arthroplasty, that is an indirect expression of severity of arthropathy.…”

Section: Introductionmentioning

confidence: 99%