Successful perioperative management using prothrombin complex concentrates in patients with severe congenital protein C deficiency

Inoue, Kosuke; Arakawa, Yuki; Noguchi, Junjirō; Irikura, Tomoya; Watakabe, Mai; Hiraki, Takamasa; Honda, Mamoru; Mitani, Yuichi; Mori, Makiko; Fukuoka, Kohei; Oshima, Koichi; Kawashima, Hiroshi; Kurihara, Jun; Koh, Katsuyoshi

doi:10.1002/pbc.29380

Cited by 2 publications

(2 citation statements)

References 9 publications

(10 reference statements)

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“…Although DOACs are available for use in both adult and pediatric patients with inherited thrombophilia, 46–48 the clinical utility needs to be assessed according to the age of pediatric patients. PCC, which is limitedly licensed for hemophilia B in Japan, has been effectively used for PC‐deficient patients with biallelic variants 49,50 . Prenatal diagnosis of severe PC deficiency may allow initiation of preemptive replacement, leading to subsequent complication‐free control.…”

Section: Discussionmentioning

confidence: 99%

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The clinical and genetic landscape of early‐onset thrombophilia in Japan

Egami,

Ishimura,

Ochiai

et al. 2023

Pediatric Blood & Cancer

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ObjectivesTo determine the optimal management for early‐onset thrombophilia (EOT), the genetic and clinical features of protein C (PC)‐, protein S (PS)‐, or antithrombin (AT)‐deficient patients of ≤20 years of age were studied in Japan.Methods/resultsClinical and genetic information of all genetically diagnosed cases was collected through the prospective, retrospective study, and literature review. One‐hundred‐one patients had PC (n = 55), PS (n = 29), or AT deficiency (n = 18). One overlapping case had PC‐ and PS‐monoallelic variant. Fifty‐five PC‐deficient patients (54%) had 26 monoallelic or 29 biallelic variant(s), and 29 (29%) PS‐deficient patients had 20 monoallelic or nine biallelic variant(s). None of the patients had AT‐biallelic variants. The frequent low‐risk allele p.K193del (PC‐Tottori) was found in five patients with monoallelic (19%) but not 29 with biallelic variant(s). The most common low‐risk allele p.K196E (PS‐Tokushima) was found in five with monoallelic (25%) and six with biallelic variant(s) (67%). One exceptional de novo PC variant was found in 32 families with EOT. Only five parents had a history of thromboembolism. Thrombosis concurrently developed in three mother–newborn pairs (two PC deficiency and one AT deficiency). The prospective cohort revealed the outcomes of 35 patients: three deaths with PC deficiency and 20 complication‐free survivors. Neurological complications were more frequently found in patients with PC‐biallelic variants than those with PC‐, PS‐, or AT‐monoallelic variants (73% vs. 24%, p = .019).ConclusionsWe demonstrate the need for elective screening for EOT targeting PC deficiency in Japan. Early prenatal diagnosis of PC deficiency in mother–infant pairs may prevent perinatal thrombosis in them.

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Section: Discussionmentioning

confidence: 99%

“…PCC, which is limitedly licensed for hemophilia B in Japan, has been effectively used for PC-deficient patients with biallelic variants. 49,50 Prenatal diagnosis of severe PC deficiency may allow initiation of preemptive replacement, leading to subsequent complication-free control.…”

Section: Discussionmentioning

confidence: 99%

The clinical and genetic landscape of early‐onset thrombophilia in Japan

Egami,

Ishimura,

Ochiai

et al. 2023

Pediatric Blood & Cancer

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Platelets at the intersection of inflammation and coagulation in the APC‐mediated response to myocardial ischemia/reperfusion injury

Slotabec,

Seale,

Wang

et al. 2024

The FASEB Journal

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Thromboinflammation is a complex pathology associated with inflammation and coagulation. In cases of cardiovascular disease, in particular ischemia–reperfusion injury, thromboinflammation is a common complication. Increased understanding of thromboinflammation depends on an improved concept of the mechanisms of cells and proteins at the axis of coagulation and inflammation. Among these elements are activated protein C and platelets. This review summarizes the complex interactions of activated protein C and platelets regulating thromboinflammation in cardiovascular disease. By unraveling the pathways of platelets and APC in the inflammatory and coagulation cascades, this review summarizes the role of these vital mediators in the development and perpetuation of heart disease and the thromboinflammation‐driven complications of cardiovascular disease. Furthermore, this review emphasizes the significance of the counteracting effects of platelets and APC and their combined role in disease states.

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Successful perioperative management using prothrombin complex concentrates in patients with severe congenital protein C deficiency

Cited by 2 publications

References 9 publications

The clinical and genetic landscape of early‐onset thrombophilia in Japan

The clinical and genetic landscape of early‐onset thrombophilia in Japan

Platelets at the intersection of inflammation and coagulation in the APC‐mediated response to myocardial ischemia/reperfusion injury

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