120) Overall, 4 (8.2%) died as a consequence of ICB (3 patients) and IAB (1 patient). |
PMID:24322277 DOI:10.1177/1076029613515070 |
2015 Clinical and applied thrombosis/hemostasis : official journal of the International Academy of Clinical and Applied Thrombosis/Hemostasis |
* Congenital Factor VII Deficiency in Children at Tertiary Health Care Facility in Pakistan. |
- This study presents the demographics, clinical spectrum, and outcome of patients with congenital factor VII (FVII) deficiency at a tertiary care center over a period of 12 years. Of the 49 patients, 27 (55%) patients were males. Consanguinity was found in 92% of the patients. The median age of symptom onset was 2.4 (interquartile range [IQR]: 1.1-6.5) years with a median age of 5.8 (IQR: 3.1-10) years at diagnosis. Life-threatening complications like intracranial bleeding (ICB) and intra-abdominal bleeding (IAB) were observed in 8 (16.4%) patients. We found that 11 (55%) of the 20 patients with FVII coagulant activity (FVIIc) <1% were either asymptomatic or showed mild phenotype. In contrast, 9 (53%) of the 17 patients with FVIIc >5% were affected by severe symptoms. Age <1 year was the only identified risk factor associated with development of life-threatening bleeding episodes (P = .042; odds ratio 6.46). Overall, 4 (8.2%) died as a consequence of ICB (3 patients) and IAB (1 patient). |
(1)52 with | (17)4 MSCs | (33)3 subsequently | (49)2 motivation |
(2)30 was | (18)4 education | (34)2 Education | (50)2 of |
(3)20 *null* | (19)4 presenting | (35)2 achieved | (51)2 outcome |
(4)18 and | (20)4 required | (36)2 age | (52)2 pain |
(5)15 had | (21)4 safety | (37)2 as | (53)2 population |
(6)12 who | (22)4 to | (38)2 assessment | (54)2 population, |
(7)11 care | (23)4 were | (39)2 being | (55)2 received |
(8)7 is | (24)3 characteristics | (40)2 can | (56)2 remained |
(9)7 survival | (25)3 comfort | (41)2 computational | (57)2 remains |
(10)7 underwent | (26)3 died | (42)2 data | (58)2 reported |
(11)6 developed | (27)3 group | (43)2 days | (59)2 response |
(12)6 outcomes | (28)3 information | (44)2 empowerment | (60)2 responses |
(13)6 satisfaction | (29)3 life | (45)2 factors, | (61)2 symptoms |
(14)5 groups | (30)3 on | (46)2 for | (62)2 the |
(15)5 in | (31)3 postoperative | (47)2 immunomigration | |
(16)5 presented | (32)3 referral | (48)2 including |
add keyword