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N.G. Andersson et al.
Table 5. Known and unknown family history of hemophilia.
All Known family history Unknown family history Family of hemophilia of hemophilia history not
known n n(%) n(%) n
ICH 20 8(1.7) 12(2.7) 0
Major bleeds 44
Vaginal delivery 633 VD without instruments 541 VD instrumental 68 Not known with/without instruments 24
Cesarean section 293 Planned CS 134
Reason: Hemophilia
22 (4.7)
299 (64.2) 285
14
13
167 (35.8)* 84
37 12
20 (4.5) 2
322 (72.4) 12 274 5 48 6 10 1
123
(27.6) 3 49 1
- 0 - 0
Combined hemophilia and maternal
or fetal status
Maternal
Fetal 9120
16
0 2 0
9/1 3/0 0
466 445 15
32 1
Combined maternal/fetal
Other/unknown
Total number
926
*Significant at P<0.05 in comparison to unknown family history of hemophilia. n: number; ICH: intracranial hemorrhage;VD: vaginal delivery; CS: Cesarean section.
found in the rate of major bleeds and intracranial hemor- rhage in neonates with moderate and severe hemophilia between vaginal delivery and Cesarean section: major bleeds occurred in 4.3% neonates born by VD and in 5.8% after CS (P=not significant, ns); ICH in 2.4% follow- ing VD and 1.7% after CS (P=not significat, ns). Further analysis of subgroups by MOD (VD with and without instruments, CS prior to and during labor) revealed instru- mental VD as a risk factor for major bleeds and intracra- nial hemorrhage compared to non-instrumental VD (RR 7.39 major bleed; 6.96 ICH) and CS prior to labor. Neonates with moderate hemophilia had a similar risk regarding ICH compared to severe hemophilia, but a lower risk for other major bleeds. No other significant dif- ferences were found between the subgroups of vaginal delivery without instruments, CS prior and CS during labor. The CS rate was higher in neonates with a known family history and reason was hemophilia in more than half of the cases. However, no significant difference was found between the group with and without a known fam- ily history of hemophilia regarding major bleeds and ICH. The comparison of planned CS (including CS prior to labor in 93.2% but also including some cases during labor) to planned VD (including VD without instruments in 77% and also instrumental VD and CS during labor) also showed no significant difference in the group with known family history
To our knowledge, this study represents the largest prospective, monitored series recording comprehensive data on mode of delivery and neonatal bleeds in patients with moderate and severe hemophilia. The data come from countries with a good and quite uniform standard of health care which should make the results relevant and applicable for these countries, although the results may also be applicable to countries with different health care standards. Due to the inclusion of all consecutive cases in the participating centers, selection bias should be low but cannot be totally excluded. Missing data is a problem for
most registries. Earlier publications of the PedNet registry show high quality data regarding baseline data and first 75 exposure days including bleeds, with only 4% missing data.14 The subanalysis on detailed information on mode of delivery (vaginal with instruments, CS prior to or dur- ing labor, reason for CS) was available in 813 of 926 (87.8%) patients, which means that in 12.2% of included patients these data are missing, which is still acceptable for analysis. However, the frequencies for both major bleeds and ICH are low, and small differences between the groups analyzed cannot be detected due to the limited number of included patients. For example, to show a dif- ference between ICH in vaginal delivery of 2.4% to CS of 1.7%, over 12,000 patients would have to be included, which is not feasible. We excluded mild hemophilia from our analysis since the diagnosis in these patients is often made at an older age and undiagnosed cases born during the study period have not yet been included in the PedNet Registry.15
Both term and preterm neonates were included in the calculations and one could question if data should have been presented separately since ICH is a well-known complication of, in particular, delivery of an extremely premature neonate.16 Our series included 849 children with data on gestational week of birth, of whom 62 (7.3%) were born prematurely, but only 11 children (1.3%) were born before the 33rd gestational week, i.e. very or extremely premature. There were three major bleeds in the premature group (5.2%) and no cases of ICH. In term births, 41 major bleeds in 787 neonates (6.4%) occurred, and 20 ICH (20 of 787; 2.5%). Since there was no significant difference in the frequency of bleeds between the term and preterm groups we considered it justified to merge them together in the calculations. The premature group was still a rather small group, and a much larger group would be needed in order to draw any conclusions between the more extremely premature and less premature on this issue. In another neonatal series,
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