Details:

Alternative Title:
Haemophilia
Personal Author:
KULKARNI, R. ; PRESLEY, R. J. ; LUSHER, J. M. ; SHAPIRO, A. D. ; GILL, J. C. ; ... More +
KULKARNI, R. ; PRESLEY, R. J. ; LUSHER, J. M. ; SHAPIRO, A. D. ; GILL, J. C. ; MANCO-JOHNSON, M. ; KOERPER, M. A. ; ABSHIRE, T. C. ; DIMICHELE, D. ; HOOTS, W. K. ; MATHEW, P. ; NUGENT, D. J. ; GERAGHTY, S. ; EVATT, B. L. ; SOUCIE, J. M. Less -
Description:
Aim

To describe the prevalence and complications in babies ≤2 years with haemophilia.

Methods

We used a standardized collection tool to obtain consented data on eligible babies aged ≤2 years with haemophilia enrolled in the Centers for Disease Control and Prevention Universal Data Collection System surveillance project at US Hemophilia Treatment Centers (HTCs).

Results

Of 547 babies, 82% had haemophilia A, and 70% were diagnosed within one month of birth. Diagnosis was prompted by known maternal carrier status (40%), positive family history (23%), bleeding (35%) and unknown 2%; 81% bled during the first two years. The most common events were bleeding (circumcision, soft tissue, oral bleeding) and head injury. There were 46 episodes of intracranial haemorrhage (ICH) in 37 babies (7%): 18 spontaneous, 14 delivery related, 11 traumatic, 2 procedure related and 1 unknown cause. Of the 176 central venous access devices (CVADs) in 148 (27%) babies, there were 137 ports, 22 surgically inserted central catheters and 20 peripherally inserted central catheters. Ports had the lowest complication rates. Inhibitors occurred in 109 (20%) babies who experienced higher rates of ICH (14% vs. 5%; P = 0.002), CVAD placement (61% vs. 19%; P < 0.001) and CVAD complications (44% vs. 26%; P < 0.001). The most common replacement therapy was recombinant clotting factor concentrates.

Conclusion

Bleeding events in haemophilic babies ≤2 years were common; no detectable difference in the rates of ICH by the mode of delivery was noted. Neonatal factor exposure did not affect the inhibitor rates. Minor head trauma, soft tissue and oropharyngeal bleeding were the leading indications for treatment.


Subjects:
[+]
Article Child, Preschool Data Collection Female Hemophilia A Humans Infant Infant, Newborn Male
Source:
Haemophilia. 23(2):207-214.
Pubmed ID:
27813214
Pubmed Central ID:
PMC5354941
Document Type:
Journal Article
Funding:
CC999999/Intramural CDC HHS/United States
Name as Subject:
Centers for Disease Control and Prevention (U.S.)
Place as Subject:
United States
Collection(s):
CDC Public Access
Main Document Checksum:
[+]
urn:sha256:157000ff4895007587b03d56c133c8027a95757fac9a39aa04740084532c62a8
Download URL:
https://stacks.cdc.gov/view/cdc/44638/cdc_44638_DS1.pdf
File Type:

Supporting Files

• 	nihms836612.nxml	xml
  	nihms836612f1.gif	gif
  	nihms836612f1.jpg	jpeg

More +