0
We're unable to sign you in at this time. Please try again in a few minutes.
Retry
We were able to sign you in, but your subscription(s) could not be found. Please try again in a few minutes.
Retry
There may be a problem with your account. Please contact the AMA Service Center to resolve this issue.
Contact the AMA Service Center:
Telephone: 1 (800) 262-2350 or 1 (312) 670-7827  *   Email: subscriptions@jamanetwork.com
Error Message ......
Article |

Initial Presentations of Pediatric Hemophiliacs

James H. Conway, MD; Margaret W. Hilgartner, MD
Arch Pediatr Adolesc Med. 1994;148(6):589-594. doi:10.1001/archpedi.1994.02170060043007.
Text Size: A A A
Published online

Objective:  To examine the manner of presentation and time elapsed before diagnosis in a current population of pediatric patients with coagulopathies compared with a historically similar group presented by Baehner and Strauss in 1966.

Design:  Comparative retrospective patient series.

Setting:  A large pediatric (hemophilia) referral center in New York, NY.

Population:  Sixty-five male patients presented for evaluation of a presumed coagulopathy between 1974 and 1989. Nine patients were excluded for inadequate data or follow-up. Of the 56 patients included, 47 were were factor VIII deficient (32 severely affected, 15 not severely affected) and nine were factor IX deficient (six severely affected, three not severely affected).

Interventions:  None.

Results:  Severely affected neonates were diagnosed younger than 1 month significantly more often in this patient population compared with the 1966 population (68.4% vs <10%, P<.001). Patients not severely affected were also diagnosed younger than 1 month significantly more often than in 1966(50% vs 2.5%, P<.001). Workup in these groups was initiated because of either bleeding events or family history with similar frequency (48.6% vs 51.4%); historically, diagnosis had ensued primarily after bleeding events. By 1 year of age, all severely affected infants had been diagnosed in our population, a statistically significant improvement compared with the historical group (100% vs <40%, P<.001); patients not severely affected were also diagnosed more frequently (72.2% vs 15%, P<.001). Eighty-five percent of diagnoses made after 1 month of age were based on bleeding events, despite a positive family history of 28.6%. Overall, 35.7% of diagnoses resulted after a positive family history was elicited.

Conclusions:  In this population, diagnoses were made earlier than in the comparison group. Attention to family history and early bleeding events continues to contribute to the early diagnosis of factor deficiencies.(Arch Pediatr Adolesc Med. 1994;148:589-594)

Topics

Sign in

Create a free personal account to sign up for alerts, share articles, and more.

Purchase Options

• Buy this article
• Subscribe to the journal

Figures

Tables

References

Correspondence

CME
Meets CME requirements for:
Browse CME for all U.S. States
Accreditation Information
The American Medical Association is accredited by the Accreditation Council for Continuing Medical Education to provide continuing medical education for physicians. The AMA designates this journal-based CME activity for a maximum of 1 AMA PRA Category 1 CreditTM per course. Physicians should claim only the credit commensurate with the extent of their participation in the activity. Physicians who complete the CME course and score at least 80% correct on the quiz are eligible for AMA PRA Category 1 CreditTM.
Note: You must get at least of the answers correct to pass this quiz.
You have not filled in all the answers to complete this quiz
The following questions were not answered:
Sorry, you have unsuccessfully completed this CME quiz with a score of
The following questions were not answered correctly:
Commitment to Change (optional):
Indicate what change(s) you will implement in your practice, if any, based on this CME course.
Your quiz results:
The filled radio buttons indicate your responses. The preferred responses are highlighted
For CME Course: A Proposed Model for Initial Assessment and Management of Acute Heart Failure Syndromes
Indicate what changes(s) you will implement in your practice, if any, based on this CME course.
Submit a Comment

Multimedia

Some tools below are only available to our subscribers or users with an online account.

Sign in

Create a free personal account to sign up for alerts, share articles, and more.

Purchase Options

• Buy this article
• Subscribe to the journal

Related Content

Customize your page view by dragging & repositioning the boxes below.

Jobs
brightcove.createExperiences();