Get 20M+ Full-Text Papers For Less Than $1.50/day. Start a 14-Day Trial for You or Your Team.

Learn More →

Initial Presentations of Pediatric Hemophiliacs

Initial Presentations of Pediatric Hemophiliacs Abstract 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) References 1. Baehner RL, Strauss HS. Hemophilia in the first year of life . N Engl J Med . 1966;275:524-528.Crossref 2. Buchanan GR. Hemophilia . Pediatr Clin North Am . 1980;27:309-326. 3. Kasper CK, Dietrich SL. Comprehensive management of hemophilia . Clin Haematol . 1985;14:489-512. 4. Schulman I. Pediatric aspects of the mild hemophilias . Med Clin North Am . 1962;46:93-105. 5. Buchanan GR. Coagulation disorders in the neonate . Pediatr Clin North Am . 1986;33:203-220. 6. Montgomery RR, Marlar RA, Gill JC. Newborn hemostasis . Clin Haematol . 1985; 14:443-460. 7. Bray GL, Luban NLC. Hemophilia presenting with intracranial hemorrhage . AJDC . 1987;141:1215-1217. 8. Franze I, Forrest TS. Sonographic diagnosis of a subdural hematoma as the initial manifestation of hemophilia in a newborn . J Ultrasound Med . 1988:7: 149-152. 9. Yonker PG, Graham-Pole J, Mehta P. Presentation of hemophilia A in the newborn period . J Fla Med Assoc . 1985;72:99-100. 10. Rohyans JA, Miser AW, Miser JS. Subgaleal hemorrhage in infants with hemophilia: report of two cases and review of the literature . Pediatrics . 1982; 70:306-307. 11. Olson TA, Alving BM, Cheshier JL, Landes RD, Ruyman FB. Intracerebral and subdural hemorrhage in a neonate with hemophilia A . Am J Pediatr Hematol Oncol . 1985;7:384-387. 12. Nixon RG, Brindley GW. Hemophilia presenting as compartment syndrome in the arm following venipuncture: a case report and review of the literature . Clin Orthop . 1989;244:176-181. 13. Tarsitano JJ, Cohen SM. Revelation and initial diagnosis of mild hemophilia from dental findings: report of case . J Am Dent Assoc . 1988;76:823-825. 14. Lazerson J. Persistent hematoma after mouth trauma . Hosp Pract (Off Ed) . 1981;16:28-29. 15. Sonis AL, Musselman RJ. Oral bleeding in classic hemophilia . Oral Surg . 1982; 53:363-366.Crossref 16. Daffos F, Forestier F, Kaplan C, Cox W. Prenatal diagnosis and management of bleeding disorders with fetal blood sampling . Am J Obstet Gynecol . 1988:158: 939-946.Crossref 17. Baty BJ, Drayna D, Leonard C, White R. Prenatal diagnosis of factor VIII deficiency to help with the management of pregnancy and delivery . Lancet . 1986; 1:207.Crossref http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Archives of Pediatrics & Adolescent Medicine American Medical Association

Initial Presentations of Pediatric Hemophiliacs

Loading next page...
 
/lp/american-medical-association/initial-presentations-of-pediatric-hemophiliacs-doK3FpltkI

References (18)

Publisher
American Medical Association
Copyright
Copyright © 1994 American Medical Association. All Rights Reserved.
ISSN
1072-4710
eISSN
1538-3628
DOI
10.1001/archpedi.1994.02170060043007
Publisher site
See Article on Publisher Site

Abstract

Abstract 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) References 1. Baehner RL, Strauss HS. Hemophilia in the first year of life . N Engl J Med . 1966;275:524-528.Crossref 2. Buchanan GR. Hemophilia . Pediatr Clin North Am . 1980;27:309-326. 3. Kasper CK, Dietrich SL. Comprehensive management of hemophilia . Clin Haematol . 1985;14:489-512. 4. Schulman I. Pediatric aspects of the mild hemophilias . Med Clin North Am . 1962;46:93-105. 5. Buchanan GR. Coagulation disorders in the neonate . Pediatr Clin North Am . 1986;33:203-220. 6. Montgomery RR, Marlar RA, Gill JC. Newborn hemostasis . Clin Haematol . 1985; 14:443-460. 7. Bray GL, Luban NLC. Hemophilia presenting with intracranial hemorrhage . AJDC . 1987;141:1215-1217. 8. Franze I, Forrest TS. Sonographic diagnosis of a subdural hematoma as the initial manifestation of hemophilia in a newborn . J Ultrasound Med . 1988:7: 149-152. 9. Yonker PG, Graham-Pole J, Mehta P. Presentation of hemophilia A in the newborn period . J Fla Med Assoc . 1985;72:99-100. 10. Rohyans JA, Miser AW, Miser JS. Subgaleal hemorrhage in infants with hemophilia: report of two cases and review of the literature . Pediatrics . 1982; 70:306-307. 11. Olson TA, Alving BM, Cheshier JL, Landes RD, Ruyman FB. Intracerebral and subdural hemorrhage in a neonate with hemophilia A . Am J Pediatr Hematol Oncol . 1985;7:384-387. 12. Nixon RG, Brindley GW. Hemophilia presenting as compartment syndrome in the arm following venipuncture: a case report and review of the literature . Clin Orthop . 1989;244:176-181. 13. Tarsitano JJ, Cohen SM. Revelation and initial diagnosis of mild hemophilia from dental findings: report of case . J Am Dent Assoc . 1988;76:823-825. 14. Lazerson J. Persistent hematoma after mouth trauma . Hosp Pract (Off Ed) . 1981;16:28-29. 15. Sonis AL, Musselman RJ. Oral bleeding in classic hemophilia . Oral Surg . 1982; 53:363-366.Crossref 16. Daffos F, Forestier F, Kaplan C, Cox W. Prenatal diagnosis and management of bleeding disorders with fetal blood sampling . Am J Obstet Gynecol . 1988:158: 939-946.Crossref 17. Baty BJ, Drayna D, Leonard C, White R. Prenatal diagnosis of factor VIII deficiency to help with the management of pregnancy and delivery . Lancet . 1986; 1:207.Crossref

Journal

Archives of Pediatrics & Adolescent MedicineAmerican Medical Association

Published: Jun 1, 1994

There are no references for this article.