Risk of Bleeding and Inhibitor Development After Circumcision of Previously Untreated or Minimally Treated Severe Hemophilia A Children

Elalfy, Mohsen Saleh; Elbarbary, Nancy Samir; Eldebeiky, Mohamed; el-Danasoury, Azza S

doi:10.3109/08880018.2012.704624

Cited by 16 publications

(16 citation statements)

References 32 publications

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“…This study showed that the rate of inhibitor development after a median of 16 exposure doses was not different from that in 36 matched control patients in the non‐circumcised group. Three patients in the circumcised group (12%), in contrast to four patients (11.1%) in the non‐circumcised group developed an inhibitor . The author concluded that there was no increased risk of inhibitor development for patients undergoing neonatal circumcision despite early exposure to factor concentrates.…”

Section: Discussionmentioning

confidence: 95%

“…Bleeding outcomes after circumcision in previously untreated or minimally treated children with severe haemophilia A was recently reported by Elalfy et al . . In a cohort of 25 patients with severe haemophilia A (ages 7–34 months), all of whom were treated with a single dose of factor VIII (50 U kg −1 , target factor levels 100%), no children had peri‐operative bleeding.…”

Section: Discussionmentioning

confidence: 99%

“…The incidence of bleeding complications from circumcision in males with haemophilia is variable depending on the severity of the disease, the age at which circumcision is performed, the method of circumcision, the factor replacement given, as well as adjunctive hemostatic agents used. While there is little uniformity in the literature given the above variables and lack of standard definition for bleeding, the incidence of bleeding with circumcision in patients with haemophilia has been reported between 0% and 56% [4][5][6][7][8][9][10][11][12].…”

Section: Discussionmentioning

confidence: 99%

See 2 more Smart Citations

Neonatal circumcision in severe haemophilia: a survey of paediatric haematologists at United States Hemophilia Treatment Centers

et al. 2014

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Neonatal circumcision in patients with severe haemophilia has not been well studied. We performed a survey of paediatric haematologists from Hemophilia Treatment Centers (HTC) across the United States to better understand the attitudes toward and management of neonatal circumcision in haemophilia patients. Response rate to our survey was 40% (n = 64/159). Thirty-eight percent of respondents (n = 24) said that they would allow this procedure in the newborn period but in many cases this was against medical advice. The most reported concern regarding neonatal circumcision in haemophilia patients was the risk of development of an inhibitor (n = 25; 39%) followed by the concern for bleeding (n = 22; 34%) and issues related to vascular access in the neonate (n = 11; 17%). All respondents recommended at least one preprocedure dose of factor replacement. Twenty-two percent (n = 14) of respondents did not use more than one dose of factor replacement but 32% (n = 21) used 1-2 postoperative doses. The remainder of paediatric haematologists surveyed recommended between 3-5 (16%; n = 10) and 6-10 (3%, n = 2) additional days postoperatively. There was wide variation in both techniques of circumcision as well as adjuvant haemostatic agents used. Only 22% of respondents said that they had an established protocol for management of circumcision in the newborn haemophilia patient. These survey results highlight the need for evidence-based guidelines regarding the optimal management of circumcision in neonates with severe haemophilia.

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Section: Discussionmentioning

confidence: 95%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

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Neonatal circumcision in severe haemophilia: a survey of paediatric haematologists at United States Hemophilia Treatment Centers

et al. 2014

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“…Circumcision is frequently performed in children with IBDs, with variable outcomes. 529,539 The rate of bleeding complications in haemophilia patients varies from low (0-6%) 512,[540][541][542] to high incidence 543 depending on the centre and protocol used. However, when the IBD is not diagnosed before intervention, the bleeding rate can be even higher.…”

Section: General Perioperative Management Recommendationsmentioning

confidence: 99%

Management of severe perioperative bleeding

Kozek‐Langenecker

Ahmed

Afshari

et al. 2017

European Journal of Anaesthesiology

694

316

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: The management of perioperative bleeding involves multiple assessments and strategies to ensure appropriate patient care. Initially, it is important to identify those patients with an increased risk of perioperative bleeding. Next, strategies should be employed to correct preoperative anaemia and to stabilise macrocirculation and microcirculation to optimise the patient's tolerance to bleeding. Finally, targeted interventions should be used to reduce intraoperative and postoperative bleeding, and so prevent subsequent morbidity and mortality. The objective of these updated guidelines is to provide healthcare professionals with an overview of the most recent evidence to help ensure improved clinical management of patients. For this update, electronic databases were searched without language restrictions from 2011 or 2012 (depending on the search) until 2015. These searches produced 18 334 articles. All articles were assessed and the existing 2013 guidelines were revised to take account of new evidence. This update includes revisions to existing recommendations with respect to the wording, or changes in the grade of recommendation, and also the addition of new recommendations. The final draft guideline was posted on the European Society of Anaesthesiology website for four weeks for review. All comments were collated and the guidelines were amended as appropriate. This publication reflects the output of this work.

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“…This study demonstrates that early circumcision before 36 months of age and with minimal CFCs surgical prophylaxis is both effective and safe regarding the risk of development of inhibitors although a longer follow‐up period to at least 20 exposure days would have given more concrete conclusion. It also indicates that this approach reduced the need for CFCs surgical prophylaxis to only two doses which is lower compared to other published studies …”

Section: Type Of Surgical Proceduresmentioning

confidence: 70%

Low‐dose surgical prophylaxis: Optimization of use of World Federation of Hemophilia Humanitarian Aid donated clotting factor concentrates to developing countries

Ekiaby¹,

Haffar

2020

Haemophilia

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Background Patients with hemophilia (PWH) might need surgical interventions during the course of their lives. Such medical interventions pose hemostatic challenges and requests infusion of clotting factor concentrates (CFCs) during peri and postoperative for variable periods to prevent bleeding and until complete wound healing. Access to CFCs to PWH living in resource limited settings is usually a challenge which makes surgical interventions either risky or not practical. Recently World Federation of Hemophilia (WFH) started a humanitarian aid program to channel CFCs into resource limited countries and which allowed the possibility to perform surgical interventions for PWH in these countries. Aim of work To study safety and efficacy of using lower doses of CFCs for surgical prophylaxis. Methods Review of literature and our center experience to demonstrate safety and efficacy of low dose surgical prophylaxis using CFCs Results Several elements can help using lower doses of CFCs for surgical prophylaxis in resource limited setting. These elements include severity of hemophilia, type of surgical procedure, the use of hemostatic surgical techniques, the type of CFCs, the mode of infusion of CFCs and finally the use of adjunctive therapies Conclusion Management of surgical procedures for PWH in a multidisciplinary specialized hemophilia treatment centers with proper understanding of hemostatic and surgical challenges of the procedure can allow for safe and effective use of lower doses of CFCs for surgical prophylaxis.

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Risk of Bleeding and Inhibitor Development After Circumcision of Previously Untreated or Minimally Treated Severe Hemophilia A Children

Abstract: Two doses factor concentrate and gelatin sponge application were generally enough to prevent bleeding after circumcision of severe hemophilia A. Circumcision and low-dose FVIII protocol were not an additional risk for development of high-titer inhibitor.

Cited by 16 publications

References 32 publications

Neonatal circumcision in severe haemophilia: a survey of paediatric haematologists at United States Hemophilia Treatment Centers

Neonatal circumcision in severe haemophilia: a survey of paediatric haematologists at United States Hemophilia Treatment Centers

Management of severe perioperative bleeding

Low‐dose surgical prophylaxis: Optimization of use of World Federation of Hemophilia Humanitarian Aid donated clotting factor concentrates to developing countries

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