A single centre experience in circumcision of haemophilia patients: Izmir protocol

Yılmaz, Deniz; Akın, Mehmet; Ay, Yılmaz; Balkan, Can; Çelik, Ahmet; Ergün, Orkan; Kavaklı, Kaan

doi:10.1111/j.1365-2516.2010.02324.x

Cited by 27 publications

(29 citation statements)

References 12 publications

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“…We did not find any ratio in the literature for the RFD group. This ratio for haemophilic patients was reported as 8.9% by Zü lfikar et al [26], as 6% by Yılmaz et al [28] and as 20.3% by Sasmaz et al [29].…”

Section: Discussionmentioning

confidence: 64%

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Surgical interventions in childhood rare factor deficiencies

Salcioglu¹,

Tuğcu²,

Akçay³

et al. 2013

Blood Coagulation &Amp; Fibrinolysis

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Congenital rare factor deficiencies may present in infancy by life-threatening bleedings or may not show any symptoms until adulthood. It is reported more commonly in countries having consanguineous marriages. Data regarding surgical interventions of rare congenital factor deficiencies are based on case reports and records of guidelines. There are no well documented and separately prepared directories related to pre-surgical and prophylactic approaches of surgical interventions of these deficiencies. Our retrospective study consisted of 171 rare factor deficiencies that were followed up in our clinic, and of whom 61 had 88 surgical interventions between 1990 and 2012. Of these patients, 45 were having factor VII deficiency, and factor V, X, XI, XIII and fibrinogen deficiencies were present in five, four, three, two and two patients, respectively. In 23 patients, factor coagulant activities were under 5% (37.7%), in 15 it was between 5 and 30% (24.6%), and in 23 between 30 and 50% (37.7%). Twenty-eight were symptomatic and 33 were asymptomatic. Information of 51 (83.6%) male and 10 (16.4%) female patients with an age range of 5-25 years (13 ± 5.27), whose age at presentation ranged between 3 weeks and 18 years (7 ± 4.66), were retrieved from patient records and from the records contained in the data-processing environment introduced in 2005. The rate of familial consanguinity was 49.2%. Of the surgical interventions, 24 (27.3%) were major, 24 (27.3%) were minor and 40 (45.4%) were circumcision. We used fresh frozen plasma in 32, recombinant factor (rF)VIIa in 20, prothrombin complex concentrate in five and fibrinogen in three patients during surgical interventions. In 18 patients, antifibrinolytic agents were also used. In 27 patients, surgical interventions were applied without any replacement therapy. No additional doses were required after surgical prophylaxis doses. Thrombotic events were not observed. Antibody occurrence was not detected in these patients. In our study, we evaluated preparation for surgical procedures, factor replacement therapy before surgical intervention and postoperative follow-up in patients with rare coagulation factor deficiency.

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Section: Discussionmentioning

confidence: 64%

“…Many studies exist on circumcision of haemophilic patients. An effort to establish a circumcision protocol may be observed particularly in the studies from our country [26][27][28][29]. Excluding circumcision of patients with factor XI deficiency, only anecdotal data are available regarding circumcision of RFD patients.…”

Section: Discussionmentioning

confidence: 98%

Surgical interventions in childhood rare factor deficiencies

Salcioglu¹,

Tuğcu²,

Akçay³

et al. 2013

Blood Coagulation &Amp; Fibrinolysis

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“…Kavaklı K et al (14)(15)(16)(17)(18) from Turkey introduced "İzmir Protocol" to the world. In İzmir Protocol, circumcision is performed under general anesthesia in inhibitor negative patients, fibrin glue is used during the operation, factor support is given for two days with a target level of 50-100% and tranexamic acid is used (Table 2).…”

Section: Discussionmentioning

confidence: 99%

Kanama bozukluğu olan çocuklarda sünnet deneyimi

Özdemir¹,

Çelik²,

Bulut³

et al. 2011

tpa

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Tur kish Arc hi ves of Pe di at rics, pub lis hed by Ga le nos Pub lis hing Circumcision in children with bleeding diathesisAd dress for Cor res pon den ce: Tiraje Celkan MD, İstanbul University Cerrahpaşa Medical Faculty, Division of Pediatric Hematology-Oncology, İstanbul, Turkey E-mail: tirajecelkan@yahoo. Original Article Sum maryAim: Circumcision is one of the most commonly performed procedures in children, however there are few reports which describe circumcision in patients with bleeding disorders (BD). The aim of this study is to present our experience in circumcision of children with BD. Material and Method:We retrospectively searched the patient records of 18 children (3-14 years old) who were followed up at Cerrahpaşa Medical Faculty Pediatric Hematology Clinic for BD and who were circumcised at Cerrahpaşa Medical Faculty Pediatric Surgery Clinic between 2000 and 2010. Management strategies, complications and outcomes were reviewed. Results: Seven children had a BD related to the first phase of hemostasis; 3 children had Glanzman thrombasthenia, 2 had Bernard-Soulier syndrome and 2 had chronic immune thrombocytopenic purpura. Other 11 children were followed up for a BD related to the 2nd phase of hemostasis; 3 had von-Willebrand disease (vWD), 1 had factor V deficiency, 1 had haemophilia B and 6 children had haemophilia A. No patient with inherited platelet disorder and/or thrombocytopenia required platelet replacement. Circumcision was performed with fibrinolytics and fibrin glue in these patients and there was no bleeding. In children with haemophilia (without inhibitors) and vWD, 2-3 doses of factor replacement therapy (15-25/kg/d) which was started before surgery were given until after 24-48 hours of circumcision. Fibrinolytics and fibrin glue were also used in these patients and bleeding was not observed in any patient. In 2 hemophilia A patients with low responsing inhibitors, by-pass agents were used for 12 and 18 days respectively. Bleeding was controlled with sequential use of rFVIIa and aPCC. Conclusions: Children with a BD can be safely circumcised in a center with experience. In circumcision of children with BD, antifibrinolytics and local hemostatic agents are helpful in decreasing factor doses in haemophilics and offer the possibility to omit platelet transfusion in patients with platelet disorders. (Turk Arch Ped 2011; 46: 304-8)

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“…3 Few publications are available in the literature about circumcision in patient with hemophilia. 3,9,11,12,19,20 The are several techniques described to induce hemostasis during circumcision in a patient with hemophilia such as fibrin glue and fibrin glue combined with the infusion of the deficient factor. 11,21 Up to our knowledge, no study described the use of the harmonic scalpel in circumcision and this will be the first report.…”

Section: 11mentioning

confidence: 99%

Outcome of harmonic scalpel circumcision in children with hemophilia

2017

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Background: There are many techniques described to decrease the risk of bleeding after circumcision in patients with bleeding diathesis such as monopolar diathermy, bipolar scissors and fibrin glue. Up to our knowledge, no previous study described the use of the harmonic scalpel in patients with bleeding diathesis. Our aim was to evaluate postoperative outcome after harmonic scalpel circumcision in boys with hemophilia.Methods: This was a prospective observational study carried out in Sohag University Hospitals, Sohag, Egypt. The patients were recruited from Outpatient pediatric hematology clinic from January 2014 till January 2017. All procedures were done under general anesthesia. After strict sterilization, the dorsal aspect of the prepuce was incised using harmonic scalpel up to the coronal sulcus, then the whole prepuce was cut circumferentially using the harmonic scalpel. It is a sutureless technique.Results: 26 boys with hemophilia A and 5 with hemophilia B were included. Their median age was 5 and 8 years respectively. The most common indication for circumcision was religious (20/31), the intraoperative blood loss ranged between 0-5 ml, operative time ranged between 3-5 minutes, there was no intraoperative complication, one case of rebleeding managed conservatively by factor administration, two cases of wound infection responded to antibiotic therapy, hospital stay ranged between 1-3 days. It was a sutureless technique.Conclusions: Harmonic scalpel circumcision is a safe technique in children with hemophilia, it decreased the risk of rebleeding, decreased operative time and it is also a sutureless technique and may decrease the cost by reducing the amount of factor replacement.

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A single centre experience in circumcision of haemophilia patients: Izmir protocol

Cited by 27 publications

References 12 publications

Surgical interventions in childhood rare factor deficiencies

Surgical interventions in childhood rare factor deficiencies

Kanama bozukluğu olan çocuklarda sünnet deneyimi

Outcome of harmonic scalpel circumcision in children with hemophilia

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