Radiosynoviorthesis in children with haemophilia

Pasta, Gianluigi; Mancuso, Maria Elisa; Perfetto, O. S.; Solimeno, L. P.

doi:10.1055/s-0037-1621502

Cited by 11 publications

(3 citation statements)

References 14 publications

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“…Am häufigsten betroffen von rezidivierenden Einblutungen sind ▪ das Kniegelenk, ▪ das obere Sprunggelenk und ▪ das Ellenbogengelenk [13]. Der Nachweis der Synovialitis sollte aktuell sein.…”

Section: Besonderheiten In Der Diagnostik Und Indikationsstellung Zur...unclassified

Radiosynoviorthese bei Hämophilie

Boddenberg-Pätzold¹,

Sigl-Kraetzig²,

Kampen³

2022

Radiopraxis

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Die Radiosynoviorthese (RSO) ist fester Bestandteil der Therapie bei Patienten mit Hämophilie mit rezidivierenden Gelenkeinblutungen. 70–90% der Patienten erleben eine verminderte Blutungsfrequenz, nachlassende Schmerzen und verbesserte Beweglichkeit. Der Artikel beschreibt die Pathomechanismen der Erkrankung und die Besonderheiten bei der RSO der zumeist noch jungen Patienten, die prätherapeutische Diagnostik, die Durchführung der RSO und die Nachsorge.

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Section: Besonderheiten In Der Diagnostik Und Indikationsstellung Zur...unclassified

Radiosynoviorthese bei Hämophilie

Boddenberg-Pätzold¹,

Sigl-Kraetzig²,

Kampen³

2022

Radiopraxis

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“…In these symptomatic subjects, several treatment options are proposed, depending on the severity of joint alterations. Oral analgesics, physical maintenance, physiotherapy, use of braces, and intra-articular injections with several types of products (hyaluronic acid, rifampicin, radiocolloids) are the best treatment options now available in early or mild stages of arthropathy [3][4][5]. In cases of poor clinical effects or in the worst cases, surgery is needed: need for arthroscopy is indicated by the presence of persistent synovitis and soft tissue alterations with low grades of arthropathy, while joint arthroplasty remains the gold standard for end-stage haemophilic arthropathy [6,7].…”

Section: Introductionmentioning

confidence: 99%

Major Orthopaedic Surgery in Persons with Haemophilia A with and without Inhibitors Treated by Emicizumab: A Mid-Term, Large, and Successful Series at a Single Center

Carulli,

Daniele,

Linari

et al. 2024

JCM

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Introduction: Patients with Haemophilia (PWH) need orthopaedic treatments and often they undergo surgery. Classically, PWH with inhibitors have to face such procedures earlier than other patients. Major orthopaedic surgery is not easy and complications are frequent. Emicizumab is the first monoclonal antibody introduced for haematological prophylaxis for PWH with inhibitors, achieving an efficacious haemostasis also in patients with severe haemophilia A with inhibitors, later demonstrated for PWH without inhibitors. A few years ago, emicizumab was also proposed for PWH undergoing surgery, as it supports excellent bleeding control. The literature on orthopaedic surgery using an emicizumab protocol is scarce: only isolated case reports with short-term follow-ups are available. Aim: The purpose of this study is the assessment of the mid-term outcomes of major orthopaedic surgery performed in a population of patients with and without inhibitors and an emicizumab regimen. Methods: We reviewed the records of 13 PWH (eight with high-titre inhibitors, five without) with a mean age of 54.6 years, undergoing 15 orthopaedic surgical procedures between 2017 and 2022: primary knee and hip arthroplasty, revision, pseudotumor excision, or amputation. Their prophylaxis consisted of the combination of emicizumab and boluses of rFVIIa (PWH with inhibitors) or rFVIII (PWH without inhibitors). The clinical parameters of evaluation were: VAS, Haemophilic Joint Health Score (HJHS), and standard radiologic studies. Follow-up was conducted at 1, 3, 6 months, and then yearly. The survival rate of all implants was also assessed. Results: The mean follow-up was 38.8 months (range: 12–65). All patients were successfully treated without complications during surgery. During the postoperative period, a patient affected by a septic complication two months after his pseudotumor excision underwent an above-the-knee amputation. All patients were regularly discharged to the rehabilitative ward, reporting satisfaction for pain reduction and improved joint and global function at the VAS and HJHS scores. No revisions or implant failures were recorded. Conclusions: A prophylaxis regimen with emicizumab and factor replacement in PWH with or without inhibitors undergoing major orthopaedic surgery ensures effective bleeding control and good postoperative clinical outcomes at mid-term follow-up, and may be routinely adopted in dedicated high-volume hospitals. This series is the most consistent to date reported at a single Haemophilia centre.

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“…A proper therapeutic approach to acute hemorrhages is essential in the development and evolution of joint damage in these patients. 19 Multidisciplinary teams working with patients with hemophilia should include the use of scales such as VERITAS-PRN to initially establish the degree of adherence of on-demand treatment patients and, subsequently, address those aspects that can be improved in the prevention of future complications in case of more severe bleeding episodes.…”

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confidence: 99%

Validation of the Spanish Version of the VERITAS-PRN Scale to Assess Adherence to on Demand Regimens in Patients with Hemophilia

Torres-Ortuño

Cuesta‐Barriuso

Nieto-Munuera

et al. 2021

PPA

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Introduction:The episodic or on-demand administration of clotting factor concentrates in hemophilia patients in the event of hemorrhage is employed to restore hemostasis. Adherence to on-demand treatments needs to be assessed in order to improve patient management, avoiding adverse effects and serious clinical complications. Aim: To validate the Spanish version of the treatment adherence scale in patients with hemophilia, namely, Validated Hemophilia Regimen Treatment Adherence Scale-PRN (VERITAS-PRN). Methods: Eighty-five patients were recruited in three hemophilia patient associations in Spain. The VERITAS-PRN scale was adapted through a back-translation process from English to Spanish. A native Spanish bilingual translator translated the scale from English to Spanish, and subsequently another native English bilingual translator translated the scale from Spanish to English. The scale was applied twice (two months apart) to assess test-retest reliability. Results: Internal consistency reliability was slightly lower in the VERITAS-PRN in Spanish (0.80) versus the English version (0.85). There were no differences (p > 0.05) between the means of the dimensions or in the total scores between the sample of patients in Spain and the USA. The test-retest reliability coefficient of the scores on the total scale was 0.80 [CI,]. The test-retest reliability coefficient was greater than 0.90 in all subscales. Conclusion:The Spanish version of VERITAS-PRN has high consistency and empirical validity. This scale is useful for assessing the degree of adherence to treatment in adult patients with hemophilia following episodic infusion treatment.

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Radiosynoviorthesis in children with haemophilia

Cited by 11 publications

References 14 publications

Radiosynoviorthese bei Hämophilie

Radiosynoviorthese bei Hämophilie

Major Orthopaedic Surgery in Persons with Haemophilia A with and without Inhibitors Treated by Emicizumab: A Mid-Term, Large, and Successful Series at a Single Center

Validation of the Spanish Version of the VERITAS-PRN Scale to Assess Adherence to on Demand Regimens in Patients with Hemophilia

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