Desmopressin testing in haemophilia A patients and carriers: results of a multi centre survey.

نویسندگان

  • R Knöfler
  • J Koscielny
  • J T Tauer
  • B Huhn
  • A Gneuss
  • E Kuhlisch
  • A Hofmann
  • S Petros
  • V Aumann
  • D Franke
  • K Kentouche
  • G Syrbe
  • K Seeger
  • H Haberland
  • R Klamroth
چکیده

INTRODUCTION Desmopressin (DDAVP) testing (DT) in patients (pts) with haemophilia A (HA) and carriers (CHA) is up to now not standardized. This prompted us to evaluate results of DT carried out between 1996 and 2011 in centres of the Competence Network Haemorrhagic Diatheses East. PATIENTS AND METHOD An increase of the factor VIII activity (FVIII) above 50% or at least the two fold of initial values within 120 min after DDAVP was defined as complete response (CR). Data from 80 patients (31 children, 49 adults) of whom 64 suffered from HA (sub-HA: n=48; mild: n=14; moderate: n=2) and 16 patients CHA were evaluated. RESULTS In 34 patients DDAVP was given i.v. (dose range: 0.26-0.6 µg/kg body weight, mean: 0.33), in 31 intranasally (i.n. 300-600 µg) and in 15 s.c. (15-40 µg). The maximal FVIII increase was reached 60 min after DDAVP. For i.v. application the mean FVIII increase was 3.1-fold, for i.n. 2.1-fold and for s.c. 2.4-fold. A CR was detected in 71 patients, a non-response in 9. Mild side effects such as flush, headaches or nausea were observed in 11 patients (14%). CONCLUSION For desmopressin testing in patients with haemophilia A and carriers i.v. application at 0.3 µg/kg body weight and the determination of FVIII before and 60 min after desmopressin infusion is recommended.

برای دانلود متن کامل این مقاله و بیش از 32 میلیون مقاله دیگر ابتدا ثبت نام کنید

ثبت نام

اگر عضو سایت هستید لطفا وارد حساب کاربری خود شوید

منابع مشابه

Guideline for the treatment of haemophilia in South Africa.

This guideline has been prepared by the authors for and on behalf of the Medical and Scientific Advisory Council (MASAC) of the South African Haemophilia Foundation to facilitate the appropriate management of individuals with haemophilia in South Africa. Individuals with haemophilia and their physicians should be advised by a Comprehensive Haemophilia Treatment Centre. Strategies that help to p...

متن کامل

Do patients with haemophilia undergoing cardiac surgery have good surgical outcomes?

A best evidence topic in cardiac surgery was written according to a structured protocol. The question addressed was whether patients with haemophilia undergoing cardiac surgery have good surgical outcomes. Haemophilia A and haemophilia B are sex-linked recessive inherited diseases affecting males only, with females acting as carriers. The conditions result in various degrees of factor VIII or f...

متن کامل

Prenatal diagnosis for haemophilia: a nationwide survey among female carriers in the Netherlands.

Carriers of haemophilia face difficult choices regarding prenatal diagnosis, but little is known about the determinants that influence their decisions. The aim of this study was to assess the incidence of prenatal diagnosis and potential determinants affecting the choice for prenatal diagnosis. A nationwide survey was performed among all women who underwent carriership testing for haemophilia i...

متن کامل

Approach to Penicillin Allergy – A Survey

Individual approaches to the diagnosis and management of penicillin allergy are practiced    by clinicians.   This cross-sectional survey of physicians was aimed at exploring their ways of dealing with diagnosis and management of penicillin G allergy.   Of the 235 respondents, 63% believed patients’ self-reported history of penicillin allergy and avoided using penic...

متن کامل

Evaluation of inhibitor antibodies in haemophilia A population

Background: Inhibitory antibody to exogenous Factor VIII (FVIII) is a major complication of hemophilia treatment. This study was conducted to determine the prevalence of inhibitor antibody directed against FVIII.Methods: From May 2010 to May 2011, 52 patients with severe hemophilia A admitted in Amirkola Children’s Hospital were evaluated. Those who had abnormal mixing study, antibody against F...

متن کامل

ذخیره در منابع من


  با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید

برای دانلود متن کامل این مقاله و بیش از 32 میلیون مقاله دیگر ابتدا ثبت نام کنید

ثبت نام

اگر عضو سایت هستید لطفا وارد حساب کاربری خود شوید

عنوان ژورنال:
  • Hamostaseologie

دوره 32 4  شماره 

صفحات  -

تاریخ انتشار 2012