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  • Acquired antithrombin deficiency  (1)
  • Early vs. later onset of prophylaxis  (1)
  • 1
    Electronic Resource
    Electronic Resource
    Radiological and orthopedic score in pediatric hemophilic patients with early and late prophylaxis (1998)
    Springer
    Annals of hematology 77 (1998), S. 171-174 
    Details
    ISSN: 1432-0584
    Keywords: Key words Hemophilic arthropathy ; Early vs. later onset of prophylaxis ; Radiological and orthopedic score ; Target joints ; Joint bleeding
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract  In order to evaluate joint alteration, 17 patients with hemophilia A and B were investigated over a period of 4 years (1993–1997). Patients were subdivided into two groups, according to therapy regimens. In group 1 (n=10) prophylactic treatment was initiated until the third year of life. In group 2 (n=7) patients received prophylactic treatment at the age of 5 years and above. To assess alterations in knee, elbow, and ankle joints, the radiological score and the physical examination score of the Orthopedic Advisory Committee of the World Federation of Hemophilia were used. The sum of the scores of these six joints was defined as the patient-dependent score. Patients of group 1 (median age at the end of observation: 10 years) reached a median radiological score of 1.0 (range: 0–13) and an orthopedic score of 0 (range: 0–4), whereas patients of group 2 (median age: 14 years) had a radiological score of 20 (range: 2–47) and an orthopedic score of 8 (range: 0–12), which shows a significant difference (p〈0.01). In both treatment groups a manifestation or progression of arthropathic alteration was seen in those children who had repeated joint bleeding (〉5) prior to the onset of prophylactic treatment (r=0.90, p〉0.01). Altogether, two of 60 joints in group 1 and 12 of 42 joints in group 2 had a radiological score ≥4. Elbow joints were more often affected than knee and ankle joints. In conclusion, the number of joint bleedings before prophylactic treatment was started influenced the progression of arthropathy even in patients with early onset of prophylaxis. The aim of treatment in severe hemophilia should be early prophylaxis before repeated joint bleeding occurs in order to prevent osteoarthropathic alteration.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/s002770050436
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    Paper (German National Licenses)
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  • 2
    Electronic Resource
    Electronic Resource
    Treatment of consumption coagulopathy with antithrombin concentrate in children with acquired antithrombin deficiency – A feasibility pilot study (1999)
    Springer
    European journal of pediatrics 158 (1999), S. S187 
    Details
    ISSN: 1432-1076
    Keywords: Key words Antithrombin concentrate ; Children ; Septicaemia ; Acquired antithrombin deficiency
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Consumption coagulopathy is a serious problem in childhood. In addition to treatment of the underlying disease, consumption coagulopathy was previously treated with heparin. Nowadays it is treated by substitution of coagulation factors, especially antithrombin (AT) concentrate, alone or in combination with heparin. In this pilot study we administered AT concentrate (dosage 80 U/kgbw/d), without additional heparin treatment, to 29 children beyond infancy with acquired AT deficiency. Antithrombin, platelet count, fibrinogen, PT, and APTT were assayed before and during the course of AT substitution. These coagulation parameters returned to normal 48 hours after normalisation of the plasma AT level. AT levels normalised within 24 h of initial substitution in all children. Lethal outcome due to the underlying disease was observed in only two children. Conclusion Data of this pilot study suggest that, concomitantly with the treatment of the underlying disease, consumption coagulopathy in childhood can be managed successfully with early substitution of AT concentrate.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/PL00014353
    Library Location Call Number Volume/Issue/Year Availability
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    Paper (German National Licenses)
    Fulltext
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