Background: Fibrinogen is a protein playing pleiotropic role in human body. It is engaged in maintaining hemostasis. Congenital fibrinogen disorders comprise quantitative and qualitative fibrinogen anomalies. The symptoms range from bleeding, thrombosis to asymptomatic at all what is the most common case. Hypodysfibrinogenemia with lower level of fibrinogen of reduced activity, is the least common of all congenital fibrinogen disorders. Case report: A 31-year-old woman was reported at the 21 weeks of gestation, suffered from genital tract bleeding and there was a history of stillbirth. Clinical examination with no pathology, however laboratory tests revealed coagulation abnormalities due to prolonged thrombin test, decreased protein S and lower fibrinogen level (70 mg/dl). Autoimmune diseases were excluded and the diagnosis was widened with rotational thromboelastometry and genetic test for hypodysfibrinogenemia. The patient was treated with fibrinogen substitution and prophylactic dose of heparin throughout pregnancy and 2 weeks following labour. At 39 week of gestation Caesarean section was done, with no complications. Results: Genetic test revealed heterozygous mutation in fibrinogen gamma gene confirming hypodysfibrinogenemia. Due to bleeding manifestation in this patient of congenital fibrinogen disorders, fibrinogen substitution was implemented with heparin as a paranticoagulant prophylaxis, what turned out to be successful and enabled the patient to maintain the pregnancy. Conclusions: As hypodysfibrinogenemia symptoms are diverse the management is difficult and each patient’s therapy should be planned separately. Pregnancy may be the first time when congenital fibrinogen disorders reveal and it is especially challenging to prevent from obstetrical complications.
Currently in Poland there is no regulation aiming at framing the complexity of substitution process arising at pharmacy level. In practice such process exists in variety type of products, both generic and biological scope. Lack of integrity in terms of this process between authorised medical professions who held it, might lead to further costs both for the health care system and patients. The aim of the study is to explain, that the economic advantage, which very often is the result of performed substitution process, in some types of products such as bio-logical ones, should be preceded with the decision of the treating physi-cian. Only then, in the cooperation between persons prescribing and dispensing certain medicinal product the process of substitution may fully use its potential of money saving along with the control over a certain patient. The study brings the yet unknown classification of dif-ferences in costs between actually performed automatic substitution of products containing human insulin in the pharmacy setting in Poland in four year period in five Voivodeships.
The purpose of the study is to analyze the act of substitution between biological products. Diabetes mellitus notes the greatest increase in the projected causes of deaths globally till 2030. The proper drug substitution process may help to increase the control over the disease management. In this paper authors try to identify and explain the challenges for the health policies and legislations in emerging markets and low- and middle-income countries. Analysis of retrospective data covering prescribed-dispensed insulin products was performed. The study is based on the law and economics approach with the application of the planning theory and modeling. The study shows the scope of substitution process of insulin and highlights the necessity of univocal legal approach profiled for this therapy area. Prognosis of created base model indicates at constant presence of substitution process of insulin. Substitution is to bring financial savings for patients, it is yet however unknown whether savings arising from the act of purchase will still have positive impact on undertaken therapy process in long-term period. These findings may inform about important factors and how emerging markets and low- and middle-income countries can increase progress of the substitution process.
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