Therapeutische Umschau. Revue thérapeutique
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Case Reports
[An infant with umbilical cord and intracranial hemorrhage--severe factor XIII deficiency].
Based on the description of a severe bleeding disorder in a young child a short overview on the genetics, the epidemiology, the pathophysiology, the clinical manifestations and the laboratory diagnosis of factor XIII deficiency is presented. The impressive clinical signs with bleeding starting in the neonatal period (prolonged bleeding from the umbilical cord), followed by severe, life-threatening episodes of intracranial hemorrhages should raise the clinical suspicion of FXIII deficiency. ⋯ The diagnosis of factor XIII deficiency is difficult but has important therapeutic consequences: patients with documented severe deficiency should be put on regular substitution with factor XIII concentrates. Appropriately timed periodic infusions of such factor XIII concentrates enable patients to live normal lives, free from catastrophic bleeding episodes.
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Congenital afibrinogenemia is a rare autosomal recessive hemostatic disorder leading to unclottable global coagulation tests. Furthermore, it is associated with abnormal platelet aggregation and with severe bleeding episodes if untreated. Surprisingly, thrombotic complications may be observed quite frequently in afibrinogenemic patients following replacement of fibrinogen. A case of congenital afibrinogenemia is described in a patient who suffered from severe bleeding episodes in the absence of replacement therapy but developed a deep vein thrombosis with multiple pulmonary emboli after fibrinogen replacement and surgical treatment of a hip fracture, despite conventional heparin prophylaxis.
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Opioids do not yield adequate analgesic effects in at least one-third of all patients suffering from chronic pain. Nonetheless, in contrast to former opinion there is no type of pain that is principally resistant to opioids, which means that the individual patient's response to opioid administration has to be investigated to determine adequate treatment. Opioids fail most frequently in cases of neuropathic, osseous or sympathetically maintained pain. ⋯ A differential indication of the large number of possible coanalgesics should be determined with the help of a detailed pain history focussing on the pain quality. Similar to the WHO analgesic ladder used in (tumor) pain therapy, co-analgesic therapy should only be supplemented by invasive therapeutic procedures after various combinations and alternative substances have proven fruitless. Experience shows that this is necessary only for a small number of patients.
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The diagnosis and therapy of pain is routine in common practice of almost all clinical fields. Whilst acute pain may be controlled fairly easily, the treatment of chronic pain patients may be frustrating if conventional approaches for analgesia are chosen. Only a specialized and multidisciplinary procedure is beneficial for advanced stages of pain chronification. Precisely, an adequate treatment program has to consider biological, psychological and social aspects of chronic pain.