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At least 415 records · Page 23Linked to original sources
PubMed1982

Spontaneous intracranial bleeding in hemorrhagic diathesis.

Eight patients with spontaneous intracranial bleeding secondary to hemophilia A, afibrinogenemia, and thrombocytopenia are described. The clinical picture was that of increased intracranial pressure of gradual onset with variable signs and symptoms. Both the hemostatic defect and the intracranial bleeding were proved by laboratory investigations and CT scanning. Mortality was 75% (six of eight patients). The two remaining patients recovered completely following combined medical and surgical treatment, and are alive and well.

Adolescent↗
PubMed2003

Platelet-mediated microvascular inflammation and thrombosis in thrombocythemia vera: a distinct aspirin-responsive arterial thrombophilia, which transforms into a bleeding diathesis at increasing platelet counts.

Erythromelalgia is the main, pathognomonic and presenting symptom in patients with essential thrombocythemia and thrombocythemia associated with polycythemia vera. Complete relief from erythromelalgic and acrocyanotic pain is obtained with the cyclooxygenase inhibitors aspirin and indomethacin, but not with sodiumsalicylate, dipyridamol, sulfinpyrozone and ticlopedine. Thus, cyclooxygenase metabolites are necessary for erythromelalgia to develop. Local platelet consumption in erythromelalgic areas became evident by the demonstration of arteriolar fibromuscular intimal proliferation and occlusions by platelet-rich thrombi in skin biopsies, by the findings of shortened platelet survival times, significant higher levels of platelet activation markers beta-thromboglobulin, thrombomoduline and increased urinary thromboxane B2 excretion in thrombocythemia patients suffering from erythromelalgia. Aspirin treatment of erythromelalgia in thrombocythemia patients resulted in the disappearance of the erythromelalgic, thrombotic signs and symptoms, correction of the shortened platelet survival times, and a significant reduction of the increased levels of beta-TG, PF4, TM and urinary TxB2 excretion to normal. Erythromelalgia is frequently preceded or followed by atypical transient neurologic, ocular or coronary ischemic symptoms, which specifically respond to low-dose aspirin or reduction of platelet counts to normal. The broad spectrum of acropareshesias, erythromelalgia and acrocyanotic ischemia together with the episodic and transient atypical TIAs and ocular or coronary ischemic symptoms are caused by spontaneous activation and aggregation of hypersensitive platelets in the end-arterial microvasculature involving the peripheral, cerebral and coronary circulation of thrombocythemia patients. These microvascular circulation ischemic disturbances in thrombocythemia vera already occur at platelet counts in excess of 400 x 10(9) l(-1). Low-dose aspirin is highly effective and safe in the cure and prevention of thrombotic and ischemic events and does not elicit bleedings at platelet counts below 1000 x 10(9) l(-1). Spontaneous hemorrhages usually occur at very high platelet counts far in excess of 1000 x 10(9) l(-1) (HT) due to an acquired von Willebrand factor deficiency at increasing platelet counts. At platelet counts between 1000 and 2000 x 10(9) l(-1), thrombosis and bleeding (ETT and HT) frequently occur in sequence or paradoxically and low-dose aspirin does prevent thrombotic complications but aggravates or may elicit bleeding symptoms. Reduction of the platelet count to below 1000 x 10(9) l(-1) by platelet lowering agents usually results in the disappearance of the bleeding tendency and improvement of the von Willebrand syndrome, but the thrombotic tendency persists as long as platelet counts are above the upper limit of normal.

Aspirin↗
PubMed1992

A natural history of Dupuytren's contracture treated by surgical fasciectomy: the influence of diathesis (76 hands reviewed at more than 10 years).

Fifty-six patients (51 males and 5 females) were reviewed more than 10 years after surgical fasciectomy for Dupuytren's disease (average 12 years 7 months). 76 hands were operated upon (20 bilateral cases) for a total of 137 fingers (out of 152 affected digits). The average pre-operative score based on Tubiana's classification was of 4.28. To-date, at maximum follow-up time, it is of 2.45. 34 recurrences occurred before re-operation (i.e. 45%), whereas at maximum follow-up, 74% of hands are still defective, although only 22% cause functional impairment. The method of improvement ratio classifies patients into two categories: satisfactory results and failures. This original method of qualification emphasizes three negative factors: recurrences, initial severity of disease, and failure to achieve full finger extension post-operatively. Results as a whole are in accordance with those mentioned in literature. Improved results depend on early surgery for severe cases, which must therefore be diagnosed at an early stage. Surgery often sets back the disease, without curing it. Further efforts in fundamental research on the etiology of this disease will be needed.

Disease Susceptibility↗
PubMed2003

Pathogenesis and management of the bleeding diathesis in acute promyelocytic leukaemia.

Life-threatening bleeding, which remains a challenging complication of acute leukaemia, is particularly characteristic of the subtype, acute promyelocytic leukaemia (APL). The clinical picture and laboratory abnormalities are most compatible with the diagnosis of disseminated intravascular coagulation (DIC). Evidence for diffuse activation of the coagulation system, hyperfibrinolysis and systemic elaboration of non-specific protease activity can usually be demonstrated and occurs most commonly during induction chemotherapy. While both host- and tumour-associated mechanisms can be implicated in the pathogenesis of the coagulopathy, leukaemic cell properties appear to be the proximate cause of activation of the haemostatic mechanisms. In this chapter we summarize the current state of knowledge of the pathogenesis of the coagulopathy of APL and the therapeutic approaches that have proved most useful for the management of this complication. Special attention is devoted to the use of all-trans-retinoic acid (ATRA), which has revolutionized the treatment of APL and markedly ameliorated the APL-related coagulopathy.

Disseminated Intravascular Coagulation↗
PubMed2002

Acquired hemophilia a presenting as a bleeding diathesis in a postpartum patient: diagnosis and management.

Antibodies against Factor VIII (FVIII) can develop in non-hemophiliac patients, causing the rare condition of acquired hemophilia. In 7.3% of the patients, the FVIII inhibitors appear either during pregnancy or in the postpartum period. In this case report, we present a non-hemophiliac patient, who presented five months postpartum with intermittent heavy vaginal bleeding, easy bruising, hemarthrosis, and recurrent frank hematuria. The woman presented to the emergency room with hematuria. Coagulation screening tests showed a prolonged APTT. Using a standard diagnostic algorithm, a Factor VIII inhibitor was detected. The treatment, cause of disease, and prognosis of this woman is presented in this paper as well as a literature review of acquired hemophilia A associated with pregnancy.

Adult↗
PubMed2001

The Children's Attributional Style Interview: developmental tests of cognitive diathesis-stress theories of depression.

This paper presents an initial assessment of the Children's Attributional Style Interview (CASI), a newly designed measure for assessing attributional style in young children (age 5 and up). The CASI was used to conduct prospective tests of the reformulated helplessness and the integrated hopelessness/self-esteem theories of depression in a sample of 147 5-10-year-old children. For comparison, the same tests were also conducted with the Children's Attributional Style Questionnaire-Revised, a commonly used measure for assessing attributional style in older children (age 8 and up). The CASI evidenced support of the reformulated helplessness theory and partial support of the integrated hopelessness/self-esteem theory. The CASI also demonstrated good internal consistency. Thus, our findings provide initial support for the CASI as a methodologically sound measure of attributional style for children as young as 5 years old. Although preliminary, our findings also suggest possible developmental differences in how attributional style interacts with self-esteem and negative life stress. The CASI should prove to be a useful tool in furthering the understanding of the origins and development of attributional style in childhood, as well as its contribution to the understanding of the development and prevention of depressive symptomatology in children.

Child↗
PubMed2004

Shame-proneness as a diathesis for dissociation in women with histories of childhood sexual abuse.

This study examined whether shame-proneness is associated with dissociation among abused women. Participants were 99 hospitalized women with and without reported histories of childhood sexual abuse. Hypotheses were that childhood sexual abuse and shame-proneness would each be associated with dissociation, and that the relationship between sexual abuse and dissociation would be greater among women with higher shame-proneness. Multiple regression analysis indicated that shame-proneness was independently related to dissociation, but childhood sexual abuse was not. As predicted, the combination of shame-proneness and childhood sexual abuse was associated with dissociation.

Adult↗
PubMed1999

[Isolated increased aPTT with anamnestic hemorrhagic diathesis--severe FXI deficiency].

A 47-year-old patient with several episodes of bleeding after tonsillectomy, after an excision of a sacral dermoid and after urinary tract surgery presented with a mechanical ileus and was admitted to the department of visceral surgery. Preoperative analysis revealed a prolonged activated partial thromboplastin time (aPTT) of 93 seconds (normal range: 40-60 seconds), whereas the prothrombin time and thrombin time were normal. A mixture of 1 volume patient plasma and 1 volume normal plasma gave a normal aPTT, both before and after incubation of the plasma mixture at 37 degrees C for 1 hour. The patient's history and the prolonged aPTT as the only abnormal clotting test indicated deficiency of either factor VIII, factor IX, factor XI or von Willebrand factor with consecutively diminished factor VIII. Laboratory analysis revealed a factor XI of 4% indicating severe factor XI deficiency. Laparotomy was successfully performed without any hemorrhagic complications under fresh frozen plasma substitution.

Diagnosis, Differential↗
PubMed1999

[Life-long hemorrhagic diathesis in a young man with unclottable global coagulation tests--congenital afibrinogenemia].

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.

Adult↗