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At least 1,423 records · Page 79Linked to original sources
PubMed2006

Recurrent aphthous stomatitis as an expression of pathergy in atopics.

BACKGROUND: Recurrent Aphthous Stomatitis (RAS) may be part of the "atopic background". Recently it has been reported that aphtous stomatitis could be an expression of atopic diathesis. The aim of this study is to verify whether the prevalence of posttraumatic aphthae is higher in patients with RAS and atopic diathesis than in patients with RAS without atopy. MATERIAL AND METHODS: In the past three years 39 new patients between 14 and 56 years of age and affected by idiopathic RAS have been observed. Atopic status and history of posttraumatic aphthosis were evaluated in all patients. RESULTS: The results appear to show that in our population the prevalence of posttraumatic aphthae is higher among atopic patients. CONCLUSIONS: Not only RAS but also pathergy could be considered an expression of the rich clinical/ symptomatic constellation of atopic diathesis.

Adolescent↗
PubMed1984

[3 operated cases of congenital deficiency of Factor XIII associated with intracranial hematomas].

Three operated cases of congenital deficiency of factor XIII (fibrin-stabilizing factor) associated with intracranial hematomas were described and the diagnosis, replacement therapy of the factor were discussed. Congenital deficiency of factor XIII is quite rare coagulation disorder and only 100 patients were reported in the literatures in which we could find only one case who had craniotomy for associated intracranial hemorrhage. Case 1: A 41-year-old female with the history of unknown hemorrhagic diathesis complained of headache and right hemiparesis on August 2, 1980. CT scan showed left parietal intracerebral hematoma caused by unknown hemorrhagic diathesis and operated on under fresh blood transfusion. Postoperative state was uneventful but bleeding from the operated wound and rebleeding in the operated hematoma cavity were found on 5th postoperative day. The screening test for factor XIII was abnormal but replacement therapy with fresh plasma and factor XIII failed to control hemorrhagic diathesis. The patients died of GI bleeding and recurrent intracerebral hematoma on 21st postoperative day. Case 2: A 1.4-year-old boy with the history of umbilical bleeding on delivery and diagnosed as congenital deficiency of factor XIII in the other hospital fall down and struck his occiput on September 20, 1980. He vomited and became stuporous two days after injury, and was transferred to Ryukyu University Hospital. CT can revealed epidural hematoma at the left posterior fossa which extended to the supratentorium. The hematoma was successfully evacuated under infusion of fresh plasma and he showed uneventful recovery without rebleeding by postoperative appropriate replacement therapy.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗
PubMed1993

[Topical treatment of oral bleeding in children with clotting disturbances].

Owing to substitutive therapy an inhibition is possible of bleeding from oral mucosa as well as stomatological procedures in children with haemorrhagic diathesis. For elimination or limiting of substitutive therapy in 29 children with congenital haemorrhagic diathesis caused by plasma or platelet disorders, tissue glue was used. The preparation Tissucol, IMMUNO, was given during 37 stomatological procedures in which 44 tooth were extracted, and for post-traumatic bleeding from oral mucosa. Very good and good results were obtained in 95% of the performed stomatological procedures. That makes possible a statement that Tissucol is effective in maintaining of local haemostasis in children with haemorrhagic diathesis. This method is safer and less expensive than blood-derived preparations used for substitutive therapy, and is especially valuable in the treatment of children with haemophilia type A with antibodies against factor VIII.

Administration, Topical↗
PubMedDate not supplied

[Contribution of methods of exploration of the distal vascular bed to the diagnosis and general comprehension of Raynaud's phenomenon].

Current vascular investigations permit a better understanding and placing of Raynaud's Phenomenon from the nosological point of view. This work contains a study of the contributions made by digital rheography, arteriography and pulp biopsy. These methods allow us on the one hand to define the concept of Raynaud's diathesis, an essential element necessary to the appearance of vasomotor crises, and on the other hand to identify the possible triggering causes on which depend certain cases of Raynaud's phenomenon of idiopathic appearance, sometimes clinically latent. The general conception of Raynaud's Phenomenon, defined from the semeiological point of view and corresponding to a pathology and a diathesis common to the various conditions in which it may appear, is confirmed by the data provided by these investigations. The diagnostic approach in Raynaud's phenomenon has thus a double perspective: --to identify and define the Raynaud's diathesis, --to seek the possible causes which may be clinically latent.

Biopsy↗
PubMed1997

Desmopressin--a haemostatic drug: state-of-the-art review.

Desmopressin is a well tolerated and convenient haemostatic drug that can be used in a number of clinical conditions with bleeding diathesis. It has several effects on the haemostatic system, causing endogenous release of coagulation factor VIII, von Willebrand factor, tissue plasminogen activator and also increasing platelet adhesiveness and shortening bleeding time. The effect is virtually immediate and lasts for several hours. Side-effects are few and mostly mild. Desmopressin is suitable for home use in selected patients with bleeding diathesis. The optimal dosage is 0.3 microgram kg-1 intravenously, but the drug may also be administered subcutaneously or intranasally. In addition to the established indications, haemophilia A, von Willebrand's disease and platelet dysfunction, the drug may also be used safely in other patients with unexpected bleeding during or after surgery. The effectiveness of general prophylactic use of desmopressin, in order to diminish surgical blood loss and transfusion requirements, has not been proven, except in patients with known bleeding diathesis caused by congenital or acquired haemostatic disorders, e.g. in those taking aspirin.

Deamino Arginine Vasopressin↗
PubMed1997

[Thrombocyte dysfunction in children with albinism].

Seven children with albinism were examined for haemorrhagic diathesis. In 6 patients coagulation screening tests (prothrombin time, activated partial thromboplastin time, thrombin time) were normal, in 1 patient prolongation of activated partial thromboplastin time due to mild factor XI deficiency was found. Platelet counts were in the reference range, however, the template bleeding time of 6 out of 7 patients was prolonged. Evaluation of platelet aggregation and secretion in a lumi-aggregometer demonstrated that only the child with normal bleeding time had normal platelet function, and 6 children with prolonged bleeding time had impaired aggregation and release reaction. Five out of these 6 patients had severe decrease of ATP secretion even when high dose of thrombin was used as agonist. The number of dens granules in the platelets of these patients, as measured by the uptake of mepacrine, was significantly less than in normal controls. These findings clearly suggested storage pool deficiency. In one of these patients storage pool deficiency was associated with mild factor XI deficiency. The high frequency of haemorrhagic diathesis in albino children emphasizes, the importance of the screening of patients with albinism for bleeding diathesis.

Adolescent↗
PubMed1987

Acquired hemophilia. A natural history study of 16 patients with factor VIII inhibitors receiving little or no therapy.

Rarely, a patient develops an antibody against factor VIII coagulant activity. The resultant hemorrhagic diathesis is clinically distinct from inherited hemophilia, being characterized by few hemarthroses but frequent skin and other soft-tissue hematomas. Hematuria may be troublesome. These patients represent therapeutic challenges. This study is one institution's results with 16 such patients followed up over an average of 31 months (range, four to 120 months; median, 19 months). It describes the largest group from a single institution receiving essentially no immunosuppressive agents, yet has one of the better overall results. Two patients experienced fatal hemorrhage and five patients underwent spontaneous remission. Long-term survival is not incompatible with persistence of the inhibitor. We conclude that this hemorrhagic diathesis is clinically distinct, less fatal than usually perceived, and may undergo spontaneous remission. Clearly, there is no mandate for any particular therapeutic regimen, such as immunosuppression, in the attempt to rid the patient of the antibody.

Adult↗
PubMed1997

Acquired von Willebrand's disease: a concise review.

Acquired von Willebrand's disease (AvWD), an adult-onset bleeding diathesis, has most commonly been found in patients with an underlying lymphoproliferative disease or monoclonal gammopathy. Other malignancies, autoimmune diseases, hypothyroidism, and drugs have also been associated with AvWD. We have included an illustrative case history of a patient with a bleeding diathesis consistent with AvWD and a monoclonal gammopathy who required emergent cardiac surgery. Our review of the literature determined that most cases of AvWD are due to a circulating antibody that combines with the high molecular weight multimers (HMWM) of von Willebrand factor (vWF). These vWF multimer-antibody complexes are subsequently cleared from the circulation either by the reticuloendothelial system or by adsorption onto tumor cells. Clearance of the HMWM of vWF thus results in extremely low functional levels and variable antigenic levels. Mixing studies which are traditionally used to diagnose factor inhibitors are useful only if removal of vWF-antibody complexes can be accomplished in vitro. Treatment with intravenous immunoglobulin has recently been shown to be the most effective therapy for patients with an underlying lymphoproliferative disorder or monoclonal gammopathy. This therapeutic strategy is based on the observed immune complex clearance phenomenon that appears to be operative in most cases. Other AvWD-associated diseases require treatment specifically directed at the underlying disorder.

Antibodies↗
PubMed1998

Platelet von Willebrand factor in Hermansky-Pudlak syndrome.

The Hermansky-Pudlak Syndrome (HPS) is an autosomal recessive inherited disorder characterized by oculocutaneous albinism, tissue accumulation of ceroid pigment, and a mild to moderate bleeding diathesis attributed to storage-pool deficient (SPD) platlets. Patients have platelet aggregation and release abnormalities. In addition, low levels of plasma von Willebrand factor (vWF) antigen in some HPS patients have been associated with a greater bleeding tendency than would be predicted from either condition alone. Other HPS patients have severe bleeding despite normal levels of plasma vWF, suggesting that at least one additional factor is responsible for their bleeding diathesis. Because platelet vWF levels have been well correlated with clinical bleeding times in patients with von Willebrand's disease, we have measured the platelet vWF activity and antigen levels in 30 HPS patients and have attempted to correlate their clinical bleeding with these values. The platelet vWF activity levels in patients was significantly lower than that of normal subjects (P < 0.0001). The patients as a group also had slightly lower values of plasma vWF activity when compared with normals (P-0.03). In 11 of the HPS patients, the multimeric structure of plasma vWF showed a decrease in the high molecular weight multimers and an increase in the low molecular weight multimers. In correlating the platelet and plasma vWF values with the bleeding histories, we were not able to show a predictable relationship in the majority of the patients.

Adenosine Triphosphate↗
PubMed1999

Surviving cancer: the importance of sexual self-concept.

Sexual self-schemas are cognitive generalizations regarding sexual aspects of the self; they represent a core component of one's sexuality. We contend that individual differences in sexual self-view are an important cognitive diathesis for predicting sexual difficulty or dysfunction. We illustrate the role of sexual self-schemas in sexual behavior and responsiveness in healthy female and male samples. Next, we examine the diathetic properties of sexual self-schemas. Finally, we discuss an empirical test of the proposed diathesis-stress interaction, reviewing the role of women's sexual self-views on sexual morbidity following diagnosis and treatment for gynecologic cancer.

Adult↗
PubMed2000

Molecular genetic studies of bipolar disorder and puerperal psychosis at two polymorphisms in the estrogen receptor alpha gene (ESR 1).

A number of lines of evidence point to the possible involvement of estrogen pathways in the pathophysiology of bipolar disorder in general and puerperal psychosis in particular. There is strong evidence from clinical, follow-up, and genetic studies to support the hypothesis that most cases of puerperal psychosis are manifestations of an affective disorder diathesis with a puerperal trigger and that genes influence susceptibility to both diathesis and trigger. The nature of the trigger is unknown but in view of the abrupt onset at a time of major physiological change it is widely believed that biological, probably hormonal, mechanisms are of paramount importance, with estrogen receiving the most attention to date. We have undertaken a case control association study of bipolar disorder and puerperal psychosis at two known polymorphisms within the estrogen receptor alpha gene (ESR 1) in a sample of 219 unrelated bipolar probands and 219 controls. We could exclude these polymorphisms from an important contribution to susceptibility to bipolar disorder with a high level of confidence. We found no support for the hypothesis that they contribute specific susceptibility to the puerperal trigger, but due to the small numbers of puerperal probands (n = 26) no firm conclusions can be drawn regarding their involvement in puerperal psychosis. Am. J. Med. Genet. (Neuropsychiatr. Genet.) 96:850-853, 2000.

Adult↗
PubMed2007

Association of acquired von Willebrand syndrome with AL amyloidosis.

Acquired loss of functional von Willebrand factor (VWF) has been termed the acquired von Willebrand syndrome (AVWS). AVWS is a rare adult-onset bleeding diathesis that is clinically similar to congenital von Willebrand disease (VWD), and occurs with a variety of autoimmune, lymphoproliferative, or myeloproliferative disorders. We have identified four patients with AVWS in association with immunoglobulin light chain (AL) amyloidosis. These patients, lacking any pre-existing or family history of abnormal bleeding, developed cutaneous, mucosal, or gastrointestinal bleeding in the course of their disease without deficiency of clotting factor X or other factors; the activated partial thromboplastin time (aPTT) was prolonged in three out of the four cases. Despite normal VWF antigen levels, VWF ristocetin cofactor activity (VWF:RCo) was low. Electrophoresis patterns of high molecular weight (HMW) VWF multimers were abnormal in two of the four cases. Two of the patients were treated with high-dose intravenous melphalan followed by autologous stem cell transplantation (HDM/SCT) and achieved hematologic remission. In these two patients, the bleeding diathesis improved and the coagulation parameters normalized, confirming a causal relationship between the plasma cell dyscrasia and the AVWS. AVWS should be considered in AL amyloidosis patients with hemorrhagic diatheses and normal clotting factor levels.

Adult↗
PubMed1978

Hemorrhagic death associated with a high titer factor V inhibitor.

An acquired bleeding diathesis was first noted in a 51-year-old patient 11 days following an exploratory laparotomy. Laboratory studies indicated the cause of bleeding to be the development of a circulating anticoagulant which inhibited factor V activity. The inhibitor, an immunoglobulin of the IgG class, was separated by use of Sephadex G-200 filtration, disc electrophoresis, and isoelectric focusing. Despite vigorous immunosuppressive, antifibrinolytic, and replacement therapy, including the use of a "prothrombin complex" and plasmaphoresis, the bleeding diathesis could not be reversed and the patient died of hemorrhage. Although inhibitors to factor V are not usually associated with major life-threatening hemorrhage, this case demonstrates that the development of such an inhibitor can be an ominous finding. The use of an aminoglycoside antibiotic in this and other patients so reported may be one of the contributing causes to the development of such an inhibitor.

Antibodies↗
PubMed2005

Personality traits as intermediary phenotypes in suicidal behavior: genetic issues.

A genetic contribution to the risk of suicidal behavior is now supported by many studies. It probably involves specific factors acting on their own, independently of the genetic transmission of associated psychiatric disorders. A history of childhood maltreatment, adverse events, psychosocial stress, psychological traits and major psychiatric disorders all appear to contribute to the global risk of suicide attempt or completion. The interplay between previously identified risk factors, different as they are in nature and degree of complexity, still remains to be clarified. A stress-diathesis model has been proposed, where trait-like genetic and developmental risk factors (the diathesis) interact through still unknown mechanisms with actual (stress-related) factors to create the conditions for a suicidal gesture. Disentangling the effects of these risk factors, and specifically the effects of the genetic factors influencing these different pathological conditions, appears to be a difficult task. Indeed the results of candidate gene association studies suggest that genetic vulnerability factors for various related psychiatric phenotypes (major psychiatric disorders and personality traits) partly overlap with more specific factors predisposing to suicidal behavior. Personality traits are partly under genetic control and may be closer to the genetic effects than psychiatric syndromes. We review here the available data on the genetics of personality traits presumably involved in suicidal behavior, focusing on the association studies carried out with serotonin-related genes. We suggest that future studies on the genetic vulnerability to suicidal behavior should include the investigation of endophenotypes, with the aim of deciphering the mechanisms underlying the genetic susceptibility to these closely associated phenotypes.

Aggression↗
PubMed2005

Predicting psychological distress in college students: the role of rumination and stress.

Psychological distress among college students represents a serious health concern. The aim of this research was to investigate, for the first time, whether interactions between rumination and different measures of stress could differentially predict components of psychological distress, within a diathesis-stress framework. This self-report study employed a longitudinal design, spanning a period of 6 months. One hundred sixty-one undergraduate college students completed selected measures of psychological distress, rumination, and stress at two time points 6 months apart. Both independent and interaction effects were examined through hierarchical regression analyses. Rumination and stress were found to interact significantly to predict the social dysfunction components of psychological distress. Other main effects are reported. The evidence supported the proposed diathesis-stress model and extended previous research by relating rumination to different components of psychological distress prospectively.

Adult↗
PubMed1994

Family studies of non-insulin-dependent diabetes mellitus in South Indians.

Though a genetic basis for non-insulin-dependent diabetes mellitus (NIDDM) is clear, the likely mode of inheritance is not known. The segregation of NIDDM was studied in 64 nuclear South Indian pedigrees (449 individuals) ascertained through an affected proband having both parents and more than 1 sibling alive and available for oral glucose tolerance testing. A high proportion of parents were found to be of abnormal glucose tolerance [89 of 128 (70%) diabetic and 11 of 128 (9%) impaired]. Complex segregation analysis was performed using (1) POINTER which implements the mixed model and distinguishes major gene, multifactorial and non-transmitted environmental contributions to affection and (2) COMDS which implements an oligogenic model with major gene, modifier gene and environmental contributions to a) affection and b) diathesis (an ordered polychotomy amongst non-affected family members, based on 2-h plasma glucose level). Using POINTER, there was no formal support for a major gene and the most parsimonious solutions were achieved with multifactorial models. Using COMDS, we found i) significant improvements in models when information on glucose levels in nondiabetic family members (diathesis) was included, ii) support for segregation of a diallelic gene as well as background familial resemblance, and iii) under the best-supported model, this diallelic locus featured incomplete dominance (d = 0.8) and a disease-predisposing allele frequency of 14%. In South Indians, segregation of NIDDM is inadequately described by simple major gene models: more complex models provide more satisfactory descriptions. This finding, if applicable in other populations, has important implications for the search for diabetes-susceptibility genes.

Blood Glucose↗
PubMed1975

Pathoanatomical features of the kidney in myelomonocytic and chronic lymphocytic leukemia.

The kidneys of 18 autopsy cases of myelomonocytic leukemia (MML) were examined for MML-specific features. Nine cases of chronic lymphocytic leukemia (CLL) served as controls. The kidneys of the cases of MML showed macroscopically detectable signs of hemorrhagic diathesis and secondary uric acid diathesis more often than those of CLL. In the MML group most of the kidneys weighed more than the normal average for the corresponding age group, but the average renal weights for the 2 groups were about the same. Renal weight and grade of leukemic infiltration, particularly in MML, revealed no significant positive correlation. In most of the cases of MML there were unevenly distributed poorly defined leukemic, infiltrates in the renal cortex and medulla. The histology resembled that of pyelonephritis. In CLL, on the other hand, the leukemic infiltrates were usually sharply defined and localized in foci in the outer cortex and the corticomedullary border region. Renal dysfunction in cases of MML has been attributed by others to hyperlysozymemia. It was found occasionally but there was no MML-typical morphological substrate in our material. Hyaline droplet change of the tubular epithelium was more frequent and more pronounced in MML than in CLL. However, we also determined that it was nonspecific and that it was not a parameter of cell damage. Tubular hyaline droplet change and the morphological criteria of acute renal failure were not positively correlated with the degree of leukemic infiltration of the kidneys or with the leukemic proliferation as a whole. Instead, they were considered to be signs and symptoms of accompanying or secondary diseases which complicated the leukemia.

Acute Kidney Injury↗
PubMed1976

Management of bleeding disorders in traumatic-haemorrhagic shock states with deep frozen fresh plasma.

Coagulation disorders in traumatic-haemorrhagic shock need not represent a simple coagulation problem. They may also occur as a complex of local and disseminated intravascular consumption, dilution, extravascular loss and depressed synthesis of coagulation factors. In the severely bleeding patient with a haemorrhagic diathesis heparin is contrainedicated because it does not normalize coagulability immediately. Therefore, it fails to stop haemorrhage and the shock becomes untreatable. Fresh frozen plasma, however, has proved to be suitable as a simultaneous substitution therapy for the coagulation disorder and the hypovolaemic shock. 25 patients suffering from severe traumatic-hemorrhagic shock associated with coagulation disorders and haemorrhagic diathesis were successfully treated with fresh frozen plasma, after conventional shock therapy had failed over a period of 2 hours. The success was documented clinically and by numerous laboratory tests. Thrombocytopenia has only a secondary responsibility for the haemorrhagic state.

Blood Preservation↗