[Some clinical and immunological characteristics of pneumonia in children with lymphatic hypoplastic diathesis].
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Acute Disease↗
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[Some hemocoagulative effects of antifibrinolytic therapy in the minor hemorrhagic diathesis of hepatic cirrhosis].
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Antifibrinolytic Agents↗
[Immunologic reactivity of newborn infants with manifestations of allergic diathesis].
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Disease Susceptibility↗
[New possibilities in the management of hemorrhagic diathesis caused by factor deficiency and thrombocytopenia: recombinant active factor VII concentrate].
Recombinant Factor VIIa, a new therapeutic tool to treat severe bleeding caused by inhibitory haemophilia cases, some cases of thrombocytopenia and thrombocytopathy (e.g. severe type III von Willebrand disease) receives growing attention in clinical practice. Exogeneous FVIIa-in a supraphysiological concentration (clearly over 6 U/ml) seems to be able to generate quickly and safely (without thrombotic side effects) thrombin--the final enzyme of clotting--in physiological, or pathological conditions. A concise review about the possible mechanisms of action, indications, monitoring and clinical experience gained sofar with FVIIa is given in this report.
Factor VII↗
Genetic deficiency of factor VII and hemorrhagic diathesis. A case report and literature review.
FVII deficiency is a rather rare inherited hemocoagulation disorder that predisposes to hemorrhagic events, especially from mucous membranes, that are not predictable and severe as in hemophilia A. This defect produces prolonged prothrombin time (PT), reduced activity of FVII and normal activated partial thromboplastin time (aPTT). We report the case of a 43-year-old obese woman with severe deficiency of factor VII (FVII), probably genetic in nature, and meno-metrorrhagia associated with multiple fibromas of uterus. Our patient had no history of bleeding in infancy and young age, and in the past, before the disease was diagnosed, underwent major surgery operations (thyroidectomy and caesarian section) without hemorrhage. Patient's relatives with mild heterozygous deficiency of FVII (the father, a brother, a sister, a sister's daughter and the patient's son) did not show any bleeding tendency. This case report is discussed in the light of literature data ((source: Medline from 1964 to 1996). The different forms of congenital (isolated or combined with other clotting disorders) and acquired FVII deficiency, with the appropriate therapies, are reviewed. The clinician must consider FVII deficiency in cases of recurrent bleeding, and this disease, even if rather rare, should not be underestimated in clinical practice because it is potentially fatal.
Adult↗
An epidemiological study of hand eczema. II. Prevalence of atopic diathesis in hairdressers, compared with a control group of teachers.
The aim of this cross-sectional study was to estimate the prevalence of atopic dermatitis in hairdressers compared with a control group of elementary school teachers. Both groups consisted of employees who reported exanthema on the hands and/or forearms at the time of completing a questionnaire. The mean age for the affected hairdressers was 26 years (range: 17-63) and for the teachers 44 years (range: 28-65). The mean duration of employment was 85 months for the hairdressers (range: 2-552) and 177 months for the teachers (range: 24-360). The frequency of atopic dermatitis was 12.7% (C.I.: 6.3-19.2) in the group of hairdressers and 25% (C.I.: 7.7-42.3) in the group of teachers. No difference was observed between the groups regarding atopic mucosal symptoms or familial atopy.
Adolescent↗
[Indices of cytochemical studies in children with various manifestations of allergic diathesis].
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Alkaline Phosphatase↗
A process model for the development of schizophrenia.
Less than two decades ago scientists interested in the etiology of schizophrenia were still vigorously debating the nature-nurture controversy, with many adhering to the view that the illness grew either out of a constitutional deficit or a pathological family environment. Today, virtually all researchers adopt interactional models that include both constitutional and environmental factors. Interactional models are most often expressed in diathesis-stress terms: Development of schizophrenia requires both a biological vulnerability (diathesis) and stressful life circumstances that facilitate expression of the illness. Modern debate within the field now primarily focuses on understanding the characteristics of the biological diathesis, the stressful environment, and their interaction. Scientists using diathesis-stress theories have employed several research strategies (Rosenthal 1970): (1) consanguinity studies exploring the distribution of illness among the relatives of schizophrenics, (2) twin studies comparing the concordance of schizophrenic illness in monozygotic and dizygotic twin pairs reared together or apart, (3) retrospective studies examining the premorbid behavior and development of schizophrenic parents, and (4) prospective studies of children at high risk for schizophrenia. The population most frequently followed in prospective high-risk studies has been offspring of schizophrenics. Biological offspring of schizophrenics who are reared by their parents are at extremely high risk for becoming schizophrenic themselves, approximately ten times greater risk than the general population. The present paper will make use of the Israeli High-Risk Study to test a diathesis-stress model for the transmission of schizophrenia. The goodness of fit of this model to the data will be explored using a simple decision tree data analytic approach.
Child↗
Invasive squamous-cell carcinoma in ThinPrep specimens: diagnostic clues in the cellular pattern.
Despite the increasing utilization of the ThinPrep Pap Test (TP), limited data exist regarding the cytomorphologic features and patterns of invasive squamous-cell carcinoma in TP specimens. We analyzed a series of TP specimens from patients with histologically confirmed invasive squamous carcinomas of the cervix. Patients with biopsy-proven invasive squamous-cell carcinoma of the cervix with a TP cervical cytologic specimen within the previous 2 mo were identified. The TP slides were analyzed for overall cellularity (percent circle coverage by epithelial cells), tumor cellularity, tumor diathesis, inflammation, coexistent dysplasia, and keratinization. Tumor cellularity was defined as <5%, 5-50%, and >50% of slide cellularity. In all 13 cases that were identified, a cytologic diagnosis of either invasive squamous-cell carcinoma or suspicious for invasive squamous-cell carcinoma was made. In 7/13 cases (54%), epithelial cells covered <40% of the slide circle. Epithelial cells covered >40% of the slide circle in 6/13 cases (46%). Tumor cellularity covered <5% of the slide circle in 4/13 cases (31%), 5-50% in 7/13 cases (54%), and >50% in 2/13 cases (15%). A tumor diathesis was present in 12/13 cases (92%). Inflammation was absent in 1/13 cases (8%), mild in 8/13 cases (62%), moderate in 2/13 cases (15%), and severe in 1/13 cases (8%). Coexistent dysplasia was identified in 12/13 cases (92%). Keratinization was identified in 9/13 specimens (69%). In the vast majority of patients, a diagnosis of squamous-cell carcinoma was rendered on the TP cervical specimen, despite a pattern of decreased cell coverage. It could be hypothesized that tumor diathesis and inflammation may be the etiology for decreased cellularity by blocking filter coverage by epithelial cells. This cellular pattern with diathesis in the ThinPrep smear may be a useful clue to look carefully for diagnostic cells of squamous-cell carcinoma.
Adult↗
Background features of endometrial carcinoma on ThinPrep cytology.
Conventional smear screening has had little impact on the detection of endometrial carcinoma. Recent studies on liquid-based preparations show promise with increased detection rates of endometrial lesions. The purpose of this 2-yr retrospective study (February 1, 2002-February 29, 2004) was to evaluate the background features on ThinPrep slide preparations from endometrial carcinomas. During this period, 20 (0.06%) cases were diagnosed as adenocarcinoma, of which 15 (75%) showed endometrial carcinoma on cervical or vaginal biopsy. Classic tumor diathesis was present on the slides in 8 (53%) of the cases, the majority of which were from high-grade tumors. In 1 (7%) case, the tumor diathesis consisted of precipitated protein in a watery background. Tumor diathesis was absent in 6 (40%) of the cases, the majority of which were low-grade lesions. Background features on ThinPrep Pap tests from patients with endometrial features range from classic tumor diathesis to clean; findings dependent on the histologic type and grade of the tumor and the extent of the disease process.
Adenocarcinoma↗
Percutaneous embolization to control intractable epistaxis.
Recurrent epistaxis is a common manifestation of patients with a bleeding diathesis. Two patients with epistaxis secondary to a bleeding diathesis managed by local conservative techniques are reviewed. (A case of polycythemia vera and a case of liver failure secondary to hepatoma are reviewed.) Recently bilateral, percutaneous carotid angiography examination was performed on a patient with a bleeding diathesis and intractable epistaxis. At the time of the angiographic examination, embolization of both internal maxillary arteries with Gelfoam particles was accomplished and dramatic control of the epistaxis was achieved. In a patient with severe epistaxis secondary to a bleeding diathesis that is unresponsive to local measures, percutaneous Gelfoam embolization offers substantial advantages over surgical intervention.
Adult↗
Gout in Maoris.
Historical evidence suggests that the Maori people of New Zealand were virtually untroubled by gout or obesity at a time when these disorders, along with other elements of the gouty diathesis, were rife in the best fed and hardest drinking sections of the Northern European population. By the mid 20th century, however, the apparent decline of the gout in Europe and North America and the breakup of the gouty diathesis in those lands had been more than compensated by their large-scale reappearance in the Maori and in other indigenous inhabitants of the Pacific Basin who, at first sight, appeared to have become one large gouty family. Half the Polynesian population of New Zealand, Rarotonga, Puka Puka, and the Tokelau Islands proved to be hyperuricemic by accepted European and North American standards, the associated gout rate reaching 10.2% in Maori males aged 20 and over. The trends towards hyperuricemia and gout, on the one hand, and towards obesity, diabetes mellitus, hypertension, and associated degenerative vascular disorders, on the other hand, which manifest themselves separately in some Polynesian Pacific Islanders, run together in the Maori and Samoan people, presenting a combined problem of considerable importance to the public health. The appearance of these traits under conditions of plenty in the descendants of hardy and wide-ranging Polynesian voyagers, suggests the emergence of a formerly favorable ancestral polygenic variation through selection for survival under harder conditions. This may now have lost its primitive survival value with a paradoxic shift towards increased prevalence of obesity and the gouty diathesis in more affluent environmental conditions. This may now constitute a genetic load, with recent environmentally determined increase in morbidity and mortality rates from degenerative vascular disorders. There is no satisfactory evidence that overproduction of uric acid differs in mechanism from its European counterparts, although more work remains to be done to determine whether there is any difficulty in renal handling of an increased uric acid load. A high Maori morbidity rate from gout and morbidity and mortality rates from associated components of the gouty diathesis in the face of readily available skilled medical advice and care, indicate the need for greater future attention to help education and health care delivery, at least while conditions of plenty continue. Continuation of previous epidemiologic surveillance may then be required in order to provide a continuing index of the effectiveness of these measures, as well as an opportunity for further research into the interrelationships of these associated disorders.
Adult↗
Factor XIII: inherited and acquired deficiency.
Factor XIII (XIII), an enzyme found in plasma (present as a pro-enzyme), platelets and monocytes, is essential for normal haemostasis. It may also have a role to play in the processes of wound healing and tissue repair. Inherited XIII deficiency results in a life-long, severe bleeding diathesis which, if untreated, carries a very high risk of death in early life from intracranial bleeding. XIII is a zymogen requiring thrombin and calcium for activation. In plasma, XIII has two subunits: the 'a' subunit, which is the active enzyme, and the 'b' subunit which is a carrier protein. Activated XIII modifies the structure of clot by covalently crosslinking fibrin through an epsilon (gamma-glutamyl)lysine link. It also crosslinks other proteins, including fibronectin and alpha-2-plasmin inhibitor (alpha-2PI), into the clot through the same link. Clot modified by XIII is physically stronger, relatively more resistant to fibrinolysis and may be a more suitable medium for the ingrowth of fibroblasts. Inheritance of factor XIII is autosomal recessive. The majority of patients with the inherited defect show no XIII activity and absence of 'a' subunit protein in plasma, platelets and monocytes. At the molecular level, the defect is not a major gene rearrangement or deletion, but most likely a single point mutation which may be different in each family. Because of the severity of the bleeding diathesis, prophylaxis is desirable and has been shown to be very effective as the in vivo half-life of plasma XIII is long, and low plasma levels are sufficient for haemostasis. Acquired inhibitors have been reported in only two cases with inherited XIII deficiency. Acquired XIII deficiency has been described in a variety of diseases and bleeding has been controlled by therapy with large doses of XIII in such conditions as Henoch-Schönlein purpura, various forms of colitis, erosive gastritis and some forms of leukaemia. Large dose XIII therapy has also been used in an endeavour to promote wound healing after surgery and bone union in non-healing fractures. The use of XIII in these conditions remains controversial. Very rarely a bleeding diathesis results from the development of a specific inhibitor to XIII arising de novo, often as a complication in the course of a disease or in association with long-term drug therapy. The bleeding diathesis in these patients is difficult to treat.
Amino Acid Sequence↗
Aggressivity, suicide attempts, and depression: relationship to cerebrospinal fluid monoamine metabolite levels.
BACKGROUND: We have proposed a stress-diathesis model for suicidal behavior, in which major depression is a stressor and the diathesis is shared with aggression. Neurotransmitter correlates of the stress or diathesis have not been adequately evaluated by previous studies, because they did not simultaneously examine the relationship of multiple neurotransmitters to all three psychopathologies in the same population. In the present study we investigated the relationship of monoamine metabolites to aggressivity, suicidal behavior, and depression in patients with mood disorders. METHODS: Ninety-three drug-free subjects with a major depressive episode underwent lumbar puncture and psychiatric evaluation. Cerebrospinal fluid CSF levels of 5hydroxyindolacetic acid (5-HIAA), homovanillic acid (HVA) and methoxy-hydroxy-phenylglycol (MHPG) were assayed. The relationships between monoamine metabolites and clinical variables were statistically evaluated. RESULTS: Higher lifetime aggressivity correlated significantly with lower CSF 5-HIAA. Lower CSF 5-HIAA and greater suicidal intent were found in high-lethality suicide attempters compared with low-lethality suicide attempters. Low-lethality attempters did not differ biologically from nonattempters. No correlation between CSF HVA and any of the psychopathological variables was found. Only aggression showed a trend statistically in correlating positively with CSF MHPG levels. CONCLUSIONS: Lower CSF 5-HIAA concentration was independently associated with severity of lifetime aggressivity and a history of a higher lethality suicide attempt and may be part of the diathesis for these behaviors. The dopamine and norepinephrine systems do not appear to be as significantly involved in suicidal acts, aggression, or depression. The biological correlates of suicide intent warrant further study.
Adult↗
Safety and efficacy of holmium: YAG laser lithotripsy in patients with bleeding diatheses.
PURPOSE: We assessed the safety and efficacy of ureteroscopy and holmium:YAG laser lithotripsy for treating upper urinary tract calculi in patients with known and uncorrected bleeding diathesis. MATERIALS AND METHODS: We retrospectively reviewed the charts at 2 tertiary stone centers to identify patients with known bleeding diathesis who were treated with holmium:YAG laser lithotripsy for upper urinary tract calculi. A total of 25 patients (29 upper urinary tract calculi) underwent ureteroscopic holmium laser lithotripsy. Bleeding diathesis involved warfarin administration for various conditions in 17 patients, liver dysfunction in 3, thrombocytopenia in 4 and von Willebrand's disease in 1. The mean international normalized ratio, platelet count and bleeding time were 2.3, 50 x 109/l. and greater than 16 minutes in patients on warfarin and in those with liver dysfunction, thrombocytopenia and von Willebrand's disease, respectively. RESULTS: Overall the stone-free rate was 96% (27 of 28 cases) and 29 of 30 procedures were completed successfully without significant complication. In a patient treated concomitantly with electrohydraulic lithotripsy significant retroperitoneal hemorrhage required blood transfusion. CONCLUSIONS: Upper tract urinary calculi in patients with uncorrected bleeding diathesis can be safely managed by contemporary small caliber ureteroscopes and the holmium laser as the only modality of lithotripsy. Ureteroscopic holmium laser lithotripsy without preoperatively correcting hemostatic parameters limits the risk of thromboembolic complications and costs associated with an extended hospital stay. Avoiding electrohydraulic lithotripsy is crucial for decreasing bleeding complications in this cohort of patients.
Adult↗
Drug-induced changes in selenium-dependent glutathione peroxidase activity in the chick.
The ability of aurothioglucose and D(-)-penicillamine hydrochloride to inhibit selenium-dependent glutathione peroxidase (SeGSH-Px) in vitro and to increase exudative diathesis in vitamin E-deficient chickens was studied. Aurothioglucose and penicillamine competitively inhibited SeGSH-Px in inverse proportion to the concentration of hydrogen peroxide and reduced glutathione, respectively, in chick liver postmitochondrial supernatant assay preparations. Neither drug inhibited glutathione reductase or superoxide dismutase at the concentrations tested; however, both inhibited catalase in a semilogarithmic fashion. This was true for both the purified bovine enzyme and chick liver homogenate. Aurothioglucose and penicillamine injected subcutaneously at the back of the neck increased exudative diathesis in vitamin E-deficient chickens fed 0.1 ppm Se, and effectively overcame the protective effect of selenium 72 h after injection in chicks fed vitamin E-free, low selenium diets supplemented with 0.0-0.1 ppm Se. Assays of plasma and of liver, lung and kidney postmitochondrial supernatants indicated that all observed reductions in SeGSH-Px activity preceded increases in exudative diathesis. Plasma and liver SeGSH-Px activities were lower at early times (6-24 h) after treatment with high doses of either drug. Lung SeGSH-Px activities were only lower in chicks receiving 240 mg penicillamine/kg 6 h after treatment; kidney SeGSH-Px activities were only lower in chicks treated with the highest dose of aurothioglucose 48 h after treatment. Brain SeGSH-Px activities were unaffected by drug treatment and the heart had higher SeGSH-Px activities only at 6 h after treatment with the highest dose of either drug compared to saline controls. Catalase activities in liver homogenates were only significantly altered by penicillamine; the highest dose caused the activity to be higher than that in saline-treated chicks. The cause of the lower SeGSH-Px activities could be either lower enzyme concentrations in tissues of the drug-treated groups and/or direct inhibition. Whatever the mechanism, it is concluded that exudative diathesis can be used to determine which drugs reduce SeGSH-Px activity in the chick.
Administration, Oral↗
Uremic bleeding: pathogenesis and therapy.
BACKGROUND: We reviewed current understanding of the pathophysiology of the uremic bleeding diathesis and discuss accepted therapeutic interventions that minimize the risk of bleeding in the uremic patient. METHODS: Computerized literature searches and references from previous publications, including articles describing original research and reviews pertaining to the pathophysiology of and clinical approach to uremic bleeding. RESULTS: The most common hemorrhagic manifestations in uremia are prolonged bleeding from puncture sites; nasal, gastrointestinal and genitourinary bleeding; and subdural hematomas. The most useful clinical laboratory test to assess both bleeding risk and response to therapy is bleeding time. It correlates better with clinical bleeding complications than indices of azotemia (eg, blood urea nitrogen [BUN], creatinine) or in vitro platelet aggregation tests. A low hematocrit is also correlated with increased bleeding risk. Anemia plays an important role in the bleeding diathesis of uremia and its correction with red cell transfusions or human recombinant erythropoietin is critical. Anticoagulation during hemodialysis may transiently exacerbate the bleeding diathesis. Hemodialysis and peritoneal dialysis improve the hemostatic defect and renal transplantation totally corrects it. Cryoprecipitate has been largely replaced by desmopressin acetate, which acts promptly (in less than 1 hour) but has a short duration of action (hours) and exhibits tachyphylaxis. Conjugated estrogens are slower in the onset of action (about 6 hours) but their effect lasts for about 2 weeks. CONCLUSIONS: The pathophysiology of the bleeding diathesis of uremia is complex and incompletely understood but useful clinical tests and therapies have evolved empirically. Broadly available dialysis and the advent of erythropoietin are likely to reduce the magnitude of this problem.
Bleeding Time↗
Depression spectrum disease, I: The role of gene-environment interaction.
OBJECTIVE: This study used an adoption study design to separate genetic from environmental factors in the etiology of depression spectrum disease, a type of major depression characterized by families in which male relatives are alcoholic and females are depressed. The genetic etiology hypothesis of depression spectrum disease proposes that an alcoholic genetic diathesis predisposes to depression in females but alcoholism, not depression, in males. METHOD: The study examined 197 adult offspring (95 male and 102 female) of alcoholic biological parents and used logistic regression models to determine the contribution to major depression in male and female adoptees that could be explained by the genetic alcoholic diathesis combined with an environmental factor that was characterized by psychiatrically or behaviorally disturbed adoptive parents. RESULTS: Major depression in females was predicted by an alcoholic diathesis only when combined with the disturbed adoptive parent variable. The same regression model failed to predict depression in males. Other possible environmental confounding factors contributing to an increased chance of depression were found in females: fetal alcohol exposure, age at the time of adoption, and a family with an adopted sibling who had a psychiatric problem. These variables did not diminish the significance of the prediction of depression with the alcohol genetic diathesis and disturbed parent model. CONCLUSIONS: The results show that a genetic factor is present for which alcoholism is at least a marker, and which exerts its effect in women as a gene-environment interaction leading to major depression. This finding suggests that an important etiologic factor in depression spectrum disease is gene-environment interaction.
Adolescent↗