Environmental factors promoting the effective use of a computer-assisted clinical case for second-year osteopathic medical students

We report a 38-year-old female of Puerto Rican descent with Hermansky-Pudlak syndrome and decreased levels of von Willebrand factor. Histologic and ultrastructural findings of non-sunexposed skin showed melanocytes with short dendritic processes and decreased numbers of melanosomes. Ultrastructural examination of platelets revealed greatly reduced numbers of delta granules. Recognition of this syndrome is important because skin neoplasms, ceroid deposition and hemorrhagic manifestations can be causes of morbidity and of potential death in patients affected with this syndrome.     

Anesthetic management of a patient with Bernard-Soulier syndrome

Bernard-Soulier syndrome (BSS) is a hereditary hemorrhagic disease characterized by prolonged bleeding time due to abnormal platelet aggregation and giant platelets. Transfusion of platelet-rich plasma is the only treatment available for the hemorrhagic episodes in patients with this disease. A 20-year-old female with BBS was scheduled for sagittal osteotomy of the mandibular rami under general anesthesia. Anesthesia was induced with fentanyl and diazepam, and was maintained with nitrous oxide, fentanyl, and 0.5% enflurane. No exacerbation of the bleeding tendency was observed during or after the surgery. We consider that the use of halothane should be avoided in patients with BBS because it may inhibit the aggregation of platelets and prolong the bleeding time.     

An explanation for failure to predict cyclosporine area under the curve using a limited sampling strategy: a beginner''s second note

PURPOSE: To highlight the hemorrhagic character of metastatic hepatocellular carcinoma. PATIENT AND METHOD: A case report of a patient with mandibular metastatic hepatocellular carcinoma, who had severe postbiopsy hemorrhagic episodes, and literature review is presented. CONCLUSIONS: Mandibular metastatic hepatocellular carcinoma is a hemorrhagic tumor because of its hypervascular nature. Any rapidly enlarging swelling with ill-defined mandibular destruction suggestive of malignancy should be considered for metastatic hepatocellular carcinoma. Only a needle biopsy should be attempted in view of the hemorrhagic nature of the tumor. Palliative radiotherapy can be useful for the control of local expansile symptoms of the tumor and because of its possible role in the prevention of hemorrhage.     

Development and validation of a visual acuity chart for Australian Aborigines and Torres Strait Islanders

Portal hypertension syndrome is a common evolutive complication of several hepatic and extrahepatic diseases, being liver cirrhosis responsible for more than 80% of cases. When diagnosed it has prognostic value because of the high incidence of hemorrhagic, metabolic and infectious complications that these patients may develop. Clinical suspicion must be confirmed by objective complementary studies that provide information about the etiology and severity of the disease. In this review article we describe the contribution of ultrasonography in the evaluation of patients with portal hypertension as an objective diagnostic method and the usefulness of doppler ultrasound in the non-invasive hemodynamic assessment of the splanchnic and portal circulation.     

Anterior lenticonus and Alport's syndrome

A case of anterior lenticonus is reported in a 26 year old man with hemorrhagic nephritis related to familial Alport's syndrome. Anterior lenticonus is seen only as a part of Alport's syndrome. The authors describe its clinical, histologic and therapeutic aspects. They also discuss the other ocular manifestations, the nephritis and the sensorineural deafness.     

The clinico-epidemiological characteristics of Omsk hemorrhagic fever in 1988-1992

Materials on morbidity in Omsk hemorrhagic fever at the period of 1988-1992 in the districts of the Novosibirsk region are presented. Cases of the disease were registered mainly in September-October (83.3% of cases). Transmission of the disease by contact played the leading role in the epidemiology of Omsk hemorrhagic fever. The disease took the course of medium severity with faintly pronounced hemorrhagic syndrome.     

Delayed postpartum uterine dehiscence. A case report

BACKGROUND: Delayed postpartum uterine dehiscence with hemorrhagic shock may be caused by inadequate treatment of postpartum endomyometritis. CASE: A woman developed a delayed uterine dehiscence six weeks postpartum. Inadequate antibiotic treatment for postpartum endomyometritis was thought to be the etiology. The patient required a hysterectomy for definitive treatment because of associated hemorrhagic shock. CONCLUSION: Only one other case of uterine dehiscence that resulted from endomyometritis has been reported. Broad-spectrum antibiotic therapy is indicated in postpartum endomyometritis to avoid uterine dehiscence with hemorrhagic shock.     

The hemostatic system function of patients with severe forms of diphtheria

Hemostasis of 100 patients with severe diphtheria infection was studied throughout the disease. The patients were found to have marked procoagulant, anticoagulant and fibrinolytic disorders. Antithrombogenic activity of the vascular wall was also abnormal. The above impairments correlated with the symptoms severity and are interpreted as DIC syndrome which ran subclinically or as hemorrhagic syndrome. The majority of the patients underwent a hyperhypocoagulant phase of DIC syndrome.     

Removal of a torn Racz catheter from lumbar epidural space

A 45-year-old man with the acquired immune deficiency syndrome (AIDS) developed disseminated Mycobacterium tuberculosis infection and was started on isoniazid, rifampin, pyrazinamide and ethambutol. The treatment was interrupted because of side effects. On resumption of treatment be developed a rapidly progressive neurological illness characterized by left hemiparesis, right gaze preference, convulsions, coma, evidence of cerebral edema on computed tomography scan and death 9 days later. Autopsy showed the presence of miliary tuberculosis affecting the lungs, liver, spleen, lymph nodes and bone marrow. The brain showed evidence of acute hemorrhagic leukoencephalitis (AHL)-the first such case in a patient with AIDS. We speculate that treatment-induced lysis of mycobacteria with concomitant release of mycobacterial lipoproteins may have activated T-lymphocytes to cause AHL in this patient.     

Freshly frozen preserved plasma for the treatment of intravascular coagulation in polytraumatized patients

Coagulation disorders in hemorrhagic shock need not represent an isolated intravascular coagulation. They may also occur as a complex of local disseminated intravascular consumption, extravascular consumption, dilution, and reduced synthesis of coagulation factors. In the severely bleeding patient with hemorrhagic diathesis heparin is contraindicated because it does not normalize coagulability. Therefore, it fails to stop hemorrhage and shock remains untreatable. Fresh frozen plasma, however, has proved to be suitable as simultaneous substitution therapy of coagulopathy and of hypovolemic shock. 11 patients suffering from traumatic-hemorrhagic shock associated with intravascular coagulation and hemorrhagic diathesis were successfully treated with fresh frozen plasma, after conventional shock therapy had failed over a period of hours.     

The potentials of heparin monotherapy in patients with hemorrhagic vasculitis and kidney involvement

A retrospective analysis was done of case histories and outpatient case records of 25 patients with hemorrhagic vasculitis presenting with renal lesion, and 12 patients with hemorrhagic vasculitis without kidney damage. Heparin therapy was instituted as subcutaneous injections 4 times daily, 450-500 units/kg/24 h. A positive effect of heparin therapy correlated with the early start of the treatment and presence of the urinary syndrome. A possibility is shown of forecasting of the heparin therapy efficacy in the above patient populations in respect of a decline in the urinary excretion of products of the fibrinogen/fibrin cleavage more than two-fold a week after the start of treatment. If no such decline occurs the treatment is to be supplemented by prednizolon and curantil.     

Thrombolytic therapy using r-TPA in Budd-Chiari syndrome in the course of myeloproliferative diseases

Budd-Chiari syndrome is a rather unusual clinical entity; among others, myeloproliferative disorders not infrequently are reported as a cause of this syndrome. In the past prognosis of Budd-Chiari syndrome was usually very poor. However, in recent years treatment with fibrinolytic agents has proved to be often successful in Budd-Chiari syndrome, as well as in other thrombotic disorders. In particular, r-TPA has appeared to be effective, due to its thrombospecificity. Three cases of Budd-Chiari's syndrome associated with myeloproliferative disorders are described, in which r-TPA administration, together with treatment of underlying disease, resulted in a complete recanalization of sovrahepatic veins. r-TPA, due to its thrombospecificity, has been shown to be more effective than other thrombolytic agents; its use is associated with a lower number of hemorrhagic events and it may be repeated in the case of uncompleted response.     

Effects of 5-HT1A receptor agonists on patterns of rat motor activity in relation to effects on forebrain monoamine synthesis

The majority of cases of hemolytic-uremic syndrome and a smaller proportion of cases of thrombotic thrombocytopenic purpura have recently been shown to result from a toxin produced by enteric bacteria, referred to as verotoxin, or Shiga-like toxin. The predominant toxin-producing bacterial strain in North America is E. coli O157:H7, which causes hemorrhagic colitis in humans after ingestion of contaminated meat. The toxin is believed to gain entry to the circulation from the bowel wall; it then binds to specific glycolipid receptors abundant on renal vascular endothelial cells. The toxin inactivates ribosomes inside the cells, thereby killing them and producing the clinical manifestations of hemolytic-uremic syndrome. Recognition of the etiology of hemolytic-uremic syndrome may lead to better prospects for prevention and treatment.     

Circulating anticoagulant in the procainamide-induced lupus syndrome

An elderly man with procainamide hydrochloride-induced lupus syndrome had a circulating anticoagulant against factor XI and a biologic false-positive (BFP) test result for syphilis. This was not associated with hemorrhagic problems. The activity of the circulating anticoagulant and the BFP disappeared within days following discontinuation of procainamide and the administration of corticosteroids.     

Sex cord-stromal and steroid cell tumors of the ovary

Gonadal cell types that derive from the coelomic epithelium (sex cords) or mesenchymal cells of the embryonic gonads include granulosa cells, theca cells, fibroblasts, Leydig cells, and Sertoli cells. Ovarian tumors of these cell types are called sex cord-stromal tumors. This group of tumors represents approximately 8% of ovarian neoplasms and affects all age groups. The more common types are granulosa cell tumors (GCTs), fibrothecomas, and Sertoli-Leydig cell tumors. Sex cord-stromal tumors are of interest partly because of their hormonal effects, which are rare for other ovarian neoplasms. These effects include estrogenic effects (pseudoprecocious puberty, endometrial bleeding, endometrial hyperplasia and carcinoma) and virilization. The variety of gross appearances of these tumors, ranging from large multicystic masses to small solid masses, would appear to preclude a specific radiologic diagnosis. However, in many patients, both clinical and radiologic clues can suggest the diagnosis, including predominantly fibrous content at ultrasound or magnetic resonance imaging (fibrothecoma), large hemorrhagic multicystic mass in a child with pseudoprecocious puberty (juvenile GCT), and associated syndromes such as Peutz-Jeghers syndrome (sex cord tumor with annular tubules) or Ollier disease and Maffucci syndrome (juvenile GCT).     

Pathogenesis of dengue virus diseases: missing pieces in the jigsaw

The mechanisms involved in the pathogenesis of dengue hemorrhagic fever and dengue shock syndrome remain unresolved. Antibody-dependent enhancement of infection has long been thought to play a central role; however, this remains unverified. The alternative hypothesis that virus variation, virulence and dynamics may account for severe dengue disease, particularly in children, should be considered.     

Replication of Marburg virus in human endothelial cells. A possible mechanism for the development of viral hemorrhagic disease

Marburg and Ebola virus, members of the family Filoviridae, cause a severe hemorrhagic disease in humans and primates. The disease is characterized as a pantropic virus infection often resulting in a fulminating shock associated with hemorrhage, and death. All known histological and pathophysiological parameters of the disease are not sufficient to explain the devastating symptoms. Previous studies suggested a nonspecific destruction of the endothelium as a possible mechanism. Concerning the important regulatory functions of the endothelium (blood pressure, anti-thrombogenicity, homeostasis), we examined Marburg virus replication in primary cultures of human endothelial cells and organ cultures of human umbilical cord veins. We show here that Marburg virus replicates in endothelial cells almost as well as in monkey kidney cells commonly used for virus propagation. Our data support the concept that the destruction of endothelial cells resulting from Marburg virus replication is a possible mechanism responsible for the hemorrhagic disease and the shock syndrome typical of this infection.     

Therapeutic dilemmas: balancing the risks of bleeding, thrombosis, and leukemic transformation in myeloproliferative disorders (MPD)

With these uncertainties, which patients with ET and PV should receive myelosuppressive therapy and accept its possible risks? Remembering the generalization from the literature that most patients with ET who are to have a catastrophic thrombosis either have it at the time of diagnosis or after preceding, less severe thrombotic symptoms, it is acceptable to omit myelosuppressive therapy in asymptomatic patients with ET. Young patients with PV and no thrombotic manifestations can similarly be managed with phlebotomy alone. There is no evidence in the literature to show that myelosuppressive therapy should be used simply because the platelet count is high or to prevent transition to myeloid metaplasia. In patients with ET or PV with previous thrombotic manifestations, the risk/benefit ratio probably favors myelosuppressive therapy. The PVSG studies also suggest that patients over the age of 70 with PV are at particular risk for thrombosis and should be treated with myelosuppression. No myelosuppressive agent has been shown to be superior to hydroxyurea. Patients who fail on hydroxyurea should not be treated with 32P or alkylating agents. Anagrelide or interferon would be more appropriate. The PVSG-05 study suggests that high doses of aspirin, i.e. approximately 1.0 grams per day, are not indicated. Trials of low dose aspirin to prevent thrombosis are encouraged. There is no way to predict which patients will have hemorrhagic complications and, as mentioned, one study suggests that there will be few (44). Patients with thrombocytosis and pathological bleeding, i.e. hemorrhagic thrombocythemia, generally improve with myelosuppressive therapy. This syndrome is to be distinguished from patients who bleed with normal or low platelet counts, generally in the setting of myeloid metaplasia (14). These patients have an acquired disorder of platelet function due to platelet production from abnormal megakaryocytes. An occasional patient will benefit from increasing the platelet count by splenectomy.     

Retinoic acid receptor-gamma in human epidermis preferentially traps all-trans retinoic acid as its ligand rather than 9-cis retinoic acid

Brain injury in the premature infant is an extremely important problem, in part because of the large absolute number of infants affected yearly. The 2 principal brain lesions that underlie the neurological manifestations subsequently observed in premature infants are periventricular hemorrhagic infarction and periventricular leukomalacia. The emphases of this article are the neuropathological features, pathogenesis, and potential means of prevention of these 2 lesions. Recent work suggests that the ultimate goal, prevention of the lesions, is potentially achievable. Periventricular hemorrhagic infarction may be avoidable by prevention of germinal matrix-intraventricular hemorrhage, and periventricular leukomalacia by detection of impaired cerebrovascular autoregulation, prevention of impaired cerebral blood flow, and interruption of the cascade to oligodendroglial cell death by such agents as free radical scavengers.