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Biomedical subjects

E J Young

Publications and source records attributed to E J Young.

At least 19 recordsLinked to original sources

Laforin is a cell membrane and endoplasmic reticulum-associated protein tyrosine phosphatase.

Lafora disease (LD) is the only progressive myoclonus epilepsy with polyglucosan bodies. Among conditions with polyglucosan bodies, LD is unique for the subcellular location of its polyglucosans in neuronal perikarya and dendrites and not in axons. Here we report that the protein encoded by the EPM2A gene, which is mutated in LD, localizes at the plasma membrane and the endoplasmic reticulum and that it is a functional protein tyrosine phosphatase. The significance of these findings in the epilepsy of LD and in the origin and characteristic subcellular location of Lafora bodies is discussed.

Amyotrophic Lateral Sclerosis↗

Thrombocytopenic purpura associated with brucellosis: report of 2 cases and literature review.

Mild hematologic abnormalities are common in the course of human brucellosis; however, they generally resolve promptly with treatment of the disease. Occasionally, thrombocytopenia is severe and can be associated with bleeding into the skin (purpura) and from mucosal sites. We describe 2 patients infected with Brucella melitensis who presented with thrombocytopenic purpura, and we review 41 additional cases from the literature. Patients ranged in age from 2 to 77 years, and both sexes were affected equally. In the majority of cases, examination of the bone marrow revealed abundant megakaryocytes. Possible mechanisms involved in thrombocytopenia include hypersplenism, reactive hemophagocytosis, and immune destruction of platelets. Recognition of this complication is essential, since hemorrhage into the central nervous system is associated with a high mortality rate.

Adolescent↗

Disseminated herpesvirus infection during pregnancy.

A case of disseminated herpes simplex virus infection during pregnancy is reported, and 26 similar cases are reviewed. The present case was unusual in that it occurred in the 12th week of gestation, whereas all but one of the other cases occurred in the second or third trimester. This rare complication is generally preceded by a flu-like prodrome with or without oral or genital lesions. The clinical presentation is (1) encephalitis without hepatic involvement, (2) hepatitis with or without central nervous system findings, or (3) disseminated skin lesions. When the liver is involved, rapidly progressive hepatic necrosis and severe coagulopathy are common and the mortality is high. Early diagnosis and early institution of antiviral chemotherapy with acyclovir greatly improve the outcome for both mother and infant.

Adult↗

Immunoprophylaxis against klebsiella and pseudomonas aeruginosa infections. The Federal Hyperimmune Immunoglobulin Trial Study Group.

To determine if passive immunization could decrease the incidence or severity of Klebsiella and Pseudomonas aeruginosa infections, patients admitted to intensive care units of 16 Department of Veterans Affairs and Department of Defense hospitals were randomized to receive either 100 mg/kg intravenous hyperimmune globulin (IVIG), derived from donors immunized with a 24-valent Klebsiella capsular polysaccharide plus an 8-valent P. aeruginosa O-polysaccharide-toxin A conjugate vaccine, or an albumin placebo. The overall incidence and severity of vaccine-specific Klebsiella plus Pseudomonas infections were not significantly different between the groups receiving albumin and IVIG. There was some evidence that IVIG may decrease the incidence (2.7% albumin vs. 1.2% IVIG) and severity (1.0% vs. 0.3%) of vaccine-specific Klebsiella infections, but these reductions were not statistically significant. The trial was stopped because it was statistically unlikely that IVIG would be protective against Pseudomonas infections at the dosage being used. Patients receiving IVIG had more adverse reactions (14.4% vs. 9.2%).

Antibodies, Bacterial↗

Mosquito-transmitted malaria acquired in Texas.

Malaria was endemic in the United States before World War II. However, locally acquired malaria was thought to have been eradicated. Since the mid-1980s, cases of locally acquired malaria have been described. We report the case of a 62-year-old man who came to the Houston Veterans Affairs Medical Center with fever, malaise, and weakness and was found to have Plasmodium vivax infection on peripheral blood smear. He had not left the country for 37 years and had no previous history of malaria. On specific questioning, he mentioned heavy exposure to mosquitoes. Thus, malaria was presumably transmitted locally by mosquitoes. Subsequently, two other cases of apparently locally acquired, mosquito-transmitted malaria were identified in Houston. Symptoms, signs, and general laboratory test results do not typically suggest a specific diagnosis. Therefore, malaria should be considered in all patients with febrile illnesses, even those without a history of travel.

Animals↗

The current spectrum of Staphylococcus aureus infection in a tertiary care hospital.

Staphylococcus aureus remains a prominent cause of community- and hospital-acquired infection. This study reviewed 162 cases of S. aureus infection occurring in 120 adults who were hospitalized at a Veterans Affairs Medical Center and referred for consultation to the Infectious Disease Service. There were 37 cases of skin and soft tissue infection, 5 pyomyositis, 34 osteomyelitis, 13 septic arthritis, 19 pneumonia, 3 empyema, 5 pyelonephritis, 37 vascular infection, 3 epidural abscess, and 6 miscellaneous infections. Bacteremia was documented in 56 of 119 (47%) cases in which blood cultures were obtained, indicating the serious nature of the infections in many cases. Staphylococcus aureus is widely prevalent in healthy persons. Given its ubiquity and the capacity to cause a broad array of infections, an effective host response must play an important role in preventing infection. This host response is immunologically nonspecific, in that it depends upon the effectiveness of mechanical barriers to invasion and, once invasion takes place, the interaction of PMN, complement, and antibody that is probably present in serum of all immunologically competent adults rather than sensitization of B or T lymphocytes by any identifiable antigens specific to S. aureus. Analysis of the present cases calls attention to S. aureus as an opportunistic pathogen, 1 that only infrequently causes serious infection in otherwise healthy persons. Nearly every patient in this series had 1 or more medical condition thought to predispose to infection; 279 such conditions were identified, representing an average of 2.3 per person. A break in the natural barrier to infection was also present in the majority of cases, for example, trauma, wound, or pre-existing decubitus ulcer in skin and soft tissue infections; endotracheal tube in pneumonia; and a catheter bypassing urethra or skin in urinary and vascular infections, respectively. The tendency for patients to be infected with S. aureus repeatedly (mean number of infections, 1.4 per patient) reflects the chronicity of many predisposing factors and, perhaps, of colonization as well. Staphylococcus aureus has a special predilection to cause infections involving prosthetic devices, perhaps related to its affinity for fibronectin, laminin, and other serum proteins that can mediate attachment to foreign material; 46 of 162 (28%) infections were associated with the presence of a foreign body. Such infections are difficult to eradicate with antibiotic therapy alone, perhaps because of a change in the metabolic state of adherent bacteria, and removal of the foreign body is generally required for cure.(ABSTRACT TRUNCATED AT 400 WORDS)

Abscess↗

Concomitant neurocysticercosis and brucellosis.

A young Mexican woman had headache and left arm weakness develop shortly after immigrating to the United States. A solitary cerebral cysticercus was found at surgery, but, instead of the expected finding of clear fluid, the cyst contained pus from which Brucella melitensis was cultured. Although the patient had no signs or symptoms suggestive of brucellosis, agglutination studies revealed IgM and IgG antibodies consistent with active brucellosis. Clinicians should be alert to the possibility of multiple infections in immigrants from countries where parasites and bacteria that are uncommon in the United States are endemic.

Adult↗

Infections associated with prosthetic devices: magnitude of the problem.

The number and diversity of prosthetic devices inserted into patients continue to increase each year. Despite technological advances in the design and manufacture of prostheses and improved surgical techniques, infection remains a serious and potentially fatal complication. Although the incidence of serious infections remains low (averaging a few per cent for totally implanted devices), the consequences of an infected prosthesis can be disastrous. The incidence of infections related to temporary or partially implanted devices is even higher than for prostheses completely covered by the skin. In addition to excess morbidity and occasionally mortality, prosthesis-related infections add to the costs of medical care and to prolonged hospitalization. the magnitude of the problem is greatly underappreciated, in part due to the care of such infections on a piecemeal basis by numerous and diverse health care specialists.

Bacterial Infections↗