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Biomedical subjects

D Armstrong

Publications and source records attributed to D Armstrong.

At least 523 records · Page 29Linked to original sources

Dolichol and dolichyl phosphate levels in brain tissue from English setters with ceroid lipofuscinosis.

Human ceroid lipofuscinosis (CL) is an inherited disease marked by cerebromacular degeneration and early death. We have utilized the canine model to investigate the possible role of dolichol and dolichyl phosphate in the developmental pathology of CL. We found that while brain levels of dolichol increase with age in both affected and unaffected dogs, the amount in the diseased animal was similar to that in controls. Brain levels of dolichyl phosphate ranged from 20 to 35 micrograms/g in control dogs at all ages examined, but increased substantially during development in the affected dogs, a value of 113 +/- 24 micrograms/g (mean +/- SD) being obtained in the end-stage animals. In addition to the results obtained in the canine model, dolichyl phosphate levels in human brain tissues from a 5-year-old with late infantile CL and from a 19-year-old with juvenile CL were found to be 153 and 382 micrograms/g, respectively, compared with a control that assayed 26 micrograms/g. The preliminary findings with human tissues provide further evidence for an association of elevated brain dolichyl phosphate levels with CL. Whether the increase is primary or secondary remains to be determined.

Adult↗

Infectious complications of the acquired immune deficiency syndrome.

The cause of AIDS is unknown. In the absence of a specific etiologic agent or diagnostic test, a case can only be recognized when complications of the immune deficiency such as infection or Kaposi's sarcoma occur. Defective T-cell function is the principal immunologic defect; there are also defects, however, in B-cell function that may have some clinical significance. It has not yet been possible to reverse the immunologic deficiency, and this failure has been the principal prognostic factor in this illness. A number of the infectious complications of AIDS, however, can be diagnosed and successfully treated.

Acquired Immunodeficiency Syndrome↗

Clinical correlations of serial quantitative blood cultures determined by lysis-centrifugation in patients with persistent septicemia.

The potential clinical value of colony counts determined by the lysis-centrifugation blood culture method was studied by reviewing the records of eight patients with persistent septicemia in whom colony counts were available on at least 3 days. Colony counts of the five patients who survived decreased steadily as the patients improved. One of the three patients who died had counts repeatedly below 1.0 CFU/ml while she was clinically stable and higher counts when her condition deteriorated. Two patients died despite decreasing colony counts. One was improving and died unexpectedly of an unrelated cause; the other died of candidiasis, but declining serial arabinitol/creatinine ratios suggested a partial response to therapy. In addition, septicemia related to infected intravenous catheters was documented by demonstrating large differences in colony counts determined simultaneously from two different sites in two patients and by demonstrating a precipitous drop in CFU per milliliter after removal of the infected catheter in one patient. Routine availability of colony counts appears to be an important advantage of the lysis-centrifugation method.

Adolescent↗

Influence of N-acetylhomocysteine thiolactone on cultured retinal cells in canine neuronal lipofuscinosis. An ultrastructural study.

An SH- group donor drug, N-acetylhomocysteine thiolactone (0.2 mg/ml), was added to retinal cultures from an adult dog affected with ceroid lipofuscinosis (NCL). This substance was applied every 3rd day to the culture medium. After 1, 2 and 3 weeks in culture the cells were fixed for electron microscopy. Ultrastructurally the NCL-specific lipopigments revealed extensive fading out or vacuolation, revealing randomly arranged lamellar or 'fingerprint' membranes as well as structures resembling degenerated mitochondria. Additionally extensive fragmentation of the pigment bodies was present. The significance of these results are discussed.

Animals↗

Bacterial infections of the CNS in neutropenic patients.

In neutropenic patients, fever and mental status changes are frequently the only overt clinical manifestations of bacterial infections of the CNS. Prominent headache and meningeal signs are exceptional. CNS infections may occur even in patients receiving large doses of broad-spectrum antibiotics. CSF culture and Gram's stain are required to establish or exclude the diagnosis and are often positive, even in patients receiving antibiotics for other indications. The CSF cell counts and chemistries are helpful if abnormal, but, when normal, provide no assurance that infection is not present. The CSF glucose can be lowered in the absence of pleocytosis, but a low CSF glucose is neither sensitive (27% in this series) nor specific. Lumbar puncture is hazardous in many neutropenic patients because of simultaneous thrombocytopenia; lumbar puncture should be performed by an experienced physician after platelet transfusions. The outcome of CNS infection depends on the underlying clinical disorder and on bone marrow recovery. The use of third-generation cephalosporins, new semisynthetic penicillins, and intrathecal administration of aminoglycosides may improve outcome.

Agranulocytosis↗

Bronchoalveolar lavage in the diagnosis of diffuse pulmonary infiltrates in the immunosuppressed host.

The usefulness of bronchoalveolar lavage in the diagnosis of pulmonary infiltrates in the immunosuppressed patient was studied in 97 patients. In immunosuppressed patients, the available diagnostic procedures are often invasive and have variable yield and a potential for serious complications. Bronchoalveolar lavage had an overall diagnostic yield of 66% (61 of 92 diseases). It was most effective in the diagnosis of opportunistic infections, including infection with Pneumocystis carinii (18 of 22 cases), cytomegalovirus pneumonia (10 of 12 cases), fungal pneumonia (5 of 6 cases), and mycobacterial disease (4 of 5 cases). The technique was also helpful in suspected pulmonary hemorrhage (7 of 9 cases) but was less useful for diagnosing malignancy (10 of 22 cases) and drug-induced toxicity (6 of 15 cases). Findings of bronchoalveolar lavage could be combined with those of transbronchial biopsies, brushings, and washings in the diagnosis of most of the diseases. The procedure was safe, even in thrombocytopenic patients and those requiring mechanical ventilatory support. Bronchoalveolar lavage is a valuable procedure for evaluation of pulmonary disease in the immunosuppressed host.

Adolescent↗

Fungal infections in the immunocompromised host.

Invasive fungal infections cause significant morbidity and mortality in patients with impaired immune defences. Defects in neutrophil function and neutropenia predispose to disseminated Candida, Aspergillus and Mucoraceae infections while altered T-lymphocyte mononuclear phagocyte function predisposes to infection with C. neoformans, Histoplasma and Coccidioides. Fungal infections in the immunocompromised host are difficult to diagnose and difficult to treat successfully. The diagnosis is often missed or delayed because of the non-specific clinical features, the failure to isolate or difficulty in interpreting the presence of the fungus from routine microbiological cultures, and the limited usefulness of available serological tests. The assay for cryptococcal antigen is the only currently available reliable serological test used to diagnose an invasive fungal infection. Definitive diagnosis is made by histopathological demonstration of the fungus in tissue or a positive culture from a sterile body site. Invasive procedures are often necessary to obtain adequate tissue for histology and culture. The treatment of invasive fungal infection in the immunocompromised host is amphotericin B with or without 5FC. The usual recommended dose is 1.5 to 3 g total amphotericin B over 6 to 12 weeks. The optimal dose and duration of therapy for each infection is not known. Treatment failures and relapses are common in patients who do not achieve remission of their underlying disease. Ketoconazole, a new broad-spectrum oral antifungal medication, does not appear to be effective therapy for invasive fungal infection in the immunocompromised patient based on results of small clinical trials. New diagnostic methods and therapeutic approaches are necessary to improve the outcome of these infections. Areas of current research include serological assays for fungal antigens and metabolites which may allow earlier diagnosis, treatment with combinations of antifungal agents, and the development of new antifungal agents.

Antifungal Agents↗

Computed tomographic diagnosis of distal radio-ulnar joint disruption.

Distal radio-ulnar joint dislocation may be a difficult diagnosis using standard wrist radiographs. We report a patient in whom standard films did not convincingly demonstrate a dislocation which was well seen with computed tomographic scans of the wrist. Computed tomography may be the method of choice for demonstrating distal radio-ulnar joint dislocation when this is suspected clinically but not seen with standard films.

Adult↗

Toxoplasmosis. Problems in diagnosis and treatment.

Forty-two cases of Toxoplasma gondii infection were analyzed, 25 in patients with neoplastic disease and 17 in apparently normal patients. Infection in normal hosts was usually manifested by adenopathy and ran a benign course. Infection in patients with neoplastic diseases was usually manifested by fever and/or neurologic symptoms. Patients with leukemias and lymphomas, particularly Hodgkin's Disease, were at highest risk for infection. Most, but not all, patients developed serologic titers indicative of infection. T. gondii infections were fatal in all eight cases with central nervous system involvement despite treatment in five cases. Leukopenia was a significant complication of treatment with sulfadiazine and pyrimethamine despite the use of folinic acid.

Adolescent↗

Severe pneumococcal infection in patients with neoplastic disease.

A study of pneumococcal bacteremia in 56 patients with neoplastic disease from January 1, 1972 to June 30, 1980 is presented and compared to an earlier study between 1955 and 1971. Patients at highest risk were those with Hodgkin's disease who had been splenectomized, multiple myeloma and chronic lymphocytic leukemia showing an attack rate of 15.6/1000, 12.5/1000, and 10.8/1000, respectively. The attack rate was more than three times higher among patients with Hodgkin's disease in the present series compared to the previous series. In 32% of cases there was no identifiable source for the infection. Four splenectomized patients with Hodgkin's disease developed pneumococcal meningitis and two died. The overall mortality rate was 32% versus a rate of 18% for those treated with appropriate antibiotics for more than 24 hours. There was a significant improvement in overall survival when compared with our previous series. As before, almost one fourth (24%) of our isolates were not among those included in the pneumococcal vaccine presently available. Antibiotic prophylaxis should be considered in high risk patients.

Adolescent↗

Pasteurella multocida septicemia. Experience at a cancer hospital.

Pasteurella multocida most commonly infects patients with animal contacts. Life-threatening systemic disease is distinctly uncommon in otherwise healthy persons and usually occurs in patients with chronic predisposing disease. Two cases of sepsis occurred in a cancer hospital, and we surmise that specific predisposing factors existed in our patients as in prior reported cases of sepsis in patients without cancer. These factors include animal contact, open wounds, and, most important, advanced hepatic disease.

Adult↗

Postmortem observations on beta-glucuronidase deficiency presenting as hydrops fetalis.

This study reports a case of type VII mucopolysaccharidosis (beta-glucuronidase deficiency) presenting as lethal hydrops fetalis. Skin fibroblast cultures established postmortem revealed deficient beta-glucuronidase activity. Mucopolysaccharides were stored in various cells of the brain, heart, kidney, liver, and spleen. The stages of maturation of the bones, kidneys, and brain were discrepant, the brain being the least mature organ. A delay in central nervous system maturation may account for psychomotor retardation in some patients with this enzyme deficiency.

Brain↗

Computed tomographic and nuclear magnetic resonance correlation of canine ceroid-lipofuscinosis with aging.

Canine ceroid lipofuscinosis is a degenerative neuronal disorder which has as a distinct pathologic counterpart, neuronal ceroid lipofuscinosis or Batten's disease in humans. The disease occurs in English setters and is associated with abnormal accumulation of autofluorescent lipopigments. As such, a study of this animal model may allow insight into the aging process as well. This investigation explores the computed tomographic features of canine ceroid-lipofuscinosis and correlates these findings (progressive atrophy, inherent changes in CT/NMR tissue characteristics) with clinical and pathologic features.

Animals↗

The fabrication of nurse--patient relationships.

This paper argues that until about 10 years ago the caring role of the nurse was restricted primarily to the biological functioning of the patient. The more recent concern with communication and patient 'psychology' in the nursing literature suggests that nursing has succeeded, through this analysis, in constituting a new identity for the patient.

Communication↗

Mucormycosis following bone marrow transplantation.

Disseminated mucormycosis, presenting with a cutaneous lesion, developed in a 20-year-old woman following bone marrow transplantation. The infecting organism was identified as Rhizopus rhizopodiformis. Despite early diagnosis and therapy with amphotericin B, the patient died.

Adult↗