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Biomedical subjects

D P Perl

Publications and source records attributed to D P Perl.

126 records · Page 7Linked to original sources

Macroscopic pathology of the lungs in Legionnaires' disease.

We used the whole lung section technique to review the macroscopic pathology in 12 patients who died with Legionnaires' disease. None of these patients had been treated with erythromycin. Consolidation was evenly distributed throughout all lobes without a consistent segmental distribution. The smallest lesions were around bronchioles or bounded by lobular septa. In most cases there was confluent involvement of multiple lobules. Extensive consolidation made distinction between a lobar and confluent lobular distribution difficult. Abscesses were present in two cases and nodular infiltrates in two others. In five additional patients, Legionnaires' disease had been treated with erythromycin. Four had a clinical response to treatment, and the fifth had diffuse staphylococcal pneumonia as the predominant lesion. Because the lungs of all five patients contained bacteria other than the Legionnaires' disease bacterium at the time of autopsy, it was difficult to ascertain the role of Legionnaires' disease bacterium in the pathology.

Bacteria↗

Neurological manifestations of hereditary hemorrhagic telangiectasia (Rendu-Osler-Weber disease): report of 2 cases and review of the literature.

Two cases of hereditary hemorrhagic telangiectasia (HHT) with neurological involvement are presented. One patient had multiple vascular malformations including telangiectasias of the brain, medulla, and spinal cord and a berry aneurysm of the internal carotid artery; she also had a large cerebellar abscess, presumably reflecting the presence of a pulmonary arteriovenous fistula. The second patient had an idiopathic subarachnoid hemorrhage. In more than 200 reported patients with HHT involving the nervous system, 61% had lesions seondary to a pulmonary arteriovenous fistula (cerebral hypoxemia, paradoxical and septic emboli, and brain abscess). The findings emphasize the need for early surgical correction of such fistulas. In 36% of the patients with neurological involvement and HHT, vascular malformations of the brain and spinal cord were documented, and in 3%, portal-systemic encephalopathy was noted. Multiple lesions were frequent. HHT should be considered a generalized vascular dysplasia (universal or systemic angiomatosis), and not simply a benign mucocutaneous disease.

Aged↗

The pathology of Legionnaires' disease. Fourteen fatal cases from the 1977 outbreak in Vermont.

Fourteen fatal cases from the 1977 Vermont outbreak of Legionnaires' disease have been analyzed. Serious underlying diseases were present in all patients. The only consistent lesions were in the lungs. Bronchopneumonia was present in all cases and was confluent in most. No lobe of the lung was preferentially involved and consolidation was usually bilateral. Abscesses were evident macroscopically in only two cases. Microscopically, there was an extensive alveolar infiltrate of polymorphonuclear neutrophils and macrophages. Lysis of the inflammatory cells was frequently present and was associated with an increased number of bacteria. Coagulative necrosis of lung was present in a few cases, and the possibility of a bacterial toxin must be considered. Bacteria were well stained by the Dieterle stain and appeared Gram-negative in tissue imprints from the unfixed lung.

Adolescent↗

Partial trisomy 8 (trisomy 8q2106 leads to 8qter).

A case of trisomy for part of the long arm of chromosome 8, confirmed by G-banding analysis, in a white male infant is described. The mother carried a reciprocal translocation between chromosome 8 and chromosome 13 (46,XX,t(8;13),(q21:q34). The patient had inherited the translocated chromosome 13 and was thus trisomic for the distal half of the long arm of chromosome 8. He had many of the clinical features of the full trisomy 8 syndrome. As compared with previously reported cases with trisomy of the distal end of chromosome 8, he was more dysmorphic and showed greater developmental retardation.

Abnormalities, Multiple↗

Diencephalic amnesia: a reappraisal.

The anatomical basis of memory disorder related to lesions of the diencephalon is a controversial matter. A study of a patient who developed severe amnesia in association with bilateral metastatic tumour invasion of the medial and posterior thalamus is reported. The findings in this case have led to further discussion of this unsettled issue.

Amnesia↗

Pneumocystis carinii pneumonia in the United States: epidemiologic, diagnostic, and clinical features.

Analysis of 194 patients with confirmed Pneumocystis carinii pneumonia in the United States over a 3-year period revealed that P. carinii pneumonia occurred almost exclusively in the immunosuppressed host who had a serious underlying disease. The epidemiologic features of pneumocystis pneumonia primarily reflected those of the underlying disease. P. carinii was identified antermortem in 81% of the cases usually by biopsy or needle aspiration of the lung, procedures associated with considerable morbidity and morbidity and mortality. Laboratory identification of P. carinii was usually accurate, but errors resulting from fault staining tecnique occurred. Most patients had been ill less than 2 weeks with bilateral diffuse interstitial pneumonia. Leukopenia (white blood cell count less than or equal to 3,00/mm3) and probably severe hypoxia were negative prognostic factors. Although treatment with pentamidine was effective, the drug frequently cased adverse reactions, particularly impaired renal function, when given with other nephrotoxic agents.

Adolescent↗

Genetics and Alzheimer's disease: notes of an environment watcher (with apologies to L. Thomas).

Support has recently been voiced for the genetic hypothesis of Alzheimer's disease; frequently at the expense of considerations of environmental factors. In the adult, genetic diseases rarely exist without modification by environment forces. Atherosclerosis leading to myocardial infarction may provide a useful model to consider genetic and environmental factors in Alzheimer's disease. Familial hypercholesterolemia produces a premature inherited form of myocardial infarction, whereas, atherosclerosis with myocardial infarction, seen in advanced age, relates more closely to environmental factors such as smoking, diet, etc.

Alzheimer Disease↗

Proposal to revise the morphologic criteria for the diagnosis of Alzheimer's disease.

Ten years have passed since the drafting of the original National Institute on Aging/American Association of Retired Persons or Khatchaturian criteria for the neuropathologic diagnosis of Alzheimer's disease. In that time, much progress has been made in the study of this disorder. It is clear that although the Khatchaturian criteria have been useful, advances in our understanding of the morphologic substrate of the disease needs to be incorporated in any newly proposed diagnostic criteria. We propose diagnostic criteria to be employed in establishing the diagnosis of Alzheimer's disease in a research setting. Here, we require that a level of density and distribution of senile plaques and neurofibrillary tangles be identified and that other superimposed conditions, such as major cerebral infarcts and/or Parkinson's disease changes, are absent. We also propose separate criteria for diagnosing individuals who die in the early stages of the disease. These latter cases are of extreme research interest and are characterized by a distribution of neurofibrillary tangles that remains primarily restricted to the entorhinal cortex and hippocampus.

Aged↗