Clinical case seminar: a 33-year-old woman with a pituitary mass and panhypopituitarism.
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Biomedical subjects
Publications and source records attributed to C A Ellsworth.
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We performed Southern analysis of mitochondrial DNA (mtDNA) in 6 tissues from a patient with Kearns-Sayre syndrome and found a single deletion of 4.9 kb in all tissues. The percentage of deleted mtDNAs varied widely between tissues, from only 4% in smooth muscle to approximately 50% in skeletal muscle. Samples of DNA obtained from 3 different skeletal muscles and from separate areas of individual tissues showed little variation in percentage of deleted mtDNA. Biochemical analysis showed no clear correlation between mitochondrial enzyme activity and deleted mtDNAs.
The physiologic measurements of a subpopulation of mononuclear cells derived from head and neck lymphoid tissues are similar to those of dendritic cells are described. Dendritic cells are a subpopulation of bone marrow-derived leukocytes that were originally identified in rodents and now described in man as having central control of T-lymphocyte functions. We describe a technique for the enrichment of dendritic cells obtained from tonsils utilizing a bovine serum albumin (BSA) gradient and note that they have the light and electron microscopic appearance of dendritic cells. The measured oxidative mitogenic response and interferon-gamma production in complete leukocyte cultures was compared with BSA gradient-separated preparations. The denser cells, comprised mostly of normal appearing lymphocytes, would not undergo a mitogenic response nor produce normal amounts of interferon when stimulated unless the dendritic cell-rich, less-dense fraction, was added back. The dendritic cells derived from tonsils seem to behave as a potent accessory cell for these T-lymphocyte-associated functions.
Erythrocytosis, increased whole blood oxygen affinity, and iron overload were found in a 37-yr-old man. Electrophoretic techniques to demonstrate a hemoglobin variant showed no abnormality. Structural studies of the hemoglobin from this patient revealed an abnormal hemoglobin previously described as Hemoglobin Olympia, a high-affinity variant. Study of three generations in this family showed increased hepatic iron or iron absorption in some members of all three generations studied. The findings in this family are consistent with an increase in iron absorption due to the consequences of Hemoglobin Olympia and the heterozygous state for hemochromatosis (allele for hemochromatosis associated with HLA-A3, B7) or the presence in the family pedigree of three different alleles for hemochromatosis (alleles for hemochromatosis associated with HLA-A3. B7, HLA-A3, B15, and HLA-A9, B44) with the heterozygous state being manifest with increased iron absorption.
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Experimentally induced chronic arachnoiditis was studied over a one-year period in beagle dogs. All animals demonstrated severe extensive changes at postmortem which were strikingly similar to those reported in man. Arachnoid inflammation, fibrosis, and adhesions were often associated with nerve roots embedded in collagen. These changes were present in all four experimental dogs, and were absent in four controls. In spite of having pathologically severe changes of arachnoiditis, two of four dogs appeared clinically normal. We suggest that the clinical appearance of chronic arachnoiditis in this animal model may reflect the human experience, wherein presentations may vary from merely complaints of back pain to severe sensory-motor impairment.