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Biomedical subjects

K Berry

Publications and source records attributed to K Berry.

At least 55 records · Page 3Linked to original sources
PubMed1991

Hepatic hemangioblastoma. An unusual presentation in a patient with von Hippel-Lindau disease.

We report a case of multiple capillary hemangioblastomas of the liver occurring in a patient with von Hippel-Lindau disease and a history of previous cerebellar and spinal hemangioblastomas. Although rare examples of this tumor have previously been recorded in the pancreas, kidney, and urinary bladder, this appears to be the first recorded case with hepatic involvement. The histology and immunohistochemical appearance of this neoplasm are identical with those of the cerebellar tumor. We believe it represents a separate primary neoplasm rather than metastatic disease.

Adult↗
PubMed1990

Transgenic rearranged T cell receptor gene inhibits lymphadenopathy and accumulation of CD4-CD8-B220+ T cells in lpr/lpr mice.

The lpr gene in homozygous form induces development of CD4-CD8-B220+ T cells and lymphadenopathy in MRL and C57BL/6 mice. Although the propensity for excessive production of T cells is related to an intrinsic T cell defect, a thymus is also required because neonatal thymectomy eliminates lymphadenopathy. Recent evidence suggests that excessive production and release of autoreactive T cells from the thymus of lpr/lpr mice might lead to downregulation of CD4 and CD8 as a "fail safe" tolerance mechanism that occurs during late thymic or post-thymic development. To test this hypothesis, T cell receptor (TCR) transgenic mice that produce large numbers of immature thymocytes recognizing the H-2Db and male H-Y antigens were backcrossed with C57BL/6-lpr/lpr mice and MRL-lpr/lpr mice. It was predicted that Db male lpr/lpr mice would produce large numbers of autoreactive T cells during early thymic development that would lead to an accelerated lymphoproliferative disease. In contrast, Db female lpr/lpr mice would produce large numbers of Db H-Y-reactive T cells, but might not develop lymphadenopathy because the male H-Y antigen would not be present. Unexpectedly, there was complete elimination of lymphadenopathy in both male and female TCR transgenic lpr/lpr mice. The elimination of lymphadenopathy was not due to a failure of thymic maturation since the thymus of H-2Db female lpr/lpr mice contained nearly normal numbers of mature thymocytes. Elimination of lymphadenopathy was also not due to a lack of autoreactive T cells in the peripheral lymph nodes (LN) since there was an increased syngeneic mixed lymphocyte proliferative response of LNT cells from transgenic lpr/lpr compared with +/+ mice in vitro. Hypergammaglobulinemia and autoantibody production in the transgenic lpr/lpr was present at levels comparable with or higher than control nontransgenic lpr/lpr mice, suggesting a dissociation of autoantibody production from the lymphoproliferative disease in the TCR transgenic mice. Conversely, the development of lymphadenopathy and production of CD4-CD8-B220+ T cells appear to be intimately linked, as both were completely eliminated in T cells expressing the transgenic TCR. We propose that lymphoproliferation and production of CD4-CD8-6B2+ T cells in lpr/lpr mice is related to decreased expression of the TCR, and providing the T cells with a rearranged TCR transgene overcomes this defect.

Animals↗
PubMed1990

Kinetic analysis of chemotactic peptide-induced actin polymerization in neutrophils.

Definition of the kinetics of ligand-activated actin polymerization in the neutrophil is important for ultimately understanding the mechanisms utilized for regulation of actin polymerization in this non-muscle cell. To better define the kinetics of formyl peptide (fMLP)-induced actin polymerization in neutrophils we determined F-actin content at 5 second intervals after activation of human neutrophils with a range (10(-11)-10(-9) M) of fMLP concentrations. The state of actin polymerization was monitored by quantifying F-actin content with NBD phallacidin binding in both flow cytometric and extraction assays. Results demonstrate three successive kinetic periods of fMLP-induced actin polymerization in neutrophils, a lag period, a 5 second period when rate of polymerization is maximal, and a period of declining rate of actin polymerization as F-actin content approaches a maximum. The duration of the lag period, the maximum rate of polymerization, and the maximum extent of polymerization all depend upon the fMLP concentration. The lag period varies from 0 to 12 seconds and is followed in 5-10 seconds by a 5 second burst of actin polymerization when the rate is as great as 9% increase in F-actin content per second. After the 5 second burst of polymerization, the rate of polymerization rapidly declines. The study defines three distinct kinetic periods of fMLP-induced actin polymerization during which important rate-limiting biochemical events occur. The mechanistic and motile implications of kinetic periods are discussed.

Actins↗
PubMed1990

Acute sensory neuronopathy with preserved SEPs and long-latency reflexes.

A case of spontaneous acute sensory neuronopathy is described in a previously healthy 19-year-old male. He had marked sensory ataxia with relative sparing of cutaneous sensation. There was no recovery over 4 years. Sural nerve biopsy showed modest axonal loss. Sensory evoked potentials were mildly abnormal in the face of absent sensory nerve action potentials. The long-latency (R2) reflex was preserved whereas the short-latency (R1) reflex was absent. The findings indicate that loss of large-diameter (group Ia) ganglion cells are primarily responsible for this syndrome.

Acute Disease↗
PubMed1990

Dominantly inherited apathy, central hypoventilation, and Parkinson's syndrome: clinical, biochemical, and neuropathologic studies of 2 new cases.

We describe 2 new patients from a family in which 10 persons in 3 successive generations had a dominant neuropsychiatric disorder characterized by apathy, central hypoventilation, and parkinsonism. Neuropathologically, both patients showed severe neuronal loss and reactive gliosis in the substantia nigra. Neurochemical studies showed a marked depletion of dopamine in substantia nigra, putamen, and caudate nucleus, as well as reduction in serotonin content in the substantia nigra. Glutamate contents were low in frontal cortex and thalamus, and gamma-aminobutyric acid (GABA) contents were low in thalamus and substantia nigra of both patients. In addition, phosphoethanolamine contents were reduced in all brain regions of both patients, especially in the substantia nigra. One patient with severe symptoms had low levels of homovanillic acid, 5-hydroxyindoleacetic acid, and GABA in his CSF repeatedly for 3 years before death (aged 58), while the 2nd patient died (aged 51) of an unrelated cause before developing any symptoms of the familial disorder. Because brain deficiencies of multiple neurotransmitters appear to be involved, this disorder is unlikely to respond to treatment; however, neurochemical studies of CSF may make presymptomatic diagnosis feasible.

Adult↗
PubMed1989

Examination of brains of AIDS cases for human immunodeficiency virus and human cytomegalovirus nucleic acids.

The role of direct virus infection as a determining factor in acquired immunodeficiency syndrome (AIDS) dementia was investigated using in situ hybridisation for human immunodeficiency virus (HIV) and human cytomegalovirus (HCMV). Four of the five AIDS dementia patients in this series demonstrated HIV infected cells distributed in widely different parts of the brain, but only one case showed HCMV infected cells. The greater abundance of HIV was in subcortical white matter in nodular areas consisting of monocyte/macrophage infiltrates. The cells were occasionally arranged as a multinucleated syncitium. In two cases, a few large cells with the appearance of neurons were positive for HIV hybridisation. By appropriate treatment with ribonuclease, it was shown that hybridisation was primarily to HIV RNA. HCMV infected cells were observed in small numbers in only one of the positive cases, suggesting that HCMV is not a determining factor in AIDS dementia. HCMV positive cells were located in the grey matter, with an appearance suggestive of neurons. Cells expressing the MHC-class II antigen HLA-DR, a marker of reactive microglia and macrophages, were observed to be extensive in affected brain sections in the one case examined. These cells were present in greater number than HIV infected cells. In this case, extensive numbers of HIV infected cells were noticed along the peripheral margin of the substantia innominata. This could indicate infection in this case of a critical brain region from the cerebrospinal fluid.

Acquired Immunodeficiency Syndrome↗
PubMed1988

Duodenal tuberculosis.

The clinical and radiological features in 30 cases of duodenal tuberculosis are presented. The patients are divisible into two groups on the basis of their presenting features. Twenty-two had symptoms and signs of gastric or duodenal obstruction, and eight patients had dyspepsia suggesting peptic ulceration. In the first group, laporotomy showed that the duodenal obstruction was due to extrinsic compression by matted tuberculous lymph nodes in the majority; there was only five intrinsic strictures. In patients with dyspepsia there were bulbar and post-bulbar ulcers accompanied by more widespread mucosal changes, induration, and periduodenal lymphadenopathy. Bypass procedures were performed to relieve obstruction; no resection was possible because of the adherence of the lymph node masses.

Adolescent↗
PubMed1988

Lymphocytic adenohypophysitis.

Lymphocytic adenohypophysitis (LAH) is an uncommon disorder in the spectrum of pituitary disease. Twenty-three cases proven by biopsy or at autopsy have been reported since 1962. We report 2 further cases and review the etiology, immunology and pathology of the disease. The diagnosis should be considered in a female patient who presents during the post-partum period with the clinical picture of a non-functional or prolactin cell pituitary adenoma and evidence of hypopituitarism.

Adult↗
PubMed1988

Brain lesions in a Pacific white-sided dolphin (Lagenorhynchus obliquidens).

A young, male, free-ranging Pacific white-sided dolphin (Lagenorhynchus obliquidens) was found disoriented and died after being held in captivity for several months. Malacic lesions in several areas of the brain were associated with helminth eggs. The appearance and location of these eggs suggested they were of the genus Nasitrema.

Animals↗
PubMed1987

Cytochrome c oxidase deficiency in Leigh syndrome.

We studied 6 mitochondrial enzymes in crude extracts and isolated mitochondria from 5 children with pathologically proven subacute necrotizing encephalomyelopathy (Leigh syndrome). Samples were taken from brain (5 patients), skeletal muscle (4 patients), liver (4 patients), kidney (4 patients), heart (1 patient), and cultured fibroblasts (3 patients). An isolated defect of cytochrome c oxidase (COX) activity was found in brain (decrease of activity to 15 to 39% of the normal mean), muscle (9 to 20%), kidney (1 to 67%), and in the 1 available heart (4%) from a patient with cardiopathy. COX activity was also decreased in liver of 3 patients (2 to 13% of normal) and in cultured fibroblasts of 2 patients (18 and 27%), but it was normal in both liver and fibroblasts from 1 patient. Immunotitration using polyclonal antibodies against human heart COX showed essentially normal amounts of cross-reacting enzyme protein in various tissues from different patients. Electrophoresis of COX immunoprecipitated from brain mitochondrial extracts showed normal patterns of COX subunits in 2 patients. This study confirms the theory that COX deficiency is an important cause of Leigh syndrome.

Brain↗
PubMed1987

Acetylcholine receptor antibodies in myasthenia gravis: use of a qualitative assay for diagnostic purposes.

We have modified the techniques of Lindstrom and of Tindall to measure serum acetylcholine receptor antibody using human antigen bound to 125I-alpha Bungarotoxin. By using 10 microliters of serum and precipitating antigen-antibody complexes with an excess of staph A, we found that only one out of 43 patients with clinically diagnosed active generalized Myasthenia Gravis had no antibodies. In pooling these results with the results of tests done for diagnostic purposes we found positive results in 54/55 generalized active MG, 8/21 MG in remission, 16/37 ocular MG and 0/55 healthy controls. Two out of 38 non MG were also positive and their clinical diagnosis of botulism and penicillamine treated rheumatoid arthritis have been confirmed by a one year follow-up. Most of these sera were also tested for reactivity with fetal calf AchR. Six out of 49 samples positive with the human receptor were negative with calf receptor. We conclude that our technique is extremely useful for the diagnosis of Myasthenia Gravis and that fetal calf antigen cannot replace human antigen in the assay.

Animals↗
PubMed1987

Pathological and clinical study of Müller's muscle in Graves' ophthalmopathy.

Specimens of Müller's muscle from patients with Graves' disease were examined for evidence of morphologic, histochemical, immunohistochemical and electron microscopic changes. No scarring, significant inflammation or shared antigenicity between Müller's muscle and thyroglobulin-related antigens was found. There were moderate numbers of mast cells in 48 of 66 specimens. Computed tomography in 16 patients with Graves' ophthalmopathy showed enlargement of the superior rectus-levator complex in 17 of 28 orbits with lid retraction. However, no correlation was found between the amount of lid retraction and the degree of enlargement of the muscle complex.

Ciliary Body↗
PubMed1983

Cytoplasmic body myopathy. Report on a family and review of the literature.

A 15-year-old girl who was seen for scoliosis presented with cardiorespiratory failure associated with a respiratory infection. She was found to have weakness predominant in the face, sternomastoid, proximal limb, respiratory, spinal and cardiac muscles. The serum creatine kinase level was slightly elevated and the electrocardiogram was abnormal. The electromyograph was consistent with a myopathy. The course was malignant. Her 14-year-old brother had similar findings and succumbed at the age of 14 and one-half years from cardiorespiratory failure. The mother had minimal weakness of proximal limb muscles since early life. The tendon reflexes were normal as was the serum creatine kinase level. The course was benign. On light microscopy the muscle biopsy in the girl showed fibre diameter variation, centrally placed nuclei, necrosis, fibrosis and cytoplasmic bodies. The muscle biopsy in the brother and mother had similar findings except that the inclusion bodies were not seen in the mother. On electron microscopy, the girl showed typical cytoplasmic bodies, involving predominantly type 1 fibres. The mother also had these structures. The literature is reviewed and the origin, pathogenesis and aetiology of the cytoplasmic body are discussed.

Adolescent↗
PubMed1983

Inclusion body myositis (IBM): myopathy or neuropathy?

Inclusion body myositis (IBM) is described in six elderly patients (three women) and in a young familial patient. They all showed the morphologically characteristic vacuoles containing osmiophilic membranous whorls and intracytoplasmic or intranuclear inclusions. There is a well-delineated bimodal age spectrum of IBM, with onset in the second and sixth decades, but otherwise the disorder seems to be a specific entity. Clinical, electrophysiologic, and morphologic features suggest a neurogenic origin in some cases.

Aged↗
PubMed1983

Acute hemorrhagic leukoencephalopathy. A clinical, pathological, and radiological correlation.

Two patients with acute hemorrhagic leukoencephalopathy, one of which was pathologically proven, were serially studied with CT scanning. Both patients showed marked distinctive low density white matter changes throughout both hemispheres, which correlated with clinically involved areas. One patient recovered from the disease, perhaps due to steroid treatment, and showed slow but complete resolution of CT scan changes. We feel that CT scan findings significantly help in the diagnosis of this disease, which may be amenable to early treatment with steroids.

Acute Disease↗