Felty's syndrome and hairy cell leukaemia.
Explore the source record for details and available documents.
Biomedical subjects
Publications and source records attributed to N Lee.
Explore the source record for details and available documents.
Primary prostatic lymphoma is a very rare condition for which the treatment is ill defined. A case of this condition from Australia is reported and treatment recommendations reviewed.
The efficacy and toxicity of a regimen consisting of amsacrine (m-AMSA), cytarabine, and thioguanine for remission-induction therapy in poor prognosis categories of acute myeloid leukaemia (AML) were determined in a single arm study of 46 patients. The study group consisted of 17 patients with disease refractory to daunorubicin plus cytarabine-based induction regimens, 22 patients with disease which had relapsed during daunorubicin plus cytarabine maintenance therapy, or following completion of this maintenance programme after receiving > or = 500 mg daunorubicin/m2, and 7 previously untreated patients where cardiac disease contraindicated anthracycline therapy. Complete remission (CR) was attained in 46%, and probability of survival was comparable to published results for first-line treatment with daunorubicin plus cytarabine regimens. There was no statistically significant difference in CR rate or probability of survival between these three categories of poor prognosis AML, and cardiotoxic complications were uncommon despite extensive anthracycline exposure in the majority. In the 43% of patients who were 60-76 years of age, there was no statistically significant difference in CR rate or probability of survival relative to patients < 60 years. This observation fails to support the view that less myelotoxic regimens with lesser efficacy should be the basic approach to treatment of AML in patients > or = 60 years of age.
Explore the source record for details and available documents.
OBJECTIVE: Measurements of the volume of the hippocampus have been used preoperatively to determine the side of the hippocampus involved in patients with intractable temporal lobe epilepsy. However, the method used is time consuming and requires special training. The purpose of this study was to determine the validity of using measurements of the volume of the hippocampal body as a substitute for measurements of the volume of the whole hippocampus in patients with temporal lobe epilepsy. SUBJECTS AND METHODS: The study group consisted of 33 patients with intractable temporal lobe epilepsy due to presumed hippocampal sclerosis and 30 control subjects. Of the 33 patients with intractable temporal lobe epilepsy, 30 had hippocampal sclerosis and three had normal findings on pathologic examination of the hippocampus. T2-weighted fast spin-echo images were used to determine hippocampal volumes. Volumes were calculated by summing the sectional areas of the entire hippocampus and of the hippocampal body. Correlation between the volume of the hippocampal body and the volume of the whole hippocampus was determined. The sensitivity and specificity of measurement of the volume of the hippocampal body for lateralizing the foci responsible for seizures in patients with presumed hippocampal sclerosis were compared with those of measurement of the volume of the whole hippocampus. RESULTS: Significant linear relationships were noted between volumes of the hippocampal body and volumes of the whole hippocampus (p < .001). The sensitivity and specificity of measurements of volumes of the hippocampal body were identical to those of measurements of volumes of the whole hippocampus (87% and 100%, respectively). CONCLUSIONS: Our results show that segmental MR measurements of the body of the hippocampus are as accurate as measurements of the whole hippocampus for lateralizing temporal lobe epilepsy before surgery. Because segmental measurements are less time consuming and require less experience to perform, they are considered the procedure of choice.
A large-scale community survey in Shatin, Hong Kong, is presented with a modified two-phase design using flagged and nonflagged subsamples. A modified Self-Reporting Questionnaire and the Diagnostic Interview Schedule (version III) were used as the screening and diagnostic instruments, respectively. Lifetime rates for 19 Diagnostic Interview Schedule/DSM-III diagnoses are presented. The most common diagnoses in Shatin were tobacco dependence, generalized anxiety disorder, alcohol abuse and/or dependence, all phobias, and dysthymic disorder. The male-predominant disorders were tobacco dependence, alcohol abuse/dependence, pathological gambling, and antisocial personality. The female-predominant disorders were generalized anxiety disorder, all phobias, dysthymic disorder, major depressive disorder, obsessive-compulsive disorder, and bereavement.
The mechanisms by which chronic ethanol exposure produces neuronal damage have not been established. Potentially ethanol may reduce normal neurotrophic influences necessary for neuronal survival, growth, and function. We hypothesized that chronic ethanol exposure might produce a decrease in the synthesis, availability, upregulation, delivery, and/or the biological activity of normally occurring neurotrophic factors, or may alter the capacity of target neurons to respond to these factors. The available evidence leading to this hypothesis and supporting data from our laboratory are discussed.
We report a family in which autosomal dominant congenital nystagmus cosegregates with a balanced 7;15 translocation. Ophthalmic investigation showed predominantly horizontal nystagmus with a small rotatory component and no significant loss of visual function. This finding suggests a possible localisation for autosomal dominant congenital nystagmus (McKusick 164100).
Explore the source record for details and available documents.
We investigated the single- and multiple dose pharmacokinetics of a new controlled-release formulation (Orfil retard enteric coated tablet) of valproic acid in comparison with those of the plain tablet as a reference. Twelve healthy volunteers were given each formulation of 300 mg in the single-dose study. In the steady-state multiple-dose study, twelve epileptic patients received 1200 mg/day of the reference drug (300 mg 9 AM, 300 mg 3 PM, 600 mg 9 PM) and the test formulation (600 mg 9 AM, 600 mg 9 PM) with at least one week interval in cross-over manner. The AUC values of the test controlled release formulation were 91.7% (95% confidence interval: 78.4-100.4%) of the reference drug in the single-dose study and 98.2% (95% confidence interval: 86.2%-109.9%) in the steady-state study. The AUC's of the two formulations were not significantly different by ANOVA test. The Cmax and Tmax values of the test formulation were significantly different from the values of the reference in single-(Tmax: 158.4%, Cmax: 52.5% of the reference) and multiple-dose study (Tmax: 153.5% of the reference). The MRT values of the test formulation were also significantly greater (129.4% of the reference) in the single-dose study. Regarding the controlled-release characteristics of the test formulation, fluctuation index and percentage fluctuation of the twice a day dosage regimen of the test formulation were comparable with those of the thrice a day dosage regimen of the conventional tablet. Area deviation was even smaller in the test regimen of the controlled release formulation.(ABSTRACT TRUNCATED AT 250 WORDS)
This article explores the question of whether different social control mechanisms contribute to social disorganization and consequent deviance. Two groups of Yup'ik Eskimo were compared on reported felonies and misdemeanors. One group belongs to a sovereignty movement called the "Yupi'it Nation." Some member villages in this group have abolished their own tribal courts. The other group has maintained relationships with the state of Alaska and relies on Western law enforcement to maintain social order. There are statistically significant differences in amounts of reported felonies and misdemeanors. This may be due to differential deviance, differential reporting, or a combination of both. Because of the political position of the sovereignty villages, however, it seems clear that they are using more traditional methods of dealing with disruptive behavior. Use of traditional social control may contribute to social cohesiveness, thereby reducing deviance. Differential Deviance and Social Control Mechanisms.
A rare case of double primary tumors of the liver is reported. A 69-year-old male presented with fever and right upper quadrant pain. On admission blood culture grew Salmonella group B. Laboratory data showed leucocytosis, mild elevation of aspartate aminotransferase, alanine aminotransferase and sugar, positive-HBsAg, and normal range CEA and AFP. Abdominal sonography disclosed a well demarcated solid mass and another cystic lesion with a ragged wall in the left lobe of liver. Abdominal CT revealed a mixed density solid mass in the medial segment, and laterally located cystic mass with internal septa. A preoperative diagnosis of double tumors of the left lobe of the liver was made and the patient underwent a left hepatic lobectomy. Hepatocellular carcinoma and cystadenocarcinoma were diagnosed by histopathological examination. The patient has been well without tumor recurrence after one and a half year's follow-up.
We describe a case of hypercalcemia without lytic bone lesions complicating myeloid blast crisis of chronic myeloid leukemia (CML). Serum levels of parathyroid hormone-related protein (PTHrP) were elevated during the initial hypercalcemic period and became undetectable during chemotherapy-induced chronic phase, only to become elevated again during subsequent recurrent blastic periods repeatedly associated with hypercalcemia. Previously reported cases of hypercalcemia complicating CML are reviewed. It is suggested that PTHrP was responsible for the hypercalcemia in this case and may be an important mediator of hypercalcemia in CML.
An adenosine deaminase (ADA) deficient patient with severe combined immunodeficiency (SCID) developed resistance to therapeutic injections of bovine ADA conjugated to polyethylene glycol (PEG-ADA). This 18-year-old girl was diagnosed as having partial ADA deficiency at age 7 years, and was started on bovine conjugated PEG-ADA at age 15 years. The weekly dose of 15 U/kg led to clinical improvement with resolution of sinusitis and bronchitis within 2 months and normalization of some T cell functions. After 5 months, however, she developed an inhibitory antibody to ADA, became refractory to treatment with PEG-ADA, and clinically and immunologically deteriorated. This antibody was successfully suppressed over a 4-month period with a combination of prednisone (2 mg/kg/day), intravenous immunoglobulin (2 g/kg/dose), and discontinuing the PEG-ADA injections for 7 weeks. The PEG-ADA injections were then restarted at a higher dose (20 U/kg/dose, twice a week). With the suppression of the inhibitory antibody, her clinical and immunologic status improved to previously achieved level. She has subsequently continued treatment for over 36 months, receiving a single weekly dose of PEG-ADA (20 U/kg/week) with sustained clinical and immunologic improvement, including weakly positive antigen-specific T cell proliferative responses to tetanus and Candida.
The effect of chronic ethanol treatment (CET) for 21-26 weeks on the neurotrophic activity contained in the rat hippocampus (HPC) was determined with a bioassay in cultures of dissociated dorsal root ganglion cells (DRG) obtained from E7-8 chick embryos. Extracts of the HPC from CET or pair-fed control rats were used as experimental media, and neuronal survival and neurite-outgrowth of DRG cultures were determined. Both neuronal survival (-25%) and neurite-outgrowth (-50%) were reduced in the presence of HPC extracts from CET rats relative to controls. These data suggest that CET reduces the neurotrophic content of the HPC which may result in damage to septohippocampal neurons.
IL-2 has three cysteine residues. The cysteines at positions 58 and 105 of active IL-2 form an intramolecular disulfide bond while that at position 125 remains as a free form. To evaluate the importance of correct disulfide bond, mutant proteins (muteins) that have triple and double substitutions of cysteines with alanines, namely A58/105/125 and A58/125, were made by polymerase chain reaction method respectively. Thymidine incorporation assay on CTLL-2 cells showed that although these two muteins were only 0.5-2.0% as potent as that of wild type IL-2, they were 50-200 fold more active than A58, a mutein that has substitution of cysteine at position 58 with alanine. Binding inhibition study showed that the relative affinity of muteins A58/125 and A58/105/125 for high affinity IL-2 receptors was 5-25 fold higher than that of A58. These results suggest that the dramatic decrease in the activity of mutein A58 may result from the formation of an incorrect disulfide bond between the cysteines at positions 105 and 125.
Internalization of IL-2 is important for its biological activities. The internalization of IL-2 was regulated by the duration of glutathione (GSH) treatment in CTLL-2 and CT-4R cells. Flow cytometric studies showed that the level of surface IL-2 receptors was not increased by GSH treatment. Northern blot analysis showed that the mRNA of IL-2Rp55 and IL-2Rp70, the two major components of the high-affinity IL-2 receptors, was increased 6 hr after GSH treatment. The appearance rate of membrane IL-2 receptors in GSH-treated cells was faster than that of the untreated cells. GSH also shortened the half-life (from 5 to less than or equal to 3 hr) and thus increased the turnover of the surface high-affinity IL-2 receptors. These results suggest that although GSH does not affect the level of surface IL-2 receptors, GSH may regulate the internalization of IL-2 by enhancing the synthesis and turnover of surface IL-2 receptors.
In germinal centers, B lymphocytes are intimately associated with follicular dendritic cells (FDCs). It has been hypothesized that FDCs are involved in the regulation of B-cell growth and differentiation through cell-cell interactions. In this study, highly enriched preparations of FDCs were isolated by cell sorting using the FDC restricted monoclonal antibody DRC-1. When irradiated FDCs were cultured with mitogen stimulated B cells, B cell 3H-TdR uptake was inhibited by up to 80%. This inhibitory effect was not seen when paraformaldehyde fixed FDCs were added to B-cell cultures, suggesting that the FDCs needed to be metabolically active. Moreover, supernatants from cultured FDCs were similarly able to inhibit B-cell proliferation. These results demonstrate that FDCs may downregulate the clonal expansion of B cells that occurs within lymphoid follicles as part of the normal physiologic immune response. Potentially, the loss of the inhibitory role of FDCs in vivo may be of importance in certain infectious and neoplastic processes in which germinal centers are affected.