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A patient with neutropenia and life-threatening infections secondary to T-γ lymphoproliferative disease, who did not respond to treatment with recombinant human G-CSF (filgrastim), was treated with filgrastim plus cyclosporine A (CyA). The patient achieved a good response in the absolute neutrophil count and subsequently required a dose reduction in the filgrastim. The patient was eventually discontinued from the CyA but continues on filgrastim alone. While on therapy, the large granular lymphocytes disappeared from the circulation and the beta-TCR rearrangement, which was present prior to beginning therapy, became undetectable. The patient had no significant toxicity to the CyA or the filgrastim and he has not experienced any serious infections or required hospitalization. Filgrastim has proven to be relatively nontoxic and of some benefit to patients with this disease and should probably be utilized first when treatment is necessary. However, if improvement is not observed, these findings suggest that a trial of the combination of CyA plus filgrastim may be beneficial.  相似文献   
3.
We describe 3 patients who had oral mucosal lesions with features of traumatic eosinophilic granuloma (TEG) and containing CD30+ atypical cells. In 1 patient, the oral lesion was followed by skin nodules. All lesions were evaluated histologically, by immunohistochemical analysis, and by polymerase chain reaction (PCR) analysis of the T-cell receptor (TCR) gamma chain gene. All oral lesions were characterized by a dense and deeply infiltrative lymphoproliferation, showing epitheliotropism and massive eosinophilia. They contained atypical large lymphoid cells, which expressed T-cell markers and CD30. PCR analysis showed a monoclonal rearrangement of the TCR gamma chain gene in all lesions and, in 1 patient, the same rearrangement in the oral and cutaneous specimens. The lesions in these patients seem to be the oral counterpart of the spectrum of primary cutaneous CD30+ lymphoproliferative disorders and should be recognized as such to avoid a diagnosis of large T-cell lymphoma and possible consequent overtreatment. However, they represent only a subset among several others within the complex and heterogeneous category of disorders referred to as TEG.  相似文献   
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Tumoral monoclonal immunoglobulin (Ig) light chain non-fibrillar deposits ('aggregomas'), which can be considered analogous to solitary light chain amyloidomas, are a rare presenting feature of B-cell dyscrasias. It is not certain if they are truly localized or if in reality they represent an initial expression of a silent systemic non-amyloid light chain deposition disease (LCDD). This report describes three patients, two of whom presented with cervical masses and the third with a solitary lung nodule, each comprising granular aggregates of monoclonal kappa light chain. Extracted deposits from the lymph node of one patient were shown by N-terminal amino acid sequence analysis to belong to the variable-region kappa I (Vkappa I) light chain subgroup, the first reported kappa-LCDD protein encoded by the L9 gene and the first report of an expressed protein related to this gene. Extracted deposits from the lung nodule of the second patient belonged to the Vkappa IV light chain subgroup encoded by the B3 germ line gene. The N-terminal amino acid sequences of the light chains from the aggregomas were compared with the related germ line sequences and to the N-terminal amino acid sequences of the nine other known kappa-LCDD light chains reported thus far from patients with systemic LCDD.  相似文献   
7.
Bromocriptine, a potent dopamine D2 receptor agonist, suppresses lipogenesis and improves glucose intolerance and insulin resistance. Recent evidence suggests that bromocriptine may produce these effects by altering central nervous system (CNS) regulation of metabolism. To determine whether or not the CNS plays a critical role in these bromocriptine-mediated effects on peripheral metabolism, we compared the metabolic responses to bromocriptine when administered peripherally versus centrally in naturally obese and glucose intolerant Syrian hamsters. Male hamsters (BW 194 +/- 5 g) were treated with bromocriptine or vehicle either intraperitoneally (i.p., 800 microgram/animal) or intracerebroventricularly (i.c.v., 1 microgram/animal) daily at 1 h after light onset for 14 days while held on 14-hour daily photoperiods. Glucose tolerance tests (GTTs, 3 g glucose/kg BW) were conducted after treatment. Compared to control animals, bromocriptine i.p. significantly reduced weight gain (11.7 vs. -2.4 g) and the areas under the glucose and insulin GTT curves by 29 and 48%, respectively. Similarly, compared with vehicle-treated controls, bromocriptine i.c.v. at 1 microgram/animal substantially reduced weight gain (8.7 vs. -6.3 g), the areas under the glucose and insulin GTT curves by 31 and 44% respectively, and the basal plasma insulin concentration by 41% (p < 0.05). Furthermore, both treatments significantly improved insulin-mediated suppression of hepatic glucose production during a hyperinsulinemic-euglycemic clamp. Thus, daily administration of bromocriptine at a very low dose i.c.v. replicates the metabolic effects of bromocriptine administered i.p. at a much higher dose. This finding demonstrates for the first time that the CNS is a critical target of bromocriptine's metabolic effects.  相似文献   
8.
This study compared neck range of movement recording using three different methods goniometers (EGM), inclinometers (INC) and a three-dimensional video analysis system (IMG) in simultaneous and synchronized data collection. Twelve females performed neck flexion-extension, lateral flexion, rotation and circumduction. The differences between EGM, INC, and IMG were calculated sample by sample. For flexion-extension movement, IMG underestimated the amplitude by 13%; moreover, EGM showed a crosstalk of about 20% for lateral flexion and rotation axes. In lateral flexion movement, all systems showed similar amplitude and the inter-system differences were moderate (4–7%). For rotation movement, EGM showed a high crosstalk (13%) for flexion-extension axis. During the circumduction movement, IMG underestimated the amplitude of flexion-extension movements by about 11%, and the inter-system differences were high (about 17%) except for INC–IMG regarding lateral flexion (7%) and EGM–INC regarding flexion-extension (10%). For application in workplace, INC presents good results compared to IMG and EGM though INC cannot record rotation. EGM should be improved in order to reduce its crosstalk errors and allow recording of the full neck range of movement. Due to non-optimal positioning of the cameras for recording flexion–extension, IMG underestimated the amplitude of these movements.  相似文献   
9.

Background :

Nowadays obesity is a chronic disease considered one of the greatest problems in public healthy. Showing to be effective in a short and long term, the bariatric surgery has emerged as an optional treatment for morbid obesity.

Aim:

Identify the profile of patients seeking bariatric surgery.

Methods:

Were interviewed 100 patients in preoperative nutritional monitoring of bariatric surgery. The study was conducted by applying a questionnaire prepared according to the research objectives.

Results:

From the individuals that were seeking bariatric surgery, 78% were female, 62% were married and 69% reported physical activity. The average age of those surveyed was 37±10.83 years and mean body mass index (BMI) was 43.51± 6.25 kg/m². The comorbidity more prevalent in this group was high blood pressure (51%). In previous treatments for weight reduction, 92% have already done hypocaloric diet followed by anorectic drug (83%). The success of these treatments was reported by 92% of patients; however, the weight lost was recovered in less than one year of 75%. Patients with diabetes mellitus and dyslipidemia had higher BMI values. The patients with comorbidities showed lower levels of BMI.

Conclusion:

The profile of patients who sought surgical treatment for their obesity were predominantly women with a family background of obesity and obesity-related comorbidities, especially hypertension and diabetes mellitus.  相似文献   
10.
 Autoimmune lymphoproliferative disease (ALD) is a rare familial disorder. Clinical and laboratory features of this disease include a generalized lymphadenopathy, splenomegaly, increased levels of circulating CD3+ with low levels of CD4+, CD8+ T-cells, and autoimmune phenomena, characteristics that the autoimmune lymphoproliferative syndrome (ALPS) have in common. Treatment usually consists of different supportive therapies. We report on the case of a young man affected by ALD who became resistant to steroids and was unresponsive to cyclosporine. Nevertheless, he was successfully treated with interferon (IFN)-α, resulting in a long-lasting, clinically complete remission. Received: 29 November 1999 / Accepted: 29 June 2000  相似文献   
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