Clinical, radiologic and pathologic characteristics of the Carney triad: a case report and literature review.

This report describes a patient with a gastrointestinal stromal tumor (GIST) and upper gastrointestinal hemorrhage as initial presentations of the Carney triad, a synchronous or metachronous condition involving gastric leiomyosarcoma, extra-adrenal paraganglioma and pulmonary chondroma. A 26-year-old woman presented with dizziness and several episodes of melena over 3 days. Physical examination revealed pale conjunctiva but normal hemodynamic status. Gastroendoscopy showed one antral submucosal tumor with evidence of an associated bleeder (the bleeder over the mucosal surface of the gastric tumor). An abdominal computed tomography scan showed an antral submucosal tumor, two secondary lesions over segment II/V of the liver and a retroperitoneal tumor. Surgical removal of the tumor and subsequent recovery were uneventful. The gastric GIST with liver metastasis and retroperitoneal paraganglioma were confirmed by pathologic study. The patient was treated with imatinib mesylate for the GIST with liver metastasis, and continued follow-up treatment at our hospital. An abdominal computed tomography scan at 32 months after surgery showed no change in the liver metastatic lesions and no evidence of local recurrence. Another follow-up visit at 33 months after surgery confirmed the stable condition. Of nearly 100 cases reported in the literature, this case is the first to be reported in Taiwan. This case highlights the possibility of this rare syndrome occurring in young female patients with one of the three components of the triad and the need for further diagnostic studies for early identification of tumors when curative surgery is still possible.

Inhibition of nuclear factor kappaB is associated with neuroprotective effects of glycyrrhizic acid on glutamate-induced excitotoxicity in primary neurons.

Glycyrrhizic acid is an herbal drug with a broad spectrum of antiviral activities and pharmacological effects and multiple sites of action. We investigated whether glycyrrhizic acid protects against glutamate-induced excitotoxicity and the underlying mechanisms. We found that glycyrrhizic acid protected against neurotoxicity in rat primary neuronal cultures and hippocampal slices by suppression of the glutamate-induced apoptosis. Glycyrrhizic acid conferred neuroprotective properties in a concentration-dependent manner, as determined by cell survival, apoptosis, and Ca(2+) influx. Glycyrrhizic acid selectively inhibited the Ca(2+) influx activated through N-methyl-D-aspartate (NMDA) receptor by glutamate, but not through membrane depolarization elicited by high K(+) induction. Glycyrrhizic acid treatment also diminished glutamate-induced DNA fragmentation and cleavage of poly (ADP-ribose) polymerase (PARP). Electrophoretic mobility shift assay (EMSA) indicated that glycyrrhizic acid inhibited the binding activity of nuclear factor kappaB (NF-kappaB) to its target elements. Western blot analysis of NF-kappaB inhibitor (IkappaBalpha) protein revealed that the inhibitory effect of glycyrrhizic acid on glutamate-induced activation of NF-kappaB activity was attributable to the inhibition of IkappaB kinase activity. Thus, the site of action of glycyrrhizic acid could be a downstream consequence of Ca(2+)entry through NMDA receptors and that NF-kappaB may be one downstream target in this process. These observations suggest that glycyrrhizic acid may be of therapeutic value for the prevention of cerebral damage elicited by the glutamate.

Functional assays of HLA A2-restricted epitope variant of latent membrane protein 1 (LMP-1) of Epstein-Barr virus in nasopharyngeal carcinoma of Southern China and Taiwan.

Human leukocyte antigen (HLA) A2 was consistently associated with increased risk for nasopharyngeal carcinoma (NPC) in Chinese populations. Previously we have reported that an Epstein-Barr virus (EBV) strain carrying an HLA A2-restricted epitope variant of LMP-1 is prevalent in NPC in southern China and Taiwan (Lin et al., J. Gen. Virol. 85: 2023-2034, 2004). The variant has mutation selectively involved one of the two anchor residues in position 2 (125 L-->F) and an additional mutation in position 5 (129 M-->I). Functional assays of the epitope variant were carried out in the present work. The stabilization assay on T2 cells indicated that the variant peptide YFL (YFLEILWRL) prevalent in NPC binds to HLA A2 molecules less efficiently than the prototype peptide YLL (YLLEMLWRL). A dose-dependent binding of the HLA A2 molecules with added peptides was observed. In ex vivo cytotoxic T lymphocyte (CTL) assays with CD8-enriched effectors from A2-positive donors revealed that the YLL-specific CTL was able to lyse EBV-infected B cells expressing HLA A2, whereas the CTL recognition was abrogated with the peptide YFL. Cytokine (IFN-gamma) responses, measured both by intracytoplasmic staining and ELISPOT assays after peptide stimulation, also indicated that the variant epitope peptide failed to give an IFN-gamma response. The IFN-gamma response was almost entirely restricted to those tetramer-positive cells. These results show that EBV isolates from NPC of southern China and Taiwan is dominated by an HLA A2-restricted 'epitope-loss variants' of LMP-1, which would allow the virus to resist immune recognition and may in part contribute to the prevalence of NPC in these populations.

Effect and correlation of serum total IgE, eosinophil granule cationic proteins and sensitized allergens in atopic dermatitis patients with or without rhinitis.

To understand the relevance of serological parameters and eliciting allergens in the patients with atopic dermatitis (AD) and patients with atopic dermatitis in combination with rhinitis (ADR), we compared the serum total IgE, major basic protein (MBP) and eosinophil cationic protein (ECP) levels and identified the sensitized allergens in these two groups. The serum levels of total IgE, MBP and ECP were not significantly different in these two groups. Patients with ADR had a higher positive rate of aeroallergens including housedust, mite (D.F), mite (D.P) and cockroach than patients with AD. However, a high positive rate of seafood allergens (include crab, shellfish and shrimp) was observed in patients with AD. The serum IgE level correlated well with serum MBP level (r = 0.603, P = 0. 019) in patients with ADR. There was a strong correlation between serum MBP and ECP level (r = 0.773, P = 0.005) in patients with AD and in patients with ADR (r = 0.721, P = 0.008). We also found that numbers of sensitized allergens correlated well with total IgE in patients with AD (r = 0.760, P = 0.001) and in patients with ADR (r = 0.487, P = 0.004). These results suggested that the aeroallergens are the most important allergens causing and aggravating atopic diseases in Taiwan. Seafood may play an important role in the pathogenesis of AD. We suggest the measurement of serum total IgE combined with Multiple Allergens Simultaneous Test-Chemiluminescent Assays(AST-CLA) test could be helpful in the diagnosis of atopic diseases.

Langerhans cell histiocytosis of bone--a case report.

Langerhans cell histiocytosis of bone causes bone destruction and frequent bone reactions that mimic benign and malignant neoplasms as well as osteomyelitis. The bony lesion of Langerhans cell histiocytosis consists of histiocytes with variable numbers of eosinophils, lymphocytes, and neutrophils. We present a rare case of Langerhans cell histiocytosis of bone, including imaging studies and pathology. In addition, we discuss the classification and pathology of Langerhans cell histiocytosis.

Is a one-step operation for breast cancer patients presenting nipple discharge without palpable mass feasible?

Although spontaneous nipple discharge without mass is a common complaint, only about 5-15% of patients with nipple discharge have cancer. The standard preoperative evaluations, including mammography, physical examination, ultrasonography and squeezing cytology, do not provide enough definite data to make a decision for surgery. We have investigated whether preoperative intraductal aspiration cytology and galactography supply sufficient information about the location and nature of the lesion, and have carried out a one-step operative procedure by adding frozen section diagnosis during surgery. A retrospective review of Kaohsiung Medical University Hospital patients presenting symptoms of nonpalpable mass was conducted from January 1989 to June 2000. The medical charts, pathology and cytology reports, and imaging studies were reviewed. Of 487 patients with spontaneous nipple discharge, 190 with pathologic discharge had complete preoperative galactography and intraductal aspiration cytology, and 176 received surgery. Fourteen cases with negative cytology and normal galactography, who had not had an operation, did not develop cancer during an average 7 years follow-up. The diagnostic accuracy rates of 35 cancer patients using galactography and cytology were 91.4% and 88.6%, respectively, and 97.1% in combination, which is better than those of 141 patients with benign lesions (77.3% and 84.4%, and 90.0% in combination, P<0.05). The results show a 91.5% preoperative diagnostic accuracy rate in all patients with nipple discharge, and can be used to discuss the diagnosis with the patient during the preoperative period. All 35 cases with cancer received the one-step procedure under general anesthesia. Sixteen patients received mastectomies, and the other 19 cases had ductolobular resections, depending on their preoperative evaluations and frozen section in pathology. There were no false positives or false negatives in frozen section when comparing permanent histology and residual cancer in mastectomy specimens. There were no symptoms of recurrence in any of the patients who had undergone the two different procedures during 7 years of follow-up. In patients with spontaneous nipple discharge without palpable mass, the preoperative intraductal cytology and galactography were reliable methods to evaluate intraductal lesions. The one-step procedure will be selected for those patients if the frozen section is added. The ductolobular resection with an adequate surgical margin should be the first choice for those nonpalpable breast cancers with nipple discharge. Due to the limited number of breast cancer cases studied, more cases and a long follow-up period are necessary in future.

Vírus da hepatite G / Hepatitis G virus

O vírus da Hepatite G (HGV) foi recentemente reconhecido como agente transmissível que afeta o homem. OHGV foi detectado no sangue com a presença ou não de “marcadores” dos vírus da hepatite C e da Hapatite B. Diferentemente destes dois últimos, a presença do HGV no ser humano não parece estar associada a seqüelas clínicas típicas e pode ser benigno. Também não leva à produção de anticorpos que serviriam como base para t estes sorológicos. Atualmente, a detecção do HGV se faz mediante o PCR.