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To examine the possible role of transscleral outflow routes, enucleated human and porcine eyes underwent noncontact neodymium:yttrium aluminum garnet (Nd:YAG) laser cyclophotocoagulation 3 mm posterior to the limbus. Pars plana lesions were verified histologically. The eyes were perfused with saline solution at 50 mm Hg perfusion pressure, placing the tip of the needle into the hyaloid orbicular space. The outflow facility was 0.072 microliter/min/mm Hg in paired controls and 0.105 microliter/min/mm Hg in human lasered eyes, a difference of 31%. In porcine eyes the difference was 43%. Since concepts of aqueous production, impaired circulation, and inflammation do not apply to enucleated eyes, the increase may be related to pars plana transscleral flow facilitated by disruption of the neuroepithelial barrier.  相似文献   
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Teratomas are true neoplasms composed of tissues from all three germinal layers and may exhibit variable levels of maturity. They have an unknown origin and eccentric microscopic appearance. Teratomas arising from the oral cavity are rare in the newborn; only 22 cases have been reported in the literature. We describe a female neonate with an oral teratoma originating from the tongue that was successfully treated with surgery.  相似文献   
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BACKGROUND: The treatment of hepatitis C virus (HCV)-related cirrhosis is difficult due to high frequency of adverse effects. We retrospectively reviewed the case records of patients with HCV cirrhosis to evaluate the efficacy and tolerability of pegylated (peg) interferon and ribavirin treatment in these patients. METHODS: Medical records of 28 patients with HCV-related compensated cirrhosis were reviewed. The treatment protocol was a combination therapy of peg interferon alfa-2b (1 microg/Kg/week) plus oral ribavirin (10-12 mg/Kg/day). Primary endpoint was sustained virological response, with additional endpoints of drug tolerance, clinical or biochemical worsening and death. RESULTS: End-of-treatment virlogic response was seen in 24 of 28 patients (85%) and sustained virologic response in 15 of 28 (53%) patients. Biochemical end-of-treatment response and sustained response were seen in 20 and 16 patients (71% and 57%), respectively. Treatment had to be stopped in 3 patients due to decompensation of liver status in two and drug intolerance in one, while dose modification was required in two patients. CONCLUSIONS: Combination therapy with peg interferon plus ribavirin seems effective in patients with liver cirrhosis. High relapse rate, poor biochemical recovery and possibility of decompensation are issues that need to be kept in mind.  相似文献   
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Although Hedgehog (HH) signaling plays a critical role in patterning the ventral midbrain, its role in early midbrain specification is not known. We examined the midbrains of sonic hedgehog (Shh) and smoothened (Smo) mutant mice where HH signaling is respectively attenuated and eliminated. We show that some ventral (Evx1+) cell fates are specified in the Shh-/- mouse in a Ptc1- and Gli1-independent manner. HH-independent ventral midbrain induction was further confirmed by the presence of a Pax7-negative ventral midbrain territory in both Shh-/- and Smo-/- mice at and before embryonic day (E) 8.5. Midbrain signaling centers are severely disrupted in the Shh-/- mutant. Interestingly, dorsal markers are up-regulated (Wnt1, Gdf7, Pax7), down-regulated (Lfng), or otherwise altered (Zic1) in the Shh-/- midbrain. Together with the increased cell death seen specifically in Shh-/- dorsal midbrains (E8.5-E9), our results suggest specific regulation of dorsal patterning by SHH, rather than a simple deregulation due to its absence.  相似文献   
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Melanoma     
The incidence of melanoma in the US is rising at a rate second only to that of lung cancer in women. Early stage melanoma is curable, but once metastatic, it is almost uniformly fatal. The immunotherapy of melanoma is a new and exciting therapeutic modality that is being extensively investigated worldwide. Interferon-alpha has an approximately 16% response rate in metastatic melanoma. In the randomised trials to date, no combination of chemotherapeutic or hormonal agent with interferon-alpha has proven to be superior to dacarbazine, the reference agent for the treatment of metastatic melanoma. The role of interferon-alpha-2b in the adjuvant therapy of localised melanoma at high risk for relapse has recently been established, with the results of 2 large randomised trials conducted by the US Intergroup, one showing improvement in both relapse-free survival and overall survival, and the other in relapse-free survival only. Interferon-gamma has not been effective in the adjuvant setting or in metastatic disease, but is part of combination protocols used for regional therapy for extremity melanomas. Interleukin-2 has an overall response rate of 15 to 20% in metastatic melanoma and produces some complete and durable remissions. The US Food and Drug Administration has recently approved the use of high-dose bolus administration of recombinant interleukin-2 for the therapy of metastatic melanoma. Results of combination chemotherapy and immunotherapy regimens containing interleukin-2 (biochemotherapy) are promising, and ongoing research will determine whether a survival impact will be confirmed in randomised studies. Vaccine therapy is another exciting area of research, and clinical trials are ongoing in both metastatic melanoma and as adjuvant therapy. A bewildering array of vaccines (whole cell, carbohydrate and peptide) is available, and it remains to be seen which of these numerous preparations will be most effective. Adjuvant therapy trials with a ganglioside GM2 vaccine and others are ongoing. Numerous peptide vaccines are also being investigated for metastatic melanoma, singly and in combination with other immunotherapeutic agents.  相似文献   
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Genetic studies on closed populations benefit from comprehensive, searchable genealogy resources. To support studies of Anabaptists, a computerized database has been developed that merges two large genealogy books, the "Fisher Family History" and the "Amish and Amish Mennonite Genealogies." The former is more current but the latter is more comprehensive for the 18th and 19th centuries. Therefore, the merger of the two books is significantly more useful than either book alone. We demonstrate the utility of the merged database with two results: an increase in inbreeding coefficients and the identification of parent-child relationships that do not exist in either book. Am. J. Med. Genet. 86:156-161, 1999. Published 1999 Wiley-Liss, Inc.  相似文献   
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