Medulloblastoma (MDB) is an aggressive tumor of Central Nervous System (CNS). Radiotherapy after radical surgery has an important role in treatment of standard and high risk patients and is followed by intensive chemo...Medulloblastoma (MDB) is an aggressive tumor of Central Nervous System (CNS). Radiotherapy after radical surgery has an important role in treatment of standard and high risk patients and is followed by intensive chemotherapy. To explore modifications of protein expression induced by in vivo radiotherapy proteomic analysis was performed on a case of Anaplastic MDB. 2D-gel electrophoresis and MALDI-TOF mass spectrometry detected qualitative differences of protein expression in Anaplastic MDB at diagnosis and in relapse after radiotherapy. Relevant proteomic data were confirmed by western blot and Real-Time PCR analysis, validating the presence of Sthatmin 1 (STMN1), Heat shock protein 60 (HSP60), HSP27 and Disulfide Isomerase (ER60) among the six proteins present in both samples. The most relevant modification induced by radiotherapy was a drastic reduction of the total number of proteins (60.6%) and the appearance of few new proteins. The modifications and the striking simplification of proteins expressed by the tumor after radiotherapy may allow to tailor subsequent chemotherapy on a rational basis. A proteomic guided chemotherapy may be of great benefit to patients.展开更多
Background/Purpose: Severe gastrointestinal dysmotility (GID) impairs patients’ quality of life and is almost uniformly fatal after complications of parenteral nutrition. Intestinal and multivisceral transplants have...Background/Purpose: Severe gastrointestinal dysmotility (GID) impairs patients’ quality of life and is almost uniformly fatal after complications of parenteral nutrition. Intestinal and multivisceral transplants have been used as alternative treatment of these disorders. We studied patients with GID treated with transplantation in our center, and reviewed their outcome to determine the therapeutic efficacy of multivisceral transplants. Methods: The transplant database was searched for patients with GID from 1994 to 2001. We excluded patients with Hirschsprung disease, scleroderma, and diabetic enteropathy. We reviewed explanted organs, histochemistry, and immunohistochemistry and classified cases by etiology. Results: We selected 12 children with GID from 124 patients transplanted. Nine presented before 1 year and 3 started with symptoms between 2 and 8 years. By combined clinical and histopathological features, 6 were classified as megacystis microcolon intestinal hypoperistalsis syndrome, 4 as chronic idiopathic intestinal pseudoobstruction, and 2 as intestinal neuronal dysplasias. Six patients died during the follow-up from 21 to 546 days after transplant. The Kaplan-Meier actuarial survival rates were 66.7% at 1 year and 50% at 3 years. Conclusions: Multivisceral transplantation is a valuable therapeutic alternative for children with severe GID who cannot be adequately managed with parenteral nutrition.展开更多
文摘Medulloblastoma (MDB) is an aggressive tumor of Central Nervous System (CNS). Radiotherapy after radical surgery has an important role in treatment of standard and high risk patients and is followed by intensive chemotherapy. To explore modifications of protein expression induced by in vivo radiotherapy proteomic analysis was performed on a case of Anaplastic MDB. 2D-gel electrophoresis and MALDI-TOF mass spectrometry detected qualitative differences of protein expression in Anaplastic MDB at diagnosis and in relapse after radiotherapy. Relevant proteomic data were confirmed by western blot and Real-Time PCR analysis, validating the presence of Sthatmin 1 (STMN1), Heat shock protein 60 (HSP60), HSP27 and Disulfide Isomerase (ER60) among the six proteins present in both samples. The most relevant modification induced by radiotherapy was a drastic reduction of the total number of proteins (60.6%) and the appearance of few new proteins. The modifications and the striking simplification of proteins expressed by the tumor after radiotherapy may allow to tailor subsequent chemotherapy on a rational basis. A proteomic guided chemotherapy may be of great benefit to patients.
文摘Background/Purpose: Severe gastrointestinal dysmotility (GID) impairs patients’ quality of life and is almost uniformly fatal after complications of parenteral nutrition. Intestinal and multivisceral transplants have been used as alternative treatment of these disorders. We studied patients with GID treated with transplantation in our center, and reviewed their outcome to determine the therapeutic efficacy of multivisceral transplants. Methods: The transplant database was searched for patients with GID from 1994 to 2001. We excluded patients with Hirschsprung disease, scleroderma, and diabetic enteropathy. We reviewed explanted organs, histochemistry, and immunohistochemistry and classified cases by etiology. Results: We selected 12 children with GID from 124 patients transplanted. Nine presented before 1 year and 3 started with symptoms between 2 and 8 years. By combined clinical and histopathological features, 6 were classified as megacystis microcolon intestinal hypoperistalsis syndrome, 4 as chronic idiopathic intestinal pseudoobstruction, and 2 as intestinal neuronal dysplasias. Six patients died during the follow-up from 21 to 546 days after transplant. The Kaplan-Meier actuarial survival rates were 66.7% at 1 year and 50% at 3 years. Conclusions: Multivisceral transplantation is a valuable therapeutic alternative for children with severe GID who cannot be adequately managed with parenteral nutrition.
