Background:Chronic progressive external ophthalmoplegia(CPEO)is a mitochondrial encephalomyopathy caused by multiple mtDNA abnormalities.There is little information about the changes of ocular fundus with CPEO.The aim...Background:Chronic progressive external ophthalmoplegia(CPEO)is a mitochondrial encephalomyopathy caused by multiple mtDNA abnormalities.There is little information about the changes of ocular fundus with CPEO.The aim of this work was to measure and evaluate changes in the macular retinal thickness and optic nerve head in patients with CPEO using spectral-domain optical coherence tomography and to compare the findings with those of healthy individuals.Methods:Totally,18 CPEO patients were enrolled in this study.Healthy volunteers matched for gender,age,and diopter settings were included as a control group.The retinal thickness of macular central fovea,inner and outer retinal layer thickness of perifoveal macular,optic nerve head parameters,and peripapillay retinal nerve fiber layer thickness(pRNFLT)for all included cases were measured using spectral-domain optical coherence tomography.A paired t test was used to compare the differences in the studied parameters between the two groups.The correlations between macular retinal thickness,pRNFLT,disease duration,and age of onset were also analyzed.Results:Among the macular parameters,retinal thickness of macular central fovea(t=—2.135,P<0.05)and outer retinal layer thickness(t=—1.994,P<0.05)of patients in the CPEO group were statistically significant lower than those of patients in the normal control group.For the optic nerve head parameters,the patients in the CPEO group showed a larger rim volume(t=—2.499,P<0.05)and nerve head volume(t=—2.103,P<0.05).The overall pRNFLT of patients in the CPEO group was statistically significant lower than that of patients in the control group(t=—4.125,P<0.05).The comparison of pRNFLT in eight sectors showed that the pRNFLT of patients in the CPEO group was statistically significant lower than that of the control group mainly in the inferior and temporal sectors.The degree of pRNFL defect negatively correlated with the disease duration(r=—0.583,P<0.05).Conclusions:The retinal thickness of patients with CPEO was significantly thinner,which was mostly the outer retina.The patients,optic discs had a low volume and the loss of the retinal nerve fiber layer was obvious.With the extension of the disease duration,the retinal nerve fiber layer defect was even more significant.展开更多
Background: Central neurological involvement is the most frequent extra hematological manifestation of chronic lymphocytic leukemia;it is multifactorial and rarely due to a cerebral localization of the disease. We rep...Background: Central neurological involvement is the most frequent extra hematological manifestation of chronic lymphocytic leukemia;it is multifactorial and rarely due to a cerebral localization of the disease. We report a case of cerebral localization of chronic lymphoid leukemia whose clinical and radiological aspects were very suggestive of progressive multifocal leukoencephalopathy. Case Presentation: A 65-year-old patient who was HIV-negative (human immunodeficiency virus), had consulted for bilateral axillary, cervical and inguinal lymphadenopathy associated with major asthenia and hyper lymphocytosis (lymphocyte count was 11 giga/l). Chronic lymphocyticleukemia with TP53 mutation was diagnosed and treatment with Ibrutinib 420 mg/day was initiated. After 2 months of treatment, the evolution was marked by the onset of neurological disorders whose clinical-radiological presentation and temporal evolution had led to the diagnosis of progressive multifocal leukoencephalopathy. In the absence of virological evidence in the cerebrospinal fluid analysis, a stereotactic biopsy of the brain lesions had been performed, making it possible to formally rule out this infectious hypothesis and to demonstrate cerebral invasion by tumour cells. Immuno-chemotherapy combining Rituximab-Cyclophosphamide-Doxorubicin-Vincristine-Prednisone-Ibrutinib (RCHOP-Ibrutinib) with intrathecal chemotherapy resulted in a very good clinical-radiological response. Conclusion: The appearance of neurological manifestations in the context of chronic lymphocytic leukemia must systematically lead to a search for a cerebral localization of the disease. In the absence of virological evidence in the cerebrospinal fluid, any suspicion of progressive multifocal leukoencephalopathy in this context should lead to the histological study of brain lesions.展开更多
A more than 5 year follow up study about 48 cases of chronic atrophic gastritis(CAG) and 100 cases of chronic superficial gastritis (CSG) is reported in CAG, it is found that 35.5% of the patients were improved, 41....A more than 5 year follow up study about 48 cases of chronic atrophic gastritis(CAG) and 100 cases of chronic superficial gastritis (CSG) is reported in CAG, it is found that 35.5% of the patients were improved, 41.6% stabilized, 12.5% deteriorated, 6.2% recovered, and 4.2% cancerated. In CSG, it is found that 40% of the patients were improved, 48% stabilized, 6% deteriorated, 5% recovered and 1% cancerated. It is concluded that CAG group has a higher rate of cancerization than that of CSG group.展开更多
Background: There are many programs which focus on late-stage chronic kidney disease (CKD), and it is considered that further evidence needs to be generated regarding the effectiveness of the programs used before rena...Background: There are many programs which focus on late-stage chronic kidney disease (CKD), and it is considered that further evidence needs to be generated regarding the effectiveness of the programs used before renal replacement therapy. Study Design: A cohort study. Settings & Participants: Patients over 15 years of age who had been diagnosed with CKD according to the KDOQI (Kidney Disease Outcomes Quality Initiative) guidelines and who had undergone conventional treatment (CT) or a renal protection program (RPP). These were patients of two Colombian health insurance companies. Predictors: Age, sex, marital status, comorbidities, CKD stage, and clinical indicators. Outcomes: First CKD progression, and need for renal replacement therapy (RRT). Measures: Clinical marker. Results: The RPP is structurally and functionally different from the CT. It offers the interdisciplinary management of patients, a greater number of medical appointments, and patients start to receive treatment at younger ages and at earlier stages of their condition. The clinical markers of the patients following the RPP are within adequate ranges, and their renal function is less impaired, despite the differences in basal conditions. Upon finishing the study, we found that patients who received CT had a higher risk of receiving nephrotoxic drugs and not receiving nephroprotective drugs. The explanatory variables for the first progression were age, stage, history of dyslipidemia, and hemoglobin, potassium, and albumin levels. These variables, together with glycemia levels were also valid for RRT, except for history of dyslipidemia, as it was not significant. Upon adjusting for the explanatory variables, it was found that belonging to the RPP and attending more appointments had a protective effect in the process of controlling renal damage. Limitations: A possible selection bias. Conclusions: Belonging to a structured renal protection program is an effective way to keeping the clinical markers associated with renal impairment within normal ranges.展开更多
基金supported by a grant of Beijing Municipal Science and Technology Commission(No.Z151100003915126).
文摘Background:Chronic progressive external ophthalmoplegia(CPEO)is a mitochondrial encephalomyopathy caused by multiple mtDNA abnormalities.There is little information about the changes of ocular fundus with CPEO.The aim of this work was to measure and evaluate changes in the macular retinal thickness and optic nerve head in patients with CPEO using spectral-domain optical coherence tomography and to compare the findings with those of healthy individuals.Methods:Totally,18 CPEO patients were enrolled in this study.Healthy volunteers matched for gender,age,and diopter settings were included as a control group.The retinal thickness of macular central fovea,inner and outer retinal layer thickness of perifoveal macular,optic nerve head parameters,and peripapillay retinal nerve fiber layer thickness(pRNFLT)for all included cases were measured using spectral-domain optical coherence tomography.A paired t test was used to compare the differences in the studied parameters between the two groups.The correlations between macular retinal thickness,pRNFLT,disease duration,and age of onset were also analyzed.Results:Among the macular parameters,retinal thickness of macular central fovea(t=—2.135,P<0.05)and outer retinal layer thickness(t=—1.994,P<0.05)of patients in the CPEO group were statistically significant lower than those of patients in the normal control group.For the optic nerve head parameters,the patients in the CPEO group showed a larger rim volume(t=—2.499,P<0.05)and nerve head volume(t=—2.103,P<0.05).The overall pRNFLT of patients in the CPEO group was statistically significant lower than that of patients in the control group(t=—4.125,P<0.05).The comparison of pRNFLT in eight sectors showed that the pRNFLT of patients in the CPEO group was statistically significant lower than that of the control group mainly in the inferior and temporal sectors.The degree of pRNFL defect negatively correlated with the disease duration(r=—0.583,P<0.05).Conclusions:The retinal thickness of patients with CPEO was significantly thinner,which was mostly the outer retina.The patients,optic discs had a low volume and the loss of the retinal nerve fiber layer was obvious.With the extension of the disease duration,the retinal nerve fiber layer defect was even more significant.
文摘Background: Central neurological involvement is the most frequent extra hematological manifestation of chronic lymphocytic leukemia;it is multifactorial and rarely due to a cerebral localization of the disease. We report a case of cerebral localization of chronic lymphoid leukemia whose clinical and radiological aspects were very suggestive of progressive multifocal leukoencephalopathy. Case Presentation: A 65-year-old patient who was HIV-negative (human immunodeficiency virus), had consulted for bilateral axillary, cervical and inguinal lymphadenopathy associated with major asthenia and hyper lymphocytosis (lymphocyte count was 11 giga/l). Chronic lymphocyticleukemia with TP53 mutation was diagnosed and treatment with Ibrutinib 420 mg/day was initiated. After 2 months of treatment, the evolution was marked by the onset of neurological disorders whose clinical-radiological presentation and temporal evolution had led to the diagnosis of progressive multifocal leukoencephalopathy. In the absence of virological evidence in the cerebrospinal fluid analysis, a stereotactic biopsy of the brain lesions had been performed, making it possible to formally rule out this infectious hypothesis and to demonstrate cerebral invasion by tumour cells. Immuno-chemotherapy combining Rituximab-Cyclophosphamide-Doxorubicin-Vincristine-Prednisone-Ibrutinib (RCHOP-Ibrutinib) with intrathecal chemotherapy resulted in a very good clinical-radiological response. Conclusion: The appearance of neurological manifestations in the context of chronic lymphocytic leukemia must systematically lead to a search for a cerebral localization of the disease. In the absence of virological evidence in the cerebrospinal fluid, any suspicion of progressive multifocal leukoencephalopathy in this context should lead to the histological study of brain lesions.
文摘A more than 5 year follow up study about 48 cases of chronic atrophic gastritis(CAG) and 100 cases of chronic superficial gastritis (CSG) is reported in CAG, it is found that 35.5% of the patients were improved, 41.6% stabilized, 12.5% deteriorated, 6.2% recovered, and 4.2% cancerated. In CSG, it is found that 40% of the patients were improved, 48% stabilized, 6% deteriorated, 5% recovered and 1% cancerated. It is concluded that CAG group has a higher rate of cancerization than that of CSG group.
文摘Background: There are many programs which focus on late-stage chronic kidney disease (CKD), and it is considered that further evidence needs to be generated regarding the effectiveness of the programs used before renal replacement therapy. Study Design: A cohort study. Settings & Participants: Patients over 15 years of age who had been diagnosed with CKD according to the KDOQI (Kidney Disease Outcomes Quality Initiative) guidelines and who had undergone conventional treatment (CT) or a renal protection program (RPP). These were patients of two Colombian health insurance companies. Predictors: Age, sex, marital status, comorbidities, CKD stage, and clinical indicators. Outcomes: First CKD progression, and need for renal replacement therapy (RRT). Measures: Clinical marker. Results: The RPP is structurally and functionally different from the CT. It offers the interdisciplinary management of patients, a greater number of medical appointments, and patients start to receive treatment at younger ages and at earlier stages of their condition. The clinical markers of the patients following the RPP are within adequate ranges, and their renal function is less impaired, despite the differences in basal conditions. Upon finishing the study, we found that patients who received CT had a higher risk of receiving nephrotoxic drugs and not receiving nephroprotective drugs. The explanatory variables for the first progression were age, stage, history of dyslipidemia, and hemoglobin, potassium, and albumin levels. These variables, together with glycemia levels were also valid for RRT, except for history of dyslipidemia, as it was not significant. Upon adjusting for the explanatory variables, it was found that belonging to the RPP and attending more appointments had a protective effect in the process of controlling renal damage. Limitations: A possible selection bias. Conclusions: Belonging to a structured renal protection program is an effective way to keeping the clinical markers associated with renal impairment within normal ranges.
