Introduction: Amyloidosis are systemic conditions and carpal tunnel syndrome (CTS) precedes the principal systemic complications and can be used as an early marker. Our objective was to determine the frequency of amyl...Introduction: Amyloidosis are systemic conditions and carpal tunnel syndrome (CTS) precedes the principal systemic complications and can be used as an early marker. Our objective was to determine the frequency of amyloid deposition in idiopathic CTS and its systemic impact. Methods: We retrospectively evaluated patients with CTS between September 2019 to January 2020. Samples from the anterior carpal ligament were pathologically evaluated and amyloid deposition was confirmed by apple-green birefringence on polarized light using Congo red stain. When amyloid was detected we performed genetic testing for transthyretin variants (ATTRv), immunofixation electrophoresis in serum and urine for light chains and multidisciplinary evaluation. Results: Thirty consecutive patients were included, 19 women, 11 men, mean age 70 years old (range 42 - 89 years). We identified 3 patients (10%) with amyloid deposits (mean age: 78.6 years, 2 men, 1 woman). Genetic testing for ATTRv and light chains studies were negative. During follow-up: The first patient required aortic valve replacement. The second patient developed progressive cardiac failure with syncopal episodes, atrioventricular block and atrial fibrillation and required a pacemaker and anticoagulation. The third patient had unexplained chronic edemas. The cardiac evaluation in all 3 patients revealed left ventricular hypertrophy and myocardial uptake (Perugini Score > 2) in their nuclear bone scintigraphies with technetium pyrophosphate. Two patients were treated with tafamidis and one patient died due to refractory cardiac insufficiency. Discussion: Our findings underline the importance of investigating amyloidosis in idiopathic CTS. The identification of deposits allows early diagnosis of cardiac amyloidosis leading to timely intervention and treatment.展开更多
Modification signs in extreme weather events may be directly related to alterations in the thermodynamic panorama of the atmosphere that need to be better understood. This study aimed to make a first interconnection b...Modification signs in extreme weather events may be directly related to alterations in the thermodynamic panorama of the atmosphere that need to be better understood. This study aimed to make a first interconnection between climate extremes and thermodynamic patterns in the city of Rio de Janeiro. Maximum and minimum air temperature and precipitation extreme indices from two surface meteorological stations (ABOV and STCZ) and instability indices based on temperature and humidity from radiosonde observations (SBGL) were employed to investigate changes in the periods 1964-1980 (P1), 1981-2000 (P2), and 2001-2020 (P3). Statistical tests were adopted to determine the significance and magnitude of trends. The frequency of warm (cold) days and warm (cold) nights are increasing (decreasing) in the city. Cold (Warm) extremes are changing with greater magnitude in ABOV (STCZ) than in STCZ (ABOV). In ABOV, there is a significant increase of +84 mm/decade in the rainfall volume associated with severe precipitation (above the 95<sup>th</sup> percentile) and most extreme precipitation indices show an increase in frequency and intensity. In STCZ, there is a decrease in extreme precipitation until the 1990s, and from there, an increase, showing a wetter climate in the most recent years. It is also verified in SBGL that there is a statistically significant increase (decrease) in air temperature of +0.1°C/decade (-0.2°C/decade) and relative humidity of +1.2%/decade (-3%/decade) at the low and middle (high) troposphere. There is a visible rising trend in most of the evaluated instability indices over the last few decades. The increasing trends of some extreme precipitation indices are probably allied to the precipitable water increasing trend of +1.2 mm/decade.展开更多
Sulfur mass-independent fractionation(S-MIF)preserved in Archean sedimentary pyrite is interpreted to reflect atmospheric chemistry.Small ranges in Δ^(33)S that expanded into larger fractionations leading up to the G...Sulfur mass-independent fractionation(S-MIF)preserved in Archean sedimentary pyrite is interpreted to reflect atmospheric chemistry.Small ranges in Δ^(33)S that expanded into larger fractionations leading up to the Great Oxygenation Event(GOE;2.45–2.2 Ga)are disproportionately represented by sequences from the Kaapvaal and Pilbara Cratons.These patterns of S-MIF attenuation and enhancement may differ from the timing and magnitude of minor sulfur isotope fractionations reported from other cratons,thus obscuring local for global sulfur cycling dynamics.By expanding the Δ^(33)S record to include the relatively underrepresented São Francisco Craton in Brazil,we suggest that marine biogeochemistry affected S-MIF preservation prior to the GOE.In an early Neoarchean sequence(2763–2730 Ma)from the Rio das Velhas Greenstone Belt,we propose that low δ^(13)Corg(<-30‰)and dampened Δ^(33)S(-0.4‰to-0.7‰)in banded iron formation reflect the marine diagenetic process of anaerobic methane oxidation.The overlying black shale(TOC up to 7.8%)with higher δ^(13)Corg(-33.4‰to-19.2‰)and expanded Δ^(33)S(2.3‰±0.8‰),recorded oxidative sulfur cycling that resulted in enhance preservation of S-MIF input from atmospheric sources of elemental sulfur.The sequence culminates in a metasandstone,where concomitant changes to more uniform δCorg(-30‰to--25‰),potentially associated with the RuBisCO I enzyme,and near-zero Δ^(33)S(-0.04‰to 0.38‰)is mainly interpreted as evidence for local oxygen production.When placed in the context of other sequences worldwide,the Rio das Velhas helps differentiate the influences of global atmospheric chemistry and local marine diagenesis in Archean biogeochemical processes.Our data suggest that prokaryotic sulfur,iron,and methane cycles might have an underestimated role in pre-GOE sulfur minor isotope records.展开更多
文摘Introduction: Amyloidosis are systemic conditions and carpal tunnel syndrome (CTS) precedes the principal systemic complications and can be used as an early marker. Our objective was to determine the frequency of amyloid deposition in idiopathic CTS and its systemic impact. Methods: We retrospectively evaluated patients with CTS between September 2019 to January 2020. Samples from the anterior carpal ligament were pathologically evaluated and amyloid deposition was confirmed by apple-green birefringence on polarized light using Congo red stain. When amyloid was detected we performed genetic testing for transthyretin variants (ATTRv), immunofixation electrophoresis in serum and urine for light chains and multidisciplinary evaluation. Results: Thirty consecutive patients were included, 19 women, 11 men, mean age 70 years old (range 42 - 89 years). We identified 3 patients (10%) with amyloid deposits (mean age: 78.6 years, 2 men, 1 woman). Genetic testing for ATTRv and light chains studies were negative. During follow-up: The first patient required aortic valve replacement. The second patient developed progressive cardiac failure with syncopal episodes, atrioventricular block and atrial fibrillation and required a pacemaker and anticoagulation. The third patient had unexplained chronic edemas. The cardiac evaluation in all 3 patients revealed left ventricular hypertrophy and myocardial uptake (Perugini Score > 2) in their nuclear bone scintigraphies with technetium pyrophosphate. Two patients were treated with tafamidis and one patient died due to refractory cardiac insufficiency. Discussion: Our findings underline the importance of investigating amyloidosis in idiopathic CTS. The identification of deposits allows early diagnosis of cardiac amyloidosis leading to timely intervention and treatment.
文摘Modification signs in extreme weather events may be directly related to alterations in the thermodynamic panorama of the atmosphere that need to be better understood. This study aimed to make a first interconnection between climate extremes and thermodynamic patterns in the city of Rio de Janeiro. Maximum and minimum air temperature and precipitation extreme indices from two surface meteorological stations (ABOV and STCZ) and instability indices based on temperature and humidity from radiosonde observations (SBGL) were employed to investigate changes in the periods 1964-1980 (P1), 1981-2000 (P2), and 2001-2020 (P3). Statistical tests were adopted to determine the significance and magnitude of trends. The frequency of warm (cold) days and warm (cold) nights are increasing (decreasing) in the city. Cold (Warm) extremes are changing with greater magnitude in ABOV (STCZ) than in STCZ (ABOV). In ABOV, there is a significant increase of +84 mm/decade in the rainfall volume associated with severe precipitation (above the 95<sup>th</sup> percentile) and most extreme precipitation indices show an increase in frequency and intensity. In STCZ, there is a decrease in extreme precipitation until the 1990s, and from there, an increase, showing a wetter climate in the most recent years. It is also verified in SBGL that there is a statistically significant increase (decrease) in air temperature of +0.1°C/decade (-0.2°C/decade) and relative humidity of +1.2%/decade (-3%/decade) at the low and middle (high) troposphere. There is a visible rising trend in most of the evaluated instability indices over the last few decades. The increasing trends of some extreme precipitation indices are probably allied to the precipitable water increasing trend of +1.2 mm/decade.
基金São Paulo Research Foundation(FAPESP grants2016/02933-2 to ABS,12/15824-6 to EPO and 2015/16235-2 to PP)the Agouron Institute(to WPG)for financial support。
文摘Sulfur mass-independent fractionation(S-MIF)preserved in Archean sedimentary pyrite is interpreted to reflect atmospheric chemistry.Small ranges in Δ^(33)S that expanded into larger fractionations leading up to the Great Oxygenation Event(GOE;2.45–2.2 Ga)are disproportionately represented by sequences from the Kaapvaal and Pilbara Cratons.These patterns of S-MIF attenuation and enhancement may differ from the timing and magnitude of minor sulfur isotope fractionations reported from other cratons,thus obscuring local for global sulfur cycling dynamics.By expanding the Δ^(33)S record to include the relatively underrepresented São Francisco Craton in Brazil,we suggest that marine biogeochemistry affected S-MIF preservation prior to the GOE.In an early Neoarchean sequence(2763–2730 Ma)from the Rio das Velhas Greenstone Belt,we propose that low δ^(13)Corg(<-30‰)and dampened Δ^(33)S(-0.4‰to-0.7‰)in banded iron formation reflect the marine diagenetic process of anaerobic methane oxidation.The overlying black shale(TOC up to 7.8%)with higher δ^(13)Corg(-33.4‰to-19.2‰)and expanded Δ^(33)S(2.3‰±0.8‰),recorded oxidative sulfur cycling that resulted in enhance preservation of S-MIF input from atmospheric sources of elemental sulfur.The sequence culminates in a metasandstone,where concomitant changes to more uniform δCorg(-30‰to--25‰),potentially associated with the RuBisCO I enzyme,and near-zero Δ^(33)S(-0.04‰to 0.38‰)is mainly interpreted as evidence for local oxygen production.When placed in the context of other sequences worldwide,the Rio das Velhas helps differentiate the influences of global atmospheric chemistry and local marine diagenesis in Archean biogeochemical processes.Our data suggest that prokaryotic sulfur,iron,and methane cycles might have an underestimated role in pre-GOE sulfur minor isotope records.
