To provide strategies for monitoring and treating severe lung involvement in Gaucher disease.
We reviewed the chart of a 5-year-old boy who developed rapidly progressive, severe infiltrative lung involvement of Gaucher disease (GD) and improved after allogeneic hematopoietic stem cell transplant (HSCT), along with other case studies reported before December 2019. He was diagnosed with GD (homozygous mutation at c.1448T>C, p.L483P), and started receiving enzyme replacement therapy (ERT) at 17months old. He developed respiratory distress symptoms after 45months of ERT; chest imaging reported diffuse interstitial infiltration of the bilateral lungs and consolidations at the right lungs. Allogeneic HSCT using cells from a matched unrelated donor was performed four months upon progressive respiratory symptoms.
His respiratory symptoms subsided in one month; chest imaging improvement, pulmonary function test improvement, and normalized activity of β-glucocerebrosidase were reported in three months.
This is the first report of a patient who received early and regular ERT but developed severe infiltrative lung involvement and recovered after allogeneic HSCT. https://www.selleckchem.com/products/pyrotinib.html Based on study results, we suggest regular chest imaging, even for asymptomatic patients. For patients with severe lung involvement, rapid deterioration, and unresponsive to higher ERT dosages, allogeneic HSCT should be considered.
This is the first report of a patient who received early and regular ERT but developed severe infiltrative lung involvement and recovered after allogeneic HSCT. Based on study results, we suggest regular chest imaging, even for asymptomatic patients. For patients with severe lung involvement, rapid deterioration, and unresponsive to higher ERT dosages, allogeneic HSCT should be considered.We report a rare presentation of Pott's disease caused by M. bovis, suggesting transmission from infected cattle, and only the second case described so far in scientific reports. Noteworthy of this case was that the strain was only isolated on Stonebrink medium, a sodium pyruvate-containing culture medium for the isolation of mycobacteria. This medium is frequently ignored in diagnostic laboratories and in the laboratory manuals of most international health organizations. In general laboratories use a culture medium that contains glycerol, a carbon substrate considered inhibitory for the growth of M. bovis. The use of glycerol-containing medium therefore likely contributes towards underestimating zoonotic tuberculosis. Our case suggests that, in order to improved surveillance efforts for zoonotic TB and increase the notification rate for M. bovis to human TB, the use of pyruvate-containing media should be promoted, particularly in developing countries with a high prevalence of bovine TB, but also through the World Health Organization' (WHO) End TB Strategy and the Roadmap for Zoonotic TB.Surface-enhanced fluorescence (SEF) is rapidly becoming one of the main spectroscopic techniques for the detection of a variety of biomolecules and biomarkers. The main reasons for this trend are the high sensitivity and selectivity, robustness, and speed of this analytical method. Each year, the number of applications that utilize this phenomenon increases and with each such work, the complexity and novelty of the used substrates, procedures, and analytes rises. To obtain a clearer view of this phenomenon and research area, we decided to combine 76 valuable research articles from a variety of different research groups into this mini-review. We present and describe these works concisely and clearly, with a particular interest in the quantitative parameters of the experiment. These sources are classified according to the nature of the analyte, on the contrary to most reviews, which sort them by substrate nature. This point of view gives us insight into the development of this research area and the consequent increase in the complexity of the analyte nature. Moreover, this type of sorting can show possible future routes for the expansion of this research area. Along with the analytes, we can also pay attention to the substrates used for each situation and how the development of substrates affects the direction of research and subsequently, the choice of an analyte. About 108 sources and several interesting trends in the SEF research area over the past 25 years are discussed in this mini-review.There is evidence of underperformance of the Global Health Indicators, particularly in the WHO Afro-region. Yet, quality, effective healthcare delivery, and access to information about best practice remains a challenge to nurses and midwives in the WHO Afro-region. For nurses and midwives to have the capacity to practice safely and competently they need to engage in mandatory Continuing Professional Development (CPD). However a composite picture is not available for future project planners, researchers, and policy developers. Published literature from the past five years and professional body webpages were searched. The results of shining a light on the WHO Afro-region member states' CPD status revealed strengths, weaknesses, opportunities, and threats. The strengths lay in the beginnings of mandatory CPD and annual licensure renewal, while the weaknesses revealed inequity of CPD distribution across the region. The opportunities showed international academic partnership with possibilities for further engagement, and the threats were evident in the health context of the Afro-region, the shortage of nurses and the lesser participation of nurses in CPD programs. The illumination of the CPD status in the Afro-region suggests that a revised CPD landscape is necessary to strengthen the relevance and response capacity of nurses and midwives, as key contributors towards the Global Health Indicators.
To quantify the effects of the systematic internal and external femoral component rotations and tibial slope on the medial and lateral tibiofemoral gaps in total knee endoprostheses.
Nineteen knee cadaver specimens with an intact ligament apparatus were fixed in a custom frame, facilitating physiological flexion motion. Virtual total knee arthroplasty (TKA) was performed on three-dimensional models obtained from computed tomographyscans (0° and 90° flexions) with systematically altered femur rotations and tibial slopes.
Both the femur rotation and the tibial slope influenced the medial and lateral tibiofemoral flexion gaps (
<0.001), and the effects differed between the medial and lateral sides (
<0.001). The medial tibiofemoral flexion gap increased by 2.90±0.34mm and decreased by 2.66±0.26mm for 7° external and internal femur component rotations, respectively (both with
<0.001). The lateral tibiofemoral flexion gap decreased by 3.11±0.31mm and increased by 3.29±0.33mm for 7° external and internal femur component rotations, respectively (both with
<0.
To provide strategies for monitoring and treating severe lung involvement in Gaucher disease.
We reviewed the chart of a 5-year-old boy who developed rapidly progressive, severe infiltrative lung involvement of Gaucher disease (GD) and improved after allogeneic hematopoietic stem cell transplant (HSCT), along with other case studies reported before December 2019. He was diagnosed with GD (homozygous mutation at c.1448T>C, p.L483P), and started receiving enzyme replacement therapy (ERT) at 17months old. He developed respiratory distress symptoms after 45months of ERT; chest imaging reported diffuse interstitial infiltration of the bilateral lungs and consolidations at the right lungs. Allogeneic HSCT using cells from a matched unrelated donor was performed four months upon progressive respiratory symptoms.
His respiratory symptoms subsided in one month; chest imaging improvement, pulmonary function test improvement, and normalized activity of β-glucocerebrosidase were reported in three months.
This is the first report of a patient who received early and regular ERT but developed severe infiltrative lung involvement and recovered after allogeneic HSCT. https://www.selleckchem.com/products/pyrotinib.html Based on study results, we suggest regular chest imaging, even for asymptomatic patients. For patients with severe lung involvement, rapid deterioration, and unresponsive to higher ERT dosages, allogeneic HSCT should be considered.
This is the first report of a patient who received early and regular ERT but developed severe infiltrative lung involvement and recovered after allogeneic HSCT. Based on study results, we suggest regular chest imaging, even for asymptomatic patients. For patients with severe lung involvement, rapid deterioration, and unresponsive to higher ERT dosages, allogeneic HSCT should be considered.We report a rare presentation of Pott's disease caused by M. bovis, suggesting transmission from infected cattle, and only the second case described so far in scientific reports. Noteworthy of this case was that the strain was only isolated on Stonebrink medium, a sodium pyruvate-containing culture medium for the isolation of mycobacteria. This medium is frequently ignored in diagnostic laboratories and in the laboratory manuals of most international health organizations. In general laboratories use a culture medium that contains glycerol, a carbon substrate considered inhibitory for the growth of M. bovis. The use of glycerol-containing medium therefore likely contributes towards underestimating zoonotic tuberculosis. Our case suggests that, in order to improved surveillance efforts for zoonotic TB and increase the notification rate for M. bovis to human TB, the use of pyruvate-containing media should be promoted, particularly in developing countries with a high prevalence of bovine TB, but also through the World Health Organization' (WHO) End TB Strategy and the Roadmap for Zoonotic TB.Surface-enhanced fluorescence (SEF) is rapidly becoming one of the main spectroscopic techniques for the detection of a variety of biomolecules and biomarkers. The main reasons for this trend are the high sensitivity and selectivity, robustness, and speed of this analytical method. Each year, the number of applications that utilize this phenomenon increases and with each such work, the complexity and novelty of the used substrates, procedures, and analytes rises. To obtain a clearer view of this phenomenon and research area, we decided to combine 76 valuable research articles from a variety of different research groups into this mini-review. We present and describe these works concisely and clearly, with a particular interest in the quantitative parameters of the experiment. These sources are classified according to the nature of the analyte, on the contrary to most reviews, which sort them by substrate nature. This point of view gives us insight into the development of this research area and the consequent increase in the complexity of the analyte nature. Moreover, this type of sorting can show possible future routes for the expansion of this research area. Along with the analytes, we can also pay attention to the substrates used for each situation and how the development of substrates affects the direction of research and subsequently, the choice of an analyte. About 108 sources and several interesting trends in the SEF research area over the past 25 years are discussed in this mini-review.There is evidence of underperformance of the Global Health Indicators, particularly in the WHO Afro-region. Yet, quality, effective healthcare delivery, and access to information about best practice remains a challenge to nurses and midwives in the WHO Afro-region. For nurses and midwives to have the capacity to practice safely and competently they need to engage in mandatory Continuing Professional Development (CPD). However a composite picture is not available for future project planners, researchers, and policy developers. Published literature from the past five years and professional body webpages were searched. The results of shining a light on the WHO Afro-region member states' CPD status revealed strengths, weaknesses, opportunities, and threats. The strengths lay in the beginnings of mandatory CPD and annual licensure renewal, while the weaknesses revealed inequity of CPD distribution across the region. The opportunities showed international academic partnership with possibilities for further engagement, and the threats were evident in the health context of the Afro-region, the shortage of nurses and the lesser participation of nurses in CPD programs. The illumination of the CPD status in the Afro-region suggests that a revised CPD landscape is necessary to strengthen the relevance and response capacity of nurses and midwives, as key contributors towards the Global Health Indicators.
To quantify the effects of the systematic internal and external femoral component rotations and tibial slope on the medial and lateral tibiofemoral gaps in total knee endoprostheses.
Nineteen knee cadaver specimens with an intact ligament apparatus were fixed in a custom frame, facilitating physiological flexion motion. Virtual total knee arthroplasty (TKA) was performed on three-dimensional models obtained from computed tomographyscans (0° and 90° flexions) with systematically altered femur rotations and tibial slopes.
Both the femur rotation and the tibial slope influenced the medial and lateral tibiofemoral flexion gaps (
<0.001), and the effects differed between the medial and lateral sides (
<0.001). The medial tibiofemoral flexion gap increased by 2.90±0.34mm and decreased by 2.66±0.26mm for 7° external and internal femur component rotations, respectively (both with
<0.001). The lateral tibiofemoral flexion gap decreased by 3.11±0.31mm and increased by 3.29±0.33mm for 7° external and internal femur component rotations, respectively (both with
<0.
0 Comments
0 Shares
16 Views
0 Reviews
