P. Mogayzel | Johns Hopkins University (original) (raw)
Papers by P. Mogayzel
Pediatrics in review, May 1, 2014
Journal of Cystic Fibrosis, 2016
Bone disease, specifically low bone mineral density, is a common and undertreated complication th... more Bone disease, specifically low bone mineral density, is a common and undertreated complication that begins during childhood in patients with cystic fibrosis (CF). This case describes a male baseball player, aged 14 years, with undiagnosed CF who sustained a left midshaft femoral fracture while running toward base; 8 months later, he sustained a right midshaft femoral fracture under similar conditions. After the second fracture, further evaluation revealed low bone mineral density and CF. There is no previously published report of pathologic fractures occurring in the femoral shaft in an athlete with undiagnosed CF. Patients with CF have a higher fracture rate. Low-energy fractures of major bones in athletically active individuals should be viewed with suspicion for an underlying process.
European Respiratory Journal, 2013
Zhonghua er ke za zhi = Chinese journal of pediatrics, 2019
Objective: To assess the diagnostic value of sweat conductivity testing in Chinese children with ... more Objective: To assess the diagnostic value of sweat conductivity testing in Chinese children with cystic fibrosis (CF). Methods: This is a retrospective study. Sweat conductivity tests were conducted in 45 CF children (CF group) and 200 non-CF children (non-CF group) diagnosed with other chronic pulmonary diseases at the No. 2 Department of Respiratory Medicine, Beijing Children's Hospital from May 2014 to June 2018. Pearson's chi-square test was used to assess the differences between CF and non-CF groups. A receiver operating characteristic curve was constructed to calculate the best cut-off value to diagnose or rule out CF. The pulmonary function parameters (forced expiratory volume in the first second, forced vital capacity,forced expiratory flows at 75% of exhaled vital capacity) of CF children over 6 years old were analyzed. The relationship between sweat conductivity and pulmonary function was compared between the two groups (80-120mmol/L vs.>120mmol/L). Results: The...
Transplantation, 2002
Wolters Kluwer Health may email you for journal alerts and information, but is committed to maint... more Wolters Kluwer Health may email you for journal alerts and information, but is committed to maintaining your privacy and will not share your personal information without your express consent. For more information, please refer to our Privacy Policy. ... Skip Navigation Links Home > July 27, ...
Molecular & Cellular Proteomics, 2006
Atherosclerosis, 1988
In the present study the biosynthesis of glycosaminoglycans (GAGS) by neonatal rat aortic smooth ... more In the present study the biosynthesis of glycosaminoglycans (GAGS) by neonatal rat aortic smooth muscle cells in culture was studied. Heparan sulfate (HS) was the predominant GAG of the cell layer accounting for 32-49% of the total GAGS depending on the time in culture. The presence of low sulfated chondroitin sulfate (LSC) in aortic smooth muscle cell cultures is reported here for the first time. The effect of ascorbate on the synthesis and accumulation of these macromolecules resulted in a relative increase of C4S and DS in the cell layer. In contrast, the distribution of the GAGS which were secreted into the medium was not significantly effected by the addition of ascorbate. While HS was always found to be a minor component, the other GAGS were present in about equal concentrations. The total GAG accumulation in the medium was much greater (91-97s) than that of the cell layer (3-9s) indicating that the cells are synthesizing relatively large amounts of GAGS, although incorporation of these macromolecules into the extracellular matrix was consistently low.
American Journal of Transplantation, 2007
Lung transplantation in children poses distinctly different challenges from those seen in the adu... more Lung transplantation in children poses distinctly different challenges from those seen in the adult population. This consensus statement reviews the experience in the field of pediatric lung transplantation and highlights areas that deserve further investigation.
Journal of Cystic Fibrosis, 2021
A53. CYSTIC FIBROSIS: CLINICAL PEDIATRIC STUDIES, 2010
Clinical Pediatrics, 2010
Improvements in therapy have increased the median predicted age of survival in cystic fibrosis (C... more Improvements in therapy have increased the median predicted age of survival in cystic fibrosis (CF) to 37.4 years in 2007. The primary cause of mortality is progressive lung disease, which begins in infancy. Because early diagnosis of CF has been demonstrated to improve outcomes, the majority of states within the United States now universally screen newborns for CF. Nevertheless, screening is associated with false positives and false negatives and is not a definitive means of diagnosis.
American Journal of Respiratory and Critical Care Medicine, 2011
severe allergic asthma was undertaken. Following a rigorous evaluation process (Fig 1 in the orig... more severe allergic asthma was undertaken. Following a rigorous evaluation process (Fig 1 in the original article), 8 total studies were selected; primary outcomes evaluated were reduction of steroid use and asthma exacerbations. Secondary outcomes measured included asthma symptoms, health-related quality of life, adverse effects, lung function, and rescue medication needs. Following the steroid-reduction phase, subjects receiving O were more likely to be able to withdraw completely from corticosteroids compared with those randomized to placebo (relative risk [RR] ¼ 1.80; 95% confidence interval [CI], 1.42-2.28; P ¼ .00001). Asthma exacerbation rate per 100 patient-years was 37.6 in the O group and 69.9 in the placebo group (RR ¼ 0.57; 95% CI, 0.48-0.66 and number needed to treat for benefit ¼ 10; 95% CI, 7-13, respectively) (Fig 2 in the original article). With regard to safety, there was no difference in the number of subject withdrawals between the O group and placebo-treated subjects. No significant adverse effects were seen in subjects taking O in studies less than 1-year duration. Longer clinical studies are required for evaluation of long-term efficacy and safety of 0 in patients with moderate-to-severe asthma.
Hydroxyethylmethacrylate (HEMA) hydrogels have a peculiar crater-like topography which renders th... more Hydroxyethylmethacrylate (HEMA) hydrogels have a peculiar crater-like topography which renders them ideal for studying cell-to-substrate contact formation. Cultured rabbit aortic smooth muscle cells (SMC) were grown on collagen-HEMA hydrogels, and the ultrastructure of developing cell attachment sites was studied. By 3 hours after cell seeding, both the rounded and spreading SMC appeared anchored to the hydrogel via extra-cellular connective tissue-like material. The fully formed attachment site present at 5-8 day was characterized by large bundles of intracellular myofilaments inserting onto areas of increased electron density along the plasmalemmal membrane. Large amounts of extracellular connective tissue-like material also appeared attached to the areas of increased electron density. Fully formed cell substratum attachment sites were not observed when either elastin-HEMA or hydrogels polymerized in the absence of protein were employed. The growth and collagen synthesis by human ...
Pediatric Transplantation, 2001
This case report describes an atypical form of acute pulmonary allograft rejection that was refra... more This case report describes an atypical form of acute pulmonary allograft rejection that was refractory to conventional therapy. The rejection manifested primarily as interstitial lymphocytic infiltrates with little perivascular involvement. Despite aggressive therapy the patient died within 7 months of transplant. The timely recognition and treatment of unusual forms of allograft rejection is vital in the management of pulmonary transplant patients.
Journal of Cystic Fibrosis
Scanning electron microscopy, 1984
Hydroxyethylmethacrylate (HEMA) hydrogels have a peculiar crater-like topography which renders th... more Hydroxyethylmethacrylate (HEMA) hydrogels have a peculiar crater-like topography which renders them ideal for studying cell-to-substrate contact formation. Cultured rabbit aortic smooth muscle cells (SMC) were grown on collagen-HEMA hydrogels, and the ultrastructure of developing cell attachment sites was studied. By 3 hours after cell seeding, both the rounded and spreading SMC appeared anchored to the hydrogel via extra-cellular connective tissue-like material. The fully formed attachment site present at 5-8 day was characterized by large bundles of intracellular myofilaments inserting onto areas of increased electron density along the plasmalemmal membrane. Large amounts of extracellular connective tissue-like material also appeared attached to the areas of increased electron density. Fully formed cell substratum attachment sites were not observed when either elastin-HEMA or hydrogels polymerized in the absence of protein were employed. The growth and collagen synthesis by human ...
The Biochemical journal, Jan 15, 1989
We show here that cultured neonatal-rabbit aortic smooth-muscle cells produce and accumulate sign... more We show here that cultured neonatal-rabbit aortic smooth-muscle cells produce and accumulate significant amounts of insoluble elastin. When grown in the presence of ascorbic acid, the amount of insoluble elastin in these cultures decreases, whereas the accumulation of collagen increases. These changes have been attributed to increased hydroxylation of proline in elastin. The function of ascorbic acid in proline hydroxylation is thought to be that of a reductive cofactor that maintains the proper oxidation state of molecular iron in the enzyme complex. This study shows that both ascorbic and isoascorbic acids act similarly to modify the accumulation of elastin and collagen in culture. On the other hand, cultures grown in the presence of dithiothreitol, a reducing agent previously shown to act as a cofactor for prolyl hydroxylase, do not demonstrate altered elastin accumulation. These studies are consistent with the suggestion that there is a specific role for ascorbic acid in this ce...
The Journal of biological chemistry, Jan 5, 1989
Neonatal rat aortic smooth muscle cell cultures produce two major soluble elastin molecules terme... more Neonatal rat aortic smooth muscle cell cultures produce two major soluble elastin molecules termed protropoelastin (77 kDa) and tropoelastin (71 kDa). Cell layer extracts are protroproelastin-enriched, while protropoelastin, tropoelastin, and significant amounts of discrete elastin fragments (Mr of 66,000, 61,000, 56,000, and 45,000) are present in preparations from the medium of these cultures. To determine the role of the various elastin molecules in the metabolism of elastin in neonatal rat aortic smooth muscle cell cultures, the amino termini of these proteins were sequenced. All soluble elastin components present in the medium were purified as a single peak by high performance liquid chromatography; further separation of the components was achieved by polyacrylamide gel electrophoresis and electroblotting. The bands were excised and sequenced. The amino-terminal sequences of protropoelastin, tropoelastin, and the 66-kDa, 61-kDa, and 56-kDa fragments were identical: Gly-Gly-Val-...
Journal of Histochemistry & Cytochemistry, 1988
These studies demonstrate that the strong binding capacity of elastin for Congo red can be used t... more These studies demonstrate that the strong binding capacity of elastin for Congo red can be used to advantage in aortic smooth muscle cell cultures. A fibrous elastin network fluoresces when Congo red is added. Congo red does not alter accumulation of elastin or of total protein, even when the cells are grown in the presence of the dye for long periods of time, indicating that it is not toxic. Porcine pancreatic elastase was used to solubilize elastin in these cultures, to determine the molar ratio of Congo red to elastin, thus making it possible to estimate the amount of elastin solubilized when the cultures are injured. Congo red binding to elastin will be useful in studying elastin accumulation and/or degradation in vitro and in vivo.
Pediatrics in review, May 1, 2014
Journal of Cystic Fibrosis, 2016
Bone disease, specifically low bone mineral density, is a common and undertreated complication th... more Bone disease, specifically low bone mineral density, is a common and undertreated complication that begins during childhood in patients with cystic fibrosis (CF). This case describes a male baseball player, aged 14 years, with undiagnosed CF who sustained a left midshaft femoral fracture while running toward base; 8 months later, he sustained a right midshaft femoral fracture under similar conditions. After the second fracture, further evaluation revealed low bone mineral density and CF. There is no previously published report of pathologic fractures occurring in the femoral shaft in an athlete with undiagnosed CF. Patients with CF have a higher fracture rate. Low-energy fractures of major bones in athletically active individuals should be viewed with suspicion for an underlying process.
European Respiratory Journal, 2013
Zhonghua er ke za zhi = Chinese journal of pediatrics, 2019
Objective: To assess the diagnostic value of sweat conductivity testing in Chinese children with ... more Objective: To assess the diagnostic value of sweat conductivity testing in Chinese children with cystic fibrosis (CF). Methods: This is a retrospective study. Sweat conductivity tests were conducted in 45 CF children (CF group) and 200 non-CF children (non-CF group) diagnosed with other chronic pulmonary diseases at the No. 2 Department of Respiratory Medicine, Beijing Children's Hospital from May 2014 to June 2018. Pearson's chi-square test was used to assess the differences between CF and non-CF groups. A receiver operating characteristic curve was constructed to calculate the best cut-off value to diagnose or rule out CF. The pulmonary function parameters (forced expiratory volume in the first second, forced vital capacity,forced expiratory flows at 75% of exhaled vital capacity) of CF children over 6 years old were analyzed. The relationship between sweat conductivity and pulmonary function was compared between the two groups (80-120mmol/L vs.>120mmol/L). Results: The...
Transplantation, 2002
Wolters Kluwer Health may email you for journal alerts and information, but is committed to maint... more Wolters Kluwer Health may email you for journal alerts and information, but is committed to maintaining your privacy and will not share your personal information without your express consent. For more information, please refer to our Privacy Policy. ... Skip Navigation Links Home > July 27, ...
Molecular & Cellular Proteomics, 2006
Atherosclerosis, 1988
In the present study the biosynthesis of glycosaminoglycans (GAGS) by neonatal rat aortic smooth ... more In the present study the biosynthesis of glycosaminoglycans (GAGS) by neonatal rat aortic smooth muscle cells in culture was studied. Heparan sulfate (HS) was the predominant GAG of the cell layer accounting for 32-49% of the total GAGS depending on the time in culture. The presence of low sulfated chondroitin sulfate (LSC) in aortic smooth muscle cell cultures is reported here for the first time. The effect of ascorbate on the synthesis and accumulation of these macromolecules resulted in a relative increase of C4S and DS in the cell layer. In contrast, the distribution of the GAGS which were secreted into the medium was not significantly effected by the addition of ascorbate. While HS was always found to be a minor component, the other GAGS were present in about equal concentrations. The total GAG accumulation in the medium was much greater (91-97s) than that of the cell layer (3-9s) indicating that the cells are synthesizing relatively large amounts of GAGS, although incorporation of these macromolecules into the extracellular matrix was consistently low.
American Journal of Transplantation, 2007
Lung transplantation in children poses distinctly different challenges from those seen in the adu... more Lung transplantation in children poses distinctly different challenges from those seen in the adult population. This consensus statement reviews the experience in the field of pediatric lung transplantation and highlights areas that deserve further investigation.
Journal of Cystic Fibrosis, 2021
A53. CYSTIC FIBROSIS: CLINICAL PEDIATRIC STUDIES, 2010
Clinical Pediatrics, 2010
Improvements in therapy have increased the median predicted age of survival in cystic fibrosis (C... more Improvements in therapy have increased the median predicted age of survival in cystic fibrosis (CF) to 37.4 years in 2007. The primary cause of mortality is progressive lung disease, which begins in infancy. Because early diagnosis of CF has been demonstrated to improve outcomes, the majority of states within the United States now universally screen newborns for CF. Nevertheless, screening is associated with false positives and false negatives and is not a definitive means of diagnosis.
American Journal of Respiratory and Critical Care Medicine, 2011
severe allergic asthma was undertaken. Following a rigorous evaluation process (Fig 1 in the orig... more severe allergic asthma was undertaken. Following a rigorous evaluation process (Fig 1 in the original article), 8 total studies were selected; primary outcomes evaluated were reduction of steroid use and asthma exacerbations. Secondary outcomes measured included asthma symptoms, health-related quality of life, adverse effects, lung function, and rescue medication needs. Following the steroid-reduction phase, subjects receiving O were more likely to be able to withdraw completely from corticosteroids compared with those randomized to placebo (relative risk [RR] ¼ 1.80; 95% confidence interval [CI], 1.42-2.28; P ¼ .00001). Asthma exacerbation rate per 100 patient-years was 37.6 in the O group and 69.9 in the placebo group (RR ¼ 0.57; 95% CI, 0.48-0.66 and number needed to treat for benefit ¼ 10; 95% CI, 7-13, respectively) (Fig 2 in the original article). With regard to safety, there was no difference in the number of subject withdrawals between the O group and placebo-treated subjects. No significant adverse effects were seen in subjects taking O in studies less than 1-year duration. Longer clinical studies are required for evaluation of long-term efficacy and safety of 0 in patients with moderate-to-severe asthma.
Hydroxyethylmethacrylate (HEMA) hydrogels have a peculiar crater-like topography which renders th... more Hydroxyethylmethacrylate (HEMA) hydrogels have a peculiar crater-like topography which renders them ideal for studying cell-to-substrate contact formation. Cultured rabbit aortic smooth muscle cells (SMC) were grown on collagen-HEMA hydrogels, and the ultrastructure of developing cell attachment sites was studied. By 3 hours after cell seeding, both the rounded and spreading SMC appeared anchored to the hydrogel via extra-cellular connective tissue-like material. The fully formed attachment site present at 5-8 day was characterized by large bundles of intracellular myofilaments inserting onto areas of increased electron density along the plasmalemmal membrane. Large amounts of extracellular connective tissue-like material also appeared attached to the areas of increased electron density. Fully formed cell substratum attachment sites were not observed when either elastin-HEMA or hydrogels polymerized in the absence of protein were employed. The growth and collagen synthesis by human ...
Pediatric Transplantation, 2001
This case report describes an atypical form of acute pulmonary allograft rejection that was refra... more This case report describes an atypical form of acute pulmonary allograft rejection that was refractory to conventional therapy. The rejection manifested primarily as interstitial lymphocytic infiltrates with little perivascular involvement. Despite aggressive therapy the patient died within 7 months of transplant. The timely recognition and treatment of unusual forms of allograft rejection is vital in the management of pulmonary transplant patients.
Journal of Cystic Fibrosis
Scanning electron microscopy, 1984
Hydroxyethylmethacrylate (HEMA) hydrogels have a peculiar crater-like topography which renders th... more Hydroxyethylmethacrylate (HEMA) hydrogels have a peculiar crater-like topography which renders them ideal for studying cell-to-substrate contact formation. Cultured rabbit aortic smooth muscle cells (SMC) were grown on collagen-HEMA hydrogels, and the ultrastructure of developing cell attachment sites was studied. By 3 hours after cell seeding, both the rounded and spreading SMC appeared anchored to the hydrogel via extra-cellular connective tissue-like material. The fully formed attachment site present at 5-8 day was characterized by large bundles of intracellular myofilaments inserting onto areas of increased electron density along the plasmalemmal membrane. Large amounts of extracellular connective tissue-like material also appeared attached to the areas of increased electron density. Fully formed cell substratum attachment sites were not observed when either elastin-HEMA or hydrogels polymerized in the absence of protein were employed. The growth and collagen synthesis by human ...
The Biochemical journal, Jan 15, 1989
We show here that cultured neonatal-rabbit aortic smooth-muscle cells produce and accumulate sign... more We show here that cultured neonatal-rabbit aortic smooth-muscle cells produce and accumulate significant amounts of insoluble elastin. When grown in the presence of ascorbic acid, the amount of insoluble elastin in these cultures decreases, whereas the accumulation of collagen increases. These changes have been attributed to increased hydroxylation of proline in elastin. The function of ascorbic acid in proline hydroxylation is thought to be that of a reductive cofactor that maintains the proper oxidation state of molecular iron in the enzyme complex. This study shows that both ascorbic and isoascorbic acids act similarly to modify the accumulation of elastin and collagen in culture. On the other hand, cultures grown in the presence of dithiothreitol, a reducing agent previously shown to act as a cofactor for prolyl hydroxylase, do not demonstrate altered elastin accumulation. These studies are consistent with the suggestion that there is a specific role for ascorbic acid in this ce...
The Journal of biological chemistry, Jan 5, 1989
Neonatal rat aortic smooth muscle cell cultures produce two major soluble elastin molecules terme... more Neonatal rat aortic smooth muscle cell cultures produce two major soluble elastin molecules termed protropoelastin (77 kDa) and tropoelastin (71 kDa). Cell layer extracts are protroproelastin-enriched, while protropoelastin, tropoelastin, and significant amounts of discrete elastin fragments (Mr of 66,000, 61,000, 56,000, and 45,000) are present in preparations from the medium of these cultures. To determine the role of the various elastin molecules in the metabolism of elastin in neonatal rat aortic smooth muscle cell cultures, the amino termini of these proteins were sequenced. All soluble elastin components present in the medium were purified as a single peak by high performance liquid chromatography; further separation of the components was achieved by polyacrylamide gel electrophoresis and electroblotting. The bands were excised and sequenced. The amino-terminal sequences of protropoelastin, tropoelastin, and the 66-kDa, 61-kDa, and 56-kDa fragments were identical: Gly-Gly-Val-...
Journal of Histochemistry & Cytochemistry, 1988
These studies demonstrate that the strong binding capacity of elastin for Congo red can be used t... more These studies demonstrate that the strong binding capacity of elastin for Congo red can be used to advantage in aortic smooth muscle cell cultures. A fibrous elastin network fluoresces when Congo red is added. Congo red does not alter accumulation of elastin or of total protein, even when the cells are grown in the presence of the dye for long periods of time, indicating that it is not toxic. Porcine pancreatic elastase was used to solubilize elastin in these cultures, to determine the molar ratio of Congo red to elastin, thus making it possible to estimate the amount of elastin solubilized when the cultures are injured. Congo red binding to elastin will be useful in studying elastin accumulation and/or degradation in vitro and in vivo.