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Curtis R. Morris - One of the best experts on this subject based on the ideXlab platform.
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Chloride-sensitive renal microangiopathy in the stroke-prone spontaneously hypertensive rat.
Kidney International, 2001Co-Authors: Masae Tanaka, Olga Schmidlin, Jean L. Olson, Curtis R. MorrisAbstract:Chloride-sensitive renal microangiopathy in the stroke-prone spontaneously hypertensive rat. Background In the stroke-prone spontaneously hypertensive rat (SHRSP) fed a low-normal NaCl diet, we recently reported that supplemental KCl, but not KHCO 3 or K-citrate (KB/C), exacerbated hypertension and induced Hyperreninemia and strokes. We now ask the following question: In these SHRSP, is either such selectively Cl - -sensitive hypertension or Hyperreninemia a pathogenetic determinant of renal microvasculopathy? Methods SHRSPs were randomized to either supplemental KCl, KB/C, or nothing (control) at 10 weeks of age. Four and 14 weeks afterward, we assessed renal microangiopathy histologically and measured plasma renin activity (PRA). From randomization, blood pressure was measured radiotelemetrically and continually; proteinuria was measured periodically. Results KCl, but not KB/C, amplified renal microangiopathy and proteinuria. Four weeks after randomization, when KCl initially exacerbated hypertension, renal microangiopathy, hyperproteinuria, and Hyperreninemia had not yet occurred. However, across all groups, the increment of SBP at four weeks strongly predicted its final increment, severity of renal microangiopathy, proteinuria, and PRA 14 weeks after randomization. Then, the severity of renal microangiopathy varied directly with the levels of systolic blood pressure (SBP; R 2 = 0.9, P R 2 = 0.7, P R 2 = 0.8, P Conclusions In the SHRSP, ( 1 ) like cerebral microangiopathy, renal microangiopathy is selectively Cl - sensitive and hence, systemic microangiopathy is as well; ( 2 ) Cl - likely amplifies microangiopathy by exacerbating hypertension and possibly also by increasing PRA; and ( 3 ) Cl - might increase blood pressure and PRA by further constricting the renal afferent arteriole.
Generoso Andria - One of the best experts on this subject based on the ideXlab platform.
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Complex chromosomal rearrangements causing Langer-Giedion syndrome atypical phenotype: genotype-phenotype correlation and literature review.
American journal of medical genetics. Part A, 2013Co-Authors: Gerarda Cappuccio, Rita Genesio, Valentina Ronga, Alberto Casertano, Antonella Izzo, Maria Pia Riccio, Carmela Bravaccio, Maria Carolina Salerno, Lucio Nitsch, Generoso AndriaAbstract:Langer-Giedion syndrome (LGS) is caused by a deletion of chromosome 8q23.3-q24.11. The LGS clinical spectrum includes intellectual disability (ID), short stature, microcephaly, facial dysmorphisms, exostoses. We describe a 4-year-old girl with ID, short stature, microcephaly, distinctive facial phenotype, skeletal signs (exostoses on the left fibula, coccyx agenesis, stubby and dysmorphic sphenoid bone, osteoporosis), central nervous system malformations (hypoplastic and dysmorphic corpus callosum and septum pellucidum), pituitary gland hypoplasia and Hyperreninemia. Array-CGH revealed complex chromosomal rearrangements. A diagnosis of LGS was confirmed by the detection of a 8q23.3-q24.1 deletion. Associated chromosomal abnormalities were a 21q22.1 deletion and a balanced reciprocal translocation t(2;11)(p24;p15) de novo, confirmed by FISH analysis. We document the patient's atypical findings, never described in LGS patients, in order to update the genotype-phenotype correlation. We speculate that the disruption of regulatory elements mapping upstream CYP11B2 involved in the deleted region could cause Hyperreninemia.
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Complex chromosomal rearrangements causing Langer-Giedion syndrome atypical phenotype: genotype-phenotype correlation and literature review.
American Journal of Medical Genetics Part A, 2013Co-Authors: Gerarda Cappuccio, Rita Genesio, Valentina Ronga, Alberto Casertano, Antonella Izzo, Maria Pia Riccio, Carmela Bravaccio, Maria Carolina Salerno, Lucio Nitsch, Generoso AndriaAbstract:Langer–Giedion syndrome (LGS) is caused by a deletion of chromosome 8q23.3–q24.11. The LGS clinical spectrum includes intellectual disability (ID), short stature, microcephaly, facial dysmorphisms, exostoses. We describe a 4-year-old girl with ID, short stature, microcephaly, distinctive facial phenotype, skeletal signs (exostoses on the left fibula, coccyx agenesis, stubby and dysmorphic sphenoid bone, osteoporosis), central nervous system malformations (hypoplastic and dysmorphic corpus callosum and septum pellucidum), pituitary gland hypoplasia and Hyperreninemia. Array-CGH revealed complex chromosomal rearrangements. A diagnosis of LGS was confirmed by the detection of a 8q23.3–q24.1 deletion. Associated chromosomal abnormalities were a 21q22.1 deletion and a balanced reciprocal translocation t(2;11)(p24;p15) de novo, confirmed by FISH analysis. We document the patient's atypical findings, never described in LGS patients, in order to update the genotype–phenotype correlation. We speculate that the disruption of regulatory elements mapping upstream CYP11B2 involved in the deleted region could cause Hyperreninemia. © 2013 Wiley Periodicals, Inc.
Francesco Paneni - One of the best experts on this subject based on the ideXlab platform.
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Synergic effects of renin and aldosterone on right ventricular function in hypertension: a tissue Doppler study.
Journal of cardiovascular medicine (Hagerstown Md.), 2015Co-Authors: Mario Gregori, Benedetta Giammarioli, Giuliano Tocci, Alberto Befani, Giuseppino Massimo Ciavarella, Andrea Ferrucci, Francesco PaneniAbstract:BACKGROUND Right ventricular dysfunction (RVD) is associated with poor cardiovascular outcome. The renin-angiotensin-aldosterone system is involved in alterations of the left ventricular geometry and function. Detrimental effects of the renin-angiotensin-aldosterone system on the right ventricular function are being postulated, but data supporting this assumption are still lacking. The aim of the study was to assess the impact of Hyperreninemia, hyperaldosteronism or their combination on right ventricular function in hypertensive individuals. METHODS Plasma renin activity (PRA) and plasma aldosterone concentrations (PACs) were measured in 116 hypertensive patients, divided as follows: normal PRA and PAC (n = 38); high PRA and normal PAC (hypereninemia) (n = 26); normal PRA and high PAC (hyperaldosternism) (n = 27); high PRA and PAC (HRA) (n = 25). Echocardiographic evaluation of the left and right ventricles (RV), including tissue Doppler imaging, was performed. RVD was identified by tissue Doppler Imaging-derived Myocardial Performance Index, calculated with a multisegmental approach. RESULTS Indices of the right ventricular structure and function, as well as the prevalence of RVD, were higher in Hyperreninemia and hyperaldosternism groups as compared with the normal group, and a further increase was observed in the HRA patients. Regression models showed a similar risk of RVD in the Hyperreninemia and hyperaldosternism patients, regardless of systemic and pulmonary pressure, as well as left ventricular dysfunction. Notably, patients with both Hyperreninemia and hyperaldosternism exhibited the strongest association with RVD as compared with patients with only Hyperreninemia or hyperaldosternism. CONCLUSIONS Isolated Hyperreninemia or hyperaldosternism determines a similar impairment of the right ventricular function, whereas their combination is further detrimental. Renin and aldosterone may represent early biomarkers of right ventricular dysfunction in hypertension.
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Synergic effect of high renin and aldosterone levels on inappropriate left ventricular mass and systolic function: A tissue Doppler study
International journal of cardiology, 2013Co-Authors: Mario Gregori, Giuliano Tocci, Alberto Befani, Giuseppino Massimo Ciavarella, Andrea Ferrucci, Francesco PaneniAbstract:Left ventricular dysfunction (LVD) is a harmful condition leading to increased cardiovascular morbidity and mortality. Among the complex pathophysiological factors contributing to the development of LVD, the renin–angiotensin–aldosterone system (RAAS) has been recognized to play a pivotal role. Hyperactivation of RAAS promotes structural and functional changes leading to myocardial fibrosis, stiffness and left ventricular hypertrophy [1–4]. A large proportion of patients with altered RAAS homeostasis presents with isolated increase in plasma renin activity (PRA) or aldosterone concentrations (PAC). However, data about the impact of isolated Hyperreninemia or hyperaldosteronism on LV geometry and function are not exhaustive. Moreover, it remains unclearwhether the coexistence of high renin and aldosterone has a synergic effect on cardiac damage. LV mass (LVM) exceeding compensatory values for individual cardiac load has been recently described as inappropriate left ventricular mass (ILVM). This adverse phenotype of cardiac hypertrophy is emerging as a key determinant of cardiovascular risk [5]. In the present study we investigated the individual contribution of isolated PRA and PAC elevations on ILVM and LVD. PRA and PAC were measured in 133 young newly diagnosed hypertensive subjectswho had never been treatedwith RAAS blockers or other antihypertensive drugs. The study population was stratified as follows: 1) normal PRA and PAC (N) [n = 45]; 2) high PRA and normal PAC (Hyperreninemia, HR)
Masae Tanaka - One of the best experts on this subject based on the ideXlab platform.
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Chloride-sensitive renal microangiopathy in the stroke-prone spontaneously hypertensive rat.
Kidney International, 2001Co-Authors: Masae Tanaka, Olga Schmidlin, Jean L. Olson, Curtis R. MorrisAbstract:Chloride-sensitive renal microangiopathy in the stroke-prone spontaneously hypertensive rat. Background In the stroke-prone spontaneously hypertensive rat (SHRSP) fed a low-normal NaCl diet, we recently reported that supplemental KCl, but not KHCO 3 or K-citrate (KB/C), exacerbated hypertension and induced Hyperreninemia and strokes. We now ask the following question: In these SHRSP, is either such selectively Cl - -sensitive hypertension or Hyperreninemia a pathogenetic determinant of renal microvasculopathy? Methods SHRSPs were randomized to either supplemental KCl, KB/C, or nothing (control) at 10 weeks of age. Four and 14 weeks afterward, we assessed renal microangiopathy histologically and measured plasma renin activity (PRA). From randomization, blood pressure was measured radiotelemetrically and continually; proteinuria was measured periodically. Results KCl, but not KB/C, amplified renal microangiopathy and proteinuria. Four weeks after randomization, when KCl initially exacerbated hypertension, renal microangiopathy, hyperproteinuria, and Hyperreninemia had not yet occurred. However, across all groups, the increment of SBP at four weeks strongly predicted its final increment, severity of renal microangiopathy, proteinuria, and PRA 14 weeks after randomization. Then, the severity of renal microangiopathy varied directly with the levels of systolic blood pressure (SBP; R 2 = 0.9, P R 2 = 0.7, P R 2 = 0.8, P Conclusions In the SHRSP, ( 1 ) like cerebral microangiopathy, renal microangiopathy is selectively Cl - sensitive and hence, systemic microangiopathy is as well; ( 2 ) Cl - likely amplifies microangiopathy by exacerbating hypertension and possibly also by increasing PRA; and ( 3 ) Cl - might increase blood pressure and PRA by further constricting the renal afferent arteriole.
Gerarda Cappuccio - One of the best experts on this subject based on the ideXlab platform.
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Complex chromosomal rearrangements causing Langer-Giedion syndrome atypical phenotype: genotype-phenotype correlation and literature review.
American journal of medical genetics. Part A, 2013Co-Authors: Gerarda Cappuccio, Rita Genesio, Valentina Ronga, Alberto Casertano, Antonella Izzo, Maria Pia Riccio, Carmela Bravaccio, Maria Carolina Salerno, Lucio Nitsch, Generoso AndriaAbstract:Langer-Giedion syndrome (LGS) is caused by a deletion of chromosome 8q23.3-q24.11. The LGS clinical spectrum includes intellectual disability (ID), short stature, microcephaly, facial dysmorphisms, exostoses. We describe a 4-year-old girl with ID, short stature, microcephaly, distinctive facial phenotype, skeletal signs (exostoses on the left fibula, coccyx agenesis, stubby and dysmorphic sphenoid bone, osteoporosis), central nervous system malformations (hypoplastic and dysmorphic corpus callosum and septum pellucidum), pituitary gland hypoplasia and Hyperreninemia. Array-CGH revealed complex chromosomal rearrangements. A diagnosis of LGS was confirmed by the detection of a 8q23.3-q24.1 deletion. Associated chromosomal abnormalities were a 21q22.1 deletion and a balanced reciprocal translocation t(2;11)(p24;p15) de novo, confirmed by FISH analysis. We document the patient's atypical findings, never described in LGS patients, in order to update the genotype-phenotype correlation. We speculate that the disruption of regulatory elements mapping upstream CYP11B2 involved in the deleted region could cause Hyperreninemia.
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Complex chromosomal rearrangements causing Langer-Giedion syndrome atypical phenotype: genotype-phenotype correlation and literature review.
American Journal of Medical Genetics Part A, 2013Co-Authors: Gerarda Cappuccio, Rita Genesio, Valentina Ronga, Alberto Casertano, Antonella Izzo, Maria Pia Riccio, Carmela Bravaccio, Maria Carolina Salerno, Lucio Nitsch, Generoso AndriaAbstract:Langer–Giedion syndrome (LGS) is caused by a deletion of chromosome 8q23.3–q24.11. The LGS clinical spectrum includes intellectual disability (ID), short stature, microcephaly, facial dysmorphisms, exostoses. We describe a 4-year-old girl with ID, short stature, microcephaly, distinctive facial phenotype, skeletal signs (exostoses on the left fibula, coccyx agenesis, stubby and dysmorphic sphenoid bone, osteoporosis), central nervous system malformations (hypoplastic and dysmorphic corpus callosum and septum pellucidum), pituitary gland hypoplasia and Hyperreninemia. Array-CGH revealed complex chromosomal rearrangements. A diagnosis of LGS was confirmed by the detection of a 8q23.3–q24.1 deletion. Associated chromosomal abnormalities were a 21q22.1 deletion and a balanced reciprocal translocation t(2;11)(p24;p15) de novo, confirmed by FISH analysis. We document the patient's atypical findings, never described in LGS patients, in order to update the genotype–phenotype correlation. We speculate that the disruption of regulatory elements mapping upstream CYP11B2 involved in the deleted region could cause Hyperreninemia. © 2013 Wiley Periodicals, Inc.