scholarly journals Early Vascular Aging in Children With Tuberous Sclerosis Complex

2021 ◽  
Vol 9 ◽  
Author(s):  
Piotr Skrzypczyk ◽  
Anna Maria Wabik ◽  
Michał Szyszka ◽  
Sergiusz Józwiak ◽  
Przemysław Bombiński ◽  
...  
Keyword(s):  
Blood Pressure ◽  
Carotid Artery ◽  
Tuberous Sclerosis ◽  
Tuberous Sclerosis Complex ◽  
Common Carotid Artery ◽  
Healthy Children ◽  
Vascular Aging ◽  
Serum Cystatin C ◽  
Serum Cystatin ◽  
Arterial Damage

Objectives: Experimental data indicate that activating mutations in the mTOR (mammalian target of rapamycin) pathway may lead to abnormal arterial wall structure. Vascular anomalies like arterial stenoses are reported in pediatric patients with tuberous sclerosis complex (TSC). In addition, large renal lesions (angiomyolipoma—AML and cysts) are risk factors for arterial hypertension in adult patients with TSC. This study aimed to assess blood pressure, including central blood pressure and arterial damage (early vascular aging—EVA) in children with TSC.Materials and Methods: In a group of 33 pediatric patients with TSC (11.13 ± 4.03 years, 15 boys, 18 girls), we evaluated peripheral and central office blood pressure, 24-h ambulatory blood pressure, and arterial damage: aortic pulse wave velocity (aPWV) [m/s], [Z-score], augmentation index (AIx75HR [%]), common carotid artery intima-media thickness (cIMT) [mm], [Z-score], stiffness of common carotid artery (E-tracking), renal lesions in magnetic resonance and ultrasonography, and selected biochemical parameters. The control group consisted of 33 healthy children (11.23 ± 3.28 years, 15 boys, 18 girls).Results: In TSC group 7 (21.2%) children had arterial hypertension, 27 (81.8%) children had renal angiomyolipomas, 26 (78.8%)—renal cysts, and 4 (12.1%) patients were treated with mTOR inhibitors (2 patients with everolimus and 2 patients with sirolimus) at the moment of evaluation. Children with TSC had higher central systolic blood pressure (AoSBP) (98.63 ± 9.65 vs. 90.45 ± 6.87 [mm Hg], p < 0.001), cIMT (0.42 ± 0.05 vs. 0.39 ± 0.03 [mm], p = 0.011), cIMT Z-score (0.81 ± 1.21 vs. 0.16 ± 0.57, p = 0.007), aPWV (4.78 ± 0.81 vs. 4.25 ± 0.56 [m/s], p = 0.003) and aPWV Z-score (−0.14 ± 1.15 vs. −0.96 ± 0.87, p = 0.002) compared to healthy children, without differences in AIx75HR (8.71 ± 15.90 vs. 5.24 ± 11.12 [%], p = 0.319) and stiffness of common carotid artery. In children with TSC AoSBP correlated positively with serum cystatin C concentration (r = 0.377, p = 0.030) and with maximum diameter of renal cyst (R = 0.419, p = 0.033); mean arterial pressure (MAP) 24 h Z-score correlated with serum cystatin C concentration (R = 0.433, p = 0.013); and aPWV Z-score with daily urinary albumin loss [mg/24 h] (R = 0.412, p = 0.029).Conclusions: Children with tuberous sclerosis complex are at risk of elevated central blood pressure and early vascular aging. In children with TSC, blood pressure and arterial stiffness are related to renal involvement.

scholarly journals Assessment of Subclinical Renal Glomerular and Tubular Dysfunction in Children with Beta Thalassemia Major

Children ◽  
2021 ◽  
Vol 8 (2) ◽  
pp. 100
Author(s):  
Asmaa A. Mahmoud ◽  
Doaa M. Elian ◽  
Nahla MS. Abd El Hady ◽  
Heba M. Abdallah ◽  
Shimaa Abdelsattar ◽  
...  
Keyword(s):  
Cystatin C ◽  
Kidney Injury ◽  
Thalassemia Major ◽  
Beta Thalassemia ◽  
Good Survival ◽  
Control Group ◽  
Healthy Children ◽  
Serum Cystatin C ◽  
Serum Cystatin ◽  
Beta Thalassemia Major

Background: A good survival rate among patients with beta thalassemia major (beta-TM) has led to the appearance of an unrecognized renal disease. Therefore, we aimed to assess the role of serum cystatin-C as a promising marker for the detection of renal glomerular dysfunction and N-acetyl beta-D-glucosaminidase (NAG) and kidney injury molecule 1 (KIM-1) as potential markers for the detection of renal tubular injury in beta-TM children. Methods: This case-control study was implemented on 100 beta-TM children receiving regular blood transfusions and undergoing iron chelation therapy and 100 healthy children as a control group. Detailed histories of complete physical and clinical examinations were recorded. All subjected children underwent blood and urinary investigations. Results: There was a significant increase in serum cystatin-C (p < 0.001) and a significant decrease in eGFR in patients with beta-TM compared with controls (p = 0.01). There was a significant increase in urinary NAG, KIM-1, UNAG/Cr, and UKIM-1/Cr (p < 0.001) among thalassemic children, with a significant positive correlation between serum cystatin-C, NAG and KIM-1 as regards serum ferritin, creatinine, and urea among thalassemic patients. A negative correlation between serum cystatin-C and urinary markers with eGFR was noted. Conclusion: Serum cystatin-C is a good marker for detection of glomerular dysfunction. NAG and KIM-1 may have a predictive role in the detection of kidney injury in beta-TM children.

scholarly journals Cystatin C-Based Evaluation of Kidney Function of HIV-Infected Children in Benin City, Southern Nigeria

2012 ◽  
Vol 2012 ◽  
pp. 1-8 ◽  
Author(s):  
Moses Temidayo Abiodun ◽  
Nosakhare J. Iduoriyekemwen ◽  
Phillip O. Abiodun
Keyword(s):  
Kidney Disease ◽  
Hiv Infection ◽  
Kidney Function ◽  
Cystatin C ◽  
Mean Value ◽  
Control Group ◽  
Healthy Children ◽  
Serum Cystatin C ◽  
Serum Cystatin ◽  
The Mean

Background. Human immunodeficiency virus (HIV) is now a confirmed risk factor for kidney disease with an increased burden in persons of African descent.Method. We measured the serum cystatin C levels of 205 ART-naive, HIV-infected children by an ELISA technique and compared them with the levels of apparently healthy children.Result. The mean ± SD serum cystatin C level of children with HIV infection was 1.01 ± 0.44 mg/L, significantly higher than the mean value in the control group, that is, 0.72 ± 0.20 mg/L (P=0.000). The mean ± SD cystatin C-based estimated GFR of children with HIV infection was 102.7 ± 31.0 mL/min/1.73 m2, significantly lower than 126.9 ± 28.5 mL/min/1.73 m2in the control group, (P=0.014). A significantly higher proportion of HIV-infected children compared to controls had eGFR < 90 mL/min/1.73 m2(21.5% versus 5.4%;P=0.00). The prevalence of chronic kidney disease (CKD) among the HIV-infected children was 10.7%. The cystatin C-based eGFR of the HIV-infected children ≥5 years old correlated positively with their CD4 count (r=0.23;  P=0.022).Conclusion. There is a high prevalence of CKD among HIV-infected children, requiring regular monitoring of their kidney function using a cystatin C-based method.

Relating drug-induced changes in carotid artery mechanics to cardiovagal and sympathetic baroreflex control

2005 ◽  
Vol 83 (5) ◽  
pp. 439-446 ◽  
Author(s):  
Deborah D O'Leary ◽  
Craig D Steinback ◽  
Angela D Cechetto ◽  
Blaine T Foell ◽  
Jane C Topolovec ◽  
...  
Keyword(s):  
Blood Pressure ◽  
Carotid Artery ◽  
Common Carotid Artery ◽  
Baroreflex Sensitivity ◽  
Muscle Sympathetic Nerve Activity ◽  
Pressure Change ◽  
Drug Induced ◽  
Baroreflex Control ◽  
Arterial Blood ◽  
Induced Changes

Previous evidence indicates that sensitivity of the baroreflex cardiovagal and sympathetic arms is dissociated. In addition, pharmacologic assessment of baroreflex sensitivity (BRS) has revealed that cardiovagal, but not sympathetic, BRS is greater when blood pressure is increasing versus falling. The origin of this hysteresis is unknown. In this study, carotid artery distensibility and absolute distension (diameter) were assessed to test the hypothesis that vessel mechanics in barosensitive regions affect the BRS of cardiovagal, but not sympathetic, outflow. R-R interval (i.e. time between successive R waves), finger arterial blood pressure, muscle sympathetic nerve activity, and carotid artery dimensions (B-mode imaging) were measured during sequential infusions of sodium nitroprusside (SNP) and phenylephrine (PHE). Systolic and diastolic common carotid artery diameters and pulse pressure were recorded to calculate distensibility of this vessel under each drug condition. Cardiovagal BRS was greater when blood pressure was increasing versus decreasing (p < 0.01). Sympathetic BRS was not affected by direction of pressure change. Distensibility did not differ between SNP and PHE injections. However, compared with SNP, infusion of PHE resulted in larger absolute systolic and diastolic carotid diameters (p < 0.001). Therefore, cardiovagal reflex hysteresis was related to drug-induced changes in common carotid artery diameter but not distensibility. The lack of sympathetic hysteresis in this model suggests a relative insensitivity of this baroreflex component to carotid artery dimensions and provides a possible mechanism for the dissociation between cardiovagal and sympathetic BRS.Key words: Oxford method, baroreflex hysteresis, cardiovagal, MSNA, distensibility.

Use of the common carotid artery in blood pressure measurement in rats. A possible source of error

1980 ◽  
Vol 58 (9) ◽  
pp. 1126-1127 ◽  
Author(s):  
S. C. Pang ◽  
T. M. Scott
Keyword(s):  
Blood Pressure ◽  
Carotid Artery ◽  
Common Carotid Artery ◽  
Pressure Measurement ◽  
Blood Pressure Measurement ◽  
Neck Surgery ◽  
Artery Cannulation ◽  
Blood Pressures ◽  
The Common ◽  
Source Of Error

Comparison of blood pressures obtained by femoral and common carotid artery cannulation has shown that in the rat anaesthetized with Nembutal an increase in blood pressure occurs after neck surgery and common carotid artery cannulation.

scholarly journals Blood Pressure and Glomerular Filtration Rate in Youth With Tuberous Sclerosis Complex

2021 ◽  
Author(s):  
Efthymia Vargiami ◽  
Stella Stabouli ◽  
Christina Sidira ◽  
Maria Kyriazi ◽  
Athanasia Anastasiou ◽  
...  
Keyword(s):  
Blood Pressure ◽  
Glomerular Filtration Rate ◽  
Glomerular Filtration ◽  
Tuberous Sclerosis ◽  
Kidney Function ◽  
Tuberous Sclerosis Complex ◽  
Filtration Rate ◽  
Renal Involvement ◽  
Childhood And Adolescence ◽  
Renal Imaging

Abstract Background Renal involvement is very common in tuberous sclerosis complex (TSC) and is characterised by the development of angiomyolipoma and cysts. The aims of the present study were to assess kidney function and clinical features of renal involvement in TSC, including kidney function, and blood pressure (BP) levels in children, adolescents and young adults. Methods Non-selected patients with a definite diagnosis of TSC attending the paediatric neurology outpatient department of a tertiary hospital were included in a cross-sectional study. All participants had a renal imaging study within 6 months of ambulatory blood pressure (BP) and glomerular filtration rate (GFR) assessment. Data on demographics, history, genotype, kidney function at diagnosis and last imaging were collected. Results The median age of the patients was 15 years (IQR range 9 to18). 23.5% of the participants had ambulatory BP hypertension. Systolic BP levels correlated significantly with GFRDTPA values despite the absence of hyperfiltration. Greater increase in GFR from initial TSC diagnosis till the age of the assessment, resulted in higher GFR levels in childhood and adolescence in those that developed hypertension and possibly in those with angiomyolipoma or cysts. All patients with ambulatory BP hypertension had angiomyolipomas or cysts on renal imaging studies. Conclusions Hypertension may present with increased frequency in young patients with kidney disease associated with TSC. Routine ambulatory BP measurement could be part of the annual clinical assessment in patients with TSC.

scholarly journals Blood pressure and glomerular filtration rate in youth with tuberous sclerosis complex

2022 ◽  
Author(s):  
Efthymia Vargiami ◽  
Stella Stabouli ◽  
Christina Sidira ◽  
Maria Kyriazi ◽  
Athanasia Anastasiou ◽  
...  
Keyword(s):  
Blood Pressure ◽  
Glomerular Filtration Rate ◽  
Glomerular Filtration ◽  
Tuberous Sclerosis ◽  
Tuberous Sclerosis Complex ◽  
Filtration Rate

scholarly journals Detection of Occult Glomerular Dysfunction in Glucose Six Phosphate Dehydrogenase Deficiency Anemia

2016 ◽  
Vol 8 ◽  
pp. e2016038 ◽  
Author(s):  
Gehan Abdel Hakeem ◽  
Emad Abdel Naem ◽  
Salwa Swelam ◽  
Laila Aboul Fotoh ◽  
Abdel Azeem Al Mazary ◽  
...  
Keyword(s):  
Cystatin C ◽  
Dehydrogenase Deficiency ◽  
Kidney Injury ◽  
Deficiency Anemia ◽  
Variable Degree ◽  
Healthy Children ◽  
Serum Cystatin C ◽  
Serum Cystatin ◽  
Chronic Kidney Injury ◽  
Hemolytic Crisis

Background: Glucose six phosphate dehydrogenase deficiency (G6PD) anemia is associated with intravascular hemolysis. The freely filtered hemoglobin can damage the kidney. We aimed to assess gloumerular status in G6PD children.Methods: Sixty children were included. Thirty G6PD deficiency anemia children were enrolled during the acute hemolytic crisis and after hemolytic episode. Another thirty healthy children were included as controls. Serum cystatin C, creatinine levels and urinary albumin/creatinine (A/C) ratio were measured for glomerular filtration rate (GFR) calculation.Results:Significantly higher urinary A/C ratio and lower GFR were found during hemolysis and after the hemolytic episode compared to controls. Also, significant higher serum cystatin C, creatinine and A/C ratio and lower GFR during acute hemolytic crisis compared to the same children after the hemolytic episode subsided. Conclusions:G6PD deficiency anemia is associated with a variable degree of glomerular dysfunction during acute hemolytic episodes. This glomerular dysfunction can result in chronic subclinical or occult chronic kidney injury.

Associations of Prematurity and Low Birth Weight With Blood Pressure and Kidney Function in Middle-aged Participants of the Brazilian Longitudinal Study of Adult Health – ELSA-Brasil

2021 ◽  
Author(s):  
Julia Ines F. Branda ◽  
Bianca Almeida-Pititto ◽  
Isabela Bensenor ◽  
Paulo A. Lotufo ◽  
Sandra Roberta G. Ferreira
Keyword(s):  
Blood Pressure ◽  
Longitudinal Study ◽  
Birth Weight ◽  
Propensity Score ◽  
Low Birth Weight ◽  
Kidney Function ◽  
Adult Health ◽  
Kidney Dysfunction ◽  
Serum Cystatin C ◽  
Serum Cystatin

Abstract BackgroundBased on the Developmental Origins of Health and Disease, adverse intrauterine environment – reflected by low birth weight (LBW) and prematurity – may induce fetal programming that favors kidney dysfunction in adulthood. We examined the association of LBW and prematurity with blood pressure (BP) and kidney function markers in non-diabetic middle-aged adults without kidney disease from the Brazilian Longitudinal Study of Adult Health (ELSA-Brasil). MethodsA cross-sectional analysis of 768 subjects aged 35-54 years was conducted. Comparisons were performed according to birth weight: LBW (<2.5 kg) or normal birth weight (NBW) (2.5-4.0 kg). Associations of LBW and prematurity with BP levels and kidney function markers (glomerular filtration rate - eGFR, albumin-creatinine ratio - ACR and serum cystatin C) were tested by multiple linear regression using adjustments based on Directed Acyclic Graphs. Propensity score matching was applied to control imbalances. ResultsMean age of participants was 45.5 ± 4.6 years and 56.8% were female; 64 (8.3%) participants reported LBW and 39 (5.0%) prematurity. The LBW group had higher systolic (p = 0.015) and diastolic BP (p = 0.014) and ACR values (p = 0.031) and lower eGFR (p = 0.015) than the NBW group, but no group difference for serum cystatin C was found. The preterm group had higher mean levels of systolic and diastolic BP, but no difference in kidney function markers was evident. In a regression model adjusted for sex, skin color and family history of hypertension, both systolic and diastolic BP levels were associated with LBW, but this association disappeared after adding prematurity, which remained associated with BP (p = 0.017). Having applied propensity score matching, LBW was associated with ACR values (p=0.003), but not with eGFR or BP levels. ConclusionThe study findings of independent associations of prematurity with higher BP levels, and of LBW with markers of kidney function, in adulthood support that early life events predict risk for hypertension and kidney dysfunction in adulthood. The study design precluded the inferring of causality, and prospective studies are needed to further investigate the hypothesis raised.

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