Immunological mechanisms of arterial damage in pediatric patients with primary hypertension.

Primary hypertension is a disease that is being diagnosed with increasing frequency in pediatric patients, and many of them are found to have hypertension-mediated organ damage (HMOD), including arterial damage. The pathophysiology of primary hypertension and the formation of HMOD is multifactorial. One mechanism studied in recent years is the subclinical inflammation accompanying the elevation of blood pressure. Experimental studies, studies in adults and children, revealed the involvement of immune mechanisms in the formation of vascular lesions in the course of primary hypertension. The paper summarizes the current knowledge on this subject and points to possible therapeutic targets. Particular emphasis is placed on data from pediatric patients with primary hypertension, as a relation between arterial damage (early vascular aging) and immune system activation had already been found in children. The correct identification of immunological mechanisms may not only broaden our understanding of primary hypertension as a disease but also, more importantly, lead to the most effective methods of its treatment.

Nephrological and urological symptoms in patients with Robinow syndrome - a report of two cases.

Robinow syndrome is a rare congenital syndrome described in 1969 by Meinhard Robinow. The genetic background is heterogeneous - mutations of DVLI1, DVLI3, WNT5A genes (mild, autosomal dominant inheritance) or ROR2 gene (severe, autosomal recessive inheritance) are responsible for the syndrome. The syndrome is characterized by facial dysmorphism, skeletal defects, short stature, cardiovascular and urinary system abnormalities. CASE REPORT: We report nephrological and urological problems in two 4-year-old male patients with Robinow syndrome. The first patient has a horseshoe kidney located mainly on the right side, right vesicoureteral reflux grade II, dysfunctional voiding, buried penis, and retractile testicles. The second patient has recurrent urinary tract infections; diagnostic findings include left kidney duplication, grade II left vesicoureteral reflux, large posterior urethral diverticulum, dysfunctional voiding, buried penis, glanular hypospadias, and bilateral cryptorchidism. CONCLUSIONS: Patients with Robinow syndrome require multidisciplinary care, including nephrology-urology care. Nephrological and urological manifestations in children with Robinow syndrome are diverse, and urinary tract defects may be atypical and complex.

Reduced Blood Pressure Dipping Is A Risk Factor for the Progression of Chronic Kidney Disease in Children.

Background: Elevated blood pressure and proteinuria are well-established risk factors for chronic kidney disease (CKD) progression in children. This study aimed to analyze risk factors for CKD progress, emphasizing detailed ambulatory blood pressure (ABPM) data. Methods: In 55 children with CKD II−V, observed for ≥1 year or until initiation of kidney replacement therapy, we analyzed ABPM, clinical, and biochemical parameters. Results: At the beginning, the glomerular filtration rate (eGFR) was 66 (interquartile range­IQR: 42.8−75.3) mL/min/1.73 m2, and the observation period was 27 (16−36) months. The mean eGFR decline was 2.9 ± 5.7 mL/min/1.73 m2/year. eGFR decline correlated (p < 0.05) with age (r = 0.30), initial proteinuria (r = 0.31), nighttime systolic and mean blood pressure (r = 0.27, r = 0.29), and systolic and diastolic blood pressure dipping (r = −0.37, r = −0.29). There was no relation between mean arterial pressure during 24 h (MAP 24 h Z-score) and eGFR decline and no difference in eGFR decline between those with MAP 24 h < and ≥50 th percentile. In multivariate analysis, systolic blood pressure dipping (beta = −0.43), presence of proteinuria (beta = −0.35), and age (beta = 0.25) were predictors of eGFR decline. Conclusions: Systolic blood pressure dipping may be a valuable indicator of CKD progression in children.

Circadian Blood Pressure Profile in Pediatric Patients with Primary Hypertension.

Our study aimed to evaluate factors affecting circadian BP profile and its association with hypertension-mediated organ damage (HMOD) in pediatric patients with primary hypertension (PH). The study included 112 children (14.7 ± 2.1 age, 79 boys, 33 girls) with untreated PH. Non-dipping was defined as a nocturnal drop in systolic or diastolic BP (SBP, DBP) < 10%, and a nocturnal drop >20% was defined as extreme dipping. The nocturnal SBP drop was 10.9 ± 5.9 (%), and the DBP drop was 16.2 ± 8.5 (%). Non-dipping was found in 50 (44.6%) children and extreme dipping in 29 (25.9%) patients. The nocturnal SBP decrease correlated with BMI Z-score (r = −0.242, p = 0.010) and left ventricular mass index (LVMI) (r = −0.395, p = 0.006); diastolic DBP decrease correlated with augmentation index (AIx75HR) (r = 0.367, p = 0.003). Patients with a disturbed blood pressure profile had the highest LVMI (p = 0.049), while extreme dippers had the highest augmentation index (AIx75HR) (p = 0.027). Elevated systolic and diastolic BP dipping were risk factors for positive AIx75HR (OR 1.122 95CI (1.009−1.249) and OR 1.095 95CI (1.017−1.177). We concluded that disturbed circadian BP profile was common in children with PH and should not be considered a marker of secondary hypertension. A disturbed circadian BP profile may be associated with higher body weight. In pediatric patients with PH, non-dipping is associated with increased left ventricular mass, and extreme dipping may be a risk factor for increased arterial stiffness.

Monogenic hypertension.

Monogenic hypertension (MH) is a rare form of arterial hypertension (AH) in which a single gene mutation is responsible for developing the disease. This article discusses the pathogenesis, genetics, phenotype, and treatment of monogenic forms of AH. According to Guyton's hypothesis, mutations responsible for MH development most often lead to increased renal sodium reabsorption, in a mineralocorticoid-dependent or -independent mechanism, resulting in fluid retention and increased blood pressure. MH most often appears in childhood or adolescence and is characterized by moderate to severe blood pressure elevation and resistance to standard treatment. The coexistence of water-electrolyte abnormalities, most commonly hypokalemia and metabolic alkalosis, is characteristic but not always present. Monogenic AH should also be considered in patients with precocious or delayed puberty, growth deficiency, brachydactyly, and severe symptoms or hypertension mediated-organ damage. Identifying patients with monogenic hypertension is of utmost importance to implement appropriate treatment and reduce the risk of cardiovascular complications.

Evaluation of Active Renin Concentration in A Cohort of Adolescents with Primary Hypertension.

Our study aimed to assess active renin concentration in children with primary hypertension. Thus, we evaluated active renin concentration, clinical parameters, office and ambulatory blood pressure, and biochemical parameters in 51 untreated adolescents with primary hypertension (median: 14.4 [interquartile range-IQR: 13.8-16.8] years) and 45 healthy adolescents. Active renin concentration did not differ between patients with hypertension and healthy children (median: 28.5 [IQR: 21.9-45.2] vs. 24.9 [IQR: 16.8-34.3] [pg/mL], p = 0.055). In the whole group of 96 children, active renin concentration correlated positively with serum potassium and office and ambulatory systolic and diastolic blood pressures. Among children with hypertension, patients with isolated systolic hypertension had lower renin concentration than patients with systolic-diastolic hypertension (26.2 [IQR: 18.6-34.2] vs. 37.8 [IQR: 27.0-49.6] [pg/mL], p = 0.014). The active renin concentration did not differ between patients with isolated systolic hypertension and healthy children. In multivariate analysis, diastolic blood pressure Z-score (beta = 0.238, 95 confidence interval [0.018-0.458], p = 0.035) was the only predictor of active renin concentration in the studied children. We concluded that active renin concentration is positively associated with blood pressure and potassium in children, and diastolic blood pressure was the strongest predictor of renin level. Patients with isolated systolic hypertension may differ from patients with systolic-diastolic hypertension in less severe activation of the renin-angiotensin-aldosterone system.

Burnout Syndrome among Pediatric Nephrologists-Report on Its Prevalence, Severity, and Predisposing Factors.

BACKGROUND AND OBJECTIVES: Burnout is an occupation-related syndrome comprising emotional exhaustion, depersonalization, and reduced feelings of work-related personal accomplishments. There are reports on burnout among adult nephrologists and general pediatricians, but little is known about burnout among pediatric nephrologists. The aim of our study was to assess the prevalence and severity of burnout syndrome among Polish pediatric nephrologists. MATERIALS AND METHODS: A 25-item study survey consisting of abbreviated Maslach Burnout Inventory and additional self-created questions about work-related factors was completed by 97 physicians affiliated with the Polish Society of Pediatric Nephrology. Women comprised 75.3%, with median time of professional experience in the study group was 15 years. RESULTS: A high level of emotional exhaustion, depersonalization, and reduced feeling of personal accomplishments were observed in 39.2%, 38.1%, and 21.6% of the participants, respectively. At least a medium level of burnout in all three dimensions were observed in 26.8% of the participants and 8.2% of them presented high three-dimensional burnout. About 41.2% of the participants stated that they would like to take part in burnout prevention and support programs. According to the study participants, excessive bureaucracy in healthcare systems, rush at work, and overtime work were the main job-related problems that could influence burnout intensity. CONCLUSIONS: Burnout is an important factor in the professional landscape of pediatric nephrology. Actions aimed at reducing the risk of occupational burnout among pediatric nephrologists should be applied, both at the personal and institutional levels.

24-hour central blood pressure and immune system activation in adolescents with primary hypertension - a preliminary study.

Introduction: Adult and pediatric data suggest a positive relationship between the extent of subclinical inflammation, blood pressure, and hypertension-mediated organ damage (HMOD) in primary hypertension (PH). 24-hour (24-h) ambulatory blood pressure (ABPM) and central blood pressure (CBP) are strong predictors of HMOD. Our study aimed to analyze the relationship between 24-h central ABPM, subclinical inflammation, and clinical data in adolescents with PH. Material and methods: In 28 untreated adolescents with PH (14.50 ±2.27 years) and 25 healthy peers (14.76 ±2.83 years), we analyzed 24-h peripheral and central ABPM, markers of subclinical inflammation (neutrophil-to-lymphocyte ratio - NLR, platelet-to-lymphocyte ratio - PLR, mean platelet volume - MPV), and clinical and biochemical data. Results: Patients with PH had higher 24-h peripheral and central blood pressure than healthy peers. In all 53 patients, we found significant (p < 0.05) positive correlations between NLR, PLR and 24-h central systolic, diastolic, and mean blood pressure (24-h cSBP, 24-h cDBP, 24-h cMAP), between 24-h central augmentation index corrected for heart rate 75 (24-h cAIx75HR) and platelet count. In 28 patients with PH, 24-h cAIx75HR correlated with low-density lipoprotein (LDL) cholesterol (R = 0.442), and ambulatory arterial stiffness index with body mass index (BMI) (R = 0.487), uric acid (R = 0.430), and high-density lipoprotein (HDL) cholesterol (R = -0.428). Conclusions: Increased central 24-h blood pressure may be associated with immune system activation in adolescents with primary hypertension. In adolescents with primary hypertension, dyslipidemia and hyperuricemia are risk factors for increased arterial stiffness. Further studies on central and peripheral blood pressure in terms of their relationship with inflammation in these patients are needed.

Early Vascular Aging in Children With Tuberous Sclerosis Complex.

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.

Arterial stiffness in children with primary hypertension is related to subclinical inflammation.

INTRODUCTION: The immune system can trigger an inflammatory process leading to blood pressure elevation and arterial damage. The aim of the study was to assess the relation between subclinical inflammation and arterial damage in pediatric patients with primary hypertension (PH) and to establish the usefulness of neutrophil-to-lymphocyte (NLR) and platelet-to-lymphocyte (PLR) ratios, and mean platelet volume (MPV) as markers of arterial damage in these subjects. MATERIAL AND METHODS: In 119 children with PH (14.94 ±2.76 years) and 45 healthy children (14.91 ±2.69 years) we analyzed markers of subclinical inflammation (NLR, PLR, MPV), clinical and biochemical parameters, office blood pressure, ambulatory blood pressure monitoring (ABPM), central blood pressure, aortic pulse wave velocity (aPWV), augmentation index corrected for heart rates 75 (AIx75HR), carotid intima media thickness (cIMT), and common carotid artery stiffness (E-tracking). RESULTS: Children with PH were characterized by significantly higher neutrophil (3.9 ±1.7 vs. 3.0 ±1.0 [1000/µl], p < 0.001) and platelet counts (271.9 ±62.3 vs. 250.3 ±60.3 [1000/µl], p = 0.047), NLR (1.9 ±1.5 vs. 1.3 ±0.4, p = 0.010), PLR (131.4 ±41.9 vs. 114.7 ±37.6, p = 0.020), aPWV (5.36 ±0.88 vs. 4.88 ±0.92 m/s, p = 0.004), and cIMT (0.46 ±0.07 vs. 0.43 ±0.07 mm, p = 0.002) compared to healthy children. In PH children NLR correlated positively (p < 0.05) with: systolic, diastolic and mean blood pressure in ABPM (r = 0.243, r = 0.216, r = 0.251), aPWV [m/s] (r = 0.241), aPWV Z-score (r = 0.204), and common carotid artery PWVbeta [m/s] (r = 0.202). CONCLUSIONS: There is a link between arterial stiffness and subclinical inflammation in pediatric patients with primary hypertension. Neutrophil-to-lymphocyte ratio may serve as a promising marker of arterial stiffness in pediatric patients affected by primary hypertension.

Treatment of idiopathic nephrotic syndrome with two steroid dosing regimens - one-year observational study.

INTRODUCTION: The aim of the study was to compare the first year of disease in children with idiopathic nephrotic syndrome (INS) treated according to two prednisone dosing regimens: a weight-based schedule (2 mg/kg/24 h in the 1st month, 2 mg/kg/48 h in the 2nd month, with dose tapering during the following 4 months), and a body surface area (BSA)-based schedule (60 mg/m2/24 h in the 1st month, 40 mg/m2/48 h in the 2nd month, with dose tapering during the following 4 months). MATERIAL AND METHODS: In 2 groups of children treated with weight- and BSA-based regimens (20 patients, 3.13 ±1.01 years, treated in 2010-2013 and 20 patients, 5.13 ±2.86 years, treated in 2014-2016) clinical and anthropometrical parameters, number of INS relapses, total prednisone dose (mg/kg/year), and steroid adverse effects were compared during the first year of disease. RESULTS: Children treated with the weight-based steroid regimen received a higher total annual prednisone dose (259.06 ±79.54 vs. 185.83 ±72.67 mg/kg/24 h, p = 0.004) and had a shorter (though not significantly) period without prednisone (38.25 ±55.83 vs. 75.90 ±73.06 days, p = 0.062) compared to patients treated with the BSA-based regimen. There was no difference in number of relapses between groups (2.20 ±1.64 vs. 1.60 ±1.67, p = 0.190) but more patients relapsed in the weight-based group (19/20 vs. 13/20, p = 0.044). No differences in Z-score values of height, weight, and body mass index (BMI) were observed. No steroid-related adverse events were noted except for arterial hypertension (4/20 vs. 5/20 patients, p = 1.000). CONCLUSIONS: The BSA-based regimen of prednisone dosing in children with INS reduces exposure to steroids and risk of relapse, as well as increases days off steroids in the first year compared to the weight-based regimen with a high second-month dose.

NT-proBNP as a Potential Marker of Cardiovascular Damage in Children with Chronic Kidney Disease.

Assessing cardiovascular disease (CVD) in children with chronic kidney disease (CKD) is difficult. Great expectations have been associated with biomarkers, including the N-terminal pro-brain natriuretic peptide (NT-proBNP). This study aimed to determine the correlation between NT-proBNP and cardiovascular complications in children with CKD. Serum NT-proBNP, arterial stiffness, common carotid artery intima-media thickness (cIMT), echocardiographic (ECHO) parameters (including tissue Doppler imaging), and biochemical and clinical data were analyzed in 38 pediatric patients with CKD (21 boys, 12.2 ± 4.2 years). Mean NT-proBNP in CKD patients was 1068.1 ± 4630 pg/mL. NT-proBNP above the norm (125 pg/mL) was found in 16 (42.1%) subjects. NT-proBNP correlated with glomerular filtration rate (GFR) (r = -0.423, p = 0.008), and was significantly higher in CKD G5 (glomerular filtration rate grade) patients compared to CKD G2, G3, and G4 children (p = 0.010, p = 0.004, and p = 0.018, respectively). Moreover, NT-proBNP correlated positively with augmentation index (AP/PP: r = 0.451, p = 0.018, P2/P: r = 0.460, p = 0.016), cIMT (r = 0.504, p = 0.020), and E/E' in ECHO (r = 0.400, p = 0.032). In multivariate analysis, logNT-proBNP was the only significant predictor of cIMT Z-score (beta = 0.402, 95CI (0.082-0.721), p = 0.014) and P2/P1 (beta = 0.130, 95CI (0.082-0.721), p = 0.014). Conclusions: NT-proBNP may serve as a possible marker of thickening of the carotid artery wall in pediatric patients with CKD. The final role of NT-proBNP as a biomarker of arterial damage, left ventricular hypertrophy, or cardiac diastolic dysfunction in CKD children needs confirmation in prospective studies.

[Hypertensive urgencies and emergencies in pediatric patients]. / Nadcisnieniowe stany pilne i nagle u chorych pediatrycznych.

Hypertensive crisis is a sudden rise in blood pressure that is significantly above normal values. Depending on the severity of symptoms, hypertensive crisis can be classified as hypertensive urgency, i.e. severe arterial hypertension (AH) without organ failure and damage with nonspecific symptoms (pain, dizziness, nosebleeds, nausea, vomiting), and hypertensive emergency, i.e. severe AH with organ failure and/or acute organ damage. The most common causes of hypertensive crisis in neonates and infants are vascular diseases (thrombus or stenosis of the renal artery, coarctation of the aorta) or renal parenchymal diseases, in older children kidney diseases and renal artery stenosis, in adolescents also intoxications or pregnancy. In neonates and infants, nonspecific symptoms caused by acute heart failure predominate, and in older children, symptoms from the central nervous system are most typical. Fast- and short-acting medications are used in the treatment of hypertensive urgencies and emergencies; a gradual normalization of blood pressure within 36-48 hours is recommended. Hypertensive emergencies are treated with intravenous drugs (e.g., labetalol, hydralazine), and hypertensive urgencies with intravenous or oral drugs such as nifedipine, clonidine, and minoxidil. Hypertensive emergencies are treated with intravenous drugs (e.g., labetalol, hydralazine), and hypertensive urgencies with intravenous or oral drugs such as nifedipine, clonidine, and minoxidil. Emergency conditions are treated with intravenous drugs (e.g., labetalol, hydralazine), urgent conditions with intravenous or oral drugs such as nifedipine, clonidine, and minoxidil. Some causes of hypertensive crisis require different management, e.g. alpha-blockers in pheochromocytoma. In all patients, evaluation of target organ damage and extensive diagnostics for secondary forms of hypertension is necessary.

Serum Sclerostin Is Associated with Peripheral and Central Systolic Blood Pressure in Pediatric Patients with Primary Hypertension.

Recent studies showed the significance of the canonical Wnt/beta-catenin pathway and its inhibitor-sclerostin, in the formation of arterial damage, cardiovascular morbidity, and mortality. The study aimed to assess serum sclerostin concentration and its relationship with blood pressure, arterial damage, and calcium-phosphate metabolism in children and adolescents with primary hypertension (PH). Serum sclerostin concentration (pmol/L) was evaluated in 60 pediatric patients with PH and 20 healthy children. In the study group, we also assessed calcium-phosphate metabolism, office peripheral and central blood pressure, 24 h ambulatory blood pressure, and parameters of arterial damage. Serum sclerostin did not differ significantly between patients with PH and the control group (36.6 ± 10.6 vs. 41.0 ± 11.9 (pmol/L), p = 0.119). In the whole study group, sclerostin concentration correlated positively with height Z-score, phosphate, and alkaline phosphatase, and negatively with age, peripheral systolic and mean blood pressure, and central systolic and mean blood pressure. In multivariate analysis, systolic blood pressure (SBP) and height expressed as Z-scores were the significant determinants of serum sclerostin in the studied children: height Z-score (ß = 0.224, (95%CI, 0.017-0.430)), SBP Z-score (ß = -0.216, (95%CI, -0.417 to -0.016)). In conclusion, our results suggest a significant association between sclerostin and blood pressure in the pediatric population.

Serum Periostin as a Potential Biomarker in Pediatric Patients with Primary Hypertension.

Experimental studies suggest that periostin is involved in tissue repair and remodeling. The study aimed to evaluate serum periostin concentration as potential biomarker in pediatric patients with primary hypertension (PH). We measured serum periostin, blood pressure, arterial damage, biochemical, and clinical data in 50 children with PH and 20 age-matched healthy controls. In univariate analysis, children with PH had significantly lower serum periostin compared to healthy peers (35.42 ± 10.43 vs. 42.16 ± 12.82 [ng/mL], p = 0.038). In the entire group of 70 children serum periostin concentration correlated negatively with peripheral, central, and ambulatory blood pressure, as well as with aortic pulse wave velocity (aPWV). In multivariate analysis, periostin level significantly correlated with age (ß = -0.614, [95% confidence interval (CI), -0.831--0.398]), uric acid (ß = 0.328, [95%CI, 0.124-0.533]), body mass index (BMI) Z-score (ß = -0.293, [95%CI, -0.492--0.095]), high-density lipoprotein (HDL)-cholesterol (ß = 0.235, [95%CI, 0.054-0.416]), and triglycerides (ß = -0.198, [95%CI, -0.394--0.002]). Neither the presence of hypertension nor blood pressure and aPWV influenced periostin level. To conclude, the role of serum periostin as a biomarker of elevated blood pressure and arterial damage in pediatric patients with primary hypertension is yet to be unmasked. Age, body mass index, uric acid, and lipid concentrations are key factors influencing periostin level in pediatric patients.

[Uric acid in children and adolescents with primary hypertension]. / Kwas moczowy u dzieci i mlodziezy z nadcisnieniem tetniczym pierwotnym.

Increased concentration of uric acid may play a role in the pathogenesis of primary hypertension (PH). AIM: The aim of the study was to assess concentration of uric acid and to assess its correlation with selected clinical and biochemical parameters in children with PH. MATERIALS AND METHODS: In a group of 57 untreated pharmacologically children with PH (44 boys, 13 girls, mean age 14.99±2.84 years) following parameters were assessed: serum uric acid concentration, blood pressure in office measurement and in 24-hour ambulatory blood pressure monitoring (ABPM), and selected clinical and biochemical parameters. Control group consisted of 20 healthy children (mean 14.11±2.99 years). RESULTS: Serum uric acid concentration was significantly higher in children with PH compared to healthy children (5.72±1.38 vs. 4.55±1.07 mg/dL; p=0.001). In patients with PH, its concentration was significantly higher in boys compared to girls ((6.12±1.20 mg/dL vs. 4.35±1.13 mg/dL, p<0.001), no such difference was found in healthy children. In the PH group, uric acid concentration correlated positively with age (r=0.426, p=0.001), height (r=0.557, p<0.001), weight (r=0.518, p<0.001), weight Z- score (r=0.296, p=0.025), BMI (r=0.316, p=0.017), neutrophil count (r=0.280, p=0.035), systolic blood pressure (r=0.375, p=0.004) and pulse pressure (r=0.444, p=0.001) in ABPM and negatively with HDL cholesterol, heart rate (r=-0.310, p=0.02 (1=-0.309, p=0.020) and nighttime diastolic blood pressure dip (r=-0.268, p=0.044) in ABPM. In multivariate analysis, the determinants of uric acid concentration in children with PH were sex (Β = 0,367, 95%CI(0.122-0.611), p=0.004) and weight Z-score (Β= 0.254, 95%CI(0.005-0.504), p=0.046). CONCLUSIONS: Children with PH have increased serum uric acid concentration compared to healthy children. The risk factors for hyperuricemia in pediatric patients with PH are male sex and high body weight.

Circulating calcification inhibitors are associated with arterial damage in pediatric patients with primary hypertension.

BACKGROUND: Circulating calcification inhibitors: fetuin A (FA) and osteoprotegerin (OPG) together with soluble ligand of receptor activator of nuclear factor kappa-B (sRANKL) have been linked to vascular calcifications and arterial damage. This study aimed to evaluate relationships between FA, OPG, sRANKL, and arterial damage in children with primary hypertension (PH). METHODS: In this cross-sectional single-center study, calcification inhibitors (FA, OPG, sRANKL) levels were measured in blood samples of 60 children with PH (median age 15.8, IQR: [14.5-16.8] years) and 20 age-matched healthy volunteers. In each participant, peripheral and central blood pressure evaluation (BP) and ambulatory BP monitoring (ABPM) were performed. Arterial damage was measured using common carotid artery intima media thickness (cIMT), pulse wave velocity (PWV), augmentation index (AIx75HR), and local arterial stiffness (ECHO-tracking-ET) analysis. RESULTS: Children with PH had significantly higher peripheral and central BP, BP in ABPM, thicker cIMT, higher PWV, and AIx75HR. FA was significantly lower in patients with PH compared to healthy peers without differences in OPG, sRANKL, and OPG/sRANKL and OPG/FA ratios. In children with PH, FA level correlated negatively with cIMT Z-score and ET AIx; sRANKL level correlated negatively with ABPM systolic blood pressure (SBP), SBP load, diastolic BP load, and AIx75HR; OPG/sRANKL ratio correlated positively with SBP load, while OPG/FA ratio correlated positively with ET AIx. In multivariate analysis, FA was a significant determinant of cIMT (mm) and cIMT Z-score. CONCLUSIONS: This study reveals that in children with primary hypertension, arterial damage is related to lower fetuin A concentrations.

Acute post-streptococcal glomerulonephritis - immune-mediated acute kidney injury - case report and literature review.

Acute post-streptococcal glomerulonephritis (APSGN) is an immunological complication of infection with group A ß-hemolytic streptococcus (GAS). The disease manifests as microscopic or gross hematuria, arterial hypertension, edema, and acute kidney injury and has most commonly a self-limiting course. We report a very severe case of APSGN in a 5-year-old girl with superimposed generalized infection. The girl presented significant overhydration, a very low glomerular filtration rate (GFR) (11.2 ml/min/1.73 m2), hyperuricemia (12.7 mg/dl), nephrotic proteinuria, and gross hematuria. Her immunological tests allowed for the diagnosis of APSGN (elevated antistreptolysin O [ASO] titer, low C3, and normal C4 complement factors). She also showed very high inflammatory indicators suggestive of sepsis. She received supportive treatment together with ceftriaxone and a single dose of rasburicase. Her renal function recovered, and urinalysis normalized. Gallbladder deposits complicated the treatment. This article summarizes the existing knowledge on APSGN with particular emphasis on the immunological mechanisms of the disease. The proposed immunological pathway leading to glomerular injury is discussed. In children, APSGN has an excellent prognosis, including in cases with severe renal impairment in the early stages of the disease.

[Hypertensive crisis in a 3-year-old boy - neurovascular conflict?] / Przelom nadcisnieniowy u 3-letniego chlopca ­ konflikt naczyniowo-nerwowy?

Hypertensive crisis is a sudden rise in blood pressure with accompanying clinical symptoms. The disease is extremely rare in small children and is always a complication of secondary hypertension. CASE REPORT: 3-year-old boy (spontaneous delivery, 37 week of gestation, birth weight 2170g, 10 AS, unremarkable family history) was admitted to a hospital because of weight loss (1.5 kg, i.e. approx. 15% in 6 months), anorexia, abdominal and limb pain and lethargy. On admission, very high blood pressure values (190/150 mm Hg), lean subcutaneous tissue, frequent blinking, height 88 cm (<3c), body weight 9.5 kg (<3c). In additional tests: blood morphology, parameters of renal function, ions, gasometry, catecholamine urinary excretion, steroid profile and daily cortisol profile were within normal limits. Elevated plasma renin activity was found. In imaging studies kidneys, adrenal glands and renal arteries were normal. Normotension was not obtained on two antihypertensive drugs - metoprolol and amlodipine. In angio-CT tortuous right vertebral artery, extending to the left on the anterolateral surface of the medulla oblongata - possible compression of the vessel of the left side of medulla - was found. Diagnosis of neurovascular conflict was made. The patient was consulted by neurosurgeon who declare no possibility of surgical treatment of anomalies. In the treatment, according to the literature, a drug blocking the renin-angiotensin-aldosterone-enalapril system was used, which normalized blood pressure. At the same time, intensive nutritional treatment was used. Resolution of symptoms and weight gain was observed. In further follow-up patients' parents withdrew enalapril lawlessly, which did not lead to recurrent rise in blood pressure. The latter may suggest other, transient cause of hypertensive crisis e.g. intoxication. CONCLUSIONS: Severe hypertension in pediatric patients can give symptoms as weight loss and behavioral disorders. In the diagnostic of hypertensive crisis in children, neuroimaging studies and toxicological tests should be performed.

Serum Klotho is correlated to cardiovascular complications of chronic kidney disease in children.

AIM: Aim of the study was to investigate soluble Klotho (sKl), fibroblast growth factor 23 (FGF23) concentrations, and their correlations with cardiovascular complications in children with CKD. MATERIALS AND METHODS: 38 children with CKD stages 2 - 5 were compared to 38 healthy controls in terms of: plasma FGF23, serum sKl, peripheral and central blood pressure, arterial stiffness (pulse wave velocity - (PWV)), carotid intima media thickness (cIMT), left ventricular mass index (LVMI), and diastolic function. Correlations between FGF23, sKl, and cardiovascular parameters were investigated. RESULTS: The CKD group was characterized by higher FGF23, lower sKl concentrations, higher peripheral and central blood pressure, arterial stiffness, cIMT, left ventricular mass index, and decreased E/A ratio compared to the control group. In CKD children, sKl correlated negatively with diastolic blood pressure (DBP), mean arterial pressure (MAP), central systolic, diastolic, and mean blood pressure, PWV, and LVMI. In multivariate analysis, higher sKl was a significant predictor of lower peripheral and central DBP and lower LVMI and E/A, whereas higher FGF23 was a predictor of higher of LVMI. CONCLUSION: (1) In children with CKD, decreased sKl might be a marker of elevated central blood pressure. (2) Both sKl decrease and FGF23 increase could possibly contribute to left ventricular hypertrophy in this group of patients.