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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.
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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.
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Esgotamento Psicológico , Nefrologistas , Adulto , Causalidade , Criança , Feminino , Humanos , Pediatras , PrevalênciaRESUMO
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.
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Hipertensão , Hipopotassemia , Humanos , Hipertensão/diagnóstico , Mineralocorticoides , MutaçãoRESUMO
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.
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Nanismo , Deformidades Congênitas dos Membros , Nefrologia , Refluxo Vesicoureteral , Criança , Masculino , Humanos , Adulto Jovem , Adulto , Refluxo Vesicoureteral/complicações , Refluxo Vesicoureteral/diagnóstico , Deformidades Congênitas dos Membros/genética , Nanismo/genética , SíndromeRESUMO
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.
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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.
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Hipertensão Essencial , Adolescente , Espessura Intima-Media Carotídea , Estudos Transversais , Humanos , Análise de Onda de Pulso , alfa-2-Glicoproteína-HSRESUMO
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.
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Hipertensão , Hiperuricemia , Adolescente , Pressão Sanguínea , Monitorização Ambulatorial da Pressão Arterial , Criança , Feminino , Humanos , Masculino , Fatores de Risco , Ácido ÚricoRESUMO
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.
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Insuficiência Cardíaca , Hipertensão , Adolescente , Anti-Hipertensivos/uso terapêutico , Pressão Sanguínea , Criança , Emergências , Feminino , Humanos , Hipertensão/tratamento farmacológico , Lactente , Recém-Nascido , GravidezRESUMO
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.
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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.
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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.
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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.
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Pressão Sanguínea/fisiologia , Glucuronidase/sangue , Insuficiência Renal Crônica , Estudos de Casos e Controles , Criança , Fator de Crescimento de Fibroblastos 23 , Fatores de Crescimento de Fibroblastos/sangue , Humanos , Hipertrofia Ventricular Esquerda/epidemiologia , Hipertrofia Ventricular Esquerda/etiologia , Proteínas Klotho , Insuficiência Renal Crônica/complicações , Insuficiência Renal Crônica/epidemiologia , Rigidez Vascular/fisiologiaRESUMO
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.
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Hipertensão , Anti-Hipertensivos/uso terapêutico , Pressão Sanguínea , Criança , Pré-Escolar , Humanos , Hipertensão/tratamento farmacológico , Hipertensão/etiologia , Masculino , Artéria RenalRESUMO
Tubulointerstitial nephritis (TIN) is an inflammatory process primarily involving the renal interstitium and is the cause of acute kidney injury (AKI) in 3-7% of cases confirmed by renal biopsy in children. Aciclovir may have a nephrotoxic effect by crystallization in renal tubules or by inducing an immunologic process that leads to development of TIN. We report 2 male patients, aged 10 and 8 years, with nephrotic syndrome (NS), in whom disease relapse was triggered by varicella zoster infection. The patients received intravenous aciclovir which resulted in AKI due to acute TIN with the glomerular filtration rate 19.5 and 24.9 ml/min/1.73 m2, respectively. The diagnosis was confirmed by kidney biopsy in one of these patients. Initiation of glucocorticosteroids and withdrawal of aciclovir resulted in resolution of proteinuria and symptoms of AKI. In children with active NS treated with intravenous aciclovir, a possibility of AKI due to TIN should be taken into account.
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INTRODUCTION: Perinatal period is characterized by an increased risk of thrombosis due to low resources and limited compensatory capacity of the coagulation system in early stages of life. CASE REPORT: We report a case of a second pregnancy female infant born at 39 weeks by caesarean section, due to pre-labor rupture of membranes, with body weight of 3,570 γ and Apgar score 10. The pregnancy was complicated by hypothyroidism, uterine myoma, urinary tract infections, and mother's appendectomy at 16 Hbd. At 3 months, the girl was admitted to our hospital due to kidney calcifications, which were incidentally found during ultrasound scan. In laboratory workup, no abnormalities in calcium and phosphate homeostasis were detected. However, in ultrasound scan, linear calcifications along pyramids were visualized in both kidneys. Due to atypical location of nephrocalcinosis, Doppler scan was performed, showing lack of visible blood flow from renal veins to inferior vena cava (IVC), with compensatory flow from renal veins to paravertebral plexuses, and IVC obliteration with a massive calcification in the hepatic section. Magnetic resonance confirmed obliteration of IVC and common iliac veins, segmental dilatation of IVC, and compensatory blood flow from kidneys and lower limbs to paravertebral plexuses. Clinical picture and formation of collateral circulation suggested intrauterine thrombosis. Congenital thrombophilia was excluded in laboratory examination. CONCLUSIONS: The differential diagnosis of calcifications in renal parenchyma (nephrocalcinosis) should include renal vein thrombosis. Massive fetal and perinatal thrombosis can be asymptomatic due to high ability to form collateral circulation at the early stage of life.
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Background: Renalase is kidney-derived molecule initially considered as catecholamine-inactivating enzyme. However, recent studies suggest that renalase exerts potent cardio- and nephroprotective actions, not related to its enzymatic activity. Purpose: To assess renalase level in children with chronic kidney disease (CKD). Material and methods: Serum renalase, BMI, arterial stiffness, peripheral and central blood pressure, intima-media thickness (IMT), medications, and biochemical parameters were analyzed in 38 children with CKD (12.23 ± 4.19 years) (stage G2-5). Control group consisted of 38 healthy children. Results: In the study group, GFR was 25.74 ± 8.94 mL/min/1.73 m2; 6 children were dialyzed; 26 had arterial hypertension. Renalase level was higher in the study group compared to control group (p < 0.001). In CKD children renalase correlated (p < 0.05) with BMI Z-score (r = -0.36), alfacalcidol dose (r = 0.41), GFR (r = -0.69), hemoglobin (r = -0.48), total cholesterol (r = 0.35), LDL-cholesterol (r = 0.36), triglycerides (r = 0.52), phosphate (r = 0.35), calcium-phosphorus product (r = 0.35), parathormone (r = 0.58), and pulse wave velocity Z-score (r = 0.42). In multivariate analysis GFR (ß = -0.63, p < 0.001), triglycerides (ß = 0.59, p = 0.002), and alfacalcidol dose (ß = -0.49, p = 0.010) were determinants of renalase. Conclusions: In children with CKD there is a strong correlation between renalase level and CKD stage. Furthermore, in these patients renalase does not correlate with blood pressure but may be a marker of arterial stiffness.
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Monoaminoxidase/sangue , Insuficiência Renal Crônica/enzimologia , Adolescente , Conservadores da Densidade Óssea/sangue , Estudos de Casos e Controles , Criança , Feminino , Taxa de Filtração Glomerular , Humanos , Hidroxicolecalciferóis/sangue , Masculino , Insuficiência Renal Crônica/sangue , Insuficiência Renal Crônica/fisiopatologia , Triglicerídeos/sangue , Rigidez VascularRESUMO
AIM: Our aim was to assess common carotid artery intima-media thickness (cIMT) in children with idiopathic nephrotic syndrome (INS) and to find relation between cIMT and clinical and biochemical parameters in these patients. MATERIALS AND METHODS: In 50 children with INS we retrospectively evaluated: cIMT ((mm) and Z-score) and selected clinical and biochemical parameters. The control group consisted of 20 healthy children aged 9.46 ± 2.29 years. RESULTS: Children with INS had higher cIMT (0.45 ± 0.05 vs. 0.40 ± 0.05 (mm), p = 0.0002) and cIMT Z-score (1.72 ± 1.01 vs. 0.43 ± 1.01, p < 0.0001) than the control group. In the INS group, children with arterial hypertension had significantly higher cIMT (p = 0.0148) than normotensive children. In 50 children, with INS we found correlations between cIMT and disease duration (r = 0.40, p = 0.0040), number of INS relapses (r = 0.51, p< 0.0001), cumulative prednisone dose (r = 0.45, p = 0.0010), and BMI (r = 0.35, p = 0.0120); whereas, cIMT Z-score correlated only with the number of INS relapses (r = 0.41, p = 0.0160) and cumulative prednisone dose (r = 0.36, p = 0.0362). We found no relation between cIMT and response to corticosteroids, treatment used, and biochemical parameters. CONCLUSION: 1. Idiopathic nephrotic syndrome predisposes to atherosclerotic lesions in affected children. 2. The severity of atherosclerotic lesions is dependent mainly on the number of INS relapses, but disease vintage, cumulative steroid dose, body mass index, and presence of arterial hypertension may also be predisposing factors.â©.
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Artéria Carótida Primitiva/diagnóstico por imagem , Espessura Intima-Media Carotídea , Hipertensão/complicações , Síndrome Nefrótica/complicações , Adolescente , Anti-Inflamatórios/administração & dosagem , Pressão Arterial , Aterosclerose/diagnóstico por imagem , Aterosclerose/etiologia , Índice de Massa Corporal , Estudos de Casos e Controles , Criança , Pré-Escolar , Estudos Transversais , Feminino , Humanos , Hipertensão/fisiopatologia , Masculino , Síndrome Nefrótica/tratamento farmacológico , Prednisona/administração & dosagem , Recidiva , Estudos Retrospectivos , Fatores de Risco , Fatores de TempoRESUMO
Acute poststreptococcal glomerulonephritis (APSGN) is a complication of infection with group A beta-hemolytic streptococcus. The disease manifests as microscopic/gross hematuria, arterial hypertension, edema, and acute kidney injury and has most commonly self-limiting course. AIM: The aim of study was the analysis of clinical course of APSGN in period of increased incidence in the first half of 2018. MATERIALS AND METHODS: We analyzed following parameters in children hospitalized due to APSGN in January-June 2018: age, sex, anthropometric parameters, preceding infection, clinical signs, renal function, biochemical and immunological tests (including antristreptolysins (ASO) and complement), urinalysis, renal ultrasonography, and treatment. The incidence of APSGN in years 2007-2018 was analyzed. RESULTS: We found 11 children (6 boys, 5 girls) aged 5.01±2.44 years. The disease was preceded by pharyngitis in 8, skin infection in 1 with latent period 16.40±5.77 days. Clinical symptoms were: gross hematuria in 8, edema in 6, hypertension in 5, renal function impairment 6, and hyperkalemia in 5; all patients had lowered C3 complement factor; ASO was elevated in all patients except for a boy with skin infection. During hospitalization clinical symptoms resolved in all children; significant elevation in GFR (p=0.018) and C3 (p=0.034), and decrease in proteinuria (p=0.039) were observed. Four patients with abnormal ultrasonographic kidney image were characterized by worse kidney function (p=0.018), higher potassium concentration (p=0.052), higher proteinuria (p=0.073) and erythrocyturia (p=0.015) than remaining children. In follow-up (after 142,00±89,20days) all children had normal renal function and blood pressure, 1 patient had proteinuria, and 4 had erythrocyturia. CONCLUSIONS: In most cases APSGN is characterized by rapid resolution of symptoms and good prognosis, but patients require periodic follow-up visits. Abnormal initial ultrasonographic kidney image may be a marker of worse clinical course of APSGN.
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Glomerulonefrite , Infecções Estreptocócicas , Doença Aguda , Criança , Pré-Escolar , Feminino , Glomerulonefrite/complicações , Glomerulonefrite/diagnóstico , Glomerulonefrite/tratamento farmacológico , Humanos , Rim/microbiologia , Masculino , Proteinúria/etiologia , Infecções Estreptocócicas/complicações , Infecções Estreptocócicas/diagnóstico , Infecções Estreptocócicas/tratamento farmacológicoRESUMO
Distal renal tubular acidosis is a defect of acidification of urine in distal tubule. Full-blown form is characterized by polyuria, growth deficiency, nephrolithiasis or nephrocalcinosis. Mutations in genes encoding Cl-/HCO3 - exchanger (autosomal dominant) or H+-ATPase (autosomal recessive) are the most frequent in children. A CASE REPORT: In a boy aged 2,5 years, healthy, with proper development, metabolic acidosis with hyperchloremia, hypokalaemia, normal glomerular filtration rate and alkaline urine was discovered during hospitalization because of pneumonia. USG showed normal length kidney with nephrocalcinosis type IIB. The family history revealed nephrocalcinosis on the part of the boy's father. He also had metabolic acidosis in blood gas test. Genetic test in a boy and his father showed mutation of SLC4A1(17q21-q22) gene encoding Cl-/HCO3 - exchanger. The boy was treated with 8,4% NaHCO3 - orally (1mEq/kg/24h) and KCl (0,3 mEq/kg/24h). We obtained normalization of blood gas test and potassium concentration. CONCLUSIONS: Every child, with accidentally discovered metabolic acidosis, even with normal development, should be diagnosed in case of renal tubular acidosis. Electrolytes, gas blood test, urinalysis and USG are needed in the closest family members of child with diagnosed renal tubular acidosis.
Assuntos
Acidose Tubular Renal , Hipopotassemia , Nefrocalcinose , Acidose Tubular Renal/diagnóstico , Acidose Tubular Renal/genética , Pré-Escolar , Taxa de Filtração Glomerular , Humanos , Hipopotassemia/diagnóstico , Masculino , Mutação , Nefrocalcinose/diagnóstico , Nefrocalcinose/genéticaRESUMO
INTRODUCTION: Cerebral salt wasting syndrome (CSWS), characterized by natriuresis, polyuria, and hypovolemia, is a rare complication of central nervous system injury or disease. A CASE STUDY: 12-year-old girl was admitted with second attack of nephrotic syndrome (NS). On admission she presents with edema, blood pressure 110/60 mm Hg, proteinuria 145 mg/kg/24h, hypoalbuminemia (1.7 g/dL), GFR 94.4 mL/min/1.73m2, sodium 133 mmol/L. On 5th day the patient developed thrombosis of right subclavian and axillary vein and was treated with recombinant tissue plasminogen activator (0.3 mg/kg/h i.v.). 45 minutes after onset of the infusion severe headache appeared. Computed tomography revealed subarachnoid hemorrhage in a region of left occipital lobe and posterior 1/3 part of sickle of the brain. Control ultrasonography examination revealed resolution of the thrombus. No deficits were found on neurologic examination. Proteinuria subsided on 11th day of hospitalization. After the hemorrhage hypovolemia, hypotension (80/40 - 100/60 mm Hg, heart rate 100/min), polyuria, and pathologic natriuresis (up to 13.0 mmol/kg/24h) were observed. Cerebral salt wasting syndrome was recognized. The girl was supplemented with oral and intravenous sodium (up to 10 mmol/ kg/24h). In following days gradual decrease of diuresis and urinary sodium loss was observed. The patient was discharged home after 41 days with normal diuresis (1.5l/24h) and natriuresis (1.44 mmol/kg/24h). CONCLUSIONS: Treatment of thromboembolic complications in children with NS poses a risk of central nervous system bleeding. Serum sodium concentration and diuresis must be strictly monitored in patients with central nervous system lesion, especially in the course of nephrotic syndrome.