Gadolinium-based contrast agents (GBCAs) are widely used in magnetic resonance imaging (MRI) to help with the diagnostic and monitoring processes of many diseases, including neurological disorders. Initially, it was assumed that GBCAs carry minimal risk, are safe and well tolerated. But recent reports of GBCA-associated deposition in many body tissues have raised concerns about the broader health impacts of gadolinium exposure. The aim of this review was to summarise knowledge regarding gadolinium deposition, primarily in the brain structures, and of potential GBCA-associated toxicity. Moreover, we discuss the current recommendations on the use of GBCAs, as well as alternative contrast agents and imaging techniques.

A 19-month-old, previously healthy girl was admitted to a regional hospital due to a high fever refractory to empirical antibiotic therapy, cough, and dyspnoea. Laboratory markers of inflammation were markedly elevated. A computed tomography demonstrated enlarged mediastinal and hiatal lymph nodes and the presence of fluid in the pericardium. Transthoracic echocardiography performed in our hospital demonstrated, as well as pericardial effusion, a "banana-like" shaped encapsulated mass (50 × 20 mm) in the pericardium adjacent to the lateral left ventricular (LV) wall. LV systolic function was preserved; there was no constrictive physiology or evident signs of pericardial tamponade.

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.

Jednostronna agenezja nerki może występować jako wada izolowana lub współistnieć z innymi anomaliami rozwojowymi układu moczowego. Najczęściej rozpoznawany jest odpływ pęcherzowo-moczowodowy i wodonercze, rzadziej moczowód olbrzymi, zdwojenie moczowodu, ureterocele, ektopia nerki i spodziectwo. O P I S P R Z Y P A D K U : W pracy opisano przypadek chłopca, u którego w wieku 2,5 roku rozpoznano złożoną wadę układu moczowego-agenezję nerki lewej i ektopię nerki prawej z wodonerczem i moczowodem olbrzymim. Badanie USG wykonano z powodu bólów brzucha. Wcześniej chłopiec nie demonstrował żadnych objawów klinicznych, które mogłyby wskazywać na istnienie wady układu moczowego. Na podstawie badania izotopowego, urografii i cystouretrografii mikcyjnej rozpoznano zwężenie przypęcherzowe moczowodu. W wieku 3 lat wykonano operację przeszczepienia moczowodu metodą Cohena. Pomimo usunięcia przeszkody w odpływie moczu, utrzymywało się znacznego stopnia wodonercze ze zwężeniem warstwy miąższowej nerki. W wieku 11,5 roku u chłopca rozpoznano nadciśnienie tętnicze, w wieku 14,5 roku stwierdzono niewielki wzrost stężenia kreatyniny w surowicy i izostenurię. W ciągu 12 lat obserwacji badania ogólne i posiewy moczu były prawidłowe. W N I O S K I: Dziecko z jednostronną agenezją nerki i uropatią zaporową w drugiej nerce wymaga regularnej kontroli w poradni nefrologicznej, monitorowania ciśnienia tętniczego krwi i czynności nerki. Obecność anomalii rozwojowej w jedynej nerce może prowadzić do rozwoju przewlekłej choroby nerek i schyłkowej niewydolności nerek.

BACKGROUND AND AIMS Adult data show that in people with tuberous sclerosis complex (TSC), blood pressure is related to the extent of renal involvement. Central blood pressure and 24-h ambulatory blood pressure are stronger predictors of a cardiovascular burden compared to office peripheral blood pressure. The study aimed to analyse 24-h central blood pressure and its relation with renal involvement in paediatric patients with TSC. METHOD In a group of 32 children with TSC (9.6 ± 4.4 years, 16 boys, 16 girls), we evaluated peripheral and central 24-h ambulatory blood pressure, office peripheral blood pressure, medications used, renal lesions in magnetic resonance and ultrasonography, including the presence of angiomyolipoma (AML), atypical AML, cysts and their maximal diameter (mm) and laboratory parameters. RESULTS In the studied group, 20 (62.5%) children had renal angiomyolipomas, including 5 (15.6%) with atypical angiomyolipomas, 22 (68.8%) with renal cysts and 4 children (12.5%) were treated with mTOR inhibitors (2 patients with everolimus and 2 patients with sirolimus) at the moment of evaluation, glomerular filtration rate (GFR) was 127.4 ± 36.7, one patient had GFR 80.5, 22 patients had GFR between 90 and 139 and 9 patients had hyperfiltration defined as GFR > 140 mL/min/1.73 m2. A total of 4 (12.5%) children had elevated blood pressure in ABPM. The 24-h central systolic and diastolic blood pressure correlated with maximal diameter of renal cysts (mm) (r = 0.359, P = .044; r = 0.466, P = .029) and urinary albumin loss (mg/24-h) (r = 0.511, P = .004; r = 0.478, P = .021), 24-h central systolic, mean blood pressure and pulse pressure with serum cystatin-C (ng/mL) (r = 0.523, P = .005; r = 0.464, P = .015; r = 0.419, P = .029); 24-h peripheral MAP Z-score correlated with cyst size (mm) (r = 0.571, P = .021). No significant correlations between presence and AML size and peripheral and central blood pressure were found in the study group. There were following positive correlations of arterial stiffness parameters: 24-h central augmentation index (24hcAI x 75HR) with total cholesterol (r = 0.357, P = .045) and ambulatory arterial stiffness index (AASI) with uric acid (r = 0.520, P = .003). Size of AML correlated with age (r = 0.468, P = .007), whereas there was no correlation between cyst size and age (r = 0.145, P = .416). CONCLUSION