Thrombotic complications in children with first-episode steroid-sensitive nephrotic syndrome: A single-center experience.

OBJECTIVE: The aim of the study was to evaluate the clinical course and risk factors of venous thromboembolic complications (VTEC) in children with a first episode of steroid-sensitive nephrotic syndrome (SSNS). MATERIALS AND METHODS: We retrospectively analyzed the medical records of children hospitalized due to SSNS in one pediatric nephrology unit between 2012 and 2019. Demographic data, clinical symptoms at the onset of NS, and laboratory parameters were compared between patients with and without VTEC. RESULTS: Among 106 children (4.7 ± 3.06 years of age) with a first episode of SSNS, 5 VTEC were diagnosed during 2 - 60 days after onset of NS, on the basis of clinical symptoms and/or results of imaging studies. These were thromboses of femoral vein, central part of the kidney, dorsal veins of the hand, venous sinuses of the brain, and superficial vein in the popliteal fossa region. We found significant higher serum fibrinogen level (p = 0.022) and D-dimers (p = 0.0001) in children with VTEC vs. those without VTEC, but AUC analysis showed that only D-dimers significantly differentiate thrombosis. The clinical risk factors of VTEC were vascular cannulation (100%), infections (80%), and diuretics (80%). In children with VTEC, low molecular weight heparin was used. The outcome was a full recovery in all patients. CONCLUSION: VTEC occurs in 4.72% of children with a first episode of SSNS. The course of VTEC in children with SSNS may be asymptomatic. The clinical risk factors of VTEC in children with SSNS are vascular cannulation, infections, and diuretics. High D-dimer levels are a sensitive indicator of thrombosis.

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.

COVID-19-Vaccination-Induced Myocarditis in Teenagers: Case Series with Further Follow-Up.

Presently, the whole globe is struggling the tough challenge of the COVID-19 pandemic. Vaccination remains the most effective and safe COVID-19 weapon for adults and in the paediatric population. Aside from possible mild and moderate post-vaccination side effects, more severe side effects may occur. We retrospectively analysed a group of 5 teenagers aged from 15 to 17 years with obesity/overweight (BMI ranging from 24.8 to 30) who presented typical myocarditis symptoms following the first or second dose (3 and 2 patients, respectively) of the COVID-19 vaccine. In the whole study group, a significant increase in troponin serum concentration was observed (1674-37,279.6 ng/L) with a further quick reduction within 3-4 days. In all patients, ST segments elevation or depression with repolarisation time abnormalities in electrocardiography were noticed. Chest X-ray results were within normal limits. Echocardiography showed normal left ventricular diameter (47-56.2 mm) with ejection fraction between 61-72%. All patients were diagnosed with myocarditis based on cardiac magnetic resonance (CMR) imaging. During further hospitalisation, swift clinical improvement was notable. Follow-up in the whole study group was obtained after 106-134 days from initial CMR, revealing no myocarditis symptoms, proper troponin level, and no ECG or echocardiographic abnormalities. At the same time, persistent myocardium injury features were detected in the whole study group, including ongoing myocarditis. COVID-19-vaccine-induced myocarditis seems to be a mild disease with fast clinical recovery, but the complete resolution of the inflammatory process may last over 3 months. Further follow-up and investigation for assessing subsequent implications and long-term COVID-19-vaccine-induced myocarditis is required.

Cardiac Magnetic Resonance Follow-Up of Children After Pediatric Inflammatory Multisystem Syndrome Temporally Associated With SARS-CoV-2 With Initial Cardiac Involvement.

BACKGROUND: Pediatric inflammatory multisystem syndrome temporally associated with SARS-CoV-2 (PIMS-TS) is an inflammatory disease occurring in a small minority of children a few weeks after acute infection. Cardiac manifestations are common, but little is known about the potentially persistent heart changes after PIMS-TS. PURPOSE: To analyze the frequency and type of myocardial complications of PIMS-TS with initial cardiac involvement assessed with cardiac magnetic resonance imaging (MRI), including parametric imaging, performed 3 months after hospitalization. STUDY TYPE: Retrospective. POPULATION: Nineteen consecutive children (median age 10 years, interquartile range (IQR) 10-15 years, 74% male). FIELD STRENGTH/SEQUENCE: Balanced steady state free precession (bSSFP, cine imaging), modified Look-Locker (T1 mapping), T2-prepared bSSFP (T2-mapping), dark-blood T2-weighted turbo spin echo with fat suppression and phase sensitive inversion recovery (late gadolinium enhancement (LGE)) sequences at 1.5 T. ASSESSMENT: Patients were scanned after a median of 99 days (IQR 89-104 days) from the diagnosis. MR data were reviewed by three independent observers, with 13, 2, and 5 years' experience in cardiac MRI. Pre- and post-contrast T1, T2, extra-cellular volume, and T2 signal intensity (T2 SI) ratio were calculated. Diagnosis of acute myocarditis was based on modified Lake Louise criteria. Cardiac MRI parameters were compared, where possible, to previously published pediatric normal values. STATISTICAL TESTS: Interclass correlation coefficient and Bland-Altman repeatability analysis. A P-value <0.05 was considered statistically significant. RESULTS: Despite cardiac involvement including decreased left ventricular ejection fraction (LVEF) (median LVEF = 47%, IQR 43%-53%) and increased troponin I (median 101 ng/mL, IQR 50-661 ng/mL) during hospitalization, there were no persistent cardiac changes observed in cardiac MR at follow-up. All patients had normal size and function of the left ventricle and normal precontrast T1 and T2 relaxation times. There were no signs of LGE. Persistent, mild pericardial effusion (8-9 mm) was found in three (16%) patients. DATA CONCLUSION: There were no persistent changes on cardiac MRI in a group of children approximately 3 months post hospitalization due to PIMS-TS with cardiac involvement. This supports the hypothesis that cardiac involvement during PIMS-TS is a form of transient inflammatory response rather than direct and potentially persistent injury from the virus. LEVEL OF EVIDENCE: 4 TECHNICAL EFFICACY: Stage 3.

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.

Invitation to participate in a multi-center study for validation of cerebral computed tomography angiography and computed tomography perfusion in the determination of cerebral circulatory arrest during brain death/death by neurological criteria diagnosis procedure in paediatric population below 12 years of age.

We would like to invite paediatric intensive care units (PICU) to join our multi-center trial concerning patient population < 12 y/o and aiming at: • validation of computed tomography angiography (CTA)/computed tomography perfusion (CTP) tests for brain death/death by neurological criteria (BD/DNC) diagnosis procedures, • validation of duplex Doppler insonation of extracranial segments of the internal cerebral arteries and the vertebral arteries for choosing an optimal time for CTA/CTP testing.

The Role of Cardiac Magnetic Resonance in Evaluation of Idiopathic Ventricular Arrhythmia in Children.

The aim of the study was to assess the role of cardiovascular magnetic resonance (CMR) in the diagnosis of idiopathic VA in children. This retrospective single-centre study included a total of 80 patients with idiopathic ventricular arrhythmia that underwent routine CMR imaging between 2016 and 2020 at our institution. All patients underwent a 3.0 T scan involving balanced steady-state free precession cine images as well as dark-blood T2W images and assessment of late gadolinium enhancement (LGE). In 26% of patients (n = 21) CMR revealed cardiac abnormalities, in 20% (n = 16) not suspected on prior echocardiography. The main findings included: non-ischemic ventricular scars (n = 8), arrhythmogenic right ventricular cardiomyopathy (n = 6), left ventricular clefts (n = 4) and active myocarditis (n = 3). LGE was present in 57% of patients with abnormal findings. Univariate predictors of abnormal CMR result included abnormalities in echocardiography and severe VA (combination of >10% of 24 h VA burden and/or presence of ventricular tachycardia and/or polymorphic VA). CMR provides valuable clinical information in many cases of idiopathic ventricular arrhythmia in children, mainly due to its advanced tissue characterization capabilities and potential to assess the right ventricle.

[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.

Lung ultrasound-a new diagnostic modality in persistent tachypnea of infancy.

Lung ultrasound (LUS) has been increasingly used in diagnosing and monitoring of various pulmonary diseases in children. The aim of the current study was to evaluate its usefulness in children with persistent tachypnea of infancy (PTI). This was a controlled, prospective, cross-sectional study that included children with PTI and healthy subjects. In patients with PTI, LUS was performed at baseline and then after 6 and 12 months of follow-up. Baseline results of LUS were compared to (a) baseline high-resolution computed tomography (HRCT) images, (b) LUS examinations in control group, and (c) follow-up LUS examinations. Twenty children with PTI were enrolled. B-lines were found in all children with PTI and in 11 (55%) control subjects (P < .001). The total number of B-lines, the maximal number of B lines in any intercostal space, the distance between B-lines, and pleural thickness were significantly increased in children with PTI compared to controls. An irregularity of the pleural line was found in all patients with PTI and in none of the healthy children. There were no significant changes in LUS findings in patients with PTI during the study period. The comparison of HRCT indices and LUS findings revealed significant correlations between the mean lung attenuation, skewness, kurtosis and fraction of interstitial pulmonary involvement, and the number of B-lines as well as the pleural line thickness. LUS seems to be a promising diagnostic tool in children with PTI. Its inclusion in the diagnostic work-up may enable to reduce the number of costly, hazardous, and ionizing radiation-based imaging procedures.

Children With Acute Myocarditis Often Have Persistent Subclinical Changes as Revealed by Cardiac Magnetic Resonance.

BACKGROUND: Many children presenting with myocarditis may not fully recover and have long-term complications, including dilated cardiomyopathy. Magnetic resonance imaging (MRI) has a potential for early detection of persistent changes with long-term implications, but is not performed routinely in the monitoring of myocarditis. PURPOSE: To monitor adolescents who present with acute myocarditis using MRI and routine diagnostic tests over the short- to mid-term. STUDY TYPE: Prospective. POPULATION: Eighteen consecutive adolescents (median age 15.5, interquartile range 14.8-16.9 years, 78% male) with acute myocarditis. FIELD STRENGTH: A 3T scanner including cine steady-state free precession (SSFP), dark-blood T2 -weighted (T2 W) images, and late gadolinium enhancement (LGE). ASSESSMENT: The diagnosis of acute myocarditis was based on clinical symptoms and signs and MRI criteria (cine, T2 -W images, LGE). Follow-up MRI was performed after median 7 months (range 6-9 months). Other routine diagnostic tests included electrocardiogram (ECG), high-sensitivity troponin levels, transthoracic echocardiography, and Holter monitoring. STATISTICAL TESTS: Fisher's exact test, Wilcoxon test for paired samples, Mann-Whitney test for independent samples, Kruskal-Wallis test. RESULTS: At baseline, 17 patients (94%) had elevated troponin levels and/or ST-T changes on resting ECG; ECG showed depressed left ventricular ejection fraction (LVEF<50%) in four patients (22%). At follow-up there was a complete recovery in 16 patients (89%) observed with routinely performed tests, with two cases of persistent ventricular arrhythmia. Despite normal left ventricular volume and LVEF, MRI disclosed ongoing active inflammation in five patients (28%), healed myocarditis with persistent scars in eight patients (44%), and complete resolution of initially observed changes in five patients (28%). DATA CONCLUSION: In children with acute myocarditis, despite normalization of other routinely assessed parameters (including LVEF), there is a high prevalence of persistent MRI changes showing ongoing disease or remnant scars at follow-up. MRI may allow early detection and prevention of long-term complications of myocarditis in the follow-up care of children with acute myocarditis. LEVEL OF EVIDENCE: 2 Technical Efficacy: Stage 3 J. Magn. Reson. Imaging 2020. J. Magn. Reson. Imaging 2020;52:488-496.

Massive thrombosis in an infant with suspected nephrocalcinosis: case report and literature review.

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.

Normal values of native T1 and T2 relaxation times on 3T cardiac MR in a healthy pediatric population aged 9-18 years.

BACKGROUND: Native myocardial T1 and T2 relaxation times are diagnostic tools used in clinical practice for adult and pediatric populations. Use of a mapping technique requires accurate knowledge of normal ranges in healthy patients, which is lacking in pediatric populations. PURPOSE: To establish normal values for native T1 and T2 mapping in healthy pediatric subjects of different ages and sex. STUDY TYPE: Prospective. POPULATION: Thirty-eight healthy children (9-18 years; mean age 14.0 ± 2.7). FIELD STRENGTH: Cardiac MR with a 3T scanner. T1 and T2 mapping using MyoMaps software. ASSESSMENT: T1 and T2 relaxation times were calculated from a 0.7-1.0 cm2 region of interest placed at the mid-ventricular short-axis slice in the interventricular septum by two observers. Inter- and intraobserver variability was assessed. STATISTICAL TESTS: The Student's t-test or the Mann-Whitney test for unpaired samples was applied to compare one continuous variable between two category groups. One-way analysis of variance (ANOVA) or a Kruskal-Wallis test was applied to compare one continuous variable between three category groups. Correlation between two continuous variables was assessed with a Pearson or Spearman test. RESULTS: The mean native T1 relaxation time was 1223 ± 29 msec and T2 relaxation time was 43 ± 4.5 msec. There was no correlation between T1 /T2 values and age or body surface area (for T1 P = 0.94 and 0.90 and for T2 P = 0.19 and 0.64, respectively). There was weak correlation between T1 values and body mass index (BMI) (r = 0.448, P = 0.005). T2 values were significantly higher in females compared with males (44.6 ± 4.2 vs. 40.4 ± 3.8 msec, P = 0.002). We found a significant rise of T2 relaxation time in the pubertal period (age 13-15 years) comparing to prepubertal (age 9-12 years). Inter- and intraobserver agreement of T1 (r = 0.93; r = 0.99) and T2 (r = 0.96; r = 0.95) were high. DATA CONCLUSION: We report normal values of native T1 and T2 relaxation times obtained with Myomaps software for 3T cardiac MR in a healthy pediatric population. LEVEL OF EVIDENCE: 2 Technical Efficacy: Stage 2 J. Magn. Reson. Imaging 2020;51:912-918.

[Evaluation of renal lesions in children with tuberous sclerosis - summary of the first year of follow-up program]. / Ocena zmian w nerkach u dzieci chorych na stwardnienie guzowate ­ podsumowanie pierwszego roku programu opieki.

Tuberous sclerosis complex (TSC) is a genetic disease that leads to formation of tumors i.e. in brain kidneys, heart, lungs, and skin. AIM: The aim of the study was to summarize center's experience in the first year of program of nephrologic follow-up in patients with TSC. MATERIALS AND METHODS: During 12 months 30 children with TSC (14 boys and 16 girls aged from 3 months to 17 years 11 months, mean 7.57±5.02 years) were hospitalized. Following parameters were evaluated: genetic and biochemical tests, blood pressure in ambulatory blood pressure monitoring (ABPM), kidney lesions in ultrasonography (30 patients) and in magnetic resonance (14 patients). RESULTS: Genetic tests were performed in 6 children - in 5 TSC2 mutation was found, in one boy with TSC and numerous renal cysts only PKD1 mutation was revealed. Mean GFR was 130.81±23.23 mL/ min/1.73 m2. Four children (13.3%) had arterial hypertension. Renal lesions were found in 28 (93.3%) children: 18 patients had angiomyolipomas (AML) (mean diameter 15.4±12.5, max 38 mm), 23 patients had renal cysts (mean diameter 7.6±7.0, max 30 mm); 13 patients had AMLs and cysts. A dysplastic lesion (39x26x15 mm) in right kidney was found in one girl. Children with AML were older than remaining patients (10.08±4.55 vs. 4.25±3.50 [years], p<0.001). Children with cysts were characterized by higher systolic (p=0.017), diastolic (p=0.027) and mean (p=0.014) arterial pressure, and mean arterial pressure Z-score (p=0.025) in ABPM. Maximal kidney cyst diameter correlated positively with systolic, diastolic, mean arterial pressure, mean arterial pressure Z-score, and diastolic blood pressure load in ABPM (r = 0.61-0.75, p = 0.033-0.005). Two children with numerous AML with diameter >30 mm were treated with sirolimus. CONCLUSIONS: Because of common focal lesions in kidneys children with TSC should be kept under regular nephrologic follow-up. Presence of large renal cysts may predispose children with TSC to arterial hypertension.

[Cerebral salt wasting syndrome as a complication of subarachnoid hemorrhage in a girl with nephrotic syndrome - a case report]. / Mózgowy zespól utraty soli jako powiklanie krwawienia podpajeczynówkowego u dziewczynki z zespolem nerczycowym ­ opis przypadku.

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.

Cardiovascular magnetic resonance with parametric mapping in long-term ultra-marathon runners.

PURPOSE: There is a direct reverse dose-effect relationship between the amount of physical activity and cardiovascular risk. It is unknown whether this is true for extreme, persistent endurance training. The aim of the study was to assess structural changes of the heart in long-time ultra-marathon runners with special focus on myocardial fibrosis using parametric mapping. METHOD: We studied a group of 30 healthy, male ultra-marathon runners (mean age 40.9 ±â€¯6.6 yrs, median 9 yrs of running with frequent competitions) and 10 matched controls not engaged in any regular activities. All of them underwent cardiovascular magnetic resonance (CMR) with 3 T scanner including T1-mapping, late gadolinium enhancement (LGE) and extracellular volume (ECV) quantification. RESULTS: Athletes demonstrated significantly larger heart chambers and left ventricular (LV) mass. LV systolic function was unchanged. 73.3% of athletes fulfilled volumetric criteria for dilated cardiomyopathy or arrhythmogenic right ventricular cardiomyopathy. Non-ischemic, small volume LGE was found in 8 athletes and in 1 control (27% vs. 10%, p = 0.40). It was localised at insertion points (5 athletes, 1 control) or in the septum or infero-lateral wall (3 athletes). Athletes with insertion point LGE had higher right ventricular end-diastolic volume index in comparison to athletes without LGE (p = 0.04), which suggests its relation to volume overload. There were no differences between athletes and non-athletes in terms of ECV values (26.1% vs. 25%, p = 0.29). CONCLUSIONS: Ultra-marathon runner's hearts demonstrate a high degree of structural remodelling, but there is no significant increase in focal or diffuse myocardial fibrosis.

Carotid intima-media thickness in children with idiopathic nephrotic syndrome: A single center cross-sectional study
.

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.
.

Neonatal idiopathic aneurysm of the common iliac artery.

Arterial aneurysms are very rare in children, with iliac aneurysms being even more uncommon. We report the case of a 61-day-old girl with a right common iliac artery aneurysm that was an incidental finding on the third day of life. The aneurysm was treated by direct aneurysm puncture and endovascular coil embolization. The patient was discharged with no complications, and complete exclusion of the aneurysm was confirmed on the 3-month follow-up examination.

[Zwiekszenie czestosci zachorowania na ostre popaciorkowcowe klebuszkowe zapalenie nerek w pierwszej polowie roku 2018 - doswiadczenie jednego osrodka]. / Increased frequency of acute poststreptococcal glomerulonephritis in the first half of 2018 ­ single-center experience.

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.

[Accidentally diagnosed distal renal tubular acidosis with nephrocalcinosis - a case report]. / Przypadkowo rozpoznana kwasica kanalikowa dystalna z nefrokalcynoza ­ opis przypadku.

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.