A Pilot Comparative Study between Creatinine- and Cystatin-C-Based Equations to Estimate GFR and Kidney Ultrasound Percentiles in Children with Congenital Anomalies of the Kidney and Urinary Tract.

Congenital anomalies affecting the kidneys present significant challenges in pediatric nephrology, needing precise methods for assessing renal function and guiding therapeutic intervention. Bedside Schwartz formula with the cystatin-C-based Full Age Spectrum formula and Chronic Kidney Disease in Children (CKiD) U 25 formula used in estimating glomerular filtration rate (eGFR) and also to assess if the eGFR in association with kidney length percentiles can be a monitoring parameter for the progression of chronic kidney disease in children with congenital anomalies of the kidney and urinary tract (CAKUT). A total of 64 pediatric patients (median age at diagnostic was 12 months with an interquartile range of 2 to 60) were diagnosed with congenital anomalies in the kidney and urinary tract between June 2018 and May 2023 at "Louis Turcanu" Emergency Hospital for Children in Timisoara, Romania. Baseline characteristics, CAKUT types, associated pathologies, CKD staging, and eGFR using creatinine and cystatin C were analyzed. The mean age at the moment of examination was 116.50 months; (65, 180). Chronic kidney disease staging revealed a predominance of patients in CKD stages G1 and A1. Analysis of eGFR methods revealed a small mean difference between eGFR estimated by creatinine and cystatin C, with a moderate-strong positive correlation observed between the eGFR and ultrasound parameters. Using cystatin-C-based formulas for eGFR, in conjunction with ultrasound measurements, may offer reliable insights into renal function in pediatric patients with congenital anomalies affecting the kidney and urinary tract. However, the economic aspect must be taken into consideration because cystatin C determination is approximately eight times more expensive than that of creatinine. An interdisciplinary approach is crucial for managing patients with CAKUT.

Factors Impacting the Reduction in Neophobia Prevalence in Phenylketonuria Patients.

Food neophobia (FN), the fear of sampling new foods, can have a significant impact on children's eating habits. Children with phenylketonuria (PKU), a hereditary condition that inhibits the body's capacity to metabolize phenylalanine, should take this attitude with caution. Patients with PKU must follow a rigorous phenylalanine (Phe)-restricted diet to avoid brain malfunction that can include intellectual disability, seizures, and behavioral difficulties. The novelty of our work stems from the fact that we explored the origins of this incorrect intake pattern, which exacerbates PKU patients' already fragile health. We conducted a cross-sectional study on 34 previously diagnosed phenylketonuria patients and a control group ranging in age from 7 months to 40 years, with a sex ratio of M/F 2:1. The Food Neophobia Scale (FNS) was used to determine neophobia. We used JASP (version 0.18.1) statistical analysis to examine the relationship between neophobia and PKU condition, age and nutritional status at the time of study, diet compliance, parental educational level, period from birth to PKU diagnosis, and environmental (rural/urban) provenience of PKU patients. According to the data, 61.76% of patients with PKU were neophobic, as were 70.57% of the control group. Food neophobia was associated with PKU patients' present age, the period from birth to PKU diagnosis, and parental educational level.

Impact of Cancer Type and Treatment Protocol on Cardiac Function in Pediatric Oncology Patients: An Analysis Utilizing Speckle Tracking, Global Longitudinal Strain, and Myocardial Performance Index.

Pediatric hemato-oncology patients undergoing anthracycline therapy are at risk of cardiotoxicity, with disease type and treatment intensity potentially affecting cardiac function. Novel echocardiographic measures like speckle tracking echocardiography (STE), global longitudinal strain (GLS), and the myocardial performance index (MPI) may predict early changes in cardiac function not detected by traditional methods. This study aimed to assess the impact of cancer type and treatment protocol on these parameters and their potential in predicting long-term cardiac complications. We conducted a single-center, retrospective cohort study of 99 pediatric oncology patients and 46 controls that were assessed at 3, 6, and 12 months. The median age was 10.7 ± 4.4 years for cases and 10.2 ± 3.6 years for controls. STE, GLS, and MPI were measured, and statistical analyses were performed to determine any significant correlations with cardiotoxicity. Significant variations were observed in traditional cardiac function measurements between the patient and control groups, with a lower average ejection fraction (EF) of 62.8 ± 5.7% in patients vs. 66.4 ± 6.1% in controls (p < 0.001), poorer GLS of -16.3 ± 5.1 in patients compared to -19.0 ± 5.4 in controls (p = 0.004), and higher MPI values of 0.37 ± 0.06 in patients compared to 0.55 ± 0.10 in controls, indicating worse overall cardiac function (p < 0.001). However, differences in cardiac function measurements by cancer histology or treatment protocol were not statistically significant. Regression analyses showed that the combination of GLS, SMOD, and MPI increased the odds of cardiac toxicity with an odds ratio of 7.30 (95% CI: 2.65-12.81, p < 0.001). The study underscores the predictive value of the combined GLS, SMOD, and MPI measurements in pediatric oncology patients undergoing anthracycline treatment for cardiotoxicity. Although variations across cancer types and treatment protocols were not significant, the study emphasizes the potential utility of these novel echocardiographic measures in early detection and long-term prediction of anthracycline-induced cardiotoxicity. Further studies in larger, multi-center cohorts are required for validation.

Correlation of Speckle-Tracking Echocardiography with Traditional Biomarkers in Predicting Cardiotoxicity among Pediatric Hemato-Oncology Patients: A Comprehensive Evaluation of Anthracycline Dosages and Treatment Protocols.

Speckle tracking-echocardiography (STE) is a novel non-invasive imaging tool capable of quantifying myocardial deformation, and thus holds promise in detecting early subclinical myocardial injury. This study aimed to evaluate the correlation of STE with traditional biomarkers in predicting anthracycline-induced cardiotoxicity in the context of varying dosages and treatment protocols in pediatric hemato-oncology patients. We conducted a retrospective study involving pediatric hemato-oncology patients undergoing anthracycline-based chemotherapy. A total of 99 patients were included in the final analysis, with 82 receiving Doxorubicin, of which 58.5% were males, and 17 receiving Epirubicin, of which 70.6% were males, with a median of 10 years old. Traditional biomarkers, such as Troponin I (cTnI) and B-type natriuretic peptide (BNP), were compared with STE parameters, including the global longitudinal strain (GLS), Simpson method of discs (SMOD), and myocardial performance index (MPI). A comprehensive evaluation was conducted based on different dosages of anthracyclines and different treatment protocols, with a follow-up period of one year post-chemotherapy. It was observed that the cTnI levels in the Doxorubicin group were significantly higher (3.2 ng/mL, p = 0.002) than in the Epirubicin group (2.7 ng/mL). However, BNP and NT-proBNP levels were not significantly different between the two groups (p = 0.096 and p = 0.172, respectively). Regarding STE parameters, a significant negative correlation was observed between the anthracycline dose and GLS (Rho = -0.411, p = 0.001), indicating increased cardiotoxicity with dose elevation. The SMOD and MPI gave significantly better values in the Epirubicin group (59.2 and 0.41 vs. 54.4 and 0.36, respectively). However, the ROC analysis did not find GLS, SMOD, or MPI to be significant independent predictors of cardiotoxicity (p > 0.05). There was also considerable variation in cardiotoxicity between the Doxorubicin and Epirubicin study groups, suggesting that the risk of cardiotoxicity is not solely determined by dose. Our study underlines the potential of STE as a sensitive tool for the early detection and prediction of anthracycline-induced cardiotoxicity in pediatric hemato-oncology patients, but only in association with the clinical findings and cardiac biomarkers. While traditional biomarkers still play a role, STE can offer a more accurate prediction of cardiac risk, potentially leading to better management and outcomes for these patients.

Renal Impairment Impact and Survival Analysis in a Romanian Cohort of HIV-1(F1)-Infected Children and Adolescents.

Human immunodeficiency virus (HIV) is a lentivirus that is transmissible through blood and other body fluids. During the late 1980s and early 1990s, an estimated 10,000 Romanian children were infected with HIV-1 subtype F nosocomially through contaminated needles and untested blood transfusions. Romania was a special case in the global acquired immunodeficiency syndrome (AIDS) pandemic, displaying the largest population of HIV-infected children by parental transmission between 1987-1990. In total, 205 HIV-infected patients from the western part of Romania were analyzed in this retrospective study. Over 70% of them had experienced horizontal transmission from an unknown source, while vertical transmission was identified in only five cases. Most patients had a moderate to severe clinical manifestation of HIV infection, 77.56% had undergone antiretroviral (ARV) treatment, most of them (71.21%) had experienced no adverse reactions and many of those with HIV (90.73%) had an undetectable viral load. Renal impairment was detected in one third of patients (34.63%). Patients born before 1990, male patients, patients diagnosed with HIV before the age of 10, and those undernourished or with renal impairment had a shorter average survival time than the group born after 1990, female patients, patients receiving ARV treatment, patients with a normal body mass index (BMI) and those without renal impairment. Periodical monitoring of the estimated glomerular filtration rate (eGFR) level, as well as the detection of protein excretion, should be taken into consideration worldwide when monitoring HIV-positive patients; this in order to detect even asymptomatic chronic kidney disease (CKD), and to manage these patients and prolong their lives.

Antibiotic Resistance Patterns of Uropathogens Causing Urinary Tract Infections in Children with Congenital Anomalies of Kidney and Urinary Tract.

Background: Urinary tract infections (UTI) are common in children worldwide. Congenital anomalies of kidney and urinary tract (CAKUT) increase the risk of UTI and consequently antibiotic resistance. Antibiotic resistance represents an important public health issue worldwide. We aimed to evaluate the local trend in terms of bacterial uropathogen resistance in the western part of Romania in children with CAKUT and UTI. Methods: 252 children with CAKUT were admitted to our hospital over a five-year period. Of them, 91 developed at least one UTI episode, with a total number of 260 positive urine cultures. We collected data about age at diagnosis of CAKUT, sex, origin environment, type and side of CAKUT, number of UTIs, type of uropathogen, and uropathogens antibiotic resistance. Results: Distribution of uropathogens was Escherichia coli (38.84%), Klebsiella spp. (21.15%), Enterococcus spp. (15.76%), Proteus spp. (8.07%), Pseudomonas spp. (8.07%), Enterobacter spp. (2.3%), other Gram-negative bacteria (2.3%), and other Gram-positive bacteria (3.45%). High antibiotic resistance was detected for ampicillin, amoxicillin, and second-generation cephalosporins. Escherichia coli presented high resistance for cefepime and ceftriaxone. Pseudomonas spp. remained susceptible to amikacin, quinolones, and colistin. Vancomycin, teicoplanin, linezolid, and piperacillin/tazobactam remained effective in treating Gram-positive UTI. Conclusions: High antibiotic resistance was identified for frequently used antibiotics. Lower antibiotic resistance was observed for some broad-spectrum antibiotics. Understanding uropathogens' antibiotic resistance is important in creating treatment recommendations, based on international guidelines, local resistance patterns, and patient particularities.

Challenges in the Surgical Treatment of Atrioventricular Septal Defect in Children With and Without Down Syndrome in Romania-A Developing Country.

Background: Atrioventricular septal defect (AVSD) is a cardiac malformation that accounts for up to 5% of total congenital heart disease, occurring with high frequency in people with Down Syndrome (DS). We aimed to establish the surgical challenges and outcome of medical care in different types of AVSD in children with DS compared to those without DS (WDS). Methods: The study included 62 children (31 with DS) with AVSD, evaluated over a 5 year period. Results: Complete AVSD was observed in 49 (79%) children (27 with DS). Six children had partial AVSD (all WDS) and seven had intermediate types of AVSD (4 with DS). Eight children had unbalanced complete AVSD (1 DS). Median age at diagnosis and age at surgical intervention in complete AVSD was not significantly different in children with DS compared to those WDS (7.5 months vs. 8.6). Median age at surgical intervention for partial and transitional AVSDs was 10.5 months for DS and 17.8 months in those without DS. A large number of patients were not operated: 13/31 with DS and 8/31 WDS. Conclusion: The complete form of AVSD was more frequent in DS group, having worse prognosis, while unbalanced AVSD was observed predominantly in the group without DS. Children with DS required special attention due to increased risk of pulmonary hypertension. Late diagnosis was an important risk factor for poor prognosis, in the setting of suboptimal access to cardiac surgery for patients in Romania. Although post-surgery mortality was low, infant mortality before surgery remains high. Increased awareness is needed in order to provide early diagnosis of AVSD and enable optimal surgical treatment.

Lymphoproliferative disorder in a twin female teenager post kidney transplantation.

Post-transplant lymphoproliferative disorder (PTLD) is defined as a heterogeneous group of lymphoid and plasmocytic proliferations with variable malignant potential. They often arise in immunocompromised post solid organ transplant (SOT) patients linked with Epstein-Barr virus (EBV) infection. Clinical manifestations include fever, lymphadenopathy and organ involvement. Diagnosis of PTLD requires morphopathological tissue examination. Treatment of EBV-related PTLD in SOT patients includes immunosuppressive (IS) agents' reduction, use of antiviral medication, anti-B-lymphocyte antibodies and chemotherapy for high-risk patients. We report a case of late EBV-related PTLD occurring in a young female, coming from twins, nine years after renal transplant from deceased donor. Both sisters were diagnosed at the age of 10 with chronic kidney disease (CKD) based on nephronophthisis and underwent the first simultaneous renal transplant from deceased donor in Romania. PTLD Hodgkin's-like lymphoma and EBV-positive lesions were to be found in autopsy. Routine EBV viral load testing and immune condition in SOT patients could identify PTLD risk factors therefore early treatment can be applied. Monitoring EBV serology and immunological parameters are preferred as strategy for PTLD prevention.

Late onset of HIVAN in a young female - a case report.

INTRODUCTION: Human Immunodeficiency Virus associated Nephropathy (HIVAN) is a relatively frequent pathology among HIV patients, especially in black patients. Among about 800 HIV-infected patients from the Western Romania cohort, mainly of subtype F, none were diagnosed documented with renal biopsy with HIV-associated nephropathy. Renal alterations etiology seems to be complex. Several renal abnormalities have been described among HIV-infected patients. Patient, Methods and Results: We discuss the case of a 24-year-old white Caucasian female HIV-infected in 1990 by horizontal transmission, in her first year of life. She was diagnosed as late-presenter stage C3 at the age of 10, when she was admitted in coma secondary to toxoplasmic encephalitis. The clinical evolution was favorable under antiretroviral treatment until 2003 when dyslipidemia and arterial hypertension appeared. The first clinical manifestations of nephropathy were detected in 2006, with altered values of creatinine clearance. A 7-year follow-up of renal impairment shows a descending trend of creatinine clearance values. We analyzed the repeated ultrasound findings and renal biopsy was performed in 2013 revealing aspects of HIVAN. It has become obvious that HIVAN is caused by direct effects of HIV-1 virus over kidney structure and also that within the renal cells, viral replication is still permitted. In our case, the viral load peaked in 2011 at the same time the renal function significantly deteriorated. Her lifestyle changes must be taken under consideration - in the last year she has been under a low protein regimen. Compliance to antiretroviral treatment improves survival rate with a delayed deterioration of renal function to end-stage renal disease. CONCLUSIONS: Renal biopsy remains the most important feature in order to diagnose HIVAN. Suspicion of HIVAN diagnosis should be taken under consideration in the presence of constant proteinuria as well as decreased creatinine clearance levels.