Multidisciplinary management of people with spina bifida across the lifespan.

The average worldwide prevalence of neural tube defects (NTDs) is 1.0 per 1000 births. Its development is multifactorial due to genetic and non-genetic factors. Spina bifida (SB) is one of main representatives of NTD. The spinal cord lesion level is the main determinant of the level of paralysis, numbness, and difficulties with bladder/bowel functions. Myelomeningocele prenatal repair reduces hydrocephalus and hindbrain herniation and improves motor function. The severity of hydrocephalus is associated with poorer neurodevelopmental outcomes whether operated on prenatally or after birth. People with SB tend to have a lower IQ and cognitive difficulties. Early diagnosis, proactivity, and lifelong multidisciplinary follow-up are key protective issues. Invasive urological interventions should be considered in selected patients after failure of conservative treatment. Transition to adult care should be well planned as it is challenging. Health literacy is directly associated with success at transition. Sexuality and fertility should be addressed before/during puberty. Overall, the rates of fecal and urinary continence and skin breakdown increase with age, whereas the ability to ambulate declines with age. Bowel and urinary incontinence are independent predictors of lower health-related quality of life (HRQoL) in adults with SB. Bowel incontinence has negative impact on HRQoL regardless of frequency or amount. Long-term caregiver support should be offered at diagnosis. Survival at a mean of 50 years is poor, at 32%, due to central nervous system deaths, cancer, urological disease, and sepsis. Challenges to implementation of recommended practices exist, especially in low and middle-income countries.

Thromboprophylaxis after kidney transplantation in children: Ten-year experience of a single Brazilian center.

BACKGROUND: Kidney transplantation is the gold standard treatment for children with end-stage chronic kidney disease. Graft thrombosis is an important cause of graft failure, with high morbidity, mortality, and impact on quality of life and to the health system. The role of thromboprophylaxis in this setting is still uncertain. We describe the demographic characteristics and thrombotic risk factors in pediatric renal transplant recipients, determining the rate of renal graft thrombosis, and discuss the role of thromboprophylaxis. METHODS: This retrospective study reviewed 96 pediatric renal transplantations between 2008 and 2017 in a single hospital. Patients were assigned to one of two groups: children who did not receive thromboprophylaxis after transplantation and those who did. We reported their characteristics, comparing the incidence of graft thrombosis and hemorrhagic complications between the groups. RESULTS: Forty-nine patients (51%) received thromboprophylaxis. Thrombosis occurred in 5 patients who did not receive thromboprophylaxis (5.2%) compared with none in the group that did (p = .025). In all patients, renal graft thrombosis resulted in early graft loss. Thirteen patients had hemorrhagic complications. Seven were unrelated to pharmacological thromboprophylaxis (2 major, 1 moderate, and 4 minor bleeding, which either did not receive thromboprophylaxis or had bleeding prior to thromboprophylaxis), while six occurred during heparinization (2 major, 1 moderate, and 3 minor bleeding). There was no significant difference in the rate of hemorrhagic complications between the groups (p = .105). CONCLUSIONS: The rate of renal graft thrombosis was 5.2%. Thrombosis remains an important cause of early graft loss. Thromboprophylaxis was associated with a reduction in graft thrombosis without increased risk of bleeding.

Study of the association between generic and disease-specific quality of life and behavior problems in pediatric patients with chronic kidney disease stage 3 or higher and the quality of life and mental health of their primary caregivers.

BACKGROUND: Chronic kidney disease (CKD) can affect quality of life and mental health of patients and their primary caregivers (PCs) in different disease stages. METHODS: This prospective, cross-sectional, descriptive, comparative, and analytical study of patients with stage 3-5 CKD, aged 8-18 years, assesses the association between patients' general and disease-specific health-related quality of life (HRQOL) and behavioral problems and their PCs' QoL and mental health status. PedsQL 4.0, PedsQL ESRD, CBCL, and YSR questionnaires were used to evaluate 80 patients while their PCs were assessed by SF-36 and MINI questionnaires. The study participants were divided into three groups: G1: stage 3-4 CKD, G2: peritoneal dialysis and hemodialysis, and G3: kidney transplantation. RESULTS: Lower indexes in PedsQL 4.0 and PedsQL ESRD were demonstrated in G2 patients. No deviant internalizing and/or externalizing conducts were shown by the CBCL questionnaire while the YSR questionnaire demonstrated significant differences in all domains with higher scores in G2 patients without reaching clinical range for individual syndromes. G3 PCs presented the lowest SF-36 scores and the highest occurrence of psychiatric diagnoses, according to MINI, when compared to other PCs. Parallelism was found between scores of PedsQL 4.0 and PedsQL ESRD with tendency to higher scores in the PC's view compared to the patient's view. CONCLUSIONS: CKD affects HRQOL of patients and their PCs, as well as patients' behavior problems and their PCs' mental health. PCs' perceptions of patients' QOL and behavioral problems may be related to their mental health status and QOL self-perceptions.

Kidney impairment in fetal growth restriction: three-dimensional evaluation of volume and vascularization.

OBJECTIVES: Renal development is impaired in fetal growth restriction (FGR). Renal size can be considered a surrogate of renal function in childhood, and could be impaired in that condition. Our aim was to evaluate the ratio of total renal volume, measured by three-dimensional ultrasound, to estimated fetal weight (TRV/EFW) among fetuses with and without growth restriction. Furthermore, we correlated TRV/EFW with fetal Doppler velocimetry and renal vascularization indexes and evaluated the association of renal volume and vascular parameters with adverse neonatal events in growth-restricted fetuses. METHODS: In a retrospective cohort, TRV and renal vascularization of growth-restricted and normal fetuses were evaluated by three-dimensional ultrasonography and VOCAL technique. Independent samples t-tests and Mann-Whitney test were used for comparisons between groups. Logistic regression model was applied to evaluate the association between renal characteristics and adverse neonatal events. RESULTS: Seventy-one growth-restricted fetuses were compared to 194 controls. The TRV/EFW was lower in the growth-restricted group (P < .001). In our sample, this ratio did not correlate with Doppler velocimetry parameters, renal vascular indexes or any adverse neonatal events. CONCLUSION: The TRV/EFW ratio is decreased in FGR. Further studies are needed to investigate the association of this ratio with long-term renal outcomes.

First-year profile of biomarkers for early detection of renal injury in infants with congenital urinary tract obstruction.

BACKGROUND: Diagnosis of renal function impairment and deterioration in congenital urinary tract obstruction (UTO) continues to be extremely challenging. Use of renal biomarkers in this setting may favor early renal injury detection, allowing for a reliable choice of optimal therapeutic options and prevention or minimization of definitive renal damage. METHODS: This longitudinal, prospective study analyzed the first-year profile of two serum renal biomarkers: creatinine (sCr) and cystatin C (sCyC); and six urinary renal biomarkers: neutrophil gelatinase-associated lipocalin (NGAL), kidney injury molecule-1 (KIM-1), transforming growth factor beta-1 (TGF-ß1), retinol-binding protein (RBP), cystatin C (uCyC), and microalbuminuria (µALB) in a cohort of 37 infants with UTO divided into three subgroups: 14/37 with unilateral hydro(uretero)nephrosis, 13/37 with bilateral hydro(uretero)nephrosis, and 10/37 patients with lower urinary tract obstruction (LUTO), compared with 24 healthy infants matched by gestational age and birth weight. RESULTS: All urine biomarkers showed significantly higher values at the first month of life (p ≤ 0.009), while NGAL (p = 0.005), TGF-ß1 (p < 0.001), and µALB (p < 0.001) were high since birth compared to controls. Best single biomarker performances were RBP in bilateral hydronephrosis and LUTO subgroups and KIM-1 in unilateral hydronephrosis subgroup. Best biomarker combination results for all subgroups were obtained by matching RBP with TGF-ß1 or KIM-1 and NGAL with CyC ([AUC] ≤ 0.934; sensitivity ≤ 92.4%; specificity ≤ 92.8%). CONCLUSIONS: RBP, NGAL, KIM-1, TGF-ß1, and CyC, alone and especially in combination, are relatively efficient in identifying surgically amenable congenital UTO and could be of practical use in indicating on-time surgery.

Prevalence and correlates of depression, anxiety, and stress in medical residents of a Brazilian academic health system.

BACKGROUND: Several studies correlate medical residency with the occurrence of mental health disorders, Burnout Syndrome and quality of life impairment. It has been demonstrated that mental health disorders increase medical errors and lead to less effective patient care. Considering such context, this study aimed to evaluate the prevalence of anxiety, depression, stress and to identify its correlates with Burnout Syndrome and quality of life in a sample of medical residents and fellow physicians of the largest Brazilian academic health system. METHODS: In 2017, 1648 participants were voluntarily and anonymously surveyed online about demographic characteristics, Burnout Syndrome, mental symptoms, and quality of life measured by validated questionnaires. Responses were captured through REDCap platform and multivariate statistical analyses were performed with STATA 15. RESULTS: A total of 606 (36.8%) residents/fellows physicians completed the survey. Depression symptoms were present in 19%, anxiety symptoms in 16% and stress symptoms in 17.7% of the sample. Burnout Syndrome was present in 63% of the sample. Multivariate analysis showed a statistical significant positive correlation between Burnout Syndrome and depression, anxiety and stress symptoms and a negative correlation between mental symptoms and quality of life scores. CONCLUSIONS: Mental health symptoms prevalence in this study is similar to other studies and their occurrence is positively correlated with Burnout Syndrome among medical residents/fellow physicians of the largest Brazilian academic health system. These results are relevant and must be confirmed by multicentric longitudinal studies. This study reinforces the importance of debating interventions to improve mental health among doctors in training.

Monosymptomatic nocturnal enuresis in pediatric patients: multidisciplinary assessment and effects of therapeutic intervention.

BACKGROUND: Few studies manage patients with isolated monosymptomatic enuresis (MNE) with multidisciplinary evaluation and pre- and long-term post-intervention monitoring. METHODS: This was a prospective study of MNE patients, aged 6-16 years, diagnosed by multidisciplinary assessment. Of the 140 initial applicants (58.6%) with MNE, 82 were included in the study and randomized for therapeutic intervention in three treatment groups, namely: alarm, desmopressin and alarm + desmopressin. Therapeutic response was evaluated 12 months after treatment withdrawal. RESULTS: Of the 82 patients [mean age 9.5 (SD ± 2.6) years, n = 62 males (75.6%)], 91.1% had a family history of nocturnal enuresis (NE) in first-/second-degree relatives, 81.7% had constipation and 40.7% had mild-to-moderate apnea. Prior to randomization, management of constipation and urotherapy led to remission in seven of the 82 patients; 75 patients were randomized to intervention. There were 14/75 (18.7%) dropouts during the intervention, especially in the alarm group (p = 0.00). Initial complete/partial response was achieved in 56.6% of the alarm group, 70% of the desmopressin group and 64% in the combined group (p = 0.26). Continued success occurred in 70% of the alarm group, 84.2% of the desmopressin group and 100% of the combined group (p = 0.21). Recurrence occurred in 3/20 (15%) patients in the alarm group and 1/19 (5.2 %) patients of the desmopressin group. Post-intervention Child Behavior Checklist (CBCL) and PedsQL 4.0 scores showed significant improvement. CONCLUSIONS: The three therapeutic modalities were effective in managing MNE with low relapse rates; the alarm group showed the highest dropout rate. Therapeutic success was associated with improvement of behavioral problems and quality of life scores.

Developmental changes in polyunsaturated fetal plasma phospholipids and feto-maternal plasma phospholipid ratios and their association with bronchopulmonary dysplasia.

BACKGROUND: Docosahexaenoic (C22:6) and arachidonic acid (C20:4) are long-chain polyunsaturated fatty acids (LC-PUFA), essential to fetal development, and preferentially transported by plasma phospholipids. OBJECTIVE: To characterize fetal and maternal plasma phospholipid changes during gestation, and to investigate whether LC-PUFA phospholipid profiles are associated with bronchopulmonary dysplasia (BPD). DESIGN: Cord plasma and parturient serum from N = 108 pregnancies [24-42 week postmenstrual age (PMA)] were collected. Phosphatidylcholine (PC) and phosphatidylethanolamine (PE) were analyzed with tandem mass spectrometry. PMA-associated changes were quantified, and break point analyses served to describe nonlinear changes during gestation. RESULTS: PC and PE were lower in cord than in parturient samples. In parturients, PC decreased until 33 week PMA, but then re-increased, whereas in cord plasma, concentrations linearly decreased. Fetal PC and PC sub-group values correlated with maternal values. C20:4-PC was twofold higher in cord than in maternal samples throughout gestation. C22:6-PC values, however, exceeded maternal values only beyond 33 week PMA. Consequently, early preterm C20:4-PC-to-C22:6-PC ratio largely exceeded term infant values. In infants born before 28 week PMA, a low C20:4-PC-to-C22:6-PC ratio was associated with BPD severity. CONCLUSIONS: Fetal plasma LC-PUFA-PC composition correlates with maternal values. Fetal C20:4-PC exceeds maternal values throughout gestation, whereas C22:6-PC exceeds maternal values only beyond 33 week PMA, resulting in a low fetal C20:4-PC/C22:6-PC ratio only toward end gestation. A low C20:4-PC/C22:6-PC ratio before 28 week PMA is associated with BPD severity. These data point to a concept of PMA-adjusted ARA and DHA supplementation and, potentially, cord plasma phospholipid analysis for BPD prediction.

Impact of a multidisciplinary evaluation in pediatric patients with nocturnal monosymptomatic enuresis.

BACKGROUND: Enuresis (NE) is a clinical condition of multifactorial etiology that leads to difficulties in child/adolescent social interaction. METHODS: This was a prospective study on the impact of multidisciplinary assessment of 6- to 17-year-old patients with monosymptomatic nocturnal enuresis (MNE), including a structured history, clinical/neurological examination, bladder and bowel diaries, sleep diary and questionnaires, psychological evaluation [Child Behavior Checklist (CBCL) and PedsQL 4.0 questionnaires], urinary sonography, blood and urine laboratory tests, polysonography (PSG), and balance evaluation. RESULTS: A total of 140 enuretic participants were evaluated, of whom 27 were diagnosed with NE complicated by urinary disorder, four with hypercalciuria, three with nephropathy and one with attention-deficit hyperactivity disorder. Among the 87 participants who underwent PSG, six were diagnosed with severe apnea. Of the 82 MNE patients who underwent full assessment, 62 were male (75.6 %), and the mean age was 9.5 (±2.6) years. A family history of NE was diagnosed in 91.1 % of first- and second-degree relatives, constipation in 89.3 % and mild/moderate apnea in 40.7 %. Balance control alteration was identified by physical therapy evaluation of MNE patients. Participants' quality of life evaluation scores were significantly lower than those of their parents. CONCLUSION: Enuresis is a multifactorial disorder that requires a structured diagnostic approach.

Choline concentrations are lower in postnatal plasma of preterm infants than in cord plasma.

BACKGROUND: Choline is essential to human development, particularly of the brain in the form of phosphatidylcholine, sphingomyelin and acetylcholine, for bile and lipoprotein formation, and as a methyl group donator. Choline is actively transported into the fetus, and maternal supply correlates with cognitive outcome. Interruption of placental supply may therefore impair choline homeostasis in preterm infants. OBJECTIVE: Determination of postnatal plasma concentrations of choline and its derivatives betaine and dimethylglycine (DMG) in preterm infants compared to cord and maternal blood matched for postmenstrual age (PMA). DESIGN: We collected plasma of very low-birth-weight infants undergoing neonatal intensive care (n = 162), cord plasma of term and preterm infants (n = 176, 24-42-week PMA), serum of parturients (n = 36), and plasma of healthy premenopausal women (n = 40). Target metabolites were analyzed with tandem mass spectrometry and reported as median (25th/75th percentiles). RESULTS: Cord plasma choline concentration was 41.4 (31.8-51.2) µmol/L and inversely correlated with PMA. In term but not in preterm infants, cord plasma choline was lower in girls than in boys. Prenatal glucocorticoid treatment did not affect choline levels in cord plasma, whereas betaine was decreased and DMG increased. In parturients and non-pregnant women, choline concentrations were 14.1 (10.3-16.9) and 8.8 (5.7-11.2) µmol/L, respectively, whereas betaine was lowest in parturients. After delivery, preterm infant plasma choline decreased to 20.8 (16.0-27.6) µmol/L within 48 h. Betaine and DMG correlated with plasma choline in all groups. CONCLUSIONS: In preterm infants, plasma choline decreases to 50 % of cord plasma concentrations, reflecting choline undernourishment and postnatal metabolic adaptation, and potentially contributing to impaired outcome.

Plasma phospholipids indicate impaired fatty acid homeostasis in preterm infants.

BACKGROUND: During fetal development, docosahexaenoic (DHA) and arachidonic acid (ARA) are particularly enriched in brain phospholipids. After preterm delivery, fetal enrichment of DHA and ARA via placental transfer is replaced by enteral and parenteral nutrition, which is rich in linoleic acid (LA) instead. Specific DHA and ARA enrichment of lipoproteins is reflected by plasma phosphatidylcholine (PC) species, whereas plasma phosphatidylethanolamine (PE) composition reflects hepatic stores. OBJECTIVE: We profiled PC and PE species in preterm infant plasma, compared with cord and maternal blood, to assess whether current feeding practice meets fetal conditions in these patients. DESIGN: Preterm infant plasma (N = 171, 23-35 w postmenstrual age (PMA), postnatal day 1-103), cord plasma (N = 194) and maternal serum (N = 121) (both 24-41 w PMA) were collected. After lipid extraction, PC and PE molecular species were analyzed using tandem mass spectrometry. RESULTS: Phospholipid concentrations were higher in preterm infant than in cord plasma after correction for PMA. This was mainly due to postnatal increases in LA-containing PC and PE, resulting in decreased fractions of their DHA- and ARA-containing counterparts. These changes in preterm infant plasma phospholipids occurred during the time of transition to full enteral feeds (day 0-10 after delivery). Thereafter, the fraction of ARA-containing phospholipids further decreased, whereas that of DHA slowly reincreased but remained at a level 50% of that of PMA-matched cord blood. CONCLUSIONS: The postnatal increase in LA-PC in preterm infant plasma results in decreased fractions of DHA-PC and ARA-PC. These changes are also reflected by PE molecular composition as an indicator of altered hepatic fatty acid homeostasis. They are presumably caused by inadequately high LA, and low ARA and DHA supply, at a stage of development when ARA-PC and DHA-PC should be high, probably reducing the availability of DHA and ARA to the developing brain and contributing to impaired neurodevelopment of preterm infants.

Health-related quality of life of children and adolescents with CKD stages 4-5 and their caregivers.

BACKGROUND: Renal replacement therapies may affect the quality of life of patients and their primary caregivers (PC). METHODS: This study describes the perception of health-related quality of life (HRQoL) of children/adolescents with CKD stages 4-5, as well as of their PC (n = 64), in comparison to healthy peers and their PC (n = 129), respectively, based on the Peds QL™ 4.0 and Short Form-36 (SF-36) questionnaires and selected biomarkers. RESULTS: Patients reported a deleterious impact on physical capacity and on social and school activities. A negative influence on emotional aspects was reported by older patients, but not by their PC. Hemodialysis, followed by peritoneal dialysis, had a more negative impact on patients' physical functioning domain. PC HRQol proxy reports differed from those of their children, especially in older patients. PC of both groups presented similar SF-36 scores. An association was demonstrated between the magnitude of treatment target inadequacies, lower specific dominion scores in the patients/PC proxy reports and PC SF-36 general health scores. CONCLUSION: The HRQoL of patients with CKD stages 4-5 is negatively affected to different degrees depending on age and treatment modality. The results suggest an association between worsening HRQoL parameters and inadequate control of recognized therapeutic CKD treatment targets.

Pediatric hypertension as an early manifestation of cardiovascular disease in children.

In adults, cardiovascular events associated with arterial hypertension (AH) have a major impact on morbidity and mortality. In light of recent findings, AH in children has been interpreted as early cardiovascular disease (CVD), while exposure to CV risk factors in children proves to be a predictor of subclinical CVD in adults. The American College of Cardiology/American Heart Association has recently updated the classifications for measuring blood pressure (BP) in adults and children. Primary AH in children is generally asymptomatic, and it is associated with a family history of AH, overweight/obesity, and normal morphofunctional characteristics of the urinary system. The younger the child and the higher the BP, the greater the likelihood of secondary AH. The investigation into the etiology of AH begins with a detailed anamnesis, which should include clinical information and details on the use of medication, smoking, and alcohol consumption from the perinatal period to the time of consultation. Modifying risk factors by reducing weight, decreasing alcohol consumption and increasing vegetable intake from childhood to adulthood has been associated with the resolution of AH in the childhood-adulthood transition, and with the reversal of cardiometabolic adverse effects in non-obese adult individuals. Pharmacological therapy should be initiated in cases of symptomatic AH, AH secondary to chronic kidney disease or diabetes mellitus, presence of target organ lesions, stage 2 AH with no modifiable cause and resistant AH unresponsive to lifestyle changes.

Health-related quality of life for pediatric patients with end-stage kidney disease: A systematic review and meta-analysis of the Pediatric Quality of Life Inventory (PedsQL).

INTRODUCTION: Health-related quality of life (HRQoL) studies demonstrate the impact of end-stage renal disease (ESRD) on the physical and psychosocial development of children. While several instruments are used to measure HRQoL, few have standardized domains specific to pediatric ESRD. This review examines current evidence on self and proxy-reported HRQoL among pediatric patients with ESRD, based on the Pediatric Quality of Life Inventory (PedsQL) questionnaires. METHODS: Following PRISMA guidelines, we conducted a systematic review and meta-analysis on HRQoL using the PedsQL 4.0 Generic Core Scale (GCS) and the PedsQL 3.0 ESRD Module among 5- to 18-year-old patients. We queried PubMed, Embase, Web of Science, CINAHL, and Cochrane databases. Retrospective, case-controlled, and cross-sectional studies using PedsQL were included. FINDINGS: Of 435 identified studies, 14 met inclusion criteria administered in several countries. Meta-analysis demonstrated a significantly higher total HRQoL for healthy patients over those with ESRD (SMD:1.44 [95% CI: 0.78-2.09]) across all dimensional scores. In addition, kidney transplant patients reported a significantly higher HRQoL than those on dialysis (PedsQL GCS, SMD: 0.33 [95% CI: 0.14-0.53]) and (PedsQL ESRD, SMD: 0.65 [95% CI: 0.39-0.90]) concordant with parent-proxy reports. DISCUSSION: Patients with ESRD reported lower HRQoL in physical and psychosocial domains compared with healthy controls, while transplant and peritoneal dialysis patients reported better HRQoL than those on hemodialysis. This analysis demonstrates the need to identify dimensions of impaired functioning and produce congruent clinical interventions. Further research on the impact of individual comorbidities in HRQoL is necessary for developing comprehensive, integrated, and holistic treatment programs.

Risk factors of acute kidney injury in very low birth weight infants in a tertiary neonatal intensive care unit.

OBJECTIVE: Acute kidney injury (AKI) in the neonatal period is associated with worst outcomes as increased mortality and increased length of hospital stay. Very low birth weight (VLBW) newborns are at higher risk for developing several other conditions that are associated with worst outcomes. Understanding the risk factors for AKI may help to prevent this condition and improve neonatal care for this population. METHODS: This retrospective cohort study included 155 very low birth weight newborns admitted between 2015 and 2017. The authors compared the newborns who developed neonatal AKI with the non-AKI group and analyzed the main risk factors for developing AKI in the population. The authors also performed an analysis of the main outcomes defined as the duration of mechanical ventilation, length of stay, and death. RESULTS: From the cohort, a total of 61 (39.4%) patients had AKI. The main risk factors associated with Neonatal AKI were necrotizing enterocolitis (aOR 7.61 [1.69 - 34.37]; p = 0.008), neonatal sepsis (aOR 2.91 [1.17 - 7.24], p = 0.021), and hemodynamic instability (aOR 2.99 [1.35 - 6.64]; p = 0.007). Neonatal AKI was also associated with an increase in the duration of mechanical ventilation in 9.4 days (p = 0.026) and in an increase in mortality 4 times (p = 0.009), after adjusting for the other variables. CONCLUSION: The present results highlight the importance of minimizing sepsis and necrotizing enterocolitis, as well as the importance of identifying hemodynamic instability, to prevent AKI and diminish the burden of morbimortality in VLBW newborns.

The challenges of the pandemic and the vaccination against covid-19 in pediatric patients with kidney disease.

The covid-19 vaccine confers direct protection and reduces transmission rates of the virus and new variants. Vaccines from Pfizer/BioNTech and CoronaVac have been cleared for children in Brazil. They are safe, effective, and immunogenic. There are no known complications associated with the use of steroids or vaccines in pediatric patients with covid-19 and nephrotic syndrome. With or without immunosuppression, these patients are not at increased risk of severe covid-19, and steroids are safe for them. A milder form of covid-19 occurs in patients with chronic kidney disease without the need for hospitalization. The vaccine response may be reduced and/or the duration of antibodies after vaccination may be shorter than in the general population. However, considering risk of exposure, vaccination against covid-19 is recommended. It is believed that patients with hemolytic-uremic syndrome are at higher risk of severe covid-19. Vaccination is recommended, although specific data on the safety and efficacy of the covid-19 vaccine are limited. There is agreement that the benefits of induced immunity outweigh the risks of immunization. Vaccination against covid-19 is recommended for children and adolescents needing kidney transplantation or who have undergone transplantation. These patients present decreased immune response after vaccination, but immunization is recommended because the benefits outweigh the risks of vaccination. Current recommendations in Brazil stipulate the use of the messenger RNA vaccine. This paper aims to provide pediatric nephrologists with the latest knowledge about vaccination against covid-19 for children with kidney disease.

PCRRT-ICONIC critical care pediatric nephrology course: the global prevalence of COVID-19 and associated sequelae.

After nearly three years of the COVID-19 pandemic, research has affirmed that COVID-19 is more than just a respiratory virus. There have been significant breakthroughs made surrounding the development of acute kidney injury (AKI) and chronic kidney disease (CKD), in pediatric populations. Additionally, patient populations susceptible to renal complications consist of pediatric transplant recipients, multisystem inflammatory syndrome (MIS-C), and dialysis. Although research is gradually becoming more available surrounding this prevalent topic, knowledge is sparse on the deleterious effects of COVID-19 on pediatric patients with kidney disease and requires more in-depth analysis. The virtual international conference, Pediatric Critical Care Nephrology & Dialysis Course, on August 7th, 2021, reviewed the severe cases of COVID-19 in the global pediatric population. By integrating international perspectives, statistics, techniques, and treatments for managing renal complications, we further develop scientific understanding of the renal complications seen in children with COVID-19 globally.

Combined Focused Next-Generation Sequencing Assays to Guide Precision Oncology in Solid Tumors: A Retrospective Analysis from an Institutional Molecular Tumor Board.

BACKGROUND: Increasing knowledge of cancer biology and an expanding spectrum of molecularly targeted therapies provide the basis for precision oncology. Despite extensive gene diagnostics, previous reports indicate that less than 10% of patients benefit from this concept. METHODS: We retrospectively analyzed all patients referred to our center's Molecular Tumor Board (MTB) from 2018 to 2021. Molecular testing by next-generation sequencing (NGS) included a 67-gene panel for the detection of short-sequence variants and copy-number alterations, a 53- or 137-gene fusion panel and an ultra-low-coverage whole-genome sequencing for the detection of additional copy-number alterations outside the panel's target regions. Immunohistochemistry for microsatellite instability and PD-L1 expression complemented NGS. RESULTS: A total of 109 patients were referred to the MTB. In all, 78 patients received therapeutic proposals (70 based on NGS) and 33 were treated accordingly. Evaluable patients treated with MTB-recommended therapy (n = 30) had significantly longer progression-free survival than patients treated with other therapies (n = 17) (4.3 vs. 1.9 months, p = 0.0094). Seven patients treated with off-label regimens experienced major clinical benefits. CONCLUSION: The combined focused sequencing assays detected targetable alterations in the majority of patients. Patient benefits appeared to lie in the same range as with large-scale sequencing approaches.

Pediatric peritoneal dialysis in Brazil: a discussion about sustainability. A document by the Brazilian Society of Nephrology, the Brazilian Society of Pediatrics, the Brazilian Association of Organ Transplantation, and the Brazilian Association of Dialysis and Transplant Centers.

INTRODUçÃO: A diálise peritoneal (DP) é importante para a pediatria. Este estudo mostrou dados de centros brasileiros que utilizam DP pediátrica. MÉTODO: Estudo transversal, observacional, descritivo com questionário eletrônico. Incluiu-se pacientes de 0-18 anos em DP cadastrados nos bancos de dados dos diversos centros. Questionário preenchido anonimamente, sem dados de identificação. Foi adotada metodologia quantitativa. RESULTADOS: 212 pacientes estão em DP no Brasil (agosto, 2021). 80% têm menos de 12 anos de idade. A maioria realiza DP automatizada e 74% são dependentes do Sistema Único de Saúde. Em 25% dos centros faltou material de DP e em 51% os pacientes pediátricos foram convertidos de DP para HD. CONCLUSÃO: A maioria dos pacientes tinha menos de 12 anos e era dependente do SUS. A escassez de insumos aconteceu em 25% dos centros. Esses dados apontam para o problema da sustentabilidade de DP, única alternativa de TRS em crianças muito pequenas.