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Introduction: Hemodialysis is the most used dialysis modality in Brazil for the treatment of end-stage chronic kidney disease and severe acute kidney injury. Non-tunneled, short-term, double-lumen catheters allow immediate vascular access in patients without a definitive vascular access for dialysis treatment. Implantation is performed using the Seldinger technique, traditionally based on anatomical landmarks or with puncture under direct visualization by ultrasonography. Confirmation of the placement of the catheter is usually made with a chest X-ray after the end of the procedure., Objective: To describe the use of ultrasonography to confirm the proper positioning of the guidewire during catheter implantation in real time, ensuring the desired path., Methods: We used the Seldinger technique for catheter implantation. The confirmation of the position of the guidewire occurred after the introduction of 50 cm of this wire, as described in the aforementioned technique. A convex transducer was placed longitudinally below the xiphoid process to visualize the guidewire in the inferior vena cava, or transversely at the same location to visualize it in the cavoatrial junction or right ventricle, using the two-dimensional mode of the ultrasound device. After viewing the guidewire, the catheter implantation proceeded., Results and Discussion: The technique was performed in 1549 patients, and regarded 2596 catheter implantations over a period of 5 years and 9 months. The only complication observed was local hematoma, occurring in 0.1% of cases., Conclusion: Confirmation of guidewire position with ultrasonography during catheter implantation is recommended because it is safe, low cost and ensures correct catheter direction., (© 2022. The Author(s) under exclusive licence to Italian Society of Nephrology.)

Background and Objectives: The Brazilian Neonatal Resuscitation Program releases guidelines based on local interpretation of international consensus on science and treatment recommendations. We aimed to analyze whether guidelines for preterm newborns were applied to practice in the 20 Brazilian Network on Neonatal Research centers of this middle-income country., Methods: Prospectively collected data from 2014 to 2020 were analyzed for 8514 infants born at 230/7 to 316/7 weeks' gestation. The frequency of procedures was evaluated by gestational age (GA) category, including use of a thermal care bundle, positive pressure ventilation (PPV), PPV with a T-piece resuscitator, maximum fraction of inspired oxygen (Fio2) concentration during PPV, tracheal intubation, chest compressions and medications, and use of continuous positive airway pressure in the delivery room. Logistic regression, adjusted by center and year, was used to estimate the probability of receiving recommended treatment., Results: For 3644 infants 23 to 27 weeks' GA and 4870 infants 28 to 31 weeks' GA, respectively, the probability of receiving care consistent with guidelines per year increased, including thermal care (odds ratio [OR], 1.52 [95% confidence interval (CI) 1.44-1.61] and 1.45 [1.38-1.52]) and PPV with a T-piece (OR, 1.45 [95% CI 1.37-1.55] and 1.41 [1.32-1.51]). The probability of receiving PPV with Fio2 1.00 decreased equally in both GA groups (OR, 0.89; 95% CI, 0.86-0.93)., Conclusions: Between 2014 and 2020, the resuscitation guidelines for newborns <32 weeks' GA on thermal care, PPV with a T-piece resuscitator, and decreased use of Fio2 1.00 were translated into clinical practice., (Copyright © 2022 by the American Academy of Pediatrics.)

Paracoccidioidomycosis (PCM) is an endemic fungal infection in Latin America, which manifests as an acute or chronic form and is more frequent in adult males. It is caused by Paracoccidioides brasiliensis or Paracoccidioides lutzii , which are thermodimorphic fungi. The disease can present as a severe and disseminated form involving the lungs, skin, lymph nodes, spleen, liver, and lymphoid organs of the gastrointestinal tract. Most of the primary infections are subclinical, and the cell-mediated immune response contains the infection. It is rare in transplant patients, and there are few cases described in the literature. In solid organ transplant patients, it usually results from the reactivation of a latent infection, manifesting itself after a few years of transplantation with frequent pulmonary and skin involvement. PCM is an endemic infection in Brazil; however, as it is not classified as a notifiable disease, there is no accurate database on its incidence, and case reports are important sources of information. Clinical disease in kidney transplant patients is rare and has a high mortality rate. In this scope, the present clinical case reports the challenges of the clinical management of disseminated PCM caused by Paracoccidioides brasiliensis in a kidney transplant recipient who used immunosuppressive drugs and was treated with Itraconazole., Competing Interests: The authors have declared that no competing interests exist., (Copyright © 2021, Felipe et al.)

Purpose: To assess developmental enamel defects (DED) and dental caries of preterm (PT) and/or low birth weight (LBW) children compared to full term (FT) and/or normal birth weight (NBW) infants, and to evaluate the association of DED with socioeconomic factors, gestational health, and postnatal factors.
Methods: This study included 84 two- to five-year-old children. The PT/LBW group included 42 children who received medical care at a university hospital, while FT/ NBW group included 42 subjects. Children were matched by sex and age (1:1 ratio). Descriptive statistics, bivariate analysis, and Poisson regression were used to analyse the data.
Results: Children in the PT/LBW group had a significantly higher number of teeth with DED than subjects in the FT/NBW group. No difference was observed between the groups regarding dental caries ( P >0.05). Admission to the neonatal intensive care was significantly associated with DED unit (prevalence ratio = 0.21 [95 percent confidence interval = 0.1 to 0.5]).
Conclusion: PT/LBW children presented more DED than FT/NBW children. No differences were observed between the groups for dental caries. Admission to the neonatal intensive care unit was associated with DED.

Objectives: To evaluate the prevalence of congenital heart disease and their outcomes in a Brazilian cohort of very low birth weight preterm infants., Design: Post hoc analysis of data from the Brazilian Neonatal Network database, complemented by retrospective data from medical charts and a cross-sectional survey., Setting: Twenty public tertiary-care university hospitals., Patients: A total of 13,955 newborns weighing from 401 to 1,499 g and between 22 and 36 weeks of gestational age, born from 2010 to 2017., Interventions: None., Measurements and Main Results: The prevalence of congenital heart disease was 2.45% (95% CI, 2.20-2.72%). In a multivariate regression analysis, risk factors associated with congenital heart disease were maternal diabetes (relative risk, 1.55; 95% CI, 1.11-2.20) and maternal age above 35 years (relative risk, 2.09; 95% CI, 1.73-2.51), whereas the protection factors were maternal hypertension (relative risk, 0.54; 95% CI, 0.43-0.69), congenital infection (relative risk, 0.45; 95% CI, 0.21-0.94), and multiple gestation (relative risk, 0.73; 95% CI, 0.55-0.97). The pooled standardized mortality ratio in patients with congenital heart disease was 2.48 (95% CI, 2.22-2.80), which was significantly higher than in patients without congenital heart disease (2.08; 95% CI, 2.03-2.13). However, in multiple log-binomial regression analyses, only the presence of major congenital anomaly, gestational age (< 29 wk; relative risk, 2.32; 95% CI, 2.13-2.52), and Score for Neonatal Acute Physiology and Perinatal Extension II (> 20; relative risk, 3.76; 95% CI, 3.41-4.14) were independently associated with death, whereas the effect of congenital heart disease was spotted only when a conditional inference tree approach was used., Conclusions: The overall prevalence of congenital heart disease in this cohort of very low birth weight infants was higher and with higher mortality than in the general population of live births. The occurrence of a major congenital anomaly, gestational age (< 29 wk), and Score for Neonatal Acute Physiology and Perinatal Extension II (> 20) were significantly and independently associated with death, whereas the association of congenital heart disease and death was only evident when a major congenital anomaly was present., Competing Interests: The Brazilian Neonatal Network received funding from Brazilian Ministry of Health (Ministério da Saúde do Brasil). Dr. Silva received support for article research from the National Institutes of Health. The remaining authors have disclosed that they do not have any potential conflicts of interest., (Copyright © 2020 by the Society of Critical Care Medicine and the World Federation of Pediatric Intensive and Critical Care Societies.)

Objective: To compare the behavior of preterm newborns and full-term newborns using the Newborn Behavioral Observation and to evaluate the mothers' experience when participating in this observation., Method: This was a cross-sectional study performed at a referral hospital for high-risk births, involving mothers and neonates before hospital discharge. The mothers answered the sociodemographic questionnaire, participated in the Newborn Behavioral Observation session, and evaluated the experience by answering the parents' questionnaire at the end. The characteristics of the preterm newborn and full-term newborn groups and the autonomic, motor, organization of states, and responsiveness scores were compared. Linear regression was performed to test the association of the characteristics of mothers and neonates with the scores in the autonomic, motor, organization of states, and responsiveness domains., Results: The Newborn Behavioral Observation was performed with 170 newborns (eight twins and 77% preterm newborns). Approximately 15% of the mothers were adolescents and had nine years of schooling, on average. The groups differed regarding weight for gestational age, age at observation, APGAR score, feeding, and primiparity. The linear regression adjusted for these variables showed that only prematurity remained associated with differences in the scores of the motor (p=0.002) and responsiveness (p=0.02) domains. No statistical difference was observed between the groups in the score attributed to one's own knowledge prior to the session (p=0.10). After the session, these means increased in both groups. This increase was significantly higher in the preterm newborn group (p=0.02)., Conclusions: The Newborn Behavioral Observation increased the mothers' knowledge about the behavior of their children, especially in mothers of preterm newborns, and identified differences in the behavior of preterm newborns and full-term newborns regarding the motor and responsiveness domains., (Copyright © 2017 Sociedade Brasileira de Pediatria. Published by Elsevier Editora Ltda. All rights reserved.)

Objective: To verify whether the use of the T-piece resuscitator compared with the self-inflating bag in preterm infants ventilated at birth modifies survival to hospital discharge without major morbidities., Design: Pragmatic prospective cohort study., Setting: 20 Brazilian university hospitals of Brazilian Network on Neonatal Research. Patients were 1962 inborn infants in 2014-2015 ventilated at birth with 23-33' weeks gestation and birth weight 400-1499 g without malformations. Patients transferred until the 27th day after birth were excluded., Interventions: Positive pressure ventilation at birth with T-piece resuscitator or self-inflating bag without positive end expiratory pressure valve. Intervention with ventilation followed the Brazilian Society of Pediatrics guidelines. The choice of the equipment was at the neonatologist's discretion in each delivery. The main outcome measures were survival to hospital discharge without bronchopulmonary dysplasia, severe peri-intraventricular haemorrhage and periventricular leucomalacia. Logistic regression adjusted for confounding variables was applied for main outcome., Results: 1456 (74%) were only ventilated with T-piece resuscitator and 506 (26%) with the self-inflating bag. The characteristics of those ventilated with T-Piece resuscitator versus self-inflating bag were birth weight 969 ± 277 vs 941 ± 279 g, gestational age 28.2±2.5 vs 27.8±2.7 weeks and survival to hospital discharge without major morbidities 47% vs 35%. Logistic regression adjusted for maternal characteristics, obstetric and neonatal morbidities showed that the T-piece resuscitator increased the chance of survival to hospital discharge without major morbidities (OR=1.38; 95% CI 1.06 to 1.80; Hosmer-Lemeshow goodness of fit: 0.695)., Conclusion: This study is the first that highlights the effectiveness of T-piece resuscitator ventilation in improving relevant outcomes in preterm neonates., Competing Interests: Competing interests: None declared., (© Article author(s) (or their employer(s) unless otherwise stated in the text of the article) 2018. All rights reserved. No commercial use is permitted unless otherwise expressly granted.)

Background: This study explored the possible role of FGF23 in pediatric hypercalciuria., Methods: Plasma FGF23 was measured in 29 controls and 58 children and adolescents with hypercalciuria: 24 before treatment (Pre-Treated) and 34 after 6 months of treatment (Treated). Hypercalciuric patients also measured serum PTH hormone, 25(OH)vitD, phosphate, calcium, creatinine, and 24 h urine calcium, phosphate, and creatinine., Results: There were no differences in age, gender, ethnicity, or body mass index either between controls and patients, or between Pre-Treated and Treated patients. Median plasma FGF23 in controls was 72 compared with all patients, 58 RU/mL ( p = 0.0019). However, whereas FGF23 in Pre-Treated patients, 73 RU/mL, was not different from controls, in Treated patients it was 50 RU/mL, significantly lower than in both controls ( p < 0.0001) and Pre-Treated patients ( p = 0.02). In all patients, there was a correlation between FGF23 and urinary calcium ( r = 0.325; p = 0.0014). Treated patients had significantly lower urinary calcium ( p < 0.0001), higher TP/GFR ( p < 0.001), and higher serum phosphate ( p = 0.007) versus Pre-Treated patients., Conclusions: Pharmacological treatment of hypercalciuric patients resulted in significantly lower urinary calcium excretion, lower serum FGF23, and elevated TP/GFR and serum phosphate concentration, without significant changes in PTH. Further studies are indicated. This trial is registered with Clinical Registration Number RBR 8W27X5.

Aim: To investigate the prevalence of nutritional parameters of risk for cardiovascular disease (CVD) and kidney diseases in healthy preschool children., Methods: This is an observational cross-sectional study with 60 healthy children, of both genders, aged two to six years old and 56 mothers, in Belo Horizonte, Minas Gerais, Brazil. Preschool children and their families with regular activities at public schools were invited to paticipate in the study. The following characteristics were assessed: Socio-demographic condictions, clinical health, anthropometric, biochemical, lifestyle and data on food consumption. The 56 healthy children were divided into two groups, overweight (C1) and non-overweight (C2), as well as their mothers, respectively, in overweight (M1) and non-overweight (M2). Nutritional status was defined according to results obtained through the Anthro ® Software for nutritional analysis., Results: Thirty-five children were male, with mean age of 4.44 ± 1.0 years old. Eighty-nine percent of them were eutrophic, 86.7% were sedentary and they had five meals a day. Body mass index (BMI) for age and total cholesterol (TC) was higher on C1 ( P = 0.0001) and high density lipoprotein cholesterol (HDL-c) was higher on C2. Mothers were 32.5 ± 7.1 years old, mostly married and employed. Eighty-six percent of them were sedentary and 62.5% were overweight with BMI = 26.38 ± 5.07 kg/m 2 . Eighteen percent of the overweight mothers had isolated total hypercholesterolemia (TC levels elevated) and 12.5% had low HDL-c levels. The present study showed an association between overweight and obesity during the preschool years and the correspondent mothers' nutritional status of overweight and obesity (OR = 4.96; 95%CI: 0.558-44.17). There was a positive correlation between the food risk associated with CVD by children and mothers when their consumption was 4 times/wk ( P = 0.049; r = 0.516) or daily ( P = 0.000008; r = 0.892)., Conclusion: Analyzed children showed high rates of physical inactivity, high serum cholesterol levels and high consumption of food associated with risk for CVD and renal disease. Changes in habits should be encouraged early in kindergarten., Competing Interests: Conflict-of-interest statement: The authors declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Aim: To evaluate the parathyroid ultrasonography and define parameters that can predict poor response to treatment in patients with secondary hyperparathyroidism due to renal failure., Methods: This cohort study evaluated 85 patients with chronic kidney disease stage V with parathyroid hormone levels above 800 pg/mL. All patients underwent ultrasonography of the parathyroids and the following parameters were analyzed: Demographic characteristics (etiology of chronic kidney disease, gender, age, dialysis vintage, vascular access, use of vitamin D), laboratory (calcium, phosphorus, parathyroid hormone, alkaline phosphatase, bone alkaline phosphatase), and the occurrence of bone changes, cardiovascular events and death. The χ(2) test were used to compare proportions or the Fisher exact test for small sample frequencies. Student t-test was used to detect differences between the two groups regarding continuous variables., Results: Fifty-three patients (66.4%) had parathyroid nodules with higher levels of parathyroid hormone, calcium and phosphorus. Sixteen patients underwent parathyroidectomy and had higher levels of phosphorus and calcium × phosphorus product (P = 0.03 and P = 0.006, respectively). They also had lower mortality (32% vs 68%, P = 0.01) and lower incidence of cardiovascular or cerebrovascular events (27% vs 73%, P = 0.02). Calcium × phosphorus product above 55 mg(2)/dL(2) [RR 1.48 (1.06, 2.08), P = 0.03], presence of vascular calcification [1.33 (1.01, 1.76), P = 0.015] and previous occurrence of vascular events [RR 2.25 (1.27, 3.98), P < 0.001] were risk factors for mortality in this population. There was no association between the occurrence of nodules and mortality., Conclusion: The identification of nodules at ultrasonography strengthens the indication for parathyroidectomy in patients with secondary hyperparathyroidism due to renal failure.

Aim: Autosomal recessive mutations in NPHS1 gene are a common cause of congenital nephrotic syndrome (CNS). The disorder is characterized by massive proteinuria that manifests in utero or in the neonatal period during the first 3 months of life. NPHS1 encodes nephrin, a member of the immunoglobulin family of cell adhesion molecules and the main protein expressed at the renal slit diaphragm. Currently, there are approximately 250 mutations described in the NPHS1 gene distributed among all nephrin domains. The main objective of this study was to perform the analysis of the NPHS1 gene in patients with congenital nephrotic syndrome in order to determine the molecular cause of the disease., Methods: Direct sequencing of NPHS1 gene in four children was performed., Results: Each patient was heterozygous for two pathogenic mutations disclosing the molecular cause of the disease in 100% of the cases. We identified six different mutations, consisting of one in-frame deletion, one frameshift, and four missense substitutions. The p.Val736Met mutation that is described here for the first time was considered pathogenic by different mutation predictive algorithms. Regardless of the type of mutation, three patients had a bad outcome and died, Conclusions: Despite the small size of the cohort, this study contributed to the increasing number of deleterious mutations in the NPHS1 gene by describing a new mutation. Also, since we identified NPHS1 pathogenic mutations as the cause of the disease in all cases analyzed, it might be a frequent cause of CNS in the South Eastern region of Brazil, although the analysis of a larger sample is required to obtain more indicative epidemiological data., (© 2015 Asian Pacific Society of Nephrology.)

Introduction: Pediatric urolithiasis has become more prevalent in recent decades, with high recurrence rates and considerable morbidity. Most children with idiopathic urolithiasis have an underlying metabolic abnormality and proper research provides therapeutic interventions to reduce the formation of new stones and its complications., Objective: To identify demographic and clinical characteristics of pediatric urolithiasis, etiology, treatment management, disease recurrence and patient outcomes in a tertiary care pediatric hospital., Methods: A retrospective descriptive study of pediatric patients admitted to the Hospital Infantil Joana de Gusmão in Florianópolis, SC, Brazil, who were diagnosed with urolithiasis, from January 2002 to December 2012. Data were obtained from medical records. Those patients with diagnosis confirmed by imaging and 24hr urine or single sample urine were included., Results: We evaluated 106 pediatric patients (65% M). Average age at diagnosis was 8.0 ± 4.2 and 85% of them had positive family history of urolithiasis. Abdominal pain, renal colic and urinary tract infection were the main manifestations. 93.2% had metabolic abnormality and hypercalciuria was the most common. Pharmacological treatment was established in 78% of cases. Potassium citrate and hydrochlorothiazide were used. Surgical treatment was performed in 38% of patients. There was response to treatment in 39% of patients with recurrence of urolithiasis in 34.2% of them. Only 4.7% of patients continued follow-up, 6.6% were referred to other services, 8.5% were discharged and 73.8% lost follow-up., Conclusion: Pediatric urolithiasis deserves a detailed metabolic evaluation after their initial presentation for treatment, monitoring and prevention of its formation and its complications.

In the past few decades pediatric urolithiasis has become more frequent. The reason for this increase is not completely clear but has been attributed to changes in climate, nutritional habits and possibly other environmental factors. Although less frequent than adult stone disease, urolithiasis in the pediatric age group is also related to significant morbidity, particularly since stones tend to recur, and, thus, should not be underestimated. Most children with idiopathic stone disease have an underlying metabolic abnormality substantiating the importance of metabolic evaluation already following initial diagnosis of urolithiasis. Identification of the metabolic abnormality allows for more specific prescription of non pharmacological and pharmacological interventions aimed at preventing recurrent stone formation. A better understanding of the causes of kidney stone disease will provide better strategies for stone prevention in children.

Objectives. Considering the differences in location, socioeconomic background, and cultural background, the aim of this study was to try to identify possible factors associated with the increased incidence of urolithiasis by comparing American and Brazilian children with stones. Methods. Data of 222 American and 190 Brazilian children with urolithiasis were reviewed including age, gender, body mass index, imaging technique used (ultrasound and computed tomography), and 24-hour urine volume and chemistries. Results. There were no differences between age and gender at diagnosis. Brazilian children were leaner but in no population did obesity rate exceed that of the general population. Ultrasound was most commonly used to diagnose stones, even more so in Brazilians. Decreased urine flow was more common among Americans (P = .004), hypercalciuria among Brazilians (P = .001), and elevated Ca/citrate ratio among Americans (P = .009). There were no differences between the groups in the frequency of hypocitraturia, hyperuricosuria, absorptive hyperoxaluria, and cystinuria. Conclusions. Despite some differences between the populations, the leading causes of urolithiasis among both were "oliguria," hypercalciuria, and high Ca/citrate ratio. In neither country was obesity the reason for the increase in incidence of urolithiasis, nor was the use of computed tomography. The similarities between the 2 populations call for combining efforts in addressing the leading causes of pediatric urolithiasis.

The common denominator for all types of rickets is hypophosphatemia, leading to inadequate supply of the mineral to the growing bone. Hypophosphatemia can result from insufficient uptake of the mineral from the gut or its disproportionate losses in the kidney, the latter being caused by either tubular abnormalities per se or the effect on the tubule of circulating factors like fibroblast growth factor-23 and parathyroid hormone (PTH). High serum levels of the latter result in most cases from abnormalities in vitamin D metabolism which lead to decreased calcium absorption in the gut and hypocalcemia, triggering PTH secretion. Rickets is a disorder of the growth plate and hence pediatric by definition. However, it is important to recognize that the effect of hypophosphatemia on other parts of the skeleton results in osteomalacia in both children and adults. This review addresses the etiology, pathophysiologic mechanisms, clinical manifestations and treatment of entities associated with hypophosphatemic rickets due to perturbations in renal tubular function.

Purpose: Due to environmental and social changes (and possibly obesity) as new risk factors for stone formation in adults and changes in imaging techniques, we assessed whether etiologies of primary pediatric urolithiasis have changed, and if relationships exist between the condition and obesity or imaging technique., Materials and Methods: All pediatric patients with documented primary urolithiasis who underwent serum and 24-hour urine analyses between 1999 and 2010 were evaluated. Age at diagnosis, gender, body mass index and imaging technique were recorded., Results: Of the 222 patients (48% male) all had normal serum creatinine, electrolytes and minerals. Primary pediatric urolithiasis was diagnosed by ultrasound in 73% of cases and computerized tomography in 27%. Mean ± SD annual incidence of urolithiasis per 1,000 clinic visits increased from 2.4 ± 1.5 in the first half of the study period to 6.2 ± 2.1 in the second half (p <0.005). Mean ± SD age at diagnosis was 11.8 ± 3.8 years and body mass index was 21.7 ± 5.7 (rate of overweight 15%). A total of 140 patients had urine output less than 1.0 ml/kg per hour, with this being the only abnormality in 54. Hypercalciuria was observed in 46% of patients, hypocitraturia in 10% and high calcium-to-citrate ratio in 51%. Mild absorptive hyperoxaluria was noted in 3 patients and hyperuricosuria in 11, with all 14 exhibiting at least 1 additional abnormality. Cystinuria was present in 1 patient. No etiology was identified in 20 patients (9.0%)., Conclusions: Oliguria and hypercalciuria continue to be the most common etiologies of pediatric primary urolithiasis, followed by hypocitraturia. The recent increase in stone incidence is unlikely due to increased use of computerized tomography. Incidence of obesity was not higher than in the general population. Hyperoxaluria and cystinuria are rare, and thus might not be indicated in the initial analysis., (Copyright © 2013 American Urological Association Education and Research, Inc. Published by Elsevier Inc. All rights reserved.)

Background: Recent studies suggest that cytokines modulate bone turnover. Idiopathic hypercalciuria (IH) seems to be associated with bone mineral loss. Therefore, the aim of this study was to assess cytokines involved in bone turnover in patients with IH., Methods: Plasma and spot-urine levels of interleukin (IL)-1β, IL-6, IL-8, tumor necrosis factor alpha (TNF-α), transforming growth factor β1 (TGF-β1), and monocyte chemoattractant protein (MCP-1) were measured in 70 children and adolescents with IH and in 37 healthy controls. Patients with IH were subdivided according to their calciuria at the time of sample collection: ≥4 mg/kg/day (persistent IH, n=27) and below 4 mg/kg/day (controlled IH, n=43). Cytokines were determined by enzyme-linked immunoassay., Results: Plasma and urinary concentrations of IL-1β, IL-6, IL-8, and TNF-α were undetectable in all groups. No differences were found between controlled and persistent hypercalciuria for plasma and urinary levels of MCP-1 and TGF-β1. On the other hand, MCP-1 levels were significantly higher in both subgroups of IH in comparison to healthy controls. Furthermore, urinary MCP-1 levels of IH patients correlated positively with bone mineral content (p=0.013)., Conclusion: Although cytokine measurements did not allow the differentiation between persistent and controlled IH, our findings suggest that MCP-1 might play a role in patients with IH.

Idiopathic hypercalciuria (IH) is the leading metabolic risk factor for urolithiasis and affects all age groups without gender or race predominance. IH has a high morbidity with or without lithiasis and reduced bone mineral density (BMD), as described previously in pediatric patients as well as in adults. The pathogenesis of IH is complex and not completely understood, given that urinary excretion of calcium is the end result of an interplay between three organs (gut, bone and kidney), which is further orchestrated by hormones, such as 1,25 dihydroxyvitamin D, parathyroid hormone, calcitonin and fosfatonins (i.e., fibroblast growth-factor-23). Usually, a primary defect in one organ induces compensatory mechanisms in the remaining two organs, such as increased absorption of calcium in the gut secondary to a primary renal loss. Thus, IH is a systemic abnormality of calcium homeostasis with changes in cellular transport of this ion in intestines, kidneys and bones. Reduced BMD has been demonstrated in pediatric patients diagnosed with IH. However, the precise mechanisms of bone loss or failure of adequate bone mass gain are still unknown. The largest accumulation of bone mass occurs during childhood and adolescence, peaking at the end of the second decade of life. This accumulation should occur without interference to achieve the peak of optimal bone mass. Any interference may be a risk factor for the reduction of bone mass with increased risk of fractures in adulthood. This review will address the pathogenesis of IH and its consequence in bone mass.

Objective: To describe the use and determine the prevalence of off-label and unlicensed drug use prescribing in a pediatric intensive care unit in a Southeastern Brazilian hospital, Methods: Cross-sectional study of inpatients in a pediatric intensive care unit from May 2008 through January 2009. The classification according to the Brazilian regulatory agency (Agência de Vigilância Sanitária - Anvisa) approval criteria was based on the Anvisa electronic package insert list, Pharmaceuticals Dictionary, and the analysis was conducted through R software., Results: We analysed 1,054 prescription items for 73 patients. Females predominated (52%), and the patients' age ranged from 0 to 16 years. Among the prescribed items, 23.4% were off-label, 12.6% were unlicensed, 1.4% were both off-label and unlicensed, 86% had at least one item off-label, and 67% had at least one unlicensed drug. The most frequently prescribed therapeutic groups were systemic anti-bacterial, analgesic, psycholeptic, and antiasmathic agents., Conclusion: The current study results confirm the high prevalence of unlicensed and off-label drug use in a pediatric intensive care unit.

Children with idiopathic hypercalciuria (IH) may have a reduced bone mineral density (BMD), which could impact on bone health in adulthood. There is currently no strong evidence for a preferred treatment of such children. The aim of our study was to evaluate the BMD z-score before and after treating children and adolescents with IH with potassium citrate and thiazides. The study consisted of a historical cohort of 80 pediatric patients who were evaluated between October 1989 and November 2010. Bone scanning and densitometry measurements were made with dual-emission X-ray absorptiometry. Lumbar-spine BMD (g/cm(2)) and BMD z-score were evaluated before and after treatment. The t test and Mann-Whitney U test were used for statistical analysis. Forty-three boys and 37 girls were followed for a median time of 6.0 years. Median calcium excretion before and after treatment was 5.0 and 2.6 mg/kg/24 h, respectively. The BMD z-score changed significantly from -0.763 ± 0.954 (mean ± SD) to -0.537 ± 0.898 (p < 0.0001) before and after treatment, respectively. The BMD z-score of the patients improved with treatment, suggesting a beneficial effect and potential need for treatment. However, the lack of a control group points to the need for future studies to corroborate this outcome.

Objective: The ideal combination of premedication for neonatal tracheal intubation has not been established. The aim of this preliminary study was to compare the intubation conditions between propofol and midazolam as premedication for tracheal intubation in neonates., Study Design: A double-blinded, randomized, controlled trial was performed, and 20 preterm neonates (28 to 34 week) underwent tracheal intubation following the use of remifentanil associated to either propofol (n = 10) or midazolam (n = 10). Intubation conditions were scored according to a four-point scale., Result: According to the main outcome measured (identification of a 50% difference in the intubation conditions), there were no differences regarding the number of attempts and the overall intubation conditions among the groups (P = 1.00)., Conclusion: Both combinations of premedications have no differences regarding the quality of intubation, which could be of clinical interest. Besides midazolam, propofol could be a valid alternative as hypnotic for premedication for endotracheal intubation in neonates.

Unlabelled: Remifentanil is a relatively new ultrashort action synthetic opioid. Studies on the use of remifentanil in neonatology have emerged demonstrating its effectiveness and safety in neonates. The present study describes the use of remifentanil in both full-term and premature newborns, highlighting the theoretical benefits for this population in terms of both neonatal intensive care and anaesthesia. A Medline search was undertaken of all reviews and reports about the use of remifentanil in neonates published between 1996 and 2009 using MeSH search terms 'remifentanil', 'analgesia', 'anaesthesia', 'newborn' and 'neonate'. The review points that remifentanil has been used with advantages in newborns including preterm neonates and even for foetal anaesthesia. It proved to be a good option to attenuate the hemodynamic/endocrine markers of stress related to surgery. Owing to its unique pharmacokinetic profile, shorter extubation times can be achieved what makes the drug also a good option for short duration invasive procedures in NICUs (InSurE). A concern on its use is that the hemodynamic response (hypotension) may become significant when the drug is associated to other drugs like sevoflurane., Conclusion: Remifentanil seems to be an effective and safely used opioid for neonatal intensive care and anaesthesia practice., (© 2010 The Author(s)/Journal Compilation © 2010 Foundation Acta Paediatrica.)

The pathogenesis of idiopathic nephrotic syndrome (INS) remains unknown. Several findings suggest a role for the immune system. This study aimed to evaluate immune mediators in INS by measuring plasma and urinary levels of transforming growth factor beta1 (TGF-beta1), monocyte chemoattractant protein-1 (MCP-1/CCL2), regulated on activation normal T-cell expressed and secreted (RANTES/CCL5) and IL-8 (IL-8/CXCL8) in pediatric patients with INS and in age-matched healthy controls. Patients were divided according to their response to corticosteroids: steroid-sensitive (SS, n = 8), or steroid-resistant (SR, n = 24). Immune mediators were also compared in regard with disease activity (relapse and remission). Immune mediators were measured by ELISA. Plasma TGF-beta1 levels in SR patients were approximately 2.8-fold higher than control values (p < 0.05). Urinary IL-8/CXCL8 was 2.9-fold higher in INS patients in relapse (proteinuria >100 mg/m2/24 h) when compared with patients in remission (p < 0.05), and levels had a positive correlation with individual proteinuria values (p < 0.05). Urinary IL-8/CXCL8 was significantly higher in relapsed SR than in SS patients in remission. No changes in MCP-1/CCL2 and RANTES/CCL5 levels were detected. Our findings suggest that IL-8/CXCL8 and TGF-beta1 are involved in the pathogenesis of INS: IL-8/CXCL8 associated with local changes in glomerular permeability and TGF-beta1 could be related to worse response to corticosteroids.

Unlabelled: Patients with chronic renal failure(CRF) in hemodialysis(HD) programs comprise a risk group for acquisition of hepatitis C virus(HCV) infection. The objectives were to evaluate the seroprevalence of HCV in patients submitted to HD in State of Minas Gerais(MG), southwest of Brazil; to correlate this seroprevalence with the time of treatment on HD; to investigate the anti-HCV seropositivity in health professionals, to investigate the existence of a correlation between mean HCV seroprevalence and the human development index (HDI). Patients from 66 healthcare units(HU) were studied using a validated questionnaire and considering the positive values of anti-HCV(Elisa III) tests performed in these units between january and december 2003., Results: the majority of patients were male (56.2%), between 41 and 60 years old. The mean seroprevalence of HCV in the 66 healthcare units was 13+/-9.5%; the three-monthly seroprevalence was below 20%, 15% and 10% in 75%, 50% and 40% of healthcare units, respectively. When the HU were grouped according to HCV seroprevalence into low(<5%), medium(5-15%) and high seroprevalence(>15%), 20% of the units have low, 42% medium and 37.5% were found to have high seroprevalence. No correlation was found between HDI and HCV seroprevalence (r=0.42; p=0.174) but in the regions in which the HDI was higher, HCV seroprevalence was also higher. There was a positive correlation between HCV seroprevalence and time on HD in 884 patients in the 4 HU (p<0.001). The seroprevalence of HCV was investigated in 387 healthcare professionals(29%) working in 14 HU. They were divided into two groups according to their time of professional activity: <10 y (G1) and >10 y (G2). In G1, there were no cases of anti-HCV seropositivity. In G2, 3 members of the staff were anti-HCV seropositive. The mean time of work of the seropositive staff in the HU was 15.6 years. The seroprevalence of HCV was 0.8% when all the healthcare professionals were taken into consideration. There was no statistically significant difference with respect to HCV seroprevalence between G1 and G2 with respect to the time of occupational exposure (p=0.27)., Conclusion: the seroprevalence of HCV in patients on HD in MG is 13+/-9.5% and was <10% in 40% of the HU; there was no statistically significant correlation between HDI and seroprevalence of HCV in the healthcare units evaluated; there was a statistically significant correlation between HCV seroprevalence and time of treatment on HD; HCV seroprevalence in the health professionals studied was 0.8% and similar to the literature.

The aims of this study were to describe anthropometric data and glycemic and lipidic profiles of HIV-infected patients treated or not with antiretrovirals (ARV) drugs, and to assess association between these drugs and body composition changes, lipid and glucose homeostasis disturbances. There were 176 patients included (133 ARV-treated patients and 43 ARV-naïve). The patients were submitted to clinical evaluation, laboratorial analysis, ultrasonographic measurements, bioelectrical impedance analysis and skin folds thickness measurements. The ARV-treated group showed higher waist-to-hip ratio (p= 0.0002), higher intra-abdominal fat thickness measured by ultrasonography (p= 0.003) and lower bicipital (p= 0.01) and tricipital (p= 0.0002) skin folds. This group also showed higher triglyceride (p= 0.0002), total cholesterol (p= 0.00007), HDL cholesterol (p= 0.009), glucose measurements one hour (p= 0.01) and two hours (p= 0.001) after dextrose load, higher levels of fasting insulin (p= 0.03) and higher HOMAR index (p= 0.02). The antiretroviral drugs are associated with increased visceral fat and decreased peripheral fat pads. Beside that, these drugs are associated with atherogenic lipid profile and insulin resistance, two independent risk predictors of cardiovascular disease.

Unlabelled: The association between idiopathic hypercalciuria (IH) and reduced bone mineral density (BMD) has been described in adults and children. Frequently, hypocitraturia (HC) is an associated condition. To determine the effect that HC may have on bone metabolism of these patients, we studied 88 children with IH at diagnosis, divided into the following groups: group 1-44 (50%) patients with associated HC; group 2-44 (50%) patients without HC; group 3 (29 subjects), a healthy control group. Urinary and blood electrolytes, as long as urinary N-telopeptide, were measured. Lumbar spine (L2-L4) and femoral neck bone mineral density (BMD) and bone mineral content (BMC) were measured by dual energy X-ray absorptiometry. There was no difference in age between the three groups (P=0.80), but weight, height, body mass index, and bone age were lower (P<0.01) and serum intact parathyroid hormone (iPTH) was higher (P<0.05) in group 1 than in groups 2 and 3. N-telopeptide, measured in urine, did not differ between groups. The following bone densitometry parameters: lumbar spine BMC, BMC adjusted for height (BMCh), BMC adjusted for width of vertebrae (BMCw) and BMD, as well as femoral neck BMD, were significantly lower in group 1 than in groups 2 and 3 (P<0.01). When we corrected densitometry parameters for height, BMC was lower in group 1 and not in group 2 when compared with controls., Conclusions: Children with IH and associated HC may have a higher risk of bone mass loss and consequent osteopenia. Further studies are needed to assess the role that hypocitraturia may have in this form of bone disease.

Some children with idiopathic hypercalciuria (IH) develop bone alterations at some stage of the disease. The aims of this study were to evaluate bone mass in 88 children with IH (G1) at the time of diagnosis and to compare the findings with data for a control group of 29 normal children (G2). Kidney and bone metabolism markers were measured in both groups, and bone densitometry was performed. Serum alkaline phosphatase, intact parathyroid hormone, urinary calcium and uric acid were significantly higher in G1, whereas urinary volume and urinary citrate excretion were lower. The following densitometric parameters were significantly lower in G1: (1) lumbar spine (L(2)-L(4)) bone mineral density (BMD), bone mineral content (BMC), BMC corrected for height and for width of the vertebra, volumetric BMD (BMDvol), and Z score; (2) whole-body BMD; (3) femoral neck BMD. Lumbar spine BMDvol was reduced (osteopenia) in 35% of the patients compared with G2. N telopeptide, a urinary marker of bone resorption, was significantly higher in G1 than in G2, and was negatively correlated with lumbar spine BMD and BMDvol. Children with urinary lithiasis or idiopathic hyperuricosuria associated with IH showed no significant differences in bone metabolism compared with children without these associations. We conclude that (1) there is an altered bone metabolism in IH, with osteopenia already present at diagnosis in 35% of the patients; (2) N telopeptide is one of the most useful markers of bone alterations in IH, especially at an early stage of the disease; (3) investigation of bone metabolism is necessary in IH to prevent future serious consequences such as osteoporosis and bone fractures.

Objective: To obtain regional reference values for calcium, uric acid and citrate urinary excretion and establish a correlation between those excretions in 24-hour urine sample and single urine sample for their use in clinical practice., Methods: A hundred and twenty-five healthy children and adolescents were randomly chosen and submitted to the following protocol: clinical examination, biochemical analysis of blood, blood cell count, parathormone, 24-hour urine, fasting urine sample and stool test., Results: The maximum value of calcium excretion in 24-hour urine was 3.75 mg/kg; in mg/dl of the glomerular filtration rate, it was 0.10; and for the calcium/creatinine (mg/dl) ratio in the fasting urine sample was 0.25. Positive correlation was observed between calcium excretion in the 24-hour urine and the fasting sample (mg/dl and mg/dl of glomerular filtration rate). The maximum values of uric acid excretion in 24-hour urine were 600, 450, and 320 mg and 13, 15 and 18 mg/kg for adolescents, school and preschool children, respectively; in mg/dl of glomerular filtration rate, in the fasting urine sample, it was 0.47. Positive correlation was observed for the uric acid excretion in 24-hour urine and fasting urine samples. The mean values for the citrate excretion in 24-hour urine were 1.6, 1.1 and 0.5 mmol for adolescents, school and preschool children, respectively; for citrate/creatinine ratio, in the fasting urine sample the mean value was 0.3., Conclusions: The calcium and uric acid excretion in 24-hour urine showed correlation with those in the fasting urine sample, which allows their use for metabolic diagnosis, population studies and follow-up of patients with hypercalciuria and hyperuricosuria without voiding control; the citrate/creatinine ratio in the fasting urine sample can be used for controlling patients with hypocitraturia.

OBJECTIVE: To analyze the clinical history and evolution of children and adolescents with IH, emphasizing some of their peculiar features. METHODS: We followed 471 patients with IH at an outpatient clinic. Patients were submitted to the following protocol: abdominal X-ray, kidney and urinary tract ultrasonography; urinary ionogram, blood gas and biochemical analyses; 24-hour urine for measurement of calcium and other electrolytes and creatinine; urinalysis, urine culture and phase-contrast microscopy; second morning urine collected after fasting for measurement of calcium and creatinine. RESULTS: At the time of diagnosis, 6% of the patients were infants, 15% pre-school children, 55% school children, and 24% adolescents; 56% of them were boys. Clinical and laboratory findings were: 47% had hematuria and abdominal pain, 31% had isolated hematuria, 14% isolated abdominal pain, and 8% had urinary tract infection, nocturnal enuresis, suprapubic pain or urethralgia, or the frequency/urgency syndrome with urinary incontinence. Hypercalciuria was associated with urolithiasis in 56% of patients. There was association with hyperuricosuria in 18.5% of the cases, and hypocitraturia in 8.5% of the cases. Evolution was poor for 33% of the patients, with recurrence of nephrolithiasis, persistence of hematuria, and abdominal pain. CONCLUSIONS: IH must be diagnosed and treated with criteria in order to reduce consequences such as hematuria, abdominal pain, urinary stone formation and possible bone involvement. Signs and symptoms such as urgency and urinary incontinence, suprapubic pain and nocturnal enuresis may result from renal hyperexcretion of calcium.