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Febrile urinary tract infection (UTI) is currently considered the most frequent cause of serious bacterial illness in children in the first 2 years of life. UTI in paediatrics can irreversibly damage the renal parenchyma and lead to chronic renal insufficiency and related problems. To avoid this risk, an early effective antibiotic treatment is essential. Moreover, prompt treatment is mandatory to improve the clinical condition of the patient, prevent bacteraemia, and avoid the risk of bacterial localization in other body sites. However, antibiotic resistance for UTI-related bacterial pathogens continuously increases, making recommendations rapidly outdated and the definition of the best empiric antibiotic therapy more difficult. Variation in pathogen susceptibility to antibiotics is essential for the choice of an effective therapy. Moreover, proper identification of cases at increased risk of difficult-to-treat UTIs can reduce the risk of ineffective therapy. In this review, the problem of emerging antibiotic resistance among pathogens associated with the development of paediatric febrile UTIs and the best potential solutions to ensure the most effective therapy are discussed. Literature analysis showed that the emergence of antibiotic resistance is an unavoidable phenomenon closely correlated with the use of antibiotics themselves. To limit the emergence of resistance, every effort to reduce and rationalise antibiotic consumption must be made. An increased use of antibiotic stewardship can be greatly effective in this regard.

In the field of medical care, successful transition from pediatric-centered to adult-oriented healthcare can provide a sense of continuity in the development of youth, and prepare them to accept responsibility for and manage their own chronic kidney condition in complete autonomy. The so-called transition process requires the presence of some basic aspects: a multidisciplinary team, which acts as a bridge between child and adult services; a comprehensive clinical, cognitive, psychological, and social change for the young people; the involvement of family and caregivers. Within the framework of transition and chronicity during the developmental age, we selected international papers explaining models which agreed on some important steps in the transition process, although many differences can be observed between different countries. In fact, in Europe, the situation appears to be heterogeneous as regards certain aspects: the written transition plan, the educational programmes, the timing of transfer to adult services, the presence of a transition coordinator, a dedicated off-site transition clinic. We then analyzed some studies focusing on patients with renal diseases, including the first to contain a standardized protocol for transition which was launched recently in the USA, and which seems to have already achieved important positive, although limited, results. In Italy, the issue of transition is still in its infancy, however important efforts in the management of chronic kidney disease have already been initiated in some regions, including Emila Romagna, which gives us hope for the future of many young people.

Purpose: Idiopathic nephrotic syndrome (INS) is the most frequent form of childhood nephrotic syndrome. Steroids represent the best therapeutic option; however, inter-individual differences in their efficacy and side effects have been reported. To date, there is no way to predict patients’ resistance and/or dependence. Alterations in the cytokine profile of INS patients might contribute to proteinuria and glomerular damage and affect drug sensitivity. Methods: The cytokine plasma levels were measured in 21 INS children at diagnosis to investigate the association among cytokines pattern and clinical response. Patients were selected on the basis of their clinical response: 7 steroid sensitive (SS), 7 dependent (SD), and 7 resistant (SR). Significant results were then analyzed in 41 additional pediatric INS patients. Results: Within the 48 cytokines analyzed, macrophage migration inhibitory factor (MIF) was a good predictor of steroid response. Indeed, SR patients showed significantly higher MIF plasma levels compared with all others (p = 0.022; OR = 4.3, 95%CI = 1.2–25.4): a cutoff concentration of MIF > 501 pg/ml significantly discriminated SR patients (sensitivity = 85.7%, specificity = 71.4%). On the contrary, SD patients showed lower MIF plasma levels compared with others (p = 0.010; OR = 0.12, 95%CI = 9.2 × 10−3–6.7 × 10−1). Significant results were confirmed in the entire cohort. Conclusions: Our comprehensive cytokine analysis indicates that assessing MIF plasma levels at diagnosis could predict response to glucocorticoids in children with INS.

We assessed renal function outcome in children with congenital solitary kidney and evaluated prognostic risk factors.We retrospectively studied the clinical charts of 210 children presenting with congenital solitary kidney at 2 pediatric nephrology and 5 pediatric units between January 2009 and October 2012. Children 0 to 18 years old with a congenital solitary kidney confirmed by scintigraphy were enrolled. Of the patients 146 were suitable for analysis. Median followup was 4.6 years. Primary outcome was decreased estimated glomerular filtration rate, and secondary outcome was occurrence of proteinuria and/or systemic hypertension. Primary outcome-free survival analysis was performed, including multiple regression analysis of significant risk factors.Decreased estimated glomerular filtration rate was present in 12% of children at a median age of 2.2 years. Primary outcome-free survival analysis revealed an estimated event-free survival of 82% (95% CI 74% to 91%) at 10 years. Estimated survival rate was significantly decreased in children with additional congenital anomalies of the kidney/urinary tract (54% vs 88% overall) or insufficient renal length vs expected for normal congenital solitary kidney. The latter was the strongest predictor of decreased estimated outcome-free survival (49% vs 89%, p0.001). Occurrence of proteinuria and/or systemic hypertension was present in less than 5% of children.Some children with congenital solitary kidney show decreased glomerular filtration rate. Associated anomalies of the kidney/urinary tract and insufficient renal length appear to be significant risk factors. Adequate length of the congenital solitary kidney is a key parameter for maintenance of renal function and should be examined routinely during followup.

Aim Our aim was to update the recommendations for the diagnosis, treatment and follow‐up of the first febrile urinary tract infection in young children, which were endorsed in 2012 by the Italian Society of Pediatric Nephrology. Methods The Italian recommendations were revised on the basis of a review of the literature published from 2012 to October 2018. We also carried out an ad hoc evaluation of the risk factors to identify children with high‐grade vesicoureteral reflux or renal scarring, which were published in the previous recommendations. When evidence was not available, the working group held extensive discussions, during various meetings and through email exchanges. Results Four major modifications have been introduced. The method for collecting urine for culture and its interpretation has been re‐evaluated. We have reformulated the algorithm that guides clinical decisions to proceed with voiding cystourethrography. The suggested antibiotics have been revised, and we have recommended further restrictions of the use of antibiotic prophylaxis. Conclusion These updated recommendations have now been endorsed by the Italian Society of Pediatric Nephrology and the Italian Society for Pediatric Infectivology. They can also be used to compare other recommendations that are available, as a worldwide consensus in this area is still lacking.

The term osteopetrosis describes a group of rare hereditary diseases of the skeleton, characterized by an increase in bone density, caused by a defect in the development or function of osteoclasts. It comprises a clinically and genetically heterogeneous conditions ranging from infantile onset life-threatening forms to mildest adult onset forms. "Malignant" osteopetrosis is characterized by bone fragility, short stature, compressive neuropathies, hypocalcaemia, pancytopaenia. The deficiency of carbonic anhydrase II causes a moderate form, presenting classically as a triad of osteopetrosis, renal tubular acidosis (RTA), and cerebral calcification. This condition leads to specific craniofacial dysmorphisms associated with upper airway obstruction that may result in obstructive sleep apnea. Herein we report a case of osteopetrosis with RTA associated with severe OSAS successfully treated with continuous positive airway pressure (CPAP).

Urinary Tract Infections (UTIs) are common in every day pediatric practice. During the first 6 years of life, circa 6 to 7% of girls and 2.5% of boys will develop a UTI. UTI fall into two categories: non febrile lower UTI or cystitis and febrile upper UTI or pyelonephritis. The latter is considered by many to be the most common serious bacterial illness in children [1]. If UTI is suspected, urinalysis and urine culture should be performed. Collection methods used in clinical practice can be either invasive or non invasive, yet all carry a risk of contamination by bacteria not present in the bladder. Clean voided methods are preferable as they are quite easy to perform and reliable, while invasive methods should be limited to children in poor general health [2]. In the presence of clinical signs and positive urine analysis, while awaiting the results of antimicrobial sensitivity testing, antibiotic treatment should be started as soon as possible, considering local resistance patterns. The route of administration, either parenterally or orally, depends on the condition of the child and the severity of the infection and does not affect the duration of fever, recurrence of UTI or the incidence of post infectious scars. The need for imaging after a first febrile UTI has long been debated. New insights have led us to consider less aggressive imaging strategies, given the high rate of spontaneous resolution of vesico ureteral reflux with age and the good renal outcome for patients with acquired scarring. Therefore, voiding cystography and renal dimercaptosuccinic acid scintigraphy are not routinely recommended [2-4]. It is known that there are several risk factors for the recurrence of UTI. Some are not modifiable (age, gender, familiarity), while others (reflux, phimosis, bladder function, voiding habits, constipation and fluid intake) are modifiable thanks to behavioral changes and/or medical treatments. As regards preventive interventions, the most controversial is the use of antibiotic prophylaxis and currently none of the recently published guidelines recommend a routine use [2,4].

OBJECTIVE: To evaluate the yield, economic, and radiation costs of 5 diagnostic algorithms compared with a protocol where all tests are performed (ultrasonography scan, cystography, and late technetium99dimercaptosuccinic acid scan) in children after the first febrile urinary tract infections. METHODS: A total of 304 children, 2 to 36 months of age, who completed the diagnostic follow-up (ultrasonography, cystourethrography, and acute and late technetium99dimercaptosuccinic acid scans) of a randomized controlled trial (Italian Renal Infection Study 1) were eligible. The guidelines applied to this cohort in a retrospective simulation were: Melbourne Royal Children’s Hospital, National Institute of Clinical Excellence (NICE), top down approach, American Academy of Pediatrics (AAP), and Italian Society of Pediatric Nephrology. Primary outcomes were the yield of abnormal tests for each diagnostic protocol; secondary outcomes were the economic and radiation costs. RESULTS: Vesicoureteral reflux (VUR) was identified in 66 (22%) children and a parenchymal scarring was identified in 45 (15%). For detection of VUR (47/66) and scarring (45/45), the top down approach showed the highest sensitivity (76% and 100%, respectively) but also the highest economic and radiation costs (€52 268. 624 mSv). NICE (19/66) and AAP (18/66) had the highest specificities for VUR (90%) and the Italian Society of Pediatric Nephrology had the highest specificity (20/45) for scars (86%). NICE would have been the least costly (€26 838) and AAP would have resulted in the least radiation exposure (42 mSv). CONCLUSIONS: There is no ideal diagnostic protocol following a first febrile urinary tract infection. An aggressive protocol has a high sensitivity for detecting VUR and scarring but carries high financial and radiation costs with questionable benefit.

Renal hypodysplasia (RHD) is a congenital disorder, characterized by an abnormally developed kidney. Mutations in genes such as PAX2, HNF1-beta, TCF2, EYA1, that encode factors critical in early renal development, are being found. RHD is the leading cause of chronic renal failure in childhood, with or without associated urologic abnormalities such as vesicoureteric reflux and urinary tract obstruction. Antenatal detection has improved understanding of this disorder, resulting in enhanced outcomes through earlier intervention, including peritoneal dialysis. Management requires a multidisciplinary team approach that commences prior to the birth of the child.

Background Infant mortality rate (IMR) is a key public health indicator. Maternal age is a well-known determinant of pregnancy and delivery complications and of infant morbidity and mortality. In Italy the Infant Mortality Rate was 3.7/1000 during 2005, lower than the average IMR for the European Union (4.94/1000). Sicily is the Italian region with the highest IMR, 5/1000, and neonatal mortality rate (NMR), 3.8/1000, with substantial variation among its nine districts. The present study compared a high IMR/NMR district (Messina) with a low IMR/NMR district (Palermo) during the period 2004-2006 to evaluate potential determinants of the IMRs' differences between the two districts and specifically the impact of maternal age. Methods The Death Causes Registers identified all deaths during the first year of life recorded among infants born to residents of the two districts in 2004-2006. For every case, available hospital charts records were abstracted using a standardized form designed to capture information on potential determinants of infant death. For each district and for each year, IMRs and NMRs were computed. Chi-squared statistics tested the significance of differences between district-specific IMRs. A Poisson regression model was used to analyze the relationship between maternal age, district of residence and IMR. Results The 246 death registry-confirmed cases included 143 (58.1%) males and 103 (41.2%) females, with mean age at death of 33.3 days (SD: 64.5, median: 5.5). The average IMR for 2004-2006 was significantly higher for the Messina district than for the Palermo district (p = 0.0001). The IMR ratio was 1.6 (95%CI: 1.2 - 2.1). The IMRs declined from 2004 to 2006. A significant interaction (p = 0.04) between maternal age and district of residence was documented. Conclusion The association between advanced maternal age and infant deaths in the Messina district was due in part to the excess of newborns from advanced age mothers, but also to increased risk of death among such newborns. The significant interaction between district of residence and maternal age indicated that the IMR excess in the Messina district cannot be explained by disproportionately high live birth rates among older mothers and suggested the hypothesis that health care facilities in the Messina district could be less well prepared to provide assistance to the excess of high risk pregnancies and deliveries, as compared to Palermo district.