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Osteopetrorickets is a rare complication of autosomal recessive (“malignant”) osteopetrosis. Its prompt diagnosis is essential, because early suspicion of infantile osteopetrosis enables treatment with human stem cell transplantation, depending on the gene involved. It is important to identify not only the characteristic radiological changes of rickets, but also the coexistence of increased bone density, so as not to miss this very rare entity. Herein, a brief case report is presented.

Preterm birth has been associated with increased risk for developing hypertension and other chronic diseases during childhood and adulthood. The aim of the current prospective case-control study was to investigate the associations of preterm birth with ambulatory blood pressure (BP) levels and arterial stiffness during childhood and adolescence.The study population included 52 children and adolescents born preterm and 26 healthy children born full term with similar age. The participants underwent ambulatory BP monitoring (ABPM) and assessment of carotid-femoral pulse wave velocity (PWV).Preterm children presented higher night SBP z score values compared to controls, but did not differ in other ABPM parameters, office peripheral and central SBPs. Nocturnal hypertension was found in 78% (7/9) of ex-preterm children with ambulatory BP hypertension. Preterm birth was an independent predictor of PWV z score adjusted for heart rate. Estimated marginal means for PWV z score adjusted for age, sex, presence of kidney disease at birth, office BPs, night BPs, central SBP, and BMI z scores were significantly higher in preterm individuals compared to controls (0.703, 95% confidence interval [CI] 0.431-0.975 versus -0.19, 95% CI -0.574-0.536, respectively, P = 0.027). Preterm children who were overweight presented the highest values of night SBP and PWV z score.Preterm birth is associated with higher nocturnal BP and increased arterial stiffness in childhood and adolescence. Increased awareness for detection of hypertension and prevention of obesity in childhood could prevent future adverse cardiovascular outcomes in preterm individuals.

BACKGROUND AND AIMS This cross-sectional study investigates the association between adipokine profile, serum interleukin-6 (IL-6) levels and protein-energy wasting (PEW) in children with chronic kidney disease (CKD). METHOD Serum adiponectin, leptin, resistin and IL-6 were measured in 53 patients, with a median age of 12.9 years, presenting CKD stage 3–5. Body composition indices, lean tissue (LTI) and fat tissue index (FTI), were measured by bioimpedance analysis spectroscopy. PEW was defined as the presence of muscle wasting [LTI adjusted to height and age (LTI HA) Z-score RESULTS PEW was present in 8 (15.1%) patients. At least 1,2,3 and 4 PEW criteria were present in 25 (47.2%), 16 (30.2%), 9 (17%), 3 (5.6%) patients respectively. Among the PEW criteria, muscle wasting was present in 18 (34%), poor growth in 14 (30.2%), reduced appetite in 8 (15%) and low serum albumin in 13 (24.5%) patients respectively. PEW was more frequent in CKD stage 5 (P = .010). Adiponectin and resistin levels were higher in CKD stage 5 (P CONCLUSION In advanced paediatric CKD, adiponectin and resistin are probably involved in muscle wasting and systemic inflammation process respectively. Serum leptin reflects adiposity status. Apart from serum inflammatory cytokines, serum adiponectin may be a useful biomarker of PEW in children with CKD.

This cross-sectional study investigates the association between insulin resistance (IR) and serum uric acid (sUA) and relative fat (RFM) and lean mass (RLM) profiles in children with chronic kidney disease (CKD). RLM and RFM were assessed by bioimpedance spectroscopy in 41 children and adolescents. Normal weight obesity (NWO) was defined as normal height-age body mass index and RFM >85th percentile, according to age and sex. Homeostatic model assessment of insulin resistance (HOMA-IR) level >95th percentile, according to sex and pubertal stage, and sUA >7 mg/dl were used to define IR and hyperuricemia, respectively. High RFM (15 patients) and NWO (7 patients) were associated with higher HOMA-IR in total (p

In recent years, pulse wave velocity (PWV) has emerged as a surrogate marker of cardiovascular disease in children with cardiovascular risk factors. The aims of the present study were to identify determinants of PWV in children according to their weight status and to investigate the role of peripheral blood pressure and central hemodynamic parameters in the association between PWV and obesity.We included in the study healthy children and adolescents randomly selected from a school-based blood pressure screening study. All participants underwent ambulatory blood pressure monitoring and 24-h pulse wave analysis.Overweight and obese children had higher 24-h PWV, 24-h peripheral and central systolic blood pressure (SBP), and cardiac output than normal weight ones. Children with both overweight and hypertension presented the highest 24-h PWV values (p 0.001). Peripheral and central SBP, body mass index (BMI), and hemodynamic parameters, including stroke volume, cardiac output, total peripheral resistance, and cardiac index, were all associated with 24-h PWV. However, in stepwise regression analysis, 24-h peripheral and central SBP and cardiac index, but not BMI, were independent predictors of 24-h PWV. There were statistically significant differences in 24-h blood pressure and hemodynamic parameters among those on the lower and highest 24-h PWV quartile, but there were no significant differences in BMI among 24-h PWV quartile groups.Arterial stiffness is higher in overweight and obese children in the co-presence of hypertension. Peripheral and central SBP are the main determinants of 24-h PWV independent of weight status. Graphical abstract.

BACKGROUND AND OBJECTIVES In 2017, the American Academy of Pediatrics (AAP) recommended new blood pressure (BP) thresholds for the diagnosis of hypertension in children and adolescents. We assessed the impact of the AAP guideline, as compared to the Fourth Report and the 2016 European Society of Hypertension guidelines (ESH), on the prevalence of hypertension and the detection of left ventricular hypertrophy (LVH). METHODS We systematically searched for studies evaluating the impact of the 2017 AAP guidelines on the prevalence of hypertension and LVH compared with the Fourth Report or the 2016 ESH guidelines. Meta-analysis was performed to compare the overall risk of LVH between the guidelines. We used a random-effects model to synthesize quantitative data. RESULTS We included 18 observational studies in the systematic review with an overall moderate to high risk of bias. The AAP guideline identified more children with hypertension than the Fourth Report and the ESH guidelines. In the meta-analysis of three observational studies, the guidelines revealed similar associations with LVH [odds ratio (OR) = 3.89, 95% confidence interval (95% CI) 1.68-8.99 for AAP and OR = 3.19, 95% CI 1.14-8.88 for Fourth Report/ESH guidelines]. Qualitative analysis of two observational studies revealed similar predictive value of the guidelines for LVH in adult life. CONCLUSION Despite the higher prevalence of hypertension frequently reported by the adoption of AAP guideline BP thresholds compared with Fourth Report and the ESH guidelines, the new thresholds have not been proved to advance assessment of cardiovascular risk in terms of LVH currently the most accepted subclinical marker in youth.

Objective: This single center cross-sectional study aims to investigate the association between secondary hyperparathyroidism and body composition in pediatric patients with moderate (stage 3) and advanced (stage 4–5) chronic kidney disease (CKD).Methods: 61 patients (median age: 13.4 years) were included. Body composition indices, including lean tissue index (LTI) and fat tissue index (FTI), were measured using multi-frequency bio-impedance spectroscopy. Muscle wasting was defined as LTI adjusted to height-age (HA) z-score < −1.65 SD and high adiposity as FTI z-score > 1.65 SD. Serum mineral metabolism parameters, including serum intact parathormone (iPTH), calcium, phosphorus and 25-hydroxyvitamin D, as well as serum leptin were measured in each patient. In advanced CKD patients, the mean values of serum mineral laboratory parameters of the 6 months prior to body composition assessment were recorded, and alfacalcidol index, defined as weekly alfacalcidol dose (mcg/week) per pg/ml of iPTH × 1,000, was calculated.Results: In moderate CKD (31 patients), high iPTH (>90 ng/ml) was observed in 10 (32.3%) patients and was associated with higher FTI z-score (p = 0.022). Moreover, serum iPTH was negatively correlated to LTI HA z-score (rs = −0.486, p = 0.006), and positively correlated to serum leptin levels (rs = 0.369, p = 0.041). The positive correlation between FTI z-score and iPTH (rs = 0.393, p = 0.039) lost significance after adjustment for serum leptin. iPTH was positively associated with high adiposity (12 patients, 38.7%) after adjustment for the other mineral metabolism parameters (OR 1.023, 95% CI 1.002–1.045, p = 0.028). In advanced CKD (30 patients), no significant correlation was observed between iPTH and body composition indices and serum leptin levels. Eleven (36.7%) patients with muscle wasting presented lower alfacalcidol index (p = 0.017). Alfacalcidol index ≤ 24 was strongly associated with muscle wasting after adjustment for CKD stage and other mineral metabolism parameters (OR 7.226, 95% CI 1.150–45.384, p = 0.035).Conclusion: Secondary hyperparathyroidism is associated with high adiposity in moderate but not in advanced CKD, with leptin acting as a potential contributive factor. In advanced CKD, targeting higher alfacalcidol weekly dose per each unit of serum PTH seems beneficial for preventing muscle wasting.

OBJECTIVE We assessed the performance of the simplified American Academy Pediatrics (AAP) 2017 guideline table and a simplified table based on the Fourth Report blood pressure (BP) reference tables for high BP screening compared with the European Society Hypertension 2016 guideline diagnostic thresholds. METHODS We obtained data from a cross-sectional, school-based screening study in north Greece during 2013-2016. BP was measured by mercury sphygmomanometer. The simple tables' performance for high BP was assessed by receiver operator characteristic curve analysis, area under the curve (AUC), sensitivity, specificity, positive predictive value (PPV), and negative predictive value (NPV). RESULTS The study population included 1846 children aged 6-12 years and 986 adolescents aged 13-18 years. Compared with the European Society Hypertension 2016 classification, the AAP 2017 simple table showed AUC 0.93, sensitivity 95.5%, specificity 91.6%, PPV 35.9%, and NPV 99.7%, whereas the Fourth Report one showed AUC 0.96, sensitivity 99.2%, specificity 93.2%, PPV 42.1%, and NPV 99.9%. Comparing the prevalence of high BP by the two tables, we found agreement in 96.9% of the participants, and disagreement in 3.1% (kappa coefficient = 0.85, P

Lesch-Nyhan disease (LND) is a rare X-linked recessive inherited disorder caused by mutations in HPRT1 gene resulting in deficiency of hypoxanthine-guanine phosphoribosyltransferase (HPRT). LND is characterized by hyperuricemia and a spectrum of neurological and behavioral manifestations. We describe a rare case of a 14-month-old boy presenting with acute renal failure and hyperuricemia. The patient exhibited all features of LNS apart from self-injurious behavior. The enzymatic analysis demonstrated total inactivity of the HPRT, and the molecular analysis revealed a splice-site mutation in intron 3 leading to exon 4 exclusion. This splice-site mutation has been previously reported only twice.

This cohort study investigates the association between insulin growth factor-1 (IGF-1), bone mineral density, and frailty phenotype in children with chronic kidney disease (CKD). Forty-six patients (median age 14.5 years) were prospectively enrolled. Frailty phenotype was defined as the presence ≥ 3 of the following indicators: suboptimal growth/weight gain (body mass index height age 3 mg/l. Lumbar bone mineral apparent density (LBMAD) was measured by dual-energy X-ray absorptiometry, body composition by bioimpedance spectroscopy, and IGF-1 by enzyme-labeled chemiluminescent immunometric assay. Frailty phenotype (seven patients) was more frequent in advanced CKD (estimated glomerular filtration rate

This study recruited 85 healthy children and adolescents, aged 6–18 years, from a school-based blood pressure (BP) screening study and performed office BP measurements, 24-h ambulatory blood pressure monitoring (24-h ABPM) and 24-h pulse wave analysis. Prevalence of BP phenotypes was assessed, factors that may predict hypertension (HTN) in ABPM were examined and the effect of BP phenotypes, as well as school, office, and ambulatory BP parameters on pulse wave velocity (PWV), was investigated. Forty-five (54.9%) of the children were normotensives, 8 (9.7%) were white coat hypertensives (WCH), 19 (23.2%) had masked hypertension (MH), and 10 (12.2%) had sustained HTN. Estimated adjusted marginal means for 24-h PWV were 4.79 m/s (95% CI 4.65–4.94) for sustained hypertensives, 4.72 m/s (95% CI 4.62–4.82) for MH, 4.38 m/s (95% CI 4.23–4.54) for WCH, and 4.33 m/s (95% CI 4.26–4.40) for normotensives (sustained hypertensives versus normotensives and WCH, p

Antimicrobial prophylaxis is recommended for the prevention of urinary tract infections (UTI) in high-risk children. However, there is growing concern about the use of β-lactams as prophylaxis and subsequent development of antibiotic resistance.In this prospective, randomized, crossover controlled trial we compared cotrimoxazole (SXT) and second-generation cephalosporins (2GC) as UTI prophylaxis in children ranging in age from 1 to 60 months. Eligible patients were 1:1 randomized to receive either SXT or 2GC for the initial 6-month period (1 course), then switched to the other antimicrobial agent class for the subsequent course, with switching continuing after each course until the end of the study. Urethral orifice cultures (UOCs) were obtained at the time of switching antimicrobial prophylaxis.Among 97 children (mean age 13.6 months) on prophylaxis, breakthrough UTIs occurred during 13.3 % (10/75) of SXT courses and 10.3 % (8/78) of 2GC courses (p = 0.62). 2GC failed earlier than SXT (mean ± standard error: 0.81 ± 0.1 vs. 2.37 ± 0.36 months, respectively; p = 0.028). Pseudomonas aeruginosa and Enterococcus spp. were more frequently isolated after 2GC courses than after SXT courses [22.6 vs. 4.8 % (p = 0.02) and 20.7 vs. 4.8 % (p = 0.035), respectively]. Prophylaxis with 2GC significantly increased resistance to both 2GC and SXT, while SXT prophylaxis did not affect susceptibility to 2GC.While SXT and 2GC appear to be equally efficacious as UTI prophylaxis in children, the latter exert a broader effect on patients' flora and development of bacterial resistance, suggesting that SXT may be more appropriate for UTI prophylaxis than 2GC.

Pediatric kidney Tx has critically altered the outcome in ESRD pediatric patients. The aims of this study were to determine long-term graft and patient survival in a homogeneous ethnic population. We reviewed the medical charts of pediatric kidney Tx performed between 1990 and 2012 in Greece. Seventy-five kidney Txs were performed from LRD and 62 from DD. The 10- and 20-yr graft survival was higher in LRD Tx compared with DD Tx. Both patient and graft survival at 10 and 20 yr after Tx were similar in LRD Tx from grandparents compared with parents (92.9% vs. 93.4% 20-yr patient survival, 71.4% vs. 78.7% and 57.1% vs. 72.1%, 10- and 20-yr graft survival, respectively). However, there was a decreasing tendency in LRD Tx rates in period 2001-2012 compared with period 1990-2000 (47.1% vs. 62.7%). Risk factors for poor five-yr graft survival were DD Tx, and induction treatment with ALG compared with basiliximab, but their effect attenuated at 10 yr after Tx. In conclusion, Tx from LRD may offer efficient survival outcomes irrespective of donor age, suggesting that even older LRD could be an excellent option for the 1st kidney Tx in children and adolescents.

This school-based screening study assessed the prevalence of high blood pressure (BP) levels according to the European Society of Hypertension (ESH) 2016 guidelines. Moreover, risk factors for BP elevation, and the effect of geographic and seasonal factors on BP screening were investigated. BP and anthropometric measurements were obtained from 2832 children and adolescents, aged 6-18 years, during the period 2013-2016. Three BP measurements were performed using a mercury sphygmomanometer, and the mean of the last two was used for the analysis. Obesity was defined according to the International Obesity Task Force (IOTF) criteria. The prevalence of high-normal BP/hypertension and overweight/obesity was 3.7%/0.9%, and 22.9%/8.5%, respectively. The majority of the participants presenting high BP (≥90th percentile) were overweight or obese. Increased prevalence of high BP was observed during spring (5.5%) and winter (5%) compared with 2.5% in autumn (P

Atypical hemolytic uremic syndrome (aHUS) is frequently associated with gene mutations in complement-regulatory proteins and activators. Different complement C3 gene mutations have been associated with different outcomes in aHUS. We report the case of a 21-year-old male with a C3 heterozygous gene mutation (p.Ile1157Thr) who developed aHUS at the age of 10 months and had six relapses, the last at the age of 14.5 years. Each relapse was characterized by an apparent predominance of hematological manifestations with milder renal involvement and was followed by complete recovery, with creatinine values and hematological parameters usually recovering after the 3rd to 6th day of hospitalization. The patient was treated with plasma infusion, apart from the initial and the last episode, when dialysis was needed. Twenty years after the onset, he retains normal renal function, with no proteinuria or hypertension. One similar case of highly recurrent aHUS carrying the same C3 mutation as our patient with recovery of renal function has been previously reported. We further support that aHUS associated with the p.Ile1157Thr C3 mutation may be highly recurrent, but with recovered renal function. The prevalent p.Ile1157Thr C3 gene mutation has variable disease manifestations and both severe and milder renal phenotypes have been found.

Urinary tract infections (UTIs) due to extended-spectrum-β-lactamase (ESBL)-producing Enterobacteriaceae in children are becoming more frequent, and they are commonly treated initially with a second- or third-generation cephalosporin. We developed a murine model of ascending UTI caused by ESBL-producing Escherichia coli . Using this model, we investigated the renal bacterial burden, interleukin-6 (IL-6) expression, and histopathological alterations caused by ESBL- and non-ESBL-producing bacteria after 1, 2, or 6 days with or without ceftriaxone therapy. The renal bacterial burden, IL-6 concentration, and histological inflammatory lesions were not significantly different between mice infected with ESBL- and non-ESBL-producing bacteria without treatment at any of the time points examined. Following ceftriaxone administration, the bacterial burden was eliminated in the kidneys of mice infected with ESBL- and non-ESBL-producing bacteria on the 6th postinfection day. The histological analysis demonstrated that among mice treated with ceftriaxone, those infected with ESBL-producing bacteria had more profound renal alterations than those infected with non-ESBL-producing bacteria on the 6th day ( P < 0.001). In comparison, microbiological outcomes did not differ significantly between mice infected with ESBL- and non-ESBL-producing bacteria at any of the time points examined. The effectiveness of ceftriaxone in mice with UTIs due to ESBL-producing E. coli may have therapeutic implications; it is, however, hampered by limited activity on the histopathological lesions, a finding that needs further investigation.

Children with chronic kidney disease (CKD) are at high risk of developing impaired bone quality. Our aim was to investigate changes of bone quality in children with CKD in relation to their treatmant using two imaging techniques-dual energy X-ray absorptiometry and quantitative ultraSonography (QUS). Thirty-three patients with CKD (18 boys and 15 girls, mean age 10.37 ± 3.37 years) were evaluated with bone mineral density (BMD) measured by DXA at the lumbar spine and hip and with speed of sound (SOS) measured by QUS at the radius and tibia at the beginning and at the end of the study. The patient cohort consisted of 14 patients with CKD stage 3-4 not treated with dialysis (CKD group), 5 patients on peritoneal dialysis treatment (PD group) and 14 patients after kidney transplantation (RTx group). BMD measurements did not show any significant changes in CKD and PD patients during the study. There was a reduction in BMD measured at the lumbar spine, femoral neck and total hip in RTx patients that was approaching significance. During the 2-year follow-up, SOS measurements at the radius decreased significantly in PD patients, whereas SOS measurements at the tibia significantly improved in RTx patients. No significant changes in QUS parameters were recorded for patients in the CKD group. In conclusion, our study shows that QUS parameters seem to better reflect the state of hyperparathyroidism of renal osteodystrophy as they deteriorate significantly in patients on dialysis and improve after renal transplantation.

Objective:To assess the effect of ambulatory blood pressure (BP) parameters on arterial stiffness assessed by PWV in healthy children and adolescents.Design and method:Healthy children and adolescents, who consecutive volunteer to participate in the study, were recruited from a school-based BP scree

99mTc-dimercaptosuccinic acid (DMSA) scan is the golden standard for the diagnosis of acute pyelonephritis and renal scaring. We investigated the use of acute phase DMSA scan in infants presented promptly to the hospital because of the first episode of their febrile urinary tract infection (UTI).Ninety-eight infants with microbiologically confirmed first episode of febrile UTI were studied. DMSA scans were carried out within 7 days in these infants after admission. Infants with an abnormal acute DMSA scan underwent a second DMSA scan 6-12 months later.Overall, acute DMSA scan was abnormal in 16 (16.3%) of the 98 patients. There were no differences in sex, age, fever over 38.5°C, blood inflammation indices, or evidence of vesicoureteral reflux (VUR) between patients with normal and abnormal acute DMSA scan (P0.05). However, infants with grade III to V VUR as well as those with delayed treatment presented significantly increased renal involvement by acute DMSA scan (P0.05). The sensitivity and specificity of abnormal acute DMSA scan to predict grade III to V VUR were 50% and 88% respectively. Its positive and negative likelihood ratios were 4.16 and 0.57, respectively. Of 16 children with abnormal initial DMSA scan results, 14 underwent a second DMSA scan. Follow-up DMSA scans were normal in 12 of the 14 children.Parenchymal damage found in a minority of infants with febrile UTI presented promptly to the hospital. Acute phase DMSA scan should be carried out only in selected patients. An abnormal acute DMSA scan is a moderate predictor for dilated VUR and its ability to exclude VUR is restricted.

Our aim was to assess bone parameters in children with chronic kidney disease (CKD) with both dual-energy X-ray absorptiometry (DXA) and quantitative ultrasonography (QUS) and additionally with biochemical markers of bone turnover. Twenty children (12 boys and 8 girls) with CKD and a mean decimal age of 9.47 +/- 4.44 years were included in the study where anthropometric parameters (height and weight), pubertal status, bone mineral density (BMD) at lumbar spine, speed of sound (SOS) measured by QUS at radius and at tibia, and biochemical markers of bone metabolism were measured. Six patients (30%) had tibial SOS Z score

We report an unusual case of serious, multifocal, invasive infection due to community-acquired methicillin-resistant Staphylococcus aureus (CA-MRSA) in a 10-year-old girl with favorable outcome. The child manifested femoral osteomyelitis, pyomyositis, deep femoral vein thrombosis, pneumonia, encephalopathy, and disturbances of almost all organs. She remained in a critical condition for a week. Fever persisted for 6 weeks and acute phase reactants remained increased for 6 months, necessitating a 7-month antistaphylococcal therapy with a glycopeptide and clindamycin. This led to resolution of infection-associated problems during the subsequent 36 months of follow-up. CA-MRSA strain isolated from the patient harbored both staphylococcal chromosomal cassette type IV (SCCmec type IV) and Panton-Valentine leukocidin genes. A literature review of serious CA-MRSA infections indicated that only a small minority of published cases had favorable outcome.

BACKGROUND Hypertension presents high prevalence rates following kidney transplantation (Tx). The aims of the present study were to investigate the prevalence and possible risk factors for hypertension and blood pressure (BP) control over time after pediatric kidney Tx, as well as to assess possible effects of hypertension on graft survival. METHODS We reviewed the medical records of all pediatric kidney recipients followed up in our pediatric nephrology department. Hypertension was defined as systolic and/or diastolic BP greater than the 95th percentile for age and sex, or as being on antihypertensive medication. BP control was defined as normotension while on antihypertensive medication. RESULTS The study population included 74 pediatric kidney recipients (median age 11 years). The prevalence of hypertension was found 77% before Tx, 82.4%, 71.7%, and 61% at 1, 5, and 10 years after Tx, respectively. Deceased donor Tx and pre-transplant hypertension on antihypertensive medication were significant risk factors for hypertension after kidney Tx over the follow-up period. BP control among patients on antihypertensive treatment was 16.7% before Tx, 43.8%, 66.7%, and 42.9% at 1, 5, and 10 years post-Tx, respectively. Hypertensive patients at 10 years post-Tx had 8.079 times higher hazard of graft loss compared to normotensives (95% CI 1.561-41.807, P < 0.05). CONCLUSIONS Hypertension remains a frequent complication in pediatric kidney recipients even years after kidney Tx. BP control by antihypertensive treatment is unsatisfactory in about half of the patients. The adverse effects of hypertension on graft survival may appear in the long-term.

PURPOSE: We determined the prevalence, anatomical variants and coexisting complications of duplex collecting systems in children with a history of UTI. Additionally, we compared the prevalence and severity of the coexisting anomalies with those found in single systems. MATERIALS AND METHODS: We reviewed the records of children younger than 6 years who were evaluated following a first UTI during a 9-year period to identify those with duplex systems. Children without duplication anomalies comprised the control group. RESULTS: Of 774 evaluated children 63 (8%), more commonly females than males, had duplex systems. CDS were as common as IDS. VUR was the most commonly associated anomaly, with a higher prevalence in CDS (66%) and IDS (47%) compared to single systems (26%, p

Introduction: Antibiotics-induced acute interstitial nephritis (AIN) is a rare disorder in children, and the diagnosis is often delayed. However, many commonly prescribed antibiotics seem to be implicated. Patients and Methods: We reviewed the medical records of 6 children, age range from 10 months to 14 years, with biopsy-confirmed antibiotics-induced AIN. Clinical presentation, morphological findings, and outcomes are reported. Results: Symptoms of AIN started 2–4 weeks after antimicrobial therapy with β-lactam antibiotics in 5 children and with gentamicin in 1 child. All patients presented with acute renal failure and fever. The glomerular filtration rate was dramatically reduced in 2 cases and mildly reduced in 4 patients. Two of our patients had supportive treatment, 2 received corticosteroid therapy, and 2 children remained under peritoneal dialysis for 12 and 22 days, respectively. Five patients had a full recovery of their renal function, and 1 child, 2 years later, still presented impairment of the renal function. Conclusion: AIN should be considered in case of acute renal failure in children, mostly when other common causes have been excluded, and there is a history of drug exposure.

Autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy syndrome (APECED) or autoimmune polyendocrine syndrome type 1 (APS-1) is a rare autosomal recessive disease caused by mutations of the AutoImmune REgulator (AIRE) gene, an important mediator of tolerance to self-antigens. It is characterized by two out of three major components: chronic mucocutaneous candidiasis, hypoparathyroidism and Addison's disease. We present an 11-year-old girl suffering from recurrent episodes of mucocutaneous candidiasis and onychomycosis from 1 to 6 years of age, and transient alopecia at the age of 4 years. Hypoparathyroidism and dental enamel hypoplasia were diagnosed at 8 years. Autoantibodies to thyroid and adrenal glands were not detected and all other endocrine functions have remained normal. Genetic analysis revealed that the patient was homozygous for the mutation T16M in exon 1 of the AIRE gene (p.T16M, c.47C>T). This is the first APECED case reported for carrying this mutation in homozygous form. Parents were third cousins and heterozygous carriers of this mutation.

The outcome of patients with urinary tract infections caused by extended spectrum β-lactamases (ESBL)-producing bacteria (cases) was compared with that of matched controls with urinary tract infections caused by non-extended spectrum β-lactamases-producing isolates. Significantly, more case patients received inappropriate empiric therapy than controls. Nevertheless, clinical and microbiologic outcomes as well as formation of renal scars did not differ between the 2 groups.

We report a very unusual case of meningoencephalitis due to Listeria monocytogenes in a 7-month-old immunocompetent boy. Cerebrospinal fluid (CSF) culture was initially negative, but was positive on the seventh day. The disease was complicated by seizures and hydrocephalus managed with temporary ventriculostomy. The infant was discharged without obvious neurological sequelae after 30 days and developed without neurological or developmental sequelae at two years of age. Listeria is difficult to isolate and is not susceptible to third-generation cephalosporins commonly used for the empirical treatment of bacterial meningitis.

The A-1332G transition of the angiotensin II type 2 receptor (AT2R) gene was found to occur more often in males with ureteropelvic (UPJO) or ureterovesical junction obstruction (UVJO). However, other studies have shown controversial results. Tauhe frequency of this polymorphism was investigated in 275 Caucasian children (153 boys, 122 girls) with a wide spectrum of congenital anomalies both of upper (165) and lower (110) urinary tract system and in 200 controls (100 boys, 100 girls). Among the included malformations, renal agenesis and duplex collecting system (DCS) were studied for the first time. The frequency of the G allele did not differ among patients (193 of 397 total alleles, 48.6%) and controls (146 of 300, 48.7%). No significant difference was also found in the frequency of the G allele in subgroups of congenital uropathies compared with controls. When analysis was performed in males and females separately, no significant difference was found in the frequency of the G allele in male (45.1%) or female (50.8%) patients compared with male (57.0%) or female (44.5%) controls. Our data indicate that the AT2R gene A-1332G transition is not associated with the development of human congenital uropathies and further investigations should be carried out to unravel their etiology. Pediatr Res

A 33-day-old male infant was admitted to the neonatal intensive care nursery because of respiratory distress, grunting, cyanosis, and radiological findings of bilateral bronchopneumonia. He responded well to intensive therapy, but 11 days later developed hemolytic uremic syndrome, which was treated conservatively with prednisone and plasma transfusions with good response. The hemolytic uremic syndrome resolved, but he subsequently developed severe recurrent infections of unknown etiology and died at the age of 78 days. Necropsy findings revealed necrotizing enterocolitis as well as dysplasia of the thymus and other lymphoid tissues, compatible with the diagnosis of immunodeficiency disorder. Pediatr Nephrol

We describe three neonates with hyperkalemia and renal salt wasting during the 1st week of life. Endocrinological evaluation led to the diagnosis of selective hypoaldosteronism (HA) in two neonates and secondary pseudohypoaldosteronism (PHA) in one. The infant with PHA developed a urinary tract infection, and radiological investigation demonstrated a small dysplastic left kidney with vesicoureteral reflux. The electrolyte and hormonal disturbances in this infant persisted throughout the first months of life. The two infants with selective HA improved rapidly after administration of fludrocortisone orally and the electrolytes and renin values returned to normal. Secondary PHA and selective HA should be considered in the differential diagnosis in salt-losing neonates during the first days of life. Renal ultrasonography, urine culture, and assays of aldosterone and plasma renin activity should be performed in any infant presenting with hyperkalemia and salt wasting after the exclusion of congenital adrenal hyperplasia.

A 6-year-old boy presented with an acute infection caused by Mycoplasma pneumoniae associated with respiratory tract and kidney involvement. Renal manifestations included acute nephritis with decreased C3 fraction of serum complement, occurring concomitantly with the respiratory symptoms. The child had an excellent outcome, with rapid normalization of C3 and complete resolution of the acute nephritis. Pediatr Infect Dis J