Serum neutrophil gelatinase-associated lipocalin and cystatin C is associated with blood pressure in ex-preterm children and adolescents.

Background: As preterm birth is a risk factor for hypertension (HTN), biomarkers for early prediction of HTN in childhood is an emerging need. The aims of the study were to evaluate serum biomarkers in ex-preterm children and examine for associations with office peripheral and central SBP (cSBP), ambulatory BP parameters and pulse wave velocity (PWV).

Methods: This case-control study included children and adolescents born prematurely (ex-preterms) and at full term (controls).

X-linked hypophosphatemia due to a de novo novel splice-site variant in a 7-year-old girl with scaphocephaly, Chiari syndrome type I and syringomyelia.

X-linked hypophosphatemia (XLH) is a rare X-linked dominant inherited disorder caused by loss-of-function variants in the PHEX gene and characterized by renal phosphate wasting, hypophosphatemia, abnormal vitamin D metabolism, growth retardation and lower limb deformities. We describe a case of XLH-rickets in a 7-year-old girl with scaphocephaly, Chiari syndrome type I and syringomyelia, with a de novo non-canonical splice variant (c.1080-3C > G) in intron 9 of the PHEX gene, that has not been previously described.

Exploring systemic inflammation in children with chronic kidney disease: correlates of interleukin 6.

Background: Systemic inflammation (SI) is linked to chronic kidney disease (CKD) progression and multiple complications. Data regarding SI biomarkers in pediatric patients are scarce. This case-control and cross-sectional study investigates the correlation of neutrophil-lymphocyte ratio (NLR), platelet-lymphocyte ratio (PLR), total iron binding capacity (TIBC) and serum albumin to serum interleukin-6 (IL-6).

Sources of error in direct radionuclide cystography in children and the need for a standardized, manometric apparatus.

Direct radionuclide cystography (DRC) is a safe and reliable method for the detection and follow-up of vesico-ureteral reflux (VUR). Since the first DRC was performed, during the early 60, the method has undergone many refinements. Themanometric approach, which was first introduced in 1984, provides a correlation between the bladder pressure and VUR visualization.

Association Between Adipokine Profile, Systemic Inflammation, Muscle and Protein Energy Wasting in Children With Chronic Kidney Disease.

Objectives: This cross-sectional study explores the association of adipokines and interleukin-6 (IL-6) with muscle and protein energy wasting (PEW) in children with chronic kidney disease (CKD).

Methods: We measured serum adiponectin, leptin, resistin and IL-6 in 53 patients with CKD stage 3-5. Lean tissue (LTI) and fat tissue index (FTI) were estimated by bioimpedance analysis spectroscopy.

Severe hypophosphataemia can be an early sign of osteopetrorickets: a case report.

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.

Prevalence of masked hypertension and its association with left ventricular hypertrophy in children and young adults with chronic kidney disease: a systematic review and meta-analysis.

Objectives: There are limited studies using ambulatory blood pressure monitoring (ABPM) to assess blood pressure (BP) status in young patients with chronic kidney disease (CKD) on dialysis or after kidney transplantation. The aim of this meta-analysis is to estimate the prevalence of both white-coat hypertension (WCH) and masked hypertension, along with the prevalence of left ventricular hypertrophy (LVH), in children and young adults with CKD on dialysis or after kidney transplantation.

Methods: We performed a systematic review and meta-analysis of observational studies assessing the prevalence of BP phenotypes using ABPM, in children and young adults with CKD stages 2-5d.

Arterial stiffness and nocturnal hypertension in preterm children and adolescents.

Background And Objectives: 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.

Methods: The study population included 52 children and adolescents born preterm and 26 healthy children born full term with similar age.

Bilateral Gonadoblastoma in a 6-Year-old Girl With Frasier Syndrome: Need for Early Preventive Gonadectomy.

Frasier syndrome (FS) is a rare condition, caused by splice-site mutations of intron 9 in the Wilms' tumor suppressor gene 1 (WT1 gene). The WT1 protein is essential for urogenital development and patients with 46XY karyotype present with female (FS type 1) or male phenotype, gonadal dysgenesis, progressive glomerulopathy, and high risk of gonadoblastoma. We describe a female patient with an IVS9+4C>T donor splice-site mutation, who underwent a preventive gonadectomy at the age of 6 years due to imaging findings of dysplastic gonads.

Comparison of the 2017 American Academy of Pediatrics with the fourth report and the 2016 European Society of Hypertension guidelines for the diagnosis of hypertension and the detection of left ventricular hypertrophy in children and adolescents: a systematic review and meta-analysis.

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.

Association Between Secondary Hyperparathyroidism and Body Composition in Pediatric Patients With Moderate and Advanced Chronic Kidney Disease.

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). 61 patients (median age: 13.4 years) were included.

Lesch-Nyhan syndrome due to a splice-site mutation in a 14-month-old boy presenting as acute renal failure.

Lesch-Nyhan disease (LND) is a rare X-linked recessive inherited disorder caused by mutations in 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.

Association between insulin growth factor-1, bone mineral density, and frailty phenotype in children with chronic kidney disease.

Background: 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).

Methods: Forty-six patients (median age 14.5 years) were prospectively enrolled.

Association between relative fat mass, uric acid, and insulin resistance in children with chronic kidney disease.

Introduction: 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).

Material And Methods: 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.

Ambulatory hemodynamic patterns, obesity, and pulse wave velocity in children and adolescents.

Background: 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.

Methods: We included in the study healthy children and adolescents randomly selected from a school-based blood pressure screening study.

Arterial stiffness in children and adolescents with masked and sustained hypertension.

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.

Obesity and season as determinants of high blood pressure in a school-based screening study.

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.

Performance of simplified tables for high blood pressure screening in a European pediatric population.

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.

Comparison of cotrimoxazole vs. second-generation cephalosporins for prevention of urinary tract infections in children.

Background: 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.

Methods: 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.

Long-term outcome of pediatric kidney transplantation: A single-center experience from Greece.

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.

Long-Term Changes in Blood Pressure After Pediatric Kidney Transplantation.

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.

aHUS associated with C3 gene mutation: a case with numerous relapses and favorable 20-year outcome.

Background: 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.

Case-diagnosis/treatment: We report the case of a 21-year-old male with a C3 heterozygous gene mutation (p.

Longitudinal assessment of bone quality in pediatric patients with chronic kidney disease in relation to treatment modality.

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.

Primary intestinal lymphangiectasia: is it always bad? Two cases with different outcome.

Primary intestinal lymphangiectasia (PIL) or Waldmann's disease is a rare protein-losing gastroenteropathy of unknown etiology. Less than 200 cases have been reported globally. Patients may be asymptomatic or present edema, lymphedema, diarrhea, ascites and other manifestations.

Acute phase 99mTc-dimercaptosuccinic acid scan in infants with first episode of febrile urinary tract infection.

Background: 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).

Methods: Ninety-eight infants with microbiologically confirmed first episode of febrile UTI were studied.