Impact of the longitudinal quantitative assessment of juvenile systemic lupus erythematosus severity on the disease outcome.

Objectives: This study on juvenile SLE patients aimed to evaluate retrospectively the impact of a tertiary center's management policy of the disease severity on its long-term progression and cumulative damage development as well as provision of quality-driven medical care (QmC).

Methods: Disease activity was assessed by the Physician Global Assessment and SLEDAI-2K, flares by SELENA/SLEDAI, and damage by the pediatric SLICC/DI at diagnosis, 6 months post-diagnosis, and annually thereafter. At the same time, QmC was evaluated by relevant indices and quality of life was captured by the Greek version of the General Health Questionnaire only at the last visit.

Fok-I polymorphism of vitamin D receptor gene and the presence of renal dysfunction in patients with β-thalassemia major.

Recent evidence supports the presence of renal dysfunction even among young patients with β-thalassemia major. However, the possible genetic contribution has never been investigated. The aim of this study was to correlate the presence of Fok-I polymorphism of the vitamin D receptor gene with abnormal levels of early markers of renal impairment in children and young adults with thalassemia.

Comparative study of quantitative ultrasonography and dual-energy X-ray absorptiometry for evaluating renal osteodystrophy in children with chronic kidney disease.

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.

Renal dysfunction in patients with beta-thalassemia major receiving iron chelation therapy either with deferoxamine and deferiprone or with deferasirox.

There are limited studies on renal involvement in beta-thalassemia, mainly involving patients on deferoxamine, reporting both glomerular and tubular dysfunction. The aim of the present study was to investigate renal involvement in young thalassemia patients, using both conventional and early markers of renal dysfunction, and to correlate findings to iron chelation therapy. Forty-two patients aged 4-23 years were studied and, for analysis purposes, were divided into two groups based on chelation therapy (group A receiving deferasirox and group B receiving deferoxamine and deferiprone combination therapy).

Drug-induced interstitial nephritis in a child with idiopathic nephrotic syndrome.

Acute renal failure (ARF) is a rare but severe complication of active idiopathic nephrotic syndrome (INS) in children. It may be due to several causes with different outcomes. Both the clinical picture of the patient as well as laboratory, imaging and histopathological findings may help in the diagnosis.

Bilirubin levels predict renal cortical changes in jaundiced neonates with urinary tract infection.

Background: This study was undertaken to determine the incidence of urinary tract infection (UTI) and the frequency of anatomical abnormalities in newborns with unexplained jaundice and to find out if there is any correlation between bilirubin level and renal damage.

Methods: We studied 462 full-term neonates for UTI. They were aged 3-25 days, with either high (>10 mg/dL) or prolonged (>10 days) hyperbilirubinemia, with or without manifestations such as fever, vomiting, diarrhea, poor feeding, lethargy, and irritability.

Antibiotics-induced acute interstitial nephritis in 6 children.

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.

Superior sagittal sinus thrombosis in steroid-resistant nephrotic syndrome.

An 8(1/2)-year-old-female child with steroid-resistant nephrotic syndrome developed sagittal sinus thrombosis while on pulse therapy with corticosteroids, presenting with recurrent vomiting, headache, and impaired consciousness. The diagnosis was established by cranial computed tomography, magnetic resonance imaging, and magnetic resonance angiography. She gradually recovered without neurologic sequelae while being treated with low-molecular-weight heparin (2 mg/kg/day).

Transforming growth factor-beta1 in the urine of young children with urinary tract infection.

Urinary tract infection (UTI) is a frequent cause of morbidity during the first years of life and may lead to renal insufficiency. Transforming growth factor-beta1 (TGF-beta) is both immunoregulatory and an important mediator of interstitial fibrosis. TGF-beta was detected in the urine of 52% of 48 children aged 1-24 months with a first episode of UTI (94% due to Escherichia coli) and no obstructive nephropathy compared with 0 of 20 healthy young children (P<0.

Urinary bladder volume and pressure at reflux as prognostic factors of vesicoureteral reflux outcome.

Background: Controversy exists as to whether the outcome of vesicoureteral reflux (VUR) can be prognosticated by direct radionuclide cystography (DRC).

Objective: To correlate the quantitative data obtained by DRC with disease outcome in infants with VUR and positive DRC 1 year after diagnosis.

Materials And Methods: The medical records of 109 children with known primary VUR diagnosed during the first year of life were studied retrospectively.

Cyclic voiding cystourethrography: is vesicoureteral reflux missed with standard voiding cystourethrography?

Vesicoureteral reflux (VUR) may occur intermittently and cyclic voiding cystourethrography (VCUG) can enhance the ability of the method to detect reflux. We undertook this prospective study to assess how often VUR may occur intermittently during VCUG and to evaluate the reliability of the method by performing cyclic VCUG. Two hundred seventy-five children younger than 2 years underwent two cycles of VCUG.

Partial hypoxanthine-Guanine phosphoribosyltransferase deficiency as the unsuspected cause of renal disease spanning three generations: a cautionary tale.

Hypoxanthine-guanine phosphoribosyltransferase (HPRT) deficiency is an X-linked defect of purine metabolism. Clinical manifestations are usually related to the degree of enzyme deficiency: complete HPRT deficiency (Lesch-Nyhan syndrome) presenting with severe neurologic or renal symptoms, or partial HPRT deficiency (Kelley-Seegmiller syndrome) manifesting as a gout-urolithiasis syndrome. A 3-generation kindred is described in which the recognition of partial HPRT deficiency in 2 adolescent male siblings presenting with uric acid lithiasis led to the diagnosis in 2 maternal uncles already in renal failure of unknown cause.