Evagelia Farmaki

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

Background99mTc-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).MethodsNinety-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.ResultsOverall, 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 (P>0.05). However, infants with grade III to V VUR as well as those with delayed treatment presented significantly increased renal involvement by acute DMSA scan (P<0.05). 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.ConclusionsParenchymal 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.

Simultaneous changes in serum HMGB1 and IFN-α levels and in LAIR-1 expression on plasmatoid dendritic cells of patients with juvenile SLE.. New therapeutic options?

We investigated the simultaneous changes in serum levels of HMGB1 and IFN-α as well as in LAIR-1 expression on plasmatoid dendritic cells (pDCs) of juvenile systemic lupus erythematosus (jSLE) patients in order to explore their involvement in the disease pathogenesis and their correlation with disease activity and other characteristics. In total, 62 blood samples were studied from 26 jSLE patients (18 girls), aged 8–16 years. Twenty healthy subjects (16 girls) of comparable age were included as healthy controls (HCs). Concentrations of serum HMGB1 and IFN-α were assessed by ELISA and LAIR-1 expression on pDCs by five-color flow cytometry. The disease activity index was assessed by SLEDAI and ECLAM scores. It was found that mean serum levels both of HMGB1 and IFN-α were significantly increased in jSLE patients compared to HCs and in jSLE patients with active disease with or without active nephritis compared to those with inactive disease. Mean serum levels of HMGB1 were positively correlated with levels of IFN-α and both were positively correlated with the SLEDAI and ECLAM scores. The expression of LAIR -1 on pDCs of jSLE patients was significantly lower than that of HCs. In conclusion, our findings indicate that serum HMGB1 not only represents a potential marker of disease activity but together with the lack of LAIR-1 inhibitory function may contribute to the sustained inflammatory action of IFN-α in jSLE. In this regard, blocking the action of HMGB1 and its receptors or enhancing the expression/inhibitory function of LAIR-1 on pDCs should be included in future immune interventions for controlling jSLE.

Pandemic influenza A 2009 (H1N1) vaccination in high risk children with chronic renal diseases: Acceptance and perceptions

We aimed to evaluate the acceptance of pandemic influenza A 2009 vaccination in our high risk children with chronic renal diseases. . A total of 64 children/parents of pediatric nephrology department were approached to fill in a standardised questionnaire on influenza immunization profile. The H1N1 vaccination rates were 57.1% for transplant recipients, 61.5% for patients on peritoneal dialysis (PD), 36.4% for patients with various stages of chronic renal disease (CRD) and 26.7% for patients with glomerulonephritis (GN) on immunosuppressive therapy. Children on renal transplantation or PD had a fourfold higher rate of being vaccinated than children with GN (p=0.04). Causes of denying vaccination included fear of adverse effects (48.9%), lack of sufficient data on the new vaccine (31.9%) and others (19.2%). Patients being vaccinated were all urged by their pediatric nephrologist (100%), while patients not vaccinated were negatively influenced by media (41.4%), friends (24.1%), pediatrician (20.7%) and others (13.8%). Regarding parents education, higher level was associated with increased rate of children vaccination (p=0.04). It seems that patients with severe renal disease had better compliance with vaccination. The pediatric nephrologists had the most significant positive influence in contrast to the media which had the most negative influence.

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. 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.

The portrait of Familial Mediterranean Fever in N. Greek pediatric patients: a 30-year experience

Familial Mediterranean Fever (FMF), is the second commonest autoinflammatory disease in pediatric Greek patients (pts) after PFAPA. So far, long-term follow-up case series in Greek FMF pts have not been emerged.

PReS-FINAL-2299: Novel biomarkers for the assessment of pediatric systemic lupus erythematosus nephritis (preliminary report)

Results The pSLE nephritis patients had significantly higher serum levels of anti-NCS [median: 48.89 (IQR: 31.4880.81) U/ml versus 12.5 (11.5-27.8) U/ml, p < 0.001], anti-C1q [22.75 (12.77-56.4) U/ml versus 12.5 (12.512.5) U/ml, p < 0.001], anti-GBM [3.88 (2.25-6.94) U/ml versus 2.2 (2.2-2.4) U/ml, p = 0.002] and HMGB1 [9.9 (5.7-32.23) ng/ml versus 2.5 (2.5-2.5) ng/ml, p < 0,001], than the patients with nephritis of other causality. Serum anti-GBM levels were significantly higher in the pSLE nephritis patients compared to the pSLE patients without nephritis [3.88 (2.25-6.94) U/ml versus 2.25 (2.2-2.83) U/ml, p = 0.014], while this was not true for the rest of the biomarkers. In the pSLE nephritis patients no correlation was found between serum antiGBM levels and pSLE nephritis disease activity. Serum anti-NCS and anti-C1q levels were positively correlated with the ECLAM score in the pSLE patients as a whole (p = 0.002, rho = 0.492 and p = 0.007, rho = 0.461, respectively). Conclusion In this pure Caucasian Northern Greek pSLE population, high serum anti-GBM levels were found to be associated with the presence of nephritis, but not with the nephritis disease activity. Serum anti-GBM, anti-NCS, anti-C1q and HMGB1 may be used to differentiate patients with pSLE nephritis from patients with nephritis of other causality. Furthermore, serum anti-NCS and anti-C1q may be useful for the estimation of pSLE disease activity.

A Greek multicenter study comparing the clinical and immunologic phenotypes between adult and juvenile- onset lupus

Results At diagnosis the mean (SD) ages were 12.25(0.27) and 33.93(1.32) yrs, whereas the mean follow-up 6.63 (0.59) and 11.6 (0.7) yrs, for jSLE and aSLE respectively. General features, hepatosplenomegaly, lymphadenopathy and haematology abnormalities were more frequent in jSLE patients (p1=0.001, p2=0.025 and p3<0.0001, respectively), whereas photosensitivity was commoner in those with aSLE (p=0.047). The main difference was the higher mean number of organ/system involvement in the jSLE group (p=0.0006). At the end of 5 yrs, the cumulative number of clinical manifestations was similar in both groups. Anti-dsDNAs, anti-cardiolipin, anti-Sm, anti-URNP antibodies and low C3 and C4 were significantly commoner in jSLE (p<0.01).