Low Birth Weight is Associated with More Severe Course of Steroid-Sensitive Nephrotic Syndrome in Children, Multicentric Study.

BACKGROUND: Several previous studies have reported a more severe course of nephrotic syndrome in children with low birth weight. PATIENTS: Cohort of 223 children with idiopathic nephrotic syndrome. METHODS: We aimed to investigate the association between course of nephrotic syndrome and low birth weight. Data from seven paediatric nephrology centres were used. RESULTS: Children with low birth weight had 3.84 times higher odds for a more severe course of steroid-sensitive nephrotic syndrome (95% CI 1.20-17.22, P=0.041), and those with low birth weight and remission after 7 days had much higher odds for a more severe course of disease (OR 8.7). Low birth weight children had a longer time to remission (median 12 vs. 10 days, P=0.03). They had a higher need for steroid-sparing agents (OR for the same sex=3.26 [95% CI 1.17-11.62, P=0.039]), and the odds were even higher in females with low birth weight (OR 6.81). There was no evidence of an association either between low birth weight and focal segmental glomerulosclerosis or between low birth weight and steroid-resistant nephrotic syndrome. DISCUSSION: We conducted the first multicentric study confirming the worse outcomes of children with NS and LBW and we found additional risk factors. CONCLUSIONS: Low birth weight is associated with a more severe course of steroid-sensitive nephrotic syndrome, while being female and achieving remission after 7 days are additional risk factors.

How the 99mTc-DMSA scintigraphy findings are reflected in the adopted treatment of primary vesicoureteral reflux: One centre experience.

BACKGROUND: Renal changes may have long-term sequalae and prevention is a main goal of management of primary vesicoureteral reflux (VUR). AIM: This study aims to reveal how much 99mTc-DMSA scintigraphy findings are reflected in the adopted surgical or non-surgical treatment in children with diagnosed primary VUR and to give the clinicians information regarding their final therapeutic decision. PATIENTS AND METHODS: A total 207 children with primary VUR who underwent non-acute 99mTc-DMSA scan were retrospectively evaluated. The presence of renal changes, their grading, differential function asymmetry (<45%) and grade of VUR were compared with subsequent choice of therapy. RESULTS: Altogether 92 (44%) children had asymmetric differential function, 122 (59%) showed presence of renal changes, 79 (38%) had high-grade VUR (IV-V). Patients with renal changes had lower differential function (41%vs.48%) and higher grade of VUR. The incidence of high-grade changes (G3+G4B) afflicting more than one third of the kidney was significantly different between grade I-II, III and IV-V VUR (9%, 27%, 48%, respectively). Renal changes were detected in 76% of surgically and 48% of non-surgically treated patients, high-grade 99mTc-DMSA changes in 69% and 31%, respectively. In children with no scars/dysplasia (G0+G4A), non-surgical treatment prevailed in 77%. The independent predictors for surgical intervention were presence of renal changes and higher grade of VUR, but not functional asymmetry. DISCUSSION: Over the last 20 years, there has been a shift toward more non-surgical management of VUR. The long-term outcome of this approach should be thoroughly studied. This is the first study analysing renal status in VUR patients using 99mTc-DMSA scan and its grading regarding the adopted treatment. Renal changes in almost half of non-surgically treated children with VUR should be an indicator for earlier diagnosis and effective treatment of acute pyelonephritis and VUR. We recommend distinguishing grade III VUR, as a moderate-grade VUR, because it is linked to higher incidence of high-grade 99mTc-DMSA changes (G3+G4B); our findings of 65% of grade III VURs treated non-surgically should be cautionary. Grade III VUR does not mean a low-risk condition and should alert the clinician to evaluate the extent of renal changes and unmask high-risk cases. CONCLUSIONS: Our data strengthens the need to investigate the extent of renal changes in VUR patients regarding treatment decision. Performing 99mTc-DMSA scan individualizes the treatment of VUR patients; its grading can distinguish grade III-VUR as a separate risk entity because it differs significantly in terms of incidence of high-grade renal changes and chosen therapy.

Comparison of planar DMSA scan with an evaluation based on SPECT imaging in the split renal function assessment.

BACKGROUND: Validation of the 99mTc-DMSA planar scintigraphy accuracy for split renal function assessment and comparison with evaluation based on SPECT imaging both with and without CT attenuation correction. MATERIALS AND METHODS: For split renal function assessment two methods were used: A) planar scintigraphy based on anterior and posterior projections using correction for kidney depth calculated by the geometric mean; B) semi-quantitative evaluation based on SPECT (B1) and attenuation-corrected SPECT/CT (B2) images using locally developed software for kidney segmentation and voxel-based analysis. All three methods were performed with a phantom simulating body including pair of kidneys. For patient study methods A and B1 were applied on a group of 140 children and adolescents with various renal diseases. Renal function ratios were compared both mutually and with physically measured activity ratios in the phantom. RESULTS: Method A provided results which were closest to measured reference values (average absolute difference of 0.9 percentage points [pp]). Method B1 was noticeably worse (2.1pp), whereas attenuation correction (B2) improved tomography results considerably (1.3 pp). The superiority of planar imaging could be caused among others by differences in creation of planar range of interest compared to tomographic volume of interest. However all the differences were under the threshold of any clinical importance. The comparison between method A and B1 based on patient study also showed differences mostly of none clinical importance. CONCLUSION: Routine evaluation of split renal function using planar technique with correction of the kidney depth is at least equivalent to tomographic evaluation, and there is no need to update the established clinical practice.

Tubulointerstitial nephritis and uveitis syndrome in a mother and her son.

A mother and her son, both with tubulointerstitial nephritis and uveitis syndrome (TINU) are reported. The nephritis presented itself at 13 years in the mother and at 10 years in her son. Glomerular filtration (GFR) decreased in both, and renal biopsies confirmed the diagnosis. Nephritis preceded the onset of uveitis in both. Clinical course and renal function improved quickly on oral steroids in the boy. The mother's hyperazotemia decreased spontaneously (without steroids), but not to normal range, and remained stable for 35 years of follow-up. Local steroids due to recurrences of uveitis were repeatedly needed in both. We believe this is the first report on familial occurrence of inherited TINU syndrome in two generations.