The Association of Nephroblastoma Overexpressed (NOV) and Endothelial Progenitor Cells with Oxidative Stress in Obstructive Sleep Apnea.

OBJECTIVE: Obstructive sleep apnea (OSA) is a sleep disorder characterized by intermittent hypoxia, chronic inflammation, and oxidative stress and is associated with cardiometabolic disease. Several biological substrates have been associated with OSA such as nephroblastoma overexpressed (NOV), endothelial progenitor cells (EPC), and circulating endothelial cells (CEC). Few studies have looked at the association of NOV with OSA while the EPC/CEC relationships with OSA are unclear. In this study, we hypothesize that (1) NOV is associated with the severity of OSA independent of BMI, identifying a protein that may play a role in the biogenesis of OSA complications, and (2) EPCs and CECs are also associated with the severity of OSA and are biomarkers of endothelial dysfunction in OSA. METHODS: 61 subjects underwent overnight polysomnography (PSG), clinical evaluation, and blood analysis for NOV, EPC, CEC, interleukin 6 (IL-6), and other potential biomarkers. RESULTS: NOV and EPCs were independently associated with the oxygen desaturation index (ODI) after adjusting for potential confounders including body mass index (BMI), age, and sex (NOV p = 0.032; EPC p = 0.001). EPC was also independently associated with AHI after adjusting for BMI, age, and sex (p = 0.017). IL-6 was independently associated with AHI, but not with ODI. CONCLUSION: NOV and EPC levels correlate with the degree of OSA independent of BMI, indicating that these biomarkers could potentially further elucidate the relationship between OSA patients and their risk of the subsequent development of cardiovascular disease.

Long noncoding RNA MIAT acts as an oncogene in Wilms' tumor through regulation of DGCR8.

OBJECTIVE: Recent researches have proved that long noncoding RNAs (lncRNAs) cover an important role in malignant tumors. Our study showed how lncRNA myocardial infarction-associated transcript (MIAT) functions in the development of Wilms' tumor. PATIENTS AND METHODS: Real Time-quantitative Polymerase Chain Reaction (RT-qPCR) was utilized to detect the MIAT expression in Wilms' tumor patients. The MIAT expression level and the patients' overall survival time were analyzed. Then, we conducted functional experiments to identify the changes in the biological behaviors of Wilms' tumor cells due to the loss of MIAT. Moreover, further experiments were performed to explore the potential mechanism. RESULTS: By comparing with MIAT expression in adjacent tissues, the MIAT expression level was significantly higher in Wilms' tumor samples. Moreover, the cell growth ability of Wilms' tumor cells was inhibited due to the loss of MIAT. The migrated and invaded ability of the Wilms' tumor cells was inhibited due to the loss of MIAT. Furthermore, the expression of DGCR8 was downregulated due to the loss of MIAT. In addition, it was found that the DGCR8 expression was positively correlated to MIAT expression in Wilms' tumor tissues. CONCLUSIONS: The above results suggested that MIAT could promote the cell proliferation and the metastasis of Wilms' tumor by upregulating DGCR8, which indicated that MIAT might be a potential target for the diagnosis and therapy of Wilms' tumor.

Phenotype evolution and health issues of adults with Beckwith-Wiedemann syndrome.

BACKGROUND: Beckwith-Wiedemann syndrome (BWS) phenotype usually mitigates with age and data on adulthood are limited. Our study aims at reporting phenotype evolution and health issues in adulthood. METHODS: 34 patients (16 males), aged 18-58 years (mean 28.5) with BWS were enrolled. RESULTS: 26 patients were molecularly confirmed, 5 tested negative, and 3 were not tested. Final tall stature was present in 44%. Four patients developed Wilms' Tumor (2, 3, 5, and 10 years, respectively); one hepatoblastoma (22 years); one acute lymphoblastic leukemia (21 years); one adrenal adenoma and testicular Sertoli cell tumor (22 and 24 years, respectively); and three benign tumors (hepatic haemangioma, uterine myoma, and mammary fibroepithelioma). Surgery for BWS-related features was required in 85%. Despite surgical correction several patients presented morbidity and sequelae of BWS pediatric issues: pronunciation/swallow difficulties (n = 9) due to macroglossia, painful scoliosis (n = 4) consistent with lateralized overgrowth, recurrent urolithiasis (n = 4), azoospermia (n = 4) likely consequent to cryptorchidism, severe intellectual disability (n = 2) likely related to neonatal asphyxia and diabetes mellitus (n = 1) due to subtotal pancreatectomy for intractable hyperinsulinism. Four patients (two males) had healthy children (three physiologically conceived and one through assisted reproductive technology). CONCLUSIONS: Adult health conditions in BWS are mostly consequent to pediatric issues, underlying the preventive role of follow-up strategies in childhood. Malignancy rate observed in early adulthood in this small cohort matches that observed in the first decade of life, cumulatively raising tumor rate in BWS to 20% during the observation period. Further studies are warranted in this direction.

[Long-term renal function in Wilms tumor survivors]. / Función renal a largo plazo en supervivientes de tumor de Wilms.

OBJETIVE: To evaluate long-term renal function and morbimortality in non-syndromic Wilms tumor (WT) survivors. METHODS: Retrospective study about WT patients treated in 1993-2017, according to SIOP protocols. Mortality, glomerular filtration rate (GFR), prevalence of hypertension and requirement of dialysis and renal transplant were evaluated. Chronic kidney disease (CKD) was defined as GFR <90 ml/min/1.73 m2. RESULTS: Thirty-nine children were treated in the 25 analyzed years. Median time of follow-up was 6 years (0.5-21 years). 48% (19 patients) debuted with stage I or II. Four cases had high-grade histo-logy. Mortality rate was 10%. GFR data were found in 37 patients. Chronic kidney disease (grade I-II) turned up in 6 patients (16%). No patient required renal replacement therapy or renal transplant. 16% of patients developed CKD in both unilateral and bilateral WT, (p>0.05); OR 1.04 (IC 95% 0.09-10.9). Identical results were obtained comparing patients treated with or without radiotherapy (16%). Children with stage I-III had CKD in 11% vs. 40% of patients with stage IV (p=0.12); OR 5.3 (IC 95% 0.61-45). None of them presented hypertension in addition. CONCLUSIONS: In the current study the prevalence of CKD was low but not negligible, although no patients required renal replacement therapy or renal transplant. Bilateral renal involvement and radiotherapy were not associated with CKD development. Metastatic disease determines a higher risk of CKD.

OBJETIVOS: Evaluar la función renal y la morbimortalidad a largo plazo, en supervivientes de tumor de Wilms (TW) no sindrómico. MATERIAL Y METODOS: Estudio retrospectivo de pacientes con TW entre 1993-2017 tratados según protocolos SIOP. Evaluamos mortalidad, filtrado glomerular (FG), prevalencia de hipertensión arterial (HTA), necesidad de diálisis y trasplante renal. Se definió enfermedad renal crónica (ERC) como FG <90 ml/min/1,73 m2. RESULTADOS: En los 25 años analizados se trataron 39 pacientes con edad media diagnóstica de 3,6 años (0,3-11 años). Mediana de seguimiento 6 años (0,5-21 años). El 48% (19 pacientes) debutaron con estadio I o II. Cuatro pacientes presentaron histología de alto riesgo (10%). La mortalidad fue del 10%. El 16% (6 pacientes) desarrolló ERC (grados I-II). Ningún paciente precisó terapia renal sustitutoria (TRS) o trasplante. La presencia de ERC tanto en enfermedad unilateral como bilateral fue del 16%, p>0,05; OR 1,04 (IC 95% 0,09-10,9). Se obtuvieron idénticos resultados (16%) comparando pacientes que recibieron radioterapia frente a aquellos que no. Los pacientes en estadio I, II y III presentaron una prevalencia de ERC del 11% vs. 40% en estadio IV (p=0,12); OR 5,3 (IC 95% 0,61-45). Ningún paciente asoció HTA crónica. CONCLUSIONES: En el presente estudio la prevalencia de ERC en supervivientes de TW no sindrómico es baja pero no desdeñable, aunque ninguno precisó trasplante renal o TRS. La presencia de enfermedad bilateral y la radioterapia no se asociaron al desarrollo de ERC. La enfermedad metastásica condiciona un riesgo mayor de ERC.

MiR-155-5p affects Wilms' tumor cell proliferation and apoptosis via targeting CREB1.

OBJECTIVE: The aim of this study was to explore the role of microRNA-155-5p (miR-155-5p) in regulating the proliferation and apoptosis of Wilm's tumor (WT), and to investigate the possible underlying mechanism. PATIENTS AND METHODS: The expression levels of miR-155-5p in 37 pairs of WT clinical samples, as well as WT cell line (G401), were detected by quantitative reverse transcription polymerase chain reaction (qRT-PCR). MTT (3-(4,5-dimethylthiazol-2-yl)-2,5-diphenyl tetrazolium bromide) assay and flow cytometry assay were used to detect the effects of miR-155-5p on cell proliferation, cycle and apoptosis. Target gene prediction software was applied to screen the potential downstream target gene of miR-155-5p. QRT-PCR, Western blot (WB) and luciferase reporter gene assay proved that cAMP-response element binding protein 1 (CREB1) was the target gene of miR-155-5p. Besides, rescue experiment was conducted to further explore the effect of CREB1 on WT cells. RESULTS: The expression levels of miR-155-5p in WT tissues and cells were both significantly down-regulated. Importantly, miR-155-5p was found to be involved in the malignant behavior of WT cells. MTT assay and flow cytometry assay demonstrated that miR-155-5p significantly inhibited the proliferation, caused stagnation of cells in G0/G1 phase, and promoted cell apoptosis. CREB1 was verified as a functional target gene of miR-155-5p, which was negatively regulated by miR-155-5p. Rescue experiments indicated that restoring the expression of CREB1 could interfere with the effects of miR-155-5p on WT cells. CONCLUSIONS: MiR-155-5p could regulate the proliferation, cell cycle and apoptosis of WT cells. These effects were achieved by regulating the expression of CREB1. Furthermore, our study might provide a new theoretical basis for the basic research of WT.

Fetal programming and Wilms tumor.

BACKGROUND: The "fetal programming" hypothesis has been evaluated in many adult diseases including cancer, but not for Wilms tumor. Wilms tumor has been related to high birthweight, but little is known about other growth metrics such as a baby's birth length, ponderal index, or placenta size, which can shed additional light on growth patterns. METHODS: Cases of Wilms tumor (N = 217) were taken from the Danish Cancer Registry, and controls (N = 4340) were randomly selected from the Population Register and matched to cases by sex and age. Linkage to the Medical Births Registry provided information on gestational factors and fetal growth measurements, while linkage to the Patient Register provided information on maternal and child health conditions. RESULTS: Despite having typically normal to higher birthweights, Wilms tumor cases had smaller placentas (≤540 g; odds ratio (OR) = 4.24; 95% confidence interval (CI), 1.84-9.78) and a lower placenta-to-birthweight ratio (OR = 1.81; 95% CI, 1.17-2.82, per 1 SD decrease). Small placentas were more common among Wilms cases without congenital anomalies (OR = 6.43; 95% CI, 1.95-21.21). Wilms tumor cases had a higher prevalence of high birthweight (>4000 g; OR = 1.57; 95% CI, 1.11-2.22), birth length 55 cm or longer (OR = 1.74; 95% CI, 1.09-2.78), and being large for gestational age (OR = 1.79; 95% CI, 1.08-2.96). CONCLUSIONS: Our study corroborates earlier studies showing associations with high birthweight and suggests associations between Wilms tumor and decreased placental size and low placenta-to-birthweight ratio.

[Effect of LINE1-ORF1p overexpression on the proliferation of nephroblastoma WT_CLS1 cells].

OBJECTIVE: To prepare the LINE1-ORF1p polyclonal antibody, and to study the effect of LINE1-ORF1p on the proliferation of nephroblastoma WT_CLS1 cells. METHODS: A genetic engineering method was used to achieve prokaryotic expression of LINE1-ORF1p, and rabbits were immunized with LINE1-ORF1p to prepare polyclonal antibody. Indirect ELISA was used to evaluate antibody titer, and Western blot and immunohistochemistry were used to evaluate the specific ability of antibody to recognize LINE1-ORF1p. The eukaryotic expression vector pEGFP-N1-LINE1-ORF1 was constructed and used to transfect WT_CLS1 cells. Western blot and qRT-PCR were used to measure the protein and mRNA expression of LINE1-ORF1, respectively, and cell proliferation assay and colony-forming assay were used to evaluate the effect of LINE1-ORF1p on the proliferation of WT_CLS1 cells and the formation of tumor cell clone. RESULTS: The LINE1-ORF1p antibody prepared had a titer of >1:16 000 and could specifically recognize LINE1-ORF1p in cells and tumor tissue. WT_CLS1 cells transfected with pEGFP-N1-LINE1-ORF1 had significant increases in the mRNA and protein expression of LINE1-ORF1 and significantly enhanced cell proliferation ability and colony formation ability (P<0.05). CONCLUSIONS: LINE1-ORF1p can promote the growth of nephroblastoma cells and the formation of tumor cell clone, and may be involved in the pathogenesis of nephroblastoma.

Estimated glomerular filtration rate after nephrectomy for Wilms tumor.

BACKGROUND: The aim of this study was to assess long-term residual kidney function after unilateral nephrectomy for non-syndromic Wilms tumor (NSWT). METHODS: Of the patients who underwent one-sided NSWT at Tohoku University Hospital between 1977 and 2003, nine were followed up until age ≥18 years. For these nine patients, we retrospectively evaluated estimated glomerular filtration rate (eGFR) in childhood (3-10 years old), adolescence (11-17 years old) and adulthood (≥18 years). RESULTS: Mean age at the last follow up was 23.0 years. Tumor classification was as follows: stage I tumor, n = 6; stage II tumor, n = 3; mixed-type nephroblastoma, n = 8; and congenital mesoblastic nephroma, n = 1. Mean eGFR was 101.3 ± 21.2 mL/min/1.73 m2 in childhood, 106.0 ± 32.1 mL/min/1.73 m2 in adolescence and 100.5 ± 20.7 mL/min/1.73 m2 in adulthood. Therefore, no significant change in eGFR was observed over the three life stages evaluated. Further, none of the patients met the diagnostic criteria for chronic kidney disease by early adulthood. CONCLUSIONS: eGFR after unilateral nephrectomy in patients with NSWT remained ≥60 mL/min/1.73 m2 during the transition from childhood to early adulthood, with no development of chronic kidney disease or end-stage kidney failure.

Analysis of treatment of large abdominal malignancies in children complicated with abdominal compartment syndrome: Report of six cases.

To explore effective treatment of large abdominal malignancies in children complicated with abdominal compartment syndrome (ACS).Six children with large abdominal malignancies complicated with ACS were admitted to our department from January 2013 to January 2016, and the changes in their breathing, heart rate, oxygen saturation, abdominal circumference, bladder pressure, and urine output, as well as the treatment measures and outcomes, were retrospectively analyzed.The 6 children included 1 child with bilateral nephroblastoma, 1 child with abdominal alveolar rhabdomyosarcoma, 1 child with right ovarian malignant teratoma complicated with abdominal glioma, 1 child with abdominal malignant teratoma, 1 child with right nephroblastoma, and 1 child with left adrenal gland neuroblastoma. All patients were treated in a timely manner. The first 4 children underwent abdominal cavity decompression through surgical resection of the tumor, and the ACS was successfully cured allowing for follow-up care, whereas the last 2 patients failed to receive emergency surgery and eventually died due to the gradual aggravation of ACS.Decompression through surgical resection of the tumor is the only effective measure for treating large abdominal malignancies in children complicated with ACS.

Prospective analysis of long-term renal function in survivors of childhood Wilms tumor.

BACKGROUND: Considering the improved outcome, a better understanding of the late effects in Wilms tumor survivors (WT-S) is needed. This study was aimed at evaluating renal function and determining the prevalence of clinical and subclinical renal dysfunction in a cohort of WT-S using a multimodal diagnostic approach. METHODS: Thirty-seven WT-S were included in this prospective cross-sectional single center study. To evaluate renal function, glomerular filtration rate (GFR) and urinary protein excretion were assessed. Additionally, kidney sonomorphology and blood pressure were analyzed. RESULTS: All examined WT-S (mean age 28.7 years, mean follow-up 24.8 years) had been treated with a combination of surgery and chemotherapy; 59.5% had received adjuvant radiotherapy. Impaired glomerular renal function was detected in a considerable proportion of WT-S, with age-adjusted cystatin-based GFR estimation below age norm in 55.9%. A lower cystatin-based estimated GFR (eGFR) correlated with longer follow-up time and higher irradiation dose. In 5 patients (13.5%) albuminuria was identified. Analysis of sonomorphology detected compensatory contralateral renal hypertrophy in 83.3% of WT-S. Chronic kidney disease (CKD) ≥ stage II was present in 55.9% of WT-S. Blood pressure measurements revealed arterial hypertension in 15 (40.5%) WT-S (newly diagnosed n=10). In 24.3% both CKD ≥ stage II and arterial hypertension were determined. CONCLUSION: Even though WT-S are believed to carry a low risk for end-stage renal disease, in this study, a remarkable number of WT-S presented with previously unidentified subclinical signs of renal function impairment and secondary morbidity. Therefore, it is important to continue regular follow-up, especially after transition into adulthood.

Promotion of Wilms' tumor cells migration and invasion by mono-2-ethyhexyl phthalate (MEHP) via activation of NF-κB signals.

Wilms tumor is a pediatric kidney cancer associated with inactivation of the WT1 tumor-suppressor gene in 5-10% of cases. There is an urgent need to illustrate risk factors which can trigger the motility of Wilms tumor cells. Our present study revealed that mono-(2-ethylhexyl) phthalate (MEHP) exposure can significantly increase the in vitro migration and invasion of G401 and WiT49 cells. Real time PCR and western blot analysis revealed that MEHP treatment can increase the expression of metalloproteinase-2 (MMP-2) and MMP-9, while had no effect on the expression of MMP-1, MMP-3, or MMP-12. The si-MMP-2 and si-MMP-9 can reverse MEHP induced migration and invasion of G401 cells. MEHP can activate both ERK1/2 and p65 in WT cells, while had no obvious effect on Akt or PKA. However, only BAY 11-7082, the inhibitor of NF-κB, while not ERK1/2 (PD 98059), can reverse MEHP induced migration and invasion of WT cells. BAY 11-7082 also can attenuate MEHP induced up regulation of MMP-2 and MMP-9. The inhibitor of estrogen receptor reversed MEHP induced activation of NF-κB and up regulation of MMP-2/-9. In addition, MEHP also increased the mRNA and protein expression, nuclear translocation, and transcriptional activities of NF-κB in WT cells. Collectively, our study found that MEHP stimulated the Wilms' tumor progression via NF-κB signals.

Bilateral Wilms Tumor With Ureteral Extension.

Wilms tumor is the most common renal tumor in children. However, tumor extension into the ureter is exceedingly rare. We present a case of bilateral Wilms tumor with unilateral ureteral extension into the bladder. This case illustrates the importance of thoughtful diagnostic evaluation and surgical planning to obtain a good oncologic outcome while preserving renal function.

Extrarenal Wilms' Tumor of the Female Genital System: A Case Report and Literature Review.

Extrarenal Wilms' Tumors (ERWTs) are rare. There have been only 25 cases of ERWT arising from the female genital system reported in the literature. In this paper, we report a 60-year-old woman with a complaint of vaginal bleeding and a polypoid mass in the uterine cavity by sonography that was demonstrated as ERWT by pathology after resection. The pathological characteristics, histological origination, diagnosis, therapy and prognosis of ERWT in female reproductive system are discussed in this paper in the purpose of improving the diagnosis and therapy of this rare tumor.

Comprehensive renal function evaluation in patients treated for synchronous bilateral Wilms tumor.

OBJECTIVES: The purpose of this study was to perform a comprehensive assessment of long-term renal function in patients treated at our institution for synchronous bilateral Wilms tumor (BWT) and to determine the optimal method for estimating glomerular filtration rate (eGFR). METHODS: Surgical approach, adjuvant therapy, and pathology reports were reviewed for patients with at least six months follow-up from definitive surgery. eGFRs, as assessed by the Schwartz and Chronic Kidney Disease in Children (CKiD) formulas, were compared to measured GFR (mGFR) determined by 99mTc-DTPA scanning. Urine studies, including microalbumin, ß-microglobulin, and FENa were also reviewed. RESULTS: Forty-two patients were identified. Of 36 living patients, 28 (77.8%) had greater than 6months follow-up, with a median overall follow-up of 5.2years (range: 1.4-13.4). The median mGFR was 97mL/min/1.73m2, while the median eGFRSchwartz and eGFRCKiD were 103.3mL/min/1.73m2 and 79.7mL/min/1.73m2, respectively, (p=0.13 and p=0.75, compared to mGFR). Eleven (39.3%) patients had at least one abnormal urine study (microalbumin >30µg/g creatinine, n=3; ß-2 microglobulin >133µg/g creatinine, n=9; FENa>1%, n=4). CONCLUSIONS: In our series, few patients had an abnormally low GFR. Neither method for estimating GFR gave a significantly different result from measured GFR, suggesting that the Schwartz equation is adequate, although specific urine tests may be more sensitive for detecting subtle renal dysfunction. LEVEL OF EVIDENCE: Level IV - retrospective case series with no comparison group.

An 11-year experience of acquired von Willebrand syndrome in children diagnosed with Wilms tumour in a tertiary referral centre.

Wilms tumour (WT) is the commonest primary malignant renal tumour of childhood. Acquired von Willebrand syndrome (avWS) is a well-described paraneoplastic phenomenon, but it is uncommon and may not be detected until clinically significant bleeding is encountered during interventional procedures. Previous studies on small cohorts of patients have determined an incidence of between 4 and 8%. We have performed a retrospective study on cases of WT presenting over an 11.5-year period to a paediatric haematology/oncology unit in a tertiary referral centre to review the incidence of avWS, bleeding phenotype, management, and response to treatment of the primary pathology.

Renal Function Recovery after Nephrectomy or Nephron-Sparing Surgery in Children with Unilateral Renal Tumor.

Introduction Children with unilateral renal tumor (URT) and preoperative renal dysfunction (PRD) may benefit from nephron-sparing surgery (NSS). To test this hypothesis, we studied the outcome of baseline renal function after nephrectomy or NSS among children with URT. Materials and Methods Retrospective records review of children with URT who underwent nephrectomy (25 children) or NSS (11 children) at our institution. We analyzed the estimated glomerular filtration rate (eGFR) changes over time among patients, stratified by both preoperative renal function (with or without PRD) and surgical extent (NSS vs. nephrectomy). The primary end point was evaluation of compensatory recovery of preoperative eGFR after surgery. Only children older than 2 years at surgery were included in the study. Renal dysfunction was defined as an eGFR < 90 mL/min/1.73 m2. Results After nephrectomy or NSS, patients with PRD presented, on average during adolescence, a significant increase in eGFR, whereas patients without PRD presented, on average during adolescence, a stable eGFR. However, after nephrectomy, 5 of 17 (29%) and 7 of 8 (87%) adolescent patients with baseline eGFR ≤ or > 100 mL/min/1.73 m2, respectively, achieved or maintained two-kidney eGFR values (T-KEV) (p = 0.01). After NSS, four adolescent patients with PRD and seven without PRD achieved or maintained T-KEV. Conclusion The majority of children with URT and low baseline eGFR present with an impaired renal function recovery after nephrectomy and may benefit from NSS. Collaborative studies are needed to support present findings.

Wilms Tumor: An Uncommon Entity in the Adult Patient.

Wilms tumor, the most common kidney tumor in children, is rarely seen in adults, making it a challenge for the adult oncologist to diagnose and treat. Unlike with renal cell carcinoma, patients with Wilms tumor should receive adjuvant chemotherapy with or without radiation therapy. Adult oncologists may not be familiar with pediatric oncology protocols, so it is important to consult with pediatric oncologists who have more experience in this disease. Multimodal therapy based on pediatric protocols improved the outcomes of adults with Wilms tumor worldwide. We report a rare case of a 24-year-old woman with a slow-growing mass of the left kidney during a 4-year period. The mass was surgically removed and final diagnosis confirmed by pathology to be Wilms tumor. The patient received adjuvant chemotherapy and has been free of disease since 2014.

Measurement of glomerular filtration rate by dynamic contrast-enhanced magnetic resonance imaging using a subject-specific two-compartment model.

Measuring glomerular filtration rate (GFR) by dynamic contrast-enhanced (DCE) magnetic resonance imaging (MRI) as part of standard of care clinicalMRIexams (e.g., in pediatric solid tumor patients) has the potential to reduce diagnostic burden. However, enthusiasm for this relatively newGFRtest may be curbed by the limited amount of cross-calibration studies with referenceGFRtechniques and the vast variety ofMRtracer model algorithms causing confusion on the choice of model. To advanceMRI-basedGFRquantification via improvedGFRmodeling and comparison with associated(99m)Tc-DTPA-GFR, 29 long-term Wilms' tumor survivors (19.0-43.3 years, [median 32.0 ± 6.0 years]) treated with nephrectomy, nonnephrotoxic chemotherapy ± radiotherapy underwentMRIwith Gd-DTPAadministration and a(99m)Tc-DTPA GFRtest. ForDCE-MRI-basedGFRestimation, a subject-specific two-compartment (SS-2C) model was developed that uses individual hematocrit values, automatically defines subject-specific uptake intervals, and fits tracer-uptake curves by incorporating these measures. The association between reference(99m)Tc-DTPA GFRandMR-GFRs obtained bySS-2C, three published 2C uptake, and inflow-outflow models was investigated via linear regression analysis. Uptake intervals varied from 64 sec to 141 sec [96 sec ± 21 sec] and hematocrit values ranged from 30% to 49% [41% ± 4%]; these parameters can therefore not be assumed as constants in 2C modeling. OurMR-GFRestimates using theSS-2C model showed accordingly the highest correlation with(99m)Tc-DTPA-GFRs (R(2) = 0.76,P < 0.001) compared with other models (R(2)-range: 0.36-0.66). In conclusion,SS-2C modeling ofDCE-MRIdata improved the association betweenGFRobtained by(99m)Tc-DTPAand Gd-DTPA DCE-MRIto such a degree that this approach could turn into a viable, diagnosticGFRassay without radiation exposure to the patient.