Standardization of pediatric uroradiological terms: a multidisciplinary European glossary.

To promote the standardization of nephro-uroradiological terms used in children, the European Society of Paediatric Radiology uroradiology taskforce wrote a detailed glossary. This work has been subsequently submitted to European experts in pediatric urology and nephrology for discussion and acceptance to improve the quality of radiological reports and communication between different clinicians involved in pediatric urology and nephrology.

Standardization of pediatric uroradiological terms: A multidisciplinary European glossary.

To promote the standardization of nephro-uroradiological terms used in children, the European Society of Pediatric Radiology uroradiology taskforce wrote a detailed glossary. This work has been subsequently submitted to European experts in pediatric urology and nephrology for discussion and acceptance to improve the quality of radiological reports and communication among different clinicians involved in pediatric urology and nephrology.

Nephropathia epidemica (puumala virus infection) in Austrian children.

From 2000 to 2007, 19 Austrian children (aged 6-18 years) had serologically verified nephropathia epidemica. Common clinical features were abdominal/flank/back pain, fever, nausea, vomiting, headache, and transient visual disturbances. Acute renal failure was present in 18 (95%) patients. All patients recovered completely. Childhood nephropathia epidemica in Austria takes a similar course to those reported for Northern European Puumala virus strains.

Complement inhibitor eculizumab in atypical hemolytic uremic syndrome.

BACKGROUND AND OBJECTIVES: Atypical hemolytic uremic syndrome (aHUS) is associated with a congenital or acquired dysregulation of the complement alternative pathway that leads to continuous complement activation on host cells causing inflammation and damage. Eculizumab, a humanized mAb against complement protein C5, inhibits activation of the terminal complement pathway. DESIGN, SETTING, PARTICIPANTS, & MEASUREMENTS: We report an adolescent with relapsing unclassified aHUS. On admission, a high plasma creatinine level indicated a poor prognosis, and hemodialysis had to be started. Plasma exchanges were initially effective against the microangiopathic hemolytic activity and allowed a temporary improvement of renal function with termination of hemodialysis after 7 wk. Subsequently, plasma exchanges (three times per week) failed to prevent ongoing aHUS activity and progressive renal failure. After 12 wk, aHUS treatment was switched to eculizumab. RESULTS: Eculizumab was effective in terminating the microangiopathic hemolytic process in two aHUS relapses; however, after normalization of complement activity, aHUS recurred and ultimately led to anuric end-stage renal failure. CONCLUSIONS: In this patient, complement inhibition by eculizumab temporarily terminated the microangiopathic hemolytic activity. Nevertheless, renal damage as a result of preceding and subsequent aHUS activity resulted in end-stage renal failure; therefore, therapeutic success may depend on early administration of eculizumab. The optimal duration of treatment may be variable and remains to be determined.

Three-dimensional ultrasonography-based virtual cystoscopy of the pediatric urinary bladder: a preliminary report on feasibility and potential value.

OBJECTIVE: The purpose of this study was to validate the feasibility and potential of 3-dimensional ultrasonography (3DUS)-based virtual cystoscopy in the pediatric urinary bladder. METHODS: Twenty patients (age range, newborn-14 years) underwent urinary tract ultrasonography and 3DUS of the urinary bladder. From this data set, virtual cystoscopy was reconstructed for visualization of the inner bladder surface. Three-dimensional ultrasonography was compared with 2-dimensional ultrasonographic (2DUS) findings, voiding cystourethrography (VCUG) results, and reports from cystoscopy or surgery when available. RESULTS: Three-dimensional ultrasonography was feasible in all patients. Data quality was sufficient for virtual cystoscopy without major motion artifacts. The 3DUS results matched all other findings; particularly, 3DUS superiorly visualized the ureteral ostium and the bladder neck configuration; in 5 patients, 3DUS depicted pathologically shaped ostia not detected by 2DUS. This correlated with the presence of vesicoureteral reflux on VCUG. Performing virtual cystoscopy added 1 minute to the investigation time (range, 0.5-2 minutes) and 3 minutes for postprocessing and viewing (range, 2-5 minutes). CONCLUSIONS: Three-dimensional ultrasonography-based virtual cystoscopy is feasible in the pediatric urinary bladder without sedation. It reveals surface information not accessible by 2DUS, improving detection of pathologic conditions such as atypically shaped ureteral ostia. Three-dimensional ultrasonography-based cystoscopy may become a valuable adjunct to 2DUS of the pediatric urinary tract, improving selection criteria for further imaging such as VCUG, and potentially may help reduce the need for endoscopic cystoscopy. However, these preliminary results still have to be confirmed in prospective studies with larger patient numbers.

Value of comprehensive renal ultrasound in children with acute urinary tract infection for assessment of renal involvement: comparison with DMSA scintigraphy and final diagnosis.

The aim of this study was to evaluate the value of comprehensive renal ultrasound (US), i.e., combining greyscale US and amplitude-coded color Doppler sonography (aCDS), for assessment of urinary tract infection (UTI) in infants and children, compared to (1) (99m)Tc DMSA scintigraphy and (2) final diagnosis. Two hundred eighty-seven children with UTI underwent renal comprehensive US and DMSA scintigraphy. The results were compared with regard to their reliability to diagnose renal involvement, using (1) DMSA scintigraphy and (2) final diagnosis as the gold standard. Sixty-seven children clinically had renal involvement. Sensitivity increased from 84.1% using only aCDS to 92.1% for the combined US approach, using DMSA scintigraphy as the reference standard. When correlated with the final diagnosis, sensitivity for DMSA scintigraphy was 92.5%; sensitivity for comprehensive US was 94.0%. Our data demonstrate an increasing sensitivity using the combination of renal greyscale US supplemented by aCDS for differentiation of upper from lower UTI. Sensitivity for DMSA and comprehensive US was similar for both methods compared to the final diagnosis. Comprehensive US should gain a more important role in the imaging algorithm of children with acute UTI, thereby reducing the radiation burden.

Two decades' experience of renal replacement therapy in paediatric patients with acute renal failure.

During the past 20 years, childhood renal replacement therapy (RRT) and the treatment of underlying diseases experienced extensive advances. We reviewed the data of our critically ill patients with acute renal failure (ARF) and RRT, comparing two decades from 1985 to 1994 and from 1995 to 2004. There were 87 patients with a mortality rate of 45% in the first decade, decreasing to 28 patients with a mortality rate of 39% in the second decade. The mortality rate decreased from 51% to 20% in patients older than one year, while the mortality rate in patients younger than one year increased from 38% to 88%. Yet, the absolute number of these non-survivors younger than one year decreased from 16 to seven patients. The decrease of RRT was mainly caused by a decrease of ARF secondary to heart surgery, oncologic disorders and sepsis. Whereas the majority of patients (75%) were treated with continuous haemofiltration in the first decade, 75% of patients were treated with continuous haemodiafiltration in the second decade. In conclusion, advances in the diagnosis and treatment of underlying disorders have reduced the need for RRT in critically ill paediatric patients during the past 20 years. In addition, there was a tendency for a decrease in the overall mortality, which might be caused by changing treatment policies and advances in RRT technology. Nevertheless, the high mortality rate in small infants is challenging.

Skin transplantation to monitor clinical donor-related tolerance in mixed hematopoietic chimerism.

Mixed hematopoietic chimerism usually carries with it the tolerance to any other tissue from the same donor. Consequently, the establishment of a sustained chimerism may allow long-term acceptance of transplanted organs without immunosuppression. We report a girl with refractory severe aplastic anemia who developed low recipient level hematopoietic chimerism following transplantation of maternal highly purified CD34+ cells without prophylactic immunosuppression. Renal thrombotic microangiopathy led to chronic renal failure and she received skin allografts from her mother in view of a future kidney donation. The maternal skin grafts were accepted without immunosuppression and the hematopoietic chimerism remained stable. Skin transplantation may be a helpful and easily applicable tool to monitor donor-related tolerance in hematopoietic chimerism clinically. It should contribute to minimize the risks of subsequent solid organ transplantation from the same donor without immunosuppression.

Successful treatment of chronic recurrent multifocal osteomyelitis with tumor necrosis factor-alpha blockage.

We describe the case of an 18-year-old girl with chronic recurrent multifocal osteomyelitis (CRMO) over a period of 10 years. She had suffered predominantly from very painful recurrent swelling of her cheeks. Various therapeutic regimens including nonsteroidal antiinflammatory drugs and steroids had shown only a partial or temporary response. Because tumor necrosis factor-alpha-blocking agents have been successfully applied in Crohn's-associated CRMO and the related SAPHO (synovitis, acne, pustulosis, hyperostosis, and osteitis) syndrome, tumor necrosis factor-alpha-blocking therapy with infliximab was initiated. Thereafter, apart from 1 mild episode, no additional recurrences were observed during 21 months of follow-up. Infliximab was well tolerated, and steroids were tapered off. Our observation indicates that infliximab may be an effective therapeutic option in CRMO.

Potential impact of pediatric MR urography on the imaging algorithm in patients with a functional single kidney.

OBJECTIVE: The purpose of this study was to evaluate the potential of MR urography in the assessment of children with a suspected "functional single kidney." SUBJECTS AND METHODS: Sixty patients (age range, 2.7 weeks to 15.7 years) who had been referred for assessment of a suspected functional single kidney underwent MR urography in addition to detailed sonography of the urinary tract and the currently indicated standard imaging. The results of the conventional imaging ((99m)Tc-dimer captosuccinic acid scintigraphy, voiding cystourethrography, and genitography) were compared with the results of sonography and MR urography; surgical findings served as the gold standard if available. RESULTS: Twenty-six patients had a single kidney. The other diagnoses were six contralateral multicystic dysplastic kidneys, two normal ectopic kidneys, one crossfused double system, and 25 ectopic or dysplastic renal buds. Scintigraphy detected all normal kidneys, two ectopic kidneys, and two dysplastic renal buds. Detailed sonography missed two ectopic kidneys and two orthotopic dysplastic renal buds, but one additional renal bud that could not be confirmed on other imaging techniques (accuracy, 91.7%; sensitivity, 88.2%; specificity, 96.2%) was suspected. MR urography results were correct in all patients and verified in all 13 with surgical correlation. CONCLUSION: MR urography allows a reliable assessment of renal and ureteral anatomy and of dysplastic or ectopic renal buds, even in non- or poorly functioning systems. MR urography therefore has the potential to replace the currently used excretory urography and scintigraphy. In patients with a suspected functional single kidney a detailed sonographic study and MR urography should be considered the diagnostic algorithm of choice.

De novo HNF-1 beta gene mutation in familial hypoplastic glomerulocystic kidney disease.

Mutations in the gene encoding the transcription factor hepatocyte nuclear factor (HNF)-1 beta are associated with maturity-onset diabetes of the young (type V), non-diabetic renal disease, and occasionally genital malformations in females. Recently, familial hypoplastic glomerulocystic kidney disease (GCKD) has been added to the clinical spectrum of HNF-1 beta gene mutations. Familial hypoplastic GCKD is a rare, dominantly inherited disorder characterized by small kidneys containing glomerular cysts, abnormal pelvicalyceal anatomy, and chronic renal failure. A family with hypoplastic GCKD occurring in the father and the daughter was screened for mutations in the HNF-1 beta gene. The sequence of exon 4 of the HNF-1 beta gene revealed a C insertion at codon 334 resulting in a frameshift mutation (P334fsinsC) in two family members. The P334fsinsC allele co-segregated with hypoplastic GCKD in the family. Oral glucose tolerance testing was normal in the 11-year-old girl. In her 38-year-old father, impaired glucose tolerance was detected. These studies provide further evidence that familial hypoplastic GCKD is associated with HNF-1 beta gene mutations. HNF-1 beta gene mutation screening may prove useful in patients with small cystic kidneys and chronic renal failure, in whom a definite renal diagnosis could otherwise only be established by renal biopsy.

Future expectations--what paediatric nephrologists and urologists await from paediatric uroradiology.

Cooperation between paediatric nephrology/urology and paediatric radiology is essential for timely and correct diagnosis and therapy of kidney and urinary tract disorders. We need the direct contact between doctors before or after investigations, interdisciplinary discussions, and rapid access to the images. This should lead to optimal settings for investigations, a reduction of radiation burden and the number of investigations, and further improvement in the management of patients. Modern sonography including colour Doppler sonography, amplitude-coded Doppler sonography, and eventually 3D-ultrasound is and will be the method of choice as the basic, non-invasive investigation. These investigations should become the routine in all institutions. Refinement and standardisation of already established investigations are needed. Recently introduced investigations, like MRI, will have to show their impact on future diagnostic imaging. Future introduction of new non-invasive methods is welcome, e.g. to correctly diagnose vesicoureteral reflux without catheterisation/puncture of the bladder.