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The case of a 12-year-old boy with sports-induced recurrent macrohematuria and left-sided flank pain is reported. After extensive laboratory and imaging diagnostics, the diagnosis of nutcracker syndrome is made based on the characteristic clinical manifestation. Under a conservative approach and abstention from the triggering sport, a clinical as well as image-morphologically confirmed maturation occurred.

Several scientific fields, including Astrophysics, Astroparticle Physics, Cosmology, Nuclear and Particle Physics, and Research with Photons, are estimating that by the 2020 decade they will require data handling systems with data volumes approaching the Zettabyte distributed amongst as many as 1018 individually addressable data objects (Zettabyte-Exascale systems). It may be convenient or necessary to deploy such systems using multiple physical sites. This paper describes the findings of a working group composed of experts from several

The MAGIC data center has recently introduced a new computing service, which is available to the whole collaboration. This service will be complemented with DataCooker, an analysis system currently in development. This system implements the MAGIC data analysis chain and integrates it in the data center infrastructure. DataCooker is designed to provide an easy access to the computing service without the need to know its details or deal with the complexities of its use.

Data transfers between Supercomputing centers and Grid sites may be complicated since, in general, Supercomputing centers are not completely incorporated in the Grid environment. In particular, the Port d'Informació Científica (PIC) had to transfer data of cosmological simulations from the Barcelona Supercomputing Center (BSC) to Grid storage resources in order to post-process those data at PIC. The data connection is limited to the Secure Shell (SSH) protocol using the scp command. Besides a limitof bandwidth, we had to deal with a lack of automation and reliability of the transfers. PIC managed to control those scp transfers through gLite's File Transfer Service (FTS). Therefore, the discs containing the data at the BSC are mounted via SSH to a local Storage Resource Management (SRM) server at PIC. Once established the access to the data through the SRM server, the data can be transfered to any other SRM server, including the storage system at PIC, using grid protocols. Although we cannot improve the bandwidth, we reach a level of automation and reliability comparable to transfers from the Tier-0 to Tier-1 sites of the Large Hadron Collider (LHC) experiments.

The Silver-Russell syndrome (SRS) is a rare imprinting disease associated with pre- and postnatal growth retardation, craniofacial features, and asymmetry. Genitourinary abnormalities are seen in up to 20% of affected individuals. Apart from structural renal anomalies, cryptorchidism and hypospadias occur frequently in boys, while girls often have anomalies similar to those in Mayer-Rokitansky-Küster-Hauser syndrome with congenital hypoplasia or aplasia of the uterus and upper part of the vagina. Frequently hypospadias repair and orchiopexy are difficult because of lack of buccal mucosa due to facial dysmorphism and intraabdominal position of the testicles, respectively. Anesthetic problems with SRS children can be profound and mostly concern a difficult airway due to facial dysmorphism. Especially the young, very thin SRS patients are prone to hypoglycemia and hypothermia and require close perioperative monitoring. Children with SRS and their families face challenges from birth to adulthood. In case of urogenital abnormalities, they should receive multidisciplinary care by pediatric urologists/surgeons and pediatric anesthesiologists in a center of expertise in rare diseases.

ZusammenfassungEs wird über den Fall eines 12-jährigen Jungen mit sportinduzierter, rezidivierender Makrohämaturie und linksseitigen Flankenschmerzen berichtet. Nach umfangreicher laborchemischer sowie bildgebender Diagnostik wird in Zusammenschau des charakteristischen klinischen Bildes die Diagnose Nussknackersyndrom gestellt. Unter konservativen Maßnahmen sowie Verzicht auf den auslösenden Sport kam es zu einer klinischen sowie bildmorphologisch bestätigten Maturation.

Besides physical examination, ultrasonography is the most valuable diagnostic tool to assess the scrotum and testes in the case of an acute scrotum or scrotal pathology. In infants and toddlers the examination can be challenging. Due to the limited patient compliance, the small testicular size (< 0.5 ml), and low blood flow velocity (< 3 cm/s), it can be difficult to achieve a proper flow curve when assessing blood flow. The examiner's skills are as important as adequate equipment (i. e., linear ultrasound probe, 12-14 MHz) and optimal program settings (Doppler scale < 3 cm/s, gate 1 mm). However, if there is doubt, surgical exploration is unavoidable.

Zusammenfassung Das Silver-Russell-Syndrom (SRS) ist ein Imprinting-Defekt vorrangig mit intrauteriner und postpartal persistierender Wachstumsretardierung, sowie Gesichtsschädeldysmorphie. Die Diagnosestellung ist aufgrund der großen Symptomvariabilität und der genetischen Heterogenität erschwert. 20 % der Betroffenen weisen urogenitale Fehlbildungen auf. Während die Nierenanomalien häufig nicht therapierelevant sind, bedürfen die zumeist proximalen Hypospadien komplexer Rekonstruktionen. Dabei muss berücksichtigt werden, dass aufgrund der kraniofazialen Fehlbildung Mundschleimhaut nicht oder nur begrenzt zur Verfügung steht. Uni- oder bilaterale Hodenhypo- und -aplasien sowie intraabdominale Fehllagen der Hoden erfordern nicht selten aufwändige Rekonstruktionskonzepte, da primär stets ein hodenerhaltendes Vorgehen anzustreben ist. Mädchen weisen oft Mayer-Rokitansky-Küster-Hauser-Syndrom-ähnliche Anomalien mit Hypo- oder Aplasie von Uterus und proximaler Vagina auf. Bedarf die Behandlung von Kindern mit seltenen Erkrankungen generell einer großen kinderanästhesiologischen Erfahrung, so ist dies bei SRS-Patienten besonders der Fall. Speziell die kraniofaziale Dysmorphie kann den Einsatz von fiberoptischen Intubationshilfen notwendig machen. Aufgrund des erhöhten Hypoglykämie- und Hypothermierisikos ist zudem auf die perioperative Homöostase zu achten. Von der Geburt bis in das Erwachsenenalter stehen SRS-Patienten und deren Eltern oft vor großen Herausforderungen. Für eine optimale primäre Versorgung und geordnete Transition sollten deshalb diese Patienten auch aus kinderurologischer und kinderanästhesiologischer Sicht künftig möglichst nur in Kliniken mit entsprechender Fachkompetenz und Infrastruktur behandelt werden.

Laparoscopy is an established diagnostic procedure but is less frequently used as a therapeutic method for impalpable testes. To compare the results of the classic two-stage orchidopexy described by Fowler-Stephens with a testicular vessel-sparing single-stage approach in the management of intra-abdominal testes we retrospectively analyzed our data.From January 2005 to September 2010, 105 patients (mean age 32 months) underwent laparoscopy for impalpable testes. In cases of intra-abdominal testes, laparoscopic orchidopexy was performed either in a two-stage procedure including initial ligation of the testicular vessels or as a direct single-stage procedure without ligation of the vessels. The results of both approaches were evaluated postoperatively clinically and by ultrasonography. Among the 122 impalpable testes 63 were located intra-abdominally; single-stage orchidopexy was performed in 29 testes whereas a two-stage approach was conducted in 14 testes. Fourteen gonads had to be removed due to atrophy and in six cases no testis was found at all. In the other 59 cases inguinal exploration followed resulting in 22 orchidopexies, 34 removals and 3 blind-ending vessels. During a mean follow-up of 17 months none of the 29 testes treated by single-stage orchidopexy atrophied or reascended. By contrast, two cases of atrophy, one reascent and one subumbilical wound infection occurred after the two-stage procedure. Considering our excellent experiences with single-stage management, we conclude that the single-stage approach is a reliable, safe and efficacious treatment modality of intra-abdominal testes. However, the two-stage procedure including testicular vessel ligation should be restricted to high abdominal testes with very short vessels.

Zusammenfassung Hintergrund Die (Duplex-)Sonographie ist neben der körperlichen Untersuchung das wichtigste diagnostische Verfahren zur Beurteilung des akuten Skrotums und skrotaler Pathologien. Im Säuglings- und Kleinkindalter gestaltet sich aber die Anwendung des Diagnostikums durchaus als schwierig und ist deshalb nicht uneingeschränkt auf alle Altersgruppen anwendbar. Problematik Kleine Hodenvolumina (< 0,5 ml) und langsame systolische Blutflussgeschwindigkeiten (< 3 cm/s) erschweren oft die Ableitung und Interpretation der intratestikulären Blutflusskurven, zusätzlich zur oft eingeschränkten Patientencompliance. Schlussfolgerung Für den Erfolg der Methode ist ein entsprechendes Equipment (lineare Ultraschallsonde 12–14 MHz) und eine optimale Geräteeinstellung (Dopplerskalierung < 3 cm/s, Gate 1 mm, niedriger Wandfilter) ebenso wichtig wie ein versierter Untersucher. Die Umsetzung der Sonographie darf nicht zur unnötigen Zeit- und damit Therapieverzögerung führen. Im Zweifelsfall gilt deshalb stets die Prämisse Hoden- bzw. Befundfreilegung.