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This study aimed to predict the modification of the alignment between the tibial tubercle (TT) and the trochlear groove (TG) that occurs during femoral or tibial hemiepiphysiodesis. MRI scans of 541 knees were retrospectively reviewed to determine the distances between the cranial insertion of the patellar tendon on the TT and the femoral physis (FP)/tibial physis (TP). Thereafter, we developed a trigonometric formula to calculate the predicted change of the TT-TG distance that occurs during hemiepiphysiodesis around the knee using both the planned angular correction as well as the length between the physis (both distal femoral and proximal tibial) and the insertion of the patellar tendon of the TT. This study showed that TT-FP and TT-TP distances vary very little with sex and age during growth and the mean values of FP-TT and TP-TT distances (55 and 7 mm, respectively) can thus be used in clinical settings for calculating a rough estimate of the translation of the TT position that will occur during 'guided growth'. On this subject, one can expect a 1 mm simultaneous lateral or medial transfer of the TT for every 1° of angular correction during distal femoral hemiepiphysiodesis. For proximal tibial hemiepiphysiodesis, an angular correction of 8° should roughly translate into a simultaneous 1 mm transfer of the TT. This study puts forward the hypothesis that a simultaneous modification of the TT-TG distance has to be expected following hemiepiphysiodesis, whether femoral or tibial. LEVEL OF EVIDENCE III.

Objectives To test the performance of a single 3D IR T2-Weighted sequence compared to a Whole-body MRI protocol including DWI, T1-Weighted and STIR 3D IR (3S) in a pediatric population. Methods Two radiologists (15 and 30 years of experience),reviewed WBMRIs: first the STIR alone and 2 weeks later the 3S protocol. The indications were variable. Only positive findings were explicitly reported. A third reader compared the results to gold standard (GS) exams specific for the pathology. Agreement between the two readers, sensitivity and positive predictive value of STIR were calculated. Results fifty-four WBMRIs were included (16 suspected child abuse, 8 chronic recurrent multifocal osteomyelitis (CRMO), 11 lymphomas, 4 osteosarcomas, 9 neuroblastomas, 6 histiocytosis). The mean age was 6 years 10 months, range: 1 month to 15 years. Agreement between readers was of 0.87 [0.82–0.91] for 3D STIR, and 0.89 [0.83–0.93] for the 3S protocol. For reader 1 sensitivity of 3D STIR was 81.6% and of 3S 81.0%. For reader 2 it was 74.1% for 3D STIR and 74.7% for 3S. For both readers and for both protocols, the positive predictive value (PPV) depended on the type of disease (for example 100% histocytosis and osteosarcomas, >90% for child abuse, >85% CRMO but Conclusions Sensitivities were not different between the 2 protocols, for each reader and were different between the 2 readers for each protocol.

The fission yeast Schizosaccharomyces pombe has been an invaluable model system in studying the regulation of the mitotic cell cycle progression, the mechanics of cell division and cell polarity. Furthermore, classical experiments on its sexual reproduction have yielded results pivotal to current understanding of DNA recombination and meiosis. More recent analysis of fission yeast mating has raised interesting questions on extrinsic stimuli response mechanisms, polarized cell growth and cell-cell fusion. To study these topics in detail we have developed a simple protocol for microscopy of the entire sexual lifecycle. The method described here is easily adjusted to study specific mating stages. Briefly, after being grown to exponential phase in a nitrogen-rich medium, cell cultures are shifted to a nitrogen-deprived medium for periods of time suited to the stage of the sexual lifecycle that will be explored. Cells are then mounted on custom, easily built agarose pad chambers for imaging. This approach allows cells to be monitored from the onset of mating to the final formation of spores.

In this paper we illustrate the principal extraspinal pathologies causing sciatica and new approaches for the study of structures such as the lumbosacral plexus (LSP). Visualisation of the LSP in its entirety is difficult with conventional two-dimensional MRI sequences owing to its oblique orientation. In our institution, we have found that the utilisation of three-dimensional short tau inversion-recovery sampling perfection with application-optimised contrasts using different flip angle evolutions sequence is helpful, allowing multiplanar and maximum intensity projection reconstructions in the coronal oblique plane and curvilinear reformats through the plexus. Diffusion tensor imaging enables the observation of microstructural changes and can be useful in surgical planning. The normal anatomy of the LSP, its different extraspinal pathologies and differential diagnoses are thoroughly presented.

During asymmetric cell division, spindle positioning is critical for ensuring the unequal inheritance of polarity factors. In budding yeast, the mother-bud neck determines the cleavage plane and a correct nuclear division between mother and daughter cell requires orientation of the mitotic spindle along the mother-bud axis. A surveillance device called the spindle position/orientation checkpoint (SPOC) oversees this process and delays mitotic exit and cytokinesis until the spindle is properly oriented along the division axis, thus ensuring genome stability. Cytoskeletal proteins called septins form a ring at the bud neck that is essential for cytokinesis. Furthermore, septins and septin-associated proteins are implicated in spindle positioning and SPOC. In this review, we discuss the emerging connections between septins and the SPOC and the role of the mother-bud neck as a signaling platform to couple proper chromosome segregation to cytokinesis.

Partial rhombencephalosynapsis (PRECS) has been recently reported in association with Chiari II (CII). However, its existence as a true malformation is challenged due to the anatomical changes potentially induced by CII. The aim of this report was to investigate the contribution of midbrain/hindbrain tractography in this setting. A 13-year-old boy with a known CII malformation and operated myelomeningocele was referred for brain imaging after a first complex partial seizure. In addition to the classical features of CII, MRI showed partially fused cerebellar hemispheres and multiple supratentorial abnormalities. Diffusion tensor imaging (DTI) color map and tractography showed absent transverse fibers on the midsection of the cerebellum, scarce fibers of the middle cerebellar peduncle (MCP), absence of the middle pontine crossing tract, and fibers running vertically in the medial part of the cerebellum. Vertical mediocerebellar fibers are a feature of classical RECS and the paucity or absence of MCP fibers is mainly described in CII. In our patient, DTI and FT therefore demonstrated structural characteristics of both RECS and CII confirming their potential coexistence and suggesting possible shared embryological pathway.

Internal hernias are an extremely rare complication after pediatric liver transplantation, and its presentation with cholestasis has not been described to date. We report the case of a 12-yr-old boy who presented with moderate abdominal pain 11 yr after split liver transplantation and biliary-enteric anastomosis. He developed severe jaundice within 24 h of initial presentation. Imaging studies revealed ascites, dilation of the intrahepatic bile ducts, a dilated Roux-en-Y-loop, with the loop truncated at the level of the mesenteric artery, which performed a narrow right-to-left loop. At laparotomy, a transmesenteric internal hernia at the root of the jejunal mesentery was identified, originating from the creation of the Y-loop; the Roux-en-Y-loop and its adjacent intestinal loops had slipped through the opening. The Roux-en-Y loop was ischemic from strangulation, and the rest of the intestine well perfused. No surgical resection was necessary following reduction. The patient recovered completely. We discuss diagnosis and management of internal hernias, and review radiological signs. Internal transmesenteric hernias can occur at any time after liver transplantation and prompt diagnosis and surgical treatment are vital.

We present a case of intra-articular fibroma of the tendon sheath of the knee involving a teenager. The diagnosis, radiologic investigations, and treatment with a literature review are presented for this rare occurrence.

To assess the contemporary bacteriologic epidemiology of pediatric osteoarticular infection with particular regard to children's ages, because Kingella kingae has gained increasing recognition as the predominant pathogen for osteoarticular infection in young children.Retrospective file review of enrolled children from 0 to 15 years of age, admitted to our institution from 2007 to 2015 for suspected osteoarticular infection (217 cases). Information on age, sex, the bone or joint infected, imaging studies, and laboratory data (including bacterial investigations) were collected for analysis.Microorganism identification was possible for 138 infected children (63.6%), through blood (cultures or polymerase chain reaction [PCR]) and/or operative samples (cultures or PCR). Thirty-one patients (14.3%) were found to both have positive blood cultures and operative samples. The results of positive bacteriology specimens identified the most common causative pathogen for osteoarticular infection as K kingae (47.8% of microbiologically confirmed osteoarticular infections of all ages, and 87.7% in children between the ages of 6 and 48 months), significantly more common than Staphylococcus aureus (35.5% of microbiologically confirmed osteoarticular infections of all ages, and 78.2% in children4 years of age).Use of the appropriate PCR assays demonstrated that K kingae currently is the major bacterial cause of pediatric osteoarticular infection, especially in children4 years of age in whom K kingae is more common than S aureus. PCR assays should be used in routine microbiologic laboratory evaluation to improve diagnostic performance. However, despite the use of molecular methods, there are many osteoarticular infections in which no microorganism is detected, which suggests that these infections may be caused by other as yet unrecognized fastidious microorganisms.

BACKGROUND: Late-onset noninfectious pulmonary complications (LONIPCs) are life-threatening complications of bone marrow transplantation (BMT). Several pathological patterns are described in the literature with different prognoses, and with different relationships to graft-versus-host disease (GVHD). The role of high-resolution CT (HRCT) is not yet well established. OBJECTIVE: To illustrate different patterns of LONIPCs on HRCT in allogeneic versus autologous BMT in order to investigate the correlation with chronic GVHD (cGVHD). MATERIALS AND METHODS: A total of 67 HRCT scans were performed in 24 patients with noninfectious pulmonary disease at least 3 months after BMT (16 allogeneic, 8 autologous). Abnormality patterns and extension on HRCT images were correlated with the clinical outcome and with the severity of cGVHD. RESULTS: Of 24 patients, 9 showed LONIPCs (1 autologous, 8 allogeneic). There was a significant association between abnormalities on HRCT and severe cGVHD (P = 0.038), with no specific pattern. Prognosis seemed to be related to the severity of cGVHD and not to the extent of abnormalities on HRCT. CONCLUSION: The significant association between abnormalities on HRCT and severe GVHD suggests that LONIPCs can be a pulmonary manifestation of the disease. HRCT is a useful tool when combined with clinical data.

OBJECTIVE: The purpose of our study was to illustrate three cases of bilateral hydrosalpinx in postpubertal girls operated on for Hirschsprung's disease and to discuss the possible cause: iatrogenic or congenital. We identified bilateral hydrosalpinx in three postpubertal sexually inactive girls with Hirschsprung's disease treated, respectively by Duhamel, Soave pull-through, and Martin procedures. No history of surgical complications or pelvic inflammation had been reported. CONCLUSION: Hirschsprung's disease is rare in girls and bilateral hydrosalpinx is also extremely uncommon in sexually inactive adolescents. We think there may be a possible common cause: either a postsurgical complication or a congenital defect of the autonomous innervation in the context of a neurocristopathy. Because of the rarity of both conditions, the association is unlikely to be coincidental. The cause of this association is unclear and further studies are required to find its prevalence and to estimate the possible impact on fertility.

Cell fusion is universal in eukaryotes for fertilization and development, but what signals this process is unknown. Here, we show in Schizosaccharomyces pombe that fusion does not require a dedicated signal but is triggered by spatial focalization of the same pheromone–GPCR (G-protein-coupled receptor)–MAPK signaling cascade that drives earlier mating events. Autocrine cells expressing the receptor for their own pheromone trigger fusion attempts independently of cell–cell contact by concentrating pheromone release at the fusion focus, a dynamic actin aster underlying the secretion of cell wall hydrolases. Pheromone receptor and MAPK cascade are similarly enriched at the fusion focus, concomitant with fusion commitment in wild-type mating pairs. This focalization promotes cell fusion by immobilizing the fusion focus, thus driving local cell wall dissolution. We propose that fusion commitment is imposed by a local increase in MAPK concentration at the fusion focus, driven by a positive feedback between fusion focus formation and focalization of pheromone release and perception.

Children with biliary atresia are prone to developing progressive hepatic fibrosis and biliary cirrhosis following the Kasai operation. The only treatment is liver transplantation. To assess liver fibrosis by acoustic radiation force impulse elastography (ARFI) in children who had Kasai operation, with the goal of identifying an ARFI value cut-off for children requiring liver transplantation. Of the 32 post-Kasai children included, 19 were transplanted or listed for transplantation (group A), while 13 were not on the list during their follow-up (group B). We recorded biopsies, blood samples and ARFI values over time, including at Kasai operation and at transplantation. We estimated an association between groups and continuous variables using generalized estimating equations, and we compared categorical variables using the Fisher exact test. Portal hypertension signs were similar in both groups, whereas ARFI values were higher in group A (mean±standard deviation=3.3±1.2 m/s) than in group B (2.0±0.7 m/s; P=.0003). Eighteen of 19 (94.7%) children in group A and 6/13 (46.2%) children in group B presented with two consecutive ARFI values ≥2 m/s (sensitivity=7%, specificity=53.8%; P=0.003). We found that children who were transplanted had two consecutive ARFI values ≥2 m/s during follow-up. ARFI for evaluation of post-Kasai liver fibrosis may assist the long-term assessment of biliary atresia and may even guide treatment decisions.

In 1996, a gap between the literature evidence for the prevention of chemotherapy-induced emesis and the prescription pattern in clinical practice was demonstrated in a drug utilization study. This study, carried out in 103 Italian oncological centers (77 new; 26 old that participated in the previous study) evaluates three different intervention strategies to implement these guidelines. In cancer patients submitted to chemotherapy, prescriptions of antiemetics were prospectively monitored for 2 consecutive weeks in 1999. Simple diffusion of guidelines was evaluated in an observational study in the 77 new centers, while the double combination of simple diffusion and “audit and feedback” strategy was randomly compared in the old centers with the triple combination of the same two strategies plus an “educational outreach visit.” Simple diffusion of guidelines improved the prescription but only for acute and delayed emesis induced by high-moderate emetogenic chemotherapy. No significant difference was detected in the prescriptions against cisplatin-induced emesis. The inappropriate use of 5-HT3 antagonists for prophylaxis of low emetogenic chemotherapy was found most frequently. Similar poor results were achieved by the audit and feedback strategy, while the educational outreach visit significantly increased the prescription of the optimal prophylaxis for cisplatin-induced acute and delayed emesis. A combination of interventions, including an educational outreach visit, seems to be a good strategy for transferring the results of antiemetic research to oncological practice.

Background Evidence-based guidelines, consensus conferences and experts’ opinion are rarely promptly transferred to patient care. We audited prescriptions of adjuvant systemic therapies for Italian breast cancer patients and compared them with recommendations of an International Consensus Panel. Patients and methods Disease characteristics and adjuvant therapies for 768 breast cancer patients referred to 87 Italian centers from 16 to 23 March 2000 were evaluated for adherence to the published recommendations. Results Endocrine therapy was not prescribed for 102 of 541 patients (19%) with endocrine-responsive disease and for 22 of 45 patients (49%) with unknown hormonal receptor status. Instead, endocrine therapy was prescribed for 22 of 182 patients (12%) with endocrine-unresponsive disease. Adjuvant chemotherapy was prescribed for 98% of the patients. The type of chemotherapy was the cyclophosphamide, methotrexate, 5-fluorouracil regimen for 453 of 754 (60%), while 253 of 754 (34%) received an anthracycline-based regimen. The proportion of patients with anthracyclines increased with the number of involved axillary nodes and grading, and decreased with age. Endocrine therapy was administered to 482 of 768 (63%) and was mainly represented by an antiestrogen. Conclusions Lack of adherence to evidence-based guidelines for adjuvant treatment of Italian breast cancer patients was as high as 19%. It might be wise for national health authorities to promote education on life-saving procedures, like adjuvant systemic treatments, in cancer medicine.

In many cell types, septins assemble into filaments and rings at the neck of cellular appendages and/or at the cleavage furrow to help compartmentalize the plasma membrane and support cytokinesis. How septin ring assembly is coordinated with membrane remodeling and controlled by mechanical stress at these sites is unclear. Through a genetic screen, we uncovered an unanticipated link between the conserved Rho1 GTPase and its effector protein kinase C (Pkc1) with septin ring stability in yeast. Both Rho1 and Pkc1 stabilize the septin ring, at least partly through phosphorylation of the membrane-associated F-BAR protein Syp1, which colocalizes asymmetrically with the septin ring at the bud neck. Syp1 is displaced from the bud neck upon Pkc1-dependent phosphorylation at two serines, thereby affecting the rigidity of the new-forming septin ring. We propose that Rho1 and Pkc1 coordinate septin ring assembly with membrane and cell wall remodeling partly by controlling Syp1 residence at the bud neck., Molecular Biology of the Cell, 26 (18), ISSN:1939-4586, ISSN:1059-1524

The fission yeast Schizosaccharomyces pombe is an invaluable model system for studying the principles that drive sexual differentiation and the meiotic cell division cycle. We describe a simple protocol for microscopic observation of the entire sexual life cycle that can be adapted to focus on specific stages of sexual differentiation. After growth to exponential phase in a nitrogen-rich medium, cell cultures are switched to a nitrogen-deprived medium until the population is enriched for the specific stage of the sexual lifecycle to be studied. Cells are then mounted in easily constructed customized agarose pad chambers for imaging.

International audience; This article reviews the most relevant state-of-the-art magnetic resonance (MR) techniques, which are clinically available to investigate brain diseases. MR acquisition techniques addressed include notably diffusion imaging (diffusion-weighted imaging (DWI), diffusion tensor imaging (DTI), and diffusion kurtosis imaging (DKI)) as well as perfusion imaging (dynamic susceptibility contrast (DSC), arterial spin labeling (ASL), and dynamic contrast enhanced (DCE)). The underlying models used to process these images are described, as well as the theoretic underpinnings of quantitative diffusion and perfusion MR imaging-based methods. The technical requirements and how they may help to understand, classify, or follow-up neurological pathologies are briefly summarized. Techniques, principles, advantages but also intrinsic limitations, typical artifacts, and alternative solutions developed to overcome them are discussed. In this article, we also review routinely available three-dimensional (3D) techniques in neuro MRI, including state-of-the-art and emerging angiography sequences, and briefly introduce more recently proposed 3D quantitative neuro-anatomy sequences, and new technology, such as multi-slice and multi-transmit imaging.

Whole-body MRI (WBMRI) is a non-irradiating imaging technique, suitable to investigate the extent of multisystemic diseases in pediatric patients. However its real impact on diagnosis and management of non-oncological pediatric diseases remains unclear. We present our experience of pediatric WBMRI in various pathologies.We retrospectively reviewed medical files of all non- oncologic patients who underwent WBMRI from January 2008 to November 2012. The MRI protocol included T1 weighted and 3D SPACE Inversion Recovery (IR) and fat saturated diffusion weighted (DWI) sequence. We reviewed medical records in order to determine if performing WBMRI added useful information for diagnostic purposes and/or changed clinical management.Forty-two children were included in the study (19 F, 23 M) (median age 4.7 years). Twenty-one children underwent general anesthesia. WBMRI was a useful tool to provide correct diagnosis in chronic recurrent multifocal osteomyelitis (CRMO), and to identify the origin of fever or arthralgia of unknown etiology. WBMRI allowed to determine the extent of disease in juvenile idiopathic arthritis (JIA), chronic granulomatous disorder, enchondromatosis, Langerhans cell histiocytosis, and in the assessment of tumor burden in neurofibromatosis type I. For the battered child syndrome, the influence on management was rather minimal. For each of these pathologies we performed a review of recent literature.WBMRI is easy to perform in children and allows the assessment of systemic diseases or discovery of lesions ignored by clinical examination. WBMRI influenced the decisional process and clinical management of various pathologies in our series.

Laura Merlini,1 Giacomo Cartenì,2 Stefano Iacobelli,3 Caterina Stelitano,4 Mario Airoldi,5 Peter Balcke,6 Felix Keil,7 Ferdinand Haslbauer,8 Laura Belton,9 Beatriz Pujol10 1Department of Medical Oncology, Ospedale Civile S, Bortolo, Vicenza, 2Department of OncoHematology, Azienda Ospedaliera di Rilievo Nazionale "Antonio Cardarelli", Napoli, 3Department of Medical Oncology, Ospedale Clinicizzato SS Annunziata, Chieti, 4Department of Hematology, Azienda Ospedaliera "Bianchi Melacrino Morelli", Reggio Calabria, 5Department of Medical Oncology, Azienda Ospedaliero Universitaria Le Molinette, Torino, Italy; 61st Medical Department, General Hospital St Pölten and Karl Landsteiner Institute of Oncology, St Pölten, 73rd Medical Department (Hematology and Oncology), Hanusch Krankenhaus der Wiener Gebietskrankenkasse, Vienna, 8Department of Oncology, Landeskrankenhaus Vöcklabruck, Vöcklabruck, Austria; 9Contract biostatistician, Amgen Ltd, Uxbridge, UK; 10Research and Development Haematology/Oncology, Amgen Europe, Zug, Switzerland Purpose: To describe the prevalence and management of anemia in cancer patients. Methods: This cross-sectional, observational survey was conducted in Italy and Austria. Centers prespecified one day, during a 4-month enrollment window, to report specific data collected during normal clinical practice for patients with non-myeloid tumors attending for chemotherapy (±radiotherapy) treatment. The primary endpoint was the prevalence of anemia as determined using a prespecified algorithm: hemoglobin (Hb) ≤10 g/dL on/within 3 days prior to visit; ongoing anemia treatment; physician diagnosis of anemia, together with ≥1 anemia symptom. Results: Between November 18, 2010 and March 18, 2011, data for 1412 patients were collected (Italy n = 1130; Austria n = 282). Most patients (n = 1136; 80%) had solid tumors; 809 (57%) had received ≤ 3 chemotherapy cycles. The prevalence of anemia was 32% (95% confidence interval: 29.4%–34.2%); 196 patients (14%) were deemed anemic based on Hb ≤10 g/dL, 131 (9%) on ongoing anemia treatment, and 121 (9%) on physician diagnosis/anemia symptom. Overall, 1153 patients (82%) had Hb data; mean (standard deviation [SD]) Hb levels were 11.7 (1.7) g/dL. In total, 456 patients (32%) had anemia symptoms: fatigue (n = 392; 28%), depression (n = 122; 9%), and dyspnea (n = 107; 8%) were most common. Fifty-one patients (4%) had had their current chemotherapy cycle delayed due to anemia. On visit day, or ≤ 28 days prior, 91 (6%), 188 (13%), and 81 patients (6%) had evidence of whole blood/red blood cell transfusion, erythropoiesis-stimulating agent use, or iron use, respectively. Conclusion: On the prespecified study day, one-third of patients with non-myeloid tumors undergoing chemotherapy were found to be anemic and 13% had evidence of erythropoiesis-stimulating agent use then or in the 28 days prior. Keywords: erythropoiesis-stimulating agent, hemoglobin, iron, management, transfusion&nbsp

International audience

Cell pairing is central for many processes, including immune defense, neuronal connection, hyphal fusion, and sexual reproduction. How does a cell orient toward a partner, especially when faced with multiple choices? Fission yeast Schizosaccharomyces pombe P and M cells, which respectively express P and M factor pheromones [1, 2], pair during the mating process induced by nitrogen starvation. Engagement of pheromone receptors Map3 and Mam2 [3, 4] with their cognate pheromone ligands leads to activation of the Gα protein Gpa1 to signal sexual differentiation [3, 5, 6]. Prior to cell pairing, the Cdc42 GTPase, a central regulator of cell polarization, forms dynamic zones of activity at the cell periphery at distinct locations over time [7]. Here we show that Cdc42-GTP polarization sites contain the M factor transporter Mam1, the general secretion machinery, which underlies P factor secretion, and Gpa1, suggesting that these are sub-cellular zones of pheromone secretion and signaling. Zone lifetimes scale with pheromone concentration. Computational simulations of pair formation through a fluctuating zone show that the combination of local pheromone release and sensing, short pheromone decay length, and pheromone-dependent zone stabilization leads to efficient pair formation. Consistently, pairing efficiency is reduced in the absence of the P factor protease. Similarly, zone stabilization at reduced pheromone levels, which occurs in the absence of the predicted GTPase-activating protein for Ras, leads to reduction in pairing efficiency. We propose that efficient cell pairing relies on fluctuating local signal emission and perception, which become locked into place through stimulation.

The first step towards cytokinesis in budding yeast is the assembly of a septin ring at the future site of bud emergence. Integrity of this ring is crucial for cytokinesis, proper spindle positioning, and the spindle position checkpoint (SPOC). This checkpoint delays mitotic exit and cytokinesis as long as the anaphase spindle does not properly align with the division axis. SPOC signalling requires the Kin4 protein kinase and the Kin4-regulating Elm1 kinase, which also controls septin dynamics. Here, we show that the two redundant ubiquitin-ligases Dma1 and Dma2 control septin dynamics and the SPOC by promoting the efficient recruitment of Elm1 to the bud neck. Indeed, dma1 dma2 mutant cells show reduced levels of Elm1 at the bud neck and Elm1-dependent activation of Kin4. Artificial recruitment of Elm1 to the bud neck of the same cells is sufficient to re-establish a normal septin ring, proper spindle positioning, and a proficient SPOC response in dma1 dma2 cells. Altogether, our data indicate that septin dynamics and SPOC function are intimately linked and support the idea that integrity of the bud neck is crucial for SPOC signalling., PLoS Genetics, 8 (4), ISSN:1553-7390, ISSN:1553-7404

Background: K. kingae is a common causative organism in acute osteoarticular infections (OAIs) in children under 4years of age. Differentiation between K. kingae and Gram-positive cocci (GPC) is of great interest therapeutically. Objective: Our aim was to identify early distinguishing MRI features of OAIs. Materials and methods: Thirty-one children younger than 4years of age with OAI underwent MRI at presentation. Of these, 21 were caused by K. kingae and ten by GPC. Bone and soft tissue reaction, epiphyseal cartilage involvement, bone and subperiosteal abscess formation were compared between the two groups. Interobserver agreement was measured. Results: Bone reaction was less frequent (P = 0.0066) and soft tissue reaction less severe (P = 0.0087) in the K. kingae group. Epiphysis cartilage abscesses were present only in the K. kingae group (P = 0.0118). No difference was found for bone abscess (P = 0.1411), subperiosteal abscess (P = 1) or joint effusion (P = 0.4414). Interobserver agreement was good for all criteria. Conclusion: MRI is useful in differentiating K. kingae from GPC in OAI. Cartilaginous involvement and modest soft tissue and bone reaction suggest K. kingae

Internal hernias are an extremely rare complication after pediatric liver transplantation, and its presentation with cholestasis has not been described to date. We report the case of a 12-yr-old boy who presented with moderate abdominal pain 11 yr after split liver transplantation and biliary-enteric anastomosis. He developed severe jaundice within 24 h of initial presentation. Imaging studies revealed ascites, dilation of the intrahepatic bile ducts, a dilated Roux-en-Y-loop, with the loop truncated at the level of the mesenteric artery, which performed a narrow right-to-left loop. At laparotomy, a transmesenteric internal hernia at the root of the jejunal mesentery was identified, originating from the creation of the Y-loop; the Roux-en-Y-loop and its adjacent intestinal loops had slipped through the opening. The Roux-en-Y loop was ischemic from strangulation, and the rest of the intestine well perfused. No surgical resection was necessary following reduction. The patient recovered completely. We discuss diagnosis and management of internal hernias, and review radiological signs. Internal transmesenteric hernias can occur at any time after liver transplantation and prompt diagnosis and surgical treatment are vital.

Summary Purpose This study aimed to assess the feasibility of magnetic resonance (MR) neurography in children, and the potential roles of diffusion-weighted imaging (DWI) and fiber-tracking (FT) techniques. Methods Five pediatric patients (age range: 6–12 years) underwent magnetic resonance imaging (MRI) for various clinical indications: neurogenic bladder (case 1); persistent hand pain following minor trauma (case 2); progressive atrophy of the lower left extremity muscles (case 3); bilateral hip pain (case 4); and palpable left supraclavicular mass (case 5). All studies were performed using a 1.5-T Avanto MRI scanner (Siemens, Erlangen, Germany). The protocol included 3D T2-weighted STIR and SPACE imaging, T1-weighted fat-saturation post-gadolinium imaging and diffusion tensor imaging (DTI) with tractography. ADC (N × 10−3 mm2/s) and FA values were calculated from regions of interest (ROIs) centered on the nerves. Nerve-fiber tracks were calculated using a fourth-order Runge-Kutta algorithm (NeuroD software). Results MR neurography allowed satisfactory visualization of all neural structures, and FA and ADC measurements were feasible. The final diagnoses were Tarlov cysts, median-nerve compression, sciatic perineurioma, Charcot-Marie-Tooth disease and plexiform neurofibroma in a patient with NF-1. Discussion FA and ADC measurements are of little value because of the lack of normal reference values. Nerve-fiber tractography (FT) may be of value in the characterization of tumor pathology, and is also helpful in the planning of surgical treatments. Conclusion MR neurography is feasible in pediatric patients. However, a considerable amount of work has yet to be done to establish its role in the clinical management of the wide range of peripheral nerve diseases.

BACKGROUND: Congenital ocular motor apraxia (COMA) occasionally shares with Joubert syndrome (JS) and related disorders (JSRDs) a peculiar malformation, the 'molar tooth sign' (MTS). In JSRDs, the absence of superior cerebellar peduncles (SCP) decussation is reported. OBJECTIVE: To investigate whether COMA demonstrates similar abnormal axonal pathways. MATERIALS AND METHODS: Eight healthy age-matched controls, three children with clinical COMA and one child with clinical JSRD underwent examination with a 1.5-T MRI scanner. Diffusion-weighted imaging (DWI), colour-coded fractional anisotropy maps and three-dimensional diffusion tensor imaging (DTI) tractography of the cerebellorubral network were analyzed. RESULTS: On DTI cartography, the 'red dot' originally supposed to represent the SCP decussation in the midbrain was present in controls as well in those with COMA but absent in the single case with JS. In none of the subjects including controls was 3-D FT able to depict the SCP decussation. When seeded, the red dot resulted in the ventral tegmental decussation (VTD). It was normal in controls and in patients with COMA but was absent in our single patient with JSRD. MTS was identified in alla patients with COMA and in the patient with JSRD. CONCLUSION: MTS can be present in both COMA and JSRD but the underlying anatomy depicted by fibre tracking is distinct. The main difference is the integrity of the VTD in COMA.

Antenatal hydronephrosis (ANH) is a frequent anomaly detected on fetal ultrasound scans. There is no consensus recommendation for the postnatal follow-up and/or the necessity to perform a voiding cystourethrography (VCUG) to diagnose vesicoureteral reflux (VUR). We conducted a cohort/non-randomized trial of 121 patients with ANH, defined as an anterior posterior diameter (APD) ≥ 5 mm after the 20th week of gestation, to evaluate the ability of the antenatal and postnatal ultrasonography results to predict VUR. All infants had two successive ultrasounds at 5 days and 1 month, respectively, after birth. A VCUG was performed at 6 weeks in children with a persistent APD ≥ 5 mm and/or an ureteral dilatation observed on at least one of two postnatal ultrasounds. In total, 88 patients had VCUG and nine had VUR, with five having high-grade reflux (>grade II). The risk of VUR increased significantly with the degree of APD detected on the postnatal ultrasound scan (p = 0.03). The odds ratios were 5.0 [95% confidence interval (CI) 0.5-51.2] for APD = 7-9 mm and 9.1 (95% CI 1.0-80.9) for APD ≥ 10 mm. The results of this study show that among our patient cohort antenatal ultrasound was not predictive of reflux. There was, however, a relation between the importance of the postnatal renal pelvis diameter and the risk of VUR. A cut-off of 7 mm showed a fair ability of ultrasonography to predict VUR and a cut-off of 10 mm enabled all severe refluxes in the 88 patients who had a VCUG to be diagnosed.

Vasculitides and aneurysm formation are well-known complications in Wiskott-Aldrich syndrome (WAS), most often appearing later in life, usually in second decade. The authors report the case of a 5-month-old boy with a genetically and phenotypically severe Wiskott-Aldrich syndrome and sequential formation and spontaneous thrombosis of hepatic aneurysms. This case demonstrates that aneurysm formation may develop early in the course of severe WAS phenotypes. Because of the progressive nature of these manifestations, surgical or interventional procedures are not advisable. Early allogeneic hematopoietic stem cell transplantation (HSCT) should be considered before the manifestation of irreversible organ damage.

Metabolic diseases of the brain are a rare occurrence. They may be either occurring as inherited diseases causing a destruction of myelin, or they may be the result of toxicity. Neuroimaging, especially magnetic resonance imaging, plays an important role in the detection and classification of these rare diseases. Magnetic resonance spectroscopy is an important tool in the characterization and diagnosis of inherited metabolic diseases.

Noninflammatory tubal abnormalities are rare in children and usually not well covered by traditional educational material. The presenting symptoms are nonspecific and are common to many other conditions, so its preoperative diagnosis is rarely made. The purpose of this study was to review the hospital charts and imaging findings in children and sexually inactive adolescents who showed fallopian tube pathology. Understanding of the pertinent findings of previous imaging examinations might assist radiologists in making the correct preoperative diagnosis and increase the likelihood of preserving the fallopian tubes. The clinical entities described in this article include isolated tubal torsion, paratubal cysts, hydrosalpinx, undescended/ectopic fallopian tube, and tubal inguinal hernia.

Mild antenatal renal pelvic dilatation (ARPD) revealed by prenatal ultrasound (US) raises the question whether or not screening for vesicoureteral reflux (VUR) is mandatory. The aim of our study was to suggest guidelines for postnatal management of infants with mild ARPD defined as an antero-posterior (AP) dilatation5 and10 mm.Therefore we assessed the value of postnatal US at day 30 to predict VUR, the incidence of VUR at day 30 and the rate of spontaneous resolution at 1 year. Two hundred (200) infants with ARPD were included and had renal US and voiding cystourethrography (VCUG) at day 30. If VUR was present, VCUG was repeated 1 year later.Incidence of VUR was 10% (20/200) at day 30 after birth and only 3% (6/200) 1 year later. VUR at day 30 was twice as frequent in children with postnatal dilatation (11%) than in nondilated kidneys (6%).Considering the low incidence of VUR at 1 year, screening for VUR in mild ARDP seems not to be justified. However follow-up by US to detect increase in dilatation and clinical monitoring for signs of urinary infection is required.

Objectifs Determiner la demarche radiologique la plus adequate pour diagnostiquer precocement une fracture de rate chez l’enfant. Materiels et methodes Nous avons etudie retrospectivement 22 cas de fracture de rate sur 2 ans. Sur les 22 enfants, 4 ont beneficie d’une TDM en premiere intention. Dix-huit enfants ont eu un US en premiere intention, suivi d’une TDM. Les lesions ont ete controlees par echographie sur une periode de 5 jours a 4 mois. Resultats Chez les 4 enfants ayant eu une TDM en premiere intention, l’examen montrait une fracture de rate (n = 4), du liquide intra-peritoneal (n = 4), une lesion grele (n = 1) et une contusion renale et pancreatique (n = 1). Chez les 18 enfants ayant eu un US en premiere intention, du liquide intraperitoneal a ete retrouve dans tous les cas, la fracture splenique n’etait visible que dans 3 cas. Cet examen a montre en outre 2 epaississements de paroi digestive et un epanchement pleural. Les US de controle ont revele la disparition du liquide peritoneal entre 1 jour et 4 mois. Tous les enfants ont beneficie d’un traitement conservateur avec surveillance echo-clinique. Conclusion L’US est l’examen de premiere intention dans le cadre du traumatisme abdominal chez l’enfant. La presence de liquide intra-peritoneal meme en quantite minime est un signe d’appel qui doit entrainer la realisation immediate d’une TDM. La surveillance echographique du liquide peritoneal est indispensable pour s’assurer de la guerison de la lesion.

Tumors of the pediatric facial skeleton represent a major challenge in clinical practice because they can lead to functional impairment, facial deformation, and long-term disfigurement. Their treatment often requires a multidisciplinary approach, and radiologists play a pivotal role in the diagnosis and management of these lesions. Although rare, pediatric tumors arising in the facial bones comprise a wide spectrum of benign and malignant lesions of osteogenic, fibrogenic, hematopoietic, neurogenic, or epithelial origin. The more common lesions include Langerhans cell histiocytosis and osteoma, while rare lesions include inflammatory myofibroblastic and desmoid tumors; juvenile ossifying fibroma; primary intraosseous lymphoma; Ewing sarcoma; and metastases to the facial bones from neuroblastoma, Ewing sarcoma, or retinoblastoma. This article provides a comprehensive approach for the evaluation of children with non-odontogenic tumors of the facial skeleton. Typical findings are discussed with emphasis on the added value of multimodality multiparametric imaging with computed tomography (CT), magnetic resonance imaging (MRI) with diffusion-weighted imaging (DWI), positron emission tomography CT (PET CT), and PET MRI. Key imaging findings and characteristic histologic features of benign and malignant lesions are reviewed and the respective role of each modality for pretherapeutic assessment and post-treatment follow-up. Pitfalls of image interpretation are addressed and how to avoid them.

The online version of the original article can be found at http://dx.doi.org/10.1007/s00234-015-1500-1 .

Background In children, septic arthritis (SA) of the hip is either primary or concomitant with acute haematogenous osteomyelitis (AHO). However, seldom, patients with isolated SA at presentation, may later show osteomyelitis in the metaphysis. The aim of this study was to elaborate a physiopathological hypothesis based on the peculiar MRI findings to explain the onset of AHO after SA. Methods Cases of acute infection of the hip admitted between January 2010 and December 2013 were retrospectively reviewed to assess radiographic and MRI features, as well as bacteriological findings. Only children with isolated SA were included in this study, whereas cases of concomitant SA and AHO at presentation were excluded. Results Ten patients met the inclusion criteria. Six (1–11 months) demonstrated, on the initial MRI, decreased perfusion on gadolinium enhanced fat-suppressed T1-weighted sequence of the femoral epiphysis and developed one month later metaphyseal AHO. Four (5–14 years) did not show decreased perfusion and did not develop AHO on follow-up. The type of germ involved influenced neither the type of enhancement pattern nor the outcome. Conclusions Age under one year and decreased perfusion of the affected femoral epiphysis increases the risk of secondary AHO. Our study is the first report in human medicine supporting the physiopathological hypothesis described by Alderson et al. in an animal model: primary infection can originally affect the joint, then penetrate the epiphyseal cartilage, and finally spread into the metaphyseal region through transphyseal vessels present only in the first 12/18 months of life.