Upscaling ground-based backpack gamma-ray spectrometry to spatial resolution of UAV-based gamma-ray spectrometry for system validation.

Advances in the development of gamma-ray spectrometers have resulted in devices that are ideal for use in conjunction with the increasingly reliable systems of autonomously flying uncrewed aerial vehicles (UAVs) that have recently become available on the market. Airborne gamma-ray spectrometry (GRS) measurements have many different applications. Here, the technique is applied to a former uranium mining and processing site, which is characterized by relatively low specific activities and, hence, low count rates, requiring relatively large detectors and correspondingly big size UAVs. The future acceptance of the use of such UAV-based GRS systems for radionuclide mapping depends on their ability to measure absolute specific activities of natural radionuclides such as U-238 in near-surface soil that are consistent with the results of established and proven ground-based systems. To determine absolute specific activities on the ground, the gamma radiation data from airborne detectors must be corrected for attenuation caused by the flight altitude above ground. In recent years, mathematical procedures for altitude correction have been developed, that are specifically tailored to the working range of several tens of meters typical for UAVs. However, very limited experimental validation of these theoretical approaches is available. A very large dataset consisting of about 3000 UAV-based and 19,000 backpack-based measurements was collected at a low-grade uranium ore dump in Yangiabad, Uzbekistan. We applied different geostatistical interpolation methods to compare the data from both survey techniques by upscaling backpack data to airborne data. Compared to backpack systems, UAV-based systems have lower spatial resolution, so measurements average over larger areal units (or in geostatistical terminology: "spatial support"). Taking into account the change in spatial support, we illustrate that (1) the UAV-based measurements show good agreement with the upscaled backpack measurements and that (2) UAV surveys provide good delineation of contrasts of the relatively smooth U-238 specific activity distribution typical for former uranium mining and processing sites. We are able to show that the resolution of UAV-based systems is sufficient to map extended uranium waste facilities.

Experience with NORM waste disposal in different European countries.

Industrial processes generate various quantities of waste that can be contaminated with radionuclides of natural origin (naturally occurring radioactive material ('NORM waste')). The efficient management of this waste is essential for any industries affected by NORM waste generation. To obtain an overview of current practices and approaches in Europe, the IRPA Task Group on NORM conducted a survey among task group members and other experts from European countries. The results revealed significant differences in methods and approaches in the European countries. In many countries, landfills are used to dispose of small- and medium-sized quantities of NORM waste with limited activity concentration. But our survey indicated that despite a uniform reference basis for national legislation in Europe, there are different framework conditions for the disposal of NORM waste in practice. In some countries, the disposal is hampered because the interface between the radiation protection system and the waste regime is not yet clearly regulated. Particular practical problems include the lack of acceptance of waste because of the 'radioactivity' stigma and only vague specifications by the legislators on acceptance obligations of the waste management sector.

The European NORM Association (ENA)-promoting radiation protection in the field of norm in Europe.

The European NORM Association (ENA) was founded in 2017, merging previously informal European networks. It has the statute of an International Non-profit Organization established under Belgian Law. The objective of ENA is to promote and advance radiation protection in the context of exposure to NORM. It operates as a European platform and forum for discussion, dissemination and exchange of information, training and education and by supporting scientific knowledge and new directions of research related to NORM issues. A key activity of ENA is to share practical solutions. To this end, ENA gathers radiation protection practitioners, regulators, scientists and industry representatives in order to support the management of NORM in compliance with European standards and according to best practices. Since its foundation, ENA has organized three workshops where topical issues on NORM have been discussed. It has established close working relationships and links with IAEA, HERCA, IRPA and other international initiatives-getting a recognition at international level. ENA has set up working groups on NORM in the industry, in the environment, in building materials and, as recently as in 2021, a working group on decommissioning of NORM facilities. A series of webinars have been organized to present case studies on NORM decommissioning and discuss associated challenges and practical solutions.

Pediatric computed tomography doses in Germany from 2016 to 2018 based on large-scale data collection.

PURPOSE: Accumulating evidence from epidemiological studies that pediatric computed tomography (CT) examinations can be associated with a small but non-zero excess risk for developing leukemia or brain tumor highlights the need to optimize doses of pediatric CT procedures. Mandatory dose reference levels (DRL) can support reduction of collective dose from CT imaging. Regular surveys of applied dose-related parameters are instrumental to decide when technological advances and optimized protocol design allow lower doses without sacrificing image quality. Our aim was to collect dosimetric data to support adapting current DRL to changing clinical practice. METHOD: Dosimetric data and technical scan parameters from common pediatric CT examinations were retrospectively collected directly from Picture Archiving and Communication Systems (PACS), Dose Management Systems (DMS), and Radiological Information Systems (RIS). RESULTS: We collected data from 17 institutions on 7746 CT series from the years 2016 to 2018 from examinations of the head, thorax, abdomen, cervical spine, temporal bone, paranasal sinuses and knee in patients below 18 years of age. Most of the age-stratified parameter distributions were lower than distributions from previously-analyzed data from before 2010. Most of the third quartiles were lower than German DRL at the time of the survey. CONCLUSIONS: Directly interfacing PACS, DMS, and RIS installations allows large-scale data collection but relies on high data-quality at the documentation stage. Data should be validated by expert knowledge or guided questionnaires. Observed clinical practice in pediatric CT imaging suggests lowering some DRL in Germany is reasonable.

[Using simulated patients to test competency in child protection medicine in the third section of the medical examination]. / Einsatz von Simulationspersonen zur Prüfung von Handlungskompetenz im Bereich der Kinderschutzmedizin im Dritten Abschnitt der Ärztlichen Prüfung.

During the COVID-19 pandemic, the third section of the medical examination could be performed with simulation patients. Simulations enable standardized examinations of medical competency according to the educational objectives of the national learning objectives catalogue. The evaluation of student's medical response to child abuse and neglect seems to be an appropriate opportunity to increase attention of prospective physicians for potential child abuse. The presented case reports the simulation of a pediatric nonaccidental trauma.

Variability in the distance from the end of the gray matter to the end of the conus medullaris: a case-triggered histological investigation.

OBJECTIVE: The background for this investigation was the dramatic course of a 14-year-old girl with a spontaneous hemorrhage in the area of the conus medullaris resulting in a complete cross-sectional syndrome with bladder and bowel dysfunction. Despite immediate surgical treatment, the patient showed close to no postoperative improvement. Subsequent histopathological examination of the removed masses revealed a cavernoma. To better understand the link between the site and symptoms of conus medullaris lesions, the authors performed a literature search and then histological examination of the conus medullaris of 18 cadaveric specimens from body donors. METHODS: After a literature search regarding the histological features of the structure of the conus medullaris did not lead to satisfying results, the authors performed histological examination of the conus medullaris in 18 cadaveric specimens from body donors. The largest (a) and smallest (b) diameters of the conus medullaris were measured, noting individual variations in the distance from the caudal ending of the gray matter to the macroscopically visible end of the conus medullaris. Correlations of these differences with sex, body height, gray matter transverse diameter, and cross-sectional area at the end of the gray matter were analyzed. RESULTS: Gray matter displayed in the form of a butterfly figure was found along almost the entire length of the conus medullaris. The specific slide containing the end of the gray matter was noted. The distance between the caudal ending of the gray matter in the conus and the macroscopical end of the conus medullaris was defined as the gray matter to cone termination (GMCT) distance. There were great individual variations in the distance from the caudal ending of the gray matter to the macroscopically visible end of the conus medullaris. Analysis of the correlations of these differences with sex, body height, gray matter transverse diameter, and cross-sectional area at the end of the gray matter showed no significant sex-specific differences in the GMCT distance. Patient body height and transverse diameter at the end of the gray matter were found to be correlated positively with the GMCT distance. Moreover, greater height also correlated positively with the cross-sectional area at the end of the gray matter. CONCLUSIONS: This report is, to the authors' knowledge, the first published description of the histological structure of the conus medullaris and can serve as the basis for a better understanding of neurological deficits in patients with a conus medullaris syndrome. Findings that gray matter can be detected far into the conus medullaris, with large individual differences in the endpoint of the gray matter, are important for operative care of intramedullary masses and vascular malformations in this area. It is therefore important to use electrophysiological monitoring during these operations.

European Federation of Societies for Ultrasound in Medicine and Biology (EFSUMB): An Update on the Pediatric CEUS Registry on Behalf of the "EFSUMB Pediatric CEUS Registry Working Group".

The European Federation of Ultrasound in Medicine and Biology (EFSUMB) created the "EFSUMB Pediatric Registry" (EFSUMB EPR) with the purpose of collecting data regarding the intravenous application of pediatric contrast-enhanced ultrasound (CEUS). The primary aim was to document the current clinical practice and usefulness of the technique and secondarily to assess CEUS safety in children. We issue the preliminary results of this database and examine the overall practice of CEUS in children in Europe.

Assessment of Waldeyer's ring in pediatric and adolescent Hodgkin lymphoma patients-Importance of multimodality imaging: Results from the EuroNet-PHL-C1 trial.

BACKGROUND: In the EuroNet Pediatric Hodgkin Lymphoma (EuroNet-PHL) trials, decision on Waldeyer's ring (WR) involvement is usually based on clinical assessment, that is, physical examination and/or nasopharyngoscopy. However, clinical assessment only evaluates mucosal surface and is prone to interobserver variability. Modern cross-sectional imaging technology may provide valuable information beyond mucosal surface, which may lead to a more accurate WR staging. PATIENTS, MATERIALS, AND METHODS: The EuroNet-PHL-C1 trial recruited 2102 patients, of which 1752 underwent central review including reference reading of their cross-sectional imaging data. In 14 of 1752 patients, WR was considered involved according to clinical assessment. In these 14 patients, the WR was re-assessed by applying an imaging-based algorithm considering information from 18 F-fluorodeoxyglucose positron emission tomography, contrast-enhanced computed tomography, and/or magnetic resonance imaging. For verification purposes, the imaging-based algorithm was applied to 100 consecutive patients whose WR was inconspicuous on clinical assessment. RESULTS: The imaging-based algorithm confirmed WR involvement only in four of the 14 patients. Of the remaining 10 patients, four had retropharyngeal lymph node involvement and six an inconspicuous WR. Applying the imaging-based algorithm to 100 consecutive patients with physiological appearance of their WR on clinical assessment, absence of WR involvement could be confirmed in 99. However, suspicion of WR involvement was raised in one patient. CONCLUSIONS: The imaging-based algorithm was feasible and easily applicable at initial staging of young patients with Hodgkin lymphoma. It increased the accuracy of WR staging, which may contribute to a more individualized treatment in the future.

General advice in ultrasound based elastography of pediatric patients.

Ultrasound elastography including transient elastography (TE), point shear wave elastography, (pSWE) and two (three)- dimensional shear wave elastography (2D-SWE) have been introduced mainly for the evaluation of the liver. All the techniques are also feasible for the examination of spleen, whereas pSWE and 2D-SWE can be used for the assessment of the pancreas, kidney, gastrointestinal tract and other organs. Strain elastography also plays a role for non-liver applications. The aim of the current report is to highlight unique features and techniques for the elastographic examinations in children and to report initial results in non-liver applications.

Ultrasound elastography of the lower uterine segment in women with a previous cesarean section: Comparison of in-/ex-vivo elastography versus tensile-stress-strain-rupture analysis.

OBJECTIVES: The purpose of this study was to assess, if the biomechanical properties of the lower uterine segment (LUS) in women with a previous cesarean section (CS) can be determined by ultrasound (US) elastography. The first aim was to establish an ex-vivo LUS tensile-stress-strain-rupture(break point) analysis with the possibility of simultaneously using US elastography. The second aim was to investigate the relationship between measurement results of LUS stiffness using US elastography in-/ex-vivo with results of tensile-stress-strain-rupture analysis, and to compare different US elastography LUS-stiffness-measurement methods ex-vivo. STUDY DESIGN: An explorative experimental, in-/ex-vivo US study of women with previous CS was conducted. LUS elasticity was measured by point Shear Wave Elastography (pSWE) and bidimensional Shear-Wave-Elastography (2D-SWE) first in-vivo during preoperative examination within 24 h before repeat CS (including resection of the thinnest part of the LUS = uterine scar area during CS), second within 1 h after operation during the ex-vivo experiment, followed by tensile-stress-strain-rupture analysis. Pearson's correlation coefficient and scatter plots, Bland-Altman plots and paired T-tests, were used. RESULTS: Thirty three women were included in the study; elastography measurements n = 1412. The feasibility of ex-vivo assessment of LUS by quantitative US elastography using pSWE and 2D-SWE to detect stiffness of LUS was demonstrated. The strongest correlation with tensile-stress-strain analysis was found in the US elastography examination carried out with 2D-SWE (0.78, p < 0.001, 95%CI [0.48, 0.92]). The laboratory experiment illustrated that, the break point - as a surrogate marker for the risk of rupture of the LUS after CS - is linearly dependent on the thickness of the LUS in the scar area (Coefficient of correlation: 0.79, p < 0.001, 95%CI [0.55, 0.91]). Two extremely stiff LUS-specimens (outlier or extreme values) rupture even at less stroke/strain than would be expected by their thickness. CONCLUSION: This study confirms that US elastography can help in determining viscoelastic properties of the LUS in women with a previous CS. The data from our small series are promising. However whether individual extreme values of high stiffness and consecutive restricted biomechanical resilience can explain the phenomenon of rupture during TOLAC in cases of LUS with adequate thickness remains a question which prospective trials have to analyze before US elastography can be introduced into clinical practice.

Intra- and inter-observer variation and accuracy using different shear wave elastography methods to assess circumscribed objects - a phantom study.

AIMS: The elastic properties of circumscribed tissues (e.g., tendons, lymph nodes, prostates, brain tumors) are of considerable clinical interest. The purpose of this study was thus to compare the Intra-/Inter-observer variation and accuracy in vitro of point shear wave elastography (pSWE) with that of 2D-SWE and to assess 2D-SWE's precision with variable ROI (vROI) incircumscribed objects. MATERIAL AND METHODS: Round targets (Elasticity QA Phantom Model 049) were examined for varying degrees of stiffness (8, 14, 45, and 80 kPa) and diameters (20/10 mm). Three ultrasound systems and four probes were applied (pSWE: Acuson/S3000 9L4/4C1 and Epiq7 C51, 2D-SWE: Aplio/500 PVT375BT). Three different ROIs were used, namely fixed ROI (fROI) and variable ROI: rectangular-best-fitted ROI, and round-best-fitted ROI. Each measurement was performed twice by four observers. RESULTS: A total of 3,604 measurements were conducted. The intra-observer variation of 2D-SWE measurements indicated better agreement (Intraclass Correlation Coefficient (ICC) = 0.971; 95% CI=[0.945; 0.985]), than for the pSWE measurements (ICC = 0.872; 95% CI=[0.794; 0.92]). With both methods, the shear wave elastography applied showed low inter-observer variation: ICC = 0.980; 95% CI=[0.970; 0.987]. However, a significant difference was observed between fROI (pSWE) and vROI (2D-SWE) on circumscribed objects in terms of accuracy. The lowest degree of observationerror was detected in situations where the ROI was not "best fitted", but placed within the target of 3mm from the border (target diameter: 20mm; mean relative error = 0.15). CONCLUSIONS: When estimating the elastic properties of circumscribed tissues, the different measurement techniques performed by commercial shear wave elastography systems reveal a strong susceptibility for observational errors, depending upon the fixed vs. variable ROI of the pSWE vs. 2D-SWE technique.

Patella Dislocation in Children and Adolescents.

Introduction Patellar dislocation is one of the commonest knee injuries in adolescents. Although treatment usually leads to good results, the influence of anatomical and functional factors on therapeutic strategy has been underestimated, especially in cases of recurrence. Patients and Methods The course of treatment has been analysed in 88 patients with 136 patellar dislocations. The importance of anatomical conditions was studied using X-ray and MRI findings. The treatment results were critically evaluated in comparison with current recommendations. Results From 2000 to 2015, 109 patellar dislocations occurred in 88 patients; a further 27 previous dislocations were reported by the patients (mean age 14 years, 47 boys and 41 girls). About one-third of patients (35.2 %) suffered one or more recurrences. Almost half (48.6 %) of the dislocations occurred during physical exercise, particularly ball sports. Osteochondral flake fracture was found in 9 % of the patients, and a lesion of the medial patellofemoral ligament in 96 %. There was an anatomical predisposition to patellar dislocation in almost all cases. The sulcus angle, patellar and trochlear dysplasia, and patellar height were highly significantly different between the patient group and controls. The TT-TG distance was subsequently calculated, but had no impact on therapy. Seventy-seven patients were treated conservatively and 32 patients surgically. The conservative procedure included partial immobilisation for six weeks. Surgical reconstruction or tightening was performed in 27 cases; in five, in combination with other surgical procedures. Plasty of the medial patellofemoral ligament with a tendon graft was performed in five patients, and osteochondral or meniscal lesions were repaired in 10 patients. Recurrences occurred in 41.7 % of conservatively treated knees and in 29.6 % of surgically treated knees (without reconstruction with a tendon graft). No recurrence was seen after reconstruction of the medial patellofemoral ligament with a tendon graft. Fifty-four patients underwent a follow-up examination. Fourteen of these (25.9 %) had suffered a recurrence. The outcome 16 months after the end of treatment was mostly good, as were the results of self-assessment (Larson-Lauridsen Score). Conclusion An anatomical predisposition is detectable in almost all cases of patellar dislocation, but frequently occurs with an accident event, e.g. in ball sports. Primary patellar dislocations without serious concomitant injuries may be treated conservatively. In the event of recurrence, the indication for surgery is given, even in young patients and in any patient with an osteochondral flake fracture. Tightening reconstruction of the MPFL used to be frequently performed, but is associated with a high rate of recurrence.

Safety of gadobutrol in more than 1,000 pediatric patients: subanalysis of the GARDIAN study, a global multicenter prospective non-interventional study.

BACKGROUND: Gadobutrol is a gadolinium-based contrast agent, uniquely formulated at 1.0 mmol/ml. Although there is extensive safety evidence on the use of gadobutrol in adults, few studies have addressed the safety and tolerability of gadobutrol in pediatric patients. OBJECTIVE: This subanalysis of data from the GARDIAN study evaluated the safety and use of gadobutrol in pediatric patients (age <18 years). MATERIALS AND METHODS: The GARDIAN study was a large phase IV non-interventional prospective multicenter post-authorization safety study performed in Europe, Asia, North America and Africa. A total of 23,708 patients were included who were scheduled to undergo cranial or spinal MRI, liver or kidney MRI, or MR angiography with gadobutrol enhancement. The primary study endpoint was the overall incidence of adverse drug reactions (ADRs) and serious adverse events (SAEs) following gadobutrol administration. RESULTS: The GARDIAN study included 1,142 children (age <18 years) who received gadobutrol at a mean dose of 0.13 (range 0.04-0.50) mmol/kg body weight. Gadobutrol was well tolerated in these children, with low rates of ADRs (0.5%) and no SAEs, consistent with results in adults enrolled in the GARDIAN study. Rates of adverse events and ADRs were unrelated to pediatric age or gadobutrol weight-adjusted dose. There were no symptoms suggestive of nephrogenic systemic fibrosis. Investigators rated the contrast quality of gadobutrol-enhanced images as good or excellent in 97.8% of pediatric patients, similar to the main study population. CONCLUSION: Gadobutrol is very well tolerated and provides excellent contrast quality at the recommended weight-adjusted dose in children (age <18 years), similar to the profile in adults.

Headache as the leading symptom in a case of cerebral tuberculosis.

The advent of new and acute headaches poses a diagnostic challenge. The differential diagnosis comprises numerous diseases and syndromes, the prevalence of which varies depending on the geographical region. Due to increased magnitudes in international migration, the usual differential diagnostic spectrum has to be enlarged in individual cases. The presented case illustrates this dilemma and shows that, for example, tuberculosis deserves to be taken into consideration.

Pharmacokinetics and Safety of Macrocyclic Gadobutrol in Children Aged Younger Than 2 Years Including Term Newborns in Comparison to Older Populations.

OBJECTIVES: This clinical study evaluated the pharmacokinetics (PK) and safety data of macrocyclic extracellular contrast agent gadobutrol in pediatric subjects aged younger than 2 years. MATERIALS AND METHODS: Pediatric subjects (term newborns to those aged younger than 2 years) with normal renal function undergoing magnetic resonance imaging with gadobutrol (0.1 mmol/kg body weight [BW]) were prospectively enrolled in this open-label, multicenter clinical trial to evaluate PK as a primary end point. Plasma PK was analyzed using a population-based PK approach. Safety and qualitative efficacy (evaluation of images) were secondary end points. Safety and tolerability were assessed throughout study participation (approximately 7 days). Imaging efficacy variables were assessed by investigators. RESULTS: Forty-four subjects were evaluated for safety and efficacy; 43 subjects were eligible for PK evaluation including 9 term newborns and infants aged younger than 2 months. Gadobutrol PK in pediatric subjects aged younger than 2 years were adequately described by a linear 2-compartmental model with elimination from the central compartment. Total median systemic exposure (area under the curve) of gadobutrol was estimated at 776 µmol · h/L (range, 544-1470 µmol · h/L). Simulated median concentration at 20 minutes after injection of gadobutrol (C20) was 339 µmol/L (range, 230-456 µmol/L). Safety and tolerability profile were similar to older populations. In 1 subject (2.3%), vomiting was reported as a mild adverse event related to gadobutrol, and there were no reported serious adverse events. The evaluation of gadobutrol-enhanced images provided improved diagnosis, increased confidence in diagnosis, and contributed to subject clinical management. CONCLUSIONS: The PK profile of gadobutrol in children aged younger than 2 years including newborns is similar to that in older children and adults. At the dose of 0.1 mmol/kg BW, gadobutrol had a favorable safety profile and was well tolerated with similar profile across the age range 0 to younger than 2 years and compared with older children and adults. Extrapolation of efficacy data from adults to the younger pediatric population, including term newborns, is justified. The recommended standard dose of gadobutrol (0.1 mmol/kg BW), as used in the population aged 2 years and older, is also appropriate in children aged younger than 2 years.

Inflammatory pseudotumor (IPT) and inflammatory myofibroblastic tumor (IMT): a representative literature review occasioned by a rare IMT of the transverse colon in a 9-year-old child.

AIMS AND BACKGROUND: Inflammatory pseudotumor (IPT) and inflammatory myofibroblastic tumor (IMT) represent different entities. However, it is only in recent years that this has been taken into increasing consideration. Some authors still use both terms synonymously or interchangeably. Inflammatory myofibroblastic tumor is a real neoplasm because of the proliferation of myofibroblastic cells. Inflammatory pseudotumor is a more inflammatory reactive or regenerative entity and shows an overlapping with immunoglobulin G4-related disease. METHODS AND STUDY DESIGN: To analyze the current situation, 443 publications from the last 5 years (2009 to February 2014) were included. Reports involved 938 patients and 956 organ sites. The age distribution is twin peaked with one maximum in childhood and the other between 50 and 60 years of age. This distribution is questionable due to the more frequent occurrence of IPT in the liver and of IMT in the lung. Inflammatory pseudotumors mainly occur in older patients; IMTs in children and young adults. RESULTS AND CONCLUSIONS: The liver and biliary tract were the most commonly affected of all body regions, at 32%. This was followed by the lung, including the respiratory tract, at 27%, and by the gastrointestinal tract, at 10%. Lesions of the large bowel, as in the present case of a 9-year-old boy, are very rare. There were organ-related as well as nonspecific clinical symptoms, such as fever, weight loss, and fatigue. Laboratory test results revealed anemia and elevated inflammation-dependent parameters. The patterns in medical imaging are variable and nonspecific. Morphology often suggests a malignant process. For this reason, therapy in most cases is surgical, but this is required more often in IMTs. Many IPTs could be treated conservatively.

Ovarian borderline tumors in pre-menarche girls.

By reason of a new case of an ovarian mucinous borderline tumor (BOT) in a pre-menarche girl, a research of current literature was implemented. Low-grade malignant epithelial tumors are extremely rare in young children and, as far as we know, only a few case reports exist. The patients presented with vomiting, pain, and a swollen lower abdomen. Pre-operative diagnosis primarily consists of imaging techniques. At Stage Ia, the tumor is confined to the ovary without penetration of the capsule, no malignant ascites or peritoneal implants. Treatment consists of removal of the tumor combined with concurrent salpingo oophorectomy, appendectomy, omentectomy, and peritoneal lavage. Although the treatment recommendations are not uniform, basically, preservation of fertility is the main objective. The prognosis is very good, but recurrence is possible even after 10 years.

SOX10 mutation with peripheral amyelination and developmental disturbance of axons.

In this study we describe a case of a term infant with the neurological variant of Waardenburg syndrome type 4 (i.e., PCWH = peripheral demyelinating neuropathy, central dysmyelinating leukodystrophy, Waardenburg syndrome, and Hirschsprung disease, as defined in OMIM #609136) due to a novel heterozygous base exchange (c.671C>G) in exon 4 of SOX10. Magnetic resonance imaging suggested central myelin deficiency with cerebral and cerebellar hypoplasia. Hirschsprung disease was confirmed by rectal biopsy. Sural nerve biopsy revealed hypoplasia due to amyelination (with the exception of a single, small myelinated fiber) and severe reduction in the number of axons.

Self-localization of polyacrylic acid molecules on polar ZnO(0001)-Zn surfaces.

The adsorption of single polyacrylic acid (PAAc) molecules was investigated on stepped hydroxide-stabilized polar ZnO(0001)-Zn surfaces using atomic force microscope (AFM) topography and force distance spectroscopy. Stepped surfaces of ZnO(0001)-Zn were prepared by a wet chemical etching procedure and PAAc molecules were adsorbed from aqueous NaClO(4) solutions. AFM single molecule topography studies could be utilized to show that polyacrylic acid molecules specifically adsorb on the non-polar (10-10) step edge faces at low ionic strengths. The radius of gyration of the dissolved PAAc in aqueous solution was measured by means of static light scattering experiments yielding a radius of gyration of R(g)=136 nm at pH 7.4 in 50 mM NaClO(4)/NaOH solution, which is in good agreement with the size of the adsorbed PAAc molecules as measured using AFM. The obtained results could be rationalized in terms of binding-site configurations at step edges and the effect of the chemical environment on both local electric double layer charge and molecular conformation of the PAAc molecules. The point of zero charge of the ZnO(10-10) surface was measured with chemical force microscopy to be pH(PZC)=10.2 ± 0.2. The specific adsorption of polyacrylic acid at non-polar ZnO step-edges can be explained by coordinative bonds formed between the carboxylic acid group and the Zn-surface atoms. On the hydroxide stabilized polar surface only weak hydrogen bonds can be formed in addition to van-der-Waals forces. Thus a "diffusion and trapping" mechanism keeps the adsorbed PAAc molecules mobile on the ZnO(0001)-Zn surface terraces due to small interaction forces until they are trapped at the (10-10) step faces by stronger coordinative bonds from the carboxylic groups to zinc atoms located in the first atomic layer of the crystal structure.

Is neck flexion during a cerebrospinal fluid puncture a potentially hazardous maneuver?

INTRODUCTION: Cerebrospinal fluid puncture (CSFP) is a diagnostically meaningful procedure. We describe an acute tetraplegia in a patient as complication after CSFP. CASE HISTORIES: Cervical myelopathy due to posterior os odontoideum subluxation was diagnosed, and an occipitocervical fusion was performed surgically. No significant improvement of the neurological status was observed within the following 3 years. CONCLUSIONS: Neck flexion as performed during CSFP is a potentially hazardous maneuver. When patients show inconstant symptoms of craniocervical pathology or signs of cervical myelopathy, an os odontoideum should be suspected.