Both the planning and operation of complex, multi-energy systems increasingly rely on optimization. This optimization requires the use of mathematical models of the system components. The model most often used to describe thermal storage, and especially in the common mixed-integer linear program (MILP) formulation, is a simple integrator model with a linear loss term. This simple model has multiple inherent drawbacks since it cannot be applied to represent the temperature distribution inside of the storage unit. In this article, we present a novel approach based on multiple layers of variable size but fixed temperature. The model is still linear, but can be used to describe the most relevant physical phenomena: heat losses, axial heat transport, and, at least qualitatively, axial heat conduction. As an additional benefit, this model makes it possible to clearly distinguish between heat available at different temperatures and thus suitable for different applications, e.g., space heating or domestic hot water. This comes at the cost of additional binary decision variables used to model the resulting hybrid linear dynamics, requiring the use of state-of-the-art MILP solvers to solve the resulting optimization problems. The advantages of the more detailed model are demonstrated by validating it against a standard model based on partial differential equations and by showing more realistic results for a simple energy optimization problem.

Moving towards a sustainable heat supply calls for decentralized and smart heating grid solutions. One promising concept is the decentralized feed-in by consumers equipped with their own small production units (prosumers). Prosumers can provide an added value regarding security of supply, emission reduction and economic welfare, but in order to achieve this, in addition to advanced hydraulic control strategies also superordinate control strategies and appropriate market models become crucial. In this article we study methods to find a global optimum for the local energy community or at least an acceptable approximation to it. In contrast to standard centralized control approaches, based either on expert rules or mixed integer linear optimization, we adopt an agent-based, decentralized approach that allows for incorporation of nonlinear phenomena. While studied here in small-scale systems, this approach is particularly attractive for larger systems, since with an increasing number of interacting units, the optimization problem becomes more complex and the computational effort for centralized approaches increases dramatically. The agent-based optimization approach is compared to centralized optimization of the same prosumer-based setting as well as to a purely central setup. The comparison is based on the quality of the optimization solution, the computational effort and the scalability. For the comparison of these three approaches, three different scenarios have been set up and analysed for four seasons. In this analysis, no approach has emerged as clearly superior to the others; thus each of them is justified in certain situations. (c) 2020 Elsevier Ltd. All rights reserved.

The continuous increase of (volatile) renewable energy production and the coupling of different energy sectors such as heating, cooling and electricity have significantly increased the complexity of urban energy systems. Such multi-energy systems (MES) can be operated more efficiently with the aid of optimization-based energy management systems (EMS). However, most existing EMS are tailor-made for one specific system or class of systems, i.e. are not generally applicable. Furthermore, only limited information on the actual savings potential of the usage of an EMS under realistic conditions is available. Therefore, this paper presents a novel modular modeling approach for an EMS for urban MES, which also enables the modeling of complex system configurations. To assess the actual savings potential of the proposed EMS, a comprehensive case study was carried out. In the course of this the influence of different user behavior, changing climatic conditions and forecast errors on the savings potential was analyzed by comparing it with a conventional control strategy. The results showed that using the proposed EMS in conjunction with supplementary system components (thermal energy storage and battery) an annual cost savings potential of between 3 and 6% could be achieved.

GOALS OF WORK:To describe the course of hepatitis C in a cohort of 105 survivors after childhood cancer.PATIENTS AND METHODS:Data on chemo/radiotherapy, clinical status, serial alanine aminotransferase (ALT) evaluation, and virological parameters after the end of treatment were collected for each patient. Liver biopsies, when performed, were centrally evaluated by a pathologist.MAIN RESULTS:All patients were alive at the end of follow-up and did not show hepatic insufficiency. ALT evaluation along the entire follow-up showed a moderate (87%) or a remarkable (13%) cytolytic pattern. Young age at diagnosis, hematopoietic stem cell transplantation, and duration of infection significantly correlate with a worse hepatic activity. Type of tumor and chemo and/or radiotherapy regimens did not influence the pattern of hepatic cytolysis. Liver biopsy, centrally reviewed in 30% of the cohort, showed one case of cirrhosis and mild fibrosis in 71% of the group. Higher degrees of fibrosis did not seem to be related to any exposition to chemo/radiotherapy but correlated significantly with the more remarkable cytolytic course.CONCLUSIONS:The outcome of hepatitis C in our patients is comparable to the one described in European cohorts of adult cancer survivors and perinatally infected subjects. Nevertheless, progression to high degrees of hepatic damage has to be monitored by a careful follow-up.

Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis with encephalitis is a life-threatening condition that requires treatment with immunochemotherapy; refractory patients are eligible for allogeneic hematopoietic stem cell transplantation. We report on an adolescent female who failed to respond to induction immunochemotherapy and was salvaged by allogeneic hematopoietic stem cell transplantation from her human leukocyte antigen-identical, Epstein-Barr virus-seropositive brother leading to rapid clearance of Epstein-Barr virus from blood and cerebrospinal fluid.

Pheochromocytomas are rare tumors of the adrenal gland occurring sporadically or as part of familial cancer syndromes. Here we report on the case of a pheochromocytoma due to the germline missense mutation c.491A>G (Q164R) in exon 3 of the von Hippel–Lindau gene in a girl as young as 2.75 years. Extended analyses of her relatives showed that the mutation occurred de novo in the patient's father who was subsequently diagnosed with bilateral pheochromocytomas and a retinal angioma. To the best of our knowledge, this is the youngest patient presenting with pheochromocytoma so far described in the literature. The same VHL mutation has been reported in a patient who developed a pheochromocytoma at the age of 10 years; therefore, for known VHL Q164R mutation carriers, we suggest screening for pheochromocytoma beginning at 2 years of age. © 2010 Wiley‐Liss, Inc.

A 4¾‐year‐old male with Down syndrome (DS) presented with unsteady gait and fatigue. Neuroimaging revealed a cerebellar mass with concomitant obstructive hydrocephalus and additional metastatic lesions. He was successfully treated and is still in complete remission 5 years from diagnosis. The present case illustrates that, although not yet reported, medulloblastoma can also occur in patients with DS. Pediatr Blood Cancer 2009;53:1150–1151. © 2009 Wiley‐Liss, Inc.

Evaluamos el pronóstico clínico a largo plazo de 33 pacientes tratados para un traumatismo arterial de la extremidad superior en el Regensburg University Medical Center entre 1996 y 2004. Durante el seguimiento, junto a los parámetros clínicos, de cada paciente se obtuvo la puntuación en la escala Disabilities of Arm, Shoulder, and Hand (DASH) al igual que la puntuación del cuestionario Short Form-36 Health Survey (SF-36). Los hallazgos de la evaluación clínica se compararon con los resultados obtenidos en la puntuación de la DASH y en el cuestionario SF-36. El tiempo de seguimiento mediano fue de 42 meses. En el momento del seguimiento se habían reconstruido todas las lesiones arteriales y se mantenía la permeabilidad de los vasos. La prevalencia de lesiones nerviosas y/u ortopédicas concomitantes fue elevada (24/33, 72%). La influencia de una lesión nerviosa fue con diferencia mayor que las de otros factores en el pronóstico funcional a largo plazo. Además, un traumatismo contuso y la necesidad de una fasciotomía inmediata fueron marcadores adicionales de un restablecimiento funcional deficiente. Las puntuaciones obtenidas tanto en la DASH como en el SF-36 se correlacionaron con la evaluación clínica de la gravedad de los déficit funcionales. Un traumatismo vascular de la extremidad superior se asocia casi siempre con lesiones ortopédicas y/o nerviosas concomitantes. La afectación del plexo braquial y de los nervios periféricos de la extremidad superior es una variable predictora de un peor pronóstico funcional a largo plazo.

Brain abscesses in children with leukemia or other malignancies are rare and potentially fatal. We report on four children who developed brain abscesses during treatment for acute lymphoblastic leukemia (ALL). All patients received multimodal broad-spectrum antibiotic therapy and liposomal amphotericin-B in combination with hyperbaric oxygen. First-line antimicrobial treatment was modified when a causative organism was isolated. All four patients survived, with two patients showing complete resolution of neurological and MRI abnormalities and with two patients still having residual lesions. To date, all patients are in remission with three patients still receiving antileukemic therapy. Brain abscesses can be successfully managed by a multimodality approach even in severely immunocompromised cancer patients.

Nous avons évalué les résultats cliniques à long terme de 33 patients soignés pour un traumatisme artériel du membre supérieur au centre médical universitaire de Ratisbonne entre 1996 et 2004. Les paramètres cliniques, le score d'handicap du bras, d'épaule, et de la main (DASH) et le formulaire court d'enquête épidémiologique Form-36 (SF-36) de chaque patient étaient rassemblées au cours du suivi. Les résultats de l'évaluation clinique étaient comparés aux résultats obtenus des scores DASH et du questionnaire SF-36. La durée médiane de suivi était de 42 mois. Toutes les lésions artérielles avaient été reconstruites et étaient perméables au moment du suivi. La prédominance des lésions nerveuses et/ou orthopédiques concomitantes était élevée (24/33, 72%). L'influence des lésions nerveuses avait de loin plus d'impact que les autres facteurs sur les résultats fonctionnels à long terme. En outre, les traumatismes explosifs et le besoin d'aponévrotomies immédiates étaient d'autres marqueurs de mauvais rétablissement fonctionnel. Le score DASH et le SF-36 étaient corrélés à l'évaluation clinique de la sévérité des déficits fonctionnels. Le traumatisme vasculaire du membre supérieur est presque toujours associé à des lésions orthopédiques et/ou nerveuses concomitantes graves. L'atteinte du plexus brachial et des nerfs périphériques du membre supérieur est un facteur prédictif de mauvais résultats fonctionnels à long terme.

BACKGROUND AND PURPOSE:Radiotherapy is an integral part of various therapeutic regimens in pediatric and adult oncology. Endocrine dysfunction, neurologic and psychiatric deficits, secondary malignancies and radiation-induced necrosis are well-known possible late effects of cranial irradiation. However, only sporadic cases of radiation-induced cavernous hemangiomas (RICH) have been reported so far.PATIENTS AND METHODS:Pediatric patients who underwent cranial radiation therapy for malignant diseases between January 1980 and December 2003 were retrospectively analyzed. After the end of therapy they entered a detailed follow-up program.RESULTS:Of 171 patients, eight (three patients with medulloblastoma, three patients with acute lymphoblastic leukemia, and one patient each with ependymoma and craniopharyngioma) developed intracerebral cavernoma 2.9-18.4 years after irradiation representing a cumulative incidence (according to the Kaplan-Meier method) of 2.24%, 3.86%, 4.95%, and 6.74% within 5, 10, 15, and 20 years following radiation therapy, respectively. In patients treated in the first 10 years of life, RICH occurred with shorter latency and significantly more often (p = 0.044) resulting in an even higher cumulative incidence.CONCLUSION:These findings and previously published cases show that cavernous hemangiomas may occur after irradiation of the brain several years after the end of therapy irrespective of the radiation dose and type of malignancy. Particularly children < 10 years of age at the time of irradiation are at higher risk. Since patients with RICH frequently do not show symptoms but hemorrhage is a possible severe complication, imaging of the central nervous system should be performed routinely for longer follow- ups, particularly in patients who were treated as young children.

The aim of this study was to quantify the severity of late effects by a simple numerical score (late effects severity score, LESS) in patients who received radiochemotherapy for medulloblastoma or ependymoma. The LESS was correlated with neurocognitive and quality of life (QoL) outcomes.

We assessed the long-term clinical outcome of 33 patients treated for arterial trauma of the upper extremity at the Regensburg University Medical Center between 1996 and 2004. Along with clinical parameters, the Disabilities of Arm, Shoulder, and Hand (DASH) score and the Short Form-36 Health Survey (SF-36) score of each patient were collected at the time of follow-up. Findings of the clinical assessment were compared to results obtained with the DASH score and the SF-36 questionnaire. The median follow-up time was 42 months. All arterial injuries had been reconstructed and were open at the time of follow-up. The prevalence of concomitant neural and/or orthopedic injuries was high (24/33, 72%). The influence of neural injury was by far greater than the impact of other factors on the long-term functional outcome. Furthermore, blunt trauma and the need for immediate fasciotomy were further markers for deficient functional recovery. Both the DASH and the SF-36 scores correlated with the clinical assessment of the severity of functional deficits. Upper extremity vascular trauma is almost always associated with severe concomitant orthopedic and/or neural injuries. The involvement of the brachial plexus and the peripheral nerves of the upper extremity is a predictor of worse long-term functional outcome.

Background: Focal nodular hyperplasia (FNH) is a benign hepatic lesion of unknown etiology. Although uncommon in children, a cumulative incidence is reported in oncologic patients after ending their therapy. Differential diagnosis to other focal liver lesions especially to metastases is often difficult.Patients and methods: We report on four children (female n=2, male n=2; age at initial diagnosis: 9 months, 20 months, 11.5 and 14 years) with different non-hepatic primary tumors (gastrointestinal stroma-tumor, neuroblastoma (n=2) and nephroblastoma) who developed focal liver lesions 2, 2.5, 3 and 8 years after successful treatment of their primary malignancy, respectively.Results: Diagnosis of focal nodular hyperplasia was established by sonography, computed tomography and magnetic resonance imaging. In addition percutaneous needle biopsy was performed in two patients. Median interval from the end of chemotherapy to the onset of FNH was 3.9 years (range 2-8 years).Conclusion: Diagnosis of FNH has to be included in the differential diagnosis of uncertain liver lesions. Biopsy might be avoided by using special imaging techniques like MRI, CT and ultrasound. A wait and see strategy is recommended, specific treatment is not necessary.

Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is a rare type of cutaneous lymphoma and is classified as a post-thymic lymphoproliferative disorder that belongs to a new subset ofmature, peripheral T-cell neoplasms by the World Health Organization (WHO) [1]. The disease is characterized clinically by an indolent course of recurrent, self-healing, or progressive subcutaneous nodules. These nodules both clinically and histologically resemble panniculitis. Alternatively, a rapidly progressive course can be seen with subcutaneous nodules accompanied by constitutional symptoms and, in some cases, the development of a potentially fatal hemophagocytic syndrome with significant cytopenia [2]. Here we report a teenager with SPTCL whose disease was studied by positron emission tomography (PET) at the time of diagnosis and after completion of the treatment.

Introduction: Opsoclonus-myoclonus-syndrome (OMS) is a rare, idiopathic or paraneoplastic syndrome, which is predominantly characterized by opsoclonus (rapid, multi-directional conjugate eye movements), a movement disorder in terms of a myoclonic ataxia and irritability. In adulthood an association with different types of cancer has been reported, whereas in childhood neuroblastomas (NB) are found frequently.

Hemophagocytic lymphohistiocytosis (HLH) during childhood cancer treatment is a rare adverse event posing major diagnostic and therapeutic challenges. Between 1995 and 2006, 6 children developed HLH while on conventional chemotherapy (n=4) or after allogeneic stem cell transplantation (n=2). Treatment of HLH included dexamethasone and etoposide, 2 children additionally received infliximab or daclizumab. Three children survived, whereas 3 children died 2, 5, and 47 days after diagnosis of HLH. HLH is a severe adverse event of childhood cancer therapy. Early diagnosis and immediate initiation of adequate treatment are mandatory to overcome this severe condition.

Three adolescents with central nervous system (CNS) negative acute myeloid leukaemia (AML) refused cranial irradiation for CNS prophylaxis. Instead, these patients received four doses of 50 mg of intrathecal (IT) liposomal cytarabine on day 1, 15, 43 and 71 of maintenance therapy. Corticosteroids were given to prevent chemical arachnoiditis. All patients developed bilateral papilloedema after the third or fourth dose of liposomal cytarabine, local side effects were observed in two patients. Under prolonged dexamethasone therapy, side effects resolved completely. As reversible neuroophthalmological side effects caused by intrathecal liposomal cytarabine can also be observed under low-dose maintenance therapy in patients with CNS negative AML, it should be used with caution in children and adolescents.