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ZUSAMMENFASSUNGDas pädiatrische Kurzdarmsyndrom als eine Form des chronischen Darmversagens ist eine seltene und schwerwiegende Erkrankung. Durch den Einsatz heimparenteraler Ernährung können Überleben, Wachstum und Entwicklung sichergestellt werden. Jedoch bedeutet die Notwendigkeit eines permanenten zentralvenösen Katheters eine Einschränkung der Lebensqualität und geht mit impliziten Risiken einher wie Katheter-Infektionen, metabolischen Entgleisungen und Hepatopathie. Die orale und enterale Ernährung ist daher von herausragender Bedeutung: erstens zur Förderung der Adaptation des verbliebenen Darmes mit dem primären Behandlungsziel, die parenterale Ernährung reduzieren und schließlich beenden zu können; zweitens, um Risiken und Komplikationen beispielsweise die mit chronischem Darmversagen assoziierte Hepatopathie zu minimieren, und drittens, um die Entwicklung oro-fazialer Fähigkeiten zu fördern zur Vermeidung oraler Aversionen und Essstörungen mit größtmöglicher sozialer Teilhabe und Lebensqualität. Die Betreuung von Kindern mit Kurzdarmsyndrom durch bzw. mit einem spezialisierten Zentrum für Intestinale Rehabilitation verbessert Outcome und Prognose.

Short bowel syndrome (SBS) is a severe, life-threatening condition and one of the leading causes of intestinal failure in children. Here we were interested in changes in muscle layers and especially in the myenteric plexus of the enteric nervous system (ENS) of the small bowel in the context of intestinal adaptation. Twelve rats underwent a massive resection of the small intestine to induce an SBS. Sham laparotomy without small bowel transection was performed in 10 rats. Two weeks after surgery, the remaining jejunum and ileum were harvested and studied. Samples of human small bowel were obtained from patients who underwent resection of small bowel segments due to a medical indication. Morphological changes in the muscle layers and the expression of nestin, a marker for neuronal plasticity, were studied. Following a SBS, muscle tissue increases significantly in both parts of the small bowel, jejunum, and ileum. The leading pathophysiological mechanism of these changes is hypertrophy. Additionally, we observed an increased nestin expression in the myenteric plexus in the remaining bowel of SBS. Our human data also showed that in patients with SBS, the proportion of stem cells in the myenteric plexus have risen by more than two times. Our findings suggest that the ENS is tightly connected with changes in intestinal muscle layers and is critically involved in the intestinal adaptation process to SBS.

Endoskopische Diagnostik und Interventionen bei Kindern erfordern ein hohes Mas an Expertise aus unterschiedlichen Bereichen. Die kleinen Dimensionen, die Vulnerabilitat der Patienten*innen und die Seltenheit der Erkrankungen und Problematiken einerseits und die Notwendigkeit modernster Endoskopietechnik andererseits konnen nur durch ein adaquat aufgestelltes Team gemeistert werden. Wir beschreiben typische Indikationen, personelle und technische Anforderungen und machen Vorschlage zur Prozessorganisation. Die Notwendigkeit zum interdisziplinaren Vorgehen wird an 3 exemplarischen Beispielen geschildert. Kein Fachgebiet allein kann die vielfaltigen Herausforderungen der Kinderendoskopie alleine bewaltigen. Die Organisation sollte deshalb niederschwellige Kooperationen begunstigen. Vor allem im Bereich der Fruhgeborenenversorgung und Kindern mit Darmversagen und Motilitatsstorungen bedarf es weiterer Technikentwicklungen.

Endoscopic diagnostics and interventions in children require a high level of expertise from different fields. The small dimensions, the vulnerability of the patients and the rarity of the diseases and problems as well as the necessity for the most modern endoscopic technology can only be mastered by an adequately constructed team.We describe the typical indications, personnel and technical requirements and make suggestions for process organization. The necessity for an interdisciplinary approach is described using three illustrative examples.No single specialty alone can cope with the manifold challenges of pediatric endoscopy. The organization should therefore favor low-threshold collaborations.Further development of techniques is needed especially in the field of premature infant care and children with intestinal failure and motility disorders.EINFüHRUNG: Endoskopische Diagnostik und Interventionen bei Kindern erfordern ein hohes Maß an Expertise aus unterschiedlichen Bereichen. Die kleinen Dimensionen, die Vulnerabilität der Patienten*innen und die Seltenheit der Erkrankungen und Problematiken einerseits und die Notwendigkeit modernster Endoskopietechnik andererseits können nur durch ein adäquat aufgestelltes Team gemeistert werden.Wir beschreiben typische Indikationen, personelle und technische Anforderungen und machen Vorschläge zur Prozessorganisation. Die Notwendigkeit zum interdisziplinären Vorgehen wird an 3 exemplarischen Beispielen geschildert.Kein Fachgebiet allein kann die vielfältigen Herausforderungen der Kinderendoskopie alleine bewältigen. Die Organisation sollte deshalb niederschwellige Kooperationen begünstigen.Vor allem im Bereich der Frühgeborenenversorgung und Kindern mit Darmversagen und Motilitätsstörungen bedarf es weiterer Technikentwicklungen.

Patient education programs (PEPs) to improve disease management are part of standard and regular treatment in adolescents with diabetes. In Germany, youth with inflammatory bowel disease (IBD) receive individual counseling but not PEPs in group settings. Generic PEPs have been developed in order to improve transition from child-centered to adolescent health services. The aim of the study was to investigate the effects of a transition-oriented PEP on quality of life (QoL) and self-management in young patients with IBD (PEP naive), compared to patients with diabetes (familiar with PEPs). A 2-day transition workshop was oriented at improving psychosocial skills and addressed both generic as well as specific aspects of the condition. A controlled trial on the outcomes of a generic transition-oriented PEP was conducted in 14- to 20-year-old patients with IBD (n = 99) and diabetes (n = 153). Transition competence and QoL were assessed at baseline and 6-month follow-up. Results show that the intervention lead to a significant increase in QoL only in patients with IBD. The PEP significantly improved transition competence in both groups, however to a higher extent in subjects with IBD.Transition-oriented PEPs can have differential effects in different patient groups. However, this needs further longitudinal investigations. What is Known: • To date, evidence has accumulated concerning the effectiveness of patient education programs (PEPs) in pediatric health care for chronic conditions such as type 1 diabetes, asthma, atopic dermatitis, or obesity but is less documented in inflammatory bowel disease (IBD). In particular, PEPs in the transition period have not been investigated in youth with IBD. • The current study focuses on evaluating a PEP for transition preparation and management designed to be generically used across different chronic conditions since many aspects of managing chronic conditions share commonalities across conditions. The 2-day workshop included condition-specific modules adapted to the specific medical needs but was otherwise similar in quality and organization among different conditions. What is New: • The transition-oriented PEP was effective in enhancing self-management and transition management skills in both patients with IBD and diabetes; however, effects were higher in youth with IBD. A significant impact of the intervention on patients' QoL compared to the control group was only identified in youth with IBD. • We recommend that patients with IBD have access to PEP as a standard treatment as well as to a transition program during the course of illness.

“Going Green—Education for Sustainability,” a German-American blended learning project for the EFL and STEM classrooms, asks students to challenge commonly held stereotypes about how both cultures approach sustainable development. Since the pilot project (2014), over 3,000 secondary school students in Germany and the US have enrolled in a shared learning management system (Moodle), worked collaboratively both online and offline, developed green action plans and shared them with the school and wider community as part of a competition.This article outlines the conceptual perspective of Going Green that includes the aspects of (a) teaching ‘publics,’ (b) countering expectations and misconceptions, (c) raising awareness of counter-narratives, and (d) expanding the knowledge base of the target culture (sustainable policies in the US). These components together facilitate learning objectives beyond interactional and communicative competencies by promoting learner agency and community-based actions. Attitudinal data drawn from the last two project cycles (2016–17, 2017–18) reflect a heterogeneous view of learners’ expectations and understandings regarding sustainable policies in the US and Germany. Finally, we investigate how narratives and counter-narratives of sustainable development on both sides of the Atlantic can be exploited in the technology-enhanced foreign language classroom in order to facilitate the aforementioned goals. Narratives and counter-narratives play a critical role in cultural learning and can fundamentally shape how foreign language learners construct and make sense of cultural images (Bruner; Warner). Perhaps one of the most instructive examples in this regard is the discourse on sustainable development, defined as economic, social, and environmental “development that meets the needs of the present without compromising the ability of future generations to meet their own needs” (UNWCED). Climate change communication research has long acknowledged the impact of narrative structure, theoretical and ideological framing, and audience appropriacy to communicate effectively sustainability-related messages and inspire climate issue engagement (Roser-Renouf and Maibach). The approaches to sustainable development in Germany and the United States differ considerably, as do their perceptions on both sides of the Atlantic. In this article, we first introduce the Going Green project along with its pedagogical rationale and a discussion of cultural learning in this setting. In so doing, we focus on the role of mainstream vs. counter-narratives of sustainable development in the U.S. and pedagogic approaches to teaching them in the EFL classroom. In order to contextualize some of the potential challenges of such an approach, we report a questionnaire survey of previous project participants’ perceptions and preconceptions of sustainable development in the U.S. The reported data illustrate that learners are often unaware of non-mainstream publics and the impact of the local and state government-levels to support sustainable development in the U.S. We close by discussing the role of such counter-narratives in the teaching of culture and how they are implemented in the Going Green curriculum.

Summary Organ donor shortage for infant liver transplant recipients has lead to an increase in splitting and living donation. For cases in which even transplantation of the left lateral graft (Couinaud’s segments II + III) results in a ‘‘large for size situation’’ with an estimated graft body weight ratio (GBWR) of >4%, monosegmental liver transplantation was developed. This, however, bears complications because of greater parenchymal surface and suboptimal vascular flow. We exclusively use the left lateral graft from living donors or split grafts. Temporary abdominal closure is attempted in cases of increased pressure. We report of 41 pediatric transplants in 38 children £10 kg. Within this group, there were 23 cases with a GBWR of ‡4, and 15 cases with a GBWR

To evaluate the efficacy of cisapride in improving tolerance of enteral feeding, 59 premature infants were randomized into a blinded placebo-controlled study. Treatment was initiated with the introduction of enteral feeding and continued until 150 ml/kg/day of milk were tolerated. Only in extremely low birth weight (ELBW) infants, was the time to tolerate full enteral feeding shorter in the treatment group, whereas ECG recordings showed a significantly prolonged QTc interval during treatment. Two children developed cardiac rhythm disturbances. In conclusion premature infants may not benefit from routine use of the drug to improve enteral feeding, and seem to be more vulnerable to its side effects.

Male gender predisposes to severe sepsis and septic shock. This effect has been ascribed to higher levels of testosterone. The ESPNIC ARDS database was searched, to determine if there was evidence of a similar male preponderance in severe sepsis in prepubertal patients in spite of low levels of male sex hormones at this age. A total of 72 patients beyond neonatal age up to 8 years of age with sepsis were identified. The male/female (M/F) ratio was 1.7 (1.0;2.7) and differed significantly from non-septic ARDS patients in this age group [n = 209; M/F = 1.0 (0.8;1.3)]. The highest M/F-ratio was observed in the first year of life. The gender-ratio was the same as reported in adult patients with sepsis. In infants between 1 month and 12 months of age, the ratio was 2.8 (1.2;6.1) (Chi2= 5.6; P< 0.01), in children from 1 year to 8 years of age it was 1.2 (0.7;2.2) (n.s.). In a subgroup of patients with severe sepsis or septic shock, caused by other bacteria than Neisseria meningitidis, the M/F-ratio was 2.1 (1.2;3.6) (Chi2= 4.9; P

Objective The genetic basis of inflammatory bowel disease (IBD) is incompletely understood. The aim of this study was to identify rare genetic variants involved in the pathogenesis of IBD. Design Exome sequencing and immunological profiling were performed in a patient with early onset Crohn9s disease (CD). The coding region of the gene encoding X-linked inhibitor of apoptosis protein ( XIAP ) was sequenced in samples of 275 paediatric IBD and 1047 adult-onset CD patients. XIAP genotyping was performed in samples of 2680 IBD patients and 2864 healthy controls. Functional effects of the variants identified were investigated in primary cells and cultured cell lines. Results Our results demonstrate the frequent occurrence of private variants in XIAP in about four percent of male patients with paediatric-onset CD. While XIAP mutations are known to be associated with the primary immunodeficiency (PID) X-linked lymphoproliferative disease type 2 (XLP2), CD patients described here exhibited intestinal inflammation in the absence of XLP2 and harboured a spectrum of mutations partially distinct from that observed in XLP2. The majority of XIAP variants identified was associated with a selective defect in NOD1/2 signalling, impaired NOD1/2-mediated activation of NF-κB, and altered NF-κB-dependent cytokine production. Conclusions This study reveals the unanticipated, frequent occurrence of XIAP variants in male paediatric-onset CD. The link between XIAP and NOD1/2, and the association of XIAP variants with XLP2, support the concept of PID in a subset of IBD patients. Moreover, these studies provide a rationale for the implementation of XIAP sequencing in clinical diagnostics in male patients with severe CD.

The objective of this study was to prove the superiority of doxapram compared to theophylline therapy in apneas of prematurity in very low birth weight infants. Therefore all VLBW infants (gestational age < 35 weeks) were randomized if they had in a 2 hours-interval more than 2 apneas, 4 bradycardias or 4 oxygen desaturations. They received either theophylline (loading dose 5 mg/kg b. w., 3 mg/kg b. w. bid) or doxapram by continuous infusion of 0.5 mg/kg/h. Apneas, bradycardias and desaturations were recorded from the trend analysis of our monitoring system over the first 3-days and a 7 days period and compared statistically (Mann-Whitney U-test). Plasma levels of both drugs and a polysomnographic recording were obtained during steady state conditions in parallel to a behavioral observation according to Prechtl. The recorded events were again compared using the Mann-Whitney U-test. Twenty patients were treated with theophylline, 14 with doxapram. In 9 patients of each group we could perform a polysomnography and behavioral observation. The incidence of apneas, bradycardias and desaturations in a 7 days-interval was not significantly different between both groups. Analyzing the first 3 days of treatment, however, we could detect a significantly lower rate of apneas in the doxapram group (2.5 apneas compared to 7 in the theophylline group, p < 0.037). In the polysomnographic recording and in our behavioral observations we could not record any significant differences between both groups. Therefore we can conclude that theophylline and doxapram are comparable in the treatment of apneas of prematurity, however, doxapram is superior to theophylline in reducing the rate of apneas in the first 3 days of treatment.

A prospective randomized study was performed to evaluate the efficacy of a low dose (5 mg/kg/day bid) vancomycin compared with a low dose (5 mg/kg/day once daily) teicoplanin therapy to prevent CONS sepsis in VLBW-infants. All infants received this therapy after their 4th day of life or after an eventual therapy of early onset sepsis as long as an i.v. line was in place or 1500 g body weight. Twenty-seven infants were treated with vancomycin (birth weight 1103 +/- 286 g, gest. age 28.8 +/- 1.9 weeks), 28 with teicoplanin (birth weight 1133 +/- 226 g, gest. age 29.04 +/- 2.2 weeks). The infants were observed for clinical and laboratory signs of sepsis. On day 4 of therapy and every 3rd day during therapy serum creatinine levels, tracheal aspirates, stool cultures and vancomycin/teicoplanin peak and trough levels were obtained. We could not detect any case of blood culture positive sepsis and 1 case of suspected sepsis (neg. blood cultures) in both groups, as compared with a former CONS sepsis rate of 24% in our institution's VLBW infants without antibiotic prophylaxis. Nine patients in the vancomycin and five in the teicoplanin group had tracheal colonization with CONS. In both groups peak and trough levels of antibiotics were in the bactericidal range. Serum creatinine was not normal in both groups. We conclude that teicoplanin is preventing CONS sepsis as well is vancomycin. The minimal inhibitory concentrations of both antibiotics against grampositive isolates in units using this strategy have to be observed carefully to detect emerging resistance.

Chronic intestinal failure (CIF) in childhood is caused by congenital malformations and inflammatory diseases of the gut. Its reported prevalence is 13.7 per million population. Long-term home parenteral nutrition has dramatically improved the life expectancy and quality of life of children with CIF. The affected children are now treated with parenteral nutrition at home as soon as their medical state and family circumstances allow.The authors present data from a patient registry and review publications retrieved by a selective literature search.Children with CIF can now be expected to survive beyond adolescence, at the very least, and enjoy good quality of life. This goal can only be achieved if nutritional therapy is carried out safely and the affected children's development is closely monitored by an interdisciplinary team that consists of primary care physicians/family doctors, neonatologists, pediatric gastroenterologists, and pediatric surgeons. Moreover, the prevention, early detection, and appropriate treatment of complications such as infection, liver disease, renal dysfunction, and disturbances of bone metabolism is of vital importance. The patients' families must be supported by specially qualified ambulatory nurses and social workers. Treatment with parenteral, enteral, and oral nutrition and surgery enables most infants with CIF to meet all their nutritional needs orally by the time they start going to school. For children who suffer from intractable complications, intestinal transplantation provides a real and increasing chance of survival.

Organ donor shortage for infant liver transplant recipients has lead to an increase in splitting and living donation. For cases in which even transplantation of the left lateral graft (Couinaud's segments II + III) results in a "large for size situation" with an estimated graft body weight ratio (GBWR) of4%, monosegmental liver transplantation was developed. This, however, bears complications because of greater parenchymal surface and suboptimal vascular flow. We exclusively use the left lateral graft from living donors or split grafts. Temporary abdominal closure is attempted in cases of increased pressure. We report of 41 pediatric transplants in 38 children ≤10 kg. Within this group, there were 23 cases with a GBWR of ≥4, and 15 cases with a GBWR4. There was no statistical difference in vascular or biliary complications. Despite a more frequent rate of temporary abdominal closure, we did not find a higher rate of intra-abdominal infections. Overall, patient and graft survival was excellent in both groups (one death, three re-transplants). We noticed, however, that the ventro-dorsal diameter of the graft appears to be more relevant to potential graft necrosis than the actual graft size. In conclusion, the usage of monosegmental grafts seems unnecessary if transplantation of left lateral grafts is performed by an experienced multidisciplinary team, and temporary abdominal closure is favored in cases of increased abdominal pressure.

Long-term total parenteral nutrition (TPN) in children is often complicated by the development of cholestasis, liver fibrosis, and liver failure. High doses of intravenous lipids may be involved in the pathogenesis of hepatobiliary dysfunction. The purpose of this study was to determine whether the use of 2 newly developed lipid emulsions could reduce liver damage.Three groups of prepubescent rabbits received TPN including a lipid emulsion either based on soybean oil, olive oil, or soybean oil with n-3 fatty acids added. Enterally fed animals served as controls. After 21 d the animals were killed. Serum samples were obtained at the beginning and end of the study period. Specimens were processed for histological evaluation using a specific score to assess the severity of liver damage.Biochemical parameters did not predict the extent of liver damage. Hydropic degeneration as an indicator of toxic liver injury was the predominant histological alteration regardless of the type of lipids infused. The extent of fibrosis did not significantly differ among treatment groups except for animals infused with n-3 fatty acids exhibiting increased fibrotic transformation as compared with controls.In our animal model, the use of a lipid emulsion with a reduced amount of polyunsaturated fatty acids was not superior to a lipid emulsion based on soybean oil. Long-term application of n-3 fatty acids was associated with more extensive fibrosis. Therefore, intravenous n-3 fatty acids containing lipid preparations (fish oil) should not be used in patients for long-term TPN.

The chemiluminescence (CL) technique with scavengers for superoxide anion (superoxide dismutase) and hydrogen peroxide (catalase) was used to characterize the generation of reactive oxygen species (ROS) inside and outside the human neutrophil after stimulation with both soluble (formyl-methionyl-leucyl-phenylalanine, FMLP) and particulate (urate crystals, zymosan, oxidized LDL) stimuli. Depending on the stimulus used, ROS generation differed in composition and absolute amounts. The ratio between extracellularly and intracellularly produced ROS ranged from 0.3 (zymosan) to 4.2 (FMLP). While enhancing substantially FMLP-stimulated CL, horseradish peroxidase inhibited CL induced by particulate stimuli by 40-80%. Furthermore, an azide-insensitive and therefore peroxidase-independent part of CL was found in FMLP-, LDL- and zymosan-stimulated cells. The results indicate that different agonists may lead through distinct chemical pathways to neutrophil luminol-amplified light generation.

Neonatal asphyxia is a major topic of neonatal research. However, no clear-cut physiologic parameters exist which enable an early identification of neonatal infants who are either at risk to develop brain damage or posthypoxic heart failure. Parameters indicating dysfunction of the heart and kidneys as creatinine and creatinine kinase have been evaluated. In our study, 47 asphyxiated infants (umbilical artery pH

American studies journal; Vol. 54, No. 06, 2010. 2199-7268, Special Edition: Urban Culture, Urban Landscapes: Growing Up in the American City

American studies journal; Vol. 62, No. 07, 2017. 2199-7268, Special Edition: Miscellaneous Articles