bluuebunny/crossref_metadata_2025_split huggingface dataset:
Real-time monitoring system for evaluating the acid-producing activity of oral squamous cell carcinoma cells at different environmental pH
Authors: Morishima Hiromitsu , Washio Jumpei , Kitamura Jun , Shinohara Yuta , Takahashi Tetsu , Takahashi Nobuhiro 
Published: August 2017

AbstractThis study aimed to establish a real-time monitoring system for evaluating the acid-producing activity of cells and the effects of microenvironmental pH on their metabolism. Oral squamous cell carcinoma (HSC-2, HSC-3) and normal (HaCaT) cells were used. Their acid-producing activity from glucose, glutamine, and glutamate was monitored at various pH values using a pH stat system. Their production of lactic acid and ammonia was also measured. The acid-producing activity was monitored successfully. Both the cancer and normal cells produced acids from glucose, glutamine, and glutamate. All of the cells decreased their acid-producing activity as the environmental pH fell, but in glucose-derived acid-producing activity the cancer cells were more acid-tolerant than HaCaT cells. In the cancer cells, the proportion of lactic acid among all acids produced from glucose at the acidic environment tended to be higher than that in HaCaT cells. All of the cells produced ammonia from glutamine, while only HaCaT cells produced ammonia from glutamate. We established a real-time monitoring system for evaluating the acid-producing activity of cells. Our results suggest that the cancer cells possess acid-tolerant glucose metabolism with a tendency of metabolic shift to lactic acid production at acidic pH and they metabolise glutamate without ammonia production.
X-linked Dilated Cardiomyopathy with Mutation in the 5′ Splice Site Intron 1 of Dystrophin Gene with Utrophin Upregulation
Authors: Chan Sophelia Hoi-shan , Ho Ronnie Siu-lun , Lo Ivan Fai-man , Kan Amanda Nim-chi , Lun Kin-Shing 
Published: August 2017

AbstractWe report a teenage boy presented with dilated cardiomyopathy (DCM) with no initial skeletal involvement and initial normal creatine kinases. One year after the heart transplantation, he had exercise-induced transient muscle weakness with elevated creatine kinases (CKs). Muscle biopsy showed normal structures and normal dystrophin immunohistochemical labeling, but utrophin, which is an autosomal homologue of dystrophin, was overexpressed at sarcolemma. Sanger sequencing confirmed a heterozygous c.31 + 1G > A, 5′ splice site point mutation at the first intron of dystrophin gene. A review of previous reports of patients with different point mutations in the same region, the first exon–intron boundary that involved the muscle promotor to exon 1, confirmed a high correlation of cardiospecific phenotype sparing the muscles with this specific site of mutations. The confirmation of upregulation of brain and Purkinje isoforms of dystrophin protein in the skeletal muscles but not in the heart in past studies help to explain the skeletal sparing presentation. X-linked DCM (XLDCM) is an important cause of isolated cardiomyopathy. Routine immunohistochemical staining study including dystrophin in cardiac muscle biopsy, and dystrophin and utrophin labelling on skeletal muscle biopsy for patients with subsequent muscle symptoms or raised creatine kinases, help in the early diagnosis of the XLDCM. Future experimental study to determine the aberrant pre-mRNA splicing of this specific splice site mutation involving exon 1 and intron 1 will help to understand better the underlying complex mechanism of the splicing regulation.
Sleep and Epilepsy
Authors: Bazil Carl 
Published: August 2017

AbstractThe neurophysiology of the brain is complicated and nuanced. It is responsible for the normal sleep/wake states that every person experiences, and for the changes in brain neurophysiology that result in epileptic seizures and in disorders of sleep. It is therefore not surprising that sleep, sleep disorders, and epilepsy interact on many levels. The sleep state influences the detection of interictal epileptiform discharges, important for diagnosis of epilepsy. The state of sleep also influences whether a seizure will occur at a given time, and this differs considerably for various epilepsy syndromes. Sleep disruption of any kind, including from sleep disorders, can worsen epileptic seizures and contribute to intractability. Finally, anticonvulsant medications can influence sleep and sleep disorders in both positive and negative ways. Understanding this interplay between epilepsy and sleep is helpful in the optimal treatment of all patients with epileptic seizures.
Pediatric Intestinal Rehabilitation and Transplantation Registry: Initial Report from a European Collaborative Registry
Authors: Boscarelli Alessandro , Hermans Dominique , Dall'Oglio Luigi , Diamanti Antonella , d'Aische Aloys , Pakarinen Mikko , Reding Raymond , Morini Francesco , Bagolan Pietro , Fusaro Fabio , Totonelli Giorgia , Tambucci Roberto 
Published: August 2017

Introduction Short bowel syndrome (SBS) is the main cause of intestinal failure (IF) in the pediatric population. To promote the standardization of care of these patients, the registry of Pediatric Intestinal Rehabilitation and Transplantation (PIRAT) has been established. The aim of this study is to describe patients with IF using PIRAT database.
Materials and Methods Data from two tertiary care European referral Centers registered in PIRAT (https://www.studeon.eu/pirat) were analyzed (1994–2015). Neonatal SBS-related IF was defined as need for parenteral nutrition (PN) to sustain life and growth for more than 75 days, after extensive bowel resection during neonatal period. Data included patient demographics, disease at birth, residual small intestine, and intestinal autonomy (PN on/off).
Results In this study, 114 children with SBS-related IF were identified (male 60%). Median gestational age was 35.3 weeks (interquartile range [IQR]: 33.0–38.0); median birth weight was 2,440 g (IQR: 1,700–2,990). The main causes of SBS were intestinal atresia in 31 (27%), midgut volvulus in 29 (25%), necrotizing enterocolitis in 23 (20%), and gastroschisis in 12 (11%). Nine (7.9%) patients died on PN (six sepsis, two IF-associated liver disease, and one multiorgan failure). Median residual small bowel length was 46 cm (IQR: 13.0–92.5). Ileocecal valve was resected in 48 patients (42%). Intestinal autonomy was achieved in 68% patients.
Conclusion We present the web-based registry PIRAT and the first results of patients with IF registered from two European Centers. PIRAT could give the opportunity to create a dedicated international network (IF-net) to standardize, improve, and spread the therapeutic paths for the rare and heterogeneous condition of SBS-related IF.
Postnatal Germ Cell Development in the Cryptorchid Testis: The Key to Explain Why Early Surgery Decreases the Risk of Malignancy
Authors: Clasen-Linde Erik , Li Ruili , Reinhardt Susanne , Kvist Kolja , Vikraman Jaya , Southwell Bridget , Hutson John , Cortes Dina , Thorup Jorgen 
Published: August 2017

Purpose Cryptorchidism is a risk factor for testicular malignancy and surgical treatment lowers this risk. This study aimed to investigate the germ cell behavior in prepubertal cryptorchid testes using immunohistochemical markers for germ cell malignancy to understand how early orchiopexy may possibly prevent cancer developing.
Materials and Methods Histology sections from 1,521 consecutive testicular biopsies from 1,134 boys aged 1 month to 16.5 years operated for cryptorchidism were incubated with antibodies including antiplacental-like alkaline phosphatase (PLAP), anti-Oct3/4, anti-C-kit, and anti-D2–40.
Results Oct3/4 and D2–40-positive germ cells are found throughout the first 2 years of life, with declining frequency thereafter. After 2 years, they should have disappeared and may indicate neoplasia. PLAP-positive cells were seen in 57 to 82% and C-kit-positive cells in 5 to 21% of cryptorchid testes between 4 and 13 years. Not until puberty did PLAP and C-kit-positive undifferentiated spermatogonial stem cells vanish. Only 0.3% of the present material had obvious prepubertal intratubular germ cell neoplasia (ITGCN) and they all had syndromic cryptorchidism. An additional three boys (0.3%) older than 2 years had weak Oct3/4 expression in undescended testes, but all cases were D2–40 negative.
Conclusion Prepubertal ITGCN was rare and mostly seen in syndromic cryptorchidism. In nonsyndromic cryptorchidism PLAP-positive undifferentiated spermatogonial stem cells persisted in a significant proportion of nontreated undescended testes and they will be especially sensitive to long-lasting abnormally high temperature that may be the single most important cause facilitating the accumulation of mutations during cell replication and the development of ITGCN to be prevented by orchiopexy.
Nonspanning Total Wrist Arthrodesis with a Low-Profile Locking Plate
Authors: Rancy Schneider , Ek Eugene , Paul Sophia , Hotchkiss Robert , Wolfe Scott 
Published: August 2017

Purpose This study aims to compare the outcomes and complications of our technique for nonspanning total wrist arthrodesis using a locking plate with the standard carpometacarpal spanning technique.
Methods A retrospective review of charts was performed to identify patients who underwent total wrist arthrodesis by the senior author (S.W.W.). We compared the outcomes of 15 cases of nonspanning wrist fusion with a 2.4/2.7 mm locking T plate to 11 cases of spanning wrist fusion with a 2.7/3.5 mm locking compression plate. Minimum follow-up was 3 months. Indications for fusion included rheumatoid arthritis, posttraumatic arthritis, Kienböck's disease, primary osteoarthritis, juvenile inflammatory arthropathy, psoriasis, brachial plexopathy, failed hemi or total wrist arthroplasty, failed four-corner fusion, and failed proximal row carpectomy. The primary outcome was fusion. Secondary outcomes included time to union, patient-rated wrist evaluation score, numerical rating scale pain score, grip strength, and complications.
Results All the wrists got fused. There were no significant differences in objective and subjective outcomes between cohorts. There were three complications (27%) in the spanning group, including tendon rupture and peri-implant fracture at the third metacarpal. This was compared with three complications (20%) in the nonspanning group, consisting of hardware removal.
Discussion We achieved similar fusion rates employing both spanning and nonspanning total wrist arthrodesis techniques, without necessitating carpometacarpal arthrodesis in the latter. Complications associated with our method were comparably less severe than those reported in the literature. We advocate nonspanning arthrodesis as an alternative method for total wrist fusion with a high union rate and minimal risk of complications at the carpometacarpal joint.
Level of Evidence Therapeutic level IV.
Spectrum of Neurocutaneous Syndromes in Children in Neurology Practice
Authors: Kaur Sukhjot , Kaur Simrat , Goraya Jatinder 
Published: August 2017

AbstractNeurocutaneous syndromes (NCSs) are a well-recognized cause of neurological dysfunction in children. While there is large body of literature describing individual neurocutaneous disorders, composite studies describing the relative frequency of these disorders and their modes of presentation in pediatric neurology practice are sparse. Charts of 87 children, 54 boys and 33 girls, with NCSs were reviewed. Tuberous sclerosis was diagnosed in 47 children, followed by neurofibromatosis type 1 and ataxia-telangiectasia in 10 each. Six children had Sturge-Weber syndrome, five had hypomelanosis of Ito, and three had Sjogren-Larsson syndrome. Sixty children presented with seizures, of which 46 had tuberous sclerosis. Others presented with developmental delay and ataxia/movement disorder. Family history of NCS was noted in 20 (21.6%) children. Thirty-four (39%) children had developmental delay or intellectual disability at the time of presentation. Our study reveals that tuberous sclerosis is the most common NCS in pediatric neurology practice.
Verletzungen der Halswirbelsäule im Kindes- und Jugendalter
Authors: Jarvers J. , Völker A. , von der Höh N. , Josten C. , Heyde C. 
Published: August 2017

ZusammenfassungKommt es bei Säuglingen und Kleinkindern eher zu Verletzungen des zervikothorakalen Übergangs und der oberen HWS, so finden sich mit zunehmendem Alter zumeist Verletzungen der unteren HWS, aufgrund der zu der erwachsenen Wirbelsäule mehr und mehr angeglichenen Anatomie und Biomechanik.Aufgrund des seltenen Auftretens von HWS-Verletzungen im Kindes- und Jugendalter bedarf es einer genauen Kenntnis möglicher Verletzungsformen und der physiologischen Entwicklung der HWS einschließlich möglicher Anomalien, um die Verletzungen nicht zu übersehen und um die adäquate Therapie einzuleiten. Je nach Lokalisation und Verletzungen kommen konservative Therapiemaßnahmen mit Ruhigstellung in Orthesen oder dem Halofixateur in Betracht. Bei entsprechendem Verletzungsmuster können jedoch auch im Kindesalter Osteosynthesen und Fusionen notwendig werden.
Operative Therapie – Mitralklappenrekonstruktion oder Mitralklappenersatz?
Authors: Sündermann Simon , Falk Volkmar 
Published: August 2017

ZusammenfassungDas Auftreten einer Mitralklappeninsuffizienz (MI) basiert auf 2 völlig unterschiedlichen pathologischen Mechanismen: Bei der primären MI kommt es zu degenerativen Veränderungen an der Mitralklappe. Hier ist die Goldstandardtherapie die chirurgische Mitralklappenrekonstruktion mit exzellenten Langzeitergebnissen. Die sekundäre MI ist die Folge einer ischämischen oder dilatativen Kardiomyopathie mit Veränderungen der Ventrikelgeometrie, die zu einer Veränderung der Klappengeometrie führen. Für eine erfolgreiche Rekonstruktion ist es notwendig, dass genügend viables Myokard vorhanden ist, sodass ein reverses Remodeling wahrscheinlich ist. Außerdem müssen verschiedene Faktoren wie der Sphericity-Index und die Symmetrie des Tetherings berücksichtigt werden, um den Erfolg einer Rekonstruktion abzuschätzen. Liegt eine ungünstige Konstellation vor, sollte ein Mitralklappenersatz oder eine alternative Therapie wie z. B. die Mitraclip-Implantation angestrebt werden.
Arthrosonographic Reference Values of the Shoulder Joint in Healthy Children and Adolescents: A Cross-Sectional Multicentre Ultrasound Study
Authors: Trauzeddel Ralf , Windschall Daniel , Trauzeddel Ralf , Nirschl Christine , Ganser Gerd , Palm-Beden Katharina , Berendes Rainer , Haller Maria , Krumrey-Langkammerer Manuela , Nimtz-Talaska Antje , Schoof Philipp , Lehmann Hartwig 
Published: August 2017

Abstract
Background Defining of gray scale ultrasound standard reference values of the shoulder joint in childhood and adolescence during maturation. Patients: We examined 445 healthy girls and boys between 1 year and 18 years of age.
Method A cross-sectional multicentre grey-scale ultrasound study was performed to examine the shoulder joint on both sides. The children were divided according to their gender and were further classified into six age groups, which constituted three-year age ranges, to record anatomical development changes. We measured the capsule-bone distance (BCD) as a representation of the intracapsular cavity, as well as the thickness of the joint capsule and joint cartilage. Values were expressed in mean±standard deviation (SD) and minimum-maximum (min-max). The shape of the joint capsule and capsule-bone junction zone was qualitatively analysed.
Results The joint cartilage thickness decreased with increasing age in all joints independently from sex and body side. However, the BCD and the capsule thickness increased with age. There was no intraarticular fluid visible. The joint capsule had a predominantly concave form, whereas the capsule-bone junction was mostly sharp.
Discussion This study is the first describing age-related normal values of the intracapsular cavity, joint capsule and cartilage thickness as well as their respective shape in a large cohort of healthy children.
Conclusion The findings could be helpful to differentiate between physiological and pathological joint conditions and thereby distinguishing age-related variations from alterations caused by inflammation.
Should Breast Cancer Surgery Be Done in an Outpatient Setting?
Authors: Formago Margaret , Schrauder Michael , Rauh Claudia , Hack Carolin , Jud Sebastian , Hildebrandt Thomas , Schulz-Wendtland Rüdiger , Frentz S. , Graubert S. , Beckmann Matthias , Lux Michael 
Published: August 2017

Abstract
Introduction The care of patients with breast cancer is extremely complex and requires interdisciplinary care in certified facilities. These specialized facilities provide numerous services without being correspondingly remunerated. The question whether breast cancer surgery should be performed in an outpatient setting to reduce costs is increasingly being debated. This study compares inpatient surgical treatment with a model of the same surgery performed on an outpatient basis to examine the potential financial impact.
Material and Methods A theoretical model was developed and the DRG fees for surgical interventions to treat primary breast cancer were calculated. A theoretical 1-day DRG was then calculated to permit comparisons with outpatient procedures. The costs of outpatient surgery were calculated based on the remuneration rates of the AOP (Outpatient Surgery) Contract and the EBM (Uniform Assessment Scale) and compared to the costs of the 1-day DRG.
Results The DRG fee for both breast-conserving surgery and mastectomy is higher than the fee paid in the context of the EBM system, although the same procedures were carried out in both systems. If a hospital were to carry out breast-conserving surgery as an outpatient procedure, the fee would be € 1313.81; depending on the type of surgery, the hospital would therefore only receive between 39.20% and 52.82% of the DRG fee. This was the case even for a 1-day treatment. Compared to the real DRG fees the difference would be even more striking.
Conclusion Carrying out breast cancer surgery as an outpatient procedure would result in a significant shortfall of revenues. Additional services from certified centers, such as the interdisciplinary planning of treatment, psycho-oncological and social-medical care with the involvement of relatives, detailed documentation, etc., which are currently provided without surcharge or adequate remuneration, could no longer be maintained. The quality of processes and excellent results which have been achieved and ultimately the care given by certified facilities would be significantly at risk.
Interventionell-radiologische Behandlung von Interkostalarterienblutungen – eine retrospektive Analyse
Authors: Kupczyk Patrick , Meyer Carsten , Thomas Daniel , Schild Hans , Pieper Claus 
Published: August 2017

Zusammenfassung
Hintergrund Interkostalarterienblutungen sind potenziell lebensbedrohliche Verletzungen, die neben einer konservativen und chirurgischen Therapie auch einer interventionell-radiologischen Notfallmaßnahme zugeführt werden können. Wir berichten über unsere Erfahrungen mit der notfallmäßigen Interkostalarterienembolisation.
Material und Methoden Retrospektiv wurden alle Patienten identifiziert, die in einem Zeitraum von 7 Jahren aufgrund einer akuten arteriellen Blutung mittels Interkostalarterienembolisation behandelt wurden. Neben technischem und klinischem Erfolg wurden weitere klinische und prozedurale Parameter sowie die Überlebenszeit der Patienten erfasst.
Ergebnisse Zwischen 2010 und 2017 wurden 27 Embolisationsprozeduren bei 24 Patienten (14 Männer, mittleres Alter 65,7 ± 13,9 Jahre) durchgeführt. Die Mehrzahl der Patienten litt an iatrogenen Interkostalarterienblutungen (n = 17; 70,1%; vorwiegend nach Thorakozentese). Bei 5 Patienten war zuvor ein frustraner Versuch einer thorakoskopischen Blutstillung erfolgt. 25/27 Interventionen waren primär technisch erfolgreich. In 2 Fällen kam es zu Nachblutungen über Kollateralen, sodass eine Reintervention notwendig war (sekundärer technischer Erfolg). In 15 Fällen war eine sekundäre chirurgische Hämatomausräumung erforderlich. Die Interkostalarterienembolisation war bei 23/24 Patienten klinisch erfolgreich, eine Patientin verstarb trotz erfolgreicher Embolisation an den Folgen des ausgedehnten Hämatothorax. Eine spinale Ischämie trat als Majorkomplikation auf.
Schlussfolgerung Die Interkostalarterienembolisation ist eine effektive interventionell-radiologische Notfallmaßnahme bei Patienten mit akuten Blutungen und stellt eine Alternative zur chirurgischen Therapie, auch nach erfolgloser chirurgischer Blutstillung, dar. Angesichts der potenziell schweren Komplikationsmöglichkeiten sollte der Eingriff durch einen erfahrenen Interventionalisten durchgeführt werden.
Indikationsfremde Anwendung von Medikamenten und Medizinprodukten in der Notfallmedizin
Authors: Ittner Karl , Koppenberg Joachim , Walter Ute 
Published: August 2017

ZusammenfassungWenn zugelassene Arzneimittel außerhalb der in der entsprechenden Fachinformation dargelegten Beschreibungen angewendet werden, dann spricht man von einer nicht zulassungskonformen Anwendung oder von einem Off-Label-Use. Wie in fast allen medizinischen Fachgebieten gibt es auch im Rettungsdienst sogenannte Off-Label-Use-Pharmakotherapien. Sofern evidenzbasierte Informationen zu einer nicht zulassungskonformen Anwendung vorliegen, und insbesondere im konkreten Notfall keine zulassungskonforme Möglichkeit besteht, dann ist diese gerechtfertigt. Verwendet ein Notarzt aber ein Medizinprodukt außerhalb der Zulassung, dann stellt er ein neues Produkt her und haftet persönlich bei einem Patientenschaden.
Moderne Diagnostik der Mitral- und Trikuspidalklappe – was ist wirklich notwendig?
Authors: Spieker Maximilian , Kelm Malte , Westenfeld Ralf 
Published: August 2017

ZusammenfassungMitral- und Trikuspidalklappenerkrankungen weisen eine hohe, mit dem Alter ansteigende Prävalenz in der Bevölkerung auf. Parallel mit den modernen und innovativen Therapieverfahren haben sich die Anforderungen an die Diagnostik von Vitien weiterentwickelt. Den Grundstein für eine erfolgreiche Therapie bildet auch weiterhin die echokardiografische Bildgebung, die präzise Informationen über Art und Ausmaß der Läsion, den Mechanismus, die Ätiologie sowie über die technische Durchführbarkeit einer Intervention liefern muss. Neue Innovationen im Bereich der Echokardiografie erlauben eine noch sensitivere Beschreibung der Pathomorphologie, eine valide Quantifizierung des Schweregrades und die Unterstützung bei der Klappenintervention. Neben den diagnostischen Untersuchungen spielt die klinische Beurteilung des Patienten eine wichtige Rolle und fordert eine kritische Wertung der Untersuchungsergebnisse unter Berücksichtigung der Klinik des Patienten.
Interventionelle Therapie der Mitralklappe: direkte und indirekte Anuloplastie – neue Optionen
Authors: Rottländer Dennis , Degen Hubertus , Haude Michael 
Published: August 2017

ZusammenfassungDer Nutzen der konventionellen Mitralklappenoperation bei Patienten mit funktioneller Mitralklappeninsuffizienz ist unklar. Es wurden hohe Rezidivraten nach 2 Jahren beschrieben. Der chirurgische Goldstandard, die Anuloplastie, zielt auf die Reduktion der Dimensionen des Mitralklappenanulus. In der interventionellen Kardiologie unterscheidet man die indirekte (Carillon) von der direkten Anuloplastie (Cardioband, Mitralign, Accucinch). Das Carillon Mitral Contour System führt nach 12 Monaten zu einer signifikanten Reduktion der Mitralklappeninsuffizienz, einem vermehrten Reverse Remodeling, einer Verbesserung der klinischen Symptomatik und einer Zunahme der 6-Minuten-Gehstrecke. Das Cardioband reduziert die Mitralklappeninsuffizienz und führt zu einer Verbesserung der Herzinsuffizienzsymptomatik und 6-Minuten-Gehstrecke. Das Mitralign System reduziert bei 50% der Patienten die Mitralklappeninsuffizienz, führt zu einem Reverse Remodeling und bessert die Herzinsuffizienzsymptomatik. Das GDS Accucinch System befindet sich noch in ersten klinischen Testungen und klinische Studienergebnisse stehen aus. Eine individuelle Verfahrenswahl erscheint sinnvoll.