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Hintergrund: Eine bewährte und häufig angewandte Operation bei Patienten (Pat) mit idiopathischer Skoliose (IS) ist die dorsale transpedikuläre Korrekturspondylodese in Freihandtechnik. Neuere Verfahren mit fluoroskopisch- und CT-gestützter Navigation sind mutmaßlich risikoärmer und exakter. Fragestellung: Ist die Freihandtechnik bei IS bezüglich der schraubenassoziierten Komplikationen und intraoperativen Strahlenbelastung sicher? Material und Methoden: Anhand prospektiv erfasster Daten (2017–2018) von 39 konsekutiven Pat (durchschn. Alter 18,7 J.) mit thorakal einbogiger IS (61,7° ±13,9) eines Skoliosezentrums wurden folgende Parameter (Mittelwert ± Standardabweichung) ausgewertet: Gesamtstrahlenprodukt, Durchleuchtungszeit, fusionierte Segmente, Operationszeit, Blutverlust und schraubenassoziierte Komplikation. Es erfolgte ein Literaturvergleich mit Daten zur intraoperativen Strahlenbelastung bei Navigationsverfahren. Ergebnisse: Das Gesamtstrahlenprodukt/Pat betrug 71,7 ± 44,0 cGy * cm2, die Durchleuchtungszeit 17,4 ± 8,6 s (7,8 Segmente), die Operationszeit 183,5 ± 54,2 min und der Blutverlust 379,5 ± 183 ml. Keine schraubenassoziierte Komplikation im gesamten Kollektiv. Die Korrektur der Hauptkrümmung betrug 75,7 %. Der Vergleich der erhobenen Daten mit denen aus der Literatur zeigte eine für den Pat 1,25- bis 12,5-fach höhere Strahlenexposition bei fluoroskopisch- gestützter Navigation und 9,25- bis 12,3-fach höhere bei CT-gestützten Verfahren. Schlussfolgerung: Freihändiges Setzen von Pedikelschrauben ist bei entsprechender Erfahrung mit vergleichbarer Genauigkeit und geringerer Strahlenbelastung für den Pat verbunden als navigierende Verfahren. Hinsichtlich des geringen Alters der Pat sollte ein strahleninduziertes Langzeitrisiko für maligne Erkrankungen berücksichtigt werden.

How do core business and manufacturing capabilities enable strategies for sustainable manufacturing, and what are those capabilities? This paper proposes a Capability Methodology for Sustainable Manufacturing (CMSM) for allowing top management of manufacturing companies to address these questions. A diagnostic tool was developed from three case studies based on a set of interview questions aimed at identifying core capabilities and sustainability issues in manufacturing companies. Interview data was coded and mapped through a relational matrix formulation that describes four archetypes for the development of sustainability strategies. The matrix maps the degree of complexity of the sustainability concept as understood by the company, and the scope of the product life cycle being considered. It is argued that the methodology helps bring awareness to managers of any gaps or mismatches between their actual core capabilities and the desired outcomes for sustainable manufacturing.

Hearing impairment is clinically and genetically heterogeneous. There are >400 disorders in which hearing impairment is a characteristic of the syndrome, and family studies demonstrate that there are at least 30 autosomal loci for nonsyndromic hearing impairment. The genes that have been identified encode diaphanous (HDIA1), alpha-tectorin (TECTA), the transcription factor POU4F3, connexin 26 (GJB2), and two unconventional myosins (MYO7A and MYO15), and four novel proteins (PDS, COCH, DFNA5, DFNB9). The same clinical phenotype in hearing-impaired individuals, even those within the same family, can result from mutations in different genes. Conversely, mutations in the same gene can result in a variety of clinical phenotypes with different modes of inheritance. For example, mutations in the gene encoding MYO7A cause Usher syndrome type IB, autosomal-recessive nonsyndromic hearing impairment (DFNB2), and autosomal-dominant nonsyndromic hearing impairment (DFNA11). Additionally, the mouse ortholog of the MYO7A gene is the shaker-1 gene. Mouse models such as shaker-1 have facilitated the identification of genes that cause hearing impairment in humans. The availability of high-resolution maps of the human and mouse genomes and new technologies for gene identification are advancing molecular understanding of hearing impairment and the complex mechanisms of the auditory system.

Sera from patients with rheumatic autoimmune diseases were screened for antibodies directed against RNA polymerase I by using a solid-phase radioimmunoassay. Significant levels of the antibodies were detected in the sera of all patients with either systemic lupus erythematosus or mixed connective tissue disease and in 78% of the individuals with rheumatoid arthritis. No detectable anti-RNA polymerase I antibodies were found in the sera from healthy subjects. Individuals taking hydralazine, three of whom exhibited symptoms of drug-induced lupus, had barely detectable levels of the antibodies. Immunoglobulins obtained from sera containing anti-RNA polymerase I antibodies, as determined by the radioimmunoassay, could inhibit RNA polymerase I activity in vitro. Sera from patients with systemic lupus erythematosus contained immunoglobulins directed against the polymerase I-associated polypeptide of Mr 65,000 as well as against the polypeptides of Mr 120,000 or Mr 25,000, or both. Sera from individuals with rheumatoid arthritis reacted with the polypeptide of Mr 65,000 only. The antibodies in the sera of patients with mixed connective tissue disease were directed against the Mr 42,000 polypeptide or a combination of the Mr 65,000, 42,000, and 25,000 polypeptides. These data suggest that the production of anti-RNA polymerase I antibodies may be a unique characteristic of individuals with rheumatic autoimmune diseases and that the production of antibodies against specific polypeptides of RNA polymerase I may be indicative of the particular class of disease.

A fluorometric method for determining bethanidine in blood plasma is described. The bethanidine is extracted into chloroform, a drug‐dye complex with eosin Y is formed, and the fluorescence is measured (excitation, 535 nm; fluorescence, 560 nm). The assay detects 0.02 μM bethanidine (4 ng/ml) in plasma. The relative fluorescence of several body constituents and antihypertensive drugs is negligible. The plasma levels of bethanidine in four hypertensive patients receiving this drug were measured.

Neonatal hyperphenylalaninaemia caused by mutations in the gene encoding phenylalanine hydroxylase (PAH) represents a wide spectrum of metabolic phenotypes, ranging from classical phenylketonuria (PKU) to mild hyperphenylalaninaemia (MHP). The marked interindividual heterogeneity is due to the expression of multiple PAH mutations in genetic compounds. We have investigated four unusual families in which both PKU and MHP were present. In each family three different mutations in the PAH gene were identified, including two associated with PKU and one associated with MHP. The unexpected outcome of discordant phenotypes within the families described is explained by previously unrecognized parental MHP. By mutation analysis we have also predicted the phenotypical outcome in a hyperphenylalaninaemic infant born to a mother who before pregnancy had been diagnosed as having MHP. Our results demonstrate the utility of nucleic acid analysis in follow-up in PKU screening programmes.

A number of hearing-impaired individuals with near-normal thresholds for frequencies above 8 000 Hz who are characterized by good speech articulation have recently been identified. It is unclear whether this unusually good articulation is linked to the ability of such persons to process speech signals containing energy that falls within the range of their residual, high-frequency hearing (for example, certain consonants). This paper reports a detailed study of the signal-processing capabilities of one patient with a long-standing, severe to profound hearing loss in the standard audiometric range, and normal left-ear thresholds in the region of 13 000 Hz. Measures obtained include difference limens for frequency and intensity, gap detection, rate matching, temporal integration, and tone-on-tone masking profiles (psychoacoustical tuning curves). In addition, measures of speech production and perception were made when the patient was using an experimental hearing aid designed to transpose speech range frequencies into the region of her residual high-frequency hearing. Results indicate the patient can efficiently process signals which fall within the band of her best hearing, and suggest her ultra-audiometric hearing may be useful in speech communication.Nous avons etudie la capacite de traiter les signaux auditifs chez une patiente presentant une deterioration severe a profonde dans le champ conventionnel d'audibilite, et des seuils d'audition presque normaux pour des frequences aux environs de 13 000 Hz. Nous avons enregistre les seuils differentiels de frequence et d'intensite, la detection d'une interruption dans un signal, l'integration temporelle, les courbes d'accord psycho-acous-tique de Zwicker et Schorn, la capacite d'extraire l'information concernant le taux de repetition a partir de signaux de haute frequence spectrale. Nous avons aussi effectue des mesures de perception et de production de la parole lorsque la patiente utilisait une prothese experimentale transposant les frequences de la parole dans la region de 13 000 Hz.Les resultats indiquent que cette personne est capable de traiter les signaux aux environs de 13 000 Hz au moins aussi bien que ceux qui tombent dans la region de sa deterioration auditive, ce qui permet de penser qu'elle est en mesure d'utiliser le residu auditif ultra-audiometrique pour la communication parlee.

In three generations of the S. family there were 16 members with severe deafness all of whom were examined. By history all members were born with at least some hearing, with progressive severe loss by later childhood. Sonographic and speech analysis gave further evidence of at least some hearing in early childhood. Audiologic tests on 3 members of the family suggested a cochlear location of the defect. Other tests done on 10 affected members including physical, neurological, and vestibular examinations showed no abnormalities. The deafness appears to be transmitted as an autosomal recessive. We suggest that this entity be called "Recessive early-onset neural deafness".

A non-surgical method for recording human acoustic nerve action potentials is described. Data obtained from 18 subjects with normal hearing are presented, and a relationship between stimulus intensity and latency of the N1 component of the acoustic nerve response is given. Possible application of this relationship as a reference in diagnosing hearing disorders is discussed.

Sehr geehrte Leserin,

The deafness mouse has profound sensorineural hearing loss with degeneration of hair cells soon after birth. The mode of inheritance is recessive, and there are no associated phenotypic anomalies. Thus, this mouse provides a model for recessive, nonsyndromic, prelingual deafness. We have mapped the gene causing deafness in the mouse to Chromosome (Chr) 19 by analysis of 230 intersubspecific backcross progeny. No recombinants were found with the microsatellite marker D19Mit14. The loci for two guanine nucleotide-binding proteins are tightly linked to this marker, and they are being investigated as possible candidate genes. The identification of the defective gene in the mouse will help to explain the mechanism that causes hair cell degeneration and is likely to identify a homologous gene for deafness in humans.

Antibodies against nuclear protein kinase NII were detected by radioimmunoassay in the sera of patients with systemic lupus erythematosus and mixed connective tissue disease but not in sera from normal individuals or from patients with rheumatoid arthritis. Immunoglobulins derived from sera containing the anti-kinase antibodies inhibited the activity of protein kinase NII in vitro but had no effect on the activity of cytoplasmic cyclic AMP-dependent protein kinase.

We recommend individualized evaluation strategies for cochlear implant candidates that consider new developments in hearing aid technology. Four categories of patients are presented. Category I patients can be helped by modern ear level high-powered hearing aids and implantation is not warranted. Categories II and III patients leave us uncertain; they have improved sound detection with hearing aids, but achieve no open set word recognition. Category IV patients are unquestionably candidates for implants because no method of stimulation helps them. Experience has taught us that the most appropriate evaluation plan reflects the patient's history and is sensitive to the emotional and financial consequences of aborted candidacy. Those patients who are candidates can be guided through each component of the evaluation process with ever increasing confidence of ultimate success at the time of surgery.

Correction to: Leukemia (2015) 29, 647–659; doi:10.1038/leu.2014.233; published online 29 August 2014 Following the publication of this article, the authors noted that there were errors in Figure 4a and c. The correct figure appears below:

Purpose: The efficacy of dextromethorphan (DM) for treating cough is uncertain, and its use is not supported by the American Academy of Pediatrics. Still, DM is often given to children as an antitussive. Dosing of DM is based on age, not weight. We sought to determine if a dose-response relationship existed among a cohort of children given a single nocturnal dose of DM for cough due to an upper respiratory infection.Methods: In a double-masked, placebo-controlled trial of over-the-counter cough medications, 33 children received DM. Dose given was based on the drug label: children 2-5 years - 7.5 mg; children 6-11 years - 15 mg; children 12-18 years - 30 mg. This equated to a range of 0.35-0.94 mg/kg/dose. Subjective parental assessments of cough and sleep were assessed via questions using a 7-point Likert scale that compared symptoms to the prior night without medication. Three dose ranges were compared.Results: (See Table)Conclusion: While no statistically significant differences were detectable for the outcomes studied, there was a trend towards improved symptom control with higher doses of DM. This should be considered in future evaluations of the efficacy of DM.Clinical Pharmacology & Therapeutics (2004) 75, P23–P23; doi: 10.1016/j.clpt.2003.11.087