[Congenital Intermittent Third-degree Atrioventricular Block Associated with Retinoid Exposure in Pregnancy]. / Konnataler intermittierender AV-Block III° nach Retinoideinnahme in der Schwangerschaft.

BACKGROUND: The retinoid acitretin, which has been approved as an effective therapy for severe keratinization disorders, is highly teratogenic, and exposure in the first trimester of pregnancy is associated with the risk of miscarriage and various malformations, including congenital heart defects. Cardiac conduction system disorders have not been described so far. CASE REPORT: A 24-year-old woman was treated with acitretin for dyskeratosis follicularis until pregnancy was diagnosed at 12 weeks of gestation. The female infant was born after 35 weeks gestation by cesarean section because of intermittent fetal bradycardia. The baby was vigorous at birth (Apgar 9, 10, 10 at 1, 5 and 10 min) but displayed intermittent third-degree atrioventricular block. A search for maternal autoantibodies and viral infections gave negative findings. CONCLUSION: The spectrum of disorders caused by intrauterine retinoid exposure appears to include atrioventricular conduction failure.

IB80-A Novel Infectious Bronchitis Virus Genotype (GVIII).

Since mid-2015, there has been an increasing number of chicken samples that are positive for infectious bronchitis virus (IBV) in a screening PCR but which do not show positive results in any established, variant-specific PCR tests (793B, QX, D1466, Massachusetts, D274, Italy 02, Arkansas, Variant 2, Q1). Partial sequencing of the viral genome of those samples shows great similarities, but nucleotide similarity in the S1 gene is only about 57%-61% when compared to any other known GI-GVII IBV genotype and lineage. With nucleotide identity in the S1 gene of approximately 80%, the closest related strain in the National Center for Biotechnology Information database (as of March 15, 2020) is the North American PA/1220/98 isolate (AY789942) designated as a unique variant by Valastro et al. in 2016. Due to its divergence from other IBV strains, we propose that strain, designated IB80, is the type strain of a novel IBV genotype GVIII. So far, IB80 has been detected in commercial layer and broiler parent flocks, frequently showing severe drops in egg production as well as in broiler flocks in Europe and beyond.

IB80­un nuevo genotipo del virus de la bronquitis infecciosa (GVIII). Desde mediados del 2015, ha habido un número creciente de muestras de pollo que resultan positivas para el virus de la bronquitis infecciosa (IBV) por la detección mediante PCR de escrutinio, pero que no muestran resultados positivos en ninguna prueba de PCR específica para las variantes establecidas (793B, QX, D1466, Massachusetts, D274, Italia 02, Arkansas, variante 2, Q1). La secuenciación parcial del genoma viral de esas muestras muestra grandes similitudes, pero la similitud de nucleótidos en el gene S1 es solo del 57% al 61% en comparación con cualquier otro genotipo y linaje GI-GVII conocidos del virus de bronquitis. Con una identidad de nucleótidos en el gene S1 de aproximadamente el 80 %, la cepa relacionada más cercana en la base de datos del Centro Nacional de Información Biotecnológica (al 15 de marzo de 2020) es el aislamiento norteamericano PA/1220/98 (AY789942) designado como variante única por Valastro et al. en 2016. Debido a su divergencia con otras cepas del virus de bronquitis infecciosa, se propone que la cepa, denominada IB80, es la cepa tipo de un nuevo genotipo GVIII del virus de bronquitis infecciosa. Hasta ahora, se ha detectado IB80 en parvadas de reproductoras de pollos de engorde y ponedoras comerciales, y con frecuencia muestra disminuciones severas en la producción de huevo, así como en parvadas de pollos de engorde en Europa y otras regiones.

[Anatomic and subjective success of structured surgical treatment strategy in the management of chronic epiphora - a postoperative analysis of contentment]. / Anatomischer und subjektiver Erfolg einer strukturierten chirurgischen Therapiestrategie zum Management der chronischen Epiphora - eine postoperative Zufriedenheitsanalyse.

BACKGROUND: Epiphora is the result of hypersecretion or reduced tear outflow because of relative or absolute obstruction of the lacrimal system. For the treatment a specific surgical procedure is usually required. Herein we present the subjective and objective findings of 243 consecutive patients with non-irritative induced epiphora, who underwent an operation in a standardised therapy scheme, which is used at the eye departments of the Universities of Lübeck and Würzburg. PATIENTS AND METHODS: In a retrospective cohort study 243 patients were included who underwent lateral tarsal strip (n = 72), external dacryocystorhinostomy (DCR) in the case of an absolute nasolacrimal duct obstruction (NLDO) (n = 116) and relative NLDO (n = 25), DCR with canalicular surgery (n = 18) and simple canalicular surgery (n = 12) with a follow-up of at least 6 month. Common patient and cosmetical satisfaction, intensity of symptoms and further symptoms of 168 patients were evaluated by sending a questionnaire with subsequent telephone survey. Objective findings were recorded from hospital charts. RESULTS: 59.5% of the patients were free of complaints, 19.6% had an absolute decrease of symptoms (epiphora outside and in windy weather) and 20.8 % persistent epiphora (symptoms both in- and outside persisted). 81% of patients with absolute NLDO and 57% with relative NLDO were graded as being free of symptoms, 7% (24%) as a relative decrease and 12% (19%) as a persistence of symptoms. There was a significant difference in success between these indications (p = 0.018). Patency of the lacrimal system assessed by irrigation was found in 95% (absolute NLDO). Commonly, 73.8% were satisfied, 7.7% indifferent and 18.4 % not satisfied. 4 patients were cosmetically not satisfied. CONCLUSIONS: Use of a standardised therapy scheme reduces the symptoms in 79.1% of the patients. In patients with absolute NLDO external DCR achieves an anatomic success rate of 95% and a subjective improvement in 88% with excellent cosmetic results. In comparison with absolute NLDO the success rate of relative NLDO is lower, but still with a subjective improvement in 81%. Our results show that postoperative success depends on the preoperative cause of the epiphora.

Coenzyme Q(10) is decreased in fibroblasts of patients with methylmalonic aciduria but not in mevalonic aciduria.

The content of coenzyme Q(10) (CoQ(10)) was examined in skin fibroblasts of 10 patients with mevalonic aciduria (MVA) and of 22 patients with methylmalonic aciduria (MMA). Patients with these inborn errors of metabolism are thought to be at risk for CoQ(10) depletion either by direct inhibition of the proximal pathway of CoQ(10) synthesis (MVA) or indirectly by inhibition of mitochondrial energy metabolism (MMA). We demonstrated that CoQ(10) concentrations were not significantly different from controls in MVA patients, suggesting that there may be upregulatory effects. On the other hand the CoQ(10) content in fibroblasts of patients with MMA was significantly reduced.

Reconfigurable re-entrant cavity for wireless coupling to an electro-optomechanical device.

An electro-optomechanical device capable of microwave-to-optics conversion has recently been demonstrated, with the vision of enabling optical networks of superconducting qubits. Here we present an improved converter design that uses a three-dimensional microwave cavity for coupling between the microwave transmission line and an integrated LC resonator on the converter chip. The new design simplifies the optical assembly and decouples it from the microwave part of the setup. Experimental demonstrations show that the modular device assembly allows us to flexibly tune the microwave coupling to the converter chip while maintaining small loss. We also find that electromechanical experiments are not impacted by the additional microwave cavity. Our design is compatible with a high-finesse optical cavity and will improve optical performance.

[Congenital ectropion in ichthyosis congenita mitis and gravis]. / Angeborenes Ektropium bei Ichthyosis congenita mitis und gravis.

BACKGROUND: Congenital ichthyosis is a generalized hyperkeratinization of the skin at birth. Depending on clinical aspects and severity, three forms of congenital ichthyosis have been defined: mitis, tarda, and gravis. Desquamation of the parchment-like hyperkeratinized skin begins shortly after birth and may require several weeks to complete. Skin alterations in the eyelid cause shortening of the anterior lamella, subsequently resulting in ectropion. This affects the upper eyelid more often than the lower and can lead to complications such as chronic palpebral or bulbar conjunctivitis and keratinization or exposure keratopathy. Here we present two case reports illustrating the course of ichthyosis congenita mitis and gravis. PATIENTS AND METHODS: Patient 1 (ichthyosis congenita mitis): a male baby prematurely born at 34+2 weeks of gestation was delivered by cesarean section. The entire body was covered by a parchment-like hyperkeratinized skin. Both eyes showed ectropion of the upper and the lower eyelid, which was more obvious with enforced lid closure. Frequent application of external ointment and spontaneous desquamation led to resolution of the ectropion. Patient 2 (ichthyosis congenita gravis): a male baby prematurely born at 35+4 weeks of gestation was delivered by cesarean section. At birth the child showed the signs of a collodion baby with ectropion of all four eyelids in combination with a characteristic "fish mouth" and rudimentary external ears. The child died on the 14th day of life of septicaemia. CONCLUSION: In mild forms of congenital ichthyosis surgical treatment of eyelid ectropion is not required. In more severe cases a skin graft may become necessary. Various although limited sources of graft material which are discussed can be considered.

Verification of redox-processes as switching and retention failure mechanisms in Nb:SrTiO3/metal devices.

Nanoscale redox reactions in transition metal oxides are believed to be the physical foundation of memristive devices, which present a highly scalable, low-power alternative for future non-volatile memory devices. The interface between noble metal top electrodes and Nb-doped SrTiO3 single crystals may serve as a prominent but not yet well-understood example of such memristive devices. In this report, we will present experimental evidence that nanoscale redox reactions and the associated valence change mechanism are indeed responsible for the resistance change in noble metal/Nb-doped SrTiO3 junctions with dimensions ranging from the micrometer scale down to the nanometer regime. Direct verification of the valence change mechanism is given by spectromicroscopic characterization of switching filaments. Furthermore, it is found that the resistance change over time is driven by the reoxidation of a previously oxygen-deficient region. The retention times of the low resistance states, accordingly, can be dramatically improved under vacuum conditions as well as through the insertion of a thin Al2O3 layer which prevents this reoxidation. These insights finally confirm the resistive switching mechanism at these interfaces and are therefore of significant importance for the study and application of memristive devices based on Nb-doped SrTiO3 as well as systems with similar switching mechanisms.

Mortality while awaiting liver retransplantation: predictability of MELD scores.

INTRODUCTION: Model for End-stage Liver Disease (MELD) scores at the time of listing on the transplant waiting list have been shown to accurately predict 3-month mortality in adults. There is no data assessing the accuracy of the MELD scores in predicting mortality of patients awaiting liver retransplantation. We sought to determine the outcome of patients listed for retransplantation at a single center and the accuracy of MELD scores in predicting mortality on the transplant waiting list. METHODS: A retrospective review of adult patients at a single center listed for a second liver transplantation during the years 1993 to 2000. MELD scores and a concordance statistic were calculated at the time of initial listing and initial transplant as well as the time of relisting for a second transplant and at 2, 4, 6, 8, 12, and 24 weeks after relisting. RESULTS: Of the 63 patients in the study, 43 (68%) received a second transplant, and 20 (32%) died while awaiting retransplantation. Of the patients receiving a second transplant, 13 (30%) died within 1 year of receiving the transplant. The most common cause of death on the waiting list was sepsis (50%), hepatorenal syndrome (20%), and multiorgan failure (10%), whereas the majority of deaths posttransplantation were sepsis-related (69%). At the time of relisting the c-statistic for MELD scores predicting death after 1 week on the waiting list was 0.78 (P = .007). After 3 months on the waiting list, the c-stat was largely unchanged (0.76, P = .04). CONCLUSIONS: We have shown that MELD scores may predict mortality on the transplant waiting list for patients listed for a second transplant.

Adiponectin before and after weight loss in obese children.

Adiponectin is decreased in obesity and seems to be involved in insulin resistance. The influences of age, gender, puberty, and weight loss on adiponectin have not been studied in obese children. We measured body fat mass based on skinfold thickness, age, pubertal stage, gender, adiponectin, and insulin resistance (homeostasis model assessment) in 42 obese children. We analyzed adiponectin and homeostasis model assessment 1 yr later in these obese children and separated them into two groups according to degree of weight loss (decrease in sd score for body mass index, >or=0.5 vs. <0.5). Adiponectin was negatively correlated to percentage body fat (r = -0.44; P = 0.002), insulin resistance (r = -0.33; P = 0.016), and age (r = -0.41; P = 0.003). Adiponectin levels were significantly (P = 0.017) higher in pubertal girls compared with boys, but there was no significant difference in prepubertal children in respect to gender (P = 0.833). Adiponectin was significantly (P < 0.001) lower in pubertal compared with prepubertal children. The significant weight loss in 16 children was associated with a significant increase in adiponectin (P = 0.010) and a decrease in insulin resistance (P = 0.013), whereas there were no changes in the 26 children without significant weight loss. Adiponectin levels in obese children were negatively correlated to age, body fat, and insulin resistance and were decreased in puberty. Significant weight loss led to an increase in adiponectin levels and an improvement of insulin resistance.

SODA (severity of dyspepsia assessment): a new effective outcome measure for dyspepsia-related health.

The aim of this research was to develop and evaluate an instrument for measuring dyspepsia-related health to serve as the primary outcome measure for randomized clinical trials. Building on our previous work we developed SODA (Severity of Dyspepsia Assessment), a multidimensional dyspepsia measure. We evaluated SODA by administering it at enrollment and seven follow-up visits to 98 patients with dyspepsia who were randomized to a 6-week course of omeprazole versus placebo and followed over 1 year. The mean age was 53 years, and six patients (6%) were women. Median Cronbach's alpha reliability estimates over the eight visits for the SODA Pain Intensity, Non-Pain Symptoms, and Satisfaction scales were 0.97, 0.90, and 0.92, respectively. The mean change scores for all three scales discriminated between patients who reported they were improved versus those who were unchanged, providing evidence of validity. The effect sizes for the Pain Intensity (.98) and Satisfaction (.87) scales were large, providing evidence for responsiveness. The effect size for the Non-Pain Symptoms scale was small (.24), indicating lower responsiveness in this study sample. SODA is a new, effective instrument for measuring dyspepsia-related health. SODA is multidimensional and responsive to clinically meaningful change with demonstrated reliability and validity.

Using the national registry of HIV-infected veterans in research: lessons for the development of disease registries.

Disease-specific registries have many important applications in epidemiologic, clinical and health services research. Since 1989 the Department of Veterans Affairs has maintained a national HIV registry. VA's HIV registry is national in scope, it contains longitudinal data and detailed resource utilization and clinical information. To describe the structure, function, and limitations of VA's national HIV registry, and to test its accuracy and completeness. The VA's national HIV registry contains data that are electronically extracted from VA's computerized comprehensive clinical and administrative databases, called Veterans Integrated Health Systems Technology and Architecture (VISTA). We examined the number of AIDS patients and the number of new patients identified to the registry, by year, through December 1996. We verified data elements against information obtained from the medical records at five VA sites. By December 1996, 40,000 HIV-infected patients had been identified to the registry. We encountered missing data and problems with data classification. Missing data occurred for some elements related to the computer programming that creates the registry (e.g., pharmacy files), and for other elements because manual entry is required (e.g., ethnicity). Lack of a standardized data classification system was a problem, especially for the pharmacy and laboratory files. In using VA's national HIV registry we have learned important lessons, which, if taken into account in the future, could lead to the creation of model disease-specific registries.

Current experience with tacalcitol ointment in the treatment of psoriasis.

Various controlled studies have demonstrated the efficacy and safety of tacalcitol ointment (4 micrograms/g) in the treatment of psoriasis. Further data are now available from a multicentre post-marketing surveillance study involving more than 5000 outpatients. Once-daily treatment with tacalcitol resulted in a marked decrease of mean sum score (erythema, infiltration, scaling) from 6.3 to 2.7 score points in an average period of 61 days. Efficacy was assessed by dermatologists as 'very good' or 'good' in 71% of the patients. Local tolerance was stated to be 'very good' or 'good' in 94% of the patients. Only 1% of the patients experienced skin irritation. No case of hypercalcaemia was observed. Compared with conventional therapies, 52% of the patients saved up to half an hour of treatment time daily because of the once-daily application. Good local tolerance and a convenient treatment regimen may also help compliance.

The effect of chain membership on hospital costs.

OBJECTIVE: To compare the cost structures of hospitals in multihospital systems and independently owned hospitals. DATA SOURCES: The American Hospital Association's Annual Survey from 1990 for data on hospital costs and attributes. Area characteristics came from the Area Resource File, and the Medicare case-mix index came from the Health Care Financing Administration. Data on wages are from the Bureau of the Census' State and Metropolitan Area Data Book. The Guide to Hospital Performance from HCIA, Inc. provided data on quality of care. STUDY DESIGN: Separate cost functions were estimated for chain and independent hospitals. Hybrid translog cost functions included measures of outputs, input prices, and hospital and area characteristics. The estimation method accounted for the simultaneous determination of costs and chain membership, and for any nonrandom selection of hospitals into chains. Several economic cost measures were calculated to compare the cost structures of the two types of hospitals. DATA EXTRACTION METHODS: Data from all sources were merged at the hospital level to form the study sample. PRINCIPAL FINDINGS: Hospitals in multihospital systems were less costly than independently owned hospitals. Among independent hospitals, for-profits had the highest costs. There were no statistically significant differences in costs by ownership among chain members. Economies of scale were enjoyed in both types of hospitals only at high volumes of output, while economies of scope occurred at all volumes for chain hospitals, but only at low and medium volumes for independent hospitals. CONCLUSIONS: This study provides support for the idea that growth of the multihospital system sector can provide a market solution to the problem of constraining costs. It does not, however, support the property rights theory that proprietary hospitals are more efficient than nonprofit hospitals.

Coenzyme Q10 reduces the toxicity of rotenone in neuronal cultures by preserving the mitochondrial membrane potential.

Defects in mitochondrial energy metabolism due to respiratory chain disorders lead to a decrease in mitochondrial membrane potential (DeltaPsim) and induce apoptosis. Since coenzyme Q10 (CoQ10) plays a dual role as an antioxidant and bioenergetic agent in the respiratory chain, it has attracted increasing attention concerning the prevention of apoptosis in mitochondrial diseases. In this study the potential of CoQ10 to antagonize the apoptosis-inducing effects of the respiratory chain inhibitor rotenone was explored by video-enhanced microscopy in SH-SY5Y neuroblastoma cells. The cationic fluorescent dye JC-1 which exhibits potential-dependent accumulation in mitochondria was used as an indicator to monitor changes in DeltaPsim. The relative changes in fluorescence intensity after incubation with rotenone for 15 minutes were calculated. Pre-treatment with CoQ10 (10 or 100 microM) for 48 h led to a significant reduction of rotenone-induced loss of DeltaPsim. These results suggest, that cytoprotection by CoQ10 may be mediated by raising cellular resistance against the initiating steps of apoptosis, namely the decrease of DeltaPsim. Whether these data may provide new directions for the development of neuroprotective strategies has to be investigated in future studies.

Rates of health services utilization and survival in patients with heart failure in the Department of Veterans Affairs medical care system.

The objective of this study was to describe patterns of hospital and clinic use and survival for a large nationwide cohort of patients with heart failure. A retrospective cohort study of patients treated in the Veterans Affairs medical care system was conducted using linked administrative databases as data sources. In 1996, the average heart failure cohort member had 1-2 hospitalizations, 14 inpatient days, 6-7 visits with the primary physician, 15 other visits for consultations or tests, and 1-2 urgent care visits per 12 months. The overall risk-adjusted 5-year survival rate was 36%. Hospital use rates in the cohort fell dramatically between 1992 and 1996. One-year survival rates increased slightly over the period. Patients with heart failure are heavy users of services and have a very poor prognosis. Utilization and outcome data indicate the need for major efforts to assure quality of care and to devise innovative ways of delivering comprehensive services.

Impacts of PPS on Medicare Part B expenditures and utilization for hospital episodes of care.

The universe of Medicare claims for four states from 1983 through 1986 is used to examine the impacts Medicare's prospective payment system (PPS) makes on hospital lengths of stay and on Part B expenditures for physician services associated with hospital stays. The descriptive results show that lengths of stay have fallen, while both inpatient and outpatient expenditures have risen. The regression results, on the other hand, show that PPS has reduced both lengths of stay and expenditures, and that other factors, such as increases in physician fees and patients' severity of illness, account for expenditure increases.

Cost implications of regionalizing open heart surgery units.

This study calculated the potential change in costs from regionalizing open heart surgery units in a geographic network of the Department of Veterans Affairs (VA). It used data from the VA's cost accounting system, and the authors conducted a sensitivity analysis. Under consolidation, savings from closing an open heart surgery unit would be partially offset by the costs of treating nonemergency cases at other VAs, treating emergency cases at non-VA hospitals, and transporting patients to regionalized facilities. Nevertheless, the potential savings from consolidation would exceed $3 million, or 18% of the network's costs of treating open heart surgery patients.

How the physician fee schedule affects Medicare patients' out-of-pocket spending.

Simulations of the redistributive effects of the new Medicare fee schedule have focused primarily on physicians, but patients may also be affected. Using a national sample of Medicare patients, we studied the fee schedule's potential impact on both components of out-of-pocket spending: copayments and balance bill amounts. While the fee schedule would raise copayments for the average patient, this effect would be more than offset by the balance billing limits (also imposed by Congress). Nevertheless, almost 10% of patients, particularly those who are black or living in rural areas, would experience large increases in their liability. Finally, the fee schedule with balance billing limits also serves as a "catastrophic coverage" program; patients with extraordinarily high medical expenses would enjoy substantial reductions in their out-of-pocket payments.

Clinical and socioeconomic determinants of health care use among HIV-infected patients in the Department of Veterans Affairs.

This study estimates the impact of clinical and socioeconomic characteristics on health care use for HIV-infected patients. Data come from the Department of Veterans Affairs (VA) HIV Registry, which electronically extracts data from patients' automated medical records, and from patient interviews. Unlike prior studies, this analysis includes a staging system incorporating CD4 count and AIDS-defining diagnoses. Results showed that clinical factors were the most important determinants of health care use; socioeconomic variables were seldom significant. These findings were expected, since the VA is an equal access system, providing care regardless of socioeconomic status.

The health status of veterans using mobile clinics in rural areas.

Between 1992 and 1994, the Department of Veterans Affairs (VA) experimented with mobile clinics to provide health care for rural veterans. The objective was to assess the health status of rural mobile clinics' patients and compare this with patients receiving care in VA hospital-based clinics. This study hypothesized that hospital-based clinic patients would be more ill (i.e., have a greater reduction in health status). The Medical Outcomes Study (MOS) Short Form was used to evaluate patients' health status. Most patients sought care for the management of chronic disease. Patients in both groups had similar types of diseases. Mobile clinic patients were as ill as hospital-based patients (i.e., similar health status scores). This study shows that rural veterans have a case mix and a reduction in health status similar to that of VA hospital-based patients. Planners should account for this health reduction when planning the kinds of facilities and services needed in rural areas.