Memory in posttraumatic stress disorder: properties of voluntary and involuntary, traumatic and nontraumatic autobiographical memories in people with and without posttraumatic stress disorder symptoms.

One hundred fifteen undergraduates rated 15 word-cued memories and their 3 most negatively stressful, 3 most positive, and 7 most important events and completed tests of personality and depression. Eighty-nine also recorded involuntary memories online for 1 week. In the first 3-way comparisons needed to test existing theories, comparisons were made of memories of stressful events versus control events and involuntary versus voluntary memories in people high versus low in posttraumatic stress disorder (PTSD) symptom severity. For all participants, stressful memories had more emotional intensity, more frequent voluntary and involuntary retrieval, but not more fragmentation. For all memories, participants with greater PTSD symptom severity showed the same differences. Involuntary memories had more emotional intensity and less centrality to the life story than voluntary memories. Meeting the diagnostic criteria for traumatic events had no effect, but the emotional responses to events did. In 533 undergraduates, correlations among measures were replicated and the Negative Intensity factor of the Affect Intensity Measure correlated with PTSD symptom severity. No special trauma mechanisms were needed to account for the results, which are summarized by the autobiographical memory theory of PTSD.

THE LESSER-KNOWN PIANO CHAMBER MUSIC OF CAMILLE SAINT-SAENS: A RECORDING PROJECT

Throughout his long and industrious lifetime, Camille Saint-Saens (1835-1921) devoted himself unconditionally to music both as a composer and a performer. Saint-Saens was a self-described traditionalist and musical purist, yet his works are distinctly expressive and imaginative, and they reflect the composer's own unique musical language which incorporates recognizably modem traits such as chromaticism and frequent modulation. As a performer, Saint-Saens preferred to premiere his own works and often included his chamber music in his concert programs. Regarded primarily as a symphonic composer in the present day, however, his extensive and varied collection of chamber music works is sadly neglected. Six varied small-ensemble works with piano from his chamber music repertoire have been selected for study and recording for this project: Piano Trio No. 1 in F Major, Op. 18 (1864); Sonata for Cello and Piano No. 1 inC Minor, Op. 32 (1872); two pieces for two pianos, Le Rouet d'Omphale (The Spinning Wheel ofOmphale), Op. 31 (1871) and Phaeton, Op. 39 (1874); piano duet Konig Harald Haifagar (King Harald Haarfager), Op. 59 (1880); and a wind quartet, Caprice sur des airs Danois et Russes (Caprice on Danish and Russian Airs) for Flute, Oboe, Clarinet and Piano, Op. 79 (1887). Analyses of the forms and harmonic structures of these compositions will be included in this dissertation paper as well as studies from the viewpoint of Saint-Saens' compositional style, ensemble characteristics, and writing for the piano. The recordings for this project were made in four sessions in LeFrak Concert Hall at Queens College, the City University of New York. On September 24, 2003, Op. 31, Op. 39 and Op. 59 were recorded with Professor Morey Ritt, piano. On March 2, 2004, Op. 18 was recorded with Elena Rojas, violin, and Clare Liu, cello, and on March 15, 2004, Op. 32 was recorded, also with Ms. Liu. The Caprice, Op. 79 was recorded on June 27, 2008 with Laura Conwesser, flute; Randall Wolfgang, oboe; and Steve Hartman, clarinet. The recordings may be found on file in the library at the University of Maryland, College Park.

SOLO AND CHAMBER CLARINET WORKS BY AMERICAN- BORN WOMEN COMPOSERS: 1990-2005

During the period of 1990-2005, American-born women composers have contributed significantly to the standard clarinet repertoire. Pioneering composers such as Joan Tower, Margaret Brouwer, and Libby Larsen have created staples for clarinet literature. Yet, there is very little scholarly research on women composers of clarinet music, most being concentrated on Joan Tower. Through my research, I have discovered over seventy-five works by more than fifty composers in the following genres: solo clarinet; clarinet and piano; clarinet and voice, with or without piano; and small chamber pieces for up to five players. This performance dissertation project consists of three recitals featuring solo and chamber works by nine living women composers, and program notes containing pertinent biographical and compositional information. My intent is to increase recognition women composers, both prominent and lesser known, who are writing high-quality, accessible clarinet literature. Each woman selected is making a full or partial living from the sales of her compositions, has received recognition through awards, commissions, grants, and frequent performances, and has composed works that are both performer and audience accessible. Recital 1: Trios for Clarinet, Violin, and Piano Commissioned by the Verdehr Trio and Composed by American-Born Women Composers. Composers: Jennifer Higdon, Joan Tower, Margaret Brouwer, and Libby Larsen. Recital II: Programmatic Clarinet Works by American-Born Women Composers. Composers: Andrea Clearfield, Stella Sung, and Karen Amrhein. Recital III: Works for Solo Clarinet, Clarinet and Piano, and Clarinet Concerto Genres by American-Born Women Composers. Composers: Persis Parshall Vehar, Jenni Brandon, Margaret Brouwer, and Libby Larsen.

Mitochondrial mutations in adenoid cystic carcinoma of the salivary glands.

BACKGROUND: The MitoChip v2.0 resequencing array is an array-based technique allowing for accurate and complete sequencing of the mitochondrial genome. No studies have investigated mitochondrial mutation in salivary gland adenoid cystic carcinomas. METHODOLOGY: The entire mitochondrial genome of 22 salivary gland adenoid cystic carcinomas (ACC) of salivary glands and matched leukocyte DNA was sequenced to determine the frequency and distribution of mitochondrial mutations in ACC tumors. PRINCIPAL FINDINGS: Seventeen of 22 ACCs (77%) carried mitochondrial mutations, ranging in number from 1 to 37 mutations. A disproportionate number of mutations occurred in the D-loop. Twelve of 17 tumors (70.6%) carried mutations resulting in amino acid changes of translated proteins. Nine of 17 tumors (52.9%) with a mutation carried an amino acid changing mutation in the nicotinamide adenine dinucleotide dehydrogenase (NADH) complex. CONCLUSIONS/SIGNIFICANCE: Mitochondrial mutation is frequent in salivary ACCs. The high incidence of amino acid changing mutations implicates alterations in aerobic respiration in ACC carcinogenesis. D-loop mutations are of unclear significance, but may be associated with alterations in transcription or replication.

Thermodynamic and dynamic contributions to future changes in regional precipitation variance: focus on the Southeastern United States

© 2014, Springer-Verlag Berlin Heidelberg.The frequency and severity of extreme events are tightly associated with the variance of precipitation. As climate warms, the acceleration in hydrological cycle is likely to enhance the variance of precipitation across the globe. However, due to the lack of an effective analysis method, the mechanisms responsible for the changes of precipitation variance are poorly understood, especially on regional scales. Our study fills this gap by formulating a variance partition algorithm, which explicitly quantifies the contributions of atmospheric thermodynamics (specific humidity) and dynamics (wind) to the changes in regional-scale precipitation variance. Taking Southeastern (SE) United States (US) summer precipitation as an example, the algorithm is applied to the simulations of current and future climate by phase 5 of Coupled Model Intercomparison Project (CMIP5) models. The analysis suggests that compared to observations, most CMIP5 models (~60 %) tend to underestimate the summer precipitation variance over the SE US during the 1950–1999, primarily due to the errors in the modeled dynamic processes (i.e. large-scale circulation). Among the 18 CMIP5 models analyzed in this study, six of them reasonably simulate SE US summer precipitation variance in the twentieth century and the underlying physical processes; these models are thus applied for mechanistic study of future changes in SE US summer precipitation variance. In the future, the six models collectively project an intensification of SE US summer precipitation variance, resulting from the combined effects of atmospheric thermodynamics and dynamics. Between them, the latter plays a more important role. Specifically, thermodynamics results in more frequent and intensified wet summers, but does not contribute to the projected increase in the frequency and intensity of dry summers. In contrast, atmospheric dynamics explains the projected enhancement in both wet and dry summers, indicating its importance in understanding future climate change over the SE US. The results suggest that the intensified SE US summer precipitation variance is not a purely thermodynamic response to greenhouse gases forcing, and cannot be explained without the contribution of atmospheric dynamics. Our analysis provides important insights to understand the mechanisms of SE US summer precipitation variance change. The algorithm formulated in this study can be easily applied to other regions and seasons to systematically explore the mechanisms responsible for the changes in precipitation extremes in a warming climate.

Recognition of Planar Segments in Point Cloud Based on Wavelet Transform

© 2005-2012 IEEE.Within industrial automation systems, three-dimensional (3-D) vision provides very useful feedback information in autonomous operation of various manufacturing equipment (e.g., industrial robots, material handling devices, assembly systems, and machine tools). The hardware performance in contemporary 3-D scanning devices is suitable for online utilization. However, the bottleneck is the lack of real-time algorithms for recognition of geometric primitives (e.g., planes and natural quadrics) from a scanned point cloud. One of the most important and the most frequent geometric primitive in various engineering tasks is plane. In this paper, we propose a new fast one-pass algorithm for recognition (segmentation and fitting) of planar segments from a point cloud. To effectively segment planar regions, we exploit the orthonormality of certain wavelets to polynomial function, as well as their sensitivity to abrupt changes. After segmentation of planar regions, we estimate the parameters of corresponding planes using standard fitting procedures. For point cloud structuring, a z-buffer algorithm with mesh triangles representation in barycentric coordinates is employed. The proposed recognition method is tested and experimentally validated in several real-world case studies.

PIANIST-COMPOSERS WHOSE WORKS INCLUDE PIANISTIC INNOVATIONS

Throughout the piano’s history, certain composers have created innovations in the areas of virtuosity and sonority. These innovations came not only from the composers’ imagination, but also from the development of instruments and changes in musical style from one period to another. To investigate what kinds of innovations these pianist composers made, I divided them into technique and sound from Mozart to Cowell. I chose two-piano music (Sonata in D major, K.448 by Mozart and Rachmaninoff’s Second Suite) to demonstrate their experiments with varieties of textures and sonorities, using different registers of the two pianos orchestrally. En Blanc et noir by Debussy shows this composer’s deep interest and originality in piano sonorities. For solo piano music, Beethoven’s Piano Sonata Op.53 shows extensive technical invention. His use of long pedal effects shows a pianistic possibility not explored by Mozart. Hummel’s Piano Sonata in D major represents orchestral devices as well as pianistic techniques showing recent developments in the instrument. Chopin’s Ballade No.3 and Scherzo No.3 show virtuosic moments and also the expanded range of the keyboard. His Nocturne Op.27, no.2, with its sonorities resulting from the combination of pedal, and widespread accompaniments derived from Alberti bass figures, is a perfect example of Chopin’s characteristic sound-world. “Vallée d’Obermann” by Liszt uses many virtuosic techniques as well as the extreme wide ranges of keyboard in both hands to create dramatic contrasts of texture. Debussy’s etude, “Pour les Sonorités opposés” is probably the first etude designed for sonority rather than for keyboard virtuosity. Albeniz’s “Evocación” and “Triana” show Spanish atmosphere. Prokofiev’s Sonata no.3 shows frequent motoric driving elements that demand percussive virtuosity. Cowell’s piano music is some of the earliest to explore the sonorities of tone clusters and playing on the strings. This performance dissertation consists of three recitals performed in the Orchestra Room, Leah Smith Hall, and Gildenhorn Recital Hall at the University of Maryland, College Park. These recitals are documented on compact disc recordings that are housed within the University of Maryland Library System.

Adaptive intervention design in mobile health: Intervention design and development in the Cell Phone Intervention for You trial.

BACKGROUND/AIMS: The obesity epidemic has spread to young adults, and obesity is a significant risk factor for cardiovascular disease. The prominence and increasing functionality of mobile phones may provide an opportunity to deliver longitudinal and scalable weight management interventions in young adults. The aim of this article is to describe the design and development of the intervention tested in the Cell Phone Intervention for You study and to highlight the importance of adaptive intervention design that made it possible. The Cell Phone Intervention for You study was a National Heart, Lung, and Blood Institute-sponsored, controlled, 24-month randomized clinical trial comparing two active interventions to a usual-care control group. Participants were 365 overweight or obese (body mass index≥25 kg/m2) young adults. METHODS: Both active interventions were designed based on social cognitive theory and incorporated techniques for behavioral self-management and motivational enhancement. Initial intervention development occurred during a 1-year formative phase utilizing focus groups and iterative, participatory design. During the intervention testing, adaptive intervention design, where an intervention is updated or extended throughout a trial while assuring the delivery of exactly the same intervention to each cohort, was employed. The adaptive intervention design strategy distributed technical work and allowed introduction of novel components in phases intended to help promote and sustain participant engagement. Adaptive intervention design was made possible by exploiting the mobile phone's remote data capabilities so that adoption of particular application components could be continuously monitored and components subsequently added or updated remotely. RESULTS: The cell phone intervention was delivered almost entirely via cell phone and was always-present, proactive, and interactive-providing passive and active reminders, frequent opportunities for knowledge dissemination, and multiple tools for self-tracking and receiving tailored feedback. The intervention changed over 2 years to promote and sustain engagement. The personal coaching intervention, alternatively, was primarily personal coaching with trained coaches based on a proven intervention, enhanced with a mobile application, but where all interactions with the technology were participant-initiated. CONCLUSION: The complexity and length of the technology-based randomized clinical trial created challenges in engagement and technology adaptation, which were generally discovered using novel remote monitoring technology and addressed using the adaptive intervention design. Investigators should plan to develop tools and procedures that explicitly support continuous remote monitoring of interventions to support adaptive intervention design in long-term, technology-based studies, as well as developing the interventions themselves.

Effects of chronic mild traumatic brain injury on white matter integrity in iraq and afghanistan war veterans

Mild traumatic brain injury (TBI) is a common source of morbidity from the wars in Iraq and Afghanistan. With no overt lesions on structural MRI, diagnosis of chronic mild TBI in military veterans relies on obtaining an accurate history and assessment of behavioral symptoms that are also associated with frequent comorbid disorders, particularly posttraumatic stress disorder (PTSD) and depression. Military veterans from Iraq and Afghanistan with mild TBI (n = 30) with comorbid PTSD and depression and non-TBI participants from primary (n = 42) and confirmatory (n = 28) control groups were assessed with high angular resolution diffusion imaging (HARDI). White matter-specific registration followed by whole-brain voxelwise analysis of crossing fibers provided separate partial volume fractions reflecting the integrity of primary fibers and secondary (crossing) fibers. Loss of white matter integrity in primary fibers (P < 0.05; corrected) was associated with chronic mild TBI in a widely distributed pattern of major fiber bundles and smaller peripheral tracts including the corpus callosum (genu, body, and splenium), forceps minor, forceps major, superior and posterior corona radiata, internal capsule, superior longitudinal fasciculus, and others. Distributed loss of white matter integrity correlated with duration of loss of consciousness and most notably with "feeling dazed or confused," but not diagnosis of PTSD or depressive symptoms. This widespread spatial extent of white matter damage has typically been reported in moderate to severe TBI. The diffuse loss of white matter integrity appears consistent with systemic mechanisms of damage shared by blast- and impact-related mild TBI that involves a cascade of inflammatory and neurochemical events. © 2012 Wiley Periodicals, Inc.

Influence of the thalamus on spatial visual processing in frontal cortex.

Each of our movements activates our own sensory receptors, and therefore keeping track of self-movement is a necessary part of analysing sensory input. One way in which the brain keeps track of self-movement is by monitoring an internal copy, or corollary discharge, of motor commands. This concept could explain why we perceive a stable visual world despite our frequent quick, or saccadic, eye movements: corollary discharge about each saccade would permit the visual system to ignore saccade-induced visual changes. The critical missing link has been the connection between corollary discharge and visual processing. Here we show that such a link is formed by a corollary discharge from the thalamus that targets the frontal cortex. In the thalamus, neurons in the mediodorsal nucleus relay a corollary discharge of saccades from the midbrain superior colliculus to the cortical frontal eye field. In the frontal eye field, neurons use corollary discharge to shift their visual receptive fields spatially before saccades. We tested the hypothesis that these two components-a pathway for corollary discharge and neurons with shifting receptive fields-form a circuit in which the corollary discharge drives the shift. First we showed that the known spatial and temporal properties of the corollary discharge predict the dynamic changes in spatial visual processing of cortical neurons when saccades are made. Then we moved from this correlation to causation by isolating single cortical neurons and showing that their spatial visual processing is impaired when corollary discharge from the thalamus is interrupted. Thus the visual processing of frontal neurons is spatiotemporally matched with, and functionally dependent on, corollary discharge input from the thalamus. These experiments establish the first link between corollary discharge and visual processing, delineate a brain circuit that is well suited for mediating visual stability, and provide a framework for studying corollary discharge in other sensory systems.

Geochemical and isotopic evidence for seawater contamination of the hydrothermal system of Taal volcano, Luzon, the Philippines

The hydrologic structure of Taal Volcano has favored development of an extensive hydrothermal system whose prominent feature is the acidic Main Crater Lake (pH<3) lying in the center of an active vent complex, which is surrounded by a slightly alkaline caldera lake (Lake Taal). This peculiar situation makes Taal prone to frequent, and sometimes catastrophic, hydrovolcanic eruptions. Fumaroles, hot springs, and lake waters were sampled in 1991, 1992, and 1995 in order to develop a geochemical model for the hydrothermal system. The low-temperature fumarole compositions indicate strong interaction of magmatic vapors with the hydrothermal system under relatively oxidizing conditions. The thermal waters consist of highly, moderately, and weakly mineralized solutions, but none of them corresponds to either water-rock equilibrium or rock dissolution. The concentrated discharges have high Na contents (>3500 mg/kg) and low SO4/Cl ratios (<0.3). The Br/Cl ratio of most samples suggests incorporation of seawater into the hydrothermal system. Water and dissolved sulfate isotopic compositions reveal that the Main Crater Lake and spring discharges are derived from a deep parent fluid (T≃300°C), which is a mixture of seawater, volcanic water, and Lake Taal water. The volcanic end member is probably produced in the magmatic-hydrothermal environment during absorption of high-temperature gases into groundwater. Boiling and mixing of the parent water give rise to the range of chemical and isotopic characteristics observed in the thermal discharges. Incursion of seawater from the coastal region to the central part of the volcano is supported by the low water levels of the lakes and by the fact that Lake Taal was directly connected to the China sea until the sixteenth century. The depth to the seawater-meteoric water interface is calculated to be 80 and 160 m for the Main Crater Lake and Lake Taal, respectively. Additional data are required to infer the hydrologic structure of Taal. Geochemical surveillance of the Main Crater Lake using the SO4/Cl, Na/K, or Mg/Cl ratio cannot be applied straightforwardly due to the presence of seawater in the hydrothermal system.

Prevention of recurrences in frequently relapsing herpes labialis with thymopentin a randomized double-blind placebo-controlled multicenter study

The present randomized, placebo-controlled double-blind multicenter study included a population of 36 subjects with frequent recurrences (at least once a month) of herpes labialis. Most of the patients had failed to respond adequately to previous treatment with other therapeutic tools, including acyclovir. Either 50 mg of thymopentin or of placebo was administered 3 times a week, by the subcutaneous route, for 6 weeks. Subsequently, the patients were observed for nearly 6 months on the average. The results achieved with thymopentin for the individual parameters were significantly superior to those obtained with placebo; thus significant improvement was seen in patients on thymopentin in the duration of the longest symptomfree period (prolonged from 2.1 weeks to 20.9 weeks, p = 0.000), in the number of relapses (reduced from 1.6 to 0.4 episodes/month, p = 0.001), and in the total duration of herpes symptoms per month (shortened from 2.0 to 0.3 weeks, p = 0.000). Placebo treatment also resulted in considerable improvement (p < 0.05 or 0.01), but was significantly inferior to the improvement obtained with thymopentin. The longest symptomfree period in the placebo group was prolonged from 2.4 to 11.2 weeks. The number of relapses per month was reduced from 1.4 to 0.8, and the total duration of herpes symptoms per month from 2 to 0.9 weeks. The results of intergroup analyses, in which the observed parameters and the improvement achieved in either group were compared, significantly favored thymopentin treatment. The effect of thymopentin was in all but one parameters superior to that of placebo and highly significant (p < 0.01). © 1985 Humana Press Inc.

We want to touch IT. Are hybrid laptops just new fancy gadgets or a new revolution?

While the number of traditional laptops and computers sold has dipped slightly year over year, manufacturers have developed new hybrid laptops with touch screens to build on the tactile trend. This market is moving quickly to make touch the rule rather than the exception and the sales of these devices have tripled since the launch of Windows 8 in 2012, to reach more than sixty million units sold in 2015. Unlike tablets, that benefit from easy-to-use applications specially designed for tactile interactions, hybrid laptops are intended to be used with regular user-interfaces. Hence, one could ask whether tactile interactions are suited for every task and activity performed with such interfaces. Since hybrid laptops are increasingly used in educational situations, this study focuses on information search tasks which are commonly performed for learning purposes. It is hypothesized that tasks that require complex and/or less common gestures will increase user's cognitive load and impair task performance in terms of efficacy and efficiency. A study was carried out in a usability laboratory with 30 participants for whom prior experience with tactile devices has been controlled. They were asked to perform information search tasks on an online encyclopaedia by using only the touch screen of and hybrid laptop. Tasks were selected with respect to their level of cognitive demand (amount of information that had to be maintained in working memory) and the complexity of gestures needed (left and/or right clicks, zoom, text selection and/or input.), and grouped into 4 sets accordingly. Task performance was measured by the number of tasks succeeded (efficacy) and time spent on each task (efficiency). Perceived cognitive load was assessed thanks to a questionnaire given after each set of tasks. An eye tracking device was used to monitor users' attention allocation and to provide objective cognitive load measures based on pupil dilation and the Index of Cognitive Activity. Each experimental run took approximately one hour. The results of this within-subjects design indicate that tasks involving complex gestures led to a lower efficacy, especially when the tasks were cognitively demanding. Regarding efficacy, there is no significant differences between sets of tasks excepted for tasks with low cognitive demand and complex gestures that required more time to be achieved. Surprisingly, users that declared the biggest experience with tactile devices spent more time than less frequent users. Cognitive load measures indicate that participants reported having devoted more mental effort in the interaction when they had to use complex gestures.

Seroprotection against tetanus in patients attending an emergency department in Belgium and evaluation of a bedside immunotest.

BACKGROUND: In most emergency departments, tetanus prophylaxis currently relies on vaccination history. Bedside evaluation of tetanus immunity may improve this process. OBJECTIVES: (i) To determine the seroprevalence of tetanus immunity; (ii) to evaluate the accuracy of vaccination history in assessing tetanus immunity; (iii) to identify factors predictive of seroprotection and incorrect history. METHOD: In a prospective observational study, tetanus immunity was assessed in 784 adults using Tétanos Quick Stick (TQS). A questionnaire was completed to obtain vaccination and general histories. Immunity assessed by TQS and by vaccination history were compared with anti-tetanus antibody levels measured by the enzyme-linked immunosorbent assay (seroprotection threshold >0.15 IU/ml). RESULTS: Overall, 64.2% of patients were protected according to TQS results. Four independent predictors of seroprotection were identified: young age, birthplace in Belgium, male sex and occupational medicine consultation. TQS performance was good: kappa=0.71, sensitivity 85.3%, specificity 87.2%, positive predictive value 92.1% and negative predictive value 77.2%. Seven hundred and sixty-two participants responded to the vaccination history: 23.4% said they were protected, 22.1% that they were not and 54.5% did not know. History performance was poor: kappa=0.27, sensitivity 60.3%, specificity 73.3%, positive predictive value 81.8% and negative predictive value 45.8%. Compared with history, TQS offered a significantly better sensitivity, negative and positive predictive values, but specificity was similar. No predictor of an incorrect history was identified. CONCLUSION: Lack of protective immunity against tetanus is frequent but poorly evaluated by history taking. Several demographic characteristics are good predictors of seroprotection. TQS could be a valuable tool in selected patients to improve tetanus prophylaxis in the emergency department.

Revisiting human IL-12Rβ1 deficiency: A survey of 141 patients from 30 countries

Interleukin-12 receptor β1 (IL-12Rβ1) deficiency is the most common form of Mendelian susceptibility to mycobacterial disease (MSMD). We undertook an international survey of 141 patients from 102 kindreds in 30 countries. Among 102 probands, the first infection occurred at a mean age of 2.4 years. In 78 patients, this infection was caused by Bacille Calmette-Guérin (BCG; n = 65), environmental mycobacteria (EM; also known as atypical or nontuberculous mycobacteria) (n = 9) or Mycobacterium tuberculosis (n = 4). Twenty-two of the remaining 24 probands initially presented with nontyphoidal, extraintestinal salmonellosis. Twenty of the 29 genetically affected sibs displayed clinical signs (69%); however 8 remained asymptomatic (27%). Nine nongenotyped sibs with symptoms died. Recurrent BCG infection was diagnosed in 15 cases, recurrent EM in 3 cases, recurrent salmonellosis in 22 patients. Ninety of the 132 symptomatic patients had infections with a single microorganism. Multiple infections were diagnosed in 40 cases, with combined mycobacteriosis and salmonellosis in 36 individuals. BCG disease strongly protected against subsequent EM disease (p = 0.00008). Various other infectious diseases occurred, albeit each rarely, yet candidiasis was reported in 33 of the patients (23%). Ninety-nine patients (70%) survived, with a mean age at last follow-up visit of 12.7 years ± 9.8 years (range, 0.5-46.4 yr). IL-12Rβ1 deficiency is characterized by childhood-onset mycobacteriosis and salmonellosis, rare recurrences of mycobacterial disease, and more frequent recurrence of salmonellosis. The condition has higher clinical penetrance, broader susceptibility to infections, and less favorable outcome than previously thought. © 2010 Lippincott Williams & Wilkins.