Search

1 3-hydroxy-5-trifluoromethyl-N-[2-(2-thienyl)-2-phenyl-ethenyl]- benzo(B) thiophene-2-carboxamide (L-652,343) is a 5-lipoxygenase and cyclo-oxygenase inhibitor in vitro. 2 In psoriasis increased concentrations of arachidonic acid transformation products are found in the lesional skin which may be important in the pathogenesis of the disease. We have measured the effect of orally administered L-652,343 on the concentration of LTB4 and prostaglandins in the lesional skin. 3 Eight patients with stable chronic plaque psoriasis received 500 and 250 mg of L-652,343, 12 h apart. A chamber technique was used to collect skin exudate samples from abraded plaques before and at 4, 24 and 48 h after the first dose. Exudates were analysed for LTB4 by a neutrophil chemokinesis assay and for PGE2 and PGD2 by RIA. 4 PGE2 and PGD2 levels were significantly reduced at 4 and 24 h after the first dose of L-652,343 but LTB4 levels were not affected indicating inhibition of the cyclo-oxygenase pathway but not of the 5-lipoxygenase pathway. This shows the importance of confirming that the action of 5- lipoxygenase inhibiting drugs in vitro occurs in vivo. [ABSTRACT FROM AUTHOR]

Monohydroxy acids (HETEs) and leukotriene B4 (LTB4) metabolites of arachidonic acid were measured in skin of healthy volunteers after ultraviolet B irradiation, and in the uninvolved skin of psoriatics after topical dithranol application. Exudate was collected from suction bullae on control and inflamed abdominal skin, and analysed for 12-HETE and PGE2 by GC-MS and LTB4 by bioassay. 12-HETE and PGE2 were raised at 24 h but not at 72 h after u.v.B irradiation: control and 24 h values were 13.7 and 41.5 ng ml-1 (P less than 0.05, n = 6) for 12-HETE respectively, and 4.5 and 30.2 ng ml-1 (P less than 0.01, n = 6) for PGE2. Dithranol application raised PGE2 levels from 23.1 ng ml-1 in control exudate to 62 ng ml-1 (P less than 0.01, n = 6) at 24 h before declining to base levels at 72 h. However, 12-HETE was raised at 72 h (200 ng ml-1, P less than 0.01, n = 5) but not at 24 h (104 ng ml-1) compared to control levels (50 ng ml-1, n = 5). The levels of the LTB4 were low (less than 100 pg ml-1), and no significant increases were observed. Arachidonic acid in inflamed skin can be metabolised by the cyclo-oxygenase and lipoxygenase pathway. It is probable that the lipoxygenase product 12-HETE is involved in these inflammatory reactions. [ABSTRACT FROM AUTHOR]

1 Clinically normal human abdominal skin in 11 subjects, was irradiated with three times the minimal-erythema dose of ultraviolet B (u.v. B) irradiation. 2 Erythema was assessed visually, and exudate was recovered by a suction bulla technique from normal skin, and from skin at 6, 24 and 48 h after irradiation. 3 Erythema was moderate at 6 h, but increased to a maximum at 24 h, which was maintained at 48 h. 4 6- oxo-PGF1 alpha was markedly raised at 6 h, moderately raised at 24 h, but had returned to control levels at 48 h, when the erythema was still maximal. 5 Prostaglandin I2, the precursor of 6-oxo-PGF1 alpha and 6- oxo-PGF1 alpha may, therefore, play a part in the early inflammatory process after u.v. B irradiation, but is unlikely to be responsible for the erythema still present at 48 h. [ABSTRACT FROM AUTHOR]

1 Corticosteroids are known to suppress evoked histamine release from human skin in vitro. Therefore the effect of prednisone, on cold evoked histamine release and clinical response was studied in six patients with acquired cold urticaria. 2 Following cold challenge all six patients developed an immediate urticarial response of the forearm and elevation of histamine levels, measured by superfusion cascade bioassay of the venous blood draining the forearm. 3 After treatment with prednisone, in an oral dose of 20-25 mg for 1 to 5 days, a suppression of evoked histamine release occurred in all but one patient. However, there was no corresponding decrease of erythema or oedema, though pruritus was improved in most patients. 4 The lack of clinical improvement in erythema and oedema, despite reduction in histamine levels, suggests that histamine may not be the principal mediator of the vascular response to cold challenge in acquired cold urticaria. [ABSTRACT FROM AUTHOR]

1 Clinically normal human abdominal skin was irradiated with three minimal-erythema doses of ultraviolet B irradiation, (u.v.B). 2 Erythema was assessed visually, and exudate recovered by a suction bulla technique from normal skin, and at 10 min, 2, 6, 18, 24 and 48 h after irradiation. 3 Erythema was barely visible at 2 h, but increased to maximum at 24 h, which was maintained at 48 h. 4 Increased arachidonic acid and prostaglandin E2, F2 alpha and D2 concentrations in the exudate, measured by gas chromatography-mass spectrometry, accompanied the developing erythema, with the maximal rise of arachidonic acid, prostaglandin E2 and D2 occurring at the height of the erythema at 24 h. 5 At 48 h, still at the height of the erythemal response, arachidonic acid and PGE2 levels had returned to near normal. 6 Concentrations of arachidonic acid and of its products from the cyclo- oxygenase pathway, parallel the development of i.u.B. erythema in the first 24 h. [ABSTRACT FROM AUTHOR]

Fifty shoulders in 46 patients underwent stabilization surgery for multidirectional instability. Univariate analysis showed no significant differences (P>0.05) for age, gender, or workers' compensation in patient satisfaction or American Shoulder and Elbow Surgeons (ASES) score. Only the ASES score was lower with prior surgery (P=0.001). There was a significantly increased ASES score (P<0.05) for arthroscopic versus open treatment. Subjective variable analysis showed that satisfaction and ASES score were significantly associated with questions regarding pain, instability, and upper extremity use (P<0.01). Increased range of motion showed a trend toward higher ASES scores (P<0.074). Patient satisfaction (P=0.013) was associated with greater forward elevation and greater external rotation (P=0.056). Multivariate analysis (P<0.05) showed that independent determinants of patient satisfaction with outcome included change in instability symptoms and ASES score. Subjective variables of symptoms and motion had the greatest correlation with patient satisfaction and ASES score following surgery for multidirectional shoulder instability. To improve patient satisfaction, an expanded focus on these subjective points may be beneficial.Full article available online at OrthoSuperSite.com/view.asp?rID=28894 [ABSTRACT FROM AUTHOR]

From early on, infants show a preference for infant-directed speech (IDS) over adult-directed speech (ADS), and exposure to IDS has been correlated with language outcome measures such as vocabulary. The present multi-laboratory study explores this issue by investigating whether there is a link between early preference for IDS and later vocabulary size. Infants' preference for IDS was tested as part of the ManyBabies 1 project, and follow-up CDI data were collected from a subsample of this dataset at 18 and 24 months. A total of 341 (18 months) and 327 (24 months) infants were tested across 21 laboratories. In neither preregistered analyses with North American and UK English, nor exploratory analyses with a larger sample did we find evidence for a relation between IDS preference and later vocabulary. We discuss implications of this finding in light of recent work suggesting that IDS preference measured in the laboratory has low test-retest reliability.

The Fontan procedure is used to palliate complex forms of congenital heart disease. This results in adverse hepatic sequelae now known as Fontan-associated liver disease (FALD). Conventional laboratory measures of liver disease do not correlate well with FALD severity. Cytokeratin-18 (CK-18) is a measure of cell death and is sensitive in detecting other causes of liver disease. Our aim was to assess the use of a novel measure of liver disease, CK-18, in Fontan patients. This is a single-center, prospective, cross-sectional study of Fontan patients aged 8-21 years old. We performed ultrasound elastography, echocardiography, magnetic resonance imaging, and serum laboratory testing. Novel laboratory test CK-18 levels in Fontan subjects were compared to healthy age-matched controls. Thirteen Fontan patients were evaluated with a median age 15 years (10, 14), 4 Hypoplastic left heart syndrome, 11 were male, and 5 were symptomatic. Fontan patients had normal AST/ALT, but a significantly elevated liver stiffness by elastography (median 13.4 kPa). Hepatic stiffness by elastography was associated with diastolic-indexed (rho = 0.58, p = 0.04) ventricular volumes. Compared to 10 aged-matched controls, CK-18 was higher in the Fontan group-cleaved CK-18 protein (p < 0.01) and full CK-18 protein, (p = 0.02). CK-18 was positively associated with AST and ALT. Elevated CK-18 levels were found in Fontan patients compared to controls suggesting hepatic cell death even in these relatively healthy Fontan patients. CK-18 was elevated prior to changes in traditional testing. CK-18 may be a useful sensitive marker of liver disease in FALD., (© 2024. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

There is substantial evidence that infants prefer infant-directed speech (IDS) to adult-directed speech (ADS). The strongest evidence for this claim has come from two large-scale investigations: i) a community-augmented meta-analysis of published behavioral studies and ii) a large-scale multi-lab replication study. In this paper, we aim to improve our understanding of the IDS preference and its boundary conditions by combining and comparing these two data sources across key population and design characteristics of the underlying studies. Our analyses reveal that both the meta-analysis and multi-lab replication show moderate effect sizes ( d ≈ 0.35 for each estimate) and that both of these effects persist when relevant study-level moderators are added to the models (i.e., experimental methods, infant ages, and native languages). However, while the overall effect size estimates were similar, the two sources diverged in the effects of key moderators: both infant age and experimental method predicted IDS preference in the multi-lab replication study, but showed no effect in the meta-analysis. These results demonstrate that the IDS preference generalizes across a variety of experimental conditions and sampling characteristics, while simultaneously identifying key differences in the empirical picture offered by each source individually and pinpointing areas where substantial uncertainty remains about the influence of theoretically central moderators on IDS preference. Overall, our results show how meta-analyses and multi-lab replications can be used in tandem to understand the robustness and generalizability of developmental phenomena., Competing Interests: Competing Interests: The authors declare no conflict of interests., (© 2024 Massachusetts Institute of Technology.)

Purpose: To explore the associations between race and retinoblastoma diagnosis in United States children., Methods: In this analytical nonconcurrent cohort study, we used 1988-2018 data from the Surveillance, Epidemiology, and End-Results (SEER) database. Children ages 0-17 with retinoblastoma were included (n = 758); those with missing data were excluded (n = 11; final cohort: n = 747). The exposure variable was race (White, Black, Asian/Pacific Islanders, American Indian/Alaska Native), and the outcome variable was diagnosis of retinoblastoma before versus after 2 years of age. Covariates included sex, rural-urban continuum, ethnicity, decade of diagnosis, and laterality of disease. Unadjusted and adjusted logistic regression analyses were performed to calculate odds ratios and 95% confidence intervals., Results: No statistically significant association was found between racial/ethnic groups (OR = 0.61-0.99; P = 0.92) and age at diagnosis (OR = 0.86; P = 0.66). Females were more likely to be diagnosed earlier than males (OR = 0.62; 95% CI, 0.44-0.88; P = 0.042). No association was found between urban versus rural subjects (OR = 1.02; 95% CI, 0.60-1.75) or between decades (OR = 0.81; 95% CI, 0.54-1.22 and OR 0.96; 95% CI, 0.62-1.47)., Conclusions: We found no statistically significant difference between racial/ethnic groups for diagnosis of children with retinoblastoma after 2 years of age. Future studies could explore why females are more likely than males to be diagnosed before 2 years of age., (Copyright © 2024 American Association for Pediatric Ophthalmology and Strabismus. Published by Elsevier Inc. All rights reserved.)

The efficiency of spoken word recognition is essential for real-time communication. There is consensus that this efficiency relies on an implicit process of activating multiple word candidates that compete for recognition as the acoustic signal unfolds in real-time. However, few methods capture the neural basis of this dynamic competition on a msec-by-msec basis. This is crucial for understanding the neuroscience of language, and for understanding hearing, language and cognitive disorders in people for whom current behavioral methods are not suitable. We applied machine-learning techniques to standard EEG signals to decode which word was heard on each trial and analyzed the patterns of confusion over time. Results mirrored psycholinguistic findings: Early on, the decoder was equally likely to report the target (e.g., baggage) or a similar sounding competitor (badger), but by around 500 msec, competitors were suppressed. Follow up analyses show that this is robust across EEG systems (gel and saline), with fewer channels, and with fewer trials. Results are robust within individuals and show high reliability. This suggests a powerful and simple paradigm that can assess the neural dynamics of speech decoding, with potential applications for understanding lexical development in a variety of clinical disorders., (Copyright © 2022. Published by Elsevier Inc.)

Background: Food insecurity (FI) increases children's risk for illness and developmental and behavioral problems, which are ongoing concerns for congenital heart disease (CHD) patients. In 2020, 14.8% of households with children suffered from FI. The Hunger Vital Signs (HVS) asks 2 questions to assess FI. The global aim of the project is to implement HVS and connect FI families to resources., Methods: Stakeholders identified 6 critical drivers in implementing FI screening at an outpatient cardiology clinic and conducted plan-do-study-act (PDSA) cycles to implement HVS. Over the 13-month study period, time series analyses were performed to assess our process measure (FI screening) and outcome measure (connection of FI families to resources). Demographics and severity of CHD were analyzed for FI families., Results: Screening rates increased from 0% to >85%, screening 5064 families. Process evaluations revealed roadblocks including screening discomfort. FI families were more likely to identify as Black or multiple or other ethnicity. Severe CHD patients were at higher risk for FI (n = 106, odds ratio [OR] 1.67 [1.21-2.29], P = .002). Face-to-face meetings with social work and community partnerships reduced loss to follow-up and our ability to offer all FI families individualized FI resources., Conclusion: HVS screening can be implemented in a cardiology clinic to improve identification of FI families. A written tool can combat screening discomfort and improve identification of FI families. Children with severe CHD may be at increased risk for FI. A multidisciplinary team and community partnerships can improve individualized resource distribution., (Copyright © 2022 by the American Academy of Pediatrics.)

From the earliest months of life, infants prefer listening to and learn better from infant-directed speech (IDS) than adult-directed speech (ADS). Yet, IDS differs within communities, across languages, and across cultures, both in form and in prevalence. This large-scale, multi-site study used the diversity of bilingual infant experiences to explore the impact of different types of linguistic experience on infants' IDS preference. As part of the multi-lab ManyBabies 1 project, we compared lab-matched samples of 333 bilingual and 385 monolingual infants' preference for North-American English IDS (cf. ManyBabies Consortium, 2020: ManyBabies 1), tested in 17 labs in 7 countries. Those infants were tested in two age groups: 6-9 months (the younger sample) and 12-15 months (the older sample). We found that bilingual and monolingual infants both preferred IDS to ADS, and did not differ in terms of the overall magnitude of this preference. However, amongst bilingual infants who were acquiring North-American English (NAE) as a native language, greater exposure to NAE was associated with a stronger IDS preference, extending the previous finding from ManyBabies 1 that monolinguals learning NAE as a native language showed a stronger preference than infants unexposed to NAE. Together, our findings indicate that IDS preference likely makes a similar contribution to monolingual and bilingual development, and that infants are exquisitely sensitive to the nature and frequency of different types of language input in their early environments., Competing Interests: Conflicts of Interest The authors declare that there were no conflicts of interest with respect to the authorship or the publication of this article.

Determining the meanings of words requires language learners to attend to what other people say. However, it behooves a young language learner to simultaneously encode relevant non-verbal cues, for example, by following the direction of their eye gaze. Sensitivity to cues such as eye gaze might be particularly important for bilingual infants, as they encounter less consistency between words and objects than monolingual infants, and do not always have access to the same word-learning heuristics (e.g., mutual exclusivity). In a preregistered study, we tested the hypothesis that bilingual experience would lead to a more pronounced ability to follow another's gaze. We used a gaze-following paradigm developed by Senju and Csibra (Current Biology, 18, 2008, 668) to test a total of 93 6- to 9-month-old and 229 12- to 15-month-old monolingual and bilingual infants, in 11 laboratories located in 8 countries. Monolingual and bilingual infants showed similar gaze-following abilities, and both groups showed age-related improvements in speed, accuracy, frequency, and duration of fixations to congruent objects. Unexpectedly, bilinguals tended to make more frequent fixations to on-screen objects, whether or not they were cued by the actor. These results suggest that gaze sensitivity is a fundamental aspect of development that is robust to variation in language exposure., (© 2020 International Congress of Infant Studies.)

The discovery of words in continuous speech is one of the first challenges faced by infants during language acquisition. This process is partially facilitated by statistical learning, the ability to discover and encode relevant patterns in the environment. Here, we used an electroencephalogram (EEG) index of neural entrainment to track 6-month-olds' ( N = 25) segmentation of words from continuous speech. Infants' neural entrainment to embedded words increased logarithmically over the learning period, consistent with a perceptual shift from isolated syllables to wordlike units. Moreover, infants' neural entrainment during learning predicted postlearning behavioral measures of word discrimination ( n = 18). Finally, the logarithmic increase in entrainment to words was comparable in infants and adults, suggesting that infants and adults follow similar learning trajectories when tracking probability information among speech sounds. Statistical-learning effects in infants and adults may reflect overlapping neural mechanisms, which emerge early in life and are maintained throughout the life span.

Aim: Recombinant secreted frizzled-related protein 5 (sFRP5) improved periodontal status in mice. Thus, this study aimed to investigate this finding in human periodontitis using an epidemiological approach., Materials and Methods: sFRP5 and wnt5a concentrations were determined in human serum from the Food Chain Plus cohort using ELISAs. A total of 128 patients with periodontitis and tooth loss and 245 patients with periodontitis without tooth loss were compared to 373 sex-, smoker-, age- and BMI-matched individuals in a nested case-control design., Results: Systemic sFRP5 serum levels were significantly lower in patients with periodontitis and tooth loss (2.5 [0.0-10.4] ng/ml, median [IQR]) compared to patients with periodontitis without tooth loss (6.0 [2.5-15.8] ng/ml, median [IQR], p = 0.04] and matched controls (7.0 [2.5-18.3] ng/ml, median [IQR], p = 0.02). No significant differences in sFRP5 serum levels were found among patients with periodontitis without tooth loss (6.0 [2.5-15.8] ng/ml, median [IQR]) and controls (3.1 [0.0-10.6] ng/ml, median [IQR], p = 0.06)., Conclusions: sFRP5 might serve as a novel biomarker for periodontitis severity. Modulating the inflammatory background of severe forms of periodontitis, in the time of precision medicine, needs to be revealed in further studies., (© 2019 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.)

This study explores whether children can learn a structural processing bias relevant to pronoun interpretation from brief training. Over three days, 42 five-year-olds were exposed to narratives exhibiting a first-mentioned tendency. Two characters were introduced, and the first-mentioned was later described engaging in a solo activity. In our primary condition of interest, the Gesture Training condition, the solo-activity sentence contained an ambiguous pronoun, but co-speech gesture clarified the referent. There were two comparison conditions. In the Gender Training condition the characters were different genders, thereby avoiding ambiguity. In the Name Training condition, the first-mentioned name was simply repeated. Ambiguous pronoun interpretation was tested pre- and post-training. Children in the Gesture condition were significantly more likely to interpret ambiguous pronouns as the first-mentioned character after training. Results from the comparison conditions were ambiguous: there was a small but non-significant effect of training, but also no significant differences between conditions.

Deep medial collateral ligament (MCL) injury leads to meniscal lift-off and extrusion of the medial meniscus, resulting in instability and increased medial compartment pressures with subsequent cartilage damage. Repair of the deep MCL meniscotibial ligament in concert with superficial MCL repair or reconstruction is intended to restore the native anatomy , stability, and function of the medial meniscus. We present an arthroscopically assisted technique using standard arthroscopy portals and a medial open approach.

A 5-month-old previously healthy female presented with a one-week history of fever and increased fussiness. Her presentation revealed an ill-appearing infant with an exam and cerebrospinal fluid (CSF) studies concerning bacterial meningitis; CSF cultures grew Pasteurella multocida . Additionally, brain magnetic resonance imaging (MRI) demonstrated cervical osteomyelitis. Despite multiple days of antibiotic therapy, she remained febrile with continued pain; MRI showed oligoarticular effusions, and aspiration of these joints yielded bloody aspirates. Evaluations for coagulopathy and immune complex-mediated arthropathy were negative. The patient improved following appropriate antibiotic therapy and spontaneous resolution of hemarthroses, and was discharged to a short-term rehabilitation hospital. P. multocida is a small, encapsulated coccobacillus that is part of the commensal oral flora of animals. It most commonly causes skin infections in humans, yet is a rare cause of meningitis in the pediatric population, especially in children <1 year of age. Transmission due to P. multocida is most commonly due to direct contact with animals. To our knowledge, this is the first case of oligoarticular hemarthroses and cervical osteomyelitis complicating P multocida meningitis. This case highlights the physician's potential for cognitive bias and premature anchoring, and the resulting implications in delivering excellent patient care.

The objective of this study was to assess whether migraine and tension-type headache (TTH) are best viewed as discrete entities or points on a severity continuum using taxometric analysis. Historically, classification systems have conceptualized the primary headache disorders of migraine and TTH as fundamentally different disorders that are differentiated by their characteristic symptom profiles and, as such, imply differing pathophysiologies and required treatments. Despite this categorical nosology, findings continue to emerge suggesting that migraine and TTH instead reflect dimensions of severity within the same headache construct. However, few studies have assessed this issue using taxometric statistical analyses or investigated how this taxonomic structure varies as a function of age and headache frequency. We conducted a latent-mode factor analysis of headache symptomatology obtained from 3449 individuals with headache from 2 previous, large-scale cross-sectional studies of primary headache sufferers (Martin et al., 2005, and Smitherman and Kolivas, 2013). Stratified taxometric analyses suggest that the validity of a categorical vs dimensional classification varies as a function of sample characteristics. Specifically, graphical results revealed that high headache frequency (>15 d/mo) and younger age (<24 years old) were associated with unimodal distributions suggestive of a dimensional construct of primary headache, whereas lower headache frequency and older age were associated with bimodal distributions characteristic of discrete diagnostic entities. Conceptualizing primary headache as a severity continuum was supported for young adults and those with frequent headaches. The distinctions of a categorical classification system were supported for adults (>24 years old) and those with infrequent headache.

Background: Recurrent headache sufferers are often fearful of pain, which disrupts thought processes, interferes with daily activities, and may maintain headache-related disability through avoidance and associated negative reinforcement., Objective: The aim of this cross-sectional study was to (1) examine differences in fear of pain (FOP) between headache sufferers and non-headache controls; (2) examine differences in FOP across primary headache diagnostic groups; (3) assess the extent to which FOP predicts headache variables (eg., severity, frequency, disability); and (4) determine whether FOP mediates the relationship between pain severity and headache-related disability., Methods: The sample consisted of 908 young adults (M age = 19.5 years; 64.9% female). Of those, 237 (26.1%) met the diagnostic criteria for episodic tension-type headache (TTH), 232 (25.6%) for episodic migraine (167 [18.4%] without aura and 65 [7.2%] with aura), 38 (4.2%) for chronic migraine, and 19 (2.1%) for chronic TTH; 382 (42.1%) served as non-headache controls., Results: FOP differed among groups, with headache sufferers reporting greater FOP than those without headache; migraineurs typically endorsed greater FOP than those with TTH. Among those with headache, FOP significantly predicted headache severity (R(2)  = 6.1%) and frequency (R(2)  = 4.5%), and accounted for more variance in disability (R(2)  = 17.5%) than gender, anxiety, and depression combined (13.8%). Pain severity and disability were strongly associated (r = 0.61, P < .001), and FOP partially mediated this association (indirect effect point estimate = 0.38; 95% confidence interval: 0.23-0.57)., Conclusions: FOP differentiates migraineurs from those without headache and plays a significant role in primary headache, particularly in headache-related disability. Findings build upon and extend those from previous chronic pain studies and highlight the need for longitudinal and experimental studies to further explore this construct in headache., (© 2015 American Headache Society.)

Opinion Statement: Posttraumatic stress disorder (PTSD) is often comorbid with chronic migraine (CM) and chronic tension-type headache (CTTH). Trauma-focused cognitive behavioral psychotherapies, selective serotonin reuptake inhibitors (SSRIs), and venlafaxine have demonstrated efficacy in the treatment of PTSD. Amitriptyline, topiramate, sodium valproate, and botulinum toxin A are efficacious for treatment of chronic daily headache (CDH). Treatment studies on individuals with CDH and comorbid PTSD, however, are limited. As such, multiple therapeutic agents or modes of interventions typically are necessary, such that comprehensive treatment simultaneously utilizes approaches with established efficacy for each individual condition.

Objective: Infants with "single ventricle" congenital heart disease are at high risk for sudden death following palliative surgical management. We developed a pilot telemedicine project to evaluate the feasibility of using Web-based daily reporting of clinical data with the goal of reducing unexpected admissions and sudden death., Subjects and Methods: We enrolled 9 subjects (enrolled subjects [ES]) following surgical palliation over 12 months. Parents electronically transmitted ES daily weight and oxygen saturation and then completed an automated 10-point phone questionnaire on nutrition, activity, and distress. Subject enrollment continued until a second surgical palliative procedure (n=5), sudden death (n=2), or disenrollment (n=2). We collected clinical data on all ES and 9 historical controls (HC) from the preceding 18 months and analyzed clinical management, including outpatient telephone surveillance success, scheduled and unscheduled office and emergency department visits, hospitalizations, procedures, and adverse events, including death., Results: Subject recruitment was more difficult than expected. Weight transmission success was high, but there was poor correlation between telemedicine system-measured oxygen saturation and a commercial monitor. The outpatient clinical telephone surveillance success rate for HC and ES was approximately 30%. After technical adjustments, parents of all ES (100%) were able to transmit questionnaire data. There were 9 emergency room visits for ES versus 11 unscheduled emergency room visits for HC. Sudden death occurred in 1 of 9 HC and 2 of 9 ES., Conclusions: Telemedicine monitoring for high-risk congenital heart disease patients is feasible but challenging, may reduce unscheduled visits, but may not impact the primary end point of preventing sudden death in this high-risk pediatric population.

Delayed pressure urticaria is a mechanical urticaria in which pressure causes whealing. Delayed cutaneous erythema and edema occur in association with marked subcutaneous swelling after the application of a sustained pressure stimulus to the skin. The earliest reports and theories of the pathogenesis of delayed pressure urticaria are summarized. Detailed attention is given to making the diagnosis by taking a history and provoking the lesions. The clinical features and natural history are considered. The effects of the disorder on quality of life are delineated, and management strategies are suggested.

Background: Circulating autoantibodies against FcepsilonRI, IgE, or both occur in approximately one third of patients with chronic idiopathic urticaria (CIU), but not all autoantibodies initiate histamine release., Objective: We sought to classify patients with CIU into subsets on the basis of serum bioactivity and immunoreactivity and to examine the relationship between newly defined subtype and disease severity., Methods: Sera from patients with CIU (n = 78), dermog-raphism (n = 15), and cholinergic urticaria (n = 10) and sera from healthy subjects (n = 39) were analyzed by means of Western blot analysis for anti-FcepsilonRI autoantibodies and for histamine release from basophils and dermal mast cells. In vivo reactivity of autologous serum was tested by means of intradermal injection, and CIU severity was determined on the basis of clinical interview., Results: We classified sera from patients with CIU into 5 subsets: immunoreactive histamine-releasing anti-FcepsilonRI autoantibodies (n = 20 [26%]); immunoreactive anti-FcepsilonRI autoantibodies without histamine-releasing activity (n = 12 [15%]); anti-IgE-like autoantibodies (n = 7 [9%]); serum containing a mast cell-specific histamine-releasing factor (n = 7 [9%]); and sera with no identifiable factor (n = 32 [41%]). Patients with serum histamine-releasing activity had more severe urticaria than patients without such activity. Positive skin test responses to autologous sera were associated with histamine-releasing anti-FcepsilonRI autoantibodies but not with non-histamine-releasing anti-FcepsilonRI autoantibodies. Neither healthy subjects nor patients with dermographism or cholinergic urticaria had his-tamine-releasing anti-FcepsilonRI autoantibodies., Conclusion: These data support the specificity of functional anti-FcepsilonRI autoantibodies to CIU. The identification of distinctive subsets of patients suggests that other pathogenic mechanisms occur in CIU in addition to direct ligation of FcepsilonRI by autoantibodies causing dermal mast cell degranulation. Elucidating these mechanisms might lead to new treatments for CIU.

Background: Few studies have examined the relation of iron status to diet in populations from developing countries with high levels of iron deficiency and diets of poor quality., Objective: The objective was to identify nutrients, dietary constituents, and foods that are associated with better iron status in a rural Mexican population., Design: A prospective cohort study was conducted in rural central Mexico. The subjects were 125 nonpregnant women aged 16-44 y. During the 12 mo before blood collection, food intakes were assessed repeatedly by a combination of dietary recalls, food weighing, and food diaries [mean (+/-SD) days of food intake data: 18.8 +/- 5.9 d]. Hemoglobin, hematocrit, and plasma ferritin were measured at the end of the study., Results: Higher plasma ferritin concentrations were associated with greater intakes of nonheme iron and ascorbic acid after control for age, BMI, breast-feeding, season, and the time since the birth of the last child. Higher ascorbic acid intakes, but not higher intakes of heme and nonheme iron, predicted a lower risk of low hemoglobin and hematocrit values after control for the background variables. Consumption of the alcoholic beverage pulque predicted a lower risk of low ferritin and low hemoglobin values. Seasonal variation in ferritin, hemoglobin, and hematocrit values was observed., Conclusion: Better iron status was associated with greater intakes of foods containing nonheme iron and ascorbic acid. PULQUE:a beverage containing iron, ascorbic acid, and alcohol-may influence the iron status of women in rural central Mexico.

H1-antihistamines are the cornerstone of symptomatic treatment in acute and chronic urticaria, in which they not only relieve itching, but also reduce the number, size, and duration of urticarial lesions. Relief of whealing, flaring, and erythema may be incomplete as the vascular effects of histamine are mediated to its action at H2-receptors as well as at H1-receptors, and other vasoactive substances may also be involved. In randomized, prospective, placebo-controlled, double-blind studies, the new low-sedating H1-antihistamines have been found to be effective and safe in urticaria. Sedating antihistamines, although effective, place patients at risk for adverse effects, including decreased psychomotor performance. The response to H1-antihistamines in some types of urticaria, for example, in urticarial vasculitis, is unsatisfactory. An H2-antihistamine administered concurrently with an H1-antihistamine may modestly enhance relief of itching and wheal formation in some patients with urticaria refractory to treatment with an H1-antihistamine alone. The available evidence does not justify the routine addition of H2-antihistamine treatment to H1-antihistamine treatment.

Typical urticarial lesions are transient cutaneous swellings of sudden onset, often itchy, persisting for less than 24 hours and resolving to leave normal appearing skin. Angioedema lesions are similar subcutaneous lesions. Atypical urticarias persist for longer than 24 hours, may be painful and bruised in appearance and accompanied with severe systemic symptoms. Conditions where prolonged weals are present include delayed pressure urticaria and urticarial vasculitis. These conditions do not respond well to antihistamine therapy. In delayed pressure urticaria, weals appear after a delay of hours at sites of sustained pressure on the skin and occur in association with ordinary chronic 'idiopathic' urticaria. Weals of urticarial vasculitis show histological features of venulitis, and can be accompanied by arthralgia and abdominal pain. Rarely, the condition is due to infective or autoimmune disease. Urticarial diseases, sometimes with features of urticarial vasculitis, and with associated systemic features include Schnitzler's Syndrome, Still's disease and Muckle-Wells syndrome. The latter syndrome is linked with chromosome 1q44, as is autosomal dominant cold urticaria, an unusual physical urticaria. Persistent cholinergic erythema, a variant of cholinergic urticaria, has been mistaken for a drug eruption or cutaneous mastocytosis. Rarely, food and exercise induced urticaria and anaphylaxis occur when exercise follows a specific food or any meal within a few hours. The early stages of inflammatory disease may be mistaken for urticaria and angioedema, but lesions usually persist for longer than 48 hours and are accompanied by epidermal changes.

Chronic idiopathic urticaria (CIU) may be severe and refractory to standard therapies. We describe two patients with CIU, neither of whom had detectable autoantibodies, in whom control of the disease was achieved with methotrexate.

Urticaria is a common skin condition. Although an episode may be mild and last only a few days, chronic urticaria can significantly affect the quality of life. The condition is frequently misunderstood by patients who believe the condition is always the result of an allergy and is dangerous.

We report two cases of Schnitzler's syndrome in which anti-interleukin-1alpha autoantibodies and functional autoantibodies against the high affinity IgE receptor (FcepsilonRIalpha) or against IgE were absent. One patient responded well to TL-01 phototherapy, a treatment which may be considered in patients with Schnitzler's syndrome if, as is usually the case, they are unresponsive to antihistamine therapy.

Background: Previous studies defining the histopathologic features of patients with chronic idiopathic urticaria (CIU) were performed on wheals of uncertain duration and before the identification of functional autoantibodies against FcepsilonRI and/or IgE, now known to be present in approximately 30% of patients with CIU., Objective: We sought to determine the timing of the inflammatory infiltrate in the wheals of patients with CIU and to detect differences between patients with and without autoantibodies., Methods: Immunohistochemistry was used to identify neutrophils (neutrophil elastase), T lymphocytes (CD3), and activated eosinophils (EG2) in biopsy specimens from uninvolved skin and wheals present for less than 4 hours and greater than 12 hours in 22 patients with CIU, as well as in biopsy specimens from the skin of 12 healthy control subjects. Patients were identified as having functional autoantibodies on the basis of their serum-evoked histamine release in vitro from the basophils of 2 healthy donors., Results: EG2(+), neutrophil elastase+, and, to a lesser extent, CD3(+) cells were found in greater numbers in wheals undergoing biopsy at less than 4 and greater than 12 hours than in uninvolved skin (P <.05). Patients without autoantibodies (n = 12) had significantly more EG2(+) cells in wheals of greater than 12 hours' duration than patients with autoantibodies (n = 10; P =.02). There was no other difference between patients with and without autoantibodies in the cutaneous cellular infiltrate., Conclusion: Neutrophil and eosinophil accumulation occurs early in the evolution of a wheal in patients with CIU, but eosinophil activation may occur later or be more persistent in patients without autoantibodies.

Background: Previous studies defining the clinical features of patients with chronic idiopathic urticaria (CIU) were performed before the identification of functional autoantibodies against FcepsilonRI and/or IgE, now known to be present in approximately 30% of patients with CIU., Objective: Our purpose was to determine whether there are differences between patients with and those without autoantibodies in the clinical features or severity of CIU., Methods: The clinical features of 107 patients with CIU were evaluated prospectively. Patients were identified as having functional autoantibodies on the basis of the serum-evoked histamine release in vitro from the basophils of 2 healthy donors., Results: Patients with autoantibodies (31%) had more wheals (P = .005), a wider distribution of wheals (P = .009), higher itch scores for the most severe episodes of itching (P = .002), more systemic symptoms (P = .03), and lower serum IgE levels (P < .0005) than patients without autoantibodies., Conclusion: The presence of autoantibodies indicates a subset of patients with more severe CIU.