A Retrospective Study of Calcinosis Cutis in Patients With Systemic Lupus Erythematosus.

Calcinosis cutis (CC) is a very rare and poorly characterized finding in systemic lupus erythematosus (SLE).1 In this retrospective study, we present our experience of 10 individuals with SLE who developed CC, describing the epidemiology, diagnosis, and clinical characteristics of this rare entity.

Auditory event-related potentials and function of the medial olivocochlear efferent system in children with auditory processing disorders.

OBJECTIVE: The objectives were to investigate the function of central auditory pathways and of the medial efferent olivocochlear system (MOCS). DESIGN: Event-related potentials (ERP) were recorded following the delivery of the stimulus /da/ in quiet and in ipsilateral, contralateral, and binaural noise conditions and correlated to the results of the auditory processing disorders (APD) diagnostic test battery. MOCS function was investigated by adding ipsilateral, contralateral, and binaural noise to transient evoked otoacoustic emission recordings. Auditory brainstem responses and pure tone audiogram were also evaluated. STUDY SAMPLE: Nineteen children (7 to 12 years old) with APD were compared with 24 age-matched controls. RESULTS: Otoacoustic emissions and ABR characteristics did not differ between groups, whereas ERP latencies were significantly longer and of higher amplitudes in APD children than in controls, in both quiet and noise conditions. The MOCS suppression was higher in APD children. CONCLUSIONS: Findings indicate that children with APD present with neural deficiencies in both challenging and nonchallenging environments with an increase in the timing of several central auditory processes correlated to their behavioural performances. Meanwhile, their modulation of the auditory periphery under noisy conditions differs from control children with higher suppression.

Links between Daytime Napping, Night-Time Sleep Quality and Infant Attention: An Eye-Tracking, Actigraphy and Parent-Report Study.

The current study explored the potential influence of infant sleep, measured by parental report and actigraphy, and family functioning on attention development using eye tracking. The use of actigraphy in parallel with parental report, has the advantage of measuring participant's sleep throughout the night without parental observation and the ability to objectively assess sleep quality. An eye-tracking version of the Gap-Overlap task was used to measure visual attention. Questionnaires and behavioural assessment were used to assess family function, and general cognitive development. Fifty infants (Mean age = 13.44 months, SD = 3.10) participated in the study, 23 of which had full final datasets. Results show that daytime sleep duration, as measured by parental report, and proportion of light sleep at night, as measured by actigraphy, are linked to visual attention. A higher proportion of light sleep, a marker of poorer sleep quality, and less daytime sleep were negatively linked with facilitation and disengagement on the Gap-Overlap task. Family functioning was not associated with attention. The results provide initial evidence that in addition to the amount of daytime sleep; quality of night-time sleep as measured by proportion of light sleep, is a potentially useful sleep variable which requires further focus in the study of attention development.

Evaluation of the Effectiveness and Tolerability of Mycophenolate Mofetil and Mycophenolic Acid for the Treatment of Morphea.

Importance: First-line systemic therapy for morphea includes methotrexate with or without systemic corticosteroids. When this regimen is ineffective, not tolerated, or contraindicated, a trial of mycophenolate mofetil (MMF) or mycophenolic acid (MPA)-referred to herein as mycophenolate-is recommended; however, evidence to support this recommendation remains weak. Objective: To evaluate the effectiveness and tolerability of mycophenolate for the treatment of morphea. Design, Setting, and Participants: A retrospective cohort study was conducted from January 1, 1999, to December 31, 2018, among 77 patients with morphea from 8 institutions who were treated with mycophenolate. Main Outcomes and Measures: The primary outcome was morphea disease activity, severity, and response at 0, 3 to 6, and 9 to 12 months of mycophenolate treatment. A secondary outcome was whether mycophenolate was a well-tolerated treatment of morphea. Results: There were 61 female patients (79%) and 16 male patients (21%) in the study, with a median age at disease onset of 36 years (interquartile range, 16-53 years) and median diagnostic delay of 8 months (interquartile range, 4-14 months). Generalized morphea (37 [48%]), pansclerotic morphea (12 [16%]), and linear morphea of the trunk and/or extremities (9 [12%]) were the most common subtypes of morphea identified. Forty-one patients (53%) had an associated functional impairment, and 49 patients (64%) had severe disease. Twelve patients received initial treatment with mycophenolate as monotherapy or combination therapy and 65 patients received mycophenolate after prior treatment was ineffective (50 of 65 [77%]) or poorly tolerated (21 of 65 [32%]). Treatments prior to mycophenolate included methotrexate (48 of 65 [74%]), systemic corticosteroids (42 of 65 [65%]), hydroxychloroquine (20 of 65 [31%]), and/or phototherapy (14 of 65 [22%]). After 3 to 6 months of mycophenolate treatment, 66 of 73 patients had stable (n = 22) or improved (n = 44) disease. After 9 to 12 months of treatment, 47 of 54 patients had stable (n = 14) or improved (n = 33) disease. Twenty-seven patients (35%) achieved disease remission at completion of the study. Treatments received in conjunction with mycophenolate were frequent. Mycophenolate was well tolerated. Gastrointestinal adverse effects were the most common (24 [31%]); cytopenia (3 [4%]) and infection (2 [3%]) occurred less frequently. Conclusions and Relevance: This study suggests that mycophenolate is a well-tolerated and beneficial treatment of recalcitrant, severe morphea.

Cutaneous Lupus Erythematosus Patients With a Negative Antinuclear Antibody Meeting the American College of Rheumatology and/or Systemic Lupus International Collaborating Clinics Criteria for Systemic Lupus Erythematosus.

OBJECTIVE: Systemic lupus erythematosus (SLE) is a disorder that is heterogeneous and can be difficult to diagnose. One hallmark of the disease is the presence of antinuclear antibodies (ANAs), a feature that has been incorporated into multiple classification criteria over the years. In this study, we used a database of patients with cutaneous lupus erythematosus (CLE) to determine how many had a negative ANA and met criteria for SLE using the American College of Rheumatology (ACR) and/or Systemic Lupus International Collaborating Clinics (SLICC) criteria. METHODS: We used a database of 301 biopsy-proven CLE patients that contained information including ANA status and the presence of features of SLE. The database was searched for patients who had a negative ANA result and whether or not they met SLE criteria using the ACR and/or SLICC criteria. RESULTS: Of the 301 patients with biopsy-proven CLE and a known ANA, 111 had a negative ANA test (36.9%) and 27 had an ANA test that fluctuated (33.3%). In all, 20 ANA-negative patients met SLE criteria (18.0%), and 12 patients with a fluctuating ANA test met SLE criteria (44.4%). Of all patients who had either a negative or fluctuating ANA result and who met criteria for SLE (n = 32), 27 patients had involvement of ≥1 organ system other than skin (84.4%), and 13 patients had involvement of ≥2 organ systems other than skin (40.6%). CONCLUSION: Our results show that an ANA is not always present in patients with systemic disease. This fact should be taken into consideration when devising SLE classification criteria to be used for clinical trials.

Assessing the Correlation Between Disease Severity Indices and Quality of Life Measurement Tools in Pemphigus.

Pemphigus, an autoimmune blistering disease that affects the skin and mucous membranes, adversely impacts patients' quality of life (QOL). While there are various QOL measurement tools that can be used in this disease, few studies have assessed how a patient's change in disease severity can affect their QOL. This study aims to identify which disease severity index correlates best with the change in QOL. Fifty pemphigus patients completed QOL surveys with disease severity scored over two visits. QOL was assessed with the Autoimmune Bullous Disease Quality of Life (ABQOL), Dermatology Life Quality Index (DLQI), Skindex-29, and Short Form Survey 36 (SF-36). Disease severity was scored with the Pemphigus Disease Area Index (PDAI) and Autoimmune Bullous Skin Disorder Intensity Score (ABSIS). Correlations between the change in QOL scores and change in disease severity were analyzed using Spearman's coefficient (r). The change in PDAI showed a strong correlation (r = 0.60-0.79) with changes in the ABQOL, Skindex-29 symptoms (Skindex-S), and Skindex-29 functioning (Skindex-F) subscales for all patients (n = 50). For patients with mucosal disease (n = 24), the change in PDAI showed a strong correlation with changes in the ABQOL and Skindex-S subscale. For patients without mucosal disease, the change in PDAI showed a strong correlation with the Skindex-S. The change in ABSIS showed a strong correlation with Skindex-S for all patients and patients with no mucosal involvement, but showed no strong correlations for patients with mucosal involvement. The changes in PDAI always had a stronger correlation than the changes in ABSIS scores to changes in the ABQOL, DLQI, and Skindex-29 subscales, except where the PDAI and ABSIS scores were about the same for the Skindex-S subscale in patients with no mucosal involvement (r = 0.76 and r = 0.77, respectively). PDAI is superior to ABSIS in its correlation with validated QOL tools. The QOL tools that appear to be of most use in clinical trials and patient management are the Skindex-S and ABQOL.

From first symptoms to diagnosis of amyotrophic lateral sclerosis: perspectives of an Irish informal caregiver cohort-a thematic analysis.

OBJECTIVES: Informal caregivers play an integral part in the management of amyotrophic lateral sclerosis (ALS). The objective of this study was to explore the journey from first problem symptoms to diagnosis from the perspective of informal caregivers providing care to people with ALS. DESIGN: As part of a semistructured interview, information was collected on a range of caregiver demographic details, and from an open-ended question their experiences of the time of symptom onset to diagnosis. We carried out descriptive statistical analysis and thematic analysis of qualitative data. SETTING AND PARTICIPANTS: Home interviews with informal caregivers (n=74) of people with ALS attending the National ALS/Motor Neuron Disease Clinic at Beaumont Hospital, Dublin, Ireland. RESULTS: This was a largely female and spousal cohort of caregivers, living with the patient for whom they provided informal care. The majority of patients were men and were spinal onset. Caregivers described the time from first symptoms to diagnosis. Using a primarily inductive approach, the coding was data driven and the codes and themes derived from the content of these descriptions. Two main themes were identified (1) problem signs and symptoms (A) noticing and (B) reaction; (2) interaction with the health services. CONCLUSIONS: Exploring the perspectives of caregivers from first problem symptoms to diagnosis provides valuable insights into the development of the condition, impediments to its recognition, help-seeking behaviours and interactions with healthcare services. The journey from early symptoms to diagnosis is important for future decision-making, affects readiness for caregiving and could negatively impact on caregiver health and well-being. The early acknowledgement by healthcare professionals of stressors along the journey to diagnosis, and appreciation of their possible impact on caregivers is important. The separate needs of caregivers should be assessed on a regular basis.

Ear effect and gender difference of spontaneous otoacoustic emissions in children with auditory processing disorder.

Spontaneous otoacoustic emissions (SOAEs) are found in most healthy ears, and can be used to measure the health of the cochlear structures and feedback mechanism. According to existing literature, right ears tend to exhibit greater numbers of SOAEs than left ears (Bilger et al., 1990) and females tend to show higher incidence of SOAEs than males (Moulin et al., 1993). The SOAE prevalence has not been extensively studied in children with Auditory Processing Disorder (APD), a disorder with unknown etiology that reduces one's ability to process auditory information. This study examined the prevalence and ear advantage of SOAEs between genders in children diagnosed with APD. SOAEs were investigated in 19 children (7 girls and 12 boys) with APD and 2 4 typically developing children (14 girls and 10 boys) aged 7-12. Right ear advantage was more prevalent in control (71%) than APD subjects (42 %). However, over 30% more females exhibited a right ear advantage than males in each group. Although the results are not significant, our findings indicate that the lack of right ear advantage for SOAE is more prevalent in children with APD, particularly in males, suggesting that cochlear mechanisms or their control might be somehow affected in APD.

Pediatric temperature measurement and child/parent/nurse preference using three temperature measurement instruments.

This study compared the tympanic thermometer with the electronic and chemical dot thermometers used at the axillary site and evaluated child, parent, and nurse preferences for method of temperature measurement. The child's (n = 146) temperature was measured using each of the three methods. Each child, parent, and nurse was asked to select a preference for device and site. Results were analyzed using the Bland-Altman method. Results showed that most of the paired readings fell within the Bland-Altman limits of agreement (LOA). When the chemical dot and tympanic temperature readings were compared with the electronic axillary reading, the tympanic thermometer was found to be in closer agreement. The chemical dot thermometer placed in the axilla consistently read higher than the electronic thermometer in the same site. Children, parents, and nurses preferred the tympanic thermometer.