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The urinary cystatin C/creatinine ratio was compared between children with or without tubular toxicity, according to a standard assessment of tubular dysfunction. However, although the urinary cystatin C/creatinine ratio was increased in children with tubular toxicity, this marker does not provide additional information to the well-known markers of tubulopathy.

Monitoring of plasma cystatin C may be substituted to radionucleide glomerular exploration in children treated by cisplatin and/or ifosfamide.

Monitoring of plasma cystatin C may be substituted to radionucleide glomerular exploration in children treated by cisplatin and/or ifosfamide.

This study aims to assess surgical outcomes and survival following first, second and third pelvic exenterations for pelvic malignancy.

Consecutive patients undergoing pelvic exenteration for pelvic malignancy at a quaternary referral centre from January 1994 and December 2017 were included. Demographics and surgical outcomes were compared between patients who underwent first, second and third pelvic exenterations by generalized mixed modelling with repeated measures. Survival was assessed using Cox proportional hazards models and Kaplan-Meier plots.

Of the 642 exenterations reviewed, 29 (4.5%) were second and 6 (0.9%) were third exenterations. Patients selected for repeat exenteration were more likely to have asymptomatic local recurrences detected on routine surveillance (P<0.001). Postoperative wound complications increased with repeat exenteration (6%, 17%, 33%; P=0.003, respectively). Additionally, postoperative length of stay increased from 27 to 38 and 48days, respectively (P=0.004). Median sur, these data indicate that repeat exenteration does not afford a survival advantage compared with patients having a single exenteration. These data suggest that repeat exenteration for recurrent pelvic malignancy may be of questionable therapeutic value.

Psychological comorbidities have been associated with asthma in adults and children, but have not been studied in a population of children with severe asthma. The aim of this study was to test the hypothesis that symptoms of anxiety or depression are highly prevalent in pediatric severe asthma and negatively effects asthma control.

Longitudinal assessments of anxiety or depression symptoms (Patient Health Questionnaire-4 [PHQ-4]), asthma control (Asthma Control Test [ACT]), and lung function were performed in a single-center pediatric severe asthma clinic. Participant data were collected during routine clinical care. Primary outcomes were ACT and forced expiratory volume in 1 s per forced vital capacity (FEV1/FVC).

Among 43 subjects (with total 93 observations), 58.1% reported at least one anxious or depressive symptom and 18.6% had a PHQ-4 more than 2, the threshold for an abnormal test result. (L)-Dehydroascorbic After adjusting for age, sex, race, and asthma medication step, there was a significant reduction in ACT for girls with PHQ-4 more than 2 (adjusted mean [SE] ACT for PHQ-4 > 2 13.64 [0.59], ACT for PHQ-4 ≤ 2 20.64 [1.25], p = .02) but not boys. Moreover, there was a significant differential effect of mental health impairment for girls than boys. ACT for girls with PHQ more than 2 13.64 (0.59) compared with boys with PHQ-4 more than 2 17.82 (0.95), adjusted mean difference ACT by sex = 4.18 points; 95% confidence interval, 0.63-7.73; p = .033. In adjusted models, there was no association between PHQ-4 more than 2 and FEV1/FVC.

Symptoms of anxiety and depression are common. In children with severe asthma, a PHQ-4 score more than 2 is associated with worse asthma symptom control in girls, but not boys.

Symptoms of anxiety and depression are common. In children with severe asthma, a PHQ-4 score more than 2 is associated with worse asthma symptom control in girls, but not boys.The novel coronavirus disease-2019 (COVID-19), caused by the pathogen severe acute respiratory syndrome-CoV-2, is causing a global pandemic, with over 26.9 million cases and 880,000 deaths as of September 6, 2020. While there has been speculation and observational research about the impact of COVID-19 on asthma, much remains unknown. The goal of this article is to provide a scoping review on pediatric asthma and COVID-19 and summarize what we do and do not know from the first wave of the pandemic.Congenital glycosylation disorders (CDG) are inherited metabolic diseases due to defective glycoprotein and glycolipid glycan assembly and attachment. MOGS-CDG is a rare disorder with seven patients from five families reported worldwide. We report on a 19-year-old girl with MOGS-CDG. At birth she presented facial dysmorphism, marked hypotonia, and drug-resistant tonic seizures. In the following months, her motility was strongly limited by dystonia, with forced posture of the head and of both hands. She showed a peculiar hyperkinetic movement disorder with a rhythmic and repetitive pattern repeatedly documented on EEG-polygraphy recordings. Brain MRI showed progressive cortical and subcortical atrophy. Epileptic spasms appeared in first months and ceased by the age of 7 years, while tonic seizures were still present at last assessment (19 years). We report the oldest-known MOGS-CDG patient and broaden the neurological phenotype of this CDG.

Primary Sjögren’s syndrome (SS) is characterized by a lymphocytic infiltration of salivary glands (SGs) and the presence of an interferon (IFN) signature. SG epithelial cells (SGECs) play an active role in primary SS pathophysiology. We undertook this study to examine the interactions between SGECs and T cells in primary SS and the role of the interleukin-7 (IL-7)/IFN axis.

Primary cultured SGECs from control subjects and patients with primary SS were stimulated with poly(I-C), IFNα, or IFNγ. T cells were sorted from blood and stimulated with IL-7. CD25 expression was assessed by flow cytometry. SG explants were cultured for 4 days with anti-IL-7 receptor (IL-7R) antagonist antibody (OSE-127), and transcriptomic analysis was performed using the NanoString platform.

Serum IL-7 level was increased in patients with primary SS compared to controls and was associated with B cell biomarkers. IL7R expression was decreased in T cells from patients with primary SS compared to controls. SGECs stimulated with poly(I-C), IFNα, or IFNγ secreted IL-7.