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https://www.readbyqxmd.com/read/29197979/genetic-differences-in-the-aryl-hydrocarbon-receptor-and-cyp1a2-affect-sensitivity-to-developmental-polychlorinated-biphenyl-exposure-in-mice-relevance-to-studies-of-human-neurological-disorders
#1
Kelsey Klinefelter, Molly Kromme Hooven, Chloe Bates, Breann T Colter, Alexandra Dailey, Smitha Krishnan Infante, Izabela Kania-Korwel, Hans-Joachim Lehmler, Alejandro López-Juárez, Clare Pickering Ludwig, Christine Perdan Curran
Polychlorinated biphenyls (PCBs) are persistent organic pollutants that remain a human health concern with newly discovered sources of contamination and ongoing bioaccumulation and biomagnification. Children exposed during early brain development are at highest risk of neurological deficits, but highly exposed adults reportedly have an increased risk of Parkinson's disease. Our previous studies found allelic differences in the aryl hydrocarbon receptor and cytochrome P450 1A2 (CYP1A2) affect sensitivity to developmental PCB exposure, resulting in cognitive deficits and motor dysfunction...
December 2, 2017: Mammalian Genome: Official Journal of the International Mammalian Genome Society
https://www.readbyqxmd.com/read/29194859/a-nationwide-epidemiological-study-of-myasthenia-gravis-in-latvia
#2
Anete Zieda, Kristine Ravina, Ieva Glazere, Lelde Pelcere, Maruta S Naudina, Lelde Liepina, Inese Kamsa, Natalja Kurjane, Mark Woodhall, Leslie Jacobson, M Isabel Leite, Kokil Tandon, Viktorija Kenina
BACKGROUND AND PURPOSE: Myasthenia gravis (MG) is an autoimmune disorder characterized by fatigable muscle weakness due to of antibody-mediated impairment of neuromuscular transmission. The aim of this study was to investigate the incidence and prevalence of MG in Latvia, and to characterize this population by well-established clinical parameters such as age at onset, presence of associated antibodies, and thymus pathology. METHODS: cases of patients with known MG or presenting with first MG symptoms within the time period from 1 January 2010 to 31 December 2014 were selected from the database of the Neuromuscular Disease Clinic of Pauls Stradins Clinical University Hospital and Children's Clinical University Hospital...
November 30, 2017: European Journal of Neurology: the Official Journal of the European Federation of Neurological Societies
https://www.readbyqxmd.com/read/29194572/the-igg-response-against-staphylococcus-aureus-is-associated-with-severe-atopic-dermatitis-in-children
#3
J E E Totté, L M Pardo, K B Fieten, J de Wit, D V de Boer, W J van Wamel, S G M A Pasmans
BACKGROUND: An altered immune response against Staphylococcus (S.) aureus might contribute to inflammation and barrier damage in atopic dermatitis (AD). OBJECTIVES: We profiled IgG antibodies against 55 S. aureus antigens in sera of children with mild to severe AD using a Luminex assay. Additionally, we evaluated the association between IgG levels and disease severity. METHODS: In this cross-sectional study, we included children with AD of two interventional study cohorts, namely SMA (n= 131) and the older DAVOS cohort (n= 76)...
November 30, 2017: British Journal of Dermatology
https://www.readbyqxmd.com/read/29033080/reconstitution-of-th17-tc17-and-treg-cells-after-paediatric-haematopoietic-stem-cell-transplantation-impact-of-interleukin-7
#4
Katrine Kielsen, Lars P Ryder, David Lennox-Hvenekilde, Monika Gad, Claus H Nielsen, Carsten Heilmann, Marianne Ifversen, Anders Elm Pedersen, Klaus Müller
Successful reconstitution of T lymphocytes after allogeneic haematopoietic stem cell transplantation (HSCT) is needed to establish the graft-versus-leukaemia effect and an effective anti-microbial defense, but the ratio between functionally different T-cell subsets needs to be balanced to avoid graft-versus-host disease (GVHD). IL-7 is essential for T-cell generation in the thymus and peripheral T-cell homeostasis. High IL-7 levels have been associated with impaired T-cell reconstitution, increased risk of acute GVHD and treatment-related mortality, but the underlying cellular mechanisms behind these associations have not been investigated previously...
October 9, 2017: Immunobiology
https://www.readbyqxmd.com/read/29025154/hematological-alterations-in-protein-malnutrition
#5
Ed W Santos, Dalila C Oliveira, Graziela B Silva, Maristela Tsujita, Jackeline O Beltran, Araceli Hastreiter, Ricardo A Fock, Primavera Borelli
Protein malnutrition is one of the most serious nutritional problems worldwide, affecting 794 million people and costing up to $3.5 trillion annually in the global economy. Protein malnutrition primarily affects children, the elderly, and hospitalized patients. Different degrees of protein deficiency lead to a broad spectrum of signs and symptoms of protein malnutrition, especially in organs in which the hematopoietic system is characterized by a high rate of protein turnover and, consequently, a high rate of protein renewal and cellular proliferation...
November 1, 2017: Nutrition Reviews
https://www.readbyqxmd.com/read/28792717/the-relationship-between-serum-tarc-tslp-and-postn-levels-and-childhood-atopic-dermatitis
#6
Pinar Uysal, Fatih Birtekocak, Aslihan Buyukozturk Karul
BACKGROUND: The aim of the study is to evaluate the relationship between serum periostin (POSTN), thymus and activation-regulated chemokine (TARC), and thymic stromal lymphopoietin (TSLP) levels and disease severity and atopy in children with atopic dermatitis. METHODS: Sixty children with atopic dermatitis and 31 healthy controls were included in the study. The disease severity was measured by SCORAD scores. Serum POSTN, TARC, and TSLP levels were measured in all participants...
July 1, 2017: Clinical Laboratory
https://www.readbyqxmd.com/read/28760578/pre-birth-origins-of-allergy-and-asthma
#7
REVIEW
K L Gatford, A L Wooldridge, K L Kind, R Bischof, V L Clifton
Allergy is a chronic disease that can develop as early as infancy, suggesting that early life factors are important in its aetiology. Variable associations between size at birth, a crude marker of the fetal environment, and allergy have been reported in humans and require comprehensive review. Associations between birth weight and allergy are however confounded in humans, and we and others have therefore begun exploring the effects of early life events on allergy in experimental models. In particular, we are using ovine models to investigate whether and how a restricted environment before birth protects against allergy, whether methyl donor availability contributes to allergic protection in IUGR, and why maternal asthma during pregnancy is associated with increased risks of allergic disease in children...
September 2017: Journal of Reproductive Immunology
https://www.readbyqxmd.com/read/28746835/genetic-ataxia-telangiectasia-porcine-model-phenocopies-the-multisystemic-features-of-the-human-disease
#8
Rosanna Beraldi, David K Meyerholz, Alexei Savinov, Attila D Kovács, Jill M Weimer, Jordan A Dykstra, Ryan D Geraets, David A Pearce
Ataxia telangiectasia (AT) is a progressive multisystem autosomal recessive disorder caused by mutations in the AT-mutated (ATM) gene. Early onset AT in children is characterized by cerebellar degeneration, leading to motor impairment. Lung disease and cancer are the two most common causes of death in AT patients. Accelerated thymic involution may contribute to the cancer, and recurrent and/or chronic respiratory infections may be a contributing factor to lung disease in AT. AT patients have fertility issues, are highly sensitive to ionizing radiation and they present oculocutaneous telangiectasia...
November 2017: Biochimica et Biophysica Acta
https://www.readbyqxmd.com/read/28707128/thymectomy-is-a-beneficial-therapy-for-patients-with-non-thymomatous-ocular-myasthenia-gravis-a-systematic-review-and-meta-analysis
#9
Kai Zhu, Jiaoxing Li, Xin Huang, Wei Xu, Weibin Liu, Jiaxin Chen, Pei Chen, Huiyu Feng
Ocular myasthenia gravis, an autoimmune disease, is characterized by extraocular muscle weakness. Myasthenia gravis is closely associated with the functional status of the thymus gland. The efficacy of thymectomy for non-thymomatous ocular myasthenia gravis remains controversial. Here, we present the first systematic review and meta-analysis of studies assessing the outcome of thymectomy in patients with non-thymomatous ocular myasthenia gravis and found that the pooled rate of complete stable remission was 0...
July 13, 2017: Neurological Sciences
https://www.readbyqxmd.com/read/28689684/-ectopic-thymus-a-rare-cause-of-neck-mass-in-children
#10
S Kallel, M Mnejja, M Kessentini, A Ben Said, I Charfeddine, B Hammami, A Ghorbel
INTRODUCTION: Ectopic cervical thymus (ECT) is a rare embryological abnormality in children. It can be revealed by a compressive neck mass mistaken for a malignant tumor. Through a new case of ECT, we review the embryopathogenesis, diagnostic difficulties and therapeutic features. CLINICAL OBSERVATION: A 19-month-old girl presented a right cervical mass that quickly increased in size, causing intermittent dyspnea. The physical examination objectified a 6-cm, soft and compressible, painless right cervical tumefaction, extending from the mastoid area to the ipsilateral supraclavicular fossa...
July 6, 2017: Archives de Pédiatrie: Organe Officiel de la Sociéte Française de Pédiatrie
https://www.readbyqxmd.com/read/28657137/a-novel-pax1-null-homozygous-mutation-in-autosomal-recessive-otofaciocervical-syndrome-associated-with-severe-combined-immunodeficiency
#11
I Paganini, R Sestini, G L Capone, A L Putignano, E Contini, I Giotti, F Gensini, A Marozza, A Barilaro, B Porfirio, L Papi
Otofaciocervical syndrome (OFCS) is a rare disorder characterized by facial anomalies, cup-shaped low-set ears, preauricular fistulas, hearing loss, branchial defects, skeletal anomalies, and mild intellectual disability. Autosomal dominant cases are caused by deletions or point mutations of EYA1. A single family with an autosomal recessive form of OFCS and a homozygous missense mutation in PAX1 gene has been described. We report whole exome sequencing of 4 members of a consanguineous family in which 2 children, showing features of OFCS, expired from severe combined immunodeficiency (SCID)...
June 28, 2017: Clinical Genetics
https://www.readbyqxmd.com/read/28636882/foxn1-italian-founder-mutation-in-indian-family-implications-in-prenatal-diagnosis
#12
Akella Radha Rama Devi, Nagesh Narayan Panday, Shaik Mohammad Naushad
The Forkhead box N1 (FOXN1) is a transcriptional factor regulating the development, differentiation and function of thymic epithelial cells; maintaining T-lineage progenitors in bone marrow; promoting terminal differentiation of epithelial cells of hair follicles. Mutation in FOXN1 was reported to cause a rare disorder characterized by rudimentary thymus gland, T-cell immunodeficiency, congenital alopecia and nail dystrophy within an Italian community. This is the first report of FOXN1 p.R255X mutation from India, outside this endogamous Italian community...
September 5, 2017: Gene
https://www.readbyqxmd.com/read/28546728/spindle-epithelial-tumor-with-thymus-like-element-settle-a-case-report
#13
Cengiz Tavusbay, Demet Arikan Etit, Ercan Kadioglu, Mehmet Haciyanli
Spindle epithelial tumor with thymus-like element (SETTLE) is a very rare malignant neoplasm of the thyroid. It is generally encountered in children, adolescents, and young adults Objective: We aimed to present a patient with SETTLE and to review the clinical and pathologic features of this rare tumor. A 13-year-old male patient presented to our clinic with complaints of a rapidly growing lump on his neck. There was no evidence of distant metastases at time of presentation. A total thyroidectomy was performed...
June 2017: Indian Journal of Surgical Oncology
https://www.readbyqxmd.com/read/28529467/radiotherapy-for-pertussis-an-historical-assessment
#14
Edward J Calabrese, Gaurav Dhawan, Rachna Kapoor
X-ray therapy was used to treat pertussis/whooping cough during a 13-year period from 1923 to 1936 in North America and Europe. Twenty studies from clinicians in the United States reported that approximately 1500 cases of pertussis were treated by X-ray therapy usually with less than 0.5 erythema dose. Young children (<3 years) comprised about 70% to 80% of the cases, with the age of cases ranging from as young as 1 month to 50 years. In general, symptoms of severe coughing, vomiting episodes, and spasms were significantly relieved in about 85% of cases following up to 3 treatments, while about 15% of the cases showed nearly full relief after only 1 treatment...
April 2017: Dose-response: a Publication of International Hormesis Society
https://www.readbyqxmd.com/read/28440352/intrathyroidal-ectopic-thymus-in-children-a-sonographic-survey
#15
Adnan Kabaalioğlu, Murat Alp Öztek, Uğur Kesimal, Kağan Çeken, Emel Durmaz, Ali Apaydın
AIMS: Intrathyroidal ectopic thymus (IET) is being increasingly reported in the radiology literature. Most of the reports are of individual cases or small series and prevalence and natural course of the pathology is not well known. The purpose of this study is to establish the prevalence of IET in children and report long term follow-up results. MATERIAL AND METHODS: In 180 children who were examined by ultrasound (US) for other reasons, 7 patients were indentified with IET...
April 22, 2017: Medical Ultrasonography
https://www.readbyqxmd.com/read/28393508/-cervical-thymic-cyst-in-childhood
#16
M Haro-García, J Crespo Del Hierro, R de la Fuente-Cañibano, M A Alañón-Fernández
Cervical thymus cyst is a rare cause of neck mass in children. . Approximately 90 cases have been described, and just a few have been published in Spanish literature. They usually appear in early children and are diagnosed as brachial cyst. We report the case of a 6 year old boy with a swelling on the right side of the neck. The swelling had appeared a few months before. Ultrasound examination of a neck mass and MRI showed a cystic mass compatible with possible branchial cyst. Excision of the tumor was performed...
July 10, 2016: Cirugía Pediátrica: Organo Oficial de la Sociedad Española de Cirugía Pediátrica
https://www.readbyqxmd.com/read/28359333/the-immune-regulating-effect-of-xiao-er-qixingcha-in-constipated-mice-induced-by-high-heat-and-high-protein-diet
#17
Chang Qu, Guang-Hua Yang, Rong-Bo Zheng, Xiu-Ting Yu, Shao-Zhong Peng, Jian-Hui Xie, Jian-Nan Chen, Xiu-Fen Wang, Zi-Ren Su, Xiao-Jun Zhang
BACKGROUND: Xiao'er Qixingcha (EXQ) has been extensively applied to relieve dyspepsia and constipation in children for hundreds of years in China. However, the therapeutic mechanism underlying its efficacy remained to be defined. The present study aimed to clarify the possible laxative and immune-regulating effects of EXQ on two models of experimental constipation in mice, which mimicked the pediatric constipation caused by high-heat and high-protein diet (HHPD). METHODS: The two models of constipated mice were induced by HHPD or HHPD + atropine respectively...
March 31, 2017: BMC Complementary and Alternative Medicine
https://www.readbyqxmd.com/read/28288591/correlates-of-thymus-size-and-changes-during-treatment-of-children-with-severe-acute-malnutrition-a-cohort-study
#18
Maren Johanne Heilskov Rytter, Hanifa Namusoke, Christian Ritz, Kim F Michaelsen, André Briend, Henrik Friis, Dorthe Jeppesen
BACKGROUND: The impairment of immune functions associated with malnutrition may be one reason for the high mortality in children with severe acute malnutrition (SAM), and thymus atrophy has been proposed as a marker of this immunodeficiency. The aim of this study was to identify nutritional and clinical correlates of thymus size in children with SAM, and predictors of change in thymus size with nutritional rehabilitation. METHODS: In an observational study among children aged 6-59 months admitted with SAM in Uganda, we measured thymus area by ultrasound on hospital admission to treatment with F75 and F100, on hospital discharge and after 8 weeks of nutritional rehabilitation with ready-to-use therapeutic food, as well as in well-nourished healthy children...
March 14, 2017: BMC Pediatrics
https://www.readbyqxmd.com/read/28279759/what-do-we-know-about-the-structure-of-human-thymic-hassall-s-corpuscles-a-histochemical-immunohistochemical-and-electron-microscopic-study
#19
Renáta Mikušová, Veronika Mešťanová, Štefan Polák, Ivan Varga
Hassall's corpuscles are the most prominent structures in the human thymus. However, relatively few analyses have been performed to determine their function and cellular origins during development. In this study, we evaluated the cellular microenvironment of human thymic Hassall's corpuscles using histochemistry, immunohistochemistry, and transmission electron microscopy. We examined 95 human thymic tissue samples, which were perioperatively obtained from children undergoing cardiac surgery. To characterize the complex cellular microenvironment of human thymic corpuscles, we used a panel of 14 different antibodies to identify discrete cell types...
May 2017: Annals of Anatomy, Anatomischer Anzeiger: Official Organ of the Anatomische Gesellschaft
https://www.readbyqxmd.com/read/28238403/increased-sputum-levels-of-thymus-and-activation-regulated-chemokine-in-children-with-asthma-not-eosinophilic-bronchitis
#20
M J Kim, H S Lee, I S Sol, M N Kim, J Y Hong, K E Lee, Y H Kim, K W Kim, M H Sohn, K-E Kim
BACKGROUND: Thymus and activation-regulated chemokine (TARC), a member of the CC chemokine family, plays a crucial role in Th2-specific inflammation. We aimed to determine the concentration of sputum TARC in children with asthma and eosinophilic bronchitis (EB) and its relation with eosinophilic inflammation, pulmonary function, and bronchial hyper-responsiveness. METHODS: In total, 90 children with asthma, 38 with EB, and 45 control subjects were enrolled. TARC levels were measured in sputum supernatants using an ELISA...
May 2017: Allergologia et Immunopathologia
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