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Thymus children

Afshin Shirkani, Mohammad Shahrooei, Gholamreza Azizi, Hassan Rokni-Zadeh, Hassan Abolhassani, Shokrollah Farrokhi, Glynis Frans, Xavier Bossuyt, Asghar Aghamohammadi
ZAP-70 deficiency is a rare autosomal recessive form of combined immunodeficiency (CID) characterized by selective absence of circulating CD8 T cells with low, normal, or increased CD4 T cells in peripheral blood. Up to now, 14 unique mutations in the ZAP70 gene have been identified in patients with ZAP-70-related CID. We present a 3-year-old boy with a history of recurrent bacterial infections and autoimmunity. Initial laboratory findings showed a normal total lymphocyte count, but low levels of CD8 and CD4 T cells and an abnormal lymphocyte proliferation response...
October 19, 2016: Immunological Investigations
Elpis-Athina Vlachopapadopoulou, Marina Vakaki, Fotini-Eleni Karachaliou, Irene Kaloumenou, Kleanthi Kalogerakou, Christina Gali, Stefanos Michalacos
BACKGROUND: During gestation, the primordial thymus migrates from the pharynx to the anterior mediastinum, thus thymic tissue can remain at any point along this path. Intrathyroidal thymic remnants are rare, and their sonographic patterns have only recently been described. This retrospective study presents the sonographic appearance of ectopic intrathyroidal thymus and emphasizes the role of sonography in order to avoid misdiagnosis. METHODS: The population consisted of 42 children, 3...
October 19, 2016: Hormone Research in Pædiatrics
Katarína Adamicová, Tomáš Balhárek, Želmíra Fetisovová, Yvetta Mellová
Epidermolysis bullosa and Bart´s syndrome are fairly accurately documented diseases by histopathology. In the article the authors describe interesting and hitherto undescribed phenomenon in the lungs male infant with epidermolysis bullosa junctionalis and Barts syndrome, who died 17 days after birth and 13 days after surgery for pyloric atresia, on multiorgan failure within basic congenital diseases.Histologically in lung alveoli was found to the massive presence of foamy macrophages and numerous globoid formations resembling morphological and immunohistochemical "Hassall´s" bodies in a thymus of the newborn...
2016: Ceskoslovenská Patologie
S Rauthe, A Rosenwald
Lymphomas infiltrating the mediastinum are a challenge for the treating physician as well as for the pathological diagnostics. The clinical scenario is often an emergency situation, while the pathologist is usually confronted only with small biopsy samples. Classical Hodgkin's lymphoma is by far the most frequently occurring lymphoma in the mediastinum and predominantly the nodular sclerosis subtype. In small and very sclerotic specimens it can be difficult to morphologically detect Hodgkin and Reed-Sternberg cells and to identify the characteristic phenotype by immunohistochemistry...
September 2016: Der Pathologe
Martina Kovarova, Uma Shanmugasundaram, Caroline E Baker, Rae Ann Spagnuolo, Chandrav De, Christopher C Nixon, Angela Wahl, J Victor Garcia
BACKGROUND: Approximately 1.5 million HIV-positive women become pregnant annually. Without treatment, up to 45% will transmit HIV to their infants, primarily through breastfeeding. These numbers highlight that HIV acquisition is a major health concern for women and children globally. They also emphasize the urgent need for novel approaches to prevent HIV acquisition that are safe, effective and convenient to use by women and children in places where they are most needed. METHODS: 4'-Ethynyl-2-fluoro-2'-deoxyadenosine, a potent NRTI with low cytotoxicity, was administered orally to NOD/SCID/γc(-/-) mice and to bone marrow/liver/thymus (BLT) humanized mice, a preclinical model of HIV infection...
August 1, 2016: Journal of Antimicrobial Chemotherapy
N P Loginova, V A Chetvertnykh, N V Chemurziyeva
Biopsy specimens of the thymus were studied in children aged under 11 months (n = 77) with congenital heart defects and circulatory hypoxia of varying severity. Histological sections were stained with hematoxylin-eosin and Shubich's method (to demonstrate mast cells). The expression of Ki-67, CD3 and CD34 was assessed by immunohistochemistry. The ultrastructure of thymic tissues was also examined. It was found that the severity of hypoxia determined the morphological changes in the organ associated with a development of large complex of tissue reactions...
2016: Morfologi︠a︡
Wei An, Bhopal C Mohapatra, Neha Zutshi, Timothy A Bielecki, Benjamin T Goez, Haitao Luan, Fany Iseka, Insha Mushtaq, Matthew D Storck, Vimla Band, Hamid Band
CBL and CBL-B ubiquitin ligases play key roles in hematopoietic stem cell homeostasis and their aberrations are linked to leukemogenesis. Mutations of CBL, often genetically-inherited, are particularly common in Juvenile Myelomonocytic Leukemia (JMML), a disease that manifests early in children. JMML is fatal unless corrected by bone marrow transplant, which is effective in only half of the recipients, stressing the need for animal models that recapitulate the key clinical features of this disease. However, mouse models established so far only develop hematological malignancy in adult animals...
July 16, 2016: Oncotarget
Sławomir Kroczka, Katarzyna Stasiak, Marek Kaciński
BACKGROUND: Myasthenia gravis (MG) is an autoimmunologic disorder. It is characterized by various clinical symptoms and their dependency upon the exertion and the rest as well. MATERIAL AND METHODS: Between 2002-20014 in the Neurophysiology Laboratory at the Chair of Pediatric and Adolescent Neurology, Jagiellonian University in Krakow, the electrophysiological repetitive nerve stimulation study were performed in 44 children. The clinical picture and positive electrophysiological test were the ground to diagnose MG in 19 of them (12 girls and 7 boys)...
2016: Przegla̧d Lekarski
Dhanesheshwar N Lanjewar, Varsha Omprakash Bhatia, Sonali Dhaneshwar Lanjewar
BACKGROUND: Human immunodeficiency virus (HIV) infection in India has now been prevalent over three decades, and an increasing number of children are being affected with HIV. The spectrum of pathologic lesions in children with acquired immunodeficiency syndrome (AIDS) in India has not been well described. MATERIALS AND METHODS: A review of systematically conducted autopsies of 11 (10 boys and 1 girl) children with AIDS is presented. RESULTS: The mode of HIV transmission in 6 children was vertical; in one it was blood transfusion and in 4 children route was presumably vertical as these were children of orphanage...
April 2016: Indian Journal of Pathology & Microbiology
Hitokazu Esaki, Satoshi Takeuchi, Norihiro Furusyo, Kazuhiko Yamamura, Sayaka Hayashida, Gaku Tsuji, Masaki Takemura, Jun Hayashi, Masutaka Furue
Atopic dermatitis (AD) is a multifactorial T-helper (Th)2-mediated skin disease frequently associated with elevated serum immunoglobulin (Ig)E and food allergy is also a Th2- and IgE-mediated adverse immunological reaction. Our previous study indicated the relation of egg allergy history and disease severity of AD. Thus, the purpose of the study was to investigate the levels of IgE specific to major food allergens (egg, milk, wheat) and Th2 chemokines (chemokine [C-C motif] ligand [CCL]17/thymus and activation regulated chemokine [TARC] and CCL22/macrophage-derived chemokine [MDC]) and the relationship between them...
March 30, 2016: Journal of Dermatology
Paul J Galardy, Tibor Bedekovics, Michelle L Hermiston
Non-Hodgkin lymphoma (NHL) is the third most common malignancy in children, adolescents and young adults (CAYA). NHL is a diverse set of diseases that arise at key regulatory checkpoints during B or T cell development in the bone marrow, germinal centre or thymus. While advances in the use of conventional cytotoxic agents have led to dramatic improvements in survival, these cures are associated with significant acute and long-term toxicities. Moreover, the prognosis for CAYA patients with relapsed or refractory NHL remains dismal, with the vast majority dying of their disease...
May 2016: British Journal of Haematology
Marijn Stuut, Gusta van Zwieten, Jos M Straetmans, Martin Lacko, Constance T R M Stumpel
Anterior neck masses in young children can be a diagnostic challenge for otolaryngologists and radiologists. We present a rare case of herniation of normal mediastinal thymus in a four-year-old girl. Additional medical features as an inguinal hernia and trochlear nerve paresis raised the question whether there is a causal relationship or an association. A connective tissue disorder could not be diagnosed as possible causal factor to the abnormal movement of the mediastinal thymus. Awareness and recognition of this benign phenomenon is important in order to avoid unnecessary biopsy or surgery...
April 2016: International Journal of Pediatric Otorhinolaryngology
Robyn M Amos-Kroohs, Devon L Graham, Curtis E Grace, Amanda A Braun, Tori L Schaefer, Matthew R Skelton, Charles V Vorhees, Michael T Williams
The level of lead (Pb) exposure in children has decreased dramatically since restrictions on its use were implemented. However, even with restrictions, children are exposed to Pb and still present with cognitive and behavioral deficits. One prominent aspect of the exposome of these children is that many come from low social economic status (SES) conditions, and low SES is associated with stress. In order to compare the combined effects of early stress and Pb, Sprague-Dawley rats were exposed to vehicle or Pb either alone or in combination with maternal separation stress during brain development (i...
May 2016: Neurotoxicology
Tahira Bibi, Mushtaq Ahmad, Sarah E Edwards, Niaz Mohammad Tareen, Rukhsana Jabeen, Irum Abdullah
ETHNOPHARMACOLOGICAL RELEVANCE: Infection by intestinal parasitic worms (soil-transmitted helminths or geohelminths) is prevalent in many parts of the world, and poses a particular health risk to children. This paper presents findings from a preliminary study with the primary aim to document indigenous knowledge about the use of herbal medicines in the treatment of intestinal worm infections in children among the local communities of Kalat district of northern Balochistan, Pakistan. MATERIALS AND METHODS: Ethnomedicinal data were collected through a triangulation approach, that included participant-observation and rapid appraisal methods...
May 13, 2016: Journal of Ethnopharmacology
Theo van den Broek, Eveline M Delemarre, Willemijn J M Janssen, Rutger A J Nievelstein, Jasper C Broen, Kiki Tesselaar, Jose A M Borghans, Edward E S Nieuwenhuis, Berent J Prakken, Michal Mokry, Nicolaas J G Jansen, Femke van Wijk
The generation of naive T cells is dependent on thymic output, but in adults, the naive T cell pool is primarily maintained by peripheral proliferation. Naive T cells have long been regarded as relatively quiescent cells; however, it was recently shown that IL-8 production is a signatory effector function of naive T cells, at least in newborns. How this functional signature relates to naive T cell dynamics and aging is unknown. Using a cohort of children and adolescents who underwent neonatal thymectomy, we demonstrate that the naive CD4+ T cell compartment in healthy humans is functionally heterogeneous and that this functional diversity is lost after neonatal thymectomy...
March 1, 2016: Journal of Clinical Investigation
Linda Jorgov, Françoise Montravers, Sona Balogova, Christine Ragu, Hélène Pacquement, Thierry Leblanc, Samuel Abbou, Hubert Ducou-Lepointe, Judith Landman-Parker, Jean-Noёl Talbot
PURPOSE: To evaluate, in children with Hodgkin lymphoma (HL), the frequency and intensity of visually diffuse FDG uptake by selected organs at baseline (bPET) and on interim PET/CT (iPET), and to evaluate the relation between FDG uptake, metabolic response and evolution of the disease with treatment. PATIENTS AND METHODS: Thirty children with HL had bPET and then iPET after two cycles of treatment, which were blind-read retrospectively. Excluding sites with focal uptake, diffuse FDG uptake by thymus, bone marrow at iliac crests, liver, spleen, and the spinal cord at the 12th thoracic vertebra (Th12) was evaluated visually using a three-point scoring method and semiquantitatively by measuring SUVmax...
July 2016: European Journal of Nuclear Medicine and Molecular Imaging
Victoria Rebecca Carol Alexander, Joseph George Manjaly, Christopher M Pepper, Sonna N Ifeacho, Richard J Hewitt, Benjamin E J Hartley
OBJECTIVE: Head and neck teratomas are rare and malignant change is rarer still. This is a report detailing all of the teratomas treated by the ear, nose and throat or craniofacial teams over the last 28 years at our institution. Examining the common presenting and radiological features as well as our success rates compared to the current literature. METHOD: A retrospective review of all cases presenting to our institution with a head and neck teratoma were analysed...
December 2015: International Journal of Pediatric Otorhinolaryngology
Anna-Sophia Wiekmeijer, Karin Pike-Overzet, Hanna IJspeert, Martijn H Brugman, Ingrid L M Wolvers-Tettero, Arjan C Lankester, Robbert G M Bredius, Jacques J M van Dongen, Willem E Fibbe, Anton W Langerak, Mirjam van der Burg, Frank J T Staal
BACKGROUND: Severe combined immunodeficiency (SCID) represents congenital disorders characterized by a deficiency of T cells caused by arrested development in the thymus. Yet the nature of these developmental blocks has remained elusive because of the difficulty of taking thymic biopsy specimens from affected children. OBJECTIVE: We sought to identify the stages of arrest in human T-cell development caused by various major types of SCID. METHODS: We performed transplantation of SCID CD34(+) bone marrow stem/progenitor cells into an optimized NSG xenograft mouse model, followed by detailed phenotypic and molecular characterization using flow cytometry, immunoglobulin and T-cell receptor spectratyping, and deep sequencing of immunoglobulin heavy chain (IGH) and T-cell receptor δ (TRD) loci...
February 2016: Journal of Allergy and Clinical Immunology
Graciela Gavia-García, Haydeé González-Martínez, Ángel Miliar-García, Edmundo Bonilla-González, María de Los Ángeles Rosas-Trejo, Mina Königsberg, Oralia Nájera-Medina, Armando Luna-López, María Cristina González-Torres
OBJECTIVE: Malnutrition has been associated with oxidative damage by altered antioxidant protection mechanisms. Specifically, the aim of this study was to evaluate oxidative damage (DNA and lipid) and antioxidant status (superoxide dismutase [SOD], glutathione peroxidase [GPx], and catalase [CAT] mRNA, and protein expression) in thymus from malnourished rat pups. METHODS: Malnutrition was induced during the lactation period by the food competition method. Oxidative DNA damage was determined quantifying 8-oxo-7, 8-dihydro-2'-deoxyguanosine adduct by high-performance liquid chromatography...
November 2015: Nutrition
Tsuyoshi Umehara, Katsura Hakamada, Goro Oshima, Katsuyoshi Suzuki, Ken Iwanaga, Yuki Yamaguchi, Hiroyuki Arai, Yumiko Hikida, Junya Kita, Yasuhiro Hayashi
We report herein on a case of ectopic cervical thymus in a 5-year-old boy and the literature is reviewed. Swelling of the right neck was seen in the patient in his newborn period and it was diagnosed as cystic disease of the neck in a previous hospital at 4 months of age. Ultrasonography (US) and MRI revealed a cervical tumor consisting of a solid component in our hospital, and histopathologic examination showed no evidence of malignancy. The lesion revealed almost no change in size but showed a mosaic pattern on US, whereon the parents agreed to the removal of the tumor...
May 2015: Nihon Jibiinkoka Gakkai Kaiho
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