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Procedure: Gastroschisis Repair

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Total 540 results found since Jan 2013.

Double-strand break repair through homologous recombination in autosomal-recessive BCL10 deficiency
Three members of the caspase recruitment domain (CARD) family of adaptor proteins (CARD9, CARD10, and CARD11) are known to form heterotrimers with B-cell lymphoma 10 (BCL10) and mucosa-associated lymphoid tissue lymphoma translocation gene 1 (MALT1), resulting in the CARD-BCL10-MALT1 (CBM) complex. In humans and mice, the CBM complex mediates nuclear factor kappa-light-chain-enhancer of activated B cells (NF- κB) and mitogen-activated protein kinase activation in a cell-type–specific and nonredundant manner, after the stimulation of various immune receptors.
Source: Journal of Allergy and Clinical Immunology - January 17, 2019 Category: Allergy & Immunology Authors: Sonia Garc ía-Gómez, Rebeca Chaparro, Amin Safa, Ana Van Den Rym, Rubén Martinez-Barricarte, Lazaro Lorenzo, Silvia Sánchez-Ramón, Victor Toledano, Carolina Cubillos-Zapata, Eduardo López-Collazo, Maria Dolores Martín-Arranz, Eduardo Martín-Arranz Tags: Letter to the Editor Source Type: research

Double-strand break repair through homologous recombination in autosomal recessive BCL10 deficiency
DNA double-strand break (DSB) repair through homologous recombination from BCL10-/- patient is impaired. Given the relevance of DBS in the onset of cancer, it is important to highlight homologous recombination is BCL10-dependent in human fibroblasts.
Source: Journal of Allergy and Clinical Immunology - January 17, 2019 Category: Allergy & Immunology Authors: Sonia Garc ía-Gómez, Rebeca Chaparro, Amin Safa, Ana Van Den Rym, Rubén Martinez-Barricarte, Lazaro Lorenzo, Silvia Sánchez-Ramón, Victor Toledano, Carolina Cubillos-Zapata, Eduardo López-Collazo, Maria Dolores Martín-Arranz, Eduardo Martín-Arranz Source Type: research

Dysfunctional ErbB2, an EGF receptor family member, hinders repair of airway epithelial cells from asthmatic patients
Genetic and genomic data increasingly point to the airway epithelium as critical to asthma pathogenesis. Epithelial growth factor (EGF) family members play a fundamental role in epithelial differentiation, proliferation, and repair. Although expression of erythroblastosis oncogene B2 (ErbB2) mRNA, an EGF family receptor, was reported to be lower in asthmatic patients, little is understood about its functional role.
Source: Journal of Allergy and Clinical Immunology - January 9, 2019 Category: Allergy & Immunology Authors: Hideki Inoue, Takeshi Hattori, Xiuxia Zhou, Emily B. Etling, Brian D. Modena, John B. Trudeau, Fernando Holguin, Sally E. Wenzel Source Type: research

Dysfunctional ErbB2, an EGF receptor family member, hinders repair of asthmatic airway epithelial cells
Asthmatic airway epithelial cells have dysregulated wound repair, in association with lower activation of the ErbB2 pathway. Restoration of this pathway might be a novel therapeutic target, potentially restoring a disrupted airway epithelium in asthma.
Source: Journal of Allergy and Clinical Immunology - January 9, 2019 Category: Allergy & Immunology Authors: Hideki Inoue, Takeshi Hattori, Xiuxia Zhou, Emily B. Etling, Brian D. Modena, John B. Trudeau, Fernando Holguin, Sally E. Wenzel Source Type: research

The need to consider context in the evaluation of anti-infectious and immunomodulatory effects of Vitamin A and its derivatives.
Abstract Vitamin A and its derivatives (retinoids) act as potent regulators in many aspects of mammalian reproduction, development, repair, and maintenance of differentiated tissue functioning. Unlike other vitamins, Vitamin A and retinoids, which have hormonal actions, present significant toxicity, which plays roles in clinically relevant situations, such as hypervitaminosis A and retinoic acid ("differentiation") syndrome. Although clinical presentation is conspicuous in states of insufficient or excessive Vitamin A and retinoid concentration, equally relevant effects on host resistance to specific infectious ag...
Source: Current Drug Targets - December 16, 2018 Category: Drugs & Pharmacology Authors: Xavier-Elsas P, Vieira BM, Masid-de-Brito D, Barradas MG, Gaspar-Elsas MIC Tags: Curr Drug Targets Source Type: research

Septal Perforation Repair Utilizing an Anterior Ethmoidal Artery Flap and Collagen Matrix.
CONCLUSION: Anterior ethmoidal artery flap with an inlay collagen matrix is a reliable technique to repair nasal septal perforation. This technique, with robust vascularity and wide angle of rotation, enables the closure of perforations both large (<50% total septum) and with anterior locations. PMID: 30525903 [PubMed - as supplied by publisher]
Source: American Journal of Rhinology and Allergy - December 10, 2018 Category: ENT & OMF Authors: Cavada MN, Orgain CA, Alvarado R, Sacks R, Harvey RJ Tags: Am J Rhinol Allergy Source Type: research

Bilateral suprazygomatic maxillary nerve blocks vs. infraorbital and palatine nerve blocks in cleft lip and palate repair: A double-blind, randomised study
CONCLUSION Bilateral suprazygomatic maxillary nerve block is an effective and safe alternative to the traditional peripheral nerve blocks for cleft lip and cleft palate surgery, in a mixed paediatric and adult population.
Source: European Journal of Anaesthesiology - December 7, 2018 Category: Anesthesiology Tags: Loco-regional anaesthesia Source Type: research

The Iceberg Map of germline mutations in childhood cancer: focus on primary immunodeficiencies
Purpose of review The risk of cancer is higher, and its outcome is worse in patients with primary immunodeficiency (PID) than in members of the general population. Thus, the inter-relationship of malignant diseases with PIDs requires more study. Recent findings Large genetic screens identified a vast number of germline mutations in childhood cancer patient samples. Although TP53 was the most frequent single gene identified as mutated, many PID disorders like DNA repair defects are among the inborn causes of childhood cancer. We provide a comprehensive analysis of compiled data from seven recent studies that focused on...
Source: Current Opinion in Pediatrics - December 1, 2018 Category: Pediatrics Tags: ALLERGY, IMMUNOLOGY AND RELATED DISORDERS: Edited by Jordan S. Orange Source Type: research

Preoperative management of spontaneous cerebrospinal fluid rhinorrhea with acetazolamide
ConclusionThis is the first study to report the use of acetazolamide therapy as a primary treatment option for spontaneous CSF rhinorrhea. This therapy enabled surgery to be avoided in 31.3% of patients. This would indicate that in the absence of other contraindications for delaying repair, a trial of acetazolamide therapy could be considered as an initial option in the management of isolated spontaneous CSF rhinorrhea.
Source: International Forum of Allergy and Rhinology - November 15, 2018 Category: Allergy & Immunology Authors: Ashwini M. Tilak, Heather Koehn, Jos é Mattos, Spencer C. Payne Tags: ORIGINAL ARTICLE Source Type: research

The Effect of Delayed and Early Diagnosis in Siblings, and Importance of Newborn Screening for SCID
Severe combined immunodeficiency (SCID) is a form of primary immunodeficiency that affects both cellular and humoral immunity, caused by genetic defects in lymphocyte development and function. There are over 20 known genes mutations that lead to SCID including defects that cause radiosensitivity due to impairment in DNA repair (1). Mutations in DCLREC1 resulting in absent or dysfunctional Artemis protein are the most common cause of SCID associated with increased radiosensitivity and were identified as the etiology of the unusually high incidence of T-B-NK+ SCID in Athabascan-speaking tribes, Apache and Navajo (2,3).
Source: Annals of Allergy, Asthma and Immunology - November 12, 2018 Category: Allergy & Immunology Authors: Matthew S. Krantz, Cosby A. Stone, James A. Connelly, Allison E. Norton, Yasmin W. Khan Tags: Letters Source Type: research

The Iceberg Map of germline mutations in childhood cancer: focus on primary immunodeficiencies
Purpose of review The risk of cancer is higher, and its outcome is worse in patients with primary immunodeficiency (PID) than in members of the general population. Thus, the inter-relationship of malignant diseases with PIDs requires more study. Recent findings Large genetic screens identified a vast number of germline mutations in childhood cancer patient samples. Although TP53 was the most frequent single gene identified as mutated, many PID disorders like DNA repair defects are among the inborn causes of childhood cancer. We provide a comprehensive analysis of compiled data from seven recent studies that focused on...
Source: Current Opinion in Pediatrics - November 10, 2018 Category: Pediatrics Tags: ALLERGY, IMMUNOLOGY AND RELATED DISORDERS: Edited by Henry H. Bernstein Source Type: research

Advances in site-specific gene editing for primary immune deficiencies
Purpose of review Conventional gene therapy has been a successful, curative treatment modality for many primary immune deficiencies with significant improvements in the last decade. However, the risk of leukemic transformation with viral-mediated gene addition still remains, and unregulated gene addition is not an option for certain diseases in which the target gene is closely controlled. The recent bloom in genome modification platforms has created the opportunity to site-specifically correct mutated DNA base pairs or insert a corrective cDNA minigene while maintaining gene expression under control of endogenous regulat...
Source: Current Opinion in Allergy and Clinical Immunology - November 2, 2018 Category: Allergy & Immunology Tags: PRIMARY IMMUNE DEFICIENCY DISEASE: Edited by Stephen Jolles and M. Teresa (Maite) de la Morena Source Type: research

Regulatory T cell therapy for autoimmune and autoinflammatory diseases: the next frontier
FOXP3-expressing regulatory T cells (Tregs) are essential for self-tolerance with an emerging role in tissue repair and regeneration. Their ability to traffic to tissue and perform complex therapeutic tasks in response to the tissue microenvironment make them an attractive candidate for drug development. Early experiences of Treg cell therapy in graft-versus-host disease, type 1 diabetes, and organ transplant have shown that it is feasible, safe, and potentially efficacious in some settings. Many ongoing trials in a wide variety of diseases will further enhance our knowledge about the optimal approaches for Treg manufacturing and dosing.
Source: Journal of Allergy and Clinical Immunology - October 24, 2018 Category: Allergy & Immunology Authors: Jonathan H. Esensten, Yannick D. Muller, Jeffrey A. Bluestone, Qizhi Tang Source Type: research

Regulatory T-cell therapy for autoimmune and autoinflammatory diseases: The next frontier
Forkhead box P3 –expressing regulatory T (Treg) cells are essential for self-tolerance, with an emerging role in tissue repair and regeneration. Their ability to traffic to tissue and perform complex therapeutic tasks in response to the tissue microenvironment make them an attractive candidate for drug developmen t. Early experiences of Treg cell therapy in patients with graft-versus-host disease, type 1 diabetes, and organ transplantation have shown that it is feasible, safe, and potentially efficacious in some settings.
Source: Journal of Allergy and Clinical Immunology - October 24, 2018 Category: Allergy & Immunology Authors: Jonathan H. Esensten, Yannick D. Muller, Jeffrey A. Bluestone, Qizhi Tang Tags: Reviews and feature article Source Type: research

Analysis of eosinophilic esophagitis in children with repaired congenital esophageal atresia
A high prevalence of eosinophilic esophagitis (EoE) has been preliminarily reported in patients after repair of esophageal atresia (EA), but the basis of this association is unknown.
Source: Journal of Allergy and Clinical Immunology - October 24, 2018 Category: Allergy & Immunology Authors: Usha Krishnan, Chan Lijuan, Gifford J. Andrew, Marc E. Rothenberg, Ting Wen Source Type: research