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What Are Causes of Childhood Interstital Lung Disease?
Discussion Interstitial lung disease in children (chILD) is less understood than adult interstitial lung disease (ILD) and is rare. Prevalence is ~ 0.13 to 16.2 per 100,000 children under age 17 years. chILD is an general term for respiratory disorders that are heterogeneous, chronic and impair lung function. While some define diffuse parenchymal lung disease (DPLD) separately, the term ILD usually encompases DPLD. chILD has variable definitions and is “usually diagnosed if three of the following features are present: 1) respiratory symptoms (cough, rapid and/or difficult breathing, and exercise intolerance), 2) res...
Source: PediatricEducation.org - August 1, 2022 Category: Pediatrics Authors: Pediatric Education Tags: Uncategorized Source Type: news

Drug accelerates blood system ’s recovery after chemotherapy, radiation
A drug developed by UCLA physician-scientists and chemists speeds up the regeneration of mouse and human blood stem cells after exposure to radiation. If the results can be replicated in humans, the compound could help people recover quicker from chemotherapy, radiation and bone marrow transplants.The study, published in Nature Communications,also sheds light on the basic biology behind blood stem cell regeneration and the role of a specific molecular process that is blocked by the new drug. Blood stem cells reside in the bone marrow and produce all of the body ’s blood and immune cells.“We’re very excited about the ...
Source: UCLA Newsroom: Health Sciences - August 14, 2019 Category: Universities & Medical Training Source Type: news

Myeloid-Derived Suppressor Cells: Ductile Targets in Disease
Discussion MDSCs violently emerge in pathological conditions in an attempt to limit potentially harmful immune and inflammatory responses. Mechanisms supporting their expansion and survival are deeply investigated in cancer, in the perspective to reactivate specific antitumor responses and prevent their contribution to disease evolution. These findings will likely contribute to improve the targeting of MDSCs in anticancer immunotherapies, either alone or in combination with immune checkpoint inhibitors. New evidence indicates that the expansion of myeloid cell differentiation in pathology is subject to fine-tuning, as its...
Source: Frontiers in Immunology - May 2, 2019 Category: Allergy & Immunology Source Type: research

Functional Antibody Responses Following Allogeneic Stem Cell Transplantation for TP53 Mutant pre-B-ALL in a Patient With X-Linked Agammaglobulinemia
Discussion This case demonstrates successful cure of pre-B-ALL complicating XLA by alloSCT with restoration of B-cell development and functional antibody response. We are aware of only one previous case of pre-B-ALL in an XLA patient (21), which suggests that human BTK deficiency in itself does not predispose to pre-B-ALL. However, there are data to suggest that BTK may act as a tumor suppressor, and BTK deficiency may predispose to tumor development following a “second hit.” Mice with a genetic deficiency in Slp65, a gene encoding an adaptor protein that functions together with BTK, have a block in proge...
Source: Frontiers in Immunology - April 25, 2019 Category: Allergy & Immunology Source Type: research

Sanguinarine Induces Apoptosis Pathway in Multiple Myeloma Cell Lines via Inhibition of the JaK2/STAT3 Signaling
In this study, we aimed to investigate the potential anti-proliferative and pro-apoptotic activities of SNG in a panel of MM cell lines (U266, IM9, MM1S, and RPMI-8226). SNG treatment of MM cells resulted in a dose-dependent decrease in cell viability through mitochondrial membrane potential loss and activation of caspase 3, 9, and cleavage of PARP. Pre-treatment of MM cells with a universal caspase inhibitor, Z-VAD-FMK, prevented SNG mediated loss of cell viability, apoptosis, and caspase activation, confirming that SNG-mediated apoptosis is caspase-dependent. The SNG-mediated apoptosis appears to be resulted from suppres...
Source: Frontiers in Oncology - April 16, 2019 Category: Cancer & Oncology Source Type: research

Incidence and Outcome of Invasive Fungal Diseases in Children With Hematological Malignancies and/or Allogeneic Hematopoietic Stem Cell Transplantation: Results of a Prospective Multicenter Study
Conclusion: This prospective multicenter study provides information on the current incidence and outcome of IFD in the real life setting. Practice variation between the centers may help to ultimately improve antifungal management in children at highest risk for IFDs. Introduction Available data on the incidence and outcome of invasive fungal diseases (IFD) in children treated for a hematological malignancy or undergoing allogeneic hematopoietic stem cell transplantation (HSCT) are mostly based on single site, retrospective studies or on studies performed prior to the availability of newer compounds such as broad-sp...
Source: Frontiers in Microbiology - April 15, 2019 Category: Microbiology Source Type: research

A Systematic Review on Predisposition to Lymphoid (B and T cell) Neoplasias in Patients With Primary Immunodeficiencies and Immune Dysregulatory Disorders (Inborn Errors of Immunity)
Conclusions Though this is not a comprehensive summary of malignancies in PIDDs, or even lymphoproliferative disease in this area, this review summarizes the Medline-indexed published reports of B and T lymphomas in patients with PIDDs. This report highlights the diversity of malignant lymphoproliferative disorders in setting of PIDDs, and its associated challenges of diagnosis and treatment. The pathological classification and nomenclature for the lymphoid malignancies with variably reported and postulated underlying mechanisms were inconsistent and inadequate for many of these published reports. A wide range of treatmen...
Source: Frontiers in Immunology - April 15, 2019 Category: Allergy & Immunology Source Type: research

Genetic factors control regenerative properties of blood-forming stem cells, UCLA studies show
FINDINGSResearchers from the UCLA Department of Medicine, Division of Hematology Oncology and the  Eli and Edythe Broad Center of Regenerative Medicine and Stem Cell Research at UCLA have published two studies that define how key genetic factors affect blood-forming stem cells by either accelerating or hindering the cells ’ regenerative properties. The findings could one day lead to improved treatments for people undergoing common therapies for cancer such as chemotherapy and radiation. BACKGROUNDBlood-forming stem cells, or hematopoietic stem cells, are found in the bone marrow. These cells have two unique properties:...
Source: UCLA Newsroom: Health Sciences - December 5, 2016 Category: Universities & Medical Training Source Type: news

UCLA researchers unlock protein key to harnessing regenerative power of blood stem cells
In this study, the authors showed that a cell surface protein called protein tyrosine phosphatase-sigma (PTP-sigma) regulates the critical process called engraftment, which is how HSCs start to grow and make healthy blood cells after transplantation. Mamle Quarmyne, a graduate student Chute’s lab and first author of the study, demonstrated that PTP-sigma is produced (expressed) on a high percentage of mouse and human HSCs. She showed further that genetic deletion of PTP-sigma in mice markedly increased the ability of HSCs to engraft in transplanted mice.  In a complementary study, Quarmyne demonstrated that selection o...
Source: UCLA Newsroom: Health Sciences - December 8, 2014 Category: Universities & Medical Training Source Type: news

Small Molecule Imaging of Fungi by Positron Emission Tomography Scanning
This technology relates to the field of radioactive, isotopically-labeled calcofluor derivatives and uses of such compounds to detect a broad spectrum of filamentous fungi including pathogenic species such asAspergillus andMucorales, by diagnostic imaging methods such as positron emission tomography (PET) scanning.Aspergillosis and other filamentous fungal infections are increasingly common fungal lung infection with high mortality rates (over 50%) in immune compromised patients, such as those receiving chemotherapy, stem cell/organ transplantation, or HIV patients. One-year survival of the infected patients ranges from 59...
Source: NIH OTT Licensing Opportunities - July 9, 2014 Category: Research Authors: ajoyprabhu3 Source Type: research

Combined DOCK8 and CLEC7A mutations causing immunodeficiency in 3 brothers with diarrhea, eczema, and infections
We report 4 brothers from a consanguineous Italian family with an immunodeficiency syndrome characterized by severe eczema, milk and egg allergies, recurrent infections, intractable diarrhea, failure to thrive, and, in 2 of the 3 surviving brothers, lymphoma. One of the brothers (patient II-2) died at age 12 months of cardiovascular failure caused by intractable diarrhea, and the other 3 were evaluated as part of this study ( and , A). The proband (patient II-1) presented shortly after birth with severe eczema and intractable diarrhea without gastrointestinal infection. In the first year of life, he had multiple allergies ...
Source: Journal of Allergy and Clinical Immunology - February 1, 2013 Category: Allergy & Immunology Authors: Darrell L. Dinwiddie, Stephen F. Kingsmore, Sonia Caracciolo, Giuseppe Rossi, Daniele Moratto, Cinzia Mazza, Cristiano Sabelli, Rosa Bacchetta, Laura Passerini, Chiara Magri, Callum J. Bell, Neil A. Miller, Shannon L. Hateley, Carol J. Saunders, Lu Zhang, Tags: Letters to the Editor Source Type: research