Does She Have SCID?

Discussion Severe-combined immunodefiency (SCID) is actually a group of inherited disorders with an absence or dysfunction of T, B and NK cells that results in severe dysfunction of the immune system. SCID is a primary immunodeficiency. SCID was thought to be a rare disorder but with the advent of neonatal screening the incidence in the US general population is estimated at ~1 in 58,000 live births, but with numbers higher or lower depending on the specific population. Patients often have failure to thrive, oral candidiasis, and diarrhea as infants as well as a variety of infectious diseases. They can also have interstitial lung disease due to Pneumocystis jiroveci or graft-vs-host disease because of maternal lymphocyte engraphment. Without treatment to reconstitute the immune system, patients usually die at 90% long-term survival after transplantation when infants are
Source: PediatricEducation.org - Category: Pediatrics Authors: Tags: Uncategorized Source Type: news

Related Links:

Publication date: Available online 18 April 2019Source: Stem Cell ReportsAuthor(s): Friederike Matheus, Ejona Rusha, Rizwan Rehimi, Lena Molitor, Anna Pertek, Miha Modic, Regina Feederle, Andrew Flatley, Elisabeth Kremmer, Arie Geerlof, Valentyna Rishko, Alvaro Rada-Iglesias, Micha DrukkerSummaryThe neural crest (NC) gives rise to a multitude of fetal tissues, and its misregulation is implicated in congenital malformations. Here, we investigated molecular mechanisms pertaining to NC-related symptoms in Bohring-Opitz syndrome (BOS), a developmental disorder linked to mutations in the Polycomb group factor Additional sex com...
Source: Stem Cell Reports - Category: Stem Cells Source Type: research
Publication date: Available online 18 April 2019Source: Stem Cell ReportsAuthor(s): Fani Papagiannouli, Cameron Wynn Berry, Margaret T. FullerSummaryTissue homeostasis and repair relies on proper communication of stem cells and their differentiating daughters with the local tissue microenvironment. In the Drosophila male germline adult stem cell lineage, germ cells proliferate and progressively differentiate enclosed in supportive somatic cyst cells, forming a small organoid, the functional unit of differentiation. Here we show that cell polarity and vesicle trafficking influence signal transduction in cyst cells, with pro...
Source: Stem Cell Reports - Category: Stem Cells Source Type: research
Publication date: Available online 18 April 2019Source: Stem Cell ReportsAuthor(s): Yefim Zaltsman, Sayaka Masuko, Joshua J. Bensen, Laura L. KiesslingSummaryLeveraging the extraordinary potential of human pluripotent stem cells (hPSCs) requires an understanding of the mechanisms underlying cell-fate decisions. Substrate elasticity can induce differentiation by signaling through the transcriptional coactivator Yes-associated protein (YAP). Cells cultured on surfaces mimicking brain elasticity exclude YAP from their nuclei and differentiate to neurons. How YAP localization is controlled during neural differentiation has bee...
Source: Stem Cell Reports - Category: Stem Cells Source Type: research
Publication date: 18 April 2019Source: Cell, Volume 177, Issue 3Author(s): Wantong Yao, Anirban MaitraIn this issue of Cell, Lytle et al. (2019) integrate functional genomic approaches to identify molecular dependencies of pancreatic cancer stem cells that may be exploited therapeutically. The comprehensive analysis reveals an unexpected role for retinoic acid receptor-related orphan receptor gamma (RORγ), a T-cell-associated transcription factor, in defining the stemness and the aggressive behavior of pancreatic cancer.
Source: Cell - Category: Cytology Source Type: research
In conclusion, our results indicated that CEP55 played an important role in promoting the invasion and migration of U251 cell and self-renewal of glioma stem like cells which might be a new therapeutic target for glioma.
Source: Neuroscience Letters - Category: Neuroscience Source Type: research
Publication date: Available online 18 April 2019Source: Neuroscience &Biobehavioral ReviewsAuthor(s): Lisa M. ChristianAbstractBirth prior to full term is a substantial public health issue. In the US, ˜400,000 babies per year are born preterm (1 million are early term (37–386/7 weeks). Birth prior to full term confers risk both immediate and long term, including neonatal intensive care, decrements in school performance, and increased mortality risk from infancy through young adulthood. Risk for low birth weight and preterm birth are 1.5-2 times greater among African Americans versus Whites. Psychosocial str...
Source: Neuroscience and Biobehavioral Reviews - Category: Neuroscience Source Type: research
Discussion: Our findings are consistent with previous observations of increases in lipid mobilisation, coupled with preferential transfer of DHA to the fetus, with advancing gestation. However the number of eligible studies was small and further investigations are required.
Source: Prostaglandins, Leukotrienes and Essential Fatty Acids (PLEFA) - Category: Lipidology Source Type: research
Overall hand hygiene compliance increased from 2009 to 2017; associated drop seen in HA - SAB incidence
Source: The Doctors Lounge - Psychiatry - Category: Psychiatry Tags: Cardiology, Dermatology, Endocrinology, Family Medicine, Geriatrics, Gastroenterology, Gynecology, Infections, AIDS, Internal Medicine, Allergy, Critical Care, Emergency Medicine, Nephrology, Neurology, Nursing, Oncology, Ophthalmology, Orthopedics, ENT, Source Type: news
Graduates with loan forgiveness/repayment program more likely to enter primary care fields
Source: The Doctors Lounge - Psychiatry - Category: Psychiatry Tags: Cardiology, Dermatology, Endocrinology, Family Medicine, Geriatrics, Gastroenterology, Gynecology, Infections, AIDS, Internal Medicine, Allergy, Critical Care, Emergency Medicine, Nephrology, Neurology, Oncology, Ophthalmology, Orthopedics, ENT, Pathology Source Type: news
CONCLUSIONS: Our findings indicate that the mechanism underlying the therapeutic effect of cupping therapy involves upregulation of vascular and lymphatic endothelial markers (CD34+, LYVE-1+, and CD34+LYVE-1+) in local tissues, which in turn promotes local new lymphatic vessel formation through the expression of PROX1. PMID: 30993665 [PubMed - as supplied by publisher]
Source: Folia Morphologica - Category: Anatomy Tags: Folia Morphol (Warsz) Source Type: research
More News: Allergy | Allergy & Immunology | Babies | Breastfed | Children | Databases & Libraries | Department of Health | Gene Therapy | Genetics | Hospitals | Infectious Diseases | Internet | Interstitial Lung Disease | Jaundice | Laboratory Medicine | Learning | Lung Transplant | Microdeletion Syndromes | Pathology | Pediatrics | Perinatology & Neonatology | Pregnancy | Primary Immunodeficiency Disease | Stem Cell Therapy | Stem Cells | Study | Toxicology | Transplant Surgery | Transplants | Universities & Medical Training