About: Haploidentical Stem Cell Transplantation with Post-Transplant Cyclophosphamide for Primary Immune Deficiency Disorders in Children: Challenges and Outcome from a Tertiary Care Center in South India

Facets (new session)
Description
Metadata
Settings
- owl:sameAs
- Inference Rule:

About: Haploidentical Stem Cell Transplantation with Post-Transplant Cyclophosphamide for Primary Immune Deficiency Disorders in Children: Challenges and Outcome from a Tertiary Care Center in South India Goto Sponge NotDistinct Permalink

An Entity of Type : schema:ScholarlyArticle, within Data Space : wasabi.inria.fr associated with source document(s)

Attributes	Values
type	Academic Article research paper schema:ScholarlyArticle
isDefinedBy	Covid-on-the-Web dataset
has title	Haploidentical Stem Cell Transplantation with Post-Transplant Cyclophosphamide for Primary Immune Deficiency Disorders in Children: Challenges and Outcome from a Tertiary Care Center in South India
Creator	Jayakumar, Indira Patel, Shivani Ravichandran, Nikila Sivasankaran, Meena Uppuluri, Ramya Vaidhyanathan, Lakshman Raj, Revathi Ramakrishnan, Balasubramaniam Swaminathan, Venkateswaran Melarcode, Kesavan
Source	PMC
abstract	Haploidentical stem cell transplantation (haplo SCT) has emerged as an acceptable alternative to matched family donor transplantation for children diagnosed to have primary immune deficiency disorders (PIDs). We present data over 4 years on the challenges and efficacy of unmanipulated T cell replete haplo SCTs with post-transplant cyclophosphamide (PTCy) in children diagnosed to have PIDs. We performed a retrospective study in the pediatric blood and marrow transplantation unit where all children less than 18 years of age diagnosed to have PIDs and who underwent haplo SCT with PTCy from January 2014 to February 2018 were included in the study. Of the 16 transplants included in the study, 5 children were diagnosed to have Wiskott-Aldrich syndrome, 3 with congenital hemophagocytic lymphohistiocytosis, 2 each with Griscelli syndrome and Mendelian susceptibility to mycobacterial diseases, and one each with Chediak-Higashi syndrome, ORAI 1 mutation immune deficiency, severe combined immune deficiency, and Hyper IgM syndrome. The source of stem cells was PBSC in 62.5% and bone marrow in 32.5%. Engraftment by day 16–21 post hematopoietic stem cell transplantation was achieved in 75% transplants with 91% of these remaining in sustained complete chimerism. Acute skin and gut graft versus host disease of grade 2–3 were noted in 50% transplants and cytomegalovirus (CMV) reactivation in 43.7% transplants. One child with congenital HLH succumbed to refractory CMV, adenovirus, and BK virus infection. Cytokine release syndrome (CRS) was noted in 75% transplants with 2 children succumbing to the illness. Tocilizumab was successfully used early in one child. Overall mortality was found to be 37.5% with overall survival of 62.5% with a median follow-up of 23.3 months. In resource limited settings, PTCy has the potential to provide a cost-effective advantage in terms of accessibility of this curative procedure among children with PIDs.
has issue date	2019-02-18(xsd:dateTime)
bibo:doi	10.1007/s10875-019-00600-z
bibo:pmid	30778805
has license	no-cc
sha1sum (hex)	b7748287e19480de013b52a6ee4a3ddf80f60c94
schema:url	https://doi.org/10.1007/s10875-019-00600-z
resource representing a document's title	Haploidentical Stem Cell Transplantation with Post-Transplant Cyclophosphamide for Primary Immune Deficiency Disorders in Children: Challenges and Outcome from a Tertiary Care Center in South India
has PubMed Central identifier	PMC7100782
has PubMed identifier	30778805
schema:publication	J Clin Immunol
resource representing a document's body	covid:b7748287e19480de013b52a6ee4a3ddf80f60c94#body_text
is schema:about of	named entity 'age' named entity 'advantage' named entity 'gut' named entity 'procedure' named entity 'hemophagocytic lymphohistiocytosis' named entity '2018' named entity 'One' named entity 'transplants' named entity 'Hyper IgM syndrome' named entity 'ORAI' named entity 'Children' named entity 'BLOOD AND MARROW TRANSPLANTATION' named entity 'TERMS' named entity 'primary' named entity 'PIDs' named entity 'severe combined immune deficiency' named entity 'congenital' named entity 'The' named entity 'potential' named entity 'adenovirus' named entity 'congenital' named entity 'emerged' named entity 'cytomegalovirus' named entity 'study' named entity 'succumbing' named entity 'PIDs' named entity 'Immune' named entity 'CMV' named entity 'cytomegalovirus' named entity 'congenital' named entity 'refractory' named entity 'ORAI' named entity 'BK virus' named entity 'CRS' named entity 'SCT' named entity 'Cytokine release syndrome' named entity 'Chediak-Higashi syndrome' named entity 'immune deficiency' named entity 'graft versus host disease' named entity 'cyclophosphamide' named entity 'Wiskott-Aldrich syndrome' named entity 'congenital' named entity 'Mendelian' named entity 'CMV' named entity 'Stem Cell Transplantation' named entity 'Immune Deficiency' named entity 'infection' named entity 'SCID' named entity 'IL6' named entity 'infection' named entity 'GvHD' named entity 'IL6' named entity 'graft versus host disease' named entity 'chemotherapy' named entity 'survival rates' named entity 'cytokine' named entity 'nitroblue tetrazolium' named entity 'CRS' named entity 'antibodies' named entity 'total body irradiation' named entity 'cGy' named entity 'adverse effects' named entity 'cidofovir' named entity 'CRS' named entity '3, 4' named entity 'refractory' named entity 'CRS' named entity 'normal distribution' named entity 'CD34' named entity 'ARDS' named entity 'systemic viral' named entity 'HSCT'

Faceted Search & Find service v1.13.91 as of Mar 24 2020

Alternative Linked Data Documents: Sponger | ODE Content Formats:

RDF

ODATA

Microdata

About

OpenLink Virtuoso version 07.20.3229 as of Jul 10 2020, on Linux (x86_64-pc-linux-gnu), Single-Server Edition (94 GB total memory)
Data on this page belongs to its respective rights holders.
Virtuoso Faceted Browser Copyright © 2009-2024 OpenLink Software