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https://www.readbyqxmd.com/read/29412158/allogeneic-hematopoietic-stem-cell-transplantation-for-gata2-deficiency-using-a-busulfan-based-regimen
#1
Mark Parta, Nirali N Shah, Kristin Baird, Hind Rafei, Katherine R Calvo, Thomas Hughes, Kristen Cole, Meg Kenyon, Bazetta Blacklock Schuver, Jennifer Cuellar-Rodriguez, Christa S Zerbe, Steven M Holland, Dennis D Hickstein
Allogeneic hematopoietic stem cell transplantation (HSCT) reverses the bone marrow failure syndrome due to GATA2 deficiency. The intensity of conditioning required to achieve reliable engraftment and prevent relapse remains unclear. Here, we describe the results of a prospective study of HSCT in 22 patients with GATA2 deficiency using a busulfan-based conditioning regimen. The study included 2 matched related donor (MRD) recipients, 13 matched unrelated donor (URD) recipients, and 7 haploidentical related donor (HRD) recipients...
February 3, 2018: Biology of Blood and Marrow Transplantation
https://www.readbyqxmd.com/read/28288951/haploidentical-related-donor-hematopoietic-stem-cell-transplantation-for-dedicator-of-cytokinesis-8-deficiency-using-post-transplantation-cyclophosphamide
#2
Nirali N Shah, Alexandra F Freeman, Helen Su, Kristen Cole, Mark Parta, Niki M Moutsopoulos, Safa Baris, Elif Karakoc-Aydiner, Thomas E Hughes, Heidi H Kong, Steve M Holland, Dennis D Hickstein
Dedicator-of-cytokinesis 8 (DOCK8) deficiency, a primary immunodeficiency disease, can be reversed by allogeneic hematopoietic stem cell transplantation (HSCT); however, there are few reports describing the use of alternative donor sources for HSCT in DOCK8 deficiency. We describe HSCT for patients with DOCK8 deficiency who lack a matched related or unrelated donor using bone marrow from haploidentical related donors and post-transplantation cyclophosphamide (PT/Cy) for graft-versus-host disease (GVHD) prophylaxis...
June 2017: Biology of Blood and Marrow Transplantation
https://www.readbyqxmd.com/read/27641484/haploidentical-related-donor-hematopoietic-stem-cell-transplantation-with%C3%A2-post-transplantation-cyclophosphamide-for-dock8-deficiency
#3
Alexandra F Freeman, Nirali N Shah, Mark Parta, Helen C Su, Alessandra Brofferio, Irma Gradus-Pizlo, Sabah Butty, Thomas E Hughes, David E Kleiner, Daniele Avila, Theo Heller, Heidi H Kong, Steven M Holland, Dennis D Hickstein
No abstract text is available yet for this article.
November 2016: Journal of Allergy and Clinical Immunology in Practice
https://www.readbyqxmd.com/read/26453586/haploidentical-hematopoietic-cell-transplantation-with-post-transplant-cyclophosphamide-in-a-patient-with-chronic-granulomatous-disease-and-active-infection-a-first-report
#4
Mark Parta, Dianne Hilligoss, Corin Kelly, Nana Kwatemaa, Narda Theobald, Harry Malech, Elizabeth M Kang
PURPOSE: We describe haploidentical hematopoietic cell transplantation (HCT) with high-dose post-transplant cyclophosphamide (PTCy) in a boy with x-linked chronic granulomatous disease (CGD). METHODS: A persistent and life-threatening fungal infection was the indication for HSCT. Non-myeloablative conditioning with PTCy (50 mg/kg days 3 and 4) was used in the absence of fully matched donors. RESULTS: Engraftment occurred on day 24. The patient experienced Grade 2 graft-versus-host disease of the skin and gastrointestinal tract and CMV infection, both of which were controlled...
October 2015: Journal of Clinical Immunology
https://www.readbyqxmd.com/read/25885978/a-comparative-study-of-the-efficacy-of-intravenous-paracetamol-and-dexmedetomidine-on-peri-operative-hemodynamics-and-post-operative-analgesia-for-patients-undergoing-laparoscopic-cholecystectomy
#5
Sarbari Swaika, Nilakshi Parta, Surajit Chattopadhyay, Bikash Bisui, Sumantra Sarathi Banarjee, Somjit Chattarjee
BACKGROUND: Unrelieved post-operative pain may result in the physical suffering as well as multiple physiological and the psychological consequences, which may adversely affect the peri-operative outcome and contribute to increase the length of stay in hospital. OBJECTIVES: We designed this study to evaluate the effect of IV Paracetamol and Dexmedetomidine as multimodal analgesic technique on post-operative analgesia and to reduce the consumption of the systemic opioid and its adverse effects in cases of laparoscopic cholecystectomy...
September 2013: Anesthesia, Essays and Researches
https://www.readbyqxmd.com/read/25636378/matched-related-and-unrelated-donor-hematopoietic-stem-cell-transplantation-for-dock8-deficiency
#6
Jennifer Cuellar-Rodriguez, Alexandra F Freeman, Jennifer Grossman, Helen Su, Mark Parta, Heardley Murdock, Nirali Shah, Catherine Bollard, Heidi H Kong, Niki Moutsopoulos, Kelly Stone, Juan Gea-Banacloche, Steven M Holland, Dennis D Hickstein
We performed allogeneic hematopoietic stem cell transplantation in 6 patients with mutations in the dedicator-of-cytokinesis-8 (DOCK8) gene using a myeloablative conditioning regimen consisting of busulfan 3.2 mg/kg/day i.v. for 4 days and fludarabine 40 mg/m(2)/day for 4 days. Three patients received allografts from matched related donors and 3 patients from matched unrelated donors. Two patients received peripheral blood stem cells and 4 patients bone marrow hematopoietic stem cells. Tacrolimus and short-course methotrexate on days 1, 3, 6, and 11 were used for graft-versus-host-disease (GVHD) prophylaxis...
June 2015: Biology of Blood and Marrow Transplantation
https://www.readbyqxmd.com/read/25111582/nonmyeloablative-allogeneic-hematopoietic-stem-cell-transplantation-for-gata2-deficiency
#7
Jennifer Grossman, Jennifer Cuellar-Rodriguez, Juan Gea-Banacloche, Christa Zerbe, Katherine Calvo, Thomas Hughes, Fran Hakim, Kristen Cole, Mark Parta, Alexandra Freeman, Steven M Holland, Dennis D Hickstein
We treated 14 patients with GATA2 deficiency using a nonmyeloablative allogeneic hematopoietic stem cell transplantation regimen. Four patients received peripheral blood stem cells from matched related donors (MRD), 4 patients received peripheral blood stem cells from matched unrelated donors (URD), 4 patients received hematopoietic stem cells from umbilical cord blood donors (UCB), and 2 patients received bone marrow cells from haploidentical related donors. MRD and URD recipients received conditioning with 3 days of fludarabine and 200 cGy total body irradiation (TBI)...
December 2014: Biology of Blood and Marrow Transplantation
https://www.readbyqxmd.com/read/7620852/absorption-of-polychlorinated-biphenyls-dibenzo-p-dioxins-and-dibenzofurans-by-breast-fed-infants
#8
COMPARATIVE STUDY
P Dahl, G Lindström, K Wiberg, C Rappe
The absorption of polychlorinated biphenyls, dibenzo-p-dioxins and dibenzofurans was studied in four breast-fed infants. The absorption was measured by comparing the estimated total intake and the excretion in faeces, during 48 hours, at three different time points; 1, 2 and 3 months post parta. The levels of the analysed compounds in the human milk are typical for Sweden, i.e approximately 20 ppt toxic equivalents for the dibenzo-p-dioxins and dibenzofurans and for the polychlorinated biphenyls approximately 16 ppt toxic equivalents...
June 1995: Chemosphere
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