ABSTRACT
Complete hematopoietic chimerism and tolerance of a liver allograft from a deceased male donor developed in a 9-year-old girl, with no evidence of graft-versus-host disease 17 months after transplantation. The tolerance was preceded by a period of severe hemolysis, reflecting partial chimerism that was refractory to standard therapies. The hemolysis resolved after the gradual withdrawal of all immunosuppressive therapy.
Subject(s)
Hemolysis/immunology , Liver Transplantation/immunology , Transplantation Chimera/immunology , Transplantation Tolerance/immunology , Child , Female , Graft vs Host Disease , Humans , Immunosuppression Therapy , Liver Failure, Acute/surgery , T-Lymphocytes/immunology , Transplantation Tolerance/genetics , Transplantation, HomologousABSTRACT
This document considers a number of scenarios involving complex haemoglobinopathies and provides 28 recommendations at both the clinical and laboratory levels on how these should be managed.
Subject(s)
DNA/genetics , Genetic Testing/methods , Hemoglobinopathies/diagnosis , Hemoglobinopathies/genetics , DNA/analysis , Female , Genetic Carrier Screening/methods , Hemoglobin, Sickle/genetics , Humans , Male , Pedigree , Thalassemia/geneticsABSTRACT
OBJECTIVE: To review the use of recombinant activated factor VII in paediatric cardiac surgery. DESIGN: Retrospective chart review. SETTING: Paediatric intensive care unit in a stand-alone university-affiliated children's hospital. PATIENTS AND PARTICIPANTS: Cardiac surgical patients who received recombinant activated factor VII (rFVIIa, NovoSeven; NovoNordisk, Copenhagen, Denmark) between June 2002 and June 2003 at The Children's Hospital at Westmead. RESULTS: Six children undergoing cardiac surgery received rFVIIa. Recombinant activated factor VII was administered if bleeding was excessive and persisted despite appropriate investigation and attention to haemostasis by surgical and medical staff. An intravenous dose of 180 microg/kg was given and repeated 2 h later. All of the six patients responded well to rFVIIa with achievement of haemostasis. No adverse events were noted. CONCLUSIONS: Recombinant activated factor VII achieved haemostasis in six paediatric cardiac surgical patients. Good outcomes and no adverse events were noted in these children.
Subject(s)
Factor VII/therapeutic use , Recombinant Proteins/therapeutic use , Thoracic Surgery , Child, Preschool , Factor VIIa , Humans , Infant , Intensive Care Units, Pediatric , Postoperative Care , Retrospective Studies , Treatment OutcomeABSTRACT
Shwachman Diamond syndrome (SDS) is a genetic disorder characterized by pancreatic hypoplasia, recurrent infection and bone marrow dysfunction. Some cases have an abnormality of chromosome 7, such as isochromosome 7q (i(7q)), which may be associated with the development of leukemia. We present a boy who was diagnosed with SDS at 19 months of age. From age 5-14 years, bone marrow cytogenetics has shown a consistent abnormality - i(7q), with an intermittent separate abnormality - deletion 20q, from age 11 years. During this time, the boy has been clinically well without leukemic signs, managed conservatively. We suggest that deletion 20q may be a non random secondary change in SDS with i(7q).
