Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0029713 (immaturity)
 4,335 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Umbilical cord blood collected and cryopreserved at birth contains enough hematopoietic progenitor stem cells for engraftment. HLA identical sibling cord blood transplant has been performed for the first time, in a child with Fanconi anemia. Three years latter, this child is alive with a complete donor type bone marrow. Since this first attempt, several other patients with other diseases have been transplanted successfully. Cord blood banking is a safe and easy procedure. Due to the high proliferative capacity of neonatal hematopoietic progenitors and to the relative immunological functional immaturity of neonatal lymphocytes cord blood cells could be used for matched unrelated or partially mismatched transplants.
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PMID:Clinical applications of stem cell transfusion from cord blood and rationale for cord blood banking. 135 18

Myasthenia gravis is considered to be an autoimmune disease in which several factors reciprocally influence the clinical type and course. We investigated the relative importance of the following factors: anti-acetylcholine receptor antibody (AChR Ab), HLA, age at onset, autoimmunity, thymic abnormality, duration of treatment, change in AChR Ab titer and immunosuppressive therapy. The pretreatment-AChR Ab titer and HLA were shown to significantly influence the clinical type. On the other hand, the age at onset significantly influenced the clinical course. The finding that with an onset at less than 5-year-old there was a tendency for a good prognosis suggests an association between the immaturity of the muscle and immune systems, and the clinical course.
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PMID:Factors influencing the clinical type and course of myasthenia gravis. 162 31

It has been shown that human umbilical cord blood contains stem/progenitor cells comparable in number to that of adult bone marrow. We report here the first successful cases of transplantation of umbilical cord blood cells. The patients were suffering from Fanconi's anemia, complicated by severe aplastic anemia. During pregnancy, it was shown that the mother was carrying a sibling unaffected by the disease and with HLA identical to the patient. Cord blood was collected and frozen in liquid nitrogen at birth. After conditioning with low-dose cyclophosphamide (20 mg/kg) and thoraco-abdominal irradiation (5 grays), the patients received a cord blood transplant of thawed cells. Three patients have been transplanted without any immediate side-effect. One has not enough follow-up, but two patients are alive and well with complete donor hematologic reconstitution and no chronic graft versus host disease. Potential developments of this technique are an extension of applicability with regard to other diseases that might be transplanted and whether such transplants can be performed in adults. The relative immaturity of the lymphoid system at birth may be advantageous in decreasing the graft versus host reaction if these cells are used in a mismatched transplantation. Cord blood cell banks may be useful for transplants in patients lacking an HLA-identical donor.
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PMID:Transplantation of umbilical cord blood in Fanconi's anemia. 198 24

To the best of our knowledge this is the seventh case report of monozygotic twins both affected by myasthenia gravis (MG). The monozygotism was proven by sex identity, blood group and HLA determinations ('O' Rh+, A2, A19.2, B40, CW3). One paternal aunt was also affected and the three cases have high titles of anti-acetylcholine receptor antibodies and anti-striated muscle antibodies, which indicate an acquired form of MG. After several myasthenic crises the twins are now doing well with corticosteroid therapy. The paternal aunt has a more benign form of MG, with ocular and limb involvement (grade IIa of Osserman) and was submitted to thymectomy. The authors discuss the use of corticosteroid for the infantile form of MG instead of thymectomy, based on the immaturity of the immune system in the young age. The use of other immunosuppressive drugs is not advisable because of potential hazardous development of neoplasms.
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PMID:[Familial myasthenia gravis: a case report in identical twins]. 268 10

A series of 27 adult patients with chronic myelocytic leukaemia (CML) were examined for erythrocyte C3b receptor (EC3bR) expression. Twenty-five patients were successfully HLA typed and there was a positive association of CML with HLA-B40 (P less than 0.01). Only 10 (37%) of the patients were EC3bR positive (P less than 0.00001) compared to 223 healthy controls of whom 198 (89%) were EC3bR positive. The positivity of EC3bR and HLA-B40 occurred simultaneously in six patients (P less than 0.05). HLA-B40 positivity and EC3bR expression were correlated with the following variables: age at the time of CML diagnoses, duration of CML (until death or the end of follow-up period 1 July 1983), the stage of CML and simultaneous medication. EC3bR positive patients were significantly more often in remission (P less than 0.05) and also had shorter duration of the disease (P less than 0.005) then did EC3bR negative ones. No significant correlation existed between EC3bR status and the other parameters. The presence of HLA-B40 did not associate with any of the listed variables. These findings may indicate the loss of the receptor in the course of CML with increasing immaturity of cells released from bone marrow.
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PMID:Chronic myelocytic leukaemia: HLA association and decreased erythrocyte C3b receptor expression. 315 1

Study of inverse alloimmunization, previously investigated in HLA-system, was continued by the investigation of maternal--foetal alloimmunization on Gm-system immunoglobulin allotypes (Steinberg-Speiser's phenomenon) in 105 cases of the newborn (cord blood) on antigen determinants of their mothers. The work includes investigation of Gm- and InV-system allotipie, using mothers' and their infants' blood by agglutination inhibition method (according to C. Ropartz and L. Rivat). Later on, using the same method, infants' sensitization to mothers' G-class immunoglobulin specificity was investigated. Allotypes bound to subclasses of G-class immunoglobulins, discrepancy in expression of certain specities of mothers and their infants, possible immaturity of some allotypes at birth and occurrence of immune response in both mother and infant are discussed in this paper. The occurrence we noted is a contribution to investigations of immunoglobulin genetics through Gm-system allotipie bound to protein primary structure.
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PMID:[Maternal-fetal alloimmunization in the Gm and InV system (inverse type of immunization in pregnancy)]. 652 64

T cell blasts resulting from stimulating human cord blood (CB) mononuclear cells (MC) and adult peripheral blood (APB) MC with a bacterial superantigen, toxic shock syndrome toxin-1 (TSST-1), and then with human rIL-2 were investigated for their reactions to restimulation with TSST-1. Expression of TCR V beta 2, which determines the potential reactivity to TSST-1 and of CD45RO, was increased in both TSST-1-induced CB and APB T cell blasts. Most preparations of the TSST-1-induced CB T cell blasts exhibited low or no production of IL-2 and IL-4 in response to restimulation with TSST-1, while the APB T cell blasts showed high responses. TSST-1-induced T cell blasts derived from APB T cells depleted of both CD45RO+ T cells and HLA class II+ T cells showed high IL-2 production in response to restimulation with TSST-1. TSST-1-induced CB T cell blasts generated in the presence of DR+ L cells with high accessory cell activity still showed a low response to restimulation with TSST-1. These results indicate that CB T cells are inherently highly susceptible to tolerance induction by bacterial superantigens, suggesting the immunologic immaturity of CB T cells.
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PMID:Evidence for immunologic immaturity of cord blood T cells. Cord blood T cells are susceptible to tolerance induction to in vitro stimulation with a superantigen. 759 32

Umbilical cord blood (UCB) is an attractive potential alternative to bone marrow (BM) as a source of hematopoietic progenitor cells since the number of progenitors in UCB is similar or even greater than that in normal BM. It was the aim of the present study to analyze the degree of immaturity of UCB progenitor cells. UCB mononuclear (MNC) and/or CD34+ cells were tested for surface antigen phenotype, expression of cytokines receptor, effect of stem cell factor (SCF) on colony growth, resistance to mafosfamide and replating potential. We have found that 34.9 +/- 3.4% and 77.9 +/- 2.6% of UCB CD34+ cells did not express CD38 and CD45RA antigens, respectively, suggesting that UCB contains a high proportion of immature progenitor cells. By means of three-color analysis, the receptor for SCF was detected on the majority of the CD34+ HLA-DR+ subpopulation; in fact, 81.8% +/- 4.3% of CD34+ HLA-DR+ cells were defined as SCF(low) and 8.1 +/- 1.5% as SCF(high). Colony growth of MNC and CD34+ cells was enhanced by the addition of SCF to methylcellulose mixture, resulting in a statistically significant increase in CFU-GM and CFU-GEMM but not in BFU-E numbers. UCB progenitor cells showed a higher resistance to mafosfamide treatment, in comparison to BM; the addition of SCF to the culture medium resulted in a statistically significant increase in mafosfamide concentration required to inhibit 95% of colony growth (P < or = 0.05). Moreover, as shown by single colony transfer assays, the presence of SCF in primary cultures promoted a significantly higher replating potential for both untreated (42 +/- 3.3% vs 21 +/- 4.6%, P < or = 0.018) and mafosfamide-treated samples (62 +/- 5.6% vs 44 +/- 6.1%, P < or = 0.018). In conclusion, UCB is a source of progenitor cells with immature characteristics in terms of surface antigen expression, distribution of SCF receptor, resistance to mafosfamide and replating potential. Therefore, UCB progenitor cells represent an ideal candidate population for experimental programs involving gene transfer and ex vivo stem cell expansion.
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PMID:Biologic and phenotypic analysis of early hematopoietic progenitor cells in umbilical cord blood. 944 33

Hematopoietic progenitor cells are present in umbilical cord blood; placental blood (PB) previously considered as waste product now constitutes an alternative source of hematopoietic stem cells for bone marrow reconstitution. This has promoted the establishment of cord blood banks for use in unrelated transplants. The banking of PB offers many advantages: the donors do not require anesthesia, stored PB can be a valuable source of stem cells for patients from ethnic minorities underrepresented in volunteer registers, and stored PB can be made available much faster than bone marrow from unrelated donors. Preliminary clinical experience suggests that, due to the immunological immaturity of PB cells, graft versus host disease might be lower than when using bone marrow from adult donors and HLA restrictions might be less stringent. If the number of nucleated cells in PB often appears low for patients weighing more than 40 kg, clinical data suggest that the number of stem cells may be sufficient for adult transplantation. The number of cord blood banks throughout the world is increasing rapidly. In the USA and Europe, more than 10,500 PB units are stored and available for transplantation. In the next 5 years, a total of 50,000 PB will be reached which may be sufficient to provide for the majority of candidates for unrelated BM transplantation. The practices of umbilical cord blood collection, mother selection, infectious disease screening, cell manipulation and storage must be standardized. Some accreditation process should be mandatory for assessing operating procedures and the quality assurance programs of the banks, and for allowing the international exchange of placental blood between transplant centers.
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PMID:[Cord blood banks--unrelated transplants]. 957 80

Bone marrow transplantation is limited due to the lack of HLA-matched donors and to the frequent occurrence of GvHD. Hemopoietic transplants using cord blood cells are being increasingly used in pediatric patients. In this study, the immunophenotypic characteristics of cord blood cells have been investigated by one or two-color flow cytometric analysis. The CB cells were characterized by a low proportion of CD3+ T-cells, increased CD4/CD8 and CD45RA/CD45RO ratios, minimal expression of HLA-DR, increased proportion of CD5CD19 double positive B-cells, while CD3- CD8+ and CD3- CD7+ subsets, not usually found in adult PB, were detected. These data reflect the immaturity of CB cells as assessed by immunophenotypic analysis suggesting that it could be a valuable alternative source of transplantable hematopoietic progenitor cells and might alleviate some of the problems associated with bone marrow or peripheral blood transplantation.
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PMID:Phenotypic characteristics of cord blood hemopoietic cells. 968 Jan 4


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