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1.
J Pediatr Hematol Oncol ; 40(2): e129-e132, 2018 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-28816796

RESUMO

Leukemic relapse in the central nervous system (CNS) after conventional treatment is associated with a poor prognosis. The effectiveness and safety of IV infusion of human leukocyte antigen (HLA)-mismatched lymphocytes for leukemia, and intrathecal (IT) infusion of HLA-mismatched lymphocytes for cerebrospinal fluid (CSF) dissemination of medulloblastoma have been reported. A 13-year-old girl (HLA-A31) was diagnosed as relapsing from Philadelphia chromosome-positive acute leukemia in the CNS after receiving chemotherapy, tyrosine kinase inhibitors, haploidentical hematopoietic stem cell transplantation (HSCT) from her father (HLA-A31), and craniospinal irradiation. We performed an IT infusion of haploidentical lymphocytes from her mother. Peripheral blood mononuclear cells obtained from her mother (HLA-A31) were administered by IT infusion weekly. Examination of CSF 1 week after first IT showed that lymphocyte counts had increased markedly and the breakpoint cluster region/abelson-bearing cells had disappeared. Furthermore, CD3 T cells in the CSF were negative for HLA-A31, and expressed high HLA-DR. These results indicate the infused non-HSCT-donor lymphocytes did not survive, and that the HSCT donor(father)-derived lymphocytes migrated to the CSF and were activated. The patient showed partial remission for 2 months following this therapy. Serious adverse reactions and graft versus host disease were not observed. To control leukemic CNS dissemination, haploidentical nondonor lymphocytes might contribute to a graft versus leukemia effect.

2.
J Allergy Clin Immunol ; 141(1): 322-328.e10, 2018 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-28392333

RESUMO

BACKGROUND: Rare DNA breakage repair disorders predispose to infection and lymphoreticular malignancies. Hematopoietic cell transplantation (HCT) is curative, but coadministered chemotherapy or radiotherapy is damaging because of systemic radiosensitivity. We collected HCT outcome data for Nijmegen breakage syndrome, DNA ligase IV deficiency, Cernunnos-XRCC4-like factor (Cernunnos-XLF) deficiency, and ataxia-telangiectasia (AT). METHODS: Data from 38 centers worldwide, including indication, donor, conditioning regimen, graft-versus-host disease, and outcome, were analyzed. Conditioning was classified as myeloablative conditioning (MAC) if it contained radiotherapy or alkylators and reduced-intensity conditioning (RIC) if no alkylators and/or 150 mg/m2 fludarabine or less and 40 mg/kg cyclophosphamide or less were used. RESULTS: Fifty-five new, 14 updated, and 18 previously published patients were analyzed. Median age at HCT was 48 months (range, 1.5-552 months). Twenty-nine patients underwent transplantation for infection, 21 had malignancy, 13 had bone marrow failure, 13 received pre-emptive transplantation, 5 had multiple indications, and 6 had no information. Twenty-two received MAC, 59 received RIC, and 4 were infused; information was unavailable for 2 patients. Seventy-three of 77 patients with DNA ligase IV deficiency, Cernunnos-XLF deficiency, or Nijmegen breakage syndrome received conditioning. Survival was 53 (69%) of 77 and was worse for those receiving MAC than for those receiving RIC (P = .006). Most deaths occurred early after transplantation, suggesting poor tolerance of conditioning. Survival in patients with AT was 25%. Forty-one (49%) of 83 patients experienced acute GvHD, which was less frequent in those receiving RIC compared with those receiving MAC (26/56 [46%] vs 12/21 [57%], P = .45). Median follow-up was 35 months (range, 2-168 months). No secondary malignancies were reported during 15 years of follow-up. Growth and developmental delay remained after HCT; immune-mediated complications resolved. CONCLUSION: RIC HCT resolves DNA repair disorder-associated immunodeficiency. Long-term follow-up is required for secondary malignancy surveillance. Routine HCT for AT is not recommended.

3.
Child Neurol Open ; 3: 2329048X15620641, 2016 Jan-Dec.
Artigo em Inglês | MEDLINE | ID: mdl-28503601

RESUMO

A 16-month-old girl was diagnosed with Epstein-Barr virus hemophagocytic lymphohistiocytosis and transferred to our hospital on the 58th day of the hemophagocytic lymphohistiocytosis after treatment failure according to the Hemophagocytic Lymphohistiocytosis-2004 protocol. On admission to our hospital, she had a flaccid paralysis of her lower limbs. Nerve conduction studies showed a acute motor axonal neuropathy, and a diagnosis of Guillain-Barre syndrome was established. Intravenous immunoglobulin G was started on the 57th day of the Guillain-Barre syndrome. To date, her neurological recovery is incomplete. For hemophagocytic lymphohistiocytosis, after treatment failure of THP-COP regimen (pirarubicin, cyclophosphamide, vincristine, and prednisone) and 2 courses of ESCAP regimen (etoposide, prednisone, cytarabine, L-asparaginase), we are now in the process of coordinating unrelated umbilical cord blood transplantation. To the best of our knowledge, we report the youngest case of Guillain-Barre syndrome accompanied by Epstein-Barr virus hemophagocytic lymphohistiocytosis. Rapid progression of Guillain-Barre syndrome, the electrophysiological subtype of Guillain-Barre syndrome, and treatment delay possibly led to poor neurological outcome.

4.
Clin Immunol ; 160(2): 255-60, 2015 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-26172957

RESUMO

We herein describe a case of a 17-year-old boy with intractable common warts, short stature, microcephaly and slowly-progressing pancytopenia. Simultaneous quantification of T-cell receptor recombination excision circles (TREC) and immunoglobulin κ-deleting recombination excision circles (KREC) suggested very poor generation of both T-cells and B-cells. By whole exome sequencing, novel compound heterozygous mutations were identified in the patient's DNA ligase IV (LIG4) gene. The diagnosis of LIG4 syndrome was confirmed by delayed DNA double-strand break repair kinetics in γ-irradiated fibroblasts from the patient and their restoration by an introduction of wild-type LIG4. Although the patient received allogeneic hematopoietic stem cell transplantation from his haploidentical mother, he unfortunately expired due to an insufficiently reconstructed immune system. An earlier definitive diagnosis using TREC/KREC quantification and whole exome sequencing would thereby allow earlier intervention, which would be essential for improving long-term survival in similar cases with slowly-progressing LIG4 syndrome masked in adolescents.


Assuntos
Anormalidades Craniofaciais/genética , DNA Ligases/genética , Transtornos do Crescimento/genética , Síndromes de Imunodeficiência/genética , Microcefalia/genética , Pancitopenia/genética , Imunodeficiência Combinada Severa/genética , Adolescente , DNA Ligase Dependente de ATP , Humanos , Masculino
5.
Sensors (Basel) ; 12(12): 16673-84, 2012 Dec 04.
Artigo em Inglês | MEDLINE | ID: mdl-23211754

RESUMO

Series-connected thin film piezoelectric elements can generate large output voltages. The output voltage ideally is proportional to the number of connections. However, parasitic capacitances formed by the insulation layers and derived from peripheral circuitry degrade the output voltage. Conventional circuit models are not suitable for predicting the influence of the parasitic capacitance. Therefore we proposed the simplest model of piezoelectric elements to perform simulation program with integrated circuit emphasis (SPICE) circuit simulations). The effects of the parasitic capacitances on the thin-film Pb(Zr, Ti)O(3), (PZT) elements connected in series on a SiO(2) insulator are demonstrated. The results reveal the negative effect on the output voltage caused by the parasitic capacitances of the insulation layers. The design guidelines for the devices using series-connected piezoelectric elements are explained.


Assuntos
Capacitância Elétrica , Desenho de Equipamento , Dióxido de Silício/química , Simulação por Computador , Projeto Auxiliado por Computador , Transdutores , Zircônio/química
6.
Sensors (Basel) ; 12(6): 8026-38, 2012.
Artigo em Inglês | MEDLINE | ID: mdl-22969385

RESUMO

A novel gas-sealed capacitive pressure sensor with a temperature compensation structure is reported. The pressure sensor is sealed by Au-Au diffusion bonding under a nitrogen ambient with a pressure of 100 kPa and integrated with a platinum resistor-based temperature sensor for human activity monitoring applications. The capacitance-pressure and capacitance-temperature characteristics of the gas-sealed capacitive pressure sensor without temperature compensation structure are calculated. It is found by simulation that a ring-shaped structure on the diaphragm of the pressure sensor can mechanically suppress the thermal expansion effect of the sealed gas in the cavity. Pressure sensors without/with temperature compensation structures are fabricated and measured. Through measured results, it is verified that the calculation model is accurate. Using the compensation structures with a 900 µm inner radius, the measured temperature coefficient is much reduced as compared to that of the pressure sensor without compensation. The sensitivities of the pressure sensor before and after compensation are almost the same in the pressure range from 80 kPa to 100 kPa.

7.
Sensors (Basel) ; 12(1): 226-32, 2012.
Artigo em Inglês | MEDLINE | ID: mdl-22368466

RESUMO

A 1.1 µW power dissipation, voltage-output humidity sensor with 10% relative humidity accuracy was developed in the LFoundry 0.15 µm CMOS technology without post-processing. The sensor consists of a woven lateral array of electrodes implemented in CMOS top metal, a humidity-sensitive layer of Intervia Photodielectric 8023D-10, a CMOS capacitance to voltage converter, and the self-calibration circuitry.


Assuntos
Umidade , Metais/química , Óxidos/química , Semicondutores/instrumentação , Calibragem , Simulação por Computador , Eletricidade , Eletrônica , Temperatura Ambiente
8.
Sensors (Basel) ; 11(6): 6197-202, 2011.
Artigo em Inglês | MEDLINE | ID: mdl-22163949

RESUMO

A 2 µW power dissipation, voltage-output, humidity sensor accurate to 5% relative humidity was developed using the LFoundry 0.15 µm CMOS technology without post-processing. The sensor consists of a woven lateral array of electrodes implemented in CMOS top metal, a Intervia Photodielectric 8023-10 humidity-sensitive layer, and a CMOS capacitance to voltage converter.


Assuntos
Microcomputadores , Fontes de Energia Elétrica , Eletroquímica/métodos , Eletrodos , Desenho de Equipamento , Umidade , Sistemas Microeletromecânicos/métodos , Polímeros/química , Pirróis/química , Reprodutibilidade dos Testes , Software , Temperatura Ambiente
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