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2.
Pediatr Infect Dis J ; 2020 Nov 11.
Artículo en Inglés | MEDLINE | ID: mdl-33181786

RESUMEN

Combination therapy for toxoplasmosis consists of sulfadiazine, pyrimethamine and leucovorin. Although sulfadiazine can cause hypersensitivity reactions, such as fever, rash and liver dysfunction, there is no consensus on an effective therapy for congenital toxoplasmosis (CTox) without sulfadiazine. We attempted desensitization to sulfadiazine in 2 patients with CTox and sulfadiazine hypersensitivity. Desensitization was achieved for 1 patient.

5.
Biosci Trends ; 14(3): 200-205, 2020 Jul 17.
Artículo en Inglés | MEDLINE | ID: mdl-32418922

RESUMEN

Chest radiographs should be obtained at the peak of inspiration so that radiological findings can be precisely interpreted. However, this is not easily achieved, particularly in young children who do not follow the instruction to hold their breath. We developed a sensor that detects the breathing movements and conducted a randomized controlled study to determine whether the sensor would increase the proportion of chest radiographs obtained in the inspiration phase. We recruited 124 infants and children aged less than 3 years, who visited the pediatric department of a general hospital in Tokyo, Japan, and allocated them into one of two groups: with-sensor and without-sensor groups. Overall, 81% of all images were obtained during inspiration. The proportion of chest radiographs taken during inspiration was not statistically different between the two groups (81% vs. 82%). In the with-sensor group, radiologic technologists were able to obtain chest radiographs of the same quality while not observing the chest movement, but the sensor. The use of the sensor did not increase the proportion of chest radiographs taken in the inspiration phase in this study. However, this null result may indicate the possibility of utilizing the sensor for automatizing chest radiography in the future.

6.
J Pediatr Hematol Oncol ; 42(6): e459-e462, 2020 Aug.
Artículo en Inglés | MEDLINE | ID: mdl-30994506

RESUMEN

Chronic myeloid leukemia (CML) is commonly associated with major BCR-ABL transcript. We present a child with blastic phase CML associated with minor BCR-ABL transcript without prior CML diagnosis. Diagnosis was achieved by fluorescence in situ hybridization of peripheral blood neutrophils, which identified 90% as BCR-ABL positive. The patient received chemotherapy with imatinib followed by dasatinib and underwent reduced-intensity hematopoietic allogeneic stem cell transplantation with prophylactic posttransplant dasatinib for 2 years and has remained in complete molecular remission. Our intensified treatment regimen was effective compared with previous studies on minor BCR-ABL CML describing inferior outcomes with tyrosine kinase inhibitor therapy.

7.
Int J Clin Oncol ; 23(6): 1178-1188, 2018 Dec.
Artículo en Inglés | MEDLINE | ID: mdl-29869758

RESUMEN

BACKGROUNDS: Multidisciplinary therapy has increased the risk of subsequent late effects, but detailed analyses on secondary cancers in childhood cancer survivors (CCSs) are limited in Asian countries. METHODS: This was a retrospective cohort study comprising 10,069 CCSs who were diagnosed between 1980 and 2009 across 15 Japanese hospitals. We conducted secondary analyses to estimate the incidence of secondary cancer according to each primary malignancy and to elucidate the association between primary and secondary cancers. We also explored the risk factors for the development of secondary cancer in each independent primary malignancy. RESULTS: The cumulative incidence of secondary cancer at 20 years varied among primary cancers: hematological malignancy, 3.1% (95% CI 2.2-4.3); retinoblastoma, 6.6% (95% CI 1.5-16.8); pediatric solid tumor, 2.5% (95% CI 1.3-4.2); brain tumors, 5.2% (95% CI 1.7-11.8) bone/soft tissue sarcoma, 5.2% (95% CI 2.3-10.1); and others, 3.3% (95% CI 1.6-6.0) (p = 0.015). The cumulative incidence of secondary cancers is highest in those with osteosarcoma (13.1%) followed by those with hepatoblastoma (8.4%) and retinoblastoma (6.6%). Close association between the primary and secondary cancer diagnoses was found. The risk factors for secondary cancer development depended on the primary cancer, but autologous/allogeneic stem cell transplantation was a relatively common risk factor. CONCLUSION: The cumulative incidence of secondary cancer varied among primary cancers. The primary cancer was closely associated with the secondary cancer but stem cell transplantation was a common risk factor for secondary cancers among CCSs.


Asunto(s)
Neoplasias Primarias Secundarias/epidemiología , Neoplasias Primarias Secundarias/etiología , Neoplasias/terapia , Trasplante de Células Madre/efectos adversos , Sobrevivientes/estadística & datos numéricos , Adolescente , Niño , Preescolar , Femenino , Humanos , Incidencia , Lactante , Japón/epidemiología , Masculino , Pronóstico , Estudios Retrospectivos , Factores de Riesgo
8.
Jpn J Infect Dis ; 71(4): 309-311, 2018 Jul 24.
Artículo en Inglés | MEDLINE | ID: mdl-29709989

RESUMEN

Cytomegalovirus (CMV) is the most common cause of congenital infection. Pneumonitis is considered to be a rare manifestation although congenital CMV infection presents with various non-specific findings. Ganciclovir and valganciclovir are beneficial for improving neurodevelopmental sequelae and hearing outcomes of congenital CMV infection; however, treatment response evaluation is not well reported. We report a female case of congenital CMV infection presenting with pneumonitis, meningoencephalitis, and chorioretinitis. She was treated with intravenous ganciclovir for 6 weeks, and clinical features improved. Measurement of the CMV genome load by real-time polymerase chain reaction assay was performed during treatment. After the administration of ganciclovir, the CMV genome was not detected in the blood and levels decreased gradually in the urine. Physicians should consider the possibility of congenital CMV infection in neonates who present with respiratory distress. Furthermore, measurement of the CMV genome load in blood and urine may be useful for evaluating treatment response.


Asunto(s)
Antivirales/administración & dosificación , Infecciones por Citomegalovirus/tratamiento farmacológico , Citomegalovirus/aislamiento & purificación , Monitoreo de Drogas/métodos , Ganciclovir/administración & dosificación , Neumonía/tratamiento farmacológico , Carga Viral , Administración Intravenosa , Adulto , Sangre/virología , Coriorretinitis/congénito , Coriorretinitis/tratamiento farmacológico , Coriorretinitis/patología , Coriorretinitis/virología , Infecciones por Citomegalovirus/congénito , Infecciones por Citomegalovirus/patología , Infecciones por Citomegalovirus/virología , ADN Viral/sangre , ADN Viral/orina , Femenino , Humanos , Recién Nacido , Meningoencefalitis/congénito , Meningoencefalitis/tratamiento farmacológico , Meningoencefalitis/patología , Meningoencefalitis/virología , Neumonía/congénito , Neumonía/patología , Neumonía/virología , Reacción en Cadena en Tiempo Real de la Polimerasa , Orina/virología
9.
Int J Clin Oncol ; 23(5): 965-973, 2018 Oct.
Artículo en Inglés | MEDLINE | ID: mdl-29700636

RESUMEN

BACKGROUND: The Japanese Children's Cancer Group (JCCG) Neuroblastoma Committee (JNBSG) conducted a phase II clinical trial for high-risk neuroblastoma treatment. We report the result of the protocol treatment and associated genomic aberration studies. METHODS: JN-H-07 was a single-arm, late phase II trial for high-risk neuroblastoma treatment with open enrollment from June 2007 to February 2009. Eligible patients underwent five courses of induction chemotherapy followed by high-dose chemotherapy with hematopoietic stem cell rescue. Surgery for the primary tumor was scheduled after three or four courses of induction chemotherapy. Radiotherapy was administered to the primary tumor site and to any bone metastases present at the end of induction chemotherapy. RESULTS: The estimated 3-year progression-free and overall survival rates of the 50 patients enrolled were 36.5 ± 7.0 and 69.5 ± 6.6%, respectively. High-dose chemotherapy caused severe toxicity including three treatment-related deaths. In response to this, the high-dose chemotherapy regimen was modified during the trial by infusing melphalan before administering carboplatin and etoposide. The modified high-dose chemotherapy regimen was less toxic. Univariate analysis revealed that patients younger than 547 days and patients whose tumor showed a whole chromosomal gains / losses pattern had a significantly poor prognosis. Notably, the progression-free survival of cases with MYCN amplification were not inferior to those without MYCN amplification. CONCLUSIONS: The outcome of patients treated with the JN-H-07 protocol showed improvement over the results reported by previous studies conducted in Japan. Molecular and genetic profiling may enable a more precise stratification of the high-risk cohort.


Asunto(s)
Protocolos de Quimioterapia Combinada Antineoplásica/uso terapéutico , Neuroblastoma/tratamiento farmacológico , Adolescente , Biomarcadores de Tumor/genética , Carboplatino/administración & dosificación , Niño , Preescolar , Hibridación Genómica Comparativa , Etopósido/administración & dosificación , Femenino , Humanos , Quimioterapia de Inducción , Lactante , Japón , Masculino , Melfalán/administración & dosificación , Neuroblastoma/genética , Neuroblastoma/patología , Estudios Prospectivos , Tasa de Supervivencia , Resultado del Tratamiento
10.
J Infect Chemother ; 24(3): 220-223, 2018 Mar.
Artículo en Inglés | MEDLINE | ID: mdl-29138020

RESUMEN

In the era of Antiretroviral Therapy (ART) in which human immunodeficiency virus type 1 (HIV-1) infection affected children can expect a better prognosis, the importance of careful follow up of pediatric HIV-1 cases for neurological complications has been growing. We present a case of hemorrhagic Moyamoya syndrome in a child with congenital HIV-1 infection. A 10-year-old girl was referred to our hospital for the treatment of Pneumocystis Jirovecii Pneumonia (PCP: Pneumocystis pneumonia). Her HIV-1 control was poor and Moyamoya syndrome was found during the opportunistic infection screening at admission. Despite subsequent successful treatment of PCP and HIV-1 infection, we could not save her life due to the intracranial hemorrhage caused by Moyamoya syndrome. A few reported cases of Moyamoya syndrome associated with HIV-1 infection have shown negative outcomes when the control of HIV-1 infection is unsuccessful. Recently "HIV-associated vasculopathy" has been used to describe the cerebrovascular disorder related to HIV-1 infection that is caused by the endothelial dysfunction induced from chronic inflammation and cytokine imbalances due to HIV-1 infection. We assumed that "HIV-associated vasculopathy" may have contributed to the development of collateral vessels impairment related to the bleeding, although the mechanism of vascular damage with HIV-1 infection is not yet well defined. Therefore proper management of the HIV-1 infection is crucial for Moyamoya syndrome with HIV-1 cases. Furthermore it is better to take into account the risk of intracerebral hemorrhage when considering the indication and timing of the revascularization surgery, although generally hemorrhaging is rare in Moyamoya disease in children.


Asunto(s)
Infecciones por VIH/congénito , Infecciones por VIH/complicaciones , Transmisión Vertical de Enfermedad Infecciosa , Hemorragias Intracraneales/etiología , Enfermedad de Moyamoya/complicaciones , Infecciones Oportunistas/complicaciones , Neumonía por Pneumocystis/complicaciones , Infarto Cerebral/diagnóstico por imagen , Revascularización Cerebral , Niño , Enfermedad Crónica , Angiografía por Tomografía Computarizada , Femenino , Infecciones por VIH/transmisión , Humanos , Inflamación , Hemorragias Intracraneales/diagnóstico por imagen , Angiografía por Resonancia Magnética , Enfermedad de Moyamoya/diagnóstico por imagen , Infecciones Oportunistas/diagnóstico , Neumonía por Pneumocystis/diagnóstico
11.
Pediatr Blood Cancer ; 64(11)2017 Nov.
Artículo en Inglés | MEDLINE | ID: mdl-28521076

RESUMEN

PURPOSE: Perifosine is an alkylphospholipid analog that inhibits or modulates signaling through signal transduction pathways such as Akt, which is enhanced in neuroblastoma (NB) by activation of tyrosine kinase receptors. We conducted a phase I study of perifosine in Japanese patients with recurrent or refractory NB. EXPERIMENTAL DESIGN: All patients enrolled were over 2 years of age; all had refractory or relapsed NB and a performance status of greater than 50%. Perifosine was orally administered at a loading dose (100-300 mg) on day 1 and at a maintenance dose (50-150 mg) from day 2 onward. Dose-limiting toxicity (DLT) and pharmacokinetics were assessed in Step 1 and safety and efficacy in Step 2. RESULTS: Nineteen patients were recruited. No DLT was observed. Adverse reactions occurring in more than 30% of the patients were vomiting (63%), nausea (53%), and diarrhea (37%). The mean plasma concentration of perifosine was 27.5 ± 9.8 µM on day 15 and 27.3 ± 11.5 µM on day 29. The response rate (RR) in 18 patients evaluable according to modified International Neuroblastoma Response Criteria was 0%; the disease control rate (DCR) was 56%. Median progression-free survival (PFS) was 122 days. In 11 patients evaluable according to the Response Evaluation Criteria in Solid Tumors, the RR and DCR were 9% and 55%, respectively. The median PFS was not reached. CONCLUSIONS: Perifosine monotherapy was well tolerated in Japanese patients with recurrent/refractory NB. Further investigations in combination with other anticancer or molecular targeted agents are warranted.


Asunto(s)
Resistencia a Antineoplásicos/efectos de los fármacos , Recurrencia Local de Neoplasia/tratamiento farmacológico , Neuroblastoma/tratamiento farmacológico , Fosforilcolina/análogos & derivados , Terapia Recuperativa , Adolescente , Adulto , Niño , Preescolar , Femenino , Estudios de Seguimiento , Humanos , Masculino , Dosis Máxima Tolerada , Recurrencia Local de Neoplasia/patología , Estadificación de Neoplasias , Neuroblastoma/patología , Fosforilcolina/uso terapéutico , Pronóstico , Proteínas Proto-Oncogénicas c-akt/antagonistas & inhibidores , Transducción de Señal/efectos de los fármacos , Tasa de Supervivencia , Adulto Joven
12.
J Cardiol ; 70(4): 396-401, 2017 Oct.
Artículo en Inglés | MEDLINE | ID: mdl-28238564

RESUMEN

AIMS: To identify left ventricular (LV) mechanical impairment by 3D speckle-tracking echocardiography (3DSTE) in long-term childhood cancer survivors after anthracycline therapy with or without persistent LV regional diastolic wall motion abnormalities (WMA) and a preserved LV ejection fraction (EF >53%). METHODS AND RESULTS: Thirty-two patients (median: 14.6 years) and 12 age-matched controls were studied. The patients were divided into two groups according to the existence of WMA: Group 1 (with WMA: n=14), Group 2 (without WMA: n=18). 3DSTE was performed to assess LV global longitudinal strain (GLS), global circumferential strain (GCS), global radial strain (GRS), global area strain (GAS), LV torsion, LV end-diastolic volume (LVEDV), and LV end-systolic volume (LVESV). LV systolic dyssynchrony index (SDI) was calculated as the percentage of the standard deviation of time to peak strain of the 16 segments divided by the RR interval. There was no significant difference in LVEDV, LVESV, GLS, torsion, or SDI derived from LS, CS, or AS among the 3 groups. In contrast, there were significant differences in GRS, GCS, and GAS, and SDI derived from RS among the 3 groups. Compared with group 2, group 1 had significantly reduced GRS (p<0.001), GCS (p<0.01), GAS (p<0.01), and greater SDI derived from GRS (p<0.01). Moreover, the existence of WMA was correlated with GRS (p<0.001), SDI derived from GRS (p<0.001), and LVEF (p=0.036). Multiple linear regression analysis identified GRS as a significant determinant of the existence of WMA (ß=0.751, p=0.001). CONCLUSION: Childhood cancer survivors with persistent LV regional WMA show a reduced LV myocardial performance compared with those without WMA, despite a preserved LVEF.


Asunto(s)
Supervivientes de Cáncer , Ventrículos Cardíacos/diagnóstico por imagen , Ventrículos Cardíacos/fisiopatología , Disfunción Ventricular Izquierda/fisiopatología , Adolescente , Antraciclinas/uso terapéutico , Antibióticos Antineoplásicos/uso terapéutico , Niño , Diástole , Ecocardiografía Tridimensional/métodos , Femenino , Humanos , Masculino , Análisis Multivariante , Neoplasias/tratamiento farmacológico , Sístole
13.
Circ J ; 80(11): 2376-2381, 2016 Oct 25.
Artículo en Inglés | MEDLINE | ID: mdl-27746411

RESUMEN

BACKGROUND: Tenascin-C (TN-C) is an extracellular matrix glycoprotein that is heavily upregulated at sites of inflammation. We conducted a retrospective study to assess the utility of TN-C as a novel biomarker to predict the risk of developing coronary artery lesions (CAL) and resistance to intravenous immunoglobulin (IVIG) in patients with Kawasaki disease (KD).Methods and Results:We collected blood samples of 111 KD patients (IVIG-responder: 89, IVIG-resistant: 22; CAL: 8) and 23 healthy controls, and measured the serum levels of TN-C. TN-C levels on admission were significantly higher in patients than in healthy controls and in patients during convalescence after IVIG administration (69.6 vs. 20.4 vs. 39.7 ng/ml, respectively; P<0.001), and correlated positively with C-reactive protein (P<0.001), neutrophil (percentage; P=0.005), and ALT (P<0.001), and negatively with platelet count (P=0.023) and sodium level (P=0.025). On admission, TN-C levels in patients who later developed CAL were significantly higher than in those without CAL (P=0.010), and significantly higher in IVIG-resistant subjects than in IVIG-responders (P=0.003). The accuracy of TN-C testing for the prediction of IVIG resistance was comparable to that of the Kobayashi score. CONCLUSIONS: Serum TN-C could be a biomarker for predicting the risk of developing CAL and IVIG resistance during the acute phase of KD. (Circ J 2016; 80: 2376-2381).


Asunto(s)
Vasos Coronarios/metabolismo , Resistencia a Medicamentos , Inmunoglobulinas Intravenosas/administración & dosificación , Síndrome Mucocutáneo Linfonodular/sangre , Tenascina/sangre , Biomarcadores/sangre , Niño , Preescolar , Femenino , Humanos , Lactante , Masculino , Síndrome Mucocutáneo Linfonodular/tratamiento farmacológico , Estudios Retrospectivos , Factores de Riesgo
14.
Int J Clin Oncol ; 21(3): 506-16, 2016 Jun.
Artículo en Inglés | MEDLINE | ID: mdl-26620038

RESUMEN

BACKGROUND: The epidemiology of secondary cancers in childhood cancer survivors has been unknown in Asian countries. Our aim is to assess the incidence and risk factors for secondary cancers through a nationwide survey in Japan. METHODS: A retrospective cohort study comprising 10,069 children who were diagnosed with cancer between 1980 and 2009 was conducted in 15 Japanese hospitals. The cumulative incidence rate was calculated using death as the competing risk and compared by the Gray method. The standardized incidence ratio (SIR) was defined as the ratio of the number of observed cancers divided by the number of expected cancers. The risk factors were analyzed using Cox regression analysis. RESULTS: One hundred and twenty-eight patients (1.3 %) developed secondary cancers within a median follow-up of 8.4 years. The cumulative incidence rate was 1.1 % (95 % confidence interval [CI] 0.9-1.4) at 10 years and 2.6 % (95 % CI 2.1-3.3) at 20 years after primary cancer diagnosis. Sensitivity analysis, limited to 5-year survivors (n = 5,387), confirmed these low incidence rates. The SIR of secondary cancers was 12.1 (95 % CI 10.1-14.4). In the Cox analysis, the hazard ratios for secondary cancers were 3.81 (95 % CI 1.53-9.47) for retinoblastoma, 2.78 (95 % CI 1.44-5.38) for bone/soft tissue sarcomas, and 1.81 (95 % CI 1.16-2.83) for allogeneic stem cell transplantation. CONCLUSIONS: The cumulative incidence of secondary cancers in children in Japan was not high; however, the SIR was relatively high. Retinoblastoma or sarcoma in addition to allogeneic stem cell transplantation were significant risk factors for secondary cancers.


Asunto(s)
Neoplasias Óseas/terapia , Neoplasias Primarias Secundarias/epidemiología , Neoplasias de la Retina/terapia , Retinoblastoma/terapia , Sarcoma/terapia , Neoplasias de los Tejidos Blandos/terapia , Sobrevivientes/estadística & datos numéricos , Adolescente , Adulto , Niño , Preescolar , Femenino , Estudios de Seguimiento , Humanos , Incidencia , Lactante , Japón , Masculino , Modelos de Riesgos Proporcionales , Estudios Retrospectivos , Factores de Riesgo , Trasplante de Células Madre/estadística & datos numéricos , Encuestas y Cuestionarios , Tasa de Supervivencia , Factores de Tiempo , Trasplante Homólogo/estadística & datos numéricos , Adulto Joven
15.
J Pediatr Hematol Oncol ; 38(5): 398-401, 2016 07.
Artículo en Inglés | MEDLINE | ID: mdl-26535770

RESUMEN

Positron emission tomography combined with computed tomography (PET/CT) is a promising diagnostic procedure for the detection of extramedullary disease (EMD) in acute myeloid leukemia. We studied 2 children with acute myeloid leukemia who underwent PET to assess for EMD at diagnosis as well as in remission. We detected 5 EMD lesions in 2 cases with PET, only 2 of which were detectable on clinical examination. Our cases show PET's increased sensitivity over physical examination alone in assessing and monitoring the extent of this disease.


Asunto(s)
Leucemia Mieloide Aguda/diagnóstico por imagen , Tomografía Computarizada por Tomografía de Emisión de Positrones/métodos , Sarcoma Mieloide/diagnóstico por imagen , Niño , Clavícula/diagnóstico por imagen , Clavícula/patología , Fluorodesoxiglucosa F18 , Humanos , Lactante , Masculino , Órbita/diagnóstico por imagen , Órbita/patología
16.
Cytokine ; 74(2): 339-42, 2015 Aug.
Artículo en Inglés | MEDLINE | ID: mdl-25801094

RESUMEN

UNLABELLED: We present a case of Kawasaki Disease (KD) that was refractory to plasma exchange (PE), but which finally responded to concurrent intravenous methylprednisolone pulse (IVMP) and PE treatment. To determine direct and indirect evidence for the efficacy of this combination therapy, we analyzed data of patients with refractory KD by review of the literature using medical databases and cytokine profiling. For literature searches, we used the Pubmed™ and Ichushi™ databases. Search terms used included "Kawasaki disease" and "plasma exchange" to extract articles that described KD cases treated with PE. For cytokine profiling, we measured interleukin (IL)-6, soluble tumor necrosis factor-α receptor (sTNF-αR) type 1 and type 2 before and after PE and PE with IVMP. Our search revealed 201 KD patients treated with PE, of which PE treatment was effective in 188 patients (93.5%), but not in 13 cases (6.5%). All 13 cases were treated successfully with additional treatment. Of the 13 cases, only six (2.5%) had recurrence during the PE treatment period. In our case, cytokine profiling showed PE treatment decreased IL6, while sTNF-αR type1 and type2 remained at high levels. PE and IVMP decreased IL-6 and sTNFα-R type 1 and type 2 levels. CONCLUSION: PE concurrent with additional anti-inflammatory treatment such as IVMP might be a very promising treatment option for PE refractory patients.


Asunto(s)
Interleucina-6/sangre , Metilprednisolona/administración & dosificación , Síndrome Mucocutáneo Linfonodular , Intercambio Plasmático , Receptores Tipo II del Factor de Necrosis Tumoral/sangre , Receptores Tipo I de Factores de Necrosis Tumoral/sangre , Preescolar , Femenino , Humanos , Síndrome Mucocutáneo Linfonodular/sangre , Síndrome Mucocutáneo Linfonodular/terapia
17.
Ann Hematol ; 93(5): 747-52, 2014 May.
Artículo en Inglés | MEDLINE | ID: mdl-24337487

RESUMEN

Patients with severe aplastic anemia (SAA) and an absolute neutrophil count (ANC) of 0 typically have fatal outcomes. We defined fulminant AA (FAA) as ANC = 0 for at least 2 weeks prior to and after immunosuppressive therapy (IST). We analyzed the outcomes of 35 children with FAA among 288 children who enrolled in a prospective study for AA (AA-97 study). AA was classified as FAA (n = 35), very SAA (vSAA; n = 129), or SAA (n = 124). All of the children received the IST with horse anti-thymocyte globulin (ATG) and cyclosporine (CsA). A significantly lower response rate at 6 months was seen in children with FAA when compared to those with vSAA or SAA (40.0, 63.6, and 63.7 %, respectively; p = 0.027). Of 20 nonresponder patients in the FAA group, 11 were rescued by alternative donor transplantation, and 5 patients showed a late response after 6 months. Consequently, no significant difference was noted in overall survival when comparing the FAA, vSAA, and SAA groups (88.5, 95.8, and 96.8 %). These findings indicate that IST with ATG and CsA is justified as a first-line treatment for children with FAA who lack a human leukocyte antigen-matched sibling donor.


Asunto(s)
Anemia Aplásica/terapia , Suero Antilinfocítico/uso terapéutico , Ciclosporina/uso terapéutico , Inmunosupresores/uso terapéutico , Enfermedad Aguda , Adolescente , Anemia Aplásica/inmunología , Anemia Aplásica/mortalidad , Anemia Aplásica/patología , Animales , Niño , Preescolar , Femenino , Trasplante de Células Madre Hematopoyéticas , Prueba de Histocompatibilidad , Caballos , Humanos , Inmunosupresión , Masculino , Neutrófilos/efectos de los fármacos , Neutrófilos/inmunología , Neutrófilos/patología , Estudios Prospectivos , Índice de Severidad de la Enfermedad , Análisis de Supervivencia , Resultado del Tratamiento
18.
Thromb Res ; 130(6): e289-93, 2012 Dec.
Artículo en Inglés | MEDLINE | ID: mdl-23123162

RESUMEN

INTRODUCTION: Recombinant thrombomodulin (rTM), which degrades factors Va and VIIIa by activating protein C, has been developed as a new drug for treating disseminated intravascular coagulation (DIC). MATERIALS AND METHODS: Since July 2009, we have treated 25 children with DIC using rTM (380 U/kg/day, or 130 U/kg/day for newborns) as a first-line therapy. Median duration of rTM administration was 5 consecutive days (range, 2-13 days). We employed DIC criteria of the Japan Welfare and Health Ministry. The first day on which rTM treatment was given was defined as day 1. RESULTS: Median patients age was 3 years. Underlying diseases were hematological disorders (n=13) and severe infection (n=12). Overall, 20 of the 25 patients had recovered from DIC by day 7 and 22 of the 25 patients remained alive at day 28. Median Pediatric Logistic Organ Dysfunction score improved from 11 on day 1 to 2 on day 7 (p=0.009). Laboratory data (median) on day 7 (prothrombin time (PT) ratio, 1.15; fibrin and fibrinogen degradation products (FDP), 9.6 mg/l; D-dimer, 1.6 mg/l FEU; antithrombin, 112%; protein C, 105%) were significantly improved compared to results on day 1 (PT ratio, 1.39; FDP, 21.6 mg/l; D-dimer, 6.4 mg/l FEU; antithrombin, 86%; protein C, 54%). Whereas, 5 patients failed to respond and serious bleeding events were observed in 2 newborns. CONCLUSION: The efficacy of rTM cannot be assessed from the present dataset, due to several limitations such as the small heterogenous patient cohort, and the lack of age- and disease-matched controls. Nevertheless, this case-series remains important in terms of enabling further prospective control studies to evaluate the efficacy of rTM in children.


Asunto(s)
Coagulación Intravascular Diseminada/tratamiento farmacológico , Trombomodulina/uso terapéutico , Adolescente , Niño , Preescolar , Coagulación Intravascular Diseminada/sangre , Femenino , Humanos , Lactante , Recién Nacido , Masculino , Proteínas Recombinantes/uso terapéutico , Estudios Retrospectivos
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