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1.
Mol Psychiatry ; 18(10): 1067-76, 2013 Oct.
Artículo en Inglés | MEDLINE | ID: mdl-23732879

RESUMEN

One of the prevailing hypotheses suggests schizophrenia as a neurodevelopmental disorder, involving dysfunction of dopaminergic and glutamatergic systems. Accumulating evidence suggests mitochondria as an additional pathological factor in schizophrenia. An attractive model to study processes related to neurodevelopment in schizophrenia is reprogramming of somatic cells into induced pluripotent stem cells (iPSCs) and differentiating them into different neuronal lineages. iPSCs from three schizophrenia patients and from two controls were reprogrammed from hair follicle keratinocytes, because of their accessibility and common ectodermal origin with neurons. iPSCs were differentiated into Pax6(+)/Nestin(+) neural precursors and then further differentiated into ß3-Tubulin(+)/tyrosine hydroxylase(+)/DAT(+) dopaminergic neurons. In addition, iPSCs were differentiated through embryonic bodies into ß3-Tubulin(+)/Tbox brain1(+) glutamatergic neurons. Schizophrenia-derived dopaminergic cells showed severely impaired ability to differentiate, whereas glutamatergic cells were unable to maturate. Mitochondrial respiration and its sensitivity to dopamine-induced inhibition were impaired in schizophrenia-derived keratinocytes and iPSCs. Moreover, we observed dissipation of mitochondrial membrane potential (Δψm) and perturbations in mitochondrial network structure and connectivity in dopaminergic along the differentiation process and in glutamatergic cells. Our data unravel perturbations in neural differentiation and mitochondrial function, which may be interconnected, and of relevance to dysfunctional neurodevelopmental processes in schizophrenia.


Asunto(s)
Folículo Piloso/patología , Células Madre Pluripotentes Inducidas/patología , Queratinocitos/patología , Mitocondrias/metabolismo , Neurogénesis , Neuronas/patología , Esquizofrenia Paranoide/patología , Antígenos de Diferenciación/biosíntesis , Antígenos de Diferenciación/genética , Linaje de la Célula , Células Cultivadas , Dopaminérgicos/farmacología , Ectodermo/citología , Fármacos actuantes sobre Aminoácidos Excitadores/farmacología , Regulación de la Expresión Génica , Humanos , Células Madre Pluripotentes Inducidas/metabolismo , Potencial de la Membrana Mitocondrial , Modelos Neurológicos , Proteínas del Tejido Nervioso/biosíntesis , Proteínas del Tejido Nervioso/genética , Neuronas/efectos de los fármacos , Neuronas/metabolismo , Consumo de Oxígeno , ARN Mensajero/biosíntesis , ARN Mensajero/genética , Esquizofrenia Paranoide/metabolismo
2.
J Med Genet ; 50(12): 823-30, 2013 Dec.
Artículo en Inglés | MEDLINE | ID: mdl-24065356

RESUMEN

BACKGROUND: Russell Silver syndrome (RSS) leads to prenatal and postnatal growth retardation. About 55% of RSS patients present a loss-of-methylation of the paternal ICR1 domain on chromosome 11p15. CDKN1C is a cell proliferation inhibitor encoded by an imprinted gene in the 11p15 ICR2 domain. CDKN1C mutations lead to Beckwith Wiedemann syndrome (BWS, overgrowth syndrome) and in IMAGe syndrome which associates growth retardation and adrenal insufficiency. We searched for CDKN1C mutations in a cohort of clinically diagnosed RSS patients with no molecular anomaly. METHOD: The coding sequence and intron-exon boundaries of CDKN1C were analysed in 97 RSS patients. The impact of CDKN1C variants on the cell cycle in vitro were determined by flow cytometry. Stability of CDKN1C was studied by western immunoblotting after inhibition of translation with cycloheximide. RESULTS: We identified the novel c.836G>[G;T] (p.Arg279Leu) mutation in a familial case of intrauterine growth retardation (IUGR) with RSS phenotype and no evidence of IMAGe. All the RSS patients inherited this mutation from their mothers (consistent with monoallelic expression from the maternal allele of the gene). A mutation of this amino acid (p.Arg279Pro) has been reported in cases of IMAGe. Functional analysis showed that Arg279Leu (RSS) did not affect the cell cycle, whereas the Arg279Pro mutation (IMAGe) led to a gain of function. Arg279Leu (RSS) led to an increased stability which could explain an increased activity of CDKN1C. CONCLUSIONS: CDKN1C mutations cause dominant maternally transmitted RSS, completing the molecular mirror with BWS. CDKN1C should be investigated in cases with family history of RSS.


Asunto(s)
Inhibidor p57 de las Quinasas Dependientes de la Ciclina/genética , Mutación/genética , Antígeno Nuclear de Célula en Proliferación/genética , Síndrome de Silver-Russell/genética , Secuencia de Aminoácidos , Análisis de Varianza , Sitios de Unión/genética , Simulación por Computador , Inhibidor p57 de las Quinasas Dependientes de la Ciclina/metabolismo , Femenino , Retardo del Crecimiento Fetal/genética , Células HeLa , Humanos , Masculino , Datos de Secuencia Molecular , Linaje , Antígeno Nuclear de Célula en Proliferación/metabolismo , Alineación de Secuencia , Síndrome de Silver-Russell/fisiopatología
3.
Arch Pediatr ; 30(3): 142-148, 2023 Apr.
Artículo en Inglés | MEDLINE | ID: mdl-36907727

RESUMEN

BACKGROUND: We aimed to evaluate catch-up growth in children with severe Hashimoto's hypothyroidism (HH) after thyroid hormone replacement therapy (HRT). METHODS: A multicenter retrospective study was conducted including children referred for growth slowdown that led to the diagnosis of HH between 1998 and 2017. RESULTS: A total of 29 patients were included, with a median age of 9.7 years (13-172 months). Median height at diagnosis was -2.7 [-4.6; -0.1] standard deviation score (SDS), with a height loss of 2.5 [0.7; 5.4] SDS compared to height before growth deflection (p<0.0001). At diagnosis, the median TSH level was 819.5 mIU/L [100; 1844], the median FT4 level was 0 pmol/L [undetectable; 5.4], and the median anti-thyroperoxidase antibody level was 1601 UI/L [47; 25,500]. In the 20 patients treated only with HRT, there were significant differences between height at diagnosis and height at 1 year (n = 19, p<0.0001), 2 years (n = 13, p = 0.0005), 3 years (n = 9, p = 0.0039), 4 years (n = 10, p = 0.0078), and 5 years (n = 10, p = 0.0018) of treatment but not in the case of final height (n = 6, p = 0.0625). Median final height was -1.4 [-2.7; 1,5] SDS (n = 6), with a significant difference between height loss at diagnosis and total catch-up growth (p = 0.003). The other nine patients were also given growth hormone (GH). They were smaller at diagnosis (p = 0.01); however, there was no difference in final height between those two groups (p = 0.68). CONCLUSION: Severe HH can lead to a major height deficit, and catch-up growth seems to be insufficient after treatment with HRT alone. In the most severe cases, administration of GH may enhance this catch-up.


Asunto(s)
Hormona de Crecimiento Humana , Hipotiroidismo , Humanos , Niño , Estudios Retrospectivos , Hipotiroidismo/diagnóstico , Hipotiroidismo/tratamiento farmacológico , Trastornos del Crecimiento/etiología , Yoduro Peroxidasa , Estatura
4.
Rev Mal Respir ; 38(5): 530-534, 2021 May.
Artículo en Francés | MEDLINE | ID: mdl-33965265

RESUMEN

BACKGROUND: The intrathoracic manifestations of IgG4-related disease include a range of conditions and severity, and can on occasion cause acute respiratory failure as reported in the case described here. OBSERVATION: A 69-year-old male former smoker, was admitted to our hospital with dyspnea, fever, cough, fatigue, and a 3-month history of weight loss. He received high flow oxygen therapy and non-invasive ventilation for severe respiratory failure. Chest computed tomography revealed multifocal condensations and ground glass opacities, accompanied by thickening of the perilymphatic interstitium, mediastinal lymphadenopathy and bilateral pleural effusion. Elevated serum concentrations of IgG4 suggested an IgG4-Related Disease. He developed renal failure and underwent a renal biopsy. Histopathological analysis of which supported the diagnosis by showing dense lymphocytic infiltrate with a count of IgG4+ cells/hpf higher than 60, and storiform fibrosis - a swirling, "cartwheel" pattern of fibrosis which may have a patchy distribution. The patient responded well to steroid therapy. CONCLUSION: Although respiratory symptoms are usually mild in IgG4-relatd disease, thoracic features can evolve into acute respiratory failure with few extra thoracic manifestations.


Asunto(s)
Enfermedad Relacionada con Inmunoglobulina G4 , Enfermedades Pulmonares Intersticiales , Derrame Pleural , Anciano , Humanos , Inmunoglobulina G , Enfermedad Relacionada con Inmunoglobulina G4/complicaciones , Enfermedad Relacionada con Inmunoglobulina G4/diagnóstico , Enfermedades Pulmonares Intersticiales/complicaciones , Enfermedades Pulmonares Intersticiales/diagnóstico , Masculino , Tomografía Computarizada por Rayos X
5.
Rev Mal Respir ; 37(7): 590-594, 2020 Sep.
Artículo en Francés | MEDLINE | ID: mdl-32739035

RESUMEN

INTRODUCTION: Eosinophilic pneumonias are characterized by an increase in lung eosinophils. These disorders can be induced by drug reactions. CASE REPORT: A 57-year-old woman suffering from bipolar disorder and treated by sodium divalproate for more than 2 years was hospitalised in the department of respiratory medicine for dyspnoea and cough. The investigations showed severe hypoxaemia, airflow limitation, multiple ground-glass opacities and crazy paving on the chest CT-scan and a blood eosinophilia. A significant alveolar eosinophilia was found in the broncho-alveolar lavage. A complete assessment of possible causes was made. Finally, we made the diagnosis of eosinophilic pneumonia secondary to sodium divalproate. The treatment was stopped and systemic corticosteroid therapy was not introduced. The patient showed an improvement of her dyspnoea in a few days. Lung function and the CT-scan were normal within a few months. CONCLUSIONS: Sodium divalproate, frequently used in the treatment of bipolar disorder, is a rare cause of eosinophilic lung disease, even years after its introduction. Rapid diagnosis and withdrawal of treatment led to complete resolution in the reported case.


Asunto(s)
Eosinofilia Pulmonar/inducido químicamente , Ácido Valproico/efectos adversos , Trastorno Bipolar/tratamiento farmacológico , Disnea/inducido químicamente , Disnea/diagnóstico , Disnea/etiología , Femenino , Humanos , Enfermedad Iatrogénica , Persona de Mediana Edad , Eosinofilia Pulmonar/complicaciones , Eosinofilia Pulmonar/diagnóstico , Privación de Tratamiento
6.
Rev Mal Respir ; 37(10): 823-828, 2020 Dec.
Artículo en Francés | MEDLINE | ID: mdl-33071063

RESUMEN

Pulmonary veno-occlusive disease (PVOD) is a rare cause of pulmonary hypertension. Heritable and sporadic forms have been distinguished. Hypoxemia, profound reduction in the diffusion of carbon monoxide and haemodynamic confirmation of pre-capillary pulmonary hypertension are the major diagnostic criteria. Thoracic CT scanning and a response to pharmaceutical therapy provide additional information to confirm the diagnosis. A 52-year-old patient, three of whose siblings had pulmonary hypertension, was admitted with dyspnoea, malaise and palpitations. Right heart catheterisation confirmed pre-capillary pulmonary hypertension. A search for an EIF2AK4 mutation was carried out, and this showed a composite biallelic heterozygous mutation compatible with the diagnosis of familial PVOD, identical to that showed in one of his brothers. Given the signs of severity of the disease and the diagnosis of PVOD, whose response to pharmaceutical therapy is often poor, the patient was placed on a waiting list for lung transplantation. Despite a similar diagnosis in 3 brothers and follow-up proposed 11 years before the diagnosis, pulmonary hypertension appeared within a few weeks and led immediately to a severe clinical situation. Annual clinical and echocardiographic monitoring had been strongly advised to the patient, but had not allowed diagnosis at a mild or moderate stage of the disease. This clinical case shows that the identification of factors predicting the development of heritable PVOD at a pre-symptomatic stage is an important issue for clinical research.


Asunto(s)
Mutación , Proteínas Serina-Treonina Quinasas/genética , Enfermedad Veno-Oclusiva Pulmonar/genética , Heterocigoto , Humanos , Hipertensión Pulmonar/diagnóstico , Hipertensión Pulmonar/genética , Hipertensión Pulmonar/terapia , Masculino , Persona de Mediana Edad , Enfermedad Veno-Oclusiva Pulmonar/diagnóstico , Enfermedad Veno-Oclusiva Pulmonar/terapia , Radiografía Torácica , Índice de Severidad de la Enfermedad , Hermanos
7.
J Med Virol ; 81(1): 42-8, 2009 Jan.
Artículo en Inglés | MEDLINE | ID: mdl-19031461

RESUMEN

Enteroviruses (EV) are the main etiological agents of aseptic meningitis. Diagnosis is made by detecting the genome using RT-PCR. The aim of the study was to evaluate the impact of a positive diagnosis on the management of infants, children, and adults. During 2005, 442 patients were admitted to hospital with suspected meningitis. Clinical and laboratory data and initial treatment were recorded for all patients with enteroviral meningitis. The turnaround time of tests and the length of hospital stay were analyzed. The results showed that EV-PCR detected EV in 69 patients (16%), 23% (16/69) were adults. About 18% of CSF samples had no pleocytosis. After positive PCR results, 63% of children were discharged immediately (mean 2 hr 30 min) and 95% within 24 hr. Infants and adults were discharged later (after 1.8 and 2 days, respectively). The use of antibiotics was significantly lower in children than in infants and adults. The PCR results allowed discontinuation of antibiotics in 50-60% of all patients treated. Patients received acyclovir in 16% of cases (7% children vs. 50% adults) and 23% (11% vs. 69%) underwent a CT scan. Clinical data were compared between patients whose positive EV-PCR results were available within 24 hr (n = 32) and those whose results were available > 24 hr after collection of CSF (n = 14). Duration of antibiotic treatment (difference: 2.3 days; P = 0.05) was reduced between the two groups. No statistical difference in the length of stay was observed. The EV-PCR assay should be performed daily in hospital laboratory practice and considered as part of the initial management of meningitis.


Asunto(s)
Infecciones por Enterovirus/diagnóstico , Infecciones por Enterovirus/terapia , Enterovirus/aislamiento & purificación , Meningitis Aséptica/terapia , Meningitis Aséptica/virología , Reacción en Cadena de la Polimerasa de Transcriptasa Inversa , Adolescente , Adulto , Anciano , Anciano de 80 o más Años , Antibacterianos/uso terapéutico , Antivirales/uso terapéutico , Manejo de Caso , Niño , Preescolar , Enterovirus/genética , Femenino , Humanos , Lactante , Recién Nacido , Tiempo de Internación , Masculino , Persona de Mediana Edad , Factores de Tiempo , Tomografía Computarizada por Rayos X
8.
Science ; 283(5403): 845-8, 1999 Feb 05.
Artículo en Inglés | MEDLINE | ID: mdl-9933168

RESUMEN

Stem cell homing and repopulation are not well understood. The chemokine stromal cell-derived factor-1 (SDF-1) and its receptor CXCR4 were found to be critical for murine bone marrow engraftment by human severe combined immunodeficient (SCID) repopulating stem cells. Treatment of human cells with antibodies to CXCR4 prevented engraftment. In vitro CXCR4-dependent migration to SDF-1 of CD34+CD38-/low cells correlated with in vivo engraftment and stem cell function. Stem cell factor and interleukin-6 induced CXCR4 expression on CD34+ cells, which potentiated migration to SDF-1 and engraftment in primary and secondary transplanted mice. Thus, up-regulation of CXCR4 expression may be useful for improving engraftment of repopulating stem cells in clinical transplantation.


Asunto(s)
Antígenos CD , Quimiocinas CXC/fisiología , Trasplante de Células Madre Hematopoyéticas , Células Madre Hematopoyéticas/fisiología , Receptores CXCR4/fisiología , ADP-Ribosil Ciclasa , ADP-Ribosil Ciclasa 1 , Animales , Anticuerpos , Antígenos CD34/análisis , Antígenos CD34/inmunología , Antígenos de Diferenciación/análisis , Quimiocina CXCL12 , Quimiocinas CXC/farmacología , Quimiotaxis , Ensayo de Unidades Formadoras de Colonias , Sangre Fetal , Movilización de Célula Madre Hematopoyética , Humanos , Interleucina-6/farmacología , Glicoproteínas de Membrana , Ratones , Ratones Endogámicos NOD , Ratones Noqueados , Ratones SCID , NAD+ Nucleosidasa/análisis , Receptores CXCR4/biosíntesis , Receptores CXCR4/inmunología , Factor de Células Madre/farmacología , Acetato de Tetradecanoilforbol/farmacología , Regulación hacia Arriba
9.
Rev Mal Respir ; 36(4): 538-542, 2019 Apr.
Artículo en Francés | MEDLINE | ID: mdl-30686554

RESUMEN

INTRODUCTION: The natural history of orphan lung diseases is often unclear. We report the long-term follow-up of a case of bronchiectasis due to pulmonary non amyloid light chain deposition disease (LCDD). CASE REPORT: A 50-year-old woman who was a smoker, was diagnosed with diffuse thin walled bronchiectasis of uncertain origin after presenting with a respiratory tract infection. Ten years later, the combination of bronchiectasis, the appearance of pulmonary cysts and the identification of increased kappa free light chains evoked the diagnosis of pulmonary LCDD. The diagnosis was confirmed by lung biopsy. No immunoproliferative disorder was identified. During the 12 years follow-up, dyspnea worsened progressively and bronchiectasis and lung cysts extended leading to multicystic lung disease. Pulmonary function tests did not show any ventilatory defect but a small decrease in carbon monoxide transfer factor occurred. CONCLUSION: We describe the evolution of a rare presentation of isolated pulmonary LCDD, characterized by cystic diffuse atypical bronchiectasis with thin walls, associated with progressive cystic destruction of the lung parenchyma. The possibility of pulmonary LCDD should be considered in cases of atypical bronchiectasis of unknown etiology.


Asunto(s)
Bronquiectasia/etiología , Amiloidosis de Cadenas Ligeras de las Inmunoglobulinas/complicaciones , Paraproteinemias/complicaciones , Bronquiectasia/diagnóstico , Bronquiectasia/patología , Femenino , Humanos , Cadenas Ligeras de Inmunoglobulina/metabolismo , Amiloidosis de Cadenas Ligeras de las Inmunoglobulinas/diagnóstico , Amiloidosis de Cadenas Ligeras de las Inmunoglobulinas/patología , Persona de Mediana Edad , Paraproteinemias/diagnóstico , Paraproteinemias/patología , Fumadores
10.
Bioresour Technol ; 99(14): 6162-7, 2008 Sep.
Artículo en Inglés | MEDLINE | ID: mdl-18207390

RESUMEN

The present work deals with the harvesting of Arthrospira platensis (Spirulina) from a diluted culture medium. This cyanobacterium was retained by the European Space Agency as food supply for long term manned spatial missions, and integrated in the MELiSSA project: an artificial microecosystem which supports life in space. Membranes techniques seem to be adapted to efficiency, reliability and safety constraints, even if a well-known limitation is the progressive fouling and permeation flux decrease. Among usual solid/liquid separation processes, Arthrospira harvesting is performed by tangential ultrafiltration (tubular inorganic membrane 50 kD Céram-Inside from Tami, Nyons, France). To ensure a reliable separation step with the best biomass quality, a good comprehension of the ultrafiltration progress and fouling phenomenon is needed, in particular, the link between operating parameters, permeation flux and cleanability. Comparative experiments were made between limiting and critical flux with different suspensions: fresh biomass, stressed biomass and a suspension of Arthrospira platensis enriched with exopolysaccharides.


Asunto(s)
Cianobacterias/aislamiento & purificación , Membranas Artificiales , Medios de Cultivo
11.
Rev Mal Respir ; 35(9): 978-982, 2018 Nov.
Artículo en Francés | MEDLINE | ID: mdl-30318432

RESUMEN

INTRODUCTION: The anti programmed death-1 (PD-1) and the programmed death ligand 1 (PD-L1) antibodies are used as immunotherapies in the treatment of many solid tumours. Cases of interstitial pneumonitis induced by anti PD-1 have been widely described, but there are fewer data with anti PD-L1. Avelumab is a new immunotherapy of the anti PD-L1 class. CASE REPORT: A 66-year-old woman, ex-smoker, had been treated with avelumab and axitinib since November 2016 for renal cell cancer. Interstitial pneumonitis was discovered accidentally 4 months after the beginning of the treatment, with ground glass opacities, intra-lobular crosslinking and adenopathy of the 4R zone on the CT scan. An exhaustive assessment did not reveal any respiratory function defect or an infectious or immunological cause. The radiological abnormalities regressed spontaneously after cessation of treatment confirming the diagnosis of drug-induced pneumonitis. CONCLUSION: Avelumab can induce interstitial lung disease. The mechanism is uncertain and requires further studies. Monitoring of respiratory function and CT scanning are necessary for its early management.


Asunto(s)
Anticuerpos Monoclonales/efectos adversos , Antineoplásicos Inmunológicos/efectos adversos , Enfermedades Pulmonares Intersticiales/inducido químicamente , Anciano , Anticuerpos Monoclonales/administración & dosificación , Anticuerpos Monoclonales Humanizados , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapéutico , Carcinoma de Células Renales/tratamiento farmacológico , Femenino , Humanos , Neoplasias Renales/tratamiento farmacológico , Enfermedades Pulmonares Intersticiales/diagnóstico
12.
J Clin Invest ; 104(9): 1199-211, 1999 Nov.
Artículo en Inglés | MEDLINE | ID: mdl-10545519

RESUMEN

The chemokine SDF-1 plays a central role in the repopulation of the bone marrow (BM) by circulating CD34(+) progenitors, but the mechanisms of its action remain obscure. To extravasate to target tissue, a blood-borne cell must arrest firmly on vascular endothelium. Murine hematopoietic progenitors were recently shown in vivo to roll along BM microvessels that display selectins and integrins. We now show that SDF-1 is constitutively expressed by human BM endothelium. In vitro, human CD34(+) cells establish efficient rolling on P-selectin, E-selectin, and the CD44 ligand hyaluronic acid under physiological shear flow. ICAM-1 alone did not tether CD34(+) cells under flow, but, in the presence of surface-bound SDF-1, CD34(+) progenitors rolling on endothelial selectin rapidly developed firm adhesion to the endothelial surface, mediated by an interaction between ICAM-1 and its integrin ligand, which coimmobilized with SDF-1. Human CD34(+) cells accumulated efficiently on TNF-activated human umbilical cord endothelial cells in the absence of SDF-1, but they required immobilized SDF-1 to develop firm integrin-mediated adhesion and spreading. In the absence of selectins, SDF-1 also promoted VLA-4-mediated, Gi protein-dependent tethering and firm adhesion to VCAM-1 under shear flow. To our knowledge, this is the first demonstration that SDF-1 expressed on vascular endothelium is crucial for translating rolling adhesion of CD34(+) progenitors into firm adhesion by increasing the adhesiveness of the integrins VLA-4 and LFA-1 to their respective endothelial ligands, VCAM-1 and ICAM-1.


Asunto(s)
Antígenos CD34/metabolismo , Médula Ósea/metabolismo , Quimiocinas CXC/fisiología , Endotelio Vascular/metabolismo , Células Madre Hematopoyéticas/metabolismo , Integrinas/metabolismo , Adhesión Celular , Quimiocina CXCL12 , Selectina E/metabolismo , Sangre Fetal/metabolismo , Citometría de Flujo , Humanos , Receptores de Hialuranos/metabolismo , Molécula 1 de Adhesión Intercelular/metabolismo , Selectina-P/metabolismo , Estrés Mecánico , Linfocitos T/metabolismo , Molécula 1 de Adhesión Celular Vascular/metabolismo
13.
J Clin Invest ; 106(11): 1331-9, 2000 Dec.
Artículo en Inglés | MEDLINE | ID: mdl-11104786

RESUMEN

The chemokine stromal-derived factor-1 (SDF-1) controls many aspects of stem cell function. Details of its regulation and sites of production are currently unknown. We report that in the bone marrow, SDF-1 is produced mainly by immature osteoblasts and endothelial cells. Conditioning with DNA-damaging agents (ionizing irradiation, cyclophosphamide, and 5-fluorouracil) caused an increase in SDF-1 expression and in CXCR4-dependent homing and repopulation by human stem cells transplanted into NOD/SCID mice. Our findings suggest that immature osteoblasts and endothelial cells control stem cell homing, retention, and repopulation by secreting SDF-1, which also participates in host defense responses to DNA damage.


Asunto(s)
Quimiocinas CXC/genética , Daño del ADN , Células Madre/metabolismo , Animales , Células de la Médula Ósea/citología , Células de la Médula Ósea/metabolismo , Línea Celular , Células Cultivadas , Quimiocina CXCL12 , Ciclofosfamida/farmacología , Relación Dosis-Respuesta en la Radiación , Endotelio Vascular/citología , Endotelio Vascular/metabolismo , Citometría de Flujo , Fluorouracilo/farmacología , Regulación de la Expresión Génica/efectos de los fármacos , Regulación de la Expresión Génica/efectos de la radiación , Humanos , Ratones , Ratones Endogámicos BALB C , Ratones Endogámicos C57BL , Ratones SCID , Osteoblastos/citología , Osteoblastos/metabolismo , ARN Mensajero/genética , ARN Mensajero/metabolismo , Células Madre/citología , Células Tumorales Cultivadas
14.
Abdom Radiol (NY) ; 42(7): 1880-1887, 2017 07.
Artículo en Inglés | MEDLINE | ID: mdl-28357531

RESUMEN

PURPOSE: The first reports of hepatic steatosis following pancreaticoduodenectomy (PD) were published several years ago; however, clear risk factors remain to be identified. Therefore, the aim of this study was to identify the risk factors for hepatic steatosis post-PD. METHODS: We studied 90 patients who had undergone PD between September 2005 and January 2015. The inclusion criteria were as follows: available unenhanced CT within one month before PD and at least one unenhanced CT acquisition between PD and chemotherapy initiation. Using scanners, we studied the liver and spleen density as well as the surface areas of visceral (VF) and subcutaneous fat (SCF). These variables were previously identified by univariate and multivariate analyses. RESULTS: Hepatic steatosis occurred in 25.6% of patients at 45.2 days, on average, post-PD. Among the patients with hepatic steatosis, the average liver density was 52 HU before PD and 15.1 HU post-PD (p < 0.001). The Patients with hepatic steatosis lost more VF (mean, 28 vs. 11 cm2) and SCF (28.8 vs. 13.7 cm2) (p < 0.01 and p = 0.01, respectively). Portal vein resection and extensive lymph node dissection were independent risk factors in the multivariate analysis (odds ratio [OR] 5.29, p = 0.009; OR 3.38, p = 0.04, respectively). CONCLUSION: Portal vein resection and extensive lymph node dissection are independent risk factors for post-PD hepatic steatosis.


Asunto(s)
Hígado Graso/diagnóstico por imagen , Escisión del Ganglio Linfático , Neoplasias Pancreáticas/diagnóstico por imagen , Neoplasias Pancreáticas/cirugía , Pancreaticoduodenectomía , Complicaciones Posoperatorias/diagnóstico por imagen , Adulto , Anciano , Anciano de 80 o más Años , Hígado Graso/epidemiología , Femenino , Humanos , Metástasis Linfática , Masculino , Persona de Mediana Edad , Complicaciones Posoperatorias/epidemiología , Estudios Retrospectivos , Factores de Riesgo , Tomografía Computarizada por Rayos X
15.
Cancer Radiother ; 21(8): 774-783, 2017 Dec.
Artículo en Francés | MEDLINE | ID: mdl-29132802

RESUMEN

PURPOSE: Prophylactic radiotherapy to prevent procedure-tracts metastases from malignant pleural mesothelioma remains controversial and clinical practice varies. The purpose was to assess the efficacy of local radiotherapy in a single fraction of 10Gy in preventing malignant seeding at intervention pleural site in patients with malignant pleural mesothelioma. MATERIAL AND METHODS: This is a retrospective cohort study, including patients with histological confirmed malignant pleural mesothelioma treated by prophylactic irradiation to prevent interventional site metastases with a unique fraction of 10Gy with 6 to 18MeV, from January 1990 to December 2013 in the institut de cancérologie de Lorraine (Nancy, France). RESULTS: Ninety-one patients were treated by irradiation in intervention site, involving 120 intervention pleural sites, 91 thoracoscopies, 17 thoracotomies with chest drain and 12 CT or ultrasound guided needle biopsies. The median follow-up was 7 months (interquartile between 3 and 15 months). The overall survival was 43.5% at 12 months. The local progression free survival was 43.7% at 12 month. The incidence of local recurrence was 8% at 12 months. The median interval from radiotherapy to local recurrence was 4 months (2; 32). No grade II or higher toxicity was observed. CONCLUSION: Irradiation of pleural intervention sites with a single fraction of 10Gy is effective, well tolerated, simple, fast and cost effective.


Asunto(s)
Neoplasias Pulmonares/cirugía , Mesotelioma/cirugía , Siembra Neoplásica , Neoplasias Pleurales/cirugía , Dosis de Radiación , Prevención Secundaria/métodos , Adulto , Anciano , Anciano de 80 o más Años , Estudios de Cohortes , Femenino , Francia , Humanos , Masculino , Mesotelioma Maligno , Persona de Mediana Edad , Metástasis de la Neoplasia/prevención & control , Estudios Retrospectivos
16.
Diagn Interv Imaging ; 97(3): 355-60, 2016 Mar.
Artículo en Inglés | MEDLINE | ID: mdl-26546291

RESUMEN

PURPOSE: To describe the multidetector row computed tomography (MDCT) imaging features of HCC that develops in patients who are free from underlying liver cirrhosis and to determine if the MDCT presentation of this specific tumor differs from that of the more common HCC that develops in patients with liver cirrhosis using a retrospective case-control study. PATIENTS AND METHODS: The MDCT examinations of 38 patients with HCC in non-cirrhotic liver (group 1) were quantitatively and qualitatively analyzed and compared to those obtained in 38 patients with HCC in cirrhotic liver (group 2) matched for age and gender. Quantitative and qualitative characteristics of HCC of both groups were compared using univariate analysis. RESULTS: HCCs were significantly larger in group 1 (81.5mm±55.5) than in group 2 (44.5mm±39.1 SD; P=0.0015). In group 1, HCCs were more frequently single tumors (87%) than in group 2 (37%) (P<0.0001), encapsulated (92% vs. 47% respectively; P<0.0001), had more frequently fatty component (24% vs. 8%, respectively; P=0.0279) and internal hemorrhage (29% vs. 3%, respectively; P=0.0033). No significant differences were found between the two groups for location, hyperenhancement of HCC during the arterial phase, washout during the portal phase, endoluminal portal involvement by HCC, endoportal cruoric thrombus, invasion of adjacent organs and underlying liver steatosis. CONCLUSION: HCC in non-cirrhotic liver are larger than those observed in cirrhotic liver and more frequently present as a single encapsulated tumor. They have the same patterns of enhancement than HCC that develops in cirrhotic liver.


Asunto(s)
Carcinoma Hepatocelular/diagnóstico por imagen , Neoplasias Hepáticas/diagnóstico por imagen , Tomografía Computarizada Multidetector , Adulto , Anciano , Anciano de 80 o más Años , Estudios de Casos y Controles , Femenino , Humanos , Cirrosis Hepática , Masculino , Persona de Mediana Edad , Estudios Retrospectivos
17.
Gerokomos (Madr., Ed. impr.) ; 32(4): 263-268, dic. 2021. tab, ilus
Artículo en Español | IBECS (España) | ID: ibc-218803

RESUMEN

El objetivo de este artículo es exponer el abordaje terapéutico y la importancia del factor humano en la aparición de eventos adversos en lesiones producidas por quemaduras. Para ello presentamos el caso de una mujer de 48 años que sufrió quemaduras dérmicas profundas por exposición a aceite hirviendo en ambas extremidades inferiores con una superficie corporal total quemada del 10%. En atención primaria se estableció un plan de actuación de enfermería con visión holística para evitar la aparición de eventos adversos. Los resultados muestran que, pese a las instrucciones dadas sobre la importancia del reposo, el factor humano de la paciente impidió, a partir de los 15 días, que guardase el reposo necesario. Este hecho produjo la profundización de algunas de las lesiones. Se puede concluir que la aparición de los eventos adversos debidos al factor humano retrasó la curación de las heridas y profundizó las lesiones en ambas extremidades (AU)


The objective is to expose the therapeutic approach and importance of the human factor in the appearance of adverse events in injuries caused by burns. To do this, we present the case of a 48-year-old woman who suffered deep skin burns from exposure to boiling oil in lower extreme embassies with a burned total body surface area of 10%. In Primary Care, a nursing action plan with a holistic vision was established to avoid the appearance of adverse events. The results indicated, despite the instructions given on the importance of rest, that the human factor of the patient prevented, from 15 days, keeping the necessary rest. This fact caused the deepening of some of the injuries. It can be concluded that the appearance of adverse events due to the human factor delayed wound healing and deepened the injuries to both extremities (AU)


Asunto(s)
Humanos , Femenino , Persona de Mediana Edad , Quemaduras/enfermería , Traumatismos de la Pierna/enfermería , Vendajes , Atención de Enfermería , Índice de Severidad de la Enfermedad , Resultado del Tratamiento
18.
J Sports Med Phys Fitness ; 45(1): 13-9, 2005 Mar.
Artículo en Inglés | MEDLINE | ID: mdl-16208285

RESUMEN

AIM: The objective of the present study was to determine oxygen uptake (VO(2)) and percentage of maximum oxygen uptake (%VO2max) in obese and non-obese adolescents during various activities in standardised conditions, and the corresponding %VO2max in free-living conditions. METHODS: Twenty-seven obese and 50 non-obese adolescents aged 12 to 16 years participated in this study. Body composition was assessed by bioelectrical impedance analysis and dual energy X-ray absorptiometry (DXA), VO2max by treadmill tests, VO2 corresponding to various activities by whole body calorimetry, and time and % VO2max corresponding to various activities in free-living conditions using the heart-rate recording method and a physical activity diary. RESULTS: VO2max (l/min) was 27.4% higher in obese than in non-obese subjects (p<0.001), but not significantly different after adjustment for fat-free mass (FFM). In the whole body calorimeters, with the same activity program, % VO2max corresponding to sleep and sedentary activities were lower in obese than in non-obese girls (-15.1% and -12.3%, p<0.05), but not significantly different between obese and non-obese boys. However, walking at 4-5-6 km/h corresponded to 47-59% and 71% of VO2max, respectively, in obese, and 34-41% and 48% of VO2max in non-obese subjects (p<0.001). In free-living conditions, moderate physical activities and sports corresponded to 52% vs 35%, and 39% vs 51% of VO2max, respectively, in obese and non-obese adolescents. CONCLUSIONS: In standardised conditions %VO2max did not correspond to the same type of physical activity in obese compared to non-obese adolescents. Consequently, % VO2max is inadequate for comparing the types of physical activities of obese and non-obese adolescents in free-living conditions.


Asunto(s)
Actividad Motora/fisiología , Obesidad/fisiopatología , Consumo de Oxígeno/fisiología , Absorciometría de Fotón , Adolescente , Análisis de Varianza , Composición Corporal , Niño , Impedancia Eléctrica , Metabolismo Energético , Femenino , Frecuencia Cardíaca/fisiología , Humanos , Masculino
19.
Mol Immunol ; 37(15): 871-87, 2000 Oct.
Artículo en Inglés | MEDLINE | ID: mdl-11282392

RESUMEN

More than 35 years ago, study of an unknown immunoglobulin (Ig) in the serum from a myeloma patient led to the discovery of IgD. Subsequently, the finding that it also exists as a membrane-bound Ig stimulated a large number of studies during the 70s. Then, the interest on IgD shrank, largely because of the lack of known function of secretory IgD (secIgD) and of a stagnating knowledge of the functions of surface IgD. In the recent years, very significant advances followed the tremendous accumulation of data on the physiology of the B cell receptor, of which IgD is the major component, on the role of secIgD in normal and diseased individuals. This review, which is focused on human IgD but integrates data in the mouse and other species when needed, summarizes present data on the structure, synthesis and functions of both membrane and secIgD, IgD receptors and the involvement of IgD in various diseases, especially the hyperIgD syndrome.


Asunto(s)
Inmunoglobulina D/genética , Inmunoglobulina D/fisiología , Animales , Membrana Celular/inmunología , Membrana Celular/metabolismo , Humanos , Inmunoglobulina D/biosíntesis , Inmunoglobulina D/inmunología , Receptores de Superficie Celular/inmunología , Receptores Fc/inmunología , Receptores Inmunológicos/inmunología
20.
Diagn Interv Imaging ; 96(6): 547-62, 2015 Jun.
Artículo en Inglés | MEDLINE | ID: mdl-24776810

RESUMEN

Radioembolization (RE) is a selective internal radiotherapy technique in which yttrium-90 blended microspheres are infused through the hepatic arteries. It is based on the fact that primary and secondary hepatic tumors are vascularized mostly by arterial blood flow whereas healthy hepatocytes obtain their blood supply mostly from the portal network. This enables high radiation doses to be delivered, sparing the surrounding non-malignant liver parenchyma. Most of the complications are caused by unexpected particles passing into the gastrointestinal tract through branches originating from the main hepatic arterial supply. Knowledge of this hepatic arterial network and of its variations and the technical considerations this raises are required in preparation for treatment. This work describes the specific anatomical features and techniques for this anatomy through recent literature illustrated by cases from our own experience.


Asunto(s)
Embolización Terapéutica/métodos , Neoplasias Hepáticas/radioterapia , Microesferas , Radioisótopos de Itrio/uso terapéutico , Variación Anatómica , Arteria Hepática/anatomía & histología , Arteria Hepática/diagnóstico por imagen , Humanos , Hígado/irrigación sanguínea , Hígado/diagnóstico por imagen , Neoplasias Hepáticas/irrigación sanguínea , Radiografía
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