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1.
Abdom Radiol (NY) ; 2024 Sep 14.
Artículo en Inglés | MEDLINE | ID: mdl-39277562

RESUMEN

Chronic contained rupture of abdominal aortic aneurysm (CCR-AAA) is a rare subtype of abdominal aortic rupture that can mimic other retroperitoneal lesions. We report a case of CCR-AAA in a man in his sixties who presented with a 10-month history of right low back pain and weight loss. Contrast-enhanced computed tomography (CT) revealed a lobulated retroperitoneal mass around the abdominal aorta, initially misdiagnosed as a possible hemorrhagic retroperitoneal tumor. Despite multiple imaging studies including CT, magnetic resonance imaging (MRI), and 18F-fluorodeoxyglucose positron emission tomography (FDG-PET)/CT, as well as a CT-guided biopsy, the correct diagnosis remained elusive for 34 months. Key findings included subtle aortic wall irregularity on CT, high signal intensity on T1-weighted MRI suggesting hematoma, peripheral FDG uptake on PET/CT, and histological findings of biopsy tissue consistent with organizing hematoma. Surgery confirmed the diagnosis, revealing an organized hematoma with a defect in the right wall of the abdominal aortic aneurysm. This case demonstrates that CCR-AAA can present with atypical radiological features, potentially leading to misdiagnosis. When encountering a para-aortic mass with a hemorrhagic component, careful observation of the AAA morphology and aortic wall contour is crucial for an accurate diagnosis of CCR-AAA.

2.
Cureus ; 16(7): e63972, 2024 Jul.
Artículo en Inglés | MEDLINE | ID: mdl-39104995

RESUMEN

Meningoencephalitis caused by varicella-zoster virus (VZV) is a serious condition requiring prompt antiviral treatments, but magnetic resonance imaging (MRI) findings are often normal, limiting early diagnostic utility. We report a case of severe VZV-associated meningoencephalitis characterized by diffuse T2 hyperintense lesions covering the brain surface on MRI, presumed to be vasogenic edema. An immunocompetent 78-year-old Japanese woman presented with a disturbance of consciousness preceded by seven days of headache. On admission, she was in a semi-coma with intermittent convulsive seizures and had a localized skin rash with blisters on her back. Brain MRI showed diffuse T2 hyperintensity on the brain surface with an elevated apparent diffusion coefficient and the marked gadolinium-contrast enhancement of the pia-arachnoid membrane and vessel walls. Polymerase chain reaction using cerebrospinal fluid revealed the presence of VZV, and then she was diagnosed with VZV-associated meningoencephalitis. Treatment with acyclovir and corticosteroids was initiated, leading to some clinical improvement; however, the patient developed acute non-occlusive mesenteric ischemia and died on the 10th day of hospitalization. The characteristic MRI findings observed in our patient may be useful in considering the pathogenesis and early diagnosis of this rare entity.

3.
Int J Hematol ; 120(4): 520-524, 2024 Oct.
Artículo en Inglés | MEDLINE | ID: mdl-39003680

RESUMEN

Rosai-Dorfman-Destombes disease (RDD) is a rare histiocytosis characterized by accumulation of S100 + , CD68 + , and CD1a- histiocytes, with emperipolesis. It occurs predominantly in black adolescents and young adults, but rarely in Japanese children. Recently, oncogenic mutations in mitogen-activated protein kinase (MAPK) pathway genes were reported in 30-50% of patients with RDD, and several studies have described treatment of adult patients with MAPK inhibitors. Here, we present the case of a Japanese boy with refractory RDD without signs of cardiofaciocutaneous (CFC) syndrome who harbored MAP2K1 p.Lys59del and responded to trametinib. The patient had lymph node, nasal cavity, kidney, upper respiratory tract, and intracranial involvement. RDD progressed after multi-agent chemotherapy, but responded to trametinib (0.025 mg/kg). Trametinib did not eliminate the mass lesions, but trametinib plus minimal prednisolone (0.1 mg/kg) resulted in a good outcome for more than 15 months, without significant adverse effects. MAP2K1 p.Lys59del has been described as a germline mutation in a patient with CFC syndrome, but not as a somatic mutation in patients with malignancies. Trametinib may be a promising drug for children with RDD that is refractory to multi-agent chemotherapy. Its long-term efficacy and safety alone and in combination with chemotherapy should be investigated.


Asunto(s)
Histiocitosis Sinusal , MAP Quinasa Quinasa 1 , Mutación , Piridonas , Pirimidinonas , Humanos , Pirimidinonas/uso terapéutico , Histiocitosis Sinusal/tratamiento farmacológico , Histiocitosis Sinusal/genética , Piridonas/uso terapéutico , Masculino , MAP Quinasa Quinasa 1/genética , MAP Quinasa Quinasa 1/antagonistas & inhibidores , Niño , Resultado del Tratamiento , Inhibidores de Proteínas Quinasas/uso terapéutico
4.
Cureus ; 16(5): e60187, 2024 May.
Artículo en Inglés | MEDLINE | ID: mdl-38868262

RESUMEN

Foreign body granulomas following endovascular treatment are rare complications and are mostly reported in the brain or cutaneous vascular tissues. To the best of our knowledge, no study to date has reported on foreign body granulomas in the abdomen after injection of N-butyl-2-cyanoacrylate (NBCA)-lipiodol mixture into the abdominal arteries. This study reports a case of foreign body granuloma that appeared 12 months after the embolization of a right internal iliac artery aneurysm using an NBCA-lipiodol mixture, which posed challenges in differentiation from malignant tumors. We present a 77-year-old man who underwent embolization of a right internal iliac artery aneurysm and open surgical repair of an abdominal aortic aneurysm. A contrast-enhanced CT performed 12 months postoperatively revealed a right-sided retroperitoneal mass surrounding the iliopsoas muscle. The mass contained multiple, small, hyperdense areas, suggesting the migration of the NBCA-lipiodol mixture casts from the embolized right internal iliac artery aneurysm. The differential diagnosis included foreign body granuloma, lymphoma, and sarcoma. A biopsy of the lesion revealed a granuloma with various stages of inflammation, no hemosiderin deposition, multinucleated giant cells, and foam cells containing fat, and was diagnosed with a foreign body granuloma. Special staining for microorganisms revealed no findings suggestive of infection. Because the patient was asymptomatic, no treatment was administered. Contrast-enhanced CT at 24 months postoperatively showed shrinkage of the mass, with no change in size noted at 48 months postoperatively. This report highlights a foreign body granuloma that mimicked malignant tumors. Extravascular migration of the NBCA-lipiodol mixture casts likely contributed to granuloma formation. Radiologists should consider foreign body granulomas after embolization using NBCA into the abdominal arteries.

5.
Neuropsychopharmacol Rep ; 44(2): 417-423, 2024 Jun.
Artículo en Inglés | MEDLINE | ID: mdl-38634351

RESUMEN

AIMS: To investigate the negative attitudes of Japanese psychiatrists toward atypical long-acting injectable (LAI) antipsychotics, which are the current mainstream LAIs in Japan. METHODS: We surveyed 69 Japanese psychiatrists using a 5-point Likert scale to assess their attitudes toward atypical LAI antipsychotics. Our assessment referenced concerns identified in a study conducted in Japan a decade ago, which found significant differences when compared with a survey of German psychiatrists. We also identified the factors influencing these negative attitudes. Additionally, the results were compared with those of previous Japanese and German studies. RESULTS: More than 50% of Japanese psychiatrists expressed negative attitudes toward atypical LAI antipsychotics in various areas. These concerns included apprehensions about cost, reluctance to recommend them initially, pain from injections, complexity of switching to LAI, usage in first-episode cases, and sufficient medication adherence with oral drugs. In all three studies, cost and adequate adherence to oral medication were concerns that exceeded the average of the three negative comments. Age and experience in psychiatry influenced the psychiatrists' attitudes toward using these drugs in first-episode cases. CONCLUSIONS: These findings shed light on the reasons for the underutilization of atypical LAI antipsychotics and suggest opportunities to enhance their appropriate use in clinical settings.


Asunto(s)
Antipsicóticos , Actitud del Personal de Salud , Preparaciones de Acción Retardada , Psiquiatría , Humanos , Antipsicóticos/administración & dosificación , Antipsicóticos/uso terapéutico , Japón , Masculino , Psiquiatría/métodos , Femenino , Adulto , Persona de Mediana Edad , Inyecciones , Cumplimiento de la Medicación/estadística & datos numéricos , Cumplimiento de la Medicación/psicología , Encuestas y Cuestionarios , Pautas de la Práctica en Medicina/estadística & datos numéricos , Psiquiatras
7.
CVIR Endovasc ; 7(1): 40, 2024 Apr 25.
Artículo en Inglés | MEDLINE | ID: mdl-38662076

RESUMEN

BACKGROUND: This study aimed to assess the effectiveness and versatility of an intentional early detachment technique with detachable coils in addressing challenging vascular embolization scenarios. This novel approach aims to provide an alternative method for achieving precise coil placement when standard methods of detachable coil placement are ineffective owing to vascular anatomy or limited available equipment. MATERIALS AND METHODS: This retrospective study included 11 patients (nine males and two females; median age, 77 years) who underwent embolization procedures between October 2021 and December 2023 using the intentional early detachment technique through 1.6-Fr or 1.3-Fr microcatheters. In this technique, detachable coils were intentionally detached within the microcatheter and placed through saline flushing. The technique's technical success, complications, and clinical success were evaluated. RESULTS: The technique was applied in three distinct scenarios: tortuous vascular anatomy (four cases), inadequate system backup (three cases), and 1.3-Fr microcatheter use (four cases). The technical and clinical success rates were 100%. No complications were observed, and no cases of coil migration or malpositioning. CONCLUSION: The intentional early detachment technique is valuable for interventional radiologists and offers a solution for challenging vascular embolization scenarios. Its application is limited to specific circumstances; however, it can significantly enhance coil placement in complex cases, thereby contributing to improved patient care.

8.
Neuropsychopharmacol Rep ; 44(1): 34-41, 2024 Mar.
Artículo en Inglés | MEDLINE | ID: mdl-38183370

RESUMEN

The coronavirus disease pandemic has presented healthcare systems with unprecedented challenges globally and substantially impacted the management of chronic diseases such as schizophrenia. This narrative review highlights the usefulness of long-acting injectable antipsychotics (LAIs) as maintenance therapy for patients with schizophrenia during the pandemic. The analysis of relevant literature and psychiatric survey data revealed diverse trends in LAIs prescription and patient adherence with oral antipsychotics. Although some studies have reported a decrease in LAIs prescriptions owing to pandemic-related disruptions, others have suggested stable patient adherence with oral antipsychotics. Approximately 70% of Japanese psychiatrists reported an increase in schizophrenia relapse rates in a survey, underscoring the critical role of LAIs in maintaining therapeutic stability. The potential benefits of LAIs with extended dosing intervals have been highlighted, including improving oral medication adherence and reducing the frequency of hospital visits. In conclusion, this review emphasizes the continued need for uninterrupted LAIs therapy in conjunction with community and home-based care despite the disruptions caused by the coronavirus disease pandemic. Further development of LAIs maintenance therapy strategies considering the ongoing pandemic and potential future public health emergencies are required.


Asunto(s)
Antipsicóticos , COVID-19 , Esquizofrenia , Humanos , Antipsicóticos/uso terapéutico , Pandemias , Esquizofrenia/tratamiento farmacológico , Inyecciones Intramusculares
9.
J Stroke ; 25(3): 388-398, 2023 Sep.
Artículo en Inglés | MEDLINE | ID: mdl-37813673

RESUMEN

BACKGROUND AND PURPOSE: Differences in measurement of the extent of acute ischemic stroke using the Alberta Stroke Program Early Computed Tomographic Score (ASPECTS) by non-contrast computed tomography (CT-ASPECTS stratum) and diffusion-weighted imaging (DWI-ASPECTS stratum) may impact the efficacy of endovascular therapy (EVT) in patients with a large ischemic core. METHODS: The RESCUE-Japan LIMIT (Recovery by Endovascular Salvage for Cerebral Ultra-acute Embolism Japan-Large IscheMIc core Trial) was a multicenter, open-label, randomized clinical trial that evaluated the efficacy and safety of EVT in patients with ASPECTS of 3-5. CT-ASPECTS was prioritized when both CT-ASPECTS and DWI-ASPECTS were measured. The effects of EVT on the modified Rankin Scale (mRS) score at 90 days were assessed separately for each stratum. RESULTS: Among 183 patients, 112 (EVT group, 53; No-EVT group, 59) were in the CT-ASPECTS stratum and 71 (EVT group, 40; No-EVT group, 31) in the DWI-ASPECTS stratum. The common odds ratio (OR) (95% confidence interval) of the EVT group for one scale shift of the mRS score toward 0 was 1.29 (0.65-2.54) compared to the No-EVT group in CT-ASPECTS stratum, and 6.15 (2.46-16.3) in DWI-ASPECTS stratum with significant interaction between treatment assignment and mode of imaging study (P=0.002). There were significant interactions in the improvement of the National Institutes of Health Stroke Scale score at 48 hours (CT-ASPECTS stratum: OR, 1.95; DWIASPECTS stratum: OR, 14.5; interaction P=0.035) and mortality at 90 days (CT-ASPECTS stratum: OR, 2.07; DWI-ASPECTS stratum: OR, 0.23; interaction P=0.008). CONCLUSION: Patients with ASPECTS of 3-5 on MRI benefitted more from EVT than those with ASPECTS of 3-5 on CT.

10.
Neurol Genet ; 8(3): e680, 2022 Jun.
Artículo en Inglés | MEDLINE | ID: mdl-35620139

RESUMEN

Objective: In this study, we report the case of a 24-year-old man with intellectual disability and childhood-onset seizures. This patient had newly identified biallelic variants in the laminin subunit gamma 3 (LAMC3) gene with unreported cortical malformation. Methods: Exome sequencing. Results: Genetic analyses revealed new biallelic variants in the LAMC3 gene. An MRI examination of the brain revealed cortical malformations predominantly in the temporal lobes and mildly in the occipital, frontal, and parietal lobes. In addition, our patient also exhibited mild midline malformation in the ventral pons, which is unique to LAMC3 variants. Discussion: Patients with LAMC3 variants have been reported to exhibit cortical malformation predominantly in the occipital lobes, but this patient exhibited cortical malformation predominantly in the temporal lobes and mildly in the occipital, frontal, and parietal lobes. In addition, this patient also exhibited mild midline malformation in the ventral pons. These unique findings cast new light on the role of LAMC3 in brain development.

11.
Radiol Case Rep ; 17(7): 2424-2427, 2022 Jul.
Artículo en Inglés | MEDLINE | ID: mdl-35601381

RESUMEN

A 30-year-old woman suffering from an eating disorder and alcoholism presented with a progressively worsening gait disturbance lasting 2 weeks. Her neurological findings included impaired ocular motility and trunk ataxia. Fluid-attenuated inversion recovery imaging of the brain showed hyperintensity in the dorsal brainstem, aqueduct, thalamus, and cerebral cortex. A long hyperintense segment on T2-weighted imaging was visible in the central gray matter of the cervical spinal cord. No restricted diffusion was observed; thus, T2 elongation in the spine was suggested to be due to vasogenic edema. We diagnosed the patient with Wernicke's encephalopathy and initiated vitamin supplementation. Thereafter, her symptoms rapidly improved; magnetic resonance imaging on the 11th day of hospitalization showed normalization of the signals in her brain and spinal cord. As our case demonstrates, Wernicke's encephalopathy can induce vasogenic edema of the spinal cord, which can rapidly improve with early therapeutic intervention.

13.
J Stroke Cerebrovasc Dis ; 30(11): 106087, 2021 Nov.
Artículo en Inglés | MEDLINE | ID: mdl-34500200

RESUMEN

BACKGROUND: Meningovascular neurosyphilis, a form of early neurosyphilis, can cause infectious arteritis, which can be complicated by cerebral infarction. High-resolution vessel wall imaging (HR-VWI) is one of the techniques used to directly visualize the vessel wall. Herein, we present a rare case of meningovascular neurosyphilis, in which intracranial arterial vasculitis was evaluated using HR-VWI. CASE PRESENTATION: A 22-year-old man with no medical history of any condition was brought to the emergency room with one day history of right upper and lower extremity weakness. Diffusion-weighted magnetic resonance (MR) imaging showed a high signal from the left putamen to the corona radiata, and MR angiography showed stenosis of the right internal carotid artery (ICA) and the bilateral middle cerebral arteries (MCAs). HR-VWI showed thickening, along with smooth, intense, and concentric enhancement of the right ICA and the bilateral MCAs. The patient was diagnosed with neurosyphilis based on the findings of the blood tests and cerebrospinal fluid examination. The patient's symptoms gradually improved after treatment with intravenous penicillin G and oral antiplatelet agents. HR-VWI, performed approximately 6 months after the treatment, revealed improvement in the contrast enhancement of the vessel wall and the vascular stenosis. CONCLUSION: To the best of our knowledge, this is the first report of meningovascular neurosyphilis that evaluated the course of treatment using HR-VWI. Our report highlights the effectiveness of HR-VWI to determine the effects of treatment on meningovascular neurosyphilis.


Asunto(s)
Accidente Cerebrovascular Isquémico , Neurosífilis , Humanos , Accidente Cerebrovascular Isquémico/etiología , Angiografía por Resonancia Magnética , Masculino , Neurosífilis/diagnóstico por imagen , Adulto Joven
14.
Neuroradiology ; 63(4): 529-538, 2021 Apr.
Artículo en Inglés | MEDLINE | ID: mdl-32989557

RESUMEN

PURPOSE: The word "fixel" refers to the specific fiber population within each voxel, and fixel-based analysis (FBA) is a recently developed technique that facilitates fiber tract-specific statistical analysis. The aim of the paper is to apply FBA to detect impaired fibers for corticobasal syndrome (CBS) especially in regions that contain multiple crossed fibers. METHODS: FBA was performed in cohorts of participants clinically diagnosed with CBS (n = 10) and Parkinson's disease (n = 15) or in healthy controls (n = 9). The parameters of the diffusion weighted image were echo time, 83 ms; time, 8123.6 ms; flip angle, 90°; section thickness, 2 mm; b = 1000 s/mm2; and 32 axes. Diffusion tensor analysis was conducted using tract-based spatial statistics (TBSS), and white matter volume was estimated via voxel-based morphometry. RESULTS: A comparison of PD or HC to CBS revealed a significant difference in the dentatorubrothalamic tract of the brainstem in FBA in addition to the affected regions in voxel-based morphometry and TBSS (family-wise error-corrected p < 0.05). Reduction of the white matter fibers crossing the brainstem could not be detected via microstructural changes identified using TBSS, but it was detected using FBA. CONCLUSION: FBA has some advantages in determining the distribution of corticobasal syndrome lesions.


Asunto(s)
Enfermedad de Parkinson , Sustancia Blanca , Encéfalo , Humanos , Síndrome
15.
Dysphagia ; 36(3): 483-491, 2021 06.
Artículo en Inglés | MEDLINE | ID: mdl-32743742

RESUMEN

Muscle aging such as sarcopenia adversely affects motor activities. However, few studies have elucidated the aging physiological mechanism of tongue concerted with the changes muscle composition. The present study aimed to examine the tongue composition changes to detect the effect of tongue fat mass on tongue pressure and swallowing function with aging. Twenty community-dwelling elderly without head and neck cancer, stroke, or neuromuscular disease and 20 healthy young were included. Tongue volume, tongue fat mass, tongue lean muscle mass, and tongue fat percentage were evaluated with 3D magnetic resonance imaging (MRI) and Dixon MRI. Tongue pressure was also measured. Swallowing function among elderly individuals was assessed via videofluorography, which was evaluated using the penetration-aspiration scale (PAS) and normalized residue ratio scale (NRRS). Tongue fat mass and tongue fat percentage significantly increased with aging. The tongue fat percentage of elderly participants was 20%, which was two times greater than that of young participants. No significant difference was observed in tongue volume and tongue lean muscle mass. A significantly negative correlation was observed between tongue fat mass and tongue fat percentage as well as tongue pressure. Conversely, tongue volume was not significantly correlated with tongue pressure. Tongue muscle composition exhibited no effect in the PAS and NRRS. Increase of fat mass is a major change in tongue composition with aging, which is associated with low tongue pressure. Thus, attention must be paid not only to tongue quantity but also to the quality of tongue muscles.


Asunto(s)
Deglución , Lengua , Anciano , Humanos , Imagen por Resonancia Magnética , Músculos , Presión , Lengua/diagnóstico por imagen
17.
Jpn J Radiol ; 37(4): 277-282, 2019 Apr.
Artículo en Inglés | MEDLINE | ID: mdl-30671706

RESUMEN

PURPOSE: To evaluate the frequency, characteristics, and clinical significance of transient hyperintensity foci on T1-weighted images (T1WI) in acute disseminated encephalomyelitis (ADEM). MATERIALS AND METHODS: Patients diagnosed with ADEM underwent MR studies at the time of disease onset and every 3 months or more often thereafter. The frequency and appearance timing of abnormal signals including T1WI and their morphological characteristics were evaluated. Relations between patient symptoms and abnormal signals on MRI were also evaluated. RESULTS: Five ADEM patients were included in this study. Linear (n = 2) or nodular (n = 1) T1-hyperintensity foci appeared in 3 patients (60%, 3/5). Locations of T1-hyperintensity foci were both cortical/subcortical region and basal ganglia (n = 1), subcortical region alone (n = 1), and internal capsule (n = 1). Those T1-hyperintensity foci were located within the T2-weighted image (T2WI) and fluid-attenuated inversion recovery (FLAIR) hyperintensity foci on initial MRI. Some T1-hyperintensity foci also showed hyperintensity on diffusion-weighted image (DWI) and contrast enhancement. T1-hyperintensity appeared at 14-43 days (median, 28 days), and disappeared in 2 patients at 91 days and 627 days after disease onset. There were no neurological sequelae remained in any patients. CONCLUSION: T1-hyperintensity foci is not a rare finding (60%) and it can be observed after improvement in symptoms in ADEM.


Asunto(s)
Encefalomielitis Aguda Diseminada/diagnóstico por imagen , Imagen por Resonancia Magnética/métodos , Adolescente , Adulto , Encéfalo/diagnóstico por imagen , Encéfalo/patología , Niño , Preescolar , Medios de Contraste , Progresión de la Enfermedad , Encefalomielitis Aguda Diseminada/patología , Femenino , Humanos , Aumento de la Imagen , Masculino , Estudios Retrospectivos , Adulto Joven
18.
Clin Neurol Neurosurg ; 173: 91-95, 2018 10.
Artículo en Inglés | MEDLINE | ID: mdl-30096569

RESUMEN

OBJECTIVE: Changes in brain tissue can be detected sensitively using PRESTO (principles of echo-shifting with a train of observations) magnetic resonance imaging (MRI). The aim of this study was to evaluate the correlation between the proliferative ability of astrocytoma and intratumoral spotty signal voids seen as hypo-intense dots on PRESTO MRI. PATIENTS AND METHODS: Fifty-seven astrocytic tumors, comprising 14 astrocytomas, 12 anaplastic astrocytomas, and 31 glioblastomas, were included in this retrospective study. The tumors were classified independently by blinded radiologists according to the number of spotty signal voids detected on PRESTO-MRI as follows: spot-free (grade 0), less than 3 spots (grade 1), or more than 3 spots or a large spot (grade 2). RESULTS: Thirteen patients (92.9%) with astrocytoma were classified as PRESTO grade 0 and 1 patient (7.1%) was classified as grade 1. Seven patients (58.3%) with anaplastic astrocytoma were classified as PRESTO grade 0, 1 (8.3%) as grade 1, and 4 as grade 2 (33.3%). Three patients (9.7%) with glioblastoma were classified as grade 0, 6 (19.4%) as grade 1, and 22 (70.9%) as grade 2. There was a strong correlation between PRESTO tumor grade and the mean MIB-1 index. CONCLUSIONS: These results indicate that a grading system based on the number of spotty signal voids detected on PRESTO images would be useful for the diagnosis of astrocytic tumors and predicting their proliferative ability.


Asunto(s)
Astrocitoma/patología , Neoplasias Encefálicas/patología , Glioblastoma/patología , Imagen por Resonancia Magnética , Adulto , Anciano , Anciano de 80 o más Años , Encéfalo/patología , Femenino , Humanos , Imagen por Resonancia Magnética/métodos , Masculino , Persona de Mediana Edad , Clasificación del Tumor/métodos
19.
Rinsho Ketsueki ; 58(11): 2243-2249, 2017.
Artículo en Japonés | MEDLINE | ID: mdl-29212976

RESUMEN

Congenital leukemia is a rare subgroup of childhood leukemia. Lineage switches in leukemic cells are relatively rare events, which have been occasionally reported in congenital leukemia. To the best of our knowledge, the survival of congenital leukemia patients with lineage switch has not been previously documented. This lack of documentation may be attributable to extremely poor prognosis of these patients. We describe a case of a newborn female with initial diagnosis of MLL-AF4 positive B-precursor acute lymphoblastic leukemia, who developed lineage switch to acute monocytic leukemia following the induction therapy. Although morphological remission was temporary, she received an HLA-haploidentical bone marrow transplant from her father with non-remission status because of an early relapse at the age of 4 months. Despite many difficulties such as graft-versus-host disease, growth impairment, and psychomotor retardation, she remained in remission for 3 years and 7 months after the transplant. This successful outcome suggests that the graft-versus-leukemia effect was potentially accomplished in the patient. Taken together, early HLA-haploidentical stem cell transplant following remission is required for congenital leukemia patients with lineage switch, and it may be an effective alternative for refractory patients.


Asunto(s)
Antígenos HLA/inmunología , Leucemia Monocítica Aguda/terapia , Leucemia-Linfoma Linfoblástico de Células Precursoras B/tratamiento farmacológico , Trasplante de Células Madre , Femenino , Haplotipos , Humanos , Quimioterapia de Inducción , Recién Nacido , Leucemia Monocítica Aguda/patología , Leucemia-Linfoma Linfoblástico de Células Precursoras B/congénito , Leucemia-Linfoma Linfoblástico de Células Precursoras B/inmunología , Leucemia-Linfoma Linfoblástico de Células Precursoras B/patología , Recurrencia , Inducción de Remisión , Factores de Tiempo
20.
Nat Prod Commun ; 12(4): 499-502, 2017 Apr.
Artículo en Inglés | MEDLINE | ID: mdl-30520581

RESUMEN

Chrysin suppresses the TNFα-induced increase in the secretion of plasma plasminogen activator inhibitor 1 (PAl-1), a risk factor for thrombotic diseases, from human umbilical vein endothelial cells (HUVECs). The present study aimed to determine the association between the location of the hydroxyl groups in chrysin.to levels of-PAI-1. in the medium of HUVEC stimulated with TNFα. We cultured HUVEC for 3 h in medium containing chrysin or various flavonoids and then stimulated them with TNFα (10 ng/mL) for 12 h. Levels of PAI-1 antigen measured using ELISA showed that chrysin significantly inhibited the PAl- I increase with an IC50 of 15.6 µM. The flavones, galangin, baicalein, 5-hydroxyflavone, 6-hydroxyflavone, 7-hydroxyflavone and quercetin did not significantly inhibit the PAI- increase. Apigenin and luteolin were cytotoxic and thus their ability to inhibit PAI production could not be evaluated. Chrysin also inhibited PAI- mRNA expression whereas the other compounds did not. Hydroxyl groups located in the A-5 and A-7 positions were essential for the inhibitoryactivity, which along with cytotoxicity, was significantly influenced by adding a third hydroxyl group.


Asunto(s)
Flavonoides/química , Flavonoides/farmacología , Células Endoteliales de la Vena Umbilical Humana/metabolismo , Inhibidor 1 de Activador Plasminogénico/metabolismo , Células Cultivadas , Células Endoteliales de la Vena Umbilical Humana/efectos de los fármacos , Humanos , Estructura Molecular , Factor de Necrosis Tumoral alfa/metabolismo
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