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This study aimed to evaluate the effects of a virtual reality intervention for dementia care education in Japanese acute care nurses. Non-randomised controlled trial was used to assess the effectiveness of the intervention pre- and post-intervention and at a 1-month follow-up. This study enrolled 20 and 19 nurses in the intervention and control groups, respectively. Generalized estimating equation analyses revealed significant differences in scores on the Dementia Nursing Competency Scale in Acute Hospitals and Staff Experiences of Working with Dementia Residents questionnaire in the intervention group. A post hoc test revealed significantly higher scores on the Dementia Nursing Competency Scale in Acute Hospitals at 1 month after compared with pre-intervention. Staff Experiences of Working with Dementia Residents questionnaire scores were significantly lower at pre- than that at post-intervention and 1 month after intervention. These findings could improve competency of dementia nursing care and attitudes toward people with dementia.
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Demência , Humanos , Inquéritos e Questionários , Escolaridade , JapãoRESUMO
BACKGROUND: This study aims to evaluate the association between smoking habits and dental care utilization and cost in individuals registered with the Japan Health Insurance Association, Osaka branch. METHODS: We used the administrative claims database and specific medical check-up data and included 226,359 participants, who visited dental institutions, underwent dental examinations, and underwent specific medical checkups, with smoking data from April 2016 to March 2017. We calculated propensity scores with age, gender, exercise, eating habits, alcohol intake, and sleep. We also compared dental care utilization with the total cost of each procedure. RESULTS: According to propensity score matching, 62,692 participants were selected for each group. Compared to non-smokers, smokers were younger, and a higher proportion were men. Smokers tended to skip breakfast, have dinner just before bed, and drink alcohol. After adjusting for potential confounding factors with propensity score matching, the mean annual dental cost among smokers was significantly higher than non-smokers. The prevalence of pulpitis, missing teeth, and apical periodontitis were higher among smokers than non-smokers, while inlay detachment, caries, and dentine hypersensitivity were higher among non-smokers. CONCLUSION: This study suggests that smokers have higher dental cost consisted of progressive dental caries, missing teeth, and uncontrolled acute inflammation that necessitated the use of medications. It is suggested that smokers tend to visit the dentist after their symptoms become severe.
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Cárie Dentária , Consumo de Bebidas Alcoólicas/efeitos adversos , Consumo de Bebidas Alcoólicas/epidemiologia , Assistência Odontológica , Cárie Dentária/epidemiologia , Feminino , Humanos , Masculino , Fumantes , Fumar/efeitos adversos , Fumar/epidemiologiaRESUMO
Disaster preparedness is the most important measure that can be taken to reduce damage. However, disaster preparedness is predicted to be difficult for older adults with cognitive impairment. Thus, we investigated the effects on disaster preparedness of cognitive impairment in persons requiring special care who are 75 or older. The survey included subject characteristics such as demographic indicators, physical function, cognitive function impairments, community involvement, and disaster preparedness. Cross-sectional data from 711 subjects were divided into two groups, a cognitive impairment group and an unimpaired group. Next, to show the effects of subject characteristics in each group on disaster preparedness, a binomial logistic regression analysis was performed. Then, the effects on disaster preparedness were compared between the two groups. This study showed that persons requiring special care who are 75 or older, and have the characteristics of "living alone," "requiring care," and "needing mobility assistance" in addition to cognitive impairment, have increased difficulty making disaster preparedness. Government officials and community supporters therefore need to recognize that these people should be supported on a priority basis, and to provide continuing support for disaster preparedness.
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Disfunção Cognitiva , Planejamento em Desastres , Desastres , Idoso , Estudos Transversais , Humanos , JapãoRESUMO
Jabara (Citrus jabara Hort. ex Y. Tanaka) is a type of citrus fruit known for its beneficial effect against seasonal allergies. Jabara is rich in the antioxidant narirutin whose anti-allergy effect has been demonstrated. One of the disadvantages in consuming Jabara is its bitter flavor. Therefore, we fermented the fruit to reduce the bitterness and make Jabara easy to consume. Here, we examined whether fermentation alters the anti-allergic property of Jabara. Suppression of degranulation and cytokine production was observed in mast cells treated with fermented Jabara and the effect was dependent on the length of fermentation. We also showed that 5-hydroxymethylfurfural (5-HMF) increases as fermentation progresses and was identified as an active component of fermented Jabara, which inhibited mast cell degranulation. Mast cells treated with 5-HMF also exhibited reduced degranulation and cytokine production. In addition, we showed that the expression levels of phospho-PLCγ1 and phospho-ERK1/2 were markedly reduced upon FcεRI stimulation. These results indicate that 5-HMF is one of the active components of fermented Jabara that is involved in the inhibition of mast cell activation.
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Citrus/química , Furaldeído/análogos & derivados , Mastócitos/efeitos dos fármacos , Mastócitos/fisiologia , Extratos Vegetais/farmacologia , Receptores de IgE/metabolismo , Degranulação Celular/efeitos dos fármacos , Degranulação Celular/imunologia , Citocinas/genética , Citocinas/metabolismo , Fermentação , Alimentos Fermentados , Furaldeído/química , Furaldeído/farmacologia , Imunoglobulina E/imunologia , Mediadores da Inflamação/metabolismo , Extratos Vegetais/químicaRESUMO
A male infant suffered from partial seizures at four months of age, and developed West syndrome at eight months of age. ACTH therapy was effective for the West syndrome. However, partial seizures recurred at 14 months of age, which could not be sufficiently controlled with an anti-epileptic drug. A characteristic facial appearance, great toe abnormalities, and developmental retardation were noted. An interstitial deletion of 2q was detected by chromosomal G-banding and array comparative genomic hybridization (CGH) confirmed the deletion as arr 2q24.3q31.3 (166,303,447-180,982.972) ×1 (build19). He presented with clinical findings similar to those of the recently defined 2q31.1 deletion syndrome. The deletion extended to the SCN1A gene, a gene responsible for Dravet syndrome, mapped to the 2q24.3 region. No deletion was noted in the adjacent SCN2A gene. Thus, for interstitial deletions, detailed breakpoints should be identified by array CGH. The frequency of epilepsy varies with deletion ranges in the 2q24-q31 region, suggesting that deletions in the SCN1A gene deletion, as well as in the 2q31.1 region, are involved in the development of West syndrome.
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Cromossomos Humanos Par 2 , Espasmos Infantis/genética , Deleção Cromossômica , Humanos , Lactente , Imageamento por Ressonância Magnética , Masculino , Espasmos Infantis/diagnóstico por imagemRESUMO
Primary peritoneal carcinoma (PPC) is treated similarly to advanced epithelial ovarian carcinoma (aEOC); however, the standard approach for the management of PPC is controversial. The objective of this study was to evaluate the clinical features and prognosis of those patients. A retrospective analysis was performed of eight patients with PPC between January 2008 and December 2015. Clinicopathologic parameters, the diagnostic modality, treatment, and oncologic outcome were analyzed. The median age at the time of diagnosis was 72.5 years (range: 55-79), with a median follow-up of 26.5 months (range, 5-74). Most of the PPC developed with carcinomatosis peritonei involving ascites, while some cases developed sporadically in the peritoneal or extraperitoneal cavity without ascites. The most common initial symptom was abdominal fullness, and other symptoms were inguinal tumor, paralysis of the extremities, and respiratory disorder. The preoperative CA125 value was elevated in all patients. In four patients who did not undergo primary surgery, the final diagnoses were determined by the ascites cytology and radiological image. Initial or interval debulking surgery was performed in only two patients. All patients were treated with paclitaxel or docetaxel plus carboplatin. Five showed a complete response (CR), and one showed a partial response (PR). Among the five patients with CR, the median progression-free and overall survival periods were 15 (12-26) and 41.5 (32-74) months, respectively. Three patients without carcinomatosis peritonei showed a relatively favorable prognosis. The management of PPC is generally consistent with that of aEOC; however, in atypical cases, the treatment method should be considered individually.
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Anesthesia is sometimes used for the reduction of maternal pain in normal human term labor, but whether the drugs affect oxidative stress remains unclear. The placenta serves as an interface between the maternal and fetal vasculature. In this study, we immunohistochemically analyzed two markers for oxidative stress, namely 8-hydroxy-2'-deoxyguanosine (8-OHdG) and 4-hydroxy-2-nonenal-modified proteins (HNE), using placentas from 21 cases of normal tansvaginal delivery (V group), 20 Caesarean sections (C group), and 17 normal transvaginal deliveries with epidural anesthesia (E group). 8-OHdG staining in the nuclei of trophoblasts lining the chorionic villi was significantly stronger in the V group either compared with the C or E group (p<0.001), without significant differences in the C and E groups (p = 0.792). Moderate to intense staining by HNE of the intravascular serum of chorionic villi vasculature was frequently observed in the placentas from the V group, but less frequently of those in either C or E groups (p<0.001), nor the p value comparing the C and E groups was significant (p = 0.128) for HNE staining. Our results suggest that although the role of oxidative stress and its influences on fetal state in the placenta in labor remains unclear, it seems to be lessened by epidural anesthesia.
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Amniotic fluid contains numerous biomolecules derived from fetus and mother, thus providing precious information on pregnancy. Here, we evaluated oxidative stress of human amniotic fluid and measured the concentration of catalytic Fe(II). Amniotic fluid samples were collected with consent from a total of 89 subjects in Nagoya University Hospital, under necessary medical interventions: normal pregnancy at term, normal pregnancy at the 2nd trimester, preterm delivery with maternal disorders but without fetal disorders, congenital diaphragmatic hernia, fetal growth restriction, pregnancy-induced hypertension, gestational diabetes mellitus, Down syndrome and trisomy 18. Catalytic Fe(II) and oxidative stress markers (8-hydroxy-2'-deoxyguanosine, 8-OHdG; dityrosine) were determined with RhoNox-1 and specific antibodies, respectively, using plate assays. Levels of 8-OHdG and dityrosine were higher in the 3rd trimester compared with the 2nd trimester in normal subjects, and the abnormal groups generally showed lower levels than the controls, thus suggesting that they represent fetal metabolic activities. In contrast, catalytic Fe(II) was higher in the 2nd trimester than the 3rd trimester in the normal subjects, and overall the abnormal groups showed higher levels than the controls, suggesting that high catalytic Fe(II) at late gestation reflects fetal pathologic alterations. Notably, products of H2O2 and catalytic Fe(II) remained almost constant in amniotic fluid.
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Miller-Dieker syndrome (MDS) is characterized by facial abnormalities and lissencephaly and is caused by a microdeletion in the region containing the LIS1 gene at chromosome 17p13.3. We report a case in which postnatal neuroimaging revealed severe lissencephaly. A 9-month-old boy presented with infantile spasms syndrome. Because of the refractory course of seizures and continued poor vitality, total corpus callosotomy was performed at 28 months of age. Intraoperative electroencephalogram (EEG) showed that the bilateral synchronous epileptiform discharges disappeared immediately after the disconnection. Postoperatively, the epileptic spasms (ES) in clusters disappeared, and single ES followed by focal seizures became the main symptom. The patient smiled more and became more responsive to stimuli. Postoperative scalp interictal EEG showed desynchronized multifocal spike and wave discharges with a marked decrease in the bilateral synchronous spike and wave discharges. Our findings suggest that the corpus callosum is involved in the mechanism ES in clusters in MDS-associated lissencephaly, and total callosotomy could be a therapeutic option.
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INTRODUCTION: Primary diffuse leptomeningeal melanomatosis is an extremely rare variant of primary melanoma of the central nervous system. It is characterized by a variety of nonspecific clinical, radiological, and histopathological features requiring differential diagnosis from a variety of diseases. Here, we aimed to use our own clinical case as an example of the difficulties in the diagnosis of this disease. CASE PRESENTATION: A 14-year-old boy presented with focal to bilateral tonic-clonic seizures. Brain MRI showed diffuse cortical surface and subcortical lesions, isointense on T1-weighted images and hypointense on T2-weighted images, respectively, with diffuse leptomeningeal gadolinium enhancement. Cytology of the cerebrospinal fluid revealed atypical mononuclear cells, but characteristic melanoma cells were not found. Although we suspected meningeal carcinomatosis, we did not perform abrainbiopsy under the tentative diagnosis of Sturge-Weber syndrome. A definitive diagnosis of primary diffuse leptomeningeal melanomatosis was made with abrainbiopsy after hedevelopednon-convulsive status epilepticus. Despite treatment, he died of hydrocephalus 1 year and 8 months after onset. CONCLUSION: Primary diffuse leptomeningeal melanomatosis poses a clinical diagnostic and therapeutic challenge. Leptomeningeal enhancement extending into the cerebral sulci and signal changes in T1/T2 weighted images of cortical and subcortical lesions on MRI are key to an early decision regarding whether to perform a biopsy, even in the pediatric population.
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Melanoma , Neoplasias Meníngeas , Adolescente , Criança , Meios de Contraste , Gadolínio , Humanos , Imageamento por Ressonância Magnética , Masculino , Melanoma/diagnóstico por imagem , Melanoma/patologia , Neoplasias Meníngeas/diagnóstico por imagem , Neoplasias Meníngeas/patologiaRESUMO
BACKGROUND: Rasmussen syndrome (RS) is a rare neurological disorder characterized by unilateral chronic inflammation, drug-resistant epilepsy, and progressive neurological and cognitive deterioration. There has been no detailed pathological evaluation or finding, including focal cortical dysplasia, for bilateral RS. CASE REPORT: A 13-year-old boy presented with status epilepticus with focal to bilateral tonic clonic seizure starting from the left upper limb. At the age of 15, epilepsia partialis continua of the right face and upper extremities appeared, and MRI showed hemispheric abnormal signal intensities with left frontal lobe predominance. Three months later, MRI showed extensive abnormal signal intensities in the right occipitoparietal and left temporal lobes. Tacrolimus was useful in preventing recurrence. Because the seizures were intractable, a corpus callosotomy was performed at 16 years along with a concurrent brain biopsy from the bilateral lateral frontal cortices. We detected dysmorphic neurons in addition to inflammatory changes suspicious for RS, leading to a diagnosis of focal cortical dysplasia (FCD) type â ¡a and suspected bilateral RS. Total callosotomy and vagus nerve stimulation were not sufficiently effective. CONCLUSIONS: In bilateral RS, FCD may be present in both cerebral hemispheres. In the current case, an autoimmune response to dysmorphic neurons may have contributed to the pathogenesis of intense inflammation.
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Encefalite , Epilepsia , Malformações do Desenvolvimento Cortical , Adolescente , Eletroencefalografia , Encefalite/complicações , Epilepsia/complicações , Humanos , Inflamação , Imageamento por Ressonância Magnética/efeitos adversos , Masculino , Malformações do Desenvolvimento Cortical/complicações , Malformações do Desenvolvimento Cortical/diagnóstico por imagem , Malformações do Desenvolvimento Cortical/cirurgia , Malformações do Desenvolvimento Cortical do Grupo I , Convulsões/etiologiaRESUMO
OBJECTIVE: The aims of this study were to examine the preparedness of vulnerable people ages 75 years and older and to clarify the characteristics of older adults that are associated with disaster preparedness. METHODS: We conducted interviews with persons requiring special care ages 75 years and older living in coastal communities of western Japan, where earthquakes and tsunami disasters are a concern. The survey included participant characteristics such as demographic indicators, physical function, health status, community involvement, and disaster preparedness. Binomial logistic regression analysis was performed with participant characteristics as independent variables and disaster preparedness as the dependent variables. RESULTS: The characteristics related to disaster preparedness were age, family composition, cognitive function, level of interaction with neighbors, and participation in community activities. CONCLUSIONS: Being female, living alone, and having cognitive impairment were factors that led to decreased disaster preparedness. However, it was suggested that close human interactions in the community facilitated preparedness. Community engagement is crucial in reducing disaster damage and recovering effectively. In order to facilitate preparedness measures for persons requiring special care ages 75 and older, it is important to establish community preparedness.
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Planejamento em Desastres , Características de Residência , Idoso , Estudos Transversais , Terremotos , Feminino , Humanos , Japão , Masculino , Características de Residência/estatística & dados numéricos , Medição de Risco , TsunamisRESUMO
OBJECTIVE: The aim of this study was to determine the prognostic factors for acute encephalopathy with reduced diffusion (AED) during the acute phase through retrospective case evaluation. METHODS: The participants included 23 patients with AED. The diagnosis of AED was based on their clinical course and radiological findings. We divided the patients into severe and non-severe groups based on the neurodevelopmental outcome. The severe group included seven patients (median age, 21â¯months; range, 6-87â¯months) and the non-severe group included 16 patients (19â¯months, 9-58â¯months). Clinical symptoms, laboratory data and electroencephalogram (EEG) findings within 48â¯h from the initial seizure onset were compared between the two groups to identify neurological outcome predictors. RESULTS: The incidence of coma 12-24â¯h after onset, serum creatinine (Cr) levels within 2â¯h after onset, maximum aspartate aminotransferase (AST) levels within 24â¯h after onset, and the rate of electrographic seizures in EEG were significantly higher in the severe group (Coma, 80%; Cr, 0.40â¯mg/dl, 0.37-0.73; AST, 363 IU/L, 104-662; electrographic seizures, 80%) than the non-severe group (Coma, 0%; Cr, 0.29â¯mg/dL, 0.19-0.45; AST, 58.5 IU/L, 30-386; electrographic seizures, 0%). CONCLUSIONS: Coma 12-24â¯h after onset, elevation of Cr levels within 2â¯h after onset, elevation of AST levels within 24â¯h after onset, and non-convulsive status epileptics (NCSE) in comatose patients were early predictors of severe AED. Patients in a coma after a febrile seizure should be checked for NCSE signs in EEG to terminate NCSE without delay.
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Encefalopatias/diagnóstico , Biomarcadores/sangue , Encéfalo/fisiopatologia , Encefalopatias/epidemiologia , Criança , Pré-Escolar , Creatinina/sangue , Eletroencefalografia , Humanos , Incidência , Lactente , Prognóstico , Índice de Gravidade de Doença , Fatores de TempoRESUMO
An 11-year-old boy presented with progressive leg hypesthesia but no history of trauma. Dysuria and constipation appeared subsequent to gait difficulty. He was admitted 8days after onset. Spinal magnetic resonance imaging (MRI) revealed longitudinal hyperintensity with cord swelling and hypointensity on T2-weighted images, suggesting severe inflammation and microbleeding change, respectively. Gadolinium contrast-enhanced MRI demonstrated mild enhancement in the lesions. Platelet count and coagulation findings were normal, and cerebrospinal fluid analysis showed no pleocytosis. He was diagnosed with idiopathic acute transverse myelitis (ATM), and intravenous methylprednisolone pulse therapy and plasmapheresis were initiated. On day 14, motor dysfunction aggravated suddenly, accompanied by expanding hemorrhagic lesions. Thereafter, administration of intravenous immunoglobulin, repeated intravenous methylprednisolone pulse therapy and prednisolone for one month resulted in complete recovery four months later. Both anti-aquaporin-4 and anti-myelin oligodendrocyte glycoprotein antibodies were negative. We presented the first pediatric case showing hemorrhagic spinal lesions in the clinical course of ATM. This severe complication should be recognized in the management of ATM.
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Hemorragia/etiologia , Imunoglobulinas Intravenosas/uso terapêutico , Metilprednisolona/uso terapêutico , Mielite Transversa/tratamento farmacológico , Mielite Transversa/patologia , Prednisolona/uso terapêutico , Medula Espinal/patologia , Aquaporina 4/imunologia , Criança , Humanos , Imageamento por Ressonância Magnética/métodos , Masculino , Metilprednisolona/administração & dosagem , Mielite Transversa/complicações , Mielite Transversa/diagnóstico , Prednisolona/administração & dosagem , Resultado do TratamentoRESUMO
We diagnosed a 3-year-old girl with acute transverse myelitis (ATM). She presented with weakness of the limbs and developed urination difficulty and respiratory disturbance. Magnetic resonance imaging revealed a symmetric area of high signal intensity on T2-weighted images involving the lower end of the medulla oblongata to the level of the fourth thoracic vertebra. Anti-aquaporin-4 antibody was negative. She was treated with intravenous methylprednisolone pulse therapy, immunoglobulin therapy, and plasmapheresis; however, her clinical symptoms did not change. At 10 and 20days after symptom onset, cardiac arrest occurred on postural change, requiring cardiopulmonary resuscitation. A permanent pacemaker was implanted 23days after onset. In the presence of sympathetic nerve hypofunction, relative hyperactivity of the parasympathetic nerves may have led to severe bradycardia and cardiac arrest in the presence of an inducer, such as a postural change. This is the first reported case of pacemaker implantation for management of ATM.
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Mielite Transversa/terapia , Marca-Passo Artificial , Pré-Escolar , Feminino , Humanos , Processamento de Imagem Assistida por Computador , Imageamento por Ressonância Magnética , Mielite Transversa/diagnóstico por imagem , Mielite Transversa/fisiopatologiaRESUMO
The KCNT1 gene encodes the sodium-dependent potassium channel, with quinidine being a partial antagonist of the KCNT1 channel. Gain-of-function KCNT1 mutations cause early onset epileptic encephalopathies including migrating partial seizures of infancy (MPSI). At 5months of age, our patient presented with epileptic spasms and hypsarrhythmia by electroencephalogram. Psychomotor retardation was observed from early infancy. The patient was diagnosed with West syndrome. Consequently, various anti-epileptic drugs, adrenocorticotropic hormone therapy (twice), and ketogenic diet therapy were tried. However, the epileptic spasms were intractable. Whole exome sequencing identified a KCNT1 mutation (c.1955G>T; p.G652V). At 2years and 6months, the patient had daily epileptic spasms despite valproate and lamotrigine treatment, and was therefore admitted for quinidine therapy. With quinidine therapy, decreased epileptic spasms and decreased epileptiform paroxysmal activity were observed by interictal EEG. Regarding development, babbling, responsiveness, oral feeding and muscle tone were ameliorated. Only transient diarrhea was observed as an adverse effect. Thus, quinidine therapy should be attempted in patients with West syndrome caused by KCNT1 mutations, as reported for MPSI.
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Anticonvulsivantes/uso terapêutico , Mutação , Proteínas do Tecido Nervoso/genética , Canais de Potássio/genética , Quinidina/uso terapêutico , Espasmos Infantis/tratamento farmacológico , Espasmos Infantis/genética , Encéfalo/efeitos dos fármacos , Encéfalo/fisiopatologia , Pré-Escolar , Análise Mutacional de DNA , Eletroencefalografia , Humanos , Lactente , Masculino , Canais de Potássio Ativados por Sódio , Espasmos Infantis/fisiopatologiaRESUMO
Autoimmune autonomic ganglionopathy (AAG) is an acquired immune-mediated disorder that leads to systemic autonomic failure. Autoantibodies to the ganglionic nicotinic acetylcholine receptor (gAChR) are detected in 50% of AAG patients. We report the first pediatric case of AAG presenting with acute encephalitis. The patient was a 13-year-old boy who presented with orthostatic hypotension, followed by rapidly progressing disturbance of consciousness. Cerebrospinal fluid analysis revealed significant pleocytosis and increased neopterin concentration. Head MRI showed hyperintensities in bilateral caudate nuclei, putamen, hippocampus, and insula cortex. Severe autonomic dysfunctions such as severe orthostatic hypotension, bradycardia, dysuria, prolonged constipation and vomiting appeared. These symptoms were successfully controlled by repeated immunomodulating therapy with intravenous methylprednisolone pulse therapy and intravenous immunoglobulin. Autoantibodies to the α3 subunit of gAChR were detected at neurological onset, but were undetectable five months later. This observation indicates that AAG should be suspected in patients manifesting acute encephalitis characterized by preceding and prolonged autonomic symptoms, and immunomodulating therapy from an early stage can be effective.
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Doenças Autoimunes do Sistema Nervoso/complicações , Doenças Autoimunes do Sistema Nervoso/diagnóstico , Encefalite/complicações , Encefalite/diagnóstico , Doença Aguda , Adolescente , Autoanticorpos/metabolismo , Doenças Autoimunes do Sistema Nervoso/terapia , Encéfalo/diagnóstico por imagem , Encefalite/terapia , Humanos , Imunomodulação , Imageamento por Ressonância Magnética , Masculino , Receptores Colinérgicos/imunologiaRESUMO
BACKGROUND: Clinical phenotypic expression of SSADH deficiency is highly heterogeneous, and some infants may develop refractory secondary generalized seizures. PATIENT: A 9-month-old boy manifested partial seizures, developing severe status epilepticus, and conventional antiepileptic drugs were ineffective. Use of ketamine contributed to the control of status epilepticus, achieving a reduction in frequency of partial seizures, and improving EEG findings without apparent complications. Diffusion-weighted images showed hyperintensities in the bilateral basal ganglia and fornix, and multiple T2 hyperintensity lesions were detected. (123)I-iomazenil (IMZ) SPECT revealed a decrease in binding of (123)I-iomazenil predominantly in the left temporal region by the 18th day of hospitalization. However, repeated IMZ-SPECT on the 46th day of hospitalization demonstrated almost no accumulation across a broad region, sparing the left temporal region. The patient showed rapid regression, refractory myoclonus, and severe progressive brain atrophy. CONCLUSION: IMZ-SPECT findings demonstrated reduced benzodiazepine receptor binding and its dynamic changes in an SSADH-deficient patient. Considering the down regulation of the GABAA receptor, ketamine should be included in pharmacotherapeutic strategies for treatment of refractory status epilepticus in SSADH-deficient patients.
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Erros Inatos do Metabolismo dos Aminoácidos/diagnóstico por imagem , Erros Inatos do Metabolismo dos Aminoácidos/fisiopatologia , Deficiências do Desenvolvimento/diagnóstico por imagem , Deficiências do Desenvolvimento/fisiopatologia , Estado Epiléptico/diagnóstico por imagem , Estado Epiléptico/fisiopatologia , Succinato-Semialdeído Desidrogenase/deficiência , Erros Inatos do Metabolismo dos Aminoácidos/terapia , Atrofia , Encéfalo/diagnóstico por imagem , Encéfalo/efeitos dos fármacos , Encéfalo/fisiopatologia , Deficiências do Desenvolvimento/terapia , Imagem de Difusão por Ressonância Magnética , Progressão da Doença , Eletroencefalografia , Flumazenil/análogos & derivados , Humanos , Lactente , Masculino , Compostos Radiofarmacêuticos , Estado Epiléptico/terapia , Tomografia Computadorizada de Emissão de Fóton ÚnicoRESUMO
BACKGROUND: A high serum estradiol (E2) level is occasionally detected in postmenopausal women with common epithelial ovarian tumors with functioning stroma. It has been proven that functioning stroma has the capacity to convert androgens to estrogens. However, the mechanism of the initiation and development of functioning stroma remains unclear. CASE PRESENTATION: We present two cases of elevated E2 levels in elderly women with ovarian mucinous adenocarcinomas that contained functioning stroma. Immunohistochemical evaluation revealed high expression levels of aromatase and steroidogenic factor-1 (SF-1), which is considered to be a master regulator of steroidogenesis, in their ovarian stroma. CONCLUSIONS: These cases suggest that overexpression of SF-1 may promote estrogen biosynthesis through regulation of P450 aromatase expression in ovarian tumors with functioning stroma; this in turn induces high serum E2 levels in postmenopausal women with common epithelial ovarian tumors.
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Adenocarcinoma Mucinoso/metabolismo , Aromatase/metabolismo , Proteínas de Ligação a DNA/metabolismo , Neoplasias Epiteliais e Glandulares/metabolismo , Neoplasias Ovarianas/metabolismo , Fatores de Transcrição/metabolismo , Idoso de 80 Anos ou mais , Carcinoma Epitelial do Ovário , Dinoprostona/metabolismo , Estrogênios/biossíntese , Feminino , Humanos , Pessoa de Meia-Idade , Pós-Menopausa/metabolismo , Fatores de Processamento de RNA , Células Estromais/metabolismoRESUMO
Ovarian endometriosis is a recognized risk for infertility and epithelial ovarian cancer, presumably due to iron overload resulting from repeated hemorrhage. To find a clue for early detection and prevention of ovarian endometriosis-associated cancer, it is mandatory to evaluate catalytic (labile) ferrous iron (catalytic Fe(II)) and to study iron manipulation in ovarian endometriotic lesions. By the use of tissues from women of ovarian endometriosis as well as endometrial tissue from women with and without endometriosis, we for the first time performed histological analysis and cellular detection of catalytic Fe(II) with a specific fluorescent probe (HMRhoNox-M), and further evaluated iron transport proteins in the human specimens and in co-culture experiments using immortalized human eutopic/ectopic endometrial stromal cells (ESCs) in the presence or absence of epithelial cells (EpCs). The amounts of catalytic Fe(II) were higher in ectopic endometrial stromal cells (ecESCs) than in normal eutopic endometrial stromal cells (n-euESCs) both in the tissues and in the corresponding immortalized ESCs. ecESCs exhibited higher transferrin receptor 1 expression both in vivo and in vitro and lower ferroportin expression in vivo than n-euESCs, leading to sustained iron uptake. In co-culture experiments of ESCs with iron-loaded EpCs, ecESCs received catalytic ferrous iron from EpCs, but n-euESCs did not. These data suggest that ecESC play a protective role for cancer-target epithelial cells by collecting excess iron, and that these characteristics are retained in the immortalized ecESCs.