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1.
J Hum Genet ; 67(11): 675-678, 2022 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-35970985

RESUMO

Pierre-Robin sequence (PRS) is a rare, congenital defect presenting with micrognathia, glossoptosis, and airway obstruction with variable inclusion of a cleft palate. Overlapping PRS with neurofibromatosis type 2 (NF2) is a syndrome caused by a chromosome 22q12 microdeletion including NF2. We describe a patient with severe early-onset NF2 overlapping with PRS that showed micrognathia, glossoptosis, and a mild form of cleft palate. We detected a de novo chromosome 22q12 microdeletion including MN1 and NF2 in the patient. Previous cases of overlapping PRS and NF2 caused by the chromosome 22q12 microdeletions showed severe NF2 phenotypes with variable severity of cleft palate and microdeletions of varying sizes. Genotype-phenotype correlations and comparison of the size and breakpoint of microdeletions suggest that some modifier genes distal to MN1 and NF2 might be linked to the cleft palate severity.


Assuntos
Fissura Palatina , Glossoptose , Micrognatismo , Neurofibromatose 2 , Síndrome de Pierre Robin , Humanos , Síndrome de Pierre Robin/genética , Neurofibromatose 2/genética , Fissura Palatina/genética , Micrognatismo/genética , Cromossomos , Transativadores/genética , Proteínas Supressoras de Tumor/genética
2.
Otol Neurotol ; 43(7): e712-e719, 2022 08 01.
Artigo em Inglês | MEDLINE | ID: mdl-35802892

RESUMO

OBJECTIVES: No clinically useful prognostic factors have been identified for idiopathic sudden sensorineural hearing loss (ISSNHL). The current study therefore sought to identify useful prognostic factors for idiopathic sudden sensorineural hearing loss from blood biomarkers while attempting to classify the pathogenic mechanism and formulate treatment strategies based on these results. STUDY DESIGN: Prospective cohort study. SETTING: Tertiary referral center. METHODS: A total of 47 patients with acute phase ISSNHL were treated with steroid at an initial dose of 1 mg/kg/day and hyperbaric oxygen therapy and followed up for 6 months. Serum fibrinogen levels, peripheral blood mononu- clear cells (PBMCs), and interleukin (IL)-1ß, IL-6, and tumor necrosis factor (TNF)-α production levels from PBMCs were measured, after which patient's pre- and post- treatment hearing was compared. RESULTS: In the overall cohort, the mean improvement level, mean recovery rate, and mean fibrinogen level was 20.3 dB, 46.2%, 292.0 mg/mL, respectively. The mean levels of IL-1ß, IL-6, and TNF-α produced by peripheral blood mononu- clear cells cultured under lipopolysaccharide stimulation were 318.4, 498.1, and 857.6 pg/mL, respectively. High fibrinogen levels were associated with poor hearing progno- sis. Lipopolysaccharide-stimulated cytokine production by PBMCs did not correlate with hearing changes; however, the prognosis was significantly better in patients with low fibrinogen levels and high IL-1ß levels produced by PBMCs than in other patients. CONCLUSIONS: Our results suggest that patients with simple inflammatory-type ISSNHL responded well to standard therapy. Therefore, serum fibrinogen levels and PBMCs cytokine production may help determine the management of ISSNHL based on its pathogenic mechanism.


Assuntos
Perda Auditiva Neurossensorial , Perda Auditiva Súbita , Biomarcadores , Fibrinogênio , Glucocorticoides , Perda Auditiva Neurossensorial/tratamento farmacológico , Perda Auditiva Súbita/tratamento farmacológico , Humanos , Interleucina-6/uso terapêutico , Lipopolissacarídeos , Prognóstico , Estudos Prospectivos , Estudos Retrospectivos , Resultado do Tratamento
3.
Cureus ; 14(5): e25192, 2022 May.
Artigo em Inglês | MEDLINE | ID: mdl-35747011

RESUMO

Signet-ring cell/histiocytoid carcinoma (SRCHC) is a rare, aggressive neoplasm that often originates in the eyelid. We present a rare case of a 64-year-old male with SRCHC and papillary thyroid carcinoma (PTC) that underwent exome panel sequencing with next-generation sequencing (NGS). In addition, we reviewed reports of genetic mutations in SRCHC and compared them with our results. The imaging findings allowed us to recognize the differences in pathology between the left and right cervical nodes. For first-line treatment, an extended total maxillectomy with orbital exenteration and dissection of the left neck was performed. Two months later, total thyroidectomy and right neck dissection were performed. Two years after surgery, multiple bone metastases occurred. An exome panel sequence with NGS was used to determine the chemotherapy regimen. Notably, somatic mutations in cadherin 1 (CDH1), human epidermal growth factor receptor 2 (ERBB2), neurofibromin 1 (NF1), and tumor protein p53 (TP53) were detected. These mutations are rarely detected in PTC; therefore, cervical metastases are assumed to originate from SRCHC. To our knowledge, there have been no reports of simultaneous cancer of SRCHC and PTC. Somatic mutations in CDH1, ERBB2, NF1, and TP53 were detected in the exome panel sequence of the metastatic lymph nodes of SRCHC and correlated with previous reports of SRCHC.

4.
Pol J Radiol ; 85: e1-e7, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-32180847

RESUMO

PURPOSE: To evaluate the significance of additional coronal reconstruction images in the diagnostic ability of contrast-enhanced computed tomography (CECT) for metastatic cervical nodes in patients with head and neck squamous cell carcinomas (HNSCC). MATERIAL AND METHODS: We retrospectively assessed 97 metastatic and 141 reactive histologically proven cervical nodes of 38 patients with HNSCC, who underwent CECT before neck dissection. Observer #1, an expert radiologist in head and neck imaging, and observer #2, a general radiologist, reviewed all CECT images. The observers first assessed the presence of nodal metastasis using axial CECT alone (A-CECT). Three days later, they reassessed its presence using combined axial and coronal CECT (A&C-CECT). RESULTS: The sensitivity of A-CECT vs. A&C-CECT was 73.2% vs. 75.3% for observer #1 (p = 0.73) and 69.1% vs. 69.1% for observer #2 (p = 1.00), respectively. The specificity of A-CECT versus A&C-CECT was 92.2% vs. 97.2% for observer #1 (p < 0.05) and 92.9% vs. 95.7% for observer #2 (p = 0.22), respectively. The accuracy of A-CECT versus A&C-CECT was 84.5% vs. 88.2% for observer #1 (p < 0.05) and 83.2% vs. 85.3% for observer #2 (p = 0.30), respectively. The area under the curve (AUC) of A-CECT vs. A&C-CECT was 0.86 vs. 0.91 for observer #1 (p < 0.05) and 0.85 vs. 0.85 for observer #2 (p = 0.80), respectively. CONCLUSIONS: The specificity, accuracy, and AUC increased with the use of coronal images during the assessment by the expert radiologist. The appropriate use of coronal images allowed proper configuration recognition and improved diagnostic ability.

5.
Auris Nasus Larynx ; 47(4): 658-667, 2020 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-32089351

RESUMO

OBJECTIVE: Cancer cells secrete large amounts of lactic acid via aerobic glycolysis. We have shown that lactic acid plays an important role as a proinflammatory and immunosuppressive mediator and promotes tumor progression. Fluorine-18 fluorodeoxyglucose (FDG) uptake detected by positron emission tomography/computed tomography (PET/CT) is considered as a good indicator of aerobic glycolysis in cancer. In this study, we examined the relationships between systemic inflammatory parameters and FDG-PET/CT parameters in advanced head and neck squamous cell carcinoma (HNSCC). Furthermore, we investigated the relationships between FDG-PET/CT parameters and M2-macrophage polarization in HNSCC by assessing the ratio of CD163, a M2-macrophage marker, to CD68, a pan-macrophage marker. METHODS: This study included 73 advanced HNSCC patients. We assessed the C-reactive protein (CRP) level, white blood cell (WBC) count, neutrophil count, lymphocyte count, and monocyte count as systemic inflammatory markers. Additionally, we assessed the maximum standardized uptake value (SUVmax), mean SUV (SUVmean), metabolic tumor volume (MTV), and total lesion glycolysis (TLG) as FDG-PET/CT parameters. RESULTS: The CRP level, WBC count, and neutrophil count were correlated with whole-body FDG-PET/CT parameters. The CD163/CD68 ratio was correlated with SUVmax and SUVmean. Our results suggest that systemic inflammation, which is associated with neutrophils, develops in patients with HNSCC having tumors with a larger volume and increased glucose uptake and that M2-macrophage polarization is promoted in HNSCC with increased glucose uptake, SUVmax, and SUVmean. FDG-PET/CT has the potential to reflect cancer-related chronic inflammation and immunosuppressive conditions in cancer patients. CONCLUSIONS: FDG-PET/CT parameters appear to be useful in assessing the immune status in HNSCC.


Assuntos
Proteína C-Reativa/metabolismo , Neoplasias de Cabeça e Pescoço/diagnóstico por imagem , Inflamação/sangue , Macrófagos/metabolismo , Carcinoma de Células Escamosas de Cabeça e Pescoço/diagnóstico por imagem , Idoso , Idoso de 80 Anos ou mais , Antígenos CD/metabolismo , Antígenos de Diferenciação Mielomonocítica/metabolismo , Feminino , Fluordesoxiglucose F18 , Glicólise , Neoplasias de Cabeça e Pescoço/sangue , Neoplasias de Cabeça e Pescoço/metabolismo , Neoplasias de Cabeça e Pescoço/patologia , Humanos , Inflamação/metabolismo , Ácido Láctico/metabolismo , Contagem de Leucócitos , Contagem de Linfócitos , Masculino , Pessoa de Meia-Idade , Monócitos , Neutrófilos , Tomografia por Emissão de Pósitrons combinada à Tomografia Computadorizada , Compostos Radiofarmacêuticos , Receptores de Superfície Celular/metabolismo , Carcinoma de Células Escamosas de Cabeça e Pescoço/sangue , Carcinoma de Células Escamosas de Cabeça e Pescoço/metabolismo , Carcinoma de Células Escamosas de Cabeça e Pescoço/patologia
6.
J Int Adv Otol ; 15(3): 454-458, 2019 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-31846928

RESUMO

Muckle-Wells syndrome (MWS), a subclass of cryopyrin-associated periodic syndrome (CAPS), sometimes includes complications of bilateral progressive sensorineural hearing loss. A 48-year-old woman had been diagnosed with pediatric rheumatic arthritis at aged 6 years; however, systematic therapy with prednisolone and methotrexate showed limited efficacy for her general fatigue and arthritic pain, and it never improved the hearing level. She underwent a cochlear implant surgery for progressive profound bilateral hearing loss. After 7 years of cochlear implant surgery, she was diagnosed with MWS by genetic tests. Interleukin (IL)-1ß monoclonal antibody therapy (canakinumab) improved general fatigue and arthritic pain but showed no effect on cochlear symptoms. Owing to successful cochlear implant surgery, she reacquired the hearing and communication function while being able to understand over 90% of monosyllables and words in the sound field of her daily life at 65 dB SPL for the next 13 years of her life. This suggests that peripheral cochlear damage induced by chronic inflammation contributes to the sensorineural hearing loss in cases with MWS, and that cochlear implantation can provide long-term hearing efficacy for patients with MWS with irreversible profound hearing loss.


Assuntos
Implante Coclear , Síndromes Periódicas Associadas à Criopirina/complicações , Perda Auditiva Neurossensorial/cirurgia , Cóclea/fisiopatologia , Feminino , Audição , Perda Auditiva Neurossensorial/congênito , Perda Auditiva Neurossensorial/fisiopatologia , Humanos , Pessoa de Meia-Idade , Período Pós-Operatório , Fatores de Tempo , Resultado do Tratamento
7.
Jpn J Radiol ; 37(9): 627-635, 2019 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-31352657

RESUMO

A variety of neoplastic and non-neoplastic lesions of the parotid gland can present with a predominantly cystic architecture, and although radiologists frequently encounter cystic parotid tumors, other non-neoplastic lesions should also be included in the differential diagnoses of cystic parotid lesions. Non-neoplastic cystic lesions are usually classified as either congenital/acquired cystic lesions or inflammatory/infectious lesions. Adequate knowledge about these rare conditions is essential for appropriate diagnosis and optimal treatment strategy. This review article describes CT and MR imaging features of non-neoplastic cystic lesions of the parotid gland and provides helpful suggestions on the differential diagnoses for cystic parotid lesions.


Assuntos
Imageamento por Ressonância Magnética/métodos , Doenças Parotídeas/diagnóstico por imagem , Tomografia Computadorizada por Raios X/métodos , Cistos/complicações , Cistos/diagnóstico por imagem , Diagnóstico Diferencial , Feminino , Humanos , Masculino , Doenças Parotídeas/complicações , Glândula Parótida/diagnóstico por imagem
8.
Br J Radiol ; 92(1100): 20190054, 2019 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-31116575

RESUMO

OBJECTIVE: This study aimed to assess the imaging features and natural course of clavicle fracture following neck dissection (ND). METHODS AND MATERIALS: Eight patients with clavicle fracture following ND were included. Because bilateral clavicle fractures occurred in one patient, a total of nine clavicle fractures following ND were assessed using CT images. Pathological fracture due to bone metastasis or local recurrence was carefully ruled out. RESULTS: The time interval from ND to clavicle fracture ranged from 2 to 8 months (median, 4 months). On CT images, all the nine fractures occurred in the proximal clavicles and displayed increased bone marrow density with extraosseous soft-tissue mass formation. Osteolysis of the clavicle was not observed. Intra articular fractures were observed in three (33%), displaced fractures in two (22%), and free bone fragments in three (33%) cases. Further follow-up using CT showed that six (67%) fractures resulted in pseudoarthrosis. For the remaining three (33%) fractures with bone union during follow-up, the time interval between clavicle fracture and bone union ranged from 4 to 16 months (median, 6 months). CONCLUSION: Proximal clavicle fractures, which are rarely observed following ND, always display extraosseous soft-tissue mass formation without osteolysis. They frequently result in pseudoarthrosis; however, occasionally, they also result in bone union within 24 weeks. ADVANCES IN KNOWLEDGE: The location of proximal clavicle, the lack of osteolysis, and the presence of free bone fragments may be the key to diagnosis of clavicle fracture following ND.


Assuntos
Clavícula/diagnóstico por imagem , Clavícula/lesões , Fraturas Ósseas/diagnóstico por imagem , Esvaziamento Cervical , Complicações Pós-Operatórias/diagnóstico por imagem , Idoso , Idoso de 80 Anos ou mais , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Tomografia Computadorizada por Raios X , Resultado do Tratamento
9.
Radiol Med ; 124(3): 199-205, 2019 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-30421386

RESUMO

PURPOSE: The present study aimed to assess CT and MR imaging findings of infection-free and benign second branchial cleft cysts (SBCCs). METHODS: Eleven patients with histopathologically confirmed infection-free and benign SBCCs underwent preoperative contrast-enhanced CT (CECT) and/or MR imaging. We assessed qualitative (presence and extent of wall thickening, degree of contrast enhancement of the thickened wall on CECT images, and signal intensity of the thickened wall on T2-weighted images) and quantitative (maximum thickness of the thickened wall) imaging findings. RESULTS: Eccentric and smooth wall thickening was observed in 11/11 (100%) patients. The wall thickening extent (percentage of the thickened wall to the circumference of the wall) was small (1%-25%) in 4/11 (36%), moderate (26%-50%) in 6/11 (54%), extensive (51%-75%) in 1/11 (9%), and diffuse (76%-100%) in 0/11 (0%) patients. Mild homogeneous enhancement of the thickened wall on CECT images was observed in 7/7 (100%) patients. The signal intensity of the thickened wall on T2-weighted images was isointense relative to that of normal lymph nodes in 7/8 (88%) and mildly hyperintense in 1/8 (12%) patient. The maximum thickness of the thickened walls ranged from 2 to 4 (mean 3.4) mm. CONCLUSIONS: Infection-free and benign SBCCs are identifiable as cysts with eccentric and smooth wall thickening on CECT and MR images. The wall thickness was almost always less than half of the wall circumference, isointense relative to normal lymph nodes, and showed mild homogeneous enhancement.


Assuntos
Branquioma/diagnóstico por imagem , Imageamento por Ressonância Magnética , Tomografia Computadorizada por Raios X , Adolescente , Adulto , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Adulto Jovem
10.
Nat Commun ; 9(1): 2081, 2018 05 25.
Artigo em Inglês | MEDLINE | ID: mdl-29802314

RESUMO

The faithful shutdown of the somatic program occurs in the early stage of reprogramming. Here, we examined the effect of in vivo reprogramming on Kras-induced cancer development. We show that the transient expression of reprogramming factors (1-3 days) in pancreatic acinar cells results in the transient repression of acinar cell enhancers, which are similarly observed in pancreatitis. We next demonstrate that Kras and p53 mutations are insufficient to induce ERK signaling in the pancreas. Notably, the transient expression of reprogramming factors in Kras mutant mice is sufficient to induce the robust and persistent activation of ERK signaling in acinar cells and rapid formation of pancreatic ductal adenocarcinoma. In contrast, the forced expression of acinar cell-related transcription factors inhibits the pancreatitis-induced activation of ERK signaling and development of precancerous lesions in Kras-mutated acinar cells. These results underscore a crucial role of dedifferentiation-associated epigenetic regulations in the initiation of pancreatic cancers.


Assuntos
Carcinoma Ductal Pancreático/genética , Transformação Celular Neoplásica/patologia , Regulação Neoplásica da Expressão Gênica , Neoplasias Pancreáticas/genética , Proteínas Proto-Oncogênicas p21(ras)/metabolismo , Células Acinares/metabolismo , Células Acinares/patologia , Animais , Carcinoma Ductal Pancreático/patologia , Transformação Celular Neoplásica/genética , Reprogramação Celular/genética , Epigênese Genética , Feminino , Humanos , Sistema de Sinalização das MAP Quinases/genética , Masculino , Camundongos , Camundongos Endogâmicos C57BL , Camundongos Endogâmicos ICR , Camundongos Transgênicos , Células-Tronco Embrionárias Murinas , Mutação , Pâncreas/citologia , Pâncreas/patologia , Neoplasias Pancreáticas/patologia , Proteínas Proto-Oncogênicas p21(ras)/genética , Estômago/patologia , Neoplasias Gástricas/genética , Neoplasias Gástricas/patologia , Fatores de Transcrição/genética , Fatores de Transcrição/metabolismo , alfa-Fetoproteínas/metabolismo
11.
Cancer Sci ; 108(6): 1128-1134, 2017 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-28370718

RESUMO

Reprogramming of glucose metabolism in tumor cells is referred to as the Warburg effect and results in increased lactic acid secretion into the tumor microenvironment. We have previously shown that lactic acid has important roles as a pro-inflammatory and immunosuppressive mediator and promotes tumor progression. In this study, we examined the relationship between the lactic acid concentration and expression of LDHA and GLUT1, which are related to the Warburg effect, in human head and neck squamous cell carcinoma (HNSCC). Tumors expressing lower levels of LDHA and GLUT1 had a higher concentration of lactic acid than those with higher LDHA and GLUT1 expression. Lactic acid also suppressed the expression of LDHA and GLUT1 in vitro. We previously reported that lactic acid enhances expression of an M2 macrophage marker, ARG1, in murine macrophages. Therefore, we investigated the relationship between the lactic acid concentration and polarization of M2 macrophages in HNSCC by measuring the expression of M2 macrophage markers, CSF1R and CD163, normalized using a pan-macrophage marker, CD68. Tumors with lower levels of CD68 showed a higher concentration of lactic acid, whereas those with higher levels of CSF1R showed a significantly higher concentration of lactic acid. A similar tendency was observed for CD163. These results suggest that tumor-secreted lactic acid is linked to the reduction of macrophages in tumors and promotes induction of M2-like macrophage polarization in human HNSCC.


Assuntos
Neoplasias de Cabeça e Pescoço/metabolismo , Neoplasias de Cabeça e Pescoço/patologia , Ácido Láctico/metabolismo , Macrófagos/patologia , Antígenos CD/metabolismo , Antígenos de Diferenciação Mielomonocítica/metabolismo , Biomarcadores Tumorais/metabolismo , Carcinoma de Células Escamosas/metabolismo , Carcinoma de Células Escamosas/patologia , Linhagem Celular Tumoral , Humanos , Macrófagos/metabolismo , Receptores de Superfície Celular/metabolismo , Receptores de Fator Estimulador das Colônias de Granulócitos e Macrófagos/metabolismo , Carcinoma de Células Escamosas de Cabeça e Pescoço
12.
Nihon Jibiinkoka Gakkai Kaiho ; 120(3): 202-8, 2017 03.
Artigo em Japonês | MEDLINE | ID: mdl-30010300

RESUMO

Small cell neuroendocrine carcinoma of the head and neck is a rarely occurring poorly differentiated and high-grade malignant neoplasm characterized by highly active proliferation of neuroendocrine tumor cells. There are no established therapies for this disease. To clarify the clinical course and develop effective treatment(s) for the carcinoma, we reviewed the data of 8 patients of small cell neuroendocrine carcinoma of the head and neck treated by us between 2006 and 2014 at the Department of Otolaryngology, Gifu University School of Medicine and our affiliated hospitals. The patients consisted of 3 men and 5 women, ranging in age from 38 to 84 years old (mean : 60.9 years). The tumor arose from the nasal cavity or the paranasal sinuses in 3 cases, from the parotid grand in 2 cases, from the oropharynx in 2 cases, and from the hypopharynx in 1 case. The tumor that arose from the hypopharynx was a combined small-cell carcinoma with squamous cell carcinomas, and the one that arose from the oropharynx had already metastasized to the brain. Most of the patients were treated by chemotherapy and radiotherapy based on the treatment employed for small cell carcinoma of the lung. Only the patient in whom the tumor arose from a paranasal sinus was treated by surgery despite the definitive diagnosis of small cell carcinoma. We selected CPT-11 and a platinum agent for 4 patients, and VP-16 and a platinum agent for 3 patients as the first-line chemotherapy. Although two patients showed carcinoma-free survival, one died of recurrence of the regional lymph node metastases and five died of distant metastases despite the absence of locoregional recurrence. The 5-year survival rate was a dismal 25%, suggesting that we need to establish effective treatment(s) for the control of distant metastases in cases of the small cell neuroendocrine carcinoma of the head and neck.


Assuntos
Carcinoma Neuroendócrino/terapia , Carcinoma de Células Pequenas/terapia , Neoplasias de Cabeça e Pescoço/terapia , Adulto , Idoso , Idoso de 80 Anos ou mais , Biópsia , Feminino , Neoplasias de Cabeça e Pescoço/patologia , Humanos , Masculino , Pessoa de Meia-Idade
13.
Head Neck ; 38(9): 1387-92, 2016 09.
Artigo em Inglês | MEDLINE | ID: mdl-27002591

RESUMO

BACKGROUND: The purpose of the present study was to determine whether polaprezinc suspension in sodium alginate (P-AG) reduces the irradiation period and time to discharge after completion of radiotherapy in patients with head and neck cancer. METHODS: The incidence and severity of oral mucositis, the irradiation period, and the time to discharge in patients who received radiotherapy with head and neck cancer were investigated retrospectively from the medical records. RESULTS: The incidence of grade 3 oral mucositis was significantly lower in the P-AG group than in the control group (16.5% vs 52.0%; p = .0003). P-AG also significantly reduced median duration of radiotherapy (hazard ratio [HR] = 0.557; 95% confidence interval [CI] = 0.357-0.871; p = .0149) and median time to discharge after completion of radiotherapy (HR = 0.604; 95% CI = 0.386-0.946; p = .028). CONCLUSION: P-AG reduced the irradiation period and the time to discharge after completion of radiotherapy by preventing oral mucositis in patients with head and neck cancer. © 2016 Wiley Periodicals, Inc. Head Neck 38: 1387-1392, 2016.


Assuntos
Carnosina/análogos & derivados , Neoplasias de Cabeça e Pescoço/radioterapia , Compostos Organometálicos/uso terapêutico , Radioterapia/efeitos adversos , Estomatite/tratamento farmacológico , Administração Tópica , Idoso , Carnosina/uso terapêutico , Estudos de Casos e Controles , Feminino , Seguimentos , Neoplasias de Cabeça e Pescoço/mortalidade , Neoplasias de Cabeça e Pescoço/patologia , Humanos , Masculino , Pessoa de Meia-Idade , Mucosa Bucal/efeitos da radiação , Antissépticos Bucais , Alta do Paciente , Radioterapia/métodos , Dosagem Radioterapêutica , Estudos Retrospectivos , Índice de Gravidade de Doença , Estomatite/etiologia , Estomatite/fisiopatologia , Análise de Sobrevida , Fatores de Tempo , Resultado do Tratamento , Compostos de Zinco/uso terapêutico
14.
Pediatr Blood Cancer ; 63(5): 931-4, 2016 May.
Artigo em Inglês | MEDLINE | ID: mdl-26713883

RESUMO

Gorham-Stout disease (GSD) is a rare disorder of unknown etiology. We present a 6-year-old male with GSD involving the skull base who presented with recurrent cerebrospinal fluid (CSF) rhinorrhea, severe hearing loss, and facial palsy secondary to cerebellar herniation into the internal auditory canal. After 2 months of treatment with pegylated interferon (IFN) α-2b (50 µg/week), his hearing recovered dramatically. Two years later, new bone formation appeared radiologically and IFN was switched to sirolimus. One year after the switch, CSF rhinorrhea disappeared. Antiangiogenic therapy might inhibit proliferation of vascular endothelial cells in osteolytic lesions and lead to new bone formation.


Assuntos
Rinorreia de Líquido Cefalorraquidiano , Perda Auditiva , Audição , Interferon-alfa/administração & dosagem , Osteogênese , Osteólise Essencial , Polietilenoglicóis/administração & dosagem , Recuperação de Função Fisiológica , Base do Crânio/fisiopatologia , Pré-Escolar , Perda Auditiva/tratamento farmacológico , Perda Auditiva/patologia , Perda Auditiva/fisiopatologia , Humanos , Masculino , Osteólise Essencial/tratamento farmacológico , Osteólise Essencial/patologia , Osteólise Essencial/fisiopatologia , Proteínas Recombinantes/administração & dosagem , Base do Crânio/patologia
15.
Auris Nasus Larynx ; 42(6): 501-4, 2015 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-26025177

RESUMO

Invasive aspergillosis is a life-threatening infection in immunocompromised hosts and occurs most frequently in the lungs. Invasive laryngeal aspergillosis is extremely rare. Due to the potential progression of invasive aspergillosis, antifungal therapy must be started immediately in cases involving clinical suspicion of the disease. A 65-year-old male with agranulocytosis complained of sore throat and dysphagia. His epiglottis was covered with caseating granulomatous lesions and the tissue was easily disrupted. A histopathological examination showed an aggressive invasion of Aspergillus species and cartilage destruction. Therefore, we made a diagnosis of primary invasive epiglottic aspergillosis. The invasive aspergillosis resolved with antifungal therapy and an increase in neutrophils. It is therefore necessary to include invasive laryngeal aspergillosis in the differential diagnosis when encountering immunocompromised patients presenting with laryngeal granulomatous lesions and laryngitis-like symptoms.


Assuntos
Aspergilose/diagnóstico , Epiglotite/diagnóstico , Hospedeiro Imunocomprometido , Aspergilose/imunologia , Epiglotite/imunologia , Humanos , Masculino , Pessoa de Meia-Idade
16.
PLoS One ; 9(12): e115879, 2014.
Artigo em Inglês | MEDLINE | ID: mdl-25549093

RESUMO

BACKGROUND: To determine whether adverse events extend the duration of hospitalization, and to evaluate the effectiveness of medical intervention in ameliorating adverse events and reducing the prolonged hospital stay associated with adverse events. METHODS: A single arm intervention study was conducted from October 2012 to March 2014 in the otolaryngology ward of a 614-bed, university-affiliated hospital. Adverse events were monitored daily by physicians, pharmacists and nurses, and recorded in the electronic medical chart for each patient. Appropriate drug management of adverse events was performed by physicians in liaison with pharmacists. The Kaplan-Meier method was used to assess the length of hospitalization of patients who underwent medical intervention for adverse events. RESULTS: Of 571 patients admitted to the otolaryngology ward in a year, 219 patients (38.4%) experienced adverse events of grade ≥2. The duration of hospitalization was affected by the grade of adverse events, with a mean duration of hospital stay of 9.2, 17.2, 28.3 and 47.0 days for grades 0, 1, 2, and 3-4, respectively. Medical intervention lowered the incidence of grade ≥2 adverse events to 14.5%. The length of hospitalization was significantly shorter in patients who showed an improvement of adverse events after medical intervention than those who did not (26.4 days vs. 41.6 days, hazard ratio 1.687, 95% confidence interval: 1.260-2.259, P<0.001). A multivariate Cox proportional hazard analysis indicated that insomnia, constipation, nausea/vomiting, infection, non-cancer pain, oral mucositis, odynophagia and neutropenia were significant risk factors for prolongation of hospital stay. CONCLUSION: Patients who experienced adverse events are at high risk of prolonged hospitalization. Medical intervention for adverse events was found to be effective in reducing the length of hospital stay associated with adverse events.


Assuntos
Quimiorradioterapia/efeitos adversos , Tempo de Internação , Otorrinolaringopatias/cirurgia , Complicações Pós-Operatórias/epidemiologia , Adulto , Idoso , Feminino , Humanos , Estimativa de Kaplan-Meier , Masculino , Pessoa de Meia-Idade , Otorrinolaringopatias/tratamento farmacológico , Otorrinolaringopatias/radioterapia , Complicações Pós-Operatórias/tratamento farmacológico , Medição de Risco
17.
Nihon Jibiinkoka Gakkai Kaiho ; 117(9): 1194-9, 2014 Sep.
Artigo em Japonês | MEDLINE | ID: mdl-25726661

RESUMO

Bleeding from parathyroid cysts is rare. The retropharyngeal space has a very soft structure and if bleeding spreads to this space, airway obstruction can easily occur. We report on a 50-year-old female case with idiopathic neck bleeding from a left parathyroid cyst without any episode of injury. The patient complained of neck swelling after exercise and went to a nearby hospital. At the hospital, the doctor thought this swelling was caused by retropharyngeal bleeding from a tumor behind the left thyroid gland. Embolization of the left thyroid artery was performed. However, the next day, airway obstruction.was occurred and she was brought to our hospital. An emergency operation was performed to open the left neck swelling region. The operation findings and pathological examination showed that the bleeding was caused by a parathyroid cyst and airway obstruction had occurred because of retropharyngeal edema. In this case, the bleeding may have been caused by torsion of the neck when the patient exercised. The retropharyngeal edema probably resulted from the delay of delivery of the lymphatic and venous return and the arterial embolization. 1 year after the operation, the patient is very well and there has been no recurrence.


Assuntos
Cistos/complicações , Hemorragia/etiologia , Doenças das Paratireoides/complicações , Obstrução das Vias Respiratórias/etiologia , Feminino , Humanos , Pessoa de Meia-Idade
18.
Int J Cancer ; 133(5): 1107-18, 2013 Sep 01.
Artigo em Inglês | MEDLINE | ID: mdl-23420584

RESUMO

The activation of oncogenic signaling pathways induces the reprogramming of glucose metabolism in tumor cells and increases lactic acid secretion into the tumor microenvironment. This is a well-known characteristic of tumor cells, termed the Warburg effect, and is a candidate target for antitumor therapy. Previous reports show that lactic acid secreted by tumor cells is a proinflammatory mediator that activates the IL-23/IL-17 pathway, thereby inducing inflammation, angiogenesis and tissue remodeling. Here, we show that lactic acid, or more specifically the acidification it causes, increases arginase I (ARG1) expression in macrophages to inhibit T-cell proliferation and activation. Accordingly, we hypothesized that counteraction of the immune effects by lactic acid might suppress tumor development. We show that dichloroacetate (DCA), an inhibitor of pyruvate dehydrogenase kinases, targets macrophages to suppress activation of the IL-23/IL-17 pathway and the expression of ARG1 by lactic acid. Furthermore, lactic acid-pretreated macrophages inhibited CD8+ T-cell proliferation, but CD8+ T-cell proliferation was restored when macrophages were pretreated with lactic acid and DCA. DCA treatment decreased ARG1 expression in tumor-infiltrating immune cells and increased the number of IFN-γ-producing CD8+ T cells and NK cells in tumor-bearing mouse spleen. Although DCA treatment alone did not suppress tumor growth, it increased antitumor immunotherapeutic activity of Poly(IC) in both CD8+ T cell- and NK cell-sensitive tumor models. Therefore, DCA acts not only on tumor cells to suppress glycolysis but also on immune cells to improve the immune status modulated by lactic acid and to increase the effectiveness of antitumor immunotherapy.


Assuntos
Ácido Dicloroacético/farmacologia , Ácido Láctico/toxicidade , Neoplasias Experimentais/imunologia , Proteínas Serina-Treonina Quinases/antagonistas & inibidores , Animais , Arginase/genética , Linhagem Celular Tumoral , Feminino , Glicólise/efeitos dos fármacos , Interferon gama/biossíntese , Interleucina-17/genética , Subunidade p19 da Interleucina-23/genética , Ativação Linfocitária/efeitos dos fármacos , Macrófagos/fisiologia , Camundongos , Camundongos Endogâmicos C57BL , Poli I-C/farmacologia , Piruvato Desidrogenase Quinase de Transferência de Acetil
19.
Nihon Jibiinkoka Gakkai Kaiho ; 115(10): 910-6, 2012 Oct.
Artigo em Japonês | MEDLINE | ID: mdl-23214049

RESUMO

Surgery is not usually indicated for the involvement of cervical lymph node metastasis to the subclavian vein. Although surgery is indicated for the involvement of cervical lymph node metastasis to the venous angle, the usual visual field associated with cervical lymph node dissection cannot sufficiently visualize the subclavian vein, and the possibility exists of causing great vessel injury when involved lymph nodes are large and their mobility is restricted. In such cases, surgical excision may be avoided based on the expectation that ligating or cutting the internal jugular vein will be difficult. We examined 10 patients who underwent surgery for the adhesion or invasion of the primary tumor or involved lymph nodes to the venous angle or subclavian vein. The clavicle was removed or displaced to secure the visual field. The sternoclavicular joint was conserved and the clavicle, separated from the first rib, was lifted in 4 patients, while the medial two thirds of the clavicle was removed in 6 patients. Involved lymph nodes could be securely dissected without causing great vessel injury. A chylous leak occurred in one patient undergoing the procedure on the left side.


Assuntos
Neoplasias de Cabeça e Pescoço/cirurgia , Excisão de Linfonodo , Veia Subclávia/cirurgia , Idoso , Feminino , Neoplasias de Cabeça e Pescoço/patologia , Humanos , Veias Jugulares/cirurgia , Excisão de Linfonodo/métodos , Metástase Linfática , Masculino , Pessoa de Meia-Idade , Resultado do Tratamento
20.
Eur J Radiol ; 81(4): e612-7, 2012 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-22297182

RESUMO

PURPOSE: The purpose of this study was to describe MR findings including diffusion-weighted (DW) imaging findings in patients with mucosa-associated lymphoid tissue (MALT) lymphoma of the salivary glands. MATERIALS AND METHODS: Ten patients with histologically proven MALT lymphoma of the salivary glands were included. All patients underwent 1.5-T MR imaging, six of the ten underwent DW imaging, nine underwent CT, and eight (18)F-fluorodeoxyglucose (FDG) PET/CT. MR images were reviewed for numbers, locations, sizes, MR imaging characteristics, and apparent diffusion coefficients (ADCs). Calcium deposition and maximum standardized uptake values (SUVmax) were also assessed. RESULTS: Twenty-six tumors, ranging in number from 1 to 5 (mean, 2.6), were identified. Nine patients had tumors in the parotid glands and one in the submandibular glands. Tumors were found bilaterally in 7 patients and unilaterally in three. Tumors ranged in size from 0.6 to 5.5cm (mean, 1.8cm). Ten (38%) tumors had intratumoral cystic formations and 8 (31%) had ill-demarcated margins. DW images showed hyperintensity with extremely low ADCs (range, 0.48-0.82 [×10(-3)mm(2)/s]; mean, 0.64) for solid components of all 19 tumors in the 6 examined patients. Calcium deposition was found in one (4%) tumor on CT. SUVmax variously ranged from 1.3 to 17.7 (mean, 6.3). CONCLUSION: Salivary gland MALT lymphomas were often found bilaterally and were occasionally accompanied by intratumoral cystic formations and ill-demarcated margins. DW imaging may play a supplementary role in the diagnosis of lymphoma, because it showed restricted water molecule diffusion, whereas PET/CT showed indeterminate findings.


Assuntos
Linfoma de Zona Marginal Tipo Células B/patologia , Imageamento por Ressonância Magnética/métodos , Neoplasias das Glândulas Salivares/patologia , Glândulas Salivares/patologia , Idoso , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Reprodutibilidade dos Testes , Sensibilidade e Especificidade
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