RESUMO
BACKGROUND: The relationship between the major periodontal bacteria, Porphyromonas gingivalis, and the pathogenesis of IgA nephropathy (IgAN)-particularly with respect to galactose-deficient IgA1 (Gd-IgA1)-has not been fully elucidated. METHODS: Saliva samples from 30 IgAN patients and 44 patients with chronic kidney disease (CKD) were subjected to analysis of P. gingivalis status via polymerase chain reaction using a set of P. gingivalis-specific primers. The associations between P. gingivalis presence and clinical parameters, including plasma Gd-IgA1, were analyzed in each group. RESULTS: Compared with the CKD group, the IgAN group demonstrated significantly higher plasma Gd-IgA1 levels (p < 0.05). Compared with the P. gingivalis-negative subgroup, the P. gingivalis-positive subgroup exhibited significantly higher plasma Gd-IgA1 levels in both IgAN and CKD patients (p < 0.05). Additionally, among IgAN patients, the P. gingivalis-positive subgroup displayed significantly higher plasma Gd-IgA1 and urine protein levels, compared with the P. gingivalis-negative subgroup (p < 0.05). With respect to renal biopsy findings, the frequencies of segmental glomerulosclerosis and tubular atrophy/interstitial fibrosis were significantly greater in the P. gingivalis-positive subgroup than in the P. gingivalis-negative subgroup, according to the Oxford classification of IgAN (p < 0.05). CONCLUSION: Our findings suggest an association between the presence of P. gingivalis in the oral cavity and the pathogenesis of IgAN, mediated by increased levels of Gd-IgA1.
Assuntos
Glomerulonefrite por IGA , Insuficiência Renal Crônica , Humanos , Glomerulonefrite por IGA/patologia , Porphyromonas gingivalis/metabolismo , Galactose/metabolismo , Imunoglobulina A/metabolismo , BocaRESUMO
BACKGROUND: Subependymal giant cell astrocytoma (SEGA) is occasionally seen in tuberous sclerosis complex (TSC). Two main options are currently available for treating SEGA: surgical resection or pharmacotherapy using mammalian target of rapamycin inhibitors (mTORi). We hypothesized that opportunities for surgical resection of SEGA would have reduced with the advent of mTORi. METHODS: We retrospectively reviewed the charts of patients treated between August 1979 and July 2020, divided into a pre-mTORi era group (Pre-group) of patients treated before November 2012, and a post-mTORi era group (Post-group) comprising patients treated from November 2012, when mTORi became available in Japan for SEGA. We compared groups in terms of treatment with surgery or mTORi. We also reviewed SEGA size, rate of acute hydrocephalus, recurrence of SEGA, malignant transformation and adverse effects of mTORi. RESULTS: In total, 120 patients with TSC visited our facility, including 24 patients with SEGA. Surgical resection was significantly more frequent in the Pre-group (6 of 7 patients, 86 %) than in the Post-group (2 of 17 patients, 12 %; p = 0.001). Acute hydrocephalus was seen in 1 patient (4 %), and no patients showed malignant transformation of SEGA. The group treated using mTORi showed significantly smaller SEGA compared with the group treated under a wait-and-see policy (p = 0.012). Adverse effects of pharmacotherapy were identified in seven (64 %; 6 oral ulcers, 1 irregular menstruation) of the 11 patients receiving mTORi. CONCLUSIONS: The Post-group underwent surgery significantly less often than the Pre-group. Since the treatment option to use mTORi in the treatment of SEGA in TSC became available, opportunities for surgical resection have decreased in our facility.
Assuntos
Antineoplásicos/uso terapêutico , Astrocitoma/tratamento farmacológico , Neoplasias Encefálicas/tratamento farmacológico , Serina-Treonina Quinases TOR/antagonistas & inibidores , Esclerose Tuberosa/complicações , Adolescente , Adulto , Astrocitoma/genética , Neoplasias Encefálicas/genética , Criança , Pré-Escolar , Feminino , Humanos , Lactente , Japão , Masculino , Estudos Retrospectivos , Adulto JovemRESUMO
BACKGROUND: We previously found that worse dental caries status was associated with high pulse pressure among patients on hemodialysis, indicating that such patients might have arteriosclerosis. In this study, we used abdominal computed tomography to evaluate arteriosclerosis in patients on hemodialysis and investigated the association between arteriosclerosis and dental caries status. We also prospectively examined risk factors associated with 2-year prognosis. METHODS: The dental caries and periodontal disease statuses of 80 patients on hemodialysis were evaluated using the decayed, missing, or filled teeth (DMFT) index, and periodontal pocket depth, respectively. The aortic calcification index was semiquantitatively measured using computed tomography images of the abdominal aorta. Clinical data were also analyzed after all patients on hemodialysis provided written, informed consent to participate in the study. RESULTS: Regression analysis demonstrated a significant correlation between the DMFT and aortic calcification indexes. Multiple regression analysis showed that the DMFT index was significantly correlated with the aortic calcification index, following adjustment for age, sex, and dialysis period. Thirteen of the 80 patients died during the 2-year follow-up period; logistic regression analysis showed that mortality rate was significantly associated with the aortic calcification index, but not the DMFT index. However, periodontal pocket depth was not correlated with the aortic calcification index. CONCLUSION: These findings suggest that worse dental caries status could be associated with arteriosclerosis among patients on hemodialysis, which may indirectly affect the prognosis of arteriosclerosis in these patients.
Assuntos
Arteriosclerose/epidemiologia , Cárie Dentária/epidemiologia , Doenças Periodontais/epidemiologia , Diálise Renal , Calcificação Vascular/epidemiologia , Idoso , Aorta Abdominal/diagnóstico por imagem , Arteriosclerose/diagnóstico por imagem , Índice CPO , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Prognóstico , Insuficiência Renal Crônica/terapia , Fatores de Risco , Índice de Gravidade de Doença , Taxa de Sobrevida , Tomografia Computadorizada por Raios X , Calcificação Vascular/diagnóstico por imagemRESUMO
Myelodysplastic syndrome (MDS) is a clonal disorder arising from an alteration in multipotent stem cells, which lose the ability of normal proliferation and differentiation. Disease progression occurs in approximately 30% MDS cases. Specific chromosomal alterations seem responsible for each step in the evolution of acute myeloid leukemia (AML). Multiple genetic aberrations occur during the clonal evolution of MDS; however, few studies report the presence of the Philadelphia (Ph) chromosome. We report a rare case of Ph-positive AML, which evolved during the course of low-risk MDS. The patient, a 76-year-old man with mild leukocytopenia, was diagnosed with MDS, refractory neutropenia (RN). After 1.5 yr, his peripheral blood and bone marrow were suddenly occupied by immature basophils and myeloblasts, indicating the onset of AML. A bone marrow smear showed multilineage dysplasia, consistent with MDS evolution. Chromosomal analysis showed an additional t(9;22)(q34;q11) translocation. Because progression occurred concurrently with emergence of the Ph chromosome, we diagnosed this case as Ph-positive AML with basophilia arising from the clonal evolution of MDS. The patient was initially treated with nilotinib. A hematological response was soon achieved with disappearance of the Ph chromosome in the bone marrow. Emergence of Ph-positive AML in the course of low-risk MDS has rarely been reported. We report this case as a rare clinical course of MDS.
Assuntos
Medula Óssea/patologia , Leucemia Mieloide Aguda/genética , Síndromes Mielodisplásicas/genética , Cromossomo Filadélfia , Cariótipo Anormal , Idoso , Antineoplásicos/farmacologia , Antineoplásicos/uso terapêutico , Evolução Clonal , Progressão da Doença , Humanos , Cariotipagem , Leucemia Mieloide Aguda/tratamento farmacológico , Leucemia Mieloide Aguda/patologia , Masculino , Síndromes Mielodisplásicas/tratamento farmacológico , Síndromes Mielodisplásicas/patologia , Pirimidinas/farmacologia , Pirimidinas/uso terapêuticoRESUMO
Chronic active Epstein-Barr virus infection (CAEBV) is a systemic T- or NK-lymphoproliferative disorder (LPD) caused by EBV. Allogenic hematopoietic stem cell transplantation (HSCT) is the only curative therapy for CAEBV, but relapse sometimes occurs. Relapse is generally attributed to proliferation of recipient-derived CAEBV cells. We herein report a case of donor-derived CAEBV-like NK-cell post-transplant lymphoproliferative disease (PTLD) in a 41-year-old female after the first allogenic HSCT for CAEBV from an HLA-matched sibling donor. A second HSCT from an HLA-matched unrelated donor successfully controlled the disease, but EBV infection of cells derived from the second donor continued to be detected. Although the mechanisms underlying CAEBV and CAEBV-like NK-cell PTLD have not yet been elucidated in detail, the findings of the present case imply that host genetic factors, including familial factors, may be important in disease development.
Assuntos
Infecções por Vírus Epstein-Barr , Transplante de Células-Tronco Hematopoéticas , Transtornos Linfoproliferativos , Adulto , Infecções por Vírus Epstein-Barr/complicações , Infecções por Vírus Epstein-Barr/terapia , Feminino , Transplante de Células-Tronco Hematopoéticas/efeitos adversos , Herpesvirus Humano 4/genética , Humanos , Células Matadoras Naturais , Transtornos Linfoproliferativos/etiologia , Transtornos Linfoproliferativos/terapiaRESUMO
Shwachman-Diamond syndrome (SDS) is an autosomal recessive inherited disorder characterized by bone marrow failure, exocrine pancreatic dysfunction, and skeletal abnormalities. SDS is typically caused by a pathogenic mutation in the Shwachman-Bodian-Diamond Syndrome (SBDS) gene. Patients with SDS have an increased risk of developing acute myeloid leukemia (AML) and myelodysplastic syndromes. We identified germline biallelic SBDS mutations (p.K62X and p.I167M) in a 50-year-old AML patient who had never experienced the typical symptoms of SDS. The K62X mutation is one of the most common pathogenic mutations, whereas the significance of the I167M mutation was unclear. Based on cellular experiments, we concluded that the I167M mutation contributed to the development of AML, and chemotherapy including topoisomerase inhibitors, which induce DNA double-strand breaks, may have been toxic to this patient. Our experience indicates that some asymptomatic Shwachman-Bodian-Diamond syndrome mutations contribute to the development of leukemia, and that careful treatment selection may be warranted for patients harboring these mutations.
Assuntos
Mutação em Linhagem Germinativa/genética , Leucemia Mieloide Aguda/etiologia , Leucemia Mieloide Aguda/genética , Proteínas/genética , Síndrome de Shwachman-Diamond/genética , DNA/metabolismo , Reparo do DNA/efeitos dos fármacos , Feminino , Genes Recessivos/genética , Humanos , Leucemia Mieloide Aguda/tratamento farmacológico , Pessoa de Meia-Idade , Síndromes Mielodisplásicas/etiologia , Síndrome de Shwachman-Diamond/complicações , Inibidores da Topoisomerase/efeitos adversosRESUMO
We investigated the hematopoietic status of aplastic anemia with FDG-PET before and after immunosuppressive therapy. FDG-PET showed a patchy uptake pattern before treatment, indicating residual compensatory hypercellular marrow. Three years after successful treatment with ATG plus CsA, the heterogeneity of bone marrow uptake persisted, suggesting that expanded reconstitution of hematopoiesis may require a long time even after the achievement of hematological remission.
Assuntos
Anemia Aplástica/diagnóstico por imagem , Anemia Aplástica/fisiopatologia , Fluordesoxiglucose F18 , Hematopoese , Tomografia por Emissão de Pósitrons , Compostos Radiofarmacêuticos , Anemia Aplástica/tratamento farmacológico , Autoanticorpos/administração & dosagem , Ciclosporina/administração & dosagem , Humanos , Imunossupressores/administração & dosagem , Masculino , Pessoa de Meia-IdadeRESUMO
TAFRO syndrome and POEMS syndrome are lymphoproliferative disorders with elevated interleukin-6 and vascular endothelial growth factor (VEGF) levels; however, their underlying pathogenic mechanisms remain unclear. Similarities have been reported in the pathological findings of the lymph nodes of TAFRO syndrome, Multicentric Castleman disease (MCD), and some cases of POEMS syndrome. However, there is no consensus on the relationship among them. We encountered a case of lymphoproliferative disorder that presented with manifestations of both TAFRO syndrome and POEMS syndrome. This case may be a subtype of idiopathic MCD and will be very important for establishing the disease concept of TAFRO syndrome and POEMS syndrome.
Assuntos
Hiperplasia do Linfonodo Gigante , Síndrome POEMS , Hiperplasia do Linfonodo Gigante/complicações , Hiperplasia do Linfonodo Gigante/diagnóstico , Humanos , Linfonodos , Síndrome POEMS/complicações , Síndrome POEMS/diagnóstico , Fator A de Crescimento do Endotélio VascularRESUMO
BACKGROUND AIMS: Cell therapy with mesenchymal stromal cells (MSC) has been reported recently as a promising treatment for severe acute graft-versus-host disease (GvHD). METHODS: We designed a pilot study to treat severe hepatic or gut GvHD using MSC derived from only the donor and cultured without bovine serum. Because the number of cultured MSC is smaller using this method, we planned to treat patients by intra-arterial regional administration directly to the target organs. RESULTS: Three patients were enrolled, and the MSC could be expanded using donor serum. There were no obvious side-effects immediately after arterial injection. The maximum response was partial in one of three patients and did not continue for more than 2 months. Idiopathic pneumonia syndrome developed in two of the three patients. CONCLUSIONS: A single local arterial MSC injection was unable to save these patients' lives and so might not be more effective than multiple systemic intravenous MSC injection. Further clinical research and additional strategies are required to develop appropriate methods for using MSC to achieve extended remission of GvHD.
Assuntos
Resistência a Medicamentos , Doença Enxerto-Hospedeiro/terapia , Transplante de Células-Tronco Mesenquimais , Células-Tronco Mesenquimais/citologia , Células-Tronco Mesenquimais/efeitos dos fármacos , Esteroides/farmacologia , Células Estromais/transplante , Doença Aguda , Adulto , Resistência a Medicamentos/efeitos dos fármacos , Evolução Fatal , Feminino , Doença Enxerto-Hospedeiro/prevenção & controle , Humanos , Injeções Intra-Arteriais , Masculino , Pessoa de Meia-Idade , Projetos Piloto , Radiografia Torácica , Esteroides/uso terapêutico , Células Estromais/citologia , Tomografia Computadorizada por Raios XRESUMO
Heavy chain disease (HCD) is a rare B-cell proliferative neoplasm that is characterised by the production of truncated monoclonal immunoglobulin heavy chains without light chains. Gamma HCD is a subgroup of HCD. A 67-year-old man was admitted to our hospital with dyspnoea and lower leg oedema. Based on the results of heart catheterisation, he was diagnosed with pulmonary hypertension. Laboratory tests revealed an elevated level of IgG, and serum immunoelectrophoresis showed that IgG was a monoclonal gamma heavy chain without light chains. Finally, he was diagnosed with gamma HCD complicated by pulmonary hypertension. Bortezomib and dexamethasone therapy was initiated, but became refractory within 8 months. Therefore, the treatment was switched to lenalidomide and dexamethasone therapy, and the disease has been stably controlled for more than 2 years. To the best of our knowledge, this is the first case of gamma HCD being successfully treated by lenalidomide and dexamethasone therapy.
Assuntos
Doença das Cadeias Pesadas/tratamento farmacológico , Hipertensão Pulmonar/etiologia , Fatores Imunológicos/uso terapêutico , Lenalidomida/uso terapêutico , Idoso , Medula Óssea/patologia , Bortezomib/uso terapêutico , Dexametasona/uso terapêutico , Quimioterapia Combinada , Doença das Cadeias Pesadas/complicações , Humanos , Imunoglobulina G/sangue , MasculinoRESUMO
OBJECTIVES: It is generally thought that the recovery of damaged chorda tympani nerve (CTN) function after middle ear surgery is different in pediatric patients from that in adult patients. The purpose of this study was to investigate the changes and the progress of taste and somatosensory functions of the tongue after middle ear surgery in pediatric patients compared with those of adult patients. STUDY DESIGN: Prospective study. METHODS: Fifty-nine pediatric patients and 106 adult patients underwent middle ear surgery. Taste and somatosensory functions of the anterior tongue, the so-called CTN functions, were assessed using electrogustometry (EGM), a 2-point discriminator, an electrostimulator, and a questionnaire before and 2 weeks and 6 months after surgery. RESULTS: Two weeks after surgery, there was no significant difference in the incidence of dysgeusia and abnormal EGM thresholds between the patient groups. The incidence of tongue numbness was significantly lower in pediatric patients than in adult patients regardless of CTN manipulation. Although the lingual somatosensory thresholds of adult patients were significantly increased, those of pediatric patients were not increased. Six months after surgery, the incidences of dysgeusia and an abnormal EGM threshold were lower in pediatric patients than in adult patients. Tongue numbness disappeared, and the thresholds of lingual somatosensory tests returned to normal in most pediatric patients. CONCLUSION: Not only taste function but also lingual somatosensory function was damaged after middle ear surgery even in pediatric patients. Pediatric patients complained of tongue numbness less frequently and showed earlier recovery than adult patients. LEVEL OF EVIDENCE: 2 Laryngoscope, 130:1016-1022, 2020.
Assuntos
Nervo da Corda do Tímpano/fisiopatologia , Orelha Média/cirurgia , Procedimentos Cirúrgicos Otológicos/efeitos adversos , Recuperação de Função Fisiológica/fisiologia , Distúrbios do Paladar/fisiopatologia , Limiar Gustativo/fisiologia , Língua/fisiopatologia , Adolescente , Adulto , Idoso , Criança , Nervo da Corda do Tímpano/lesões , Feminino , Seguimentos , Humanos , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Distúrbios do Paladar/etiologia , Fatores de Tempo , Adulto JovemRESUMO
Methotrexate, hydrocortisone, vincristine, sobuzoxane, and etoposide (MTX-HOPE) chemotherapy was originally reported in 2007 as a salvage regimen for relapsed or refractory non-Hodgkin's lymphoma. To clarify the safety and efficacy of this regimen, we retrospectively analyzed patients at our institute. We analyzed 18 patients, including 16 with diffuse large B-cell lymphoma (DLBCL), one with follicular lymphoma (FL), and one with T-cell lymphoma. The median age at MTX-HOPE therapy was 79 (range: 68-87). Ten patients received more than 3 previous chemotherapy regimens. The median period from the initial treatment to the first MTX-HOPE administration was 53 months. No patient had severe renal dysfunction. The overall response rate was 78%, with 39% achieving CR and 39% achieving PR. The median OS and PFS after the initiation of MTX-HOPE were 10 months (range: 0.5-86 months) and 7 months (range: 0.2-86 months), respectively. The one-year OS rate was 44% and the two-year OS rate was 22%. The median number of treatment cycles was 7 (range: 1-46), and 6 patients received more than 10 cycles. Among eight patients who were over 80 years of age, 7 responded to the therapy, and the median OS and PFS of this subgroup were 19 months and 11 months, respectively. All patients tolerated the treatment well, mostly on an outpatient basis, except for one who died from infection and one who developed intracranial hemorrhage. MTX-HOPE may be a promising treatment option for elderly patients with refractory or relapsed malignant lymphoma.
Assuntos
Linfoma não Hodgkin/terapia , Terapia de Salvação , Idoso , Idoso de 80 Anos ou mais , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Etoposídeo/uso terapêutico , Feminino , Humanos , Hidrocortisona/uso terapêutico , Masculino , Metotrexato/uso terapêutico , Piperazinas/uso terapêutico , Terapia de Salvação/métodos , Resultado do Tratamento , Vincristina/uso terapêuticoRESUMO
BACKGROUND: Patients on hemodialysis must undergo this procedure at a hospital three times weekly and might be unable to visit a dentist. In addition, dentists might hesitate to provide oral care because such patients tend to bleed because they are medicated with anticoagulants, are susceptible to bacterial infections, and might have unusual drug reactions. We postulated that patients on hemodialysis have worse oral status than healthy people, which in turn might predispose such patients to systemic complications. METHODS: We compared the status of dental caries and periodontal diseases among 80 patients on hemodialysis and 76 healthy individuals (controls) using the decayed, missing, or filled teeth (DMFT) index, total number of C4 teeth (destruction of the entire tooth crown), and periodontal pocket depth. Clinical data were analyzed after all patients on hemodialysis and controls provided written, informed consent to participate in the study. RESULTS: Total number of C4 teeth (p = 0.021), missing teeth (MT) index (p = 0.0302), and DMFT index score ≥ 24 (p = 0.017) were significantly higher in patients on hemodialysis than controls. Pulse pressure (p = 0.0042) and the prevalence of a history of heart disease such as angina pectoris and acute myocardial infarction (p = 0.029) were higher in patients on hemodialysis with higher (≥ 24) than lower (< 24) DMFT index scores. Periodontal pocket depth was not significantly different between these two groups. CONCLUSION: Worse status of dental caries is possibly associated with arteriosclerosis among patients on hemodialysis.
Assuntos
Arteriosclerose/epidemiologia , Cárie Dentária/epidemiologia , Falência Renal Crônica/terapia , Doenças Periodontais/epidemiologia , Diálise Renal , Idoso , Idoso de 80 Anos ou mais , Comorbidade , Feminino , Humanos , Falência Renal Crônica/epidemiologia , Masculino , Pessoa de Meia-Idade , Saúde Bucal , PrevalênciaRESUMO
Angioimmunoblastic T-cell lymphoma (AITL) is a common subtype of peripheral T-cell lymphoma with an aggressive clinical course and poor prognosis after conventional chemotherapy, for which there is no current standard of care. We describe here an 87-year-old woman with AITL, whose clinical diagnosis was complicated by the presence of B immunoblasts positive for Epstein-Barr virus in the lymph nodes and monoclonal plasma cells in the bone marrow at initial presentation. Rebiopsy of the lymph node led to the correct diagnosis of AITL with concurrent smoldering plasma cell myeloma. She was treated with several courses of conventional chemotherapy, resulting in progressive disease, and then switched to the immunomodulatory drug lenalidomide, which used in Japan for the treatment of multiple myeloma. Lenalidomide was effective in controlling both AITL and plasma cell myeloma.
Assuntos
Proliferação de Células , Infecções por Vírus Epstein-Barr , Herpesvirus Humano 4 , Lenalidomida/administração & dosagem , Linfoma de Células T Periférico , Plasmócitos/metabolismo , Idoso de 80 Anos ou mais , Infecções por Vírus Epstein-Barr/sangue , Infecções por Vírus Epstein-Barr/diagnóstico , Infecções por Vírus Epstein-Barr/tratamento farmacológico , Infecções por Vírus Epstein-Barr/virologia , Feminino , Humanos , Linfoma de Células T Periférico/sangue , Linfoma de Células T Periférico/diagnóstico , Linfoma de Células T Periférico/tratamento farmacológico , Linfoma de Células T Periférico/virologiaRESUMO
OX40 (CD134), an activation-induced costimulatory molecule, is mainly expressed on CD4(+) T cells. Several reports, including previous reports from our laboratory, suggest that OX40-mediated signaling plays an important role in the development of graft-versus-host disease (GVHD) after allogeneic hematopoietic stem cell transplantation (Allo HSCT). Here, we show that peripheral blood CD4(+)OX40(+) T cells are a unique cell subset as they possess the homing receptors of lymph nodes, and some of them have an exceptional capacity to produce high levels of interleukin-2 (IL-2) upon the stimulation through T cell receptors. Stimulation with IL-7 acts selectively on CD4(+)OX40(+) T cells not only to induce antigen-independent growth but also to increase the frequency of cells with IL-2-producing potential. Simultaneous, but not sequential, ligation of the T cell receptor and OX40 induces CD4(+)OX40(+) T cells to produce far more IL-2, which causes them to proliferate abundantly and differentiate readily into Th1- or Th2-biased effector memory T cells, especially in Allo HSCT recipients. Although not all the CD4(+)OX40(+) T cells had IL-2-producing capacity, Allo HSCT recipients with chronic GVHD (cGVHD) had a significantly higher frequency of IL-2-producing OX40(+) cells in their peripheral blood CD4(+) T cell subset than Allo HSCT recipients without cGVHD. Collectively, CD4(+)OX40(+) T cells with IL-2-producing potential are expected to be privileged for growth and differentiation in lymph nodes upon antigen presentation, suggesting that they might be involved in the process of inducing or maintaining cGVHD.
Assuntos
Diferenciação Celular/imunologia , Proliferação de Células , Doença Enxerto-Hospedeiro/imunologia , Transplante de Células-Tronco Hematopoéticas , Receptores OX40/imunologia , Transdução de Sinais , Células Th1/imunologia , Células Th2/imunologia , Quimeras de Transplante/imunologia , Adulto , Idoso , Apresentação de Antígeno/imunologia , Contagem de Linfócito CD4 , Doença Crônica , Feminino , Doença Enxerto-Hospedeiro/sangue , Doença Enxerto-Hospedeiro/patologia , Neoplasias Hematológicas/sangue , Neoplasias Hematológicas/imunologia , Neoplasias Hematológicas/patologia , Neoplasias Hematológicas/terapia , Humanos , Capeamento Imunológico , Memória Imunológica/imunologia , Interleucina-2/sangue , Interleucina-2/imunologia , Interleucina-7/sangue , Interleucina-7/imunologia , Linfonodos/imunologia , Linfonodos/metabolismo , Linfonodos/patologia , Masculino , Pessoa de Meia-Idade , Receptores OX40/metabolismo , Células Th1/metabolismo , Células Th1/patologia , Células Th2/metabolismo , Células Th2/patologia , Quimeras de Transplante/sangue , Transplante Homólogo , Células Tumorais CultivadasRESUMO
OBJECTIVES: Patients after middle ear surgery often complain of taste disturbance and a lingual numbness. The purpose of this study was to objectively assess changes in the somatosensation of the tongue and taste function in patients undergoing stapes surgery. STUDY DESIGN: Prospective study. METHODS: Symptoms of taste disturbance and tongue numbness after surgery were investigated before and after surgery in 41 patients (13 males, 28 females; mean age 41.8 years) who underwent stapes surgery. Twenty-eight patients (9 males, 19 females; mean age 43.1 years) underwent sensory and taste function tests before and after surgery. Sensory function of the tongue was measured at the operated side and the nonoperated side using the 2-point discrimination test and an electrostimulator test. Taste function was assessed with electrogustometry (EGM). RESULTS: The chorda tympani nerve (CTN) was gently touched or stretched in all patients. Postoperative thresholds on the operated side were significantly higher than preoperative thresholds in all tests in the patients who underwent all three kinds of tests. Tongue somatosensory symptoms improved significantly earlier than the taste disturbance postoperatively, and the sensory thresholds returned to the baseline along with recovery of symptoms. CONCLUSION: These findings suggest that dysfunction of the CTN occurred following surgery even when the CTN was preserved, and that the sensory nerve threshold of the tongue correlated with the symptom of lingual numbness. The CTN may play a role not only in taste function but also in the somatosensory function of the tongue. LEVEL OF EVIDENCE: 4. Laryngoscope, 128:701-706, 2018.
Assuntos
Nervo da Corda do Tímpano/lesões , Disgeusia/fisiopatologia , Doenças do Nervo Facial/etiologia , Limiar Sensorial/fisiologia , Cirurgia do Estribo/efeitos adversos , Limiar Gustativo/fisiologia , Língua/inervação , Adulto , Nervo da Corda do Tímpano/fisiopatologia , Disgeusia/diagnóstico , Disgeusia/etiologia , Doenças do Nervo Facial/diagnóstico , Doenças do Nervo Facial/fisiopatologia , Feminino , Humanos , Masculino , Estudos Prospectivos , Língua/fisiopatologiaRESUMO
Tuberous sclerosis complex (TSC) is an autosomal dominant inherited disease characterized by lesions that involve multiple organs. Interdisciplinary management at individual facilities needs to be coordinated to treat multiple organ systems. We hypothesized that the number of patients, opportunities for patients to undergo examinations, and opportunities for patients to be treated would increase after establishment of a TSC board (TB) in our hospital. From August 1979 to August 2017, 76 patients were studied. We established the TB in our hospital in 2014. We divided the patients into the pre-TB group and post-TB group. Patients consisted of 33 females and 43 males (mean age, 18.7 years; median age, 15 years). The follow-up period was 2 to 457 months (mean, 51.6 months; median, 24.5 months). Twenty-four patients were in the pre-TB group, and 52 were in the post-TB group. Regular follow-up (p < 0.001), younger age (p = 0.002), opportunities for patients to undergo examinations, opportunities for patients to receive neurological treatment (p < 0.001), and mammalian target of rapamycin (mTOR) inhibitor usage (p = 0.041) were significantly higher in the post-TB group. The radial relationship around the axis of TSC coordinators may be the key to interdisciplinary management of TSC.
Assuntos
Equipe de Assistência ao Paciente , Esclerose Tuberosa , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Criança , Pré-Escolar , Feminino , Humanos , Lactente , Masculino , Pessoa de Meia-Idade , Avaliação de Resultados em Cuidados de Saúde , Diagnóstico Pré-Natal , Garantia da Qualidade dos Cuidados de Saúde , Esclerose Tuberosa/diagnóstico , Esclerose Tuberosa/terapia , Adulto JovemRESUMO
Autoimmune autonomic ganglionopathy is an autonomic disorder that occurs as a symptom of paraneoplastic neurological syndrome. To date, there have been no reports on multiple myeloma with autoimmune autonomic ganglionopathy. A 37-year-old Japanese woman suffered from orthostatic hypotension was diagnosed with multiple myeloma (IgG kappa type), and a serological examination revealed the presence of anti-ganglionic nicotinic acetylcholine receptor (anti-gAChR) antibodies. She was treated for multiple myeloma, as a result, the autonomic disturbance improved and her anti-gAChR antibody titer decreased to undetectable levels, despite the fact that she only achieved a partial remission of multiple myeloma. Treatment for multiple myeloma may improve autoimmune autonomic ganglionopathy.
Assuntos
Doenças do Sistema Nervoso Autônomo/complicações , Gânglios Autônomos/patologia , Mieloma Múltiplo/complicações , Adulto , Autoanticorpos/imunologia , Feminino , Humanos , Receptores Nicotínicos/imunologiaRESUMO
A dose modified ifosfamide, epirubicin, and etoposide (IVE) regimen was prospectively assessed for its efficacy in mobilizing peripheral blood stem cells for autologous transplantation. Two patients with Hodgkin's lymphoma and two with non-Hodgkin's lymphoma who were undergoing stem cell therapy were studied. All patients had a history of multiple treatments with insufficient stem cell mobilization. The dose modified IVE regimen consisted of ifosfamide 3 g/m(2) intravenously (IV) administered on days 1-2 in combination with epirubicin 50 mg/m(2) IV on day 1 and etoposide 200 mg/m(2) (100 mg/m(2) in two patients with complete remission) IV on days 1-3. The ifosfamide dosage was reduced to two-thirds of the original protocol. A substantial high yield of CD34(+) cells was achieved when patients were treated with a dose-modified IVE regimen, compared with that during the previous regimen (two with the ifosfamide, carboplatin, and etoposide [ICE] regimen, one with high-dose cyclophosphamide and one with the original IVE regimen). Two patients who had refractory and residual disease received a 200 mg/m(2) dose of etoposide, which resulted in tumor reduction (one patient with complete remission and one with further reduction in tumor size). After the IVE regimen, all four patients had a sufficient yield of CD34(+) cells in total, which was available for stem cell transplantation. Hematological and non-hematological toxicities were comparable in all regimens. This single-center prospective study demonstrated that the dose-modified IVE regimen can be used as a safe treatment with high mobilizing efficacy in heavily pretreated lymphoma patients.
Assuntos
Antineoplásicos/uso terapêutico , Epirubicina/uso terapêutico , Etoposídeo/uso terapêutico , Mobilização de Células-Tronco Hematopoéticas/métodos , Doença de Hodgkin/terapia , Ifosfamida/uso terapêutico , Linfoma não Hodgkin/terapia , Antígenos CD34/análise , Antineoplásicos/administração & dosagem , Epirubicina/administração & dosagem , Etoposídeo/administração & dosagem , Humanos , Ifosfamida/administração & dosagem , Pessoa de Meia-Idade , Recidiva Local de Neoplasia/terapia , Células-Tronco de Sangue Periférico/citologia , Estudos Prospectivos , Terapia de Salvação/métodosRESUMO
Streptococcus mutans is a major pathogen of human dental caries. Strains harbouring the cnm gene, which encodes Cnm, a collagen-binding protein, contribute to the development of several systemic diseases. In this study, we analysed S. mutans strains isolated from the oral cavity of immunoglobulin (Ig)A nephropathy (IgAN) patients to determine potential relationships between cnm and caries status as well as IgAN conditions. Saliva specimens were collected from 109 IgAN patients and the cnm status of isolated S. mutans strains was determined using PCR. In addition, the dental caries status (decayed, missing or filled teeth [DMFT] index) in patients who agreed to dental consultation (n = 49) was evaluated. The DMFT index and urinary protein levels in the cnm-positive group were significantly higher than those in the cnm-negative group (p < 0.05). Moreover, the urinary protein levels in the high DMFT (≥15) group were significantly higher than those in the low DMFT (<15) group (p < 0.05). Our results show that isolation of cnm-positive S. mutans strains from the oral cavity may be associated with urinary protein levels in IgAN patients, especially those with a high dental caries status.