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1.
Kyobu Geka ; 74(8): 635-639, 2021 Aug.
Artigo em Japonês | MEDLINE | ID: mdl-34334610

RESUMO

We experienced two cases of primary pulmonary amyloidosis with a localized consolidation. Case 1 is a 80-year-old man, who was found to have an abnormal chest nodular shadow with blurred margin at a medical examination. Chest computed tomography( CT) showed a localized consolidation on the periphery of the upper lobe of the right lung. A CT-guided biopsy was performed. Case 2 is a 66-year-old woman, who was found to have an abnormal chest opacity at a medical examination. Chest CT showed a localized gathering of small nodules in the right lower lobe. Gradual enlargement was noted by follow up CT and the accumulation of fluorodeoxyglucose (FDG) was shown by PET/CT. In consideration of primary lung cancer or malignant lymphoma, right lower lobectomy was performed. Both cases were pathologically diagnosed as pulmonary amyloidosis. Since no findings of amyloid deposits in other organs or of existence of any blood disorders, a diagnosis of primary pulmonary amyloidosis was made.


Assuntos
Amiloidose , Amiloidose de Cadeia Leve de Imunoglobulina , Pneumopatias , Neoplasias Pulmonares , Idoso , Idoso de 80 Anos ou mais , Amiloidose/diagnóstico por imagem , Amiloidose/cirurgia , Feminino , Humanos , Amiloidose de Cadeia Leve de Imunoglobulina/diagnóstico por imagem , Pulmão/diagnóstico por imagem , Pulmão/cirurgia , Pneumopatias/diagnóstico por imagem , Pneumopatias/cirurgia , Neoplasias Pulmonares/diagnóstico por imagem , Neoplasias Pulmonares/cirurgia , Masculino , Tomografia por Emissão de Pósitrons combinada à Tomografia Computadorizada
2.
J Card Surg ; 36(10): 3509-3518, 2021 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-34254364

RESUMO

BACKGROUND: The incidence of systemic amyloidosis is rising, and there is a concomitant rise in heart transplant for an indication of cardiac amyloidosis. METHODS: We utilized the Organ Procurement and Transplantation Network (OPTN) database to retrospectively assess survival and outcomes in adult patients undergoing heart transplant for cardiac amyloidosis from 1999 to 2019. We also compared survival among four distinct time periods: 1999-2001, 2002-2008, 2008-2015, 2016-2019. RESULTS: Of 41,103 patients, 425 (1.03%) were transplanted for an indication of restrictive cardiomyopathy due to cardiac amyloidosis (RCM-Amyloidosis). The percent of all transplants occurring for RCM-Amyloidosis increased from 0.25% in the 1999-2001 era to 1.74% in the 2015-2019 era (p < .001). Across eras, Kaplan-Meier survival functions were comparable between RCM-Amyloidosis and non-RCM patients at 1 year (88% vs. 89%, p = .56) and at 5 years (72% vs. 77%, p = .092), but worse for RCM-Amyloidosis patients at 10 years (44% vs. 59%, p = .002). With adjustment for other clinical variables in multivariable Cox regression model, RCM-Amyloidosis was not associated with increased risk of death at 1 year (hazard ratio [HR] = 1.11, p = .56) or at 5 years (HR = 1.20, p = .18), but it was associated with increased risk of death at 10 years (HR = 1.35, p = .01). Cardiac amyloidosis was not associated with any morbidity outcomes following transplant, including graft failure, acute rejection, or hospitalization for infection or rejection. CONCLUSIONS: Our data suggest a trend of improving survival among RCM-Amyloidosis patients compared with non-RCM patients across transplant eras, with current similarities in 1- and 5-year survival but a persistent, increased risk of mortality at 10 years.


Assuntos
Amiloidose , Cardiomiopatia Restritiva , Transplante de Coração , Adulto , Amiloidose/cirurgia , Humanos , Modelos de Riscos Proporcionais , Estudos Retrospectivos
4.
Nephrol Dial Transplant ; 36(2): 355-365, 2021 01 25.
Artigo em Inglês | MEDLINE | ID: mdl-33439995

RESUMO

BACKGROUND: Outcomes after renal transplantation have traditionally been poor in systemic amyloid A (AA) amyloidosis and systemic light chain (AL) amyloidosis, with high mortality and frequent recurrent disease. We sought to compare outcomes with matched transplant recipients with autosomal dominant polycystic kidney disease (ADPKD) and diabetic nephropathy (DN), and identify factors predictive of outcomes. METHODS: We performed a retrospective cohort study of 51 systemic AL and 48 systemic AA amyloidosis patients undergoing renal transplantation. Matched groups were generated by propensity score matching. Patient and death-censored allograft survival were compared via Kaplan-Meier survival analyses, and assessment of clinicopathological features predicting outcomes via Cox proportional hazard analyses. RESULTS: One-, 5- and 10-year death-censored unadjusted graft survival was, respectively, 94, 91 and 78% for AA amyloidosis, and 98, 93 and 93% for AL amyloidosis; median patient survival was 13.1 and 7.9 years, respectively. Patient survival in AL and AA amyloidosis was comparable to DN, but poorer than ADPKD [hazard ratio (HR) = 3.12 and 3.09, respectively; P < 0.001]. Death-censored allograft survival was comparable between all groups. In AL amyloidosis, mortality was predicted by interventricular septum at end diastole (IVSd) thickness >12 mm (HR = 26.58; P = 0.03), while survival was predicted by haematologic response (very good partial or complete response; HR = 0.07; P = 0.018). In AA amyloidosis, recurrent amyloid was associated with elevated serum amyloid A concentration but not with outcomes. CONCLUSIONS: Renal transplantation outcomes for selected patients with AA and AL amyloidosis are comparable to those with DN. In AL amyloidosis, IVSd thickness and achievement of deep haematologic response pre-transplant profoundly impact patient survival.


Assuntos
Amiloidose/complicações , Falência Renal Crônica/mortalidade , Transplante de Rim/efeitos adversos , Idoso , Amiloidose/cirurgia , Feminino , Humanos , Falência Renal Crônica/etiologia , Falência Renal Crônica/patologia , Masculino , Pessoa de Meia-Idade , Prognóstico , Estudos Retrospectivos , Taxa de Sobrevida , Fatores de Tempo , Transplante Homólogo
5.
Transplant Proc ; 53(1): 329-334, 2021.
Artigo em Inglês | MEDLINE | ID: mdl-32917391

RESUMO

BACKGROUND: Indications for heart transplantation are expanding to include amyloid light chain (AL) and transthyretin-related (TTR) amyloidosis. Previously, AL amyloid had been a contraindication to heart transplantation given inferior outcomes. These patients typically have biventricular failure requiring mechanical circulatory support (MCS). We report the outcomes of patients with end-stage cardiac amyloidosis who underwent cardiac transplantation, including some who were bridged to transplantation with a durable biventricular MCS METHODS: The records for patients with cardiac amyloidosis who underwent cardiac transplant between 2010 and 2018 were reviewed. Primary endpoint was post-transplant 1-year survival. Secondary endpoints included 1-year freedom from cardiac allograft vasculopathy (as defined by stenosis ≥ 30% by angiography), nonfatal major adverse cardiac events (myocardial infarction, new congestive heart failure, percutaneous coronary intervention, implantable cardioverter defibrillator/pacemaker implant, stroke), and any rejection. RESULTS: A total of 46 patients received heart transplantation with a diagnosis of either AL or TTR amyloidosis. Of these, 7 patients were bridged to transplantation with a durable biventricular MCS device (6 AL, 1 TTR) and 39 patients were transplanted without MCS bridging. The MCS group consisted of 5 total artificial hearts and 2 biventricular assist devices. The 1-year survival was 91% for the entire cohort, 83% for those with AL amyloidosis, 94% for those with TTR amyloidosis, and 86% for those who received MCS bridging. CONCLUSIONS: Cardiac transplantation can be safely performed in selected amyloidosis patients with reasonable short-term outcomes. Those bridged to transplantation with biventricular MCS appear to have short-term outcomes similar to those transplanted without MCS. Larger numbers and longer observation are required to confirm these findings.


Assuntos
Amiloidose/complicações , Cardiopatias/etiologia , Cardiopatias/cirurgia , Transplante de Coração , Resultado do Tratamento , Adulto , Idoso , Amiloidose/cirurgia , Estudos de Coortes , Feminino , Transplante de Coração/efeitos adversos , Coração Auxiliar , Humanos , Masculino , Pessoa de Meia-Idade , Complicações Pós-Operatórias/etiologia , Estudos Retrospectivos
6.
Laryngoscope ; 131(8): 1810-1815, 2021 08.
Artigo em Inglês | MEDLINE | ID: mdl-33009850

RESUMO

OBJECTIVES: Laryngeal amyloidosis (LA) is a rare disease characterized by extracellular protein deposition within the larynx. Treatment is difficult due to the frequently submucosal and multifocal nature of disease. The mainstay of treatment is surgical resection; however, recurrence rates are high. Recently, use of radiotherapy (RT), either alone or postoperatively, for LA has been adapted from the management of extramedullary plasmacytoma and has been shown to provide local disease control. Here, we describe the experience with adjuvant RT for LA at our center. STUDY DESIGN: Retrospective case series. METHODS: Retrospective study of patients with amyloidosis of the larynx, with or without other disease sites, seen at a tertiary academic center between 2011 and 2019. Outcomes included disease characteristics, recurrence rates, treatment modalities, and pre- and posttreatment voice handicap index (VHI)-10. RESULTS: Ten patients met eligibility criteria. Mean follow-up time for all patients was 62.0 ± 41.0 months; mean follow-up time after last treatment was 51 ± 55 months. All but one patient underwent surgical resection of disease. Seven patients underwent subsequent RT. Of these seven, six underwent RT at our institution; five received a dose of 45 Gray (Gy); and one received a dose of 20 Gy. All seven completed RT without toxicity-related interruption. Patients undergoing RT underwent 2.1 ± 1.3 surgical procedures prior to RT; no patients required surgery after RT. Mean pretreatment VHI-10 was 22.9 ± 8.1; mean posttreatment VHI-10 was 12.9 ± 13.3. CONCLUSION: RT after surgery for LA can provide good local control without unacceptable toxicity and may decrease the need for further surgery. LEVEL OF EVIDENCE: 4 Laryngoscope, 131:1810-1815, 2021.


Assuntos
Amiloidose/radioterapia , Doenças da Laringe/radioterapia , Laringoscopia , Radioterapia Adjuvante/métodos , Adulto , Idoso , Amiloidose/cirurgia , Feminino , Seguimentos , Humanos , Doenças da Laringe/cirurgia , Laringe/efeitos da radiação , Laringe/cirurgia , Masculino , Pessoa de Meia-Idade , Recidiva , Estudos Retrospectivos , Resultado do Tratamento
7.
Kidney Int ; 99(3): 707-715, 2021 03.
Artigo em Inglês | MEDLINE | ID: mdl-32712168

RESUMO

Longer survival using modern therapies has increased the number of patients with immunoglobulin light-chain amyloidosis receiving kidney transplantation. We evaluated 60 patients with immunoglobulin light chain amyloidosis who underwent kidney transplantation based on their hematologic response for outcomes of death, graft failure, and complications. Patient hematologic responses (light-chain in blood or urine) prior to kidney transplantation were three patients had no response, five had a partial response, six had a very good partial response, 37 had a complete response, and nine were treatment-naive patients (never treated for this disorder). After transplantation, seven of nine treatment-naive patients achieved a complete response. The median follow-up for the entire transplant cohort was 61 months. The estimated median overall survival from the time of kidney transplantation was 123 months for the entire group. Median overall survival was not reached for the very good partial response plus complete response groups, it was 47 months for no response plus partial response groups, and 117 months for the treatment-naive group (all significantly different). Median overall survival of very good partial response was 81 months, while the median was not reached in the complete response group (no significant difference). The time to amyloid recurrence was significantly longer in complete response compared to very good partial response (median 181 vs 81 months). Death-censored graft survival at one- and five-years was 98.3%, and 95.8%, respectively for all groups. Of the 60 patients, three had allograft failure, 19 died with a functioning graft, and 13 had an amyloid recurrence. Thus, outcomes after kidney transplant in patients with immunoglobulin light-chain amyloidosis seem acceptable if a very good partial response or complete response is achieved either before or after transplantation.


Assuntos
Amiloidose , Amiloidose de Cadeia Leve de Imunoglobulina , Transplante de Rim , Amiloidose/diagnóstico , Amiloidose/cirurgia , Humanos , Cadeias Leves de Imunoglobulina , Amiloidose de Cadeia Leve de Imunoglobulina/diagnóstico , Amiloidose de Cadeia Leve de Imunoglobulina/terapia , Transplante de Rim/efeitos adversos , Recidiva Local de Neoplasia , Resultado do Tratamento
8.
J Card Surg ; 35(11): 3248-3250, 2020 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-32939861

RESUMO

We present a case of coronary artery bypass grafting in a 78-year-old man with triple vessel disease and concomitant cardiac amyloidosis. Postoperatively, he developed a profound low cardiac output state and multiorgan failure. He died 3 weeks following surgery. Bypass surgery is rarely performed in patients with cardiac amyloidosis, and there is little in the literature regarding outcomes. The few published cases present a bleak picture, and hence percutaneous coronary intervention should always be preferred.


Assuntos
Amiloidose/cirurgia , Cardiomiopatias/cirurgia , Ponte de Artéria Coronária/efeitos adversos , Ponte de Artéria Coronária/métodos , Doença da Artéria Coronariana/cirurgia , Idoso , Amiloidose/complicações , Baixo Débito Cardíaco/etiologia , Baixo Débito Cardíaco/prevenção & controle , Cardiomiopatias/complicações , Doença da Artéria Coronariana/complicações , Evolução Fatal , Humanos , Masculino , Insuficiência de Múltiplos Órgãos/etiologia , Insuficiência de Múltiplos Órgãos/prevenção & controle , Intervenção Coronária Percutânea , Complicações Pós-Operatórias/etiologia , Complicações Pós-Operatórias/prevenção & controle
9.
Transplantation ; 104(8): 1703-1711, 2020 08.
Artigo em Inglês | MEDLINE | ID: mdl-32732850

RESUMO

BACKGROUND: There are limited data on the outcome of transplant recipients with familial Mediterranean fever (FMF)-associated AA amyloidosis. The aim of the present study is to evaluate demographic, clinical, laboratory, and prognostic characteristics and outcome measures of these patients. METHODS: Eighty-one renal transplant recipients with FMF-associated AA amyloidosis (group 1) and propensity score-matched transplant recipients (group 2, n = 81) with nonamyloidosis etiologies were evaluated in this retrospective, multicenter study. Recurrence of AA amyloidosis was diagnosed in 21 patients (group 1a), and their features were compared with 21 propensity score-matched recipients with FMF amyloidosis with no laboratory signs of recurrence (group 1b). RESULTS: The risk of overall allograft loss was higher in group 1 compared with group 2 (25 [30.9%] versus 12 [14.8%]; P = 0.015 [hazard ratio, 2.083; 95% confidence interval, 1.126-3.856]). Patients in group 1 were characterized by an increased risk of mortality compared with group 2 (11 [13.6%] versus 0%; P = 0.001 [hazard ratio, 1.136; 95% confidence interval, 1.058-1.207]). Kaplan-Meier analysis revealed that 5- and 10-year patient survival rates in group 1 (92.5% and 70.4%) were significantly lower than in group 2 (100% and 100%; P = 0.026 and P = 0.023, respectively). Although not reaching significance, overall, 5- and 10-year graft survival rates (57.1%, 94.7%, and 53.8%, respectively) in group 1a were worse than in group 1b (76.2%, 95%, and 77.8%, respectively; P = 0.19, P = 0.95, and P = 0.27, respectively). CONCLUSIONS: AA amyloidosis is associated with higher risk of mortality after kidney transplantation. Inflammatory indicators should be monitored closely, and persistent high levels of acute-phase reactants should raise concerns about amyloid recurrence in allograft.


Assuntos
Amiloidose/cirurgia , Febre Familiar do Mediterrâneo/complicações , Rejeição de Enxerto/mortalidade , Falência Renal Crônica/cirurgia , Transplante de Rim/efeitos adversos , Adulto , Aloenxertos/imunologia , Aloenxertos/patologia , Amiloidose/imunologia , Amiloidose/mortalidade , Amiloidose/patologia , Biópsia , Febre Familiar do Mediterrâneo/imunologia , Febre Familiar do Mediterrâneo/mortalidade , Febre Familiar do Mediterrâneo/cirurgia , Feminino , Seguimentos , Rejeição de Enxerto/imunologia , Rejeição de Enxerto/patologia , Sobrevivência de Enxerto/imunologia , Humanos , Estimativa de Kaplan-Meier , Rim/imunologia , Rim/patologia , Falência Renal Crônica/imunologia , Falência Renal Crônica/mortalidade , Falência Renal Crônica/patologia , Masculino , Pessoa de Meia-Idade , Recidiva , Estudos Retrospectivos , Proteína Amiloide A Sérica/imunologia , Proteína Amiloide A Sérica/metabolismo , Taxa de Sobrevida , Resultado do Tratamento , Adulto Jovem
10.
Pacing Clin Electrophysiol ; 43(9): 913-921, 2020 09.
Artigo em Inglês | MEDLINE | ID: mdl-32573789

RESUMO

BACKGROUND: Cardiac amyloidosis is a progressive infiltrative disease involving deposition of amyloid fibrils in the myocardium and cardiac conduction system that frequently manifests with heart failure (HF) and arrhythmias, most frequently atrial fibrillation (AF), atrial flutter (AFL), and atrial tachycardia (AT). METHODS: We performed an observational retrospective study of patients with a diagnosis of cardiac amyloid who underwent catheter ablation at our institution between January 1, 2011 and December 1, 2018. Patient demographics, procedural characteristics, and outcomes were determined by manual chart review. RESULTS: A total of 13 catheter ablations were performed over the study period in patients with cardiac amyloidosis, including 10 AT/AF/AFL ablations and three atrioventricular nodal ablations. Left ventricular ejection fraction was lower at the time of AV node ablation than catheter ablation of AT/AF/AFL (23% vs 40%, P = .003). Cardiac amyloid was diagnosed based on the results of preablation cardiac MRI results in the majority of patients (n = 7, 70%). The HV interval was prolonged at 60 ± 15 ms and did not differ significantly between AV nodal ablation patients and AT/AF/AFL ablation patients (69 ± 18 ms vs 57 ± 14 ms, P = .36). The majority of patients undergoing AT/AF/AFL ablation had persistent AF (n = 7, 70%) and NYHA class II (n = 5, 50%) or III (n = 5, 50%) HF symptoms, whereas patients undergoing AV node ablation were more likely to have class IV HF (n = 2, 66%, P = .014). Arrhythmia-free survival in CA patients after catheter ablation of AT/AF/AFL was 40% at 1 year and 20% at 2 years. CONCLUSIONS: Catheter ablation of AT/AF/AFL may be a feasible strategy for appropriately selected patients with early to mid-stage CA, whereas AV node ablation may be more appropriate in patients with advanced-stage CA.


Assuntos
Amiloidose/cirurgia , Fibrilação Atrial/etiologia , Fibrilação Atrial/cirurgia , Cardiomiopatias/cirurgia , Ablação por Cateter/métodos , Idoso , Amiloidose/complicações , Cardiomiopatias/complicações , Ecocardiografia , Eletrocardiografia , Feminino , Humanos , Masculino , Recidiva , Estudos Retrospectivos
11.
World Neurosurg ; 140: 325-331, 2020 08.
Artigo em Inglês | MEDLINE | ID: mdl-32461181

RESUMO

BACKGROUND: Solitary spinal amyloidoma (SSA) is a rare and poorly characterized disease. There are few cases described, and the knowledge of this neoplasm is limited. A more accurate description of demographics, clinical findings, and outcomes may be useful for a better understanding of this pathology, as well as therapeutic intervention, adding value to the research of localized amyloidosis. METHODS: A systematic search was carried out from when registries began until February 2020. We also include a case diagnosed and treated in our department. Descriptive statistics were used to evaluate data, demographics, clinical findings, diagnostic modalities, therapeutics, and finally neurologic outcomes. The Kaplan-Meier method was used to assess overall survival and progression-free survival. RESULTS: The final cohort comprises 35 patients. The mean age at diagnosis was 61.97 years, and 68.60% of the patients were male. SSA developed more frequently in the thoracic spine (48.60%), followed by the cervical spine (17.10%). Intradural lesions were rare, and the average neoplastic score for spinal instability was 9.5 points. The most common symptoms were impaired motor function (74.29%) and axial back pain (65.70%). After surgery, neurologic recovery was reported in 82.90% of cases. Mean progression-free survival and mean overall survival were 47.26 and 156.66 months. CONCLUSIONS: SSA is a rare subgroup of localized amyloidosis, usually being diagnosed in male patients between the sixth and eighth decades. The gold standard treatment seems to be surgical resection. SSA patients have excellent long-term survival and a low rate of local recurrence.


Assuntos
Amiloidose/cirurgia , Coluna Vertebral/cirurgia , Amiloidose/patologia , Feminino , Humanos , Masculino , Doenças da Coluna Vertebral/patologia , Doenças da Coluna Vertebral/cirurgia , Coluna Vertebral/patologia , Resultado do Tratamento
12.
JACC Heart Fail ; 8(6): 461-468, 2020 06.
Artigo em Inglês | MEDLINE | ID: mdl-32387068

RESUMO

OBJECTIVES: The purpose of this study is to report outcomes after heart transplantation in patients with cardiac amyloidosis based on a large single-center experience. BACKGROUND: Cardiac amyloidosis causes significant morbidity and mortality, often leading to restrictive cardiomyopathy, progressive heart failure, and death. Historically, heart transplantation outcomes have been worse in patients with cardiac amyloidosis compared with other heart failure populations, in part due to the systemic nature of the disease. However, several case series have suggested that transplantation outcomes may be better in the contemporary era, likely in part due to the availability of more effective light chain suppressive therapies for light chain amyloidosis. METHODS: This study examined all patients seen between 2004 and 2017, either at the Stanford University Medical Center or the Kaiser Permanente Santa Clara Medical Center, who were diagnosed with cardiac amyloidosis and ultimately underwent heart transplantation. This study examined pre-transplantation characteristics and post-transplantation outcomes in this group compared with the overall transplantation population at our center. RESULTS: During the study period, 31 patients (13 with light chain amyloidosis and 18 with transthyretin [ATTR] amyloidosis) underwent heart transplantation. Patients with ATTR amyloidosis were older, were more likely to be male, had worse baseline renal function, and had longer waitlist times compared with both patients with light chain amyloidosis and the overall transplantation population. Post-transplantation, there were no differences in post-operative bleeding, renal failure, infection, rejection, or malignancy. There was no significant difference in mortality between patients who underwent heart transplantation for amyloid cardiomyopathy and patients who underwent heart transplantation for all other indications. CONCLUSIONS: In carefully selected patients with cardiac amyloidosis, heart transplantation can be an effective therapeutic option with outcomes similar to those transplanted for other causes of heart failure.


Assuntos
Amiloidose/complicações , Cardiomiopatias/complicações , Insuficiência Cardíaca/cirurgia , Transplante de Coração , Idoso , Amiloidose/diagnóstico , Amiloidose/cirurgia , Cardiomiopatias/diagnóstico , Cardiomiopatias/cirurgia , Feminino , Insuficiência Cardíaca/diagnóstico , Insuficiência Cardíaca/etiologia , Humanos , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Resultado do Tratamento , Listas de Espera
14.
J Oral Maxillofac Surg ; 78(9): 1572-1582, 2020 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-32442425

RESUMO

PURPOSE: Localized amyloidosis of the tongue is a benign condition in which surgical management may be considered. The aim of the study was to review the current literature and report a case. MATERIALS AND METHODS: We searched the PubMed database for all relevant articles reporting cases of localized tongue amyloidosis published between 1980 and February 2020. In addition, we updated 1 case diagnosed and treated in our department. RESULTS: A 49-year-old male patient presented with an asymptomatic tongue nodule of the dorsum mimicking median rhomboid glossitis. The results of an incisional biopsy showed an amyloid on Congo red staining and positive findings for the κ light chain by immunohistochemical analysis. The findings of the systemic workup were negative. Therefore, a diagnosis of localized κ light-chain amyloidosis was made. The patient underwent a resection of the lesion, and no recurrence or progression was observed during a period of 18 months. The literature review showed 12 reports describing 21 patients (11 men, 52.3%) with localized tongue amyloidosis. The most common clinical presentation was nodular with a single lesion of the tongue dorsum (15 patients, 71.4%). All cases showed positive findings on Congo red staining. Immunohistochemical analysis findings were available for only 9 patients (42.8%) and showed light-chain amyloidosis. No case showed any systemic involvement or the development of systemic disease. Surgical excision was performed in 9 cases, with recurrence at the site of operation in 2 cases. CONCLUSIONS: Localized amyloidosis of the tongue is a rare disease in which surgical excision may be therapeutic when a multidisciplinary evaluation does not show any systemic disease. We recommend an excision when the lesion is persistent or shows an enlargement or when discomfort is reported. In the case of any further local recurrence, resection may be repeated.


Assuntos
Amiloidose , Doenças da Língua , Amiloide , Amiloidose/diagnóstico , Amiloidose/cirurgia , Biópsia , Humanos , Masculino , Pessoa de Meia-Idade , Língua/cirurgia , Doenças da Língua/diagnóstico , Doenças da Língua/cirurgia
15.
Arch. Soc. Esp. Oftalmol ; 95(5): 244-247, mayo 2020. ilus
Artigo em Espanhol | IBECS | ID: ibc-198616

RESUMO

Mujer de 35 años con disminución progresiva de visión en ambos ojos sin otros síntomas asociados manifestó como único antecedente la muerte de su hermano por amiloidosis. La agudeza visual fue de cuenta dedos a 30 centímetros en el ojo derecho y 20/70 en el ojo izquierdo. En la oftalmoscopia del ojo derecho se observó un vítreo blanco, homogéneo que no permitió valorar detalles y en el ojo izquierdo un vítreo blanquecino con opacidades en forma de bandas en la región cortical. Se realizó vitrectomía en el ojo derecho y estudio con inmunotinciones que fueron positivos para material amiloide. Publicamos un caso de amiloidosis diagnosticada con tinciones específicas en muestra vítrea cuya manifestación inicial y única fue la presencia de opacidades vítreas. El procesamiento de estas muestras debe considerarse en pacientes con antecedentes familiares, opacidades vítreas tempranas, resultados negativos de biopsia sistémica o curso atípico de la enfermedad


A 35-year-old woman with progressive decrease of vision in both eyes and with no other associated symptoms, manifested as the only antecedent, the death of her brother due to amyloidosis. The visual acuity was counting fingers at 30 centimetres in the right eye and 20/70 in the left eye. In ophthalmoscopy of the right eye, a white, homogeneous vitreous, that was difficult to assess in detail, was observed, and in the left eye a whitish vitreous with band-like opacities in the cortical region. Vitrectomy was performed in the right eye, and a study with specific stains showed positive for amyloid material. A case is reported of amyloidosis diagnosed with specific stains in a vitreous sample for which the initial and only manifestation was the presence of vitreous opacities. The processing of these samples should be considered in patients with family history, early vitreous opacities, negative results of systemic biopsy, or atypical course of the disease


Assuntos
Humanos , Feminino , Adulto , Amiloidose/diagnóstico , Amiloidose/complicações , Corpo Vítreo , Oftalmopatias/etiologia , Acuidade Visual , Vitrectomia , Amiloidose/cirurgia , Oftalmoscopia , Corpo Vítreo/cirurgia , Coloração e Rotulagem , Corantes , Oftalmopatias/diagnóstico , Biópsia
16.
Curr Opin Organ Transplant ; 25(3): 211-217, 2020 06.
Artigo em Inglês | MEDLINE | ID: mdl-32304420

RESUMO

PURPOSE OF REVIEW: To deeply investigate one type of intracellular cardiac storage disease, the Fabry disease and one example of extracellular cardiac storage disease, the cardiac amyloidosis, with the aim to collect data about cardiac treatment at the end-stage level and the feasibility of heart transplantation (HTx) in this setting of patients. RECENT FINDINGS: Some registry describes that Fabry disease and cardiac amyloidosis showed similar characteristics as hypertrophic cardiomyopathies; thus, their correct diagnosis is often missing or time consuming. A multiorgan approach is mandatory to recognize the main systemic findings of these diseases, involving also the kidneys, the brain, the autonomous system and the skin. SUMMARY: The early diagnosis of these diseases is required to start as soon as possible the correct therapy for the patients affected. However, the end-stage heart failure is common and HTx could be offered to selected patients, especially if affected by light chain cardiac amyloidosis, to allow to perform the autologous stem cell transplantation after the cardiac transplant. Considering the Fabry disease patients, the enzyme recombinant therapy is also mandatory after HTx to avoid potential release of the systemic disease on the donor graft.


Assuntos
Amiloidose/cirurgia , Doença de Fabry/cirurgia , Cardiopatias/cirurgia , Transplante de Coração/métodos , Adulto , Idoso , Transplante de Células-Tronco Hematopoéticas , Humanos , Pessoa de Meia-Idade , Transplante Autólogo
18.
J Clin Ultrasound ; 48(3): 168-173, 2020 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-32003472

RESUMO

We describe the case of a 41-year-old woman with primary Sjögren's syndrome (SS) who presented multiple recurrences of breast amyloidosis. Each recurrence of breast amyloidosis showed different sonographic features, potentially mimicking malignancy. We briefly discuss the possible cause of this variability in imaging features based on the radiologic-histologic correlation.


Assuntos
Amiloidose/complicações , Amiloidose/diagnóstico por imagem , Doenças Mamárias/complicações , Doenças Mamárias/diagnóstico por imagem , Síndrome de Sjogren/complicações , Adulto , Amiloidose/patologia , Amiloidose/cirurgia , Doenças Mamárias/patologia , Doenças Mamárias/cirurgia , Feminino , Seguimentos , Humanos , Pessoa de Meia-Idade , Recidiva , Ultrassonografia/métodos
19.
World Neurosurg ; 138: 205-213, 2020 06.
Artigo em Inglês | MEDLINE | ID: mdl-32084617

RESUMO

BACKGROUND: Management of cerebral amyloidomas has lacked consensus owing to their rarity. We present a case and review 39 reported cases of amyloidomas in the literature, comparing their initial presentation, imaging characteristics, treatment, and progression. CASE DESCRIPTION: A 56-year-old woman presented with a history of intractable headaches and progressive speech difficulty. Imaging demonstrated a 1.7 × 2.6 cm left frontal subcortical hemorrhagic mass with an associated developmental venous anomaly, characteristics initially suggestive of a cavernous malformation. Diffusion tractography imaging was employed to avoid interference with the intact arcuate fasciculus and corticospinal tracts to minimize neurologic deficits. CONCLUSIONS: To our knowledge, this is the first documented case of the use of diffusion tractography in preoperative planning for cerebral amyloidoma resection. Our systematic review of 39 reported cases of amyloidomas in the literature provided insight regarding how these rare lesions have manifested and progressed and further understanding of current theories regarding their etiology and pathophysiology. Cerebral amyloidomas are rare, localized, proteinaceous aggregates with variable presentation and prognosis and no apparent relationship to systemic amyloidosis. Biopsy may be useful in determination of progression. Diffusion tractography is a valuable tool for minimizing complications associated with resection.


Assuntos
Amiloidose/cirurgia , Encefalopatias/cirurgia , Procedimentos Cirúrgicos Minimamente Invasivos/métodos , Procedimentos Neurocirúrgicos/métodos , Feminino , Lobo Frontal/patologia , Lobo Frontal/cirurgia , Humanos , Pessoa de Meia-Idade
20.
Ugeskr Laeger ; 182(3)2020 01 13.
Artigo em Dinamarquês | MEDLINE | ID: mdl-32052728

RESUMO

Localised amyloidosis in the urinary tract is a rare and often benign condition, which is usually clinically mistaken for malignancy. I this case report, a 48-year-old man was referred to the hospital with left flank pain, and CT-urography showed a tumour with calcification in the distal ureter. During transurethral procedure, the tumour was resected macroscopically. Histology revealed the diagnosis of amyloidosis. Follow-up was without signs of systemic involvement or recurrence. This case underlines the significance of preoperative diagnostic biopsy, thus saving the patient from unnecessary major surgery.


Assuntos
Amiloidose , Ureter , Neoplasias Ureterais , Amiloidose/diagnóstico , Amiloidose/cirurgia , Humanos , Masculino , Pessoa de Meia-Idade , Recidiva Local de Neoplasia , Urografia
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