Your browser doesn't support javascript.
Mostrar: 20 | 50 | 100
Resultados 1 - 20 de 5.047
Filtrar
3.
Medicine (Baltimore) ; 98(46): e17999, 2019 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-31725668

RESUMO

INTRODUCTION: Heavy and light chain amyloidosis is an extremely rare condition. There are few reports referring to the clinical impact of cardiac involvement in heavy and light chain amyloidosis, and the significance of myocardial impairment has not yet been completely explained. PATIENT CONCERNS: A 66-year-old Japanese man was admitted to our hospital presenting with nephrotic syndrome and congestive heart failure. DIAGNOSIS: Kidney and endoscopic gastric mucosal biopsy demonstrated congophilic hyalinization in most of the glomeruli and surrounding vessel walls, which were highly positive for immunoglobulin A and lambda. Finally, the patient was diagnosed as an atypical multiple myeloma with systemic heavy and light chain amyloidosis. INTERVENTIONS: The patient was referred to hematology for further treatment and was moved to another hospital for the administration of chemotherapy using melphalan and dexamethasone. OUTCOMES: The patient was still alive after 15-month follow-up from the initial diagnosis. CONCLUSION: Initial screening and follow-up for cardiac involvement are important for heavy and light chain amyloidosis. Further investigation for the prognosis of heavy and light chain amyloidosis is required to improve the strategies of diagnosis and treatment options for patients with this disease.


Assuntos
Amiloidose/complicações , Insuficiência Cardíaca/complicações , Cadeias Pesadas de Imunoglobulinas/sangue , Cadeias Leves de Imunoglobulina/sangue , Síndrome Nefrótica/complicações , Idoso , Amiloidose/patologia , Insuficiência Cardíaca/patologia , Humanos , Amiloidose de Cadeia Leve de Imunoglobulina/complicações , Amiloidose de Cadeia Leve de Imunoglobulina/patologia , Masculino , Síndrome Nefrótica/patologia
6.
Vestn Otorinolaringol ; 84(4): 48-50, 2019.
Artigo em Russo | MEDLINE | ID: mdl-31579058

RESUMO

Amyloidosis of the larynx is rare in the pediatric age group. We present a clinical case of laryngeal amyloidosis manifesting as false vocal fold bulging in a thirteen-year-old girl with hoarseness and with initial suspicion of laryngeal tumor. Computed tomography and ultrasound scanning of the larynx were useful in determining the laryngeal process size along with fibro laryngoscopy. There was performed a microsurgical removal of the larynx formation. The results of Congo red staining of the formation were characteristic of amyloid. Systemic manifestations of the disease were not revealed.


Assuntos
Amiloidose , Doenças da Laringe , Adolescente , Amiloidose/complicações , Amiloidose/diagnóstico , Amiloidose/cirurgia , Criança , Diagnóstico Diferencial , Feminino , Rouquidão , Humanos , Doenças da Laringe/complicações , Doenças da Laringe/diagnóstico , Doenças da Laringe/cirurgia , Neoplasias Laríngeas/diagnóstico , Laringoscopia
8.
Pol Merkur Lekarski ; 47(278): 67-69, 2019 Aug 30.
Artigo em Inglês | MEDLINE | ID: mdl-31473755

RESUMO

Heart failure in patients with rheumatoid arthritis (RA) caused by secondary amyloidosis is now extremely rare. A CASE REPORT: A 42 year old female patient with rheumatoid arthritis was admitted to our cardiology unit to diagnose and find the cause of her heart failure. Echocardiography showed marked diastolic dysfunction, hypertrophic cardiomyopathy and global longitudinal strain characteristic for cardiac amyloidosis. However, the suspicion of secondary amyloidosis related to her RA was excluded based on negative results of anti-SAA test. Cardiac MRI showed typical changes for cardiac amyloidosis in agreement with the primary echocardiography. Measurement of serum free light chain ratio revealed pattern typical for light chain amyloidosis secondary to multiple myoloma, confirmed by plasmocytosis on bone marrow biopsy and histopathology of salivary gland. CONCLUSIONS: In patients with cardiac amyloidosis, despite strong clinical suggestions, the definite diagnosis should be always established because it may allow to implement effective treatment.


Assuntos
Amiloidose , Cardiomiopatias , Cardiomiopatia Hipertrófica , Insuficiência Cardíaca , Adulto , Amiloidose/complicações , Amiloidose/diagnóstico , Ecocardiografia , Feminino , Insuficiência Cardíaca/etiologia , Humanos
10.
Rinsho Ketsueki ; 60(7): 791-796, 2019.
Artigo em Japonês | MEDLINE | ID: mdl-31391368

RESUMO

Here, we present the case of a 72-year-old male who presented with swelling, stiffness, and dysesthesia in the bilateral fingers, wrists, and ankles. Although rheumatoid arthritis was initially suspected, laboratory tests were negative for rheumatoid factor (RF) and anti-cyclic citrullinated peptide (CCP) antibody. Based on the findings of immune globulin G (IgG)-λ M proteins and 26% plasma cells in the bone marrow, multiple myeloma was diagnosed. Joint sonography revealed thickening of the tendon synovial sheaths around the bilateral wrist joints, palmar flexor tendon sheaths, and extensor digitorum tendon sheaths, and magnetic resonance imaging (MRI) revealed soft tissue masses around the bilateral hip joints. Carpal tunnel syndrome associated with amyloid arthritis was suspected. Amyloid deposits were observed in synovectomy specimens, and the patient was then diagnosed with amyloid arthritis. He had concurrent pulmonary fibrosis, and treatment with lenalidomide/dexamethasone (Ld therapy) was initiated. The symptoms in the bilateral fingers, wrists, and ankles improved with the treatment course, and joint sonography revealed that thickening of tendon sheath and soft tissue masses disappeared after seven courses of Ld therapy. However, MRI still revealed soft tissue masses around the bilateral hip joints. In patients with joint symptoms that do not fulfill the diagnostic criteria for rheumatoid arthritis, differentiation with amyloid arthritis is necessary.


Assuntos
Amiloidose/complicações , Mieloma Múltiplo/diagnóstico , Idoso , Anticorpos Anti-Proteína Citrulinada , Artrite Reumatoide , Diagnóstico Diferencial , Humanos , Imagem por Ressonância Magnética , Masculino , Mieloma Múltiplo/complicações
11.
Med Hypotheses ; 130: 109269, 2019 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-31383341

RESUMO

Colchicine is the first choice of the treatment in familial Mediterranean fever (FMF). However, in FMF patients with amyloidosis, especially during creatinine level >1.5 mg/dL and nephrotic range proteinuria, colchicine may be ineffective. Interleukin-1 (IL-1) blockers could be used in colchicine resistant cases. However, starting IL-1 blocker treatment after colchicine failure may lose opportunity for effective treatment. Therefore, administering IL-1 blocker together with colchicine as first line therapy may increase the chance for suppressing the disease.


Assuntos
Amiloidose/tratamento farmacológico , Colchicina/administração & dosagem , Febre Familiar do Mediterrâneo/tratamento farmacológico , Proteína Antagonista do Receptor de Interleucina 1/farmacologia , Interleucina-1/antagonistas & inibidores , Amiloidose/complicações , Colchicina/farmacologia , Creatinina/farmacologia , Febre Familiar do Mediterrâneo/complicações , Humanos , Proteinúria/complicações , Recidiva , Resultado do Tratamento
12.
Transplant Proc ; 51(7): 2292-2294, 2019 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-31400972

RESUMO

Familial Mediterranean fever (FMF) is an important and preventable cause of chronic kidney disease due to secondary amyloidosis. Although colchicine is the first-line therapy in patients with FMF with 60% to 65% complete remission rates, 5% to 10% of patients are colchicine-resistant and 5% to 10% of them are intolerant to the therapy. Anti-interleukin-1 agents, such as anakinra and canakinumab, are safe and efficient therapeutic options in patients with colchicine resistance or intolerance. However, the data on management of these targeted agents is limited in recipients of kidney transplant (RKT). In this case series, we aim to share our experience on canakinumab therapy of 4 RKTs with FMF-related amyloidosis, who were followed up in our clinic between 2010 and 2017. All of the 4 patients with end-stage renal disease were colchicine- resistant and on other alternative therapies, which provided poor disease control. For efficient control of secondary amyloidosis, canakinumab therapy was initiated in 1 of the patients before the renal transplant, and for the remaining patients after renal transplant. Any serious adverse effect, development of proteinuria, or graft dysfunction has not been observed in any of the patients. Under the canakinumab treatment, complete clinical responses, prevent typical familial Mediterranean fever attacks with fever and arthritis and abdominal pain, normalized serum amyloid A and C-reactive protein levels were achieved in all patients. Canakinumab treatment is a safe and effective therapeutic option for RKTs with FMF who are resistant or intolerant to colchicine and anakinra.


Assuntos
Amiloidose/tratamento farmacológico , Anticorpos Monoclonais Humanizados/uso terapêutico , Febre Familiar do Mediterrâneo/tratamento farmacológico , Falência Renal Crônica/tratamento farmacológico , Transplante de Rim , Adulto , Amiloidose/complicações , Amiloidose/cirurgia , Febre Familiar do Mediterrâneo/complicações , Febre Familiar do Mediterrâneo/cirurgia , Feminino , Humanos , Proteína Antagonista do Receptor de Interleucina 1/uso terapêutico , Falência Renal Crônica/etiologia , Falência Renal Crônica/cirurgia , Masculino , Período Pós-Operatório , Resultado do Tratamento
13.
Am J Case Rep ; 20: 933-936, 2019 Jun 30.
Artigo em Inglês | MEDLINE | ID: mdl-31256189

RESUMO

BACKGROUND Intracardiac thrombosis has been known to be associated with not only hepatocellular carcinoma but also with amyloidosis and use of a cardiac implantable electronic device. We report a case of a continuous tumor thrombus with hepatocellular carcinoma from the portal vein and hepatic vein to the right atrium via the inferior vena cava in a patient with a cardiac amyloidosis and an implanted cardiac resynchronization therapy (CRT) device. CASE REPORT A 68-year-old female first admitted to our hospital because of heart failure with an AL type primary cardiac amyloidosis. After 3 years, she underwent an implantation of a CRT device for biventricular pacing following repeated episodes of heart failure and low left ventricular ejection fraction of 34% with NYHA class III. Again, she presented with symptoms of heart failure and cardiomegaly on chest x-ray at 7 years after the CRT device implantation. The echocardiography showed a huge echogenic mass occupying the right atrium, and 64 multi-detector computed tomography showed a lobulated heterogeneously enhancing mass of hepatocellular carcinoma in the right upper lobe of her liver and a continuous tumor thrombus from the portal vein and hepatic vein to the right atrium via the inferior vena cava. CONCLUSIONS Intracardiac thrombosis and heart failure occurred in a patient with hepatocellular carcinoma and cardiac amyloidosis, who had an implanted CRT device, which resulted not only in hypercoagulability by the hepatocellular carcinoma itself and the accumulation of various risk factors, but also the progression of myocardial damage with the development of amyloidosis.


Assuntos
Amiloidose/complicações , Carcinoma Hepatocelular/complicações , Cardiopatias/complicações , Insuficiência Cardíaca/complicações , Neoplasias Hepáticas/complicações , Trombose/complicações , Idoso , Amiloidose/cirurgia , Dispositivos de Terapia de Ressincronização Cardíaca , Ecocardiografia , Feminino , Cardiopatias/cirurgia , Insuficiência Cardíaca/cirurgia , Humanos , Fatores de Risco , Trombofilia , Trombose/cirurgia , Tomografia Computadorizada por Raios X
17.
Clin Nucl Med ; 44(9): e503-e509, 2019 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-31283608

RESUMO

PURPOSE: The aims of this study were to evaluate the diagnostic performance of F-florbetaben PET/CT for detecting amyloid deposits in patients with multiple myeloma (MM) and to identify the optimal PET analysis method. METHODS: Fourteen patients with MM were prospectively enrolled (6 with amyloidosis, 8 control subjects). Dynamic imaging of the kidneys was performed for 20 minutes, and the retention ratio was obtained. At 90 minutes after injection, PET was performed. All images were assessed qualitatively and quantitatively, and the SUVmax, SUVmean, and SUVratio were obtained. Variables were compared between the amyloidosis group and the control group. Amyloid deposition was confirmed according to international consensus guidelines. RESULTS: Tracer uptake was abnormal in all patients with amyloidosis. The visual detection rate was excellent (100%) in the heart, stomach, and tongue but limited in the kidneys (50%) and poor (0%) in the esophagus, liver, and colon. F-florbetaben PET/CT identified 13 unexpected cases of abnormal uptake, confirming further amyloid deposition. Both spherical and manual volumes of interest showed similar diagnostic performance when evaluating amyloidosis in target organs. There was no significant difference in diagnostic performance between the SUVmax, SUVmean, and SUVratio. CONCLUSIONS: F-florbetaben PET/CT can accurately detect systemic amyloid deposits in patients with MM. F-florbetaben PET/CT was particularly useful in the heart, stomach, and tongue but of limited value in the esophagus, liver, and colon. F-florbetaben PET/CT can provide clinical information on organ involvement and could replace pathologic examination for diagnosis of amyloidosis in the future.


Assuntos
Amiloidose/complicações , Amiloidose/diagnóstico por imagem , Compostos de Anilina , Mieloma Múltiplo/complicações , Tomografia Computadorizada com Tomografia por Emissão de Pósitrons , Estilbenos , Idoso , Estudos de Casos e Controles , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Estudos Prospectivos
18.
BMJ Case Rep ; 12(6)2019 Jun 29.
Artigo em Inglês | MEDLINE | ID: mdl-31256048

RESUMO

A previously healthy 54-year-old woman was admitted to the stroke unit with an acute ischaemic stroke attributed to atrial fibrillation newly diagnosed at the emergency room. Nevertheless, preliminary investigation on stroke aetiology revealed incidental hypoalbuminaemia in the context of nephrotic syndrome, while clinically, the patient developed progressive signs of cardiac failure raising the suspicion of an underlying disorder. Systemic amyloidosis was histologically confirmed a few weeks after hospital admission. The rare presentation and non-specific symptom constellation contributed to delayed institution of the appropriated treatment regimen at a point where multiorganic involvement was irreversible leading to death only 2 months after the first manifestation. The presented case reminds us of the importance of always keeping in mind this rarer cause of ischaemic stroke since an early diagnosis remains the key to a more hopeful prognosis.


Assuntos
Amiloidose/complicações , Amiloidose/diagnóstico , Isquemia Encefálica/diagnóstico por imagem , Isquemia Encefálica/etiologia , Acidente Vascular Cerebral/diagnóstico por imagem , Acidente Vascular Cerebral/etiologia , Amiloidose/patologia , Biópsia , Encéfalo/diagnóstico por imagem , Evolução Fatal , Feminino , Humanos , Rim/patologia , Imagem por Ressonância Magnética , Pessoa de Meia-Idade , Tomografia Computadorizada por Raios X
SELEÇÃO DE REFERÊNCIAS
DETALHE DA PESQUISA