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1.
Klin Monbl Augenheilkd ; 237(1): 35-40, 2020 Jan.
Artigo em Alemão | MEDLINE | ID: mdl-31968364

RESUMO

BACKGROUND: Primary localised orbital amyloidosis (PLOA) is a very rare disease. In contrast to the isolated manifestation, systemic involvement can be associated with potentially life-threatening consequences. However, the isolated involvement of the orbit can also lead to serious complications. MATERIAL AND METHODS: Two cases of PLOA are described and the necessary ophthalmic, internistic and immunohistochemical diagnostic testing are explained. RESULTS: The first case describes a 71-year-old woman with PLOA. In the clinic, a yellow-orange bumpy prominence in the nasal lower quadrant without further ophthalmological abnormalities was found. Extensive diagnostic testing found no systemic manifestation. The patient herself was free of complaints. In the follow-up over 4 years, patient showed slow progression without ocular complications. The second case is a 72-year-old male patient with similar clinical signs but localisation in the temporal superior quadrant. During the clinical course, multiple ophthalmological complications developed (ptosis, protrusio bulbi, diplopia, secondary glaucoma, perforated corneal ulcer in neurotrophic keratopathy). Perforating keratoplasty had to be performed. Fractioned radiotherapy led to stabilisation of the disease. The follow-up period was 4 years. CONCLUSION: PLOA can lead to visual and organ threatening complications. Accurate diagnosis is required for further diagnostic and therapeutic procedures and to counteract potential local and systemic complications. Interindividual differences in the course have to be considered.


Assuntos
Amiloidose , Blefaroptose , Exoftalmia , Ceratite , Idoso , Amiloidose/diagnóstico , Amiloidose/cirurgia , Blefaroptose/diagnóstico , Blefaroptose/cirurgia , Exoftalmia/diagnóstico , Exoftalmia/cirurgia , Feminino , Humanos , Ceratite/diagnóstico , Ceratite/cirurgia , Masculino , Órbita
3.
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
4.
Medicine (Baltimore) ; 98(38): e17256, 2019 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-31567998

RESUMO

RATIONALE: Cardiac amyloidosis, considered for the last years to be a rare disease, is one of the determinants of HFpEF. The non-specific clinical presentation and the difficulties related to endomyocardial biopsy have made cardiac amyloidosis an underdiagnosed clinical entity. Improvement of non-invasive diagnostic techniques and the development of new therapies increased clinical awareness for this form of restrictive cardiomyopathy. We here summarize echocardiography and Tc-HDP scintigraphy findings in 6 cases of cardiac amyloidosis and review the literature data of this progressive and fatal cardiomyopathy. PATIENTS CONCERNS: The main clinical manifestations were fatigue, low exercise tolerance and edemas. The right heart failure symptoms usually dominated the clinical picture. DIAGNOSES: All cases were evaluated by echocardiography; 3 cases were further examined by bone scintigraphy and 4 cases a peripheral biopsy was performed. Electrocardiography showed low-voltage QRS complexes and "pseudo-infarct" pattern in the precordial leads, contrary to the echocardiographic aspect, which revealed thickening of ventricle walls. Biatrial dilation and diastolic disfunction were observed. Impaired systolic function was detected in advanced stages of the disease. Tc-HDP scintigraphy revealed cardiac uptake of radiopharmaceutical and managed to confirm the diagnosis in 1 case of cardiac amyloidosis in which salivary gland biopsy was negative. INTERVENTIONS: The treatment was based on managing fluid balance, with the mainstream therapy represented by diuretics. Neurohormonal agents, usually used in heart failure treatment were avoided, due to poor tolerance and worsening of disease course. The management of these 6 cases was challenging due to the refractory manifestation of congestive heart failure. OUTCOMES: During follow-up, 4 of the 6 patients from the current study died in the first year after the final diagnosis was established. LESSONS: Nuclear imaging of cardiac amyloidosis has a revolutionary development nowadays. Bone scintigraphy presents promising results for identifying patients at early stages of disease and to differentiate between cardiac amyloidosis types. Further studies are necessary for the standardization of imaging protocol and development of non-invasive diagnostic tools, especially in assessing the response to treatment and disease progression, for which little is known.


Assuntos
Amiloidose/diagnóstico por imagem , Ecocardiografia , Cardiopatias/diagnóstico por imagem , Cintilografia/métodos , Adulto , Idoso , Idoso de 80 Anos ou mais , Amiloidose/diagnóstico , Amiloidose/patologia , Difosfonatos , Feminino , Coração/diagnóstico por imagem , Cardiopatias/diagnóstico , Cardiopatias/patologia , Humanos , Masculino , Pessoa de Meia-Idade , Miocárdio/patologia , Compostos de Organotecnécio
5.
Medicina (B Aires) ; 79(4): 276-279, 2019.
Artigo em Espanhol | MEDLINE | ID: mdl-31487247

RESUMO

There is limited epidemiological information on AA amyloidosis in Argentina, so the objective of this study was to describe the epidemiological characteristics of this disease in a tertiary hospital in our country. We designed a prospective clinical cohort of all consecutive patients with AA amyloidosis confirmed by immunohistochemistry in tissue from the Institutional Registry of Amyloidosis of the Hospital Italiano de Buenos Aires, in the period 04/01/2012- 12/31/2017. Of the 121 patients in the registry, 18 were included with AA for the analysis. Of the total included, 50% (9) were female, with a median age of 53.5 (interquartile range, RII 46-61) years. The 88.9% (16) of cohort presented renal compromise, all had proteinuria, and 6 required dialysis. Six had amyloid infiltration of the digestive system. The latency between the onset of the underlying disease and the diagnosis of AA had a median of 27 (RII 8-35) years. The underlying disease was of inflammatory origin in 6 cases. In 50% (9) of the patients the cause of AA amyloidosis was unknown. In the remaining 50%, these causes resemble those observed in developed countries. Furthermore, our results highlight the importance of their differential diagnosis to identify the most appropriate treatment or follow-up according to the situation presented by each patient.


Assuntos
Amiloidose/diagnóstico , Proteína Amiloide A Sérica/análise , Adulto , Idoso , Argentina , Estudos de Coortes , Feminino , Humanos , Imuno-Histoquímica , Masculino , Pessoa de Meia-Idade , Estudos Prospectivos
6.
Expert Rev Cardiovasc Ther ; 17(9): 673-681, 2019 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-31478389

RESUMO

Introduction: Cardiac amyloidosis is a disorder caused by the accumulation of abnormal protein products, amyloid, in the myocardium which subsequently impairs normal heart function. Heart failure with preserved ejection fraction has been increasingly attributed to amyloidosis and the resultant restrictive cardiomyopathy it creates. Areas covered: Amyloid transthyretin (ATTR) is one of several identified amyloid products that have been pathologically implicated in cardiac amyloidosis through advanced diagnostics. Improvements in nuclear imaging techniques, particularly scintigraphy, have enabled non-invasive diagnosis where previously endomyocardial biopsy was the only option. Despite being considered a rare disease, it is likely that ATTR cardiac amyloidosis is an underdiagnosed condition which has been supported by autopsy findings in heart failure populations. This article will review ATTR cardiac amyloidosis to provide physicians with the tools they need to establish a definitive diagnosis when there is a clinical suspicion of amyloidosis and provide the most appropriate care. Expert commentary: Increased awareness and improved diagnostic techniques will lead to earlier diagnosis and a greater understanding of the clinical presentation. The anticipated increases in the prevalence of this disease due to increased clinical awareness will require, and in-part, facilitate the development of new therapies to manage this patient population.


Assuntos
Amiloide/metabolismo , Amiloidose/diagnóstico , Insuficiência Cardíaca/diagnóstico , Pré-Albumina/metabolismo , Humanos , Miocárdio/patologia , Cintilografia
7.
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
8.
Acta méd. costarric ; 61(3): 131-133, jul.-sep. 2019. graf
Artigo em Espanhol | LILACS | ID: biblio-1019304

RESUMO

Resumen La amiloidosis sistémica constituye una enfermedad poco frecuente, donde la infiltración cardíaca es la principal causa de morbimortalidad, sin importar la causa subyacente del depósito amiloide. Se reporta el caso de una paciente femenina de 48 años con síndrome nefrótico, insuficiencia cardíaca e inmunocompromiso, estableciéndose el diagnóstico de amiloidosis primaria con infiltración cardíaca secundaria a mieloma múltiple. Se discute brevemente la enfermedad, la importancia del juicio clínico apoyado en medios diagnósticos y los retos terapéuticos actuales.


Abstract Systemic amyloidosis constitutes a non common disease in which cardiac involvement is the leading cause of morbidity and mortality, regardless of the underlying pathogenesis of amyloid production. We present the case of a 48 years old female with nephrotic syndrome, heart failure and immunocompromise in which Primary Amyloidosis with cardiac involvement secondary to Multiple Myeloma is established as diagnosis. The disease is briefly discussed, as well as the value of clinical judgment supported on diagnostic means and the therapeutic challenges now days.


Assuntos
Humanos , Feminino , Pessoa de Meia-Idade , Cardiomiopatia Restritiva/complicações , Insuficiência Cardíaca/complicações , Insuficiência Cardíaca/diagnóstico por imagem , Amiloidose/diagnóstico , Costa Rica , Amiloidose de Cadeia Leve de Imunoglobulina/complicações
9.
BMJ Case Rep ; 12(7)2019 Aug 01.
Artigo em Inglês | MEDLINE | ID: mdl-31371336

RESUMO

In this case report, we present a healthy man who was referred for removal of subconjunctival yellow lesions found during a routine eye examination. In histopathological examination, an amyloidosis was found. There were no remnants or new lesions during 1-year follow-up. There was no systemic involvement. Conjunctival amyloidosis is a rare diagnosis that often is overlooked. Failure to recognise conjunctival amyloidosis might lead to late diagnosis of systemic amyloidosis. This case may rise the awareness to this rare diagnosis.


Assuntos
Amiloidose/diagnóstico , Doenças da Túnica Conjuntiva/diagnóstico , Amiloidose/terapia , Doenças da Túnica Conjuntiva/terapia , Humanos , Masculino , Pessoa de Meia-Idade
19.
Pan Afr Med J ; 32: 158, 2019.
Artigo em Francês | MEDLINE | ID: mdl-31303928

RESUMO

Renal amyloidosis is a rare complication of adult onset Still's disease. We here report three cases of renal amyloidosis in a series of 33 cases of adult onset Still's disease. The three patients enrolled had an average age of 43 years (with a range from 33 to 58 years). The diagnosis of Still's disease was retained on the basis of febrile polyarthritis (3 cases) associated with fleeting rush (1 case), biologic inflammatory syndrome in the absence of any infectious, inflammatory or neoplastic causes. All patients were treated with corticosteroids secondarily associated with methotrexate due to destructive polyarthritis (2 cases) and to a recurrence (1 case). Renal amyloidosis had occurred 4.9 years after Still's disease (with a range from 33 months to 7 years). Amyloidosis was revealed by nephrotic syndrome (3 cases) associated with renal failure (1 case). Diagnosis was based on renal puncture biopsy (3 cases) which showed AA amyloidosis (2 cases) and untyped amyloidosis (1 case). All patients received colchicine. Outcome was favorable in a female patient while in the other two patients the disease progressed to chronic renal failure. Renal amyloidosis uncommonly results from adult onset Still's disease. Once the disease gets established it can be life-threatening.


Assuntos
Amiloidose/etiologia , Síndrome Nefrótica/etiologia , Doença de Still de Início Tardio/complicações , Corticosteroides/administração & dosagem , Adulto , Amiloidose/diagnóstico , Amiloidose/tratamento farmacológico , Biópsia por Agulha , Colchicina/administração & dosagem , Progressão da Doença , Feminino , Humanos , Falência Renal Crônica/etiologia , Metotrexato/administração & dosagem , Pessoa de Meia-Idade , Insuficiência Renal/etiologia , Doença de Still de Início Tardio/diagnóstico , Doença de Still de Início Tardio/tratamento farmacológico
20.
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
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