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1.
Medicine (Baltimore) ; 99(6): e18978, 2020 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-32028407

RESUMO

RATIONALE: AA amyloidosis (AA) is caused by a wide variety of inflammatory states, but is infrequently associated with Castleman disease (CD). CD describes a heterogeneous group of hematologic disorders that share characteristic lymph node histopathology. CD can present with a solitary enlarged lymph node (unicentric CD, UCD) or with multicentric lymphadenopathy (MCD), constitutional symptoms, cytopenias, and multiple organ dysfunction due to an interleukin-6 driven cytokine storm. PATIENT CONCERNS: We are reporting a case of a 26-year-old woman with no significant past medical history who presented with a 3-month history of fatigue and an unintentional 20-pound weight loss. DIAGNOSIS: A CT-scan of the abdomen and pelvis revealed hepatosplenomegaly and a mesenteric mass. Congo Red staining from a liver biopsy showed apple-green birefringence and serum markers were suggestive of an inflammatory process. Post-excision examination of the resected mass revealed a reactive lymph node with follicular hyperplasia with kappa and lambda stains showing polyclonal plasmacytosis consistent with CD. INTERVENTIONS: The patient underwent surgery to remove the affected lymph node. OUTCOMES: IL-6, anemia, leukocytosis, and thrombocytosis resolved or normalized 2 weeks after resection; creatinine normalized 9 months postsurgery. Twenty two months post-surgery her IFN-γ normalized, her fatigue resolved, her proteinuria was reduced by >90% and she had returned to her baseline weight. LESSONS: Our case and literature review suggest that patients presenting with UCD or MCD along with organ failure should prompt consideration of concurrent AA amyloidosis.


Assuntos
Amiloidose/etiologia , Hiperplasia do Linfonodo Gigante/complicações , Doenças Peritoneais/etiologia , Adulto , Amiloidose/sangue , Amiloidose/diagnóstico , Amiloidose/diagnóstico por imagem , Hiperplasia do Linfonodo Gigante/sangue , Hiperplasia do Linfonodo Gigante/diagnóstico , Fadiga/etiologia , Feminino , Humanos , Doenças Peritoneais/sangue , Doenças Peritoneais/diagnóstico , Doenças Peritoneais/diagnóstico por imagem , Proteína Amiloide A Sérica/análise , Tomografia Computadorizada por Raios X , Perda de Peso
3.
Cardiol Clin ; 37(4): 487-495, 2019 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-31587789

RESUMO

Light chain amyloidosis is a deadly disease in which a monoclonal plasma cell dyscrasia produces misfolded immunoglobulin light chains (AL) that aggregate and form rigid amyloid fibrils. The amyloid deposits infiltrate one or more organs, leading to injury and severe dysfunction. The degree of cardiac involvement is a major driver of morbidity and mortality. Early diagnosis and treatment are crucial to prevent irreversible end-organ damage and improve overall survival. Treatment of AL cardiac amyloidosis involves eliminating the underlying plasma cell dyscrasia with chemotherapy and pursuing supportive heart failure management.


Assuntos
Amiloidose , Cardiomiopatias , Gerenciamento Clínico , Diagnóstico Precoce , Paraproteinemias/complicações , Amiloidose/diagnóstico , Amiloidose/etiologia , Amiloidose/terapia , Cardiomiopatias/diagnóstico , Cardiomiopatias/etiologia , Cardiomiopatias/terapia , Humanos , Paraproteinemias/diagnóstico
4.
Neurology ; 93(17): e1587-e1596, 2019 10 22.
Artigo em Inglês | MEDLINE | ID: mdl-31511348

RESUMO

OBJECTIVE: To elucidate the clinical characteristics of acquired ATTR amyloidosis after domino liver transplantation (DLT) with liver grafts explanted from patients with hereditary variant ATTR (ATTRv) amyloidosis. METHODS: We evaluated the presence of amyloid deposits and clinical symptoms in 30 recipients of domino liver transplants (24 men and 6 women) who underwent DLT with liver grafts explanted from patients with ATTRv amyloidosis. We analyzed symptoms and measures of 7 cases of symptomatic acquired ATTR amyloidosis and compared those with 30 patients with ATTRv amyloidosis who were the domino liver donors. We also reviewed the literature on case studies of acquired ATTR amyloidosis. RESULTS: We found amyloid deposition in 13 of our 30 domino liver recipients. A Kaplan-Meier analysis estimated that the median time from DLT to the first detection of amyloid was 8.5 years. In the literature review, the mean time was 7.3 years, with a wide range of 0.5-13 years. Our 7 symptomatic cases and the literature cases with acquired ATTR amyloidosis presented with clinical features that differed from patients with ATTRv amyloidosis who were the domino liver donors. Patients with acquired ATTR amyloidosis showed markedly milder autonomic disturbance, which is one of the main symptoms of ATTRv amyloidosis. CONCLUSIONS: Careful monitoring is required for DLT recipients of ATTRv liver grafts because the time from DLT to disease onset has a wide range and the clinical picture of these DLT recipients is distinct from that of liver donors.


Assuntos
Amiloidose/etiologia , Transplante de Fígado , Complicações Pós-Operatórias , Adolescente , Adulto , Feminino , Humanos , Hepatopatias/cirurgia , Masculino , Pessoa de Meia-Idade , Adulto Jovem
5.
Ren Fail ; 41(1): 850-854, 2019 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-31498016

RESUMO

Previous reports of glomerular disease in adult patients with autosomal dominant dystrophic epidermolysis bullosa (EB) are limited and include post-infectious glomerulonephritis, IgA nephropathy, amyloidosis, and leukocytoclastic vasculitis. To our knowledge, membranoproliferative glomerulonephritis (MPGN) has not been described before. We report a case of a 39-year-old male with autosomal dominant dystrophic EB, presenting with bilateral leg swelling of one-week duration. There was no other significant past medical history. The physical examination was remarkable for scars and erosions over all body areas, with all extremities with blisters and ulcers covered, absent finger and toenails and bilateral lower extremity edema. Serum creatinine was 0.9 mg/dL, albumin 1.3 g/dL and urine protein excretion 3.7 g/24 h. Viral markers (hepatitis-B, C, and HIV), complement c3 and c4 levels and auto-immune antibody profile all remained negative or within normal limits. Renal ultrasound and echocardiogram were normal. Renal biopsy recovered 14 glomeruli, all with proliferation of mesangial and endothelial cells as well as an expansion of the mesangial matrix, focal segmental sclerosis and amorphous homogeneous deposits demonstrating apple-green birefringence under polarized light with Congo red stain. Our observation emphasizes the importance of recognizing MPGN and secondary amyloidosis in patients with EB, especially with the availability of newer treatment modalities.


Assuntos
Amiloidose/diagnóstico , Epidermólise Bolhosa Distrófica/complicações , Glomerulonefrite Membranoproliferativa/diagnóstico , Glomérulos Renais/patologia , Adulto , Amiloidose/etiologia , Amiloidose/patologia , Biópsia , Diagnóstico Diferencial , Glomerulonefrite Membranoproliferativa/etiologia , Glomerulonefrite Membranoproliferativa/patologia , Humanos , Masculino , Nefrose Lipoide/diagnóstico , Esclerose
6.
Transplant Proc ; 51(7): 2289-2291, 2019 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-31400971

RESUMO

INTRODUCTION: Amyloid A amyloidosis is most commonly caused by familial Mediterranean fever (FMF) in Turkey. Amyloidosis secondary to FMF is an important cause of end-stage renal failure, and kidney transplantation (KT) in these cases can be complicated, with long-term results oftentimes inferior compared with organ transplant in patients without FMF. The present study aims to show the long-term results of patients with secondary amyloidosis caused by FMF undergoing KT . METHODS: We enrolled 27 patients with a history of FMF amyloidosis undergoing KT and a control group of 614 patients undergoing KT between 2005 and 2018 at Ankara University Medical School. All data were recorded retrospectively from patients files. RESULTS: Twenty-two patients (81.5%) were treated with triple immunosuppressive therapy consisting of mycophenolate mofetil, tacrolimus, and a steroid; 5 patients (18.5%) were treated with tacrolimus, azathioprine, and prednisolone. Acute cellular rejection was seen in 3 patients (11.1%), and acute cellular- and antibody-mediated rejection occurred in 1 patient (3.7%). During the follow-up period, graft loss due to acute cellular rejection was observed in only 1 patient. One patient was lost to follow-up.


Assuntos
Amiloidose/etiologia , Febre Familiar do Mediterrâneo/complicações , Rejeição de Enxerto/epidemiologia , Falência Renal Crônica/cirurgia , Transplante de Rim/efeitos adversos , Adulto , Feminino , Rejeição de Enxerto/etiologia , Humanos , Falência Renal Crônica/etiologia , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Resultado do Tratamento , Turquia
7.
Medicine (Baltimore) ; 98(35): e16830, 2019 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-31464908

RESUMO

RATIONALE: Amyloidosis accounts for 2% of head and neck tumors. Amyloidosis that develops in the head and neck region is localized amyloidosis. Multifocal amyloidosis in the head and neck region is extremely rare. PATIENT CONCERNS: The patient presented to the clinic of otolaryngology with nasal obstruction, anosmia and left neck mass for several months. DIAGNOSIS: A left nasopharynx tumor was revealed under nasopharyngeal scope. Eosinophilic, proteinaceous material was revealed under a pathology scope in the nasopharynx tissue and neck tumor. Congo red staining demonstrated pale congophilic amorphous material with apple-green birefringence under cross-polarized light, and multifocal amyloidosis was diagnosed. Amyloidosis secondary to systemic lupus erythematosus (SLE) was confirmed after a series of investigations. INTERVENTIONS: The patient underwent local excision for multifocal amyloidosis without following management. To control underlying SLE, the patient accepted steroid pulse therapy and immunosuppressants. The patient eventually achieved disease remission. OUTCOMES: During the 6 months of follow-up in the outpatient department of otolaryngology and rheumatology, complications, recurrence of nasopharyngeal amyloidosis, and SLE flare-up were not observed. LESSONS: Head and neck amyloidosis involving the nasopharynx is a rare presentation of this disease. Head and neck multifocal amyloidosis should be taken as a hint of systemic disease. In head and neck amyloidosis, a comprehensive survey should be performed to clarify the underlying disease predisposing to amyloidosis and organ involvement.


Assuntos
Amiloidose/etiologia , Neoplasias de Cabeça e Pescoço/diagnóstico , Lúpus Eritematoso Sistêmico/diagnóstico , Neoplasias Nasofaríngeas/diagnóstico , Adulto , Amiloidose/cirurgia , Feminino , Neoplasias de Cabeça e Pescoço/patologia , Neoplasias de Cabeça e Pescoço/cirurgia , Humanos , Imunossupressores/uso terapêutico , Lúpus Eritematoso Sistêmico/tratamento farmacológico , Neoplasias Nasofaríngeas/patologia , Neoplasias Nasofaríngeas/cirurgia , Indução de Remissão , Esteroides/uso terapêutico , Resultado do Tratamento
8.
Paediatr Respir Rev ; 31: 32-34, 2019 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-31288987

RESUMO

As the life expectancy of patients with cystic fibrosis has increased, greater attention has been paid towards the diagnosis and management of the longer term consequences of the condition. A recognised but rare complication of the disease is the development of secondary amyloidosis. Whilst deposition of amyloid protein has been reported in a high proportion of patients with cystic fibrosis at post-mortem [1] and Serum Amyloid A protein has been shown to correlate with disease activity and response to antibiotics [2], the manifestation of clinical disease remains extremely uncommon. The prognosis for patients with amyloid secondary to cystic fibrosis in published reports has been historically bleak [3-6], however there may be novel approaches in the era of biological therapies. The theoretical potential for an increase in the incidence of secondary amyloid amongst the population of cystic fibrosis patients who are experiencing much longer lifespans means that it is worthwhile to consider the condition and its possible treatments in more detail. We report a case and a review of the literature.


Assuntos
Amiloidose/metabolismo , Fibrose Cística/metabolismo , Síndrome Nefrótica/metabolismo , Proteína Amiloide A Sérica/metabolismo , Amiloidose/etiologia , Amiloidose/fisiopatologia , Fibrose Cística/complicações , Fibrose Cística/fisiopatologia , Feminino , Humanos , Síndrome Nefrótica/etiologia , Adulto Jovem
13.
Medicine (Baltimore) ; 98(30): e16567, 2019 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-31348284

RESUMO

RATIONALE: Multiple myeloma is the second most common hematological malignancy. Extramedullary involvement is one of the indicators of poor prognosis. There is no consensus in treatment options and the efficacy. This article reports a case of multiple myeloma with onset of pancreas involvement. Amyloidosis secondary to multiple myeloma and a partial response to the chemotherapy treatment further emphasized its rarity. PATIENT CONCERNS: In this article, we report a 59-year-old male patient with a chief complaint of fatigue for 8 months and upper abdominal pain for 2 months. DIAGNOSIS: The patients were diagnosed as amyloidosis secondary to multiple myeloma with pancreatic occupying (head-neck junction area) lesion based on laboratory examination and pathology from lymph node puncture and skin biopsy. INTERVENTIONS: An intensive chemotherapy treatment as bortezomib, lenalidomide, dexamethasone, cisplatin, epirubicin, cyclophosphamide, and etoposide was given. Due to intolerance, treatment regimen was further adjusted to bortezomib, lenalidomide, and dexamethasone. OUTCOMES: The patient was 12 months alive. After 4 cycles of chemotherapy, a partial response was achieved and abdominal magnetic resonance imaging suggested a reduced pancreatic occupying lesion. LESSONS: This case demonstrates that pancreatic involvement, digestive system neoplasm, and amyloidosis-related clinical features may be the earliest manifestations of multiple myeloma. For these patients, an intensive chemotherapy regimen may be a possible treatment approach.


Assuntos
Amiloidose/etiologia , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Mieloma Múltiplo/patologia , Neoplasias Pancreáticas/secundário , Humanos , Linfonodos/patologia , Masculino , Pessoa de Meia-Idade , Mieloma Múltiplo/complicações , Mieloma Múltiplo/tratamento farmacológico , Neoplasias Pancreáticas/tratamento farmacológico
14.
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
15.
Int J Mol Sci ; 20(12)2019 Jun 18.
Artigo em Inglês | MEDLINE | ID: mdl-31216785

RESUMO

Transthyretin (TTR) is a thyroid hormone-binding protein which transports thyroxine from the bloodstream to the brain. The structural stability of TTR in tetrameric form is crucial for maintaining its original functions in blood or cerebrospinal fluid (CSF). The altered structure of TTR due to genetic mutations or its deposits due to aggregation could cause several deadly diseases such as cardiomyopathy and neuropathy in autonomic, motor, and sensory systems. The early diagnoses for hereditary amyloid TTR with cardiomyopathy (ATTR-CM) and wild-type amyloid TTR (ATTRwt) amyloidosis, which result from amyloid TTR (ATTR) deposition, are difficult to distinguish due to the close similarities of symptoms. Thus, many researchers investigated the role of ATTR as a biomarker, especially its potential for differential diagnosis due to its varying pathogenic involvement in hereditary ATTR-CM and ATTRwt amyloidosis. As a result, the detection of ATTR became valuable in the diagnosis and determination of the best course of treatment for ATTR amyloidoses. Assessing the extent of ATTR deposition and genetic analysis could help in determining disease progression, and thus survival rate could be improved following the determination of the appropriate course of treatment for the patient. Here, the perspectives of ATTR in various diseases were presented.


Assuntos
Amiloidose/diagnóstico , Amiloidose/metabolismo , Amiloidose/terapia , Biomarcadores , Pré-Albumina/metabolismo , Proteínas Amiloidogênicas/química , Proteínas Amiloidogênicas/genética , Proteínas Amiloidogênicas/metabolismo , Amiloidose/etiologia , Diagnóstico Diferencial , Gerenciamento Clínico , Humanos , Mutação , Pré-Albumina/química , Pré-Albumina/genética , Agregados Proteicos , Agregação Patológica de Proteínas/metabolismo , Relação Estrutura-Atividade , Fluxo de Trabalho
16.
J Zoo Wildl Med ; 50(1): 147-158, 2019 03 01.
Artigo em Inglês | MEDLINE | ID: mdl-31120673

RESUMO

Thirteen pronghorn antelope (Antilocapra americana) from a single captive herd at the Columbus Zoo and Aquarium underwent complete or partial necropsies between 1997 and 2016. Ten of the 13 animals had histologic evidence of amyloidosis resulting in a 77% prevalence. Histologic and ultrastructural changes were characterized in an attempt to determine the underlying cause of the amyloid. Amyloid detection was performed through histologic examination of hemotoxylin and eosin and Congo red-stained microscopic slides for all 13 animals. Transmission electron microscopy and mass spectrometry was performed on renal tissue from two animals. Pedigree analysis and retrospective investigation into the clinical histories was performed. Histologically, 9/10 animals had amyloid present in the kidneys, 8/10 in the liver, 9/10 in the spleen, 4/10 in the gastrointestinal tract, 3/10 in the adrenal glands, and 2/10 in the thyroid glands. Transmission electron microscopy demonstrated glomerular deposits consistent with amyloid. Mass spectrometry performed on renal specimens from two animals revealed the presence of serum amyloid A. Eight of the 10 animals diagnosed with systemic amyloidosis had a clinical history of haemonchosis (elevated fecal strongyle count), 5/10 were diagnosed with pneumonia postmortem, and 7/10 had postmortem findings consistent with negative energy balance. Serum amyloid A, and ß and γ globulin levels were evaluated in four cases of amyloidosis, and all were within normal ranges for healthy domestic cattle. It was possible that the herd's amyloidosis was associated with a hereditary defect that could be exacerbated by chronic inflammation. However, there was no significant association between the mean degree of relatedness and presence of amyloidosis. In conclusion, systemic amyloidosis in this captive population of pronghorn is common. It is likely reactive and secondary to underlying chronic inflammation caused by haemonchosis and/or pneumonia.


Assuntos
Amiloidose/veterinária , Antílopes , Proteína Amiloide A Sérica/metabolismo , Amiloidose/classificação , Amiloidose/epidemiologia , Amiloidose/etiologia , Animais , Animais de Zoológico , Feminino , Masculino , Ohio/epidemiologia , Especificidade de Órgãos , Prevalência , Estudos Retrospectivos
17.
Int J Biol Macromol ; 134: 1022-1037, 2019 Aug 01.
Artigo em Inglês | MEDLINE | ID: mdl-31128177

RESUMO

Protein and peptides are converted from their soluble forms into highly ordered fibrillar aggregates under various conditions inside the cell. Such transitions confer diverse neurodegenerative diseases including Alzheimer's disease, Huntington's disease Prion's disease, Parkinson's disease, polyQ and share abnormal folding of potentially cytotoxic protein species linked with degeneration and death of precise neuronal populations. Presently, major advances are made to understand and get detailed insight into the structural basis and mechanism of amyloid formation, cytotoxicity and therapeutic approaches to combat them. Here we highlight classifies and summarizes the detailed overview of protein misfolding and aggregation at their molecular level including the factors that promote protein aggregation under in vivo and in vitro conditions. In addition, we describe the recent technologies that aid the characterization of amyloid aggregates along with several models that might be responsible for amyloid induced cytotoxicity to cells. Overview on the inhibition of amyloidosis by targeting different small molecules (both natural and synthetic origin) have been also discussed, that provides important approaches to identify novel targets and develop specific therapeutic strategies to combat protein aggregation related neurodegenerative diseases.


Assuntos
Amiloide/química , Amiloide/metabolismo , Agregados Proteicos , Agregação Patológica de Proteínas , Dobramento de Proteína , Amiloide/toxicidade , Proteínas Amiloidogênicas/química , Proteínas Amiloidogênicas/metabolismo , Proteínas Amiloidogênicas/toxicidade , Amiloidose/tratamento farmacológico , Amiloidose/etiologia , Amiloidose/metabolismo , Amiloidose/patologia , Animais , Humanos , Interações Hidrofóbicas e Hidrofílicas , Terapia de Alvo Molecular , Pressão , Agregados Proteicos/efeitos dos fármacos , Agregação Patológica de Proteínas/tratamento farmacológico , Dobramento de Proteína/efeitos dos fármacos , Processamento de Proteína Pós-Traducional , Relação Estrutura-Atividade , Temperatura
18.
Minerva Med ; 110(5): 450-454, 2019 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-31142092

RESUMO

Juvenile idiopathic arthritis (JIA) is a chronic systemic inflammatory disease, which affects children and adolescents, characterized by significant differences when compared to inflammatory rheumatisms in adulthood. Today, in a panorama enriched in the last decades with great improvements in the diagnostic and therapeutic field, a far from negligible portion and an increasing number of patients with JIA require the continuation of treatments in adulthood. This specific population of patients, given the high incidence of extra-articular manifestations, residual irreversible disabilities, comorbidities related to an inflammatory process and extended immunosuppressive treatments during the age of development, requires precise attentions in the follow-up and a multidisciplinary approach characterized by different clinical, psychological and social aspects.


Assuntos
Artrite Juvenil/diagnóstico , Adolescente , Amiloidose/etiologia , Artrite Juvenil/complicações , Artrite Juvenil/terapia , Criança , Pré-Escolar , Progressão da Doença , Nanismo/etiologia , Oftalmopatias/etiologia , Humanos , Osteoporose/etiologia , Índice de Gravidade de Doença , Transição para Assistência do Adulto
19.
Medicine (Baltimore) ; 98(14): e15039, 2019 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-30946344

RESUMO

RATIONALE: Amyloidosis secondary to intrapulmonary Castleman disease (CD) is a rare benign disease diagnosed by histopathology. It seems to be associated with chronic inflammation, and large amounts of IL-6 produced in the germinal center of CD may enhance the production of precursor of amyloid. PATIENT CONCERNS: We reported a case of an 18-year-old woman presenting with dry cough and dyspnea on exertion for 6 months and detailed exams revealed multiple pulmonary nodules, positive antinuclear antibodies, hypocomplementemia, and thrombocytopenia. DIAGNOSES: A computed tomography-guided percutaneous lung biopsy revealed the histopathological features of pulmonary hyalinizing granuloma (PHG), but video-assisted pulmonary wedge resection for biopsy with immunohistochemical stains finally demonstrated a corrected diagnosis of intrapulmonary CD with secondary amyloidosis. INTERVENTIONS: The patient had received prednisone and Tacrolimus for 6 months. OUTCOMES: There was no significant improvement in pulmonary lesions or platelet level. Chemotherapy to CD was needed. LESSONS: Intrapulmonary CD should be considered in patients with multiple pulmonary nodules irresponsive to corticosteroid and diagnosis of PHG should be carefully considered based on small lung biopsy sample. The treatment of amyloidosis secondary to CD remains to be uncertain.


Assuntos
Amiloidose/diagnóstico , Hiperplasia do Linfonodo Gigante/diagnóstico , Granuloma/diagnóstico , Pneumopatias/diagnóstico , Neoplasias Pulmonares/diagnóstico , Adolescente , Amiloidose/etiologia , Hiperplasia do Linfonodo Gigante/complicações , Diagnóstico Diferencial , Feminino , Humanos , Pneumopatias/complicações
20.
Contrib Nephrol ; 198: 94-102, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-30991407

RESUMO

BACKGROUND: With the advancement of technology, a dialysis membrane has been developed to achieve the efficient removal of beta-2 microglobulin (ß2MG), which could not be removed with previous hemodialysis (HD) membranes. Recently, there has been an increase in the population of elderly chronic kidney disease (CKD) patients with chronic inflammation and malnutrition. The optimal extracorporeal circulation treatment for elderly CKD patients is not certain. SUMMARY: We have reported the clinical advantages, such as improvements in nutritional, inflammatory, and hemodynamic conditions, of the adsorptive HD membrane for elderly HD patients. We have also reported that the use of ß2MG adsorption columns improved the symptoms of dialysis-related amyloidosis and the number of bone cysts, which could not be improved by the high-flux hemodialyzer. Both the adsorptive HD membrane and ß2MG adsorption columns remove uremic toxins and inflammatory cytokines via adsorption without aggravating the nutritional condition of these patients. Key Messages: We should reconsider the mechanisms of adsorption, in addition to diffusion and convection, in the extracorporeal circulation treatment of elderly HD patients.


Assuntos
Membranas Artificiais , Diálise Renal/instrumentação , Microglobulina beta-2/isolamento & purificação , Adsorção , Idoso , Idoso de 80 Anos ou mais , Amiloidose/etiologia , Amiloidose/prevenção & controle , Cistos Ósseos/etiologia , Cistos Ósseos/prevenção & controle , Humanos , Masculino , Diálise Renal/efeitos adversos , Diálise Renal/métodos , Diálise Renal/tendências , Insuficiência Renal Crônica/complicações , Insuficiência Renal Crônica/terapia
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