Amiloidose renal familiar em cães da raça Shar-Pei / Family renal amyloidosis in Shar-Pei dogs / Amiloidosis renal familiar en perros Shar-Pei

A amiloidose renal familiar é uma doença incomum em cães, que afeta os rins e está associada ao acúmulo anormal de proteínas amiloides, com capacidade de promover danos orgânicos progressivos com comprometimento de funcionalidade. Caracterizada pela presença de conteúdo proteináceo glomerular, a amiloidose frequentemente está associada a quadros de falência renal, com presença de sinais clínicos variados, sendo uma condição grave e complexa. O presente artigo tem como objetivo descrever os achados clínico-laboratoriais, de imagem e histopatológicos de amiloidose familiar em dois cães da raça Shar-pei. Os animais apresentavam parentesco direto e evidenciavam sinais de cansaço, prostração e emagrecimento progressivo. As evidências clínico-laboratoriais e ultrassonográficas sugeriram a presença de glomerulonefropatia, sendo essa confirmada por exame histopatológico. Os dois cães, diante da gravidade do quadro, foram a óbito. A análise histopatológica evidenciou deposição de material proteináceo fibrilar na região glomerular e tubular, bem como infiltrado linfoplasmocítico, característicos de amiloidose renal. É essencial lembrar que a amiloidose renal familiar em cães é uma doença complexa e que as origens devem ser investigadas. O tratamento é desafiador, diante da inexistência de um manejo terapêutico definido para a doença, sendo este muitas vezes ineficaz. A empatia e o cuidado no manejo dessa condição podem ajudar a melhorar a qualidade de vida do paciente e fornecer conforto ao proprietário durante esse processo desafiador.

Family renal amyloidosis is an uncommon disease in dogs, which affects the kidneys and is associated with abnormal accumulation of amyloid proteins, capable of promoting progressive organic damage with impairment of functionality. Characterized by the presence of glomerular proteinaceous content, amyloidosis is often associated with renal failure, with the presence of varied clinical signs, being a serious and complex condition. This article aims to describe the clinical, laboratory, imaging and histopathological findings of familial amyloidosis in two Shar-pei dogs. The animals were directly related and evidenced signs of tiredness, prostration and progressive weight loss. Clinical, laboratory and ultrasonographic evidence suggested the presence of glomerulonephropathy, which was confirmed by histopathological examination. The two dogs, given the severity of the condition, died. Histopathological analysis showed deposition of fibrillar proteinaceous material in the glomerular and tubular region, as well as lymphoplasmocytic infiltrate, characteristic of renal amyloidosis. It is essential to remember that family renal amyloidosis in dogs is a complex disease and that the origins must be investigated. The treatment is challenging, given the lack of a defined therapeutic management for the disease, which is often ineffective. Empathy and care in managing this condition can help improve the patient's quality of life and provide comfort to the owner during this challenging process.

La amiloidosis renal familiar es una enfermedad poco común en perros, que afecta a los riñones y se asocia con la acumulación anormal de proteínas amiloides, con capacidad de promover daño orgánico progresivo con compromiso de la funcionalidad. Caracterizada por la presencia de contenido proteico glomerular, la amiloidosis suele asociarse a insuficiencia renal, con la presencia de signos clínicos variados, siendo una afección grave y compleja. El presente artículo tiene como objetivo describir los hallazgos clínico-laboratorios, imagenológicos e histopatológicos de la amiloidosis familiar en dos perros Sharpei. Los animales estaban directamente emparentados y presentaban signos de cansancio, postración y pérdida progresiva de peso. Los datos clínico-laboratorios y ecográficos sugirieron la presencia de glomerulonefropatía, la cual fue confirmada mediante examen histopatológico. Los dos perros, dada la gravedad del cuadro, fallecieron. El análisis histopatológico mostró depósito de material proteico fibrilar en la región glomerular y tubular, así como infiltrado linfoplasmocitario, característico de la amiloidosis renal. Es fundamental recordar que la amiloidosis renal familiar en perros es una enfermedad compleja y que es necesario investigar sus orígenes. El tratamiento es un desafío, dada la falta de un manejo terapéutico definido para la enfermedad, que muchas veces resulta ineficaz. La empatía y el cuidado en el manejo de esta afección pueden ayudar a mejorar la calidad de vida del paciente y brindar comodidad al propietario durante este desafiante proceso.

Síndrome do túnel do carpo associada à amiloidose / Carpal tunnel syndrome associated with amyloidosis

Introdução: A amiloidose é caracterizada pela deposição de proteínas nos órgãos e tecidos, e tem sido associada à síndrome do túnel do carpo (STC) quando ocorre no punho. O objetivo é descrever uma série de casos de pacientes submetidos à cirurgia para STC associado à amiloidose. Métodos: O estudo incluiu 12 pacientes que se submeteram à cirurgia para tratar a STC cuja biópsia identificou amiloidose; o seguimento foi de cinco anos. Os pacientes foram avaliados por testes clínicos, eletroneuromiografia, imagens radiológicas e biópsia. Resultados: Todos os pacientes apresentaram queixas musculoesqueléticas, sintomas severos de compressão do nervo mediano, alterações nos testes neurofisiológicos. Realizou-se a cirurgia, sinovectomia e biópsia. No pós-operatório, cinco pacientes (41%) desenvolveram dor crônica e distrofia simpático-reflexa. Conclusão: Observou-se maior frequência de dor pós-operatória na amostra, o que revela a necessidade de atenção na abordagem e tratamento dessa associação.

Introduction: Amyloidosis features protein deposition in the organs and tissues and has been associated with carpal tunnel syndrome (CTS) when it occurs in the wrist. The objective is to describe a case series of patients undergoing surgery for CTS associated with amyloidosis. Methods: The study included 12 patients who underwent surgery to treat CTS in whom amyloidosis was proven by biopsy; the follow-up period was 5 years. The patients were evaluated by clinical tests, electroneuromyography, radiological images, and biopsy. Results: All patients presented with musculoskeletal complaints, severe symptoms of median nerve compression, and changes on neurophysiological tests. Surgery, synovectomy, and biopsy were performed. In the postoperative period, five patients (41%) developed chronic pain and reflex sympathetic dystrophy. Conclusion: A higher frequency of postoperative pain was observed in the patients, demonstrating the need for caution in the approach and treatment of this association.

Localized Gastric Amyloidosis with Kappa and Lambda Light Chain Co-Expression

Esophagogastroduodenoscopy for cancer screening was performed in a 55-year-old woman as part of a health screening program, and revealed a depressed lesion approximately 20 mm in diameter in the lesser curvature of the mid-gastric body. Several biopsy specimens were collected as the lesion resembled early gastric cancer; however, histopathologic evaluation revealed chronic active gastritis with an ulcer and amorphous eosinophilic material deposition. Congo red staining identified amyloid proteins, and apple-green birefringence was shown using polarized light microscopy. Immunohistochemical staining revealed the presence of kappa and lambda chain-positive plasma cells. There was no evidence of underlying plasma cell dyscrasia or amyloid deposition in other segments of the gastrointestinal tract. Echocardiography and computed tomography of the chest, abdomen, and pelvis did not show any significant findings. Thus, the patient was diagnosed with localized gastric amyloidosis with kappa and lambda light chain coexpression.

Amyloid Arthropathy of the Hip Joint Associated with Multiple Myeloma: A Case Report / 대한고관절학회지

Amyloidosis is a disease characterized by the deposition of non-soluble fibrous protein in multiple tissues with a number of possible causes. This protein deposition can occur in any tissue, yet is most commonly seen in kidneys, heart, and gastrointestinal tracts. However, invasion to bone tissues is not often reported. The deposition of amyloid proteins in bone tissues may result in joint pain and pathological fractures; it is important to elucidate the causes and detect early to determine prognosis and treat optimally. In the present case report, with relevant literature review, the authors report a case of total hip arthroplasty in an amyloidosis patient.

Effectiveness of individual and group interventions for people with type 2 diabetes / Efetividade das intervenções individual e em grupo junto a pessoas com diabetes tipo 2 / Efectividad de las intervenciones individual y en grupo en personas con diabetes tipo 2

OBJECTIVE: to compare the effectiveness of two educational interventions used by a healthcare provider in the monitoring of individuals with type 2 diabetes mellitus (T2DM), regarding knowledge of the disease, impact on quality of life and adoption of self-care actions. METHODS: comparative, longitudinal, prospective study performed with 150 subjects with type 2 diabetes, analyzed according to the type of participation in the program (individual and/or group). Participants of the individual intervention (II) received nursing consultations every six months and those of the group intervention (GI) took part in weekly meetings for three months. Data were collected through four questionnaires: Identification questionnaire, Problem Areas in Diabetes Questionnaire (PAID), Summary of Diabetes Self-Care Activities Questionnaire (SDSCA) and the Diabetes Knowledge Scale (DKN-A). Data were analyzed using the Friedman and Mann Whitney tests, considering a statistical significance of p ≤ 0.05. RESULTS: there was an increase in knowledge about the disease in the II (p<0.003) and GI (p<0.007), with reduction of the impact on the quality of life in the II (p<0.007) and improvement in self-care actions in the GI (p<0.001). CONCLUSION: in both intervention models improvements were observed in the indicators, over the six month monitoring period. .

OBJETIVO: comparar a efetividade de duas intervenções educativas, utilizadas por uma operadora de saúde, no acompanhamento ao indivíduo com diabetes mellitus Tipo 2 (DM2), quanto ao conhecimento sobre a doença, impacto na qualidade de vida e adoção de ações de autocuidado. MÉTODOS: estudo comparativo, longitudinal, prospectivo, realizado com 150 indivíduos com diabetes tipo 2, analisados conforme a modalidade de participação no programa (individual e/ou em grupo). Os participantes da intervenção individual (II) realizaram consultas de enfermagem a cada seis meses e os da intervenção em grupo (IG), reuniões semanais por três meses. Os dados foram coletados mediante quatro questionários: Questionário de identificação, Questionário de Impacto na Qualidade de Vida em Diabetes (PAID), Questionário de Autocuidado em Diabetes (QAD) e Questionário de Conhecimento do Diabetes (DKN-A). Os dados foram analisados utilizando-se o Teste de Friedman e o Teste de Mann Whitney, considerando significância estatística para p ≤ 0,05. RESULTADOS: verificou-se aumento do conhecimento sobre a doença na II (p<0,003) e na IG (p<0,007), redução do impacto na qualidade de vida na II (p<0,007) e melhora das ações de autocuidado na IG (p<0,001). CONCLUSÃO: em ambos os modelos de intervenção foram observadas melhoras dos indicadores, ao longo dos seis meses de acompanhamento. .

OBJETIVO: comparar la efectividad de dos intervenciones educativas, utilizadas por una operadora de planes de salud, en el acompañamiento al individuo con diabetes mellitus Tipo 2 (DM2), sobre al conocimiento de la enfermedad, impacto en la calidad de vida y adopción de acciones de autocuidado. MÉTODOS: estudio comparativo, longitudinal, prospectivo, realizado con 150 individuos con diabetes tipo 2, analizados conforme la modalidad de participación en el programa (individual y/o en grupo). Los participantes de la intervención individual (II) realizaron consultas de enfermería a cada seis meses y los de intervención en grupo (IG), reuniones semanales por tres meses. Los datos fueron recolectados mediante cuatro cuestionarios: Cuestionario de identificación, Cuestionario de Impacto en la Calidad de Vida en Diabetes (PAID), Cuestionario de Autocuidado en Diabetes (CAD) y Cuestionario de Conocimiento de la Diabetes (DKN-A). Los datos fueron analizados utilizando el test de Friedman y el test de Mann Whitney, considerando significación estadística para p ≤ 0,05. RESULTADOS: se verificó aumento del conocimiento sobre la enfermedad en la II (p<0,003) y en la IG (p<0,007), reducción del impacto en la calidad de vida en la II (p<0,007) y mejoría de las acciones de autocuidado en la IG (p<0,001). CONCLUSIÓN: en los dos modelos de intervención fueron observadas mejorías de los indicadores, a lo largo de los seis meses de acompañamiento. .

Protein Transmission, Seeding and Degradation: Key Steps for alpha-Synuclein Prion-Like Propagation

Converging lines of evidence suggest that cell-to-cell transmission and the self-propagation of pathogenic amyloidogenic proteins play a central role in the initiation and the progression of several neurodegenerative disorders. This "prion-like" hypothesis has been recently reported for alpha-synuclein, a presynaptic protein implicated in the pathogenesis of Parkinson's disease (PD) and related disorders. This review summarizes recent findings on alpha-synuclein prion-like propagation, focusing on its transmission, seeding and degradation and discusses some key questions that remain to be explored. Understanding how alpha-synuclein exits cells and propagates from one brain region to another will lead to the development of new therapeutic strategies for the treatment of PD, aiming at slowing or stopping the disease progression.

Impact of sub-chronic aluminium-maltolate exposure on catabolism of amyloid precursor protein in rats / 生物医学与环境科学（英文）

<p><b>OBJECTIVE</b>To investigate the impact of sub-chronic Aluminium-maltolate [Al(mal)3] exposure on the catabolism of amyloid precursor protein (APP) in rats.</p><p><b>METHODS</b>Forty adult male Sprague-Dawley (SD) rats were randomly divided into five groups: the control group, the maltolate group (7.56 mg/kg BW), and the Al(mal)3 groups (0.27, 0.54, and 1.08 mg/kg BW, respectively). Control rats were administered with 0.9% normal saline through intraperitoneal (i.p.) injection. Maltolate and Al(mal)3 were administered to the rats also through i.p. injections. Administration was conducted daily for two months. Rat neural behavior was examined using open field tests (OFT). And the protein expressions and their mRNAs transcription related with APP catabolism were studied using enzyme-linked immunosorbent assay (ELISA) and real-time polymerase chain reaction (RT-PCR).</p><p><b>RESULTS</b>The expressions of APP, β-site APP cleaving enzyme 1 (BACE1) and presenilin-1 (PS1) proteins and their mRNAs transcription increased gradually with the increase of Al(mal)3 doses (P<0.05). The enzyme activity of BACE1 in the 0.54 and 1.08 mg/kg Al(mal)3 groups increased significantly (P<0.05). The expression of β-amyloid protein (Aβ) 1-40 gradually decreased while the protein expression of Aβ1-42 increased gradually with the increase of Al(mal)3 doses (P<0.05).</p><p><b>CONCLUSION</b>Result from our study suggested that one of the possible mechanisms that Al(mal)3 can cause neurotoxicity is that Al(mal)3 can increase the generation of Aβ1-42 by facilitating the expressions of APP, β-, and γ-secretase.</p>

A Case of Systemic Amyloidosis Found by Goiter in Crohn's Disease

Secondary amyloidosis is characterized by accumulation of insoluble, fibrous amyloid proteins in various tissues and organs, accompanied by infectious or inflammatory diseases. Amyloidosis may involve the thyroid, gastrointestinal tract, kidneys, liver, or bone marrow. Amyloidosis as a complication of Crohn's disease is rare but serious, and may worsen the prognosis. We have experienced a case of amyloid goiter and gastrointestinal amyloidosis secondary to Crohn's disease. A 74-year-old female patient with Crohn's disease was admitted to Soonchunhyang University Hospital with general weakness and poor oral intake. Anterior-neck diffuse goiter and tenderness around the navel were found. Amyloid goiter and gastrointestinal amyloidosis diagnosed by sonoguided needle biopsy of the thyroid and endoscopic biopsies of the stomach and duodenum.

Influence of H102 on the expression of amyloid protein and amyloid precursor protein in the hippocampus of APP695 transgenic mice / 中国应用生理学杂志

<p><b>OBJECTIVE</b>To observe the influence of H102 on the expression of amyloid protein and amyloid precursor protein in the hippocampus of APP695 transgenic mice.</p><p><b>METHODS</b>The 9-month-old APP695 transgenic mice were randomly divided into the model group and the H102 group; C57BL/6J mice were adopted as normal control group. The H102 group were injected with H102 in a dose of 3 microl/per mouse in lateral ventricle, once a day, for ten days; while the model group and the control group were injected with saline. The hippocampus and temporal cortex of the brain sections from transgenic mice and wild type female mice were subjected to immunohistochemistry and Congo red histological staining, and observed the difference of the protein expression under microscope. The expression of the APP protein was detected by Western blot.</p><p><b>RESULTS</b>Abeta and APP immunohistochemistry showed density of positive cell in the CA1 region of hippocampus of control group were less than model group. H102 peptide reduced the area, and density of positive cells. Congo red staining showed there were lots of amyloid plagues in the brains of model mice but not in the brains of normal control. And the Western blot showed the content of the APP protein of the model group was much higher than the H102 group. H102 significantly decreased the amyloid plagues.</p><p><b>CONCLUSION</b>The expression of APP, Abeta are increased in APP695 transgenic mice, and H102 can decrease the level of APP, Abeta in transgenic mice.</p>

A Case of Secondary Amyloid Goiter with Hypothyroidism / 대한내분비학회지

Amyloidosis results from the deposition of insoluble, fibrous amyloid proteins, nearly always in the extracellular spaces of organs and tissues. There are several varieties of amyloidosis, each of which is identified by the immunochemical nature of amyloid protein fibrils. Amyloid goiter is a very rare clinical entity and can be confused with a neoplasm. We have experienced a case of amyloid goiter with hypothyroidism secondary to tuberculosis. A 20 years old women with 5 months history of pulmonary tuberculosis was admitted with complaints of diarrhea, abdominal pain, weight loss at one year ago. She had a non-tender, diffuse and firm goiter. Also she had normal thyroid function at the first admission but was found to be hypothyroid at the second admission, 10 months later. Histologic examination revealed amyloid deposition in thyroid gland, stomach, colon and rectum.