Search results for " amyloidosis."
showing 10 items of 31 documents
Multisystem Amyloidosis in a Coal Miner with Silicosis: Is Exposure to Silica Dust a Cause of Amyloid Deposition?
2022
The over-secretion of monoclonal immunoglobulin light chains by clonal B cells followed by the aggregation and extracellular deposition of fibrillar deposits are responsible forthe clinical course AL amyloidosis. It is well documented that silica significantly increases the number of immunoglobulin-secreting cells. In the present paper, we report on a coal miner with silicosis and fast progressing primary amyloidosis with predominantly heart, kidney, and lung manifestations. Severeheart failure due to myocardial hypertrophy resulted in the patient’s death. We conclude that long-term environmental silica exposure and silica deposition may contribute to the development of monoclonal gammopath…
CO(2)-laser treatment of laryngeal amyloidosis.
2003
Four consecutive female patients (age: 14–47 years) with laryngeal amyloidosis, treated with endoscopic CO2-laser surgery, entered the study. All patients underwent periodic microlaryngoscopies following surgery to confirm the adequacy of the surgical resection. Recurrences or suspected lesions were resected and fibrin deposits were removed to prevent the formation of synechiae or healing adhesions. After two negative microlaryngoscopies, performed two months apart, the patients were followed-up approximately every six months over a period from six months to 18 years, with no evidence of recurrences. The endoscopic CO2-laser technique is highly effective in the treatment of localized laryng…
Role of Colchicine Treatment in Tumor Necrosis Factor Receptor Associated Periodic Syndrome (TRAPS): Real-Life Data from the AIDA Network
2020
Objective. To analyze the potential role of colchicine monotherapy in patients with tumor necrosis factor receptor associated periodic syndrome (TRAPS) in terms of control of clinical and laboratory manifestations. Methods. Patients with TRAPS treated with colchicine monotherapy were retrospectively enrolled; demographic, clinical and therapeutic data were collected and statistically analysed after having clustered patients according to different times at disease onset, penetrance of mutations, dosage of colchicine, and different disease manifestations. Results. 24 patients (14 males; 15 with pediatric disease onset) treated with colchicine monotherapy were enrolled. Colchicine resulted in …
Senile amyloidosis: Principles of localization in a heterogeneous form of amyloidosis
1983
In order to identify amyloid deposits in patients over 60 years of age (so-called senile amyloid), the following five tissues were investigated under the light and electron microscope : 1. pituitary gland, 2. pancreatic islets of Langerhans, 3. heart, 4. aorta, and 5. brain. In all an increasing incidence of amyloid deposits was found with increasing age, and in the brain a significant quantitative increase in amyloid deposits with increasing age was observed. Despite the biochemical heterogeneity of amyloid found in old age, all the deposits seen in tissues examined were morphologically similar. Typical amyloid fibrils were always found (diameter 60–100 A), and these were invariably deposi…
Sonographic signs of amyloidosis.
2009
INTRODUCTION: Amyloidosis is a rare group of diseases with a variety of symptoms that occurs not only in multimorbid elderly but also in young patients. However, sonographic signs of amyloidosis have not been described so far. PATIENTS AND METHODS: 30 patients with different forms of amyloidosis (19 patients with TTR-amyloidosis (familial amyloid Polyneuropathy [FAP]), 11 patients with other forms of systemic amyloidosis) were sonographically evaluated in a standardised fashion. In all patients amyloidosis was histologically verified. RESULTS: Typical signs of cardiac amyloidosis were myocardial thickness, pericardial effusion, pleural effusion and typical echorich subendocardial deposition…
Hereditary cerebral hemorrhage with amyloidosis associated with the E693K mutation of APP
2010
Objective To report the clinical, genetic, neuroimaging, and neuropathologic studies of patients with the hereditary cerebral hemorrhage with amyloidosis linked to the APP E693K mutation. Design Case series. Clinical details and laboratory results were collected by direct evaluation and previous medical records. DNA analysis was carried out in several affected subjects and healthy individuals. Neuropathologic examination was performed in 2 subjects. Setting Southern Lombardy, Italy. Patients Individuals with and without amyloidosis in 4 unrelated Italian families (N = 37). Main Outcome Measure Genotype-phenotype relationship. Results The affected individuals presented with recurrent headach…
Amyloidosis and Ocular Involvement: an Overview.
2019
Purpose: To describe the ophthalmic manifestations of amyloidosis and the corresponding therapeutic measures.Methods: The 178 patients included in the study had different types of amyloidosis, diagnosed at a single internal medicine institution (Bari, Italy). To provide a comprehensive review of the types of amyloidosis that can be associated with ocular involvement, the images and clinical descriptions of patients with amyloidosis structurally related to gelsolin, keratoepithelin and lactoferrin were obtained in collaborations with the ophthalmology departments of hospitals in Mainz (Germany) and Helsinki (Finland).Results: Overall, ocular morbidity was detected in 41 of the 178 patients w…
Evidence of impaired left ventricular systolic function by Doppler myocardial imaging in patients with systemic amyloidosis and no evidence of cardia…
2007
We examined the potential role of Doppler myocardial imaging for early detection of systolic dysfunction in patients with systemic amyloidosis (AL) but without evidence of cardiac involvement by standard echocardiography. We identified 42 patients without 2-dimensional echocardiographic or Doppler evidence of cardiac involvement. These patients had normal ventricular wall thickness and normal velocity of the medial mitral annulus. Myocardial images were obtained in these patients and in 32 age- and gender-matched healthy controls. Peak longitudinal systolic tissue velocity (sTVI), systolic strain rate (sSR), and systolic strain (sS) were determined for 16 left ventricular segments. Radial a…
Detection of Left Ventricular Systolic Dysfunction in Cardiac Amyloidosis with Strain Rate Echocardiography .
2006
Background We examined the potential role of Doppler myocardial imaging including tissue velocity imaging, strain imaging, and strain rate imaging for detection of left ventricular systolic dysfunction in cardiac amyloidosis (CA) and determined the minimum dataset required to make the diagnosis. Methods and Results Doppler myocardial imaging was performed in 103 patients with amyloidosis (AL). Peak longitudinal systolic tissue velocity, systolic strain rate (sSR), and systolic strain (sS) were determined for 16 left ventricular segments. Radial and circumferential sSR and sS were also measured. Patients with increased left ventricular wall thickness were classified with advanced-CA, and the…
Comparison of right ventricular longitudinal strain imaging, tricuspid annular plane systolic excursion, and cardiac biomarkers for early diagnosis o…
2012
Aims To determine the role of assessing right ventricular (RV) function, using standard echocardiography and Doppler myocardial imaging (DMI), in the early diagnosis of cardiac amyloidosis and in the prediction of mortality. Methods and results Patients with primary systemic (AL) amyloidosis seen at our institution from 1 February 2004 through 31 October 2005 (N = 249) were categorized by left ventricular thickness and E′ velocity and compared with 38 age- and sex-matched controls. Standard echocardiographic and DMI examination were used to measure echocardiographic parameters of RV function: systolic tissue velocity, strain rate, and strain were determined for basal and middle RV free wall…