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63rd Annual Conference of the Indian Society of Gastroenterology, ISGCON 2022-January 5th - 8th, 2023 in Jaipur.

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A real-world study on diagnosis and prognosis of light-chain cardiac amyloidosis in Southern China.

BackgroundLight-chain cardiac amyloidosis (AL-CA) has been highly valued in developed countries, but in developing countries, the recognition and diagnosis of this condition is still limited. There are currently few reports on a large number of Chinese patients with AL-CA. The present study aimed to report real-world clinical characteristics and prognosis of AL-CA in China.Methods and resultsConsecutive patients with AL-CA diagnosed at the Second Xiangya Hospital of Central South University between June 2012 and September 2020 were reviewed. A total of 170 patients with AL-CA have been recruit

Paraneoplastic Neuropathies: What's New Since the 2004 Recommended Diagnostic Criteria.

The diagnostic criteria published by the PNS (Paraneoplastic Neurological Syndromes) Euronetwork in 2004 provided a useful classification of PNS, including paraneoplastic neuropathies. Subacute sensory neuronopathy (SSN) was the most frequently observed peripheral PNS, whereas other forms of neuropathy, as sensory polyneuropathy, sensorimotor polyneuropathy, demyelinating neuropathies, autonomic neuropathies, and focal nerve or plexus lesions, were less frequent. At the time of publication, the main focus was on onconeural antibodies, but knowledge regarding the mechanisms has since expanded.

Совпадение в полном тексте источника
… It is often the predominant manifestation of anti-Hu multifocal encephalomyelitis (paraneoplastic encephalomyelitis), which in 10% cases can also manifest with dysautonomic symptoms, as hypotension, dysrhythmia, or intestinal pseudo-obstruction ( 20 ). … Symptoms can result in orthostatic hypotension, urinary symptoms, sweating abnormalities, intestinal pseudo-obstruction, gastrointestinal dysmotility, and subacute dysautonomia. Dysautonomia is typical of AL amyloid neuropathy. …

Analysis of factors associated with hematopoietic stem-cell retransplantation: a case-control study.

Objectiveto analyze the factors associated with the failure of Hematopoietic Stem Cell Transplantation (HSCT) in patients undergoing Hematopoietic Stem Cell Retransplantation (HSCR).Methodthis study implemented a quantitative approach and was a case-control type which addressed patients undergoing HSCR. To do so, a paired sample of two controls was used for each case (2:1). The case group consisted of the medical records of all patients who underwent HSCR (28) and the control group (56) of those who underwent only one transplant. Three variables guided the pairing: gender, diagnosis and type o

Peripheral Nervous, Hepatic, and Gastrointestinal Endpoints for AL Amyloidosis Clinical Trials: Report from the Amyloidosis Forum Multi-organ System Working Group.

Systemic immunoglobulin light chain (AL) amyloidosis is a heterogeneous rare disease driven by a destructive monoclonal gammopathy and typified by misfolded immunoglobulin light and/or heavy chains which aggregate and deposit in organs as insoluble amyloid fibrils. Disease heterogeneity is driven by the degree of multi-systemic involvement; cardiac, renal, neurological, and gastrointestinal (GI) systems are affected to varying degrees in different patients. While prognosis is primarily driven by hematologic response to treatment and outcomes associated with cardiac events and overall survival,

Совпадение в полном тексте источника
… Intestinal pseudo-obstruction and protein-losing enteropathy are severe manifestations, albeit uncommon. Limited data suggest GI symptoms may be more common in some subtypes of amyloidosis [ 40 , 41 ]. …

Renal Involvement in Mixed Cryoglobulinemic Vasculitis: Current Perspectives.

Cryoglobulinemia is a rare disorder characterized by the presence of abnormal proteins (cryoglobulins) in the blood that precipitate at low temperatures. It presents with a wide clinical spectrum, from mild symptoms to severe, life-threatening disease. In mixed cryoglobulinemia (MC), vascular damage results from immune complexes-typically monoclonal IgM with rheumatoid factor activity and polyclonal IgG (Type II), or polyclonal/oligoclonal IgM and IgG (Type III). These complexes can obstruct small blood vessels, leading to ischemia and leukocytoclastic vasculitis. Renal involvement occurs in a

Ataxia-Telangiectasia and Associated Bronchiectasis: Case Report and Literature Review.

Ataxia-telangiectasia is a rare, autosomal recessive primary immunodeficiency caused by mutations in the ATM gene on chromosome 11, which encodes a serine-threonine kinase essential for the recognition and repair of DNA double-strand breaks. The disease is characterized by progressive neurological impairment, immunological dysfunction, and an increased susceptibility to recurrent infections and malignancies. Pulmonary involvement represents a major source of morbidity and frequently arises from chronic infections, aspiration, and impaired airway clearance, ultimately leading to the development

Coexistence of Autoantibodies against the Golgi Complex and Ro52 Antigen in a Patient with Nonspecific Interstitial Pneumonia.

Nonspecific interstitial pneumonia (NSIP) is often associated with connective tissue diseases (CTD). The diagnosis of NSIP was confirmed in a 63-year-old man by high-resolution computed tomography and an open lung biopsy. Anti-Golgi complex autoantibodies (AGA) and anti-Ro52 antibodies were simultaneously detected at high concentrations. Autoantibodies to aminoacyl-tRNA synthetases (ARS) were negative. The patient was treated with corticosteroids for six months. During the seven-year follow-up, NSIP had a slow progression and patient had not developed the clinical features of CTD. The present

Oral and Maxillo-Facial Manifestations of Systemic Diseases: An Overview.

Many systemic (infective, genetic, autoimmune, neoplastic) diseases may involve the oral cavity and, more generally, the soft and hard tissues of the head and neck as primary or secondary localization. Primary onset in the oral cavity of both pediatric and adult diseases usually represents a true challenge for clinicians; their precocious detection is often difficult and requires a wide knowledge but surely results in the early diagnosis and therapy onset with an overall better prognosis and clinical outcomes. In the current paper, as for the topic of the current Special Issue, the authors pre

Pain in blood cancers.

Patients with blood-related cancers (BRC) suffer from a substantial symptom burden, including several pain syndromes sustained by different causes and pathogenetic mechanisms. So, with regard to pain, a multifaceted clinical scenario may be observed in this setting. Indeed, pain may be correlated to disease itself, to disease-associated complications, to iatrogenic causes or may be due to unrelated clinical conditions. A close diagnostic procedure for the assessment of the underlying causes of the pain and of its pathogenetic mechanisms may direct the treatment approach which should be based o

Sclerosing diseases of the skin.

Sclerosing skin diseases comprise a group of distinct dermatological conditions characterized by fibrotic changes that may severely impair patients' quality of life. These conditions often present with cutaneous manifestations and, in some cases, may extend to extracutaneous tissues, potentially resulting in significant morbidity and mortality. This review explores the current understanding of the four most prevalent sclerosing skin diseases - localized scleroderma (morphea), systemic sclerosis (SSc), scleromyxedema, and scleredema adultorum of Buschke - as well as sclerotic conditions induced

Advances in Extracellular Matrix-Associated Diagnostics and Therapeutics.

The extracellular matrix (ECM) is the common denominator of more than 50 chronic diseases. Some of these chronic pathologies lead to enhanced tissue formation and deposition, whereas others are associated with increased tissue degradation, and some exhibit a combination of both, leading to severe tissue alterations. To develop effective therapies for diseases affecting the lung, liver, kidney, skin, intestine, musculoskeletal system, heart, and solid tumors, we need to modulate the ECM's composition to restore its organization and function. Across diverse organ diseases, there are common denom

Integrated Pleiotropic Gene Set Unveils Comorbidity Insights across Digestive Cancers and Other Diseases.

Comorbidities are prevalent in digestive cancers, intensifying patient discomfort and complicating prognosis. Identifying potential comorbidities and investigating their genetic connections in a systemic manner prove to be instrumental in averting additional health challenges during digestive cancer management. Here, we investigated 150 diseases across 18 categories by collecting and integrating various factors related to disease comorbidity, such as disease-associated SNPs or genes from sources like MalaCards, GWAS Catalog and UK Biobank. Through this extensive analysis, we have established a

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Неделя ГЭЭ 2017 Презентации на плакате

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Pharmacological treatment options for mast cell activation disease.

Mast cell activation disease (MCAD) is a term referring to a heterogeneous group of disorders characterized by aberrant release of variable subsets of mast cell (MC) mediators together with accumulation of either morphologically altered and immunohistochemically identifiable mutated MCs due to MC proliferation (systemic mastocytosis [SM] and MC leukemia [MCL]) or morphologically ordinary MCs due to decreased apoptosis (MC activation syndrome [MCAS] and well-differentiated SM). Clinical signs and symptoms in MCAD vary depending on disease subtype and result from excessive mediator release by MC

61st Annual Conference of Indian Society of Gastroenterology, Virtual Diamond Jubilee ISGCON 2020, 19-20th December 2020.

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СРП 2013

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62nd Annual Conference of the Indian Society of Gastroenterology, February 10th - 13th, 2022, Pune.

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AL Amyloidosis: Current Chemotherapy and Immune Therapy Treatment Strategies: JACC: CardioOncology State-of-the-Art Review.

Immunoglobulin light chain (AL) amyloidosis is an incurable plasma cell disorder characterized by deposition of fibrils of misfolded immunoglobulin free light chains (FLC) in target organs, leading to failure. Cardiac involvement is common in AL amyloidosis and represents the single most adverse prognostic feature. A high index of clinical suspicion with rapid tissue diagnosis and commencement of combinatorial, highly effective cytoreductive therapy is crucial to arrest the process of amyloid deposition and preserve organ function. The clinical use of molecularly targeted drugs, such as protea