Intestinal pseudo-obstruction associated with amyloidosis

Liapis, Konstantinos; Michelis, Fotios V.; Delimpasi, Sosanna; Karmiris, Themis

doi:10.3109/13506129.2010.548085

Cited by 18 publications

(6 citation statements)

References 3 publications

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“…It has been postulated that severe diarrhea associated with gastrointestinal amyloidosis could also be a result of rapid intestinal transit [11]. The underlying pathology for most of these symptoms is amyloid infiltration and replacement of the intestinal smooth muscle causing dysmotility [12, 13], which in extreme cases can even present as pseudo-obstruction [8, 12, 14, 15]. Ischemia secondary to vascular infiltration or depletion of ganglion cells has also been noted.…”

Section: Discussionmentioning

confidence: 99%

Primary Systemic Amyloidosis with Extensive Gastrointestinal Involvement

Gaduputi

Badipatla

Patel

et al. 2013

Case Rep Gastroenterol

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We report this case of a 42-year-old woman who presented with a debilitating illness manifested by intractable nausea, vomiting, diarrhea and unchecked weight loss. The patient had multisystem involvement that presented as anemia, abnormal liver function tests and progressively deteriorating renal function necessitating dialysis. She was found to be profoundly hypoalbuminemic secondary to malabsorptive and protein-losing enteropathy in tandem with nephrotic range proteinuria. Intolerance to enteral feeding led the patient to be dependent on parenteral nutrition. Serum immunofixation revealed IgG lambda monoclonal protein. The patient underwent endoscopic evaluation with biopsies taken from the gastrointestinal tract that confirmed the diagnosis of primary systemic light-chain amyloidosis. A subsequent bone marrow biopsy revealed normocellular bone marrow with deposition of amyloid. The patient was not considered for autologous stem cell transplantation as the outcomes in patients with multisystem involvement are often poor, with a high mortality risk. Diffuse primary systemic light-chain amyloidosis involving the gastrointestinal tract is a rare entity and is to be considered among differentials in patients presenting with unexplained malabsorptive symptoms.

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Section: Discussionmentioning

confidence: 99%

Primary Systemic Amyloidosis with Extensive Gastrointestinal Involvement

Gaduputi

Badipatla

Patel

et al. 2013

Case Rep Gastroenterol

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“…Amyloid fibrils gradually accumulate in the GI wall and cause mucosal impairment leading to bleeding and intestinal pseudo-obstruction in parallel with a decrease in bowel motility via direct pressure of the neighboring tissues and/or ischemic changes due to vascular involvement (42,43). Endoscopically, mucosal polypoid lesions with friability and erosions are common findings (44,45). Due to the frequent involvement of the vascular walls, GI bleeding is more likely to occur in patients with systemic AL amyloidosis than in those with the localized form (31,45).…”

Section: Uncommon Manifestationsmentioning

confidence: 99%

“…Endoscopically, mucosal polypoid lesions with friability and erosions are common findings (44,45). Due to the frequent involvement of the vascular walls, GI bleeding is more likely to occur in patients with systemic AL amyloidosis than in those with the localized form (31,45). Abdominal CT sometimes shows thickening and dilatation of the GI tract with soft tissue infiltration (46).…”

Section: Uncommon Manifestationsmentioning

confidence: 99%

Clinical Manifestations at Diagnosis in Japanese Patients with Systemic AL Amyloidosis: A Retrospective Study of 202 Cases with a Special Attention to Uncommon Symptoms

2014

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Objective To retrospectively investigate the clinical manifestations at diagnosis in Japanese patients with systemic AL amyloidosis. Methods We reviewed the medical records of 230 Japanese patients who had visited our hospital and been diagnosed with AL amyloidosis, and abstracted those with the systemic type. The clinical data at diagnosis of systemic AL amyloidosis, including laboratory and imaging findings, were analyzed. Results Two hundred and two patients (mean, 58.7±9.5 years) were enrolled in this study. Immunofixation or immunoelectrophoresis was performed in 173 patients, 144 of whom were positive for M-protein in the serum and/or urine (κ:λ=30:114). The primary clinical manifestations at diagnosis were proteinuria and/or renal dysfunction (54.0%), congestive heart failure (24.8%), peripheral neuropathy (10.4%), hepatomegaly (7.9%) and arrhythmia (5.0%). The remaining patients developed unusual manifestations, such as solitary tumor, lymphadenopathy, gastrointestinal bleeding, intestinal pseudoobstruction, hemorrhagic tendencies and polyarthralgia. Dilatation of the intestine with marked thickening of the gastrointestinal wall on computed tomography and multiple nodular lesions with associated mucosal friability on endoscopy are characteristic findings of systemic AL amyloidosis. Conclusion The clinical pictures of Japanese patients with systemic AL amyloidosis are similar to those previously reported from the US and European nations; however, some patients with this disease develop uncommon symptoms. Conducting laboratory and histological examinations for systemic AL amyloidosis is necessary when making a differential diagnosis of these symptoms.

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“…Amyloidosis is characterized by extracellular deposition of abnormal protein. It involves multiple organs, most common being the heart, peripheral nerves and kidneys; however its involvement of the GI tract is mostly subclinical [3,4,5]. It has a varied presentation in gastrointestinal tract resulting from gastroparesis, nausea/vomiting, malabsorption and alteration in bowel habits among others [6].…”

Section: Discussionmentioning

confidence: 99%

“…It has a varied presentation in gastrointestinal tract resulting from gastroparesis, nausea/vomiting, malabsorption and alteration in bowel habits among others [6]. In the GI tract, it most commonly affects the small bowel [5]. Involvement of GI tract is defined by presence of symptoms along with proof of biopsy.…”

Section: Discussionmentioning

confidence: 99%

A Rare Cause of Intestinal Pseudo-obstruction: Gastrointestinal Amyloid

Kochar

Shah

2020

Cureus

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Amyloidosis is characterized by extracellular deposition of the amyloid protein. It can affect multiple organ systems but it most commonly affects the heart, kidney and gastrointestinal (GI) tract. It can occur sporadically or in association with other conditions like multiple myeloma, chronic inflammatory diseases, infections etc. Its involvement of the gastrointestinal tract is rare and diffuse. It has variable manifestations in GI tract from involving the stomach to the large bowel including liver. We present a case of 55 year old Caucasian male with recent diagnosis of multiple myeloma who presented with recurrent episodes of small bowel obstruction which was later found to be caused by amyloid deposition.

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Intestinal pseudo-obstruction associated with amyloidosis

Cited by 18 publications

References 3 publications

Primary Systemic Amyloidosis with Extensive Gastrointestinal Involvement

Primary Systemic Amyloidosis with Extensive Gastrointestinal Involvement

Clinical Manifestations at Diagnosis in Japanese Patients with Systemic AL Amyloidosis: A Retrospective Study of 202 Cases with a Special Attention to Uncommon Symptoms

A Rare Cause of Intestinal Pseudo-obstruction: Gastrointestinal Amyloid

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