This report concerns two patients with severe protein losing enteropathy and refractory diarrhea due to AA amyloidosis who were successfully treated with corticosteroid and octreotide. In these patients, biopsied tissues from the gastrointestinal (GI) tract showed extensive deposition of AA amyloid, which was caused by rheumatoid arthritis in one case and was of unidentified etiology in the other. Both patients manifested severe diarrhea unresponsive to conventional treatment with hypoproteinemia, and protein leakage from the small intestine to the ascending colon was confirmed by 99mTc-diethylene triamine pentaacetic acid human serum albumin (HSA-D) scintigraphy. Soon after starting a long-acting somatostatin analogue, octreotide, with co-administration of oral prednisolone, their general status improved in parallel with a rapid decrease in the volume of watery diarrhea and an increase in serum levels of albumin and IgG. Also on 99mTc-HSA-D scintigraphy protein leakage from the GI tract was apparently decreased in both patients. Combination therapy with a somatostatin analogue and corticosteroid may be effective for protein losing enteropathy with intractable diarrhea ascribable to GI amyloidosis. Because of the lack of specific therapies in this serious clinical situation, the described therapy should actively be considered as a therapeutic option not only in AA amyloidosis, but also in other types of systemic amyloidosis.
Because previous randomized clinical trials have failed to show any benefits of renal artery stenting (RAS), clinicians have been confused regarding the optimal management of patients with atherosclerotic renal artery stenosis (ARAS). Here, we report a notable case with ARAS whose clinical course could help clinicians identify patients suitable for RAS. A 66-year-old woman with ARAS and some parameter abnormalities on renal duplex ultrasound was admitted to our hospital with severe hypertension (>250 mmHg). After the initiation of antihypertensive intravenous treatment, the patient manifested acute worsening of renal function over 6 days and required dialysis. Because renal failure continued to deteriorate over more than 4 weeks, we performed stenting to the severe ostial stenosis of the right renal artery. As a result, the patient achieved dramatic improvement in renal function and successful withdrawal from dialysis and has maintained stable control of blood pressure without additional events for more than 2 years since discharge. This case suggests that careful analysis of the patient's profile, such as history of rapidly deteriorating renal failure, presence of bilateral ARAS, or a solitary functional kidney, in addition to various parameters of renal duplex ultrasound, may help predict the efficacy of RAS in patients with ARAS.
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Learning objective:
Because several randomized studies have reported negative results for renal artery stenting (RAS), an individual-based approach is required for the treatment of atherosclerotic renal artery stenosis (ARAS). To determine whether RAS could be effective in patients with ARAS, it is important to assess patient-specific characteristics such as progression of renal failure (e.g. acute worsening), presence of bilateral ARAS, or the existence of a solitary functional kidney, as well as parameters of related examinations such as the renal arterial resistive index.>
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