trimethoprim--sulfamethoxazole-drug-combination and Amyloidosis

Whipple's disease (WD) is a chronic infection caused by Thropheryma whipplei that usually manifests with intestinal, articular, pulmonary, neurological and cardiac abnormalities. Rarely, WD has been associated with renal AA amyloidosis.We report a 50 year-old male with nephrotic syndrome and renal failure whose renal biopsy revealed extensive AA amyloidosis. Amyloid was not found in other organs, namely in gastrointestinal tract and bone marrow. There was no evidence of chronic inflammatory disease, and despite detailed investigation, the diagnosis of the underlying disease remained obscure. Eight months after referral he started peritoneal dialysis. Three years later he developed anorexia, weight loss, anemia, and recurrent attacks of non-bloody diarrhea. A biopsy of the small intestine showed typical histological findings of WD and PCR was positive for T. whipplei. He was treated with ceftriaxone followed by co-trimoxazole, with remission of complaints and histological features. Three years later the patient underwent successful cadaveric kidney transplantation. In this case, AA amyloidosis preceded the manifestations of WD. To the best of our knowledge, this is the first report of kidney transplantation in a patient with amyloidosis due to WD. Recurrence of amyloidosis in renal graft is not expected.

Topics: Amyloidosis; Anti-Bacterial Agents; Ceftriaxone; Duodenum; Humans; Kidney; Kidney Transplantation; Male; Middle Aged; Nephrotic Syndrome; Serum Amyloid A Protein; Trimethoprim, Sulfamethoxazole Drug Combination; Tropheryma; Whipple Disease

Hyperkalemia is a serious electrolyte disorder and is a frequent finding in renal transplant recipients. Trimethoprim-induced hyperkalemia has been increasingly reported in recent years. We describe two renal transplant recipients who developed end-stage renal disease secondary to familial Mediterranean fever and presented with severe hyperkalemia secondary to the use of standard dose of trimethoprim. One of the patients had potential underlying adrenal insufficiency, which might be a contributing factor for the development of hyperkalemia. We concluded that renal transplant patients receiving even the standard dose of trimethoprim should be monitored closely for the development of hyperkalemia. They should be recognized as a group with increased risk in regard to their concurrent renal insufficiency, concomitant use of cyclosporine, and associated tubulointerstitial disease. Patients with secondary amyloidosis are at even greater risk, and subclinical adrenal insufficiency may be an underlying risk factor for the development of severe, life-threatening hyperkalemia among this group of patients.

Topics: Adult; Amyloidosis; Anti-Infective Agents; Familial Mediterranean Fever; Humans; Hyperkalemia; Immunosuppressive Agents; Kidney Failure, Chronic; Kidney Transplantation; Male; Trimethoprim, Sulfamethoxazole Drug Combination

Seven patients with familial amyloid neuropathy (AF amyloidosis) were studied to clarify the pathophysiology of the diarrhea associated with this disorder. Fecal weight and fat determinations, 14C-glycocholate breath tests, and a test of B12 absorption were performed before and after treatment with co-trimoxazole. Gastric emptying was assessed with conventional roentgen contrast medium and radio-opaque markers. Gastric emptying was delayed, fecal weight and fat excretion increased, and the 14C-glycholate breath test abnormal in all but one patient. In most cases co-trimoxazole reduced diarrhea, steatorrhea, and 14C-glycine deconjugation; vitamin B12 absorption returned to normal in one patient after co-trimoxazole treatment. In a jejunal mucosal biopsy specimen, amyloid was absent in the villi, but small deposits were detected along small vessels and nerves in the lamina propria. These findings suggest altered gastrointestinal motility due probably to an autonomic neuropathy which in turn leads to enteral bacterial overgrowth and subsequently to diarrhea and steatorrhea. This diarrhea can be temporarily alleviated by co-trimoxazole treatment.

Topics: Adult; Aged; Amyloidosis; Breath Tests; Celiac Disease; Diarrhea; Drug Combinations; Female; Gastric Emptying; Glycocholic Acid; Humans; Intestinal Mucosa; Male; Middle Aged; Nervous System Diseases; Sulfamethoxazole; Trimethoprim; Trimethoprim, Sulfamethoxazole Drug Combination