[Gastrointestinal amyloidosis in a patient with multiple myeloma]. / Gastro-intestinale amyloïdose bij multipel myeloom.

BACKGROUND: Gastrointestinal amyloidosis is characterized by deposition of amyloid in the gastrointestinal mucosa. This can cause an increased risk of malabsorption, obstruction, gastrointestinal bleeding and perforations. CASE DESCRIPTION: A 52-year-old male presented with rectal bleeding. Endoscopic evaluation revealed non-specific red and purple lesions in the sigmoid colon. Histological results were inconclusive. Six weeks later this patient presented at the emergency room with an intestinal perforation. He also had newly developed renal insufficiency. Further analysis led to the diagnosis of multiple myeloma. After 3 doses of bortezomib and dexamethasone the patient was admitted with abdominal pain and rectal bleeding. Revision of our earlier sigmoid biopsies revealed gastrointestinal amyloidosis. The patient died 4 months after initial presentation. CONCLUSION: Gastrointestinal amyloidosis is a rare disease. Due to non-specific symptoms, diagnosis can easily be missed in patients who have not been diagnosed previously with amyloidosis or a related disease.

Increased carotid and femoral intima-media thickness in patients after repair of aortic coarctation: influence of early repair.

BACKGROUND: In patients, after repair of aortic coarctation, abnormal function of the proximal precoarctation arterial conduits has been demonstrated, but data on arterial wall structure of proximal and distal arteries in patients are scarce. The aim of our study is to compare intima-media thickness (IMT) of the carotid and femoral arteries in controls and patients after coarctation repair and to identify independent predictors of carotid and femoral IMT. METHODS: A total of 137 patients after repair of aortic coarctation (89 male, median age 29.7 years, range 17-74 years, and median age at repair 6.3 years, range 0.01-46 years) and 46 age-matched controls underwent ambulatory blood pressure monitoring and B-mode ultrasound of the carotid and femoral arteries. RESULTS: Twenty (15%) of the 137 patients were operated on before the age of 1 year ("early repair"). The patients with an early repair were younger compared to the controls and compared with the patients with a late repair (24.5 [10.5] vs 31.6 [8.4] years, P = .005, and vs 32.1 [10.5] years, P < .001, respectively). Eighty patients (59%) had hypertension on the basis of the results of the ambulatory blood pressure monitoring (mean daytime blood pressure > or = 135/85 mm Hg). The prevalence of hypertension was not significantly different between patients with an early or late repair. Although patients with an early repair were younger, both carotid and femoral IMT were increased in these patients compared to controls (common carotid artery 0.57 [0.11] vs 0.49 [0.07] mm, P < .001; common femoral artery 0.48 [0.10] vs 0.44 [0.05] mm, P = .033). In multivariable regression analyses, age at repair was only an independent predictor of femoral and not of carotid IMT. CONCLUSIONS: Patients, after repair of aortic coarctation, have increased carotid and femoral IMT despite normal ambulatory blood pressures and successful previous repair. Early repair seems to preserve postcoarctation arterial wall structure, but seems to have only limited effect on precoarctation vessel walls, which supports the theory of a developmental defect of the proximal aorta and its branches.