[AL-amyloidosis and its treatment by eliminating the precursor protein]. / AL-amyloïdose en de behandeling door eliminatie van het voorlopereiwit.

AL amyloidosis was diagnosed in 2 patients, women aged 61 and 43 respectively. The first patient, who had a nephrotic syndrome, died soon after diagnosis as the disease appeared to be already widespread. The second patient was still alive at the last follow-up, 17 years after diagnosis, because of effective elimination of her light chains by high-dose chemotherapy. AL amyloidosis is a rare, but severe, systemic disease with high mortality. Its aetiology lies in deregulated plasma cells producing excessive numbers of free immunoglobulin light chains. These light chains are the precursor proteins of amyloid fibrils. Amyloid fibrils are deposited extracellularly in tissue leading to organ dysfunction. Symptomatology is diverse, often non-specific, and generally not very well-known. Therefore, the diagnosis is often delayed for a long time. This is unfortunate, as high-dose chemotherapy targeted at elimination of the precursor protein considerably improves prognosis. However, this type of therapy can only be given in patients with limited and moderately progressive disease.

Fatal rupture of a mycotic aneurysm of the right coronary artery post pneumococcal sepsis.

Aneurysms of the coronary arteries are rare and mycotic coronary aneurysms are even rarer. We report a unique, yet unfortunately autopsy-proven fatal case of a ruptured atherosclerotic mycotic aneurysm of the right coronary artery with streptococcus pneumoniae in a non-immunocompromised patient resulting in cor tamponade and death.

[Flank pain caused by a renal artery dissection]. / Flankpijn door een nierarteriedissectie.

BACKGROUND: A spontaneous renal artery dissection is a very rare diagnosis. The clinical presentation can vary and its course can be atypical. There are no guidelines available regarding treatment; however, the options are a conservative (medication) or interventional (radiological or surgical) approach. CASE DESCRIPTION: A 45-year-old man presented to the emergency department with hypertensive urgency after earlier episodes of flank pain. The cause appeared to be a spontaneous bilateral renal artery dissection with infarction. After a multidisciplinary consultation, the decision was made to manage the patient conservatively since symptoms had subsided, blood pressure was acceptable and renal function remained stable. Eventually, kidney function restored to normal and CT images showed almost complete recovery of the previously damaged renal parenchyma. CONCLUSION: This case demonstrates that in the event of renal artery dissection, a conservative medication policy may be a good option in clinically stable patients with non-deteriorating renal function. Timely recognition and adequate follow-up are important to prevent serious complications, such as renal ischaemia or renal infarction that could necessitate a nephrectomy.

Diagnostic use of angiotensin converting enzyme inhibitors in radioisotope evaluation of unilateral renal artery stenosis.

Iodine-123 hippurate renography, [99mTc]diethylenetriaminepentaacetic acid (DTPA) renography, and [99mTc]dimercapto succinic acid (DMSA) renal scintigraphy were performed before and during angiotensin converting enzyme (ACE) inhibition in a group of 15 hypertensive patients with angiographically "significant" unilateral renal artery stenosis. Visual and quantitative evaluation of the three radioisotope methods before ACE inhibition already disclosed abnormalities suggestive of renal artery stenosis in a high percentage (87%, 60%, and 60%, respectively) in this group of patients, but ACE inhibition further improved the diagnostic yield in all three methods (93%, 86%, and 80%). Iodine-123 hippurate renography was at least as useful as [99mTc]DTPA renography in this respect, while [99mTc]DMSA scintigraphy can be used particularly in segmental stenosis. Despite a large drop in blood pressure after ACE inhibition little adverse reactions were seen and overall renal function was fairly well maintained, the exceptions noted in patients with initially a more impaired renal function.

4-Aminopyridine and haemodialysis in the treatment of verapamil intoxication.

Verapamil, a calcium antagonist, is used as an antianginal, antidysrhythmic and antihypertensive agent. Fatal intoxications with this commonly used drug have been described. We report the effects of 4-aminopyridine and haemodialysis in a patient with severe verapamil intoxication.

Restenosis prevalence and long-term effects on renal function after percutaneous transluminal renal angioplasty.

We studied the frequency of restenosis and the effects of percutaneous transluminal renal angioplasty (PTRA) on blood pressure (BP) and particularly on renal function in all patients in whom successful dilatation was performed. Restenosis was found in 42% of the patients with an atherosclerotic renal artery stenosis and in 22% of the patients with fibromuscular dysplasia. BP improvement was seen in 70-80% of the patients with unilateral stenosis or with successful bilateral dilatation (group I) whereas only seldom was an effect on BP observed in patients with more complicated disease, such as those with an occlusion of the contralateral artery (group II). Remarkably, however, both in group I and in group II, in about half of the patients an improvement in renal function was found, even 2-3 years after the procedure. Our results thus indicate that PTRA can be useful in preservation or even improvement of renal function, even if no effect on blood pressure can be expected.