Familial amyloidotic polyneuropathy (FAP) caused by amyloidogenic transthyretin (ATTR) mutations is the most common form of hereditary amyloidosis. We investigated the diagnostic value of the bone scanning agent technetium-99m 3,3-diphosphono-1,2-propanodicarboxylic acid (99mTc-DPD) in this disease. Eight patients (four males, four females; age 54.4+/-8.3 years, range 43-67 years) with ATTR-FAP proven by immunohistochemistry and molecular analysis and a control group comprising ten oncological out-patients (five males, five females; age 53.4+/-8.5 years, range 34-66 years) without evidence of bony metastases were studied using 99mTc-DPD. Whole body tracer retention was 80.1%+/-10.3% (range 65.1%-94.8%) in FAP patients and 55.7%+/-8.1% (range 40.2%-66.7%) in controls at 3 h p.i. (P<0.001), and cardiac uptake was 7.3%+/-2.2% (range 4.2%-10.1%) in FAP patients and 3.1%+/-0.5% (range 2.3%-4.0%) in controls (P<0.001). The heart/whole body uptake ratio was 8.9%+/-1.7% (range 6.5%-11.0%) in FAP patients and 5.6%+/-0.5% (range 5.1%-6.8%) in controls (P<0.001). The three FAP patients with the highest cardiac tracer uptake had cardiomyopathy or arrhythmia. 99mTc-DPD scintigraphy is proposed as a simple and valuable diagnostic aid to evaluate the severity of the disease and the risk of concomitant heart problems.
Background and Purpose-Deep vein thrombosis and pulmonary embolism (PE) prove venous embolic activity and enforce the suspicion of paradoxical embolism in patients with stroke with patent foramen ovale. Because it has implications in secondary prevention, we investigated the frequency of silent PE in such a cohort of patients. Methods-Patients with cryptogenic stroke or transient ischemic attack and patent foramen ovale who underwent a ventilation perfusion scintigraphy were identified from a stroke registry. Blinded from clinical data, ventilation perfusion scintigraphy scans were re-evaluated independently by 2 experts. Patients showing at least a subsegmental defect were considered as having silent PE. Factors potentially associated with PE were analyzed. Results-The evaluation included 151 patients. Median age was 55.2 years and 59.9% were male. In 56 (37%) patients, silent PE was found; a deep vein thrombosis was evident in 11 (7%) patients. Atrial septal aneurysm was identified in 39 patients and hypermobile atrial septum in 37 patients. Atrial septal aneurysm and hypermobile atrial septum were independently associated with PE. In females, intake of oral contraceptives showed certain association with PE (6 of 25 versus 3 of 40; Pϭ0.07). Conclusions-Silent PE frequently occurs in patients with cryptogenic stroke and patent foramen ovale, particularly when atrial septal aneurysm or hypermobile atrial septum are present. (Stroke. 2011;42:822-824.)
In rheumatic diseases, radiation synovectomy is a reliable method. Meanwhile, radiation synovectomy is an outpatient therapy. In addition, combination with arthroscopic synovectomy is an increasing therapeutic modality. In comparison to the hitherto inpatient modality, a greater lymphatic emigration of the radionuclide and, therefore, a higher radiation exposure is possible. In 35 patients we found radionuclide emigration in 17 cases by whole body scintigraphy, resulting in a 50%-percentile with 68.27%-confidence interval of 1.8 (0.45-4.78)% of the injected yttrium-90-activity. Comparison of 3 groups with the above mentioned therapy modalities resulted in no statistical difference (p>0.05). Because of the found radionuclide emigration, a radiation dose of 0. 1 (0.05-0,18) mSv in women and 0.2 (0.1-0.38) mSv in men was calculated. For lymph nodes, liver, spleen and whole body radiation doses of 619 (154-1644) mSv, 62 (15-165) mSv, 62 (15-165) mSv and 37 (9-99) mSv were calculated. Gonadal radiation dose can be neglected and the morbidity rate for tumors because of the whole body radiation dose is low with a value of 0.4 per thousand. Therefore, radiation synovectomy can be used unlimited by patients age and independent of the therapeutic modality.
The DL50 value has an influence on therapeutic success of RIT. This influence is only discrete and not likely to produce clinically relevant effects in the practical use of RIT.
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