Erdheim-Chester disease and nuclear medicine imaging. A case report and brief review.

Erdheim-Chester disease (ECD) is a rare clonal myeloid neoplasm typically affecting adults over 50 years old, with bone lesions in almost all patients. The prognosis is poor in most cases if left untreated. Clinical manifestations are not specific, which hinders early diagnosis. The disease has distinct radiological features. However, three-phase bone scintigraphy exhibits the most typical pattern of all imaging modalities, which is the prominent strikingly symmetrical radiotracer uptake in the distal ends of the femurs and proximal and distal ends of the tibiae, sparing the epiphyses. We report a case of a 54-year-old female patient, presenting with atypical persistent knee joint pain. After an MRI scan, she underwent a three-phase bone scan, revealing the characteristic pattern, thus indicating a possible ECD diagnosis, which was eventually confirmed in biopsy material. Novel aspects of the pathophysiology and treatment of the disease, as well as a differential diagnosis from the perspective of an MSK radiologist and nuclear medicine physician, are also discussed.

Position statement on the non-invasive diagnosis of patients with ATTR pardiac amyloidosis, endorsed by the Hellenic Society of Nuclear Cardiology, for the Nuclear Medicine practitioners.

Cardiac amyloidosis is a rare condition characterized by the accumulation of abnormal proteins called amyloids in the heart tissue. These amyloids can disrupt the normal functioning of the heart and lead to a variety of symptoms and complications.

Serum leptin, adiponectin and ghrelin concentrations in post-menopausal women: Is there an association with bone mineral density?

OBJECTIVE: Adipokines and ghrelin exert well-documented effects on energy expenditure and glucose metabolism. Experimental data also support a role in bone metabolism, although data from clinical studies are conflicting. The purpose of this cross-sectional study was to investigate the association of serum concentrations of leptin, adiponectin and ghrelin with bone mineral density (BMD) in post-menopausal women. METHODS: BMD in lumbar spine and femoral neck, and circulating leptin, adiponectin and ghrelin concentrations were measured in 110 healthy post-menopausal women. Patients with secondary causes of osteoporosis were excluded. RESULTS: Osteoporosis was diagnosed in 30 (27%) women and osteopenia in 54 (49%). Serum leptin concentrations were positively correlated with both lumbar spine (r=0.343, p<0.01) and femoral neck BMD (r=0.370, p<0.01). Adiponectin concentrations were negatively associated with BMD at both sites (r=-0.321, p<0.01 and r=-0.448, p<0.01 respectively). No significant correlation between ghrelin concentrations and BMD was found. Osteoporotic women had lower body weight, body mass index (BMI) and leptin concentrations, but higher adiponectin concentrations compared with non-osteoporotic women. In multivariate stepwise regression analysis, the association of adiponectin concentrations with BMD remained significant only for femoral neck, after adjustment for body weight or BMI. CONCLUSIONS: An inverse association between adiponectin and femoral neck BMD was found in post-menopausal women, independently of body weight. The positive association between leptin and BMD was dependent on body weight, whereas no effect of ghrelin on BMD was evident.

Muscle perfusion of posterior trunk and lower-limb muscles at rest and during upper-limb exercise in spinal cord-injured and able-bodied individuals.

STUDY DESIGN: Nonrandomized-controlled trial. OBJECTIVES: To assess muscle perfusion at rest and during arm-cranking exercise (ACE) in upper and lower posterior trunk and vastus lateralis (VL) muscles in individuals with spinal cord injury (SCI) and controls (C). SETTING: Exercise Physiology-Biochemistry Laboratory. METHODS: Eight SCI with thoracic lesion and eight C received injections of radioactive tracer to trapezius (TRAP), latissimus dorsi (LAT) and VL. Radioactive counts were recorded with a γ-camera for 10 min at rest and during ACE (60% VO(2max) for 20 min). Time-count curves were generated and the isotope clearance rate, expressed as half-life time (T(1/2),min), was calculated to assess muscle perfusion. RESULTS: Resting T(1/2) was lower in TRAP and LAT vs VL (P<0.05) in SCI, however, there were no differences among muscles in C. Arm-cranking increased (P<0.001) the isotope clearance in TRAP and LAT in SCI and C, whereas no effect was found on T(1/2) in VL in both groups. T(1/2) was longer (P<0.05) in SCI vs C in VL at rest and during ACE, whereas there were no differences between groups in posterior trunk muscles. CONCLUSIONS: Resting muscle perfusion was reduced in the paralyzed limbs of SCI compared with C, whereas there was no evidence of impaired microcirculation in upper and lower back muscles in SCI. Although ACE did not induce a hyperemic response in VL, it increased hyperemia in upper and lower posterior trunk muscles in SCI, suggesting beneficial effects of this type of activity on muscle microvasculature in this region.

Intramyocardial implantation of autologous bone marrow-derived stem cells combined with coronary artery bypass grafting in patients with ischemic cardiomyopathy: a pilot study.

BACKGROUND: Ischemic cardiomyopathy has the distinctiveness of irreversible myocardial damage with scar tissue formation and mainly impaired perfusion of the remaining viable myocardium. We present results of the first series of patients with severe ischemic cardiomyopathy managed in our institution with intramyocardial implantation of autologous bone marrow stem cells at the time of coronary artery bypass grafting. The aim is to evaluate feasibility and safety of the procedure in our institution. PATIENTS AND METHODS: Nine patients with severe ischemic cardiomyopathy scheduled for elective coronary artery bypass grafting were managed with concurrent intramyocardial autologous bone marrow stem cells injection in pre-defined viable peri-infarct areas that showed poor perfusion and could not be grafted. Detailed mapping of infracted and hibernating myocardial segments was performed in all patients with single photon emission computed tomography segmental analysis. RESULTS: There was no perioperative 30-day mortality. Improvement was evident in left ventricular ejection fraction which was increased significantly from 31.3% preoperatively to 42.4%, 46.6% and 52.5% at 3, 6 and 12 months respectively. Postoperative thallium scintigraphy revealed increased perfusion in myocardial segments corresponding to areas of stem cell injection and a net reduction in the estimated infarct size at 6 and 12 months in 5/8 (62.5%) patients. CONCLUSIONS: Preliminary data from this pilot study show that intramyocardial administration of bone marrow stem cells in patients undergoing coronary bypass grafting for ischemic cardiomyopathy is safe and associated with an improvement in left ventricular function and enhanced reperfusion of non-viable myocardial territories.

The clinical significance of reverse redistribution in Tl201 cardiac SPET.

UNLABELLED: In an attempt to evaluate the clinical significance of reverse redistribution in Tl201 cardiac SPET, Tl201 SPET examinations included in the data base of our department have been retrospectively reviewed. PATIENTS: Six hundred ninety eight patients demonstrated the pattern of reverse redistribution. For 237 of these patients' cardiac catheterization data were also available. One hundred and six patients of these had a history of myocardial infarction while the rest of them were referred for angina symptoms. RESULTS: Catheterization showed that the infarction area was perfused by a stenosed but patent vessel in 79 of the 106 patients with a history of myocardial infarction (74.5%). Collateral circulation was evident in 21 of these 106 patients (19.8%). Only six patients (5.6%) showed a totally occluded vessel with no obvious evidence of collateral circulation. Of the 131 patients, with no myocardial infarction history, 14 (10.7%) had normal coronary vessels. The rest of the patients (89.3%) demonstrated stenotic vessels perfusing the same area where reverse redistribution was observed. Sixty-three of these patients have been reexamined after 9-36 months. On 15 of these patients (23.8%) the second scintigram showed ischemia in the same segments where reverse redistribution was initially detected. Another 17 of the above patients had a former scintigraphic examination that showed ischemia on the segments that currently demonstrated reverse redistribution. CONCLUSION: The results of the present retrospective study indicate that, in patients with known coronary disease, the pattern of reverse redistribution frequently indicates the ischemic area.

Somatostatin receptor scintigraphy before and after treatment with somatostatin analogues in patients with thyroid eye disease.

Octreotide, a potent synthetic long-acting somatostatin analogue, has been shown to have a beneficial effect in thyroid eye disease (TED). Orbital scintigraphy using ocetreoscan-111 is a useful study, which can be used to visualize somatostatin-receptor-bearing cells and also to select patients who might benefit from octreotide therapy. One major limitation of this therapy is that the drug must be administered parenterally and used several times daily. Lanreotide, a new somatostatin analogue, has a much longer duration of action in comparison with octreotide, and has recently been found to have a beneficial effect in the treatment of thyroid eye disease. The aim of this study was to investigate the orbital Indium-111-pentetreotide activity after treatment with octreotide and lanreotide in patients with thyroid ophthalmopathy. Fourteen patients were studied. 12 with bilateral and 2 with unilateral thyroid eye disease, (10 females and 4 males) and all with moderately severe symptoms of ophthalmopathy. All were treated with antithyroid drugs and were euthyroid at the time of the study. All patients were investigated with orbital scintigraphy using octreoscan-111 and selected for study on the basis of a positive octreoscan. Five patients received 30 mg lanreotide intramuscularly once every 2 weeks over a period of 3 months, and 5 patients received octreotide 100 microg subcutaneously thrice daily for 3 months. Four patients served as controls and received no treatment. The octreoscan-111 scintigraphy was repeated in all patients 3 months after the first examination. The NOSPECS classification and the clinical activity score (CAS) of thyroid ophthalmopathy were also evaluated before and 3 months after the initiation of treatment. All patients who received treatment had a negative follow-up octreoscan while controls had a positive octreoscan. NOSPECS score and CAS were improved with treatment, but unchanged in control patients. The reduced uptake of octreoscan may be the result of blocking of somatostatin receptors, or reduction in receptor-expressing tissues, downregulation of somatostatin receptors in target tissues, or a combination of these factors.

Characterization of the prolyl dipeptidyl peptidase gene (dppIV) from the koji mold Aspergillus oryzae.

The koji mold Aspergillus oryzae secretes a prolyl dipeptidyl peptidase (DPPIV) when the fungus is cultivated in a medium containing wheat gluten as the sole nitrogen and carbon source (MMWG). We cloned and sequenced the DPPIV gene from an A. oryzae library by using the A. fumigatus dppIV gene as a probe. Reverse transcriptase PCR experiments showed that the A. oryzae dppIV gene consists of two exons, the first of which is only 6 bp long. The gene encodes an 87.2-kDa polypeptide chain which is secreted into the medium as a 95-kDa glycoprotein. Introduction of this gene into A. oryzae leads to overexpression of prolyl dipeptidyl peptidase activity, while disruption of the gene abolishes all prolyl dipeptidyl peptidase activity in MMWG. The dppIV null mutants did not exhibit any change in phenotype other than the absence of prolyl dipeptidyl peptidase activity, suggesting that this activity is not essential. This loss of activity diminished the number of dipeptides and increased the number of larger peptides present in the MMWG culture broth. These effects were reversed by the addition of purified, recombinant DPPIV from the methylotrophic yeast expression vector Pichia pastoris. Our results suggest that the DPPIV enzyme may be of importance in industrial hydrolysis of what gluten-based substrates, which are rich in Pro residues.

Increased lung uptake during myocardial scintigraphy improves the detection and localization of coronary artery disease.

Increased lung uptake during exercise thallium 201 scanning denotes severe ventricular hypofunction. To assess whether there is any relationship between this finding and the stenosis of a single coronary artery, the authors studied 140 patients (age 57.5 +/- 12 years) with both exercise thallium and coronary angiography within three months. A planar view was acquired for two hundred forty seconds immediately postexercise and prior to the single photon emission computed tomography acquisition. Two identical regions of interest outlining the left ventricle and the right lung in the planar view were used for calculation of the lung uptake, expressed as a lung-to-heart counts ratio. The patients were divided into four angiographic groups: (I) lesions at the left anterior descending (LAD), n = 35; (II) stenosis at the circumflex (CRX), n = 35; (III) stenosis at the right coronary artery (RCA), n = 35; and (IV) another group of 35 normal subjects serving as controls. No patient had a previous myocardial infraction (MI) or any cardiac operation. Patients in groups I (0.53 +/- 0.094) and II (0.44 +/- 0.072) had higher lung uptake than those in groups III (0.43 +/- 0.061) or IV (0.42 +/- 0.050). There was no difference between groups III and IV, or II and IV, but the difference between I and IV was of statistical significance (P < 0.001).(ABSTRACT TRUNCATED AT 250 WORDS)