Evaluation of coronary artery abnormalities in Williams syndrome patients using myocardial perfusion scintigraphy and CT angiography

Background: Sudden death risk in Williams syndrome (WS) patients has been shown to be 25–100 times higher than in the general population. This study aims to detect coronary artery anomalies and myocardial perfusion defects in WS patients using noninvasive diagnostic methods. Methods: This study features 38 patients diagnosed with WS. In addition to physical examination, electrocardiography, and echocardiography, computed tomography (CT) angiography and rest/dipyridamole stress technetium-99m sestamibi (99mTc-sestamibi) single photon emission computed tomography (SPECT) myocardial perfusion scintigraphy (MPS) were performed. Results: Twenty-one (55%) patients were male; 17 (45%) were female. The average patient age was 12 ± 5 years (2.5–26 years); the average follow-up period was 7.2 ± 4.2 years (6 months–18 years). Cardiovascular abnormalities were found in 89% of patients, the most common one being supravalvar aortic stenosis (SVAS). CT angiography revealed coronary anomalies in 10 (26%) patients, the most common ones being ectasia of the left main coronary artery and proximal right coronary artery as well as myocardial bridging. SVAS was present in 80% of patients with coronary artery anomalies. 99mTc-sestamibi SPECT MPS revealed findings possibly consistent with myocardial ischemia in 29% of patients, and ischemia in 7 out of 10 patients (70%) with coronary anomalies shown on CT angiography (p = 0.03). Conclusions: Coronary artery abnormalities are relatively common in WS patients and are often accompanied by SVAS. CT angiography and dipyridamole 99mTc-sestamibi SPECT MPS seem to be less invasive methods of detecting coronary artery anomalies and myocardial perfusion defects in WS patients

A Case with Non-Small Cell Cancer in the Left Lung Diagnosed Following Observation of Bilateral Diffuse Lung Uptake of Tc-99m-MDP on Bone Scintigraphy

Extraskeletal accumulation of Technetium-99m methylene diphosphonate (Tc-99m-MDP) can be seen in bone scintigraphy both in benign and malignant lesions. In malignant lesions, this phenomenon is usually associated with microscopic calcifications due to the abnormal calcium metabolism, and occurs most frequently in the lungs. In this case report, we present a patient with a non-small lung cancer diagnosed following observation of incidental bilateral lung MDP accumulation in bone scintigraphy. Therefore, the tracer distribution in the soft tissues needs to be carefully examined on bone scans and any unexpected visible soft tissue activity should be stated on the scintigraphy report

Appearance of situs inversus totalis and polysplenia syndrome on FDG PET/CT

A 49-year-old woman with a history of breast cancer had undergone surgery, chemotherapy, and radiation therapy for local disease. The patient was referred to our PET/CT department to search for possible metastatic disease. After a 6-hour fast (serum glucose level 120 mg/dL), the patient was injected with 14.9 mCi (555 MBq) F-18 of FDG intravenously. After a I-hour waiting period, the patient was imaged using a dedicated Siemens Biograph LSO HI-REZ integrated PET/CT camera. The CT scan without intravenous contrast was used for anatomic landmarking and attenuation correction. In addition to the metastatic disease on the anterior chest wall, there was situs inversus. Also CT images of the PET/CT study showed multiple spleens along the greater curvature of the stomach in the right side of the upper abdomen

Evaluation of coronary artery abnormalities in Williams syndrome patients using myocardial perfusion scintigraphy and CT angiography

Background: Sudden death risk in Williams syndrome (WS) patients has been shown to be 25-100 times higher than in the general population. This study aims to detect coronary artery anomalies and myocardial perfusion defects in WS patients using noninvasive diagnostic methods

Protective Effects of Erythropoietin and N-Acetylcysteine on Methotrexate-Induced Lung Injury in Rats

Objective: Methotrexate (MTX) is known to have deleterious side effects on lung tissue. We aimed to investigate the effects of erythropoietin (EPO) and N-acetyl-cysteine (NAC) on MTX-induced lung injury in rats. Study Design: Animal experiment. Material and Methods: Twenty-six female Sprague-Dawley rats were divided into 4 groups. Sham group, 0.3 mL saline; MTX group, 5 mg/kg MTX; EPO group, 5mg/kg MTX and 2000 IU/kg EPO; NAC group, 5 mg/kg MTX and 200 mg/kg NAC were administered once daily for 4 consecutive days. Malondialdehyde (MDA), superoxide dismutase (SOD), catalase (CAT) and inflammation and congestion scores in lung tissues were evaluated. Results: In MTX group MDA were significantly higher, CAT and SOD were significantly lower than in sham, EPO and NAC groups (p0.005). In group MTX both scores were significantly higher than in sham (p<0.005). The congestion score of group MTX was significantly higher than those of group EPO and NAC (p<0.005). Conclusion: EPO and NAC have significant preventive effects on MTX-induced lung injury in rats. Decreased antioxidant capacity and increased MDA level may cause the oxidative damage in MTX group. Also, higher antioxidant capacity and lower MDA level may be a response to oxidative stress in EPO and NAC groups

The management and the diagnosis of fever of unknown origin

Prolonged fever presents a challenge for the patient and the physician. Fever with a temperature higher than 38.3 degrees C on several occasions that lasts for at least 3 weeks and lacks a clear diagnosis after 1 week of study in the hospital is called a fever of unknown origin (FUO). More than 200 diseases can cause FUO, and the information gathered from history taking, physical examination, laboratory and imaging studies should be evaluated with care. History taking and physical examination may provide some localizing signs and symptoms pointing toward a diagnosis. Infection, cancers, noninfectious inflammatory diseases and some miscellaneous diseases are the main etiologies, and some patients remain undiagnosed despite investigations. Tuberculosis, lymphoma and adult-onset Still's disease are the main diseases. Fluorodeoxyglucose PET is a promising imaging modality in FUO. Establishing a uniform algorithm for FUO management is difficult. Every patient should be carefully evaluated individually considering the previous FUO management experience

Immunosuppressive treatment results in patients with primary IgA nephropathy in Turkiye; the data from TSN-GOLD working group

Background Immunoglobulin A (IgA) nephropathy (IgAN) treatment consists of maximal supportive care and, for high-risk individuals, immunosuppressive treatment (IST). There are conflicting results regarding IST. Therefore, we aimed to investigate IST results among IgAN patients in Turkiye.Method The data of 1656 IgAN patients in the Primary Glomerular Diseases Study of the Turkish Society of Nephrology Glomerular Diseases Study Group were analyzed. A total of 408 primary IgAN patients treated with IST (65.4% male, mean age 38.4 ± 12.5 years, follow-up 30 (3–218) months) were included and divided into two groups according to treatment protocols (isolated corticosteroid [CS] 70.6% and combined IST 29.4%). Treatment responses, associated factors were analyzed.Results Remission (66.7% partial, 33.7% complete) was achieved in 74.7% of patients. Baseline systolic blood pressure, mean arterial pressure, and proteinuria levels were lower in responsives. Remission was achieved at significantly higher rates in the CS group (78% vs. 66.7%, p = 0.016). Partial remission was the prominent remission type. The remission rate was significantly higher among patients with segmental sclerosis compared to those without (60.4% vs. 49%, p = 0.047). In the multivariate analysis, MEST-C S1 (HR 1.43, 95% CI 1.08–1.89, p = 0.013), MEST-C T1 (HR 0.68, 95% CI 0.51–0.91, p = 0.008) and combined IST (HR 0.66, 95% CI 0.49–0.91, p = 0.009) were found to be significant regarding remission.Conclusion CS can significantly improve remission in high-risk Turkish IgAN patients, despite the reliance on non-quantitative endpoints for favorable renal outcomes. Key predictors of remission include baseline proteinuria and specific histological markers. It is crucial to carefully weigh the risks and benefits of immunosuppressive therapy for these patients