Current barriers and recommendations on the diagnosis of transthyretin amyloid cardiomyopathy: a Delphi study.

Objectives: This study has been conducted to investigate the non-invasive diagnostic journey of patients with a transthyretin amyloid cardiomyopathy (aTTR-CM) in Turkey, identify the challenges and uncertainties encountered on the path to diagnosis from the perspectives of expert physicians, and develop recommendations that can be applied in such cases. Methods: This study employed a three-round modified Delphi method and included 10 cardiologists and five nuclear medicine specialists. Two hematologists also shared their expert opinions on the survey results related to hematological tests during a final face-to-face discussion. A consensus was reached when 80% or more of the panel members marked the "agree/strongly agree" or "disagree/strongly disagree" option. Results: The panelists unanimously agreed that the aTTR-CM diagnosis could be established through scintigraphy (using either 99mTc-PYP, 99mTc-DPD, or 99mTc-HMPD) in a patient with suspected cardiac amyloidosis (CA) without a further investigation if AL amyloidosis is ruled out (by sFLC, SPIE and UPIE). In addition, scintigraphy imaging performed by SPECT or SPECT-CT should reveal a myocardial uptake of Grade ≥2 with a heart-to-contralateral (H/CL) ratio of ≥1.5. The cardiology panelists recommended using cardiovascular magnetic resonance (CMR) and a detailed echocardiographic scoring as a last resort before considering an endomyocardial biopsy in patients with suspected CA whose scintigraphy results were discordant/inconclusive or negative but still carried a high clinical suspicion of aTTR-CM. Conclusion: The diagnostic approach for aTTR-CM should be customized based on the availability of diagnostic tools/methods in each expert clinic to achieve a timely and definitive diagnosis.

68Ga-PSMA PET/CT Images of Multiple Cutaneous Metastases in a Patient With Prostate Carcinoma: Complete Response to 177Lu-PSMA-617 Treatment.

ABSTRACT: Although distant cutaneous metastasis is an uncommon presentation of prostate cancer, it remains an important diagnostic consideration as an advanced disease and a poor prognosis. Herein we present a rare case of prostate cancer patient whose multiple cutaneous metastases were treated with 177Lu-PSMA-617 radioligand treatment. To our knowledge, it is the first report in which a complete response was seen with 68Ga-PSMA PET/CT scan after 177Lu-PSMA-617 radioligand treatment.

Assessment of clinical characteristics of cardiac amyloidosis as a potential underlying etiology in patients diagnosed with heart failure with preserved ejection fraction.

BACKGROUND: Heart failure with preserved ejection fraction (HFpEF) is heterogeneous clinical syndrome. Transthyretin cardiac amyloidosis (CA) is an underdiagnosed cause of HFpEF. Red flags are extremely useful for suspecting CA. AIMS: We aimed to evaluate the frequency of cardiac and extracardiac manifestations of CA in HFpEF patients based on red flags. METHODS: Baseline characteristics of 85 patients were recorded during admission. Electrocardiogram and echocardiography were performed. All patients were examined for red flags. Cardiac scintigraphy was performed in 85 patients. RESULTS: The mean (standard deviation [SD]) age of the study group was 67.9 (9.8) years, and 52 (61.2%) patients were female. At least 1 red flag was observed in 67% of HFpEF patients. Only 4 of the patients had more than 3 red flags. The mean number of red flags in a patient with HFpEF was 1.3. Extracardiac clinical red flags were observed in only 9 (10.5%) patients. Cardiac clinical red flags were extremely rare. An electrocardiographic red flag was detected in 2 out of 10 patients and an echocardiographic red flag in 4 out of 10 patients with HFpEF. Scintigraphy showed that 17.6% of all patients have had a grade 2 or 3 cardiac uptake. The patients with wild-type transthyretin CA had twice as many red flags as those without. CONCLUSION: The results of the study showed that patients diagnosed with HFpEF had an average of 1.3 red flags suggestive of CA. In real life, extracardiac red flags are rare, while electrocardiographic and echocardiographic red flags are more common in patients with HFpEF.

Bone mineral density and vitamin D status in children with remission phase of steroid-sensitive nephrotic syndrome.

Children with idiopathic nephrotic syndrome are primarily treated with glucocorticoids (GCs), but long-term GC use can lead to undesired side effects. We investigated the bone mineral density (BMD) and 25-hydroxyvitamin D (25-OH D) levels in children with the remission phase of steroid-sensitive nephrotic syndrome (SSNS). This study included 32 patients with SSNS who had not received GC treatment in the last 6 months and a control group of 20 healthy children. Serum levels of calcium, phosphate, alkaline phosphatase, 25-(OH)D, and parathyroid hormone (PTH) were measured. BMD was determined in the lumbar spinal region using dual-energy X-ray absorptiometry (DEXA). Serum 25-(OH)D levels were lower in the SSNS patients than in the healthy children (P <0.05), with 22 patients (68.8%) having Z-scores <-1. The Z-scores were positively correlated with 25-(OH)D levels (r = 0.424, P <0.05). PTH levels were higher in patients with osteoporosis than in patients with Z-scores ≥-1 (P <0.05). Bone mineral content and BMD were positively correlated with the age of diagnosis (P <0.01). Receiver-operating characteristic curve analysis showed that the cutoff value of 25-(OH)D levels for predicting low BMD was 14.67 ng/mL with a sensitivity of 90% and a specificity of 64%. The area under the curve (AUC ± standard error) was 0.868 ± 0.064 (95% confidence interval: 0.742-0.994, P = 0.001). Decreased 25-(OH)D levels and the negative effects of long-term GC treatment on BMD persist in SSNS remission phase. Levels of 25-(OH)D <14.67 ng/mL could predict abnormal DEXA scans in children with SSNS remission phase.

Which of the fluorine-18 fluorodeoxyglucose positron emission tomography/computerized tomography parameters are better associated with prognostic factors in breast cancer?

The aim of the present study is to evaluate the relationship between the immunohistochemical and histopathological prognostic factors and the metabolic fluorine-18 fluorodeoxyglucose positron emission tomography/computerized tomography (PET/CT) parameters in breast cancer.A total of 94 female patients diagnosed with primary breast cancer (median age: 54.5 years, 94 lesions with size >15 mm) who underwent PET/CT imaging before any treatment were enrolled to this retrospective study. Maximum and average standardized uptake values (SUVmax and SUVavg), metabolic tumor volume (MTV), total lesion glycolysis (TLG), and tumor/liver uptake ratio (TLR) of the primary tumors were calculated and compared between various histopathological and immunohistochemical prognostic factor groups.All metabolic parameters were associated with clinical T stage, metabolic M stage, and nuclear grade. The MTV, TLG, and TLR were significantly higher in patients with suspected lymph node metastasis. There were significant differences according to estrogen receptor and human epidermal growth factor-2 status in the metabolic values other than MTV. In case of progesterone receptor, there were significant differences in the metabolic characteristics except for the MTV and TLG values. The Ki-67 labeling index was moderately correlated with SUVmax, SUVavg, and TLR. All metabolic characteristics except MTV were significantly higher in triple negative breast cancer compared with the other molecular subtypes.The results of the present study suggest that the TLG and TLR values have stronger associations with several prognostic factors in breast cancer (BC) compared with other metabolic parameters.