Amyloidosis is a severe disease caused by protein misfolding and deposition in tissues and organs. Thirty-eight different proteins are known to be amyloidogenic. Amyloidosis is categorized into inherited or acquired, and systemic or localized. Light-chain (AL)- and transthyretin (ATTR) amyloidosis are the two most common subtypes. Awareness, early diagnosis, accurate subtyping and relevant treatment are crucial for the management. Novel therapies of systemic AL and ATTR amyloidosis have considerably improved outcome and survival. The aim of this review is to increase awareness and knowledge on diagnosing amyloidosis.
Importance Scintigraphy and ultrasonography are common imaging modalities for the preoperative localization of enlarged parathyroid glands in primary hyperparathyroidism. When comparing the 2 modalities, the benefits of ultrasonography in terms of convenience, patient comfort, duration, cost, and lack of radiation should be taken into account. Objective To investigate whether surgeon-performed ultrasonography, with or without contrast-enhanced ultrasonography (CEUS), is noninferior to scintigraphy for localizing pathological parathyroid glands in patients with primary hyperparathyroidism. Design, Setting, and Participants Prospective, paired, noninferiority cohort study performed at a high-volume tertiary referral center for parathyroidectomy with blinded examiners and a 6-month follow-up. Participants were adults (age ≥18 years) referred for parathyroidectomy due to primary hyperparathyroidism. Of 207 eligible patients, 35 were excluded, leaving 172 enrolled in the study. Inclusion lasted from September 2019 until February 2021. Follow-up ended in December 2021. Exposures 99m Technetium-pertechnetate/ 99m technetium-sestamibi subtraction scintigraphy with 99m technetium-sestamibi single-photon emission computed tomography/computed tomography, followed by surgeon-performed ultrasonography and CEUS. Main Outcomes and Measures The sensitivity of each imaging modality in localizing pathological parathyroid glands, calculated on a per-quadrant and a per-patient basis, respectively. The a priori noninferiority margin was a lower 95% confidence limit for the difference in sensitivity not falling below −10%. Results Out of 172 participants, 139 (80.8%) were women, the median (range) age was 65 (24-87) years, and the median (IQR) follow-up was 200.5 (181-280.25) days. Quadrant sensitivity (95% CI) was 70.9% (63.2%-78.5%) for ultrasonography, 68.4% (60.4%-76.5%) for ultrasonography plus CEUS, and 67.0% (60.0%-74.0%) for scintigraphy. The sensitivity difference (95% CI) compared with scintigraphy was 3.9% (−4.1% to 11.8%) for ultrasonography and 1.5% (−6.4% to 9.3%) for ultrasonography plus CEUS, establishing noninferiority for both modalities. Per-patient sensitivity was 81.4% (74.8%-86.9%) for ultrasonography and 79.1% (72.2%-84.9%) for both scintigraphy and ultrasonography plus CEUS. The sensitivity difference compared with scintigraphy was 2.3% (−6.8% to 11.4%) for ultrasonography and 0.0% (−9.1% to 9.1%) for ultrasonography plus CEUS, establishing noninferiority for both modalities. Conclusions and Relevance In this cohort study, conventional ultrasonography by an experienced parathyroid surgeon-sonographer was noninferior to scintigraphy and may constitute a valid first-line imaging modality in patients with primary hyperparathyroidism, even without the addition of CEUS. Further imaging should be reserved for cases that are equivocal or nonlocalizing on ultrasonography.
The use of 18F-Fluoro-D-deoxy-glucose -positron emission tomography/computed tomography (FDG-PET/CT) in gastrointestinal (GI)-malignancies may not be as straightforward as in many other cancers, but the potential is clearly there in select clinical settings. The challenges include the relative non-specificity of FDG, the variable degrees of physiologic FDG-uptake, and the heterogeneous FDG-uptake in different cell types within the GI-domain, which all together hamper the use in primary diagnostics. In general, the literature is older, heterogeneous, and based on stand-alone PET, which is now largely considered obsolete. There is emerging evidence for use of hybrid PET/CT, but the literature is still relatively sparse. The main indications are preoperative staging of distant metastases, not only in limited disease but also before curative treatment of limited metastatic disease. Controversies remain concerning liver metastases but improved technology boast well for the future role of FDG-PET/CT not least concerning equivocal findings on conventional imaging. In our opinion, an important upcoming indication is early response assessment, perhaps mostly in the neoadjuvant settings of upper GI-malignancies, but standardization of response assessment criteria is lacking before a more widespread implementation is feasible. Finally, there seems to be a significant role in recurrence detection, especially in CRC.
Perfluorinated chemicals (PFCs) have been widely used and have emerged as important food contaminants. A recent study on pregnant women suggested that PFC exposure was associated with a longer time to pregnancy (TTP). We examined the association between serum concentrations of PFCs in females and TTP in 222 Danish first-time pregnancy planners during the years 1992–1995. The couples were enrolled in the study when discontinuing birth control and followed for six menstrual cycles or until a clinically recognized pregnancy occurred. Fecundability ratio (FR) was calculated using discrete-time survival models. In addition, odds ratio (OR) for TTP >6 cycles was calculated. OR for TTP >6 cycles for those with PFC concentrations above the median were 0.96 [95% confidence interval (CI): 0.54–1.64] for perfluorooctane sulfonic acid (PFOS), the major PFC, compared with those below the median. FRs for those with PFOS concentrations above the median were 1.05 (95% CI: 0.74–1.48) compared with those below the median. Other PFCs showed the same lack of association with TTP. The results were not affected by adjustment for covariates. PFOS and perfluorooctanoic acid concentrations were similar to those observed in a previous Danish study. These findings suggest that exposure to PFCs affects TTP only to a small extent, if at all.
