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A 73-year-old man with a history of marginal zone lymphoma was admitted to the emergency room for diplopia and ipsilateral headache. The Fluorine-18-fluorodeoxyglucose positron emission tomography/computed tomography (18F-FDG PET/CT) demonstrated intense and symmetrical hypermetabolism of the cavernous sinuses, and hypermetabolic lesions diffusely in the lymph nodes and bones. The diagnosis of high-grade relapse of lymphomatous disease was made. In this context, the homogenous and symmetric lesion of the cavernous sinuses, without any other encephalic or meningeal lesions, raised the hypothesis of a paraneoplastic origin. A plausible paraneoplastic link between the neuro-ophthalmological lesion and the malignant disorder is IgG4-related disease, a condition that may be associated with lymphoma. As in our case, this diagnosis is often presumptive because histopathological confirmation is difficult to obtain. [ABSTRACT FROM AUTHOR]

La collection sous-périostée orbitaire est une complication rare de la drépanocytose. Nous rapportons le cas d'une jeune fille de 16 ans, suivie pour une drépanocytose SS et hospitalisée en urgence en janvier 2019 pour une exophtalmie douloureuse. Cette jeune fille a été transfusée dans un contexte de crise vaso-occlusive (CVO) à 3 reprises dans l'année précédente. Son hémoglobine de base est à 9,5 g/dl, avec une hémoglobine S à 80 % et une hémoglobine fœtale à 14 %. Une tentative d'hydroxycarbamide en 2018 a dû être arrêtée rapidement sur une neutropénie. La patiente s'est initialement présentée aux urgences pour une CVO des membres inférieurs, ne cédant pas aux antalgiques de palier 1 et au Nefopam. A l'entrée, l'hémoglobine est à 8,8 g/dl, les réticulocytes à 129 G/L, les leucocytes à 5,8 G/L et la CRP à 40 mg/L. Un traitement par PCA (Patient Controlled Analgesi a) de Morphine est débuté rapidement. Le lendemain, la patiente se plaint de douleurs de l'hémiface droite. À l'examen, elle présente une exophtalmie, une ophtalmoplégie et un œdème palpébral droit. Elle n'a pas de baisse de l'acuité visuelle. L'hémoglobine est à 7,4 g/dl, les leucocytes à 7,6 G/L et la CRP à 100 mg/L. Un scanner cérébral met en évidence un œdème rétro-orbitaire de l'œil droit associé à une collection sous-périostée en regard du droit latéral de l'œil droit, nécessitant une intervention chirurgicale en urgence. Une antibiothérapie par Cefotaxime est mise en place en péri-opératoire. Un drainage de la collection hématique sous-périostée est réalisé et permet des prélèvements bactériologiques qui resteront négatifs. Une IRM orbitaire post chirurgicale ne montre pas d'atteinte du nerf optique, mais la persistance d'une exophtalmie de grade 2 et une infiltration des tissus graisseux intra coniques. La patiente est transférée en post-opératoire immédiat dans le service de médecine interne mais présente à 48 heures un syndrome thoracique aigu, avec déglobulisation, pour lequel elle est transfusée de deux culots globulaires et transférée en unité de soins continus. L'antibiothérapie est maintenue pendant 7 jours. L'évolution clinique est rapidement favorable sur le plan clinique et biologique. Sur la gravité de l'atteinte orbitaire et le risque de récidive, un programme d'érythraphérèse est initié. L'espace médullaire hématopoïétique étant minime dans les os de la face, notamment dans ceux de l'orbite, il est rare de rencontrer des infarctus osseux orbitaires chez le drépanocytaire. Ils sont plus fréquents chez les enfants du fait de la plus grande proportion de tissu hématopoïétique médullaire par rapport à l'adulte [1]. Une trentaine de cas sont décrits dans la littérature, avec un âge moyen de 14 ans [2]. Dans environ la moitié des cas, on note la présence d'un hématome sous-périosté [1,2]. Les premières manifestations sont une douleur et un œdème localisés. L'atteinte peut être bilatérale dans un tiers des cas environ [2]. La principale complication est le syndrome de compression optique, responsable d'une exophtalmie, d'une paralysie oculomotrice, d'une hypoesthésie cornéenne et à l'extrême d'une compression du nerf optique [1] , engageant alors le pronostic visuel. Le diagnostic différentiel peut être difficile avec une ethmoïdite, une cellulite de la face ou une ostéomyélite [1]. L'élévation des leucocytes et le syndrome inflammatoire ne sont pas discriminants [2]. À l'imagerie, l'absence de pathologie sinusienne et la localisation de la collection sur la paroi latérale de l'orbite sont des arguments en défaveur d'un abcès [2]. L'IRM permet de statuer sur la nature hématique ou non de la collection et de mettre en évidence l'infarctus osseux en regard [2]. En cas d'ostéomyélite, il apparaît, après injection de Gadolinium, un rehaussement en plage, tandis qu'il est fin et serpigineux en cas d'infarctus osseux [3]. Le traitement médical semble le plus souvent suffisant, comprenant le traitement de la crise et l'utilisation par certaines équipes de corticostéroïdes [1,2] , à utiliser avec précaution dans le contexte de drépanocytose. La chirurgie est réservée aux cas de compression persistante du nerf optique [2]. L'hématome sous-périosté secondaire à un infarctus osseux orbitaire est une complication rare mais grave de la drépanocytose, dont le diagnostic doit être rapide et le traitement adéquat, afin de préserver le pronostic visuel. La survenue de cette complication doit faire discuter la mise en place d'un traitement de fond de la drépanocytose. [ABSTRACT FROM AUTHOR]

Background: Selective internal radiotherapy based on transarterial radioembolization (TARE) with yttrium-90 ( 90 Y) microspheres is an established treatment for primary or metastatic liver disease., Purpose: The objective of this work is to optimize the dosimetry of patients treated with 90 Y TARE, using positron emission tomography (PET) images., Methods: The NEMA 2012 PET phantom was filled with nearly 3.9 GBq of 90 Y activity and acquired at days 0, 3, 5, 7, and 9 on a classic time-of-flight PET/computed tomography (CT) scanner (Philips TF64) and on a silicon photomultiplier (SiPM)-based PET/CT scanner (Philips Vereos). Acquisitions were carried on following the guidelines proposed in a previously published multicentric trial and images were reconstructed by varying and combining the available parameters. Comparisons were performed to identify the best set(s) of parameters leading to the most accurate 90 Y-PET image(s), in terms of activity distribution. Then, for both scanners, the best images were analyzed with Simplicit 90 Y, a personalized dosimetry software using multicompartmental Medical Internal Radiation Dose model. The comparison between measured and true doses allowed to identify the image granting the most consistent dose estimations and, therefore, to designate the set of parameters to be applied on patients' data for the reconstruction of optimized clinical images. Posttreatment dosimetry of four patients was then realized with Simplicit 90 Y using optimized imaging datasets., Results: Based on activity distribution comparisons and dose estimations over phantom and patients data, the SiPM-based PET/CT system appeared more suitable than the photomultiplier tube-based TF64 for 90 Y-PET imaging. With the SiPM-based PET/CT system, reconstructed images with a 2-mm voxel size combined with the application of the point spread function correction led to the most accurate results for quantitative 90 Y measures., Conclusions: For the SiPM-based PET/CT scanner, an optimized set of reconstruction parameters has been identified and applied on patients' data in order to generate the most accurate image to be used for an improved personalized 90 Y-PET dosimetry, ensuring a reliable evaluation of the delivered doses., (© 2022 American Association of Physicists in Medicine.)

Preoperative molecular imaging is paramount to direct surgery in primary hyperparathyroidism (pHPT). We investigated the diagnostic performance of 18 F-fluorocholine ( 18 F-FCH) PET/CT compared with 11 C-methionine ( 11 C-MET) PET/CT for localization of hyperfunctioning parathyroid tissue in patients with pHPT and negative or inconclusive 99m Tc-sestaMIBI ( 99m Tc-MIBI) SPECT findings. Methods: Fifty-eight patients with biochemical evidence of pHPT and negative or inconclusive 99m Tc-MIBI SPECT findings were referred for presurgical detection and localization of hyperfunctioning parathyroid tissue by 11 C-MET and 18 F-FCH PET/CT. The PET/CT results were classified into 3 categories (positive, inconclusive, or negative) based on the nodular aspect of tracer uptake and the visualization of corresponding nodules on CT. The PET/CT results were correlated with the surgical and histopathologic findings, which were used as the gold standard. Results: Fifty-three patients were included for analysis. 18 F-FCH PET/CT was positive in 39 patients (74%), inconclusive in 5 (9%), and negative in 9 (17%), compared with 25 (47%), 12 (23%), and 16 (30%), respectively, for 11 C-MET PET/CT. 18 F-FCH localized 11 additional foci (6 positive and 5 inconclusive), compared with 11 C-MET. Twenty-six patients (sex ratio, 10/16 M/F) underwent surgery, with resection of 31 lesions (22 adenomas, 6 hyperplastic glands, and 3 carcinomas) and 1 normal gland. At follow-up, 21 patients (81%) were considered cured after surgery, whereas 3 patients (12%) had persistence of hypercalcemia. With inconclusive cases being considered negative, 18 F-FCH PET/CT correctly localized 26 lesions in 24 of 26 patients (92%), compared with 16 lesions in 15 of 26 patients (58%) localized by 11 C-MET PET/CT. Per-patient-based sensitivity and positive predictive value were 96% and 96%, respectively, for 18 F-FCH and 60% and 94%, respectively, for 11 C-MET ( P < 0.0001). Per-lesion-based sensitivity and positive predictive value were 84% and 90%, respectively, for 18 F-FCH and 52% and 94%, respectively, for 11 C-MET ( P < 0.0001). Conclusion: In the presence of biochemical evidence of pHPT with negative or inconclusive 99m Tc-MIBI SPECT findings, 18 F-FCH PET/CT performs better than 11 C-MET PET/CT for the detection of pathologic parathyroid tissue, allowing localization of parathyroid adenoma or hyperplasia in 96% of patients., (© 2022 by the Society of Nuclear Medicine and Molecular Imaging.)

Background: Digital PET/CT systems make use of a new technology with higher sensitivity and other better technological features than the analog ones. They require adaptation of the trade-off between performance, tracer dose and acquisition time. The aim of the study was to explore the performance of 18 F-JK-PSMA-7 imaging when performed on a digital PET/CT with an adapted protocol, in a population of patients with prostate cancer patients (PCa). Influence of previous therapy on PET/CT performance is generally disregarded in PSMA-based imaging, despite potential influence of hormono-chemotherapy on the target expression. This potential influence was also tested in this work., Methods: A total of 54 PCa patients experiencing biochemical recurrence were included in the study, in which we analysed the diagnostic performance of digital 18 F-JK-PSMA-7 PET/CT. Compared to our protocol applied for acquisition on an analog system, administered dose and acquisition time were reduced by 20% and 50% respectively. We specifically took into consideration the influence of previous treatments on recurrence detection., Results: We detected overall 18 F-JK-PSMA-7-positive lesions in 38/54 patients (70.3%). There was no statistically significant difference regarding the detection rate between the groups of patients who had hormono-chemotherapy any time after initial diagnosis and those who never got any hormonal or chemotherapeutic treatment. Regarding the SUV max values, there was not significant difference between the two groups of patients neither in pelvic ganglions nor in other metastatic sites or the prostate region., Conclusion: 18F -JK-PSMA7 PET/CT with administered dose and acquisition time adapted to the digital technology provides valuable information in PCa patients with biochemical recurrence., (© 2022. The Author(s).)

Objective: To identify the velocity of disease progression as a predictor of severity in patients with Guillain-Barre syndrome (GBS)., Materials and Methods: Prospective observational study of patients with confirmed diagnosis of GBS between May and August 2019 in four hospitals in Peru. The disease progression velocity (DPV) was defined as the time since the onset of neurological symptoms and the maximum peak of neurological severity., Results: Of 94 cases with GBS, the average age was 42 years; 73 (77.8%) patients presented severe GBS, the average hospital stay was 19 days; 45 (47.8%) patients haddiarrheal symptoms previously, in 63 (67.1%) patients the onset of motor weakness was located in the upper limbs and in 31 (32.9%) it was located in the lower limbs, 9 (10.0%) patients presented some type of dysautonomy; admission to mechanical ventilation was needed in 8 (8.5%) patients, and the deceased were 2 (2.0%). The DPV≤ 1 day has a 79% probability of developing severe disease, the two and three day DPV have the probability of 61% and 38% respectively of progressing to severe forms., Conclusion: DPV is a predictor of poor prognosis when it is less than 2 days and with a possible requirement for mechanical ventilation. The speed of progression of neurological disease is a practical and accessible clinical evaluation method that should be evaluated in patients with GBS.

Background and Objectives: Selection for surgery in patients with colorectal liver metastases (CRLM) remains inaccurate. We evaluated if CRLM baseline metabolic characteristics, assessed by [18]F-fluorodeoxyglucose-positron emission tomography/computed tomography ( 18 FDG-PET/CT), could predict postoperative outcomes., Methods: In a retrospective series of patients undergoing surgery for CRLM, we defined two groups: the long-term survival (LTS) and early relapse (ER) groups, where the postoperative recurrence-free survivals were ≥5 years or <1 year, respectively. We analyzed the patients in whom baseline 18 FDG-PET/CT was available. Clinicopathologic parameters, clinical risk score (CRS), and baseline 18 FDG-PET/CT characteristics were compared between LTS and ER groups. A metabolic CRS (mCRS) was implemented, adding one point to the standard five-point CRS when the highest tumor standardized uptake values (SUV max )/normal liver mean SUV (SUV mean(liver) ) ratios were >4.3, defining low- and high-risk mCRS by scores of 0 to 2 and 3 to 6, respectively., Results: From a series of 450 patients operated for CRLM (mean follow-up of 58 months), we included for analysis 23 and 30 patients in the LTS and ER groups, respectively. Clinicopathologic parameters and CRS were similar in the LTS and ER groups. Median SUV max /SUV mean(liver) ratios were higher in ER vs LTS patients (4.2 and 2.8, P = .008, respectively). mCRS was increased in ER patients (P = .024); 61% of LTS patients had low-risk mCRS and 73% of the ER patients had high-risk mCRS (P = .023)., Conclusions: 18 FDG-PET/CT characteristics combined with traditional CRS may represent a new tool to improve selection for surgery in patients with CRLM., (© 2019 Wiley Periodicals, Inc.)

Background: Idiopathic pulmonary fibrosis (IPF) is characterized by a progressive and irreversible respiratory failure. Non-invasive markers of disease activity are essential for prognosis and evaluation of early response to anti-fibrotic treatments., Objectives: The aims of this study were to determine whether fluorodeoxyglucose ([18F]-FDG) lung uptake is reduced after initiation of pirfenidone or nintedanib and to assess its possible use as a prognostic factor., Methods: [18F]-FDG PET/CT was performed in IPF patients and in a murine model of pulmonary fibrosis. PET/CTs were performed at day 8 and day 15 post-instillation of bleomycin in pirfenidone- or vehicule-treated mice. In IPF patients, PET-CT was performed before and 3 months after the initiation of pirfenidone or nintedanib., Results: In bleomycin-treated mice, pirfenidone significantly reduced the [18F]-FDG uptake compared to vehicule-treated mice at day 15 (p < 0.001), whereas no difference was observed at day 8 after bleomycin administration. In IPF patients, [18F]-FDG lung uptake before and after 3 months of treatment by nintedanib (n = 11) or pirfenidone (n = 14) showed no significant difference regardless the antifibrotic treatment. Moreover, no difference was noticed between patients with progressive or non-progressive disease at one year of follow up., Conclusions: Pirfenidone significantly reduces the lung [18F]-FDG uptake during the fibrotic phase in a mouse model of IPF. However, these preclinical data were not confirmed in IPF patients 3 months after the initiation of antifibrotic therapy. [18F]-FDG seems therefore not useful in clinical practice to assess the early response of IPF patients to nintedanib or pirfenidone.

Autosomal dominant deficiency of signal transducer and activator of transcription 3 (STAT3) is the main genetic etiology of hyper-immunoglobulin (Ig) E syndrome. We documented the molecular, cellular, and clinical features of 60 patients with heterozygous STAT3 mutations from 47 kindreds followed in France. We identified 11 known and 13 new mutations of STAT3. Low levels of interleukin (IL)-6-dependent phosphorylation and nuclear translocation (or accumulation) of STAT3 were observed in Epstein-Barr virus-transformed B lymphocytes (EBV-B cells) from all STAT3-deficient patients tested. The immunologic phenotype was characterized by high serum IgE levels (96% of the patients), memory B-cell lymphopenia (94.5%), and hypereosinophilia (80%). A low proportion of IL-17A-producing circulating T cells was found in 14 of the 15 patients tested. Mucocutaneous infections were the most frequent, typically caused by Staphylococcus aureus (all patients) and Candida albicans (85%). Up to 90% of the patients had pneumonia, mostly caused by Staph. aureus (31%) or Streptococcus pneumoniae (30%). Recurrent pneumonia was associated with secondary bronchiectasis and pneumatocele (67%), as well as secondary aspergillosis (22%). Up to 92% of the patients had dermatitis and connective tissue abnormalities, with facial dysmorphism (95%), retention of decidual teeth (65%), osteopenia (50%), and hyperextensibility (50%). Four patients developed non-Hodgkin lymphoma. The clinical outcome was favorable, with 56 patients, including 43 adults, still alive at the end of study (mean age, 21 yr; range, 1 mo to 46 yr). Only 4 patients died, 3 from severe bacterial infection (aged 1, 15, and 29 yr, respectively). Antibiotic prophylaxis (90% of patients), antifungal prophylaxis (50%), and IgG infusions (53%) improved patient health, as demonstrated by the large decrease in pneumonia recurrence. Overall, the prognosis of STAT3 deficiency may be considered good, provided that multiple prophylactic measures, including IgG infusions, are implemented.

Computed tomography (CT) is used increasingly to measure liver volume in patients undergoing evaluation for transplantation or resection. This study is designed to determine a formula predicting total liver volume (TLV) based on body surface area (BSA) or body weight in Western adults. TLV was measured in 292 patients from four Western centers. Liver volumes were calculated from helical computed tomographic scans obtained for conditions unrelated to the hepatobiliary system. BSA was calculated based on height and weight. Each center used a different established method of three-dimensional volume reconstruction. Using regression analysis, measurements were compared, and formulas correlating BSA or body weight to TLV were established. A linear regression formula to estimate TLV based on BSA was obtained: TLV = -794.41 + 1,267.28 x BSA (square meters; r(2) = 0.46; P <.0001). A formula based on patient weight also was derived: TLV = 191.80 + 18.51 x weight (kilograms; r(2) = 0.49; P <.0001). The newly derived TLV formula based on BSA was compared with previously reported formulas. The application of a formula obtained from healthy Japanese individuals underestimated TLV. Two formulas derived from autopsy data for Western populations were similar to the newly derived BSA formula, with a slight overestimation of TLV. In conclusion, hepatic three-dimensional volume reconstruction based on helical CT predicts TLV based on BSA or body weight. The new formulas derived from this correlation should contribute to the estimation of TLV before liver transplantation or major hepatic resection.

Capsule opacification occurs when lens epithelial cells remains on the capsule after extracapsular cataract surgery. The cells divide and form fibers. The best way to prevent opacification is to remove all epithelial cells from the capsule. Our study compared the efficacy of different mechanical polishing methods in eyes in which cataracts were removed by endocapsular phacoemulsification through a miniature capsulorhexis. The capsular bag was polished using one of five techniques: metallic scrapers, silicone scrapers, Rentsch capsule curettes, irrigation/aspiration tip, or ultrasound irrigation/aspiration tip. Then a circular central portion of the anterior capsule was removed and processed for histology and scanning electron microscopy. A control group comprised unpolished central anterior capsules obtained by capsulorhexis before or after endophacoemulsification. The cleanest capsules were obtained by polishing with the ultrasound irrigation/aspiration tip.