Real-Life Utilization of Criteria Guidelines for Diagnosis of Cardiac Sarcoidosis (CS).

Despite the increasing recognition of cardiac involvement in systemic sarcoidosis, the diagnosis of cardiac sarcoidosis (CS) remains challenging. Our aim is to present a comprehensive, retrospective case series of CS patients, focusing on the current diagnostic guidelines and management of this life-threatening condition. In our case series, patient data were collected retrospectively, including hospital admission records and rheumatology and cardiology clinic visit notes, detailing demographic, clinical, laboratory, pathology, and imaging studies, as well as cardiac devices and prescribed medications. Cases were divided into definite and probable CS based on the 2014 Heart Rhythm Society guidelines as well as presumed CS based on imaging criteria and clinical findings. Overall, 19 CS patients were included, 17 of whom were diagnosed with probable or presumed CS based on cardiac magnetic resonance imaging (CMR) and/or cardiac positron emission tomography using 18F-Fluorodeoxyglucose (PET-FDG) without supporting endomyocardial biopsy (EMB). The majority of CS patients were male (53%), with a mean age of 52.9 ± 11.8, with CS being the initial manifestation of sarcoidosis in 63% of cases. Most patients presented with high-grade AVB (63%), followed by heart failure (42%) and ventricular tachyarrhythmia (VT) (26%). This case series highlights the significance of utilizing updated diagnostic criteria relying on CMR and PET-FDG given that cardiac involvement can be the initial manifestation of systemic sarcoidosis, requiring prompt diagnosis and treatment to prevent morbidity and mortality.

Long-term outcomes during 37 years of pediatric kidney transplantation: a cohort study comparing ethnic groups.

BACKGROUND: This study aimed to evaluate short- and long-term outcomes of kidney transplantation over 37 years in a national referral center and compare outcomes between Israeli Jewish and Arab children. METHODS: Data on 599 pediatric transplantations performed in 545 children during 1981-2017, including demographic parameters, kidney failure disease profile, and pre-transplant dialysis duration, were retrieved from our computerized database and patient files. Patient and graft survival were estimated using the Kaplan-Meier method. RESULTS: Twenty-year patient survival was 91.4% for live donor (LD) and 80.2% for deceased donor (DD) kidney recipients. Respective 10-year and 20-year graft survival rates for first kidney-only transplants were 75.2% and 47.0% for LD and 60.7% and 38.4% for DD grafts. Long-term graft survival improved significantly (p < 0.001) over the study period for recipients of both LD and DD allografts and reached 7-year graft survival of 92.0% and 71.3%, respectively. The proportion of DD transplantations was higher in the Arab subpopulation: 73.8% vs. 48.4% (p < 0.001). Graft survival was not associated with age at transplantation and did not differ between the Arab (N = 202) and Jewish children (N = 343). Median (IQR) waiting time on dialysis did not differ significantly between the Arab and Jewish children: 18 (10-30) and 15 (9-30) months, respectively (p Mann-Whitney = 0.312). CONCLUSIONS: Good and progressively improving long-term results were obtained in pediatric kidney transplantation at our national referral center, apparently due to expertise gained over time and advances in immunosuppression. Equal access to DD kidney transplant and similar graft survival were found between ethnic groups.

Focal segmental glomerulosclerosis in pediatric kidney transplantation: 30 years' experience.

From 1982 to 2011, 53 kidney transplantations (KT) for pediatric focal segmental glomerulosclerosis (FSGS) were recorded in the National Israeli Kidney Transplant Registry (NIKTR): 22-primary (1◦) FSGS, 25-proved/suspected genetic-secondary (2◦) FSGS, six lost/incomplete files/other. Half (56%) of 23 patients with 2◦ FSGS were Israeli-Arabs vs 29% of 1◦ FSGS KT recipients. 1◦ FSGS recurrence occurred in 64% (14/22) of 22 KT in 17 patients aged (median) 14 years vs 1/25 of 2◦ FSGS (P<.001). Early graft days/nonfunction occurred in 9/14 (64%), 2/8 (25%) and 2/25 (4%) of recurrent 1◦ FSGS (rFSGS), nonr1◦ FSGS and 2◦ FSGS, respectively. Twelve biopsies performed in nine of these grafts at (median) 8 days (range 5-60 days) post-KT showed: ATN-5, suspected rejection-4, rFSGS-2, normal kidney-1; rFSGS was diagnosed eventually in 8/9. Dialysis need during the first month post-KT was significantly associated with FSGS recurrence: 6/14 (43%) for rFSGS vs 2/8 (25%) for non-rFSGS. Plasmapheresis (PP) achieved complete and partial rFSGS remission in 5/9 and 2/9 grafts, respectively. Three grafts were excised during the first 60 days post-KT for: nonfunction (1) and bleeding (2). Remaining grafts' GFR was: 78, 42, and 91 mL/min (median) at 5.3, 4.75, and 8 years follow-up for non-rFSGS, rFSGS, and 2◦ FSGS grafts, respectively. CONCLUSIONS: Early PP implementation should be considered after KT for 1◦ FSGS patients with early graft dysfunction despite delayed proteinuria and nonspecific biopsy.

Is 18F-FDG PET/CT useful for imaging and management of patients with suspected occult recurrence of cancer?

UNLABELLED: Rising serum tumor markers may be associated with negative imaging in the presence of cancer. CT and (18)F-FDG PET may yield incongruent results in the assessment of tumor recurrence. The present study evaluates the incremental role of (18)F-FDG PET/CT for the diagnosis and management of cancer patients with increasing levels of tumor markers as the sole indicator of potential recurrence after initial successful treatment. METHODS: Thirty-six cancer patients with increasing levels of tumor markers during follow-up and negative CT underwent (18)F-FDG PET/CT, which showed 111 sites of increased tracer uptake. PET/CT was compared with PET results on a site-based analysis for characterization of (18)F-FDG foci and on a patient-based analysis for diagnosis of recurrence. The clinical impact of PET/CT on further patient management was evaluated. RESULTS: Thirty patients (83%) had recurrence in 85 malignant sites (77%). For the site-based analysis, PET had a sensitivity, specificity, accuracy, positive predictive value, and negative predictive value of 96%, 50%, 85%, 85%, and 82%, respectively, as compared with the performance indices of PET/CT of 100%, 89%, 97%, 97%, and 100%, respectively. There was a statistically significant difference between the specificity (P < 0.05) and accuracy (P < 0.001) of PET and PET/CT for precise characterization of suspected lesions. For the patient-based analysis, PET had a sensitivity, specificity, and accuracy of 93%, 50%, and 86%, respectively, as compared with PET/CT with values of 93%, 67%, and 89%, respectively (P = not significant). PET/CT was the single modality that directed further management and treatment planning in 12 patients (33%). CONCLUSION: The results of this study indicate that PET/CT may improve the accuracy of occult cancer detection and further lead to management changes in patients with increasing levels of tumor markers as the sole suspicion of recurrent malignancy.

Combined functional and structural evaluation of cancer patients with a hybrid camera-based PET/CT system using (18)F-FDG.

UNLABELLED: Correct diagnosis and definition of the functional and anatomic status of lesions in cancer patients are of clinical importance. The value of hybrid imaging using a gamma camera-based PET/CT and (18)F-FDG in determining the relationship between mass and cancer was assessed. METHODS: Hybrid imaging was performed using a device combining low-dose CT and gamma camera-based PET. Ninety-one patients with histologically proven malignancy and 190 suspected sites of disease were evaluated. Camera-based PET was performed after the injection of 296-370 MBq (18)F-FDG. The presence of organomegaly or an abnormal mass on CT and of abnormal uptake of (18)F-FDG was assessed for each suspected lesion. The presence of malignancy at each site was determined by biopsy, imaging follow-up, or clinical outcome. RESULTS: Five imaging patterns were found. Pattern 1 showed congruent abnormal (18)F-FDG uptake and a mass on CT in 110 of the lesions. One hundred two sites (93%) had active cancer. Pattern 2 showed a mass on CT, larger than the area of abnormal (18)F-FDG uptake, and was found in 5 lesions. Active malignancy was proven in 3 sites (60%). Pattern 3 showed an abnormal mass on CT with no (18)F-FDG uptake and was found in 52 lesions. Thirteen of these lesions (25%) had active tumor. Pattern 4 showing abnormal (18)F-FDG uptake with no mass on CT was found in 23 lesions. Sixteen of these sites (70%) were malignant. Pattern 5 showed normal CT findings and no abnormal (18)F-FDG uptake in 11 patients. Two of these patients (18%) had active disease. Hybrid imaging was of value in establishing the correct relationship between CT and (18)F-FDG findings in 98 of the 190 lesions (52%). CONCLUSION: A range of patterns presenting with or without abnormal (18)F-FDG uptake on camera-based PET and a mass on CT may occur in suspected cancer sites. Both structural changes on CT and increased cell metabolism expressed by abnormal (18)F-FDG uptake should be considered in oncologic imaging. Hybrid imaging, a combined physiologic and anatomic modality, appears to provide new diagnostic opportunities in characterizing function and morphology in malignancies.

Clinical pretreatment risk factors and Ga-67 scintigraphy early during treatment for prediction of outcome of patients with aggressive non-Hodgkin lymphoma.

BACKGROUND: Clinical pretreatment risk factors indicate the severity of disease in patients with aggressive non-Hodgkin lymphoma (NHL). Ga-67 scintigraphy during treatment is an early indicator of treatment-related features of lymphoma cells. The ability of risk factors and Ga-67 to predict disease outcome was compared in 139 patients with aggressive NHL. METHODS: Pretreatment clinical risk factors and Ga-67 scintigraphy performed after one cycle and at mid-treatment were evaluated for their correlation with response rate and as predictors of 5-year failure-free survival (FFS). Univariate analysis was performed to determine the ability of pretreatment risk factors and Ga-67 early during treatment to predict FFS. Subsequently, multivariate analysis was performed on the variables with significant univariate results using the Cox proportional hazards method. The predictive value of risk factors and Ga-67 scintigraphy was calculated to determine their suitability in selecting patients with poor outcome. RESULTS: Response rate correlated with stage of disease (P < 0.01) and the international prognostic index (IPI) score (P < 0.05). Five-year FFS was predicted by stage of disease (P < 0.004), performance status (P < 0.02), and the IPI score (P < 0.01). Response rate correlated with results of Ga-67 scintigraphy after one cycle of chemotherapy (P < 0.001) and at mid-treatment (P < 0.001). Five-year FFS was predicted by Ga-67 after one cycle of chemotherapy (P < 0.0004) and at mid-treatment (P < 0.0001). Positive Ga-67 after the first cycle of treatment predicted 64% of patients who had failure of treatment. A positive study at mid-treatment predicted 77% of patients who had treatment failure. Cox analysis showed Ga-67 after one course (P < 0.0012) and at mid-treatment (P < 0.0002) as being the most significant variables in predicting FFS. CONCLUSIONS: Ga-67 scintigraphy demonstrates early the effect of treatment in patients with aggressive NHL. It is a better predictor than pretreatment risk factors of both response rate and FFS. Positive Ga-67 early during treatment may be used as an independent test in selecting patients who will not respond favorably to current protocol treatment for early therapeutic modifications.