Clinical Characteristics and Classification Criteria Performance in a Single-Center Cohort of 114 Patients With Immunoglobulin G4-Related Disease.

INTRODUCTION/OBJECTIVES: Immunoglobulin G4-related disease (IgG4-RD) is a heterogeneous fibroinflammatory condition. The 2019 American College of Rheumatology (ACR)/European League Against Rheumatism (EULAR) Classification Criteria for IgG4-RD were published to provide unified classification criteria in clinical research. The purpose of this study was to characterize demographics, disease manifestations, and treatments of patients with IgG4-RD and assess performance of the Classification Criteria in a heterogeneous cohort with a large population of Black patients. METHODS: This was a medical records review of all patients referred to a specialized IgG4-RD clinic. Demographics, serology, histopathology, disease manifestations, and treatment information were collected and analyzed. An ACR/EULAR Classification Criteria score for IgG4-RD was calculated to compare performance in definite diagnosis, probable diagnosis, and mimicker groups. RESULTS: A total of 198 patients were evaluated. Eighty-five (43%) were mimickers. Of the remaining 114, 58 (51%) were classified as definite, and 56 (49%) as probable cases by treating clinicians. Pancreatitis was the most common presentation (37%) among 28 different organ manifestations of IgG4-RD. In patients with definite clinical diagnosis of IgG4-RD, 84% met the IgG4-RD Classification Criteria (i.e., score ≥20) with mean score of 29. Only 9% of the probable cases met this threshold with an average score of 8. None of the mimickers met the Classification Criteria. CONCLUSIONS: This study highlights the broad spectrum of IgG4-RD and validates the use of the ACR/EULAR Classification Criteria for IgG4-RD, including a large proportion of Black patients. As shown in this study, IgG4-RD is a heterogeneous disease and continues to represent a diagnostic challenge to clinicians.

Gastric per-oral endoscopic myotomy for refractory gastroparesis: a detailed description of the procedure, our experience, and review of the literature.

BACKGROUND AND AIMS: Gastric per-oral endoscopic myotomy (G-POEM) was introduced four years ago as an investigational procedure for refractory gastroparesis. The safety and efficacy were currently evaluated. With our recent studies on G-POEM, we share our experience and knowledge through the discussion of a detailed description of the procedure and review of the literature. To our knowledge, this is the first systemic review on this new therapeutic endoscopic procedure. METHODS: The indications and contraindications, various aspects of the procedure, and efficacy assessment are discussed based on our experience and current available data. RESULTS: Preoperative preparation, detailed description of the procedure, post-procedural care, and results in the literature are presented. The procedure is safe and effective. 70-80% of patients have significant improvement in overall symptoms and quality of life in short-term (6 months) follow-up, as assessed by Gastric Cardinal Symptom Index and Short Form 36. CONCLUSIONS: G-POEM is a feasible and effective procedure for refractory gastroparesis based on early and limited data. Well-designed prospective studies are expected to advance and evaluate this new procedure in the future.

Semiautomated CT-Based Quantification of Donor Kidney Volume Applied to a Predictive Model of Outcomes in Renal Transplantation.

OBJECTIVE: The purpose of this study was to assess the use of semiautomated CT-based quantification of renal graft volume as a preoperative predictor of graft function. MATERIALS AND METHODS: All transplants over a 3-year period in which donors underwent CT and for which recipient outcomes were available were included. Two blinded readers used a commercially available reconstruction tool to independently measure donated kidney cortical volume and total parenchymal kidney volume. Transplant characteristics obtained by chart review included subject demographics, recipient pretransplant weight, immunologic matching, and recipient creatinine values at multiple time points. Intraclass correlation of measurements by the two readers was calculated. The ratios between donated kidney cortical volume and recipient pretransplant weight were correlated with graft function over 24 months and used in logistic regression models to calculate the odds of development of diminished renal function. RESULTS: After application of the inclusion and exclusion criteria, 153 transplants were included in the study. Donated kidney cortical and total parenchymal volume measurements had high correlation (R > 0.9) and high reproducibility (intraclass correlation coefficient, 0.93-0.94). Unadjusted correlations existed between estimated glomerular filtration rate (eGFR) and the ratio between donated kidney cortical volume and recipient pretransplant weight 12 months (R = 0.8489) and 24 months (R = 0.6839) after transplant. After adjustment for transplant parameters, recipients in the highest tertile for ratio between donated kidney cortical volume and recipient pretransplant weight (2.7 mL/kg) had higher mean eGFR values at all time points in the 24 months than did recipients in the lower tertiles (1.2 and 1.6 mL/kg). Recipients in the highest tertile had a significantly lower risk of development of diminished renal function 12 and 24 months after transplant (adjusted odds ratios, 0.25 at 12 months [95% CI, 0.09-0.66]; 0.27 at 24 months [95% CI, 0.10-0.71]). CONCLUSION: The CT-derived ratio between donated kidney cortical volume and recipient pretransplant weight is a noninvasively and readily obtained reproducible biomarker that is predictive of 12- and 24-month renal transplant outcomes.

Proximal and Distal Gastric Retention Patterns in Gastroparesis and the Impact of Gastric Per-Oral Endoscopic Myotomy: A Retrospective Analysis Using Gastric Emptying Scintigraphy.

Gastroparesis is a debilitating disease of insufficient gastric emptying and visceral hypersensitivity characterized by nausea, vomiting, early satiety, and bloating. Gastric emptying scintigraphy (GES), in combination with typical symptoms and normal esophagogastroduodenoscopy findings, is used to diagnose the disease. Gastric per-oral endoscopic pyloromyotomy (G-POEM) has emerged as a novel technique for treating gastroparesis, with up to an 80% success rate. This procedure involves myotomy of the distal stomach. We hypothesize that responders to this therapy are characterized by more distal dysmotility than nonresponders, as defined by GES retention patterns. Methods: We used regional gastric emptying measurements from diagnostic GES to determine the proximal or distal predominance of disease for each patient. We then compared treatment response and symptoms in each patient to total gastric half-emptying time (T½), proximal gastric T½, and a ratio comparing the 2 values. Results: In total, 47 patients underwent G-POEM during the study period. A significant difference (P < 0.01) was found in proximal-to-total T½ ratio between responders and nonresponders. A significant difference between pre- and postprocedural proximal-to-total T½ ratios was identified for each patient. No correlations were identified between motility patterns and symptoms or in motility patterns among the different etiologies of the disease. Conclusion: Proximal-to-total T½ ratio may represent an important patient selection factor for G-POEM versus other treatment modalities going forward. Local retention patterns in GES may not inform the symptom profile in gastroparesis.

Gastric Emptying Scintigraphy Before Gastric per Oral Endoscopic Myotomy: Imaging May Inform Treatment.

Gastric emptying scintigraphy (GES) helps to diagnose gastroparesis and is typically only used for whole stomach retention patterns. However, it may provide significantly more information when looking specifically at proximal and distal retention patterns. This article reviews global GES changes following gastric per oral endoscopic myotomy; how global, proximal, and distal GES measurements correlate to gastroparesis symptoms; and how proximal and distal GES may serve as proxies for the various mechanisms involved in gastroparesis. The authors' data on how GES may be used to select which patients will have success from G-POEM is also reviewed.

Endoscopic needle-knife treatment for symptomatic esophageal Zenker's diverticulum: A meta-analysis and systematic review.

OBJECTIVE: The aim of this study was to assess the efficacy and safety following endoscopic management of Zenker's diverticulum (ZD) using a needle-knife technique. METHODS: A systematic search of PubMed, Embase and Cochrane library databases was performed. All original studies reporting efficacy and safety of needle-knife technique for treatment of ZD were included. Pooled event rates across studies were expressed with summative statistics. Main outcomes, such as rates of immediate symptomatic response (ISR), adverse events and recurrence, were extracted, pooled and analyzed. Heterogeneity among studies was assessed using the R statistic. The random effects model was used and results were expressed with forest plots and summative statistics. RESULTS: Thirteen studies included 589 patients were enrolled. Pooled event rates for ISR, overall complication, bleeding and perforation were 88% (95% confidence interval [CI] 79-94%), 13% (95% CI 8-22%), 5% (95% CI 3-10%) and 7% (95% CI 4-12%), respectively. The pooled data demonstrated an overall recurrence rate of 14% (95% CI 9-21%). Diverticulum size of at least 4 cm and less than 4 cm demonstrated pooled adverse event rates of 17% (95% CI 10-27%) and 7% (95% CI 2-18%), respectively. When using diverticuloscope as an accessory, pooled ISR and adverse events rates were 84% (95% CI 58-95%) and 10% (95% CI 3-26%), respectively. CONCLUSION: Flexible endoscopic procedures using needle-knife offers a relatively safe and effective treatment of symptomatic ZD, especially for ZD of <4 cm in diameter.

Yield of computed tomography of the cervical spine in cases of simple assault.

BACKGROUND: Computed tomography (CT) of the cervical spine (C-spine) is routinely ordered for low-impact, non-penetrating or "simple" assault at our institution and others. Common clinical decision tools for C-spine imaging in the setting of trauma include the National Emergency X-Radiography Utilization Study (NEXUS) and the Canadian Cervical Spine Rule for Radiography (CCR). While NEXUS and CCR have served to decrease the amount of unnecessary imaging of the C-spine, overutilization of CT is still of concern. METHODS: A retrospective, cross-sectional study was performed of the electronic medical record (EMR) database at an urban, Level I Trauma Center over a 6-month period for patients receiving a C-spine CT. The primary outcome of interest was prevalence of cervical spine fracture. Secondary outcomes of interest included appropriateness of C-spine imaging after retrospective application of NEXUS and CCR. The hypothesis was that fracture rates within this patient population would be extremely low. RESULTS: No C-spine fractures were identified in the 460 patients who met inclusion criteria. Approximately 29% of patients did not warrant imaging by CCR, and 25% by NEXUS. Of note, approximately 44% of patients were indeterminate for whether imaging was warranted by CCR, with the most common reason being lack of assessment for active neck rotation. CONCLUSIONS: Cervical spine CT is overutilized in the setting of simple assault, despite established clinical decision rules. With no fractures identified regardless of other factors, the likelihood that a CT of the cervical spine will identify clinically significant findings in the setting of "simple" assault is extremely low, approaching zero. At minimum, adherence to CCR and NEXUS within this patient population would serve to reduce both imaging costs and population radiation dose exposure.

Utility of computed tomographic imaging of the cervical spine in trauma evaluation of ground-level fall.

BACKGROUND: Computed tomography (CT) of the cervical spine (C-spine) is routinely ordered for low-risk mechanisms of injury, including ground-level fall. Two commonly used clinical decision rules (CDRs) to guide C-spine imaging in trauma are the National Emergency X-Radiography Utilization Study (NEXUS) and the Canadian Cervical Spine Rule for Radiography (CCR). METHODS: Retrospective cross-sectional study of 3,753 consecutive adult patients presenting to an urban Level I emergency department who received C-spine CT scans were obtained over a 6-month period. The primary outcome of interest was prevalence of C-spine fracture. Secondary outcomes included fracture stability, appropriateness of imaging by NEXUS and CCR criteria, and estimated radiation dose exposure and costs associated with C-spine imaging studies. RESULTS: Of the 760 patients meeting inclusion criteria, 7 C-spine fractures were identified (0.92% ± 0.68%). All fractures were identified by NEXUS and CCR criteria with 100% sensitivity. Of all these imaging studies performed, only 69% met NEXUS indications for imaging (50% met CCR indications). C-spine CT scans in patients not meeting CDR indications were associated with costs of $15,500 to $22,000 by NEXUS ($14,600-$25,600 by CCR) in this single center during the 6-month study period. CONCLUSION: For ground-level fall, C-spine CT is overused. The consistent application of CDR criteria would reduce annual nationwide imaging costs in the United States by $6.8 to $9.6 million based on NEXUS ($6.4-$15.6 million based on CCR) and would reduce population radiation dose exposure by 0.8 to 1.1 million mGy based on NEXUS (0.7-1.9 million mGy based on CCR) if applied across all Level I trauma centers. Greater use of evidence-based CDRs plays an important role in facilitating emergency department patient management and reducing systemwide radiation dose exposure and imaging expenditures. LEVEL OF EVIDENCE: Diagnostic study, level III.