Pediatric to Adult Transition in Sickle Cell Disease: Survey Results from Young Adult Patients.

BACKGROUND/AIMS: We surveyed sickle cell disease (SCD) patients who transitioned from pediatric care at Texas Children's Hematology Center (TCHC) to adult care to determine the characteristics of patients with an adult SCD provider, continuation rates of pre-transition therapies, and patient perceptions of the transition process. METHODS: A cross-sectional study was conducted by telephone survey of 44 young adults with SCD, aged 19-29 years, who transitioned from TCHC to adult care within the last 15 years. RESULTS: Findings of the 23-item questionnaire revealed that transitioned patients with current adult providers (68.2%) were more likely to have seen a provider within 6 months of transition (p = 0.023) and to have been on hydroxyurea and/or monthly blood transfusions pre-transition (p = 0.021) than transitioned patients without a provider; 83% of patients on pre-transition hydroxyurea reported continuing hydroxyurea after transition. Transition challenges included inadequate preparation, difficulty finding knowledgeable adult providers, and lack of healthcare insurance/coverage. CONCLUSION: Transition to adult providers is predicted by establishing care with an adult SCD provider within 6 months of transition and being on pre-transition disease-modifying therapy. Transition may be improved if pediatric hematology centers assist and verify adult provider contact within 6 months of transition and engage patients of all disease severity during transition.

The American Association for the Surgery of Trauma Organ Injury Scale 2018 update for computed tomography-based grading of renal trauma: a primer for the emergency radiologist.

The most widely used trauma injury grading system is the Organ Injury Scale (OIS) by the American Association for the Surgery of Trauma (AAST). The AAST OIS for renal trauma was revised in 2018 to reflect necessary updates based on decades of experience with computed tomography (CT)-based injury diagnosis and, specifically, to better incorporate vascular injuries, which were not comprehensively addressed in the original OIS. In this review article, we describe CT findings of the AAST OIS for the kidney according to the 2018 revision, with an emphasis on real-world application, and highlight important differences from the prior grading scheme. Routine use of this grading system allows for a standardized classification of the range of renal injuries to aid in management, adding value in the imaging care of trauma patients.

Tumefactive brain parenchymal neurosarcoidosis.

BACKGROUND: Enhancing brain parenchymal disease, and especially tumefactive lesions, are an uncommon manifestation of neurosarcoidosis. Little is known about the clinical features of tumefactive lesions and their impact on management and outcomes, which this study aims to characterize. METHODS: Patients with pathologically-confirmed sarcoidosis were retrospectively reviewed and included if brain lesions were: (1) intraparenchymal, (2) larger than 1 cm in diameter, and (3) associated with edema and/or mass effect. RESULTS: Nine patients (9/214, 4.2%) were included. Median onset age was 37 years. Diagnosis was confirmed by brain parenchymal biopsies in 5 (55.6%). Median modified Rankin scale (mRS) score was 2 (range 1-4) at initial presentation. Common manifestations included headache (77.8%), cognitive dysfunction (66.7%), and seizures (44.4%). Sixteen lesions were present in 9 patients. The frontal lobe (31.3%) was most affected, followed by the subinsular region (12.5%), basal ganglia (12.5%%), cerebellum (12.5%), and pons (12.5%). MRI characteristics of the dominant lesions included spherical morphology (77.8%), perilesional edema (100.0%), mass effect (55.6%), well-demarcated borders (66.7%), and contrast enhancement (100.0%; 55.6% heterogeneous). Leptomeningitis was frequently present (77.8%). All required corticosteroid-sparing treatments, and most (55.6%) needed at least a third line of treatment (infliximab used in 44.4%). All patients relapsed (median 3 relapses, range 1-9). Median last mRS was 1.0 after median follow-up of 86 months, with significant residual deficits in 55.6%. CONCLUSION: Tumefactive brain parenchymal lesions are uncommon, usually affect the supratentorial brain along with leptomeningitis, and are refractory to initial treatments with a high risk of relapse. Significant sequelae were encountered despite a favorable median last mRS.

Pachymeningitis in Biopsy-Proven Sarcoidosis: Clinical Course, Radiographic Findings, Response to Treatment, and Long-term Outcomes.

BACKGROUND AND OBJECTIVES: Meningeal inflammation is one of the most common manifestations of neurosarcoidosis, occurring in 16%-69% of affected patients. While the clinical and radiographic features of leptomeningitis in neurosarcoidosis are well known, those of pachymeningitis are far less clear. Our primary aim was to study the clinicoradiographic features of pachymeningeal involvement in neurosarcoidosis and its evolution over time in response to treatment. METHODS: Patients with a diagnosis of neurosarcoidosis seen at Emory University (January 2011-August 2021) were included if pachymeningeal involvement was evident by MRI and the patient's sarcoidosis was pathologically confirmed (from a CNS or non-CNS site). RESULTS: Twenty-six of 215 (12.1%) patients with neurosarcoidosis qualified for inclusion. Pathologic confirmation came from CNS tissue in 50%. The median age of onset was 43.5 years; most were male (16/26, 61.5%). Symptoms were primarily related to pachymeningitis in 20/26 (76.9%). Headache (19/26, 73.1%), visual dysfunction (12/26, 46.2%), and seizures (7/26, 26.9%) were the most common symptoms. All patients had cranial pachymeningitis; only a single patient undergoing spinal imaging (1/11, 9.1%) had spinal pachymeningitis. The falx cerebri (16/26, 61.5%) was the most commonly affected dural structure, but the anterior and middle cranial fossae and tentorium cerebelli were frequently involved (12/26 each, 46.2%). The pachymeningeal lesions were unifocal (11/26, 42.3%) or multifocal (15/26, 57.7%) in distribution, nodular morphologically (23/25, 92.0%), and homogeneously enhancing (24/25, 96.0%). Symptomatic improvement occurred with steroids initially in 22/25 (88.0%). Ultimately, 23/26 (88.5%) required initiation of steroid-sparing immunosuppressants, including 8/26 (30.8%) eventually undergoing TNF inhibition. Pachymeningeal relapses occurred in 7/26 (26.9%). The median clinical follow-up was 48 months. The median modified Rankin scale score at last follow-up improved to 1.0 from 2.0 at presentation. DISCUSSION: Pachymeningitis due to sarcoidosis often presents with headaches, visual dysfunction, and seizures; it usually affects the dura of the falx cerebri, anterior and middle cranial fossae, and tentorium cerebelli and tends to require steroid-sparing immunosuppressants. It has the potential to relapse, but the prospect for recovery is good.

Comparison of 3D constructive interference in steady state (CISS) and T2 sampling perfection with application optimized contrasts using different flip angle evolution MR imaging of the intracranial trigeminal nerve and central skull base neuroforamina.

BACKGROUND AND PURPOSE: Due to surgical advancements, the accurate detection of perineural disease spread has become increasingly important in the management and prognostication of head and neck cancers, though MR evaluation has thus far been limited by technical and logistic challenges. The purpose of this study was to specifically evaluate the relative capability of 3D CISS and 3D T2-SPACE imaging to delineate the proximal intracranial divisions of the normal trigeminal nerve, an area important in determining the resectability of intracranial perineural disease. MATERIALS AND METHODS: A single center HIPAA-compliant, IRB approved retrospective review of 40 patients with clinical temporal bone/internal auditory canal MR imaging was conducted. 20 patients with 3D CISS images and 20 patients with 3D T2-SPACE images met inclusion criteria. Two radiologists scored the sequences on a 3-point scale based on ability to visualize anatomic structures surrounding the trigeminal nerve in Meckel's cave, intracranial trigeminal divisions, skull base neuroforamina, and proximal extracranial mandibular division. RESULTS: The following anatomic locations scored significantly better in the T2-SPACE sequence compared to the CISS sequence for both raters: intracranial V3 (p < .05), foramen ovale (p < .05), and extracranial V3 (p < .01). The average scores for the anterior Meckel's cave and foramen rotundum were higher for the T2-SPACE sequence, although not significantly. Percent interobserver agreement ranged from 50 to 90% and 65-100% for the different anatomic locations on the CISS and T2-SPACE sequences, respectively. CONCLUSION: 3D T2-SPACE was found to be superior to 3D CISS in the evaluation of the distal intracranial and extracranial portions of the normal trigeminal nerve.

Reducing low value services in surgical inpatients in Taiwan: Does diagnosis-related group payment work?

Reducing low-value care is a top priority in health care. However, how prospective payment methods such as diagnosis-related group (DRG) payment scheme reduce the use of low-value services is unclear. This study aimed to assess frequency of low-value preoperative testing services among surgical inpatients over time and to investigate whether the 2010 Tw-DRG policy has reduced utilization of these services under the National Health Insurance program in Taiwan. The nationwide National Health Insurance claims data in Taiwan from 2008 to 2013 were used. The difference-in-differences (DID) method was adopted. Utilization of three low-value preoperative testing services (chest x-ray, echocardiogram, and stress testing) were assessed. The prevalence of the three preoperative tests ranged from 0.13 per 100 admissions (preoperative stress testing) to 78.12 per 100 admissions (preoperative chest x-ray). Following the implementation of the Tw-DRG policy, the predicted probability of low-value care use was significantly reduced from 67.91% to 64.93% in the DRG group but remained relatively stable in the comparison group (from 69.44% to 68.43%) in 2010. The use of three selected preoperative tests had only a minor temporary reduction in 2010, but later increased over time. The 2010 Tw-DRG policy did not significantly moderate the growth of low-value preoperative use. Hospital financial incentives alone may be insufficient for reducing the provision of low-value care.

Review of Multimodality Imaging of Renal Trauma.

Blunt trauma accounts for more than 95% of traumatic renal injury and results from shear forces from rapid acceleration or deceleration and/or collision against the spine or ribs. The use of multiphasic contrast-enhanced computed tomography (CT) has proven pivotal in the evaluation and management of traumatic kidney injury, and CT imaging features provide the basis for nonsurgical staging. This article describes the epidemiology and mechanisms of blunt and penetrating traumatic renal injury and reviews the range of findings from various imaging modalities, with a particular emphasis on contrast-enhanced CT.

Absence of CHEK2*1100delC mutation in families with hereditary breast cancer in North America.

The CHEK2*1100delC mutation has been reported to confer a twofold increased risk of breast cancer among carriers. The frequency of the mutation varies among populations. The highest frequency has been described in Northern and Eastern European countries; the frequency may be much lower in North America. In this study, our aim was to determine the frequency of CHEK2*1100delC in members of breast cancer families who tested negative for a deleterious mutation in BRCA1/2 at the University of Michigan Comprehensive Cancer Center. We genotyped 102 members from 90 families for CHEK2*1100delC. Most of these families had several cases of breast cancer or ovarian cancer (or both), as well as multiple members with other cancer types in a single lineage. No CHEK2*1100delC mutations were detected in any of the 102 individuals, including 51 women diagnosed with breast cancer at an early age (<45 years), 8 women with bilateral breast cancer, 3 men with breast cancer, and 8 women with ovarian cancer. Our data are consistent with the reported very low frequency of CHEK2*1100delC mutations in North American populations (compared with Northern Europe), rendering CHEK2*1100delC such an unlikely culprit in BRCA1/2 negative families that routine testing of these families appears unwarranted.