Computed tomographic findings in TBX4 mutation: a common cause of severe pulmonary artery hypertension in children.

BACKGROUND: Mutations in the T-Box 4 (TBX4) gene are a lesser-known cause of heritable pulmonary arterial hypertension (PAH). Patients with heritable PAH typically have worse outcomes when compared with patients with idiopathic PAH, yet little is known about the phenotypical presentation of this mutation. OBJECTIVE: This article reviews the pattern of chest CT findings in pediatric patients with PAH and TBX4 mutations and compares their radiographic presentation with those of age-matched patients with PAH but without TBX4 mutations. MATERIALS AND METHODS: A retrospective chart review of the pulmonary arterial hypertension database was performed. Pediatric patients with PAH-confirmed TBX4 mutations and an available high CT were included. Fifteen (9 females) patients met the inclusion criteria. Fourteen (8 females) age-matched controls with diagnosed PAH but without TBX4 mutations were also evaluated. The median age at diagnosis was 7.4 years (range: 0.1-16.4 years). Demographic information and clinical outcomes were collected. CTs of the chest were reviewed for multiple airway, parenchymal, and structural abnormalities (16 imaging findings in total). Chi-square tests were used to compare the prevalence of each imaging finding in the TBX4 cohort compared to the control group. RESULTS: Patients with TBX-4 mutations had increased presence of peripheral or subpleural irregularity (73% vs 0%, P < 0.01), cystic lucencies (67% vs 7%, P < 0.01), and linear or reticular opacity (53% vs 0%, P < 0.01) compared to the control group. Ground glass opacities, bronchiectasis, and centrilobular nodules were not significantly different between the two patient groups (P > 0.05). CONCLUSION: TBX4 mutations have distinct imaging phenotypes in pediatric patients with PAH. Compared to patients without this mutation, patients with TBX-4 genes typically present with peripheral or subpleural irregularity, cystic lucencies, and linear or reticular opacity.

Varicoceles: Overview of Treatment from a Radiologic and Surgical Perspective.

A testicular varicocele is the result of the expansion of the venous pampiniform plexus of the scrotum. Often painless, a significant number of patients experience orchialgia, swelling, testicular atrophy, and abnormalities in spermatic parameters. Treatment of symptomatic varicocele involves a radiologic or surgical intervention to obstruct the reflux of venous drainage. Testicular anatomy, diagnostic evaluation and imaging, options for surgical intervention, and a step-by-step description of retrograde embolization and antegrade scrotal sclerotherapy are discussed. Furthermore, included is an overview of postprocedural management and patient outcomes for radiological interventions, and the most up-to-date evidence on the efficacy of varicocele treatments as well as how they compare to each other.

What Happened and Why: Responding to Racism, Discrimination, and Microaggressions in the Clinical Learning Environment.

Introduction: Within clinical learning environments, medical students are uniquely faced with power differentials that make acts of racism, discrimination, and microaggressions (RDM) challenging to address. Experiences of microaggressions and mistreatment are correlated with higher rates of positive depression screening and lower satisfaction with medical training. We developed a curriculum for medical students beginning clerkship rotations to promote the recognition of and response to RDM. Methods: Guided by generalized and targeted needs assessments, we created a case-based curriculum to practice communication responses to address RDM. The communication framework, a 6Ds approach, was developed through adaptation and expansion of established and previously learned communication upstander frameworks. Cases were collected through volunteer submission and revised to maintain anonymity. Faculty and senior medical students cofacilitated the small-group sessions. During the sessions, students reviewed the communication framework, explored their natural response strategies, and practiced all response strategies. Results: Of 196 workshop participants, 152 (78%) completed the evaluation surveys. Pre- and postsession survey cohort comparison demonstrated a significant increase in students' awareness of instances of RDM (from 34% to 46%), knowledge of communication strategies to mitigate RDM (presession M = 3.4, postsession M = 4.6, p < .01), and confidence to address RDM (presession M = 3.0, postsession M = 4.4, p < .01). Discussion: Students gained valuable communication skills from interactive sessions addressing RDM using empathy, reflection, and relatability. The workshop empowered students to feel prepared to enter professional teams and effectively mitigate harmful discourse.

Ocular adverse effects of therapeutic biologics.

Biological drugs, termed biologics, are medications that contain or are derived from a living organism (human, animal, or microorganism). With new biological agents being approved by the Food and Drug Administration (FDA) every year, clinicians need to know potential ocular adverse effects that are associated with these drugs. This review provides an overview of ocular adverse effects of biological medications used to treat both ophthalmic and non-ophthalmic diseases. We searched PubMed for relevant case reports, case series, reviews, and clinical trials reporting ocular adverse effects caused by biologics. This review was conducted in June 2021 and investigated the drugs listed in the most updated (April 2021) FDA Purple Book Database of Licensed Biological Products. This review focuses on monoclonal antibodies, interleukins, and receptor fusion proteins. We explore ocular side effects of 33 biological drugs, stating whether they are frequent, common, or rare.

Assessing the Efficacy of Four Diagnostic Devices and Four Nomograms in Posterior Chamber Phakic Intraocular Lens Size Selection.

PURPOSE: To assess the efficacy of different devices and nomograms in predicting the most optimal Implantable Collamer Lens (ICL) (STAAR Surgical) size. METHODS: This retrospective study includes 73 patients (142 eyes) who underwent ICL placement surgery. Anterior segment measurements were obtained by the OPD-Scan III (Marco), the Lenstar LS 900 (Haag-Streit AG/Alcon Laboratories, Inc), the Galilei G4 (Ziemer), and the VuMax ultrasound biomicroscopy device (UBM) (Sonomed Escalon). The efficacy of the four devices and four nomograms was assessed by comparing the indicated ICL sizes to the patient's postoperative vault measurements. RESULTS: Using white-to-white (WTW) measurements obtained by the Lenstar LS 900 and Galilei G4 coupled with the U.S. Food and Drug Administration's (FDA) (also called the manufacturer's) nomogram predicted the highest percentage (33% and 43%) of the 13.7 mm ICL sizes, respectively. Using the VuMax UBM coupled with the Dougherty nomogram indicated the highest percentage (80%) of the 12.6 mm ICL size. The ICL sizes predicted by UBM using the Parkhurst nomogram were comparable to those deemed ideal according to postoperative vault measurements. CONCLUSIONS: The Parkhurst nomogram predicted the most appropriate ICL size. When using WTW measurements, the optimized WTW nomogram showed promising results, especially in eyes whose preoperative calculations suggest low postoperative vaults. Moreover, clinicians should use caution when using the FDA nomogram because it showed a propensity toward indicating oversized ICLs. [J Refract Surg. 2022;38(2):106-111.].

Endophthalmitis Caused by Abiotrophia Defectiva after Anterior Vitrectomy in a Child.

Endophthalmitis caused by Abiotrophia defectiva has never before been reported in a child. We report a case of a 12-year-old female with a history of penetrating keratoplasty, followed years later by open globe injury and repair, and subsequent anterior vitrectomy, which resulted in endophthalmitis caused by Abiotrophia defectiva. We review previous cases of endophthalmitis by this organism and argue that its detection will become more common with the use of 16S rDNA PCR and MALDI-TOF mass spectrometry.

Agreement of anterior segment measurements between four diagnostic imaging devices in myopic patients.

PURPOSE: To compare the inter-device agreement of anterior chamber depth (ACD), central corneal thickness (CCT), flat keratometry (K1), steep keratometry (K2), corneal astigmatism (ΔK), and white-to-white (WTW) measurements. METHODS: This is a retrospective study with 73 myopic patients (142 eyes). We extracted and compared anterior segment measurements (ACD, CCT, K1, K2, ΔK, and WTW) of four devices (OPD-Scan III, Pentacam HR, Lenstar LS 900, and Galilei G4), and performed pairwise agreement comparisons between them. RESULTS: Agreement analyses revealed that the most agreement occurred: in Pentacam HR and Galilei G4 for ACD measurements, in Pentacam HR and Lenstar LS 900 for CCT measurements, in OPD-Scan III and Galilei G4 for WTW measurements, in OPD-Scan III and Pentacam HR for K1 and K2 measurements, and OPD-Scan III and Galilei G4 for ΔK measurements. CONCLUSION: OPD-Scan III is interchangeable with both Galilei G4 and Lenstar LS 900 for WTW measurements. OPD-Scan III and the Pentacam-HR are interchangeable in K1, K2, and ΔK measurements. OPD-Scan III and Lenstar LS 900, and OPD-Scan III and Galilei G4 are interchangeable in K1 and ΔK measurements, respectively.

Association of Federal Regulations in the United States and Canada With Potential Corneal Donation by Men Who Have Sex With Men.

Importance: Federal policy in the United States prohibits corneal donation by men who have had sex with another man (MSM) in the preceding 5 years, whereas Canada enforces a 12-month ban. The potential consequences of these policies on corneal donations should be evaluated. Objective: To estimate the number of potential corneal donations associated with MSM deferral policies in the United States and Canada. Design, Setting, and Participants: A nonvalidated telephone survey study was conducted of all 65 eye banks in the United States and Canada to investigate how many potential corneal donors were disqualified in 2018 because of federal MSM restrictions. Published demographic data were also used to arrive at a separate estimate. Survey data were gathered from May 2019 to February 2020. Main Outcomes and Measures: Eye banks were asked if they keep records of referrals disqualified specifically because of the federal MSM restrictions and, if so, how many referrals they disqualified in 2018 owing to MSM status. Results: Fifty-four of 65 eye banks (83%) responded to the survey, with 30 eye banks reporting they do not keep specific records of MSM deferrals. The remaining 24 eye banks reported disqualifying 360 referrals in 2018 because of MSM status, equating to 720 corneas. The 24 eye banks accounted for 46.2% of corneal donations in the United States and Canada in 2018, yielding an estimate of approximately 1558 corneas rejected that year because of MSM status. A separate estimate using published MSM demographic data indicates that up to 3217 potential corneal donations may have been disqualified in 2018 because of these federal policies. Conclusions and Relevance: Findings suggest that between 1558 and 3217 corneal donations were disqualified in 2018 because of federal regulations prohibiting corneal donation by men who have had sex with another man in the preceding 5 years in the United States or 1 year in Canada. With modern virologic testing that is reliable within days of HIV exposure and given the global shortage of corneal tissue, these policies should be reevaluated using current scientific evidence to increase the availability of vision-restoring surgery worldwide.