We report a 23-year-old female patient with ophthalmic features of albinism, including refractive errors, nystagmus, depigmented fundus, and foveal hypoplasia. She presented for a rhegmatogenous ...retinal detachment, which was surgically reattached with no complications. Further genetic testing revealed the presence of a heterozygous pathogenic oculocutaneous albinism OCA2 gene mutation, conferring carrier status. To the best of our knowledge, this is the first reported case of typical ocular phenotype of albinism, specifically nystagmus, in a patient who is carrier for oculo-cutaneous albinism. Further research is required to expand the genotype-phenotype relationship in carriers of oculocutaneous albinism. Ophthalmic Surg Lasers Imaging Retina 2024;55:349–353.
•The leading cause of pediatric choroidal neovascular membrane (CNVM) is idiopathic followed by inflammatory.•The most common first-line treatment was intravitreal of anti–vascular endothelial growth ...factor (anti-VEGF).•Average number of total anti-VEGF injections was 2.3, with a retreatment rate of 52.2%.•OCTA showed decrease in CNVM vessel density, vessel-length density, and height of RPED.
To report the clinical and imaging characteristics, including optical coherence tomography angiography (OCTA), and treatment outcomes of choroidal neovascular membranes (CNVMs) in children.
Retrospective clinical cohort study.
Thirty eyes from 25 children (56% girls) with CNVM from 2 centers were examined from 2005 to 2022. Clinical features, imaging findings, treatment regimens, and outcomes are described.
The most common causes of CNVM were idiopathic (48%) and inflammatory (20%). At diagnosis, most CNVMs were unilateral (80%), active (83.3%), and juxtafoveal (46.7%). Twenty-five eyes (83.3%) of 21 patients (84%) were treated. The most common first-line treatment was intravitreal injection of anti–vascular endothelial growth factor (anti-VEGF) (92%), with a retreatment rate of 52.2% at an average of 237 days. The average number of total injections per eye was 2.3. Injections were safely administered in the clinic (52.2%). A gain of 3 lines or 15 ETDRS (Early Treatment Diabetic Retinopathy Study) letters was observed at final visit. The average duration of follow-up was 56.46 ± 42.51 months. No ocular or systemic complication related to treatment was reported. Sixteen eyes (64%) had OCTA images at both presentation and final visit, which showed a decrease in CNVM vessel density and vessel-length density, and in the height of retinal pigment epithelium detachment (RPED).
There are a variety of underlying etiologies for pediatric CNVMs, which are most often unilateral. Treatment with intravitreal anti-VEGF can be beneficial and does not often require frequent or chronic dosing. OCTA demonstrated a decrease in the CNVM vessel density and vessel-length density as well as in the height of RPED.
The Covid pandemic and associated lockdown forced medical schools globally not only to deliver emergency remote teaching, but to consider alternative methods of high stakes assessment. Here we ...outline our approach to the resit virtual OSCE ("VOSCE") for final year medical students that we undertook during "lockdown" in the current pandemic.
The original 'pre Covid' examination blueprint was reviewed and modified for the virtual environment in both format and content. In anticipation of the new format delivery, a number of pre-training sessions took place for all parties, and standardised templates were developed.
A total of 9 students undertook the VOSCE, which took the form of a two-part exam (a communication and clinical examination component, and a practical procedures component). The VOSCE was completed by all students, examiners, simulated patients and invigilators on an online digital platform with no issues with regards to technical problems.
A total of 6 students passed the VOSCE and as such progressed to graduation. The limitation of assessing some particular types of skills across the remote format (such as practical procedures) was recognised. The training and the templates developed were helpful in case the VOSCE format needs to be adopted in future at short notice and/or expanded in future.
To provide an overview of the impact of retinopathy of prematurity (ROP), and the challenges in the screening, diagnosis, and treatment of ROP worldwide.
A comprehensive search was conducted using ...the PubMed database from January 2011 to October 2021 using the following keywords: retinopathy of prematurity, laser, and anti-vascular endothelial growth factor (VEGF). Data on patient characteristics, ROP treatment type, and recurrence rates were collected. The countries included in these studies were classified based on 2021-2022 World Bank definitions of high, upper-middle, lower-middle, and low-income groups. Moreover, a search for surgical outcomes for ROP and screening algorithms and artificial intelligence for ROP was conducted.
Thirty-nine studies met the inclusion criteria. ROP treatment and outcomes showed a trend towards intravitreal anti-VEGF injections as the initial treatment for ROP globally and the treatment of recurrent ROP in high-income countries. However, laser remains the treatment of choice for ROP recurrence in middle-income countries. Surgical outcomes for ROP stage 4A, 4B and 5 are similar worldwide. The incidence of ROP and ROP-related visual impairment continue to increase globally. Although telemedicine and artificial intelligence offer potential solutions to ROP screening in resource-limited areas, the current models require further optimization to reflect the global diversity of ROP patients.
ROP screening and treatment paradigms vary widely based on country income group due to disparities in resources, limited access to care, and lack of universal guidelines.
Swept-source optical coherence tomography angiography (SS-OCTA) was used to measure the age-dependent changes in macular choriocapillaris (CC) flow deficits (FDs) in normal eyes.
A prospective, ...cross-sectional study.
Subjects with normal eyes ranging in age from their 20s to their 80s were imaged using a 100-kHz SS-OCTA instrument (PLEX Elite 9000, Carl Zeiss Meditec, Dublin, California, USA). Both 3 × 3-mm and 6 × 6-mm scans were used to image the macular CC. Visualization of the CC and quantification of FDs were performed using a previously validated algorithm. The percentage of FDs (FD%) in the central 1-mm circle (C1), 1.5-mm rim (R1.5), and 2.5-mm circle (C2.5) from the 3 × 3-mm and 6 × 6-mm scans and FD% in the 2.5-mm rim (R2.5) and 5-mm circle (C5) from the 6 × 6-mm scans were measured and correlated with age and axial length.
A total of 164 subjects were enrolled, with at least 10 subjects from each decade of life. No meaningful correlations were found between FD% and axial length (|r| < 0.30). FD% in all fields increased with increasing age (all r > 0.50; all P < .001); however, the greatest increases were found in the central macula C1 regions and the smallest increases in the peripheral macula R2.5 regions.
In normal aging, the FD% increased with age across the central 5 mm of the macula, but the greatest increase was found in the central 1-mm region of the macula.
We present Submillimeter Array observations of a Keplerian disk around the Class I protobinary system L1551 NE in 335 GHz continuum emission and submillimeter line emission in super(13)CO (J = 3-2) ...and C super(18)O (J = 3-2) at a resolution of ~120 x 80 AU. The 335 GHz dust-continuum image shows a strong central peak closely coincident with the binary protostars and likely corresponding to circumstellar disks, surrounded by a ~600 x 300 AU feature elongated approximately perpendicular to the FeII jet from the southern protostellar component suggestive of a circumbinary disk. The super(13)CO and C super(18)O images confirm that the circumbinary continuum feature is indeed a rotating disk; furthermore, the C super(18)O channel maps can be well modeled by a geometrically thin disk exhibiting Keplerian rotation. We estimate a mass for the circumbinary disk of ~0.03-0.12 M sub(middot in circle), compared with an enclosed mass of ~0.8 M sub(middot in circle) that is dominated by the protobinary system. Compared with several other Class I protostars known to exhibit Keplerian disks, L1551 NE has the lowest bolometric temperature (~91 K), highest envelope mass (-0.39 M sub(middot in circle)), and the lowest ratio in stellar mass to envelope + disk + stellar mass (~0.65). L1551 NE may therefore be the youngest protostellar object so far found to exhibit a Keplerian disk. Our observations present firm evidence that Keplerian disks around binary protostellar systems, "Keplerian circumbinary disks," can exist. We speculate that tidal effects from binary companions could transport angular momenta toward the inner edge of the circumbinary disk and create the Keplerian circumbinary disk.
Development of cardiac dysfunction is associated with increased morbidity and mortality in patients with sepsis. Increasing evidence shows that gender determines the degree of inflammatory response ...of the host and that females tolerate sepsis better than males. It is unknown whether gender affects the cardiac dysfunction in animals or patients with sepsis. To investigate this, male or female C57BL/6 mice were subjected to either lipopolysaccharide (LPS)/peptidoglycan (PepG) co-administration or cecal ligation and puncture (CLP). At 18 hours after LPS/PepG injection or 24 hours after CLP, cardiac function was evaluated by echocardiography. The septic insult caused a significant cardiac dysfunction in both genders. However, the cardiac dysfunction was significantly less pronounced in females in comparison with males subjected to LPS (3 mg/kg)/PepG (0.1 mg/kg) or CLP. Compared with males injected with LPS (3 mg/kg)/PepG (0.1 mg/kg), western blotting analysis of the myocardium from females injected with LPS/PepG revealed i) profound increases in phosphorylation of Akt and eNOS; ii) significant decreases in phosphorylation of IκBα, nuclear translocation of the NF-κB subunit p65, decreased expression of iNOS and decreased synthesis of TNF-α and IL-6 in the heart. However, the gender dimorphism of the cardiac dysfunction secondary to LPS/PepG was not observed when higher doses of LPS (9 mg/kg)/PepG (1 mg/kg) were used. In conclusion, the cardiac dysfunction caused by sepsis was less pronounced in female than in male mice. The protection of female hearts against the dysfunction associated with sepsis is (at least in part) attributable to cardiac activation of the Akt/eNOS survival pathway, decreased activation of NF-κB, and decreased expression of iNOS, TNF-α and IL-6. It should be noted that the observed gender dimorphism of the cardiac dysfunction in sepsis was not seen when a very severe stimulus (high dose of LPS/PepG co-administration) was used to cause cardiac dysfunction.
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