Low-dose diazoxide is safe and effective in infants with transient hyperinsulinism.

OBJECTIVE: Transient hyperinsulinism (THI) is the most common form of recurrent hypoglycaemia in neonates beyond the first week of life. Although self-resolving, treatment can be required. Consensus guidelines recommend the lower end of the diazoxide 5-15 mg/kg/day range in THI to reduce the risk of adverse events. We sought to determine if doses <5 mg/kg/day of diazoxide can be effective in THI. DESIGN, PATIENTS, MEASURMENTS: Infants with THI (duration <6 months) were treated with low-dose diazoxide from October 2015 to February 2021. Dosing was based on weight at diazoxide start: 2 mg/kg/day in infants 1000-2000 g (cohort 1), 3 mg/kg/day in those 2000-3500 g (cohort 2) and 5 mg/kg/day in those >3500 g. RESULTS: A total of 73 infants with THI (77% male, 33% preterm, 52% small-for-gestational age) were commenced on diazoxide at a median age of 11 days (range 3-43) for a median duration of 4 months (0.3-6.8), with no difference between cohorts. The mean effective diazoxide dose was 3 mg/kg/day (range 1.5-10); 35% (26/73) required an increase from their starting dose, including 60% (9/15) of cohort 1. There was no association between perinatal stress risk factors or treatment-related characteristics and dose increase. Adverse events occurred in 13 patients (18%); oedema (12%) and hyponatraemia (5%) were the most common. Two infants developed suspected necrotising enterocolitis (NEC); none had pulmonary hypertension. CONCLUSION: Diazoxide doses <5 mg/kg/day are effective in THI. While the nature of the association between diazoxide and NEC was unclear, other adverse events were mild. We suggest considering starting doses as low as 2-3 mg/kg/day in THI to balance the side effect risk while maintaining euglycaemia.

Standardised practices in the networked management of congenital hyperinsulinism: a UK national collaborative consensus.

Congenital hyperinsulinism (CHI) is a condition characterised by severe and recurrent hypoglycaemia in infants and young children caused by inappropriate insulin over-secretion. CHI is of heterogeneous aetiology with a significant genetic component and is often unresponsive to standard medical therapy options. The treatment of CHI can be multifaceted and complex, requiring multidisciplinary input. It is important to manage hypoglycaemia in CHI promptly as the risk of long-term neurodisability arising from neuroglycopaenia is high. The UK CHI consensus on the practice and management of CHI was developed to optimise and harmonise clinical management of patients in centres specialising in CHI as well as in non-specialist centres engaged in collaborative, networked models of care. Using current best practice and a consensus approach, it provides guidance and practical advice in the domains of diagnosis, clinical assessment and treatment to mitigate hypoglycaemia risk and improve long term outcomes for health and well-being.

Incidence of primary open angle glaucoma in the Andhra Pradesh Eye Disease Study (APEDS).

BACKGROUND: To report 15-year incidence rate of primary open angle glaucoma (POAG) in the Andhra Pradesh Eye Disease Study (APEDS). METHODS: A population-based longitudinal study was carried out at three rural study sites. Phakic participants aged ≥40 years who participated at baseline (APEDS I) and the mean 15-year follow-up visit (APEDS III) were included. A comprehensive ophthalmic examination was performed on all participants. Mean intraocular pressure (IOP) was average of IOPs of right and left eyes. The definition of glaucoma was based on the International Society of Geographical and Epidemiological Ophthalmology (ISGEO) classification. The main outcome measure was incidence of POAG during the follow-up period in participants without glaucoma or suspicion of glaucoma at baseline. RESULTS: Data from the available and eligible participants from the original cohort (1241/2790; 44.4%) were analysed. The mean age (standard deviation) of participants at baseline was 50.2 (8.1) years; 580 (46.7%) were men. Thirty-six participants developed POAG [bilateral in 17 (47.2%)] over 15 years. The incidence rate of POAG per 100-person years (95% confidence interval) was 2.83 (2.6, 3.08). Compared to baseline, the reduction in mean IOP [median (range) mm Hg] was -0.75 (-7.5, 9) in participants with incident POAG and -2.5 (-14.5, 14.5) in those without. The inter-visit difference in mean IOP was a significant risk factor on logistic regression analysis. CONCLUSION: We report the long-term incidence of POAG in rural India. A longitudinal change in IOP, specifically a less pronounced reduction in IOP with increasing age, was a novel risk factor.

Prevalence and pattern of retinopathy of prematurity at two national referral hospitals in Uganda: a cross-sectional study.

BACKGROUND: Retinopathy of prematurity (ROP) is a leading cause of blindness in children and an ROP epidemic is predicted this decade in sub-Saharan Africa. With the increasing survival rate of preterm babies in Uganda, and no data on ROP prevalence, there is a need to assess the burden of ROP to inform preventive strategies and targeted screening. METHODS: We conducted a two-center cross-sectional study of preterm (< 37 weeks gestational age) infants from the neonatal units of Kawempe National Referral Hospital (KNRH) and Mulago Specialised Women and Neonatal Hospital (MSWNH) from August 2022 to October 2022. An ophthalmologist examined all participants using an indirect ophthalmoscope with a + 20D convex lens and captured digital images using a Volk iNview™ Fundus Camera. The collected data were entered into Epidata 4.2 and exported to Stata 14.0 for analysis. RESULTS: 331 preterm infants enrolled in this study. The oxygen received was unblended. The mean gestational age was 30.4 ± 2.7 weeks, and the mean birth weight was 1597 ± 509 g. 18/101 (17.8%) were found to have any ROP amongst the preterm infants recruited from MSWNH, 1/230 (0.4%) from KNRH [95% CI] had any stage of ROP (i.e. stage 5). Of these, 8 (42.1%) had stage 2 ROP. Infants with a birth weight below 1500 g were 10 times more likely to have ROP than those among infants with a birth weight more than 1500 g [AOR: 10.07 (2.71-37.44)]. Infants who were not fed exclusively on breast milk had higher odds of having ROP than those exclusively fed on breast milk [AOR: 7.82(1.92-31.82)]. CONCLUSION: 6% of preterm infants born in two tertiary hospitals in Uganda were found to have ROP. Lack of exclusive feeding on breast milk and birth weight of less than 1500 g were strong predictors of ROP. The higher prevalence of ROP in MSWNH calls for cautious use of oxygen among preterms. We recommend targeted ROP screening for those at risk.

Impact of COVID-19 pandemic on retinopathy of prematurity services in low resource settings.

BACKGROUND AND OBJECTIVES: Retinopathy of prematurity is an increasingly important cause of blindness in children in low- and middle-income countries. Timely screening and treatment greatly reduce blindness. This study assessed the impact of the COVID-19 pandemic on ROP services in low- and middle-income countries. METHODS: An online survey with closed and open-ended questions in Microsoft Teams was sent to 304 ophthalmologists providing screening, treatment and/or vitreoretinal services (January 15 to March 12 2021). Categories were used to report impact on services. 28 participants were purposively selected for interview. RESULTS: 184/201 completed forms from 32 countries were received. Two thirds of participants worked in government facilities and 45.6% were paediatric ophthalmologists. A moderate to severe impact was reported by 13% for screening, 9% for treatment and 16% for vitreoretinal surgery with some variation between government, private and not-for-profit sectors. 22% thought services would take a year to recover. Fifteen ophthalmologists from 12 countries were interviewed. Many reported fewer neonatal admissions, and several reported more ROP blind infants once restrictions lifted. Themes associated with services continuing included limited lockdown, autonomy and flexibility, commitment, advocacy and technology. Themes associated with a negative impact include fear and panic, closure of facilities, COVID infection or quarantining of health workers and parents, lack of transport and exacerbation of poverty. CONCLUSIONS: The COVID pandemic had a very variable impact on the provision of ROP services. In some countries where the impact was great, more infants may have become blind from ROP. Lessons can be learnt for planning pandemic preparedness.

Accuracy and impact on quality of life of real-time continuous glucose monitoring in children with hyperinsulinaemic hypoglycaemia.

Objective: Continuous glucose monitoring (CGM) is the standard of care for glucose monitoring in children with diabetes, however there are limited data reporting their use in hyperinsulinaemic hypoglycaemia (HH). Here, we evaluate CGM accuracy and its impact on quality of life in children with HH. Methods: Real-time CGM (Dexcom G5 and G6) was used in children with HH aged 0-16years. Data from self-monitoring capillary blood glucose (CBG) and CGM were collected over a period of up to 28days and analysed. Quality of life was assessed by the PedsQL4.0 general module and PedsQL2.0 family impact module, completed by children and their parents/carers before and after CGM insertion. Analysis of accuracy metrics included mean absolute relative difference (MARD) and proportion of CGM values within 15, 20, and 30% or 15, 20, and 30 mg/dL of reference glucose values >100 mg/dL or ≤100 mg/dL, respectively (% 15/15, % 20/20, % 30/30). Clinical reliability was assessed with Clarke error grid (CEG) analyses. Results: Prospective longitudinal study with data analysed from 40 children. The overall MARD between reference glucose and paired CGM values (n=4,928) was 13.0% (Dexcom G5 12.8%, Dexcom G6 13.1%). The proportion of readings meeting %15/15 and %20/20 were 77.3% and 86.4%, respectively, with CEG analysis demonstrating 97.4% of all values in zones A and B. Within the hypoglycaemia range (<70 mg/dL), the median ARD was 11.4% with a sensitivity and specificity of 64.2% and 91.3%, respectively. Overall PedsQL child report at baseline and endpoint were 57.6 (50.5 - 75.8) and 87.0 (82.9 - 91.2), and for parents were 60.3 (44.8 - 66.0) and 85.3 (83.7 - 91.3), respectively (both p<0.001). Conclusion: Use of CGM for children with HH is feasible, with clinically acceptable accuracy, particularly in the hypoglycaemic range. Quality of life measures demonstrate significant improvement after CGM use. These data are important to explore use of CGM in disease indications, including neonatal and paediatric diabetes, cystic fibrosis and glycogen storage disorders.

Reducing the Impact of the COVID-19 Pandemic on Retinopathy of Prematurity Screening: Successful Strategies Learnt from a Multi-Center Tele-Medicine Program in India.

PURPOSE: Control of blindness due to retinopathy of prematurity (ROP) requires timely screening and treatment within 48-72 h. Anticipating that the coronavirus disease 2019 (COVID-19) pandemic would disrupt ROP services, we devised strategies ''on-the''-go"" to ameliorate this possiblity. We describe the successful outcomes of this approach in preventing infant blindness during the pandemic. METHODS: Data on the number of preemies recruited, screened and treated in the Karnataka Internet-assisted Diagnosis of Retinopathy of Prematurity (KIDROP) program were collected in a retrospective (2019, interval 1) - prospective (2020, interval 2) manner. We summarize 10 key strategies that were developed as we faced logistic, operational and implementation challenges. These included pragmatic methods of enhancing enrolment, transporting for screening and ensuring timely treatment in the outreach. RESULTS: The total number of ROP screening sessions was 20,598 (7,197 new) and 14,371 (5,773 new) during interval 1 and 2 respectively. Of these, 166 (2.3%) and 157 (2.7%) infants required treatment during interval 1 and 2 respectively. All infants needing treatment during the COVID period, were treated on time which was possible due to successful implementation of the 'on-the-go' strategies throughout the state of Karnataka. The fiscal equivalent of the blindness prevented during this period is USD 15.6 million. CONCLUSION: The greater decline in the number of ROP screening episodes in neonatal units in government hospitals was because several were converted to 'COVID only" hospitals. KIDROP's multi-zonal, decentralized strategy, which uses non-physician-based imaging in a telemedicine network, ensured that essential ROP services continued even during the lockdown.

Retinopathy of prematurity among infants admitted to two neonatal intensive care units in Ethiopia.

OBJECTIVE: This study was conducted to determine the prevalence and risk factors for retinopathy of prematurity (ROP) in two neonatal intensive care units (NICUs) in Addis Ababa, Ethiopia. METHODS AND ANALYSIS: A prospective screening survey was conducted from June 2019 to June 2020 in two level 3 public NICUs. Infants with a birth weight (BW) of ≤1500 g or gestational age (GA) of ≤32 weeks and those with a BW of >1500 g and GA of >32 weeks with an unstable clinical course were included. Data on demographic and neonatal characteristics, neonatal and maternal comorbidities, and therapeutic interventions were collected. Logistic regression analysis was used to identify predictors of ROP. RESULTS: Two hundred and two infants were included: mean BW: 1658g (range: 700-2400 g) and mean GA: 32.4 weeks (range: 26-34 weeks). 32.2% had any stage of ROP, and 6.4% had Type 1 ROP. Lower BW, smaller GA and total days on oxygen were independent risk factors for severe ROP (Type 1 or worse). All 13 neonates with severe ROP were treated. CONCLUSION: ROP is emerging as a concern in Ethiopia. ROP screening should include neonates with BW of <1800 g or GAs of ≤33 weeks, but further studies are needed in level 2 and private NICUs. Screening guidelines need to be developed and implemented in all hospitals with NICUs.

Improved screening of retinopathy of prematurity (ROP): development of a target product profile (TPP) for resource-limited settings.

BACKGROUND: As more preterm infants survive, complications of preterm birth, including retinopathy of prematurity (ROP), become more prevalent. ROP rates and blindness from ROP are higher in low-income and middle-income countries, where exposure to risk factors can be higher and where detection and treatment of ROP are under-resourced or non-existent. Access to low-cost imaging devices would improve remote screening capabilities for ROP. METHODS: Target product profiles (TPPs) are developed early in the medical device development process to define the setting, target user and range of product requirements. A Delphi-like process, consisting of an online survey and consensus meeting, was used to develop a TPP for an ROP imaging device, collecting feedback on a proposed set of 64 product requirements. RESULTS: Thirty-six stakeholders from 17 countries provided feedback: clinicians (72%), product developers (14%), technicians (6%) and other (8%). Thirty-six per cent reported not currently screening for ROP, with cited barriers including cost (44%), no training (17%) and poor image quality (16%). Among those screening (n=23), 48% use more than one device, with the most common being an indirect ophthalmoscope (87%), followed by RetCam (26%) and smartphone with image capture (26%). Consensus was reached on 53 (83%) product requirements. The 11 remaining were discussed at the consensus meeting, and all but two achieved consensus. CONCLUSIONS: This TPP process was novel in that it successfully brought together diverse stakeholders to reach consensus on the product requirements for an ROP imaging devices. The resulting TPP provides a framework from which innovators can develop prototypes.

Cataract services for all: Strategies for equitable access from a global modified Delphi process.

Vision loss from cataract is unequally distributed, and there is very little evidence on how to overcome this inequity. This project aimed to engage multiple stakeholder groups to identify and prioritise (1) delivery strategies that improve access to cataract services for under-served groups and (2) population groups to target with these strategies across world regions. We recruited panellists knowledgeable about cataract services from eight world regions to complete a two-round online modified Delphi process. In Round 1, panellists answered open-ended questions about strategies to improve access to screening and surgery for cataract, and which population groups to target with these strategies. In Round 2, panellists ranked the strategies and groups to arrive at the final lists regionally and globally. 183 people completed both rounds (46% women). In total, 22 distinct population groups were identified. At the global level the priority groups for improving access to cataract services were people in rural/remote areas, with low socioeconomic status and low social support. South Asia and Sub-Saharan Africa were the only regions in which panellists ranked women in the top 5 priority groups. Panellists identified 16 and 19 discreet strategies to improve access to screening and surgical services, respectively. These mostly addressed health system/supply side factors, including policy, human resources, financing and service delivery. We believe these results can serve eye health decision-makers, researchers and funders as a starting point for coordinated action to improve access to cataract services, particularly among population groups who have historically been left behind.

Spectrum of neuro-developmental disorders in children with congenital hyperinsulinism due to activating mutations in GLUD1.

Background: Hyperinsulinism/hyperammonemia (HI/HA) syndrome is the second most common type of congenital hyperinsulinism caused by an activating GLUD1 mutation. Objective: The aim of this study was to determine the clinical profile and long-term neurological outcomes in children with HI/HA syndrome. Method: This study is a retrospective review of patients with GLUD1 mutation, treated at two centers in the UK and Russia, over a 15-year period. Different risk factors for neuro-developmental disorders were analysed by Mann-Whitney U test and Fisher's exact P test. Results: We identified 25 cases with GLUD1 mutations (12 males). Median age of presentation was 7 months (12 h-18 months). Hypoglycaemic seizures were the presenting feature in 24 (96%) cases. Twenty four cases responded to diazoxide and protein restriction whilst one patient underwent partial pancreatectomy. In total, 13 cases (52%) developed neurodevelopmental manifestations. Epilepsy (n = 9/25, 36%), learning difficulties (n = 8/25, 32%) and speech delay (n = 8/25, 32%) were the most common neurological manifestation. Median age of presentation for epilepsy was 12 months with generalised tonic-clonic seizures being the most common (n = 4/9, 44.4%) followed by absence seizures (n = 3/9, 33.3%). Early age of presentation (P = 0.02), diazoxide dose (P = 0.04) and a mutation in exon 11 or 12 (P = 0.01) were associated with neurological disorder. Conclusion: HI/HA syndrome is associated with wide spectrum of neurological disorders. These neurological manifestations were more frequent in cases with mutations affecting the GTP-binding site of GLUD1 in our cohort.

Regional variation in the incidence of pseudo-exfoliation in the Andhra Pradesh Eye Disease Study (APEDS).

BACKGROUND: To report the 15-year incidence rate of pseudo-exfoliation (PXF),  PXF glaucoma and regional variation among rural participants in the Andhra Pradesh Eye Disease Study (APEDS) III. METHODS: This population-based longitudinal study was carried out at three rural study sites. Individuals of all ages who participated at baseline with a mean 15-year follow-up visit were included. Detailed Comprehensive ophthalmic examination was performed on all participants. The main outcome measure was development of PXF during the follow-up period in participants who were phakic in one or both eyes without PXF at baseline. RESULTS: Among 5395 participants, 5108 (94.6%) met the inclusion criteria. There were 93 (1.82%; 95% confidence interval (CI), 1.47-2.22) cases of incident PXF. Their median baseline age (1st, 3rd quartiles) was 51 (44, 59) years and the male: female ratio was 1.3:1. There was no case of incident PXF in participants aged <30 years at baseline. The incidence rate per 100 person years (95% CI) among all ages and those aged ≥30 years at baseline was 1.73 (1.64-1.82) and 3.73 (3.53-3.93), respectively. PXF material was located on iris as well as anterior surface of lens and it was often bilateral. Participants living in two study sites and increasing age were associated with the incidence of PXF. The 15-year incidence of PXF glaucoma (95% CI) in participants ≥30 years of age at baseline was 0.33% (0.14-0.66). CONCLUSION: There is significant regional variation in incidence of PXF in south India which warrants further investigation.

Universal newborn eye screening: a systematic review of the literature and review of international guidelines.

Background: This systematic review assessed the effectiveness of universal screening for newborn eye abnormalities compared with no screening in improving infant vision and health outcomes. Methods: We searched CENTRAL (Cochrane Library), MEDLINE, Embase, Global Health, Global Index Medicus, clinical trials databases, and bibliographies of relevant articles. We included randomized and observational studies of all newborns, regardless of illness or risk factors, that compared universal screening for any eye abnormality by eight weeks of age with no universal screening. Two authors independently selected studies, extracted data, and evaluated the risk of bias. We used GRADE to assess the certainty of evidence. We also reviewed available recommendations on newborn eye screening. Results: Fourteen studies were identified but only three compared universal red reflex screening with no screening. Findings suggest that universal red reflex testing in maternity wards (MWs) may increase the number of newborns with congenital cataracts referred for eye care from MWs or well-baby clinics (WBCs) in the first year of life (risk ratio (RR) = 9.83, 95% confidence interval (CI) = 1.36-71.20; low certainty evidence). However, the effect of screening in WBC is uncertain (RR = 6.62, 95% CI = 0.87-50.09). The effect of MW or WBC screening on referral from any health care facility (MWs, WBCs, paediatrician clinic, other) in the first year is uncertain (MW screening: RR = 1.22, 95% CI = 0.63-2.39; WBC screening: RR = 0.97, 95% CI = 0.46-2.05). However, referral or surgery by 6 weeks of age may be higher with universal MW screening (early referral: RR = 4.61, 95% CI = 1.12-19.01; early surgery: RR = 8.23, 95% CI = 1.13-59.80; low certainty evidence). The effect of WBC screening on early referral and surgery is uncertain (early referral: RR = 1.98, 95% CI = 0.43-9.19; early surgery: RR = 3.97, 95% CI = 0.50-31.33; very low certainty evidence). Universal red reflex testing may increase clinical conjunctivitis (OR = 1.22, 95% CI = 1.01-1.47; low certainty evidence) but the effect on confirmed bacterial conjunctivitis is uncertain (OR = 1.20, 95% CI = 0.76-1.90; very low-certainty evidence). Nine guidelines recommended universal newborn eye screening using red reflex testing. Conclusions: Evidence supports the role of red reflex testing shortly after birth to increase early identification, referral, and surgery for congenital cataracts.

Improving the Quality of Health Care in Special Neonatal Care Units of India: A Before and After Intervention Study.

BACKGROUND: We evaluated the efficacy of training health care workers (HCWs) in point-of-care quality improvement (POCQI) and a preterm newborn health care package (PHCP), followed by remote mentoring and supportive supervision in improving health care practices, neonatal survival, and morbidities in special neonatal care units (SNCUs). METHODS: This pre- and postintervention quality improvement study was conducted at 3 SNCUs in Madhya Pradesh, India from February 2017 to February 2019. Clinical care teams comprising doctors and nurses from the study sites were trained in POCQI and the PHCP. The teams identified, prioritized, and analyzed problems and designed quality improvement initiatives at their respective health facilities. Change ideas were tested by the local teams using sequential plan-do-study-act cycles. Facilitators maintained contact with the teams through quarterly review meetings, fortnightly videoconferencing, on-demand phone calls, and group chat service. State SNCU coordinators made follow-up visits to supplement coaching. Study research staff independently collected data on admissions, health care practices, and outcomes of neonates. FINDINGS: A total of 156 HCWs were trained in the POCQI methodology and PHCP. Sixteen quality improvement projects were formulated and implemented. Among 13,821 enrolled neonates (birth weight 2275±635 g; gestation: 35.8±2.8 weeks), improvement was seen in reduction of use of oxygen (36.1% vs. 48.0%; adjusted odds ratio [aOR]=0.60, 95% confidence interval [CI]=0.55, 0.66), antibiotics (29.4% vs. 39.0%; aOR=0.76, 95% CI=0.68, 0.85), and dairy milk (33.8% vs. 49.4%; aOR=0.34, 95% CI=0.31 to 0.38). Enteral feeds were started within 24 hours of admission in a larger number of neonates, resulting in fewer days to reach full feeds. There was no effect on survival at discharge from the hospital (aOR=0.93; 95% CI=0.80, 1.09). CONCLUSION: A collaborative cross-learning quality improvement approach with remote mentoring, coaching, and supportive supervision was successful in improving the quality of care at SNCUs.

Systematic review of the guidelines for retinopathy of prematurity.

As the characteristics of preterm infants with retinopathy of prematurity (ROP) vary, depending on the quality of neonatal care they received, universal screening criteria for ROP are not possible. The purpose of this study was to compare ROP guidelines for those planning to develop guidelines, particularly in resource-poor settings.A systematic review was undertaken of PubMed, Embase and guidelines registers to identify national or international policy statements or guidelines in English, issued or operational in 2010-2021. Bibliographies of two publications were also reviewed. The searches identified 633, 1081 and 317 records, respectively, and 157 records were retrieved from other sources. 23 publications were included in the analysis.Most included documents defined cut-offs for screening as <32 weeks gestational age (GA) or ≤1500g birth weight (BW). The highest values were in the Philippines (<35 weeks GA; <2000g) and India (≤34 weeks GA; <2000g). The lowest were in high-income countries, i.e. the United States of America (≤32 weeks for GA) and New Zealand (<1250g for BW). Most guidelines included additional risk factors to consider. The most frequent indication for when screening should start was a combination of GA and chronological age. All but one document defined when screening could stop. There was general consensus on the indications, timing and methods of treatment. Indications for anti-VEGF therapy varied between countries.Guidelines were identified for a limited number of countries with none from low-income settings. Variation in the indications for screening reflects the varying exposure to risk factors in different settings.

Factors associated with adherence to treatment in patients with open angle glaucoma in Sierra Leone, West Africa: patient demographics and questionnaire.

BACKGROUND: Glaucoma is a significant cause of blindness worldwide. It is more common, presents earlier and is more aggressive in those of African descent. Non-adherence and poor knowledge of glaucoma is a significant barrier to treatment and has been associated with low health literacy. We aim to establish the factors contributing to late presentation, treatment non-adherence and disease progression in glaucoma patients in Sierra Leone. This will help better understand the challenges eye services face, highlight fields requiring development in patient-clinician interaction and identify areas or specific vulnerable patient groups in which resources should be focused. METHODS: Prospective, consecutive recruitment of 120 patients with POAG attending the Lowell and Ruth Gess Eye Hospital and the Connaught Government Teaching Hospital, Freetown, Sierra Leone between February and April 2020. Data were collected from 3 sources: (1) review of clinical notes since first attendance, (2) semi-structured interviews and (3) assessment of study participant's drop instillation technique using a structured checklist. Descriptive statistics was performed for demographic data and other relevant data points. Logistic regression was used for analysis of target variables. RESULTS: The average age was 62 years with more males (52.6%). Agricultural workers and informal street traders represented 13.2% of participants' occupation. 25.8% of participants had no formal school, and 47.4% had either a degree or a diploma. This is out of proportion with the general population and may represent a hidden demographic of glaucoma patients. Drop instillation technique was successful in 52% of study participants. Notable responses to the questionnaire were 30% of patients did not know the name of their eye condition and 22% had no knowledge of glaucoma. CONCLUSION: Investment in a wide-ranging and robust screening programme and public health campaigns targeting these vulnerable groups and high-risk individuals, for example with a positive family history, alongside improved patient education and staff training is required to improve glaucoma care. Support from government, international organisations and the private sector is required to reduce the economic burden of blindness in Sierra Leone.

Variation in Glycemic Outcomes in Focal Forms of Congenital Hyperinsulinism-The UK Perspective.

Context: In focal congenital hyperinsulinism (CHI), localized clonal expansion of pancreatic ß-cells causes excess insulin secretion and severe hypoglycemia. Surgery is curative, but not all lesions are amenable to surgery. Objective: We describe surgical and nonsurgical outcomes of focal CHI in a national cohort. Methods: Patients with focal CHI were retrospectively reviewed at 2 specialist centers, 2003-2018. Results: Of 59 patients with focal CHI, 57 had heterozygous mutations in ABCC8/KCNJ11 (51 paternally inherited, 6 de novo). Fluorine-18 L-3,4 dihydroxyphenylalanine positron emission tomography computed tomography scan identified focal lesions in 51 patients. In 5 patients, imaging was inconclusive; the diagnosis was established by frozen section histopathology in 3 patients, a lesion was not identified in 1 patient, and 1 declined surgery. Most patients (n = 56) were unresponsive to diazoxide, of whom 33 were unresponsive or partially responsive to somatostatin receptor analog (SSRA) therapy. Fifty-five patients underwent surgery: 40 had immediate resolution of CHI, 10 had persistent hypoglycemia and a focus was not identified on biopsy in 5. In the 10 patients with persistent hypoglycemia, 7 underwent further surgery with resolution in 4 and ongoing hypoglycemia requiring SSRA in 3. Nine (15% of cohort) patients (1 complex surgical access; 4 biopsy negative; 4 declined surgery) were managed conservatively; medication was discontinued in 8 children at a median (range) age 2.4 (1.5-7.7) years and 1 remains on SSRA at 16 years with improved fasting tolerance and reduction in SSRA dose. Conclusion: Despite a unifying genetic basis of disease, we report inherent heterogeneity in focal CHI patients impacting outcomes of both surgical and medical management.

Visual impairment and blindness among children from schools for the blind in Maharashtra state, India: Changing trends over the last decade.

PURPOSE: To determine the causes of severe visual impairment and blindness in children in schools for the blind in Maharashtra, India. METHODS: Children aged <16 years, enrolled in the schools for the blind in Maharashtra state, India were examined between October 2018 and December 2019. The anatomical sites and etiology for blindness were recorded using the World Health Organization's standard reporting form. Causes of blindness were compared among different regions of the state and also by different age groups. RESULTS: Of the 1,969 students examined from 39 schools for the blind, 188 children (9.5%) had severe visual impairment and 1,666 children (84.6%) were blind. Whole globe anomalies (794, 42.8%) were the most common anatomical site of vision loss in children, followed by corneal (289, 15.6%) and retinal abnormalities (280, 15.2%). Corneal causes were second most common in the poorer districts of Vidarbha (15.3%) and Marathwada (14.6%), whereas retinal causes were second most common in the wealthier regions of western Maharashtra (18.3%) and Khandesh (24.1%). Nearly one-third (593, 32%) of children were blind from potentially avoidable causes. Preventable blindness consisting of corneal causes and retinopathy of prematurity was seen in 281 (15.2%) cases, whereas treatable causes comprising of lens-related causes, glaucomas, refractive errors, amblyopia, and uveitis accounted for another 311 (16.8%). Among the younger children (≤10 years), the proportion of corneal blindness was lower (83/623, 13.3% vs. 206/1232, 16.7%) and that of retinal blindness was higher (119/623, 19% vs. 163/1232, 13.2%) than the older children. CONCLUSION: Whole globe anomalies constitute a major cause of SVI and blindness in Maharashtra. There seems to be an increase in the proportion of retinal blindness, especially retinopathy of prematurity, suggesting a need for increased screening coverage.