Latest Visit Research Articles

Clinical performance of a custom-designed soft contact lens in patients with keratoconus and intolerance to rigid contact lenses.

This study evaluated the efficacy and safety of YOUSOFT® soft contact lens (CL) (TOMEY-CL) in patients with keratoconus and intolerance to rigid CLs. Retrospective observational study. Thirty-six eyes of 20 patients (14 men and 6 women) with keratoconus and rigid CL intolerance were included in the study. Four patients were unilateral. The mean age was 33.1 ± 11.7 (± standard deviation) years, the mean spherical refractive error was -4.99 ± 3.97 D, and the mean cylindrical refractive error was -3.39 ± 2.13 D. The following examinations were performed at baseline and were repeated at every visit; refractometry, visual acuity, corneal topography and pachymetry with an anterior OCT system. Corneal endothelial cell density was evaluated at baseline and every 6 months. Slit-lamp examination was also performed to confirm the CL condition and ocular health at every visit. Seventeen patients (85%) were able to continue using the Yousoft. Among 3 patients (15%) who dropped out, 2 were dissatisfied with their visual outcomes and one had trouble with lens handling. Uncorrected visual acuity was 1.08 ± 0.43 (range: 0.22 to 2.00) logMAR at baseline, and best CL-corrected visual acuity was 0.01 ± 0.15 (range: -0.18 to 0.40) logMAR, showing a significant improvement after wearing Yousoft (P < 0.0001). The endothelial cell density did not change significantly, with 2373 ± 482 at baseline and 2402 ± 464 cells/mm2 at the latest visit after lens prescription (P = 0.351). There were no severe complications such as corneal infiltrates or infectious keratitis throughout the study period. This study showed the efficacy and acceptable safety of Yousoft for patients with keratoconus and intolerance to rigid CLs.

Clinical presentation patterns of patients with coeliac disease in Gaza Strip: a cross-sectional descriptive study

BackgroundCoeliac disease is an autoimmune disease of the small intestine precipitated by the ingestion of gluten in genetically susceptible individuals. Clinical presentation of patients with coeliac disease patients varies depending on age and has been associated with other autoimmune conditions. In the Gaza strip, the patterns of clinical presentations and associations have not been reported. We aimed to assess these factors. MethodsThis cross-sectional descriptive study was done in July, 2015. We administered a structured questionnaire to all patients who were diagnosed as having coeliac disease and were registered at Ard El Insan Association, a Palestinian charitable society that provides preventive and therapeutic services, family guidance, and health education to patients with nutritional diseases through clinics in the Gaza Strip. The questionnaire focused on the presenting symptoms, coexisting diseases, and the diet of each patient. Patients provided verbal consent for their data to be used in this study. We used SPSS Statistics version 23.0 for data management and descriptive statistical analysis. Findings55 patients aged 2–40 years were included in our study with a mean age of 20 (SD 11·7) years. 23 (42%) patients were younger than 18 years. 19 (34%) were boys or men and 36 (66%) were girls or women. The mean age of the first presentation with gastrointestinal complaints was 10 (SD 9) years, whereas the mean age of diagnosis was 16 (10) years. The most common presenting symptoms were anorexia (76%), abdominal distention (73%), and diarrhoea (73%). In addition, 78% of patients had poor weight gain, 71% had iron deficiency anaemia, 40% had arthralgia, and 33% had short stature and had experienced delayed puberty. 18 (33%) patients had a family history of coeliac disease. The proportion of patients who had other autoimmune diseases was 23%, with the most frequent being urticaria (9%), type 1 diabetes (4%), asthma (4%), alopecia (4%), and thyroiditis (2%). 44 (80%) patients adhered to therapeutic diets. The mean level of haemoglobin at diagnosis was 10·0 (SD 1·7) g/L, but at the latest visit was 11·6 (1·2) g/L. InterpretationOur findings suggest that coeliac disease occurs notably more frequently in girls and women than in boys and men in Gaza Strip and that diagnosis can take 5–6 years after the first gastrointestinal complaint. On the other hand, a substantial number of patients presented with non-gastrointestinal symptoms. Therefore, an awareness of the broad spectrum of presentations of coeliac disease is necessary to avoid delays in diagnosis. FundingNone.

Physical growth and social prognosis of esophageal atresia after 15 years of age

BackgroundThe mortality rate of esophageal atresia (EA) has significantly improved, but late complications remain problematic. We evaluated the physical growth, late complications, and social prognosis of postoperative patients with EA who have reached 15 years of age.MethodsEA patients who were treated at our institution from 1984 to 2003 were enrolled. The follow-up, physical growth at the last visit, late complications and treatment, academic status, and employment situation were evaluated.ResultsTwenty-nine EA patients were registered, and the 23 surviving patients (79.3%) were followed. Anthropometry at the latest visit tended to be below the standard values. Fundoplication was performed in 6 (26.1%) of 9 (39.1%) patients with gastroesophageal reflux. Anastomotic stenosis was found in 12 patients (52.2%), and 2 (8.7%) were treated with re-anastomosis. Thirteen patients were attending a regular school, and one was attending a school for disabled children. Four had jobs from 18 years of age. Follow-up was aborted during early childhood in nine patients.ConclusionsThe physical size of EA was smaller than in the healthy population of the same age. Late complications had not affected the physical growth but were sometimes recognized in adolescence. The social prognosis of the patients was largely favorable.

Changes in corneal thickness, corneal volume, and densitometry after long-term orthokeratology wear

PurposeTo investigate changes in the corneal volume, corneal densitometry and pachymetry of young myopes wearing over-night orthokeratology (OK) contact lenses. MethodsThe medical records of 28 right eyes of young myopes wearing OK between 2013 and 2018 were reviewed retrospectively. The baseline refractive error, best corrected visual acuities at baseline and uncorrected visual acuity at the most recent visit were recorded. Corneal volume of the central 10 mm cornea; densitometry at central, nasal and temporal cornea; and pachymetry along the horizontal and vertical meridians were collected from the Pentacam® HR at baseline, after one night of lens wear, and at the latest visit. ResultsThe mean age of subjects was 12.03 ± 3.80 years at the time of OK lens fitting and wore OK overnight for a mean duration of 666 days (range 206–1736 days). The baseline spherical equivalent refractive error was −3.03 ± 1.56 D (range −1.00 to −6.00 D). The corneal volume increased significantly after OK wear (p = 0.001). Corneal densitometry increased after OK wear, but the change did not reach statistical significance (p = 0.113). Pachymetry in the central cornea did not change significantly across all visits (p > 0.05) but increased significantly in the mid-peripheral regions of the cornea. Baseline refractive error was not found to be correlated with the changes in corneal volume, corneal densitometry, or pachymetry. ConclusionThe increase in corneal volume and densitometry and no significant change in the central corneal thickness may indicate the presence of corneal oedema from long-term OK wear. The baseline refraction was not correlated with the changes in corneal volume, densitometry or pachymetry.

Functional outcome of unstable pelvic fractures treated in a level III hospital in a developing country: a 10-year prospective observational study

BackgroundUnstable pelvic fractures are severe and life-threatening injuries with high morbi-mortality rates. Management of these fractures is a major challenge in orthopaedic practice in limited resource communities. The aim of this study is to evaluate the functional outcome of unstable pelvic fractures managed in a hospital with limited diagnostic and therapeutic facilities.MethodologyThis was a hospital-based prospective observational study carried out from 1st of January 2009 to 31st of December 2018 at the Limbe Regional Hospital, a level III health institution in the South-West region of Cameroon.ResultsA total of 68 patients were included in the study. The ages ranged from 18 to 80 years with a mean age of 39 ± 5 years. The average follow-up duration at the latest visit was 36 months (range 3–84 months). There were 59 cases that were evaluated. The overall average Majeed score was fair. Poor outcomes were noted in patients aged 60 years and above, those with co-morbidities, and those managed conservatively.ConclusionAlthough the functional outcomes following unstable pelvic fractures have improved with modernised diagnostic and therapeutic modalities, it is not the case in poor resource settings where the lack of these modalities makes the management challenging, consequently affecting the functional outcome.

Assessment of Acute Rejection by Global Longitudinal Strain and Cardiac Biomarkers in Heart-Transplanted Patients.

AimsThe aim of this study was to evaluate left ventricular global longitudinal strain (LVGLS), N-terminal pro brain natriuretic peptide (Nt-ProBNP), and Troponin T as non-invasive markers for acute cellular rejection (ACR) diagnosis and severity assessment after heart transplantation (HTx).MethodsWe retrospectively included all HTx patients transplanted from 2013 to 2019. At each visit, the patients were subjected to endomyocardial biopsy (EMB), measurement of Nt-ProBNP and Troponin T, and protocoled echocardiography with assessment of LVGLS. Sudden drop in graft function (SDGF) was defined as a drop in LVGLS ≥-2% in combination with either an increase in Troponin T ≥20% or Nt-ProBNP ≥30% compared with levels at the latest visit.ResultsWe included 1,436 EMBs from 83 HTx patients. The biopsies were grouped as 0R (n = 857), 1R (n = 538), and ≥2R (n = 41). LVGLS was lower and Troponin T and Nt-ProBNP higher in the 2R group than in the 0R and 1R groups (LVGLS: -12.9 ± 3.8% versus -16.9 ± 3.1% and -16.1 ± 3.3%; Troponin T: 79 [33;230] ng/l versus 27 [13;77] ng/l and 27 [14;68] ng/l; Nt-ProBNP: 4,174 [1,095;9,510] ng/l versus 734 [309;2,210] ng/l and 725 [305;2,082], all p < 0.01). A SDGF was seen at 45 visits of which 19 had ≥2R ACR. EMBs showed ACR in 20 cases without SDGF. Finally, neither was SDGF seen nor did the EMB show rejection in 1,136 cases. Thus, the sensitivity of SDGF for ≥2R ACR detection was 49% (32–65) and specificity 98% (97–99). The positive predictive value (PPV) was 42% (31–55) and the negative predictive value (NPV) 98% (98–99). The diagnostic value improved in a sub-analysis excluding EMBs within 3 months after HTx, clinically interpreted false positive ≥2R ACR cases, and cases with ≥2R ACR who recently (<2 weeks) were treated with intravenous methylprednisolone due to ≥2R ACR (sensitivity 75% (48–93), specificity 97% (96–98), NPV 99% (99–100), and PPV 39% (27–52).ConclusionsPatients with ≥2R ACR have lower LVGLS and higher Troponin T and Nt-ProBNP than patients without 2R rejection. A non-invasive model combining changes in LVGLS and Troponin T or Nt-ProBNP showed excellent negative predictive value and moderate sensitivity and may be used as a gatekeeper to invasive biopsies after HTx.

Nonadherence to follow-up recommendations is common for dogs and cats undergoing orthopedic surgery.

To evaluate owner adherence to recommendations for follow-up examination of dogs and cats following orthopedic procedures and identify factors associated with adherence versus nonadherence. Medical records of 485 dogs and cats that underwent orthopedic surgery. Cases were categorized as urgent or elective. Information obtained from the medical records consisted of species, age, body weight, proximity to the hospital, procedure cost, recommendations for coaptation, use of financial aid, and number of owners. Cases were considered adherent to follow-up recommendations if, at the latest visit or communication, no further visits were recommended. Cases were considered nonadherent if owners did not return for recommended follow-up visits. Overall adherence to follow-up recommendations was 65.8% (319/485). Elective cases were 1.6 times as likely to be adherent to follow-up recommendations as were urgent cases, dog cases were 2.4 times as likely to be adherent as were cat cases, and cases with multiple owners listed were 2.1 times as likely to be adherent as were cases with 1 owner listed. Distance from the hospital had a statistically significant association with adherence, but the effect was not clinically important. Age, weight, coaptation, procedure cost, and use of financial aid were not significantly associated with adherence. The percentage of dogs and cats lost to follow-up following orthopedic surgery at an academic veterinary teaching hospital was substantial (166/485 [34.2%]). Efforts to improve follow-up adherence are especially indicated for animals undergoing urgent procedures, animals with single owners, and cats.

Characteristics and long-term outcomes of childhood glaucoma: a retrospective-cohort study

Purpose: To evaluate the clinical characteristics and treatment outcomes of patients with childhood glaucoma. Methods: We retrospectively reviewed the data of patients with childhood glaucoma who visited the glaucoma clinics at the Queen Sirikit National Institute of Child Health and the King Chulalongkorn Memorial Hospital between January 2008 and January 2018. The diagnosis was based on the Childhood Glaucoma Research Network classification. We recorded their clinical characteristics and requirement of any glaucoma interventions. Results: A total of 691 eyes from 423 patients were included in this study. The patients predominantly comprised boys. The average follow-up duration was 71.3±63.8 months. The mean age at presentation was 3.9±4.4 years. Most patients presented with a high initial intraocular pressure (IOP). The average intial IOP of all patients was 28.5±11.2 mmHg. Glaucoma associated with non-acquired ocular anomalies (22.9%) was the most common subtype, followed by primary congenital glaucoma (20.8%). We recorded a family history of glaucoma in 6.4% of patients of the 234 patients with an available family history. Most patients had bilateral glaucoma (63.4%) and required at least one intervention (51.5%). The average IOP at the latest follow-up visit was 19.1±10.8 mmHg. All glaucoma types had significantly lower IOP, compared to that at their baselines (all p&lt;0.001). Moreover, most patients had an unfavourable visual acuity (49.5%) at their latest visit. Conclusions: Secondary glaucoma associated with non-acquired ocular anomalies is the most common subtype of glaucoma. The majority of patients had unfavourable visual outcomes. These real-world findings are fundamental to acquire a better understanding of childhood glaucoma.

Characteristics and long-term outcomes of childhood glaucoma: a retrospective-cohort study

Purpose: To evaluate the clinical characteristics and treatment outcomes of patients with childhood glaucoma. Methods: We retrospectively reviewed the data of patients with childhood glaucoma who visited the glaucoma clinics at the Queen Sirikit National Institute of Child Health and the King Chulalongkorn Memorial Hospital between January 2008 and January 2018. The diagnosis was based on the Childhood Glaucoma Research Network classification. We recorded their clinical characteristics and requirement of any glaucoma interventions. Results: A total of 691 eyes from 423 patients were included in this study. The patients predominantly comprised boys. The average follow-up duration was 71.3±63.8 months. The mean age at presentation was 3.9±4.4 years. Most patients presented with a high initial intraocular pressure (IOP). The average intial IOP of all patients was 28.5±11.2 mmHg. Glaucoma associated with non-acquired ocular anomalies (22.9%) was the most common subtype, followed by primary congenital glaucoma (20.8%). We recorded a family history of glaucoma in 6.4% of patients of the 234 patients with an available family history. Most patients had bilateral glaucoma (63.4%) and required at least one intervention (51.5%). The average IOP at the latest follow-up visit was 19.1±10.8 mmHg. All glaucoma types had significantly lower IOP, compared to that at their baselines (all p<0.001). Moreover, most patients had an unfavourable visual acuity (49.5%) at their latest visit. Conclusions: Secondary glaucoma associated with non-acquired ocular anomalies is the most common subtype of glaucoma. The majority of patients had unfavourable visual outcomes. These real-world findings are fundamental to acquire a better understanding of childhood glaucoma.

The new 2019-EULAR/ACR classification criteria specific domains at diagnosis can predict damage accrual in 670 childhood-onset systemic lupus erythematosus patients.

To evaluate if the 2019-European League Against Rheumatism/American College of Rheumatology (EULAR/ACR) criteria at diagnosis of childhood-onset systemic lupus erythematosus (cSLE) are associated with higher rates of early damage scored by Systemic Lupus International Collaborating Clinics/American College of Rheumatology (SLICC/ACR) Damage Index (SDI). This retrospective multicenter study included 670 cSLE patients with ≤5 years of disease duration. All patients fulfilled both 2019-EULAR/ACR and 1997-ACR classification criteria. Total score of 2019-EULAR/ACR criteria and each of its specific domains were assessed at diagnosis as predictors of damage accrual at the last visit, according to the presence of any organ damage (defined by SDI ≥ 1). Median disease duration was 2.8 (IQR 1.8-3.8) years and 200 (29.9%) patients had at least one organ damage (SDI ≥ 1). The most frequent domains were neuropsychiatric (12%), renal (7%), and musculoskeletal (6%). There was a higher frequency of renal (58% vs 43%, p = 0.0004) and neuropsychiatric domain (21% vs 7%, p < 0.0001) of 2019-EULAR/ACR criteria in patients with damage (SDI ≥ 1) compared to those without damage (SDI = 0). Patients scoring renal or neuropsychiatric domains of the 2019-EULAR/ACR criteria at diagnosis were associated with renal damage (odds ratio 9.701, 95% confidence interval 3.773-24.941, p < 0.001) or neuropsychiatric damage (OR 9.480, 95% CI 5.481-16.399, p<0.0001) at latest visit, respectively. cSLE patients with positive anti-dsDNA at diagnosis were also associated with renal damage by the latest visit (OR 2.438, 95% CI 1.114-5.3381, p = 0.021). Constitutional, hematologic, mucocutaneous, serosal, and musculoskeletal domains and specific criteria as well as other immunologic criteria were not associated with damage accrual. Median of SLEDAI-2K was significantly higher in patients with global damage (19.5 (2-51) vs 14 (0-51), p<0.001). 2019-EULAR/ACR score >25 was associated with more overall (SDI ≥ 1) (38% vs 25%, p = 0.0002) and renal damage (11% vs 5%, p = 0.023). The 2019-EULAR/ACR criteria at diagnosis were associated with a higher rate of early damage in cSLE patients, especially for renal and neuropsychiatric damage. Of note, damage was particularly associated with high disease activity at diagnosis and 2019-EULAR/ACR score >25.

Challenging the Minimum Effective Antipsychotic Dose During Maintenance: Implications From 10-Year Follow-Up of First Episode Psychosis.

Background: Contradictory messages regarding the necessity of long-term antipsychotic treatment after first episode psychosis arouse deliberations in clinical practice. We explored if there is an alternative beyond the dichotomy of maintenance treatment and discontinuation of medications.Methods: We conducted a retrospective observational study by reviewing medical records at the study hospital of a cohort of patients since their participation in an early psychosis study starting from 2006, with special interests in patients able to maintain good functioning under treatment with a low antipsychotic dose.Results: Of the 81 patients with first-episode psychosis, 55 patients (67.9%) had follow-up information for longer than 5 years. The majority (n = 46, 83.6%) had non-affective psychosis, 20 patients (36.4%) had full-time employment/education by the time of their latest visit; among them, 15 patients received dosage of antipsychotics no more than the minimum effective dose [chlorpromazine equivalent (CPZE) dose, 200 mg/day]. Besides, 10 of 55 patients (18.2%) only received very low dose antipsychotics (CPZE < 50 mg/day) during maintenance, which was significantly correlated to good functioning. Being male, having a history of hospitalization, and being on clozapine therapy were correlated to poorer functioning. Antipsychotic-free status was achieved only in two non-psychotic patients.Conclusions: A substantial proportion of patients could achieve good functioning under low-dose antipsychotic maintenance after first-episode psychosis, even if they could not completely withdraw antipsychotics in the long term. Optimizing the balance between preventing relapse and preserving functioning by fine-tuning antipsychotic dosage during maintenance is a challenge warranting more clinical attention.

Na\xefve CD4+ cell counts significantly decay and high HIV RNA levels contribute to immunological progression in long-term non-progressors infected with HIV by blood products: a cohort study

BackgroundSome long-term non-progressors (LTNPs) have decreasing CD4+ T cell counts and progress to AIDS. Exploring which subsets of CD4+ T cell decreasing and the determinants associated with the decay in these patients will improve disease progression surveillance and provide further understanding of HIV pathogenesis.MethodsTwenty-five LTNPs infected with HIV by blood products were classified as decreased (DG) if their CD4+ cell count dropped to < 400 cells/μL during follow-up or as non-decreased (non-DG) if their CD4+ cell count was ≥400 cells/μL. Laboratory and clinical assessments were conducted at 6 consecutive visits to identify DG characteristics.ResultsThe LTNPs were infected with HIV for 12 (IQR: 11.5–14) years, and 23 were classified as the B′ subtype. Six individuals lost LTNP status 14.5 (IQR: 12.5–17.5) years after infection (DG), and the CD4+ T cell count decreased to 237 (IQR: 213–320) cells/μL at the latest visit. The naïve CD4+ T cell count decrease was greater than that of memory CD4+ T cells [− 128 (IQR: − 196, − 107) vs − 64 (IQR: − 182, − 25) cells/μL)]. Nineteen individuals retained LTNP status (non-DG). At enrolment, the viral load (VL) level (p = 0.03) and CD8+CD38+ percentage (p = 0.03) were higher in DG than non-DG individuals. During follow-up, viral load and CD8+CD38+ percentage were significantly increased and negatively associated with CD4+ cell count [(r = − 0.529, p = 0.008), (r = − 0.476, p = 0.019), respectively]. However, the CD8+CD28+ percentage and B cell count dropped in DG and were positively correlated with CD4+ T cell count [(r = 0.448, p = 0.028), (r = 0.785, p < 0.001)].ConclusionImmunological progression was mainly characterized by the decrease of naïve CD4+ T cell in LTNPs infected with HIV by blood products and it may be associated with high HIV RNA levels.

Long-term Outcomes of Cataract Surgery in Patients With Chronic Ocular Graft-Versus-Host Disease.

The aim of this study was to evaluate the outcomes of cataract surgery in patients with chronic ocular graft-versus-host disease (GVHD). A retrospective review was performed on 77 eyes of 42 patients with chronic ocular GVHD that underwent cataract surgery between January 2014 and February 2020 in a tertiary institution. Posterior subcapsular cataract was seen in 53 (68.8%) of 77 eyes, with a mean preoperative corrected distance visual acuity (CDVA) of 0.61 ± 0.49 logarithm of the minimum angle of resolution (logMAR). Postoperatively, with a mean follow-up of 21 months, CDVA improved to 0.17 ± 0.22 logMAR at the latest visit (P < 0.0001). With latest available refraction, 42 (57.5%) of 73 eyes were within 0.5 diopters of target refraction, and 59 eyes (80.8%) were within 1.0 diopter. Postoperative complications included superficial punctate keratopathy within 1 month postoperatively (19 eyes, 24.7%), posterior capsular opacification requiring yttrium-aluminum-garnet (YAG) laser capsulotomy (36 eyes, 46.8%), corneal epithelial defect (7 eyes, 9.1%), filamentary keratopathy (5 eyes, 6.5%), cystoid macular edema (3 eyes, 3.9%), and infectious crystalline keratopathy (1 eye, 1.3%). Lower preoperative National Institutes of Health ocular GVHD severity scores were associated with a better postoperative CDVA (grade 1, 0.13 ± 0.16 logMAR; grade 2, 0.16 ± 0.23 logMAR; and grade 3, 0.36 ± 0.21 logMAR; P = 0.004). Cataract surgery improves visual acuity long term in most patients with chronic ocular GVHD. Close postoperative monitoring is important to detect ocular surface inflammation secondary to chronic ocular GVHD, particularly in severe ocular GVHD.

Treatment of bone and joint infections by ceftazidime/avibactam and ceftolozane/tazobactam: a cohort study

Ceftazidime/avibactam (C/A) and ceftolozane/tazobactam (C/T) are two novel antibacterials with known efficacy against Gram-negative bacteria (GNB). We aimed to describe the efficacy and safety of surgical management combined with C/A or C/T treatment for bone and joint infections (BJIs). We conducted an observational, bicentric study of patients treated with C/A or C/T for a BJI between May 2016 and June 2019. Failure was defined as the need for unplanned additional antibiotic treatment or orthopaedic surgery, or death due to the BJI up to the patient's latest visit. Overall, 15 patients were included. Nine patients were treated with C/A, mainly for polymicrobial BJI due to multidrug-resistant (MDR) bacteria (Enterobacteriaceae, n=7). Six patients were male, the median age was 66 years and the median Charlson comorbidity index (CCI) was 5. It was the first septic episode at the site in 3/9 patients. The cure rate was 7/9 (median follow-up, 272 days). Two patients showed C/A-related confusion. Five patients were treated with C/T for BJI involving MDR Pseudomonas aeruginosa. Four patients were male, the median age was 53 years and the median CCI was 2. All patients had previous septic episodes at the infection site. The cure rate was 3/5 (median follow-up, 350 days). One patient was successfully treated by C/T then C/A for multistage spondylodiscitis. In our experience, C/A and C/T are two effective and safe options, even as salvage treatment for BJI due to MDR-GNB despite the absence of label, however more data are warranted.

Automated detection of hippocampal sclerosis: Comparison of a composite MRI-based index with conventional MRI measures

PurposeThis study aims to compare the performance of an MRI-based composite index (HSI) with conventional MRI-based measures in hippocampal sclerosis (HS) detection and postoperative outcome estimation. MethodsSeventy-two temporal lobe epilepsy (TLE) patients with pathologically confirmed HS and fifteen TLE patients without HS were included retrospectively. The T1-weighted and FLAIR images of these patients were processed with AccuBrain to quantify the hippocampal volume (HV) and the hippocampal FLAIR signal. The HSI index that considered both HV and hippocampal FLAIR signal was also calculated. Two experienced neuropathologists rated the HS severity with the resected tissue and reached an agreement for all cases. The asymmetry indices of the MRI measures were used to lateralize the sclerotic side, and the original MRI measures were applied to detect HS vs. normal hippocampi. Operating characteristic curve (ROC) analyses were performed for these predictions. We also investigated the sensitivity of the ipsilateral MRI measures in characterizing the pathological severity of HS and the associations of the MRI measures with postoperative outcomes (Engel class categories). ResultsWith the optimal cutoffs, the asymmetry indices of HSI and HV both achieved excellent performance in differentiating left vs. right HS (accuracy = 100 %), and the absolute value of the asymmetry index of HSI performed best in differentiating unilateral vs. bilateral HS (accuracy = 91.7 %). Regarding the detection of HS, HSI performed better in sensitivity (94.4 % vs. 87.5 %) while HV performed better in specificity (93.6 % vs. 89.4 %) when the contralateral site of unilateral HS and both sides of non-HS patients were considered as the normal reference, and HSI performed even better than HV when only both sides of non-HS patients were considered as the normal reference (AUC: 0.956 vs. 0.934, p = 0.038). The ipsilateral HSI presented the strongest association with the pathological rating of HS severity (r = 0.405, p < 0.001). None of the ipsilateral or contralateral MRI measures was associated with the postoperative outcomes. Among the asymmetry indices, only the absolute value of the asymmetry index of HV presented a significant association with the Engel classifications for the Year 2∼3 visit (r = -0.466, p = 0.004) or the latest visit with >1 year follow-up (r = -0.374, p = 0.003) while controlling for disease duration and follow-up duration. ConclusionThe HSI index and HV presented comparable good performance in HS detection, and HSI may have better sensitivity than HV in differentiating pathological HS severity. Higher magnitude of HV dissymmetry may indicate better post-surgical outcomes for HS patients.

Thirty percent of children and young adults with familial hypercholesterolemia treated with statins have adherence issues

ObjectiveTo assess adherence to lipid lowering therapy (LLT), reasons for poor adherence, and achievement of LDL-C treatment goals in children and young adults with familial hypercholesterolemia (FH). MethodsRetrospective review of the medical records of 438 children that started follow-up at the Lipid Clinic, Oslo University hospital, between 1990 and 2010, and followed-up to the end of July 2019. Based on information on adherence to the LLT at the latest visit, patients were assigned to “good adherence” or “poor adherence” groups. Reasons for poor adherence were categorized as: “lack of motivation”, “ran out of drugs”, or “side effects”. ResultsThree hundred and seventy-one patients were included. Mean (SD) age and follow-up time at the latest visit was 24.0 (7.1) and 12.9 (6.7) years; 260 patients (70%, 95% CI: 65–74%) had “good adherence” and 111 (30%, 95% CI: 25–35%) had “poor adherence”. “Lack of motivation” was the most common reason for poor adherence (n = 85, 23%). In patients with good adherence, compared to patients with poor adherence, age at latest visit (24.6 versus 22.0 years; p = 0.001), years of follow-up (13.5 versus 11.4 years; p = 0.003), and number of visits (8.1 versus 6.5 visits; p<0.001) were significantly higher, whereas LDL-C at the latest visit was lower, (3.1 (0.8) versus 5.3 (1.6) mmol/L; p<0.001) and percentage of patients reaching LDL-C treatment goal was higher, (34.5% versus 2.7%; p<0.001). Gender, BMI, age at first visit and premature cardiovascular disease in first degree relatives were not significantly associated with adherence. ConclusionThirty percent of young patients with FH had poor adherence to LLT, with lack of motivation as the main reason. Higher age, more visits and more years of follow-up were associated with good adherence.

Characteristics and long-term outcomes of childhood glaucoma: a retrospective-cohort study.

Purpose: To evaluate the clinical characteristics and treatment outcomes of patients with childhood glaucoma. Methods: We retrospectively reviewed the data of patients with childhood glaucoma who visited the glaucoma clinics at the Queen Sirikit National Institute of Child Health and the King Chulalongkorn Memorial Hospital between January 2008 and January 2018. The diagnosis was based on the Childhood Glaucoma Research Network classification. We recorded their clinical characteristics and requirement of any glaucoma interventions. Results: A total of 691 eyes from 423 patients were included in this study. The patients predominantly comprised boys. The average follow-up duration was 71.3±63.8 months. The mean age at presentation was 3.9±4.4 years. Most patients presented with a high initial intraocular pressure (IOP) of 28.5±11.2 mmHg. Glaucoma associated with non-acquired ocular anomalies (22.9%) was the most common subtype, followed by primary congenital glaucoma (20.8%). We recorded a family history of glaucoma in 6.4% of patients. Most patients had bilateral glaucoma (63.4%) and required at least one intervention (51.5%). The average IOP at the latest follow-up visit was 19.1±10.8 mmHg. All glaucoma types had significantly lower IOP, compared to that at their baselines (all p<0.001). Moreover, most patients had an unfavourable visual acuity (49.5%) at their latest visit. Conclusions: Secondary glaucoma associated with non-acquired ocular anomalies is the most common subtype of glaucoma. All subtypes, including primary glaucoma, were sporadic. The majority of patients had unfavourable visual outcomes. These real-world findings are fundamental to acquire a better understanding of childhood glaucoma.

Modified tunneling technique for root coverage of anterior mandible using minimal soft tissue harvesting and volume-stable collagen matrix: a retrospective study.

PurposeIn this study, we aimed to evaluate the clinical validity of the modified tunneling technique using minimal soft tissue harvesting and volume-stable collagen matrix in the anterior mandible.MethodsIn total, 27 anterior mandibular teeth and palatal donor sites in 17 patients with ≥1 mm of gingival recession (GR) were analyzed before and after root coverage. For the recipient sites, vertical vestibular incisions were made in the interdental area and a subperiosteal tunnel was created with an elevator. After both sides of the marginal gingiva were tied to one another, a prepared connective tissue graft and volume-stable collagen matrix were inserted through the vestibular vertical incision and were fixed with resorbable suture material. The root coverage results of the recipient site were measured at baseline (T0), 3 weeks (T3), 12 weeks (T12), and the latest visit (Tl). For palatal donor sites, a free gingival graft from a pre-decided area avoiding the main trunk of the greater palatine artery was harvested using a prefabricated surgical template at a depth of 2 mm after de-epithelization using a rotating bur. In each patient, the clinical and volumetric changes at the donor sites between T0 and T3 were measured.ResultsDuring an average follow-up of 14.5 months, teeth with denuded root lengths of 1–3 mm (n=12), 3–6 mm (n=11), and >6 mm (n=2) achieved root coverage of 97.01%±7.65%, 86.70%±5.66%, and 82.53%±1.39%, respectively. Miller classification I (n=12), II (n=10), and III (n=3) teeth showed mean coverage rates of 97.01%±7.65%, 86.91%±5.90%, and 83.19%±1.62%, respectively. At the donor sites, an average defect depth of 1.41 mm (70.5%) recovered in 3 weeks, and the wounds were epithelized completely in all cases.ConclusionsThe modified tunneling technique in this study is a promising treatment modality for overcoming GR in the anterior mandible.

Clinical and Genetic Findings in CTNNA1-Associated Macular Pattern Dystrophy

Clinical and Genetic Findings in CTNNA1-Associated Macular Pattern Dystrophy

The early- to medium-term results of a hemispherical, porous coated acetabular shell with multiple different bearing combinations are excellent with the exception of metal-on-metal.

This study prospectively reports survivorship and radiographic and clinical outcomes following primary elective total hip arthroplasty (THA) using a novel single hemispherical, porous-coated acetabular cup with five different bearing combinations and a minimum of fiveyear follow-up. Continuing post-market release monitoring of this cup, we prospectively enrolled 108 patients (121 THA) between 2009 and 2015. We followed this cohort by examining survivorship, in addition to clinical and radiological outcomes for metal-on-metal (MoM) compared with non-MoM bearing combinations (ceramic-on-ceramic, oxinium-on-polyethylene, ceramic-on-metal, and metal-on-polyethylene). All 108 (121 hips) patients were followed up. Average age at time of surgery was 45.1years (range 19 to 71years) of which 42.1% were males. A total of seven (5.8%) cups were revised, all of which were MoM. No osteolysis was observed in any of the patients at the latest visit with a mean follow-up of 9.1 ± 1.7years (range 4.4-10.7years). With MoM excluded, survivorship of the cup at fiveyears is 97.8%. Survivorship for MoM implants was 90.0%. Validated hip scores showed significant improvements for all bearing types and no significant difference between groups at latest follow-up (p = 0.614). There was no cup migration with any bearing surface. This cup showed excellent survivorship at fiveyear follow-up, except for patients receiving a MoM articulation. While there were concerns over the early survivorship of this cup, our cohort and joint registry data confirm excellent outcomes.