bluuebunny/crossref_metadata_2025_split huggingface dataset:
Oral Dexamethasone versus Prednisolone for Management of Children with West Syndrome
Authors: Deswal Monica , Lekhwani Seema , Vaswani Narain D. , Bala Kiran , Kaushik Jaya S. 
Published: August 2023

Objective:
To compare the efficacy of oral dexamethasone and prednisolone in the treatment of newly diagnosed children aged 3–36 months of West syndrome.

Methods:
An open-labeled, randomized controlled clinical trial with parallel group assignment was conducted among children aged 3–36 months with newly diagnosed West syndrome. They were randomized to receive either oral dexamethasone (0.6 mg/kg/day QID) (n = 20) or oral prednisolone (4 mg/kg/day BD) (n = 20). Proportion of children who achieved spasm freedom at 2 weeks was the primary outcome. Secondary outcome measures were proportion of children who achieved electroclinical resolution, greater than 50% reduction in spasms frequency, time to cessation of spasms, and adverse effects at 2 weeks.

Results:
The efficacy of oral dexamethasone was comparable to oral prednisolone in terms of proportion of children who achieved spasms cessation (13 [65%] vs. 8 [40%]; P = 0.21), electroclinical remission (13 [65%] vs. 8 [40%] P = 0.21), greater than 50% reduction of spasms (3 [15%] vs. 7 [35%] P = 0.65), and time to cessation of spasms (5.31 [2.81] vs. 4.37 [1.41] P = 0.39). Adverse effect profile was also comparable with irritability (18 [90%] vs. 12 [60%] P = 0.06] being most common.

Conclusion:
There was no difference in electroclinical remission at 2 weeks between oral dexamethasone and prednisolone in children with infantile spasms in this small pilot trial. Further evaluation is suggested with an adequately powered study and long-term follow-up.
Management of Children and Adolescents with Wilson Disease and Neurological Worsening Following D–Penicillamine Therapy
Authors: Kumar Madhan , Murugan T. P , Lionel Arul P. , Thomas Maya M. , Mannam Pavithra , Yoganathan Sangeetha 
Published: August 2023

Background:
Most centers in developing countries prefer chelation therapy with D-penicillamine for the management of Wilson's disease (WD) because of its easy availability and affordability. Neurological worsening following treatment with D-penicillamine is not uncommon. However, there is a paucity of Indian data on the incidence of neurological worsening in children and adolescents with WD following chelation therapy. Our study objectives were to identify the prevalence of neurological worsening in children and adolescents with WD following chelation with D–penicillamine therapy and to describe the management options and outcomes in these patients.

Materials and Methods:
In this retrospective chart review, children and adolescents with an established diagnosis of WD from 2010 to 2020 were identified from the hospital electronic database. Among these patients, data of children and adolescents with neurological worsening following D–penicillamine therapy were extracted and analyzed.

Results:
Neurological worsening was observed in 27/122 (22.1%) children and adolescents with WD on chelation therapy with D-penicillamine. Fifteen patients with neurological worsening following D-penicillamine therapy were managed with zinc monotherapy. Four patients were managed with a combination therapy of zinc and trientine. Five patients were treated with trientine monotherapy. Re-challenging with D-penicillamine at a lower dose followed by a slow dose escalation was attempted in three patients. Gradual clinical and functional status improvement was observed in 24 cases while one patient succumbed to pneumonia.

Conclusion:
Children and adolescents with WD who had neurological worsening on D-penicillamine therapy may be managed with trientine. Zinc monotherapy with copper restricted diet was also found to be effective in non-affordable patients.
P-tau231 as a Diagnostic Biomarker for Alzheimer's Disease and Mild Cognitive Impairment
Authors: Nabizadeh Fardin , Salehi Negin , Ramezannezhad Elham , Sadeghmousavi Shaghayegh , Khalili Elham 
Published: August 2023

Objective:
Some previous studies have shown that cerebrospinal fluid (CSF) levels of p-tau231 were significantly higher in patients with Alzheimer's disease (AD) compared to that in patients with mild cognitive impairment (MCI) and normal control (NC), whereas some other studies did not. Due to contradictory results, we aimed to conduct a systematic review and meta-analysis study on previous investigations to examine the potential role of CSF p-tau231 as a biomarker of AD and MCI.

Method:
PubMed, Scopus, and Web of Science were searched in March 2021 for studies on the CSF level of p-tau231 in AD, MCI, and NC. The statistical analysis was performed via standardized mean difference (SMD) methodology with a 95% confidence interval.

Results:
A total of 10 studies including 1141 subjects were included. The present study showed that CSF level of p-tau231 was significantly higher in AD patients compared to that in MCI patients (SMD = 160.94 [11.11, 310.78], P = 0.04) and NC patients (SMD = 436.21 [164.88, 707.54], P < 0.00). Moreover, comparison of MCI and NC showed a significantly higher level of CSF p-tau231 in MCI compared to NC (SMD = 341.44 [59.73, 623.14], P = 0.02).

Conclusion:
P-tau231 showed to be a valuable biomarker of discrimination AD, MCI, and NC based on our findings. This meta-analysis showed that the CSF p-tau231 can reliably differentiate AD patients from MCI and NC patients. Furthermore, based on our findings the level of CSF p-tau231 was significantly higher in MCI compared to NC. Therefore, p-tau231 can be added to the list of potential biomarkers for the diagnosis of AD and MCI in further studies. However, further investigations are needed to confirm our findings.
Medication-Adherence and Management of Risk Factors for Secondary Prevention of Stroke Using Smartphone-Based Application
Authors: Babu Veena , Sylaja P N , Soman Biju , Varma Ravi Prasad , Manju M S , Geethu G L , Kumar Suresh B 
Published: August 2023

Background:
In LMICs, the medication adherence and risk factor control are suboptimal in the post-stroke follow-up period. With shortage of physicians, smartphone-based interventions can help stroke survivors in secondary stroke prevention.

Objectives:
We aim to validate a digital innovative technology-based intervention to improve the awareness, medication adherence, control of risk factors through timely intervention of physician among the stroke survivors.

Methods:
MAMOR is a smartphone-based application to improve the stroke awareness by heath education materials, reminders to timely adherence of medication, alerts on control of risk factors, video files, and timely physician intervention. The study will involve development of the app using contextual research (Delphi qualitative method) followed by a randomized, single center, double arm-controlled trial with 1:1 assignment. The app will be evaluated over a period of 6 months with a target to enroll 192 participants. Process evaluation will be conducted. The sample size was calculated as 192, considering medication adherence of 43.8%, 20% increase in medication adherence by app, power of 80%, and 10% loss to follow-up.

Results:
The primary outcome will be medication adherence, changes in the lifestyle and behavioral and control of vascular risk factors. The secondary outcome will include vascular events and functional outcome.

Conclusion:
This study will be one among the few studies for secondary prevention of stroke through digital technology innovation in LMICs with resource constraints. The evidences generated from this study will provide translational evidence for other similar settings for stroke survivors.
Comparison of C-MACTM Videolaryngoscope with Macintosh Laryngoscope in Anticipated Difficult Airway
Authors: Ramamoorthy Srivats V , Paneerselvam Sakthirajan , Elakkumanan Lenin Babu , Rudingwa Priya , Govindaraj Kirthiha 
Published: August 2023

Background:
Failure to intubate is one of the dreaded outcomes of airway management. Videolaryngoscopes have the potential to increase intubation success by overcoming the shortcomings of direct laryngoscopy. This study aimed to compare the first-attempt intubation success rate of the C-MACTM videolaryngoscope and Macintosh laryngoscope in patients with anticipated difficult airways.

Methods:
Eighty adult patients with multiple predictors of difficult airway scheduled for surgery under general anaesthesia were randomised either into Group C (first using the C-MACTM videolaryngoscope) or Group D (first using the Macintosh laryngoscope). Laryngoscopy was done sequentially with both laryngoscopes in a patient based on the randomisation. The primary outcome was the first-attempt intubation success rate. Intubation time, Cormack–Lehane view, use of airway adjuncts and the need for external laryngeal manipulations constituted the secondary outcomes.

Results:
The first-attempt intubation success rate was 100% in both groups with the P value 1.0 and the intubation time was 26 s in Group C and 24.4 s in Group D with P = 0.17. The C-MACTM videolaryngoscope provided better grades of laryngoscopic view than direct laryngoscopy (P = 0.02). Tracheal tube introducer-assisted intubation (P = 0.01) and requirement of laryngeal manipulation to aid intubation (P = 0.01) were higher with the Macintosh laryngoscope.

Conclusion:
The Macintosh laryngoscope was comparable to the C-MACTM videolaryngoscope in terms of intubation success, intubation time and complications. However, an increased requirement for external laryngeal manipulation, lifting force and the use of an introducer for intubation were noted in the Macintosh group.
Assessing the bleeding severity in patients with type I von Willebrand using the International Society on Thrombosis and Hemostasis-Bleeding Assessment Tool (ISTH-BAT) questionnaire
Authors: Hafez Rania , Hennes Shery N , Abdelaal Azza , Tawfik Nabawia M , Hussein Safinaz 
Published: August 2023

Abstract

Background
In the recent decade, bleeding evaluation methods have evolved to standardize the severity of bleeding symptoms in a uniform manner. In 2010, the International Society on Thrombosis and Hemostasis-Bleeding Assessment Tool (ISTH-BAT) was developed and validated.

Aim and objectives
The aim of the study was to evaluate bleeding score (BS) in Egyptian patients with von Willebrand disease (VWD) type I and correlate it with laboratory parameters.

Patients and methods
This cross-sectional cohort study was conducted at Assiut University Hospital. It included 100 participants that were divided into 49 patients with type I VWD and 51 healthy participants with no known problem with bleeding or bruising. The duration of the study was ~12 months.

Results
Study revealed that there was significant relation between BS severity and laboratory parameters such as level of von Willebrand factor: Ag (P=0.02) while there was no significant relation between BS and sex (P=0.390). By using receiver operating characteristic curve analysis, ISTH-BAT BS can detect type I VWD patients in total with excellent accuracy (98%), area under the curve was 0.989 with sensitivity and specificity of 96 and 100%, respectively (P<0.001).

Conclusion
The ISTH-BAT BS is designed to reflect the severity of bleeding. Our results demonstrate the inherent variability of this bleeding pattern. We also found that the ISTH-BAT BS has high sensitivity and specificity to detect type I VWD patients in total with excellent accuracy.
Predictive factors of response to eltrombopag and romiplostim in Egyptian immune thrombocytopenia patients: a single center experience
Authors: El Demerdash Doaa , Mohamady Nagham , Abdelghany Wafaa , Youssef Heba 
Published: August 2023

Abstract

Background
Thrombopoietin receptor agonists (TPO-RA) are a well-established treatment in patients with immune thrombocytopenia (ITP). Predictors of response to some lines of treatment in ITP have been reported; yet, to date there are no predictors of response to TPO-RA were identified. We aimed to identify predictive factors of response to TPO-RA in adult ITP patients to avoid unwanted adverse effects and to individualize the treatment.

Patients and methods
We investigated demographic features, clinical-laboratory data as well as previous lines of treatment in 48 adult ITP patients who received TPO-RA for at least 3 months duration to detect reliable predictive factors of response to TPO-RA, in addition, health-related quality of life and fatigue burden was assessed in all studied ITP patients using 2 questionnaires which are 36-item short-form health survey and functional assessment of chronic illness therapy.

Results
The percentage of platelet change from days 0 to 28 of initiation of romiplostim can be a predictive factor of response to treatment with romiplostim (P=0.008) but none of the other studied factors has influenced response to TPO-RA. Both 36-item short-form health survey domains, as well as functional assessment of chronic illness therapy questionnaires had no statistically significant difference between the romiplostim and eltrombopag groups.

Conclusion
Slow or minimal change of platelet count during the first month of therapy with romiplostim could be used as a predictive factor of no response to romiplostim in ITP patients; In addition, none of the demographic features, initial clinical-laboratory features, previous lines of treatment with splenectomy or rituximab, or even the number of previous lines of therapy have influenced response to TPO-RA.
Prognostic significance of activin A in adult Egyptian patients with acute lymphoblastic leukemia
Authors: Azzazi Mohamed O , Abd-Allah Hegab Hany M , El-Ghammaz Amro M S , Shawky Verna A R , Hafez Heba M S , Helal Reham A A 
Published: August 2023

Abstract

Background
Activin A belongs to the transforming growth factor-beta superfamily of cytokines that exert a plethora of biological functions, including developmental differentiation, sex determination, control of cellular proliferation, migration, and immune responses. Activins are dimeric glycoproteins that play a significant role in reproduction and in endocrine-active tumors, although inhibins and activins have primarily been described in human gonads and identified as modulators of follicle-stimulating hormone production of the pituitary gland, they have also been detected in several solid tumor types, including endocrine-responsive endometrial, ovarian, and breast carcinomas. Their differential expression has suggested their important role in malignant cell transformation, as well as possible roles in cancer differentiation, proliferation, and growth tumors.

Aim
The aim was to assess expression of activin A in the serum of adult patients with acute lymphoblastic leukemia (ALL) and its influence on remission and survival of ALL patients.

Patients and methods
Serum activin A was measured using enzyme-linked immunosorbent assay in 30 ALL patients recruited from Hematology and Bone Marrow Transplantation Unit, Ain Shams University Hospitals, and followed for 1 year in comparison with 15 healthy controls.

Results
Serum level of activin A was elevated in ALL patients in comparison with the control group with a statistically significant difference (P<0.001). A statistically significant negative correlation was detected between age of the patients and activing-A level (P=0.035). The comparison between different outcomes of the patients (remitted, relapsed, and died patients) above and below the mean level of activin A (265.667 ng/ml), was statistically significant (P<0.001). A statistically significant negative correlation was detected between activing-A level in ALL patients and overall survival (P<0.001), and by using the log-rank test, a statistically significant difference (P<0.001) was detected in ALL patients above and below the mean level of activin A. However, a statistically nonsignificant difference was detected between the mean activing-A level in Philadelphia chromosome-positive patients and Philadelphia chromosome-negative patients (P=0.839).

Conclusion
Activin A can be a useful poor prognostic biomarker in ALL patients, also, it can be used as a predictor for aggressiveness of the disease, resistance, and survival.
Characteristics of TP53 mutation in adult precursor B-cell acute lymphoblastic leukemia
Authors: Samy Heba , Fouad Dina A , Ali Basma S.M , Attia Hend 
Published: August 2023

Abstract

Objectives
TP53 is the most intensively studied gene in cancer. However, data on the frequency and prognostic significance of TP53 mutations in acute lymphoblastic leukemia (ALL) are still lacking. This study aimed to determine the characteristics of TP53 mutation, its correlation with clinical and laboratory parameters and other cytogenetic alterations, and their impact on patient outcome on day 21 after induction therapy.

Patients and methods
This is a prospective cohort clinical study that was conducted on 41 de-novo adult ALL patients, who presented to the Hematology/Oncology Unit of Ain-Shams University Hospitals, where all studied patients were subjected to the treatment regimen. TP53 mutation was investigated in 41 patient samples using the RT-PCR.

Results
TP53 mutation was detected in 19.5% of studied cases. A highly significant association was detected between 17P deletion and TP53 mutation (P<0.0001). A significant association was detected between TP53 mutation and abnormal karyotyping (P=0.032). The authors found a clear association between TP53 mutation and hypodiploidy (P=0.001) and MYC rearrangements (P=0.001). In contrast, TP53 mutation was clearly underrepresented in ALL patients with t(9;22)(q34;q11). A highly significant association between TP53 mutation and the poor outcome on day 21 (P=0.002) was observed. The patients with TP53 mutation revealed either failure of remission (50%) or incomplete remission (50%). Logistic regression analysis of factors influencing the patient outcome showed that advanced age (>34 years), high total leukocyte count (>40 × 109/l), and abnormal fluorescence in-situ hybridization and karyotyping results due to cytogenetic abnormalities are independent predictors of poor outcome with failure of induction of complete remission on day 21.

Conclusion
TP53 alterations strongly identify high-risk adult precursor B-ALL patients with poor outcome in this study; yet, this needs further investigation on a larger sample size with a longer follow-up. Investigations of TP53 mutation especially in adult B-cell ALL (accounting 75% of adult ALL) may help with the selection of patients in need of intensive therapeutic strategy or may help with designation of new innovative targeted therapies.
Nothing is impossible if you have the right tools and techniques to support
Authors: Paritekar Prajakta , Haldipurkar Suhas , Shetty Vijay 
Published: August 2023

Background:
Zonular weakness is a known complication that a surgeon can encounter during cataract surgery.

Purpose:
The purpose of this video is to make surgeons aware about the different tools and techniques available to handle zonulopathy.

Synopsis:
This video describes the various causes of zonular weakness and different tools available to manage zonular weakness intraoperatively. The surgical technique of using the tools is also demonstrated in the video.

Highlights:
Causes of zonular weakness and surgical techniques to handle it intraoperatively.

Video Link:
https://youtu.be/xoyOP6tiiZ4

Key words: Assi anchor, capsule hooks, CTR, CTS, zonular dialysis
An approach to reduce Descemet’s membrane scrolling: Relevance to Descemet’s membrane endothelial keratoplasty (DMEK)
Authors: Dua Harminder S , Freitas Rui , Sadek Youssef , Ting Darren SJ , Nubile Mario , Mohammed Imran , Said Dalia G 
Published: August 2023

Purpose:
We aimed to determine whether Descemet’s membrane (DM) scrolling occurs primarily along the vertical or horizontal axis and establish whether oval trephination along the axis of least scrolling can reduce the grade of the scroll.

Methods:
The longest limbus-to-limbus axis on 28 sclerocorneal discs was taken as the horizontal axis. The horizontal (n = 7) or (right angles to it) vertical (n = 6) axis was marked on DM before peeling it off. The direction and grade of scrolling was observed. Narrow strips (3–4 mm wide) were then cut along the two axes (n = 4 each) and the scrolling pattern was observed. Ellipses (7 × 9 mm) of DM were punched along the two axes (n = 6 each) and the scrolls graded. Immunofluorescent staining for elastin on horizontal and vertical tissue sections from three DM samples was performed. The intensity and thickness of elastin staining were measured.

Results:
Twenty-four (85.72%) DM samples showed scrolling along the horizontal axis, none showed scrolling along the vertical axis, and four (14.28%) samples showed a spiral scroll, regardless of which axis was marked (grade 3.7 and 3.6). Vertically oval discs showed significantly reduced scrolling (grade 1.2) compared to horizontally oval discs (grade 3.5). Narrow strips of DM showed a similar scrolling pattern. Immunohistology showed no difference in any of the parameters examined along the two axes or from the center to the periphery.

Conclusion:
DM scrolls primarily along the horizontal axis. Vertically oval DM samples show minimal scrolling, which can be an advantage in DMEK. Differential scrolling is not determined by the distribution of elastin.
Prognostic factors for visual outcome after surgical repair of limbal corneal laceration at a tertiary eye care center in South India
Authors: Shetty Prerana A , Natarajan Ramya , Bhalerao Sushank A , Vuyyuru Sowjanya , Thigale Uma Yogesh , Tara Divya 
Published: August 2023

Purpose:
To determine the factors affecting the visual outcome after surgical repair of limbal corneal lacerations at a tertiary eye care center in South India.

Methods:
A retrospective analysis of patients diagnosed with limbal tears between 2011 and 2021 was conducted. Demographic information such as age, gender, cause of injury, and size of the laceration was recorded. Comprehensive ocular examination was performed, including gentle B scan evaluation whenever not contraindicated for detailed posterior segment evaluation. Only those cases with a minimum follow-up of one year were included. Postoperative best-corrected visual acuity, intraocular pressure (IOP), cornea clarity, and integrity of the wound at last follow-up were noted.

Results:
Out of the 20 patients, 15 (75%) were males and 5 (25%) were females. The mean age was 42.6 ± 22.4 years. All 20 patients had a penetrating injury, with four (20%) injured by a stick, two (10%) by an iron rod, three (15%) due to road traffic accident (RTA), three (15%) by glass, and eight (40%) with other nonspecific objects [two (10%) with needle, two (10%) with elastic rope, two (10%) with bangle, and two (10%) with metal]. The average time between the injury and the surgery was 48 hours (2 days). Four (20%) patients underwent a second surgery within a week of repair. After limbal tear repair, at final follow-up at 3 years, 7 (35%) had VA worse than 20/800, 3 (15%) had VA between 20/100 and 20/800, and 10 (50%) achieved VA better than 20/80.

Conclusion:
Preoperative visual acuity (VA), mode of injury, and size of wound affect the final visual outcome after surgical repair of limbal corneal laceration. Preoperative VA and mode of injury were statistically significant even in the multivariate analysis.