Outcome of pediatric living donor liver transplant: Experience from Pakistan; a resource limited setting.

INTRODUCTION: Liver transplantation (LT) has emerged as a lifesaving modality for many liver diseases in children. Pediatric LT is an established treatment in the Western world but is relatively a new procedure in resource-limited countries like Pakistan. The study aims to highlight the outcomes and survival of pediatric recipients from the first pediatric liver transplant center in Pakistan. METHOD: A retrospective analysis of pediatric LT was done from 2012 to 2019. The study was conducted in the Hepatobiliary and liver transplant department of Shifa International Hospital (SIH), Islamabad. A detailed analysis for indications for pediatric LT, survival, and complications was done. RESULTS: Forty-five patients under 18 years of age underwent Living donor liver transplant (LDLT) in SIH. Median age was 9 years and M:F of 2:1. Cryptogenic liver disease followed by Wilson disease were the two most common indications of LT. The majority of patients had chronic liver disease 34 (75%) while 11 (24%) had acute liver failure. The right lobe graft was the most common type of graft 19 (42.2%). Thirty days, 1-year, 3-year, and 5-year survival was 77.8%, 75.6%, 73.3%, and 60.6% respectively. Mortality was highest in patients with biliary atresia 4 (33%). Causes of death included pulmonary embolism, sepsis, surgical complications, and acute kidney injury. Mean survival was 88.850 months (±7.899) (CI 73.369-104.331). CONCLUSION: Pediatric LDLT has offered disease-free survival for patients. Survival can improve further with nutritional rehabilitation and anticipation and management of post-operative complications.

Vitamin D deficiency: A risk factor for myopia in children - a cross sectional study in a tertiary care centre.

Objective: To study the role of vitamin D deficiency as a risk factor for myopia in children aged 5-15 years. METHODS: The cross-sectional observational study was conducted from January to September 2019 at the Ophthalmology and Paediatric departments of Shifa Foundation Community Health Centre, Islamabad, Pakista. It comprised patients with suspected / symptomatic vitamin D deficiency who were enrolled from the paediatric outpatient department and referred to the ophthalmology clinics for eye exam. Apart from taking detailed ocular history, slit lamp examination, Snellen's distance visual acuity, auto-refraction to calculate spherical equivalent, and amplitude scan for measuring the axial length were performed. An average of 3 measurements was taken for both refraction and axial length calculation. Myopia was labelled if mean spherical equivalent was ≤0.25 dioptres. Vitamin D deficiency was defined as serum 25-hydroxyvitamin D level < 20ng/ml. Data was analysed using SPSS 23. RESULTS: There were 72 subjects with a mean age of 10.11±2.69 years; 37(51.4%) boys and 35(48.6%) girls. Myopia was seen in 40(55.6%) patients, while 32(44.4%) were emmetropic. The overall mean vitamin D level was 20.25±12.18 ng/ml. There was no significant association between myopia and vitamin D deficiency (p=0.115). Significant associations were found between myopia and relatively older age (p=0.005), higher height (p=0.001), more weight (p=0.001) and higher body mass index value (p=0.008). Conclusion: Low vitamin D levels were not significantly associated with myopia in children aged 5-15 years, but significant associations were found between myopia and relatively older age, and various anthropometric measures.

Impact of COVID Pandemic and Hybrid teaching on Final year MBBS students' End of clerkship Exam performance.

BACKGROUND & OBJECTIVES: The novel coronavirus (SAR-CoV-2) pandemic has revolutionized medical education worldwide. Most medical schools have adopted the online teaching and assessments. Students attending modified clerkships and assessments under the stress of the pandemic, perform and score differently when compared to normal clerkships. We aimed to identify the impact of COVID-19 on final year MBBS students' EOC (End of Clerkship) examination by comparing them with their scores prior to the COVID and with scores of the previous final year. METHODS: This cross-sectional study was conducted at Shifa College of Medicine. Final year MBBS students' scores of years 2019 and 2020 were included. Students' EOC MCQ and OSCE scores were compared in pre-COVID and COVID affected rotations of the same year and with the previous year (2019). Data were analyzed in SPSS version 21, means scores were calculated, and one-way ANOVA was applied. Pearson correlation was calculated for correlation assessment of MCQ and OSCE scores. RESULTS: There were 118 students. The mean EOC, OSCE, and MCQ scores in rotations one to four were 72.8±6.4, 73.3± 8.1, 71.6± 7.4, 72.7± 6.7 and 44.4± 8, 47.2± 8.4, 46.1± 8.2, 48.8± 8.1, respectively. One-way ANOVA results before and after COVID lockdown were statistically insignificant (p=0.3) for OSCE and significant for MCQ in the final year class of 2020 (p=0.001). The Pearson correlation assessment between MCQ and OSCE scores (n=416) had a significant positive correlation (r=0.42, p=0.000). The overall comparison between scores of the final year class of 2019 and 2020 showed significant improvement in Surgery and Obstetrics/Gynae scores in 2020. CONCLUSION: During the COVID pandemic, the final year students' performance in EOC MCQ and OSCE over all remained unaffected.

Frequency and types of haemoglobinopathies in children with microcytic anaemia.

OBJECTIVE: To study the frequency and types of haemoglobinopathies in children with microcytic anaemia. METHODS: The prospective study was conducted at the Paediatric Out-patient Department of Shifa Falahi Community Health Centre, Islamabad, Pakistan, from July to December, 2018, and comprised patients aged from 3 months to 14 years who had haemoglobin <10mg/dl and mean corpuscular volume <70fL. Serum ferritin and haemoglobin electrophoresis were done to check for iron deficiency anaemia and haemoglobinopathies. Data was analysed using SPSS 23. RESULTS: Of 175 subjects, 33(18.9%) had haemoglobinopathies and 142(81.1%) had iron deficiency anaemia. Thalassemia trait 18(10.3%) was the leading cause amongst haemoglobinopathies, followed by thalassemia major 8(4.6 %) and intermedia 5(2.9%). There were 2(1.1%) patients with haemoglobin D. CONCLUSIONS: The prevalence of haemoglobinopathies was high. Identification of haemoglobinopathies is important for proper treatment, antenatal screening and future genetic counselling.

Students' perception and scores in Paediatrics end-of-clerkship and final professional Objective Structured Clinical Examination (OSCE): A comparative study.

OBJECTIVES: This study aims to understand students' perception of the usefulness of end-of-clerkship (EOC) as compared to professional exam Objective Structured Clinical Examinations (OSCE) and to compare student performance in both examinations. METHODS: We conducted a cross-sectional study of medical students who were studying in the final year at Shifa College of Medicine, Shifa Tameer-e-Millat University, and appeared in both end-of-clerkship and final professional exams. The study was conducted in October 2019. The data was collected through a self-constructed questionnaire. The scores of all participants were also recorded. The data obtained was analyzed on IBM's statistical package for the social sciences (SPSS) version 23 (IBM, Armonk, NY). Descriptive statistics were used for qualitative variables. Mean and standard deviation was calculated for quantitative variables. Chi-square test was also applied to assess for significant differences. RESULTS: Out of 115 participants, 57 (49.6%) were males and 58 (50.4%) were females. Most of the participants (75.7%) agreed that both OSCEs were well-structured. Participants found that both the exams give a good cross-section of paediatrics and allowed them to learn something new (p=0.00). 45% of participants perceived that the end-of-clerkship OSCE exam helped in orienting them for the final professional exam by identifying their weaknesses. CONCLUSION: Although the EOC OSCE served as a good preparatory exercise, it did not alleviate the stress levels. However, the results in both OSCEs were comparable and students were satisfied with the current weightage of marks distribution.

A Clinico-Pathological Study Of Wilsons Disease; 8 Years' Experience Of A Tertiary Care Hospital.

BACKGROUND: Wilson's disease is a genetically transmitted disease and has a variety of clinical manifestations. We evaluated the various clinical and biochemical presentations of Wilson's disease (WD) at different ages. METHODS: This cross-sectional study was conducted in Shifa international hospital and Shifa Foundation Falahi Clinic (SFFC), Islamabad. Data from Jan 2010 to Dec 2018 was retrieved from hospital medical record on a structured proforma. All patients who had twenty-four hours urinary copper level of ≥100 mcg/day were included in the study. Their presenting symptoms, clinical signs and lab investigations were noted. RESULTS: Mean age was 13±4.588 years. Male to female ratio was 1.5:1. Hepatic disease was seen in 35 (68.6%) patients mainly in <10 yrs age group. Pure neurological Wilson's was seen in 14 (27.45%) cases, which were >10 years of age while 18(35.3%) had hepato-neurological manifestations. Keyser Fleischer rings were present in 26 (51%) of total patients and 14 (100%) of neurological cases. Hepatic transaminases were elevated in 36 (70 %) patients. Low serum cerruloplasmin was seen in 37 (72.5%) cases. Mean value of haemoglobin was 10.38±2.772. Mean 24 hours urinary copper was 597.6±605.446. Consanguinity was seen among 33 (64.7%) families. Family history of WD was positive in 21 (41.2%) patients. CONCLUSIONS: Hepatic form of WD is more common, yet neurological presentation is seen in patients >10 years of age.

Wilson Disease in Children; Chelation Therapy or Liver Transplantation? A 10-Year Experience from Pakistan.

BACKGROUND Wilson disease (WD) is a rare genetic disorder with vast clinical presentations and a higher incidence in areas where consanguinity is common. Most patients can be treated with oral chelation, but some require advanced surgical intervention, like liver transplantation (LT). This study aims to review outcomes of WD patients presenting to a tertiary care center over a period of 10 years. MATERIAL AND METHODS This retrospective analysis was conducted at Shifa International Hospital, Islamabad, Pakistan. Patients <18 years who were diagnosed with WD per ESPAGHAN guidelines from 2010 to 2020 were included. Presentation, diagnosis, treatment, and LT and its complications were recorded. Follow-ups were recorded, and patients were contacted by phone in cases of interrupted follow-up. Frequencies and percentages of variables were calculated. RESULTS A total of 48 patients with WD were identified. Symptomatic disease was seen in 45 patients, with 3 diagnosed on screening. The hepatic form was common (62.2%). Mean age at diagnosis was 9.74 (range 5-17) years, 28 (58.3%) were male, while 17 (35.4%) were female. Urinary copper was increased in all patients (645.82±528.40). Oral treatment with penicillamine was given to 34 (75.5%) patients; 4 (8.9%) died while on oral treatment. Living donor LT was performed in 11 (22.9%) patients, who had a mean King's Wilson index of 11 (range, 6-14). Currently, all LT patients are alive, with maximum graft survival of 7 years. CONCLUSIONS LT offers a promising treatment with good outcomes in pediatric WD. However, timely diagnosis and management with oral chelation therapy can prolong survival without LT.

A Comparison Between Written Assessment Methods: Multiple-choice and Short Answer Questions in End-of-clerkship Examinations for Final Year Medical Students.

Introduction An important aspect of a modern academic curriculum is assessment, which can be clinical and written. Written assessment includes both multiple-choice questions (MCQs) and short answer questions (SAQs). Debate continues as to which is more reliable. It is important to assess the correlation between the two different formats of written assessments, especially in the clinical subjects as they are different from the basic science subjects. Moreover, data are lacking in the correlation of the two formats of the written assessment in the clinical subjects. Therefore, we conducted this study to see the correlation between MCQs and SAQs in the end-of-clerkship examinations for final-year medical students. Materials and methods The end-of-clerkship written assessment results of the four disciplines of medicine, surgery, gynecology, and pediatrics were included. This was a retrospective correlational analytical study conducted at Shifa Tameer-e-Millat University, Islamabad, from 2013 to 2017. Data were analyzed using IBM SPSS Statistics for Windows, version 23.0 (IBM Corp., Armonk, NY); mean, standard deviation, Pearson coefficient, and p values were calculated both for MCQs and SAQs. Results A total of 481 students were involved in our study. The mean percentage scores of MCQs and SAQs in medicine were the most similar, and scores in obstetrics and gynecology had the most disparity. As compared to MCQs, the wider standard deviations were found in SAQs. Pearson correlations were 0.49, 0.47, 0.23, and 0.38 for medicine, surgery, gynecology, and pediatrics, respectively. Conclusion While we found mild to moderate significant correlation between MCQs and SAQs for final-year medical students, further investigations are required to explore the correlation and enhance the validity of our written assessments.