Medicine and Health Sciences Commons^™

Cohesion: A Core Outcome Set For The Treatment Of Neonatal Encephalopathy, Fiona A. Quirke, Shabina Ariff, Malcolm R. Battin, Caitlin Bernard, Linda Biesty, Frank H. Bloomfield, Mandy Daly, Elaine Finucane, Maira Niaz, Farhana Tabassum

Department of Paediatrics and Child Health

Background: Heterogeneity in outcomes reported in trials of interventions for the treatment of neonatal encephalopathy (NE) makes evaluating the effectiveness of treatments difficult. Developing a core outcome set for NE treatment would enable researchers to measure and report the same outcomes in future trials. This would minimise waste, ensure relevant outcomes are measured and enable evidence synthesis. Therefore, we aimed to develop a core outcome set for treating NE.
Methods: Outcomes identified from a systematic review of the literature and interviews with parents were prioritised by stakeholders (n = 99 parents/caregivers, n = 101 healthcare providers, and n = 22 …

Rural-Urban Trends In Congenital Heart Disease-Related Mortality In The United States, 1999 To 2019, Abdul Mannan Khan Minhas, Rachel A. Wyand, Robert W. Ariss, Salik Nazir, Vardhmaan Jain, Sadeer G. Al-Kindi, Michael D. Shapiro, William Campbell, Laurence Sperling, Salim S. Virani

Office of the Provost

No abstract provided.

Pharmacist-Directed Vancomycin Therapeutic Drug Monitoring In Pediatric Patients: A Collaborative-Practice Model, Kashif Hussain, Rahila Ikram, Gul Ambreen, Muhammad Sohail Salat

Department of Biological & Biomedical Sciences

Background: Therapeutic drug monitoring (TDM) of Vancomycin (VCM) is required to prevent inappropriate dosage-associated bacterial resistance, therapeutic failure, and toxicities in pediatrics. Anecdotal experience and studies show that many healthcare institutions confront barriers while implementing TDM services, this study aimed to assess a pharmacist-directed VCM-TDM service for optimizing patient care in our institution.
Materials and methods: Patients aged 1 month-18 years who received intravenous VCM were included in this quasi-experimental study. The pre-implementation phase (March-June 2018) consisted of retrospective assessment of pediatric patients, the interventional phase (July 2018 to February 2020) included educational programs and the post-implementation phase (March-June 2020) …

Indirect Determination Of Serum Creatinine Reference Intervals In A Pakistani Pediatric Population Using Big Data Analytics, Sibtain Ahmed, Jakob Zierk, Imran Siddiqui, Aysha Habib Khan

Department of Pathology and Laboratory Medicine

Background: The indirect methods of reference intervals (RI) establishment based on data mining are utilized to overcome the ethical, practical challenges and the cost associated with the conventional direct approach.
Aim: To generate RIs for serum creatinine in children and adolescents using an indirect statistical tool.
Methods: Data mining of the laboratory information system was performed for serum creatinine analyzed from birth to 17 years for both genders. The timeline was set at six years from January 2013 to December 2018. Microsoft Excel 2010 and an indirect algorithm developed by the German Society of Clinical Chemistry and Laboratory Medicine's Working …

Comparative Analysis Of Pediatric Covid-19 Infection In Southeast Asia, South Asia, Japan, And China, Judith Ju Ming Wong, Qalab Abbas, Soo Lin Chua, Ririe Fachrina Malisie, Kah Min Pon, Tomohiro Katsuta, Hongxing Dang, Pei Chuen Lee, Muralidharan Jayashree, Quratulain Maha

Department of Paediatrics and Child Health

There is a scarcity of data regarding coronavirus disease 2019 (COVID-19) infection in children from southeast and south Asia. This study aims to identify risk factors for severe COVID-19 disease among children in the region. This is an observational study of children with COVID-19 infection in hospitals contributing data to the Pediatric Acute and Critical Care COVID-19 Registry of Asia. Laboratory-confirmed COVID-19 cases were included in this registry. The primary outcome was severity of COVID-19 infection as defined by the World Health Organization (WHO) (mild, moderate, severe, or critical). Epidemiology, clinical and laboratory features, and outcomes of children with COVID-19 …

Management Of Paediatric Intracranial Ependymoma, Syeda Kubra Kishwar Jafri, Saqib Kamran Bakhshi, Muhammad Shahzad Shamim

Section of Neurosurgery

Intracranial ependymoma are relatively common paediatric brain tumours, but their eloquent location and high recurrence rate pose a significant challenge. Gross total resection or maximum safe resection followed by adjuvant radiotherapy are currently the standard recommended treatment, although there is still nearly 50% recurrence risk at 5 years. Chemotherapy has shown some promising results after recent advances in molecular understanding of ependymomas, but needs further evaluation before it could be added to the treatment regime.

Gliosarcoma In Patients Under 20 Years Of Age. A Clinicopathologic Study Of 11 Cases And Detailed Review Of The Literature, Nasir Ud Din, Hira Ishtiaq, Shabina Rahim, Jamshid Abdul Ghafar, Zubair Ahmad

Department of Pathology and Laboratory Medicine

Background: Gliosarcoma is a rare variant of IDH- wild type glioblastoma with both glial and mesenchymal differentiation. It accounts for approximately 2% of glioblastomas and has a poor prognosis similar to that of classic glioblastoma. It is seen mostly between 40 and 60 years of age with a mean age over 50 years. Pediatric gliosarcoma is even rarer than gliosarcoma in adults. We describe the clinicopathological features of gliosarcoma in patients under 20 years of age and determine whether there are significant differences from gliosarcoma in adults. We also present detailed review of published literature on pediatric gliosarcoma.
Methods: Slides …

Intramedullary Spinal Cord Lesions In Children, Ummey Hani, Sameer Saleem Tebha, Saqib Kamran Bakhshi, Muhammad Shahzad Shamim

Section of Neurosurgery

Paediatric intramedullary spinal cord lesions are uncommon pathologies, prone to result in dismal prognosis if not managed promptly and aggressively. While children usually present in good functional grades compared to adults, early recognition and treatment is important to improve outcomes. In this review, we present tumour demographics, patient factors, and treatment modalities of intramedullary spinal cord lesions in paediatric patients.

A Pathogenic Ufsp2 Variant In An Autosomal Recessive Form Of Pediatric Neurodevelopmental Anomalies And Epilepsy, Min Ni, Bushra Afroze, Chao Xing, Chunxiao Pan, Yanqiu Shao, Ling Cai, Brandi L. Cantarel, Jimin Pei, Nick V. Grishin, Stacy Hewson

Department of Paediatrics and Child Health

Purpose: Neurodevelopmental disabilities are common and genetically heterogeneous. We identified a homozygous variant in the gene encoding UFM1-specific peptidase 2 (UFSP2), which participates in the UFMylation pathway of protein modification. UFSP2 variants are implicated in autosomal dominant skeletal dysplasias, but not neurodevelopmental disorders. Homozygosity for the variant occurred in eight children from four South Asian families with neurodevelopmental delay and epilepsy. We describe the clinical consequences of this variant and its effect on UFMylation.
Methods: Exome sequencing was used to detect potentially pathogenic variants and identify shared regions of homozygosity. Immunoblotting assessed protein expression and post-translational modifications in patient-derived fibroblasts. …

Intussusception In An Infant As A Manifestation Of Covid-19, Zorays Moazzam, Areej Saleem, Alina Ashraf, Fyezah Jehan, Muhammad Arshad

Medical College Documents

Gastrointestinal manifestations of COVID-19 are rare and have primarily been limited to diarrhea or vomiting. Intussusception is the most common cause of bowel obstruction in infants, with up to 30% of pediatric intussusception cases having a preceding viral illness. We present the rare case of intussusception in a SARS-CoV-2 positive infant. This is the first documented case of survival in a SARS-CoV-2 positive patient presenting with intussusception as the primary manifestation. As our knowledge of this disease evolves, surgeons need to remain suspicious for possible gastrointestinal manifestations of COVID-19.

Primary Pediatric Cerebellar Gliosarcoma, Syed Sarmad Bukhari, Muhammad Junaid, Ali Afzal, Anisa Kulsoom

Section of Neurosurgery

Background: Primary gliosarcomas of the central nervous are rare and very few have been reported in the infratentorial compartment. Here, we describe such a lesion in a 12-year-old male.
Case description: A 12-year-old male presented with headache, ataxia, and vomiting. When Magnetic resonance studies documented a posterior fossa lesion, he underwent placement of a right ventriculoperitoneal shunt followed by a suboccipital craniectomy. The lesion proved to be a primary gliosarcoma. Unfortunately, it recurred 2 years later and required repeated resection.
Conclusion: Here, we reviewed the rare case of a 12-year-old male requiring shunt placement and suboccipital craniectomy for a primary …

A Mixed Method Multi-Country Assessment Of Barriers To Implementing Pediatric Inpatient Care Guidelines, Kirkby D. Tickell, Dorothy I. Mangale, Stephanie N. Tornberg-Belanger, Celine Bourdon, Johnstone Thitiri, Molline Timbwa, Jenala Njirammadzi, Wieger Voskuijl, Ali Faisal Saleem, Zaubina Kazi

Department of Paediatrics and Child Health

Introduction: Accelerating progress in reducing child deaths is needed in order to achieve the Sustainable Development Goal child mortality target. This will require a focus on vulnerable children-including young children, those who are undernourished or with acute illnesses requiring hospitalization. Improving adherence to inpatient guidelines may be an important strategy to reduce child mortality, including among the most vulnerable. The aim of our assessment of nine sub-Saharan African and South Asian hospitals was to determine adherence to pediatric inpatient care recommendations, in addition to capacity for and barriers to implementation of guideline-adherent care prior to commencing the Childhood Acute Illness …

Utility And Accuracy Of Primary And Secondary Ultrasonographic Signs For Diagnosing Acute Appendicitis In Pediatric Patients, W. Akhtar, Mujtaba Z. Naveed, Kumail Khandwala

Department of Radiology

Introduction Making an accurate diagnosis of acute appendicitis (AA) is vital to prevent the morbid complications associated with untreated AA. This is challenging in up to 30% of pediatric patients which is a significantly high number. Ultrasound (US) has been generally used as the initial mode of imaging in pediatric patients due to the lack of ionizing radiation. Given its variable accuracy, adjuvants such as secondary signs can be used to aid the radiologist in making an accurate diagnosis. Materials and methods Patients between the ages of two and sixteen years with acute abdominal pain suspicious for AA, who underwent …

Effect Of Two Techniques Of Parental Interaction On Children's Anxiety At Induction Of General Anaesthesia - A Randomized Trial, Alia Hussain, Fauzia Khan

Department of Anaesthesia

Objective: Several non-pharmacological techniques, such as parental presence and behavioral preparation, are used to decrease children's anxiety at anaesthesia induction. We compared the mean anxiety score in children at the time of anaesthesia induction with two different physical techniques of parental interaction and a control group with no parent present. The secondary objective was to determine the face mask acceptance during induction.
Methods: This study recruited 123 ASA I & II children, aged 1 to 8 years, undergoing day care surgery, who were randomly allocated to three groups. Children either went to the operating room (OR) alone (Gp C), or …

Torsion Of Ovarian Dysgerminoma In A Child: Role Of Computed Tomography., Kumail Khandwala, Jehanzeb Shahid, Naila Nadeem, Muhammad Usman Tariq

Department of Radiology

Dysgerminomas are malignant germ cell tumors of the ovary that most commonly occur in the adolescent population. Ovarian dysgerminomapresenting with complications like torsion is a rare entity in the pediatric age group. Cross-sectional imaging plays a crucial role in diagnosis, tumor staging before surgical resection, and for planning adjuvant chemotherapy. We report a case of a nine-year-old female who presented to the emergency room (ER) with abdominal distention and abdominal pain. Computed tomography scan revealed a large right-sided pelvic mass with areas of low attenuation, speckled calcification, peritumoral free fluid, and a twisted vascular pedicle that was likely originating from …

Comparison Of Enteral Versus Intravenous Potassium Supplementation In Hypokalaemia In Paediatric Patients In Intensive Care Post Cardiac Surgery: Open-Label Randomised Equivalence Trial (Eips), Naveed Ur Rehman Siddiqu, Quratulain Merchant, Babar Hasan, Arjumand Rizvi, Muneer Amanullah, Amina Rehmat, Anwarul Haque

Department of Paediatrics and Child Health

Objectives: The primary objective was to compare the efficacy of enteral potassium replacement (EPR) and intravenous potassium replacement (IVPR) as first-line therapy. Secondary objectives included comparison of adverse effects and number of doses required to resolve the episode of hypokalaemia.
Trial design: The EIPS trial is designed as a randomised, equivalence trial between two treatment arms.
Study setting: The study was conducted at the paediatric cardiac intensive care unit (PCICU) at Aga Khan University Hospital, Karachi.
Participants: 41 patients (aged 1 month to 15years) who were admitted to PCICU post cardiac surgery were recruited (23 IVPR arm and 18 …

Spectrum Of Complications Of Severe Dka In Children In Pediatric Intensive Care Unit, Qalab Abbas, Saba Arbab, Anwarul Haque, Khadija Nuzhat Humayun

Department of Paediatrics and Child Health

Objectives: To describe the spectrum of complications of Diabetic Ketoacidosis (DKA) observed in children admitted with severe DKA.
Methods: Retrospective review of the medical records of all children admitted with the diagnosis of severe DKA in Pediatric Intensive Care Unit (PICU) of the Aga Khan University Hospital, from January 2010 to December 2015 was done. Data was collected on a structured proforma and descriptive statistics were applied.
Results: Total 37 children were admitted with complicated DKA (1.9% of total PICU admission with 1.8% in 2010 and 3.4% in 2015). Mean age of study population was 8.1±4.6 years and 70% were …

Pediatric Critical Incidents Reported Over 15 Years At A Tertiary Care Teaching Hospital Of A Developing Country, Shemila Abbasi, Fauzia Anis Khan, Sobia Khan

Department of Anaesthesia

Background and Aims: The role of critical incident (CI) reporting is well established in improving patient safety but only a limited number of available reports relate to pediatric incidents. Our aim was to analyze the reported CIs specific to pediatric patients in our database and to reevaluate the value of this program in addressing issues in pediatric anesthesia practice. Material and Methods: Incidents related to pediatric population from neonatal period till the age of 12 years were selected. A review of all CI records collected between January 1998 and December 2012, in the Department of Anaesthesiology of Aga Khan University …

Giant Ganglioneuroma In A 5-Year Child, Hamza Abdur Rahim Khan, Fazal Wahab Khan, Saulat H. Fatimi

Department of Surgery

ABSTRACT We report a 5-year boy presenting with pain in the lower chest and upper abdomen. On evaluation with computed tomography scan of the chest, he was found to have a large 16 x 14 cm posterior mediastinal mass compressing the inferior vena cava and liver, and shifting the heart to the opposite side. Tumor was approached via right postero-lateral thoracotomy and dissected off from esophagus, heart, inferior vena cava and lungs. The entire tumor was resected without any complications. Postoperative course was unremarkable. Resection of the tumor was successful. The histopathology showed it to be giant ganglioneuroma with no …

Small Bowel Ascaris Infestation: A Diagnostic Challenge, Muhammad Waqas Khan, Sanniya Khan Ghauri

Department of Emergency Medicine

Ascariasis is a common infestation in developing countries where there is poor hygiene. A majority of the cases are asymptomatic, with a few cases presenting with mild abdominal pain and nutritional deficiencies in the long term. Here we present a case of a young boy who presented as a diagnostic dilemma, with signs of acute intestinal obstruction without any supporting radiological evidence. A barium study revealed the presence of low-burden Ascaris infestation that was managed medically.

Outcome Of Pediatric Procedural Sedation & Analgesia In A Tertiary Care Hospital In Pakistan, Humaira Jurair, Amyna Bhimani, Anwar-Ul-Haque

Department of Paediatrics and Child Health

Background and Objective: Procedural sedation and analgesia (PSA) is pharmacologically induced state which allows patients to tolerate painful procedures while maintaining protective reflexes. It is the standard of care but there is limited data from Pakistan. Our objective was to assess the safety of the procedural sedation and analgesia in pediatric population at a tertiary care setting.
Methods: A retrospective notes and record review was conducted at the Aga Khan University Hospital, Karachi over 4 years from April 2010 to August 2014. Patients were between ages 6 months to 16 years and were in low risk category. The combination of …

Ten Year Experience Of Pediatric Kidney Biopsies From A Single Center In Pakistan, A Absar, M Diamond, Y Sonia, R Arshalooz, A Safia, K Waqar, P Shahid

Section of Nephrology

There are many established registries of kidney biopsies around the world. In addition, there are several reports available in literature from many countries on pediatric kidney biopsy. This study was done to determine the indications and pathological patterns of kidney biopsies of children referred to our hospital, and compare our data with the data available from other countries. This is a cross-sectional study of pediatric kidney biopsies over a 10-year period, from January 1997 to December 2006. All biopsies were done in Aga Khan University Hospital, Karachi, Pakistan. Age range was from 1 to 14 years. Data were analyzed for …

Rhabdomyosarcoma Embryonal Type: In A Three And A Half Year Old Girl, Ameer A Khowaja, Saqib Ali Gowani, Asmatullah Khan, Saulat H. Fatimi

Department of Biological & Biomedical Sciences

Rhadomyosarcoma is the most common tumour of the soft tissues in infants and children. We report a case of a 3 1/2 year old girl who presented with a swelling over the neck. Swelling was diagnosed as Rhabdomyosarcoma (embryonal type). Tumour mass was found to be unresectable initially. The patient was subjected to multiple courses of checmotherapy, which shrunk the tumour significantly. There was simultaneous development of cystic lesions in upper lobe of right lung. Excision of the remnant rhabdomyosarcoma mass and biopsy of right lung with cystectomy of right upper lobe cystic lesion was performed. The patient fully recovered …

Chediak-Higashi Syndrome, Emaduddin Siddiqui, Shaheena Hanif

Department of Emergency Medicine

Chediak Higashi Syndrome is a rare inherited autosomal recessive disorder of immune system. Susceptibility to infection due to phagocyte dysfunction ranges from recurrent skin infection to over whelming fatal systemic infection. A five years old male child was admitted on 31st March 2006 with the complaints of pallor, fever & ear discharge for 6 month. He was treated by several medications but had temporary relief. The ear discharge was bilateral, foul smelling and of yellowish color. Past history was significant with episodes of abscess involving the different parts of body. Myeloid precursors shows giant, purple stained granules mainly in metamyelocytes, …

Immunohistochemical Evaluation Of Small Round Cell Tumors Of Childhood, Sajid H. Shah, Irshad N. Soomro, M. Shahid Siddiqui, Shahid Pervez, Sheema H. Hassan

Department of Pathology and Laboratory Medicine

Objective:

This study was done to evaluate the pediatric undifferentiated small round cell tumors with immunohistochemical staining.

Setting:

The present study included consecutive cases of small round cell tumors which were diagnosed in children (<15 years) in the section of Histopathology at the Aga Khan University Hospital, Karachi during the period of two years.

Methods:

The group of undifferentiated small round cell tumors were evaluated immunohistochemically by using a panel of antibodies on sections from routinely processed, formalin fixed, paraffin embedded tissue blocks.

Results:

The category of undifferentiated small round cell tumors included rhabdomyosarcoma (23.2%), primitive neuroectodermal tumor (17.9%), non-Hodgkin’s lymphoma (16.1%), neuroblastoma (14.2%), Ewing’s sarcoma (10.7%) in order of frequency. Osteosarcoma (Small cell variant), retinoblastoma and medulloblastoma comprised …