115 results match your criteria: "Hospital Tunku Azizah[Affiliation]"

Paediatric new onset type 1 diabetes and diabetic ketoacidosis during the COVID-19 pandemic in Malaysia.

Diabetes Res Clin Pract

November 2023

Paediatric Endocrine Unit, Department of Paediatrics, Faculty of Medicine, University Malaya, Kuala Lumpur, Malaysia.

Aims: Despite emerging evidence of increased paediatric diabetes mellitus (DM) and diabetic ketoacidosis (DKA) worldwide following the COVID-19 pandemic, studies in Asia are lacking. We aimed to determine the frequency, demographics, and clinical characteristics of new onset type 1 DM (T1DM) during the pandemic in Malaysia.

Methods: This is a retrospective multicenter study involving new onset T1DM paediatric patients in Klang Valley, Malaysia during two time periods ie 18th September 2017-17th March 2020 (pre-pandemic) and 18th March 2020-17th September 2022 (pandemic).

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Background: Identifying risk factors in patients with intravenous immunoglobulin (IVIG) resistance Kawasaki disease (KD) is vital in managing and preventing coronary artery aneurysms (CAAs). We aimed to study the risk factors for IVIG resistance KD in Malaysian children.

Methods: This retrospective observational study of children with KD was conducted at two tertiary hospitals in Malaysia from January 2014 to December 2019.

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Advances in perinatal care have led to the increased survival of preterm infants with subsequent neonatal morbidities, such as retinopathy of prematurity (ROP). This study aims to compare the differences of neonatal healthcare systems, resources, and clinical practice concerning ROP in Asia with review of current literature. An on-line survey at the institutional level was sent to the directors of 336 neonatal intensive care units (NICU) in 8 collaborating national neonatal networks through the Asian Neonatal Network Collaboration (AsianNeo).

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Introduction: Since the emergence of COVID-19, we have experienced potent variants and sub-variants of the virus with non-specific neurological manifestations. We observed a surge of the Omicron variant of COVID-19 patients with neurological manifestations where less cases of multisystem inflammatory syndrome in children (MIS-C) were reported. This article describes our experience of children with severe and rare neurological manifestations following COVID-19 infection.

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Background: Pompe disease is a rare glycogen storage disorder caused by deficiency of the lysosomal enzyme acid alpha-glucosidase (GAA), leading to glycogen deposition in multiple tissues. Infantile-onset Pompe disease (IOPD) patients present within the first year of life with profound hypotonia and hypertrophic cardiomyopathy. Treatment with enzyme replacement therapy (ERT) has significantly improved survival for this otherwise lethal disorder.

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Biliary complications post liver resection for pediatric liver tumors.

World J Pediatr Surg

July 2023

Department of Paediatric Surgery, Hospital Tunku Azizah (Kuala Lumpur Women's and Children's Hospital, Ministry of Health Malaysia), Kuala Lumpur, Malaysia.

Objective: To review biliary complications following liver resection for liver tumors in children and their associated risk factors.

Methods: Retrospectively, we reviewed children who underwent liver resection for liver tumors from 2010 to 2019. Demographic data, operative details, types of complications, interventions and outcomes were studied.

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Background: Timely and relevant information helps parents to cope when a child is diagnosed with cancer. However, obtaining and understanding information is not a straightforward process for parents.

Objectives: This article aims to explain paediatric cancer parents' information behaviour related to the care of their child.

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Introduction: Early and effective treatment is fundamental in status epilepticus (SE) management. At the initiative of the Epilepsy Council of Malaysia, this study aimed to determine the treatment gap in SE across different healthcare settings in Malaysia.

Methods: A web-based survey was sent to clinicians involved in the management of SE, across all states and at all levels of healthcare services.

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Background: Variants in the dynamin-1 () gene typically cause synaptopathy, leading to developmental and epileptic encephalopathy (DEE). We aimed to determine the genotypic and phenotypic spectrum of encephalopathy beyond DEE.

Methods: Electroclinical phenotyping and genotyping of patients with a variant were conducted for patients undergoing next-generation sequencing at our centre, followed by a systematic review.

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Hirschsprung's disease (HD) is one of the most well-known gastrointestinal motility disorders. Diagnosis and management of other lesser-known motility disorders are often challenging and tedious. We describe a teenager who was severely constipated from birth and needed intensive care admissions for life-threatening enterocolitis.

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Objectives: To summarize the role of therapeutic plasma exchange (TPE) in critically ill adults and children with severe sepsis.

Data Collection: A systematic search was performed using the following databases: Medline, EMBASE, CINAHL, and Cochrane from January 1990 till December 2022. Comparative studies of TPE in severe sepsis were selected.

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Introduction: In this study, we aimed to determine the prevalence of sleep disturbances among children aged 2-5 years with autism spectrum disorder (ASD) and their association with behaviour in these children.

Method: A cross-sectional study was conducted at Hospital Tunku Azizah, Kuala Lumpur, Malaysia, from June 2020 to December 2020. Children between 2 and 5 years old, diagnosed with ASD based on the Diagnostic and Statistical Manual of Mental Disorders, Fifth Edition (DSM-5) criteria, were recruited.

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Thyroid autoimmunity and autoimmune thyroid disease in Malaysian girls with Turner syndrome: An understudied population.

J Paediatr Child Health

July 2023

Paediatric Endocrine Unit, Department of Paediatrics, Faculty of Medicine, University Malaya, Kuala Lumpur, Malaysia.

Aims: Knowledge on the spectrum of thyroid disorders amongst Turner syndrome (TS) patients in Southeast Asia is limited. This study aimed to evaluate the prevalence of thyroid autoimmunity, the spectrum of autoimmune thyroid disease and association with age and karyotype amongst Malaysian TS girls.

Methods: A cross-sectional study was conducted at 11 paediatric endocrine units in Malaysia.

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Purpose: Primary immunodeficiency disease (PID) affects various aspects of a patient's life. However, the health-related quality of life (HRQOL) of PID among Malaysian patients is poorly described. This study aimed to determine the quality of life of PID patients and their respective parents.

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Background: Clinical pharmacy plays an integral role in optimizing inpatient care. Nevertheless, prioritising patient care remains a critical challenge for pharmacists in a hectic medical ward. In Malaysia, clinical pharmacy practice has a paucity of standardized tools to prioritise patient care.

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This case series compared clinical variables and various combinations of immunotherapy received with outcomes of patients with severe acute necrotizing encephalopathy (ANE). We performed a retrospective review of clinical variables, immunotherapy received, and outcomes (based on the modified Rankin Scale) in Malaysia between February 2019 and January 2020. Twenty-seven children (12 male), aged 7 months to 14 years (mean 4 years) at diagnosis were included.

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Infantile vitiligo and alopecia in immunodysregulation polyendocrinopathy enteropathy X-linked syndrome.

Pediatr Dermatol

November 2023

Paediatric Dermatology Unit, Department of Paediatrics, Hospital Tunku Azizah, Kuala Lumpur, Malaysia.

Immune dysregulation, polyendocrinopathy, enteropathy, X-linked (IPEX) is characterized by failure to thrive, severe chronic diarrhea, neonatal type 1 diabetes or thyroiditis, and eczematous dermatitis. We report a patient with infantile onset IPEX syndrome who developed vitiligo, alopecia, and chronic diarrhea. Awaiting stem cell transplant, he had multiple episodes of sepsis and succumbed at the age of 10 months.

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Background: Sleep disturbance in children with atopic dermatitis (AD) frequently goes unnoticed and can be associated with behavioral challenges. The aims of this study were to determine (a) the prevalence and factors associated with sleep disturbance and behavioral problems and (b) the correlation between sleep disturbance and behavioral problems in children with AD.

Methods: This cross-sectional study involved children aged 4-12 years old with moderate to severe AD.

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Article Synopsis
  • Encephalocraniocutaneous lipomatosis (ECCL) is a neurocutaneous disorder affecting the skin, eyes, and central nervous system, exemplified by a case involving a three-month-old girl with a growing corneal dermoid and other neurological abnormalities.
  • MRI findings revealed significant developmental issues including a dermoid cyst and structural brain abnormalities, leading to a diagnosis of ECCL.
  • A multidisciplinary approach involving various medical teams concluded that surgical intervention could pose more risks than benefits, opting instead for close monitoring and periodic MRI assessments to manage the condition.
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Introduction Validated tools to measure caregiving knowledge among parents of children with hematological cancers are needed to measure the clinical outcome of caregiver interventions. This study reports the development and validation of the Hematological Oncology Parents Education Caregiving Knowledge Questionnaire (HOPE-CKQ) among Malaysian parents of pediatric leukemia and lymphoma patients. Methods Initially, 60 items on caregiving knowledge were developed based on a qualitative needs assessment study.

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Background: A shortage of specialist paediatric surgeons in Malaysia led to establishment of a national postgraduate training programme in 2004. We aimed to identify sociodemographic factors impacting training experience, and to define pressure points during training to inform targeted trainee support strategies.

Methods: We conducted an anonymized online survey in June 2021 on all programme graduates.

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Anal canal and sphincter function in children with Hirschsprung disease after definitive surgery.

J Pediatr Surg

February 2023

Division of Paediatric Surgery, Department of Surgery, Faculty of Medicine, Universiti Malaya, Kuala Lumpur, Malaysia. Electronic address:

Introduction: Anorectal manometry may be useful to objectively evaluate anorectal function following definitive pullthrough for Hirschsprung Disease (HD) but there is little published data. Our study aims to investigate anorectal manometry findings and their association with bowel function.

Methodology: This was a prospective observational study.

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Early childhood nutritional deficiency has detrimental consequences on physical and cognitive development. We conducted a single-center, single-blind, two-arm pilot randomized no-treatment controlled trial (the Child of Urban Poverty Iron Project (CUPIP); NCT03819530) in a people’s housing project locale in Selangor, Malaysia, between September 2019 and February 2020, to assess the trial’s general feasibility and preliminary benefits of daily micronutrient supplementation for iron storage and anthropometric outcomes in under-5 children. Those with history of premature births, congenital abnormalities, or baseline hemoglobin <70 g/L were excluded.

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Background Postpartum depression has been linked to undesirable outcomes for mother-infant dyads, interfering with childcare and breastfeeding practices. This study aimed to determine the prevalence of depressive symptoms among mothers during the postpartum period and its association with breastfeeding and postpartum experiences. Methodology This cross-sectional study involved mothers of hospitalized infants (n = 219) at two tertiary hospitals in Klang Valley, Malaysia.

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