Mild-aortic syndrome: a cause of recurrent abdominal pain

Mid-aortic syndrome (MAS) is a rare clinical entity that is characterized by coarctation involving the distal thoracic and/or abdominal aorta and its major branches accounting for 0.5–2% of all cases of coarctation of the aorta (1). Renovascular hypertension can be a significant sequelae - it is the main symptomatic presentation of this disease among children and adolescents. We describe a 9-year-old girl who presents with recurrent abdominal pain and symptomatic hypertension. Due to significant left ventricular systolic dysfunction and uncontrolled hypertension, percutaneous balloon angioplasty was performed to treat the coarctation. To our knowledge, this is the first reported case of MAS in Malaysia. This case report highlights the clinical presentation, the role of computed tomography angiogram (CTA) in the diagnosis and current options in the management of MAS

Quality of life among adolescent survivors of childhood cancer

Over the past few decades, there has been a dramatic improvement in the long-term survival of childhood cancer patients as a result of advancement in pediatric oncology management. Previously the length of survival is used as the primary outcome evaluated in oncology treatment research, but it is now widely accepted that the quality of survival is as important as the quantity of survival. The severely toxic chemotherapy must be evaluated for its detrimental impact as well as its survival benefit. The improvement in survival rate has placed an increasing emphasis on quality of life in childhood cancer survivors. This book provides knowledge and information on the quality of life among adolescent survivors of childhood cancer, rated by survivors and their caretakers using PedsQL Generic scoring. The analysis should help shed some light on the quality of life of young survivors of childhood cancer and will be especially useful to professionals in paediatric oncology or anyone who is interested in the quality of life study in adolescent or to the caretakers of cancer patients

Mid-aortic syndrome: a rare cause of recurrent abdominal pain

Mid-aortic syndrome (MAS) is a rare clinical entity that is characterized by coarctation involving the distal thoracic and/or abdominal aorta and its major branches accounting for 0.5–2% of all cases of coarctation of the aorta (1). Renovascular hypertension can be a significant sequelae - it is the main symptomatic presentation of this disease among children and adolescents. We describe a 9-year-old girl who presents with recurrent abdominal pain and symptomatic hypertension. Due to significant left ventricular systolic dysfunction and uncontrolled hypertension, percutaneous balloon angioplasty was performed to treat the coarctation. To our knowledge, this is the first reported case of MAS in Malaysia. This case report highlights the clinical presentation, the role of computed tomography angiogram (CTA) in the diagnosis and current options in the management of MAS

Internal iliac artery thrombosis in a 2-month-old infant with incomplete Kawasaki disease

We report a case of a 2-month-old infant with incomplete Kawasaki disease with multiple coronary and systemic arteries aneurysms complicated with internal iliac arteries thrombosis. The atypical clinical presentations and severity of systemic vascular involvements discuss the importance of high index of suspicions in younger infants and treatment options in such cases

Diagnostic value of myocardial deformation pattern in children with noncompaction cardiomyopathy

The current echocardiographic diagnostic criteria for noncompaction cardiomyopathy (NCC) have variable sensitivity and low specificity. Moreover, there are limited data on the use of myocardial deformation imaging for early detection of myocardial dysfunction in children with NCC. We describe left ventricular (LV) deformation patterns in children with NCC, with the goal of identifying a potential diagnostic pattern. We prospectively enrolled 30 children with NCC (47% male; mean age 7.2 years) and 30 age- and gender-matched controls. Extent and severity of non compaction in each segment were evaluated in LV 16-segment model. Regional (base, mid and apex) and segmental (16 segments) longitudinal strain (LS), circumferential strain (CS) and radial strain (RS) were measured using speckle tracking echocardiography. In all patients with NCC, regional and segmental CS and RS at the apex were significantly decreased compared to controls (CS apex: − 19.2 ± 5.4% vs. − 30.2 ± 6.9%, p  50% and controls (sensitivity: 87%, specificity 79%, AUC 0.88, p < 0.001). Children with NCC exhibit a deformation pattern characterized by decreased apical circumferential strain, which may serve as a potential diagnostic tool for NCC. The role of decreased global LV longitudinal and basal circumferential strain should be further evaluated as a potential prognostic tool

Traditional Jones criteria: limitation in the diagnosis of rheumatic fever in patients with mitral valve repair

The present study aims to determine the limitations of traditional Jones criteria during the first episode of acute rheumatic fever (ARF) at the initial referral hospital, in a cohort of patients below 18 years old who had undergone mitral valve repair in National Heart Institute (IJN) from 2011 to 2016. Carditis followed by fever and joint involvement were the most frequent manifestations at first diagnosis. Of the 50 patients, only seven (14%) fulfilled the traditional Jones criteria for the diagnosis of the first episode of ARF. When compulsory evidence of a previous group A Beta hemolytic streptococcus (GABHS) was disregarded, this figure rose to 54%. Therefore, strict adherence to Jones criteria with absolute documentation of GABHS will lead to underdiagnoses of ARF. The application of echocardiographic diagnostic criteria of rheumatic heart disease (RHD) needs to be emphasized to allow early diagnosis and administration of secondary prophylaxis to prevent progression to severe valvular disease

Left ventricular rotational mechanics in children after heart transplantation

Background: Left ventricular (LV) dysfunction after orthotopic heart transplantation (OHT) is multifactorial and can be an indicator of graft rejection or coronary artery vasculopathy. Analysis of rotational mechanics may help in the early diagnosis of ventricular dysfunction. Studies describing the left ventricular rotational strain in children after OHT are lacking. It is important to establish the baseline rotational mechanics in pediatric OHT to pursue further studies in this population. Methods and Results: Rotational strain measured by speckle tracking was compared in 32 children after OHT, with no evidence of active rejection or coronary artery vasculopathy with 35 age-matched normal controls. Twelve OHT patients and 13 controls underwent moderate exercise with pre- and postexercise echocardiography. Torsion, slope of the systolic limb of the torsion–radial displacement loop, and the untwist rate were significantly higher in OHT patients (torsion: median 2.7°/cm [Q1–Q3, 2.3–3.2] versus 2.3°/cm [Q1–Q3, 1.9–2.7]; P=0.03, torsion–radial displacement loop: 2.7°/mm [Q1–Q3, 2.1–3.6] versus 2.0°/mm [Q1–Q3, 1.6–2.7]; P=0.008, indexed peak untwist rate: −21.6°/s/cm [Q1–Q3, −24.3 to −15.7] versus −17.1°/s/cm [Q1–Q3, −19.6 to −13.3]; P=0.01). Contrary to controls, OHT recipients were unable to increase torsion with exercise (OHT: 2.8°/cm [2.7–3.2] versus 3°/cm [2.4–3.5]; P=0.81, controls: 2.2°/cm [2–2.6] versus 3°/cm [2.4–3.7]; P=0.01, pre and post exercise, respectively). The systolic slope of the torsion–radial displacement loop relationship decreased with exercise in most OHT patients. Conclusions: Baseline rotational strain in OHT patients is higher than normal with a blunted response to exercise. The slope of torsion–radial displacement loop, and its response to exercise, may serve as a marker of left ventricular dysfunction in OHT patients

Reversible posterior leukoencephalopathy syndrome in post streptococcal glomerulonephritis

Reversible Posterior Leukoencephalopathy syndrome (RPLS) is a uncommon neurological disorder among pediatric population. Clinical features started with decreased alertness and activity, and associated with headache, visual changes, altered mental status such as seizures, confusion and abnormal behavior. Radio imaging findings commonly present typical changes in the white matter located in the posterior regions of the cerebral hemisphere and cerebellum. This syndrome commonly associated with hypertension, ecclampsia, renal failure or the used of some immunosuppression medications especially cyclosporine and tacrolimus. We describe a previously healthy 9-year-old boy who presented with acute post-streptococcal glomerulonephritis, and he developed neurological symptoms of posterior leukoencephalopathy syndrome with hypertensive crisis. His CT scan shows typical changes of non enhancing white matter with hypodensities at the occipital and parietal temporal areas. He had a rapid resolution of neurological symptoms with adequate treatment of hypertension. He was discharged well after 14 days in the ward

Diversity of respiratory viruses detected among hospitalized children with acute lower respiratory tract infections at Hospital Serdang, Malaysia

Background: The role of respiratory viruses as the major cause of acute lower respiratory tract infections (ALRTIs) in children is becoming increasingly evident due to the use of sensitive molecular detection methods. The aim of this study was to use conventional and molecular detection methods to assess the epidemiology of respiratory viral infections in children less than five years of age that were hospitalized with ALRTIs. Methods: The cross-sectional study was designed to investigate the occurrence of respiratory viruses including respiratory syncytisl virus (RSV), human metapneumovirus (HMPV), influenza virus A and B (IFV-A and B), parainfluenzavirus 1, 2, 3 and 4 (PIV 1, 2, 3 and 4), human rhinoviruses (HRV), human enterovirus (HEV), human coronaviruses (HCoV) 229E and OC43, human bocavirus (HBoV) and human adenovirus (HAdV) in hospitalized children with ALRTIs, at Hospital Serdang, Malaysia, from June 16 to December 21, 2009. The study was also designed in part to assess the performance of the conventional methods against molecular methods. Results: Viral pathogens were detected in 158 (95.8%) of the patients. Single virus infections were detected in 114 (67.9%) patients; 46 (27.9%) were co-infected with different viruses including double-virus infections in 37 (22.4%) and triple-virus infections in 9 (5.5%) cases. Approximately 70% of samples were found to be positive using conventional methods compared with 96% using molecular methods. A wide range of respiratory viruses were detected in the study. There was a high prevalence of RSV (50.3%) infections, particularly group B viruses. Other etiological agents including HAdV, HMPV, IFV-A, PIV 1-3, HBoV, HCoV-OC43 and HEV were detected in 14.5, 9.6, 9.1, 4.8, 3.6, 2.4 and 1.8 percent of the samples, respectively. Conclusion: Our results demonstrated the increased sensitivity of molecular detection methods compared with conventional methods for the diagnosis of ARTIs in hospitalized children. This is the first report of HMPV infections in Malaysia

Predictors of acute neurological complication following tetralogy of Fallot operation in Serdang Hospital, Malaysia

Background: The long waiting time for Tetralogy of Fallot (TOF) operation may potentially increase the risk of hypoxic insult. Therefore, the objective of this study is to determine the frequency of acute neurological complications following primary TOF repair and to identify the peri-operative risk factors and predictors for the neurological sequelae. Methods: A retrospective review of the medical and surgical notes of 68 patients who underwent TOF repair in Hospital Serdang, from January 2013 to December 2017 was done. Univariate and multivariate analyses of demographics and perioperative clinical data were performed to determine the risk for the development of acute neurological complications (ANC) among these patients. Results: ANC was reported in 13 cases (19.1%) with delirium being the most common manifestation (10/68, 14.7%), followed by seizures in 4 (5.9%) and abnormal movements in two patients (2.9%). Univariate analyses showed that the presence of right ventricular (RV) dysfunction, prolonged duration of inotropic support (≥7 days), prolonged duration of mechanical ventilation (≥7 days), longer length of ICU stays (≥7 days), and longer length of hospital stay (≥14 days), were significantly associated with the presence of ANCs (p0.05). The predictors for the development of postoperative delirium were pre-operative oxygen saturation less than 75% (Odds Ratio, OR=16.90, 95% Confidence Interval, 95%CI:1.36, 209.71) and duration of ventilation of more than 7 days (OR=13.20, 95%CI: 1.20, 144.98). Conclusion: ANC following TOF repair were significantly higher in patients with RV dysfunction, in those who required a longer duration of inotropic support, mechanical ventilation, ICU and hospital stay. Low pre-operative oxygen saturation and prolonged mechanical ventilation requirement were predictors for delirium which was the commonest neurological complications observed in this study. Hence, routine screening for delirium using an objective assessment tool should be performed on these high-risk patients to enable accurate diagnosis and early intervention to improve the overall outcome of TOF surgery in this country