Rickettsial Infection Diagnosed in a Clinical Context in Oman

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Intravenous Immunoglobulin in the Treatment of Vancomycin-Induced Toxic Epidermal Necrolysis

Toxic epidermal necrolysis (TEN), an uncommon but potentially life-threatening skin reaction, is frequently induced by drugs. The mucocutaneous reaction is characterised by bullous detachment of the epidermis and mucous membranes. We present a 9-month-old male with methylmalonic acidaemia, generalised hypotonia, and global developmental delay. He presented with a 3-day history of fever, cough, shortness of breath, and vomiting. Eruption appeared after 5 days of vancomycin treatment. The eruption involved almost 60% of the total body surface area and both eyes. He was successfully treated with intravenous immunoglobulin (IVIG), antibiotics,and appropriate wound management and made a full recovery with negligible sequelae despite the severity of his disease. Important components of successful treatment include early recognition, intensive care, prompt withdrawal of the causative agent, early administration of IVIG, appropriate fluid resuscitation, and control of infection. IVIG might be beneficial in the treatment of TEN; however, controlled studies are needed to evaluate IVIG compared to other modalities

Posterior Reversible Encephalopathy Syndrome in Two Omani Children with Underlying Renal Diseases

Posterior reversible encephalopathy syndrome (PRES) is a neurological condition with a combination of clinical and radiological features. Clinical symptoms include headaches, confusion, seizures, disturbed vision or an altered level of consciousness. Classic magnetic resonance imaging (MRI) findings indicate subcortical and cortical oedema, affecting mainly the posterior cerebral region. We report two paediatric cases of PRES with underlying renal diseases presenting at the Sultan Qaboos University Hospital in Muscat, Oman, in April 2010 and August 2011. The first case was an 11-year-old girl diagnosed with systemic lupus erythematosus and the second was a six-and-a-half-year-old boy on peritoneal dialysis due to multi-drug-resistant nephrotic syndrome. Both patients were hypertensive and treated with blood pressure control medications. No residual neurological dysfunction was noted in the patients at a one-year follow-up and at discharge, respectively. The role of hypertension in paediatric PRES cases, among other important risk factors, is emphasised. Additionally, MRI is an important diagnostic and prognostic tool. Prompt diagnosis and aggressive management is fundamental to preventing permanent neurological damage

Vascular Anomalies in Children Misdiagnosed with Asthma : Case series

In most asthmatic children, inhaled steroids can relieve and control the symptoms of asthma. Persistent wheezing and respiratory symptoms in young children despite appropriate treatment may indicate other diagnostic considerations. Delays in this diagnosis can result in unnecessary investigations, inappropriate treatment and further complications. We report three patients who presented to Sultan Qaboos University Hospital, Muscat, Oman, in the period between September 2010 and May 2012 with persistent wheezing due to compression of the trachea caused by vascular anomalies. All patients had double aortic arches putting pressure on the trachea, leading to respiratory manifestations and feeding problems. Following surgery, all cases showed improvement and no longer required medication. Without clinical suspicion and appropriate imaging, congenital vascular anomalies may remain undetected for years. Infants and children with chronic wheezing should be evaluated for vascular anomalies as soon as possible. General practitioners should refer all such patients to a tertiary-level hospital for further investigations and management

Semilobar Holoprosencephaly with Neurogenic Hypernatraemia : Two new cases

Holoprosencephaly (HPE) is a developmental defect of the embryonic forebrain and midface. It is due to the non-cleavage of the embryonic forebrain into two cerebral hemispheres and the incomplete development of the paramedian structures. The overall prevalence is 1.31 per 10,000 births. The aetiology could be genetic,environmental, or both. HPE is classified into alobar, semilobar, and lobar subtypes based on the degree of separation of the cerebral hemispheres. We report two new cases of semilobar HPE with neurogenic hypernatraemia. Lack of thirst and hypodypsia associated with chronic hypernatraemia in patients with HPE is highly suggestive of neurogenic hypernatraemia. Early identification of neurogenic hypernatraemia is important as it improves with forced fluid therapy and does not require any medication

Revisiting the Prevalence of Autism Spectrum Disorder among Omani Children: A multicentre study

Objectives: This study aimed to provide an updated estimate of the prevalence of autism spectrum disorder (ASD) among Omani children. Methods: This retrospective descriptive study was conducted from December 2011 to December 2018. Data were retrieved from the three main autism diagnostic centres in Oman: Sultan Qaboos University Hospital, Royal Hospital and Al-Massarah Hospital. The ASD diagnosis was made by experienced clinicians based on the fifth edition of the Diagnostic and Statistical Manual of Mental Disorders (DSM-5). The overall population prevalence estimates per 10,000 children aged 0–14 years old in Oman were calculated using the denominator of the mid-period population data. Results: A total of 1,705 ASD cases were identified with the majority of cases being male (78.1%). The overall prevalence rate of ASD was estimated at 20.35 per 10,000 children (95% confidence interval: 19.39–21.32) between 2012–2018. Boys were found to have a 3.4-fold higher prevalence of ASD than girls (31.23/10,000 versus 9.07/10,000). Regionally, the majority of cases were found in the capital, Muscat, where the highest prevalence was 36.51 cases per 10,000 children. Conclusion: The prevalence of ASD among Omani children is 15-fold higher than estimates from 2011. This increase can be attributed to improvements in diagnostic services, increased awareness of ASD, better screening programmes and changes in diagnostic criteria. In addition, this increase in prevalence suggests a need for a registry of developmental disabilities and more extensive diagnostic and rehabilitation services in Oman.Keywords: Autism Spectrum Disorder; Epidemiology; Prevalence; Oman

Transient Insulin Resistance in Propionic Acidaemia: Knowing is half the battle

Propionic acidaemia (PPA) is a disorder of amino acid and odd-chain fatty acid metabolism. Hypoglycaemia is a more commonly described finding rather than hyperglycaemia during metabolic decompensation of PPA. There is a high mortality rate in patients with organic acidaemias having severe insulin-resistant hyperglycaemia. We report a nine-month-old boy with PPA who was admitted to tertiary care hospital in Muscat, Oman, in 2018 with metabolic decompensation, persistent hyperglycaemia and transient insulin resistance. Hyperglycaemia did not respond to high insulin infusion. Plasma glucose only improved when glucose infusion rate (GIR) reached 7 mg/kg/min. The patient has full recovery and was discharged, with follow up plan. It is important to balance the GIR to achieve the targeted insulin level, beyond which the risks of hyperglycaemia start to outweigh the potential anabolic benefits of additional insulin secretion. Timely clinical attention should be given to achieve adequate caloric delivery through alternative sources other than high GIR to permit better glycaemic control, especially when insulin-resistant hyperglycaemia is present. Keywords: Propionic Acidaemia; Insulin Resistance; Infant; Case Report; Oman

Successful Management of a Neglected Case of Nephropathic Cystinosis

Cystinosis is a rare metabolic disorder characterised by lysosomal cystine accumulation leading to multi-organ damage; clinically, the kidneys are the first organ affected. Respiratory insufficiency caused by overall respiratory muscle myopathy is a life-threatening complication. Treatment with cysteamine should be initiated rapidly and continued lifelong to prolong renal function and protect the extra-renal organs. We report the case of a four-year-old Omani girl, diagnosed with infantile nephropathic cystinosis at 21 months. Cysteamine was prescribed but with no compliance to medications. She presented to the Child Health Department of Sultan Qaboos University Hospital, Oman, two years later with severe failure to thrive, electrolyte disturbance and respiratory failure. The hypoventilation and early respiratory dysfunction, due to intercostal and diaphragm myopathy, was treated by non-invasive positive-pressure ventilation. The patient was discharged after four months of intensive rehabilitation with no ventilator support. No standard treatment options have yet been established for respiratory dysfunction in cystinosis

Evidence-Based Practice : Knowledge, attitudes, practice and perceived barriers among nurses in Oman

Objectives: The aim of this study was to describe nurses’ practices, attitudes, knowledge/skills and perceived barriers in relation to evidence-based practice (EBP) in Oman. Methods: This descriptive cross-sectional study was conducted between February and November 2012. A self-reported 24-item questionnaire was used to measure EBP practices, attitudes and knowledge/skills among a convenience sample of 600 nurses working in four governmental hospitals in Muscat, Oman. Responses were scored on a one to seven rating scale. Barriers to EBP were measured on a five-point Likert scale using two subscales. Descriptive statistics and general linear regression were used to analyse the data. Results: A total of 414 nurses were included in the study. The greatest barriers to developing EBP among nurses were insufficient time for research (3.51 ± 0.97) and insufficient resources to change practices (3.64 ± 0.99). Nurses with more years of experience reported increased use of EBP (P <0.01), more positive attitudes towards EBP (P<0.001) and fewer barriers to research (P <0.01). Significant positive correlations were found between years of experience and practice (r = 0.16) and attitudes (r = 0.20). Nurses with a baccalaureate degree reported fewer barriers to research than those qualified at a diploma level (P <0.001). Nurses who perceived more barriers to research reported less use of EBP (P <0.001), less positive attitudes towards EBP (P <0.001) and limited EBP knowledge/skills (P <0.001). Conclusion: These findings provide a basis for enhancing nursing practices, knowledge and skills. Continuing education for nurses and minimising barriers is crucial to increasing the use of EBP in Oman.Keywords:

Pattern of Viral Infections among Infants and Children Admitted to the Paediatric Intensive Care Unit at Sultan Qaboos University Hospital, Oman

Objectives: The aim of this study was to describe the pattern of viral infections in infants and children admitted to the Paediatric Intensive Care Unit (PICU) at Sultan Qaboos University Hospital (SQUH) in Muscat, Oman. Methods: A retrospective review of patient records was carried out on all patients admitted to the PICU between January 2011 and December 2012. In order to detect viruses, polymerase chain reaction (PCR) technology was used to detect viruses in nasopharyngeal aspirates, tracheal aspirates, plasma, stool and urine samples. All infants and children below 13 years old, who were admitted to the PICU at SQUH during the study period and with confirmed viral infections, were included in the study. Results: A total of 373 infants and children were admitted to the PICU during the study period. Viruses were detected in 34 patients. The most frequently detected viruses were cytomegalovirus (CMV) in 29.4%; this virus was noted predominantly in immuncompromised patients (80%, P = 0.023) and was associated with increased mortality (50%, P = 0.031) and prolonged PICU stay (70%, P = 0.045). Fatalities before discharge were recorded in 23.5% of the patients. The most frequent risk factors for viral infections were an age of <12 months old (47.1%), assisted ventilation/intubation (52.9%) and a prolonged PICU stay (55.9%). Conclusion: The results of this study found that CMV was the most common viral infection among infants and children admitted to the PICU in SQUH. CMV was also the leading cause of mortality