The need for improved management of status epilepticus in children in Australia: Time from seizure onset to treatment is consistently delayed.

AIM: To determine if the management of paediatric status epilepticus (SE) follows accepted clinical practice guidelines. METHODS: Retrospective, consecutive series of patients with SE who attended the emergency departments from two NSW sites over a 12-month period. SE was defined as a convulsive seizure, 5 min or more in duration. Time to presentation to the ED, time to first- and second-line treatment, number of benzodiazepine (BZD) doses given prior to intubation and adherence to guidelines were evaluated. The outcomes included seizure duration, need for respiratory support, admission to intensive care, morbidity and mortality. RESULTS: The time from onset of seizure to ED presentation was a median (p25-p75) time of 22 (15-40) min. Forty-eight of 59 presentations received pre-hospital midazolam. The median (p25-p75) time to first-line treatment was 15 (8-25) min and to second-line treatment was 43.5 (35-59) min. There was no significant difference in the results in the two hospitals. The total number of BZD doses ranged from 1 to 7 (median 3). There was non-adherence to the clinical practice guidelines in 55 (93.2%) of 59 presentations. CONCLUSIONS: We found excessive benzodiazepine use and delay in both definitive treatment of status epilepticus and in escalation from first- to second-line anticonvulsant treatment. This raises the need for rapid escalation of treatment. We propose a 'status epilepticus code' for emergency departments.

Management of status epilepticus in children prior to medical retrieval: Deviations from the guidelines.

AIM: To evaluate the influence of adherence to treatment guidelines on outcomes in children presenting with status epilepticus (SE) using the Newborn and Paediatric Emergency Transport Service, New South Wales prospective registry. METHODS: We reviewed the treatment of children with SE, transported by the Newborn And Paediatric Emergency Transport Service to a tertiary paediatric hospital, over 1 year. We evaluated variation in management from the New South Wales clinical practice guideline. RESULTS: There was excessive administration of benzodiazepines (BZD) and a delay in administration of appropriate second-line treatment of status (median 45 min). There was excessive use of BZD, with five or more doses of BZD associated with significantly higher odds for intubation. CONCLUSION: There is considerable scope to improve clinician compliance with the SE clinical practice guidelines.

Outcomes of deviation from treatment guidelines in status epilepticus: A systematic review.

OBJECTIVE: Due to a gap between published clinical guidelines on status epilepticus SE and clinician management of SE, a systematic review was performed to investigate treatment adherence to SE guidelines and its impact on patient outcomes. METHODS: Medline and Embase searches were conducted for studies appraising adherence to SE guidelines (from 1970 and 1st April 2018). The quality of eligible studies was assessed by QUADAS- 2 criteria. Comparison was made between patients where guidelines were followed and not followed. Various patient outcomes including intubation, ICU admission, morbidity and mortality were compared. A Forest plot was used to investigate the effect of adherence on outcome. RESULTS: A total of 3424 titles and abstracts were screened from the initial search after removal of duplicates. A total of 441 full text articles were reviewed in detail, and 22 articles were included in this study. The proportion of deviations ranged from 10.7% to 66.1%. Four studies were descriptive. Eighteen studies looked at the adverse effects of non-adherence. Eight studies showed respiratory depression and intubation were associated with excessive benzodiazepine use. A subset analysis showed 5.79 times higher odds of respiratory depression and intubation], if excessive benzodiazepines were given. The next most common variations were delayed management and insufficient treatment. These variations from the guidelines were associated with prolonged seizures. CONCLUSIONS: This review provides preliminary evidence that non-adherence to SE guidelines negatively impacts on patient outcomes. Appropriate and timely treatment is imperative for rapid seizure termination and improving outcomes.

Caring for children and adolescents with epilepsy: creating an innovative electronic educational resource.

The majority of children and adolescents with epilepsy are managed in the community setting by general paediatricians. However, there is a paucity of practical, relevant information and standardised management documents assisting paediatricians. In the era of sub-specialisation, an electronic educational resource developed by specialists in epilepsy and paediatrics enhances the interface between tertiary and secondary/primary care. We aimed to create a website (www.pennsw.com.au) designed to optimise the care of all children and adolescents living with epilepsy and to assess its clinical usefulness. The site provides clinicians and families an aligned resource, including key information on epilepsy syndromes, medication usage and adverse effects, safety (sleep deprivation, water sports), driving, pregnancy, psychosocial impact of epilepsy and coping skills. General paediatricians and carers completed a questionnaire, utilising rating scales and open ended questions, to evaluate design, content and clinical usefulness. Forty-nine general paediatricians with a median 12 years of paediatric practice participated. Thirty-two carers participated. Epilepsy syndrome was focal in 59% of the children and generalised in 41%. The majority of participants (paediatricians: 84-100%, families: 69-100%) rated the website as well designed, practical, informative and clinically useful. General paediatricians considered the "Medication" pages and "Epilepsy Management Documents" as practically useful. Carers recorded the "Family Resources", seizure recording documents, the support information on mental health, and the "Coping with Epilepsy" segment as most informative. General paediatricians and carers highly valued the website, reflected by 120,000 page views in 12 months since its launch.

Refractory otitis media: an unusual presentation of childhood granulomatosis with polyangiitis.

Childhood granulomatosis with polyangiitis (cGPA), previously known as Wegener's granulomatosis, is a rare, potentially fatal necrotizing vasculitis, the symptoms of which overlap with infection. We present a 16-year-old girl who, following 6 months of treatment for persistent middle ear effusion with progressive sensorineural hearing loss, developed rapidly progressing pneumonia, with pleural effusion, and multiple cavitatory lung lesions. Investigations demonstrated high titer c-ANCA and nasal septal biopsy confirmed the diagnosis of cGPA. This case highlights the difficulty in diagnosing cGPA and the potentially life-threatening consequences of failing to do so.

The association between maternal country of birth and neonatal intensive care unit outcomes.

BACKGROUND: Immigration is increasingly common worldwide and its impact on neonatal intensive care unit outcomes is uncertain. AIMS: To determine the outcomes of children of immigrant mothers admitted to NICUs in New South Wales (NSW), Australia, between 2000 and 2006. STUDY DESIGN: Record linkage study of routinely collected state-based health databases. SUBJECTS: Infants of Australian-born (9813, 81.9%) and overseas born mothers (2166, 18.1%). OUTCOME MEASURES: NICU and childhood outcomes to a maximum 5 years of age. RESULTS: Immigrant mothers came from 122 countries, 897 (44%) from high income regions. Australian born mothers were more likely to be teenaged (Odds Ratio, 95% confidence interval: 3.07, 2.21-4.26), use drugs (3.55, 2.49-5.06) and suffer an antepartum hemorrhage (1.29, 1.14-1.48). They were less likely to have gestational diabetes (0.45, 0.38-0.54), fetal distress (0.75, 0.66-0.85) and intrauterine growth restriction (0.80, 0.67-0.93). Their infants were more likely to be admitted to the NICU for prematurity but less likely to have low 5 min Apgar scores (0.81, 0.69-0.93) or a congenital abnormality (0.79, 0.70-0.90). Infants of Middle-Eastern mothers had the lowest hospital survival rate (88.5%). Children of immigrant Asian mothers were least likely to be rehospitalized after NICU discharge (1.66, 1.27-2.17). CONCLUSIONS: NICU outcomes are affected by maternal country of birth even within the same ethnic group. Further study regarding the impact of paternal race and immigration status and duration of residency will provide data for the changing cultural environment of global perinatal care.

Clinical profile of Langerhans Cell Histiocytosis at a tertiary centre: a prospective study.

OBJECTIVE: To study the varied presentations of Langerhans Cell Histiocytosis (LCH), the differential diagnosis of the varied presentations and the time lag in achieving the diagnosis. Prospective analysis of children diagnosed to have LCH over a period of 51 mo was done. A complete history and physical examination was undertaken in all patients, followed by relevant laboratory and radiological evaluation. Biopsy of the appropriate specimen was done. The extent of the disease was documented, accordingly treated and followed up. RESULTS: There were 16 children with LCH from October 2005 through December 2009. The age ranged from 8 mo to 72 mo. Diagnosis was confirmed by CD1a/S 100 in 15 children (93.75%). The mean time to arrive at the diagnosis was 9.9 mo. Multisystem disease was documented in 11 (68.75%) children and there were 4 (25.0%) cases of pulmonary LCH. The mean time of follow-up was 14.4 mo (range, 1 mo to 50.6 mo). Most common referral diagnoses in LCH patients was recurrent pneumonia and immunodeficiency. CONCLUSIONS: There is a need for high index of suspicion for diagnosis of LCH; misdiagnosis is frequent. Pulmonary involvement in children with LCH appears common. It is possibly still underdiagnosed. Nail changes are uncommon, but may act as a marker for multisystem disease. In addition to survival data and analysis of prognostic factors, the prospective collection of data on diverse presentations is essential, along with a high index of suspicion for the diagnosis of LCH.

Transfusion of blood and components in critically ill children.

The physicians prescribing transfusions must have a thorough understanding of the various blood products, their indications and contraindications, and requirements for modification of the blood products to prevent probable adverse effects. Decision to give an RBC transfusion should not be based solely on Hb concentration, it should take in account high severity of illness; active bleeding; emergency surgery; etc. Using restrictive transfusion strategy of transfusion RBCs can decrease transfusion requirements without increasing adverse outcomes. In most circumstances, platelets should be maintained greater than 10×10(9)/L. Platelet counts greater than 20×10(9)/L are indicated for invasive procedures and greater than 50×10(9)/L for major surgeries or invasive procedures with risk of bleeding. Whenever possible, ABO-compatible platelets should be administered. Fresh frozen plasma should be transfused in multiple coagulation factor deficiencies, DIC with bleeding, replacement of rare single congenital factor deficiencies when specific concentrates are not available (e.g., protein C or factor II, V, X, XI, or XIII deficiency). During transfusion child should be monitored carefully.

Communicating oesophageal duplication cyst with heterotopic pancreatic tissue - an unusual cause of recurrent pneumonia in an infant.

UNLABELLED: Communicating oesophageal duplication cyst with heterotopic pancreatic tissue is rare congenital anomaly and unusual cause of recurrent pneumonia in children. We report a 10-month-old boy who presented with history, examination and investigations suggestive of aspiration pneumonia since birth. The imaging studies revealed a thin walled cavity communicating with the oesophageal lumen that was excised by surgery. Histopathology showed squamous epithelial lining of cyst with heterotopic pancreatic tissue. CONCLUSION: Communicating oesophageal cyst causing persistent signs and symptoms can be an unusual cause of recurrent pneumonia in an infant that can be diagnosed by further imaging studies.

School absenteeism among children and its correlates: a predictive model for identifying absentees.

OBJECTIVES: To determine the magnitude of absenteeism and its correlates and to develop a model to predict absenteeism in school children. DESIGNS: A cross-sectional study. SETTING: three government schools in Delhi. PARTICIPANTS: 704 students, aged 10 to15 years. METHODS: students were registered and interviewed using a pre designed questionnaire. The frequency and causes of school absenteeism were ascertained by school records, leave applications and one months recall. The factors were subjected to univariate analysis and a stepwise multiple logistic regression analysis and a predictive model was developed. RESULTS: The average absenteeism of a student over 6 months was 14.3±10.2 days (95% CI 13.5 -15.0). 48% children absented themselves for more than two days per month on an average. The main factors associated with school absenteeism were younger age, male sex, increasing birth order, lower levels of parental education and income, school truancy, school phobia and family reasons. The discriminating ability of the predictive model developed was 92.4% CONCLUSIONS: it is possible to identify potential absentees in school children.