Objectives Considering the dearth of literature on West syndrome (WS) from South Asian countries, this study aimed to evaluate the management practices in South Asia by an online survey and meta‐analysis. Methods An online questionnaire was sent to 223 pediatric neurologists/pediatricians in India, Pakistan, Myanmar, Sri Lanka, Bhutan, Nepal, and Bangladesh. Their responses were evaluated and supplemented by a meta‐analysis. Results Of 125 responses received (response rate: 56%), around 60% of responders observed male preponderance and an approximate lead‐time‐to‐treatment (LTTT) of 4‐12 weeks. The commonest etiology observed was a static structural insult (88.6% of responders). Most commonly used first‐line drug (country‐wise) was as follows: India—adrenocorticotropin hormone (ACTH, 50%); Pakistan—oral steroids (45.5%); Myanmar, Sri Lanka, and Nepal—oral steroids (94.4%); Bangladesh—ACTH (2/2); Bhutan—vigabatrin (3/5). ACTH and vigabatrin are not available in Myanmar and Nepal. The most commonly used regime for ACTH was maximal‐dose‐at‐initiation‐regime in India, Sri Lanka, and Bangladesh and gradually escalating‐regime in Pakistan. Maximum dose of prednisolone was variable—most common response from India: 3‐4 mg/kg/d; Pakistan, Bhutan, and Bangladesh: 2 mg/kg/d; Sri Lanka, Nepal, and Myanmar: 5‐8 mg/kg/d or 60 mg/d. The total duration of hormonal therapy (including tapering) ranged from 4 to 12 weeks (67/91). Most responders considered cessation of spasms for four weeks as complete response (54/111) and advised electroencephalography (EEG; 104/123) to check for hypsarrhythmia resolution. Difficult access to pediatric EEG in Bhutan and Nepal is concerning. More than 95% of responders felt a need for more awareness. The meta‐analysis supported the preponderance of male gender (68%; confidence interval [CI]: 64%‐73%), structural etiology(80%; CI 73%‐86%), longer LTTT (2.4 months; CI 2.1‐2.6 months), and low response rate to hormonal therapy(18% and 28% for ACTH and oral steroids respectively) in WS in South Asia. Significance This study highlights the practices and challenges in the management of WS in South Asia. These include a preponderance of male gender and structural etiology, a longer LTTT, difficult access to pediatric EEG, nonavailability of ACTH and vigabatrin in some countries, and low effectiveness of hormonal therapy in this region.
Background: NHS England commissioned four independent service providers to pilot low-calorie diet programmes to drive weight loss, improve glycaemia and potentially achieve remission of Type 2 Diabetes across 10 localities. Intervention fidelity might contribute to programme success. Previous research has illustrated a drift in fidelity in the design and delivery of other national diabetes programmes. Aims:(1) To describe and compare the programme designs across the four service providers; (2) To assess the fidelity of programme designs to the NHS England service specification. Methods:The NHS England service specification documents and each provider's programme design documents were double-coded for key intervention content using the Template for Intervention Description and Replication Framework and the Behaviour Change Technique (BCT) Taxonomy. Results:The four providers demonstrated fidelity to most but not all of the service parameters stipulated in the NHS England service specification. Providers included between 74% and 87% of the 23 BCTs identified in the NHS specification.Twelve of these BCTs were included by all four providers; two BCTs were consistently absent. An additional seven to 24 BCTs were included across providers. Conclusions:A loss of fidelity for some service parameters and BCTs was identified across the provider's designs; this may have important consequences for programme delivery and thus programme outcomes. Furthermore, there was a large degree of variation between providers in the presence and dosage of additional BCTs. How these findings relate to the fidelity of programme delivery and variation in programme outcomes and experiences across providers will be examined.
ObjectivesTo study the course of West syndrome (WS) and coronavirus disease-19 in children with WS who contracted SARS-CoV-2 infection. Methods This ambispective study was conducted at a tertiary-care center in North India between December 2020 and August 2021 after approval from the Institute Ethics Committee. Five children with WS, positive for COVID-19 based on RT-PCR, fulfilled the inclusion criteria. Results One child with COVID-19 during the first wave was retrospectively included while four children (of the 70 children screened) were prospectively enrolled. The median age at onset of epileptic spasms was 7 mo (2 boys), and that at presentation with COVID-19 was 18.5 mo. Three had underlying acquired structural etiology. Three were in remission following standard therapy, while two had ongoing spasms at the time of COVID-19 illness. During the illness, two of those in remission continued to be in remission while one child had a relapse. The children with ongoing epileptic spasms had variable course [one had persistent spasms and other had transient cessation lasting 3 wk from day 2 of COVID-19 illness, but electroencephalography (on day 8 of COVID-19 illness) continued to show hypsarrhythmia]. Fever was the most typical symptom (and sometimes the only symptom) of COVID-19, with a duration ranging from 1-8 d. Two children had moderate COVID-19 illness requiring hospitalization, while the rest had a mild illness. All the affected children had complete recovery from COVID-19. ConclusionThe severity of COVID-19 illness in children with WS is often mild, while the subsequent course of WS is variable.
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