Factors Affecting Quality of Life in Adolescent Siblings of Children With Autism Spectrum Disorder.

OBJECTIVE: To compare the quality of life (QoL) of adolescent siblings of children with autism spectrum disorder (ASD-Sibs) with siblings of typically developing children (TD-Sibs), and study the factors affecting the QoL. METHODS: Between 1 February, 2021 and 31 September, 2021, 40 children aged 10-18 years, whose sibling was suffering from ASD, were enrolled (Study group). 40 age- and sex-matched siblings of children with no clinically apparent neuro- developmental abnormality or behavioral problem were also enrolled (Control group). Severity of autism was assessed by using the childhood autism rating scale 2 (CARS-2) score. QoL was assessed by a validated version of the World Health Organi-zation Quality of Life questionnaire Brief version (WHO QoL BREF), and compared between cases and controls using Wilcoxon rank sum test. RESULTS: The mean (SD) age of study participants was 13.55 (2.75) years. The mean (SD) CARS-2 score of our sample was 35.78 (5.23). Mild to moderate autism was seen in 23 (57.5%) children, and 13 (32.5%) had severe autism. The median (IQR) QoL in ASD-Sibs was worse than TD-Sibs in physical domain (24 (19,26) vs 32 (29,32); P<0.001), psychological domain (22 (17,23) vs 25 (23,25); P<0.001), social domain (11 (8,12) vs 13 (11,14); P<0.001), and environmental domain (28 (26,31) vs 35 (31,35); P<0.001). Among the ASD-Sibs, severity of the sibling's ASD and the family's socioeconomic status were the only two factors significantly affecting one of the domains of QoL. CONCLUSION: The observed lower QoL score in adolescent siblings of children with ASD, more so in those whose siblings had more severe ASD, suggests the need for targeting the family as a unit while formulating plans for holistic management of children with ASD.

Practical Aspects of ASD Management-What Pediatricians Should Know.

The increasing prevalence of autism spectrum disorder (ASD) warrants higher levels of clinical attention to optimally manage children with ASD. There is mounting evidence that early intervention programs can help improve developmental functioning, maladaptive behaviors, and core ASD symptoms. The most thoroughly investigated and evidence-based therapies have been developmental, behavioral, and educational interventions mediated by either professionals or parents. Other commonly available interventions include speech and language therapy, occupational therapy, and social skills training. Pharmacological interventions, where needed, are used as an adjunct to treat severe problem behaviors and manage medical and psychiatric comorbidities. Complementary or alternative medicine (CAM) approaches have not proven to be of any benefit, and some of them may be harmful to the child. As the child's first point of contact, the pediatrician is well-positioned to effectively guide the families to therapies that are evidence-based and safe and also collaborate with various specialists to provide seamless, coordinated care for these children so as to improve their developmental outcomes and social functioning.

Antiseizure Medication Withdrawal in Seizure-Free Patients: What is New for the Pediatrician?

The American Academy of Neurology (AAN) has recently updated its practise advisory on antiseizure medication withdrawal. The recommendations have been formulated for pediatric as well as adult epilepsy, with emphasis on the risk factors for seizure relapse, occurrence of status epilepticus or death on drug withdrawal, and effect on quality of life in both age groups. We herein list the important aspects of the updated recommendations in pediatric epilepsy for the benefit of the general pediatricians. The full update is available at the AAN website.

Diagnosis and Management of Global Development Delay: Consensus Guidelines of Growth, Development and Behavioral Pediatrics Chapter, Neurology Chapter and Neurodevelopment Pediatrics Chapter of the Indian Academy of Pediatrics.

JUSTIFICATION: Global developmental delay (GDD) is a relatively common neurodevelopmental disorder; however, paucity of published literature and absence of uniform guidelines increases the complexity of clinical management of this condition. Hence, there is a need of practical guidelines for the pediatrician on the diagnosis and management of GDD, summarizing the available evidence, and filling in the gaps in existing knowledge and practices. PROCESS: Seven subcommittees of subject experts comprising of writing and expert group from among members of Indian Academy of Pediatrics (IAP) and its chapters of Neurology, Neurodevelopment Pediatrics and Growth Development and Behavioral Pediatrics were constituted, who reviewed literature, developed key questions and prepared the first draft on guidelines after multiple rounds of discussion. The guidelines were then discussed by the whole group in an online meeting. The points of contention were discussed and a general consensus was arrived at, after which final guidelines were drafted by the writing group and approved by all contributors. The guidelines were then approved by the Executive Board of IAP. Guidelines: GDD is defined as significant delay (at least 2 standard deviations below the mean with standardized developmental tests) in at least two developmental domains in children under 5 years of age; however, children whose delay can be explained primarily by motor issues or severe uncorrected visual/hearing impairment are excluded. Severity of GDD can be classified as mild, moderate, severe and profound on adaptive functioning. For all children, in addition to routine surveillance, developmental screening using standardized tools should be done at 9-12 months,18-24 months, and at school entry; whereas, for high risk infants, it should be done 6-monthly till 24 months and yearly till 5 years of age; in addition to once at school entry. All children, especially those diagnosed with GDD, should be screened for ASD at 18-24 months, and if screen negative, again at 3 years of age. It is recommended that investigations should always follow a careful history and examination to plan targeted testing and, vision and hearing screening should be done in all cases prior to standardized tests of development. Neuro-imaging, preferably magnetic resonance imaging of the brain, should be obtained when specific clinical indicators are present. Biochemical and metabolic investigations should be targeted towards identifying treatable conditions and genetic tests are recommended in presence of clinical suspicion of a genetic syndrome and/or in the absence of a clear etiology. Multidisciplinary intervention should be initiated soon after the delay is recognized even before a formal diagnosis is made, and early intervention for high risk infants should start in the nursery with developmentally supportive care. Detailed structured counselling of family regarding the diagnosis, etiology, comorbidities, investigations, management, prognosis and follow-up is recommended. Regular targeted follow-up should be done, preferably in consultation with a team of experts led by a developmental pediatrician/ pediatric neurologist.

Clinical profile and predictors for outcome in children presenting with Guillain-Barré syndrome.

INTRODUCTION: Acute Flaccid Paralysis (AFP) is a group of diverse clinical conditions with Guillain-Barré syndrome (GBS) as one of the most common cause. The aim of this study was to study the clinical features and predictors for the requirement of ventilation in children with GBS. MATERIALS AND METHODS: This is a prospective observational study done at a tertiary care hospital where all consecutive children less than 15 years who presented with AFP were enrolled. Demographic characteristics, symptomatology, and physical findings of those patients who were diagnosed with GBS were recorded using a pre-defined questionnaire. Univariate analysis was done to identify clinical variables associated with a higher requirement of ventilation. RESULTS: Of a total of 53 children with AFP enrolled in the study, a total of 30 patients were diagnosed with GBS. A total of 12 (40%) patients required ventilation, while five of these patients eventually died. The following variants of GBS were identified: AIDP (13/30), AMAN (12/30), and ASMAN (2/30). Lower limbs were affected in 97% of the patients, whereas upper limbs were affected in 83% of the patients. Deep tendon reflexes of the upper limb and lower limb were preserved in 56% and 7% of the patients, respectively. Presence of antecedent URTI was associated with a lower requirement of ventilation. Presence of bulbar palsy, lower upper limb power on presentation, and absence of deep tendon reflex in upper limbs were associated with a higher requirement of ventilation. CONCLUSION: GBS is an important cause of AFP in India with no significant difference between the variants in terms of frequency and prognosis. Simple physical findings can be used by primary care physicians to predict the requirement of higher levels of care.

Unusual imaging presentation of infantile atypical Kawasaki disease.

Kawasaki disease is a systemic medium vessel vasculitis of unknown etiology affecting children under 5 years of age. There are no specific diagnostic tests, and thus, the diagnosis of the disease is primarily made on the basis of clinical criteria. Unusual presentations of Kawasaki disease have been variably reported from different parts of the world. However, presentation of the disease in the form of peripheral thromboembolism and florid non-coronary aneurysms has rarely been described This report describes the imaging findings in infantile atypical Kawasaki disease with aneurysms of multiple medium-sized arteries, including coronary arteries, emphasizing the detection of clinically silent aneurysms in the disease.

An Unusual Presentation of Disseminated Histoplasmosis: Case Report and Review of Pediatric Immunocompetent Patients from India.

Histoplasmosis is a progressive disease caused by dimorphic intracellular fungi and can prove fatal. Usually, it is present in immunocompromised individuals and immunocompetent individuals in the endemic zones. We report an unusual presentation of progressive disseminated histoplasmosis. The patient in the present case report was immunocompetent child and had fever, bone pains, gradual weight loss, lymphadenopathy and hepatosplenomegaly. Disseminated histoplasmosis (DH) was diagnosed on microscopic examination and fungal culture of bone marrow, blood, skin biopsy and lymph node aspirate. The patient died on seventh day of amphotericin B. In the absence of predisposing factors and classical clinical presentation of febrile neutropenia, lung, adrenal and oropharyngeal lesions, the disease posed a diagnostic challenge. Progressive disseminated histoplasmosis in children can be fatal despite timely diagnosis and therapy. In India, disseminated histoplasmosis is seen in immunocompetent hosts. All the pediatrics immunocompetent cases from India are also reviewed.

Lawsonia inermis-mediated synthesis of silver nanoparticles: activity against human pathogenic fungi and bacteria with special reference to formulation of an antimicrobial nanogel.

Lawsonia inermis mediated synthesis of silver nanoparticles (Ag-NPs) and its efficacy against Candida albicans, Microsporum canis, Propioniabacterium acne and Trichophyton mentagrophytes is reported. A two-step mechanism has been proposed for bioreduction and formation of an intermediate complex leading to the synthesis of capped nanoparticles was developed. In addition, antimicrobial gel for M. canis and T. mentagrophytes was also formulated. Ag-NPs were synthesized by challenging the leaft extract of L. inermis with 1 mM AgNO3. The Ag-NPs were characterized by Ultraviolet-Visible (UV-Vis) spectrophotometer and Fourier transform infrared spectroscopy (FTIR). Transmission electron microscopy (TEM), nanoparticle tracking and analysis sytem (NTA) and zeta potential was measured to detect the size of Ag-NPs. The antimicrobial activity of Ag-NPs was evaluated by disc diffusion method against the test organisms. Thus these Ag-NPs may prove as a better candidate drug due to their biogenic nature. Moreover, Ag-NPs may be an answer to the drug-resistant microorganisms.

Facile Iodine-Catalyzed Michael Addition of Indoles to α,α'-Bis(arylmethylene)cyclopentanones: An Efficient Synthesis of E-2-(3-Indolylphenylmethyl)-5-phenylmethylenecyclopentanones.

Iodine-catalyzed reaction of indoles with α,α'-bis(arylmethylene)cyclopentanones afforded one diastereomer of the corresponding Michael adducts, namely, E-2-(3-indolylphenylmethyl)-5-phenylmethylenecyclopentanones, in a good yield. The products form a new group of indole derivatives.