Adolescent Health Academy Statement on the Care of Transgender Children, Adolescents, and Youth.

JUSTIFICATION: The transgender community has been long stigmatized, and discriminated against, and faces numerous mental and physical problems. Certain indicators of transgender personality appear during childhood and more often before puberty begins. This puts the onus on Pediatricians to identify and offer evidence-based care for their benefit. There is an urgent and deep-felt need to understand the medical, legal, and social aspects of the care of transgender children. Hence, Adolescent Health Academy decided to release a statement on the care of transgender children, adolescents, and youth. OBJECTIVES: To review the existing international and national guidelines and recommendations to formulate a statement for the Pediatricians on (a) terminologies and definitions; (b) legal status in India; and (c) implications for pediatric practice. PROCESS: A task force was convened by the Adolescent Health Academy as the writing committee to draft the guidelines. These were approved by all the members of the task force and the Executive Board of Adolescent Health Academy (2022). RECOMMENDATIONS: Gender identity develops in childhood and adolescence as a feeling of self, and it should be respected to mitigate gender dysphoria. The law permits transgenders the right of self-affirmation and it upholds their dignity in society. The transgender community is prone to victimization, and prejudice leading to a high risk of substance abuse, suicidal ideation, and mental health issues. Pediatricians are the primary care providers of children and adolescents including those with gender incongruence, so they should be abridged with gender-affirmative practices. Gender-affirmative care involves pubertal suppression, hormonal therapy, and surgery which should be done in conjugation with the social transition, by a gender-affirmative care team.

Opsoclonus-myoclonus-ataxia syndrome in children.

Opsoclonus-myoclonus-ataxia syndrome is a rare neuroimmunologic disorder typically presenting in previously healthy infants and toddlers. It is characterized by a clinical triad of (1) erratic saccadic intrusions; (2) myoclonus and/or ataxia; (3) behavioral features, typified by developmental plateauing, irritability and insomnia. About half of cases are associated with an underlying neuroblastoma and diagnostic imaging is essential once OMAS is suspected. A thorough workup, including serum, urine, and cerebrospinal fluid studies is critical to identify underlying biomarkers of OMAS itself or neuroblastoma. Historically, many children had relatively poor long-term outcomes, with residual neurologic and/or neuropsychiatry sequelae typical. More recent concepts have emphasized combined immunotherapy regimens that offer hope for better outcomes in children with this remarkable, challenging disease.

The Phenomenology of Tics and Tic-Like Behavior in TikTok.

BACKGROUND: Pediatricians and pediatric subspecialists worldwide have reported a marked increase in functional (conversion) disorders with tic-like behaviors during the COVID-19 pandemic. These patients often report frequent viewing of Tourette syndrome (TS) TikTok videos, suggesting disease modeling. We aimed to evaluate tic phenomenology in videos posted on TikTok. METHODS: The 100 most-viewed videos under #tourettes in TikTok were randomly assigned to two of three primary reviewers (<2 years independent practice), all pediatric neurologists specializing in movement disorders, for extraction and classification of tic phenomenology. Initial disagreements were solved by consensus. If not resolved, one of five senior reviewers (>2 years independent pediatric movement disorder practice) served as a tiebreaker. In addition, two primary and one senior reviewer rated each video on a Likert scale from 1 = "All the tics are typical of TS" to 5 = "None of the tics are typical of TS". Median scores and Spearman correlation between primary and senior reviewers were calculated. RESULTS: Six videos without tic-like behaviors were excluded. Most videos depicted coprophenomena (coprolalia: 53.2%; copropraxia: 20.2%), often with unusual characteristics. Frequently, videos demonstrated atypical phenomenology such as very strong influence by the environment (motor: 54.3%; phonic: 54.3%), aggression (19.1%), throwing objects (22.3%), self-injurious behaviors (27.7%), and long phrases (>3 words; 45.7%). Most videos portrayed atypical, nontic behaviors (median [IQR] Likert ratings: 5 [4-5]). Primary vs. senior rater scores demonstrated moderate agreement (r = 0.46; P < 0.001). CONCLUSIONS: TS symptom portrayals on highly viewed TikTok videos are predominantly not representative or typical of TS.

Selenium-rich mushrooms cultivation on a wheat straw substrate from seleniferous area in Punjab, India.

Intensive rice-wheat cultivation cycle in Northern belt of India in general and in the State of Punjab in particular results in large volumes of straw and other post-harvest residue annually. The agricultural area, bordering the districts of Nawanshahr and Hoshiarpur, is popularly known as the seleniferous belt of India. The agri-residues, generated in seleniferous region of this state, are observed to contain significantly high concentration of selenium (Se). The present study was aimed to evaluate the Se uptake by different mushroom species: Pleurotus sajor-caju, Pleurotus ostreatus, Pleurotus citrinopileatus, Agaricus bisporus, and Volvariella volvacea, cultivated on Se-rich wheat and paddy straw from the seleniferous region. Wheat (Pleurotus species and A. bisporus) and paddy straw (V. volvacea) was inoculated with the mycelium spawn and left for 7-20 days, depending on the species, to grow. Control mushrooms were grown analogously using the agricultural residues from non-seleniferous area of the State of Punjab. All fruiting bodies were collected and analyzed in triplicate. Se was quantified using inductively coupled plasma sector field mass spectrometry. The Se accumulation was high in all species under study, being the highest in A. bisporus (1396 µg/g vs. 46.8 µg/g in controls - dry weight) and V. volvacea (231 µg/g vs. 3.77 µg/g - dry weight). The observed biological efficiency and total yield for all mushroom species showed good and unaltered productivity in Se-rich conditions, if compared to the controls. The Se-rich mushrooms can be prospective Se-supplements sourcing and biofortified foods, providing readily bioavailable and accessible Se for the diets deficient of this biologically essential element.

Selenium uptake by edible oyster mushrooms (Pleurotus sp.) from selenium-hyperaccumulated wheat straw.

In an effort to produce selenium (Se)-fortifying edible mushrooms, five species of oyster mushroom (Pleurotus sp.), were cultivated on Se-rich wheat straw collected from a seleniferous belt of Punjab, India. Total selenium was analyzed in the selenium hyperaccumulated wheat straw and the fruiting bodies. Significantly high levels (p<0.0001) of Se uptake were observed in fruiting bodies of all mushrooms grown on Se-rich wheat straw. To the best of our knowledge, accumulation and quantification of selenium in mushrooms has hitherto not been reported with substrates naturally enriched with selenium. The results demonstrate the potential of selenium-rich agricultural residues as substrates for production of Se-enriched mushrooms and the ability of different species of oyster mushrooms to absorb and fortify selenium. The study envisages potential use of selenium-rich agricultural residues towards cultivation of Se-enriched mushrooms for application in selenium supplementation or neutraceutical preparations.

Mitochondrial disease in childhood: nuclear encoded.

Primary mitochondrial disorders are clinically and genetically heterogeneous, caused by an alteration(s) in either mitochondrial DNA or nuclear DNA, and affect the respiratory chain's ability to undergo oxidative phosphorylation, leading to decreased production of adenosine triphosphophate and subsequent energy failure. These disorders may present at any age, but children tend to have an acute onset of disease compared with subacute or slowly progressive presentation in adults. Varying organ involvement also contributes to the phenotypic spectrum seen in these disorders. The childhood presentation of primary mitochondrial disease is mainly due to nuclear DNA mutations, with mitochondrial DNA mutations being less frequent in childhood and more prominent in adulthood disease. The clinician should be aware of the pediatric presentation of mitochondrial disease and have an understanding of the myriad of nuclear genes responsible for these disorders. The nuclear genes can be best understood by utilizing a classification system of location and function within the mitochondria.

Selenium bioaccessibility and speciation in biofortified Pleurotus mushrooms grown on selenium-rich agricultural residues.

Cultivation of saprophytic fungi on selenium-rich substrates can be an effective means to produce selenium-fortified food. Pleurotus florida, an edible species of oyster mushrooms, was grown on wheat straw from the seleniferous belt of Punjab (India) and its potential to mobilize and accumulate selenium from the growth substrate was studied. Selenium concentration in biofortified mushrooms was 800 times higher compared with control samples grown on wheat straw from non selenium-rich areas (141 vs 0.17 µg Se g(-1) dry weight). Seventy-five percent of the selenium was extracted after in vitro simulated gastrointestinal digestion and investigation of the selenium molecular fractions by size exclusion HPLC-ICP-MS revealed that proteins and any other high molecular weight selenium-containing molecule were hydrolyzed to peptides and low molecular weight selenocompounds. Analysis of the gastrointestinal hydrolysates by anion exchange HPLC-ICP-MS showed that the bioaccessible selenium was mainly present as selenomethionine, a good bioavailable source of selenium, which accounted for 73% of the sum of the detected species. This study demonstrates the feasibility of producing selenium-biofortified edible mushrooms using selenium-rich agricultural by-products as growth substrates. The proposed approach can be used to evaluate whether selenium-contaminated plant waste materials harvested from high-selenium areas may be used to produce selenium-biofortified edible mushrooms based on the concentration, bioaccessibility and speciation of selenium in the mushrooms.

Update on nuclear mitochondrial genes and neurologic disorders.

The majority of primary mitochondrial disorders are due to nuclear gene mutations, not aberrations within the mitochondrial genome. The nervous system is frequently involved due to its high-energy demands. Many nonspecific neurologic symptoms may be present in mitochondrial disease; however, there are well-recognized red flags that should alert the clinician to the possibility of mitochondrial disease. There is an ever increasing number of nuclear gene mutations discovered that play a role in primary mitochondrial disease and its neurologic symptomatology. Neurologists need to be aware of the wide neurologic presentation, the red-flag symptoms, and the nuclear gene mutations involved in the pathophysiology of mitochondrial disease to diagnose and manage this patient population.