Pediatric Group Research Articles

Rare case of Chronic Myeloid Leukemia (chronic phase) in pediatric age group: Diagnostic and pathological perspective

Rare case of Chronic Myeloid Leukemia (chronic phase) in pediatric age group: Diagnostic and pathological perspective

Practice guideline of influenza vaccination and antiviral drugs use in children(2024 editon)

Influenza is the most common acute respiratory infectious diseases among children in China. The annual seasonal influenza outbreak has a significant impact on children's health, poses a significant disease and socioeconomic burden, and arouses a wide public concern. In recent years, a number of new influenza vaccines and influenza antivirals have been approved for use in children, expanding the choices for prevention and treatment of influenza. Pediatric Infection Group, Chinese Society of Infectious Diseases of Chinese Medical Association and Infection Group, Pediatric Expert Committee of National Health Commission Capacity Building and Continuing Education, and China Clinical Practice Guidelines Alliance Methodology Committee organized a multidisciplinary guideline development group (GDG) consisting of experts in pediatrics, infectious diseases,epidemiologists,health policy,and guideline methodology proposed eight key issues concerning influenza vaccination and antiviral use from a practice perspective. Through research question construction, evidence retrieval and synthesis, evidence appraisal and evidence-to-decision discussion, recommendations and implementation suggestions were formulated. This guideline is intended to provide guidance for pediatricians at different facility levels and healthcare providers engaged in vaccination services,and institutional professionals dealing with prevention and control of infectious diseases.

Prevalence of Keratoconus and Keratoconus Suspect among a sample of Iraqi Children with Refractive Errors: A Cross-Sectional Study

Background: Keratoconus (KC) is a chronic, bilateral, non-inflammatory degenerative condition with severe consequences. Objective: To assess the prevalence of KC and Keratoconus Suspect (KCS) in a pediatric population with astigmatic error ≥1 diopter (D) and non-axial myopia. Methods: Between March and October 2022, a cross-sectional study was done with a group of 6–18-year-old kids with an astigmatic error of ≥1 D or non-axial myopia who went to the pediatric ophthalmology clinic at Ibn Al-Haitham Teaching Eye Hospital in Baghdad, Iraq, for regular checkups. All patients got a thorough eye examination and corneal imaging using the Corneal Tomography System (CSO) Sirius equipment and Scheimpflug technology. Results: The majority of patients were females aged 110–14 years (55%). The frequencies of KC and SKC were 13.5 and 119.6%, respectively. The proportion of KC was substantially higher for those above the age of 14. Patients with KC exhibited considerably larger cylinder and axis measurements. Participants with inferior cones and an asymmetric Bow Tie exhibited greater rates of KC (51.2% and 29.4%, respectively). Except for Pachy-Thin and corneal volume mean values, all other metrics were considerably greater in KC and KCS patients than in control patients. Conclusions: The high prevalence of KC and KCS emphasizes the importance of this issue and the necessity for a systematic strategy for screening in pediatric age groups with refractive error.

Prevalence of pathogenic variants in cardiomyopathy-associated genes in acute myocarditis: a systematic review and meta-analysis

Abstract Background Acute myocarditis is an inflammatory condition that may precede the development of dilated or arrhythmogenic cardiomyopathy. Objectives To investigate the reported prevalence of pathogenic/likely pathogenic (P/LP) variants in cardiomyopathy-associated genes in patients with acute myocarditis. Methods For this systematic review and meta-analysis, we searched Pubmed and Embase databases on March 04, 2023. Observational studies evaluating the prevalence of P/LP variants in cardiomyopathy-associated genes in patients with acute myocarditis were included. Studies were stratified into adult and pediatric age groups and for the following scenarios: (a) complicated myocarditis (i.e., presenting with acute heart failure, reduced left ventricular ejection fraction, or life-threatening ventricular arrhythmias); (b) uncomplicated myocarditis. The study followed PRISMA guidelines. Results Of 732 studies identified, 8 met the inclusion criteria, providing data for 586 patients with acute myocarditis. A total of 89 P/LP variants in cardiomyopathy-associated genes were reported in 85 patients. In uncomplicated myocarditis the pooled prevalence was 4.2% (95% CI 1.8-7.4%, I2 1.4%), while in complicated myocarditis pooled prevalence was 21.9% (95% CI 14.3-30.5%, I2 38.8%) and 44.5% (95% CI 22.7-67.4%, I2 52.8%) in adults and children, respectively. P/LP variants in desmosomal genes were predominant in uncomplicated myocarditis (64%), while sarcomeric gene variants were more prevalent in complicated myocarditis (58% in adults and 71% in children). Conclusions Genetic variants are present in a large proportion of patients with acute myocarditis. The prevalence of genetic variants and the genes involved vary according to age and clinical presentation.Central Illustration

Endoscopic Epilepsy Surgery: Systematic Review and Meta-Analysis

AbstractEndoscopic epilepsy surgery is a fast emerging minimally invasive alternative to open surgery. The approach minimizes the extent of bone and brain resection and reduces surgical morbidity. This systematic review and meta-analysis sought to evaluate the favorable outcome of seizure improvement in patients undergoing endoscopic epilepsy surgery. The search was conducted by two independent researchers using PubMed and Web of Science until January 2023 to find studies reporting results of patients who underwent endoscopic epilepsy surgery. We extracted data on the clinical profile and outcomes of the patients from the eligible studies. Fifteen studies yielded 340 patients, of which 293 underwent endoscopic epilepsy surgery. The patient cohort consisted of 189 (55.6%) males. A total of 171 (58.3) patients had a favorable outcome of either Engel I or II or > 90% seizure control. Thirteen studies were included in our meta-analysis, and demonstrated improved seizure control after endoscopic epilepsy surgery, with a pooled seizure freedom rate of 58% (95% CI: 0.43–0.71, I2 = 77.1%, τ2 = 0.6836). Studies focusing on pediatric populations reported a higher proportion of positive outcomes, with a rate of 73.27% (95% CI: 62–82%, I2 = 0.0%). In comparison, mixed-age populations showed a lower success rate of 48% (95% CI: 32–65%, I2 = 79.0%). Furthermore, there was significant difference in treatment outcomes between the pediatric and mixed age groups (p = 0.014). The hypothalamic hamartomas (HH) patient population demonstrated a favorable outcome proportion of 61.71% (95% CI: 48.92–73.06%), with a moderate level of heterogeneity (I 2 = 62.9%, tau2 = 0.4266). Five patients developed postoperative complications, and there were three deaths. Our findings suggest that endoscopic epilepsy surgery is particularly effective in pediatric populations and among patients with HH, underscoring the importance of considering patient demographics and disease characteristics in clinical decision-making. The heterogeneity across studies necessitates cautious interpretation of the pooled results, advocating for tailored approaches in treatment planning. Prospective trials are required to establish class I evidence for the role of endoscopic epilepsy surgery compared with the recognized open surgical techniques.

Long-term safety of mepolizumab for up to ∼10 years in patients with severe asthma: open-label extension study

Objectives Long-term safety monitoring of mepolizumab is necessary to support real-world use for the treatment of severe asthma. This Long-Term Access Program assessed the safety and benefit:risk of mepolizumab in pediatric, adolescent, and adult patients with severe asthma. Materials and methods This was a multicenter, Phase IIIb safety, open-label extension study of multiple prior studies assessing mepolizumab in addition to standard of care (Aug 2015 − Aug 2022). Adults/adolescents (≥12 years of age) received mepolizumab 100 mg subcutaneously (SC) every 4 weeks until mepolizumab was commercialized. Pediatric patients (6–11 years of age) received mepolizumab 40 mg or 100 mg SC (bodyweight <40 or ≥40 kg, respectively) every 4 weeks. Safety was assessed every 4 weeks and benefit:risk every 12 weeks. Results Of the 514 patients enrolled, 57% were female and the mean age was 51.1 (standard deviation: 14.9) years; 24 (5%) patients were 6–17 years of age. Total cumulative mepolizumab exposure across all mepolizumab studies included in this analysis was 1500.59 patient-years; median exposure was 2.03 (range, 0.08 to 9.97) years. Overall, 37 (7%) patients experienced on-treatment serious adverse events (SAEs): 34/502 (7%) in the 100 mg SC group and 3/7 (43%) in the 40 mg SC pediatric group. Two patients experienced SAEs considered to be treatment-related by the investigator. Infections were the most common SAEs of special interest (9 [2%] patients). Physician-assessed benefit:risk of mepolizumab supported continued treatment over the study period. Conclusions This long-term safety analysis of mepolizumab was consistent with previous reports, with no emerging safety concerns; most patients had a favorable benefit:risk up to ∼10 years. Clinical trial identifier NCT00244686 (GSK ID 201956)

Survival of pediatric patients with ependymoma in a tertiary cancer center in Rio de Janeiro, Brazil

IntroductionEpendymoma is the third most frequent central malignant nervous system tumor in the pediatric age group. There is scarce data in the literature on survival of these patients, especially in upper and lower middle-income countries. We aimed to describe the clinical and demographic characteristics, treatment, and outcome of pediatric patients with ependymoma admitted to a public cancer hospital.MethodsRetrospective analysis of medical records of patients with ependymoma, admitted to the Pediatric Oncology department (0-20 years) during the period of 2000-2022. Data on patient, disease characteristics, and treatment were analyzed. Overall survival (OS) was calculated using the Kaplan-Meier method.ResultsSeventy-two patients were evaluated; median age at diagnosis was 6.5 years (range: 1-20), 63% were male, 54% of the tumors were in the posterior fossa (PF-EPN), 45% were classified as WHO grade 3, and 68% were operated on in other institutions before referral. Regarding treatment, 72% underwent radiotherapy and 33% of patients underwent chemotherapy. Almost 70% percent of the patients had relapses. The median follow-up time was 5.2 years (Range: 0,1-21,4). The OS in 5 years was 67%. Totally resected tumors had OS in 5 years of 88% (p: 0.028).ConclusionThe results achieved in this series show a survival gap between UMIC and HIC. Relapses occurred mainly in the first ten years and then reached a plateau, with the majority of patients experiencing endocrinological and neurological sequelae.

Epidemiological profile and clinical characteristics of ocular and periocular tumors in North and Central India.

To describe the epidemiological profile and clinical characteristics of ocular and periocular tumors in patients presenting to three tertiary care referral centers in North and Central India. Hospital-based consortium study. Retrospective, descriptive, observational study. A total of 3184 patients were diagnosed with 3557 ocular and periocular tumors over 11 years from 2010 to 2021. Of these, 2395 (67.33%) were benign, 84 (2.36%) were premalignant, and 1078 (30.30%) were malignant. The most common location was the ocular surface (n = 1294, 37.09%), followed by the eyelid (n = 1185, 33.97%), intraocular (n = 624, 17.88%), and orbit (n = 454, 13.01%). The most common tumors were retinoblastoma (n = 483, 13.57%), ocular surface squamous neoplasia (OSSN) (n = 301, 8.46%), and dermoid cyst (n = 167, 4.69%). In the pediatric age group, retinoblastoma was the most common tumor (n = 483, 13.57%), while in adults, it was OSSN (n = 301, 8.46%). The stage at presentation for malignant tumors was divided into in-situ (57.14%), local spread (8.16%), and metastasis (32.83%). For specific tumor locations, the stages were 78.83%, 17.51%, and 2.18%, respectively, for eyelid tumors; 51.76%, 27.05%, and 17.64%, respectively, for orbital tumors; 88.37%, 5.81%, and 5.19%, respectively for ocular surface tumors; and 35.71%, 14.15%, and 50.28%, respectively, for intraocular tumors. Identifying the epidemiological characteristics of ocular and periocular tumors will aid in early diagnosis and timely intervention. Intraocular tumors showed delayed diagnosis, advanced stages at presentation, and required patients to travel longer distances for treatment, indicating the need for strengthened diagnostic and treatment facilities to improve access to care.

Clinico-demographic profile of burns in the Middle-East and North-Africa (MENA) region: a systematic review and meta-analysis

Burn injuries remain a public health issue with risk of mortality and multiple post-burn morbidities. This article aims to systematically review and meta-analyse data about burns in the Middle-East and North-Africa (MENA) region. Methods Pubmed, Web of Science, Scopus, Global Index Medicus, Cochrane Library and Google scholar data were searched from their dates of inception till April 2024. Results One hundred and one studies were included in this review, representing data from twelve countries in the region. Data was sub-grouped into two groups: pediatric group and emergency room (ER) group. The pooled random-effect estimates were male gender 58.6% [95% CI 56.9–60.2%], accidental injury 91.1% [95% CI 85.9–94.5%], scald burn 50% [95% CI 46.7–53.4%], at home injury 79.9% [95% CI 76.7–82.8%], upper limbs involvement 51.7% [95% CI 46.8–56.6%], mean body surface area burned (BSAB) 17.2% [95% CI 14.69–19.84%], second-degree burns 56.5% [95% CI 49–63.8%], length of stay 11.18 days [95% CI 9.68–12.68%] and mortality rate 9.1% [95% CI 7.5–11%]. All included studies were of good quality. Conclusion This review shows scarcity of studies from many countries in the MENA region. These data will inform policy makers and burn specialists about the extent of this public issue in the region.

Comparative Analysis of Burn Injuries in Toddler and Preschool Children: Implications for Triage and Outcome Assessment.

This study aims to compare the demographic, clinical characteristics, and outcomes of burn injuries in toddler and preschool children, and to validate the American Burn Association (ABA) Burn Triage Decision Matrix in the Turkish pediatric population. A retrospective analysis was conducted on 684 pediatric burn patients (494 toddlers, 190 preschoolers) admitted to our burn center over a 5-year period. Variables including gender, burn etiology, burn area, depth, treatment modalities, complications, length of hospital stay, and mortality were analyzed. The performance of the ABA Burn Triage Decision Matrix was evaluated in both groups. Scalding was the predominant cause of burns in both groups, with a significant difference in the involvement of anterior trunk (p = 0.027). The mean Total Body Surface Area (TBSA) was comparable between the groups (p = 0.286). There was no significant difference in mortality rates (p = 0.385), treatment modalities, and complications. The ABA Burn Triage Decision Matrix demonstrated consistency in triaging the severity of burn injuries, with a notable discrepancy observed in the moderate risk category of toddler group. This study highlights the distinct characteristics and outcomes of burn injuries in different pediatric age groups. The ABA Burn Triage Decision Matrix's validation suggests its utility in enhancing triage accuracy and resource allocation in pediatric populations, especially in disaster-prone regions.

The management of isolated meniscal tears in skeletally immature children. An international expert consensus.

The prevalence and appreciation of meniscal tears in children have increased in both number and complexity. There is currently a paucity of high-quality evidence that can guide surgeons in treating skeletally immature patients with meniscal injuries. The aim of this study was to develop comprehensive recommendations for the management of isolated meniscal tears in skeletally immature children. An international, two-round, modified Delphi consensus was completed. Included 'experts' were identified as having an established adult knee practice, including children and either: (1) Faculty at an international paediatric knee conference, (2) Active members of complex national paediatric multi-disciplinary groups or (3) Members of faculty on recognised national/international instructional courses aimed at teaching the management of meniscal lesions to knee surgeons. The currently available literature was reviewed, and areas of poor quality, inconclusive or absent evidence were examined and formed the focus of the study. A threshold of 70% was used to define consensus for our study based on other similar Delphi consensus studies in the literature. A total of 43 experts (Round 1) and 41 experts (Round 2) took part in the Delphi study, including surgeons from Europe, the United States of America and South America. 34 statements were identified exploring three main domains-clinical assessment, management and complex tears (bucket handle, discoid and radial). Following Round 1, consensus was reached on 17 (50%) statements; subsequently, after completion of Round 2, consensus was reached on 28 (82%) statements, leaving six (18%) with no consensus. The areas of no consensus included investigation of painless clicking, the most sensitive clinical test for meniscal pathology, treatment of small radial tears (less than 1/3 width), ability to reduce chronic bucket handle tears and timing of surgery. This is the first modified Delphi consensus that provides evidence for surgeons treating skeletally immature children with isolated meniscal tears. A valuable level of consensus was reached on the assessment and management of simple and specialist meniscal tears. These consensus statements can both inform clinical practice and be used in the development of further high-quality research studies. Level V.

Trauma-induced coagulopathy across age pediatric groups: A retrospective cohort study evaluating testing and frequency.

Trauma-induced coagulopathy (TIC) is associated with negative outcomes. Pediatric TIC has been described most often in older children. Children undergo normal developmental hemostasis, but it is unknown how this process impacts the risk of TIC across childhood. To understand variations in coagulation testing and TIC across pediatric age groups. We evaluated testing patterns of coagulation studies at presentation and over the first 72 h of hospitalization by pediatric age group at a large, Level I trauma center, 2015-2020. The frequency of TIC was determined using published, age-specific reference ranges and controlling for injury severity. We performed subgroup analyses of those with isolated severe traumatic brain injury (TBI) and those who presented directly from the scene of injury. Data from 2409 pediatric patients were available; 333 patients had isolated severe TBI. Children <1 year were least likely to be tested for TIC at presentation and over the first 72 h, even among the most injured. Fibrinogen testing was uncommon, regardless of injury severity. TIC was common: 22% of patients had TIC at presentation and 35% by 72 h. Greater injury severity was associated with TIC. Children 1-4 and 5-9 years had a higher frequency of TIC at presentation and over 72 h compared to older children in the least injured cohort. We saw no difference in frequency of TIC between age groups in the subset with isolated severe TBI. Using age-specific criteria, patients most often met TIC criteria by INR/PT, followed by platelet count, and least commonly by aPTT. The presence of TIC was associated with in-hospital mortality (OR 4.10, 95% CI 2.06-8.17). Significant sampling bias exists in clinical data collection among injured children and adolescents. Contrary to previous reports and using age-specific TIC criteria, younger children are not at lower risk of TIC than older children when controlling for injury severity.

Contingent negative variation for assessment of neurological deficits in children with hypothyroidism

Objectives: Hypothyroidism is one of the most common endocrine disorders. Its effect on the central nervous system is more pronounced, especially in the pediatric age group. Despite receiving adequate thyroid hormone replacement therapy, several patients continue to suffer from neurological impairments including cognitive dysfunction. Contingent negative variation (CNV) is an event-related potential (ERP) that is considered as an indicator of cognitive function. In this study, CNV was recorded in children with hypothyroidism. To the best of our knowledge to date, there have been no studies of CNV in hypothyroidism. Materials and Methods: This prospective study included 52 children between 8 and 15 years of age who were newly diagnosed with hypothyroidism. Diagnosis of hypothyroidism was based on laboratory thyroid function tests. CNV ERP was recorded in the enrolled children at the time of diagnosis, 1-month, and 6-month follow-up. Initial CNV (iCNV) and late CNV (lCNV) amplitudes and latencies were recorded each time. Results: Although the amplitudes of iCNV and lCNV appeared to increase during follow-ups, the changes were not statistically significant (P &gt; 0.05). Similarly, there appeared to be a modest reduction in latencies of iCNV or lCNV during follow-up; however, these changes were not statistically significant either. Conclusion: Our study did not show any significant changes in neurophysiological parameters. This may be attributed to a shorter time period of follow-up of six months and a smaller sample size. There is a possibility that CNV parameters may show more pronounced changes after a prolonged duration of treatment.

Prognostic Value of Early Nerve Conduction Studies in Suspected Guillain-Barré Syndrome in Pediatric Age Group: An Observational Study

Prognostic Value of Early Nerve Conduction Studies in Suspected Guillain-Barré Syndrome in Pediatric Age Group: An Observational Study

Management of neonates at risk of early onset sepsis: a probability-based approach and recent literature appraisal : Update of the Swiss national guideline of the Swiss Society of Neonatology and the Pediatric Infectious Disease Group Switzerland.

This guideline, developed through a critical review of the literature, facilitates a probability-based approach to the management of neonates at risk of early onset sepsis. • Neonatal exposure to antibiotics is disproportionally high compared with the incidence of early onset sepsis with implications for future health and antimicrobial resistance. • A probability-based approach may facilitate a more balanced management of neonatal sepsis and antibioticstewardship.

Comparison of predictors of survival among fulminant myocarditis patients undergoing veno-arterial extracorporeal membrane oxygenation in the adult and pediatric populations

Background: Fulminant myocarditis (FM) is a potentially life-threatening disease that requires emergency care. Our study aims to explore clinical outcomes and predictors of survival when using veno-arterial extracorporeal membrane oxygenation (VA-ECMO) support for the treatment of FM in adult and pediatric patients to analyze differences between both populations. Methods: PubMed, Scopus, Web of Science, and Cochrane databases were searched for studies reporting the effect of VA-ECMO on patients diagnosed with fulminant myocarditis. Statistical analysis was performed using R version 4.2.2. Results: Forty-three studies were included in our analysis with a total of 1,268 patients. Survival rates were 65% and 71% among adult and pediatric patients, respectively. Patients who didn’t suffer from cardiac arrest prior to VA-ECMO had better chances of survival in both populations; adults (OR 0.44; P&lt;0.01) and pediatric (OR = 0.32; P= 0.006). Younger age was associated with higher survival among the adults (MD= -8.81; P&lt;0.01). Additionally, pre-ECMO LVEF was higher among survivors in the pediatric group (MD= 8.23; P&lt;0.01). Furthermore, no significant association was detected between sex, VA-ECMO duration, systolic blood pressure, lactate levels, and survival rates among both groups. Conclusion: Using VA-ECMO in patients with fulminant myocarditis can significantly improve survival outcomes, with improved prognosis observed with younger age among adults and absence of prior history of cardiac arrest in both groups.

Utility of cell index in the diagnosis of healthcare-associated ventriculitis and meningitis: an analytical cross-sectional study

BackgroundThe diagnosis of healthcare-associated ventriculitis and meningitis (HCAVM) can be complex because multiple factors confound the interpretation of cerebrospinal fluid (CSF) tests. The cell index (CI) may help in the diagnosis of HCAVM. It does not incur additional medical cost and it avoids delays from the turnaround time of CSF cultures. It is derived by calculating the ratio of CSF white blood cell (WBC) and red blood cell (RBC) divided by the ratio of peripheral WBC and RBC. This study aimed to evaluate the diagnostic utility of this parameter.MethodsAn analytic, observational, cross-sectional study was conducted at the University of the Philippines – Philippine General Hospital. All admitted pediatric and adult patients from 2015 to 2022 who underwent external ventricular drain (EVD) insertion for hydrocephalus secondary to intracranial hemorrhage (ICH), acute ischemic stroke, intracranial neoplasms, traumatic brain injury, or congenital hydrocephalus were screened. Records of patients fulfilling the inclusion criteria were then reviewed.ResultsA total of 363 patients underwent EVD insertion from 2015 to 2022. Of these, 161 were included in the study. Two-thirds (66.5%) were adults ≥ 19 years old whereas the remaining were pediatric patients 1 to < 19 years old. There were no patients < 12 months old as they fulfilled at least one exclusion criteria. Forty-nine of them were later confirmed to have HCAVM based on the CDC/NHSN criteria. A CI cut-off of ≥ 1.21 gave a maximum sensitivity of 30.6% and specificity of 86.4%. Receiver operating characteristic area under the curve (AUC-ROC) analysis was 0.585. Subgroup analysis by age showed sensitivity of 52.9% in the pediatric age group and 3.13% in adults. Subgroup analysis by neurologic indication showed sensitivity of 27.6% for ICH and 35.0% for neoplasms. Subsequent AUC-ROC analyses, however, showed that CI failed to adequately diagnose HCAVM in these subgroups.ConclusionsIn our population of neurologic patients who underwent EVD insertion, the cell index is not a reliable parameter in the diagnosis of HCAVM.

Collagenising gastritis

It is a rare condition defined histologically by superficial subepithelial collagen layer. Collagenous gastritis occurs in bimodal age distribution both paediatric and adults.Sub epithelial collagen distribution favours the body or fundus in paediatric age group and antrum in adults. Now present a case of collagenising gastritis in 50 year male who presented with vomiting to medical gastroenterology department.

DICER-1 Mutation Associated Sarcoma of Thyroid in An Adult Male

Abstract Introduction/Objective DICER-1 is an autosomal dominant tumor predisposition syndrome caused by germline DICER1 mutations. In the thyroid, it is associated with an early-onset multinodular goiter (MNG) and low-grade thyroid carcinomas. The aggressive variants thyroblastoma and poorly differentiated carcinoma are however seen in the pediatric age group. We report a case of DICER-1-associated (rhabdomyo) sarcoma without primitive components in an adult male. Methods/Case Report A 44-year-old healthy male with a family history of MNG, presented with a large left neck mass confirmed on imaging with largest 6.4cm, and extension into the mediastinum. FNA showed a malignant spindle cell- rich neoplasm with negative expression for Keratins, TTF-1, PAX-8, Calcitonin, INSM- 1 and SOX-10. The CEA and calcitonin were normal. The lymphoma was excluded by flow cytometry. The patient underwent a left thyroidectomy with central and left selective neck dissection. On gross examination, the left lobe of the thyroid was replaced by a tan- white, partially encapsulated solid mass. Microscopic examination revealed nests and sheets of ovoid to spindle- shaped cells with eosinophilic cytoplasm in fascicles with hypocellular areas with loose stroma. Foci of rhabdomyoblastic differentiation with straps cells, positive for desmin and myogenin were identified. Brisk mitotic activity and tumor necrosis were present. No primitive thyroid epithelium or blastemal component was identified. Tumor cells were negative for S100, SOX10 CD34, CD31, SMA, calponin, CD99, NKX2.2, SS18-SSX which excluded malignant peripheral nerve sheath tumor, angiosarcoma, leiomyosarcoma, Ewing’s sarcoma, and synovial sarcoma. Next-generation sequencing showed a DICER1 c.4517G&amp;gt;A (p.W1506) and c.5437G&amp;gt;C (p.E1813Q) mutation, both variants reported as germ-line variants. A final diagnosis of DICER-1-associated rhabdomyosarcoma was rendered. The patient underwent rehabilitation at another facility and lost to follow-up. Results (if a Case Study enter NA) NA Conclusion The case highlights the expanding spectrum of DICER-1-associated malignant tumors and the importance of keeping it in the differential diagnosis of unusual malignant tumors of the thyroid even in adults.

Diving deep into lupus: Gastrointestinal involvement insights from the Oman lupus study.

This multicenter longitudinal study investigated the prevalence of gastrointestinal (GI) manifestations in lupus patients and determined the risk factors associated with mortality. This study is part of the Oman Lupus Study, which included 1160 patients who met the classification criteria for systemic lupus erythematosus (SLE) from January 2006 to February 2020. All patients were screened for GI symptoms and involvement. We identified 91 patients with GI manifestations, with a prevalence rate of 8.53% in the pediatric group and 7.75% in the adult group, and this difference was not statistically significant (p = .755). Ischemic colitis was significantly associated with longer disease duration (p < .001) and positivity for B2-glycoprotein I (B2GPI) autoantibodies (p < .0001). Moreover, a significant correlation was found between ischemic colitis and hematologic manifestations (p = .001), lupus nephritis (p = .007), pulmonary complications (p = .000-.039), and some cardiac complications (p = .012-.269). Mortality rates were greater in patients with GI involvement (24.37%), including those with ischemic colitis (p = .005), chronic peritonitis (p < .001), and spleen/liver infarction (p = .001). Sepsis, thrombocytopenia, and different internal organ involvement rates were significantly associated with increased mortality. This research provides significant insights into GI manifestations in lupus patients. A higher mortality rate was found to be associated with organ involvement, disease duration, autoantibody profile, and specific complications. Considering this fact, it is vital to prioritize management strategies to improve clinical outcomes in this group of patients.