Delayed presentation of Wilkie's syndrome after scoliotic curve correction surgery: a case report.

BACKGROUND: Superior mesenteric artery (SMA) syndrome, also known as Wilkie's syndrome, is a rare but serious complication following scoliosis correction surgery. It occurs as a result of mechanical compression of third part of duodenum between the SMA and aorta. This condition occurs most commonly in significantly underweight patients with deformities, and usually during the first week following spinal deformity corrective surgeries. The angle between the abdominal aorta and the SMA gets reduced following spinal lengthening during deformity correction surgery causing compression of third part of duodenum resulting in development of SMA syndrome. CASE PRESENTATION: We present a case of 17-year-old male with congenital scoliosis with a 70-degree scoliotic curve who underwent spinal deformity correction surgery with posterior instrumented fusion. Post-operative course was uneventful and the patient was discharged after suture removal on post-operative day 15. The patient presented after 21-days of symptom onset on post-operative-day 51, with a 3 week history of post-prandial vomiting, abdominal pain and distension which resulted in rapid weight loss of 11 kg. A CT-angiogram showed obstruction at third part of duodenum. After reviewing clinical and radiological profile of the patient, a diagnosis of SMA syndrome was made. Conservative management was tried, but due to rapid deterioration of patient condition and symptoms of complete intestinal obstruction, the patient was treated surgically by gastro-jejunostomy and side-to-side jejuno-jejunostomy, which improved his condition. CONCLUSION: SMA syndrome can occur much later than previously reported cases and with potentially life-threatening symptoms following scoliosis correction. Having a high index of suspicion, early recognition of condition and institution of appropriate treatment are essential to prevent occurrence of severe complications including risk of intestinal perforation and mortality. This case highlights management of delayed onset of SMA syndrome, with presentation further delayed after symptom onset, as is common in developing parts of the world, due to limited availability and accessibility of resources, and low socio-economic status of large segments of the population.

Diagnostic Utility of GATA3 and ISL1 in Differentiating Neuroblastoma From Other Pediatric Malignant Small Round Blue Cell Tumors.

Accurate diagnosis of neuroblastoma may be challenging, especially with limited or inadequate specimen and at the metastatic sites due to overlapping imaging, histopathologic, and immunohistochemical (immunohistochemistry [IHC]; infidelity among various lineage-associated transcription factors eg FLI1, transducin-like enhancer 1, etc) features. GATA3 and ISL1 have recently been described as markers of neuroblastic differentiation. This study aims at determining the diagnostic utility of GATA3 and ISL1 in differentiating neuroblastoma from other pediatric malignant small round blue cell tumors.We evaluated GATA3 and ISL1 expression in 74 pediatric small round blue cell tumors that included 23 NMYC-amplified neuroblastomas, 11 EWSR1-rearranged round cell sarcomas, 7 SYT::SSX1-rearranged synovial sarcomas, 5 embryonal rhabdomyosarcomas, 10 Wilms tumors (nephroblastomas), 7 lymphoblastic lymphoma, 7 medulloblastoma, and 4 desmoplastic small round cell tumor.All 23 neuroblastomas (moderate to strong staining in >50% of the tumor cells), 5 T-lymphoblastic lymphomas (moderate to strong staining in 40%-90% of the tumor cells), and 2 desmoplastic small round cell tumors (weak to moderate staining in 20%-30% of the tumor cells) expressed GATA3, while other tumors were negative. ISL1 immunoreactivity was observed in 22 (96%) neuroblastomas (strong staining in in >50% of the tumor cells, n = 17; moderate to strong staining in 26%-50% of the tumor cells, n = 5), 3 embryonal rhabdomyosarcoma (moderate to strong staining in 30%-85% of the tumor cells), 1 synovial sarcoma (weak staining in 20% of the tumor cells), and 7 medulloblastoma (strong staining in 60%-90% of the tumor cells). Other tumors were negative. Overall, GATA3 showed 86% specificity, 100% sensitivity, and 90% accuracy for neuroblastoma, with a positive predictive value (PPV) and negative predictive value (NPV) of 77% and 100%, respectively. ISLI showed 72% specificity, 96% sensitivity, and 81% accuracy for neuroblastoma, with a PPV and NPV of 67% and 97%, respectively. After the exclusion of T-lymphoblastic lymphoma and desmoplastic small round cell tumors, GATA3 had 100% specificity, sensitivity, accuracy, and PPV and NPV for neuroblastoma. Similarly, in pediatric small round blue cell tumors, ISL1 had 100% specificity, sensitivity, accuracy, PPV, and NPV for neuroblastoma, after embryonal rhabdomyosarcoma, synovial sarcoma, and medulloblastoma were excluded. CONCLUSIONS: GATA3 and ISL1 may be valuable in the diagnostic work-up of neuroblastoma and may reliably be used to support the neuroblastic lineage of pediatric small round blue cell tumors. Furthermore, dual positivity helps in challenging scenarios, when there is equivocal imaging, overlapping IHC features, limited specimen, and the lack of facility for a molecular work up.

Multifaceted Spindle Cell/Sclerosing Rhabdomyosarcoma With Role of Immunohistochemistry in Avoiding Misdiagnosis: A Multi-Institutional Study of 45 Distinct Tumors.

Background. Spindle cell/sclerosing rhabdomyosarcoma is a rare neoplasm and has an aggressive clinical course. Because of its rarity, we performed a multi-institutional collaboration to comprehend the overarching clinical, histopathological, and immunohistochemical characteristics of a cohort of spindle cell/sclerosing rhabdomyosarcoma. Materials and Methods. Forty-five patients with spindle cell/sclerosing rhabdomyosarcoma were identified. Demographics, clinical, histopathological, and immunohistochemistry data were reviewed and recorded. Results. The patients' age ranged from 1 to 85 years with a male to female ratio of 1.2:1. There were 15 children/adolescents and 30 adults. Eighteen (40%) tumors were located in the head and neck region. Twenty-four (53%) tumors displayed a bimorphic cellular arrangement with hypercellular areas having short, long, and sweeping fascicular and herringbone pattern, and hypocellular areas with stromal sclerosis and associated hyalinized and/or chondromyxoid matrix. Histomorphological differentials considered were leiomyosarcoma, malignant peripheral nerve sheath tumor, fibrosarcoma, nodular fasciitis, liposarcoma, synovial sarcoma, sarcomatoid carcinoma, solitary fibrous tumor, dermatofibrosarcoma protuberans, and schwannoma. Six tumors exhibited marked stromal sclerosis. The myogenic nature was confirmed by immunohistochemistry. Positivity for at least one skeletal muscle-associated marker (MyoD1 and/or myogenin) was observed. Conclusion. Spindle cell/sclerosing rhabdomyosarcoma diagnosis can be challenging as a number of malignant spindle cell neoplasm mimic this entity. Thus a correct diagnosis requires immunohistochemical work up with a broad panel of antibodies. In view of rarity of this neoplasm, further studies on a large cohort of patients with clinical follow-up data are needed for a better understanding of this tumor.

Evaluation of programmed cell death ligand 1 expression in a contemporary cohort of penile squamous cell carcinoma and its correlation with clinicopathologic and survival parameters: A study of 134 patients.

OBJECTIVES: Penile squamous cell carcinomas (PCs) are rare malignancies with a dismal prognosis in a metastatic setting; therefore, novel immunotherapeutic modalities are an unmet need. One such modality is the immune checkpoint molecule programmed cell death ligand 1 (PD-L1). We sought to analyze PD-L1 expression and its correlation with various clinicopathologic parameters in a contemporary cohort of 134 patients with PC. METHODS: A cohort of 134 patients with PC was studied for PD-L1 immunohistochemistry. The PD-L1 expression was evaluated using a combined proportion score with a cutoff of 1 or higher to define positivity. The results were correlated with various clinicopathologic parameters. RESULTS: Overall, 77 (57%) patients had positive PD-L1 expression. Significantly high PD-L1 expression was observed in high-grade tumors (P = .006). We found that 37% of human papillomavirus (HPV)-associated subtypes and 73% of other histotype tumors expressed PD-L1, while 63% of HPV-associated tumors and 27% of other histotype tumors did not (odds ratio, 1.35; P = .002 when compared for HPV-associated groups vs all others). Similarly, PD-L1-positive tumors had a 3.61-times higher chance of being node positive than PD-L1-negative tumors (P = .0009). In addition, PD-L1 high-positive tumors had a 5-times higher chance of being p16ink4a negative than PD-L1 low-positive tumors (P = .004). The PD-L1-positive tumors had a lower overall survival and cancer-specific survival than PD-L1-negative tumors. CONCLUSIONS: Overall, PD-L1 expression is associated with high-grade and metastatic tumors. Lower PD-L1 expression is observed more frequently in HPV-associated (warty or basaloid) subtypes than in other, predominantly HPV-independent types. As a result, PD-L1 positivity, including higher expression, portends lower overall and cancer-specific survival. These data provide a rational for further investigating PD-L1-based immunotherapeutics in PC.

Evaluation of liver and splenic stiffness by acoustic radiation force impulse for assessment of esophageal varices.

BACKGROUND: In routine clinical practice, assessment of portal hypertension (PHT) among patients with liver cirrhosis is done by a upper gastrointestinal endoscopy (UGIE); however, its invasive nature limits its use. Recent advances in ultrasound imaging make it possible to evaluate the tissue stiffness of the liver and spleen reflecting the severity of underlying fibrosis. Liver stiffness and spleen stiffness can be used to predict the presence of esophageal varices/PHT among cirrhotic patients. AIM: To predict the presence or absence of esophageal varices by measuring the stiffness of the liver and spleen by ultrasonography (USG)-based acoustic radiation force impulse (ARFI). METHODS: This cross-sectional study included 90 subjects with liver cirrhosis. Liver and splenic stiffness were measured along with the USG abdomen, UGIE and aspartate aminotransferase to platelet ratio index (APRI). RESULTS: Liver and spleen stiffness were significantly higher in cirrhotic patients compared to chronic hepatitis B. The best cut-off value of liver stiffness (LS) obtained by the receiver operating characteristic (ROC) curve was 2.16 m/s for predicting esophageal varices (AUROC 0.78, p 0.0002). The best cut-off value of splenic stiffness (SS) obtained by the ROC curve was 3.04 m/s for predicting esophageal varices (AUROC 0.698, p 0.0274). When both LS and SS were taken together, the accuracy in predicting esophageal varices increased to 92.22%. An equation to predict "esophageal varices = (0.225 LS + 0.377SS) - 0.555" was derived. CONCLUSION: LS and SS values of ≥ 2.16 m/s and 3.04 m/s, respectively, predict esophageal varices independently; however, combined assessment is better with 92% accuracy.

Missed injuries associated with maxillofacial trauma.

Background: Maxillofacial trauma in polytrauma settings is often associated with multiple injuries both trivial and life threatening, and their timely detection is the mainstay of definitive trauma management for preventing mortality and morbidity. Emergency management of all the patients reporting to our maxillofacial unit is either done by our center or they have been managed at the peripheral health care facility and relatively stable patient is referred to us. Anecdotally, we found inadequacies in transport methods, diagnosis, and detection of associated injuries in the patients referred to us from the peripheral health care facility. To substantiate our finding, this observational study has been planned. Objective: To identify, diagnose, and document missed injuries associated with the maxillofacial trauma. Materials and Methods: All the trauma patients referred to the maxillofacial unit directly from the peripheral health care facility during the period of October 2017 to March 2019 were included in this study. Results: We observed a total of 270 patients having both pure maxillofacial trauma and patients having documented other injuries associated with maxillofacial injuries. In our maxillofacial unit, functioning as a secondary screen, head to toe clinical examination was performed to document any previously missed out injuries. Missed injuries diagnosed by us included spinal injuries, temporal bone fractures, fractures of the styloid process, and even head injury. Conclusion: Frequent reassessment of trauma patients at all levels of trauma care and training health care personnel particularly those at peripheral health care facility and those involved in prehospital care are pivotal in managing the trauma patients in most efficient manner.

A Comparative Study of the Effect of Tooth Mousse Plus Remin Pro and Fluor Protector Gel on Enamel Erosion: An In Vitro Study.

Background: The purpose of this in vitro study was to assess the effect of Tooth Mousse Plus, Remin Pro, and Fluor Protector Gel on enamel erosion, measuring mean weight loss after exposure to a demineralizing agent. Materials and methods: A total of 60 sound-extracted permanent incisors were sectioned and enamel specimens were randomly distributed to different groups. The initial weight of all specimens was registered. The samples were randomly divided into four groups (n = 30). Group I specimens were treated with tap water (control). Groups II, III, and IV were treated with Tooth Mousse, Remin Pro, and Fluor Protector Gel application. After that, specimens were placed all together in a plastic container with 6 mL of a soft drink and immersed for 8 minutes at room temperature, dried, and weighed. Specimens were weighed after each immersion period and mean weight loss was calculated. The data was analyzed for probability distribution using the Kolmogorov-Smirnov test. The intergroup comparison was done using a one-way analysis of variance (ANOVA) followed by post hoc analysis. Results: According to pairwise comparisons in post hoc analysis, the weight of specimens at baseline was significantly greater than the weight of specimens on day 12. The difference in the mean weight of the specimen from baseline to day 12 was 2.833 mg for group I, 2.367 mg for group II, 1.467 mg for group III, and 2.133 mg for group IV. Conclusion: Tooth Mousse Plus, Remin Pro, and Fluor Protector Gel have no significant effect on dental erosion. How to cite this article: Shukla K, Saxena A, Joshi J, et al. A Comparative Study of the Effect of Tooth Mousse Plus Remin Pro and Fluor Protector Gel on Enamel Erosion: An In Vitro Study. Int J Clin Pediatr Dent 2023;16(S-1):S57-S62.

Comparative yield of molecular diagnostic algorithms for autism spectrum disorder diagnosis in India: evidence supporting whole exome sequencing as first tier test.

BACKGROUND: Autism spectrum disorder (ASD) affects 1 in 100 children globally with a rapidly increasing prevalence. To the best of our knowledge, no data exists on the genetic architecture of ASD in India. This study aimed to identify the genetic architecture of ASD in India and to assess the use of whole exome sequencing (WES) as a first-tier test instead of chromosomal microarray (CMA) for genetic diagnosis. METHODS: Between 2020 and 2022, 101 patient-parent trios of Indian origin diagnosed with ASD according to the Diagnostic and Statistical Manual, 5th edition, were recruited. All probands underwent a sequential genetic testing pathway consisting of karyotyping, Fragile-X testing (in male probands only), CMA and WES. Candidate variant validation and parental segregation analysis was performed using orthogonal methods. RESULTS: Of 101 trios, no probands were identified with a gross chromosomal anomaly or Fragile-X. Three (2.9%) and 30 (29.7%) trios received a confirmed genetic diagnosis from CMA and WES, respectively. Amongst diagnosis from WES, SNVs were detected in 27 cases (90%) and CNVs in 3 cases (10%), including the 3 CNVs detected from CMA. Segregation analysis showed 66.6% (n = 3 for CNVs and n = 17 for SNVs) and 16.6% (n = 5) of the cases had de novo and recessive variants respectively, which is in concordance with the distribution of variant types and mode of inheritance observed in ASD patients of non-Hispanic white/ European ethnicity. MECP2 gene was the most recurrently mutated gene (n = 6; 20%) in the present cohort. Majority of the affected genes identified in the study cohort are involved in synaptic formation, transcription and its regulation, ubiquitination and chromatin remodeling. CONCLUSIONS: Our study suggests de novo variants as a major cause of ASD in the Indian population, with Rett syndrome as the most commonly detected disorder. Furthermore, we provide evidence of a significant difference in the diagnostic yield between CMA (3%) and WES (30%) which supports the implementation of WES as a first-tier test for genetic diagnosis of ASD in India.

Evaluation of Relationship between Body Mass Index (BMI) and Dental Development in the Children in Age-group of 6-13 years of Malwa Region: A Cross-sectional Study.

Aim: To evaluate the relationship between body mass index (BMI) and dental development in the children in age-group of 6-13 years of Malwa region. Materials and methods: A total of 250 orthopantomograms (OPGs) of children aged 6-13 years (130 males and 120 females) collected from the Department of Paediatric and Preventive Dentistry, Government College of Dentistry, Indore, Madhya Pradesh, India, who came for their routine dental treatment. The chronological age, height, and weight were recorded, followed by calculating the BMI of each patient using Centers for Disease Control and Prevention (CDC) growth charts. The dental age was calculated using Cameriere's method. The comparison of the dental and chronological age was done using Wilcoxon signed-rank test. Results: The dental age of underweight patients was significantly lesser than that of the normal, overweight, and obese patients (p-value of <0.05). The dental age of the obese patients were greatest and significantly greater than that of the underweight, normal, and overweight patients (p-value of <0.05). Conclusion: Dental age is significantly associated with the BMI of children aged 6-13 years. The dental age of obese and overweight children is significantly greater than the chronological age. Clinical significance: Predicting the stage of dental development and eruption periods in children with mixed dentition can help with the sequencing and timing of orthodontic, prosthodontic, and surgical procedures. How to cite this article: Selkari V, Saxena A, Parihar A, et al. Evaluation of Relationship between Body Mass Index (BMI) and Dental Development in the Children in Age-group of 6-13 years of Malwa Region: A Cross-sectional Study. Int J Clin Pediatr Dent 2023;16(2):333-337.

Association of Early Childhood Caries and Multiple Variable Factors in 3-6-year-old Children.

Introduction: Dental caries is a globally prevailing condition. It is a common finding in all age-groups, whether it is young children or adults. Caries not only affects the oral health of an individual, but also the overall health of the individual. Aims and Objectives: This article focuses on the association of ECC with BMI, SES status, maternal education, birth order, and number of siblings in age group of 3 to 6 year old children. Material and methods: The study was planned and conducted in the Department of Pedodontics and Preventive Dentistry, Government College of Dentistry, Indore, Madhya Pradesh, India. The study consisted of 200 samples, including both groups. Group I included 100 patients with ECC and group II included 100 patients caries free. Children of age 3-6 years were randomly selected and evaluated for ECC and parameters like weight, height, number of siblings, birth order, SES status, and mothers' education. Results: Body mass index (BMI) had no significant association with the occurrence of ECC. Statistical significant association was observed between the number of siblings and ECC. The "no caries" was significantly associated with "no sibling" or "one sibling". A significant association between SES status and ECC was observed. The upper and upper middle class had more number of caries free children, whereas the number of participants with ECC was significantly more in the upper lower class. There was a pronounced association between ECC and maternal education. Conclusion: Researches like these help us to broaden our aspects of understanding that caries is not caused by only one factor but a magnitude of factors. It's prevention should take into consideration not only the dietary habits but also on increasing awareness about importance of oral hygiene and how it can be affected by other social varients.This article focuses on the association of ECC with BMI, SES status, maternal education, birth order, and number of siblings in 3-6-year-old children. How to cite this article: Sahu P, Agrawal A, Jain D, et al. Association of Early Childhood Caries and Multiple Variable Factors in 3-6-year-old Children. Int J Clin Pediatr Dent 2023;16(1):42-47.

Late infantile form of multiple sulfatase deficiency with a novel missense variant in the SUMF1 gene: case report and review.

BACKGROUND: Multiple sulfatase deficiency (MSD) is a rare lysosomal storage disorder caused due to pathogenic variants in the SUMF1 gene. The SUMF1 gene encodes for formylglycine generating enzyme (FGE) that is involved in the catalytic activation of the family of sulfatases. The affected patients present with a wide spectrum of clinical features including multi-organ involvement. To date, almost 140 cases of MSD have been reported worldwide, with only four cases reported from India. The present study describes two cases of late infantile form of MSD from India and the identification of a novel missense variant in the SUMF1 gene. CASE PRESENTATION: In case 1, a male child presented to us at the age of 6 years. The remarkable presenting features included ichthyosis, presence of irritability, poor social response, thinning of corpus callosum on MRI and, speech regression. Clinical suspicion of MSD was confirmed by enzyme analysis of two sulfatase enzymes followed by gene sequencing. We identified a novel missense variant c.860A > T (p.Asn287Ile) in exon 7 of the SUMF1 gene. In case 2, a two and a half years male child presented with ichthyosis, leukodystrophy and facial dysmorphism. We performed an enzyme assay for two sulfatases, which showed significantly reduced activities thereby confirming MSD diagnosis. CONCLUSION: Overall, present study has added to the existing data on MSD from India. Based on the computational analysis, the novel variant c.860A > T identified in this study is likely to be associated with a milder phenotype and prolonged survival.

A novel case of two siblings harbouring homozygous variant in the NEUROG1 gene with autism as an additional phenotype: a case report.

INTRODUCTION: NEUROG1 gene is yet to be associated with a set of human phenotypes in the OMIM database. Three cases have previously been diagnosed with cranial dysinnervation due to biallelic variants in the NEUROG1 gene. This is the fourth and a novel report of a sibling pair harboring a homozygous variant in the NEUROG1 gene with autism as an additional phenotype. A brief review of the literature in conjunction with a genotype-phenotype correlation has been described. A potential hypothesis for the presence of the autistic phenotype in the present case has also been elucidated. CASE PRESENTATION: A female aged 6 years and 9 months born to endogamous and phenotypically healthy parents was diagnosed with global developmental delay, autism spectrum disorder, hearing loss, corneal opacity and no eye blinking. Her MRI of the brain revealed mild peritrigonal white matter hyperintensity, and MRI and CT scan of the temporal bones showed abnormal cranial nerves. The proband's younger sister, aged 4-years, was similarly affected. Whole exome sequencing was performed in the proband, which revealed a novel homozygous, likely pathogenic, truncating frameshift variant, c.228_231dup (p.Thr78ProfsTer122) in exon 1 of the NEUROG1 gene (ENST00000314744.4). Segregation analysis by Sanger sequencing showed the proband and her younger sister to be homozygotes and their parents to be heterozygous carriers. CONCLUSION: This is the fourth report across the globe with a variant identified in the NEUROG1 gene to be associated with cranial dysinnervation phenotype. An additional phenotype of autism in two female siblings was a novel observation. We provide a hypothetical framework which could explain the pleiotropic effect of a dysfunctional NEUROG1 protein leading to autism and posit it as a candidate for diagnosis of autism spectrum disorder with congenital cranial dysinnervation disorder.

SS18-SSX Expression in a Contemporary Cohort of Primary Renal Synovial Sarcoma: A Multi-Institutional Experience of Fourteen Patients.

Primary renal synovial sarcoma is a rare aggressive mesenchymal neoplasm of the kidney that accounts for less than 1% of renal sarcomas. Herein, we describe the clinicopathologic and molecular findings of 14 renal synovial sarcoma patients in one of the largest case series to date and to our knowledge, the only renal synovial sarcoma series to use novel SS18-SSX IHC. Clinicopathologic, IHC, molecular, management, and follow-up data were reviewed and analyzed. Macroscopically, the tumors had either homogeneous, tan-white, and solid (n = 10), variegated and solid (n = 3), or variegated and solid-cystic (n = 1) cut surfaces. Spindle cell (n = 10), round cell (n = 3), and round to epithelioid morphologies (n = 1) were observed. SS18-SSX IHC was positive in all 14 tumors (diffuse, n = 10; multifocal, n = 2; focal, n = 2). All the tumors harbored SS18::SSX1/2 gene rearrangement. Metastases to the liver, brain, and lung (n = 1); liver and bone (n = 1); liver and diaphragm (n = 1) were identified. Adjuvant chemotherapy was administered in 11/12 patients. Follow-up was available for 10 patients (time period range: 5 to 24 months). Four patients died of disease, and six patients are alive with no recurrence or metastasis. As SS18-SSX IHC showed an excellent concordance with the FISH results, this may reliably be used in the IHC panel of spindle/round cell sarcomas of the kidney and as a molecular surrogate for renal synovial sarcoma, particularly in a resource-limited setting. Also, the tumors with focal SS18-SSX expression had lower break apart signals in the FISH assay (19% and 23% in two tumors with focal SS18-SSX IHC positivity).

Reporting Trends, Practices, and Resource Utilization in Neuroendocrine Tumors of the Prostate Gland: A Survey among Thirty-Nine Genitourinary Pathologists.

Background. Neuroendocrine differentiation in the prostate gland ranges from clinically insignificant neuroendocrine differentiation detected with markers in an otherwise conventional prostatic adenocarcinoma to a lethal high-grade small/large cell neuroendocrine carcinoma. The concept of neuroendocrine differentiation in prostatic adenocarcinoma has gained considerable importance due to its prognostic and therapeutic ramifications and pathologists play a pivotal role in its recognition. However, its awareness, reporting, and resource utilization practice patterns among pathologists are largely unknown. Methods. Representative examples of different spectrums of neuroendocrine differentiation along with a detailed questionnaire were shared among 39 urologic pathologists using the survey monkey software. Participants were specifically questioned about the use and awareness of the 2016 WHO classification of neuroendocrine tumors of the prostate, understanding of the clinical significance of each entity, and use of different immunohistochemical (IHC) markers. De-identified respondent data were analyzed. Results. A vast majority (90%) of the participants utilize IHC markers to confirm the diagnosis of small cell neuroendocrine carcinoma. A majority (87%) of the respondents were in agreement regarding the utilization of type of IHC markers for small cell neuroendocrine carcinoma for which 85% of the pathologists agreed that determination of the site of origin of a high-grade neuroendocrine carcinoma is not critical, as these are treated similarly. In the setting of mixed carcinomas, 62% of respondents indicated that they provide quantification and grading of the acinar component. There were varied responses regarding the prognostic implication of focal neuroendocrine cells in an otherwise conventional acinar adenocarcinoma and for Paneth cell-like differentiation. The classification of large cell neuroendocrine carcinoma was highly varied, with only 38% agreement in the illustrated case. Finally, despite the recommendation not to perform neuroendocrine markers in the absence of morphologic evidence of neuroendocrine differentiation, 62% would routinely utilize IHC in the work-up of a Gleason score 5 + 5 = 10 acinar adenocarcinoma and its differentiation from high-grade neuroendocrine carcinoma. Conclusion. There is a disparity in the practice utilization patterns among the urologic pathologists with regard to diagnosing high-grade neuroendocrine carcinoma and in understanding the clinical significance of focal neuroendocrine cells in an otherwise conventional acinar adenocarcinoma and Paneth cell-like neuroendocrine differentiation. There seems to have a trend towards overutilization of IHC to determine neuroendocrine differentiation in the absence of neuroendocrine features on morphology. The survey results suggest a need for further refinement and development of standardized guidelines for the classification and reporting of neuroendocrine differentiation in the prostate gland.

Clinical and demographic comparison of celiac disease diagnosed during adulthood versus childhood and adolescence: A single-center experience.

Background and Aim: Celiac disease (CeD) is mainly reported from the northern and western parts of India. In central India, it is believed to be a disease of children, with limited data among adults diagnosed for the first time after the age of 18 years. Hence, we aimed to describe CeD's clinical and demographic features among adults and children/adolescents in central India. Methods: This is a retrospective analysis of a prospectively maintained database of all patients diagnosed for CeD from 2010 to 2019. The disease in adults was confirmed when symptoms developed for the first time after 18 years and had positive anti-transglutaminase antibodies with villous atrophy on duodenal biopsy. It was compared with pediatric patients with CeD diagnosed during the same time period. Results: Of the 170 patients diagnosed with CeD, 118 were adults and 52 were children or adolescents. The mean age of presentation of adult CeD was 37.3 ± 11.93 years, while in the pediatric and adolescent group it was 9.19 ± 5.4 years. Classical presentation with chronic, painless, small-bowel-type diarrhea was seen in 44.1% of adults compared to 57.7% in the pediatric age group. Among the adult patients, 55.9% presented with nonclassical symptoms, which included abdominal pain (40.7%) and weight loss (36.4%). The common presenting symptom in children other than diarrhea was weight loss (50%) and abdominal pain (34.6%). Conclusion: CeD is common in central India, with an increasing number of patients being diagnosed for the first time after 18 years of age and presenting more often with nonclassical symptoms.

Evaluation of symptoms along with depression, anxiety and stress levels of Sars-CoV-2 positive patients during the second wave in the city of Indore, Central India - a cross sectional study.

INTRODUCTION: The consequences of the second wave hitting India have drastically laid a huge impact on the mental state of patients. The second wave had proven to be far more dangerous and hence the psychological evaluation needed to be conducted to know the scenario of patients suffering from SARS-CoV-2. OBJECTIVE: This study was undertaken to evaluate the symptoms of SARS-CoV-2 patients along with the existing depression, anxiety and stress levels amongst them. MATERIAL AND METHODS: An observational, cross-sectional questionnaire-based survey was conducted among 351 patients infected with SARS-CoV-2 during the second wave in Indore, Central India. The questionnaire consisted of questions pertaining to socio-demographic characteristics, clinical signs and symptoms. Evaluation of depression, anxiety and stress levels were done by use of 21 item Depression, Anxiety, Stress Scale (DASS-21). RESULTS: The most common symptom amongst patients was cough (42.2%) followed by fever (40.2%). Sixty-nine (19.6%) patients were asymptomatic. Depression score was found to have significant, positive weak correlation with age (ρ-0.124, p-0.020, p value <.05). No significant difference was observed between the depression, anxiety and stress score of males and females. Based on the scores assigned to the responses, patients who tested positive were belonging to normal category with no diagnosed depression, anxiety or stress. CONCLUSION: The present study showed fever, cough, headache, weakness, and chest pain as the common sign and symptoms of COVID-19 during the second wave. There was a prevalence of low levels of anxiety, stress and depression amongst patients in Radha Saomi Covid Care Centre, Indore during the second wave.

Comparative evaluation of extrasinus versus intrasinus approach for zygomatic implant placement.

Edentulism, a common problem can occur either as a congenital defect or acquired later due to dental caries, periodontitis, as a consequence of aging, maxillofacial trauma or post-ablation in tumor resections. The rehabilitation of the missing teeth can be done using dental implants. To overcome the deficiency of available bone, processes like sinus augmentation with substituted bone graft and Le Fort I osteotomy with interpositional bone graft have been described in the literature. In order to overcome the associated limitations with these procedures, implants were designed that can be placed in specific anatomical areas like zygoma. This study aims to compare two different types of surgical approaches (Intrasinus vs Extrasinus) for the placement of zygomatic implants to treat atrophic maxilla. The placement of zygomatic implant through both extrasinus and intrasinus approaches were evaluated on the basis of different parameters and it was observed that postoperative pain and swelling was significantly found in intra sinus approach as compared to extra sinus approach. However, in the intranasal approach, poor patient compliance or low satisfaction rate was observed as compared to extra sinus approach. On the basis of the results of the study and post operative evaluation based on various criteria, it was concluded that extra sinus approach has got an edge over intra sinus approach.

The outcome of conventional open reduction approach vs endoscopic approach for orbital floor repair.

Introduction: Blunt trauma to the orbital rim is a frequent cause of both orbital fractures and damage to the surrounding facial bones and soft tissues. The inception of endoscopy and minimal invasive intervention has revolutionized the surgical treatment of blow out fracture of orbital walls. Thus, the present study was conducted to compare the outcome of conventional open reduction approach with endoscopic approach for orbital floor repair. Materials and method: The study included 10 patients with clinical and radiographic evidence of orbital floor fracture, divided randomly into two groups: Group I-operated using endoscopic approach; and Group II- operated using conventional external approach. All the patients were evaluated clinically preoperatively, immediate post-operative and after 1 month for Diplopia, Extrinsic ocular motility, Enophthalmos and Hypoglobus. Results: The study consisted of 40% females and 60% males, with mean age of Endoscopic and Conventional group being 27.20 ± 6.14 years and 27.60 ± 7.23 years respectively. In relation to diplopia, an insignificant difference was observed statistically. The mean change in enophthalmos and hypoglobus in endoscopic and conventional group showed a significant difference statistically (p < 0.001). Unrestricted ocular motility was present only in one case of each group and significantly increased to 5 cases in both the groups (p = 0.048). The mean duration of surgery in endoscopic and conventional group showed a significant difference statistically. Conclusion: Diplopia, hypoglobus and Enophthalmos correction was better achieved in endoscopic group as compared to conventional group. But duration of surgery was more in endoscopic group as compared to conventional group.

Solitary Fibrous Tumor of the Adrenal Gland.

OBJECTIVES: Solitary fibrous tumor (SFT) is a mesenchymal neoplasm that can arise at various anatomic locations. It is characterized by inv12(q13q13)-derived NAB2::STAT6 fusion, resulting in the nuclear expression of STAT6. Primary SFT of the adrenal gland is rare. We launched a multi-institutional collaboration to comprehend the overarching demographics, clinical and follow-up, macroscopic, microscopic, IHC, and FISH features of 9 patients with SFT of the adrenal gland. METHODS: We added a series of 9 patients to the collection of adrenal SFTs where the clinicopathologic parameters, including clinical presentation, imaging, histopathology, IHC, molecular profiles, and management and follow-up data, were analyzed comprehensively. A modified 4-variable risk stratification model, including age, tumor size, and necrosis, was applied. RESULTS: Our series consisted of 6 male and 3 female patients, ranging in age from 19 to 64 years (mean, 49.3 years). Abdominal pain (4) and fever with abdominal pain (1) were the presenting symptoms in 5 patients. In the remaining 4 patients, the tumors were detected by abdominal imaging for hypertension and diabetes. The size of the tumor ranged from 2 cm to 10.5 cm in maximum dimension. All tumors exhibited the morphology of a spindle cell SFT with a patternless architecture; 3 had a focal storiform arrangement. STAT6 positivity was observed in all tumors, and 7 were positive for CD34. Surgical resection was the primary modality of treatment. No adjuvant therapy was administered. Follow-up ranging from 7 months to 23 months was available for 7 patients. All were alive without disease recurrence or metastasis. Risk stratification placed 8 (88.9%) patients into a low-risk category and 1 into an intermediate-risk category. CONCLUSIONS: This series is the largest of adrenal SFTs to date. These tumors of the adrenal gland are predominantly spindle cell neoplasms with indolent behavior, with a wide age distribution and a slight male preponderance. Combining our cohort with the previously published cases, the majority of tumors fall into the low-risk category for the propensity to develop metastases. Owing to the rarity and age distribution associated with these tumors, the differential diagnosis is wide and requires a systematic approach for ruling out key differential diagnoses aided by STAT6 IHC.