scholarly journals Mucin 4: A Sensitive Diagnostic Marker for Pediatric Mucoepidermoid CarcinomaNick Shillingford

2020 ◽  
pp. 1-12
Author(s):  
Mackenzie Postel M S ◽  
◽  
Julia T Chu ◽  
Henry H Tran ◽  
Shamlal Mangray ◽  
...  

Background: Pediatric salivary gland-type neoplasms (SGTNs) pose a significant diagnostic problem due to histo-morphological heterogeneity. Previous reports have shown that Mucin 4 (MUC4) expression is associated with adult mucoepidermoid carcinoma (MEC). We hypothesize that MUC4 is also a sensitive marker for distinguishing MEC from other SGTNs in the pediatric population. Objective: To evaluate MUC4 expression in pediatric SGTNs. Methods: A retrospective review of 74 SGTNs diagnosed between 1993–2015 at Children’s Hospital Los Angeles, Boston Children’s Hospital, and Rhode Island Hospital was performed. H&E sections of 31 MECs were compared to 3 adenoid cystic carcinomas (AdCCs), 6 acinic cell carcinomas (AcCCs), 30 pleomorphic adenomas (PAs), 3 mammary analogue secretory carcinomas (MASCs), and one sialoblastoma (SB). Samples underwent immunohistochemical staining for MUC4, with expression score criteria: 0% positivity = 0, 1-10% = +, 11-50% = ++, 51-90% = +++, >90% = ++++. Results: All MECs were MUC4-positive, with 25 (80.65%) having an expression score ≥ +++. AdCCs and PAs demonstrated no to minimal MUC4-positivity. Subsets of AcCCs and MASCs were unexpectedly MUC4-positive. As a novel marker for pediatric MEC, MUC4’s sensitivity = 100%, specificity = 79.41%, positive predictive value = 75.86%, and negative predictive value = 100%. Conclusion: MUC4 is a sensitive marker for pediatric MEC

2008 ◽  
Vol 18 (2) ◽  
pp. 76-86 ◽  
Author(s):  
Lauren Hofmann ◽  
Joseph Bolton ◽  
Susan Ferry

Abstract At The Children's Hospital of Philadelphia (CHOP) we treat many children requiring tracheostomy tube placement. With potential for a tracheostomy tube to be in place for an extended period of time, these children may be at risk for long-term disruption to normal speech development. As such, speaking valves that restore more normal phonation are often key tools in the effort to restore speech and promote more typical language development in this population. However, successful use of speaking valves is frequently more challenging with infant and pediatric patients than with adult patients. The purpose of this article is to review background information related to speaking valves, the indications for one-way valve use, criteria for candidacy, and the benefits of using speaking valves in the pediatric population. This review will emphasize the importance of interdisciplinary collaboration from the perspectives of speech-language pathology and respiratory therapy. Along with the background information, we will present current practices and a case study to illustrate a safe and systematic approach to speaking valve implementation based upon our experiences.


2020 ◽  
Vol 7 (Supplement_1) ◽  
pp. S693-S694
Author(s):  
Lauren Sommer ◽  
Jesus G Vallejo ◽  
Kristina G Hulten ◽  
Sheldon L Kaplan ◽  
Jonathon C McNeil

Abstract Background The emergence of community-acquired methicillin-resistant Staphylococcus aureus (MRSA) in the late 1990s-early 2000s complicated the empiric management of suspected staphylococcal infection in children. Rising clindamycin resistance rates in many communities adds further to management challenges. Ceftaroline, an anti-MRSA cephalosporin, represents an attractive therapy option. Little data are available, however, regarding the frequency of reduced susceptibility (RS) to ceftaroline among MRSA isolates from a general pediatric population. Methods Isolates were selected from an ongoing S. aureus surveillance study at Texas Children’s Hospital. Invasive MRSA isolates from 2015-2018 were included. Isolates were initially screened for ceftaroline RS with E-test; all isolates with a ceftaroline E-test MIC ≥ 1.5µg/ml underwent ceftaroline broth dilution. Ceftaroline RS was regarded as an MIC ≥ 2 µg/ml; full ceftaroline resistance was defined as an MIC ≥ 8 μg/ml. Accessory gene regulator (agr) groups were characterized by PCR. Results 201 viable isolates were included. The ceftaroline MIC50 and MIC90 were 0.5 and 1 µg/ml, respectively (Figure 1). Six isolates had MIC ≥ 2 µg/ml (2.9%) with two having MIC ≥ 8 µg/ml (0.9%). All ceftaroline RS isolates were from healthcare associated infections. Ceftaroline RS isolates were more often associated with clindamycin-resistance and agr group II (Figure 2). Infections with ceftaroline RS were associated with central venous lines, recent ICU admission, preceding antibiotic exposure (specifically cephalosporins) and prior MRSA infection. One subject with MRSA CLABSI had a ceftaroline susceptible MRSA infection followed < 1 month later by a ceftaroline resistant infection (MIC =32 μg/ml); the isolates were identical by PFGE. Only 3 subjects had previously received ceftaroline, none of which subsequently developed a ceftaroline RS isolate. Figure 1. Ceftaroline MIC Distribution Figure 2. Clinical And Microbiologic Associations with Reduced Ceftaroline Susceptibility Conclusion Ceftaroline RS occurs in 2.9% of invasive MRSA isolates in children and is most prominent among healthcare-associated infections. These isolates were associated with clindamycin resistance and agr group II. While ceftaroline RS is rare among invasive MRSA infections, the lack of preceding ceftaroline exposure is concerning and warrants careful surveillance. Disclosures Sheldon L. Kaplan, MD, Allergan (Research Grant or Support)Pfizer (Grant/Research Support) Jonathon C. McNeil, MD, Allergan (Research Grant or Support, Allergan provided ceftaroline powder for use in studies described in this abstract)


2008 ◽  
Vol 132 (7) ◽  
pp. 1079-1103 ◽  
Author(s):  
Megan K. Dishop ◽  
Supriya Kuruvilla

Abstract Context.—Primary lung neoplasms are rare in children, but they comprise a broad and interesting spectrum of lesions, some of which are familiar from other tissue sites, and some of which are unique to the pediatric lung. Objective.—To determine the relative incidence of primary and metastatic lung tumors in children and adolescents through a single-institution case series, to compare these data to reports in the medical literature, to discuss the clinical and pathologic features of primary tumors of the tracheobronchial tree and lung parenchyma in children, and to provide recommendations for handling pediatric lung cysts and tumors. Data Sources.—A 25-year single institutional experience with pediatric lung tumors, based on surgical biopsies and resections at Texas Children's Hospital from June 1982 to May 2007, an additional 40 lung tumors referred in consultation, and a review of the medical literature. Conclusions.—A total of 204 pediatric lung tumors were diagnosed at our institution, including 20 primary benign lesions (9.8%), 14 primary malignant lesions (6.9%), and 170 secondary lung lesions (83.3%). The ratio of primary benign to primary malignant to secondary malignant neoplasms is 1.4:1:11.6. The common types of lung cancer in adults are exceptional occurrences in the pediatric population. The most common primary lung malignancies in children are pleuropulmonary blastoma and carcinoid tumor. Other primary pediatric lung tumors include congenital peribronchial myofibroblastic tumor and other myofibroblastic lesions, sarcomas, carcinoma, and mesothelioma. Children with primary or acquired immunodeficiency are at risk for Epstein-Barr virus–related smooth muscle tumors, lymphoma, and lymphoproliferative disorders. Metastatic lung tumors are relatively common in children and also comprise a spectrum of neoplasia distinct from the adult population.


2021 ◽  
Vol 64 (3) ◽  
pp. 232-238
Author(s):  
Soo-Hong Kim ◽  
Yong-Hoon Cho ◽  
Hae Young Kim

Pusan National University Children’s Hospital (PNUCH) opened in November 2008 as the second National Children’s Hospital in Korea and celebrated its 10th anniversary in 2018. In the last decade, birth rates and pediatric populations have been declining sharply and has been more serious in Busan, Ulsan, and Gyeongsangnam-do where the PNUCH is located, affecting the prevalence of index diseases. The number of the index diseases was investigated by year according to changes in the population. Patients who underwent surgery at PNUCH for 10 years were included. Changes in pediatric population and the number of live births were analyzed using the data from Statistics Korea. The number of the index diseases was analyzed retrospectively through the review of electronic medical records. Statistical analysis was performed through Poisson regression. The number of live births and pediatric population decreased from 69,528 to 48,600 (30.1%) and 1,880,284 to 1,444,961 (23.2%), respectively. Operations of pediatric surgery decreased by 32.3% compared to that during the peak season, but the prevalence rate ratio of the pediatric population increased to 1.03 (<i>P</i><0.001). The number of emergency operation increased due to the largest surge in acute appendicitis operations. Both esophageal atresia and Hirschsprung disease decreased, while hypertrophic pyloric stenosis significantly increased. The absolute number of pediatric surgery cases decreased. However, considering the changes in pediatric population and the number of live births, the relative number of pediatric surgery cases does not changed. The decrease in the number of each disease would be related to the decrease in pediatric population and the number of live births. We should consider the increase of emergency operations such as acute appendicitis.


2018 ◽  
Vol 55 (5) ◽  
pp. 711-720 ◽  
Author(s):  
Zakia Hammal ◽  
Jeffrey F. Cohn ◽  
Erin R. Wallace ◽  
Carrie L. Heike ◽  
Craig B. Birgfeld ◽  
...  

Objective: To compare facial expressiveness (FE) of infants with and without craniofacial microsomia (cases and controls, respectively) and to compare phenotypic variation among cases in relation to FE. Design: Positive and negative affect was elicited in response to standardized emotion inductions, video recorded, and manually coded from video using the Facial Action Coding System for Infants and Young Children. Setting: Five craniofacial centers: Children’s Hospital of Los Angeles, Children’s Hospital of Philadelphia, Seattle Children’s Hospital, University of Illinois–Chicago, and University of North Carolina–Chapel Hill. Participants: Eighty ethnically diverse 12- to 14-month-old infants. Main Outcome Measures: FE was measured on a frame-by-frame basis as the sum of 9 observed facial action units (AUs) representative of positive and negative affect. Results: FE differed between conditions intended to elicit positive and negative affect (95% confidence interval = 0.09-0.66, P = .01). FE failed to differ between cases and controls (ES = –0.16 to –0.02, P = .47 to .92). Among cases, those with and without mandibular hypoplasia showed similar levels of FE (ES = –0.38 to 0.54, P = .10 to .66). Conclusions: FE varied between positive and negative affect, and cases and controls responded similarly. Null findings for case/control differences may be attributable to a lower than anticipated prevalence of nerve palsy among cases, the selection of AUs, or the use of manual coding. In future research, we will reexamine group differences using an automated, computer vision approach that can cover a broader range of facial movements and their dynamics.


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