Outcomes in ovarian Sertoli-Leydig cell tumor: A report from the International Pleuropulmonary Blastoma/DICER1 and Ovarian and Testicular Stromal Tumor Registries.

OBJECTIVE: Sertoli-Leydig cell tumors (SLCTs) are rare sex cord-stromal tumors, representing <0.5% of all ovarian tumors. We sought to describe prognostic factors, treatment and outcomes for individuals with ovarian SLCT. METHODS: Individuals with SLCT were enrolled in the International Pleuropulmonary Blastoma/DICER1 Registry and/or the International Ovarian and Testicular Stromal Tumor Registry. Medical records were systematically abstracted, and pathology was centrally reviewed when available. RESULTS: In total, 191 participants with ovarian SLCT enrolled, with most (92%, 175/191) presenting with FIGO stage I disease. Germline DICER1 results were available for 156 patients; of these 58% had a pathogenic or likely pathogenic germline variant. Somatic (tumor) DICER1 testing showed RNase IIIb hotspot variants in 97% (88/91) of intermediately and poorly differentiated tumors. Adjuvant chemotherapy was administered in 40% (77/191) of cases, and among these, nearly all patients received platinum-based regimens (95%, 73/77), and 30% (23/77) received regimens that included an alkylating agent. Three-year recurrence-free survival for patients with stage IA tumors was 93.6% (95% CI: 88.2-99.3%) compared to 67.1% (95% CI: 55.2-81.6%) for all stage IC and 60.6% (95% CI: 40.3-91.0%) for stage II-IV (p < .001) tumors. Among patients with FIGO stage I tumors, those with mesenchymal heterologous elements treated with surgery alone were at higher risk for recurrence (HR: 74.18, 95% CI: 17.99-305.85). CONCLUSION: Most individuals with SLCT fare well, though specific risk factors such as mesenchymal heterologous elements are associated with poor prognosis. We also highlight the role of DICER1 surveillance in early detection of SLCT, facilitating stage IA resection.

Meditation for Anxiety: A Holistic Approach to Treatment.

This comprehensive integrated literature review provides evidence-based information on mindfulness meditation as a primary care treatment for anxiety. A variety of databases were utilized for article collection including MEDLINE Complete, CINAHL Plus with full text, Cochrane library, APA PsycInfo, APA PsycTests, Health Source: Nursing/Academic Edition, and PsycARTICLES. Research suggests anxiety can be treated effectively with a minimum of 8 weeks of meditation practice. Meditation is a cost effective and safe treatment modality that can be incorporated into the care of patients struggling with anxiety.

A Prospective, Randomized Controlled, Single-Blinded Study to Assess the Effect of a 33-Gauge Needle Versus a 34-Gauge Needle on Pain Experienced During Injection of Local Anesthetic on the Face.

BACKGROUND: Most pain experienced by patients during Mohs micrographic surgery is associated with the initial injection. Previous studies have shown that a smaller gauge needle (33-gauge vs 30-gauge) is associated with less patient-reported pain. OBJECTIVES: To evaluate patient-reported pain levels following injection with a 33-gauge versus a 34-gauge needle.  Methods: During this prospective, randomized, controlled, single-blinded study, 480 patients were randomized into a 33-gauge versus a 34-gauge needle group. Pain levels following needle insertion were recorded using the validated numerical rating scale (VNRS)-11 scale.  Results: Injection of local anesthetic with a 34-gauge needle is associated with significantly less pain compared to a 33-gauge needle across all subgroups (P=0.007, average pain level 0.49 [34-gauge group] vs 0.79 [33-gauge group] rated on a 0-10 pain scale). Females, first-time Mohs patients, patients under age 65, patients with basal cell carcinoma, and those with tumor locations on the nose experienced the most pain reduction with the use of a 34-gauge needle.  Limitations: This was a single-blinded study; thus, the injector was able to see which needle was being used. This knowledge could have subconsciously affected the angle, speed, or force used to insert the needle. CONCLUSIONS: Injections with a 33-gauge and a 34-gauge needle are both tolerated well and associated with minimal pain. While the pain reduction associated with using a 34-gauge needle is statistically significant, the use of a 34-gauge needle may be most clinically relevant for certain patient subgroups. J Drugs Dermatol. 2023;22(11): doi:10.36849/JDD.7689.

PAX1 expression in thymic epithelial neoplasms and morphologic mimics.

Thymic epithelial neoplasms are morphologically diverse and can pose a diagnostic challenge that is complicated by a lack of immunohistochemistry (IHC) markers that are entirely sensitive and specific for thymic epithelium. Polyclonal PAX8 is often used in this context, but it is not a specific marker. The PAX1 transcription factor shares significant homology with PAX8 and plays an integral role in thymic development in humans and murine models. This study evaluated the role of PAX1 IHC in differentiating thymic epithelial neoplasms from morphologic mimics on whole slide tissue sections. The PAX1 antibody stained all 74 thymoma cases; however, there was wide variability in staining intensity within each subtype. The antibody was less sensitive in thymic carcinomas and thymic neuroendocrine tumors compared to thymomas and demonstrated weak staining in a subset of morphologic mimics (21 squamous cell carcinomas, 6 pulmonary neuroendocrine tumors, 1 mesothelioma, 1 lymphoblastic lymphoma, and 1 granulosa cell tumor). With a H-score positive threshold of 75, the antibody had 100% specificity, and sensitivities of 92%, 56%, and 47% in thymomas, thymic neuroendocrine tumors, and thymic carcinomas respectively. The PAX1 antibody showed frequent geographic reduction in staining consistent with compromised antigenicity from variable formalin fixation. PAX1 IHC has a moderate-to-high sensitivity for thymic epithelial neoplasms; however, the wide staining variability and fixation effects may lead to difficulty with consistent interpretation. This marker is unlikely to supplant the role of PAX8 in diagnostic practice, but it may be a useful addition to immunohistochemistry panels when evaluating for thymic primary tumors.

Liver Pathology Related to Onco-Therapeutic Agents.

Oncotherapeutic agents can cause a wide range of liver injuries from elevated liver functions tests to fulminant liver failure. In this review, we emphasize a newer generation of drugs including immune checkpoint inhibitors, protein kinase inhibitors, monoclonal antibodies, and hormonal therapy. A few conventional chemotherapy agents are also discussed.

Epidemiological Factors Associated with Gross Diagnosis of Pulmonary Pathology in Feedyard Mortalities.

Respiratory disease continues to be the major cause of mortality in feedyard cattle, with bronchopneumonia (BP) and acute interstitial pneumonia (AIP) as the two most common syndromes. Recent studies described a combination of these pathological lesions with the presence of AIP in the caudodorsal lungs and BP in the cranioventral lungs of necropsied cattle. This pulmonary pathology has been described as bronchopneumonia with an interstitial pneumonia (BIP). The epidemiological characteristics of BIP in U.S. feedyard cattle are yet to be described. This study's objectives were to describe the agreement between feedyard clinical and necropsy gross diagnosis and to characterize epidemiological factors associated with four gross pulmonary diagnoses (AIP, BIP, BP, and Normal pulmonary tissue) observed during feedyard cattle necropsies. Systemic necropsies were performed at six feedyards in U.S. high plains region, and gross pulmonary diagnoses were established. Historical data were added to the dataset, including sex, days on feed at death (DOFDEATH), arrival weight, treatment count, and feedyard diagnosis. Generalized linear models were used to evaluate epidemiological factors associated with the probability of each pulmonary pathology. Comparing feedyard clinical diagnosis with gross pathological diagnosis revealed relatively low agreement and the frequency of agreement varied by diagnosis. The likelihood of AIP at necropsy was higher for heifers than steers and in the 100-150 DOFDEATH category compared with the 0-50 DOFDEATH (p = 0.05). The likelihood of BIP increased after the first treatment, whereas the DOFDEATH 0-50 category had a lower likelihood compared with the 150-200 category (p = 0.05). These findings highlight the importance of necropsy for final diagnosis and can aid the development of future diagnosis and therapeutic protocols for pulmonary diseases.

Predicting recurrence in pancreatic neuroendocrine tumours: role of ARX and alternative lengthening of telomeres (ALT).

BACKGROUND: While many pancreatic neuroendocrine tumours (PanNET) show indolent behaviour, predicting the biological behaviour of small nonfunctional PanNETs remains a challenge. Nonfunctional PanNETs with an epigenome and transcriptome that resemble islet alpha cells (ARX-positive) are more aggressive than neoplasms that resemble islet beta cells (PDX1-positive). In this study, we explore the ability of immunohistochemistry for ARX and PDX1 and telomere-specific fluorescence in situ hybridisation (FISH) for alternative lengthening of telomeres (ALT) to predict recurrence. METHODS: Two hundred fifty-six patients with PanNETs were identified, and immunohistochemistry for ARX and PDX1 was performed. Positive staining was defined as strong nuclear staining in >5% of tumour cells. FISH for ALT was performed in a subset of cases. RESULTS: ARX reactivity correlated with worse disease-free survival (DFS) (P = 0.011), while there was no correlation between PDX1 reactivity and DFS (P = 0.52). ALT-positive tumours (n = 63, 31.8%) showed a significantly lower DFS (P < 0.0001) than ALT-negative tumours (n = 135, 68.2%). ARX reactivity correlated with ALT positivity (P < 0.0001). Among nonfunctional tumours, recurrence was noted in 18.5% (30/162) of ARX-positive tumours and 7.5% (5/67) of ARX-negative tumours. Among WHO grade 1 and 2 PanNETs with ≤2 cm tumour size, 14% (6/43) of ARX-positive tumours recurred compared to 0 of 33 ARX-negative tumours and 33.3% (3/9) ALT-positive tumours showed recurrence versus 4.4% (2/45) ALT-negative tumours. CONCLUSION: Immunohistochemistry for ARX and ALT FISH status may aid in distinguishing biologically indolent cases from aggressive small low-grade PanNETs, and help to identify patients who may preferentially benefit from surgical intervention.

Determining Frequency of Common Pulmonary Gross and Histopathological Findings in Feedyard Fatalities.

Pulmonary disease is often associated with feedlot cattle mortality, and the most common syndromes include bronchopneumonia, acute interstitial pneumonia, and bronchopneumonia with an interstitial pneumonia. The study objective was to utilize gross necropsy and histopathology to determine the frequency of pulmonary lesions from three major syndromes and agreement between gross and histopathological diagnosis. A cross sectional, observational study was performed at six U.S. feedyards using a full systematic necropsy to assess mortalities during summer 2022. A subset of mortalities had four lung samples submitted for histopathological diagnosis. Gross necropsy was performed on 417 mortalities, 402 received a gross diagnosis and 189 had a histopathological diagnosis. Descriptive statistics were used to evaluate pulmonary diagnosis frequency based on method (gross/histopathology), and generalized linear mixed models were used to evaluate agreement between histopathological and gross diagnoses. Using gross diagnosis, bronchopneumonia represented 36.6% of cases with acute interstitial pneumonia and bronchopneumonia with an interstitial pneumonia representing 10.0% and 35.8%, respectively. Results identified bronchopneumonia with an interstitial pneumonia as a frequent syndrome which has only been recently reported. Histopathological diagnosis had similar findings; bronchopneumonia represented 32.3% of cases, with acute interstitial pneumonia and bronchopneumonia with an interstitial pneumonia representing 12.2% and 36.0%, respectively. Histopathological diagnosis tended (p-VALUE = 0.06) to be associated with gross diagnosis. Pulmonary disease was common and both diagnostic modalities illustrated three primary syndromes: bronchopneumonia, acute interstitial pneumonia, and bronchopneumonia with an interstitial pneumonia with similar frequencies. Improved understanding of pulmonary pathology can be valuable for evaluating and adjusting therapeutic interventions.

Image Classification and Automated Machine Learning to Classify Lung Pathologies in Deceased Feedlot Cattle.

Bovine respiratory disease (BRD) and acute interstitial pneumonia (AIP) are the main reported respiratory syndromes (RSs) causing significant morbidity and mortality in feedlot cattle. Recently, bronchopneumonia with an interstitial pattern (BIP) was described as a concerning emerging feedlot lung disease. Necropsies are imperative to assist lung disease diagnosis and pinpoint feedlot management sectors that require improvement. However, necropsies can be logistically challenging due to location and veterinarians' time constraints. Technology advances allow image collection for veterinarians' asynchronous evaluation, thereby reducing challenges. This study's goal was to develop image classification models using machine learning to determine RS diagnostic accuracy in right lateral necropsied feedlot cattle lungs. Unaltered and cropped lung images were labeled using gross and histopathology diagnoses generating four datasets: unaltered lung images labeled with gross diagnoses, unaltered lung images labeled with histopathological diagnoses, cropped images labeled with gross diagnoses, and cropped images labeled with histopathological diagnoses. Datasets were exported to create image classification models, and a best trial was selected for each model based on accuracy. Gross diagnoses accuracies ranged from 39 to 41% for unaltered and cropped images. Labeling images with histopathology diagnoses did not improve average accuracies; 34-38% for unaltered and cropped images. Moderately high sensitivities were attained for BIP (60-100%) and BRD (20-69%) compared to AIP (0-23%). The models developed still require fine-tuning; however, they are the first step towards assisting veterinarians' lung diseases diagnostics in field necropsies.

Quantitative p53 immunostaining aids in the detection of prevalent dysplasia.

AIMS: The lack of accepted scoring criteria has precluded the use of p53 in routine practice. We evaluate the utility of automated quantitative p53 analysis in risk stratifying Barrett's oesophagus (BE) patients using non-dysplastic BE (NDBE) biopsies in a multicentric cohort of BE progressor (P) and non-progressor (NP) patients. METHODS: NDBE biopsies prior to the diagnosis of advanced neoplasia from 75 BE-P, and index and last surveillance biopsies from 148 BE-NP were stained for p53, and scored digitally as 1+, 2+ and 3+. A secondary cohort of 30 BE-P was evaluated. RESULTS: Compared with BE-NP, BE-P was predominantly men (p=0.001), ≥55 years of age (p=0.008), with longer BE segments (71% vs 33%; p<0.001). The mean number of 3+p53 positive cells and 3+ positive glands were significantly more in BE-P versus BE-NP NDBE biopsies (175 vs 9.7, p<0.001; 9.8 vs 0.1; p<0.001, respectively). At a cut-off of ≥10 p53 (3+) positive cells, the sensitivity and specificity of the assay to identify BE-P were 39% and 93%. On multivariate analysis, scoring p53 in NDBE biopsies, age, gender and length of BE were significantly associated with neoplastic progression. 54% of patients classified as prevalent dysplasia showed an abnormal p53 immunohistochemical stain. These findings were validated in the secondary cohort. CONCLUSIONS: Automated p53 analysis in NDBE biopsies serves as a promising tool for assessing BE neoplastic progression and risk stratification. Our study highlights the practical applicability of p53 assay to routine surveillance practice and its ability to detect prevalent dysplasia.

GLI1 Immunohistochemistry Distinguishes Mesenchymal Neoplasms With GLI1 Alterations From Morphologic Mimics.

Glioma-associated oncogene 1 ( GLI1 ) alterations have been described in pericytoma with t(7;12), gastroblastoma, plexiform fibromyxoma, and an emerging class of GLI1 -rearranged or amplified mesenchymal neoplasms including "nested glomoid neoplasm". The immunophenotype of these tumor types is nonspecific, making some cases difficult to diagnose without sequencing. The utility of GLI1 immunohistochemistry (IHC) in distinguishing nested glomoid neoplasms and pericytomas with t(7;12) from morphologic mimics is unknown. To investigate the diagnostic value of GLI1 IHC, we determined its sensitivity and specificity in a "test cohort" of 23 mesenchymal neoplasms characterized by GLI1 alterations, including 12 nested glomoid neoplasms (7 GLI1 -rearranged, 4 GLI1 amplified, and 1 unknown GLI1 status), 9 pericytomas with t(7;12), 1 gastroblastoma, and 1 malignant epithelioid neoplasm with PTCH1 :: GLI1 fusion. GLI1 IHC was 91.3% sensitive in this cohort; all tumors except 2 pericytomas with t(7;12) expressed GLI1. GLI1 was also expressed in 1 of 8 (12%) plexiform fibromyxomas. Nineteen of 22 GLI1-positive tumors showed nuclear and cytoplasmic staining, while 3 showed nuclear staining only. GLI1 IHC was 98.0% specific; among morphologic mimics [40 well-differentiated neuroendocrine tumors, 10 atypical lung carcinoids, 20 paragangliomas, 20 glomus tumors, 20 solitary fibrous tumors, 10 Ewing sarcomas, 10 alveolar rhabdomyosarcomas (ARMS), 10 BCOR -altered sarcomas, 10 myoepitheliomas, 9 myopericytomas, 9 epithelioid schwannomas, 9 ossifying fibromyxoid tumors, 10 biphasic synovial sarcomas, 10 PEComas, 31 gastrointestinal stromal tumors, 10 inflammatory fibroid polyps, 11 pseudoendocrine sarcomas], 5 of 249 tumors expressed GLI1 (2 well-differentiated neuroendocrine tumors, 1 ARMS, 1 Ewing sarcoma, 1 BCOR -altered sarcoma). GLI1 IHC was also performed on a separate cohort of 13 molecularly characterized mesenchymal neoplasms in which GLI1 copy number gain was identified as a putatively secondary event by DNA sequencing (5 dedifferentiated liposarcoma [DDLPS], 2 adenosarcomas, 2 unclassified uterine sarcomas, 1 leiomyosarcoma, 1 ARMS, 1 intimal sarcoma, 1 osteosarcoma); 2 DDLPS, 1 ARMS, and 1 unclassified uterine sarcoma expressed GLI1. Lastly, because pleomorphic sarcomas sometimes show GLI1 amplification or copy number gain, GLI1 IHC was performed on a separate "pleomorphic sarcoma" cohort: GLI1 was expressed in 1 of 27 DDLPS, 1 of 9 leiomyosarcomas, and 2 of 10 pleomorphic liposarcomas, and it was negative in 23 well-differentiated liposarcomas and 9 unclassified pleomorphic sarcomas. Overall, GLI1 IHC was 91.3% sensitive and 98.0% specific for mesenchymal tumor types with driver GLI1 alterations among morphologic mimics. GLI1 expression was less frequent in other tumor types with GLI1 copy number gain. Given its specificity, in the appropriate morphologic context, GLI1 IHC may be a useful diagnostic adjunct for mesenchymal neoplasms with GLI1 alterations.

REPRINT OF: Physical and Mental Health Effects of Intimate Partner Violence for Men and Women.

EDITOR'S NOTE: This article is a reprint of a previously published article. For citation purposes, please use the original publication details: Coker AL, Davis KE, Arias I, et al. Physical and mental health effects of intimate partner violence for men and women. Am J Prev Med. 1985;1(6):1-8. BACKGROUND: Few population-based studies have assessed the physical and mental health consequences of both psychological and physical intimate partner violence (IPV) among women or men victims. This study estimated IPV prevalence by type (physical, sexual, and psychological) and associated physical and mental health consequences among women and men. METHODS: The study analyzed data from the National Violence Against Women Survey (NVAWS) of women and men aged 18 to 65. This random-digit-dial telephone survey included questions about violent victimization and health status indicators. RESULTS: A total of 28.9% of 6790 women and 22.9% of 7122 men had experienced physical, sexual, or psychological IPV during their lifetime. Women were significantly more likely than men to experience physical or sexual IPV (relative risk [RR]=2.2, 95% confidence interval [CI]=2.1, 2.4) and abuse of power and control (RR=1.1, 95% CI=1.0, 1.2), but less likely than men to report verbal abuse alone (RR=0.8, 95% CI=0.7, 0.9). For both men and women, physical IPV victimization was associated with increased risk of current poor health; depressive symptoms; substance use; and developing a chronic disease, chronic mental illness, and injury. In general, abuse of power and control was more strongly associated with these health outcomes than was verbal abuse. When physical and psychological IPV scores were both included in logistic regression models, higher psychological IPV scores were more strongly associated with these health outcomes than were physical IPV scores. CONCLUSIONS: Both physical and psychological IPV are associated with significant physical and mental health consequences for both male and female victims.

Navigating the complex realm of drug access in paediatric care.

A Drug Access Navigator (DAN) is a role that has been established in patient care settings with the goal of expediting the process by which patients obtain drugs through the Health Canada Special Access Program (SAP), individual drug companies' patient access programs (PAP), and public and private drug plans. In the paediatric setting, the process of accessing many drugs has grown increasingly complex. The majority of paediatric prescriptions involve 'off-label' drug use. This leads to an increased need for drug access through the SAP and/or PAPs and demonstrates that the need for a DAN may be particularly prominent in the paediatric population. To our knowledge, there is no evidence in the literature of a DAN functioning in a paediatric setting, though there is a demonstrable need. We plan to address this need by introducing a DAN for shared use at BC Children's Hospital, a paediatric tertiary care centre.

Teaching Through Collaboration: Flexibility and Diversity in Caregiver-Child Interaction Across Cultures.

Teaching supports the high-fidelity transmission of knowledge and skills. This study examined similarities and differences in caregiver teaching practices in the United States and Vanuatu (N = 125 caregiver and 3- to 8-year-old child pairs) during a collaborative problem-solving task. Caregivers used diverse verbal and nonverbal teaching practices and adjusted their behaviors in response to task difficulty and child age in both populations. U.S. caregivers used practices consistent with a direct active teaching style typical of formal education, including guiding children's participation, frequent praise, and facilitation. In contrast, Ni-Vanuatu caregivers used practices associated with informal education and divided tasks with children based on difficulty. The implications of these findings for claims about the universality and diversity of caregiver teaching are discussed.

Female adnexal tumor of probable Wolffian Origin - A report of two cases at one institution.

•FATWOs are rare gynecologic neoplasms of low malignant potential derived from mesonephric (Wolffian) duct remnants.•FATWOs have diverse presentations from vague abdominal symptoms to incidental diagnosis.•In general, FATWOs require no additional management beyond initial surgical intervention.

Outbreak of Severe Hypoglycemia After Ingestion of a Male Enhancement Supplement - Virginia, August-November 2019.

In August 2019, the Virginia Poison Center (VPC) and the Blue Ridge Poison Center (BRPC) were contacted concerning patients experiencing repeated episodes of marked hypoglycemia following ingestion of a male enhancement supplement tablet marketed as "V8" in convenience stores in central Virginia. Over the following 3 months, the Virginia Department of Agriculture and Consumer Services (VDACS) and the Virginia Department of Health (VDH) conducted an investigation and identified 17 patients meeting the case definition (severe hypoglycemia within 48 hours of consuming an over-the-counter male enhancement supplement in a man with no history of use of insulin or other medication used to control blood glucose). Analysis of the V8 tablets revealed that most contained glyburide, a sulfonylurea oral hypoglycemic used in the treatment of diabetes and associated with prolonged hypoglycemia following overdose (1). To stem this outbreak, V8 was removed from stores when found, and public service announcements were released. The public health implications of V8 use include the potential for substantial morbidity from hypoglycemic episodes and the potential for mortality if health care services are not accessed in a timely manner when hypoglycemia occurs. The presence of V8 in the market poses a serious threat to public health because of its potentially life-threatening adverse effects.

Pediatric Melanoma: A 35-year Population-based Review.

BACKGROUND: Melanoma is a rare neoplasm in the pediatric population. Recent publications suggest a possible increase in incidence over the past few decades. The purpose of this study was to analyze trends in pediatric patients diagnosed with malignant melanoma in British Columbia (BC) in the past 35 years. METHODS: A retrospective review was performed. All patients in BC diagnosed with melanoma before 18 years of age from 1979 to 2014 were included. Patient demographics, melanoma description, treatment details, and survival data were collected. RESULTS: Seventy-eight subjects were identified for the study. Patients were equally distributed by sex. Sixty-one (78%) of the subjects were diagnosed in the postpubertal age (≥12 years old). The most common sites of occurrence were the extremities (n = 33) and the trunk (n = 27), with the location on the trunk showing the highest mortality rate (22%). All patients were surgically treated and some had additional chemotherapy (12) and/or radiotherapy (12). Fatal outcome was recorded in 12 of the 78 subjects, 10 of whom had postpubertal diagnosis. The average time from date of diagnosis to date of death was 9.3 years. CONCLUSIONS: The incidence of melanoma in the pediatric population remains exceedingly rare: less than 2.5 per million children younger than 18 years. The diagnosis is rarely made before puberty; the incidence is equal in males and females and has not changed over a 35-year time period in BC. Our study shows 85% survival with the majority of patients having had surgical excision only.

Validity and reliability of the Physical Activity Questionnaire for Children (PAQ-C) and Adolescents (PAQ-A) in individuals with congenital heart disease.

OBJECTIVE: To assess the criterion validity, internal consistency, reliability and cut-point for the Physical Activity Questionnaire for Children (PAQ-C) and Adolescents (PAQ-A) in children and adolescents with congenital heart disease-a special population at high cardiovascular risk in whom physical activity has not been extensively evaluated. METHODS: We included 84 participants (13.6±2.9 yrs, 50% female) with simple (37%), moderate (31%), or severe congenital heart disease (27%), as well as cardiac transplant recipients (6%), from BC Children's Hospital, Canada. They completed the PAQ-C (≤11yrs, n = 28) or-A (≥12yrs, n = 56), and also wore a triaxial accelerometer (GT3X+ or GT9X) over the right hip for 7 days (n = 59 met valid wear time criteria). RESULTS: Median daily moderate-to-vigorous physical activity was 46.9 minutes per day (IQR 31.6-61.8) and 25% met physical activity guidelines defined as ≥60 minutes of moderate-to-vigorous physical activity per day. Median PAQ-score was 2.6 (IQR 1.9-3.0). PAQ-Scores were significantly related to accelerometry-derived metrics of physical activity (rho = 0.44-0.55, all p<0.01) and sedentary behaviour (rho = -0.53, p<0.001). Internal consistency was high (α = 0.837), as was reliability (stability) of PAQ-Scores over a 4-months period (ICC = 0.73, 95%CI 0.55-0.84; p<0.001). We identified that a PAQ-Score cut-point of 2.87 discriminates between those meeting physical guidelines and those that do not in the combined PAQ-C and-A samples (area under the curve = 0.80 (95%CI 0.67-0.92). CONCLUSION: Validity and reliability of the PAQ in children and adolescents with CHD was comparable to or stronger than previous studies in healthy children. Therefore, the PAQ may be used to estimate general levels of physical activity in children and adolescents with CHD.