Background: Juvenile systemic sclerosis (jSSc) is a systemic inflammatory and fibrotic autoimmune disease. Adult guidelines recommend obtaining a screening high-resolution computed tomography scan (CT) at diagnosis. As these recommendations are adopted as standard of care for jSSc, increased screening with CT may lead to increased detection of nodules.
View Article and Find Full Text PDFChronic pancreatitis is a debilitating disease affecting millions worldwide. These patients suffer from bouts of severe pain that are minimally relieved by pain medications and may necessitate major surgeries with high morbidity and mortality. Previously, we demonstrated that "chemical pancreatectomy," a pancreatic intraductal infusion of dilute acetic acid solution, ablated the exocrine pancreas while preserving the endocrine pancreas.
View Article and Find Full Text PDFBackground: Sarcopenia is an increasingly recognized marker of frailty in cardiac patients. Patients with a history of congenital heart disease and Fontan procedure have a higher risk of developing progressive muscle wasting. Our objective was to determine if we could use routine cardiac MRI (CMR) for the surveillance of muscle wasting.
View Article and Find Full Text PDFChronic pancreatitis is a debilitating disease affecting millions worldwide. These patients suffer from bouts of severe pain that are minimally relieved by pain medications and may necessitate major surgeries with high morbidity and mortality. Previously, we demonstrated that "chemical pancreatectomy," a pancreatic intraductal infusion of dilute acetic acid solution, ablated the exocrine pancreas while preserving the endocrine pancreas.
View Article and Find Full Text PDFMediastinal masses are categorized based on the International Thymic Malignancy Interest Group (ITMIG) classification into prevascular, visceral and paravertebral compartments. The schema is based on cross-sectional imaging, mainly CT, and helps with generating a differential diagnosis based on location of the mass. Up to half of all pediatric mediastinal tumors are malignant.
View Article and Find Full Text PDFIntroduction: Despite the clinical benefits of the cone operation for Ebstein's anomaly, significant right ventricular (RV) dysfunction is frequently seen immediately after the procedure and if persistent may portend worse long-term outcomes. In this study we sought to evaluate the predictors of RV dysfunction after the cone operation using preoperative CMR.
Methods: This was a retrospective review of 26 consecutive patients who had the cone operation.
CASE (Phila)
October 2021
• LAA is a complex, variable anatomic structure that can be mistaken for other cardiac structures. • Color and spectral Doppler may help to discern the LAA from surrounding structures. • Cross-sectional imaging such as CTA can aid in defining the morphology of the LAA.
View Article and Find Full Text PDFIntroduction: To investigate long-term metformin adherence in the Diabetes Prevention Program Outcomes Study (DPPOS) by examining: (1) predictors of long-term adherence to study metformin and (2) whether metformin adherence was associated with incident type 2 diabetes.
Research Design And Methods: DPPOS was an open-label continuation of the randomized clinical trial (Diabetes Prevention Program (DPP)) in which eligible participants randomized to the metformin group were offered study metformin and followed over 11 years. A brief structured adherence interview was administered semiannually.
Unresectable hepatocellular carcinoma (HCC) was first removed successfully with total hepatectomy and liver transplantation (LT) in a child over five decades ago. Since then, children with unresectable liver cancer have benefitted greatly from LT and a confluence of several equally important endeavors. Regional and trans-continental collaborations have accelerated the development and standardization of chemotherapy regimens, which provide disease control to enable LT, and also serve as a test of unresectability.
View Article and Find Full Text PDFBackground: Changes that occur in the remaining ovary after contralateral oophorectomy are not well described.
Objective: To determine average ovarian volume in pediatric patients after contralateral oophorectomy compared to age-matched controls with two normal ovaries.
Materials And Methods: We performed a retrospective review of ultrasound examinations and electronic medical records of patients ages 0-18 years who had unilateral oophorectomy from 2000 to 2017 (n=64).
A tenth of all pediatric liver transplantations (LTs) are performed for unresectable liver malignancies, especially the more common hepatoblastoma (HBL). Less understood are outcomes after LT for the rare hepatocellular carcinoma, nonhepatoblastoma embryonal tumors (EMBs), and slow growing metastatic neuroendocrine tumors of childhood. Pediatric LT is increasingly performed for rare unresectable liver malignancies other than HBL.
View Article and Find Full Text PDFCanal of Nuck abnormalities are a rare but important cause of morbidity in girls, most often those younger than 5 years of age. The canal of Nuck, which is the female equivalent of the male processus vaginalis, is a protrusion of parietal peritoneum that extends through the inguinal canal and terminates in the labia majora. The canal typically obliterates early in life, but in some cases the canal can partially or completely fail to close, potentially resulting in a hydrocele or hernia of pelvic contents.
View Article and Find Full Text PDFBackground: While neuroendocrine (carcinoid) tumors are increasingly recognized in the adult population, they are often not suspected in children.
Procedure: Retrospective review of all well-differentiated neuroendocrine (carcinoid) tumors was performed based on pathology reports from a quaternary pediatric medical center between January 2003 and June 2016. Clinical presentations, treatment approaches, imaging findings, and outcomes were reviewed and analyzed.
Objective: Although rare, pediatric neuroendocrine tumors including carcinoid tumor have increased in incidence over the past few decades. Despite increased recognition, survival rates have not significantly improved. Earlier detection of these tumors is necessary to improve clinical outcomes.
View Article and Find Full Text PDFCollection of salivary cortisol has become increasingly popular in large population-based studies. However, the impact of protocol compliance on day-to-day reliabilities of measures, and the extent to which reliabilities differ systematically according to socio-demographic characteristics, has not been well characterized in large-scale population-based studies to date. Using data on 935 men and women from the Multi-Ethnic Study of Atherosclerosis, we investigated whether sampling protocol compliance differs systematically according to socio-demographic factors and whether compliance was associated with cortisol estimates, as well as whether associations of cortisol with both compliance and socio-demographic characteristics were robust to adjustments for one another.
View Article and Find Full Text PDFIn experimental models of pancreatic growth and recovery, changes in pancreatic size are assessed by euthanizing a large cohort of animals at varying time points and measuring organ mass. However, to ascertain this information in clinical practice, patients with pancreatic disorders routinely undergo non-invasive cross-sectional imaging of the pancreas using magnetic resonance imaging (MRI) or computed tomography (CT). The aim of the current study was to develop a thin-sliced, optimized sequence protocol using a high field MRI to accurately calculate pancreatic volumes in the most common experimental animal, the mouse.
View Article and Find Full Text PDFBackground: Stress hormones have been hypothesized to contribute to the social patterning of cardiovascular disease but evidence of differences in hormone levels across social groups is scant.
Purpose: To examine the associations of socioeconomic and psychosocial factors with urinary levels of cortisol and catecholamines and determine whether these associations are modified by race/ethnicity.
Methods: Measures of cortisol, epinephrine, norepinephrine and dopamine were obtained on 12-h overnight urine specimens from 942 White, African American and Hispanic participants in the Multi-Ethnic Study of Atherosclerosis (MESA).
The aim of this study was to use multidetector computed tomography (MDCT) and magnetic resonance (MRA) angiography to illustrate the classification and clinical characteristics of single coronary artery anomaly (SCAA). Retrospective evaluation of 22 adult and pediatric patients with SCAA by way of a medical archiving system was performed between June 2001 and August 2012. Imaging modalities used for coronary artery evaluation included MRA and MDCT angiography.
View Article and Find Full Text PDFResidual pulmonary insufficiency in post-repair Tetralogy of Fallot (rToF) patients often mediates biventricular dysfunction which is associated with long-term adverse clinical outcomes. The objective of this study was to demonstrate the presence of impaired left ventricle (LV) circumferential strain (CS) in pediatric rToF patients as compared to controls using cardiac magnetic resonance imaging (CMRI). Additionally, bivariate analysis between right ventricle (RV) and LV functional measures in rToF patients was performed to further characterize the interventricular interactions thought to mediate LV dysfunction secondary to RV volume overload.
View Article and Find Full Text PDFIsolated fibrous gastric antral band in a neonate causing partial gastric obstruction and mimicking prepyloric antral web is not reported in the English literature, and we would like to report the first case. A premature neonate with feeding difficulties and bilious aspirate underwent upper gastrointestinal contrast examination. The upper gastrointestinal series showed a thin, radiolucent, circumferential band in the antrum with delayed gastric emptying.
View Article and Find Full Text PDFNoncompaction of the left ventricle, a genetic cardiomyopathy with a reported incidence of 0.05% to 0.24%, can lead to sudden cardiac death, particularly among children, if left undetected.
View Article and Find Full Text PDFBackground: The use of the adaptive statistical iterative reconstruction (ASIR) algorithm has been shown to reduce radiation doses in adults undergoing abdominal CT studies while preserving image quality. To our knowledge, no studies have been done to validate the use of ASIR in children.
Objective: To retrospectively evaluate differences in radiation dose and image quality in pediatric CT abdominal studies utilizing 40% ASIR compared with filtered-back projection (FBP).
Lobar displacement (LD) after heart-lung transplantation (HLT) has been reported in adults, but there are no reported pediatric cases. Its occurrence may cause vascular compromise of the displaced lung segment leading to necrosis, infection and bronchiectasis, as well as compression of contralateral lobes. We report two cases of LD in children following HLT, treated differently and with different outcomes.
View Article and Find Full Text PDFWe report on a 22-year-old male carrying a presumptive clinical diagnosis of Dubowitz-like phenotype who has been followed-up by cardiology for bicuspid aortic valve with ascending aorta and aortic root dilatation. Cardiac magnetic resonance imaging (CMRI) confirmed these findings, along with an incidental finding of left ventricular non-compaction (LVNC). Genetic workup revealed the diagnosis of 22q11.
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