Fetal endocardial fibroelastosis: ultrasonographic findings in two cases.

EFE is a rare cardiac disorder with poor prognosis and uncertain cause. Primary and secondary forms have been described. Most authors consider that all EFE is secondary--a reactive process set off in the endocardium by stress on the myocardium. We report two cases representing the primary dilated form and the secondary contracted form. The dilated form was associated with intracavitary thrombus of the left ventricle. In both cases, an unusual presence of subendocardial calcifications was noted. The ultrasonographic findings are discussed.     

The Turner syndrome research registry: Creating equipoise between investigators and participants

To address knowledge gaps about Turner syndrome (TS) associated disease mechanisms, the Turner Syndrome Society of the United States created the Turner Syndrome Research Registry (TSRR), a patient‐powered registry for girls and women with TS. More than 600 participants, parents or guardians completed a 33‐item foundational survey that included questions about demographics, medical conditions, psychological conditions, sexuality, hormonal therapy, patient and provider knowledge about TS, and patient satisfaction. The TSRR platform is engineered to allow individuals living with rare conditions and investigators to work side‐by‐side. The purpose of this article is to introduce the concept, architecture, and currently available content of the TSRR, in anticipation of inviting proposals to utilize registry resources.     

Total parenteral nutrition (TPN) and gallbladder diseases in neonates. Sonographic assessment

During a 1-year period, a prospective ultrasound (US) study of the biliary tract was performed in 41 neonates receiving total parenteral nutrition (TPN). Gallbladder sludge appeared in 18 neonates (44%) after a mean period of 10 days of TPN. The occurrence of this abnormality seems related to three main factors: prematurity with immaturity of the enterohepatic circulation of bile acids, duration of TPN, and lack of enteral nutrition. In five neonates (12%), an evolution of sludge to "sludge balls" was observed. Two neonates (5%) went on to develop uncomplicated gallstones and a spontaneous resolution occurred in one of them 6 months after the examination. This finding suggests conservative management in such cases. However, since cholecystitis or biliary tract obstruction are classical complications in older children, a US survey of neonates receiving TPN is useful to follow the abnormal gallbladder content, and rapid introduction of enteral feeding is advisable.     

Orbital Branch of the Infraorbital Artery: Further Characterization of an Important Surgical Landmark

The orbital branch of the infraorbital artery, a key vascular structure that is not universally noted in orbital textbooks and atlases, is clinically significant, since injury to it can result in perioperative hemorrhage. We conducted a cadaver dissection to document its presence, measure its location, and evaluate it histopathologically. It was present in 8 of 9 orbits and was a mean distance of 16.6?mm (range 10–23) from the inferior orbital rim. In half of the specimens, there were 2 separate structures seen. Histopathology confirmed these structures to be neurovascular bundles.     

Endoscopic Occlusion of Tracheoesophageal Fistula in Ventilated Patients Using an Amplatzer Septal Occluder

Acquired tracheoesophageal fistula (TEF) is a challenging, life threatening condition. It most commonly appears in critically ill patients requiring prolonged mechanical ventilation, who cannot withstand open neck or chest surgery. An endoscopic technique could be better tolerated by these patients. We present our experience using a cardiac Amplatzer ASD septal occluder for an endoscopic TEF repair in ventilation-dependent patients. Two high risk patients underwent the procedure under general anesthesia and close respiratory monitoring. In one patient the device was inserted through the trachea and in the other through the esophagus. In both cases fistula closure was achieved for different periods of time allowing the patients a temporary relief of symptoms. The procedure was well tolerated by the patients, and no significant adverse effect documented. The technique was successful as a temporary solution for unstable patients with TEFs and should be considered as a treatment modality for similar patients.     

Food Allergy: A Comprehensive Population-Based Cohort Study

Objective

To determine the incidence and temporal trends of food allergies.