Atlantic Health System

About: Atlantic Health System is a healthcare organization based out in Morristown, New Jersey, United States. It is known for research contribution in the topics: Health care & Medicine. The organization has 277 authors who have published 299 publications receiving 6594 citations.

Case Report: Expanded Access Treatment of an Infantile Neuroaxonal Dystrophy Patient with a Novel Stabilized Polyunsaturated Fatty Acid Drug (P3.327)

Abstract: Objective: The objective was to determine if D-PFUAs could provide clinical benefit to INAD patients. Background: Stabilized polyunsaturated fatty acid drugs (D-PUFAs) have been demonstrated in many neurodegenerative disease preclinical models to mitigate both cell death and disease symptoms. The gene defect underlying >90% of INAD disease, PLA2G6, causes increased cell death due from accelerated lipid peroxidation. These effects were reversed over controls in INAD stem cell and fibroblast studies with D-PUFA dosing, and climbing performance in a drosophila model improved. Since D-PUFAs are well distributed, were safe in preclinical toxicity studies and a clinical phase 1/2 study in Friedreich’s ataxia, and showed benefit in multiple INAD models, the risk/benefit profile for a trial in INAD was favorable. Design/Methods: The patient, a ~2.5-year-old female, had developed normally until about 15 months of age, and received a confirmed genetic diagnosis of PLA2G6-deficient INAD at around 28 months. At baseline, she had lost many previous development milestones, was mostly unresponsive, non-verbal and exhibiting classical INAD symptoms including, hypotonia, nystagmus, strabismus, and psychomotor regression, with little spontaneous motor activity. At the trial start, she discontinued 3.5–7 grams of omega 3/6 supplement, and began dosing of 1.8 g, BID of RT001 softgels. Results: Within the first week, chronic constipation resolved. After 2 months on drug, she regained sufficient bulbar function to terminate syringe feeding of liquids and returned to drinking from a child’s sippy cup. After 6 months, she improved in qualitative measures captured in videotaped therapy sessions or exams, including eye tracking, responsiveness to verbal commands, head control, lifting, and reaching and grasping for her spoon (a lost skill). Conclusions: INAD is a strictly progressive disease, only worsening with time. Stabilization of regressions and recovery of lost milestones appears to indicate effectiveness of the test treatment in a single, severely affected INAD child. Disclosure: Dr. Adams has received personal compensation for consulting, serving on a scientific advisory board, speaking, or other activities with BioMarin, Inc. Dr. Adams has received research support from BioMarin, Inc. Dr. Dastgir has nothing to disclose. Dr. Molinari has nothing to disclose. Dr. Heerinckx has nothing to disclose.

Robotic sacrocolpopexy. A review.

Abstract: The aim of this article was to provide a contemporary reference source for robotic sacrocolpopexy. This article will discuss robotic sacrocolpopexy, in terms of its history, acceptance in modern medicine, indications, materials used, technique, outcomes, and complications. Research from our institution and others was summarized to provide a comprehensive review of robotic sacrocolpopexy. In conclusion, we found out that robotic sacrocolpopexy provides a minimally invasive approach to treatment of pelvic organ prolapse with excellent outcomes and low complication rates.

Comparative chemotactic properties of WISP-1 and PlGF on human venous smooth muscle cells

Abstract: Introduction: Wnt-Inducible Secreted Protein-1 (WISP-1) and Placental Growth Factor (PlGF) were found to be induced within 72 hours in organ-cultured human saphenous vein. While both are members of the PDGF superfamily, WISP-1 is closely related to Connective Tissue Growth Factor and PlGF is closely related to VEGF. During vascular wound repair, WISP-1 may recruit vascular smooth muscle cells (VSMCs) and PlGF may recruit endothelial cells because of their respective similarities to previously characterized growth factors. We hypothesized that WISP-1 may be more effective at inducing migration of human VSMCs than PlGF. Methods: Lyophilized recombinant proteins (R&D Systems, Minneapolis, MN) were reconstituted in RPMI1640. Passage 4 VSMCs, all derived from a single saphenous vein for a single experiment, were used at 10,000 cells/well in 48-well chemotaxis chambers (Neuroprobe, Gaithersburg, MD). Each experiment was repeated 4 times using cells from a different saphenous vein for each experiment. Results: Both WISP-1 and PlGF induced a chemotactic response from the VSMCs. WISP-1 was maximally chemotactic at 1 ng/mL (approximately 0.026 nM) while the PlGF maximum was 10 ng/mL (approximately 0.36 nM). Further studies will define their differential chemotactic effect on endothelial cells. Conclusions: WISP-1 was 13.8 times more effective, on a molar basis, than PlGF at inducing VSMC chemotaxis. While both growth factors probably play a role in vascular wound repair, their performance in chemotaxis assays of VSMCs suggests that WISP-1 activity is directed toward VSMCs.

Pericardial effusion with tamponade - an uncommon presentation leading to the diagnosis of eosinophilic granulomatosis polyangiitis: A case report.

Abstract: Background Eosinophilic granulomatosis polyangiitis (EGPA) is a small vessel necrotizing vasculitis that commonly presents as peripheral eosinophilia and asthma; however, it can rarely manifest with cardiac involvement such as pericarditis and cardiac tamponade. Isolated pericardial tamponade presenting as the initial symptom of EGPA is exceedingly rare. Early diagnosis and appropriate treatment are crucial to prevent life-threatening outcomes. Case summary 52-year-old woman with no past medical history presented with progressive dyspnea and dry cough. On physical exam she had a pericardial friction rub and bilateral rales. Vital signs were notable for tachycardia at 119 beats per minute and hypoxia with 89% oxygen saturation. On laboratory exam, she had 45% peripheral eosinophilia, troponin elevation of 1.1 ng/mL and N-terminal prohormone of brain natriuretic peptide of 2101 pg/mL. TTE confirmed a large pericardial effusion and tamponade physiology. She underwent urgent pericardial window procedure. Pericardial and lung biopsy demonstrated eosinophilic infiltration. Based on the American College of Radiology guidelines, the patient was diagnosed with EGPA which manifested in its rare form of cardiac tamponade. She was treated with steroid taper and mepolizumab. Conclusion This case highlights that when isolated pericardial involvement occurs in EGPA, diagnosis is recognized by performing pericardial biopsy demonstrating histopathologic evidence of eosinophilic infiltration.