A biventricular repair for Jayce ’s one-of-a-kind heart

Amanda Mattioli was working in Afghanistan as a government contractor and had just completed a whirlwind round of travel to three separate continents when she learned she was pregnant. The helicopter unit that took her back to the main base so she could return home for her pregnancy gave her a unit sticker to commemorate her baby’s first helicopter ride. Little did she know it would also mark the beginning of a much longer journey for her and her son, William “Jayce” James. Amanda got her first hint the ride would be bumpy at her 20-week ultrasound, when she learned Jayce’s heart was on the right side of his chest, rather than the left side. She was sent to a hospital in nearby Pittsburgh for a level 2 ultrasound, where doctors discovered Jayce had three other major heart defects: complete atrioventricular canal defect (AV canal), transposition of the great arteries (TGA) and pulmonary atresia. Amanda was also referred to a geneticist. The geneticist told her it was very likely he had trisomy 18, a genetic disorder that causes severe birth defects. “She said he probably wouldn’t make it to birth, and if he did, he wouldn’t make it more than a few hours.” Amanda, overwhelmed and upset by the way this news had been delivered, returned to her local doctor and was referred to a private cardiologist in Pittsburgh for a second opinion, Dr. Prapti Kanani, a former cardiology fellow at Boston Children’s Hospital. Finding a b...
Source: Thrive, Children's Hospital Boston - Category: Pediatrics Authors: Tags: Diseases & Conditions Our Patients’ Stories atrioventricular canal Biventricular Repair Biventricular Repair Program Dr. Gerald Marx Dr. Pedro del Nido Heterotaxy syndrome Pulmonary atresia transposition of the great arteries Source Type: news

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In this report we demonstrated, for the first time, that Mn2+ triggers regulated necrosis (RN) in BV-2 cells involving two central mechanisms: parthanatos and lysosomal disruption. The occurrence of parthanatos is supported by several cellular and molecular events: (i) DNA damage; (ii) AIF translocation from mitochondria to the nucleus; (iii) mitochondrial membrane permeabilization; and (iv) PARP1-dependent cell death. On the other hand, Mn2+ induces lysosomal membrane permeabilization (LMP) and cathepsin D (CatD) release into the cytosol supporting the lysosomal disruption. Pre-incubation with CatB and D inhibitors partia...
Source: Neuroscience - Category: Neuroscience Source Type: research
Objectives: What patient characteristics and injury factors predict decreased knee range of motion (ROM) after operative management of tibial plateau fractures? Design: Prospective cohort study. Setting: Academic medical center. Patients: Over 11 years, tibial plateau fractures at a single academic institution were prospectively followed. A total of 266 patients were included in this study. Intervention: Surgical repair of tibial plateau fractures and secondary interventions due to arthrofibrosis. Main outcome measure: Clinical outcomes were evaluated using the Short Musculoskeletal Function Assessment and...
Source: Journal of Orthopaedic Trauma - Category: Orthopaedics Tags: Original Article Source Type: research
Placenta accreta spectrum (PAS) refers to an abnormally invasive implantation of the placenta into the uterine myometrium. The resultant risk is that of severe maternal hemorrhage and significant maternal morbidity and even mortality. The 2 strongest risk factors for the development of PAS are a history of a prior cesarean section and a placenta previa in the current pregnancy. Clinically, most patients are asymptomatic but some will present with vaginal bleeding and abdominal cramping. The goal of this article is to discuss the common clinical presentation and risk factors for placenta accreta spectrum, and to review in d...
Source: Clinical Obstetrics and Gynecology - Category: OBGYN Tags: Management of Abnormal Placentation Source Type: research
This article highlights the desired features for developing and managing a multidisciplinary team dedicated to the treatment of AIP in center of excellence.
Source: Clinical Obstetrics and Gynecology - Category: OBGYN Tags: Management of Abnormal Placentation Source Type: research
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Source: MCN: The American Journal of Maternal Child Nursing - Category: Nursing Tags: ONGOING COLUMNS: Hot Topics in Maternity Nursing Source Type: research
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Source: MCN: The American Journal of Maternal Child Nursing - Category: Nursing Tags: ONGOING COLUMNS: Perinatal Patient Safety Source Type: research
Biolife4D has achieved a milestone in its ultimate quest to develop human organs for transplant. The company recently announced that it has developed a cardiac patch using a highly specialized 3D printer designed to protect living cells during the printing process. The patch is designed to help patients recover heart function after an acute myocardial infarction, said Ravi Birla, PhD, the company’s chief science officer, in an interview with MD+DI. “Over several weeks, acute myocardial infarction results in scar formation and progresses to chronic heart failure, severely damp...
Source: MDDI - Category: Medical Devices Authors: Tags: 3-D Printing Source Type: news
Conclusion The intrarenal RAS is activated in kidney transplant donors immediately after kidney donation, independent of the systemic BP and filtration of increased plasma AGT, due to augmented inflammation. PMID: 30333423 [PubMed - as supplied by publisher]
Source: Internal Medicine - Category: Internal Medicine Tags: Intern Med Source Type: research
Authors: Sachiyo O, Masahiro T, Tsutomu T, Makoto I, Yutaka H, Nobumasa M, Takamichi K, Nozomi O, Shinpei M, Kazuhiro T, Yusuke K, Masanori O, Yusuke K, Yasuhisa H, Kazuo H, Yasumasa N Abstract Fanconi anemia (FA) is a disorder of chromosomal fragility characterized by progression to aplastic anemia, myelodysplastic syndrome, and leukemia. FA patients are also predisposed to solid cancers. A case of FA in an adult patient who developed tongue and superficial esophageal cancers following hematopoietic stem cell transplantation is reported. This case was considered significant because it is the first reported case of...
Source: Internal Medicine - Category: Internal Medicine Tags: Intern Med Source Type: research
In conclusion, we are able to recruit not only pregnant women but also fathers and grandparents. PMID: 30333380 [PubMed - in process]
Source: The Tohoku Journal of Experimental Medicine - Category: Research Authors: Tags: Tohoku J Exp Med Source Type: research
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