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When two-year-old Savanna Bluford enters Boston Children’s Colorectal and Pelvic Malformation Center, she quickly gravitates toward the waiting room’s interactive light board. Sporting pigtails, sparkly sneakers and an angelic smile, the playful toddler’s attention quickly turns to her doctor — the Center’s Co-Director Dr. Belinda Dickie. The two light up with smiles and exchange hugs as if old friends — and that, they are.
Savanna was born in South Carolina with a rare and complex birth defect affecting the gastrointestinal, urological and reproductive systems.
The condition, called covered cloacal malformation, occurs when the bladder, colon and vaginal channels are connected. This connection causes a mixing of stool and urine, which exit the body from the same location. The malformation also impacts the spinal cord.
In order to correct Savanna’s condition — which affects one in 250,000 children — she will undergo a multi-staged, multi-year reconstruction process to repair all three systems.
When it came time to find a pediatric surgeon that specializes in such complexities, Savanna’s parents searched the nation for an expert — and they found Dr. Dickie.
“There is just something about Dr. Dickie that truly inspires us and makes us feel confident,” says Andrika, Savanna’s mother and home health nurse. “She is passionate about Savanna’s well-being and future quality of li…

Source: Thrive, Children’s Hospital BostonCategory: Pediatrics Authors: Tags: Diseases & Conditions Our Patients’ Stories Belinda Dickie cloacal malformation Colorectal and Pelvic Malformation Center Leonel Rodriguez Source Type: news

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Publication date: January 2019Source: Biomedicine &Pharmacotherapy, Volume 109Author(s): Xujie Wang, Yijie Zhang, Yunshu Yang, Wei Zhang, Liang Luo, Fu Han, Hao Guan, Ke Tao, Dahai HuAbstractBone marrow mesenchymal stem cells (BMSCs) possess promising therapeutic effects and have been considered as a highly desirable agent for tissue injury treatment. However, little survived cells after transplanting due to severe relocated conditions (characterized by prolonged hypoxia and oxidative stress) lead to hampered benefits of BMSCs-based cell therapy. Curcumin, a natural dietary product, has attracted increasing attention o…

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Source: PhytomedicineCategory: Drugs & Pharmacology Source Type: research

We report a case of successful repair of truncus arteriosus (TA) associated with complete atrioventricular septal defect (c-AVSD) using a staged approach. TA associated with c-AVSD is a very rare congenital cardiac anomaly. No report of successful staged repair in South Korea has yet been published. We performed bilateral pulmonary artery banding when the patient was 33 days old, and total correction using an extracardiac conduit was performed at the age of 18 months. The patient recovered uneventfully and is doing well.
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ConclusionsThe primary targets of improvement for our institution are care transitions and communications regarding medications. The less-invasive procedures (robotic MV repair, TAVR) were not independently associated with higher patient-experience scores.

We describe a term neonate with critical AS and coarctation, severe left ventricular (LV) dysfunction with endocardial fibro-elastosis palliated with surgical valvotomy and hybrid as a bridge to early biventricular repair. Initial repair consisted of commissurotomy to create bicuspid morphology with placement of bilateral pulmonary artery bands and maintenance of ductal patency with prostaglandin. LV function improved over two weeks allowing for repeat surgical valvotomy and coarctation repair. Patient was discharged two weeks post-operatively with low normal LV function, mild aortic stenosis/regurgitation.

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This study aimed to investigate the in vivo tissue response of the Biosilicate® scaffolds in a model of tibial bone defect. Sixty male Wistar rats were distributed into bone defect control group (CG) and Biosilicate® scaffold group (BG).  Animals were euthanized 15, 30 and 45 days post-surgery. Stereomicroscopy, scanning electron microscopy, histopathological, immunohistochemistry and biomechanical analysis were used. Scaffolds had a total porosity of 44%, macroporosity of 15% with pore diameter of 230 μm. Higher amount of newly formed bone was observed on days 30 and 45 i…

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Lymphedema distichiasis syndrome (LDS) is a rare, autosomal dominant genetic condition, characterized by lower limb lymphedema and distichiasis. Other associated features that have been reported include varicose veins, cleft palate, congenital heart defects, and ptosis. We update a previously reported family with a pathogenic variant in FOXC2 (c.412‐413insT) where five affected individuals from the youngest generation had congenital renal anomalies detected on prenatal ultrasound scan. These included four fetuses with hydronephrosis and one with bilateral renal agenesis. A further child with LDS had prominence of the lef…

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AJ Arria was diagnosed and treated at Boston Children’s for hydronephrosis – a urological condition where urine backs up into the kidneys.
Every child has a favorite thing. Some find comfort in the softness of a blanket, while others prefer snuggling a stuffed animal. Whatever the soothing item is, every parent cringes at the thought of misplacing it.
Michelle Arria remembers the day her 18-month-old son Anthony James (AJ) visited Boston Children’s Hospital for testing. It was the day his favorite blanket was lost.
“Testing was about to begin, and I went to get AJ’s blanket, and…

Source: Thrive, Children’s Hospital BostonCategory: Pediatrics Authors: Tags: Diseases & Conditions Our Patients’ Stories Boston Children’s at Peabody Hydronephrosis minimally invasive surgery Richard Yu urology Source Type: news

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