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Samriti Dogra, Frederick Kaskel
Steroid-resistant nephrotic syndrome remains a challenge to treat, but various efforts are underway to better understand the pathogenesis and improve patient outcomes. This review provides an update on the newer advances in understanding the molecular etiologies for a variety of podocyte abnormalities, potential circulating factors that may initiate and sustain the steroid-resistant state, genetic mutations, and precision medicine treatment modalities in this continuously perplexing disorder.
October 26, 2016: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
Alicia Galindo-Ferreiro, Patricia Akaishi, Somaya Hanafi, Rajiv Khandekar, Alberto Galvez-Ruiz, Silvana Schellini, Augusto Cruz
PURPOSE: To compare outcomes of two techniques of frontalis silicone sling surgery. METHODS: This retrospective, nonrandomized chart review was performed on patients undergoing frontalis sling surgery between 2008 and 2011. Silicone rods were sutured to the tarsal plate through an eyelid crease incision (open method) or stab incisions (closed method). Data were collected on age, gender, type of surgery, preoperative and postoperative margin reflex distance (MRD), and complications...
October 25, 2016: Journal of Pediatric Ophthalmology and Strabismus
H Sprague Eustis, Adam Janot, Chirag Jhaveri
PURPOSE: If the interruption of macular fusion for an extended period of time is the key event that results in the development of monofixation syndrome, then the decreased macular input caused by dense cataracts may cause monofixation syndrome in adults. This study was designed to test this hypothesis. METHODS: The study design was a retrospective chart review that identified patients with unilateral cataracts at two southern Louisiana medical centers. Patients were assigned to the dense cataract group if a unilateral cataract had been present for at least 3 months and led to a visual acuity of worse than 20/200...
October 25, 2016: Journal of Pediatric Ophthalmology and Strabismus
Nazife Sefi-Yurdakul, Ayse Tülin Berk
PURPOSE: To compare the visual outcomes of primary and secondary intraocular lens (IOL) implantations and to identify the risk factors for the development of strabismus in patients with pediatric cataract. METHODS: The records of the pediatric patients who had undergone cataract surgery between January 1999 and November 2014 were reviewed retrospectively. The results of the cases with cataract extraction with primary IOL implantation (primary group) and cases with secondary IOL implantation (secondary group) were compared and the risk factors for the development of strabismus were investigated...
October 25, 2016: Journal of Pediatric Ophthalmology and Strabismus
Gregory L Fu, Janet L Alexander, Osamah J Saeedi
A 3-month-old boy with primary congenital glaucoma developed a transient corneal endothelial opacity after needle bleb revision with adjunctive 5-fluorouracil. This case presents a rare toxicity that, although transient, is concerning due to the potential of amblyopia. The authors review prior cases of corneal opacity associated with 5-fluorouracil. [J Pediatr Ophthalmol Strabismus. 2016;53:e54-e57.].
October 22, 2016: Journal of Pediatric Ophthalmology and Strabismus
Cheryl E Gariepy, Melvin B Heyman, Mark E Lowe, John F Pohl, Steven L Werlin, Michael Wilschanski, Bradley Barth, Douglas S Fishman, Steven D Freedman, Matthew J Giefer, Tanja Gonska, Ryan Himes, Sohail Z Husain, Veronique D Morinville, Chee Y Ooi, Sarah Jane Schwarzenberg, David M Troendle, Elizabeth Yen, Aliye Uc
: Acute recurrent pancreatitis (ARP) and chronic pancreatitis (CP) have been diagnosed in children at increasing rates over the past decade. However, as pediatric ARP and CP are still relatively rare conditions, little quality evidence is available on which to base the diagnosis and determination of etiology. OBJECTIVES: To review the current state of the literature regarding the etiology of these disorders and to developed a consensus among a panel of clinically active specialists caring for children with these disorders to help guide the diagnostic evaluation and identify areas most in need of future research...
October 25, 2016: Journal of Pediatric Gastroenterology and Nutrition
Paige C Woodham, James W Fulcher, Michael E Ward
Ligature strangulation, although typically homicidal in nature, has been reported as accidental in both the pediatric and adult populations. The unique mode of accidental strangulation with a gastric feeding tube in a 23-month-old with Down syndrome is currently unreported in the literature and has prompted us to report this case and look into safety modifications that can be made to these common medical devices. Given the number of children with gastric feeding tubes and concern over this exact scenario brought up by parents, it is unusual that a similar case has not been reported...
October 25, 2016: American Journal of Forensic Medicine and Pathology
Sébastien Pesenti, Benjamin Blondel, Gregory Armaganian, Sébastien Parratte, Gérard Bollini, Franck Launay, Jean-Luc Jouve
Patellofemoral instability in children requires surgery when recurrent dislocation occurs. The aim of this study is to report the 5-year clinical and radiographic outcomes following lateral wedge augmentation trochleoplasty of the femoral trochlea. This is a review of 23 (mean age: 12.5 years) patients who underwent surgery for recurrent patellar dislocations related to a flat trochlea. In every case, a lateral wedge augmentation trochleoplasty was performed with preservation of the growth plate. This surgery was consistently associated with a soft tissue procedure...
October 25, 2016: Journal of Pediatric Orthopedics. Part B
Jing Chen, Craig A Mullen
BACKGROUND: Pediatric cancer is rare and its symptoms are often ambiguous. The aims of this study were to investigate the time needed to make a diagnosis, assess the frequency of misdiagnosis, and to determine whether these factors affected survival. METHODS: A review of records of 364 pediatric patients diagnosed with cancer at the University of Rochester Golisano Children's Hospital between 2004 and 2012 was conducted. Data were extracted on patient and health care system-related factors and clinical outcomes...
October 25, 2016: Journal of Pediatric Hematology/oncology
Swapnali Sabhapandit, Somasheila I Murthy, Vivek M Singh, Ketaki Gaitonde, Madhumita Gopal, Kerul Marsonia, Shajeera Sajid, Kalpana Babu
PURPOSE: To report the epidemiology of uveitis in two urban centers in South India. METHODS: Case records seen between January 2014 and December 2014 at two tertiary eye centers in South India were retrospectively reviewed. RESULTS: A total of 1123 patients in the study population had uveitis. Anterior uveitis was seen in 48.9%, posterior in 20.5%, intermediate in 17.3%, and panuveitis in 13.3%. Of these cases, 68.4% were acute uveitis. Pediatric uveitis constituted 6...
October 26, 2016: Ocular Immunology and Inflammation
Naresh Shanmugam, Julius Xavier Scott, Vimal Kumar, Mukul Vij, Priya Ramachandran, Gomathy Narasimhan, Mettu Srinivas Reddy, Venugopal Kota, Deenadayalan Munirathnam, Chayarani Kelgeri, Karthick Sundaram, Mohamed Rela
BACKGROUND: Advances in chemotherapy, liver resection techniques, and pediatric liver transplantation have vastly improved survival in children with hepatoblastoma (HB). These are best managed by a multidisciplinary team (MDT) in a setting where all treatment options are available. Until recently, this was difficult to achieve in India. METHODS: All children (<16 years) with HB treated in a pediatric liver surgery and transplantation unit between January 2011 and July 2016 were reviewed...
October 26, 2016: Pediatric Blood & Cancer
Gina Cambareri, Christina Carpenter, Jeffrey Stock, Jane Lewis, Sarah Marietti
To describe a multicenter experience with management of ureteral obstruction after injection of Dx/HA for VUR in pediatric renal transplant patients. The records of all pediatric renal transplant patients who underwent Dx/HA injection for VUR and had subsequent obstruction were identified, and the management and outcomes were reviewed. Follow-up ranged from 1 to 10 years. There were four patients identified, all of whom had a history of rising creatinine, recurrent UTI, and increasing hydronephrosis which led to the diagnosis of high-grade VUR...
October 26, 2016: Pediatric Transplantation
Mathew George, John Amodio, Haesoon Lee
Subpleural lung cysts (SPC) are seen in children with Down syndrome (DS). The incidence and the long term course of these lesions are not known. It is important for pediatricians and pediatric radiologists to be aware of these lung lesions since the DS patients' longevity has increased and they have greater frequency to encounter the clinicians. Autopsy and the radiology series have shown that these lesions are often found in association with congenital heart disease, particularly the endocardial cushion defect and prematurity...
2016: Case Reports in Pediatrics
Mohammad El-Ghanem, Tareq Kass-Hout, Omar Kass-Hout, Yazan J Alderazi, Krishna Amuluru, Fawaz Al-Mufti, Charles J Prestigiacomo, Chirag D Gandhi
Arteriovenous malformations (AVMs) in the pediatric population are relatively rare but reportedly carry a higher rate of rupture than in adults. This could be due to the fact that most pediatric AVMs are only detected after rupture. We aimed to review the current literature regarding the natural history and the clinical outcome after multimodality AVM treatment in the pediatric population, as optimal management for pediatric AVMs remains controversial. A multidisciplinary approach using multimodality therapy if needed has been proved to be beneficial in approaching these lesions in all age groups...
September 2016: Interventional Neurology
Douglas Y Mah, Divya Shakti, Kimberlee Gauvreau, Steven D Colan, Mark E Alexander, Dominic J Abrams, David W Brown
Left atrial (LA) dilation has been shown to be associated with atrial fibrillation (AF) in the adult population, with some studies indicating that larger LAs are more prone to AF recurrence. The relation of LA size to AF in the pediatric and young adult population has not been investigated. In this study, all pediatric patients (aged ≤22 years) who presented to Boston Children's Hospital from January 2002 to December 2012 with AF were reviewed. Patients with significant congenital heart disease, cardiomyopathies, proven channelopathies, previous cardiac surgery, end-stage renal disease, or severe lung disease/cystic fibrosis were excluded...
September 30, 2016: American Journal of Cardiology
Jessica A Zagory, Jamie Golden, Kathleen Holoyda, Natalie Demeter, Nam X Nguyen
The optimal management of pilonidal disease in the pediatric population is still debated. We conducted a retrospective review of patients 21 years old and younger who underwent surgical management for pilonidal disease between 2009 and 2013 at a single pediatric institution. Sixty patients (41.7% male) were included in the analysis, with a mean age of 15.0 years (range, 13-20). Twelve (20%) had a prior drainage procedure for pilonidal abscess before the definitive operative treatment. After excision to the presacral fascia, 36 (60%) had primary closure, 17 (28...
October 2016: American Surgeon
C Esposito, M Escolino, B Troncoso Solar, R Iacona, R Esposito, A Settimi, I Mushtaq
OBJECTIVES: To document the imaging follow-up of laparoscopic partial nephrectomy (LPN) in children and to investigate the natural history of cystic lesions post-LPN. MATERIALS AND METHODS: We reviewed the US imaging reports performed during follow-up in 125 children (77 girls, 48 boys - average age 3.2 years) underwent LPN in 2 centers of pediatric surgery in the period 2005-2015. RESULTS: Transperitoneal approach was adopted in 83 cases while retroperitoneoscopy in 42 cases...
October 25, 2016: BJU International
Henrik Smeds, Jeremy Wales, Filip Asp, Ulrika Löfkvist, Babak Falahat, Britt-Marie Anderlid, Lena Anmyr, Eva Karltorp
OBJECTIVE: To evaluate if cochlear implantation is safe and constitutes an option for hearing rehabilitation of children with x-linked inner ear malformation. STUDY DESIGN: Retrospective patient review in combination with a multidisciplinary follow-up. SETTING: Tertiary referral hospital and cochlear implant program. PATIENTS: Ten children with severe-profound mixed hearing loss and radiological findings consistent with Incomplete Partition type 3 cochlear malformation received cochlear implants during the years 2007 to 2015...
October 21, 2016: Otology & Neurotology
James D Cooper, Aimee G Costello, Peter H Shaw
PURPOSE: To examine whether the rates of thrombosis in children (≤14 years of age) and adolescent/young adult (AYA) patients (15-22 years of age) with cancer is different. METHODS: We retrospectively studied the rates of thrombosis in children and AYA patients at the Children's Hospital of Pittsburgh during the years 2002-2010, using the tumor registry database. This list was then divided into two groups based on age at diagnosis. A review of ICD-9 codes from hospital billing records was then performed to identify patients who carried diagnoses of cancer (140...
October 25, 2016: Journal of Adolescent and Young Adult Oncology
Juan P Boriosi, Jens C Eickhoff, Kristi B Klein, Gregory A Hollman
BACKGROUND AND AIM: Both propofol and dexmedetomidine have been found to be safe and effective sedation for magnetic resonance imaging (MRI). Our program experienced an increase in patients arousing and experiencing an adverse airway event during propofol sedation for MRI in the first months of using a new 3T (Tesla) MRI scanner that was found to have a longer reverberation time compared to the previous 1.5 T MRI. In an effort to decrease patient arousal and adverse airway events during MRI, we administered a dexmedetomidine load prior to our standard propofol protocol...
October 25, 2016: Paediatric Anaesthesia
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