Steigerwald K.A.,Childrens Hospital at Montefiore |
Ilowite N.T.,Childrens Hospital at Montefiore
Expert Review of Clinical Pharmacology | Year: 2015
The purpose of this review is to summarize the newer and possible future treatments for the arthritis and systemic features in children with juvenile idiopathic arthritis (JIA), including evidence supporting their efficacy and safety. © 2015 © Informa UK, Ltd.
Chambers S.,Childrens Hospital at Montefiore
Journal of interventional cardiac electrophysiology : an international journal of arrhythmias and pacing | Year: 2011
Lead failure is a common complication of pacemaker placement in the pediatric population and is often due to insulation breaks or lead fracture. Usually these leads are replaced with new leads. This study describes the surgical technique of lead repair and investigates the longevity of such repairs. Retrospective review of lead repairs performed in the pediatric population (<21 years old) between December 1995 and December 2008. Operative records, pre-operative interrogation data, and most recent follow-up data were reviewed. Twenty-one lead repairs were performed on 17 patients. Sixteen were ventricular leads and five were atrial leads. There were 14 insulation defect repairs, six new lead tip splicings, and one lead extender splicing. The repaired leads were followed for a mean time of 3.6±3.0 years (range, 0.3-10.6 years). The freedom from repaired lead failure by Kaplan-Meier survival analysis was 89% at 5 years and 22% at 10 years. One atrial lead and two ventricular leads have been replaced since the repairs. One atrial lead has had a repeat insulation break repair since the initial repair. One of the five atrial leads had an elevated threshold at follow-up. Five of 16 ventricular leads had elevated thresholds at follow-up. All leads had normal impedance at follow-up. The option of pacemaker lead repair, if surgically feasible, offers an alternative to lead replacement in the pediatric population and may extend the longevity of pacemaker leads in this population.
Cantinotti M.,Fondazione Toscana G. Monasterio |
Lopez L.,Childrens Hospital at Montefiore
Journal of the American Society of Echocardiography | Year: 2013
Interest in diastolic function in children has increased recently. However, the strengths and limitations of published pediatric nomograms for echocardiographic diastolic parameters have not been critically evaluated, especially in the neonatal population. A literature search was performed within the National Library of Medicine using the keywords normal/reference values, power Doppler/tissue Doppler velocities, and children/neonates. The search was further refined by adding the keywords diastolic function, myocardial, mitral/tricuspid inflow, pulmonary vein, and Tei index. Thirty-three published studies evaluating diastolic function in normal children were included in this review. In many studies, sample sizes were limited, particularly in terms of neonates. There was heterogeneity in the methodologies to perform and normalize measurements and to express normalized data (Z scores, percentiles, and mean values). Although most studies adjusted measurements for age, classification by specific age subgroups varied, and few addressed the relationships of measurements to body size and heart rate (especially with higher neonatal heart rates). Although reference values were reproducible in older children, they varied significantly in neonates and infants. Pediatric diastolic nomograms are limited by small sample sizes and inconsistent methodologies for the performance and normalization of measurements, with few data on neonates. Some studies do reveal reproducible patterns in diastolic function in older children. A comprehensive pediatric nomogram of diastolic function involving a large population of normal infants and older children and using standardized methodology is warranted and would have tremendous impact in the care of children with acquired and congenital heart disease. Copyright 2013 by the American Society of Echocardiography.
Wahezi D.M.,Childrens Hospital at Montefiore |
Ilowite N.T.,Childrens Hospital at Montefiore
Expert Opinion on Pharmacotherapy | Year: 2013
Introduction: Juvenile idiopathic arthritis (JIA) is the most common chronic rheumatic disease in childhood. Therapeutic advances in the treatment of JIA are occurring at a rapid rate, resulting in more ambitious therapeutic goals and increasing numbers of children experiencing complete clinical remission. Areas covered: The purpose of this review is to summarize the available treatments for the management of arthritis and systemic features in children with JIA, including current evidence supporting the safety and efficacy of biologic agents and future therapeutic approaches. Expert opinion: With advances in pharmacotherapy, physical and functional outcomes in children with JIA have improved immensely. The establishment of research consortia among the pediatric rheumatology community has allowed for large controlled studies and enabled a better understanding of the safety and efficacy of these therapeutic agents in children. Long-term safety data remain limited and thus longer, larger safety studies are warranted. © 2013 Informa UK, Ltd.
Fradin K.N.,Childrens Hospital at Montefiore |
Rhim H.J.H.,Yeshiva University
Journal of Adolescent Health | Year: 2013
A 16-year-old boy presented with a 6-day history of fevers and myalgias and a 4-day history of diffuse crampy abdominal pain. On admission, his sclerae were icteric and he had diffuse abdominal tenderness. Erythrocyte sedimentation rate was elevated to 40; the γ-glutamyl transferase level was elevated to 168 U/L; indirect bilirubin was 5.6 mg/dL; and direct bilirubin was 3.3 mg/dL. During the next 2 days, he developed many stigmata of Kawasaki disease (KD), including conjunctivitis, desquamating rash, mucosal changes, swelling of the hands and feet, and lymphadenopathy. KD is commonly seen in young children but can also occur in adolescents and adults. Providers should be aware that these age-groups are at risk for KD and may present with atypical symptoms. Delays in diagnosis can put these adolescents at increased risk of coronary artery aneurysms, and, accordingly, a high index of suspicion is essential. © 2013 Society for Adolescent Health and Medicine.
Dodson N.A.,Childrens Hospital at Montefiore
Current Opinion in Pediatrics | Year: 2016
Purpose of review This review will focus on recent research, initiatives, and legislation regarding the issue of gun violence as it pertains to adolescents. Recent findings Homicide and suicide continue to be major killers of adolescents in the United States. Gun homicide kills teens in the most urban areas of the United States at the same rate as suicide kills teens in the most rural areas of the United States. Research on assault-injured youth sheds light on risk factors for teen gun homicide, and has found high rates of illegal gun carrying and retaliatory attitudes among at-risk teens. Suicide research continues to show a strong correlation between gun ownership and accessibility, and risk of completed suicide. Stand Your Ground laws and campus carry laws present unique threats to different populations of American teens. Summary Given the enormous toll that gun violence takes on adolescent lives, pediatricians should ask about guns in the home and become involved in efforts to strengthen laws that would decrease gun violence. Copyright © 2016 Wolters Kluwer Health, Inc. All rights reserved.
Shakked R.J.,Montefiore Medical Center |
Geller D.S.,Montefiore Medical Center |
Gorlick R.,Childrens Hospital at Montefiore |
Dorfman H.D.,Montefiore Medical Center
Archives of Pathology and Laboratory Medicine | Year: 2012
Context.-Mesenchymal chondrosarcoma is a rare, highgrade malignancy of bone or soft tissue with a unique, biphasic histology and poor prognosis. Because of its rarity and variable length of disease-free survival, the natural history of the disease remains poorly understood. Objective.-To present clinical, radiographic, and histopathologic features of mesenchymal chondrosarcoma from one of the largest case series collected by a single, senior-level bone pathologist. Design.-Twenty cases were reviewed in consultations spanning 45 years. Results.-Eighteen tumors (90%) originated in bone, and 2 tumors (10%) were of extraskeletal origin. Of the skeletal tumors, locations included craniofacial bones (n = 9; 50%), ribs and chest wall (n = 4; 22%), sacrum and spinal elements (n = 3; 17%), and lower extremities (n = 2; 11%), whereas soft tissue tumors were located about the scapula (n = 1; 50%) and lower extremity (n = 1; 50%). Plain radiographs demonstrated calcified, osteolytic lesions with extraosseous extension. Typical histologic features were identified consisting of small, round or spindled cells, interspersed with hyaline cartilage islands. Seventeen patients (85%) were treated surgically, and 8 patients (40%) received adjuvant treatment. Seven patients (35%) were living at last follow-up, 1.8 to 12.5 years after diagnosis, and 8 patients (40%) died between 1.2 and 21.8 years after diagnosis. Conclusions.-Mesenchymal chondrosarcoma presents multiple challenges. Diagnostic pitfalls include inadequate biopsy samples, which may result in sample error. Sox9 has been proposed as a unique marker for mesenchymal chondrosarcoma which may improve diagnostic specificity. Treatment and prognosis vary considerably. Patients who receive surgery and chemotherapy seem to fare better. Multicenter studies with higher sample numbers may improve our understanding of this malignancy.
Katikaneni R.,Childrens Hospital at Montefiore
The journal of investigative dermatology. Symposium proceedings / the Society for Investigative Dermatology, Inc. [and] European Society for Dermatological Research | Year: 2013
Alopecia areata is a common form of hair loss in which autoimmune-mediated destruction of hair follicles causes patchy hair loss, for which there is no adequate therapy. Parathyroid hormone (PTH) induces the hair cycle and promotes hair growth. PTH-CBD is a fusion protein of PTH and a bacterial collagen-binding domain (CBD), leading to targeted delivery to and retention in the skin collagen. We tested the effects of a single dose of PTH-CBD (low or high dose) on an animal model for alopecia areata, the C3H/HeJ engrafted mouse. In all the treated animals, there was a rapid (1-4 days) increase in hair growth, with sustained effects observed over a 2-month period (7/10 total treated mice<40% hair loss based on gray scale analysis, vs. 2/5 in vehicle control animals). Histological examination revealed massive stimulation of anagen VI hair follicles in treated animals despite an ongoing immune response. PTH-CBD thus shows promise as a therapy for alopecia areata, likely in conjunction with a mild immune suppressant, such as hydrocortisone cream.
Cantinotti M.,CNR Institute of Neuroscience |
Assanta N.,CNR Institute of Neuroscience |
Murzi B.,CNR Institute of Neuroscience |
Lopez L.,Childrens Hospital at Montefiore
Heart | Year: 2014
Haemodynamically insignificant left-to-right shunts are frequently discovered when screening for congenital heart disease, resulting in significant economic and psychosocial impact. A literature search was performed within the National Library of Medicine using the keywords small/insignificant/silent atrial septal defect (ASD), ventricular septal defect (VSD), patent ductus arteriosus (PDA) and patent foramen ovale (PFO). The search was refined by adding the keywords definition, classification and follow-up. Our analysis revealed significant heterogeneity in the evaluation and management of innocent left-to-right shunts. The definitions for small defects vary greatly, making it difficult to distinguish between physiologic and pathologic lesions (eg, a PFO vs a true ASD). Most small defects will partially or completely resolve spontaneously early in life. If spontaneous resolution does not occur, the risk for long-term complications (such as embolic events and endocarditis) is low but poses several practical and ethical issues: immediate discharge versus long-term follow-up, duration and frequency of followup, and content and method of discussions with the parents. Additionally, there is controversy pertaining to treatment for PDAs and VSDs, particularly among interventional cardiologists, even though risk/benefit analyses are lacking. Standards and guidelines using consensus opinion for the management of insignificant left-to-right shunts are needed to address the heterogeneity in diagnosis and management as well as use of resources, ethical and psychosocial issues.
Carlson M.,Oregon Health And Science University |
Airhart N.,Oregon Health And Science University |
Lopez L.,Childrens Hospital at Montefiore |
Silberbach M.,Oregon Health And Science University |
Silberbach M.,Childrens Hospital at Montefiore
Circulation | Year: 2012
Background-Girls and women with Turner syndrome are at risk for aortic dissection and rupture. However, the size of the aorta and the clinical characteristics among those with Turner syndrome and dissection have received little attention. Methods and Results-We obtained medical records from 20 individuals who voluntarily participated in the International Turner Syndrome Aortic Dissection Registry. Type A dissections occurred in 17 of 20 (85%) cases, and type B occurred in 3 cases of which 1 occurred after coarctation stent placement. Of those with spontaneous aortic dissections, 18 of 19 (95%) had an associated cardiac malformation that included a bicuspid aortic valve. In 1 individual there was no predisposing finding other than the presence of Turner syndrome. Associated pregnancy was documented in 1 of 19 (5%). More than half (13/19, 68%) came to medical attention >24 hours after the onset of symptoms. For those with type A dissections, the mean ascending aortic size index was 2.7±0.6 cm/m (n=9). Conclusions-Aortic dissection in Turner syndrome occurs in young individuals at smaller aortic diameters than in the general population or other forms of genetically triggered aortopathy. The absence of aortic valve or other cardiac malformations appears to markedly reduce the risk of aortic dissection However, aortic dissection can occur in Turner syndrome without cardiac malformations or hypertension. Individuals with Turner syndrome who are >18 years of age with an ascending aortic size index >2.5 cm/m should be considered for an aortic operation to prevent aortic dissection. © 2012 American Heart Association, Inc.