Childrens Hospital of Wisconsin

Lake Wisconsin, WI, United States

Childrens Hospital of Wisconsin

Lake Wisconsin, WI, United States

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Kumar S.,Medical College of Wisconsin | Kumar S.,Childrens Research Institute | Kumar S.,Childrens Hospital of Wisconsin | Henrickson K.J.,Medical College of Wisconsin | And 2 more authors.
Clinical Microbiology Reviews | Year: 2012

The menu of diagnostic tools that can be utilized to establish a diagnosis of influenza is extensive and includes classic virology techniques as well as new and emerging methods. This review of how the various existing diagnostic methods have been utilized, first in the context of a rapidly evolving outbreak of novel influenza virus and then during the different subsequent phases and waves of the pandemic, demonstrates the unique roles, advantages, and limitations of each of these methods. Rapid antigen tests were used extensively throughout the pandemic. Recognition of the low negative predictive values of these tests is important. Private laboratories with preexisting expertise, infrastructure, and resources for rapid development, validation, and implementation of laboratory-developed assays played an unprecedented role in helping to meet the diagnostic demands during the pandemic. FDA-cleared assays remain an important element of the diagnostic armamentarium during a pandemic, and a process must be developed with the FDA to allow manufacturers to modify these assays for detection of novel strains in a timely fashion. The need and role for subtyping of influenza viruses and antiviral susceptibility testing will likely depend on qualitative (circulating subtypes and their resistance patterns) and quantitative (relative prevalence) characterization of influenza viruses circulating during future epidemics and pandemics. © 2012, American Society for Microbiology. All Rights Reserved.

Dehner L.P.,University of Washington | Jarzembowski J.A.,Childrens Hospital of Wisconsin | Hill D.A.,Childrens National Medical Center
Modern Pathology | Year: 2012

Embryonal rhabdomyosarcoma of the uterine cervix is an uncommon presentation of the most common soft-tissue sarcoma in the first decades of life. Unlike embryonal rhabdomyosarcoma in other anatomic sites, in which 70-80% of cases present before 9 years of age, the average age in our series of 14 cervical cases was 12.4 years (median, 13 years), with an age range of 9 months to 32 years at diagnosis. Of the 14 cases, 12 presented as a polyp at the cervical os; two patients had an infiltrative mass in the cervix without a botryoid polyp. The polyps measured 1.5-5 cm and all had the histopathological pattern of the sarcoma botryoides variant of embryonal rhabdomyosarcoma, with condensations of primitive and differentiated rhabdomyoblasts beneath the surface epithelium and around endocervical glands. Nodules of benign-appearing cartilage were present in the stroma of six cases (43%). One of the embyronal rhabdomyosarcomas from the youngest patient, 9 months old, also had a distinctive microscopic focus of immature tubular profiles in a primitive stroma; these tubules expressed epithelial and neuroendocrine markers. Two patients had a pleuropulmonary blastoma, one diagnosed 9 years before the embryonal rhabdomyosarcoma of the cervix and the other recognized synchronously. This latter 9-year old had a DICER1 germline mutation. One patient presented with hirsutism and had a Sertoli-Leydig cell tumor, an incidentally detected cervical embryonal rhabdomyosarcoma, and nodular hyperplasia of the thyroid. Although a pleuropulmonary blastoma was not documented in the latter patient, ovarian sex-cord stromal tumors and nodular hyperplasia of the thyroid are manifestations of the pleuropulmonary blastoma family tumor and dysplasia syndrome (OMIM 601200). Embryonal rhabdomyosarcoma of the cervix must be distinguished from other rare entities, including adenosarcoma, malignant mixed Mullerian tumor and low-grade stromal sarcoma, as the former has a better prognosis; 12 of our 14 patients remain disease-free following conservative surgery and chemotherapy. Our study suggests that cervical embryonal rhabdomyosarcoma may be another pathological manifestation in the spectrum of extrapulmonary pathology in the setting of pleuropulmonary blastoma. © 2012 USCAP, Inc. All rights reserved.

Willoughby R.E.,Childrens Hospital of Wisconsin
Infectious Disease Clinics of North America | Year: 2015

Rabies is an acute, rapidly progressive encephalitis that is almost always fatal. Prophylaxis is highly effective but economics limits disease control. The mechanism of death from rabies is unclear. It is poorly cytopathic and poorly inflammatory. Rabies behaves like an acquired metabolic disorder. There may be a continuum of disease severity. History of animal bite is rare. The diagnosis is often missed. Intermittent encephalopathy, dysphagia, hydrophobia and aerophobia, and focal paresthesias or myoclonic jerks suggest rabies. Laboratory diagnosis is cumbersome but sensitive. Treatment is controversial but survivors are increasingly reported, with good outcomes in 4 of 8 survivors. © 2015 Elsevier Inc..

Arvedson J.C.,Childrens Hospital of Wisconsin
European Journal of Clinical Nutrition | Year: 2013

Children with cerebral palsy (CP) are at risk for aspiration with oral feeding with potential pulmonary consequences, and commonly have reduced nutrition/hydration status and prolonged stressful meal times. There is considerable variability in the nature and severity of swallowing problems in these children whose needs change over time. Children with generalized severe motor impairment (for example, spastic quadriplegia) are likely to experience greater swallowing deficits than those with diplegia, but oropharyngeal dysphagia is prevalent even in children with mild CP. This review is focused on dysphagia in children with CP: types of deficits, clinical and instrumental evaluation, management decision making and evidence of effectiveness of interventions. © 2013 Macmillan Publishers Limited All rights reserved.

Hahr J.Y.,Childrens Hospital of Wisconsin
Medical Hypotheses | Year: 2013

Autism as we know it, is caused iatrogenically and occurs reportedly one in 88 live birth [3]. Now national survey pegs autism prevalence one in 50 school-age children and the incidence is rising much fast in recent years. The author is hypothesizing idiopathic autism is caused by feeding of infant formula. Majorities of formula in the world are milk based and the molecular weight of the cow's milk is much higher than that of human breast milk. These increased solutes contributes to increased osmolality of the environment of the newborn infant, is directly affecting hemodynamics of normal homeostasis of the developing human brain cells. Formula makers fortified new substances in the process of formula making whenever they found previous unknown substances in the breast milk, for past several decades. When those solid substances were added in the process of formula making to make 20. cal/oz of infant formula, this resulted displacing free water in the formula. When new substances were added, same amount of free water has to be displaced from the formula. That is why we are seeing more autism in recent years, compared to previous several decades. © 2013 Elsevier Ltd.

Strasburger J.F.,Childrens Hospital of Wisconsin | Wakai R.T.,University of Wisconsin - Madison
Nature Reviews Cardiology | Year: 2010

The human fetal heart develops arrhythmias and conduction disturbances in response to ischemia, inflammation, electrolyte disturbances, altered load states, structural defects, inherited genetic conditions, and many other causes. Yet sinus rhythm is present without altered rate or rhythm in some of the most serious electrophysiological diseases, which makes detection of diseases of the fetal conduction system challenging in the absence of magnetocardiographic or electrocardiographic recording techniques. Life-threatening changes in QRS or QT intervals can be completely unrecognized if heart rate is the only feature to be altered. For many fetal arrhythmias, echocardiography alone can assess important clinical parameters for diagnosis. Appropriate treatment of the fetus requires awareness of arrhythmia characteristics, mechanisms, and potential associations. Criteria to define fetal bradycardia specific to gestational age are now available and may allow detection of ion channelopathies, which are associated with fetal and neonatal bradycardia. Ectopic beats, once thought to be entirely benign, are now recognized to have important pathologic associations. Fetal tachyarrhythmias can now be defined precisely for mechanism-specific therapy and for subsequent monitoring of response. This article reviews the current and future diagnostic techniques and pharmacologic treatments for fetal arrhythmia. © 2010 Macmillan Publishers Limited. All rights reserved.

Frommelt M.A.,Childrens Hospital of Wisconsin
Clinics in Perinatology | Year: 2014

Today, almost 70% of babies with hypoplastic left heart syndrome (HLHS) will survive into adulthood, although significant long-term morbidity and mortality still exists. Prenatal diagnosis of HLHS is increasingly common, allowing improved counseling, and the potential for fetal intervention if indicated. Exciting progress continues to be made in the area of fetal diagnosis and intervention, specifically catheter intervention for intact atrial septum or severe aortic stenosis. Pediatric cardiologists should be keenly aware of the flaws of staged palliation for the treatment of HLHS, and need to keep abreast of the emerging data regarding fetal diagnosis and intervention. © 2014 Elsevier Inc.

Oldham K.T.,Childrens Hospital of Wisconsin
Journal of the American College of Surgeons | Year: 2014

In summary, the Task Force does understand that change is difficult and, in the circumstance of the US health care environment, quite complex. Having acknowledged this, the Task Force firmly believes that if optimal resource standards are clear, providers will act in the best interests of their patients, infants, and children undergoing surgery in this circumstance. We intend to provide evidence to this point, to define optimal resources, and to facilitate this process. The hope and the underlying intent of these recommendations is to insure that every infant and child undergoing a surgical procedure in the United States will receive his or her care in an environment that offers all of the facilities, equipment, and, most especially, access to the professional providers who have the appropriate background and training to provide optimal care. This mustbe done while balancing the issues of access, staff, and the need to improve the value proposition. The Task Force is unanimous in its intent to advocate for this agenda.

Loomba R.S.,Childrens Hospital of Wisconsin
American Journal of Therapeutics | Year: 2015

Early treatment with intravenous immunoglobulin (IVIG) is necessary to help reduce the risk of coronary artery abnormalities, such as coronary artery aneurysms and to help alleviate symptoms, in Kawasaki disease. Some patients, however, do not respond to an initial dose of IVIG and require additional doses. Prediction of these IVIG nonresponders may be of assistance in altering initial therapy to make it more effective. The Egami score has been validated in the Japanese population to predict IVIG nonresponders but has shown to be ineffective in US populations. This study evaluates the Egami score in a Midwest US population, subdividing patients by race and the diagnosis of typical or atypical type of Kawasaki disease. Patients were included in the study if they met criteria for Kawasaki disease and received IVIG in the inpatient setting. A total of 182 patients were studied, and in all studied groups, the Egami score had poor sensitivity at predicting IVIG nonresponders. Sensitivity of the score differed between races and differed between typical and atypical Kawasaki disease. The Egami score, as well as other systems, have been validated to predict IVIG nonresponders. These, however, lack sensitivity in the US population. Other scores developed in the United States have also lacked sensitivity, likely due to the absence of race or Kawasaki disease classification as variables. The development of a sensitive scoring system to predict IVIG nonresponders in US populations will require the incorporation of race and Kawasaki disease classification, factors that seem to alter IVIG response. © 2015 by Lippincott Williams & Wilkins.

Loomba R.S.,Childrens Hospital of Wisconsin
Annals of Pediatric Cardiology | Year: 2016

Arterial desaturation may occur after the Kawashima procedure and, in the absence of venovenous collaterals is usually due to pulmonary arteriovenous malformations. Stenting of the pulmonary arteries, oxygen therapy, and pulmonary vasodilators such as sildenafil have not been able to resolve the arterial desaturation and the only way to do this has been Fontan completion. The time course of the formation of these malformations after the Kawashima and the progression of cyanosis and its resolution after the Fontan has only been demonstrated in case reports and small case series. We pool the available data to model arterial saturations in patients with pulmonary arteriovenous malformations after the Kawashima procedure. © 2016 Annals of Pediatric Cardiology | Published by Wolters Kluwer - Medknow.

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