Current Pediatric Reviews (v.12, #3)

Meet Our Editorial Board Member by Alan P. Knutsen (167-167).

Intrauterine Intervention for the Treatment of Fetal Growth Restriction by A-M. Spiroski, M. H. Oliver, J. E. Harding, F. H. Bloomfield (168-178).
Fetal growth restriction (FGR) is associated with an increased incidence of fetal and neonatal death, and of neonatal morbidity. Babies born following FGR also are at risk of a range of postnatal complications, which may contribute to an increased incidence of disease later in life. There currently are no effective clinical interventions which improve perinatal survival, intrauterine growth and later outcomes of the FGR baby. Postnatal interventions aimed at promoting or accelerating growth in FGR babies to improve outcome, particularly neurodevelopmental outcomes, may further increase the risk of metabolic dysregulation and, therefore, the risk of developing chronic disease in adulthood. An intrauterine intervention to improve nutrition and growth in the FGR fetus may have the potential to decrease mortality and improve long-term outcomes by delaying preterm delivery and mitigating the need for and risks of accelerated postnatal growth.

Allergic Bronchopulmonary Aspergillosis: AllA Radiologist Needs To Know by Mandeep Kumar Garg, Madhurima Sharma, Ritesh Agarwal, Ashutosh N. Aggarwal, Pankaj Gupta, Kushaljit Singh Sodhi, Arunaloke Chakrabarty, Niranjan Khandelwal (179-189).
Background: Allergic bronchopulmonary aspergillosis (ABPA) is an immunological lung disorder occurring due to hypersensitivity to fungus Aspergillus fumigatus. Early diagnosis of ABPA is desirable as the disease can cause irreversible damage and end stage lung disease. Diagnosis of ABPA is based upon a set of clinical, serological and radiological criteria. Radiology plays an important role in the diagnosis of ABPA. However a radiologist should also be familiar with various clinical and serological criteria, so as to have a complete understanding of the disease process and to give a confident diagnosis.
Objective: To describe various clinical, laboratory and radiological findings in ABPA with a comprehensive review of various diagnostic criteria.
Method: We searched the literature for various criteria that have been used for diagnosis of ABPA. We also extensively reviewed the radiology literature to look for various imaging findings described in ABPA. We searched the role of various imaging modalities used in evaluation of ABPA starting with conventional radiography to computed tomography to magnetic resonance imaging.
Results: In this article, we have reviewed clinical, serological and radiological aspects of ABPA, with emphasis on the radiological findings. We have also described the various diagnostic criteria of ABPA.
Conclusion: In recent years, role of radiology in ABPA has evolved. Radiology plays an important role not only in diagnosis but also classification and prognostication of the disease. A radiologist should also be familiar with the clinical spectrum and laboratory findings of the disease.

The Evolution of Diagnosis and Management of Pediatric Biliary Tract Rhabdomyosarcoma by Alpin D. Malkan, Israel Fernandez-Pineda (190-198).
Background: The discovery and accurate diagnosis of a biliary tract mass in the pediatric population can prove to be extremely challenging. The purpose of this article is to give an overview of the evolution of management with regard to biliary tract rhabdomyosarcoma (RMS) in the pediatric population.
Methods: Clinical research related to biliary tract RMS in the pediatric population is reviewed, focusing on the diagnosis, staging and treatment.
Results: Over the past century the management of biliary tract RMS has transitioned through a spectrum of radical surgical procedures with poor outcomes. Over the past several decades there have been efforts to treat with chemotherapy based on known RMS chemosensitivity, with surgical interventions being utilized to manage a complex variation of disease manifestations in a case specific manner which further highlights the difficulty of managing this rare pathologic entity. This multimodal approach has significantly changed the landscape for those diagnosed with childhood RMS. The diagnosis of biliary tract RMS remains an extreme challenge since this is a rare tumor in children, may or may not be included in the differential diagnosis, and can be confused with other pathologic entities such as choledochal cysts.
Conclusion: RMS involving the biliary tract is extremely rare, and remains the most common cause of neoplastic biliary obstruction in childhood. The goals of maintaining biliary tract integrity, overall quality of life and cure while avoiding excessive morbidity and mortality remain at the heart of this pediatric oncologic entity.

Treatment Options for Persistent Coagulase Negative Staphylococcal Bacteremia in Neonates by Despoina Gkentzi, Soultana Kolyva, Iris Spiliopoulou, Markos Marangos, Gabriel Dimitriou (199-208).
Background: Coagulase-negative staphylococci (CoNS) are the leading cause of late-onset sepsis in neonatal intensive-care units (NICUs). Despite adequate therapeutic levels and in vivo susceptibility, vancomycin is not always adequate in clearing CoNS bacteremias and other theurapeutic regimens are warranted especially when dealing with persistent CoNS bacteremias.
Methods: Retrospective analysis of neonates with persistent CoNS bacteremias in a tertiary NICU in two study periods (January 2006 to December 2007 and January 2011 to December 2012). Demographics, clinical characteristics and antibiotics used were recorded. We also performed a literature review on the treatment options for persistent CoNS bacteremia.
Results: Out of the 1485 admissions during the 4 years, 121 (8,1%) neonates developed CoNS bacteremia and 42 of them (34.7%) were persistent. The most common CoNS species was S.epidermidis (91%). Apart from the shorter mean duration of bacteremia (p = 0.003), during the second part of the study, no other differences were observed between the two study periods. Ten (23.8%) neonates were treated with vancomycin monotherapy. In 23 (54.7%) neonates, rifampicin was added to vancomycin. Linezolid was given to seven neonates (16.6%), whereas daptomycin was given to two neonates (4.7%). No differences were found when we compared the characteristics of neonates treated with different antibiotics. All neonates tolerated treatment well. Our literature review revealed 16 relevant studies, the majority of which are small case series.
Conclusion: The addition of rifampicin, linezolid and daptomycin in neonates with persistent CoNS bacteremia can provide useful therapeutic alternatives. Further studies are required to bring more insight into the field.

The outcomes of liver transplantation in the pediatric population have improved significantly over the past two decades. As long-term survival improves, potential complications related to immunosuppression, delayed growth and development, and metabolic issues have started to be seen more commonly in long-term survivors. Hence, general pediatricians should to be aware of these potential complications in order to optimize the medical care and the quality of life for children with liver transplantation.

Perinatally, the developmental window for the adjustment of hormone receptors to their target hormones is open. The hormonal imprinting which determines the relationship of hormones and receptors for life takes place in this period. The recognition ability of developing receptors is not yet entirely specific, so they could be 'cheated' by false imprinters such as related hormones, drugs, environmental pollutants or certain food components and faulty imprinting may result. This causes a functional developmental abnormality, which is then manifested, at any later time in life, as the alteration of the binding capacity of hormone receptors. With or without the presence of other factors this could cause problems in hormone regulated functions or may result in diseases. So it may be stated that faulty hormonal imprinting, caused by a broad spectrum of receptor level molecules, is acting as a functional teratogen. This means that functional maldevelopment is not restricted to intrauterine life, but can also occur after birth, in the perinatal period. The inclusion of faulty imprinting as maldevelopment widens both the list of functional teratogens and the period of teratogenicity. The importance and danger of faulty imprinting is high, especially considering its heritable (transgenerational) character. Considering the facts, the attitudes in the present-day perinatal treatments and medical training must be changed.

The United States Food and Drug Administration currently states that the use of stimulants in patients with tic disorders and/or family history of tic disorders including Tourette's syndrome is contraindicated. Patients with attention deficit hyperactivity disorder (ADHD), however, are at increased risk of tics regardless of stimulants use. After evaluating the most recent literature on the incidence of tic disorders in pediatric patients treated with stimulants for ADHD, it is reasonable to say that the incidence of tics and the severity of tics are not increased by the use of these medications. For patients with pre-existing tic disorders, the usual recommended dosing of stimulants should be used because supratherapeutic doses of this class of medications, specifically dextroamphetamine, have shown to exacerbate tic disorders.

Convulsions with Gastroenteritis: Reflections on some Cases and Tentative Diagnostic Score by Lunardi Sara, Maddaluno Simona, Barrani Monica, Ginocchio Francesca, Parmigiani Stefano (239-243).
Background: Since 1980 a form of benign convulsion during mild gastroenteritis, called Convulsion With Gastroenteritis (CWG) has been described. However diagnostic criteria have not been well defined yet given the similarity of CWG with other diseases of young age and therefore diagnosis is done by exclusion, creating anxiety in parents.
Objective and Methods: We here report a series of cases admitted at our hospital and, making a review analysis of data available, propose a diagnostic score (CWGDS) in order to rapidly individuate the disease from history.
Results: Five cases of seizures associated to gastrointestinal symptoms were admitted to the Pediatric Unit of Eastern Liguria Hospital in La Spezia from January 2014 to April 2015. All these cases could be defined as CWG given their characteristics and benign evolution. We then prepared CWGDS from literature's data and compared our cases with the score itself, having 100% agreement between clinical diagnosis and diagnosis done by CWGDS.
Conclusion: We propose a diagnostic score to help the clinician in diagnosing a benign condition such as CWG therefore avoiding as much as possible invasive procedures and reassuring parents.