Primary thrombocytosis in children

Myeloproliferative neoplasms are uncommon disorders in children, for which we have limited understanding of the pathogenesis and optimal management. JAK2 and MPL mutations, while common drivers of myeloproliferative neoplasms in adult patients, are not clearly linked to pediatric disease. Management and clinical outcomes in adults have been well delineated with defined recommendations for risk stratification and treatment. This is not the case for pediatric patients, for whom there is neither a standard approach to workup nor any consensus regarding management. This review will discuss thrombocytosis in children, including causes of thrombocytosis in children, the limited knowledge we have regarding pediatric primary thrombocytosis, and our thoughts on potential risk stratification and management, and future questions to be answered by laboratory research and collaborative clinical study.     

Update on the Management of Pediatric Chronic Hepatitis B

A primary challenge in the management of pediatric chronic hepatitis B (CHB) is the lack of established child-specific guidelines. Treatment decisions for children with CHB are extrapolated from the robust adult-focused treatment and management guidelines. However, the rationale for treating children differs from adults, and appropriate patient selection for treatment is imperative to avoid mutant virus development and an indefinite treatment course. Few approved therapies exist for children less than 10 years of age, a group who may benefit greatly from novel and effective therapeutics. It is hoped this gap will be met in the near future, as the field of CHB is rapidly evolving and new therapeutics come to market. This review focuses on the currently available pediatric data regarding treatment for CHB, and draws comparisons with adult-focused data where applicable.     

Clinical trials in pediatric uveitis

Uveitis associated with juvenile rheumatoid arthritis is the most common form of ocular inflammation in children. Prevention of permanent visual damage by this silent disease requires heightened awareness from pediatric rheumatologists and ophthalmologists. Early prediction of severity and prognosis will aid in the identification of those patients requiring more aggressive management. Generally, reports using immunosuppressives in this population are small, uncontrolled, and retrospective. Large-scale collaborative studies have been proposed and are underway. Hopefully, collaborations between pediatricians, pediatric rheumatologists, and ophthalmologists will allow the development of straightforward treatment guidelines for children suffering from chronic uveitis and its related complications.     

Current Strategies in Treating Severe Contact Dermatitis in Pediatric Patients

Allergic contact dermatitis in children is underdiagnosed and undertreated, and its incidence is increasing. Appropriate history taking and the suspicion for allergic contact dermatitis is essential, and patch testing remains the gold standard in diagnosis. Avoidance of the offending allergen, once identified, is the first goal of treatment. Medical therapies include topical corticosteroid and topical immunomodulators. In severe cases, oral corticosteroids or immunomodulators are utilized, although prospective randomized trials for the treatment of this disease in children are lacking. A PubMed literature search was performed to identify publications on allergic contact dermatitis in the pediatric population with the keywords: dermatitis, children, allergic contact dermatitis, pediatrics, contact hypersensitivity, contact allergy, treatment, and management. This review will address the major principles behind the diagnosis and management of this disease in the pediatric population, and highlight useful strategies that may result in improved treatment of this condition.     

Mycoses in pediatric patients

For more than 40 years, there has been limited progress in the treatment of invasive fungal infections. There are now numerous nuances to choosing the appropriate antifungal agent. Important advantages have been achieved in understanding the safety, tolerability, and pharmacokinetics of these agents. One of the most important aspects for successful management of pediatric invasive fungal infections is an understanding of the differences in the pharmacokinetics of the drugs in children and adults to offer optimal dosing strategies. Unfortunately there have been few antifungal studies conducted in children. Consequently most information for the pediatrician has been extrapolated from adult data. The breadth of antifungal data in children is expanding, however, with newer studies underway. Through the efforts of dedicated clinicians and collaboration, pediatric indications and dosing strategies will eventually be discovered that directly benefit pediatric patients.     

Pediatric pulmonologists approach to the pre-operative management of the asthmatic child

Objective: No consensus guidelines exist for the respiratory treatment of asthmatic children referred for elective surgery. The aim of this study was to evaluate the attitude of pediatric pulmonologists regarding the pre-operative management of these children. Methods: A survey of pre-operative management of asthmatic children was conducted. All 48 certified pediatric pulmonologists in Israel completed a questionnaire that comprised 20 questions regarding their approach to pre-operative management including six case scenarios with a variety of clinical situations and treatments of children with asthma. Results: Response rate was 100%. All believed that pre-operative treatment should be considered in all asthmatic children. Almost 50% suggested that a pediatric pulmonologist should be consulted in all pre-operative assessments. 50% recommended consultation only in individual cases. Overall, results showed a very wide variability between responders especially in pre-school and poorly controlled school children. The variability referred to the use of bronchodilators, inhaled corticosteroids and their combination during the pre-operative days, the addition of systemic CS and the length of pre-operative treatment. Almost all participants suggested either the initiation or augmentation of pre-operative treatment in high risk situations. Conclusions: This data demonstrate an important variability among pediatric pulmonologists in Israel regarding the practice of pre-operative treatment of infants and children with asthma especially for the less controlled and high risk children. This is most probably explained by the paucity of evidence-based data and the lack of established guidelines. Consensus guidelines for the pre-operative management of asthmatic children are needed.     

New devices for pediatric mechanical circulatory support

PURPOSE OF REVIEW: In adult patients with advanced heart failure, mechanical circulatory support devices (MCSDs) offer a valid treatment option. MCSDs can serve as a bridge to transplant, bridge to recovery or destination therapy. In the REMATCH trial, MCSDs outperformed medical management for long-term support of end-stage heart failure. Until recently, pediatric patients could not fully benefit from this development due to lack of suitable devices. This review provides an overview of pediatric MCSDs currently available in the US and those in the development stage. We discuss the emerging role of extracorporeal membrane oxygenation compared with MCSDs. RECENT FINDINGS: Small patients with a body-surface area below 0.7 m now have access to the pediatric Berlin Heart Excor. For larger children, several adult MCSDs may be suitable, including the Abiomed BVS-5000, Thoratec, HeartMate and DeBakey MCSD. Five pediatric MCSDs supported by NIH contracts issued in 2004 are now in preclinical testing. Other pediatric MCSDs are already in clinical use abroad on a small scale. SUMMARY: There is mounting experience with MCSDs in children with advanced heart failure. MCSDs will increasingly have to be considered as a treatment option for pediatric heart-transplant candidates and may serve as a bridge to recovery.     

Children on losing end of access to new drugs

Only five of the fourteen approved AIDS drugs have been tested in children, and there are no approved protease inhibitors for pediatric use. Eighty percent of the drugs on the market have never been tested in children. As a result, physicians are forced to prescribe AIDS medication for off-label use. Further development of pediatric AIDS drugs has decreased, largely due to the effectiveness of AZT in preventing perinatal transmission. A proposed White House rule would require pharmaceutical companies to test more drugs in children and to submit pediatric test data to the Food and Drug Administration (FDA) at the same time or shortly after a drug is approved for adults. The AIDS Policy Center and other groups are concerned that loopholes in the proposal will give manufacturers a way to avoid pediatric testing. The International Association for Physicians in AIDS Care (IAPAC) has launched a demonstration project in several States that will work to create resources and structures to provide HIV-positive children with access to all HIV drugs within three years. In 1998, the group will meet with drug companies, clinicians, and government health officials to set an agenda for pediatric HIV treatment research and to develop a pediatric HIV mentor program. Four areas for pediatric research improvement are cited: develop drug formulations, undertake combination therapy clinical trials, distribute pediatric treatment information to clinicians, and guarantee access to approved drugs, regardless of ability to pay.     

Recent Developments in Pediatric Community-Acquired Pneumonia

Community-acquired pneumonia (CAP) is the most common acute infectious cause of death in children worldwide. Consequently, research into the epidemiology, diagnosis, treatment, and prevention of pediatric CAP spans the translational research spectrum. Herein, we aim to review the most significant findings reported by investigators focused on pediatric CAP research that has been reported in 2014 and 2015. Our review focuses on several key areas relevant to the clinical management of CAP. First, we will review recent advances in the understanding of CAP epidemiology worldwide, including the role of vaccination in the prevention of pediatric CAP. We also report on the expanding role of existing and emerging diagnostic technologies in CAP classification and management, as well as advances in optimizing antimicrobial use. Finally, we will review CAP management from the policy and future endeavors standpoint, including the influence of clinical practice guidelines on clinician management and patient outcomes, and future potential research directions that are in the early stages of investigation.     

Hypertension in children and adolescents: An approach to management of complex hyper tension in pediatric patients

Although primary (essential) hypertension is detectable in childhood, secondary causes of hypertension must be considered in evaluating and managing hypertension in children and adolescents. Very young children and children with severe hypertension may have an underlying cause of the hypertension. Interventions to control elevated blood pressure (BP) are clinically important for all children with high BP. Nonpharmacologic approaches are recommended for all asymptomatic children with hypertension and prehypertension. Some children and adolescents will require pharmacologic therapy to control BP and to optimize organ protection. Recent advancements in pediatric clinical trials of antihypertensive agents have provided data on BP-lowering effects and safety in children. Little has been published on the choice and use of various classes of antihypertensive drugs for management of secondary hypertension in children and adolescents. This review focuses on the clinical management of specific types of secondary hypertension in pediatric patients.     

Dietary supplements and pediatric non-alcoholic fatty liver disease: Present and the future

Non-alcoholic fatty liver disease (NAFLD) is the most common chronic liver disease in children. High prevalence of pediatric obesity and sedentary lifestyle has augmented the incidence of NAFLD in children. Obesity is associated with an increased risk of NAFLD through various mechanisms such as intensification of insulin resistance and increased levels of inflammatory markers. There is no approved medical intervention for treatment of pediatric NAFLD; the only proven strategy in management of pediatric NAFLD is lifestyle modification. Recently, the effects of nutritional supplements have been examined in the management of pediatric NAFLD. The purpose of this review is to summarize the studies evaluating the effects of nutritional supplements on pediatric NAFLD and explain the future direction in this field.     

Complications in pediatric endoscopy

The experience of the “endoscopic community” in pediatric patients is limited, but during recent years increased skills of the endoscopists and technological improvements lead to a standardization of pediatric endoscopy and the development of specialized pediatric endoscopy unit.Adverse events related to diagnostic and therapeutic endoscopy in children are usually rare. Diagnosis, prevention and treatment of complications in pediatric endoscopy is crucial when dealing with benign diseases in children.The complication rate of diagnostic EGD and colonoscopy in children are extremely low. Therapeutic procedures have obviously an increased rate of adverse events. Esophageal dilations are the most common indication for endoscopic therapy in children and can lead to perforations which requires prompt diagnosis and management. Complications of ERCP in pediatric age are similar to those reported in adults.The experience in pediatric emergency endoscopy (mainly foreign body removal) is consolidated and related adverse events extremely rare.Sedation of children during endoscopy maybe needs further evaluation and standardization, to reduce the rate of specific complications.     

Thromboembolic diseases of childhood

Over the last decade, pediatric thrombophilia programs have emerged around the world as a new discipline in pediatric hematology. These programs specialize in the diagnosis, prevention and treatment of children with thromboembolic events (TEs) in both the venous and arterial systems. The need for separate pediatric programs has been discussed previously. (J Pediatr Hematol Oncol 1997; 19: 7-22.) The following article will update previous reviews (Hematol Oncol Clin North Am 1998; 12: 1283-1312; Thromb Haemost 1997; 78: 715-725) and will concentrate on three aspects: (1) The risk factors for acquiring TEs; (2) The confirmatory diagnostic tests used in children with TEs; and (3) The different antithrombotic agents used for prevention and treatment. The current knowledge in respect to the above points is only the "tip of the iceberg". Well-designed prospective trials are required to establish the contribution of congenital prothrombotic disorders, appropriate diagnostic strategies, and optimal therapy for children with TEs.     

Irritable bowel syndrome in children:Current knowledge,challenges and opportunities

Irritable bowel syndrome(IBS) is a common and troublesome disorder in children with an increasing prevalence noted during the past two decades. It has a significant effect on the lives of affected children and their families and poses a significant burden on healthcare systems. Standard symptom-based criteria for diagnosis of pediatric IBS have changed several times during the past two decades and there are some differences in interpreting symptoms between different cultures. This has posed a problem when using them to diagnose IBS in clinical practice. A number of potential patho-physiological mechanisms have been described, but so far the exact underlying etiology of IBS is unclear. A few potential therapeutic modalities have been tested in children and only a small number of them have shown some benefit. In addition, most of the described patho-physiological mechanisms and treatment options are based on adult studies. These have surfaced as challenges when dealing with pediatric IBS and they need to be overcome for effective management of children with IBS. Recently suggested top-down and bottom-up models help integrating reported patho-physiological mechanisms and will provide an opportunity for better understanding of the diseases process. Treatment trials targeting single treatment modalities are unlikely to have clinically meaningful therapeutic effects on IBS with multiple integrating patho-physiologies. Trials focusing on multiple combined pharmacological and non-pharmacological therapies are likely to yield more benefit. In addition to treatment, in the future, attention should be paid for possible prevention strategies for IBS.     

Special issues in pediatric inflammatory bowel disease

The incidence of pediatric inflammatory bowel disease （IBD） is rising and recent advances in diagnostics and therapeutics have improved the care provided to these children. There are distinguishing features worth noting between early onset and adult onset IBD. Physical and psychosocial development remains a critical target for the comprehensive management of pediatric IBD. Children are not just little adults and consideration must be given to the stages of development and how these stages impact disease presentation and management. The final stage will be the transition from pediatric care to that of adult oriented care and special consideration must be given to make this a successful process. This review highlights special considerations in the management of the child with IBD.     

The treatment of differentiated thyroid cancer in children: emphasis on surgical approach and radioactive iodine therapy

Pediatric thyroid cancer is a rare disease with an excellent prognosis. Compared with adults, epithelial-derived differentiated thyroid cancer (DTC), which includes papillary and follicular thyroid cancer, presents at more advanced stages in children and is associated with higher rates of recurrence. Because of its uncommon occurrence, randomized trials have not been applied to test best-care options in children. Even in adults that have a 10-fold or higher incidence of thyroid cancer than children, few prospective trials have been executed to compare treatment approaches. We recognize that treatment recommendations have changed over the past few decades and will continue to do so. Respecting the aggressiveness of pediatric thyroid cancer, high recurrence rates, and the problems associated with decades of long-term follow-up, a premium should be placed on treatments that minimize risk of recurrence and the adverse effects of treatments and facilitate follow-up. We recommend that total thyroidectomy and central compartment lymph node dissection is the surgical procedure of choice for children with DTC if it can be performed by a high-volume thyroid surgeon. We recommend radioactive iodine therapy for remnant ablation or residual disease for most children with DTC. We recommend long-term follow-up because disease can recur decades after initial diagnosis and therapy. Considering the complexity of DTC management and the potential complications associated with therapy, it is essential that pediatric DTC be managed by physicians with expertise in this area.     

全球儿童结核病防控策略的变迁

针对儿童结核病的现状,世界卫生组织、各国的卫生部等机构出台了一系列指南和建议,对规范当前儿童结核病的诊断、治疗、预防、管理起到了非常重要的指导作用。儿童结核病的防控是全球结核病控制的重要方面,笔者对世界卫生组织自1991年DOTS策略提出后至今的指南、建议、声明等出版材料中有关儿童结核病的内容进行梳理和总结,其中包括4个指南、10个建议、2个声明、2个手册、2个路线图和1个综述,以了解儿童结核病防控策略的变化,加强儿童结核病的早期诊断、早期治疗,及时解决当前存在的问题,同时也有助于指南方针的普及和推广。     

Disparities in Asthma Hospitalizations among Children Seen in the Emergency Department

We examined the race/ethnicity variation in the risk of hospitalization among children seen in the emergency department (ED) for asthma. ED and hospitalization records for children 1 to 19 years of age in New Jersey for 2004 and 2005 were linked. The dataset identified 47,548 ED and hospitalizations among 37,216 children. ED and hospitalization rates indicated persistent disparities in pediatric asthma. ED admission rates were similar across race/ethnic groups, suggesting similar management of pediatric asthma patients once they are seen in the ED. Integrating existing ED and hospitalization records will enhance asthma surveillance and the targeting of interventions to reduce race/ethnicity disparities.     

Endoscopic retrograde cholangiopancreatography in pediatrics

Endoscopic retrograde cholangiopancreatography (ERCP) is a useful, although understudied, tool in the diagnosis and treatment of pancreaticobiliary disease in pediatric populations. Although appropriately sized equipment is somewhat limited, 7.5-mm duodenoscopes are available and can be used in even the smallest infants along with a small array of devices to fit through the 2.0-mm channel. Indications for ERCP in children are similar to those in adults and include therapy of biliary and pancreatic ductal obstruction or disruption, although the etiologies of the obstructions may differ greatly between the 2 populations. Rates of complications and procedural success are similar in pediatric and adult patients. Historically, most pediatric ERCPs have been performed by experienced adult endoscopists, but during the past 15 years, there has been increased interest in pursuing therapeutic endoscopy training among pediatric gastroenterologists. Prospective multicentered studies at pediatric centers will help define the role of pediatric ERCP in the treatment of children with pancreatic and biliary disease.     

Liver neoplasia in children

Management of pediatric liver tumors has significantly improved over the last 2 decades. The management options for hepatocelluar carcinoma (HCC) are not well defined. In the pediatric context, the main clinical aims are to reduce chemotherapy toxicity (predominantly ototoxicity and nephrotoxicity) in children treated for hepatoblastoma and to investigate additional modes of treatment for HCC. An increasing number of children develop HCC in the background of chronic liver disease, and screening methods need to be better observed.