Genitourinary rhabdomyosarcoma: Which treatment,how much,and when?

ObjectiveTo review the current management of pediatric genitourinary rhabdomyosarcoma (RMS).MethodsStudies performed by the Intergroup Rhabdomyosarcoma Study Group, Children's Oncology Group (COG), International Society of Paediatric Oncology (SIOP) and others over the past 10 years were reviewed to compare the use of surgery, chemotherapy, and radiotherapy for treatment of RMS and their associated outcomes.ResultsEquivalent overall survival rates were reported in the last COG and SIOP trials, with worse event-free survival rates for bladder/prostate RMS in SIOP trials. The use of radiotherapy for local control was the main difference between current COG and SIOP protocols. Surgery is used to diagnose RMS, and for local control after chemotherapy. Chemotherapy is used for systemic control of RMS, but metastatic RMS will require new approaches.ConclusionRisk stratification and risk-based therapy are being studied to decrease morbidity from treatment of RMS. The proper role of surgery vs radiotherapy for local control and whether additional treatment with second-line chemotherapy outweighs the avoidance of radiotherapy remain to be defined.     

Antibiotic therapy for pediatric community-acquired pneumonia: do we know when, what and for how long to treat?

Community-acquired pneumonia (CAP) is a common cause of morbidity among children in developed countries and accounts for an incidence of 10-40 cases per 1000 children in the first 5 years of life. Given the clinical, social and economic importance of CAP, there is general agreement that prompt and adequate therapy is essential to reduce the impact of the disease. The aim of this discussion paper is to consider critically the available data concerning the treatment of uncomplicated pediatric CAP and to consider when, how and for how long it should be treated. This review has identified the various reasons that make it difficult to establish a rational approach to the treatment of pediatric CAP, including the definition of CAP, the absence of a pediatric CAP severity score, the difficulty of identifying the etiology, limited pharmacokinetic (PK)/pharmacodynamic (PD) studies, the high resistance of the most frequent respiratory pathogens to the most widely used anti-infectious agents and the lack of information concerning the changes in CAP epidemiology following the introduction of new vaccines against respiratory pathogens. More research is clearly required in various areas, such as the etiology of CAP and the reasons for its complications, the better definition of first- and second-line antibiotic therapies (including the doses and duration of parenteral and oral antibiotic treatment), the role of antiviral treatment and on how to follow-up patients with CAP. Finally, further efforts are needed to increase vaccination coverage against respiratory pathogens and to conduct prospective studies of their impact.     

Post‐operative radiotherapy for Ewing sarcoma: when,how and how much?

Postoperative radiotherapy in Ewing family of tumors has undergone continuous evolution over the last few decades to establish its role in the combined modality management of these tumors. The process of evolution is still far from over. This review analyzes the evidence from major multi-institutional prospective trials as well as large retrospective institutional series in Ewing tumors to determine the current standards and controversies in postoperative radiation. The indications of PORT, radiation dose-fractionation, timing, target volumes and treatment planning, as well as the late effects are reviewed. A summary of evidence based consensus is presented and unresolved aspects are discussed. Pediatr Blood Cancer 2008;51:575-580. (c) 2008 Wiley-Liss, Inc.     

Surgical treatment of neuropathic urinary incontinence in 2005. When, what, and how?

We present our current opinions on the surgical treatment of urinary incontinence in children with spina bifida. The age of treatment, preferred treatment modalities and results are discussed. We emphasize the importance of initiating treatment for incontinence at an early age as well as the use of effective surgical techniques.     

Induction therapy: Why,when, and which agent?

Krischock L, Marks SD. Induction therapy: Why, when, and which agent?
Pediatr Transplantation 2010: 14:298–313. © 2010 John Wiley & Sons A/S. Abstract: The long‐term outcome of paediatric transplantation has improved over the last decade with an increase in the armamentarium of immunosuppressive agents. However, the battle against the hostile immune response at the time of and after transplantation continues. Induction therapy can reduce early injury, to optimize the long‐term allograft survival. The goal of induction immunosuppression in paediatric transplantation is to permit the use of lower doses of maintenance immunosuppressive agents without increased rates of acute allograft rejection and chronic allograft damage. The aim of this review is to summarize the current literature relating to the use of antibody agents for induction in paediatric solid organ transplantation.     

Research Review: Altered reward function in adolescent depression: what,when and how?

Background: Conceptual models and recent evidence indicate that neural response to reward is altered in depression. Taking a developmental approach to investigating reward function in adolescent depression can elucidate the etiology, pathophysiology and course of depression, a disorder that typically begins during adolescence and has high rates of recurrence. Methods: This conceptual review describes the what, when and how of altered reward function in adolescent depression. With the goal of generating new, testable hypotheses within a developmental affective neuroscience framework, we critically review findings and suggest future directions. Peer‐reviewed empirical papers for inclusion in this critical review were obtained by searching PubMed, PsycInfo and ScienceDirect for the years 1990–2010. Results: A pattern of low striatal response and high medial prefrontal response to reward is evident in adolescents and adults with depression. Given the salience of social stimuli for positive affect and depression, reward function might be especially disrupted in response to social rewards. Because of changes in the dopamine system and reward function with aging, altered reward function in depression might be more evident during adolescence than later in life; however, low reward function may also be a stable characteristic of people who experience depression. Mechanisms of altered reward function in depression could include disrupted balance of corticostriatal circuit function, with disruption occurring as aberrant adolescent brain development. Conclusions: Future studies should examine responses to social rewards; employ longitudinal and prospective designs; and investigate patterns of functional connectivity in reward circuits. Understanding altered reward function in depression has potential implications for treatment development. A more rigorous approach to investigating anhedonia, threat–reward interactions and comorbid anxiety will be valuable to future progress in describing the role of reward function in the pathophysiology of depression.     

Food-induced anaphylaxis: who, what, why, and where?

Food-induced anaphylaxis is a leading cause of anaphylaxis treated in emergency departments and hospitals around the world. Peanuts, tree nuts, fish, and shellfish are the most commonly implicated foods. Food-induced anaphylaxis may occur in any age group and with any food. However, food-induced anaphylaxis fatalities disproportionately affect adolescents and young adults with peanut and tree nut allergy. Individuals who have both IgE-mediated food allergy and asthma are at a higher risk for food-induced anaphylaxis fatality. Delayed administration of epinephrine is also associated with fatal outcome. Often, in fatal reactions, the food allergen is unknowingly ingested away from home, in settings such as restaurants and schools. Although avoidance of food allergens is critical, timely administration of epinephrine is also of great importance in the treatment of food-induced anaphylaxis. Patients, families, and caregivers must be well educated regarding the signs, symptoms and risk factors for anaphylaxis. They must also be counseled on the importance of strict food avoidance of the implicated food allergens, compliance with having self-injectable epinephrine available at all times, and the importance of timely administration of epinephrine, even when cutaneous symptoms are lacking.     

Neonatal neutropenia: what diagnostic evaluation is needed and when is treatment recommended?

Neutropenia is a relatively frequent finding in the neonatal intensive care unit, particularly in very low birth weight neonates during the first week of life. Healthy term and preterm neonates have blood neutrophil counts within the same basic range as adults, but their neutrophil function, and their neutrophil kinetics during infection, differ considerably from those of adults. Neutrophil function of neonates, particularly preterm neonates, is less robust than that of adults and might also contribute to the increase in propensity to infection. In premature infants, early-onset neutropenia is correlated with sepsis, maternal hypertension, intrauterine growth restriction, severe asphyxia, and periventricular haemorrhage, and might be associated with an increase in the incidence of early-onset sepsis, nosocomial infection, and Candida colonisation. Some varieties of neutropenia in the NICU are very common and others are extremely rare. The most common causes of neutropenia in the NICU have an underlying cause that is often evident, and require little diagnostic evaluation. Unlike, persistent neutropenia should prompt evaluation even if it is of moderate severity. The laboratory tests to consider are those that provide a specific diagnosis. The first tests that should be ordered are a blood film, a complete blood count on the mother, and, if her blood neutrophil concentration is normal, maternal neutrophil antigen typing and an anti-neutrophil antibody screen. A bone marrow biopsy can be useful in cases with prolonged, unusual, or refractory neutropenia. Various treatments have been proposed as means of enhancing neutrophil production and function in preterm infants. Both recombinant granulocyte stimulating factor and recombinant granulocyte macrophage-colony-stimulating factor have been tried with variable success. Intravenous immunoglobulin, corticosteroids, granulocyte transfusions, and gamma interferon did not show a clear adequate beneficial role for the therapy of neonatal neutropenia.     

Pulmonary metastases after nephrectomy only for small, stage I/favorable-histology Wilms’ tumor

The prognosis for children with Wilms tumor is reported to be excellent in those who are less than 2 years of age at diagnosis and who have a stage I/favorable-histology tumor with specimen weight less than 550 g. We report on a patient with Wilms tumor who belonged to this group but who developed pulmonary metastases, and we discuss the diagnostic and therapeutic problems in such patients. The importance of careful evaluation of the renal sinus should be emphasized.     

Roux-en-Y hepaticojejunostomy or hepaticoduodenostomy for biliary reconstruction during the surgical treatment of choledochal cyst: which is better?

We reviewed our experience of Roux-en-Y hepaticojejunostomy (RYHJ) and hepaticoduodenostomy (HD) performed for the surgical repair of choledochal cyst (CC), with special emphasis on postoperative complications related to the type of biliary reconstruction performed. Eighty-six patients underwent primary cyst excision for CC from 1986 to 2002 at our institution. Forty-six cases with concurrent intrahepatic bile duct dilatation (IHBD) were excluded because HD was not used for biliary reconstruction if IHBD was present. Thus, 28 cases had RYHJ, and 12 had HD. Differences between the RYHJ and HD groups with respect to type of CC, age at cyst excision, and length of follow-up were not statistically significant. However, the incidences of postoperative complications related to biliary reconstruction, such as endoscopy-proven bilious gastritis due to duodenogastric bile reflux [4/12 (33.3%) of the HD group], and adhesive bowel obstruction/cholangitis [2/28 (7.1%) of the RYHJ group] were significantly different (p<.05). Our experience suggests that HD is not ideal for biliary reconstruction in CC because of a high incidence (33.3%) of complications due to duodenogastric bile reflux. Currently, RYHJ is our exclusive technique of choice for biliary reconstruction during the surgical repair of CC.     

Theophylline or caffeine: which is best for apnea of prematurity?

Apnea of prematurity is a common problem of the premature infant under 30 weeks gestation. Theophylline and caffeine, two methylxanthines, are widely used to treat this condition. The drugs are equally effective in preventing apnea in the premature infant. Caffeine citrate has many advantages over theophylline, however, including once-a-day dosing, more predictable plasma concentrations, earlier onset of action, and minimal side effects. Caffeine is therefore the initial drug of choice for apnea of prematurity.     

Post‐transplant lymphoproliferative disorder resembling Wilms tumor. Diagnostic dilemma: Renal biopsy or nephrectomy?

Cheng E, Fustino N, Klesse L, Chinnakotla S, Sanghavi R. Post‐transplant lymphoproliferative disorder resembling Wilms tumor. Diagnostic dilemma: Renal biopsy or nephrectomy?
Pediatr Transplantation 2011: 15: E187–E191. © 2010 John Wiley & Sons A/S. Abstract: Post‐transplant lymphoproliferative disorder is a life‐threatening neoplasm that can occur after orthotopic liver transplant. We report a 14‐month‐old female status‐post OLT with an atypical presentation of PTLD as a solitary renal mass. At eight‐wk post‐transplant, she presented with elevated transaminases, CMV counts (73 000 copies/mL), and EBV counts (35 000 copies/mL). CT scan revealed a solid heterogeneously enhancing right renal mass measuring 2.6 × 2.4 × 3.3 cm. The radiological diagnosis was Wilms tumor, although PTLD could not be excluded. Complete resection of a Wilms tumor is potentially curative. A needle biopsy would upstage the malignancy and result in radiochemotherapy that is deleterious to a liver graft. The mass was not amenable to partial nephrectomy. A total nephrectomy, given life‐long nephrotoxic immunosuppressants, was an unfavorable option. Thus, needle biopsy was performed. Histology confirmed monoclonal, EBV‐associated PTLD and diffuse large B‐cell lymphoma. Her therapy included immunosuppression reduction, cyclophosphamide, steroids, and anti‐CD20 monoclonal antibody. Concomitantly, she received Cytogam and gancyclovir. Complete remission was achieved three months after chemotherapy. This case illustrates that young age, CMV infection, and EBV infection are strong risk factors for PTLD. With such risk factors present, any mass or lesion in a solid organ transplant patient should be considered PTLD until proven otherwise.