Current Pediatric Reviews - Volume 4, Issue 3, 2008

Background: Many neonatal intensive care units have introduced restrictive transfusion policies for preterm infants. The justification for such an approach is debatable. Objective: To systematically review the evidence from randomized controlled trials (RCTs) on the benefit to risk ratio of lower versus higher red blood cell transfusion thresholds in preterm infants. Methods: Systematic review based on an electronic literature search (09/2007) in PubMed and CENTRAL (Cochrane Library, Issue 3 2007) for RCTs on different transfusion strategies in preterm infants. Results: Seven fully published RCTs including 712 preterm infants with various gestational and postnatal ages were found to be eligible for inclusion in this systematic review. Transfusion thresholds differed between trials, as did the duration of studies, the reported outcomes and the methodological quality. The largest RCT included 451 Extremely-Low-Birth- Weight (ELBW) infants, was of high methodological quality and found no difference in reducing mortality before discharge home or survival with any of either severe retinopathy of prematurity, bronchopulmonary dysplasia or brain injury. Conclusions: Clinical and methodological heterogeneity between studies prevents firm conclusions based on the totality of available evidence. According to the results of the largest RCT, maintaining a higher hemoglobin level in ELBW infants seems to confer little clinical benefit.

Congenital choledochal malformations (a.k.a. choledochal cysts) are uncommon but must be considered in the differential diagnosis of conjugated jaundice in both infant and child. They are also an important cause of recurrent abdominal pain due to the presence of a common pancreatic biliary channel and predisposition to pancreatitis. We present an updated Kings College Hospital classification based on morphology derived from previous versions in the literature and review possible aetiological factors, current diagnostic and surgical strategies.

Pediatric thoracic masses are usually found in the mediastinum. The three anatomic compartments each have distinct differential diagnoses. Castleman's Disease, a rare form of giant lymph node hyperplasia has been described in children with mediastinal masses. While most forms are localized, multicentric disease can also occur. This review provides a summary of pediatric mediastinal disease.

Duchenne muscular dystrophy is the most common form of the childhood muscular dystrophies. It follows a predictable clinical course marked by progressive skeletal muscle weakness, lost of ambulation before teen- age and death in early adulthood secondary to respiratory or cardiac failure. Becker muscular dystrophy is less common and has a milder clinical course but also results in respiratory and cardiac failure. Female carriers of both entities are also affected in a variable degree. The natural history of the cardiac involvement in these diseases has not been well established and without screening it progresses asymptomatically until the cardiac reserve has been eroded, when symptoms and signs of cardiac failure emerge. The purpose of this work is to provide recommendations for adequate cardiovascular care in this particular population based on the updated literature as referenced.

Biliary Atresia (BA) is one of the most common cause of neonatal cholestasis with unclear pathogenesis currently. Virological investigations had proved that BA might be closely connected with the pathogen infections especially viral infection. In this article, we have summarized the recent advancements in viral etiology and epidemiology of BA, presented the possible etiological factors such as cytomegalovirus (CMV), reovirus, rotavirus, human papilloma virus (HPV) and illuminated the unknowns of this disease and further research directions in the future. Epidemiologic studies had demonstrated a close relationship between BA and some kinds of explosive pathogen infections for its high seasonal and regional characters, which might present with different patterns in different countries. Virological researches had shown BA infants had a high rate of CMV infection detected both in blood and tissues samples. What's more, pregnant mice and newborn mice which were infected by reovirus and rotavirus could be developed into BA model. However, relationship between other viruses such as HPV and HHV-6 and BA needs further investigation. With the development of laboratory techniques and deep research in different areas, consummate and precise viral etiology of BA will be established to guide our clinical and research work in the future.

Severe Early Childhood Caries (S-ECC) is a public health care problem affecting toddlers and preschool children worldwide. S-ECC is an infectious disease most frequently characterized by an overwhelming mutans streptococci infection. The current community standard of care for S-ECC calls for the removal and restoration of carious teeth, application of topical fluoride agents, oral hygiene instruction, and counseling regarding decay-promoting feeding behaviors. Dental surgery alone has minimal impact on oral mutans streptococci reservoirs in the setting of S-ECC and counseling regarding feeding behaviors by dental professionals has largely been unsuccessful. Not surprisingly, clinical outcomes for S-ECC treated under sedation or general anesthesia are poor. Improved clinical outcomes for S-ECC may be realized through treatment strategies that focus on the infectious basis of this disease. Suppression of oral mutans streptococci reservoirs to non-pathogenic levels with a topical anti-microbial agent shows promise as the approach of choice. Preliminary studies using a one time intra-operative application of 10% povidone iodine solution to the dentition in the setting of S-ECC has produced persistent suppression of salivary mutans streptococci reservoirs for 3 months post dental surgery. This paper reviews the relevant literature focusing on this important pediatric health care issue.

Celiac disease (CD) is a permanent intolerance to gluten that results in damage of the small intestinal mucosa, and it is one of the common causes of chronic malabsorption in Caucasian children. In CD children, short stature could be the only presenting clinical feature, even in absence of gastroinstestinal symptoms. Generally, withdrawal of gluten from the diet leads to a rapid catch-up growth of body weight within 6-12 months, whereas the height catches up more gradually. A degree of dysfunction of the endocrine axis could be observed in children with CD, but the pituitary function usually normalized after the institution of gluten-free diet. On the other hand, it has been previously reported some patients with isolated and multiple GH deficiency (GHD) showing no catch-up growth during a gluten-free diet, in spite of reversion to seronegativity for EMA. These patients could benefit from substitutive GH therapy as idiopathic GHD ones. This review deals with the problem of linear growth in CD children and points to the importance of the evaluation of GH secretion in those children who showed no catch-up growth after the introduction of gluten-free diet in order to start a GH replacement therapy whether GHD is documented.

Malignant brain tumors are characterized by high local aggressiveness and tendency to metastasize within the central nervous system (CNS), and rarely outside it. Surgical approach represented and still represents the mainstay of treatment strategies. However, in the majority of cases, gross total resection is recommended to achieve long event-free survival and eventually the cure. In recent years neoadjuvant chemotherapy and adjuvant post-surgical radiotherapy and chemotherapy have gained relevance in facilitating complete surgical removal and in achieving and consolidating complete remission of such tumors. Post-surgical radiation therapy still represents the basic adjuvant treatment for children over three years with standard risk, non metastatic medulloblastoma. This approach is also considered a very important tool for children affected by ependymoma. However, associated chemotherapy is also needed when reduced doses of radiation therapy are administered, in cases of tumors characterized by a more aggressive biologic behaviour, such as high grade gliomas, and metastatic tumors of any origins. The majority of CNS tumors are of neuroepithelial origin and as such responsive to many antineoplastic drugs, like alkylating agents, antimetabolites, and alkaloids. However, in order to reach tumor cells inside the CNS these drugs have to overcome the blood brain barrier. A number of interesting tools able to disrupt or bypass the barrier, such as ionizing radiations, osmotic procedures or the liposomal technology applied to antiblastic drugs, exists. Starting from a dose of radiation of 30 Gy the permeability of an irradiated area is significantly increased compared to that of the unirradiated surrounding cerebral tissue. Many present treatment regimens include concurrent radiotherapy and chemotherapy in the post-surgical phase, and encouraging results have been reported. In the future better results will certainly come from modern technologies applied to radiation therapy, such as tomo-therapy, and from discovery of new drugs or alternative methods of administration of conventional ones.

Children with cerebral palsy are at increased risk of fracture. Factors that may increase risk include decreased bone mineral density, older age, increased severity of disease, immobility, poor nutritional status, anticonvulsant medication use, and abnormal serum factor levels. Still, it is not clear which patients with cerebral palsy would most benefit from risk reduction strategies and exactly what those strategies should be. The goal of this review is to raise awareness of the issue of bone health in children with cerebral palsy and to suggest areas for further research.

We report the effect of Gastrografin on thyroid function in 7 neonates. Levels of free thyroxine and thyroid stimulating hormone, serum and urine iodine concentrations were determined before and after using gastrografin. Transient hypothyroidism was seen in 3 patients. No thyroid gland dysfunction was detected in the Neonatal Screening Program for congenital hypothyroidism before the administration of Gastrografin and thyroid antibodies were negative. Urine and serum iodine concentrations were elevated after Gastrografin administration. Normal thyroid hormone concentrations were observed in the other four infants. We suggest careful evaluation of thyroid function whenever Gastrografin is used.

Inhaled corticosteroids (ICS) are currently the mainstay of therapy in persistent childhood asthma. However, systemic adverse effects of various available agents - particularly on growth, bone metabolism, and the hypothalamicpituitary- adrenal (HPA) axis - continue to concern the families of asthmatic children, and are debated within the medical community. Although there is short- and medium-term suppressive effect of usual doses of ICS on linear growth, the findings of long term studies are reassuring, suggesting attainment of the full potential of adult height. ICS do not appear to exert significant untoward effects on bone mineral density. Measurable systemic effect of ICS by sensitive measurements of function of the HPA axis does not necessarily translate into clinically significant side effects. However, use of higher than recommended doses of ICS may on occasion lead to symptomatic adrenal insufficiency and should be of concern; there may be important differences among available compounds regarding such potential. Variation in adverse effects of ICS is explained by differences in their pharmacokinetic and pharmacodynamic properties, formulation and delivery devices, and patient factors such as degree of airway obstruction, suppressive effect of airway inflammation per se on the HPA axis, genetic factors, patient training in the use of delivery devices, and compliance.