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Current Pediatric Reviews
ISSN: 1573-3963

Current Pediatric Reviews
Volume 4, Number 3, August 2008
Contents
Restrictive Versus Liberal Red Blood Cell Transfusion Strategies
for Preterm Infants: A Systematic Review of Randomized Controlled
Trials Pp. 143-150
Dirk Bassler, Marcus Weitz, Anja Bialkowski
and Christian F. Poets
[Abstract]
Congenital Choledochal Malformation –
Current Concepts and Classification Pp.
151-155
Saravanakumar Paramalingam and Mark
Davenport
[Abstract]
A Review of Pediatric Mediastinal Masses
and Castleman’s Disease Pp. 156-158
Amrita Dosanjh
[Abstract]
Cardiovascular Surveillance of Duchenne
and Becker Muscular Dystrophy and Female Carriers Pp.
159-163
Virginia Spehrs-Ciaffi, Pierre-Yves Jeannet and
Nicole Sekarski
[Abstract]
The Virus Infection and Biliary Atresia
Pp. 164-168
Jiexiong Feng and Lei Huang
[Abstract]
Clinical Outcomes for Severe Early Childhood
Caries Pp. 169-173
Jeffrey M. Karp and Robert J. Berkowitz
[Abstract]
Growth Retardation in Children with Celiac
Disease Pp. 174-177
Cristina Meazza, Pagani Sara, Giovenale
Diletta, Kamilia Laarej and Bozzola Mauro
[Abstract]
Different Treatment Strategies for Pediatric
Brain Tumors Pp. 178-186
Amedeo Fiorillo
[Abstract]
Bone Health in Children with Cerebral
Palsy Pp. 187-193
Jared A. Greenberg, Thomas J. Schnitzer
and Deborah Gaebler Spira
[Abstract]
Hypothyroidism and High Plasma and Urine
Iodine Levels Related to the Use of Gastrografin Pp.
194-197
Susana Ares, Miguel Saénz de Pipaón
Marcos, Ana Isabel Ruiz-Díaz, Gabriela Morreale de
Escobar and José Quero
[Abstract]
Safety of Inhaled Corticosteroids. Why
the Variation in Systemic Adverse Effects? Pp.
198-215
Michael B. Anthracopoulos, Kostas N. Priftis
and George Russell
[Abstract]
Abstracts

[Back to top]
Restrictive Versus Liberal Red Blood
Cell Transfusion Strategies for Preterm Infants: A Systematic
Review of Randomized Controlled Trials
Dirk Bassler, Marcus Weitz, Anja Bialkowski
and Christian F. Poets
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.
[Back to top]
Congenital Choledochal Malformation – Current Concepts
and Classification
Saravanakumar Paramalingam and Mark
Davenport
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.
[Back to top]
A Review of Pediatric Mediastinal Masses and Castleman’s
Disease
Amrita Dosanjh
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.
[Back to top]
Cardiovascular Surveillance of Duchenne and Becker Muscular
Dystrophy and Female Carriers
Virginia Spehrs-Ciaffi, Pierre-Yves Jeannet and
Nicole Sekarski
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.
[Back to top]
The Virus Infection and Biliary Atresia
Jiexiong Feng and Lei Huang
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.
[Back to top]
Clinical Outcomes for Severe Early Childhood Caries
Jeffrey M. Karp and Robert J. Berkowitz
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.
[Back to top]
Growth Retardation in Children with Celiac Disease
Cristina Meazza, Pagani Sara, Giovenale
Diletta, Kamilia Laarej and Bozzola Mauro
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.
[Back to top]
Different Treatment Strategies for Pediatric Brain Tumors
Amedeo Fiorillo
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.
[Back to top]
Bone Health in Children with Cerebral Palsy
Jared A. Greenberg, Thomas J. Schnitzer
and Deborah Gaebler Spira
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.
[Back to top]
Hypothyroidism and High Plasma and Urine Iodine Levels Related
to the Use of Gastrografin
Susana Ares, Miguel Saénz de Pipaón
Marcos, Ana Isabel Ruiz-Díaz, Gabriela Morreale de
Escobar and José Quero
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.
[Back to top]
Safety of Inhaled Corticosteroids. Why the Variation in Systemic
Adverse Effects?
Michael B. Anthracopoulos, Kostas N. Priftis
and George Russell
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 hypothalamic-pituitary-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.
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