Doctor’s Digest
February 2011
A monthly newsletter for St. Louis Children’s Hospital attending and referring medical staffs
In this issue
3 Children’s Direct Reaches
One-Year Anniversary
of Expanded Services
5 SLCH Joins ACS National
Quality Improvement Program
Focused on Pediatric Surgery
6 Case Study: Newborn Seizures
Research Update | Parent-Child Play Therapy Relieves Depression
A form of play therapy between parents and their
toddlers can relieve depression in preschoolers,
according to child psychiatry researchers at
Washington University School of Medicine in
St. Louis.
Known as parent-child interaction therapy, the playbased technique has been used successfully to treat
hyperactivity and disruptive disorders. The researchers
adapted it, adding a focus on emotional development,
to test whether it could help parents teach their
children how to regulate negative emotions, such as
guilt and sadness.
Play therapy sessions
put parents and their
children in one room
while a therapist
observes from a nearby
room.
Joan L. Luby, MD,
is diagnosing and
working to develop
therapies to
treat clinical
depression
in children
as young
as 3.
SLC9201 2/2011
Results from the pilot
study are published
online in the journal
Depression and Anxiety.
Investigators caution
that the findings are
preliminary, involving
only eight preschoolers,
but they call the
outcomes dramatic.
Depression symptoms
improved in all of the
children studied.
“There was a very large
effect,” says principal
investigator Joan L.
Luby, MD, psychiatrist
at St. Louis Children’s
Hospital and professor of
child psychiatry. “From
our past research, we know that children as young as
3 can suffer from clinical depression, but how to treat
it is an open question. Most infant-preschool mental
health providers want to avoid drug treatment, and
there is evidence that psychosocial interventions can
be uniquely effective in young children. Our findings
certainly suggest that may be true for depression.”
Therapy took place during 14 sessions over 18 weeks.
Depression severity scores decreased 44 percent.
Following therapy, most child participants no longer
met the diagnostic criteria for depression.
The study was conducted as an open trial. That is,
all children received the investigational play therapy.
Luby’s group since has conducted a more rigorous
randomized, controlled study that put some parents
and preschoolers into play therapy, while other parents
met weekly with a psychologist to learn about normal
emotional development. The researchers currently are
analyzing the data from that study.
The original therapy was developed in the 1970s by
Sheila M. Eyberg, PhD, distinguished professor of
clinical and health psychology at the University of
Florida in Gainesville.
“The original form of parent-child interaction therapy
had two components,” says lead author Shannon
N. Lenze, PhD, an instructor in the Department of
Psychiatry. “One was a child-directed interaction, and
the other was parent-directed. The first encourages
the parent to use effective praising techniques, to
play games the child wants to play, to get down on
the child’s level and be enthusiastic. The second
component teaches the parent about effective discipline
using a very specific ‘time out’ to help the child learn
to obey parent commands.”
A third novel component, which was developed for
this study, involves emotions and helps parents learn
to help their child more effectively regulate them.
continued on next page
continued from previous page
Share Your Ideas
“It’s designed to help parents understand
what’s going on with their child,” Lenze
says. “It also trains parents in how to show
empathy for the child and the emotions
that child is displaying, as well as what to do
about those emotions.”
Should you have ideas or
suggestions you would like
brought before the Children’s
Medical Executive Committee
(CMEC), contact one of your
CMEC private physician
representatives:
The focus on emotions comes from the belief
that emotionally healthy people are able to
experience a broad range of emotions, both
positive and negative. Healthy people, Luby
says, can feel emotions at peak intensity but also
can regulate them. Depressed children often can’t
do that.
Peter Putnam, MD
314.965.5437
Catherine Remus, MD
314.842.5239
Robert Strashun, MD
314.991.1217
Kathie Wuellner, MD
618.474.1711
Let Us Hear From You
If you have comments or
suggestions regarding Doctor’s
Digest, or if you would like
to share information about
your activities as a physician,
contact:
Amy Connelly
Marketing and
Communications
St. Louis Children’s Hospital
600 South Taylor Ave.
Suite 202
St. Louis, MO 63110
Mailstop 90.94.210
“It’s healthy to be able to experience sadness or
guilt, but it’s not healthy to be overwrought with
guilt and preoccupied with sad things,” Luby
explains. “We work to help parents tolerate a
child’s emotion, as opposed to just trying to quash
it or to change the subject and distract the child,
which is a very typical parental technique.”
During therapy sessions, therapists like Lenze
observe parent-child interactions through a oneway mirror. They talk directly to the parents, who
wear an earpiece and a microphone, allowing the
therapists to coach them while they are interacting
with their child. This allows the therapist to deliver
SLCH News | Updated
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The Importance of
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has been updated
to include the 2011
recommendations.
Contact Children’s
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specific instructions about how to deal with the child’s
emotions in real time.
“That’s one of the interesting techniques that Eyberg
developed,” Luby says. “Because the therapist is
watching and coaching, the parent becomes the ‘arm of
the therapist’ during the treatment session, which puts
the parent and therapist on the same page. I think that
may be one of the reasons why the technique seems to
be so effective.”
Another reason may involve brain development.
“The brain is undergoing tremendous, rapid change
during this period of development,” Luby says.
“We think it is important to identify depression and
intervene early so that we might prevent it from
becoming a chronic and relapsing disorder.”
Lenze SN, Pautsch J, Luby J. Parent-child interaction
therapy emotion development: a novel treatment for
depression in preschool children, Depression and Anxiety,
Early View, published online Dec. 13, 2010. DOI:
10.1002/da.20770
This work was supported by grants from the National
Institute of Mental Health of the National Institutes of
Health.
Upcoming Conference to Focus on
Events Pediatric Emergency
Medicine and Trauma Care
St. Louis Children’s Hospital is hosting a two-day pediatric
emergency medicine and trauma care event in April. Current
Concepts in Pediatric Trauma Care, April 15, is an afternoon
educational program focusing on the management of traumatic injuries
in children. James Kessel, MD, medical director, trauma surgery, at
University of Missouri/ University Hospital, is the keynote speaker.
The 18th Annual Clinical Advances in Pediatric Emergency
Medicine, April 16, is a full-day event featuring topics related
to pediatric emergency medicine. Steve Krug, MD, professor of
pediatrics at Northwest University Feinburg School of Medicine
and Chief, Division of Emergency Medicine at Children’s Memorial
Hospital in Chicago, is the keynote speaker.
The two-day event will be held at the Eric P. Newman Education
Center on the Washington University Medical Center campus. Visit
StLouisChildrens.org/Med_Ed for more information or to register.
Continuing education credits are available.
Children’s Direct Line 800.678.4357
StLouisChildrens.org
SLCH News | Children’s Direct Reaches One-Year Anniversary of Expanded Services
After a full year of serving as the centralized point of
contact for patient transfers, direct admissions and
dispatching St. Louis Children’s Hospital’s transport
team, Children’s Direct staff members have learned some
important lessons: There is no such thing as too much
communication. A strong medical director and support
from the hospital’s Washington University School of
Medicine partners are critical to success. Acceptance of
change takes time.
“Throughout the past year we have listened to our
customers—both external and internal—and tried to
address their concerns as quickly as possible and make
changes that enhanced our processes,” says Julie Bruns,
director of the SLCH Answer Line/Children’s Direct.
“Overall we’ve received high marks, especially in regard to
our ability to quickly connect the referring and accepting
physicians and our ability to provide preadmission clinical
information to physicians and nurses on accepting units.”
Lessons learned from taking over transfers, direct admits and transport team dispatch are
resulting in overall satisfaction with processes, communication.
Doug Carlson, MD, Children’s Direct medical director,
believes that having a single point of contact and utilizing the
expertise of attending medical control physicians has helped make
patient transfers more efficient and safe for SLCH patients.
“In addition, our experienced pediatric nurses at Children’s
Direct —all with either critical care or emergency unit experience—
are the clinical equivalent to air traffic controllers with their quick
New Children’s Direct E-Mail Aids Communication
Physicians short on time now have another way to communicate with St. Louis
Children’s Hospital. Childrens_Direct@bjc.org is a central e-mail box to which
physicians may send requests for services and general comments expressing
concerns or kudos.
“I’ve long felt it was important to have a central communication vehicle for
community pediatricians to interact with the hospital,” says Alison Nash, MD,
St. Louis Children’s Hospital medical staff president. “This e-mail address can
serve as a single point of communication that will be monitored and acted upon
by hospital staff. And physicians who use this new e-mail system will receive
follow-up so they know their concerns are being addressed.”
She adds, “The community pediatricians on the Children’s Medical Executive
Committee endorse this approach and join me in encouraging our colleagues
to utilize it.”
E-mails sent to Childrens_Direct@bjc.org will either be routed to appropriate
sources for follow-up or will be handled by Children’s Direct staff members.
They also will be tracked to ensure completion, and the resolution will be
communicated back to the sending physician.
coordination of all the complex steps to get a patient here safely,”
he says. “Our first year has taught us a great deal about how we
can continue to streamline the process. We actively seek input from
our referring physicians so we can make transferring patients to us
even easier.”
In total during 2010, Children’s Direct handled 12,806 calls. Included
in that number were 3,558 transfers, a 4 percent increase over 2009;
2,896 referral calls, an 18 percent increase; and more than 3,500
phone consultation calls, an almost 400 percent increase.
“Part of these increases were due to our making Children’s Direct
available 24 hours a day, seven days a week,” says Bruns. “In response
to our higher call volume, we’ve adjusted our staffing to ensure we
have enough people available during our busiest times.”
For the future, Children’s Direct will continue to listen to its
customers with the goal of improving its services, and investigate
other ways in which it can assist referring physicians and SLCH
staff and departments.
Chief Resident Award
Carrie Coughlin, MD
Each month, St. Louis Children’s Hospital’s chief residents honor a resident who shows exceptional dedication to his or her patients,
colleagues or profession. In February, the SLCH Chief Resident Award was presented
to Carrie Coughlin, MD, first-year pediatric resident, in recognition of her consistent dedication to providing exceptional care
to her patients and their families.
|3
CDI News | New Research Targets Kids, Flu and ‘Gut’
Teams receive funding from the Children’s Discovery Institute
Congenital heart disease, lung infection and resistance
to antibiotics are just some of the serious health issues
affecting children. Now, 11 Washington University
research teams are preparing to ask – and answer – critical
questions about these and other pediatric health problems
with help from $3.8 million in new grants from the
Children’s Discovery Institute.
In one of the funded projects, David Rudnick, MD, PhD,
will study pathways that enable the liver to regenerate, with
the potential to identify a group of drugs that might enhance
care for a child with liver disease. Other grants will help
teams led by Barbara Warner, MD, and Gautam Dantas, PhD,
study the “microbiome” — bacteria in the digestive tracts
of normal babies that may play a key role in health, disease,
nutrition and even resistance to antibiotic drugs.
Other newly-funded Institute projects will explore congenital
abnormalities of the kidneys as well as the genetic causes of
heart disease and abnormal fetal growth.
“In seeking new answers to questions about pediatric disease,
we need to collaborate and think in bold new ways. That’s what
these latest grants represent,” said Mary Dinauer, MD, PhD,
Scientific Director of the Children’s Discovery Institute, the Fred
M. Saigh Distinguished Chair in Pediatric Research at St. Louis
Children’s Hospital and professor of pediatrics at Washington
University School of Medicine. “Thanks to our committed
and generous donors, researchers may be among the first to
discover some of the sources of childhood disease and chart a
path to more effective treatments.”
The Children’s Discovery Institute encourages unique,
productive collaborations among scientists at Washington
University School of Medicine, the university’s Danforth
Campus and St. Louis Children’s Hospital. Institute-funded
projects constitute “discovery research” — preliminary
studies that may point scientists down a path that, years in
the future, could yield new treatments.
To date, awards from the Institute have resulted in
significant progress in children’s health research. Awardees
have leveraged their initial “seed funding” to gain
additional funding resources from the National Institutes
of Health (NIH) and other national organizations.
The Children’s Discovery Institute is a multidisciplinary, innovation-based research partnership
between St. Louis Children’s Hospital and
Washington University School of Medicine
that has awarded nearly $23 million in
scientific grants since its launch in 2006.
4|
“The path from discovery to treatment is often long and winding,” says
Dr. Dinauer, “but we can’t even take the first step without the kind of
research funded by the Children’s Discovery Institute.”
Children’s Discovery Institute Awards
February 2011
• Deepta Bhattacharya, PhD, will look at potential ways to
improve flu vaccines for children.
• Sun Young Ahn, MD, will study genes that may cause babies
to develop congenital abnormalities of the kidneys.
•Kyunghee Choi, PhD, will lead a collaboration to study how
heart cells might be regenerated.
• Patrick Jay, MD, PhD, will investigate some of the genetic
factors that may lead to congenital heart disease.
• Audrey McAlinden, PhD, will look at the genetics that affect
the growth of the limbs in the embryo.
• David Rudnick, MD, PhD, will study pathways that enable the
liver to regenerate, with the potential to identify a group of
drugs that might enhance care of a child with liver disease.
• Jennifer Silva, MD, will create a registry of children who
receive cardiac pacemakers, to provide a databank to help
determine the most effective use of this treatment in children.
• Ryan Gray, PhD, will examine the genetics of familial scoliosis
(curvature of the spine), which affects 3 to 4 percent of all
children.
• Michael Shoykhet, MD, PhD, will receive a Faculty Scholar
award to help him set up a laboratory for the study of nervoussystem injury in critically ill children.
• Barbara Warner, MD, will expand her study of bacteria in the
digestive tracts of normal babies that may play a key role in
health, disease and metabolism.
• Gautam Dantas, PhD, will investigate the bacteria present
inside newborn babies to understand the diversity and
development of antibiotic resistant genes.
Children’s Direct Line 800.678.4357
StLouisChildrens.org
Surgical News | SLCH Joins ACS National Quality Improvement Program Focused
on Pediatric Surgery
St. Louis Children’s Hospital (SLCH) is one of only two pediatric
facilities in Missouri to join the pediatric version of the American
College of Surgeons National Surgical Quality Improvement Program
(ACS NSQIP Peds). The success of the well-established adult NSQIP
in providing participants with high-quality surgical outcomes data was
a significant factor in SLCH’s decision to enroll in the program.
“Children’s Hospital is committed to quality improvement in the
realm of our surgical care. Information from this program will help
us identify our best practices, develop quality improvement targets
and improve patient care and outcomes,” says Jacqueline Saito, MD,
SLCH pediatric surgeon and surgeon champion for NSQIP Peds at
the hospital. “Within BJC HealthCare, Barnes-Jewish Hospital and
Boone Hospital Center in Columbia, Mo., are participants in the
adult NSQIP. We know from their experience that the program offers
valuable insight.”
One of the major advantages of the NSQIP is that surgical outcomes
data is risk-adjusted to take into account patients’ severity of illness.
“Obviously, when you care for higher-risk patients as we do at
Children’s Hospital, there is greater potential for complications to
occur,” says Dr. Saito. “If the data collected from all of the participating
hospitals is not adjusted for that inherent risk, then what may look
like a problem for us could simply be the result of caring for children
who are sicker or who undergo more complex procedures.”
surgical practices and processes, as well as insight into how our risk
profiles and outcomes compare to peer medical centers and with
national averages,” says Mara Bollini, SLCH patient safety program
director. “In total, there are about 30 pediatric facilities participating,
including SLCH peers like Cincinnati Children’s Hospital, Children’s
Hospital Boston, Children’s Hospital of Philadelphia and Johns
Hopkins Hospital.”
As SLCH’s surgeon champion, Dr. Saito will help interpret the data
and develop ways to identify strengths and areas for improvement.
She also is a member of the NSQIP Peds’ national Data Definition
Committee, which is working to refine the program’s parameters.
“Complications after surgery that are tracked in the adult NSQIP such
as a heart attack or a blood clot going to the lung are rare in children.
But we are concerned about too much pain medication causing
breathing problems in patients and about wound infections,” says
Dr. Saito. “We are working to ensure NSQIP Peds focuses on pediatricspecific issues so that the information we receive is as relevant and
therefore as informative as possible.”
A requirement for enrolling in ACS NSQIP Peds is the addition to staff
of a dedicated surgical clinical reviewer to collect and submit data on
patients. Janet Adams, RHIA, fulfills that function for SLCH.
“The program requires us to collect approximately 120 data points
that are relevant to measuring surgical outcomes in children under
18 years of age and that cover preoperative, intraoperative and
postoperative information,” says Adams. “Over the course of eightday cycles—46 per year—data are inputted from the first 35 surgeries
to fit the NSQIP criteria, with follow-up information posted for 30
days following patients’ discharge. Selections are taken from general
surgery, vascular, thoracic, orthopedics, neurosurgery, urology,
otolaryngology, gynecology and plastics.”
SLCH joined ACS NSQIP Peds and began submitting data a year ago,
and the first outcomes reports are expected within about six months.
These will include:
• Semiannual reports comparing SLCH’s risk-adjusted surgical
outcomes to other participating centers on a blinded basis for
all operations combined and by subspecialty.
• Online access to daily center-specific reports as well as those
comparing SLCH’s metrics to national averages.
• Monthly data analysis for ad hoc specialized reports.
“These reports will give us valuable information regarding our actual
|5
Case Study | Newborn Seizures
The following case study was used by James P. Keating, MD, MSc,
medical director, St. Louis Children’s Hospital Diagnostic Center,
and his co-editor, Andrews J. White, MD, division director of
pediatric rheumatology/immunology, as part of the “Patient of
the Week” (POW) series. Many of the POW case studies cover
uncommon illnesses, or common illnesses with unusual symptoms
that can be overlooked. If you would like to be added to the POW
e-mail distribution list, send an e-mail to jkeating@wustl.edu or
white_a@wustl.edu.
Newborn medicine: Laura Al-Sayed/Alan Barnette (Cape Girardeau)
PL-2: Clayton Sontheimer
NICU: Barbara Warner and team
Neurology: Seth Perlman/Brad Schlaggar/Amy Viehoever
Au: Robyn Puente
A 22-day-old girl with CC: seizures
HPI: This full-term infant was noted to be stuporous and jittery
and startled easily on DOL#1. On DOL#2, she was loaded with
phenobarbital once for clinical suspicion of seizures. However, EEG
soon after showed no evidence of seizure activity. She had some
improvements until DOL#10, when she had marked worsening
of her neurologic status with excessive irritability and jitteriness.
She was reloaded with phenobarbital and started on maintenance
phenobarbital. On DOL#12, she was clinically in status with
severe metabolic acidosis and poor perfusion and was reloaded
with phenobarbital with improvement in her cardiovascular status.
At DOL#20, she had recurrent episodes of body tightening with
arms and legs pulled in tightly accompanied by loud grunts, eye
blinking, gagging and jerking movements. Abdominal distension
and increased irritability were also noted. Keppra was added with
temporary improvement. She continued to get multiple boluses of
Keppra, lorazepam and phenobarbital for persistent clinical seizures
activities. She was subsequently started on a pentobarbital drip and
intubated prior to transfer to SLCH on DOL#22.
PMHX: Pregnancy was complicated by preterm contractions at
35 weeks. Infant female was born at 37 6/7 week EGA via vaginal
delivery to 44 yo G7P5 now 6 with unremarkable serologies. Apgars
were 4, 6, 7 at 1, 5, 10 minutes. Delivery was spontaneous and with
vacuum extraction. Nuchal cord x1 was noted. She received bagmask ventilation for 30 seconds at birth. Spontaneous right-sided
pneumothorax at birth treated with needle decompression. Cultures
were negative. NBS normal. Karotype 46XX.
Rx: Pentobarbital drip at 2 mg/kg/hour (phenobarbital and
levetiracetam were on hold at the time of transfer).
FH: One sibling who died at 2 months of age reportedly
neurologically intact, but with Hyperbilirubinemia.
SH: Her parents are from the same area of Pakistan and are otherwise
not known to be related. They live in Paducah, KY.
PE: VS T36.9, HR 164, RR 23, BP 74/51. OFC: 36.7 cm.
General: Intubated and ventilated on SIMV. HEENT: anterior
fontanelle is open, soft and flat. Palate is intact. Heart: regular rate
6|
and rhythm. Lungs: clear bilaterally. Abdomen: benign. GU: normal
Tanner I female genitalia.
Neurologic exam: Did not open her eyes and did not have
a vigorous cry or evidence of grimace with stimulation but moved
when touched. Pupils were equal, round and reactive to light from
2 to 1.5 mm bilaterally. Positive red reflex bilaterally. Minimal squint
to light. Bit on fingertip but did not suck. Increased appendicular
tone with her bilateral thumbs tightly clinched inside fists when
she was stimulated. Decreased axial tone at all times and seemed
to have decreased appendicular tone when she was left alone for
quite some time and appeared to have relaxed. Moved all extremities
asynchronously and with at least antigravity strength when
stimulated but had little spontaneous movement without any form
of stimulation. When stimulated would tend to arch back with an
extensor posture. Did not startle to a loud clap or noise. Responded
to touch in all four extremities. Deep tendon reflexes were symmetric
and 3+ throughout in the biceps, brachial radialis, patellar and
Achilles tendons. Bilateral ankle and knee clonus for 3-4 beats,
and would occasionally have some bilateral elbow clonus for 1-2
beats with stimulation.
Clinical impression: 22-day-old female infant with intractable
seizures
Considerations:
1. CNS structural abnormalities
2. Perinatal hypoxic-ischemic encephalopathy
3. Metabolic disorders, including pyridoxine-dependent seizures
4. Familial seizure disorder
5. Neuroendocrinopathies
6. Hyperekplexia
7. Genetic syndromes
Course: While the patient and the EEG were recorded (Video
EEG), excessive discontinuity and epileptiform activity were
seen. Emergence of status epilepticus after discontinuation of
pentobarbital. Dramatic elimination of the clinical seizures and flattening
of the EEG when pyridoxine 100 mgm given intravenously consistent with
epilepsy pyridoxine dependent (EPD).
MRI: 1. Multiple T1 hyperintensities scattered throughout the deep
and periventricular white matter of the bilateral frontal, parietal and
occipital lobes, right greater than left, likely representing PVL-like
injuries. 2. Small subarachnoid hemorrhage involving a left inferior
temporal gyrus. 3. Small subdural hematoma in the posterior fossa.
4. Normal MRV.
Hospital course: Pyridoxine 50 mg twice daily was initiated and she
remained seizure free the remainder of her admission.
Special studies: CSF neurotransmitter studies obtained prior to
initiation of pyridoxine showed presence of pyridoxine-dependent
seizure biomarkers. DNA analysis showed two mutations in the
antiquitin gene (ALDH7A1) consistent with alpha-aminoadipic
semialdehyde dehydrogenase (AASA) deficiency. She was discharged
home on DOL#32 with a nearly normal neurological exam, on
pyridoxine po twice daily and phenobarbital. Phenobarbital was
discontinued at the Neurology follow-up visit 3 months later. Exam
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Faculty News | Two SLCH Physicians Receive Distinguished Faculty Awards
Two members of the St. Louis Children’s Hospital medical
staff were among eleven faculty members fromWashington
University School of Medicine honored February 9 at the
2011 Distinguished Faculty Awards ceremony at the Eric
P. Newman Education Center.
Tae Sung Park, MD, the Shi Hui Huang Professor of
Neurological Surgery and professor of neurobiology and
of pediatrics, and Andrew J. White, MD, assistant professor
of pediatrics, received Distinguished Clinician Awards.
The awards were created to recognize outstanding
achievements in clinical care, community service, research
and teaching and are co-sponsored by the dean’s office,
the Office of Faculty Affairs Central Administration and
the Executive Committee of the Faculty Council.
“These dedicated and talented individuals have made
significant and lasting contributions to the School of
Medicine,” says Larry J. Shapiro, MD, executive vice
chancellor for medical affairs and dean of the School
of Medicine. “Our awardees’ efforts have touched the
personal and professional lives of countless patients,
families, colleagues and trainees. In the process, they
have enriched our academic community and beyond in
immeasurable ways.”
From left: Karen O’Malley PhD, professor of neurobiology; Larry J. Shapiro, MD, executive vice
chancellor for medical affairs and dean of the School of Medicine; T. S. Park, MD (above)/
Andrew White, MD (below) and Diana Gray, MD, associate dean for faculty affairs and
professor of obstetrics and gynecology.
Consultant members of the St. Louis Children’s Hospital
medical staff were also recognized. Jeffrey M. Michaelski,
MD, vice chairman and professor of radiation oncology,
received the Distinguished Clinician Award and Philip
E. Cryer, MD, the Irene E. and Michael M. Karl Professor
of Endocrinology and Matabolism in Medicine, received
the Daniel P. Schuster Award for Distinguished Work in
Clinical and Translational Science.
continued from previous page
at that time was remarkable for only axial hypotonia. Development
was age appropriate.
Discussion: Epilepsy, pyridoxine-dependent (EPD; PDS) is an
autosomal recessive disorder with an estimated birth incidence
between 1:400,000 and 1:750,000. There are 100 reported cases
worldwide. Patients with PDS typically present with seizures activities
within hours after birth that are refractory to typical anti-epileptics.
Affected infants may also exhibit intrauterine seizures. Seizure types
vary, including myoclonic, atonic, partial, generalized and infantile
spasms. PDS also can be associated with hyper-alertness, irritability,
tremulousness, abnormal cry, excessive startle response, abdominal
distension, vomiting and respiratory distress. Clinical diagnosis of
PDS can be made with rapid resolution of seizure activity with
administration of pyridoxine. The pathophysiology of PDS is not
fully understood. Pyridoxine is converted via a series of pathways
to its active form, pyridoxal phosphate (LPD)—an essential cofactor
for many reactions, including those involved in neurotransmitter
metabolism. Mutations in the antiquitin gene cause concomitant
depletion of LPD, which may contribute to neurotransmitter
derangements causing seizure activity. Brain imagining of patients
with PDS may show hypoplasia of the corpus callosum and
cerebellum with mega cisterna magna. Untreated seizures may also
be associated with intraventricular hemorrhage and/or subarachnoid
hemorrhage, white matter changes and hydrocephalus. The
developmental outcome of patients with pyridoxine varies, with
most patients experiencing some degree of cognitive impairment,
particularly expressive language and learning difficulties. Patients
with a history of intrauterine seizures carry the worst outcome.
Women who previous gave birth to a child with PDS are
recommended to receive pyridoxine supplementation in the final
half of subsequent pregnancies.
References for this article can be found at StLouisChildrens.org/DD
|7
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In this issue
1
4
7
Parent-Child Play Therapy Relieves Depression
New Research Target Kids, Flu and ‘Gut’
Two SLCH Physicians Receive Distinguished Awards
Faculty Update | Cole Receives Goldstein Leadership Award
F. Sessions Cole, MD, has been awarded a 2010 Samuel R. Goldstein
Leadership Award in Medical Education. Mark D. Levine, MD, and
Megan E. Wren, MD, also received the award.
The annual awards, which recognize outstanding teaching and
commitment to medical education, are among the highest honors that
School of Medicine faculty can achieve. They were established in 2000
in memory of Mr. Goldstein, a longtime friend of the medical school.
A selection committee made up of faculty and a student representative
from each class reviews all submitted nominations and selects three
awardees based on innovative teaching, curriculum development,
commitment to education and teaching evaluations. The committee
forwards its recommendations to Larry J. Shapiro, MD, executive vice
chancellor for medical affairs and dean of the School of Medicine, for
final approval.
“Sesh, Mark and Megan each exhibit excellence in and commitment
to leadership in medical student education,” Shapiro says. “The School
of Medicine and its students are extremely fortunate to have such
talented and distinguished leaders of education at our institution.”
The awards will be formally presented at a dinner this spring.
Dr. Cole is the Park J. White, MD, Professor and vice chairman of
Pediatrics. He is also assistant vice chancellor for children’s health,
director of the Division of Newborn Medicine and chief medical officer
of St. Louis Children’s Hospital.
Dr. Cole is well regarded for his
commitment to excellence in education
and outreach activities. Since his arrival
in 1986, more than 2,500 medical
students and more than 500 pediatric
residents have directly benefited from
his clinical scholarship and teaching.
He was instrumental in the formation
of community outreach programs for
medical students, such as Students
Teaching AIDS to Students (STATS),
where medical students teach AIDS
awareness to middle school students, and the Perinatal Program,
where medical students provide teenage mothers and their babies with
support and information. He also helped design an elective called
Special Topics in Reproductive Health in response to students’ requests
for a course focusing on adolescents, victims of child abuse and
teenage pregnancy, and he allowed students to control the content
and organization of the course.
Dr. Cole has received many awards and honors, including the
Clinical Teaching Award from Washington University School of
Medicine’s Classes of 1999 and 2000; Cartier First Aide Award from
the St. Louis Effort for AIDS; Torch of Youth Award from the National
Council on Youth Leadership; and FDR Leadership Award from the
March of Dimes.