Editorial
Team
Management of Congenital Craniofacial Anomalies
S. Anthony Wolfe, MD
Miami Children's Hospital
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Review Articles
Treatment
of Congenital Craniofacial Anomalies: Team Management
with Concern for the Whole Child
Samuel Berkowitz, DDS, MS, FICD
Abstract
With the institution of a team
approach to the care of children with craniofacial anomalies, the level of care improved
as each of the specialists, having gained a better understanding of the problems involved,
became more prepared to pool their efforts to solve rehabilitative problems that lend
themselves to a multidisciplinary approach. This article stresses that team members are
not only to be well-trained in their specialty, but must be experienced. It demonstrates
that records consisting of serial lateral cephalometric radiographs, dental casts,
photographs, and speech and audiologic reports must be taken for quality assurance.
Surgical advances using distraction osteogenesis are briefly discussed and its use is
demonstrated. A family centered approach to treatment, team qualifications and
responsibilities, and an operational guide for meeting their goals are reviewed in detail.
Int Pediatr. 1999;14(2):77-82
Key words: multidisciplinary
team care, credentials, experience, surgery, orthodontics, distraction osteogenesis,
longitudinal records
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Acute
Lymphocytic Leukemia in Childhood
Enrique A. Escalon, MD
Abstract
Acute leukemias constitute 97% of all childhood
leukemias. It accounts for 25% to 30% of pediatric cancer. Seventy-five percent of acute
leukemias are of the lymphocytic type. The etiology is unknown. A variety of clinical and
prognostic features are detected at presentation. With modern modalities of treatment,
approximately 70% of the patients become long-term survivors. Treatment of relapse is
improving. Late sequelae are detected in some patients. Int Pediatr. 1999;14(2):83-89.
Key words: acute lymphocytic leukemia,
childhood
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Munchausen
Syndrome by Proxy: The Syndrome of
Fabricated Illness in the Pediatric Patient
Julio Apolo, MD
Abstract
Munchausen syndrome by proxy is a serious
form of child abuse that is being identified with increasing frequency. The fabricated
illness, simulated or induced by a parent (usually the mother), results in numerous
unnecessary medical evaluations and treatments. Failure to recognize this entity leads to
significant morbidity and mortality. This review provides guidelines for the recognition
and reporting of the Munchausen syndrome by proxy. Int Pediatr. 1999;14(2):90-93.
Key words: Munchausen syndrome by proxy
(MSBP), child abuse, fabricated illness, Polle syndrome
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Vesicoureteral
Reflux in Children
Felix Ramirez-Seijas, MD; Roberto
Gugig, MD
Anselmo Cepero-Akselrad, MD; Ana Paredes, MD
Abstract
Vesicoureteral reflux is a common abnormality in
children, and can lead to the development of renal scarring with subsequent hypertension
and chronic renal failure. Most of this morbidity can be prevented with early diagnosis
and aggressive therapy. Children with VUR are usually diagnosed following a UTI, although
we have observed an increasing number diagnosed at birth following a prenatal sonogram
revealing pelvo-caleactasis or hydronephrosis. Most patients are treated medically without
the need for surgical correction since spontaneous resolution usually occurs. Long-term
prognosis is excellent. Int Pediatr. 1999;14(2):94-98.
Key words: vesicoureteral reflux, children,
urinary tract infection, ureter
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Aviation
Physiology and Interhospital Transport of Infants and Children
Andre Raszynski, MD
Abstract
Pediatric and neonatal patients are frequently
transported by air to tertiary care institutions with special expertise in pediatric and
neonatal intensive care. However, aeromedical transport has potentially deleterious
effects on the physiology of critically ill patients, primarily as a consequence of
changes in cabin barometric pressure. A fall in barometric pressure can cause expansion of
gases trapped in medical devices or patient cavities, decompression sickness, and hypoxia.
These complications can also occur when patients are transferred by commercial airlines as
their airplanes are pressurized to fly at cabin altitudes of 5000 to 8000 feet. Therefore,
medical professionals who are involved in the air transports of neonatal and pediatric
patients should be familiar with the stresses imposed by the aircraft environment on the
patient, the crew, and the equipment. Int Pediatr. 1999;14(2):99-105.
Key words: aviation physiology, interhospital
transport
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Clinical Articles
A New Infant Respiratory Chamber: A Tool for
Improving Infant Nutrition and Growth
Russell Rising, PhD; Conrad R. Cole,
MD; Debora Duro, MD; Fima Lifshitz, MD
Abstract
Equations for calculating energy requirements are
not appropriate for hospitalized infants. Therefore, a new infant respiratory chamber has
been validated for accurate assessments of the components of energy expenditure while
allowing unrestricted access. Clinically, this instrument has determined and revised
caloric requirements in one infant with intrauterine growth retardation. Older infants
with elevated thyroid stimulating hormone and normal T4 levels do not have a low basal
metabolic rate while infants seropositive for HIV have higher basal metabolic rates.
Current studies are determining the metabolic changes associated with carbohydrate
malabsorption due to fruit juice consumption. In a recent study we reported that infants
consuming fruit juices containing sorbitol, such as pear juice, have increased physical
activity, metabolic rates, and carbohydrate malabsorption as compared with ingestion of
white grape juice containing no sorbitol. Presently, we are determining if infants with a
history of colic are more sensitive to fruit juices that contain sorbitol and excess
amounts of fructose. In the future this instrument may be used to determine if infants are
prone to childhood obesity. Int Pediatr. 1999;14(2):106-110.
Key words: energy expenditure, energy
requirements, infants, respiratory chamber
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Canavan
Disease in a Patient of African Origin
Annette Perea, MD; Keith Meyer, MD;
Julio Cesar Paez, MD; Karina Yelin, MD
Sherri B. Birchansky, MD; Parul Jayakar, MD; Israel Alfonso, MD
Abstract
This patient is the second case of Canavan
disease in a patient of African origin in 226 reported cases. A 7-month-old patient of
African origin presented with neurological signs suggestive of leukodystrophy and
macrocephaly. A magnetic resonance imaging (MRI) study at 4 weeks of age revealed delayed
myelinization pattern. A second MRI study done at 6 months of age revealed significant U
fibers involvement and swollen gyri in addition to a delayed myelinization pattern. The
diagnosis of Canavan syndrome was confirmed by demonstrating a marked elevation of
N-acetylaspartic acid in urine. The purpose of this report is to recommend that Canavan
syndrome be considered in the differential diagnosis of leukodystrophy in patients of
African origin. Int Pediatr. 1999;14(2):111-113.
Key words: Canavan disease, leukodystrophy,
African origin, delayed myelinization pattern
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Osteogenic
Protein-1 is Downregulated in Bone Cells From a Patient with
Chronic Renal Failure and Severe Renal Osteodystrophy
Ana Paredes, MD; Philip Dube, DMD;
Stephen Stricker, MD; Anselmo Cepero-Akselrad, MD; Felix Ramirez-Seijas, MD
Abstract
Osteogenic protein-1 (OP-1) is a growth factor
known to induce proliferation and differentiation of normal human bone cells. Since kidney
OP-1 gene expression is decreased in renal failure and downregulation of OP-1 is a
proposed factor in the etiology of renal osteodystrophy, we sought to determine the
expression of OP-1 and other genes involved in osteoblastic differentiation in bone cells
from a chronic renal failure patient. We measured changes in messenger ribonucleic acid
(mRNA) levels for osteopontin, alkaline phosphatase, OP-1, and bone sialoprotein in bone
cells isolated from a bone biopsy performed for diagnostic purposes in a chronic dialysis
patient. Vertebral bodies from a cadaveric organ donor were used as control. Bone cells
were grown for 30 days in media ±rhOP-1. The baseline expression of OP-1 in the patient
was significantly decreased compared to that observed in control cells. The intensity of
the OP-1 mRNA signal increased in the cells treated with rhOP-1 but never reached the
level observed in the control treated cells. Lack of OP-1 in the patient’s
osteoblasts may be a factor in the inappropriate differentiation capacity observed in
these cells. The partial response in OP-1 gene expression observed after in vitro
treatment may lead to improved approaches to the prevention and treatment of renal
osteodystrophy. Int Pediatr. 1999;14(2):114-117.
Key words: Osteogenic protein-1 (OP-1),
growth factor, chronic renal failure
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Pathophysiology
and Computed Tomography Findings in a Case of Diabetic Ketoacidosis
Esperanza Pacheco, MD and Nolan
Altman, MD
Abstract
The child with diabetic ketoacidosis is at risk
for the development of cerebral edema. Any child who presents with altered mental status
and diabetes should undergo computed tomography (CT) of the brain with careful follow-up
to aid in the appropriate management. Int Pediatr. 1999;14(2):118-120.
Key words: computed tomography (CT), diabetic
ketoacidosis, cerebral edema
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Hepatic
Angiosarcoma in Children: Review and Presentation of One Case
Erick Hernandez, MD; Enrique A.
Escalon, MD; Athena C. Pefkarou, MD
John Fort, MD; Ziad Khatib, MD; Carole D. Brathwaite, MD
Abstract
We report a case of a child who presented with
abdominal pain and symptoms of an acute abdomen. A large abdominal mass was palpated and
was found to be of hepatic origin. The liver biopsy was compatible with an angiosarcoma.
The patient received intensive chemotherapy and radiotherapy with only partial control of
the disease. Successful surgical excision of the residual tumor was done, but the patient
eventually died of postoperative complications. Int Pediatr. 1999;14(2):121-124.
Key words: hepatic angiosarcoma, vascular
tumor, children, liver
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