Decreased levels of "good" cholesterol in children with Progeria may cause premature heart disease

Main Category: Pediatrics / Children's Health
Also Included In: Cholesterol
Article Date: 09 Mar 2005 - 10:00 PST


Adiponectin may be helpful in finding treatments -

In a study published in the March issue of The Journal of Pediatrics, researchers found that decreased levels of HDL cholesterol, or "good" cholesterol, may contribute to premature heart disease in children with Hutchinson-Gilford Progeria Syndrome, or Progeria. Additionally, adiponectin, a hormone that regulates the metabolism of fat and sugar, may be linked to the disease process and prove helpful in finding treatments.

Progeria is a rare, fatal genetic condition characterized by the appearance of accelerated aging in children, and affects about one in 4-8 million newborns. Although they are born looking healthy, children with Progeria begin to display characteristics of accelerated aging at around 18-24 months of age. Indicators of the disease include growth failure, loss of body fat and hair, aged-looking skin, stiffness of joints, hip dislocation, and atherosclerosis.

"All children with Progeria die between the ages of 6 and 20 years from heart failure or stroke," said Leslie Gordon, MD, PhD, lead author of the study, Medical Director of the Progeria Research Foundation (PRF), and assistant professor, Tufts University School of Medicine.

"Studying heart disease as it relates to children with Progeria can help us better understand how atherosclerosis will affect the aging population while also helping these precious children."

In the study published in The Journal of Pediatrics, a team of researchers compared cholesterol levels in children with Progeria to children who do not have the disease. The scientists discovered that, compared with children who do not have Progeria, children with Progeria in their mid- and later years have decreased levels of HDL cholesterol-or "good" cholesterol-and adiponectin, a hormone that regulates the metabolism of fat and sugar. Both factors work to remove fat from plaques in arteries, and the lower levels may contribute to accelerated plaque formation. However, LDL-or "bad" cholesterol-and triglyceride levels, which are elevated in adults with heart disease, are usually at normal levels in children with Progeria.

Dr. Gordon-along with family and friends-founded the Progeria Research Foundation in 1999 as the only organization solely dedicated to discovering the cause, treatment and cure for Progeria after her son, now eight-years-old, was diagnosed with the disease. In April 2003, PRF succeeded in the first part of its mission when the Foundation helped to discover the gene that causes Progeria. For more information about Progeria or PRF, visit ProgeriaResearch.org.

The study is reported in "Reduced Adiponectin and HDL Cholesterol Without Elevated C-reactive Protein: Clues to the Biology of Premature Atherosclerosis in Hutchinson-Gilford Progeria Syndrome" by Leslie B. Gordon, MD, PhD, Ingrid A. Harten, MS, Mary Elizabeth Patti, MD, and Alice H. Lichtenstein, PhD. The article appears in The Journal of Pediatrics, Volume 146, Number 3 (March 2005), published by Elsevier.

The Journal of Pediatrics is a primary reference for the science and practice of pediatrics and its subspecialties. This authoritative resource of original, peer-reviewed articles oriented toward clinical practice helps physicians stay abreast of the latest and ever-changing developments in pediatric medicine. The Journal of Pediatrics ranks in the top 12% of the 5,907 scientific journals receiving the most citations (Science Citation Index). The Journal is published under the Mosby imprint by Elsevier, a leading global publisher of scientific, technical, and medical journals, books, and reference works. It is a member of the Reed Elsevier plc group.

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Partial Table of Contents - Volume 146/Number 3 (March 2005)

EDITORIALS

INHALED NITRIC OXIDE FOR PRETERM INFANTS: A THERAPY IN SEARCH OF AN INDICATION? THE SEARCH CONTINUES
Neil N. Finer, MD

GROWTH FAILURE IN CYSTIC FIBROSIS: A TRUE NEED FOR ANABOLIC AGENTS?
Carla Colombo, MD, and Alberto Battezzati, MD

CHRONIC CHOLESTASIS AND DYSLIPIDEMIA: WHAT IS THE CARDIOVASCULAR RISK?
Dennis D. Black, MD

MODEST LIFESTYLE INTERVENTION ATTENUATES THE INFLAMMATORY STATE IN CHILDREN
Julia Steinberger, MD, MS

POST-TRAUMATIC STRESS AND ITS EFFECT ON HEALTH OUTCOMES IN CHILDREN
Jacqueline Grupp-Phelan, MD, MPH, and Doug Zatzick, MD

MEDICAL PROGRESS

IS THERE CLINICAL EVIDENCE SUPPORTING THE USE OF BOTANICAL DIETARY SUPPLEMENTS IN CHILDREN?
Andrea Hrastinger, PhD, et al.

ORIGINAL ARTICLES

THE SAFETY AND EFFICACY OF NITRIC OXIDE THERAPY IN PREMATURE INFANTS
J. M. Hascoet, MD, et al.

GROWTH HORMONE TREATMENT ENHANCES NUTRITION AND GROWTH IN CHILDREN WITH CYSTIC FIBROSIS RECEIVING ENTERAL NUTRITION
Dana S. Hardin, MD, et al.

THE JOURNAL OF EVALUATION OF RISK FOR ATHEROSCLEROSIS IN ALAGILLE SYNDROME AND PROGRESSIVE FAMILIAL INTRAHEPATIC CHOLESTASIS: TWO CONGENITAL CHOLESTATIC DISEASES WITH DIFFERENT LIPOPROTEIN METABOLISMS
Hironori Nagasaka, MD, PhD, et al.

REDUCED ADIPONECTIN AND HDL CHOLESTEROL WITHOUT ELEVATED C-REACTIVE PROTEIN: CLUES TO THE BIOLOGY OF PREMATURE ATHEROSCLEROSIS IN HUTCHINSON-GILFORD PROGERIA SYNDROME
Leslie B. Gordon, MD, PhD, et al.

LIFESTYLE-ONLY INTERVENTION ATTENUATES THE INFLAMMATORY STATE ASSOCIATED WITH OBESITY: A RANDOMIZED CONTROLLED STUDY IN ADOLESCENTS
Prabhakaran Balagopal, PhD, et al.

VIOLENCE EXPOSURE AND TRAUMATIC STRESS SYMPTOMS AS ADDITIONAL PREDICTORS OF HEALTH PROBLEMS IN HIGH-RISK CHILDREN
Sandra A. Graham-Bermann, PhD, and Julia Seng, PhD, CNM

SYNCOPE IN CHILDREN: DIAGNOSTIC TESTS HAVE A HIGH COST AND LOWYIELD
Leonard A. Steinberg, MD, and Timothy K. Knilans, MD

PREVALENCE, SYMPTOMS AND OUTCOME OF CONSTIPATION IN INFANTS AND TODDLERS
Vera Loening-Baucke, MD

INEFFECTIVENESS OF LACTOBACILLUS GG AS AN ADJUNCT TO LACTULOSE FOR THE TREATMENT OF CONSTIPATION IN CHILDREN: A DOUBLE-BLIND, PLACEBO-CONTROLLED RANDOMIZED TRIAL
Aleksandra Banaszkiewicz, MD, and Hania Szajewska, MD

A COMMON MUTATION IN THE SURFACTANT PROTEIN C GENE ASSOCIATED WITH LUNG DISEASE
H. Scott Cameron, MD, et al.

PULMONARY CAPILLARITIS IN CHILDREN: A REVIEW OF EIGHT CASES WITH COMPARISON TO OTHER ALVEOLAR HEMORRHAGE SYNDROMES
Jason J. Fullmer, MD, et al.

GENETIC ANALYSES IN TWO EXTENDED FAMILIES WITH DELETION 22Q11 SYNDROME: IMPORTANCE OF EXTRACARDIAC MANIFESTATIONS
Kerry A. Shooner, MS, et al.

CENTRAL NERVOUS SYSTEM HYPEREXCITABILITY ASSOCIATED WITH GLUTAMATE DEHYDROGENASE GAIN OF FUNCTION MUTATIONS
David M. Raizen, MD, PhD, et al.

RADIOGRAPHIC VERTEBRAL MORPHOLOGY: A DIAGNOSTIC TOOL IN PEDIATRIC OSTEOPOROSIS
Outi M�kitie, MD, PhD, et al.

INCIDENCE AND PREVALENCE OF HIV ENCEPHALOPATHY IN CHILDREN WITH HIV INFECTION RECEIVING HIGHLY ACTIVE ANTI-RETROVIRAL THERAPY (HAART)
Claudia A. Chiriboga, MD, et al.

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For more information, contact: The Journal of Pediatrics Editorial Office, 513-636-7140,
journal.pediatrics@cchmc.org
or Brooke Saltzer for the Progeria Research Foundation, 202-955-6222, ext. 2520,
bsaltzer@spectrumscience.com

Cincinnati Children's Hospital Medical Center

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