The new england journal of medicine

1752 n engl j med 353;16 www.nejm.org october 20, 2005

ently ill; nor should it be assumed that the mental
disorders of such people are due simply to intoler-
ance. Moreover, gender variance may, at times, re-
sult from primary psychopathologic factors.
The discussion of the political and social di-
mensions of the lives of patients with intersex dis-
orders is interesting. Consideration of major con-
tributors to the field would have added weight to
the authors speculations about the scientific and
clinical issues raised by such patients.
The Riddle of Gender is a useful introduction to po-
litical, social, and psychological issues in the field.
Once readers become acquainted with the terrain,
those who seek a more substantial knowledge of
scientific and clinical questions about the field now
known as gender psychology may choose to read
the scholarly literature in their areas of interest.
Richard C. Friedman, M.D.
WeillCornell Medical School
New York, NY 10021
abnormalities in puberty:
scientific and clinical advances
(Endocrine Development. Vol. 8.) Edited by Henriette A.
Delemarre-van de Waal. 181 pp., illustrated. Basel, Switzerland,
Karger, 2005. $149.25. ISBN 3-8055-7867-9.
his volume in the endocrine devel-
opment series is a collection of state-of-the-
art reviews, plus two chapters presenting original
data. All chapters but one originate from Europe-
an centers, and all authors are well respected in
their fields.
There is a perception in the United States, not
accepted by all, that puberty now starts at an earlier
age than it did a few decades ago. In the introducto-
ry chapter, Delemarre-van de Waal reports that the
age of puberty is stable in Europe, with some areas
actually reporting a later age of onset than has been
reported in the past. She also reviews genetic and
nutritional influences on the process. Karges and
de Roux pursue the genetic theme with isolated hy-
pogonadism, bringing the reader up to date on all
the reported mutations of the hypothalamicpitu-
itary axis.
With glucocorticoids being given to mothers of
children who may be delivered prematurely and to
mothers of children with virilizing congenital adre-
nal hyperplasia, and with cortisol levels increasing
in neonates under stress, a question arises as to
whether such factors affect the age of onset of pu-
berty. Ong discusses the present understanding of
the effects of fetal and neonatal glucocorticoid phys-
iology on puberty, using information derived from
studies of children and animals. Delemarre-van
de Waal and her colleagues present data from their
longitudinal observations of children who were
small for gestational age at birth, as well as results
from studies of puberty in rodents with fetal under-
nutrition. Polycystic ovary syndrome is one of the
constellation of disorders that occur in small-for-
gestational-age children, and Homburg reviews the
diagnosis, pathophysiology, and treatment of poly-
cystic ovary syndrome, emphasizing the need for a
high index of suspicion in cases of persistent oligo-
menorrhea or other manifestations of excessive an-
drogen production in teenage girls.
Jung and colleagues present a thorough review
of what is known and what is postulated concern-
ing the relation between hypothalamic hamarto-
mas and precocious puberty. Since medical thera-
py is recommended for these masses in sensitive
locations of the central nervous system, the chapter
by Heger and colleagues on 20-year outcomes of
treatment with gonadotropin-releasing hormone
agonists is a welcome addition and a demonstra-
tion of the many beneficial effects of treatment in
appropriately selected subjects (their list of indica-
tions for treatment is also practical). Johansson and
Ritzn present several decades of follow-up data on
the psychosocial effects of early menarche (that oc-
curring before 11 years), which suggest more norm-
breaking behavior during adolescence and lower
educational levels later, all apparently related to ini-
tiation of sexual activity at a young age as a result
of early development.
Bone health is important for all young people,
especially in this age of inadequate calcium and vi-
tamin D intake by children. Vanderschueren and
colleagues review the latest knowledge about bone
density in people with various forms of gonadal fail-
ure and the effects of therapy, and they discuss de-
layed puberty in the context of calcium and vita-
min D intake. It has now been well over 50 years
since glucocorticoid therapy for congenital adre-
nal hyperplasia was first used; Otten and colleagues
review the results of this treatment on growth, pu-
berty, and subsequent fertility in children with this
disorder. Remarkable successes in cancer therapy
allow us to turn attention to previously unthinkable
issues, such as fertility in survivors of childhood can-
cer. Beerendonk and Braat review the effects of ra-
diation and chemotherapy on future fertility and
many aspects of the preservation of fertility, rang-
t
The New England Journal of Medicine
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n engl j med 353;16 www.nejm.org october 20, 2005
notices
1753
ing from ovarian transplantation to cryopreserva-
tion of ovarian tissue or ova.
Although there are variations, the overarching
themes of the book are the long-term effects of fe-
tal life on puberty and the long-term effects of pu-
berty on later life. Although a book of this size can-
not fully cover all aspects of pubertal development,
the chosen aspects are of importance to clinicians,
and the often encyclopedic references will be of use
to researchers in the field as well.
Dennis M. Styne, M.D.
University of California, Davis, Medical Center
Sacramento, CA 95817
dmstyne@ucdavis.edu
pediatric heart failure
(Fundamental and Clinical Cardiology. Vol. 53.) Edited by Robert
E. Shaddy and Gil Wernovsky. 897 pp., illustrated. Boca Raton,
Fla., Taylor & Francis, 2005. $199.95. ISBN 0-8247-5929-X.
he timing of this textbooks arrival
is perfect. The field of pediatric cardiology
has evolved from a focus on early survival toward
an emphasis on the long-term health of children
with heart disease. The editors, who are respected
leaders in this field, have provided a state-of-the-
art reference the first of its kind on the sub-
ject of pediatric heart failure.
The introductory chapter is a wonderful histor-
ical perspective by Abraham Rudolph. He reminds
us of the difficulty in precisely defining pediatric
heart failure. He quotes Alexander Nadas, who in
his 1957 textbook stated that it might well be to re-
gard congestive failure simply as a clinical syndrome
associated with heart disease. Rudolph goes on to
highlight the important early advances that have
been made in the understanding of heart failure.
From these early advances, the readers atten-
tion is turned toward the developmental differenc-
es between the fetal and neonatal cardiovascular
systems. Important concepts are reviewed: the mo-
lecular basis for muscle contraction, the role of the
neurohormonal system and cytokines in heart fail-
ure, and the genetic causes of some cardiomyopa-
thies. In a somewhat uneven manner, the book re-
turns to these concepts in the later, more clinically
oriented chapters.
The reader will find the clinically oriented chap-
ters well organized and extensively referenced. The
authors give a complete picture of the causes, patho-
physiology, and treatment of heart failure in fetuses,
neonates, children with congenital and acquired
heart disease, and adults with congenital heart dis-
ease. Entire chapters deal with arrhythmia, right-
sided heart failure, and coronary-artery abnormali-
ties. As a testimony to the comprehensiveness of
this textbook, two chapters are worth noting
one that deals with the nutritional aspects of pedi-
atric heart failure and another that deals with the
psychological aspects.
This book bridges the gap between basic-sci-
ence concepts and clinical care recommendations.
A minor complaint is that the complex figures of
subcellular interactions would have benefited from
color presentation. Because the chapters are dis-
ease-oriented, the coverage of diagnostic testing
and decision making regarding treatment is vari-
able. There is little material on newer diagnostic
techniques, such as Doppler tissue imaging, that are
used in the assessment of pediatric heart function.
The coverage of post-transplantation heart failure
is excellent, but there is less discussion about when
pediatric heart transplantation should be recom-
mended.
The strengths of this textbook overwhelm any
shortcomings of a work in its first edition. It will
instantly become the standard reference on the sub-
ject of pediatric heart failure. It belongs in the library
of all training programs and is recommended read-
ing for all those involved in the care of children with
heart disease.
Wyman W. Lai, M.D., M.P.H.
Mount Sinai School of Medicine
New York, NY 10029
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occupational safety and health education
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The following courses will be offered in Chapel Hill, N.C.:
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The New England Journal of Medicine
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