Psychosocial short
stature (PSS) has been classified by the authors into 3 categories: (1)
Type IIA are hyperphagic children, in whom there is reversible growth
hormone (GH) insufficiency with rapid catch-up growth with a change in
their living environment but with minimal response to exogenous GH;
(2)Type IIB is a heterogeneous sub group of non-hyperphagic children who
have normal GH secretory dynamics and minimal or absent increase in
growth rate with change in their environment and variable response to
GH; and (3) Type III are children with anorexic eating habits, with an
onset as early as infancy, with failure to thrive, depression, normal GH
secretory dynamics, and significant growth response to exogenous GH.
Gohike et al report anthropometric evaluations of 46 children with PSS,
before and after change in their environment (Table 1).
Significant
improvement in height velocity SDS after intervention was observed in
all groups. ANOVA failed to show any significant differences in growth
velocity between groups. There was no significant change with treatment
in body proportion in type IIA (hyperphagic) or in type IIB
(heterogenous) children. In type III (anorexic) children, the body
proportions decreased significantly after intervention indicating
relatively shorter upper segments after treatment. In those who
received GH treatment (n = 21), there was no significant change in body
proportion after GH therapy. Body Mass Index (BMI) did not increase in
any of the groups after intervention and there were no significant
changes in bone age. Multiple regression analysis showed that the type
of PSS was a predictor for height velocity after intervention. The
greatest effect in removal from adverse home events were in the type IIA
(hyperphagic) subjects. The authors state that their findings should be
helpful to clinicians managing children with PSS because of the emphasis
on appetite disturbance and the variable treatment responses.
Gohlke BC, et al. Eur J Pediatr 2002;161:250-254.
First Editor’s
Comment:
PSS, first described in 1947 by Talbot et al,1 is often
difficult to diagnose. Variable GH secretory dynamics, and responses to
exogenous GH therapy make it important to attempt to better understand
the etiologies involved and their potential response to psychosocial
changes. The current manuscript report data on a large number of
subjects with PSS and suggested that BMI is not useful in predicting
response to treatment, but that categorization based on appetite may be
of use in predicting growth changes. It is unfortunate that their data
were not analyzed separately for those with intrauterine growth
retardation (IUGR) and for those with and without GH insufficiency.
However, the heterogeneous composition and variable treatment of these
children strengthen the conclusions based on categorization of subjects
by their eating behavior.
Reference
1.
Talbot
NB, et al. N Engl J Med 1947;236:783-789.
William L. Clarke,
MD
Second Editor’s
Comment:
PSS should be considered by pediatric endocrinologists or pediatricians
in the differential diagnosis of short stature when a short child is
seen in the clinic. If the possibility of this diagnosis is not
considered and explored in the history, the diagnosis will be missed.
PSS occurs much more frequently than is realized. Many parents of
children with PSS (particularly Type II A of the English classification)
are not concerned about their child’s stature because the parents are
psychologically rejecting the child.
PSS is a spectrum of
entities as Gohlke, Frazer, and Stanhope state. The classification is
muddy for this reason. In the patients reported by Gohlke et al there
was no child less than 3.8 years of age. In the classification listed
in Lifshitz’s Pediatric Endocrine Text1 (3rd
Edition, 1996), infants with PSS comprise a broad clinical spectrum.
This should be kept in mind so that the diagnosis of PSS is made and
treatment properly instituted, which in my opinion is not GH, but
removal of the child from the adverse environment, particularly for type
II.
Types of PSS as
Described in the 3rd Edition of Pediatric Endocrinology1
At least three
subtypes of psychosocial short stature have been recognized (Table 2).
The first (type I) occurs in infants and children 2 years of age or
younger. These infants usually have failure to thrive (nutritional
deficiency), as well as short stature, and have been very adequately
described by Krieger, Whitten, and colleagues.2-6 There is
no evidence that these children have a hormonal disturbance, such as
growth hormone deficiency, and they usually recover when sufficient
calories are ingested. Their parents do not usually blatantly reject
the child. The mothers characteristically have multiple children or
responsibilities. They are usually disorganized, and the children do
not receive the food or the attention they need, but the attention they
receive is usually adequate for infants to again grow, if they are given
adequate nourishment. Nevertheless, growth in some may be inadequate
without further psychosocial interventions, as reported by Bithoney et
al.7-9
Type II PSS has been
called transient hypopituitarism, reversible hyposomatotropism,
emotional deprivation, maternal deprivation, psychosomatic dwarfism,
abuse dwarfism, and the “garbage can” syndrome. The term PSS is
preferable to definitions that include the presence or absence of GH,
the presence or absence of overt psychologic abuse, or emotional
deprivation. This type occurs characteristically in children 3 years of
age and older. There is a greater psychologic component, and GH
response may be inadequate after stimulation with pharmacologic agents,
such as arginine or insulin. Other abnormalities indicating
adrenocorticotropic hormone (ACTH), thyroid stimulating hormone, and
gonadotropin deficiency may be noted; however, GH deficiency is the most
common endocrine aberrancy. The parents in this group usually reject
their children and abuse them psychologically. The fathers and/or
mothers are frequently chronic alcoholics. Occasionally type I patients
are observed to advance into type II, which is not surprising.
Type III of PSS was
described by Boulton et al,10 who studied seven children aged
3.6 – 11.6 years who did not have the bizarre signs and symptoms of type
II patients. They were significantly depressed and/or had a disorder of
attachment often dating from infancy. In contrast to previously reported
patients they secreted GH when tested and had a significant increase in
growth when given growth hormone treatment. A lesser response was
obtained with a placebo. The authors emphasized that type III PSS
patients did not show lack of discrimination in relationships, nor did
they display the self-destructive behavior, pain agnosia, or bizarre
eating and sleeping disorders seen in many type II patients. In
addition, the parents were not indifferent and rejecting, as are those
with PSS type II. The parents also had insight into the problem, which
was not characteristic of the parents of other patients with PSS and
several felt guilty and/or had depression.
The classifications
discussed here by the English group and that presented in Lifshitz’s
Endocrine Text are compatible. Type I, as described above, should
remain as type I and be applied to infants and very young children.
Type II pertains to children with severe PSS of the hyperphagia type.
In my opinion, type II should be limited to this group. Type III is
where further subclassifications should be placed. For example, type
IIIA could (should) be the group described by Boulton10 and
type IIIB of the type referred to by Gohlke et al. With this
classification type IIIA & B can be subdivided or a type IV added as
further subgroups are recognized. I wonder if Drs. Gohlke et al or
others agree with my thinking? A letter to the Editors of GGH
will be most welcome.
References
1.
Blizzard RM, Bulatovic A. In: Lifshitz F, ed. Pediatric Endocrinology
3rd Edition. New York: Marcel Dekker, 1996:83-93.
2.
Krieger I. Clin Pediatr 1974;13:127-133.
3.
Krieger I. Mellinger RC. J Pediatr 1971;79:216-225.
4.
Whitten C, Fischoff J. JAMA 1969;209:1675-1682.
5.
Krieger I.
In: Gardner LI, Amacher P, eds.
Endocrine Aspects of
Malnutrition: Marasmus. Kwashirkor and Psychosocial Deprivation.
Kroc Foundation, CA: Santa Ynes, 1973:129-162.
6.
Krieger I, Whitten CF. J Pediatr 1969;75:374-379.
7.
Bithoney WG, et al. Dev Behav Pediatr 1989;10(1):27-31.
8.
Bithoney WG, Newberger EH. Dev Behav Pediatr 1987;8(1):32-36.
9.
Bithoney WG, et al. Pediatr Rev 1992;13(12);453-459.
10.
Boulton TJC, et al. Acta Paediatr 1992;81:322-325.
Robert M. Blizzard,
MD
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