Psychosocial Dwarfism Research Articles

Hyperphagic short stature: A case report and review of literature

A 5½-year-old adopted girl was referred to us in view of short stature. After ruling out systemic illness, she was evaluated for growth hormone deficiency (GHD) by stimulation tests. The peak value was 3.47 ng/ml. She was then started on growth hormone (GH). At the end of 6 months of GH therapy, her height velocity was only 3 cm/year. There was a lack of attachment between the mother and the child. She had history of hyperphagia, stealing, and hoarding food. Psychiatry consultation confirmed that the child had appetite disorder, and hence was diagnosed as hyperphagic short stature (HSS). The girl and her parents are undergoing psychiatric therapy for the same. Psychosocial dwarfism seems to originate from serious disturbances in the mother–child relationship. These children mimic patients with GHD, but have poor response to GH therapy. This case underscores the importance of social environment in the growth of the individual.

Catecholaminergic Axonal Varicosities Appear to Innervate Growth Hormone-Releasing Hormone-Immunoreactive Neurons in the Human Hypothalamus: The Possible Morphological Substrate of the Stress-Suppressed Growth

Stress is considered to be a major factor in the regulation of growth. Psychosocial dwarfism, characterized with short stature, delayed puberty, and depression, is typically preceded by psychological harassment or stressful environment. It has been observed that stress suppresses GH secretion, possibly via the attenuation of GHRH secretion. However, the exact mechanism of the impact of stress on growth has not been elucidated yet. Our previous studies revealed intimate associations between neuropeptide Y (NPY)-immunoreactive (IR) axonal varicosities and GHRH-IR perikarya in the human hypothalamus. Because NPY is considered to be a stress molecule, NPY-GHRH juxtapositions may represent an important factor of stress-suppressed GHRH release. In addition to NPY, catecholamines are among the major markers of stress. Thus, in the present study, we examined the putative juxtapositions between the catecholaminergic tyrosine hydroxylase (TH)-/dopamine-β-hydroxylase-/phenylethanolamine N-methyltransferase-IR and GHRH-IR neural elements in the human hypothalamus. To reveal these juxtapositions, double-label immunohistochemistry was used. Our findings revealed that the majority of the GHRH-IR perikarya formed intimate associations with TH-IR fiber varicosities. The majority of these juxtapositions were found in the infundibular nucleus/median eminence. The lack of phenylethanolamine N-methyltransferase-GHRH associations and the small number of dopamine-β-hydroxylase-GHRH juxtapositions suggest that the vast majority of the observed TH-GHRH juxtapositions represent dopaminergic associations. The density of the abutting TH-IR fibers on the surface of the GHRH perikarya suggests that these juxtapositions may be functional synapses, and thus, in addition to NPY, catecholamines may regulate GHRH secretion via direct synaptic mechanisms.

Psychosocial dwarfism: Psychopathological aspects and putative neuroendocrine markers

There exists an extensive terminology for defining the situation of children who, in varying circumstances, suffer from affective deprivation (AD), within an unsatisfactory family situation or in institutions. Nevertheless, the neuroendocrine mechanisms (if they exist) determining it have yet to be identified. Our objective was to determine if specific neuroendocrine markers, all of them previously implicated in affective disorders, could be modified, and in which sense, in affective deprivation syndrome of the child. For this purpose, we studied three separate groups of children: (1) control group (CG); (2) children suffering from AD; and (3) children with non-organic failure to thrive (NOFT). In every case, we studied the serum levels of melatonin, serotonin, β-endorphins and adrenocorticotropic hormone (ACTH); and kynurenine pathway tryptophan metabolites (both during the day and at night). Significantly, there was a conspicuous reduction in the levels of each of the neuroendocrine markers (melatonin, serotonin, β-endorphins and ACTH) in the group suffering from affective deficiency, a diminution which was even more noticeable in the group of patients presenting delayed growth. Furthermore, as also occurs in other affective disorders, there were corresponding modifications in the metabolisation of tryptophan. We report the existence of neuroendocrine mechanisms that are associated with the above-mentioned clinical manifestations in these patients, mechanisms that may underlie the close connection existing between AD syndrome and the cause of NOFT. These data suggest that the AD syndrome and NOFT comprise a single process, but one with a different evolutionary continuum of psychosocial dwarfism.

Le nanisme psychosocial, une réalité toujours d’actualité – à propos d’un cas

Psychosocial dwarfism is a rare condition, but can still be observed. We report the case of a 5-year-old girl seen in our clinic for severe growth retardation that had been evolving for several years. A growth arrest was observed beginning at the age of 3.5years. Initial clinical examination and biological investigations were negative. As the child was hospitalized, serious disturbances in mother-child relations were observed. A significant modification of the child's behavior was observed when the mother was absent. Following questioning, the mother admitted social problems; the pregnancy had not been desired and this contributed to a lack of attachment between the mother and the child. Psychosocial dwarfism seems to originate from serious disturbances in the mother-child relationship. It seems to be caused by a partial growth hormone deficiency and a frequent context of malnutrition due to food behavior disorders. Family problems need to be taken into account and intensive medical and psychological follow-up is required. Prognosis is good if an early diagnosis is made and followed with a close and prolonged follow-up.

Associations between the human growth hormone-releasing hormone- and neuropeptide-Y-immunoreactive systems in the human diencephalon: A possible morphological substrate of the impact of stress on growth

Previous studies revealed that stress is a pivotal factor in the regulation of growth. Psychological harassment may result in psychosocial dwarfism with delayed puberty, short stature and depression. Growth hormone (GH) secretion is suppressed by stress, possibly via the attenuation of growth hormone-releasing hormone (GHRH) secretion. However, the morphological substrate of this phenomenon has not been elucidated yet.Since neuropeptide Y (NPY) levels in the plasma is increased by administration of various stressors, the common consensus is that NPY plays a crucial role in the stress response. In the present study, we examined the putative juxtapositions between the NPY- and GHRH-immunoreactive (IR) systems in the human hypothalamus using double-label immunohistochemistry. Our findings revealed that the majority of the GHRH-IR perikarya formed intimate associations with NPY-IR fiber varicosities. The majority of these juxtapositions were found in the infundibular nucleus/median eminence where NPY-IR fiber varicosities often covered a significant surface area of the GHRH neurons.Since the juxtapositions between the GHRH-IR perikarya and NPY-IR fiber varicosities may be functional synapses, they may represent the morphological substrate of stress-suppressed GH secretion. The large number of contacting elements indicates that NPY plays a pivotal role in GH release, and may be considered as a major factor in the attenuation of growth by stress in humans.

Patterns of Aberrant Eating Among Pre-adolescent Children in Foster Care

The paper reports epidemiological and phenomenological investigations of aberrant eating among 347 pre-adolescent children in court-ordered foster and kinship care, in New South Wales, Australia. A quarter of children displayed clinically significant aberrant eating problems, with no evidence of gender or age effects. Two distinct patterns were identified. The first is a pattern of excessive eating and food acquisition and maintenance behaviors without concurrent obesity (termed Food maintenance syndrome), resembling the behavioral correlates of Hyperphagic Short Stature (Psychosocial Dwarfism). Various data suggest this pattern is primarily triggered by acute stress, including maltreatment in care, against a background of complex psychopathology and developmental disabilities. The second is a cluster of pica-type eating behaviors that correlates with self-injurious behavior, and is closely associated with developmental disabilities. The paper includes recommendations for clinicians working with pre-adolescent children in care.

Psychosocial Dwarfism: A Case Report

A 4.5-yr-old boy with Down syndrome showed stunted growth for 2 yr. The patient was endocrinologically evaluated as having complete growth hormone deficiency with low serum level of IGF-I, 8.2 ng/ml, and no response of growth hormone secretion to an arginine provocation test. He was diagnosed with psychosocial dwarfism induced by his mother. Without any specific treatment, separation of the patient from his mother normalized his growth and IGF-I, 51 ng/ml. Psychosocial dwarfism should be considered as a cause of growth retardation with pituitary malfunction.

A Case‐comparison Study of the Characteristics of Children with a Short Stature Syndrome Induced by Stress (Hyperphagic Short Stature) and a Consecutive Series of Unaffected “Stressed” Children

Recently a type of growth failure (Hyperphagic Short Stature) has been described, in which there is potentially reversible severe impairment of growth hormone secretion, in association with excessively high levels of psychosocial stress. This condition is a variant of the disorder formerly known as Psychosocial Dwarfism. In the present study we compared children with Hyperphagic Short Stature (N = 25, aged 9.04 years+/-3.78, 72% male) and a closely matched sample with normal height, drawn from comparably stressful family circumstances (N = 25, aged 10.61+/-3.04, 60% male). Measures of the psychosocial environment, anthropometry, and developmental history from infancy were obtained. Many symptoms thought previously to be characteristics of psychosocial dwarfism were found to be nonspecific stress responses. Hypotonia (p < .05), enuresis/encopresis (p < .01), and sleep cycle disruption (p < .05) did differentiate the groups. Growth, appetite, and sleep are all influenced by hypothalamic nuclei, suggesting hypothalamic pathology could account for most of the clinical features of Hyperphagic Short Stature.

Book reviews

Book reviewed in this article:Gender Identities and Education: The Impact of Starting School. By B. Lloyd &amp; G. Duveen.Stories of Childhood: Shifting Agendas of Child Concern. By R. Stainton Rogers &amp; W. Stainton Rogers.Children's Welfare and the Law: The Limits of Legal Intervention. By M. King &amp; J. Trowell.Nonparametric Statistics: An Introduction. By J. D. Gibbons.SPSS/PC+ Made Simple. By P. R. Kinnear &amp; C. D. Gray.SPSS For Windows Made Simple. By P. R. Kinnear &amp; C. D. Gray.Language, Thought and Representation. By R. J. Stevenson.Becoming Men: The Development of Aspirations, Values, and Adaptational Styles. By Daniel A. Hart.Mental Lives: Case Studies in Cognition. Edited by R. Campbell.The Kaspar Hauser Syndrome of' Psychosocial Dwarfism’: Deficient Statural, Intellectual, and Social Growth Induced by Child Abuse. By John Money.Women and Gender: A Feminist Psychology. By Rhoda Unger &amp; Mary Crawford.

Review of The Kaspar Hauser Syndrome of "Psychosocial Dwarfism": Deficient Statural, Intellectual, and Social Growth Induced by Child Abuse.

Review of The Kaspar Hauser Syndrome of "Psychosocial Dwarfism": Deficient Statural, Intellectual, and Social Growth Induced by Child Abuse.

Reversibility of physiological growth hormone secretion in children with psychosocial dwarfism.

Reversibility of GH insufficiency with a change of environment is characteristic of psychosocial dwarfism, and excludes an organic endocrinopathy. However, the change in GH pulsatility has not previously been described. We therefore wished to study spontaneous GH secretion before and after change to a more favourable environment in 11 children with psychosocial deprivation and short stature in order to evaluate if separation from the families can modify their patterns of GH secretion. We describe 11 prepubertal children (6 M and 5 F; 2.2-13.5 years of age) who had growth failure and psychosocial deprivation. They were diagnosed by a multidisciplinary team as having environmental growth failure after admission to hospital for 3 weeks. Six of them were discovered to have been sexually abused. During the uninterrupted hospital admission parental access was restricted. Three sets of 18-hour GH profiles were performed on each child, except one child who had only two, during the 3-week admission. Pulse analysis of GH profiles was by Fourier transformation. On the first day of admission spontaneous GH secretion demonstrated a spectrum of abnormalities in the pattern of basal values, pulse frequency and pulse amplitude. Such GH insufficiency showed reversibility during the 3 weeks in hospital. Indeed, there was a significant increase in GH secretion which was amplitude modulated without any significant modification in pulse frequency. Our data indicate that there is abnormal physiological GH secretion in children with psychosocial deprivation, which is associated with growth failure. Despite a pathological situation, each child retained his own characteristic pattern of GH pulsatility. The pattern of reversibility of abnormal GH pulsatility provides information for the mechanism of the control of GH secretion.

The Kaspar Hauser Syndrome of 'Psychosocial Dwarfism': Deficient Statural, Intellectual, and Social Growth Induced by Child Abuse

Hauser emerged at the Haller Gate in the city of Nuremberg, Bavaria, on May 26,1828, shrouded in mystery and greatly romanticized. A 17-year-old dwarfed male, with a blank mind and without language, he exhibited a spurt of growth and intellectual and social development, becoming a highly peculiar and limited young man whose origin remained enigmatic when he was assassinated on December 14,1833. His story became the sensation of the day throughout Europe and has been enshrined in literature through the present. In his book, John Money proposes Kaspar Hauser syndrome to designate a previously poorly understood and described constellation of symptoms and findings he and others have noted in small children who had been sequestered in their homes and subjected by their caretakers to extreme neglect and other abuses, lasting to adolescence in some cases. His appears to be the first book-length treatment in the scientific literature of this

REVERSIBILITY OF PHYSIOLOGICAL GROWTH HORMONE SECRETION IN CHILDREN WITH PSYCHOSOCIAL DWAREISM

We have studied physiological growth hormone secretion in 11 (6M, 5F) short prenubertal children, age range 2.2 - 13-5 years, with psychosocial dwarfism. All patients were admitted to hospital, as a more favourable environment, as part of their medical assessment with limited parental access. 5 had been sexually abused which was only discovered during their hospital admission. An assessment of physiological growth hormone secretion was commenced within 1 hour of admission. A second assessment was made between 4 and 9 days with a third assessment at the end of the 3 week admission. All growth hormone serum profiles were obtained at 15 minute intervals for 18 hours duration between 1300 and 0700 hours. After stationarisation of the profile data, analysis was by Fournier transformation as a time series analysis to examine the frequency and amplitude of growth hormone pulses. Growth hormone insufficiency was observed during the first day of admission. This was reversible during the 3 weeks in hospital, although there was evidence that reversibility occurred in some children within 18 hours of admission to hospital. The dominant growth hormone secretory periodicity was between 120 and 165 minutes and showed no alteration during the 3 sampling occasions. However absolute spectrum power, which reflects the amplitude of the oscillatory signal, increased from 45 to 70 units. Despite changes in growth hormone pulse amplitude, profiles from an individual child showed characteristic “finger printing” with a reproducible pattern of pulses. Our findings demonstrate some important aspects about the investigation of children with psychosocial dwarfism as well as contributing to our knowledge of the pathophysiology of growth hormone secretory dynamics.

CLINICAL EXPERIENCE OF PSYCHO-SOCIAL DWARFISM

We describe 66 children (32 girls, 33 boys) from 51 families with an average age of 6.6 years (range, 0.9 to 16.5 years, all but 5 prepubertal) diagnosed by a multidisciplinary team as having environmental growth failure. In 67% the patients lived in families with 3 or more children, but in 73% it was the first or the second born child. 45% of the parents were divorced, in 31% the father was unemployed.In 56 children the birth weight was known and only in 29% it had been above 3000 g; 21% were premature, 29% had features of a low-birth-weight-syndrome (including four with Russell-Silver-Syndrome); the average birth weight was 2786 g.In all patients the predominant reason for referral was short stature or growth failure. In 28% psychosocial dwarfism had been suspected, and in a further 29% social or emotional problems were known to the referring physician but not suspected as the aetiology of the growth failure. Social services were already involved in 60% at presentation.At the first presentation and during the attendance of our clinic additional signs leading to the suspicion of psycho-social dwarfism were found; 42% behavior problems, 54% eating problems, 26% soiling, 18% nocturnal enuresis, and 12% deliberately wetting. During the observation period assessment revealed that 27 of our patients had occult sexual or physical abuse (group A), in 15 patients (group B) such abuse was highly suspected, but in 23 patients (group C) only emotional deprivation could be related to growth failure. In group A. behavior problems, bizarre eating habits, soiling and nocturnal enuresis was more common than in group C.

LONG-TERM GROWTH DATA AND GROWTH HORMONE SECRETION IN 65 PATIENTS WITH PSYCHO-SOCIAL DWARFISM

We describe the growth of 65 children (32 girls, 33 boys) from 51 families with an average age of 6.6 years (range, 0.9 to 16.5 years, all but 5 prepubertal) diagnosed by a multidisciplinary team as having environmental growth failure. 27 of our patients had occult sexual or physical abuse (group A), in 15 such abuse was highly suspected (group B), but in 23 only emotional deprivation could be related to growth failure (group C). 51 (78%) patients had an endocrine assessment, of whom 34 (67%) had repeated testing (25 (49%) during one uninterrupted hospital admission). 14 (41%) showed reversible growth hormone deficiency, 9 (26%) increased their growth hormone peak level during one admission, 6 (18%) had growth hormone deficiency in repeated testing. In all patients from group A and B the repeated testing demonstrated a noticeable result. Following an insulin stimulation test, only 4 patients demonstrated a cortisol peak concentration of less than 450 nmol/l. The average weight gain during hospital admission was 274 g/week. 27 patients had a change in their environment (10 were separated from their family), all had an increase In their height velocity SDS (from −0.76 (SD 1.6) to +2.39 (SD 2.5)), and 19 (70%) showed catchup growth (change in height velocity >2). 15 patients were treated with growth hormone before the diagnosis of psycho-social dwarfism was appreciated, and 4 (26%) achieved catch-up growth. All 7 patients who had growth hormone treatment coincident with environmental change achieved catch-up growth.

Nosology of Eating and Growth Disorders in Early Childhood

In this article the authors discuss the classification of eating disorders of infancy and early childhood, including pica, rumination disorder of infancy, nonorganic failure to thrive, and psychosocial dwarfism.

Failure to thrive: Current perspectives

The term ‘failure to thrive’ should refer, purely and simply, to the problem of failure to grow in accordance with expectations. It is a symptom, and a presenting problem, and may have a myriad of causes. For the sake of clarity, and to minimise confusion with other related disorders such as psychosocial dwarfism, the expression should be reserved for the description of infant growth (i.e. under 2 years of age). Usually the growth retardation is defined in terms of lack of appropriate weight gain. However the parameters of poor growth are defined, it is unlikely that any clearcut aetiology, in terms of physical (or organic) disease or disorder, will be discovered in most cases. For many years a distinction was made between ‘organic’ and ‘non-organic’ failure to thrive. This dichotomy is unwarranted and unwise. Paediatricians now realise that in all cases of nonorganic failure to thrive (NOFT) and in most cases of organic failure to thrive (FTT), the primary biologic insult is undernutrition. For many children with serious medical illnesses such as congenital heart disease, cerebral palsy, and gastrointestinal disorders a major cause of the associated growth failure is a nutritional intake that is insufficient to maintain a normal rate of weight gain. In cases of failure to thrive that are associated with inadequate parenting, including neglect and abuse, the cause of the associated growth impairment is not emotional but caloric deprivation.’ Having emphasised that growth failure is the essential symptom, the question arises what degree of growth retardation should arouse clinical concern? This is a contentious issue but some guidelines may

Paraphilia as a sexual disability: Outcome study in a female with a history of the Kaspar Hauser dwarfism syndrome

A longitudinal case study exemplifies paraphilic masochism as the sexological outcome in a female with a childhood history of the Kaspar Hauser syndrome of psychosocial or abuse dwarfism. The case is theoretically important with respect to the origins of paraphilia, and to case management.

Reversible Somatotropin Deficiency (Psychosocial Dwarfism) Presenting as Conduct Disorder and Growth Hormone Deficiency

The syndrome of reversible somatotropin deficiency (psychosocial dwarfism) is characterized by environment-related impairment of stature and mental growth and growth-hormone secretion. The syndrome is well illustrated by this case report of an eight-year-old boy with psychosocial dwarfism since the third year of life. It was undetected for four years, despite his having been in regular contact with a number of health workers. After he was removed from the deleterious environment, both his growth and behaviour improved markedly, and at follow-up he had maintained this progress.

Vulnerability, Stress, and Resilience in the Early Development of a High Risk Child

Abstract A young girl at risk for schizophrenia is admitted for inpatient evaluation at age 30 months with growth failure and psychosocial retardation. Her history suggests that adverse environmental experiences, particularly the traumatic loss of primary caregivers around age 15 months, resulted in symptoms consistent with the clinical picture of anaclitic depression and associated psychosocial dwarfism. Intervention in the form of hospitalization and placement by “prescribed” adoption into a favorable caregiving environment produced dramatic recovery in growth and psychological functioning. Follow-up data document sustained positive development 3½ years later. This case illustrates the power of a developmental perspective on psychopathology for conceptualizing and planning treatment for an individual child.