Impact Of Short Stature Research Articles

Impact of short stature on health-related quality of life in long-term survivors of acute lymphoblastic leukemia in childhood and adolescence

PurposeSome survivors of acute lymphoblastic leukemia (ALL) in childhood and adolescence exhibit short stature, especially if their treatment included cranial irradiation. The impact of this outcome on health-related quality of life (HRQL) is uncertain and so formed the basis for the investigation reported here.MethodsThis study examined the association between self-reported HRQL and measured height in a cohort (n = 75) of survivors of ALL more than 10 years from diagnosis. HRQL was expressed as utility scores generated from a multi-attribute preference-based measure, the Health Utilities Index (HUI) which includes the complementary systems HUI2 and HUI3. For single attributes the range is from 1.00 (no limitations) to 0.00 (lowest level of function). For overall HRQL the range is 1.00 (perfect health) to 0.00 (equivalent to being dead). A negative score is associated with states of health worse than being dead.ResultsThere were no statistically significant differences in overall HRQL between subjects <25th (n = 16, 21%), 15th (n = 11, 15%) and 10th (n = 10, 13%) centiles. A greater amount of emotional morbidity, focused on anger and depression, was manifest in those <25th and 15th centiles, with clinically important differences of 0.07 (p = 0.03) and 0.077 (p = 0.016) respectively, but not in the shortest group who were < 10th centile.ConclusionsStudies in large cohorts of young adults in the general population has reported an inconsistent relationship between height and HRQL. Results from the current study suggest that no such relationship exists in long-term survivors of ALL in childhood and adolescence.

Maternal height and risk of hypertensive disorders in pregnancy

Purpose: The relationship between maternal height and gestational hypertensive disorders was examined in a cohort of Chinese gravidae managed in 1997–2013 to clarify the association between short stature with preeclampsia (PE) and gestational hypertension (GH).Materials and methods: Retrospective study of 87 290 gravidae categorized by their height into four quartile groups. The impact of short stature, defined as height in the lowest quartile, on incidence of PE and GH was studied in relation to the presence of risk factors. The independent role of short stature was determined by regression analysis.Results: The 25th, 50th, and 75th percentile values of height were 154 cm, 158 cm, and 161 cm respectively. The incidence of PE, but not GH, was inversely correlated with height (p = .025). Short stature altered the impact of parity status, advanced age, high body mass index, infant gender, and medical history, on incidence of PE but not GH. On regression analysis, short stature increased risk of PE (adjusted RR 1.134, 95%CI 1.005–1.279) but reduced GH (adjusted RR 0.836, 95%CI 0.718–0.974).Conclusions: Maternal short stature should be defined according to distribution in a specific ethnic group, and it exerts a significant but opposite effect on the incidence of PE versus GH.

Talking about Height and Quality of Life: A Content-Analysis of Focus Group Discussions with Short-Statured Children and Their Parents in the US

Background: To understand what life is like for US children with a diagnosis of Growth Hormone Deficiency or Idiopathic Short Stature, the impact of short stature on Health related Quality of Life (HrQoL) was qualitatively examined and needs for care from the young patients and their parents perspective were identified. Methods: Focus group discussions with 26 American-English speaking and nine American-Spanish speaking children and their parents were conducted, transcribed verbatim and subsequently qualitatively analyzed by two independent raters, using an existing coding guideline, based on the multidimensional HrQoL concept and a special software (VERBI-Software MAXQDA 10). Results: A total of 1313 statements for the English-speaking and 447 statements for the Spanish-speaking families were categorized. In the US, the strongest frequency of mention was found for the HrQoL dimension “Social” across respondents, followed by “Treatment” and “Emotion”. Conclusion: Conducting and analyzing data generated from focus groups ensure that young patients’ experiences of disease are represented in the measure of outcomes for use in clinical trials and patient care.

Mood and illness experiences of adults with cystinosis

Background: Cystinosis is an autosomal recessive disorder with an estimated incidence of 1/100,000–200,000 live births. The main complications are renal disease, visual impairment, endocrine abnormalities and growth retardation. Objective: Our aim was to describe the mood and illness experiences of adults with cystinosis. Methods: Twenty-three patients attending the adult cystinosis clinic at Guy’s Hospital, London were asked to complete the Hospital Anxiety and Depression Scale (HADS) questionnaire anonymously. Eighteen months later, 21 patients who were still alive were invited to participate in a semi-structured interview aimed at exploring illness experience. Results: Eighteen patients completed the HADS questionnaire (means: depression = 7.2; anxiety = 9.2), and 12 participated in the interviews. Three significant themes emerged: (i) the main physical complaints were tiredness, the impact of short stature and side effects of cysteamine medication, especially halitosis, poor taste and nausea. (ii) Cystinosis has a major impact on relationships, autonomy and social life, including reliance on families for support to self-manage, distress at dependence, social anxiety, reduced social involvement and some positive effects on family cohesiveness. (iii) Patients use a range of individual coping strategies to deal with their illness and medication. Conclusions: Adult cystinosis patients reported comparatively high-anxiety and depression scores. Common complaints related to the complications of cystinosis and the side-effects of cysteamine, which impacted on relationships, autonomy and social life. Patients described a wide range of strategies, including benefit finding, for coping with cystinosis.

The Impact of Short Stature on Health-Related Quality of Life in Children with Chronic Kidney Disease

To compare the health-related quality of life (HRQoL) of children with chronic kidney disease (CKD) and short stature (SS) with that of children with CKD and normal height (NH), to evaluate the impact of catch-up growth and growth hormone (GH) use on HRQoL, and to describe the concordance of perceptions of HRQoL between children with SS and NH and their parents. Four hundred eighty-three children and/or parents enrolled in the multicenter Chronic Kidney Disease in Children study who had completed the Pediatric Quality of Life Inventory (Version 4.0) on at least 2 Chronic Kidney Disease in Children study visits composed this substudy population. Participants were dichotomized into NH or SS groups. The demographic characteristics that varied at baseline (sex, glomerular filtration rate, and parent education) were controlled for in the main analysis evaluating the impact of catch-up growth and use of GH on HRQoL. Multivariate modeling (controlling for confounding variables) revealed a significant association between both catch-up growth and GH use on parent-proxy reports of child physical functioning (P < .05) and social functioning (P < .05). Older children with CKD (15-17 years old) had significantly higher ratings than their parents on the Pediatric Quality of Life Inventory Physical, Emotional, Social, and School Functioning scales compared with younger children (8-14 years old). The finding that height gains and GH use are associated with increases in physical and social functioning by parent report provides additional support for interventions to improve height in children with CKD. The importance of evaluating both the parent and child perceptions of HRQoL is supported by our results.

The Impact of Short Stature on Health-Related Quality of Life in Children with Chronic Kidney Disease

The Impact of Short Stature on Health-Related Quality of Life in Children with Chronic Kidney Disease

Adjustment in conditions with short stature: a conceptual framework.

A major justification of extended indications for GH therapy in conditions with short stature is based on the objective of preventing or alleviating suspected psychosocial maladjustment. Despite more sophisticated research, results are still controversial concerning the actual seriousness of adjustment difficulties in various conditions with short stature, thus making it difficult to determine a patient's need for treatment. This paper discusses different concepts and assessment strategies of "adjustment" as a major source of the apparent heterogeneity among study findings, conclusions and treatment recommendations. A concise framework is developed that identifies and differentiates three hierarchical levels of research on adjustment in conditions with growth retardation: (1) stress exposure due to short stature, (2) quality of coping responses, and (3) occurrence of psychopathology. Choosing a particular research level and its corresponding measures may imply whether a high, medium or very low impact of short stature on adjustment is found, and the need for treatment that is inferred. The integration of these different research approaches within an integrative model may help to resolve apparent inconsistencies among empirical studies and reveal sources of confoundation due to other, short stature independent risk factors associated with the particular underlying growth disorder. The key terms of the suggested model are transformed into a clinical guideline for psychological assessment in patients with growth retardation and into three key criteria for the decision making process on extended indications for GH treatment with respect to the improvement of psychosocial adjustment.

The impact of short stature on child morbidity in a rural African community

This study was undertaken to assess whether short stature in children under 5 years of age is associated with an increased risk of acute or persistent diarrhoea, acute respiratory infection, or indeed any illness, taking account of possible confounding factors such as whether breastfed, water quality, degree of household crowding, age and gender. After initial anthropometric assessment, the morbidity and diet of a cohort of 403 Somali children were recorded for 1 year at 2-week intervals. In multivariate analyses of the findings, adjustments were made for the possible confounding factors listed. Short stature was associated with an increased incidence of acute diarrhoea (< -2 SD score, RR 1.7, 95% CI 1.3-2.2) and with a risk of developing persistent diarrhoea, but not with the incidence of acute respiratory infections. When adjusting for age only, short stature increased the risk of developing any illness, but this association was not shown when adjusting for the other possible confounding factors. There was a dose-effect relationship between different degrees of short stature and acute diarrhoea, with an increased incidence of acute diarrhoea among the shortest children. Efforts were made to account for the possibility of reverse causality, which seemed not to change the main findings. Thus, short stature seemed to increase the risk of subsequent acute diarrhoea in children under 5 years of age.

Short stature and growth hormone therapy. A national study of physician recommendation patterns

To determine current expert opinion and recommendations regarding the controversial issue of the use of growth hormone (GH) to treat short children who do not have classical GH deficiency (non-GHD children). Analysis of a national survey mailed to 534 US physician experts on the management of short stature (pediatric endocrinologists) with a response rate of 81.3%. The experts' GH treatment recommendations. The physicians reported that approximately 58% of their current patients undergoing GH therapy have classical GH deficiency, while 42% have other conditions. The proportion of physicians who recommended GH treatment of short non-GHD children ranged from 1% to 74% over all case scenarios presented. The likelihood of GH being recommended depended on the physiological growth characteristics of the child (ie, the child's height, growth rate, and predicted adult height), contingency factors (ie, strong family wishes or a reduction in GH cost), and physician beliefs (ie, the impact of short stature on well-being, the effectiveness of GH therapy). Each of these factors exerted highly significant, independent, and additive effects on decisions to recommend GH. Our results indicate that many pediatric endocrinologists consider GH treatment appropriate for selected short non-GHD children, going beyond current Food and Drug Administration-approved indications for GH. Decisions to recommend GH for a non-GHD child rest on a combination of medical, social, and perceptual factors; variations in treatment patterns stem from variations in these influences. Future GH use will likely be determined not only by the results of controlled trials, but also by family preferences, producer pricing, and physician perceptions of the value of height and GH therapy.

Growth, growth hormone and cognitive functions.

The interactions among short stature, growth hormone (GH) and cognitive functions have been extensively studied so far. However, although it seems well established that short stature is associated with cognitive problems, little effort has been made to point out the presence of specific psychological effects related to the different forms of short stature. In 'short normal' children, the presence of a scholastic underachievement seems to suggest that short stature 'itself' might predispose these patients to some of their psychosocial difficulties. The higher incidence of academic failure, in presence of a normal intellectual functioning, has been attributed to environmental and psychosocial factors, including over-protective parents and low self-esteem resulting from the impact of short stature. These problems appear to be common also to other forms of short stature (such as Turner's syndrome) where, however, they are frequently associated with other specific deficits. The in vivo model which might allow, at least in part, better understanding of GH (per se)-dependent effects is represented by GH deficiency (GHD), in which, however, the specific role of GH on psychological functioning is frequently masked by the presence of associated hormonal deficiencies. Children with isolated GHD are reported to have specific educational deficits, in particular learning disability and attention-deficit disorders, which have been tentatively attributed to a compromised intellectual potential. The psychological effects of long-term GH treatment in children with GHD still remain controversial, with some retrospective studies describing a generally beneficial outcome. Since early experiences in school are closely related to success in adult life, the possible implications that GHD during childhood holds during adulthood have been recently considered. Although regional differences have been observed in subgroups of adults with GHD, it seems that these patients have normal cognitive functions and educational attainment, but are more likely to be unemployed (and unmarried) than members of the general population. In general, patients with GHD, who have been treated at centers where psychological counselling was an integral part of the treatment program, seem to be better adjusted in adult life. For these reasons, a multidisciplinary treatment approach could allow early detection of problems in academic achievement and psychosocial development in patients with short stature, being immediately able to provide the appropriate educational and counselling interventions.

A new self-assessment questionnaire to measure well-being in children, particularly those of short stature

The impact of short stature on children is commonly assessed in terms of psychosocial consequences. Proxy respondents and formal behavioural performance tests constitute standard methods in the evaluation of growth hormone therapy in children of short stature. Therefore, a self-administered, short and simple adjective check-list was developed and tested on 342 children of both sexes, aged 9-13 years. Six dimensions covering alertness, self-esteem, mood, elation, stability and vitality were derived after factor analysis. High internal consistency reliability was found for all dimensions. Modest correlations with height, reaching statistical significance for alertness, mood and vitality, were observed. Girls described themselves as having lower self-esteem and vitality than boys, while boys had lower scores for elation. It was concluded that the measure was relevant and potentially useful in children with short stature.

PSYCHOSOCIAL STATUS OF GROWTH HORMONE DEFICIENT (GHD) PATIENTS IN ADULTHOOD

Top of pageAbstract The psychological and social status of adults previously treated in chilhood for short stature due to GHD has received little attention to date. These parameters were studied in a group of 28 adults with GHD: 16 with isolated GHD (treatment duration (id) : X= 6.7 yr, final height (fh): −2.3 SD in 12 boys and −2.4 SD in 4 girls and chronologic age at evaluation (CAe): X= 21.3 yr) and 12 with multiple pituitary GHD (dt: X= 9.9 yr, fh: X= −2.0 in 9 boys and −2.3, 0.3, 0.5 in 3 girls and CAe: X= 23.5 yr. All of them were idiopathic. A comparative study in the social aspects was performed in siblings. The study methods included: semistructured interview, special questionnaires and the following tests: Raven (intelligence), MMPI-Minnesota multiphasic personality inventory- (personality), STAI -State-treit (anxiety), Hamilton (depression) and CIMEC (local Spanish body image test). Patients showed different degrees of social maladjusment : social withdrawal, inhibition, parenteral dependency and depressive mood. The impact of short stature was never positive at school. In the cognitive area, academic achievement was low although intelligence quotients were normal. In contrast to their siblings the majority of patients lailed to med Spanish education standards. The unemployment rate was high. Self-esteem was generally low in both sexes. 60% had never experienced sexual intercourse and only 2 were married. Expectations of treatment were fulfilled in only 50% of cases. We conclude that GHD patients require psychological attention while undergoing GH treatment in chilhood.

How should the psychosocial impact of short stature and response to growth hormone treatment be assessed in children?

How should the psychosocial impact of short stature and response to growth hormone treatment be assessed in children?

Turner syndrome: A fresh look

Introduction of improved therapy for a disease often prompts a closer look and results in revision of previous concepts. The availability of biosynthetic human growth hormone (GH) has stimulated interest in a variety of growth disorders. There are two particularly important areas for clinical investigation: More complete information is needed as to which disorders respond to GH and what are the long-term beneficial and adverse effects. It is no less important to enhance our understanding of the psychological effects of short stature and its treatment. The ultimate goal of GH treatment is not to make the child taller, but to improve his or her well-being in the critical areas of family, school, and social life. It follows then that the efficacy of GH therapy cannot be evaluated by auxological measures alone. It is necessary to have a far better understanding than we now have of the impact of short stature on well-being in childhood and adult life. Biological parameters such as height do not affect the person in a direct way, but interact in a complex way to affect quality of life. It is particularly important to develop a better understanding of the effect of improving height in children with multiple anomalies or handicaps. Such children make up a large proportion of those followed in pediatric subspecialty clinics. The large-scale studies that are in progress should considerably enhance our knowledge of these important matters. The proceedings of the Turner symposium presented here are a valuable contribution toward a comprehensive understanding of one such syndrome comprised of multiple abnormalities. It represents a unique