Final Height Standard Deviation Score Research Articles

Longitudinal analysis of growth and body composition of Japanese 21-OHD patients in childhood

Substitution therapy of glucocorticoid is a major part of the treatment for 21-OHD (21-hydroxylase deficiency). However, the therapy causes two major adverse effects, impairment of linear growth and obesity, so that collecting precise growth data is essential for optimizing the therapy. We longitudinally evaluated the linear growth and the body composition of Japanese 21-OHD patients during childhood. For the present study, we chose 16 patients (eight of each sex) who were diagnosed during the newborn period, and continuously observed them in our institute until they were at least 15 years old. All patients were treated according to the guidelines from The Japanese Society for Pediatric Endocrinology. The final height standard deviation score (Ht-SDS) of all the patients was -1.18 ± 0.85 SD, and no significant differences were observed between males and females or between the simple virilizing form and the salt wasting form. As previously reported, in spite of nearly normal height at the onset of puberty, the pubertal height gains were severely impaired, resulting in reduced final heights. Body composition of the patients was evaluated with BMI-SDS. Our longitudinal data showed that BMI was increased up to +1.23 SD in males and up to +1.75 SD in females, and that adiposity rebound was precipitated. Our study should alert physicians to the risk of metabolic syndrome and provide a framework for further studies of metabolic syndrome in 21-OHD patients.

Linear Growth and Final Height Characteristics in Adolescent Females with Anorexia Nervosa

ObjectiveGrowth retardation is an established complication of anorexia nervosa (AN). However, findings concerning final height of AN patients are inconsistent. The aim of this study was to assess these phenomena in female adolescent inpatients with AN.MethodsWe retrospectively studied all 211 female adolescent AN patients hospitalized in an inpatient eating disorders department from 1/1/1987 to 31/12/99. Height and weight were assessed at admission and thereafter routinely during hospitalization and follow-up. Final height was measured in 69 patients 2–10 years after discharge. Pre-morbid height data was available in 29 patients.ResultsPatients’ height standard deviation scores (SDS) on admission (−0.285±1.0) and discharge (−0.271±1.02) were significantly (p<0.001) lower than expected in normal adolescents. Patients admitted at age ≤13 years, or less than 1 year after menarche, were more severely growth-impaired than patients admitted at an older age, (p = 0.03). Final height SDS, available for 69 patients, was −0.258±1.04, significantly lower than expected in a normal population (p = 0.04), and was more severely compromised in patients who were admitted less than 1 year from their menarche. In a subgroup of 29 patients with complete growth data (pre-morbid, admission, discharge, and final adult height), the pre-morbid height SDS was not significantly different from the expected (−0.11±1.1), whereas heights at the other time points were significantly (p = 0.001) lower (−0.56±1.2, −0.52±1.2, and −0.6±1.2, respectively).ConclusionsOur findings suggest that whereas the premorbid height of female adolescent AN patients is normal, linear growth retardation is a prominent feature of their illness. Weight restoration is associated with catch-up growth, but complete catch-up is often not achieved.

Effectiveness of rhGH treatment on final height of renal-transplant recipients in childhood

Growth retardation is a considerable clinical problem in children with chronic kidney disease (CKD). Optimization of metabolic and nutritional parameters does not always lead to improved growth. Recombinant human growth hormone (rhGH) treatment has been used to improve height. Several studies in the literature have shown increased growth velocity, although data on the final height (FH) reached are scarce. We assessed the effect of rhGH on FH standard deviation score (SDS) in children with CKD following renal transplantation (RTx), comparing it with patients who did not receive rhGH (control group) but were treated with the same protocol and followed up in a single Center. Thirty-three patients received rhGH treatment until FH. Fourteen who refused rhGH therapy were included in the controls. Prognostic factors for FH and changes in glomerular filtration rate (GFR) during follow-up were also analyzed FH SDS in rhGH-treated patients was significantly higher than in controls (-1.88 ± 1.14 vs -3.48 ± 1.19 SDS, respectively, p <0.05). In both groups, a similar reduction in GFR was observed. Height (SDS) at onset of rhGH treatment was the only statistically significant variable useful to predict response to treatment (p = 0.001). Our findings confirm that rhGH is effective to improve FH in CKD RTx patients, without affecting kidney function.

Final height in elite male artistic gymnasts

Elite male artistic gymnasts (AG) are exposed to high levels of physical and psychological stress during adolescence and experience a significant late maturation in both linear growth and pubertal development. The aim of the present study was to determine the impact of intensive physical training on the adult final height in elite male AG. This study is unique in character, as all variables were measured on the field of competition. The study was prospective and longitudinal; however, the current analysis of data is cross-sectional. Data from 86 elite male AG were obtained during the gymnastics competitions of European and World Championships. Clinical evaluation included height and weight measurements, as well as assessment of pubic hair and genital development according to Tanner's stages of pubertal development. The laboratory investigation included determination of skeletal maturation. All athletes completed a questionnaire that included questions on personal (onset and intensity of training, number of competitions per year) and family data (paternal and maternal heights). Male AG were below the 50th percentile for both final height and weight. Elite male AG had final height standard deviation score (SDS) lower than their genetic predisposition. Final height SDS was correlated positively with target height SDS (r = 0.430, p < 0.001) and weight SDS (r = 0.477, p < 0.001) and negatively to the intensity of training (r = -0.252, p = 0.022). The main factors influencing final height, by multiple regression analysis were weight SDS (p < 0.001) and target height SDS (p = 0.003). In elite maleAG, final height falls short of genetic predisposition, still well within normal limits. Considering medical and psychological risks in general, and based on the results of this research project, the International Federation of Gymnastics has increased the age limit for participants in international gymnastics competitions by 1 year.

Effect of GnRH analogue on height potential in patients with severe growth hormone insensitivity syndrome treated with IGF-I

Children with growth hormone insensitivity syndrome (GHIS) who receive insulin-like growth factor 1 (IGF-1) treatment and enter puberty with inadequate height gain are unlikely to reach adult height within the normal range. Final height standard deviation score (SDS) in most treated children is < or = -5. Combining IGF-1 with gonadotrophin-releasing hormone analogue (GnRHa) therapy may help to improve their final height. Three patients on IGF-1 treatment, two with primary GHI and one with secondary GHI, were started on GnRHa therapy at the onset of puberty. Their ages ranged from 6.4 years to 12.9 years at the start of IGF-1 therapy (120 microg/kg twice daily by subcutaneous injection). Gains in height/bone age SDSs under GnRHa therapy ranged from 0 to 0.9. Growth velocity on GnRHa therapy ranged from 4 cm/year to 4.8 cm/year. Bone maturation (measured as change in bone age divided by change in chronological age, deltaBA/deltaCA) decreased after the start of GnRHa therapy. Predicted adult height (PAH) improved in two patients and was maintained in one. Bone mineral density showed gradual improvement from baseline. Treatment with GnRHa resulted in a gain in PAH. Final height results will provide the definite answer on the effectiveness of this combined treatment.

Final height and weight of long-term survivors of childhood malignancies.

The aim of this study was to investigate growth and final height in young adults after therapy for malignant diseases. Final height and weight was studied in 50 long-term survivors (LTS) of childhood cancer (aged 17-31 years; 30 men, 20 women) 3-18 years after treatment for malignant diseases (7 acute lymphoblastic leukemia, 20 lymphoma, 8 sarcoma, 15 malignant central nervous system [CNS] tumours). None of the LTS had been treated with growth hormone (GH). A decrease in final height SDS (Standard deviation score) occurred in both LTS of malignant CNS tumours (median height SDS at diagnosis, 0.3; range, -0.9 to 2.2; median final height SDS, -1.3; range, -3.9 to 1.9; p < 0.01) and LTS of lymphoma (p < 0.05) or leukemia (p < 0.05). However, only LTS who received cranial (p < 0.05) or craniospinal (p < 0.001) irradiation (XRT) exhibited reduced final heights. LTS who had received XRT not involving the CNS or had received no XRT at all presented no reduction in final height. LTS of CNS tumours treated with high craniospinal XRT doses (24 to 56 Gy) reached lower (p < 0.01) final heights when compared with LTS of leukemia who received lower cranial XRT doses (18 to 24 Gy). Final height SDS correlated with chronological age at initiation of therapy (p < 0.05). No correlation was found between the cumulative doses of applied chemotherapeutic agents and the final height of LTS. During follow-up LTS developed an increase in weight for height index (WFH) which occurred independent of XRT. In conclusion, cranial and craniospinal XRT especially in young children with malignancies resulted in a decrease in final height SDS. As 6 of 15 LTS of malignant CNS tumours exhibited a final height SDS below -2 SD, analysis of pituitary function and substitution of GH after diagnosis of GH deficiency should be considered for these patients at a young age. Others factors not directly related to XRT are responsible for the increased risk for obesity in LTS of childhood cancer.

Endocrine Complications after Hematopoietic Stem Cell Transplantation during Childhood and Adolescence

Long-term survivors of hematopoietic stem cell transplantation (HSCT) during childhood and adolescence are at risk of developing endocrine complications. The purpose of this study was to evaluate the long-term endocrine complications and their associated risk factors among such patients. We reviewed the data from 111 patients (59 males and 52 females) who underwent HSCT at the mean age of 8.3±4.1 yr. Thirty patients (27.0%) had growth impairment, and seven (21.2%) out of 33 patients who attained final height reached final height below 2 standard deviation (SD). The final height SD score of the patients conditioned with total body irradiation (TBI) was significantly lower than that of the patients conditioned without TBI (-1.18±1.14 vs. -0.19±0.78, P=0.011). Thirteen patients (11.7%) developed hypothyroidism (11 subclinical, 2 central) 3.8±1.8 (range 1.6-6.2) yr after HSCT. Nineteen (65.5%) out of 29 females had evidence of gonadal dysfunction, and 18 (64.3%) out of 28 males had evidence of gonadal dysfunction. The risk for gonadal dysfunction was significantly higher in females conditioned with busulfan/cyclophosphamide (P=0.003). These results suggest that the majority of patients treated with HSCT during childhood and adolescence have one or more endocrine complications. Therefore, multiple endocrine functions should be monitored periodically after HSCT until they reach adult age.

Height growth during adolescence and final height after haematopoietic SCT for childhood acute leukaemia: the impact of a conditioning regimen with BU or TBI

We compared the impact of a conditioning regimen with BU (n=16) or fractionated TBI (n=42) on height growth during adolescence and final height (FH), in 58 adults transplanted for acute leukaemia before adolescence (younger than 9 for girls and 11 for boys, and prepubertal). Heights were measured at three key periods, that is, transplantation, before adolescence, and FH, and compared using height standard deviation score (SDS) and cumulative change in SDS. The influence of the conditioning regimen was assessed using multiple linear regression and adjusting for gender, central nervous system irradiation, age and leukaemia status at transplant and type of transplantation. Overall mean height SDS was near normal at transplantation and before adolescence (0.2+/-0.1 and -0.2+/-0.1, respectively), but decreased to -1.6+/-0.1 at FH. There were significant differences between the TBI and BU groups when comparing FH SDS (-1.8+/-0.2 vs -0.8+/-0.2, P=0.001), mean change in height SDS from transplantation to FH (-2+/-0.1 vs -1.1+/-0.2, P=0.002) and mean change in height SDS during adolescence (-1.6+/-0.1 vs -0.7+/-0.2, P=0.003). We conclude that preparations involving BU, although less toxic than TBI-containing regimens, also have adverse effects on growth, predominantly during adolescence.

Final height in pediatric patients after hyperfractionated total body irradiation and stem cell transplantation

Impaired linear growth has been shown to occur in individuals treated during childhood with single-dose and fractionated total body irradiation (TBI) before stem cell transplantation. Our objective was to describe the final heights attained and patient/treatment factors correlating with final height in a cohort of childhood cancer survivors treated with hyperfractionated TBI (total dose 1375 or 1500 cGy). Thirty individuals (18 men) were included in the study. The mean final height standard deviation score (s.d.s.) was -1.9 +/- 0.2, significantly lower than height s.d.s. at TBI (-0.2 +/- 0.2, P < 0.001). Final height s.d.s. was significantly correlated with age at diagnosis, age at TBI and target height (P = 0.04, P < 0.001, P < 0.001, respectively). Treatment with growth hormone (GH) (n = 7) maintained mean height s.d.s. at -2.0 from the onset of GH therapy until attainment of final height. The mean final sitting height s.d.s. was -2.2 +/- 0.2 (n = 16), significantly shorter than mean final standing height s.d.s. (P < 0.01). In conclusion, treatment with hyperfractionated TBI is associated with a reduction in standing height and an even greater reduction in sitting height. Final height after hyperfractionated TBI was similar to that reported after fractionated TBI.

A study and the growth and the development of microvascular complications in patients with type 1 diabetes mellitus

Purpose : Reduced growth and microvascular complications have been recognized as consequences of type 1 diabetes mellitus (T1DM). We assessed the effect of T1DM on growth and factors associated with the development of microvascular complications. Methods : We conducted a retrospective longitudinal evaluation of 154 patients above 16 years of age. We analyzed factors which affect final height standard deviation scores (SDS) and development of microvascular complications. Results : Final height SDS was -0.11±1.15 (-0.26±1.33 in females, 0.04±0.91 in males). Final height SDS was significantly lower than midparental height SDS and height SDS at diagnosis. There was no difference in final height SDS according to age at onset, existence or nonexistence of complications, or average HbA1C. Height SDS at onset of puberty, midparental height SDS and pubertal growth gain affected final height SDS. The number of patients with complications was 37 (24 percent). Microvascular complications developed at a younger age and after longer duration of diabetes in patients with a prepubertal onset of T1DM compared to patients with pubertal onset. Patients with complications had a higher level of average HbA1C than patients without complications. Patients whose microalbuminuria regressed had lower levels of average HbA1C, systolic BP, second 24h urine microalbumin than patients with persistant or progressed microalbuminuria. Conclusion : The results suggest that degrees of glycemic control don't affect final height, but various factors associated with T1DM can impair growth potential. Additionally, the degrees of glycemic control and puberty affect the development of microvascular complications.

Final Height of Children after Stem Cell Transplantation

Background: Growth impairment is a common complication after hematopoietic stem cell transplantation (SCT). The aim of this study was to evaluate the final adult height of patients who underwent SCT in childhood and to identify the factors that influence long-term growth in these patients. Methods: A retrospective review of 15 children who underwent SCT before puberty at Chonnam National University Hospital and reached final adult height was undertaken. To assess the severity of height reduction and to monitor the height changes longitudinally, height measurements of each patient both at the time of SCT and the final height were expressed as the height standard deviation score (SDS). Results: Seven children were males and eight were females with a median age of 12.8±2.4 years (range, 6.3∼14.7) at SCT. The median follow-up period was 7.1±2.0 years (range, 4.5∼11.1) and their final height was achieved at 18.1±1.5 years (range, 17.0∼21.8). Final height SDS values were within normal for the healthy population in all except two who had short stature (below 󰠏2.0 SDS). No patient achieved height values greater than +2.0 SDS. The final height SDS value (󰠏0.5±1.2) was not decreased from the height SDS value at SCT (󰠏0.8±0.8). The younger age group at SCT (6.1∼10.0 years, n=5) showed significantly lower final height SDS and greater ΔSDS than the older age group (10.1∼15.0 years, n=10) (󰠏1.5± 0.6 vs. 󰠏0.1±1.1, P .05) and the final height SDS (P<.05). The gender, type of disease, donor type or the presence of chronic graft-versus-host disease did not influence height. Conclusion: Growth impairment may be encountered in children after SCT. A younger age at transplant and irradiation were found to be factors associated with reduced final height. However, most patients (13/15) reached a final adult height within normal limits for the general healthy population. (Korean J Hematol 2007;42:382-391.) 󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏

Influence of gender and pubertal stage at diagnosis on growth outcome in childhood thyrotoxicosis: results of a collaborative study

To evaluate the influence of sex as well as pubertal stage at diagnosis on the growth outcome of childhood thyrotoxicosis. Retrospective, collaborative study. Longitudinal auxological evaluation in 101 patients (M/F 23/78) for 4.7 +/- 3.1 years subdivided according to pubertal stage at diagnosis into prepubertal (group I) and pubertal (group II). At diagnosis height and bone age (BA) standard deviation score (SDS) were positive both in girls and boys of groups I and II. In boys of group II, height SDS was significantly higher than in girls of the same group (P = 0.007) and in boys of group I (P = 0.026). During the follow-up, in group I, height SDS remained positive without significant differences between boys and girls, and in group II, height SDS remained significantly lower in girls than in boys. The age at onset of puberty and the age at menarche were within the normal range. Final height (FH) was within target height (TH) range in all groups The FH SDS and the height gain (FH-TH) were similar in girls and in boys in group I and significantly higher in boys than in girls (P < 0.05) in group II. The boys of group II showed a mean height gain significantly greater than that found in all the other groups. Despite the advancement of BA at presentation, there were no adverse effects on subsequent growth and FH; the growth outcome seems to be better in boys than in girls in group II.

P0678 FINAL ADULT HEIGHT OF CHILDHOOD ONSET CROHN???S DISEASE: MEAN HEIGHT DEFICIT IS 3???4CM, BUT 25% DO NOT REACH THEIR GENETICALLY DETERMINED HEIGHT RANGE

Introduction: Growth failure is an important feature of paediatric onset Crohn’s disease (CD) but there are few data regarding final adult height, in particular height deficit has rarely been described with respect to mid-parental height. The aim of this study was to determine final adult height and to examine the factors that might influence growth. Methods: We retrospectively identified 77 cases diagnosed before age 16 yrs who had achieved final adult height [defined as height > 22 yrs, or documented growth velocity <1cm/year] and in whom parental heights (Hts) were also known. No case had any other growth-inhibiting conditions. Hts were converted into standard deviation scores (SDS). Univariate analysis was undertaken of factors postulated to affect Ht: length of delay from onset of symptoms to diagnosis, pre/post pubertal onset of symptoms, site of disease activity, sex, systemic steroid therapy, and mid-parental Ht SDS. Positive factors were entered into regression models. Results: M 53%, F 47%. Median age at diagnosis 12.0 yrs after a median 1.0 yr delay. 29% had jejunal involvement. 74% had 1 or more courses of systemic steroids during follow up. Mean paternal and maternal heights were both 0.0 SDS. Mean Ht SDS of the cases were −0.7 at diagnosis improving to −0.4 at final adult height [p = 0.024]. Although the mean deficit at final adult height was 3.4cm from mid-parental height [95% CI -1.8 to -5.0cm], this included 25% with a deficit of >8cm [that is less that genetic potential]. In the regression models Ht SDS at diagnosis was negatively correlated with length of delay and positively correlated with parental SDS [R2 0.55]. Final adult height SDS was also related to parental height SDS, but the most important factors were presence of jejunal disease and height SDS at diagnosis [R2 0.64]. All these factors remained significant if length of delay was added to the model. Conclusion: This is largest group of children followed up to final adult height. The presenting features of this group, such as age, sex, height SDS and percentage with jejunal involvement are very similar to those currently reported across the British Isles [1]. The mean adult height deficit was 3–4cm, but this figure hides the 25% of cases who did not attain an adult height within the genetic range, as determined by their mid-parental height. Delay in diagnosis was related both to loss of height at diagnosis and to impaired potential for ‘catch up’. Jejunal disease predisposed to growth impairment following diagnosis. Both earlier diagnosis and improved management of cases with jejunal disease might lead to improved final adult height.

Final Height Data, Body Composition and Glucose Metabolism in Growth Hormone-Treated Short Children Born Small for Gestational Age

Extended Low birth weight has been associated with impaired insulin sensitivity, type 2 diabetes mellitus, hypertension and cardiovascular disease in later life. GH therapy is known to increase fasting and postprandial insulin levels. For this reason concern has been expressed regarding the possible detrimental effects of GH therapy in children born small for gestational age (SGA). To assess the effects of GH therapy on body composition, carbohydrate metabolism and final height in short SGA children, 165 prepubertal short children born SGA were enrolled in either a multicentre, double-blind, randomized, dose-response GH trial (n = 75) or in a GH controlled trial (n = 90). The inclusion criteria were: (1) birth length standard deviation score (SDS) below –2; (2) age 3–8 years; (3) height SDS below –2. The children’s mean (SD) age was 7.3 (2.1) years (GH dose-response trial) and 6.0 (1.5) years (GH controlled trial), birth length SDS was –3.6 and height SDS was –3.0 (0.7). In the GH dose-response trial, children were randomly assigned to either 1 mg GH/m² per day (group A, n = 41) or 2 mg GH/m² per day (group B, n = 38) (≈0.033 or 0.067 mg/kg per day, respectively). In the GH controlled trial, children were randomly assigned to 1 mg GH/m² per day (n = 60) or served as controls (n = 30). Subjects underwent standard oral glucose tolerance tests and measurement of body mass index, systolic and diastolic blood pressure and serum lipids at baseline and after 1 and 6 years of GH therapy and again 6 months after discontinuation of GH. Body composition was measured by dual energy x-ray absorptiometry at baseline and again after 3 years in the GH controlled trial. Mean (SD) final height SDS was not significantly different between the two GH dosage groups: –1.2 (0.7) in group A and –0.8 (0.7) in group B. At the start of GH therapy, 8% of children had impaired glucose tolerance (IGT). Systolic blood pressure was significantly higher in comparison with healthy peers. GH therapy induced considerably higher fasting and glucose-stimulated insulin levels after 1 and 6 years, regardless of GH dosage. After 6 years, 4% of children had IGT. Six months after discontinuation of GH, glucose levels remained normal, whereas fasting and glucose-stimulated insulin returned to levels comparable to those of healthy peers. None of the children developed diabetes. During 6 years of GH therapy both systolic and diastolic blood pressure decreased significantly and remained so after discontinuation of GH therapy. At baseline all children had reduced bone mineral content and lean body mass. Fat mass was not significantly lower than normal. Treatment with 1 mg GH/m² per day resulted in a significant increase in (and normalization of) bone mineral content and lean body mass in comparison with untreated short SGA controls. Fat mass decreased during the first year of GH but returned to values comparable to those at baseline in the following 2 years of GH therapy. We found that long-term, continuous GH therapy in short children born SGA leads to a normalization of height during childhood and to a normal final height in most children, regardless of GH dosage. Only very short or relatively older children may need a dosage of 2 mg GH/m² per day. Long-term GH therapy had no adverse effects on glucose levels and serum lipids and had a positive effect on blood pressure, even with GH dosages of up to 2 mg/m² per day. However, as has been reported in other patient groups, GH induced higher fasting and glucose-stimulated insulin levels, indicating insulin resistance. After discontinuation of GH serum insulin levels returned to normal age-reference levels. Short SGA children have a reduction in bone mineral content and lean body mass when compared with healthy controls, which significantly improved (normalized) with GH therapy at a dose of 1 mg/m² per day.

The KIGS Experience with the Addition of Gonadotropin-Releasing Hormone Agonists to Growth Hormone (GH) Treatment of Children with Idiopathic GH Deficiency

Although recombinant techniques have enabled the production of limitless amounts of human growth hormone (GH), and clinical methods for diagnosis and treatment have been greatly enhanced, the mean final heights of children with idiopathic GH deficiency (IGHD) treated with GH remain in the range of –1.3 standard deviation scores (SDS) below normal height. One of the methods used to increase height outcomes is to delay the onset and progression of puberty to allow for a longer ‘pre-pubertal’ growth phase. We reviewed the KIGS (Pharmacia International Growth Database) data of patients with IGHD who had been treated with gonadotropin-releasing hormone agonists (GnRHa) in order to see if a greater gain in height could be achieved by altering the tempo of pubertal maturation. Near-final height data were analysed in 39 adolescents (out of a total of 249) who had received GH + GnRHa therapy and were compared with similar data from 1,893 patients with IGHD treated with GH alone. The total change in height SDS in boys who received GH alone was +1.6, in contrast to +1.1 in GH + GnRHa-treated boys; the total change in height SDS in girls who received GH alone was +1.4 in contrast to +1.1 in girls treated with GH + GnRHa. The near final height SDS in girls treated with GH + GnRHa was 1.0 below the mid-parental target height (MPH), whereas there was only a –0.5 SDS difference in girls treated with GH. Approximation to the MPH did not differ in boys between the two treatment groups. These data suggest that the attainment of a substantial height SDS by manipulating the tempo of puberty is limited, but that optimizing growth during the pre-pubertal phase is a more important factor.

Final height and pubertal growth in Japanese patients with congenital hypothyroidism detected by neonatal screening

Aim: To investigate the final adult heights and pubertal growth patterns in Japanese patients with congenital hypothyroidism (CH) detected by neonatal screening. Methods: A retrospective chart review was conducted of female patients &gt;15y of age (n= 18) and male patients &gt; 18y of age (n= 9), who were detected by neonatal screening and kept on continuous thyroid hormone replacement therapy. Final height standard deviation scores (FHSDS) and target height standard deviation scores (THSDS) were determined. Parameters characterizing the pubertal growth process (such as age at onset of pubertal growth spurt and age at peak pubertal growth) were obtained from each patient's growth rate chart. Menarchial age was determined in each female patient by reviewing the medical record. The impact on FHSDS of the etiology of CH, the severity of CH, the time of initiation of therapy and the adequacy of treatment during the first year of life was assessed. Results: All patients had received initial thyroid hormone treatment no later than 50 d of age, and had reached their final height. The mean FHSDS for female and male patients were +0.17 ±0.99 and ‐0.03 ± 0.99, respectively. The mean FHSDS‐THSDS for female and male patients was +0.09 ± 0.77 and ‐0.19 ±0.53, respectively. No difference was seen in pubertal growth parameters for either gender compared with that of the reference population, except for a greater peak height velocity and pubertal height gain in male patients. The mean menarchial age was identical to that of the reference population. No significant relationship was found between the FHSDS and any of the factors investigated. Conclusion: The adult height of patients with CH detected by neonatal screening was equivalent to that of the reference population and their target height. As long as early intervention and satisfactory management are ensured, severe CH does not appear to reduce final adult height.

Treatment of GH-deficient children with two different GH doses: effect on final height and cost-benefit implications.

Treatment of GH-deficient (GHD) children with higher doses of recombinant human GH (rhGH) than conventional ones has been reported to result in higher growth velocity and increased final height. These findings, however, were observed by comparing large but heterogeneous groups of children. We wanted to verify whether the same results could be obtained in two groups of appropriately well-matched children with isolated GHD treated with high vs conventional doses of rhGH. Out of two cohorts of GHD children, cohort A (on a weekly rhGH dose of 0.3 mg/kg body weight) and cohort B (on a weekly rhGH dose of 0.15 mg/kg body weight), we selected two groups, each including 13 patients, who before treatment were matched for age, sex and height standard deviation score (SDS). They were followed up until final height. Final height SDS was significantly higher in group A (-0.45+/-0.36 (s.d.) vs -1.07+/-0.7; P=0.008), as well as height gain SDS (1.81+/-0.58 vs 1.23+/-0.62; P=0.002). The difference between final height SDS and target height SDS was positive only in group A and significantly higher in group A than in group B (0.33+/-0.51 vs -0.46+/-0.7; P=0.01). Glucose tolerance was always normal in the group treated with higher doses. The final height of children treated with higher doses of rhGH is increased, also in relation to their genetic target. The economical burden of this choice of treatment, however, has to be taken into account when evaluating the results.

Final height and pubertal growth in Japanese patients with congenital hypothyroidism detected by neonatal screening.

To investigate the final adult heights and pubertal growth patterns in Japanese patients with congenital hypothyroidism (CH) detected by neonatal screening. A retrospective chart review was conducted of female patients >15 y of age (n = 18) and male patients >18 y of age (n = 9), who were detected by neonatal screening and kept on continuous thyroid hormone replacement therapy. Final height standard deviation scores (FHSDS) and target height standard deviation scores (THSDS) were determined. Parameters characterizing the pubertal growth process (such as age at onset of pubertal growth spurt and age at peak pubertal growth) were obtained from each patient's growth rate chart. Menarchial age was determined in each female patient by reviewing the medical record. The impact on FHSDS of the etiology of CH, the severity of CH, the time of initiation of therapy and the adequacy of treatment during the first year of life was assessed. All patients had received initial thyroid hormone treatment no later than 50 d of age, and had reached their final height. The mean FHSDS for female and male patients were +0.17 +/- 0.99 and -0.03 +/- 0.99, respectively. The mean FHSDS-THSDS for female and male patients was +0.09 +/- 0.77 and -0.19 +/- 0.53, respectively. No difference was seen in pubertal growth parameters for either gender compared with that of the reference population, except for a greater peak height velocity and pubertal height gain in male patients. The mean menarchial age was identical to that of the reference population. No significant relationship was found between the FHSDS and any of the factors investigated. The adult height of patients with CH detected by neonatal screening was equivalent to that of the reference population and their target height. As long as early intervention and satisfactory management are ensured, severe CH does not appear to reduce final adult height.

Craniopharyngioma: presentation and endocrine sequelae in 36 children.

We studied the clinical presentation by age of 36 children with craniopharyngioma, and outcome by height and body mass index (BMI). Presenting symptoms included headache (51.4%), vomiting (31%), visual disturbances (22.9%), polyuria and/or polydipsia (17.1%), delayed puberty (19.4%), short stature (13.8%), and precocious puberty (2.7%). Growth deceleration was overlooked, as was diabetes insipidus (actual rate, 52% for both). Delayed puberty was observed in all patients of appropriate age. Mean height standard deviation score (SDS) at admission was significantly lower than mean target height SDS (p = 0.004), while mean final height SDS was similar (p = 0.14). BMI SDS at last follow-up was similar to mean parental BMI SDS. We conclude that although endocrinopathies are present in most patients with craniopharyngioma, they are rarely the reason for referral. While affected prepubertal children have non-endocrine complaints, most adolescents are referred because of delayed puberty. Diabetes insipidus may be more prevalent in craniopharyngioma than previously reported. When patients with hypothalamic obesity are excluded, mean BMI SDS remains within normal range and is influenced mostly by parental BMI SDS.

Effect of Gonadotropin-Releasing Hormone Agonist Treatment in Boys with Central Precocious Puberty: Final Height Results

Objective: The small number of boys present in most studies on final height (FH) after gonadotropin-releasing hormone agonist (GnRHa) treatment for central precocious puberty (CPP) offers difficulties in the evaluation of the effects of treatment on FH in males. Method: We therefore combined FH data from The Netherlands, Italy and France to study the effect of GnRHa treatment in a large group of 26 boys with CPP. Results: The mean chronological age at the start of treatment was 7.6 ± 2.0 (SD) years, bone age (BA) was 11.0 ± 2.1 years. All boys were treated with depot formulations of the GnRHa triptorelin with established gonadal suppression for a mean treatment period of 4.7 ± 2.1 years. FH was 172.9 ± 6.6 cm. FH standard deviation score (SDS) was –0.66 ± 1.22, not significantly different from the target height SDS of –0.23 ± 0.75. FH-SDS was significantly lower in the subgroup of 12 patients with organic CPP compared to patients with idiopathic CPP (–1.34 ± 1.06 vs. –0.08 ± 1.06, respectively; p = 0.01), but no difference in height gain was observed. The mean estimated height gain, defined as the difference between predicted and actual adult height was 6.2 ± 8.7 cm using the average tables of Bayley and Pinneau, and 0.3 ± 8.6 cm using the BA advance adjusted tables. Regional differences in height gain were observed between the different countries, reflecting different local practices. Conclusion: We conclude that GnRHa treatment in boys results in a FH close to target height.