Final height and pubertal development in 55 children with idiopathic growth hormone deficiency,treated for between 2 and 15 years with human growth hormone

Sixty patients with the diagnosis of idiopathic growth hormone deficiency have been followed till final height was reached, after hGH treatment lasting between 2 and 15 (average 5.4) years. Twenty-six had total and 13 partial isolated growth hormone deficiency (IGHD); 10 had GHD plus gonadotrophin deficiency (GnD); six had multiple pituitary hormone deficiency (MPHD) and five, labelled transient prepubertal GHD, had normal responses in the insulin tolerance test when retested after the end of treatment.The final height of the patients with IGHD averaged 2.3 SD below the population mean, or 2.0SD below their midparent mean. Half the boys, but only 15% of the girls, ended above the population 3rd centile. There was no difference in final height between those with total and partial deficiency nor between patients treated prepubertally and those in whom treatment started in early puberty. In the 39 patients with IGHD the correlation of final height with midparent height was 0.72, a figure identical to that occurring in the normal population. Though final height was chiefly influenced by parental height, it was also affected by the degree of smallness when treatment began being lowered by an average of 2.5 cm for every SD that the patient's height at beginning of treatment lay below the average of all IGHD children, parents' heights being allowed for.Since untreated patients end at about 6 SD below the mean, treatment during the age span represented in these patients recovered 4SD, but failed to recover the remainder. The lost 2 SD may be due to the late start of treatment (averaging 11 years of age even in our prepubertal patients). Our findings emphasise the importance of early diagnosis, so that future patients never drop to 4 or 5 SD below mean height for age, but only to 2 or 3.Patients with IGHD plus GnD had final heights averaging 1.5 SD below the population mean and those with MPHD 1.0 SD below. This was entirely due to their developing longer legs than the patients with IGHD, the final sitting heights being the same. The long legs were due to treatment with sex steroids being started relatively late. Patients with IGHD who entered puberty spontaneously did so late in time and in the boys pubertal development was normal. In the girls there was a disturbance in the normal relationship of pubertal events and in two menarche never occurred.     

Final height and predicted height in boys with untreated constitutional growth delay

Abstract We report on 49 boys with constitutional growth delay (CGD) who were initially seen in our clinic at a mean chronological age of 13.3 years (range, 7.3–16.4) and a bone age of 11.1 years (range, 6.0–13.5). All were below the 5th height percentile for chronological age. A positive family history with delayed growth and puberty in one or both parents could be elicited in 75%. All 49 patients were re-examined at a mean age of 22.9 years (range, 20.4–31.2). Measured final height was 171.3 cm (range, 161.2–181.7), which was slightly, but significantly lower than mean target height of 173.0 cm. Final height expressed as standard deviation score (SDS) of a male adult population standard was –1.0 (range. –2.4 to +0.5), also significantly lower than initial height SDS related to bone age (SDS_BA) of –0.5 (range, –1.6 to +1.2). If related to target height (Tanner), final height was found to correlate positively with the initial bone age deficit and the initial height SDS_BA. Observed final height was also compared with the predicted adult height by the methods of Bayley-Pinneau (BP), Tanner-Whitehouse Mark II (TW II) and Roche-Wainer-Thissen. Regression equations between all three prediction methods and final height showed an excellent correlation (P<0.0001). However, only by the BP method was predicted height very close to and no different from measured final height (pairedt-test). Despite this, final height in 16 of 49 patients (32.6%) differed by more than 5.0 cm from BP predicted height. An overwhelming majority of the patients *88.6%) expressed complete or reasonable satisfaction with their adult height.Conclusion In our sample of male patients with CGD, adult height came close to but did not quite reach mean target height. The BP prediction method offers a good compromise between simplicity and accuracy but must be used judiciously because individual discrepancies with attained final height may be large and unexplained.     

Growth of patients with congenital hypothyroidism detected by neonatal screening in Japan

BACKGROUND: The growth of patients with congenital hypothyroidism detected by neonatal screening in Japan was investigated. The data investigated were obtained from Medical Aid Program for Chronic Pediatric Diseases of Specified Categories registered in 2002. METHOD: The present study included 2341 patients (1030 male, 1311 female) registered as having congenital hypothyroidism. To investigate the growth of these patients, their heights plotted on cross-sectional growth charts for boys and girls, their bodyweights expressed as percentage of bodyweight for height and the frequency distribution of percentage of bodyweight for height were assessed. RESULTS: Cross-sectional growth charts of both male and female patients showed that the heights of the majority of the patients with congenital hypothyroidism were within +/-2SD. Approximately half of the patients with heights below -2SD, had some complications. The mean percentages of ideal bodyweights for height were 103.0 +/- 12.9% for both sexes (+/-SD, n = 2033), 103.3 +/- 12.7% for boys, and 102.7 +/- 12.9% for girls. The frequency distribution of percentage of bodyweight showed no tendency of shifting to either ends in comparison with normal distribution curve. CONCLUSION: Patients with congenital hypothyroidism detected by neonatal screening had normal growth in general, suggesting that the neonatal screening system is being performed efficiently from detection to treatment of the disease in Japan. However, it remains unclear whether some uncomplicated patients with a height below -2 SD are sufficiently treated or not. Close observation of these patients may be needed.     

Hypothalamo-pituitary hypothyroidism detected by neonatal screening for congenital hypothyroidism using measurement of thyroid-stimulating hormone and thyroxine

The optimal strategy in neonatal screening for congenital hypothyroidism is still a subject of controversy. In Kanagawa Prefecture in Japan, simultaneous thyroid-stimulating hormone (TSH) and T4/fT4 determination has been used, while the results of our program may provide valuable information. Cumulative findings were analysed to determine the type and frequency of thyroid disorders in infants detected by simultaneous TSH and T4/fT4 determination, and the TSH and T4/fT4 screening strategy was validated. A total of 1284130 neonates were screened between October 1979 and September 1997 and infants followed because of low T4/fT4 without elevated TSH (T4 < 51.5 nmol/L or fT4 < 9 pmol/L and TSH < 15 mU/L) were retrospectively analysed. The first survey was carried out within 6 mo of birth and the second in 1998; 258 infants were diagnosed with congenital hypothyroidism at the first medical evaluation, 15 of them with hypothalamo-pituitary hypothyroidism. However, in the second survey, only 8 children were confirmed as having hypothalamo-pituitary hypothyroidism, therefore the incidence detected by the present strategy was 1/160516. Of 8 children with hypothalamo-pituitary hypothyroidism, mental retardation was prevented in 3 owing to early treatment. CONCLUSIONS: Simultaneous measurement of TSH and T4/fT4 is a useful strategy for detecting hypothalamo-pituitary hypothyroidism, but more studies are needed to show the cost-benefits of using this strategy.     

Growth in children with congenital hypothyroidism detected by neonatal screening

A prospective analysis of physical growth in 56 children with congenital hypothyroidism compared the children's height, weight, and head circumference with population percentiles. Two measures of skeletal maturation and predictions of adult height were also compared with population standards. The mean percentiles for the children's height and head circumference were higher than population means. Although mean bone age scores were slightly higher, predictions of adult height did not differ significantly from midparental height (arithmetic mean of sum of parental heights) and population means. The duration of intrauterine hypothyroidism as measured by gestational bone age and the duration of postnatal hypothyroidism were inversely correlated with heights attained up to 9 years. This association suggests a possible long-term influence of early hypothyroidism on growth. In children with congenital hypothyroidism, maintenance of serum thyroxine levels in the upper half of the normal range results in normal growth patterns.     

中国新生儿先天性甲状腺功能低下症与苯丙酮尿症筛查22年回顾

目的 总结我国开展新生儿筛查的情况,探讨我国新生儿筛查中存在的问题和对策.方法 回顾性分析我国新生儿筛查的发展历史、模式、筛查方法 、治疗随访与患病率等临床资料.结果我国新生儿筛查从1985年开始全面开展,筛查和治疗主要分为3种模式,目前筛查覆盖率还较低.1985至2006年共有13 229 242例新生儿参加了先天性甲状腺功能低下症(CH)的筛查,检出患儿6505例,患病率为49.2/10万,同时对13 666 750例新生儿进行了苯丙酮尿症(PKU)筛查,检出患儿1170例,患病率为8.6/10万.全国CH患病率呈上升趋势,且西部地区患病率较高,而全国PKU患病率相对较稳定.结论新生儿筛杏是预防CH和PKU患儿智力和体格发育落后的关键.应进一步提高新生儿筛查的覆盖率、筛查率和治疗率,加强健康教育,普及新生儿筛查知识,积极探索筛查新技术.     

Outcome in tall stature. Final height and psychological aspects in 220 patients with and without treatment

In 135 women and 85 men who initially presented for tall stature, the outcome in treated (56 women and 33 men; cases) and untreated (controls) was investigated. At the time of height prediction, cases were significantly taller (P≤0.03) than the controls, they had higher target heights (P<0.001) and adult height predictions (P<0.001) (according to Bailey and Pinneau) compared to the controls. Bone age (according to Greulich and Pyle) and chronological age were well matched in both groups. Final height was measured after cessation of growth at a mean age above 21.5 years. The final height prediction according to Bailey and Pinneau (BP method) overestimated the final height in controls. The mean error of estimation was −0.14 cm (±3.10) in women, and −1.86 cm (±4.37) in men. Age at the time of prediction did not significantly correlate with the degree of the prediction error. Sex hormone therapy comprised a daily oral dose of 7.5 mg conjugated oestrogens in girls (plus 5 mg dydrogesterone for 10 days a month), while boys received 500 mg testosterone enantate, intramuscularly, every 2 weeks. Therapy was well tolerated. The mean corrected effect of height reducing therapy was 3.6 cm (range: 11.9 cm to −3.3 cm) in women and 4.4 cm (range: 14.2 cm to −5.2 cm) in men. Therapy was significantly more effective when started at an earlier chronological (P<0.01) and bone age (P<0.01). The residual mean growth, after therapy was stopped, was 1.8 (±1.6) cm in women and 3.1 (±2.3) cm in men. In men, post-treatment growth was inversely correlated to chronological age (P<0.01) and bone age (P<0.05) at the end of treatment, while these correlations were not significant in women. Both groups had a higher educational level than the normal population. Treated tall women reported teasing because of tallness more frequently than controls. In tall men, practical issues such as clothing size predominated. Maximum tolerated height in males was 200 cm and in females 180 cm, thus being nearly analogous to the actual professional criteria for treatment recommendation. A positive attitude to treatment was documented in over 90% of treated individuals. Conclusions Our results show that the BP method gives acceptable adult height predictions in girls, but less accurate predictions in boys. The treatment with high doses of sex hormones was low effective in both sexes and showed a wide range of response. For success, treatment must be initiated in early puberty and terminated late. The answers to a questionnaire revealed no major psychological or social maladjustment of treated individuals compared to those untreated. Received: 18 March 1997 / Accepted in revised form: 20 June 1997     

Longitudinal assessment of children with congenital hypothyroidism detected by neonatal screening

Clinical and laboratory data from 42 children (31 females and 11 males) with primary congenital hypothyroidism (CH) diagnosed by neonatal screening over a six-year period are reported. The mean age at onset of thyroid hormone therapy was 33 days. The adequacy of replacement therapy was assessed by repeated TT4, FT4, T3 and TSH serum determinations. The high serum TT4 concentrations frequently observed were not accompanied by clinical evidence of hyperthyroidism. rT3 levels determined in 28 CH children with TT4 greater than 15 micrograms/dl were clearly higher than in the controls. The mean weight, length and head circumference remained always between the 50th and 75th centile. The radiological assessment of the knee, mainly the distal femoral surface, has been considered as an important clinical value in the initial diagnosis and in the evaluation of both severity and duration of disease. The psychomotor development was assessed using Brunet-Lezine's test until age 36 months, Stanford-Binet at 4 and 5 and WISC at 6 years of age. The mean global developmental quotients (GDQ) were always between 85 and 97 at 6 to 72 months of age, only eight children were below 85. A significant correlation was found between GDQ at 6 months and the bone age. The neurological examination showed an impairment of posture, coordination and subtle deficits in motor and perceptual abilities in a small percentage of children.     

Povidone-iodine in umbilical cord care interferes with neonatal screening for hypothyroidism

Out of 1346 newborns screened for congenital hypothyroidism, 31 non-hypothyroid infants were summoned because of a thyroid stimulating hormone (TSH) level above 10 U/ml. False-positive TSH levels were significantly more frequent in the babies treated with povidone-iodine (4.6%) than in those treated with either alcohol or triple dye (0.7%).Conclusion Iodine containing solutions should be avoided in umbilical cord care of the newborn     

北京市20年新生儿疾病筛查回顾性分析

目的分析北京市1989—2009年新生儿先天性甲状腺功能减退症（CH）和苯丙酮尿症（PKU）的筛查结果,为进一步提高新生儿疾病筛查的管理水平及干预措施提供依据。方法 采集出生72h后、正常哺乳的新生儿足跟血于特定滤纸上,进行CH及PKU筛查。PKU筛查检测指标为血苯丙氨酸（Phe）浓度,分别采用细菌抑制法（1989—2003年）和荧光法（2004—2009年）;CH筛查检测指标为血促甲状腺激素（TSH）水平,分别采用放免法（1989—2003年）及时间分辨荧光免疫分析法（DELFIA）（2003—2009年）。结果 1989—2009年,北京市共筛查新生儿1745998名,筛查率由1989年的14.01%提高到2009年98.16%,可疑患儿复诊率由1991年的65.85%提高到2009年的92.18%,共确诊CH482例,发病率1：3622;PKU192例,发病率1：9094。结论 新生儿疾病筛查是包括管理、筛查、随访、诊治、评估、教育等多个环节的系统服务工程,各部门的协调配合是提高筛查管理质量的有效措施,完善的新生儿疾病筛查工作可有效降低残疾儿的发生。     

Linear growth in children with congenital hypothyroidism detected by neonatal screening and treated early: a longitudinal study

We performed a longitudinal study of a cohort of 74 children with congenital hypothyroidism (CH) detected by neonatal screening (Buenos Aires Province, Argentina) up to the age of 3 years old, in order to study linear growth and the relationship with the severity of CH at diagnosis. The mean age at diagnosis and the start of the treatment was 16.9 +/- 5.2 days. The patients were divided into group 1--severe CH (pretreatment T4 level <4 microg/dl) (n = 47)--and group 2--less severe CH (pretreatment T4 level > or = 4 microg/dl) (n = 27). Patients with CH treated early showed a sexually dimorphic pattern of growth: girls tended to be longer than boys at all ages. Boys showed some delay of growth during the first year. No difference was found in linear growth between the two groups (more/less severe CH). Height was normal in both sexes at the age of 3 years old.     

云南省部分地区新生儿先天性甲状腺功能减低症筛查结果分析

目的 总结并分析云南省部分州市先天性甲状腺功能减低症(CH)的筛查结果.方法 对2012 年7 月至2014 年4 月在云南省昭通市、曲靖市、丽江市和迪庆藏族自治州四地出生的活产婴儿236 218 例进行CH 筛查,其中男121 463 例,女114 755 例.初筛足跟血促甲状腺激素(TSH)≥ 8 μIU/L 者原血片重新复查,复查后仍为阳性者召回进一步测定静脉血TSH 和游离甲状腺素(FT4)以明确诊断.结果 236 218 名新生儿中,血片合格率为96.67%,不合格血片补采率为81.75%,初筛阳性召回率为73.02%.确诊CH 66 例,其中男性36 例,女性30 例(P>0.05).CH 发病率为1: 3 579,显著低于全国平均发病水平(1/2 034,P<0.01).患儿出生胎龄多为37~42 周,>42 周者只占3%;大部分患儿出生体重在正常范围;出生身长<50 cm 者占32%.结论 云南地区CH 发病率低于全国平均水平;CH 患儿临床特征无特异性;云南地区新生儿疾病筛查工作质量还需要进一步提高.     

广州市18年新生儿先天性甲低苯丙酮尿症和葡萄糖-6-磷酸脱氢酶缺乏症筛查

目的 探讨广州市新生儿先天性甲低、苯丙酮尿症和葡萄糖-6-磷酸脱氢酶(G6PD)缺乏症筛查方法及其对遗传代谢缺陷病的控制作用.方法 收集广州市新生儿出生3 d时的足跟血滤纸干血斑标本,检测促甲状腺素(TSH)筛查先天性甲状腺功能减低症(CH);检测苯丙氨酸(Phe)检出持续性高苯丙氨酸血症(PHPA),筛查苯丙酮尿症(PKU)和四氢生物蝶呤缺乏症(BH4D);检测红细胞G6PD活性筛查G6PD缺乏症.凡筛查阳性者按疾病诊疗常规进行确诊和治疗,将检出的CH和PHPA作为筛查干预组.将未经过新生儿筛查出现症状才就诊、临床诊断CH和PKU的患儿做为对照组.结果 1989年4月至2007年6月共筛查新生儿945 372名,检出CH 331例,PHPA 29例,G6PD缺乏症39 700例.结果 显示CH发病率为1∶2 856, PHPA为1∶32 599,G6PD缺乏症达1∶23.81.总发病率为4.24%.CH和PHPA共360例,治疗随访357例,治疗率99.2%.PHPA全部免费治疗.平均开始治疗日龄20 d,4～6岁时IQ或0～3岁DQ测定智能正常(IQ或DQ≥90)者320例(89.6%),低于正常(70≤IQ或DQ<90)者36例(10.1%), 智能残疾 (IQ或DQ<70)者1例(0.3%).对照组开始治疗年龄平均3岁,智能残疾26例,筛查组的智能发育明显好于对照组.结论 新生儿代谢病筛查是遗传代谢缺陷病的一种早期诊断和早期防治方法,对检出的CH、PKU进行早期有效治疗,可保持脑和智能发育正常,预防智能性残疾.     

先天性肾上腺皮质增生症21羟化酶缺乏患者的生长与最终身高影响因素

先天性肾上腺皮质增生症(CAH)是一组常染色体隐性遗传病,由于肾上腺皮质激素合成酶的缺陷,皮质醇的合成部分或完全受阻使促肾上腺皮质激素(ACTH)分泌过多导致肾上腺皮质增生,同时皮质醇的前体产物过多堆积并转化为性激素.21羟化酶缺乏(21-OHD)是最常见的CAH,同时也是人类最常见的常染色体隐性遗传病之一,分为经典型...     

Final height in girls with untreated constitutional delay in growth and puberty

During a 10-year period, 23 girls compared to 118 boys presented with constitutional delay in growth and puberty. Of these girls, 15 were followed to final height to determine the outcome of the untreated condition in terms of both growth and psychological well-being. At presentation chronological age was 13.2 (1.7) years [mean (S.D.)], bone age delay 2.7 (0.9) years, standing height standard deviation score (SDS) –3.4 (0.9), and predicted adult height (PAH) SDS –1.3 (0.7) (Tanner-Whitehouse II method). Final height SDS was –1.5 (0.8) measured at 18.9 (2.6) years of age. Mean age at menarche was 15.6 (0.9) years. There was no significant difference between final adult height (FH) and PAH but there was a significant difference between FH and target height (P<0.001). Psychological questionnaires revealed no significant difference in self-esteem, marital or employment status between the patient and control groups. There was no significant correlation between self-esteem and FH but 80% felt their growth delay had affected success either at school, work or socially. Of the patients, 50% would have preferred treatment to advance their growth spurt. This study demonstrates that girls with constitutional delay in growth and puberty reached their PAH, although this was lower than the midparental heights. The girls also experienced significant distress due to delayed growth and puberty and treatment to advance growth should be considered more frequently.     

Menarcheal age and growth pattern of Indian girls adopted in Sweden. II. Catch-up growth and final height

Adopted girls (n=107) previously studied regarding menarcheal age in relation to age at arrival, were analysed as to growth pattern and final height related to nutritional status at arrival and menarcheal age. It was found that most girls had catch-up growth regarding height and half of them regarding weight. Faster catch-up and later arrival age in Sweden were associated with earlier menarche. The catch-up growth was, however, incomplete, and lower the initial height for age, lower was the height for age at the succeeding measurements, and the final height. The mean final height was 154 cm, but 8% of the girls were 145 cm or shorter. The data suggest that linear growth and final height is influenced by the preadoptive nutritional condition, as well as by the degree and timing of subsequent catch-up growth, and the timing of puberty. Pubertal onset is related to the degree and timing of catch-up growth.     

Longitudinal growth and height velocity of Japanese children with Down's syndrome

Aim: To determine the natural growth pattern of Japanese children with Down's syndrome. Methods: Longitudinal height data of 85 patients (43 males, 42 females) from birth to final height were analyzed. Based on these data, semi-longitudinal standard growth curves and height velocity curves for Down's syndrome were drawn. Results: The means ± SD of final height of males and females with Down's syndrome were 153.2 ± 5.6 and 141.9 ± 4.2 cm, respectively. They were -3.0 SD and -2.8 SD for Japanese standards. Mean peak height velocities were 8.9 and 7.5 cm y[Formula: See Text], and the ages at peak height velocity were 11.6 and 10.2 y for males and females, respectively.

Conclusion: The mean height of patients with Down's syndrome was around -2 SD for normal children before puberty. Their pubertal growth spurt starts about 1 y earlier and their peak height velocity was about 1.3-1.4 cm shorter than for normal children.     

早期治疗对先天性甲状腺功能减低症患儿智力及体格发育的影响

目的：比较治疗开始时间不同的先天性甲状腺功能减低症（congenital hypothyroidism, CH）患儿治疗后智力发育、体格发育水平的不同,以寻求改善患儿预后的最佳治疗时间。方法：对2008年9月至2011年9月经新生儿疾病筛查确诊为CH,并在出生后3个月内开始应用甲状腺激素治疗的CH患儿49名,按开始治疗时间分为两组：生后1个月内治疗组（n=26）及1～3个月治疗组（n=23）。分别于6个月、1岁、2岁时,检测两组患儿体格发育情况,应用Gessell 发育量表评估智力发育商（DQ）及采用免疫荧光法测定甲状腺功能。结果：两组经甲状腺激素长期治疗,6个月、1岁、2岁时游离三碘甲状腺原氨酸（FT3）、游离甲状腺素（FT4）及促甲状腺素（TSH）水平差异无统计学意义（P>0.05）,但1个月内治疗组患儿的身长、体重均明显高于1～3个月治疗组,差异有统计学意义（P0.05）。结论：CH开始治疗时间影响患儿智力发育和体格发育;生后1个月内开始治疗者,智力及体格发育优于1～3个月开始治疗者。     

Congenital hypothyroidism: Clinical and laboratory characteristics in infants detected by neonatal screening

The clinical and laboratory characteristics of infants with neonatal hypothyroidism, and the age at which treatment was started are reviewed. The incidence of primary hypothyroidism was 1 in 3488 live births. Most of these cases were detected in a cord blood screening programme which was carried out between October 1973 and May 1980 in the Toronto region. Forty-eight infants with primary persistent hypothyroidism were classified by technetium scintiscanning as follows: 12 with athyrosis (non-visualised thyroid glands), 14 with ectopic thyroid glands, and 14 with goitrous thyroid glands. The remaining 8 infants comprised 4 with hypoplasia and 4 with transient hypothyroidism (2 idiopathic and 2 iodide induced). Although infants with athyrosis had a lower mean thyroxine value, their values overall were not significantly different from those of the other groups. Some infants, particularly those who were goitrous or ectopic, initially had normal thyroxine values. Skeletal maturation was more often delayed in athyrotic infants than in the ectopic or goitrous group. Radioactive iodine uptakes were appreciably higher in the goitrous group, and there was no significant difference between athyrotic and ectopic groups. The mean age at which treatment was started for all patients was 25 (range 6-120) days. The average age for starting treatment using initial thyroxine and secondary thyrotrophin testing in the initial stages of the cord blood screening was 36 days. The use of initial cord serum or dried blood thyrotrophin reduced the start of treatment to 14 days. It is concluded that: (1) It is important to determine the precise anatomical diagnosis, the biochemical severity, and the age at which treatment is started in order to assess the benefits of regional screening programmes in the detection of neonatal hypothyroidism. (2) Routine technetium scintiscanning before the start of treatment helps to determine the diagnosis. (3) Radioactive ¹³¹-I thyroid uptake studies are no longer routinely recommended; this is because of the radiation hazard and the lack of specificity in differentiating between the various anatomical types. (4) The feasibility of cord blood screening to detect the various causes of neonatal hypothyroidism is confirmed. (5) The time between final diagnosis and the institution of treatment can be reduced by 2 or 3 weeks if a thyrotrophin test is used initially from cord blood serum or, preferably, from dried blood spotted on filter paper; the latter is easier to post to a screening laboratory.     

Final height in Swedish children with idiopathic growth hormone deficiency enrolled in KIGS treated optimally with growth hormone

Aim: To assess final height in children with growth hormone deficiency (GHD) treated with human recombinant growth hormone (GH). Methods: Final height data for 401 Swedish children with idiopathic GHD and treated with GH, included in KIGS (Pfizer International Growth Database) between 1987 and spring 2006, were analysed retrospectively. Data were grouped according to sex, age and severity of GHD. Height at entry into KIGS, at the onset of puberty and near final height were analysed between groups. Results: Groups were heterogeneous for GHD, which ranged from partial to severe. For all groups, mean final height corrected for mid‐parental height was within the normal Swedish height range. In patients with severe GHD, mean final height was almost identical to mean normal Swedish height. About 16% of patients showed disproportionality (short legs) at final height and were significantly shorter than other patients. The parents of these children also demonstrated short stature. Conclusion: Children with idiopathic GHD receiving GH replacement therapy can achieve a final height that as a group is within the normal range and all achieve a height within their genetic potential.