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Eric P. Smith
1997 Volume 6 Issue Supple9 Pages
1-6
Published: 1997
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Keinosuke Fujita
1997 Volume 6 Issue Supple9 Pages
7-12
Published: 1997
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Turner girls, women and their families have several social problems at all stages of life. The family usually feel shock at the diagnosis of Turner syndrome. In 1991, we organized a Turner society in Osaka. Several other Turner societies have been established in different areas in Japan. A questionnaire about social problems was sent to the members of the Turner Society. Ninety-four responded, and the response rate was 56%. Half of the parents feel that during infancy the Turner girls were difficult to bring up. One third of the mothers indicated that they were too nervous to bring up the baby. During school life, half of the Turner girls were bullied. In academic performance, two thirds of Turner girls were average or better than average. Many Turner girls said that they disliked mathematics and gymnastics. Information about Turner syndrome is important for Turner girls. Not a few of them were upset when they got the information. Of 10 individuals who had a job, three Turner women had experienced difficulty in finding a job. One third of Turner women above 19 years of age have boy friends. We need to follow them up as to marriage.
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Mari Harada, Susumu Yokoya
1997 Volume 6 Issue Supple9 Pages
13-18
Published: 1997
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Yoshiaki Okada
1997 Volume 6 Issue Supple9 Pages
19-27
Published: 1997
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The quality of life (QOL) of 53 male and 18 female adult GH-deficient (GHD) patients with gonadotropin deficiency (group 2) was studied in comparison with that of 84 male and 33 female adult GHD patients without gonadotropin deficiency (group 1). Both male and female GHD patients advanced to university at a significantly higher ratio than the general population (p<0.05, p<0.01). More than two thirds of male and female patients in both groups lived with their parents and had no girl friend or boy friend. They worked with more satisfaction and got less income than the general population, but no difference between groups 1 and 2 was observed in working status. No unemployed were found among 50 male patients in group 1, but there were 4 aged 24 to 31 years among 50 male patients in group 2. The marriage rate for male patients in group 2 was significantly (p<0.01) lower than that for the general population. More male patients in group 2 than in group 1 lacked selfassertion (p<0.05), were anxious about their body and marriage (p<0.01), and also had complaints suggesting psychosomatic diseases and neurosis (p<0.05), but no difference was observed between female patients in groups 1 and 2.
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David Skuse, Jane Gilmour
1997 Volume 6 Issue Supple9 Pages
29-37
Published: 1997
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The psychosocial adaptation of children with normal short stature is a matter of concern to their parents, paediatricians and teachers. Potential areas of dysfunction include cognition, social behaviour, emotional adjustment and self-concept. We have studied a sample of 22 children who were referred to specialists for investigation of short stature (mean height below -2 standard deviation score (SDS)), aged between 6 to 11 years. We gathered parent, teacher and self-reports. Uniquely, we have also used sociometry to measure those children's social adjustment within their classrooms, as perceived by their peers. A closely matched comparison group of normally growing children was also recruited from the same school classes as the cases. We found little evidence that untreated short prepubertal children are psychosocially maladjusted, compared to peers of normal stature. They do not differ in terms of peer acceptance, self-perception or social competence. Children with short stature regarded themselves as well supported socially by parents, teachers, peers and friends. All subjects had intelligence guotients (IQ) within the normal range of ability, yet we found cognitive ability was a better predictor than height for most aspects of behavioural and emotional adjustment.
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Toshiaki Tanaka, Mari Satoh, Itsuro Hibi
1997 Volume 6 Issue Supple9 Pages
39-44
Published: 1997
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To improve the final height of short children who enter puberty at short stature, twenty-one boys who entered puberty shorter than 130 cm and twelve girls who entered puberty shorter than 125 cm were treated with a combination of GH and LHRH analog. Combined GST and GH treatment was started in boys and girls at a mean age of 11.78 (±0.82) years and 10.54 (±1.08) years, mean height of 130.0 (±4.4) cm and 122.6 (±3.2) cm, and mean height SD of -2.34 (±0.61) SD and -2.78 (±0.99) SD respectively. During the combination treatment, bone age decelerated after a bone age of 11.5 years in boys and 10 years in girls, and height velocity did not decelerate but remained at from 3 cm/year to 5 cm/year for a longer period. As a consequence of the treatment, the height SD score for bone age increased significantly. Seven boys and six girls discontinued LHRH analog treatment when the mean age was 17.11 (±1.15) years after a mean treatment period of 4.96 (±1.37) years for boys and when the mean age was 13.89 (±1.79) years after a mean treatment period of 3.21 (±1.52) years for girls. The mean height was 155.1 (±3.1) cm for boys and 143.3 (±3.0) cm for girls and their predicted adult height was 169.8 (±2.5) cm for boys and 155.8 (±3.1) cm for girls at the cessation of the combination treatment. All except one girl in our study will have achieved greater than the standard height gain during puberty. Although combined GH and LHRH analog treatment can improve the final height of short children by increasing pubertal height gain, the combination treatment seems more effective in boys than in girls.
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Katsuhiko Tachibana
1997 Volume 6 Issue Supple9 Pages
45-48
Published: 1997
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Gonadal suppression therapy is now used experimentally to improve the final height of children who enter puberty at the normal age but with short stature. For this purpose a long-acting analog of LH-releasing hormone (LHRHa) is usually used. To improve the final height, gonadal suppression must be continued for at least several years, and artificial delayed puberty may cause psychosocial problems. The accumulation of bone mineral is reported to be most active during puberty and it is related to the increased secretion of sex steroids. Suppression of sex steroid secretion at the normal pubertal age may result in poor bone mineral accumulation at this critical period and may become a risk factor for osteoporosis later in life. Gonadotropins are knownto be secreted in a pulsatile fashion even at prepubertal age. LHRHa abolishes gonadotropin secretion completely and may deteriorate pubertal development after completion of the therapy. There are reports of IgE antibody production and anaphylaxis caused by LHRHa treatment. If an antibody that cross-reacts with native LHRH is generated, it can be a cause of hypogonadism. We should take into account these possible disadvantages when giving gonadal suppression therapy to patients with basically normal puberty.
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Katsumi Ito, Hiroshi Kawaguchi
1997 Volume 6 Issue Supple9 Pages
49-53
Published: 1997
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We evaluated the 1 year effect of recombinant human GH (rhGH), of 0.5 or 1.0 IU/kg/week, on growth in 83 cases of chronic renal failure (CRF) (pre and dialysis), 23 transplanted and 18 children with nephrotic syndrome. In predialysis children height growth velocity (HV) became to be significantly increased from 3.8 at baseline to 7.8 at 6 months and 7.3 cm/year at 1 year with a dose of 0.5 IU (p<0.001). When treated with 1.0 IU, HV was increased from 4.5 to 10.3 at 6 and 8.7 cm/yaer at 1 year (P<0.001). When a comparison was done for the increase in HV, a significant difference was noted between 0.5 IU and 1.0 IU at both 6 and 12 months (P<0.01). In dialyzed children treated with 0.5 IU, HV was significantly increased from 3.5 to 5.6 at 6 months (P<0.01) and 5.4 cm/year at 1 year (P<0.01). In the 1.0 IU group, it was from 3.4 to 8.4 at 6 months (P<0.001) and 8.3 cm/year at 1 year (P<0.01). There was a significant difference in HV between 0.5 and 1.0 IU at both 6 and 12 months (P<0.01). In transplanted children, HV was significantly improved from 5.0 to 7.7 cm/year with 0.5 IU (P<0.05) and from 3.7 to 6.3 cm/year with 1.0 IU (P<0.05) during 1 year of GH treatment but there was no statistical difference between the two groups. Seven out of 23 children showed deterioration of graft function during rhGH treatment due to acute rejection (AR) in all cases. AR was noted in 2 out of 10 children (20%) in the 0.5 IU and 5 out of 13 children (38%) in the 1.0 IU group. In nephrtotic syndrome, HV was increased from 3.3 to 6.5 cm/year at 1 year and to 6.4 cm/year at 2 year (P<0.05). Our results demonstrated that rhGH is effective to stimulating HV in not only CRF but also transplants and nephrotic syndome.
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Franz Schaefer, Dieter Haffner, Elke Wühl, Burkhard Tönshoff ...
1997 Volume 6 Issue Supple9 Pages
55-58
Published: 1997
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Gen Nishimura
1997 Volume 6 Issue Supple9 Pages
59-69
Published: 1997
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“Bone dysplasia family”refers to a group of disorders which share qualitatively similar skeletal alterations and a conceivably common pathogenesis. This concept was first described by Spranger, and has provided a practical guide for the differential diagnosis of bone dysplasias. This idea has since become a pilotage for molecular investigation on this field; for instance, the discovery of heterozygous mutations of the FGFR3 gene in achondroplasia led to the elucidation of other heterozygous mutations of the FGFR3 gene in thanatophoric dysplasia and hypochondroplasia, which had been classed as allelic disorders in the“achondroplasia family”solely based on clinical and radiologic grounds. Understanding the bone dysplasia family concept can facilitate further nosologic and molecular investigations in this molecular era, which will ultimately provide information beneficial to the management of affected individuals. Common“bone dysplasia families”are outlined here, particularly from the radiological standpoint. The importance of phenotype-genotype correlation is discussed by exemplifying the radiologic findings in achondroplastic individuals with uncommon mutations of the FGFR3 gene and radiologic variabilities in a severe form of type II collagenopathy.
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Hirohiko Sano, Yoshitoshi Yamamato, Kouichi Shibazaki, Masafumi Wada
1997 Volume 6 Issue Supple9 Pages
71-72
Published: 1997
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Growth disorders induced by long term steroid hormone treatment are well known. We present here three cases of nephrotic syndrome treated with GH for severe short stature induced by long term steroid hormone therapy. Steroid hormone on alternate days (30 mg of predonisolone or 24 mg of methylpredonisolone) simultaneously for the treatment of three patients with nephrotic syndrome was necessary. Height growth before the beginning of the GH therapy was 1.5 cm, 3.0 cm and 2.6 cm per year. Height growth one year after the beginning of the GH therapy was 6.7 cm, 7.6 cm and 7.4 cm per year, respectively. Our data suggest that GH therapy is effective and safe for some cases of severe short stature patient with nephrotic syndrome.
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Shuji Abe, Nozomi Shinohara, Jun Nakae, Toshihiro Tajima, Mari Murashi ...
1997 Volume 6 Issue Supple9 Pages
73-76
Published: 1997
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Osteogenesis imperfecta (OI) is a heterogeneous inheritable disorder of the connective tissue with bone fragility. Growth deficiency and bone fracture are cardinal features of OI. We evaluated the effect of hGH treatment on growth promotion, final height and bone fracture in a girl with type I OI. At the age of 10 years, when she was 114.1 cm (-3.4 SD) tall, hGH treatment was started because the standard criteria of GH deficiency were satisfied. No episode of bone fracture had been noted prior to GH treatment, but she suffered bone fractures three times in the first two years' treatment for GH. Because of this, salmon calcitonin was added from the age of 12 years, but she suffered from bone fractures during the further GH treatment. GH treatment was terminated at the age of 17 years. Since being taken off GH therapy, one bone fracture has occurred. Her height ended up at 147.0 cm (-2.2 SD) at the age of 18 years. These findings suggest that GH treatment in a patient with type I OI seems to be effective in promoting growth, but increased frequency of bone fractures during GH treatment warrants careful selection of a patient who merits GH therapy.
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Masayoshi Sato, Tadashi Moriwake, Hiroyuki Tanaka, Taeko Ono, Hisashi ...
1997 Volume 6 Issue Supple9 Pages
77-80
Published: 1997
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GH stimulates bone growth, but the effect of GH on bone mass in childhood remains unclear. In this study, we analyzed the effect of GH on bone mass during childhood by using dual energy X-ray absorptiometry (DXA). Seventy-two males and thirty-eight females with GH deficiency were investigated. Bone mineral density (BMD), bone mineral content (BMC) and the projected area of the lumbar spine (L2-4) were measured by DXA before (B), after one year (1T) and after two years (2T) of GH therapy. Volumetric BMD (vBMD) was calculated by using a cylindrical vertebral model. Both BMC and area increased during GH therapy both in male and female subjects, but the L2-4BMDZ scores did not show a significant change in either sex after two years of therapy (males: Zscore B: -0.782 ± 0.698 2T: -0.927 ± 0.785, females: B: -0.782 ± 0.698 2T: -0.927 ± 0.785). Since BMD is affected by bone size, the assessment of bone mass during growth periods required volume-adjusted data. The absolute vBMD value also stayed unchanged both in male and female subjects. Therefore GH stimulated bone growth with an appropriate increase in bone mass in GH deficient patients during childhood.
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Tomoyo Ohyama, Makoto Sato, Hidemi Ohye, Shuji Matsubara, Jiro Takahar ...
1997 Volume 6 Issue Supple9 Pages
81-83
Published: 1997
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Tetsuro Tamura, Ryuichi Tanaka, Hitoshi Takahashi, Kenzo Kaneko
1997 Volume 6 Issue Supple9 Pages
85-86
Published: 1997
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Yasuji Inamo
1997 Volume 6 Issue Supple9 Pages
87-89
Published: 1997
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GH has some influence on cardiovascular risk factors. The effects of long-term GH replacement therapy on hemostatic and lipid parameters were examined in pediatric patients with GH deficiency. Hemostatic markers (fibrinogen, D-dimer, TAT, tPA-PAI, and PIC) and lipid parameters (total cholesterol, HDL-cholesterol, LDL-cholesterol, and Lp (a)) were evaluated in 30 pediatric patients before and during GH therapy. They were treated with recombinant GH (0.5 I.U. /kg/week) for 2.4±0.5 years. Eight patients had complete GH deficiency (aged 10.6±3.3 years; 5 males and 3 females), including 4 with craniopharyngioma (13.7±5.4 years; 2 males and 2 females), and 22 had partial GH deficiency (10.6±3.3 years; 11 males and 11 females). Statistical analysis was done with the Mann-Whitney nonparametric rank sum test. Plasma D-dimer (p<0.01) and fibrinogen (p<0.01) decreased after 2.4±0.5 years of GH treatment. There were no significant changes in total cholesterol, triglycerides, or HDL-and LDL-cholesterol, but there was an increase in lipoprotein (a) (p<0.02) and insulin (p<0.02). The free T
3 (p<0.0003) and TSH (p<0.003) levels decreased, but there was no effect on free T
4or the body mass index. Decreased hemostatic activity is a beneficial effect of GH on cardiovascular risk factors. Ceasing GH therapy by 20 years of age on completion of growth might lead to the onset of cardiovascular disease in adult life, so continuing GH therapy beyond this age may be necessary.
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Yuko Nakayama, Osamu Arisaka
1997 Volume 6 Issue Supple9 Pages
91-93
Published: 1997
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Atsuko Nimura, Shoko Ikema, Masahiro Goto, Mika Ishikawa, Sachiko Kita ...
1997 Volume 6 Issue Supple9 Pages
95-98
Published: 1997
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The bone mineral density (BMD) of the 2nd metacarpal bone of the left hand was measured in 14 children with complete GH deficiency (CGHD) and 21 children with partial GH deficiency (PGHD) by the digital image processing (DIP) method. In the pretreatment patients, mean BMD in CGHD was significantly lower than that in normal children. The BMD standard deviation (SD) score in the patients with CGHD who had started the GH treatment before 10 years of age increased by approximately 1.5 SD during GH treatment, while that in the patients with PGHD did not change significantly. Since BMD did not show a pubertal increase in the patients who received combined GH and LHRH analog treatment, their BMD SD score decreased by approximately 1.5 SD. The data demonstrate that GH influences BMD, and the level of BMD in patients with GHD should be followed in a longitudinal observation.
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Makoto Yamada, Katsura Fujii, Tomonobu Hasegawa, Yukihiro Hasegawa
1997 Volume 6 Issue Supple9 Pages
99-101
Published: 1997
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Acid-labile subunit (ALS) is one of the components of a ternary complex with insulin-like growth factor-I (IGF-I) and IGF binding protein-3 (IGFBP-3) in the circulation. The role of ALS in the circulation is not well known, but the levels of ALS are dependent on GH levels, suggesting that ALS plays an important biological role in the circulation. This study is to research whether serum ALS levels are suitable parameters for the diagnosis of GH deficiency (GHD). In this study, ALS levels were measured by Western immunoblotting in sera from patients with GHD and hypophysectomised (hypox) rats. ALS levels of the patients with GHD were less than 10% of ALS levels of control agematched children and adults. ALS levels of hypox rats were less than 10% of ALS levels of control (non-hypox) rats. The levels were partially reverted towards normal levels after rat GH treatment. Our data suggest that ALS levels are one of the best parameters for the diagnosis of GHD.
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Tateo Kuno, Hideto Yamaguchi, Sumio Miyazaki
1997 Volume 6 Issue Supple9 Pages
103-105
Published: 1997
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Mika Ishikawa, Shoko Ikema, Masahiro Goto, Reiko Horikawa, Toshiaki Ta ...
1997 Volume 6 Issue Supple9 Pages
107-109
Published: 1997
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Tomohiro Someya, Tomoyuki Watanabe, Toshiyuki Yasuda, Hiroo Niimi
1997 Volume 6 Issue Supple9 Pages
111-113
Published: 1997
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Yasuhiro Ono, Michiyo Yamamoto, Takashi Kamijo, Toshi Asai, Masamichi ...
1997 Volume 6 Issue Supple9 Pages
115-116
Published: 1997
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Satoshi Takakuwa, Noboru Igarashi
1997 Volume 6 Issue Supple9 Pages
117-119
Published: 1997
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Michitoshi Sekine, Hitoshi Seki, Youichi Hara, Hiroshi Noguchi
1997 Volume 6 Issue Supple9 Pages
121-124
Published: 1997
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Tomohiro Sasajima, Tomoyuki Hotsubo, Hideshi Tomita, Kei Numazaki, Shu ...
1997 Volume 6 Issue Supple9 Pages
125-128
Published: 1997
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We saw a case of precocious puberty in a body with Down syndrome, during treatment for autoirnmune hypothyroidism caused by interferon therapy for chronic Epstein-Barr virus infection. One year after the start of l-thyroxine supplementation, his somatic growth spurt and penile development started. His growth rate was fairly high for a patient with Down syndrome who generally have a slow pubertal growth spurt. Serum gonadotropins and testosterone were at the pubertal levels of boys of normal karyotype. We thought it better not to suppress his puberty, because it seemed that his growth rate may be reduced too much by lowered testosterone and the pubertal spurt may not recover fully after cessation of the gonadal suppression therapy. He has already gained 25cm since the start of the growth spurt and still continues to grow at the rate of 9 cm a year without any treatment.
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Tadashi Nishioka, Kazuki Sato, Hidee Arai, Hidefumi Sudo, Takashi Itab ...
1997 Volume 6 Issue Supple9 Pages
129-131
Published: 1997
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