Biomedical subjects

J Lebl

Publications and source records attributed to J Lebl.

At least 37 records · Page 2Linked to original sources

PubMed2001

Lessons from 30 years of clinical diagnosis and treatment of congenital adrenal hyperplasia in five middle European countries.

Despite the fact that congenital adrenal hyperplasia (CAH) is one of the most common inborn endocrine disorders, some patients are not identified, or may even die, in an acute salt-losing crisis. In a retrospective study covering the last 30 yr, we examined the time elapsing before diagnosis of CAH patients, in 5 Middle European countries, and the mortality rate in diagnosed patients and their siblings during childhood; we also attempted to estimate how many patients are not diagnosed clinically each year. Basic and follow-up clinical data and the family histories of 484 patients with classical forms of CAH diagnosed between 1969 and 1998 were collected and recorded in 5 Middle European countries. The sex-ratio, time elapsing before diagnosis, and mortality among siblings and patients were calculated, and the number of undiagnosed patients was estimated. We found significantly fewer genetic males (43.0%) than females (57.0%) among 484 classic CAH patients, and the percentage of diagnosed boys did not increase with time; 64.7% of them suffered from the salt-wasting (SW) form, and 35.3% from the simple virilizing (SV) form, of the disease. The diagnosis of CAH was established significantly later in males than in females in both forms [SW: 26 vs. 13 days (median), P < 0.0001; SV: 5.0 vs. 2.8 yr, P = 0.03]. Infant mortality in the general population was significantly lower than in either siblings (1.8% vs. 7.0%; P < 0.0001) or in SW (2.29% vs. 11.3%; P < 0.0001). According to our calculations, by our current praxis of clinical ascertainment, 2-2.5 SW and up to 5 SV stay undiagnosed, out of 40 expected CAH patients per year in the countries investigated. Both clinical detection and treatment of CAH patients, at least in males, were insufficient in the five Middle European countries examined during the last 30 yr. Neonatal mass screening and/or greater awareness of the medical community are discussed as ways of improving the efficacy of CAH management. Our experience may be applicable to other countries with similar health care systems.

Adrenal Hyperplasia, Congenital↗

PubMed2001

IGF-I resistance and Turner's syndrome.

The pathogenesis of growth failure in Turner's syndrome is not clear but might be attributed to a decreased sensitivity to insulin-like growth factor-I (IGF-I) in distinct cell lines or to its reduced autocrine/paracrine action. Growth hormone (GH) therapy leads to increments in IGF-I levels and to growth acceleration. In order to evaluate the pattern of overcoming IGF-I resistance through childhood and adolescence, we measured IGF-I in 78 girls with Turner's syndrome aged 4.6-18.3 years on 160 occasions without or during GH (1 IU/kg/week [0.33 mg/kg/week]) or GH+estradiol (E2) therapy and compared them with local IGF-I standards. In untreated patients, IGF-I levels were low normal (-0.71+/-0.18 SDS, mean +/- SEM). In both GH or GH+E2 treated girls, circulating IGF-I levels were persistently supraphysiological (GH only: +3.61+/-0.23 SDS; GH + estradiol: +3.18+/-0.31 SDS). The age-dependent pattern of IGF-I secretion was conserved but the pubertal increase occurred earlier. The highest standardized IGF-I levels were observed at age 8.5-9.4 years (+6.62+/-1.00 SDS) and 9.5-10.4 years (+5.61+/-1.03 SDS). GH+E2 substitution had no additional effect on circulating IGF-I. We conclude that high IGF-I levels are needed to overcome the IGF-resistance in Turner's syndrome. They reflect the action of GH therapy but not of estrogens. The earlier pubertal increase of IGF-I might be caused by exaggerated adrenal androgens.

Adolescent↗

PubMed2001

Sex hormone-binding globulin as a marker of the effect of hormonal treatment in Turner's syndrome.

OBJECTIVE: In girls with Turner's syndrome a positive effect of growth hormone (GH) therapy on statutal growth has been well documented, in spite of the fact that the levels of GH and IGF-I in these patients are usually within the normal range. It is known that plasma levels of sex hormone-binding globulin (SHBG) are negatively associated with growth hormone (GH) and with [GF-I. Limited data on SHBG levels in these patients and only scarce reports on other than gonadal steroids in these patients are available so far. The major aim of this study was to find out whether SHBG does respond to GH treatment. METHODS: Plasma levels of SHBG, estradiol, progesterone, testosterone and the adrenal steroids and their precursors, namely cortisol, 17alpha-hydroxyprogesterone, androstenedione, dehydroepiandrosterone and its sulfate were determined in 65 patients aged 2-23 years with Turner's syndrome. The patients were divided into 4 groups according to their actual treatment (untreated, sex steroids only, GH only, GH and sex steroids) and the differences between the groups were evaluated. With respect to dependence of SHBG and of steroids studied on age (with exception of cortisol), their values were expressed as per cents of medians of the physiological values. RESULTS: As expected, low levels of sex steroids were found. As to the adrenal steroids, in general, their average levels were lower than in healthy subjects but still within the physiological limits. With one exception (androstenedione), no significant differences were found between the groups. The levels of cortisol were undistinguishable from healthy subjects. Absolute as well as relative SHBG levels in untreated group and in the group treated with estrogens were normal or even lower than those reported for normal subjects of the same chronological age. Significantly reduced absolute as well as relative SHBG levels in comparison to all other groups were found in GH treated children. CONCLUSION: The results indicate that SHBG may serve as a sensitive biochemical marker of the response to the GH administration.

17-alpha-Hydroxyprogesterone↗

PubMed2001

Insulin-like growth factor-I and insulin-like growth factor-binding protein-3 in cystic fibrosis: a positive effect of antibiotic therapy and hyperalimentation.

UNLABELLED: Patients with cystic fibrosis (CF) are underweight and growth retarded. This study tested the link between serum insulin-like growth factor-I (IGF-I) and insulin-like growth factor-binding protein-3 (IGFBP-3) levels and body height, nutritional status, pulmonary function tests and activity of inflammation in 92 subjects with CF (age 2.1-18.8 y). It also analysed the effect of short-term antibiotic treatment and hyperalimentation on IGF-I and IGFBP-3 levels in 33 subjects (age 3.6-33.7y) on 41 occasions. Both IGF-I (-1.19 +/- 0.17 SD) and IGFBP-3 levels (-0.66 +/- 0.12 SD; both p < 0.0001 vs 0) were decreased in cross-sectional measurements. Their standardized values were inversely proportional to age (IGF-I: r = -0.23, p = 0.03; IGFBP-3: r = -0.29, p = 0.005) and positively correlated with SDS of height (IGF-I: r = 0.40, p < 0.0001; IGFBP-3: r = 0.36, p = 0.0005) and of mid-arm circumference (IGF-I: r = 0.39, p = 0.0001; IGFBP-3: r = 0.38, p = 0.0002), and with pulmonary function tests. After a short-term course of intensive antibiotic therapy and hyperalimentation, IGF-I normalized (from -0.66 +/- 0.20 to 0.00 +/- 0.25 SD; p < 0.0001) and IGFBP-3 increased (from -0.78 +/- 0.15 to -0.53 +/- 0.16 SD; p = 0.002). IGFBP-3 correlated inversely with erythrocyte sedimentation rate (r = -0.40, p = 0.01). CONCLUSION: The levels of IGF-I and IGFBP-3 are markedly decreased in patients with CF and tend to normalise after a short course of antibiotic treatment and hyperalimentation.

Adolescent↗

PubMed2000

[Adult height of patients after long-term treatment of idiopathic growth hormone deficiency. (Czech Registry 1991-1998)].

BACKGROUND: Final height was evaluated in patients from the Czech Register with idiopathic growth hormone deficiency treated with GH. METHODS AND RESULTS: 23 patients had the isolated growth hormone deficiency (group 1), and 37 suffered from multiple pituitary hormones deficiencies (group 2). The patients from group 1 and 2 were given growth hormone for 6.7 +/- 2.2 years and 9.6 +/- 3.0 years, respectively. The patients with isolated growth hormone deficiency reached final height -1.4 +/- 1.3 SDS, those with multiple hormone deficiencies were taller (-0.7 +/- 1.5 SDS). The height gain equalled +2.5 +/- 0.6 SDS and +3.4 +/- 1.1 SDS in group 1 and 2, respectively. 70% of the patients in group 1 and 93% of group 2 reached final heights within the target limits (+/- 2 SDS). CONCLUSION: The final height positively correlated with the target height (mid-parental height) and height at the onset of puberty (group 1 and 2). There was a negative correlation between the final height and the chronological age at the beginning of growth hormone therapy in group 1.

Adult↗

PubMed2000

Longitudinal growth in HIV-negative boys with haemophilia.

UNLABELLED: It has been shown that HIV-positive haemophilic children develop growth retardation. As not only the HIV infection but also other disease-related factors might compromise growth in these children, growth data were analysed in a longitudinal cross-sectional manner in 84 HIV-negative haemophilic patients from two university clinics. A total of 2-24 height and weight measurements (median 6) were recorded in each patient resulting in 683 single values collected between 1977-1995. Height SDS of all haemophilic boys was -0.31 +/- 2.13 (mean +/- SD, NS versus 0) and body mass index SDS was 0.21 +/- 3.49 (mean SD, NS versus 0) at first measurement and remained unchanged throughout the observation period. Neither height nor body mass index differed with respect to the severity of haemophilia (mild/moderate/severe) or the study centre (Vienna/Prague). CONCLUSION: Growth in HIV-negative patients with haemophilia is not affected in spite of the immunological abnormalities attributed to the substitution therapy or the bleeding episodes in the joints with the potential effect on the growth plate.

Adolescent↗

PubMed2000

Immune system in adults with childhood-onset growth hormone deficiency: effect of growth hormone therapy.

OBJECTIVE: To investigate the impact of growth hormone (GH) therapy in adults with childhood-onset GH deficiency on immune system. METHODS: Ten young GH deficient adults (7 males, age 19-28 years) were treated with recombinant human growth hormone for 6 months. The starting dose was 0.5 IU/m2/day (2 weeks), then it was doubled to 1.0 IU/m2/day. In 5/10 patients, the dose was further increased to 1.5 IU/m2/day at 4 weeks of therapy. Immunological studies were performed before treatment and after 6 weeks, 3 months and 6 months and included humoral (IgG, IgA, IgM, C3, C4 and immune complexes) and cellular parameters (total lymphocyte count and counts of CD3+, CD4+, CD8+ and CD19+ lymphocytes, the CD4+/CD8+ ratio and percentage of CD16+56+ and CD3+DR+). RESULTS: The cellular responses to GH therapy were subtle, but detectable, with the trend to the higher CD4+ and lower CD8+ lymphocytes and maximal changes at 6 months of therapy. They were reflected in CD4/CD8 ratio, which increased from 1.15 +/- 0.10 (mean +/- S.E.; baseline) to 1.37 +/- 0.11 (6 weeks; P < 0.05), 1.24 +/- 0.10 (3 months; n.s.) and to 1.59 +/- 0.20 (6 months; P < 0.05). The response in humoral immunity was characterized by a rapid decrease of circulating immunoglobulins (IgA: 1.40 +/- 0.25 g/l [mean+/-S.E.], baseline; 1.12 +/- 0.19, at 6 weeks; P < 0.05) and C4 (0.25 +/- 0.02 g/l, baseline; 0.19 +/- 0.01, at 6 weeks; P < 0.05) and a tendency to an increase in circulating immune complexes (29.1 +/- 8.1, baseline; 40.3 +/- 7.2, at 6 weeks; n.s.). These observations suggest a temporary immune complex formation after the onset of GH treatment which might play a partial role in developing oedema as a side effect of GH treatment, besides the known effect of GH on water retention. CONCLUSIONS: GH therapy in GH deficient young adults has a measurable effect on the increase of CD4/CD8 ratio and on the formation of immune complexes.

Adult↗

PubMed1999

Premenarchal and postmenarchal girls with insulin-dependent diabetes mellitus: ovarian and other organ-specific autoantibodies, menstrual cycle.

STUDY OBJECTIVE: To estimate various organ-specific autoantibodies and detect other endocrine autoimmune disorders and menstrual cycle characteristics in girls with Type 1 insulin dependent diabetes mellitus (IDDM). DESIGN: Prospective cohort study from 1993 to 1998, duration 4.5 years. SETTING: Diabetes & Endocrine Clinic of the University Hospital, Motol, Prague. PATIENTS: 53 IDDM girls (group A--43 postmenarchal, group B--10 premenarchal), 15.5 +/- 2.5 (8-19) years old, 6.2 +/- 4.3 years after IDDM onset. MAIN OUTCOME MEASURES: Ovarian autoantibodies directed to ooplasm, zona pellucida, membrana granulosa, theca folliculi interna, and lutein cells, insulin autoantibodies, thyroid peroxidase and thyroglobulin autoantibodies. Menstrual cycle character, endocrine glands disturbance. Diabetes control, body mass index, duration of IDDM. RESULTS: Ovarian autoantibodies in at least one of the followed structures were found in 67.9% of the IDDM girls. In the control group of 21 healthy girls of corresponding age, the positive findings in lutein cells were found in only 4.8% of the girls (P < 0.01 versus IDDM girls). The lutein cells commonly associated with theca folliculi interna cells were the most frequent immunopositive structures in diabetic girls (P < 0.05 versus another positive ovarian autoimmune structure). Autoantibodies directed to ovarian steroid producing cells were frequent in IDDM patients with both irregular and normal menstrual cycles. Irregular menstrual cycles were diagnosed in 27.9% of IDDM girls, polymenorrhea in half of them, and oligomenorrhea in the remainder. Diabetes control in our patients (glycosylated hemoglobin HbA1c in postmenarchal girls 10.1 +/- 2.0%) did not differ between those with regular and those with irregular menstrual cycles. Over a follow-up period one-third of the girls with oligomenorrhea and a long-term noncompliance (HbA1c 13.5%) developed secondary amenorrhea. Insulin autoantibodies were found in 67.8%, thyroid peroxidase autoantibodies in 12.5%, and thyroglobulin autoantibodies in 10.4% of the IDDM girls. Autoimmune thyroiditis was diagnosed in 5 IDDM patients (9.4%); hypothyroidism developed in 3 of them. Menstrual cycle was irregular in 4 of the 5 girls with autoimmune thyroiditis (polymenorrhea in 1, oligomenorrhea in another 3 girls). CONCLUSIONS: An increased incidence of various circulating autoantibodies may be markedly demonstrated in IDDM girls. Their reproductive function might have an important relationship to an evidence of ovarian autoantibodies. Menstrual cycle disturbances could be linked to the poor diabetes control, to the presence of ovarian and other autoantibodies, and also to other autoimmune disease.

Adolescent↗

PubMed1999

Lymphocyte subsets in renal transplant recipients during growth hormone therapy.

OBJECTIVE: To evaluate the effect of growth hormone treatment on growth, levels of insulin-like growth factor I (IGF-I) and lymphocyte subsets in immunosuppressed renal allograft recipients. METHODS: 18 children (aged 8.0-16.6 years) received growth hormone 1 IU/Kg/week daily for two years. Height, IGF-I levels and in 11/18 patients, lymphocyte subsets were evaluated serially. RESULTS: Standardized growth velocity increased from -1.0+1.5 to +1.2+2.2 and standardized IGF-I levels from +0.8+1.5 to +3.1+1.1 (1 year) and to +1.4+1.7 (2 years). The total lymphocyte count and the number of T lymphocytes (CD3+) decreased. The decrease was more marked in CD8+ (from 1.5+0.3 x10(9)/L to 0.9+0.3 x10(9)/L, 1 year and to 0.8+0.1 x10(9)/L, 2 years) compared to CD4+ (from 1.5+0.3 x10(9)/L to 1.0+0.2 x10(9)/L, 1 year and to 1.3+0.2 x10(9)/L, 2 years), resulting in an increment of the CD4+/CD8+ index. CONCLUSIONS: The differential effect of growth hormone treatment on CD4+ and CD8+ lymphocytes might be explained by different expression of the IGF-I receptor in these distinct subsets.

Adolescent↗

PubMed1999

[Effect of estrogens on final body height in girls with a constitutionally tall stature--risk or benefit?].

OBJECTIVE: Investigation of the effect of a long-term oestrogen treatment on the growth and development of children, elaboration of a theoretical model for remaining growth of girls on a long-term oestrogen therapy predicting reduction of the final height in girls with constitutionally tall stature, analysis of early and late side-effects of therapy. TYPE OF STUDY: Open clinical study. NAME AND PLACE OF DEPARTMENT: Endocrine out-patient clinic of the Second Paediatric Department and gynaecological out-patient clinic for children and adolescents of the Gynaecological-Obstetric Department, Second Medical School--Charles University and University Hospital Prague--Motol. METHOD: Elaboration of theoretical model for remaining growth of girls on a long-term oestrogen therapy was based on an analysis of the remaining growth of upper and lower body segment of healthy Czech girls. RESULTS: The extent of assumed reduction of the final height in girls with constitutionally tall stature treated with oestrogen declines with advancing age at the onset of treatment. The optimal age for the onset of treatment is between 10 and 11 years. CONCLUSION: Treatment can be recommended only in girls with growth prediction above 185 cm and serious psychological difficulties resulting from excessive height, always after careful analysis of the biological development and growth prediction. In every case it is necessary to evaluate the possible benefit of treatment in relation to potential risks. The greatest problem is according to the authors late onset and overrating of the therapeutic possibilities.

Adolescent↗

PubMed1998

Body composition in children receiving recombinant human growth hormone after renal transplantation.

BACKGROUND: Recombinant human growth hormone (rhGH) is an anabolic hormone promoting protein synthesis in various tissues. Therefore, changes in body composition may be expected during rhGH treatment. METHODS: We studied changes in body composition during two years of rhGH treatment in 21 children after at least one year with a functioning renal transplant. The mean +/- SD age was 12.9+/-2.5 years at the start of rhGH therapy. A whole body, dual energy X-ray absorptiometry (DEXA) exam was performed before the initiation of rhGH therapy (T0), and was repeated at one and two year intervals after initiation of the therapy (T1 and T2, respectively). RESULTS: Lean body mass increased by a median of 0.48 SDS during the first year of treatment (P = 0.022), and the median increase during two years of therapy was 0.36 SDS (P = 0.061). On the contrary, the median fat body mass decreased by 2.17 SDS during the T0 to T1 period (P = 0.04) and by 1.99 SDS during the T0 to T2 period (P = 0.055). The index for fat body mass/lean body mass (FBM/LBM) decreased by a median of 5.3% during T0 to T1 (P < 0.001), however, a slower but still significant decrease by a median of 4.2% was noted at T2 (P < 0.05). Bone mass content did not change significantly during rhGH treatment. The medians in caloric and protein intakes were stable during rhGH treatment. CONCLUSION: A significant increase of lean body mass and a decrease of fat body mass was noted during rhGH therapy in children after renal transplantation.

Adolescent↗

PubMed1997

[Late sequelae of comprehensive antineoplastic therapy in children and adolescents with solid extracranial tumors. Effect on growth, pubertal development and gonadal function].

BACKGROUND: Modern treatment of oncological diseases increases markedly the chance of long-term survival and permanent recovery. Due to frequently highly aggressive treatment it is however associated with the risk of late sequelae in the surviving patients. Comprehensive care of patients includes therefore not only control of the neoplastic disease but also efforts of maximal improvement of the quality of life of the patients. In young subjects, in view of their long-term perspective, this problem is particularly important. METHODS AND RESULTS: In 32 patients (25 boys and 7 girls) with extracranial solid tumours without primary endocrinological symptomatology (m. Hodgkin, neuroblastoma, ganglioneuroblastoma, nephroblastoma, Ewings sarcoma and others) a single examination was made assessing height, body weight, grade of sexual maturation according to Tanner, in boys testicular volume by means of a orchidometer and 20 other anthropometric dimensions. The mean age at the time of examination was 16.5 +/- 4.1 years, the mean age at the onset of treatment 6.1 +/- 4.8 years. The patients height, -0.4 +/- 0.9 SD, differs from the Czech national standard (p = 0.025). Impaired growth was recorded in 12.5% patients and had heterogenous causes. The authors proved a negative effect of radiotherapy on the growth of the spine, most markedly in children subjected to irradiation of the abdomen and chest and a highly significant reduction of the testicular volume in boys after cytostatic treatment of m. Hodgkin. CONCLUSIONS: The results are consistent with studies made abroad and indicate the necessity of comprehensive long-term follow-up of somatic growth and development of the gonads in oncological child patients.

Adolescent↗

PubMed1997

GH and TSH deficiency.

Hypothyroidism is a recognised complication of GH therapy in GH deficient children. The mechanisms involved include direct effects on thyroid function but also result from the close interrelationship of pituitary cell-lines that differentiate during embryonic development of the anterior pituitary gland. Among numerous pituitary transcriptionfactors that orchestrate pituitary organogenesis Pit-1 was the first to be recognised and is the most extensively studied. Mutations in the Pit-1 gene account for a form of combined pituitary hormone deficiency for GH, Prolactin (Prl) and TSH (CPHD). Despite the variability of the clinical presentation of this syndrome at the time of initial diagnosis, all forms finally result in severe retardation of growth and development due to GH-deficiency and hypothyroidism. More than half of the families with a combined pituitary hormone deficiency have not disclosed any Pit-1 abnormalities. Evidence is accumulating that Prop-1, a transcriptionfactor expressed temporarily in the fetal anterior pituitary, could be a candidate for patients with a Pit-1 phenotype without any Pit-1 gene abnormalities.

DNA-Binding Proteins↗

PubMed1996

[Growth in children with the exudative enteropathy syndrome due to a congenital heart defect--cor triatriatum dextrum].

Retarded growth in a child can be the sign of serious chronic disease. The authors present an account of a six-year-old boy where growth retardation persisted at least from the age of three. During this period his height dropped from the zone between the 25th and 50th percentile into the zone between the 3rd and 10th percentile. From the clinical point of view a large abdomen, loose stools and hypocalcaemia with tetany were striking, as they were moreover refractory to vitamin D2, calcitriol and calcium administration by the oral route. The authors revealed severe hypoproteinaemia, a 150 times increased value of alpha-1-antitrypsin in faeces, and exudative enteropathy syndrome was diagnosed. The cause was venous congestion due to a rare heart disease--cor triatriatum dextrum. The septum in the right atrium was resected. Immediately after surgery the consistency and frequency of stool decreased. Calcaemia and plasma protein levels reached normal levels within two months. A growth spurt of 11 cm/year followed. Fifteen months after operation the patient's height reached almost the 50th percentile.

Child↗

PubMed1995

[Growth hormone therapy in prepubertal children with chronic renal insufficiency].

BACKGROUND: Growth retardation is a serious problem in children with chronic renal failure (CRF) despite normal endogenous growth hormone (GH) secretion. Intensive medical care and dialysis do not improve height velocity. The aim of the study was to evaluate the efficacy of GH therapy in children with growth retardation secondary to CRF. METHODS AND RESULTS: Biosynthetic growth hormone was given to 7 prepubertal children (five boys and 2 girls, age with a range 3.5-14.5 years) with severe growth retardation and CRF during 1 year. The dosage of GH was 1 IU/kg/per week. GH was given daily, sc. The patients had a full examination every 3 months. Six children completed the study. Height velocity improved with GH therapy in 5 children. Renal function deterioration was accelerated in 2 children. The serum alkaline phosphatase concentration increased and the serum calcium concentration decreased during GH treatment in all children. Glucose, thyroid gland hormone concentration and lipid concentrations remained constant. CONCLUSIONS: The study showed clearly that treatment with biosynthetic GH gave an impressive improvement of height velocity in growth retarded prepubertal children with CRF.

Adolescent↗

PubMed1995

[Growth and adult height in patients with congenital adrenal hyperplasia].

BACKGROUND: The growth of children with congenital adrenal hyperplasia (CAH) can be impaired by overproduction of endogenous androgens which after the initial growth acceleration lead to early ossification of the growth plates and a low adult height, and by excessive glucocorticoid treatment as the latter antagonize the effect of growth hormone and cause growth retardation. In order to identify the life period decisive for the adult height of patients with CAH the authors analyzed retrospectively their growth data. METHODS AND RESULTS: In 35 children with 21-hydroxylase deficiency (24 girls, age 4.8 - 22.3 years and 11 boys, age 6.3 - 22.3 years) the height and bone maturation was evaluated at the age of 3, 7, 10 and 12 years and the adult height. In all girls and in 5/11 boys treatment of CAH was started at the age of 0.0 - 1.5 years (median 0.1) (early diagnosis), in 6/11 boys at the age of 4.5 - 7.0 years (median 5.2) (late diagnosis). In children with an early diagnosis growth retardation by the age of 3 years occurred (SDS of the girls' height' -1.4 +/- 0.2, n = 24, boys -0.7 +/- 0.4, n = 5). The height deficit in girls at the age of 3 predicted significantly the loss of adult height, as compared with the expected height in the family (r = 0.68, p = 0.02). By the age of 7 years the height deficit declined insignificantly (SDS of girls' height -1.0 +/- 0.2, n = 21), but bone maturation proceeded rapidly (3 years: SDS 0.1 +/- 0.4, n = 7, 7 years: SDS 1.4 +/- 0.5, n = 11). The adult height of girls (n = 12) is 147.0 - 162.5 cm (mean 156.7), i.e. -1.6 +/- 0.2 SDS, in early diagnosed boys (n = 4) 159 - 176.6 cm (mean 167.6), i.e. -1.7 +/- 0.5 SDS, in boys with a late diagnosis (n =4) 165.0 - 172.0 (mean 169.5), i.e. -1.5 +/- 0.3 SDS. CONCLUSIONS: In treated children with CAH growth retardation occurs already from the age of 3 years, apparently due to excessive glucocorticoid doses. This retardation predicts a loss of adult height. Between the age of 3 and 7 years the growth prognosis deteriorates further due to accelerated bone maturation resulting from inadequate suppression of androgens. Optimal treatment during these periods in life can improve the prerequisites for attaining the predicted adult height.

Adolescent↗

PubMed1995

[Stimulation of endogenous secretion of the growth hormone. Today in diagnosis, tomorrow in therapy?].

BACKGROUND: Treatment with growth hormone (GH) restores the natural growth rate in children with growth hormone deficiency (GHD). This is, however, achieved only after daily injections extending over many years and therefore daily short-term hypersomatotropinaemia. Stimulation of endogenous secretion of GH e.g. by oral administration of growth hormone-releasing peptide (GHRP) may help in future to eliminate these adverse aspects. This treatment could be beneficial in patients with a stimulable endogenous GH secretion. METHODS AND RESULTS: In order to find potential candidates for spontaneous secretion of GH the authors examined, using a test with sermoreline (GHRH1-29NH2), 31 children (21 boys) aged 5.8-16.5 years suffering from idiopathic (GHD), previously treated by daily GH injections. GH rose after stimulation with sermoreline to more than 14 mIU/l in 18/31 children (responders). The ratio of "responders" was higher in the sub-group of children with isolated GHD, as compared with multiple pituitary deficiency (p = 0.05) and insignificantly higher in the sub-group of children born by breech delivery (p = 0.13). CONCLUSIONS: More than half the children treated nowadays with GH could profit in future from the method of spontaneous GH secretions. The success of this procedure is more likely in children with isolated GHD and in breech delivered children.

Adolescent↗

PubMed1995

[Differential diagnosis in children with small stature].

BACKGROUND: Physiological growth is a sensitive long-term indicator of child health. Impaired growth of children may be the first manifestation of a serious chronic disease. In order to find a rational examination procedure, the authors analyzed retrospectively the diagnosis in children with impaired growth who were referred for examination to the university hospital. METHODS AND RESULTS: In the course of 5.5 years 190 children (boys/girls, 124/66) with short stature, age 2.5 to 16.5 years were examined. In 93 (68/25, 48.9%) the condition was classified as short-normal, i.e. short but healthy, incl. 25 (11/14, 13.2%) with familial short stature, in 26 (21/5, 13.7%) constitutional growth retardation (and retarded puberty) and in 42 (36/6, 22.1%) a combination of the two conditions. In 97 children (56/41, 51.1%) a pathological condition was found: in 14 girls (7.4%) Turner's syndrome, in 55 children (37/18, 29.0%) deficiency of growth hormone, incl. 11 as a results of a tumour or anomaly of the CNS, and in 28 children (19/9, 14.7%) another serious cause of a growth disorder. In three families the authors detected an autosomal dominant disorder in a parent-child pair, the parent not being aware of the disorder (renal tubular acidosis, vitamin D resistant rickets, hypochondroplasia). Based on analysis of these data the authors suggest a rational differential diagnostic procedure in children with short stature. CONCLUSIONS: The stepwise examination of children with short stature is based on the unequivocal differentiation of short-normal children and assessment of the cause of the growth disorder in the other affected children. The procedure is focused on a sparing, rapid and accurate diagnosis with subsequent early treatment of children with a serious pathological condition.

Adolescent↗