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Biomedical subjects

P Stubbe

Publications and source records attributed to P Stubbe.

17 recordsLinked to original sources

Growth response to recombinant human growth hormone of mammalian cell origin in prepubertal growth hormone-deficient children during the first two years of treatment.

In five clinical studies performed in Austria, France, the FRG, Italy, Switzerland, the UK and the USA, 304 growth hormone (GH)-deficient children were treated with recombinant human GH (rhGH) of mammalian cell origin. Two hundred and twenty-five patients were previously untreated (naive patients), and 79 were transferred from pituitary hGH after interruption of therapy for at least 6 months (transfer patients). Two treatment protocols, differing in both dose and frequency of injections, were used: (1) a dose of 0.6 IU/kg body weight per week was administered in 3 s.c. injections to 203 patients (178 naive, 25 transfer; group 1); and (2) a dose of 0.45 IU/kg body weight per week was administered in 7 s.c. injections to 101 patients (47 naive, 54 transfer; group 2). After 1 and 2 years of treatment, 143 and 109 naive, and 51 and 46 transfer patients, respectively, were still prepubertal, and their data were analyzed for efficacy. During the 1st year of treatment, both naive and transfer patients on daily injections (group 2) demonstrated better growth than those on 3 injections per week (group 1), with height velocities (HVs) of 10.6 +/- 2.7 cm/year (group 2) versus 8.6 +/- 2.0 cm/year (group 1) for naive patients (p < 0.001), and 9.9 +/- 1.9 cm/year (group 2) versus 7.2 +/- 2.7 cm/year (group 1) for transfer patients (p < 0.001). The corresponding changes in height standard deviation score (delta H SDS) for chronological age (CA) were +1.3 +/- 0.6 (group 2) versus +0.8 +/- 0.5 (group 1) for naive patients (p < 0.01), and +1.1 +/- 0.3 (group 2) versus +0.6 +/- 0.4 (group 1) for transfer patients (p < 0.001).(ABSTRACT TRUNCATED AT 250 WORDS)

Animals

Recombinant human growth hormone and oxandrolone in treatment of short stature in girls with Turner syndrome.

91 girls with Turner syndrome (TS) with a mean chronological age (CA) and bone age (BA) of 10.3 +/- 2.3 and 8.9 +/- 1.9 years, respectively, were randomly assigned to subcutaneous treatment with recombinant human growth hormone (rhGH) alone (n = 47), 2.6 IU/m2 body surface area daily or combination treatment (n = 44) with the same dose of rhGH and oxandrolone 0.1 mg/kg body weight orally, for the first 12 months of this study. During the 1st year of therapy, there was a striking increase in height velocity (HV) in both groups, from 4.0 +/- 0.8 to 6.3 +/- 1.3 cm/year [HV standard (standards of untreated Turner patients) deviation score (SDS) for CA from 0.0 +/- 0.7 to 2.9 +/- 1.3] in the rhGH group and from 4.2 +/- 1.2 to 8.5 +2- 1.7 cm/year (HV SDS-CA from +0.3 +/- 1.0 to 5.6 +/- 1.6) in the combination group. The difference between the groups was statistically significant (p < 0.01). During the 2nd year of treatment, the rhGH dose was increased to 3.4 IU/m2 daily for the rhGH-alone group, whereas in the combination treatment group the oxandrolone dose was reduced to 0.05 mg/kg daily. HV was maintained at significantly higher levels than those prior to treatment, at 5.3 +/- 1.1 cm/year (HV SDS-CA: +2.1 +/- 1.3) and 6.2 +/- 1.5 cm/year (HV SDS-CA: +3.6 +/- 1.4) in the rhGH-alone and the combination group, respectively (p < 0.001).(ABSTRACT TRUNCATED AT 250 WORDS)

Aging

[Cytological diagnosis of thyroiditis (author's transl)].

In 2347 patients fine needle aspiration of the thyroid gland was performed. In 49 cases (2.1%), a final diagnosis of thyroiditis was established. Non-specific granulomatous thyroiditis occurred most frequently (n = 24), followed by lymphocytic hypertrophic thyroiditis Hashimoto (n = 18), focal lymphocytic (n = 5), atrophic lymphocytic (n = 1) and chronic fibrosing thyroiditis (n = 1). Fine needle aspiration biopsy is most suited to diagnose granulomatous thyroiditis. In Hashimoto's thyroiditis cytological investigations are superior to estimation of thyroglobulin antibodies. The cytological method is of little value for the diagnosis of atrophic lymphocytic and chronic fibrosing thyroiditis.

Biopsy, Needle

[Juvenile goitre (author's transl)].

Clinical, cytological, serological and endocrinological examinations were performed on 50 previously untreated children and adolescent with goitre. Three girls were found to have the cytological picture of Hashimoto's thyroiditis, in one instance associated with Turner's syndrome and hyperthyroidism. Two other girls presented with hyperthyroidism. In the remaining 45 patients thyroid aspirates and thyroid hormone levels were normal. The girl with Turner's syndrome, hyperthyroidism and Hashimoto's thyroiditis had thyroglobulin antibodies. It is the authors' experience that the diagnosis of Hashimoto's thyroiditis can be made only by needle biopsy of the thyroid. If iodination of cooking and table salt were to become widespread in the Federal Republic of Germany, and increased incidence of Hashimoto's thyroiditis is to be expected amount children with goitre.

Adolescent

[Hypothyroid, iodine-deficient goitre in neonates (author's transl)].

Within one year 1750 mature neonates were examined for congenital hypothyroidism. The region of Göttingen is known to be an iodine-deficient one. Sixteen goitrous, hypothyroid neonates with a low total thyroxine, and raised serum TSH concentration were first diagnosed through this screening programme. As a result of the intra-uterine iodine deficiency, serum triiodothyronine concentration was elevated while urinary iodine excretion was reduced compared with euthyroid neonates. During iodine treatment the size of the thyroid became normal within eight days, TSH after 3.8 days and serum thyroxine after three days. Serum triiodothyronine concentration remained elevated for several weeks, presumably as a result of the persisting iodine deficiency. The results indicate that a neonatal screening programme for hypothyroidism is as essential as adding iodine to table salt for avoiding neonatal and foetal maldevelopment.

Congenital Hypothyroidism

Prolactin-producing pituitary adenoma in a 9 year old boy.

A boy aged 9 years and 8 months was evaluated for headache and an enlarged sella. His neurological status and visual fields were normal. After injection of radiographic contrast agent, computerized axial tomography showed evidence of an intrasellar tumor. The most striking endocrine abnormalities were growth hormone insufficiency after arginine infusion and after insulin-induced hypoglycemia, and excessively elevated prolactin levels ranging between 1220 ng/ml and 1560 ng/ml. A slightly granulated, acidophilic pituitary adenoma was selectively removed by the trans-sphenoidal approach. The function of the anterior pituitary improved post-operatively. Growth hormone secretion after insulin-induced hypoglycemia returned to normal, and the basal serum prolactin levels decreased, but are still three times higher than normal.

Adenoma, Acidophil

[Cryptorchidism (author's transl)].

The treatment of cryptorchidism should start at the age of two years because of pathological and anatomical studies. Ectopic testicles, inguinal hernias and postoperative situations associated with a wrong position of the testicle are primary indications for surgical corrections. Fixed inguinal, retractile or abdominal testicles may be primarily treated with human chorionic gonadotropin (HCG). The success rate after medical treatment averages 50%. The intranasal application of the releasing hormone of luteotropic hormone (LH-RH) may represent an alternative approach to the treatment of cryptorchidism. If treatment with LH-RH is to obtain the same success rate as HCG in larger series it may combine the advantage of painlessness with no androgenic side effects.

Administration, Intranasal

[The radiolucent esophageal foreign body in children (author's transl)].

The symptoms of radiolucent esophageal foreign bodies in small children may be predominantly respiratory with stridor and dyspnea. In a 1 3/12 years old girl who suffered from inspiratory stridor and only later from dysphagia as well this diagnosis was delayed for 2.5 months. Air in the upper part of the esophagus was the first striking roentgenologic symptom of the foreign body which was then clearly outlined by a barium swallow examination. The foreign body was a plastic lid of a candy tube.

Age Factors

Thyroxine-binding globulin, triiodothyronine, thyroxine and thyrotropin in newborn infants and children.

Thyroxine-binding globulin (TBG), triiodothyronine (T3), thyroxine (T4) and thyrotropin (TSH) have been determined by radioimmunoassay in plasma of newborn infants and throughout childhood until puberty. Mean maternal TBG concentration was 1.65 +/- 0.09 mg/100 ml (SEM) and significantly higher (p less than 0.01) than cord blood levels of TBG (1.16 +/- 0.08 mg/100 ml (SEM). Throughout infancy and childhood TBG remained significantly elevated (p less than 0.01) compared to a middle age control group of healthy blood donors. T3, T4 and TSH concentrations behaved postnatally as known from previous studies. The T3 and T4 increase observed immediately after birth was not a secondary phenomenon due to changes in TBG concentration since this globulin did not change significantly during this period.

Aging

Serum 3,5,3'-triiodothyronine, thyroxine, and thyrotropin in hypothyroid infants with congenital goiter and the response to iodine.

Iodine deficiency in adults caused preferred synthesis of T3; this observation has not been reported in iodine-deficient hypothyroid newborns. Serum total T4, total T3, and TSH have been determined in nine full term newborns with congenital hypothyroid goiter before and after cutaneous application of iodine. The mothers of these infants had untreated euthyroid goiter and lived during pregnancy in the area of Göttingen, West Germany, known as an iodine-deficient region. Mean total T4 in the newborns was 6.3 +/- 1.6 (mean +/- SD) micrograms/dl compared to 16.6 +/- 3.4 micrograms/dl in normal newborns at 3-4 days of age. Mean T3 in the goitrous newborns was 2.74 +/- 0.66 ng/ml compared to 1.58 +/-0.41 ng/ml in the control group of the same age. Serum TSH remained elevated during the first week of life, with a concentration of 40.9 +/- 28.7 microU/ml (control group, 4.16 +/- 1.43 microU/ml). The cutaneous application of iodine resulted in rapid disappearance of goiter and normalization of T4 and TSH within 5 days. After 30 days of iodine treatment, T3 decreased slowly but remained elevated (2.0 +/- 0.42 ng/ml vs. 1.67 +/- 0.36 ng/ml in the control group). The present findings confirm preferential T3 secretion in newborns with hypothyroid goiter. The goiter is thought to be caused by intrauterine iodine deficiency, because hypothyroid values of T4 and TSH normalized during iodine treatment. General iodine prophylaxis of the population is recommended.

Goiter

TBG-dependency of age related variations of thyroxine and triiodothyronine.

Thyroxine (T4), triiodothyronine (T3) and thyroxine-binding globulin (TBG) were determined in healthy individuals ranging in age from newborn to 95 years. T4: 10.25 +/- 1.62 microng/100 ml, T3: 1.62 +/- 0.35 ng/ml and TBG: 1.34 +/- 0.15 mg/100 ml, were found elevated until puberty compared to a middle age group with T4: 7.27 +/- 2.26 microng/100 ml, T3: 1.15 +/- 0.24 ng/ml and TBG: 0.98 +/- 14 mg/100 ml. T4 and T3 followed almost TBG concentration. In old age is dissociation between T4: 5.79 +/- 1.56 microng/100 ml, T3: 0.79 +/- 0.21 ng/ml and TBG: 1.28 +/- 0.15 mg/100 ml was found. Except for old age the ratio T4/TBG and T3/TBG minimized the age dependent variation of T4 and T3 and reduced the coefficient of variance from 26% to 17.7% for T4 and from 26.5 to 25% for T3. Age reduction of T4/TBG is 15% and of T3/TBG 13% respectively more pronounced than for T4 and T3 alone. These data indicate: 1) age related variations of T4 and T3 due to age dependency of TBG, 2) deviation of T4 and T3 values in old age from that expected by their TBG levels and 3) the importance of the routine use of hormone/TBG ratio.

Adolescent

[Pseudohypoaldsteronism. A further case report (author's transl)].

The clinical symptomatology and diagnostic procedures of pseudohypoaldosteronism in an 8 days old male newborn infant are described. The course of the disease was initially characterized by failure to thrive, renal salt loss, hyponatremia and hyperkalemia. Daily treatment with 3 g sodium chloride improved symptoms. Hydrocortisone and desoxycortiscosteronacetate were without any effect. Urinary aldosterone excretion was increased 10 to 20 times of normal. 17 ketosteroid and pregananetriol were normal. The infant died of ulcerative enterocolitis at the age of 1 1/2 years. Beside the first description of the disease by Cheek et al. (1958) 13 further publications have been collected from the literature. Symptoms at the beginning, sex, serum electrolytes, aldosterone excretion, somatic development, observation period, treatment and duration of therapy are compared with the presented case report.

17-Ketosteroids

Effects of arginine infusion in infants: increased urea synthesis associated with unchanged ammonia blood levels.

Infusion of L-arginine hydrochloride in infants and children (ages ranging from 1 day to 12 yr) at a dosage of 0.5 g/kg body weight resulted in a dramatic increase in the arginine plasma concentration, with highest values of approximately 7 mmole/liter immediately after the end of the infusion; 120 min later the mean plasma level of the amino acid had decreased to mean values of 1 mmole/liter. These fluctuations were paralleled by increased ornithine concentrations, although the mean plasma levels of this amino acid remained far below those of arginine, i.e., 0.73 and 0.22 mmole/liter after 30 and 90 min, respectively. When expressed on a molar basis, arginine administration resulted in an almost stoichiometric rise in urinary urea excretion. These findings indicate that arginine is rapidly metabolized via urea and ornithine, the latter being transformed to glucose, as evidenced by a significant rise in the blood glucose concentration. Blood gas analyses and serum urea and blood ammonia concentrations determined after the load showed no significant deviations from preinfusion levels. Thus, in contrast to the effects to be expected form studies with tissue culture homogenates, even when administered to newborn infants, arginine does not impair the turnover of the urea cycle.

Ammonia

Fanconi's anemia. I. Case histories, clinical and laboratory findings in six affected siblings.

This is a study of the largest family with Fanconi's anemia known in the world literature, namely of 6 affected siblings. All patients showed the typical features of the disease, including pancytopenia, skeletal and kidney deformities, hyperpigmentation of the skin, and physical as well as mental retardation. Four of the patients have died of their disease, the 2 patients who are still living at present have a deteriorating clinical course. Case histories, clinical and laboratory findings are reported here. In the second and third part of this study [48, 52] endocrinologic and genetic findings will be reported.

Abnormalities, Multiple

Fanconi's anemia. II. Are multiple endocrine insufficiencies a substantial part of the disease?

Three children with Fanconi's anemia belonging to a family where 6 children had the disease were investigated. One child had growth hormone deficiency, a second child showed subnormal response of testosterone to gonadotropin stimulation and the third child had a missing insulin release following arginine. This report shows that growth hormone deficiency is not necessarily liniked with Fanconi's anemia when it occurs in a family. Multiple endocrine insufficiencies do no appear to be part of the disease.

17-Hydroxycorticosteroids

[LH-RH Test in prepuberal children (author's transl)].

The LH-RH test was performed in 62 mainly prepuberal children. A dose of 25 mug/20 kg was given intravenously between 10 and 12a.m. Prepuberal healthy boys between 1 11/12 and 9 7/12 years of age reacted with a fourhold increase of LH. Prepuberal boys with an unilaterally undescended testicle showed no difference in LH response from normal. Even a low increase in LH may be followed by spontaneous puberty in children with pituitary dwarfism. The LH response in children with craniopharyngeoma was heterogeneous and appeared to depend on location of tumor and extent of operation. Birdheaded dwarfism and small stature due to steroid administration and due to unknown etiology showed normal LH responses for age. Cases with anorchia and myotonic dystrophy had an excessive LH increase. The LH response in children with Fanconi's anemia and undescended testicles and in otherwise healthy boys with undescended testicles was normal for age. A case of untreated adrenogenital syndrome without salt loss had a presumably normal increase of LH when related to bone age. Primary and pituitary hypothyroidism was shown to have a higher than normal output of LH. A boy with a tumor the 3rd ventricle hat basal levels of LH that were extremely elevated and associated with precocious puberty and diabetes insipidus. A newborn infant with anencephalus showed no increase of LH and LH-RH injection.

Adolescent