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Biomedical subjects

M B Ranke

Publications and source records attributed to M B Ranke.

At least 217 records · Page 12Linked to original sources
PubMed1986

Treatment of growth hormone deficiency.

According to the results reported in the literature and from our own experience, the following recommendations for the treatment of children with GHD can be given: In order to start GH replacement therapy in early childhood the diagnosis of GHD should be made as early as possible. The growth hormone dose during prepubertal age should not fall short of 12 IU/m2 per week. During spontaneous or induced puberty, the dose needs to be increased, possibly by a factor of two. Daily subcutaneous injections appear most suitable. Treatment with growth hormone releasing factors in cases with hypothalamic GHD, although a promising alternative to the treatment with hGH (Thorner et al, 1985), must be considered experimental at this point. Thyroxine replacement at a daily dose of 75-100 micrograms/m2 should be given in cases of secondary hypothyroidism. Glucocorticoid replacement, if required, should be given at low doses (e.g. hydrocortisone 10 (to 15) mg/m2 per day in divided doses). In cases with additional gonadotropin deficiency, sex steroids (or anabolic steroids) should be given with frequent monitoring of bone maturity not before the age of 13 in girls or 15 years in boys. In boys depot testosterone starting at low doses (e.g. 50-100 mg/month i.m.) will induce a puberty-like increment in height velocity. Since the effect of oestrogens--even in low doses--on growth is uncertain, their administration before achievement of near-normal adult height should be avoided. With the advancement of diagnostic techniques and with the experience in treatment accumulated over the past 25 years, patients with GHD need no longer become dwarfs.

Body Height↗
PubMed1986

Recombinant human growth hormone.

All batches of Met-hGH examined stimulated statural growth to approximately the same extent. The growth rates measured partly exceeded the results obtained in previous studies with pituitary preparations in the same dosage. Under treatment with SI, i.e. the preparation with the highest amount of ECP, high antibody titres with high binding capacity against GH and ECP were found. With SII all antibody determinations showed much lower titres. With Somatonorm (SIII), in the large majority of cases no antibodies were detectable. The titres registered in a few children were low and the binding capacities were negligible. The biologically determined somatomedin activity was initially pathologically low. During treatment it rose to supraphysiological levels. Also the radioimmunologically assayed somatomedin and the alkaline phosphatase increased significantly. At the start of the first series, two patients showed allergic skin reactions which turned out to be caused by the insufficiently purified preparations. Therapy with extractive preparations was free of such side-effects and fully successful. Both of the patients were atopic. A third child who was also allergic developed after 6-9 months the highest antibody titres seen, combined with a high binding capacity. Also, with this boy, treatment was switched over to pit-hGH, with very good results. Two children with pituitary dwarfism already developed in utero high antibody titres against Met-hGH but not against ECP. For this response, neither the Somatonorm nor its impurities can be implicated. Rather, it is the reaction to GH generally, which the organism recognizes as a foreign protein and thus as an antigen. One of the patients stopped growing after nine months. Likewise, pituitary GH did not lead to any further improvement.

Alkaline Phosphatase↗
PubMed1986

Clinical experience with authentic recombinant human growth hormone.

Studies of authentic recombinant human growth hormone (rhGH) began very recently in Germany. In this study, one group of patients with hGH deficiency is naïve (no previous hGH treatment), and the second group has been treated with pituitary hGH. The mean age of diagnosis of 17 naïve patients so far accepted is about 8 years; patients who have previously received pituitary hGH are older and slightly taller. No other data are yet available for analysis.

Adolescent↗
PubMed1986

Clinical experience with GRF.

Growth hormone releasing factor (GRF) is one of two main releasing hormones which control pituitary hGH production and release. This activity is retained even when the length of the GRF polypeptide is reduced to the 29 N-terminal residues. This 29-residue polypeptide has been chemically synthesized, and testing in healthy volunteers elicited secretion of hGH to a maximum level of about 40 ng/ml. In a trial of children with various disorders, the maximum hGH response was reached within 60 minutes; those children with hGH deficiency showed much lower responses than those with other disorders not related to hGH deficiency. The frequency of false-positive tests was low, and it is proposed that GRF testing could provide a useful additional tool in the diagnosis of hGH deficiency.

Child↗
PubMed1986

Isolation and partial characterization of six somatomedin-like peptides from human plasma Cohn fraction IV.

Six somatomedin-like peptides were purified from human plasma Cohn fraction IV by a six-step procedure which included ethanol precipitation, reversed-phase extraction, gel filtration, chromatofocusing and reversed-phase high pressure liquid chromatography (HPLC). Purification was monitored with a competitive protein binding assay using a crude preparations of somatomedin carrier protein. The peptides isolated were homogeneous by reversed-phase HPLC and sodium dodecyl sulphate polyacrylamide gel electrophoresis (SDS-PAGE). Their apparent isoelectric points determined by chromatofocusing were 9.2 (Sm I), (Sm II), 8.2 (Sm III), 6.7 (Sm IV), 6.3 (Sm V), and 6.15 (Sm VI). SDS-PAGE under reducing conditions revealed that they are composed of a single peptide chain with apparent molecular weights of 6800 for Sm I, II and IV and 6400 for Sm III, V, and VI. They were equally potent in the porcine costal cartilage in vitro bioassay. The basic peptides (Sm I-III) were significantly more active in radioimmunoassays for somatomedin C (SmC) and insulin-like growth factor I C-peptide (IGF-I (30 - 41], while only the slightly acidic peptides were active in a radioimmunoassay for insulin-like growth factor II C-peptide (IGF-II (33-40]. When receptor binding was tested with human placental cell membranes and Sm III as tracer, the basic peptides were significantly more potent than Sm IV-VI. With rat liver cell membranes and Sm V as tracer the slightly acidic peptides were more potent. These findings suggest 1) that human plasma may contain other somatomedin-like peptides besides the major components IGF-I/SmC and IGF-II, and 2) that the basic peptides are structurally related to IGF-I/SmC and the slightly acidic peptides are related to IGF-II.

Animals↗
PubMed1986

Effect on growth of patients with Turner's syndrome treated with low estrogen doses.

In 33 patients with Turner's syndrome growth during a one year period of treatment with low doses of estrogens was evaluated (group A: (N = 12) Presomen 5-9 micrograms/kg d; group B: (N = 9) Presomen 10-21 micrograms/kg d; group C: (N = 12) ethinylestradiol 45-155 ng/kg d) and compared to a group (N = 37) of untreated patients. The auxological evaluation was made using SDS derivations based on control data derived from 150 untreated patients. Based on chronological age (CA) SDS levels for height velocity and the increments in height at the end of treatment increased marginally. Compared to untreated patients no effect was seen when calculations were based on bone age (BA) due to an advancement in bone maturity. It is concluded that low doses of estrogens are not suitable to improve the height development in Turner's syndrome.

Age Determination by Skeleton↗
PubMed1985

[Crohn disease: initial experiences with cyclosporin A in an adolescent girl].

The diagnosis of Crohn's disease with extensive involvement of small intestine and colon was first made in a 12 3/4-year-old girl, now 15 1/2 years old. Despite continued treatment with prednisolone and salazosulfapyridine, as well as azathioprine and metronidazole, no lasting remission was obtained. Widespread severe osteoporosis with vertebral fractures made it necessary to discontinue the prednisolone, despite endoscopically and biochemically confirmed signs of activity of the disease. Administration of cyclosporin A in this situation produced phases of improved clinical and biochemical parameters. Regular control of biochemical levels failed to reveal any drug-specific acute side-effects. Because of the increased incidence of malignant lymphoma under cyclosporin A this drug should be held in reserve in the treatment of Crohn's disease, until results from controlled studies have become available.

Adolescent↗
PubMed1985

[Bilateral middle ear tuberculosis and morphologically unusual lung tuberculosis in an 11-year-old boy].

A 11 year old boy presented with chronic otorrhea and hearing impairment. After repeated tympanorevision macroscopic findings suggested the presence of middle ear tuberculosis. Chest X-ray revealed unusual abnormalities which in conjunction with normal pulmonary function and overall well-being did not support concurrent pulmonary tuberculosis. However, microbiological investigations verified this diagnosis in both sites, and proper tuberculostatic treatment proved successful.

Biopsy↗
PubMed1984

Pseudopituitary dwarfism due to resistance to somatomedin: a new syndrome.

The case of an infant is described who at birth was already small and postnatally grew extremely slowly. At age 3 the girl's height was 65 cm, weight 5.6 kg, bone age 21 months. Basal plasma GH was 36-66 ng/ml, basal SM activity was rather high, being around 2.0 U/ml. RIA- and RRA-SM were also increased. Prolonged GH administration did not raise plasma SM. There was a tendency for hypoglycemic episodes in the presence of low insulin levels. Receptor studies with skin fibroblasts showed a diminution of the specific binding of SM-C by 50%. Apparently only the specific IGF-receptor is defective whereas the insulin receptor responds to the increased SM with hypoglycemia. The observation that the high plasma SM did not suppress the enhanced GH-secretion suggests that perhaps the hypothalamic IGF-receptor was also defective.

Child, Preschool↗
PubMed1983

Cerebral gigantism of hypothalamic origin.

In five cases of Sotos Syndrome serum somatomedin activities were measured. In two of these cases elevated levels and an increased secretion of growth hormone was observed. In one case (index case) a suspected hypothalamic tumor mass could be excluded, but hydrocephalus with increased intracranial pressure was present. The pathogenesis of gigantism in this syndrome is discussed.

Adolescent↗
PubMed1983

Turner syndrome: spontaneous growth in 150 cases and review of the literature.

The spontaneous growth of 150 patients with Turner syndrome from three German centers--90 with 45,X0 constitution, 60 with other chromosomal abnormalities--has been analyzed. The mean adult height was found to be (n = 14) 146.8 cm. It was observed that growth in these patients can be divided into four phases: (1) Intrauterine growth, which is retarded; (2) Height development, which is normal up to a bone-age of about 2 years; (3) Between a bone-age of 2 and 11 years when stunting of growth is most marked; (4) After a bone-age of 11 years--the time at which puberty should normally start--the growth phase is prolonged, but total height gain is only little below normal levels. No difference in height could be observed between cases with X0 karyotype and other chromosomal variants. The data are compared with those in the literature.

Adolescent↗
PubMed1983

[Noma].

A 1 4/12 years old boy after having been ill with measles developed gingivostomatitis ulcero-gangrenosa, a disease known as noma in tropical countries. This rare disease usually occurs during severe depression of the immunologic defence like with measles, malnutrition, malignant neoplasmas or chemotherapy, and is caused by a local infection with aerobic and anaerobic bacteria, which usually are part of the normal oral flora. The course of the disease is characterized by an initially edematous swelling of the face followed by ulcerations of the gingiva, progressing as an ulcerophlegmatous inflammation of the oro-facial tissue and finally causing mutilating destruction. Vigorous general and antibiotic treatment has improved the previously poor prognosis.

Humans↗
PubMed1982

Responsiveness of cortisol and dehydroepiandrosterone to ACTH in children.

In a total of 101 children, the dehydroepiandrosterone (DHA) and cortisol (F) levels were measured before and after ACTH (Synacten) administration. F responsiveness was unchanged during development, while DHA responsiveness in healthy children was highest during adrenarche. In hypopituitary patients DHA levels were lower than in the controls, but responsiveness to ACTH showed similar changes during development. Children with Turner's syndrome and hypergonadotrophic males had the response in elevated DHA levels while ACTH-induced DHA response related to bone-age matched controls. We conclude that regulation of adrenal androgens is mediated by both ACTH and another hypothalamo-pituitary hormone, perhaps independent of gonadal activation, but requiring gonadal integrity.

Adolescent↗
PubMed1982

Amniotic fluid levels of 17 alpha-hydroxyprogesterone during human pregnancy: pre-natal diagnosis of congenital adrenal hyperplasia due to 21-hydroxylase deficiency.

Levels of 17 alpha-hydroxyprogesterone were estimated by radioimmunoassay in 174 amniotic fluid specimens obtained from 162 normal pregnancies of 16-20 weeks gestation. Another 18 specimens from 11 normal pregnancies between 25-42 weeks gestation and 7 specimens from 7 women who each previously had given birth to a child affected with CAH were also studied. There was no sex difference in AF 17 alpha-OH-progesterone levels, nor any significant downward trend in concentrations throughout the observed gestation period. Among the 7 subjects with previous history of CAH offsprings, 4 demonstrated normal levels of AF 17 alpha-OH-progesterone and 3 significantly higher values. HLA-typing of the cultivated amniotic cells confirmed these 4 cases to be heterozygous for 21-hydroxylase deficiency. The three subjects with high AF 17 alpha-OH-progesterone levels delivered babies affected with CAH, although HLA-typing in one case was non-conclusive. The failure of HLA-typing in 1 case for a confirmatory diagnosis indicates that the prenatal diagnosis of CAH must continue to rest on joint endocrinological and genetical investigations.

17-alpha-Hydroxyprogesterone↗
PubMed1976

Sex differences in binding of human growth hormone to isolated rat hepatocytes.

Since liver is a target for growth hormone action, binding of 125I-labeled human growth hormone to enzymatically isolated rat hepatocytes was studied. Specific binding was shown with hepatocytes from both male and female animals. There was a single class of receptors for human growth hormone on cells from males (affinity constant, Ka = 1.16 x 10(9) liters/mole; sites per cell, q = 6200). In males, bovine growth hormone was almost as potent as human growth hormone in displacing bound 125I-labeled human growth hormone, while ovine prolactin was about 1000 times less potent. Cells from female rats bound more 125I-labeled human growth hormone than cells from males. The cells from females contained at least two classes of receptors for human growth hormone. The receptor of highest affinity had the same affinity for human growth hormone as the single receptor found in males (Ka = 0.96 x 10(9) liters/mole). However, there were three to four times as many of these receptors per cell in females (q = 21,000). In females, bovine growth hormone and ovine prolactin were both about 20 times less potent than human growth hormone. Treatment of male rats with estrone produced cells that show the same binding characteristics as females. These results indicate that human growth hormone binds to a somatogenic receptor in hepatocytes from male rats. In females and estrogen-treated males, the receptors that bind human growth hormone recognize lactogenic as well as somatogenic properties. This suggests that the lactogenic and growth-promoting effects of human growth hormone in the rat are mediated by different receptors.

Animals↗