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Biomedical subjects

J R Bierich

Publications and source records attributed to J R Bierich.

At least 55 records · Page 3Linked to original sources
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

[Prader-Labhart-Willi syndrome].

12 patients (9 male, 3 female) with the Prader-Labhart-Willi-Syndrome (PLWS) were examined, and had medical treatment. Small stature varied in degree; body-height was found below the 25th percentile of normal height in 9 of the patients, 6 of them ranged along or below the 3rd percentile. Growth-hormone (GH) was measured after i.v.-Arginine-provocationtest; in 7 cases GH-peaks did not rise over 10 ng/ml, 4 of the children showed GH-peaks of less than 4 ng/ml. One of the boys was treated with GH for 5 years with good results, no secondary effect on carbohydrate metabolism was observed. In one girl we started with GH-therapy only recently. In 7 of 9 children at the age of puberty we found hypogonadotrophic hypogonadism. In 10 patients, who underwent i.v. GTT, 4 showed a prediabetic metabolic state with elevated plasma insulin levels, a fifth boy suffered from diabetes. The development of type-2-diabetes is caused by abnormal insulin-resistance, which is connected with overfeeding and obesity. Treatment of obesity is the most important problem in the PLWS. Regular hypocaloric food is not accepted by most of the children. Therefore, in cases of ineffective dietetic treatment, we recommend gastric partitioning with proximal gastro-jejunostomy, as we performed in two of our patients with good results regarding weight-loss. In two of six children examined, we found chromosomal abnormalities.

Adolescent↗
PubMed1983

[Stunted growth].

According to recent investigations more than half of all growth disorders are due to growth hormone (GH) deficiency. I. The cause of the classic hypothalamo-pituitary dwarfism is: 1. perinatally acquired lesions of the hypothalamus; 2. suprasellar tumours; 3. brain malformations; 4. hereditary disturbances of GH-synthesis. - II. Constitutional delay of growth and adolescent development is clinically characterised by harmonious delay of growth, skeletal development and sexual maturation. The typical aspect of the pituitary dwarf is missing. Whereas provocation tests for GH do not show reliable deviations, assessment of the spontaneous GH secretion during sleep usually demonstrates a significant GH-deficit. - III. Impairments of GH synthesis with production of a hormone which reacts in the RIA but exhibits reduced biological effectivity causes the same clinical picture as constitutional delay. The disorders I--III can successfully be treated by GH. - IV. Familial short stature is based on the summation of hereditary anlages of the parents. In principle, it is no endocrine disorder. However, the combination with disorders II and III occurs frequently ("Small/Delay" acc. to Tanner). - V. Intrauterine growth retardation is caused by maternal, placental or fetal factors. With most of the "small for dates" growth prognosis is good. However, if teratogenic agents, particularly alcohol is involved, and if the damage occurs in the first months of gestation, the prognosis is unfavorable. - VI. A great number of genetic disorders is connected with pre- and/or postnatal growth retardation, combined with typical malformations. Apart from few exceptions these syndromes are rare. Endocrine deviations are missing. - The therapeutic possibilities of GH and anabolics are thoroughly discussed.

Birth Injuries↗
PubMed1982

Undescended testes: treatment with gonadotropin.

The therapeutic success of treatment of testicular maldescent must be judged according to 2 parameters, - I. occurrence of descent, II. fertility. Ad I. Indication for hCG-treatment is always given unless an unequivocal indication for operation exists, i.e. ectopias, accompanying hernias, retention after herniotomy and advanced puberty. The optimal time for treatment is the second year of life. The large European statistics which include the prescrotal but not the retractile testes, unanimously show success rates of 50-55%. In the largest American series which does not include prescrotal testicles, descent was observed in 40% of the bilateral and in 30% of the unilateral cases. Analyzing those cases which did not respond to hormones but had to be operated, in the majority of cases ectopic, not dystopic gonads were found. Ad II. Infertility in testicular maldescent can have two reasons, 1. congenital anomalies of the primordium, 2. acquired damages due to the malposition. The few large prospective studies at hand show fertility in the majority of cases with descent after hCG, but in the minority of those coming down only after additional operation. Obviously the latter group represents a negative selection. Unilateral cases had a higher fertility rate than bilateral ones.

Child↗
PubMed1982

[Hospitalised child. Ethical and humanitarian aspects in general and clinical pediatrics (author's transl)].

Hospitalised children do not only suffer from their illness but in addition from the separation from parents and home (deprivation). The possibility for daily visiting and, if need be, simultaneous admission of the mother has greatly improved this situation. Painful investigations can be extremely stressful and always cause anxieties if the children first are not adequately informed and prepared in advance, and second are separated form their mothers. Such mistakes can be avoided if a certain person of the ward's staff is put in charge of a particular child, as a rule a nurse as a substitute for the mother. The nurse's role is not restricted to helping the doctor; her task includes establishing a personal relationship to the child. Mostly she is the person who knows the child best, and is therefore the primary contact person. For the wellbeing of the hospitalised child she is of utmost importance. Doctor, nurse, kindergarten teacher and psychologist should regard themselves as a common "therapeutical team", discussing the child's problems together and taking action in close touch with the parents.

Anxiety↗
PubMed1981

[Puberty].

Puberty commences in girls 1.5-2 years earlier than in boys. Whereas the production of sexual hormone in the female increases gradually, testosterone secretion in the male rises steeply within two years. In connection with this boys are suffering more often from emotional disturbances than girls during puberty. During the last 150 years the onset of puberty has considerably advanced. In the middle of the last century, menarche occurred at age 17; today, however, at age 12.5. This secular acceleration is caused by improved nutrition, mainly with proteins. Presupposition for the earlier onset of puberty is an earlier arriving at the developmental stage (height, weight, bone maturation) which formerly was characteristic for prepubertal children of 10/11 years. Also the physiologic variants, i.e., the so-called early normal puberty and constitutional delay of growth and adolescence, base on prepubertal differences in growth velocity and growth hormone secretion. In contrast to this, the pathologic variants of sexual development, i.e., true sexual precocity and pubertas tarda s.s., are caused by various pathologic processes located in one of the three areas: hypothalamus, pituitary, or gonads.

Adolescent↗
PubMed1981

[Investigations on the genesis of the pubertal growth spurt (author's transl)].

The pubertal growth spurt is based on synergistic effects of hGH and androgenic steroids. It fails to appear in androgen deficiency as well as in complete absence of hGH. Apart from the synergism of biological functions there are interactions with regard to the secretion of hGH and androgens. Investigating the nocturnal hGH-secretion in puberty stage 3 we found twice as high secretion rates as in stage 1. Children with anorchia or constitutional delay of growth and adolescence during treatment with testosterone secreted more than threefold amounts of hGH as before. In two cyproterone acetate-treated adolescents, and in four 12-19 years old patients with hypogonadism, the nocturnal secretion rates of hGH as well as the maximal hGH-peaks attained were lower than in normal prepubertal children. The conclusion is drawn that the androgen-secretion during adolescence is not only prerequisite to the hGH-increment necessary for the pubertal growth spurt but also to the preservation of basal hGH-secretion.

Adolescent↗
PubMed1980

[Obstetrical and postnatal complications in children of chronically alcoholic mothers (author's transl)].

The histories of mothers addicted to chronic abuse of alcohol always present severe complications during pregnancy and for most of their children during the peri- and postnatal period as well. The question arises to what extent peri- and postnatal complications may influence the clinical aspect of the alcoholembryopathy (AE) in these children. In 35 children with AE all details of their histories could be traced. By using Prechtl's concept of optimal conditions two different populations could be found amongst the children with AE, one with and the other without severe peri- and postnatal complications (exclusively peri- and postnatal asphyxia). No correlation could be found between the severity of AE and perinatal asphyxia. The result suggests reservation in rating peri- and posnatal asphyxia as an always potent factor causing brain damage.

Asphyxia Neonatorum↗
PubMed1979

[Alteration of TSH secretion in children with goitre after short-term treatment with potassium iodide (author's transl)].

300 microgram of potassium iodide daily were administered over a period of two weeks to 12 children with goitre. Basal TSH concentrations, maximal TSH increase after TRH and integrated TSH secretion after TRH were significantly decreased in this group whereas the increase of total thyroxine (T4-RIA) did not reach significance. It appears that in the development of iodine deficiency goitre in children a sensitive feed-back system is predominant. Early administration of iodine or even better general iodinated salt prophylaxis seem to be the pathogenetically correct approach in solving the common goitre problem.

Adolescent↗
PubMed1979

Long-term response to human growth hormone in 36 children with idiopathic growth hormone deficiency.

The results of long term treatment with human growth hormone (Crescormon, 12.4 IU/m2/week) in 36 patients with idiopathic growth hormone deficiency are given. Birth trauma--in particular assisted breech delivery (30%)--is the major aetiological cause. Twelve patients had isolated growth hormone deficiency (IGHD), 24 had multiple pituitary hormone deficiencies (MPHD) of which 19 were treated with additional thyroid hormones. The results were judged by the criteria of height velocity, total height gain and change of height prediction (TW2, age based). It is concluded that the growth hormone dose chosen in many cases is insufficient to maintain high growth rates after the first year of treatment, when "catch-up" no loner takes place. The tendency of patients supplemented with thyroid hormone to grow better--without additional bone-age advancement--calls for careful search for hypothyroidism and suggests the use of thyroxin in cases of doubt.

Body Height↗
PubMed1979

Alcohol embryo- and fetopathy. Neuropathology of 3 children and 3 fetuses.

Maternal chronic ethanol abuse during pregnancy causes malformations of the offspring. Three children (aged 6 months, 9 months, 4 1/2 years) and 3 fetuses (17th, 18th, and 20th gestational week) showed a wide spectrum of disorders ranging from severe dysraphic state, arhinencephaly, porencephaly, agenesis of corpus callosum, a range from hydranencephaly to microdysplasias (p.e. reduced gyration of dentate nucleus and inferior olives), and a range from gastrochisis or congenital heart defects to craniofacial dysmorphogenesis and palmar crease anomalies. The patterns of the cerebral malformations were not as uniform as the clinical phenotype of the alcohol embryopathy. The observations did not support the assumption that there exists a specific period for alcohol teratogenicity.

Abnormalities, Drug-Induced↗