Biomedical subjects

W Kiess

Publications and source records attributed to W Kiess.

211 records · Page 12Linked to original sources

PubMedDate not supplied

[Bone metabolism in 53 children and adolescents with chronic inflammatory bowel disease].

BACKGROUND: Osteopathy is a common clinical feature of chronic inflammatory bowel disease (IBD) in children and young adults at the time of primary diagnosis. The aim of the following study was to address the question of prevalance of a decreased bone density or increased bone metabolism in children with IBD. PATIENTS: We examined 63 patients (mean age 13 years; 5 - 18 years): 36 Crohn's disease (MC) patients, 16 colitis ulcerosa (CU) patient and one patient with colitis indeterminata (CID). 10 children who had been referred to the gastroenterological outpatient department due to suspected IBD symptoms were later found not to suffer from IBD. These 10 patients therefore were included in the study as controls. RESULTS: 8 of 10 CU patients and 18 of 28 MC patients showed a pathological bone density and abnormalities in bone metabolism. Repetitive bone density measurement was performed in 18 patients. In MC patients a - 0.39 SDS decrease of bone mineral density was found, without a simultaneous deterioration of clinical stage and inspite of a decreased cumulative prednisolon dosage. However in CU patients a + 0.06 SDS increase of bone mineral density was detected. These patients had a lower cumulative prednisolon dosage and a stable clinical course. CONCLUSIONS: In conclusion, pediatric IBD patients often show abnormalities in bone metabolism and decreased bone density. There is a need for multicentre, prospective randomised control trials to further identify therapeutic tools on the basis of the multifactorial etiology of bone disease in pediatric IBD patients.

Absorptiometry, Photon↗

PubMedDate not supplied

[Treatment of adolescents with type 2 diabetes].

BACKGROUND: A worldwide increased incidence of adolescents with type 2 diabetes mellitus is evident. Only few substances are available for treatment of adolescents with type 2 diabetes. We report on our experience of treatment in the diabetes centre in Leipzig, Germany. PATIENTS AND METHODS: At the moment we care for three patients with type 2 diabetes (two girls and one boy) age 16 - 17 years. We retrospectively analyzed the patients records for symptoms at onset, BMI, HbA1c and treatment for a maximum of 4 years. RESULTS: None of the adolescents had typical symptoms at onset. All had first or second degree relatives with type 2 diabetes. Diagnosis was made using oral glucose tolerance test. BMI at onset was 26 kg/m (2) (90.-97 percent) to 35.2 kg/m (2) (>99.5 percent). Fasting and stimulated insulin and c-peptide levels were elevated in all cases. An elevated HbA1c level was found in one patient. Two patients had further metabolic symptoms like hypertriglyceridemia or hyperurikemia. We started with metformin after dietary instructions in all cases. One girl is on insulin at the moment and the boy stopped metformin after weight reduction of 24.5 kg. CONCLUSIONS: In Germany type 2 diabetes is diagnosed more frequently at an early age. Adolescents with type 2 diabetes should be treated in a centre for pediatric diabetology. Treatment should consist of an individualized care for all aspects of type 2 diabetes.

Adolescent↗

PubMedDate not supplied

[Rare case of an epidemiologically important tuberculosis in childhood].

BACKGROUND: Tuberculosis is an infectious disease which is nearly forgotten in Germany because of its low incidence. CASE REPORT: We report on a 14-year-old german girl who was disregarded when active case-finding of her uncle's active pulmonary tuberculosis was carried out three years before. As a result she herself developed a severe infectious pulmonary tuberculosis. The delay between onset of symptoms and diagnosis gives cause for concern and led to active tuberculosis in her brother and her girl friend as well. The lack of information about tuberculosis in population and delay of medical detection in this case led unnecessarily to the continuing chain of infection. CONCLUSIONS: This case report shows that there are severe infectious courses of tuberculosis even in childhood which might get epidemiologically important. For earlier diagnosis and successful interruption of chains of infection tuberculosis in the German population even today has to be taken into account. Case detection through contact investigation of adults is of great importance in childhood and adolescence.

Adolescent↗

PubMedDate not supplied

An esophagobronchial fistula caused by an unusual foreign body in the esophagus leading to mediastinitis with fatal outcome.

An 8(10)/(12) year old girl with infantile cerebral paresis (ICP), severe mental retardation and seizure disorder was admitted to the intensive care unit (ICU) with clinical signs of aspiration and died ten days later. Final diagnosis was esophagobronchial fistula caused by a foreign body in the esophagus provoking aspiration pneumonia, mediastinitis and respiratory distress syndrome (ARDS). The foreign body was found to be part of a plastic toy. The clinical relevance of aspiration in mentally retarded children and the peculiar nature of the foreign body impacted in the esophagus in this case are discussed. We conclude that in cases of aspiration pneumonia in mentally retarded children the presence of unusual foreign bodies should be suspected.

Bronchial Fistula↗

PubMedDate not supplied

Cloning and sequencing of the complete cDNA encoding the human insulin receptor related receptor.

The insulin receptor related receptor (IRR) is a heterotetrameric transmembrane receptor with intrinsic tyrosine kinase activity. The IRR shares large homology with the insulin and the insulin-like growth factor-1 (IGF-I) receptor with regard to amino acid sequence and protein structure. So far, only a partial human sequence containing the complete 3' end has been reported, although the full-length human IRR cDNA had been used for transfection studies and functional analysis of the receptor. We have isolated a full-length human IRR cDNA and report on the 5' translated and untranslated region of the human IRR gene. The full length IRR sequence contains 4150 bases and shares a high degree of homology with the guinea pig IRR cDNA sequence and rat IRR sequences that had been reported earlier on by others. Sequencing of the IRR cDNA revealed that the human IRR cDNA contains 341 bases corresponding to the IRR 5' end in addition to the bases that had been reported on before. Also, this sequence contains the start codon of translation. The full length cDNA for the human IRR can now be used for functional expression studies and to elucidate the nature of the ligand for this receptor type.

Cloning, Molecular↗

PubMedDate not supplied

[Hypocalcemic tetany in 'alternative' soy milk nutrition in the first months of life].

A 14 weeks old infant was admitted to the intensive care unit with life-threatening hypocalcemic-hyperphosphatemic spasms. Hypocalcemia-hyperphosphatemia was found to have been caused by feeding a high phosphate/ low calcium soy milk. The daily uptake of calcium was calculated to have been 3.3-6 mmol that of phosphate 30 mmol. The parents strongly believed that soy milk formulas were equivalent to breast milk and cow's milk formulas and lived on a strictly vegetarian diet. Therapy with calcium (at an initial dose of 2.25 mmol/kg/day) and 1.25 OH vitamin D3 (Rocaltrol, 0.25 microgram/day) normalized Ca, PO4, vitamin D and parathyroid hormone levels rapidly. Vegetarian feeding had led to life-threatening hypocalcemic hyperphosphatemic spasms in the infant. We conclude that malnutrition and false nutritional beliefs have to be included as a potential cause of early hypocalcemia in infants.

Calcium↗

PubMedDate not supplied

Short stature and low IGF-1 and IGFBP-3 despite normal growth hormone secretion in a 4 year-old girl with primary empty sella syndrome.

A 4.1 year-old girl presented with short stature, cleft lip and additional upper incisor. Magnetic resonance imaging showed an empty sella due to a hypoplastic anterior pituitary. IGF-I and IGFBP-3 were at and below the 1st percentile, respectively. In contrast, normal spontaneous growth hormone (GH) secretion (5.5-h nocturnal sampling) was observed and pharmacological provocation raised (GH to levels between 15.4 and 53 micrograms/l. GH-binding protein levels were normal (210 pM). GH therapy led to an increase of growth velocity from 4.5 to 10.8 cm/year and a normalization of IGF-I and IGFBP-3 levels. The findings may imply an abnormal GH secretion pattern or a bioinactive GH in our patient. The data indicate that measurements of IGF-1 and IGFBP-3 may be a more sensitive test for integrative GH activity than GH testing itself.

Body Height↗

PubMedDate not supplied

Secondary central precocious puberty in a girl with McCune-Albright syndrome responds to treatment with GnRH analogue.

GnRH analogues have been used with variable success for the treatment of precocious puberty in children with McCune-Albright syndrome (MAS). In general, children with a bone age of less than 13.5 yr have been reported not to have benefitted from GnRH therapy. In contrast, we have successfully treated a young girl with MAS and--probably secondary--central precocious puberty using Decapeptyl, a long acting GnRH analogue. The girl with MAS presented at the age of six years with café au lait spots, osseous lesions and precocious puberty. At initial presentation height was 130.7 cm (> 97 percentile), weight 27.5 kg (> 97 percentile), Tanner stage B3, PH3. Bone age was 11 yr. Magnetic resonance imaging of the brain was normal. Endocrine function tests were normal with the exception of biochemical evidence of central precocious puberty: LHRH test: LH 0.9/20.3, FSH 4.3/12.7 (mU/ml), E2 15.6 pg/ml. Therapy was started with 3.75 mg GnRH analogue i.m. every four weeks and was intensified two years after the beginning of therapy to 3.75 mg i.m. every three weeks. Three years after the start of treatment bone age was 12 yr and growth velocity was 2.5 cm/year. Tanner stage was B3, PH3 and LHRH testing revealed biochemical evidence for suppression of gonadotropins: LH < 0.5/1.0, FSH 1.9/2.5 (mU/ml). We hypothesize that a subgroup of patients with MAS might present with a central form of precocious puberty. This may be particularly so in children with a bone age greater than or equal to 11 yr. Central precocious puberty in these children might follow extensive sex steroid exposure due to the peripheral precocious puberty induced by the activating mutation of the Gs protein gene. This central form of precocious puberty responds to therapy with GnRH analogues.

Brain↗

PubMedDate not supplied

Leptin as a metabolic regulator during fetal and neonatal life and in childhood and adolescence.

Body weight is regulated by a feedback loop in which peripheral signals report nutritional information to an integratory center in the brain. The cloning of the ob gene is consistent with this concept and suggests that body fat content in adult rodents is regulated by a negative feedback loop centered in the hypothalamus/1-8/. In a recent report, two severely obese children with congenital leptin deficiency due to a homozygous frame-shift mutation involving the deletion of a single guanine nucleotide in codon 133 of the ob gene have been described. This discovery provides the first genetic evidence that leptin is an important regulator of energy balance in humans. However, it has become increasingly clear that apart from leptin's function in the central nervous system and in regulation of energy balance, leptin also acts in the periphery and might be important as a hormone modulating processes in regard to reproduction, glucose metabolism and insulin resistance, as well as growth and development of many tissues and organs either directly or indirectly. This report reviews some of the topics of leptin research that are of particular importance and relevance for pediatric and adolescent medicine and for pediatric endocrinology in particular.

Adolescent↗

PubMedDate not supplied

Arginine infusion and/or acute changes of growth hormone levels do not acutely alter leptin serum levels.

UNLABELLED: Leptin, the ob gene product, is produced by differentiated adipocytes. It functions as an afferent signal to the central nervous system indicating satiety and fat mass status. It acts upon the hypothalamic-pituitary axis. Growth hormone (GH) secretion is thought to be stimulated by leptin. Conversely, leptin secretion and ob gene expression are regulated by classical neuroendocrine networks. Whether or not acute changes of GH concentrations directly alter leptin serum levels in vivo is still debated. We investigated whether or not acute changes in GH serum concentrations during arginine infusion (0.5 g/kg b. wt.) alter leptin serum levels in 45 children and adolescents (33 M, 12 F). GH and leptin serum levels were determined at -30, 0, 30, 60, 90, 120 min after arginine infusion using specific radioimmunoassays. Leptin serum concentrations remained unaltered throughout the arginine infusion in all children and adolescents whether or not GH secretion was normal. IN CONCLUSION: (1) Acute changes of GH levels do not alter leptin serum levels during acute arginine infusions over 120 min. (2) Arginine does not acutely modulate leptin secretion.

Adolescent↗

PubMedDate not supplied

Diagnosis and management of juvenile hyperthyroidism in Germany: a retrospective multicenter study.

This retrospective multicenter study was designed to survey the management of childhood and adolescent hyperthyroidism in six pediatric endocrinological units in Germany. Fifty-six patients aged between 1.1 and 17.0 yr (median 10.5 yr) were enrolled. Data were collected retrospectively from the patients' records by a trained pediatric endocrinologist using standardized questionnaires. After the diagnosis of hyperthyroidism was established on the basis of clinical and biological findings, treatment with antithyroid drugs (carbimazole, methimazole, thiamazole, propylthiouracil) was started in all patients. In 55/56 of the patients treated with antithyroid drugs, euthyroidism was achieved (98%). However, 26 patients (47%) were still hyperthyroid after discontinuation of the medication. Eight children with continued hyperthyroidism ultimately underwent subtotal thyroidectomy 13-136 (median 28) months after the initial diagnosis. Management principles of the participating centers were heterogeneous. As a consequence, prospective multicenter studies are urgently needed to establish clear standards for the diagnosis and therapy of childhood hyperthyroidism.

Adolescent↗

PubMedDate not supplied

Prediction of height velocity of prepubertal children with growth hormone deficiency in the first year of treatment with recombinant human growth hormone.

AIM: Several methods have been developed to predict the outcome of growth hormone (GH) therapy in children with growth hormone deficiency (GHD). METHODS: Over 50 factors for each of 92 prepubertal patients with GHD (26 patients with total and 45 patients with partial GHD, 21 patients with neurosecretory dysfunction) were collected and included in multiple regression analyses and other nonlinear models to predict height velocity (HV) (cm/yr) in the first year of treatment. Afterwards the model was validated by two other cohorts of patients from other universities, which followed the same treatment regime as our clinic. RESULTS: Twelve parameters had a significant correlation to HV (p<0.05) and a coefficient of determination >20%. Two parameters (In BA, In GHmax) showed a coefficient of determination >60% for children with GHD in multiple regression analysis. The validation of the mathematical model against another data set showed different results. GH was measured by the same method, but BA was scored at the first clinic as in our clinic by only one doctor and in the other university by several radiologists. The accuracy of prediction in the first clinic was significantly higher than in the other university. CONCLUSION: This model demonstrates that exact scoring of BA and precise measurement of GHmax in the stimulation test is necessary, and if carried out carefully leads to useful prediction values for determining height velocity.

Body Height↗

PubMedDate not supplied

Cord blood leptin reflects fetal fat mass.

OBJECTIVE: To explore the relationship between leptin levels and fat mass at the time of birth to test the hypothesis that the level of leptin in the fetal circulation is primarily an index of fetal fat mass. METHODS: Leptin concentration was measured in cord blood obtained from the offspring of 42 women. Trimmed, drained placental weight and neonatal morphometrics were obtained after delivery. RESULTS: The ranges in maternal weight (46.7-93.2 kg), weight gain (3.2-22.6 kg), percent body fat (10-34%), placental weight (290-688 g), birth weight (2.63-4.32 kg), neonatal fat mass (179-782 g), and cord blood leptin (1.7-26.7 ng/mL) were wide. The only morphometric variable that explained a significant portion of the variation in cord blood leptin levels was neonatal fat mass (r2 = 0.41), and this relationship was not significantly improved by best subset regression of additional fetal and placental morphometric variables (r2 = 0.46). CONCLUSION: These data support the hypothesis and suggest that in the fetus, as in the child and the adult, fat mass is the major determinant of circulating leptin levels.

Adipose Tissue↗