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Biomedical subjects

E Keller

Publications and source records attributed to E Keller.

At least 91 records · Page 5Linked to original sources
PubMed2000

[Model program for the early detection and optimal treatment of disorders of growth and physical development using a medical competence netwok].

The growth and development of a child is a mirror of his living conditions and health. For decades pediatricians have been trying to achieve an earlier identification of children with growth disorders. The measures of prevention in Germany have not been successful so far. Diagnosis for example of GH-deficiency to ensure optimal substitution with growth hormone is usually not early enough. A screening system using an auxological data bank for growth disorders has been set up by us recently. Since 1998 we have collected height and weight measurements in relation to age and sex from patients cared for by a large number of pediatricians in the region of Leipzig, Germany. By september 1999, 30,182 children of different ages have been screened. The system identifies children with short stature below the 3. centile and tall stature above the 97. centile of the German Synthetic Normcurve (Hermanussen/Burmeister 1999). In 6.81% of the children a growth disorder was identified (3.03% above 97. and 3.78% below 3. centile). In 54 cases (0.18%) we found a pathological reason for short or tall stature. A sensitive screening system has been set up for the early detection of growth disorders and obesity in childhood. We generate a data pool for multiple questions in research of human growth. We will generate yearly reports for the development of new strategies in preventive medicine.

Body Composition↗
PubMed1999

Preprotachykinin-A mRNA expression in the human and monkey brain: An in situ hybridization study.

The mRNA expression for preprotachykinin-A (PPT-A) was studied throughout the human and cynomolgus monkey brain to assess the neuroanatomical expression pattern of the PPT-A gene in primates. In situ hybridization showed that the PPT-A mRNA is expressed highly in specific regions of the postmortem human brain, including the striatum, islands of Calleja, hypothalamus (posterior, premammillary, medial mammillary, and ventromedial nuclei), superior and inferior colliculi, periaqueductal gray, and oculomotor nuclear complex. PPT-A mRNA-expressing neurons also were present in the paranigralis (ventral tegmental area) and were scattered in the bed nucleus stria terminalis throughout the sublenticular substantia innominata region, including the diagonal band of Broca and the nucleus basalis of Meynert. In the hippocampus, high PPT-A mRNA expression was localized predominantly to the polymorphic layer of the dentate gyrus; no labeled cells were present in the granular layer. Positively labeled cells also were found scattered in the CA regions as well as in the amygdaloid complex. Neocortical expression of PPT-A mRNA was localized mainly to the deep laminae (layers V/VI), except for the striate cortex (labeling was seen also in superficial layers). The subiculum, thalamus, globus pallidus, ventral pallidum, substantia nigra pars compacta, red nucleus, pontine nuclei, and cerebellum were characterized by very weak to undetectable expression of PPT-A mRNA. An expression pattern was evident in the monkey forebrain similar to that observed in the human, except for the absence of PPT mRNA-expressing cells in the medial mammillary nucleus despite intense expression in supramammillary, lateral mammillary, and premammillary nuclei. Overall, more similarities than differences are apparent between primate species in the expression pattern of the PPT-A gene. J. Comp. Neurol. 411;56-72, 1999.

Adult↗
PubMed1999

[Iatrogenic Cushing syndrome and mutatio tarda caused by dexamethasone containing nose drops].

Nasal steroid drops can produce iatrogenic Cushing's syndrome if the recommended dose is exceeded. We now report a 19-year-old male patient for whom dexamethasone nasal drops were prescribed because of nasal obstruction. He had been using these drops for more than 5 years, with a daily dexamethasone dose of 0.7-1.0 mg. It is likely that significant amounts of dexamethasone were swallowed and absorbed by the patient's gastrointestinal tract. As a consequence, the patient developed Cushing's syndrome with panhypopituitarism, growth retardation, osteoporosis and hypertension. A boyish size of the larynx resulted in a fundamental speaking frequency of a child. After discontinuing the intranasal use of the of dexamethasone and adjusting doses of hydrocortisone over 3.5 months a normalization of endocrine function occurred with the beginning of puberty and normal sexual changes. These findings show that the use of nasal steroid drops particularly in children should be limited to a short period and drugs with a low absorption dose employed.

Administration, Intranasal↗
PubMed1999

Early expression and high prevalence of islet autoantibodies for DR3/4 heterozygous and DR4/4 homozygous offspring of parents with Type I diabetes: the German BABYDIAB study.

AIMS/HYPOTHESIS: Islet autoantibodies precede the clinical onset of Type I (insulin-dependent) diabetes mellitus. The cumulative development of such autoantibodies in infants followed from birth and in particular infants with high-risk HLA genotypes is poorly defined, but such information is essential to design trials to prevent islet autoimmunity. METHODS: HLA genotypes were determined in offspring of parents with Type I diabetes who were followed from birth for at least 2 years (median follow-up: 3.1 years) and who were characterised for the expression of insulin, GAD65, IA-2 and islet cell autoantibodies at birth, 9 months, 2 and 5 years of age. RESULTS: The HLA genotypes DRB1*03/04(DQB1*57non-Asp) and DRB1*04/04(DQB1*57non-Asp) were present in 7.1% and 5.0% of offspring of parents with Type I diabetes. The frequency of both genotypes was increased in offspring who developed islet autoantibodies within the first 2 years of life (27.3% vs 5.5%, odds ratio 6.3 [p = 0.002] and 22.7% vs 4.2%, odds ratio 6.6 [p = 0.003]) and half of all offspring who developed antibodies had these genotypes. Other genotypes were not associated with an increase in risk. By life-table analysis, the cumulative risk of developing islet autoantibodies by the age of 2 years was 20% (95% CI 9.4,30.6) for offspring carrying either the DRB1*03104(DQB1*57non-Asp) or the DRB1*04/04(DQB1*57non-Asp) genotype compared with 2.7% (95% CI 1.2,4.2) for offspring without these genotypes (p < 0.0001). CONCLUSION/INTERPRETATION: These data show that early appearance of islet autoantibodies is remarkably frequent for DR3/4 heterozygous and DR4/4 homozygous offspring and indicate that primary prevention could be considered once available in an offspring cohort selected for these genotypes.

Adult↗
PubMed1999

MRI findings in Hirayama's disease: flexion-induced cervical myelopathy or intrinsic motor neuron disease?

Hirayama's disease is a benign juvenile form of focal amyotrophy affecting the upper limbs. Previous studies have suggested that the disorder is a neck flexion induced cervical myelopathy. We report clinical and magnetic resonance imaging findings in nine patients with Hirayama's disease. Cervical imaging of seven patients revealed spinal cord changes consisting of focal atrophy and foci of signal alterations. On neck flexion a forward movement and mild reduction in the anteroposterior diameter of the lower cervical cord against the vertebral bodies was noted in affected individuals as well as in five normal controls. In contrast to earlier reports, none of our patients showed complete obliteration of the posterior subarachnoid space. Measurement of the anteroposterior spinal cord diameter in each vertebral segment (C4-C7) revealed no significant differences in the degree of spinal cord flattening between the two groups. Furthermore, two of our patients had significant degenerative changes in the cervical spine (disc herniation, retrospondylosis) contralateral to the clinically affected side. These degenerative changes resulted in a marked cord compression on neck flexion but were not associated with ipsilateral clinical abnormalities or spinal cord alterations. Our results argue against a flexion-induced cervical myelopathy and support the view that Hirayama's disease is an intrinsic motor neuron disease.

Adolescent↗
PubMed1999

Titrimetric determination of Cremophor EL in aqueous solutions and biofluids: part 2: ruggedness of the method with respect to biofluids.

A titration method for Cremophor EL, as a multicomponent mixture commonly used as non-ionic emulgent for manufacturing certain parenteralia, was developed for quantitative routine analysis in biofluids. A coated wire electrode is used as the end-point indicator in potentiometric titrations of Cremophor EL with sodium tetraphenylborate. The method tolerates a broad pH range, addition of alkanols and components of drug formulations and is sufficiently rugged. Reliable results are obtained at 20 degrees C. Disturbing ions from biofluid matrices can be masked or complexed by addition of formaldehyde, ethylenediaminetetraacetic acid and sodium fluoride. Sodium hydroxide is used for the required adjustment of the samples to pH 10. Cremophor EL spiked urine samples can be determined directly, whereas the true value of the emulgent content in the case of Cremophor EL spiked plasma samples is achieved by means of a conventional method.

Alcohols↗
PubMed1999

Virtual DNA analysis as a platform for interlaboratory data exchange of HLA DNA typing results.

In 1998, the German DNA Exchange offered the possibility to report typing data as virtual DNA. Participating labs have been equipped with software based on the principle of Virtual DNA Analysis (VDA). This approach allows the combination of sequence-specific oligonucleotide (SSO), sequence-specific primer (SSP) and sequence-based typing (SBT) results. The use of all types of test kits has been allowed without any limitations, as long as basic sequence information on SSOs or SSPs was available, at least the sequence and the position of the detected motif on the sample DNA. Typing raw data of the actual SSO-SSP and, if performed, SBT information was collected. Participating labs received 20 DNA samples to type. Fourteen labs returned data on 1,250 single-locus testings. Reported data consisted of 317 SBT data, 452 SSO kits and 1,795 SSP kits with 43,312 single SSO/ SSP reactivities. One hundred and twenty-six different typing kits (unique laboratory-specific kits, commercial kits from 7 companies) have been used. In 30 (2.4%) single-locus testings, at least one single SSO/SSP reactivity has been false-positive or -negative, thus not leading to a valid result on primary evaluation. Eight of these 30 cases were due to the presence of a new DRB1*14 allele in sample no. 2. Thirty-five tests (2.8%) showed wrong allele assignments. This first attempt to collect raw typing data instead of typing interpretation on a larger scale shows the advantages of Virtual DNA Analysis like interlaboratory data exchange without loss of information, transparency of typing interpretation and reinterpretation of typing data with an updated allele database. The VDA format is a useful tool for workshops and bone marrow donor registries.

Alleles↗
PubMed1999

[The temporal changes in the diffusion coefficients in stroke].

PURPOSE: Analysis of temporal changes of ADC values at different stages after stroke. MATERIALS AND METHODS: The original images of all scans were evaluated and a region of interest analysis was performed on ADC (apparent diffusion coefficient) maps. 92 patients with symptoms of ischemic stroke were examined at 1.5 Tesla (ACS-NT, Philips Medical Systems). The study protocol included a navigated multishot SE-EPI DWI (b = 0, 189, 750 s/mm-2, TE = 120/140 ms, delta/delta = 49/60 ms), a GraSE (TR/TE = 3500/90 ms) and a FLAIR-TSE (TR/TE/TI = 6000/150/2000 ms) sequence. 8 patients had regular follow-ups. All patients had an additional CT scan. RESULTS: In hyperacute stroke (< 6 h, n = 10) the relative ADC of the infarct core dropped to about 50% and reached a minimum in the acute state (6-48 h, n = 14) during the next few hours. In subacute stroke (< 3 weeks, n = 29) the ADC began to rise significantly (p = 0.001) back to normal values and reached (p < 10(-4)) values similar to cerebrospinal fluid in the chronic state (> 3 weeks, n = 20). The combined use of DWI and conventional MRI improved the diagnostic information in 16% of all MR examination beyond the hyperacute assessment. CONCLUSION: By means of the ADC-values the age of stroke can be determined. Infarcts at different stages can be readily diagnosed with the combined use of DWI, FLAIR-TSE and GraSE. Pitfalls of DWI and conventional imaging can be avoided.

Adult↗
PubMed1999

[3D-TSE MR cholangiopancreatography with breath triggering to clarify unclear hepatopathies in children].

PURPOSE: Evaluation of 3D-TSE MR-cholangiography with respiratory triggering in the work up of hepatopathies in infants and young children. PATIENTS AND METHOD: 16 infants (4-16 years) with increased transaminases, two with recurrent pancreatitis, were examined at 1.5 T (ACS-NT II, Philips Medical Systems) using a 3D-TSE MRCP with respiratory triggering in addition to a regular MRI of the liver. The MRCP was compared to ERCP. Two radiologists and one gastroenterologist evaluated the technical quality, visualization of the pancreaticobiliary system, and the diagnostic value of the examinations. RESULTS: Technically feasible were 14/16 MRCPs and 13/16 ERCPs. Two MRCP were not of diagnostic value due to motion artifacts and in three ERCP cannulation of the papilla was not possible. 14/16 ERCP required general anaesthesia, while MRCP needed i.v. sedation in two patients only. Extrahepatic ducts/cystic duct/pancreatic duct were visualized in 14/12/8 patients using MRCP, and in 13/10/3 patients using ERCP, both without adverse effects or complications. Intrahepatic ducts were better delineated with MRCP. In 10 patients with histologically proven periportal fibrosis (n = 7) and liver fibrosis (n = 1) or antineutrophil cytoplasmatic antibodies and associated inflammatory bowel disease, MRCP and ERCP revealed pathological results. CONCLUSION: MRCP using a 3D-TSE sequence with respiratory triggering is a good non-invasive technique for delineation of the biliary tract in infants and young children for the work up to hepatopathies.

Adolescent↗
PubMed1999

[Prediction of the onset of voice mutation in singers of professional Boys' choirs: investigation of members of the Thomaner choir, Leipzig].

Die phoniatrische Betreuung der kindlichen Singstimme während des Stimmwechsels erscheint gerade bei Sängern in Knabenchören mit hoher stimmlicher Belastung wichtig. Die vorliegende Studie sucht nach Methoden, den Eintrittszeitpunkt der Mutation vorherzusagen und das Vorliegen einer Mutation differentialdiagnostisch von hyperfunktionellen Stimmstörungen oder entzündlichen Erkrankungen des Stimmorganes zu trennen. Dazu wurden 36 Knaben des Leipziger Thomanerchores im Sinne einer Longitudinalstudie alle 3 Monate über 3,5 Jahre bis zum hörbaren Einsetzen der Mutation untersucht. Es erfolgte die Bestimmung von zehn stimmlichen und acht stimmunabhängigen Parametern, die erstmals in solch umfassender Form auf ihre prädiktive Aussagekraft geprüft wurden. Die statistische Auswertung wies besonders den Serumtestosteronspiegel und die Wachstumsrate als Grössen aus, die mit dem Prämutationsverlauf korrelieren und eine konkrete Vorhersage ermöglichen. Das Vorliegen einer Mutation kann ausserdem durch den Verlauf der mittleren ungespannten Sprechstimmlage und unter Hinzuziehung des Genitalstatus bestätigt werden. Durch die exakte und prädiktive Bestimmung des Mutationsbeginnes können Erkrankungen des Stimmapparates vermieden werden, die aus dem intensiven Singen in der Mutation resultieren. Weiterhin geben die Ergebnisse dem Chorleiter wertvolle Informationen für die Planung der Besetzung seiner Knabenstimmen.

Adolescent↗
PubMed1999

Lipoprotein(a) levels in formerly small-for-gestational-age children.

Lipoprotein(a) (Lp(a)) is an independent and inherited risk factor for coronary artery disease. Concentrations of Lp(a) have been widely described in adolescents, but little is known about its concentration in children born small for gestational age (SGA). To assess the influence of intrauterine growth on Lp(a) levels we examined 50 children born SGA and 21 children born adequate for gestational age (AGA). Lp(a) blood levels (mean +/- SD) of the SGA children differed significantly (p < 0.05) from AGA children (22.3 +/- 22.1 vs. 10.9 +/- 7.6 mg/dl). 14 out of 50 adolescents of the SGA group but 1 out of 21 of the AGA group had elevated Lp(a) (>30 mg/dl) concentrations (p < 0.05). These children also had higher triglyceride (1.0 +/- 0.6 mmol/l vs. 0.74 +/- 0.38 mmol/l) levels (p < 0.05) compared to children with Lp(a) levels <30 mg/dl. Adolescents with Lp(a) levels >30 mg/dl showed a significant inverse relation between Lp(a) levels and gestational age (r = -0.68, p < 0. 005). We hypothesize that impairment of fetal growth might influence serum Lp(a) levels in later life.

Adolescent↗
PubMed1999

Gulliver G-100 - A new device to evaluate daily growth measurement in comparison with Harpenden stadiometer.

To reduce the time to monitor success of growth hormone therapy, Gulliver G-100 (G-100), a new portable height measurement device based on ultrasound technology, was developed and compared with a conventional determination system, the Harpenden stadiometer (HS). In addition, growth of 12 children was monitored at home twice per day 3 months before and 3 months during GH therapy. Mean body height of 101 children was 144.67 cm using G-100 compared to 145.16 cm using HS. The coefficient of variability of 3 measurements from each patient was 0.29 and 0.18 using G-100 or HS, respectively. Statistic analysis of these data revealed no significant difference between G- 100 or HS. Statistical analysis of short-time growth of 12 patients revealed an increase of growth velocity for 8 patients (p < 0.01) after 3 months. Calculated growth velocity using data revealed from short-time growth analysis with G-100 and using long-term growth analysis with HS did not show any significant difference. Our data reveal that G-100 is able to produce accurate results in height measurement comparable to the HS. Using G-100, the patient can be classified as a 'responder' of GH therapy already after 3 months.

Adolescent↗
PubMed1999

Norditropin SimpleXx: a liquid human growth hormone formulation, a pen system and an auto-insertion device.

Patient compliance is of vital importance for the outcome of any medical therapy. Compliance is especially a problem in long-term treatment of non-life threatening diseases, such as growth retardation in children. Until recently, all human growth hormone (hGH) products required a reconstitution process. Norditropin((R)) SimpleXx(TM) is a liquid formulation of the biosynthetic hGH product Norditropin((R)), and, together with an improved NovoPen((R)) 1.5, NordiPen(TM), and an auto-insertion device, PenMate(TM)/NordiPenMate(TM), it has been developed in order to ease the injection process for patients. A randomized, open, multicentre, crossover trial compared Norditropin((R)) SimpleXx(TM)/improved NovoPen((R)) 1.5 with freeze-dried Norditropin((R)) PenSet((R))/Nordiject((R)). A total of 67 children with GH deficiency, aged 5-18 years, were treated with either Norditropin((R)) SimpleXx(TM) for 6 weeks followed by Norditropin((R)) for 6 weeks or the opposite (sequences I and II, respectively). Acceptability/convenience and pain perception were evaluated by questionnaire after each period. The function and handling of the PenMate(TM) were evaluated in a Dutch trial by 27 GH-treated children with intrauterine growth retardation, aged 4-16 years, and their parents. All children were accustomed to using the Nordiject((R)) pen. The evaluation of the PenMate(TM) was based on a questionnaire. A similar trial was conducted in England, in which the NordiPen(TM) and the NordiPenMate(TM) were evaluated by 25 GH-treated children and their parents. Norditropin((R)) SimpleXx(TM) was found to be easier to inject by 64% of the children, and 98% of the children found the system easier to use overall. There was no difference in pain perception between the two administration systems, as judged by questionnaires and visual analogue scale score. Three out of four patients preferred to continue treatment with Norditropin((R)) SimpleXx(TM). The safety profiles of the two systems were similar. In the Dutch trial, the PenMate(TM) was found to be easy and safe to handle, even for very young children (aged 4-5 years). Of patients who took a long time to get used to the injections, 73% found that the new pen would help. A total of 88% of the children would prefer to use the PenMate(TM) in the future. Positive results of the handling tests were also reported in the British trial. The use of Norditropin((R)) SimpleXx(TM) and the auto-insertion device may improve patient compliance.

Adolescent↗
PubMed1999

Diffusion- and perfusion-weighted magnetic resonance imaging in deep cerebral venous thrombosis.

BACKGROUND: Diffusion-weighted (DWI) and perfusion-weighted (PI) MRI are highly sensitive techniques for early diagnosis of arterial infarction, but little data on venous cerebral ischemia are available. We describe a case in which DWI, PI, and fast T2-weighted sequences were performed in the acute phase of deep cerebral venous thrombosis (CVT). CASE DESCRIPTION: An 11-year-old girl with Crohn's disease developed deep CVT in which extensive edema was shown in the deep gray matter on T2-weighted sequence images. Isotropic echo-planar DWI demonstrated a local augmentation of the apparent diffusion coefficient (1.1 to 1.6x10(-3) mm2/s), consistent with vasogenic edema. In dynamic contrast-enhanced PI, the regional cerebral blood volume was increased and the passage time of the contrast bolus was markedly prolonged. Clinically, the patient recovered totally after intravenous full-dose heparinization. T2 abnormalities, apparent diffusion coefficient values (0.8 to 0.92x10(-3) mm2/s), and brain perfusion alterations resolved without damage to brain tissue. CONCLUSIONS: Unlike arterial infarction, DWI demonstrated vasogenic edema in a patient with deep CVT, which proved to be reversible in follow-up magnetic resonance imaging. PI showed areas with extensive venous congestion, but perfusion deficits were missing. Therefore, we believe that DWI and PI may play a role in detecting venous congestion in CVT and in prospective differentiation of vasogenic edema and venous infarction.

Brain Edema↗