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Biomedical subjects

E Thiele

Publications and source records attributed to E Thiele.

At least 19 recordsLinked to original sources
PubMed2001

Topiramate and metabolic acidosis in pediatric epilepsy.

PURPOSE: Topiramate (TPM) has been widely used as an adjunctive therapy for treating epilepsy. TPM is reported to have multiple mechanisms of action, including inhibition of carbonic anhydrase, which may result in metabolic acidosis from decreased serum bicarbonate (HCO3-). METHODS: Clinical data from 30 children who received TPM as adjunctive therapy for medically refractory epilepsy were reviewed at Children's Hospital, Boston. Serum HCO3- levels were assessed before, during, and after discontinuing TPM (n = 9). When multiple data were available, mean values were used for analysis. RESULTS: Of the 30 patients, 21 had a >10% decrease in HCO3- levels. The mean decrease in HCO3- among the 21 patients was 4.7 mEq/L, and maximum was 10 mEq/L. No clinical symptoms occurred, and HCO3- supplement was not needed, except for one patient who developed tachypnea from worsened acidosis after prolonged status epilepticus during a suspected viral illness. Among the 21 patients, TPM was discontinued in seven children because of a lack of efficacy, and in two because of anorexia. After discontinuing TPM, the serum HCO3- returned to the previous level before starting TPM in all nine. CONCLUSIONS: Decreased HCO3- levels occurred in the majority of patients reviewed, usually only to a small to moderate extent, but by 8 and 10 mEq/L in two cases. In patients at risk for acidosis, the decrease in HCO3- may cause significant consequences, such as severe acidosis or renal calculi. Monitoring HCO3- levels before and during TPM therapy may be indicated, especially with conditions that predispose to acidosis.

Acidosis↗
PubMed2000

Mutational analysis in a cohort of 224 tuberous sclerosis patients indicates increased severity of TSC2, compared with TSC1, disease in multiple organs.

Tuberous sclerosis (TSC) is a relatively common hamartoma syndrome caused by mutations in either of two genes, TSC1 and TSC2. Here we report comprehensive mutation analysis in 224 index patients with TSC and correlate mutation findings with clinical features. Denaturing high-performance liquid chromatography, long-range polymerase chain reaction (PCR), and quantitative PCR were used for mutation detection. Mutations were identified in 186 (83%) of 224 of cases, comprising 138 small TSC2 mutations, 20 large TSC2 mutations, and 28 small TSC1 mutations. A standardized clinical assessment instrument covering 16 TSC manifestations was used. Sporadic patients with TSC1 mutations had, on average, milder disease in comparison with patients with TSC2 mutations, despite being of similar age. They had a lower frequency of seizures and moderate-to-severe mental retardation, fewer subependymal nodules and cortical tubers, less-severe kidney involvement, no retinal hamartomas, and less-severe facial angiofibroma. Patients in whom no mutation was found also had disease that was milder, on average, than that in patients with TSC2 mutations and was somewhat distinct from patients with TSC1 mutations. Although there was overlap in the spectrum of many clinical features of patients with TSC1 versus TSC2 mutations, some features (grade 2-4 kidney cysts or angiomyolipomas, forehead plaques, retinal hamartomas, and liver angiomyolipomas) were very rare or not seen at all in TSC1 patients. Thus both germline and somatic mutations appear to be less common in TSC1 than in TSC2. The reduced severity of disease in patients without defined mutations suggests that many of these patients are mosaic for a TSC2 mutation and/or have TSC because of mutations in an as-yet-unidentified locus with a relatively mild clinical phenotype.

Adolescent↗
PubMed1996

Functional measuring of muscle tone.

Testing muscle fields requires functional conditions. The procedure for measuring has to put the musculature under conditions as they occur in physiological functioning. Furthermore a measuring instrument should be economical and easy to handle in the course of the daily routine. The Myofunctional Therapist presently has a number of measuring instruments available. Some of them need to be adjusted before every operation. Others give only comparative readings, having no absolute reference. Others measure states which do not represent normal functions of the musculature and therefore do not allow conclusions about the state of the muscle in relation to function. Some apparatuses are highly sensitive and can only be handled by highly trained persons. In this category we find the highly precise electronic equipment being highly expensive to purchase and to repair. It seems that the Function Tonometer is in all points compatible when waging the pros and cons. It directly reads the actual muscle tone under conditions which occur during normal function. The device can be used for diagnostics and documentation as well as in functional training. The output is a defined mechanical range (pressure in mbar) and needs not be adjusted before each measurement. Used in treatment, it motivates by visual feedback. The results are reproducible at any time, at any location, and by any person, even young patients. The instrument has an easy handling, favorable costs in purchase and operation, and is not easily damaged. The assembly is not electrically operated and thus is not under restrictive rules nor the need of an energy supply. The Function Tonometer is indeed a flexible and useful tool to aid in diagnosis, documentation, and treatment procedures.

Equipment Design↗
PubMed1991

[Chronic aspecific respiratory tract diseases in workers of pig-breeding farms].

To study chronic nonspecific respiratory diseases of people working in industrial swine confinement buildings, a cross sectional study was initiated. Based on history of diseases, clinical and parameters of ventilatory screening in 238 swine producers a frequency of chronic respiratory symptoms were found in 25.6%. The possible dependence on age, sex, smoking history, working conditions and duration of activity was investigated. The influence of professional and non professional factors had different intensity. The results of investigations of working conditions did not exceed the permitted maximal working place concentrations. So they could not prove the large frequency of respiratory diseases.

Age Factors↗
PubMed1990

Prospective study of the incidence of ultrasound-detected hepatic hematomas 2 and 24 hours after percutaneous liver biopsy.

Recently, a high incidence of hepatic hematomas following percutaneous liver biopsy was reported. This induced us to undertake this prospective ultrasound examination before, as well as 2 and 24 h after, percutaneous liver biopsy, using a Menghini needle (O 1.4 mm), in 93 patients for diagnostic purposes. Two hours after biopsy a small subcapsular fluid margin was found in one patient (1.1%) which disappeared within 24 h. We conclude that hepatic hematomas are not frequent following percutaneous liver biopsy and that it remains a safe diagnostic procedure to find out the reason for chronically elevated liver enzymes and evaluate suspected diffuse parenchymal lesions of the liver.

Adolescent↗
PubMed1990

[Permissible dermal exposure dose and safety level for herbicide residues as an occupational hygiene standard for the evaluation of exposure].

At preparing mixtures for spraying, application of pesticides and entering treated areas workers are subject of potential exposure, more than 90% via skin. The limitation of dermal exposure requires hygiene-toxicological threshold limit values. On the basis of toxicity data from experiments with animals a permissible dermal exposure dose for pesticide residues is determined. For that a safety and a resorption factor are used, which are fixed in dependence on the toxic effects. The permissible dermal exposure dose is the basis of the calculation of a safety value for residues on plant surfaces and reentry time.

Environmental Exposure↗
PubMed1989

[Results of occupational medicine pesticide research].

The conditions of the pesticide exposition in agriculture are described, including results of analyses of acute consequences of expositions to selected agents as well as in workers exposed to pesticide application for several years.

Agricultural Workers' Diseases↗
PubMed1985

[Respiratory function disorders caused by agrochemical exposure].

With the aim to evaluate possible influences of exposition to agrochemicals upon the respiratory system, the ventilatory parameters VC, FEV1,0, FIV1,0, and the values of PaO2 and PaCO2 were investigated in 93 males professionally exposed to mineral fertilizers, and in 69 males professionally exposed to pesticides--both groups working in agrochemical centres. A control group consisted of 162 males without exposition to agrochemicals. In exposed workers mean values of VC, FEV1,0, and PaO2 were lower (highly significant) compared with the control group. Mean values of FEV1,0, FIV1,0, and PaO2 in workers exposed to mineral fertilizers were in the borderline region. Influences upon the respiratory system should be further investigated with regard to long-term effects and to fitness for the job.

Adult↗