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Biomedical subjects

B Roth

Publications and source records attributed to B Roth.

At least 73 records · Page 4Linked to original sources
PubMed1995

Proton magnetic resonance spectroscopy reflects metabolic decompensation in maple syrup urine disease.

Using localized proton magnetic resonance spectroscopy (1H-MRS), accumulation of branched-chain amino acids (BCAA) and their corresponding 2-oxo acids (BCOA) could be non-invasively demonstrated in the brain of a 9-year-old girl suffering from classical maple syrup urine disease. During acute metabolic decompensation, the compounds caused a signal at a chemical shift of 0.9 ppm which was assigned by in vitro experiments. The brain tissue concentration of the sum of BCAA and BCOA could be estimated as 0.9 mmol/l. Localized 1H-MRS of the brain appears to be suitable for examining patients suffering from maple syrup urine disease in different metabolic states.

Adolescent↗
PubMed1995

Ketamine and strychnine treatment of an infant with nonketotic hyperglycinaemia.

UNLABELLED: Non-ketotic hyperglycinaemia (NKH) is a severe seizure disorder associated with high glycine levels. Glycine is a major inhibitory neurotransmitter in the CNS, but has also modulating effects at one of the glutamate receptors, the N-methyl-D-aspartate-(NMDA) receptor. Based on this knowledge we treated a female newborn suffering from severe NKH with the NMDA receptor blocker ketamine in association with strychnine and magnesium supplementation. This treatment led to cessation of seizures, reappearance of swallowing and sucking and improved the neurological status. Some pharmacokinetic data of strychnine and ketamine in the infant are given. CONCLUSION: Ketamine in combination with strychnine may be beneficial in non-ketotic hyperglycinaemia.

Amino Acid Metabolism, Inborn Errors↗
PubMed1995

[Obstetric prognostic factors of newborn infants with very low birth weight (< or = 1,500 gram) with reference to survival rate and early childhood development].

Prognostic factors influencing survival in 235 very low birthweight prematures (< or = 1500 g) born between 1986 and 15.11. 1993 at the Department of Obstetrics and Gynaecology, University Hospital of Cologne, were retrospectively evaluated. Chromosomal anomalies and severe congenital malformations were excluded. Of 180 singletons 84 were classified as appropriate-for-gestational-age (AGA) and 96 as small-for-gestational-age (SGA). By interrogating the attending paediatricians data regarding the early development of 62/65 surviving singletons born between 1986 to 1990 were recorded (follow-up rate 95%). Survival was significantly correlated to singleton pregnancy (p < 0.05), female sex (p = 0.001) and in the AGA-prematures to prenatal corticoid prophylaxis. With similar mean birthweight SGA-singletons showed a three weeks higher mean gestational age; the mortality showed an inverse correlation to birthweight and gestational age being 11% higher in the AGA-group compared with the SGA-group (32% versus 21%). At the age of between 11 months and 6 years severe handicaps and developmental retardations were found more often in previous AGA-prematures (6/26) than in previous SGA-prematures (4/36); type and degree of later handicap were not correlated to birthweight. According to our results survival rates of very low birthweight prematures are strongly influenced by singleton pregnancy, by fetal sex, by gestational age and in the AGA-group by prenatal corticoid prophylaxis; mortality shows an inverted correlation to birthweight and gestational age, whereas the later prognosis of survivors does not seem to be influenced by birthweight or gestational age.

Adult↗
PubMed1995

The importance of colloid osmotic pressure measurements to prevent oncotic overdosage during cardiac surgery.

OBJECTIVE: Colloidal infusion therapy during cardiac surgery has changed from the principal use of human albumin to the preference for synthetic colloids. Despite the possible interference of synthetic plasma expanders with the biuret determination of total plasma protein (TPP), perioperative infusion therapy is frequently directed on the basis of TPP and albumin levels. The hypothesis that the level of TPP or albumin does not reflect the plasma colloid osmotic pressure (COP) if synthetic plasma expanders are used was studied. MATERIALS AND METHODS: In 61 patients undergoing elective cardiac surgery the course of COP and its correlation to the TPP and albumin levels were investigated. Natural and artificial colloids were used for colloidal infusion therapy. RESULTS: No correlation between TPP/albumin levels and COP was found preoperatively and on arrival at the ICU, only a weak correlation was observed at 24 hours and 48 hours postoperatively. The wide range of the confidential interval indicates that the COP cannot be estimated correctly neither from the TPP nor the albumin level. The postoperative COP was significantly increased compared to the preoperative levels indicating oncotic overdosage. CONCLUSIONS: In order to avoid oncotic disturbances, indication for colloidal volume replacement during cardiac surgery should be controlled by oncometry if natural and synthetic colloids are administered.

Blood Proteins↗
PubMed1994

Clinical experiences with high-frequency oscillatory ventilation in newborns with severe respiratory distress syndrome.

OBJECTIVE: To generate hypotheses about which subgroups of newborns with severe respiratory distress syndrome might benefit most from high-frequency oscillatory ventilation. DESIGN: Retrospective analysis of a case series of newborns with severe respiratory distress syndrome who were treated in our department with high-frequency oscillatory ventilation. SETTING: Referral center for neonatal and pediatric intensive care medicine. PATIENTS: All newborns (n = 18), admitted between June 1991 and February 1993, of various gestational ages (26 to 41 wks), with severe respiratory distress syndrome caused by various underlying pulmonary diseases who did not respond to conventional therapy and who thus were treated with high-frequency oscillatory ventilation. MAIN OUTCOME MEASURES: Survival until discharge from our unit and persistent improvement of gas exchange. RESULTS: Eight (44%) of 18 patients survived; ten (55%) patients died. Four (22%) survivors showed marked clinical improvement with the initiation of high-frequency oscillatory ventilation. Four (22%) survivors did not respond to high-frequency oscillatory ventilation. The responder group consisted of term or near-term neonates (gestational age at least 35 wks) with pulmonary disease that was complicated by persistent pulmonary hypertension. The group of premature neonates with a gestational age of < 35 wks did not respond to high-frequency oscillatory ventilation. CONCLUSIONS: As a result of our analysis, we hypothesize that term newborns with severe respiratory distress syndrome complicated by persistent pulmonary hypertension and hypercarbia can benefit from high-frequency oscillatory ventilation. Premature neonates with ventilation-induced lung injury are not likely to respond to high-frequency oscillatory ventilation.

Female↗
PubMed1994

Value of PCR for evaluating occurrence of parasitemia in immunocompromised patients with cerebral and extracerebral toxoplasmosis.

PCR was used to evaluate the occurrence of Toxoplasma gondii parasitemia by detection of the B1 gene in blood samples in two groups of immunosuppressed patients (148 subjects) suspected of having cerebral or extracerebral infection, respectively. Group I consisted of 52 patients with AIDS with suspected cerebral toxoplasmosis. The diagnosis was clinically proven in 15 cases. Parasitemia was detected by PCR in only two of these patients (13.3%), both showing evidence of disseminated infection. Group II consisted of 96 immunocompromised patients, either with AIDS or receiving iatrogenic immunosuppressive therapy. Of these patients, 65 (34 with AIDS and 31 others) showed abnormalities only in chest radiography and were first screened for the presence of Toxoplasma DNA in bronchoalveolar lavage fluid. Blood was then analyzed when the parasite was detected in the bronchoalveolar lavage fluid. The remaining 31 subjects (22 with AIDS and 9 others) were suspected of having extracerebral, pulmonary, or disseminated toxoplasmosis, and blood was studied directly in these cases. Among the nine patients with clinically diagnosed extracerebral infection in group II, the parasite was detected by PCR in the blood of five patients (55.5%), all having pulmonary toxoplasmosis. If all patients with clinical manifestations of extracerebral toxoplasmosis (from both groups) who had not received antitoxoplasma therapy when the samples were collected are considered, PCR detected parasitemia in seven of the nine cases (77.8%). The present study indicates that examination of blood by PCR may be valuable in cases of extracerebral toxoplasmosis because of the disseminated nature of the disease. Since most cases of cerebral toxoplasmosis result from the local reactivation of latent brain cysts, detection of parasitemia by PCR is useful only in cases associated with severe cerebral infection or dissemination of this disease.

Acquired Immunodeficiency Syndrome↗
PubMed1994

Misuse of metered-dose inhalers in hospitalized patients.

Metered-dose inhalers (MDIs) have been associated with a high rate of misuse. Medical personnel also have a poor understanding of MDI technique. Hospitalized patients had their MDI technique observed before and after a series of inservices were provided to hospital personnel on correct MDI use. The rate of misuse did not change with 55 of 72 (76 percent) patients making errors in the first group before, and 45 of 55 (82 percent) patients making errors in the second group after staff education (p = 0.46). The average number of errors per patient was 2.39 in group 1 and 2.45 in group 2 (p = 0.93). Alternatives to deal with this high rate of MDI misuse are discussed.

Administration, Inhalation↗
PubMed1993

Late developmental changes of the innervation densities of the myelinated fibres and the outer hair cell efferent fibres in the rat cochlea.

The baso-apical distributions of the myelinated nerve fibres (representative for the inner hair cell afferent fibres) and the outer hair cell efferent fibres were studied during postnatal development of the rat cochlea. The myelinated fibres were counted in the primary osseos spiral lamina from semi-thin sections. The outer hair cell efferent fibres were counted in the tunnel of Corti by means of ultra-thin sections. The developmental changes of the myelinated fibres were investigated between 8 and 60 days after birth (DAB); those of the outer hair cell efferent fibres between 20 and 30 DAB. Between 12 DAB (onset of hearing) and 20 DAB the baso-apical distribution of the myelinated fibres does not change. Striking maturational changes occur later after the onset of hearing, between 20 and 30 DAB. The innervation density of the myelinated fibres increases in the lower middle region of the cochlea. In this region a maximum of innervation density appears. The efferent fibres to the outer hair cells show at 20 DAB a maximum of innervation density in the middle of the cochlea but between 20 and 30 DAB, the fibre density decreases in this region. During the same period the maximum of innervation density shifts towards the base. The change in the innervation densities of the myelinated fibres and the outer hair cell efferent fibres occurs late in development, after the onset of hearing, and after the organ of Corti shows an adult-like appearance.

Animals↗
PubMed1993

Possible role of short-term parenteral nutrition with fat emulsions for development of haemophagocytosis with multiple organ failure in a patient with traumatic brain injury.

We describe a case of life-threatening haemophagocytosis after a short term nutrition with fat emulsion in a 21-year-old woman who sustained an isolated traumatic brain injury. Hypertriglyceridemia and "creaming plasma" were observed after a three-day period of parenteral fat nutrition (Intralipid 20%). She also developed rash, high fever (40-41 degrees C), hypertension, raised intracranial pressure, hepatic and renal failure, haemolysis, marked thrombocyto- and leucopenia, coagulation disorder and pulmonary failure. These symptoms, together with a typical bone marrow smear, indicated haemophagocytosis with hyperactivation of the monocyte-macrophage system. We suggest that the hyperactivation was an effect of fat retention or agglutination of the fat particles; the initial triggering mechanism may emanate from the brain damage by hypercytokinaemia. The steroid treatment given most likely contributed to the successful outcome, as indicated by the stepwise improvement related in time to the steroid infusions.

Adult↗
PubMed1993

Holocarboxylase synthetase deficiency: early diagnosis and management of a new case.

We present a new case of holocarboxylase synthetase (HCS) deficiency, a rare autosomal recessive metabolic disorder, causing the "early-onset" form of multiple carboxylase deficiency. The patient was born at term of healthy consanguineous parents after an uncomplicated pregnancy. On the 2nd day of life she refused oral feeding, became tachydyspnoeic and showed excessive weight loss. Laboratory studies showed metabolic acidosis, marked lactic acidaemia, hyperammonaemia and increased urinary excretion of 3-hydroxyisovaleric acid, 3-methylcrotonylglycine, 3-hydroxpropionic acid and methylcitric acid. Peritoneal dialysis combined with oral supplementation of biotin (10 mg/day) started on the 3rd day of life resulted in rapid clinical recovery and normalisation of biochemical parameters. HCS deficiency was established in lymphocytes and skin fibroblasts. The activities of all biotin-dependent carboxylases were severely decreased in fibroblasts grown in medium with moderate biotin concentration (10(-8) mol/l) but normal in a high biotin medium (10(-5) mol/l). Mitochondrial carboxylase activities in lymphocytes were 23%-29% of mean normal during therapy with 20 mg of biotin/day, with the higher dose of 40 mg/day they were within (3-methylcrotoryl-CoA carboxylase, pyruvate carboxylase) or slightly below (propionyl-CoA carboxylase) the normal range. At the age of 3 years the patient's physical and psychomotor development are normal. Early biotin supplementation should be considered in newborns with lactic acidosis and organoaciduria until a final diagnosis has been established. Furthermore, the required individual dose of biotin has to be carefully evaluated biochemically for the individual patient.

Biotin↗
PubMed1993

Airway endoscopy in the diagnosis and treatment of bacterial tracheitis in children.

Children with bacterial tracheitis present with the symptoms of viral laryngotracheobronchitis or epiglottitis, but do not respond to appropriate therapy for these diseases and frequently develop acute respiratory decompensation. Since the treatment and outcome of bacterial tracheitis differ so much from those of viral laryngotracheobronchitis and epiglottitis, prompt and accurate diagnosis is essential. The aim of this study was to evaluate the significance of different diagnostic characteristics in a group of eleven patients and to compare the results to those recently reported in the pediatric and otorhinolaryngologic literature. The present study suggests that reliable predictive factors do not exist for bacterial tracheitis. No single clinical, radiological or laboratory feature was a reliable diagnostic predictor for bacterial tracheitis, nor was it any combination of these features. The only diagnostic procedure to distinguish bacterial tracheitis accurately and promptly from other forms of acute obstructive upper airway diseases was direct laryngo-tracheo-bronchoscopy. Following endoscopic removal of all tracheal secretions and pulmonary toilet, nasotracheal intubation provides sufficient airway maintenance and obviates the need for tracheostomy. Endoscopy is thus diagnostic and therapeutic at the same time. If bacterial tracheitis is suspected a direct laryngoscopy and rigid tracheobronchoscopy should be performed under general anesthesia, as prompt diagnosis and adequate treatment are essential to survival. The cultures of the purulent tracheal secretions frequently revealed Staphylococcus aureus in combination with various pathogens, particularly the involvement of Pseudomonas aeruginosa was noted in two patients. Our data imply a susceptibility of children with Down's syndrome or immunodeficiency to bacterial tracheitis.

Acute Disease↗
PubMed1993

Fertility awareness as a component of sexuality education. Preliminary research findings with adolescents.

Fertility awareness refers to the observation and interpretation of cervical mucus, often called vaginal discharge. A woman's cervical-mucus pattern indicates the time of ovulation and differentiates the fertile and infertile phases of the menstrual cycle from each other. Fertility awareness enables a woman to know when pregnancy can and cannot occur on a daily basis during each menstrual cycle. There has been, to date, almost no exploration of the appropriateness of fertility-awareness instruction for adolescents. A review of the literature on adolescent cognitive development, sexual activity, knowledge of fertility and contraceptive risk-taking behavior is presented. Based on the literature review, a theoretical rationale for fertility-awareness instruction as a unique sex-education curriculum for adolescents is proposed. The content and teaching techniques of a fertility-awareness presentation for teenagers is described. Directions for future research in fertility-awareness instruction for teenagers, and the need for long-term follow-up to assess the effects of such education on teenagers' sexual activity and contraceptive use are discussed.

Adolescent↗
PubMed1993

Expression of the two maize TATA binding protein genes and function of the encoded TBP proteins by complementation in yeast.

A single gene encodes the TATA binding protein (TBP) in yeasts and animals. Although two TBP-encoding genes (Tbp) previously were isolated from both Arabidopsis and maize, the expression and in vivo function of the encoded plant TBPs were not investigated. Here, we report that the two highly conserved maize Tbp genes are unlinked and reside within larger, ancestrally duplicated segments in the genome. We find quantitative differences in Tbp1 versus Tbp2 transcript accumulation in some maize tissues. These nonidentical expression patterns may indicate differences in the tissue-specific regulation of these genes, which might allow the two encoded maize TBP isoforms to perform nonoverlapping functions in the plant. In addition, we show that the maize TBP products, unlike animal TBPs, are functionally interchangeable with yeast TBP for conferring yeast cell viability. This is a conclusive demonstration of in vivo activity for a nonyeast TBP protein, and these complementation results point to particular amino acids in TBP that are likely to influence species-specific protein interactions.

Amino Acid Sequence↗
PubMed1992

Postnatal development of the rat organ of Corti. I. General morphology, basilar membrane, tectorial membrane and border cells.

The development of the rat organ of Corti was studied during the first postnatal weeks. The temporal and the spatial patterns of cochlear development were investigated between 4 and 24 days after birth by means of semi-thin sections at approx. ten equidistant positions along the entire cochlear duct. At all examined positions width, thickness and cross sectional area of basilar membrane, cross-sectional area of tectorial membrane, of cells of Hensen, Claudius and Boettcher and of the organ of Corti were quantitatively analyzed. The most conspicuous maturational changes occur between 8 and 12 days after birth. These are the detachment of the tectorial membrane, the first appearance of filaments within the basilar membrane, the formation of the tunnel of Corti and the opening of the inner spiral sulcus. Quantitative analysis revealed that structures of a given position along the cochlear duct do not develop synchronously. Width of the basilar membrane and cross-sectional area of the tectorial membrane are already mature at the onset of hearing (10-12 days after birth). Length, thickness and cross-sectional area of the basilar membrane as well as cross-sectional area of the organ of Corti and of the cells of Hensen, Claudius and Boettcher still develop after the onset of hearing (up to 20-24 days after birth). We suggest that basic cochlear function is established by structures which are mature before the onset of hearing. Cochlear structures which develop after the onset of hearing might be involved in this improvement during this period.

Animals↗
PubMed1992

Postnatal development of the rat organ of Corti. II. Hair cell receptors and their supporting elements.

The development of cochlear receptor cells and their supporting elements was studied by means of semi-thin and ultra-thin sections during the first postnatal weeks in the rat. The temporal and spatial patterns of the receptor cell development were investigated between the 4th and 24th days after birth. At approx. ten equidistant positions along the entire cochlear duct length of inner and outer hair cells, width of outer hair cell triad and stereocilia-length of the outer hair cells were quantitatively analyzed. Striking maturational changes take place before the 12th day after birth, that is, when the onset of hearing occurs. These changes are the formation of the tunnel of Corti, of the Nuel spaces, the appearance of filaments within the supporting elements and the change in cell shape of the hair cells. Between 4 days and 20 days after birth the maturation of outer hair cells is characterized by a decrease of organelles in the cytoplasm and establishment of the subsurface cistern. The quantitative analysis revealed a unique developmental pattern of the length of the outer hair cells, the width of the outer hair cell triad and the stereocilia length of the outer hair cells. Shortly after birth these structures have an almost constant size along the whole cochlear duct, but with increasing age the structures shorten at the cochlear base and enlarge at the apex. This pattern results in the establishment of a baso-apical gradient of the above mentioned structures. We assume that this baso-apical gradient is of central importance for the frequency representation.

Animals↗
PubMed1992

Cerebral reperfusion in brain death of a newborn. Case report.

A case of "sudden infant death" after 15 minutes of successful resuscitation of cardiovascular function is presented. While apnoic cranial nerve areflexia and electrocerebral silence persisted, angiography and transcranial Doppler sonography demonstrated nearly normal cerebral perfusion, which even increased day by day inspite of the persistence of other signs of brain death. The phenomenon "cerebral reperfusion" is concluded to be compatible with the diagnosis of brain death.

Blood Flow Velocity↗