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Biomedical subjects

S Meyer

Publications and source records attributed to S Meyer.

At least 343 records · Page 19Linked to original sources

[The importance of conventional radiographs in the diagnosis of osteosarcoma].

We report about a 12-year-old boy with a history of recurrent patella luxation. Due to persistent pain in the distal left femur a MRI examination was performed in another hospital which suggested a malignant bone tumor. Without validation of the MRI findings by conventional radiographs bone biopsy was performed. Histopathological examination yielded the diagnosis of a chondroblastic osteosarcoma. Before initiating polychemotherapy, plain radiographs for the first time, a nuclear imaging study and an additional MRI examination were performed in our hospital. The results of these studies made the diagnosis of an osteosarcoma unlikely. In particular, plain radiographs did not show any osseous lesion which was characteristic of an osteosarcoma. To establish a definite diagnosis biopsy was repeated with resection of the bone area which showed suspicious changes in MRI studies. An osteosarcoma was ruled out by histopathological examination. The pathologic changes detected in MRI were rated as bone bruise on plain radiographs and seemed to be of traumatic origin. Our case report emphasises the importance of conventional radiographs in establishing the diagnosis of an osteoarcoma respectively bone tumors and tumor-like lesions in general. They still remain the mainstay in diagnosing bone forming tumors. MRI imaging studies may show changes which mimick solid lesions but in deed can be of traumatic origin. Without informing the pathologist about the exact origin of the specimen, histopathological examination may lead to the misdiagnosis of a chondroblastic osteosarcoma if specimen, like in this case report, represents epiphyseal tissue showing cartilaginous areas with reactive bone formation.

Biopsy↗

[Solid-pseudopapillary tumor of the pancreas in a 9-year-old girl].

Solid-pseudopapillary tumor of the pancreas constitutes a very rare benign or low-grade malignant lesion occurring most commonly in young women and girls. It was first described by Frantz. Local infiltration, distant metastasis and recurrence are very rare. Until today, the histogenetic origin of the tumor cell remains to be elucidated. In 1996, solid-pseudopapillary tumor of the pancreas was introduced in the World Health Organization (WHO) classification of tumors of the exocrine pancreas. Our case report--like a recently published work by Lange et al.--intends to underline the significance of solid-pseudopapillary tumor in the differential diagnosis of a pancreatic mass.

Biomarkers, Tumor↗

[Vanishing bile duct syndrome following allogeneic bone marrow transplantation].

BACKGROUND: Cholestasis following bone marrow transplantation is not rare, but should always be a warning signal. The main causes of cholestasis following bone marrow transplantation are hepatotoxic effects of drugs, venoocclusive disease (VOD), early graft-versus-host disease (GvHD), total parenteral nutrition, viral hepatitis, sepsis and opportunistic infections. Vanishing bile duct syndrome represents a very rare cause for cholestatic symptoms. CASE REPORT: We report on a 8-year-old boy suffering from myelodysplastic syndrome, who underwent allogeneic bone marrow transplantation and developed biliary ductopenia in terms of a vanishing bile duct syndrome. CONCLUSION: Differential diagnosis for cholestasis following bone marrow transplantation should include the vanishing bile duct syndrome.

Bile Ducts↗

Gamma-hydroxybutyrate versus chlorprothixene/phenobarbital sedation in children undergoing MRI studies.

BACKGROUND: Few clinical studies have assessed gamma-hydroxybutyrate and chlorprothixene/phenobarbital sedation in children. This prospective trial compared the two regimes in children, in particular concerning differences in recovery time. METHODS: 28 pediatric oncology patients undergoing elective MRI studies at a university hospital were randomly assigned to either receive gamma-hydroxybutyrate or chlorprothixene/phenobarbital sedation. Time to induce deep sedation (Ramsay score of 5) and recovery time, the incidence of failure of sedation, the frequency of side effects, the need for therapeutic interventions, and the number of patients receiving additional midazolam were recorded. Analysis of hemodynamic parameters was performed at five defined time points. RESULTS: All 28 MRI studies were successfully completed. Recovery time was significantly shorter with gamma-hydroxybutyrate (p < 0.01). There were more side effects with chlorprothixene/phenobarbital, in particular tachycardia and hyperexcitation. Vomiting was the side effect most often seen in gamma-hydroxybutyrate sedation. Therapeutic interventions were not required in any patient. Additional midazolam was necessary to maintain satisfactory sedation in six children receiving gamma-hydroxybutyrate and four receiving chlorprothixene/phenobarbital. CONCLUSIONS: Due to its significantly shorter recovery time, gamma-hydroxybutyrate is a reasonable sedative drug for children undergoing non-invasive diagnostic procedures, and is superior to chlorprothixene/phenobarbital. In pediatric oncology patients gamma-hydroxybutyrate appears to be associated more often with vomiting. The long recovery time and its great variability make chlorprothixene/phenobarbital a less valuable alternative.

Adolescent↗

Vectors for RNAi technology in poplar.

The potential of double-stranded RNA interference (RNAi) technology was studied for down-regulation of gene expression in poplar. A set of vectors was constructed generating RNAs capable of duplex formation of sequences specific for the beta-glucuronidase (GUS) reporter gene system. These gene cassettes are driven by the CaMV-35S promoter. To address the question of gene silencing, we tested the functionality of these vectors, both in transient assays by transforming protoplasts with the RNAi constructs, and in stably transformed GUSexpressing poplar plants. Agrobacterium-mediated transformation of those GUS-expressing plants with a GUS-specific RNAi construct showed a strong down-regulation of the reporter gene. From these results we conclude that RNAi is also functional in poplar.

Base Sequence↗

Fluorescent proteins in poplar: a useful tool to study promoter function and protein localization.

The jellyfish (Aequorea victoria) green fluorescent protein (GFP) and its variants (CFP [cyan] and YFP [yellow]) were successfully used as a vital marker system for the transformation of hybrid poplar (Populus tremula x P. alba). Our results show that, in this woody plant, fluorescent proteins can be expressed: (i) transiently in protoplasts after PEG-mediated transformation, as well as in leaf cells after particle bombardment, and (ii) stably in callus cells and plants after Agrobacterium-mediated transformation. For these studies, we constructed vectors permitting easy recloning of any promoter fragments of interest. Confocal laser scanning microscopy was used both for visualization and differentiation between the different colours of the GFP variants and autofluorescence of chlorophyll and lignified xylem vessels. Peroxisomes were chosen as target organelles for GFP translocation via the peroxisomal targeting sequence PTS1 because this allowed us to concentrate the fluorochrome in the small volume of a few peroxisomes, giving a bright fluorescence over background noise.

Base Sequence↗

[Arterial tortuosity syndrome].

The arterial tortuosity syndrome is a rare congenital disorder characterized by elongation and generalized tortuosity of the major arteries including the aorta. Associated clinical features consist of excessively stretchable skin and joint laxity which is indicative of a connective tissue disorder such as Ehlers-Danlos or Cutis laxa syndrome. The gene locus of the arterial tortuosity syndrome has recently been localised on chromosome 20q13; inheritance ist autosomal recessive. - We report on a newborn with arterial tortuosity syndrome and hiatal hernia, bilateral hip dislocation, inguinal hernias and diffuse tortuosity of the great arteries including the aorta. Known gene loci involved in Ehlers-Danlos syndrome, cutis laxa syndrome and other connective tissue disorders were excluded by specific DNA markers. By homozygosity mapping with polymorphic microsatellite markers it was possible to confirm the gene locus for the ATS on chromosome 20q13. In addition to the presentation of this patient, a review of the literature is presented.

Abnormalities, Multiple↗

Severe valproic acid intoxication is associated with atrial tachycardia: secondary detoxication by hemoperfusion.

Valproic acid is an anticonvulsant drug which is associated with serious toxicity including fatal outcome in case of severe intoxication. Secondary detoxication by hemodialysis or hemoperfusion has been employed successfully in valproic acid intoxication. Cardiac arrhythmias have only been described rarely in valproic acid intoxication in humans. We report on a 15 year-old boy with severe valproic acid intoxication (valproic acid plasma level on admission: 1 150 mg/l) who presented with coma, hypernatremia and atrial tachycardia. The patient was successfully treated with hemoperfusion and intensive supportive care without implementation of a specific antiarrhythmic therapy. We conclude that patients with severe valproic acid intoxication may benefit from secondary detoxication. In addition to generally known symptoms valproic acid intoxication may also be associated with cardiac arrhythmias.

Adolescent↗

[Congenital muscular dystrophies: muscle-eye-brain disease].

Three rare autosomal recessive disorders share the combination of congenital muscular dystrophy and brain malformations including a neuronal migration defect: muscle-eye-brain disease (MEB), Walker-Warburg syndrome (WWS), and Fukuyama congenital muscular dystrophy (FCMD). In addition, ocular abnormalities are a constant feature in MEB and WWS. We report on two brothers with MEB. The clinical and radiological characteristics are demonstrated.

Brain↗

[The role of S100B-protein in neonatology, pediatric intensive care, and pediatrics].

During the last years neuromonitoring with various biochemical markers such as S100B protein has been introduced into the clinical settings of neonatal and pediatric intensive care. Several investigations have been undertaken to correlate S100B protein concentrations to the diagnosis and prognosis of neonates and children with severe cerebral disorders. This articles gives a review on the current knowledge, indications and limitations on the use of S100B protein after non-traumatic and traumatic brain injury in neonates and children.

Adolescent↗

2-substituted pi system derivatives of adenosine that are coronary vasodilators acting via the A2A adenosine receptor.

Compound 20 (CVT-3146--a 2-[(N-1-(4-N-methylcarboxamidopyrazolyl)] adenosine derivative) and compound 31 (CVT-3033--a 2-[(4-(1-N-pentylpyrazolyl)] adenosine derivative), were found to be short acting functionally selective coronary vasodilators (CV t0.5 = 5.2 +/- 0.2 and 3.4 +/- 0.5 min, respectively--rat isolated heart 50% reversal time) with good potency (EC50S = 6.4 +/- 1.2 nM and 67.9 +/- 16.7 nM, respectively), but they possess low affinity for the ADO A2A receptor (Ki = 1122 +/- 323 nM and 2138 +/- 952 nM, respectively; pig striatum).

Adenosine↗

Functional results of operation in osteogenesis imperfecta: elongating and nonelongating rods.

Gait capacity, operative intervention, and complications of operation were evaluated in 20 patients with osteogenesis imperfecta (OI). Thirty-two Bailey-Dubow (B-D) rods and 24 nonelongating rods were used. Postoperatively, gait capacity improved in eight patients, regressed in three, and remained unchanged in nine. No preoperative ambulator regressed to a nonambulatory status. The Sillence disease type was not a prognostic indicator of giat capacity. The complication rate was 72% for the B-D rod and 50% for the nonelongating rod, although the percentage requiring reoperation was similar for both types of nails. There was no difference in longevity between the two nails.

Adolescent↗

Effects of overfeeding in children with muscle dystrophies.

In muscle dystrophies as in other muscle-wasting diseases and states, a progressive loss of muscle protein occurs, probably as a result of an imbalance between muscle protein synthesis and degradation. In the present study we examined whether this progressive muscle wasting and reduced functional capacity so damaging to patients with muscular dystrophies, can be reduced or even reversed by nightly overfeeding with 1000 ml of Osmolite in addition to the voluntary daytime dietary intake. In the Duchenne muscle dystrophy (DMD) group (six patients) body weight increased significantly accompanied by a 14% increase in midarm muscle circumference with only minimal changes in triceps skin fold, indicating a relative build up of muscle mass. In the congenital muscular dystrophy (CMD) group (four patients) no changes occurred in body weight or any of the three anthropometric measurements performed. Baseline nitrogen balance was mildly positive in both groups and improved significantly in the DMD group during the 3-month experimental period of refeeding, with no changes in urinary 3-methylhistidine excretion, suggesting improved muscle protein synthesis with no change in muscle protein degradation. No changes were detected in hematological and biochemical parameters, liver function tests, pulmonary function tests, or a general activity index during the study period. Our results suggest that a reduced rate of protein synthesis rather than an increased rate of protein degradation occur in muscle dystrophies, and that overfeeding might offer promising nutritional effects, at least in DMD patients.

Adolescent↗

Perthes' disease and posterior lumbosacral union.

Delayed bone development is common in Perthes' disease, and affected children are usually smaller than normal. Somatosensory evoked potentials (SEP) studied in 25 cases of Perthes' disease were normal. Fifty-four patients with active or healed Perthes' disease were examined. Every child aged less than 8 years showed failure of posterior lumbar and/or sacral fusion. With growth, fusion increased so that at age 14 years 50% had a normal fusion and 50% had spina bifida occulta. At age greater than 16 years, the incidence was similar to that in the general population. In Perthes' disease, growth in fusion of the lumbosacral elements is delayed.

Adolescent↗

MR findings in acute and chronic coccidioidomycosis meningitis.

PURPOSE: To characterize MR findings in acute and chronic coccidioidomycosis meningitis and relate the imaging features to the clinical course. METHODS: We reviewed MR scans and clinical findings of 12 patients with coccidioidal meningitis. RESULTS: Patients with active or untreated disease were found to have hydrocephalus and intense enhancement of the cervical subarachnoid space, basilar, sylvian, and interhemispheric cisterns on postcontrast MR scans. Focal parenchymal signal abnormalities suggesting ischemia or infarction were common. Abnormal MR enhancement decreases during therapy, although patients develop cortical and/or brain stem atrophy. CONCLUSIONS: Widespread cisternal and cervical subarachnoid meningeal involvement is common in coccidioidal meningitis. Serial contrast MR imaging reflects the effects of therapy in patients with coccidioidal meningitis.

Acute Disease↗

Transient suppression of involuntary movements in cerebral palsy patients during dental treatment.

The efficacy of nitrous oxide (N2O) and oxygen (O2) inhalation in reducing involuntary movements in cerebral palsy (CP) dental patients was examined using electromyography (EMG) and H-reflex techniques. Quantification and analysis of the EMG data revealed a progressive elimination of these movements to a point resembling the state of a normal muscle at rest. There was a gradual decline in the number of bursts and an increase in the silent periods of the EMG while under N2O. The recovery period after cessation of N2O was characterized by a successive increase in the number of bursts and a simultaneous decrease in the silent periods of the EMG to a state similar to the baseline recorded prior to inhalation of N2O/O2. The H-reflex also was found to decrease gradually during inhalation of N2O/O2 to about one-half of the baseline. This parameter also returned to baseline after N2O wash-out. Our findings demonstrate quantitatively the effectiveness of N2O in reducing the central motor neuron pool excitability to an extent that suppresses involuntary movements and allows more controllable and comfortable dental management.

Adolescent↗

Nontraumatic, acute neonatal paraplegia.

Nontraumatic, acute neonatal paraplegia with associated lower limb vascular phenomena is a relatively rare condition. Three such cases are reported. The paralysis is primarily motor with minimal neurologic recovery. Contractures and bizarre deformities develop at an early stage and necessitate repeated orthopaedic intervention. The most likely etiology appears to be a vascular insult to the spinal cord.

Catheterization, Peripheral↗