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Biomedical subjects

B Böwing

Publications and source records attributed to B Böwing.

At least 19 recordsLinked to original sources

[Eicosanoid metabolism in peripheral blood cells in patients with cystic fibrosis].

BACKGROUND: The autosomal recessive inherited cystic fibrosis is the most common genetic disorder in white patients. Disturbances in the eicosanoid metabolism seem to play an important role in the pathogenesis of the disease. The present study shows examinations on the release of prostaglandin E2 (PGE2) and peptide leukotrienes (pLT) in peripheral blood cells. PATIENTS AND METHODS: In this respect heparinised blood samples of 10 cc were obtained from 25 patients with cystic fibrosis. The peripheral blood cells were separated from the remaining blood components. PGE2 and pLT were measured by a particularly developed sensitive enzyme immunoassay with specific monoclonal antibodies. Both the basal release of the metabolites and the concentrations after stimulation with arachidonic acid (AA) were explored. The control group consisted of 25 healthy individuals. RESULTS: A distinct elevation in the basal release of pLT compared to the control group was noticed. Further stimulation with AA could only be achieved to a remarkably smaller extent. In contrast to these findings no elevation of the basal release of PGE2 and no stimulative effect of AA could be detected. In general we observed a distinct shift in the eicosanoid metabolism in favour of pLT and their proinflammatory effects. CONCLUSIONS: The increased synthesis of pLT in patients with cystic fibrosis seems to play an important role in the pathogenesis of the disease. Future studies will have to proof, if we can achieve any improvement in the clinical courses in patients with cystic fibrosis using leukotriene receptor antagonists or 5-lipoxygenase inhibitors.

Adolescent↗

[Low-field magnetic resonance tomography in pediatric radiology. Possibilities, limitations and prospects].

MRI has been used in pediatric imaging for several years. It provides excellent anatomic detail and tissue characterization combined with the advantage that it is not associated with the application of ionizing radiation to the radiation sensitive infant organism. Low field MRI provides some additional advantages like a lower rate of sedations, easier monitoring of sedated patients and the option of interventional examination and therapy. The disadvantages, however, are the slightly prolonged examination times and the lower signal-to-noise ratio compared to high-field MR scanners. In the future, new techniques using modern gradient echo sequences will provide fast imaging methods with a very high signal-to-noise ratio which could partially replace conventional x-ray imaging. In the presented article we report our experiences in low-field MR imaging of pediatric patients. The possibilities as well as the limitations of this imaging modality are pointed out.

Adolescent↗

Low field thoracic MRI--a fast and radiation free routine imaging modality in children.

Radiography of the chest is the most frequently performed radiological examination in pediatric imaging. However, it is associated with the application of ionizing radiation. In order to avoid ionizing radiation in children a new and very fast MRI technique has been developed at our center as an alternative to the pediatric chest X-ray. 100 patients who had received a chest X-ray were additionally investigated in a 0.2 T low-field MR-scanner by a modified true FISP sequence with an acquisition time of 3.6-4.6 s for a coronal triple-slice scan. X-ray and MR images were independently evaluated and later compared by two pediatric radiologists. Total investigation times (door-to-door time) for X-ray and MRI were comparable. The signal-to-noise ratio for lung parenchyma was 4.6-7.3. Of 189 pathologic findings 165 were depicted on MR images as well as radiographs, 18 were noted on MRIs only, 6 on X-rays only. Overall kappa was 0.87. True FISP MRI may be a good alternative to conventional chest X-ray. The main advantages are: fast imaging free of ionizing radiation, easy performance, no need for special equipment, optional imaging in all 3 planes, good image quality, and a high diagnostic value.

Adolescent↗

Optimal tobramycin dosage in patients with cystic fibrosis--evidence for predictability based on previous drug monitoring.

A retrospective analysis of files of patients with cystic fibrosis and pulmonary exacerbations was performed to investigate whether an individual dosage of tobramycin once established by serum level determination allows a reliable prediction of the adequate dosage in a consecutive exacerbation. All patients hospitalized > or = 2 times between May 1997 and September 1998 with pulmonary exacerbation due to Pseudomonas aeruginosa infection susceptible to tobramycin were included. The initial dosage to tobramycin was 5 mg/kg body weight every 12 h followed by drug level determinations to establish the optimal dose. In a consecutive exacerbation the same dosage per kg body weight was used again and drug level determinations were repeated. Sixteen patients (six female = 38%) with a mean age of 24 years (median: 26 years, range: 9-33) were hospitalized for 49 pulmonary exacerbations (2-6 per patient, mean: 3, median: 2.5). During the first episode of tobramycin treatment in the study period all trough levels were < 2 microg/ml (median: 0.6) and the peak levels were 7.1-16.9 microg/ml (median: 11.9). In four patients the peak level was > 12 microg/ml. In 28 consecutive episodes the dosage of tobra myci n was chosen based on optimal results of previous drug level monitoring and in 27 instances (96%) the previously established optimal dose was confirmed. In five consecutive episodes the tobramycin dosage had been increased erroneously and this resulted in abnormally high peak levels in three cases. These findings suggest that a safe and therapeutic tobramycin dosage in an individual patient with cystic fibrosis is predictable based on a previously established optimal dosage.

Adult↗

Low-field magnetic resonance imaging of the pelvis in patients with anal dynamic graciloplasty: initial experience.

The aim of this study was to determine whether low-field magnetic resonance (MR) imaging can safely and accurately depict inflammatory changes in patients with anal dynamic graciloplasty, in whom high-field MR imaging is contraindicated and ultrasonography and computed tomography are inadequate. A 0.2-T field-strength MR examination was performed in six patients with anal dynamic graciloplasty malfunction in whom reoperation was contemplated. The following sequences were applied: T2-weighted turbo spinecho with fat saturation, T1-weighted conventional spin-echo, and contrast-enhanced T1-weighted conventional spin-echo with fat saturation. Results indicated that none of the patients experienced relevant discomfort, pacemaker malfunction, or electrode dislocation with low-field MR imaging. Inflammatory pelvic changes were visualized in four patients and atrophy of the transposed gracilis muscle in another. Surgery was thus avoided in the four, who underwent conservative treatment for their pelvic inflammation. It was concluded that these preliminary results demonstrate the feasibility of MR imaging with a low field strength in patients with anal dynamic graciloplasty. In such patients, in whom diagnostic imaging had been problematic, the potential for safe and accurate visualization will be a boon to treatment planning.

Adult↗

Congenital intracerebral teratoma: a rare differential diagnosis in newborn hydrocephalus.

Congenital hydrocephalus is caused by a broad spectrum of underlying disorders. In the majority of cases it is due to aqueductal stenosis and other distinct congenital anomalies, like Arnold-Chiari malformation. Nevertheless, in the differential diagnosis rare conditions such as cerebral malignancies must also be considered. We present two cases of congenital intracerebral teratoma as a differential diagnosis in congenital obstructive hydrocephalus. A teratoma is suggested when a rapidly growing hydrocephalus with a central calcified and vascularized mass is found sonographically. Regular cerebral structures usually cannot be detected. Early diagnosis in such cases is of clinical importance as the prognosis of congenital intracerebral teratoma is generally very poor.

Brain Neoplasms↗

MURCS association: case report and review.

We report on a 25 year old woman with aplasia of the Müllerian duct, unilateral renal agenesis, and anomalies of the cervicothoracic somites (MURCS association). Growth retardation and facial asymmetry were also present. A review of published reports allows MURCS association to be distinguished from related associations, sequences, and syndromes. Moreover, sporadic occurrence, the broad spectrum of associated anomalies, and the involvement of different organ systems closely related in early embryogenesis are arguments for considering MURCS association as the consequence of a developmental field defect.

Adult↗

Pulmonary artery sling associated with tracheobronchial malformations.

We describe three cases of pulmonary artery slings associated with tracheal stenoses by complete cartilaginous rings and abnormalities in the tracheobronchial branching pattern. This association implicates special problems of management that are different from the simple pulmonary artery sling. Pathologic anatomy, symptoms, diagnostic procedures, and the problems of therapy are described. Considering similar cases in the literature, we conclude that thorough diagnostic evaluation of the tracheobronchial and the cardial system should be carried out in all cases of pulmonary artery sling. Simple correction of the aberrant vessel without correcting the tracheal stenosis is of no value in these cases. In some milder cases, a conservative approach is possible and probably less harmful than an operation.

Abnormalities, Multiple↗

Diagnosis of moyamoya disease with additional renal artery stenosis by colour coded Doppler sonography.

Moyamoya disease is a rare vascular anomaly of the cerebral arteries. The etiology of the disease has not yet been clearly identified. We report the noninvasive diagnosis of Moyamoya disease in a patients with a very early onset of symptoms in infancy. The diagnosis was made by colour coded Doppler sonography and confirmed by angiography at the age of 6 months, following two episodes of cerebral infarction. A bilateral encephalodurosynangiosis was performed at the age of 7 months with subsequent slight improvement of the neurological deficits. Colour Doppler sonography revealed early vascularisation from the fascia temporalis graft into the arachnoid space. At the age of 10 months the patient developed arterial hypertension caused by left renal artery stenosis. Our case suggests, that in infancy Moyamoya disease can be suspected noninvasively by colour Doppler sonography of the cerebral arteries. Patients should be carefully screened for possible extracranial arterial stenoses which may develop in the course of time. Encephalodurosynangiosis seems to be a good therapeutic option for patients with severe neurological symptoms.

Cerebral Arteries↗

[X-ray signs of foreign body aspiration in children].

Preoperative chest X-rays were taken in both postanterior and partially lateral views of 94 children with foreign-body aspiration. Additional fluoroscopy was employed in 70 patients. In 7% of the cases, the foreign body was radiopaque; in an equal amount of cases, there were no radiological findings. In the remaining results we observed: emphysema as an indirect radiological sign in two-thirds of the cases; in less than one-third, poststenotic atelectasis; pneumonia in 10%; bronchitis in 9%; pneumothorax in 2%.

Bronchi↗

[Direct genetic diagnosis in cystic fibrosis].

The recent cloning of the cystic fibrosis gene and the simultaneous identification of the predominant mutation enabled direct gene diagnosis in the majority of all CF families. We demonstrate the superiority of now available direct genotyping in five individual cases.

Adult↗

Crohn's disease in cystic fibrosis.

We report on three patients suffering from cystic fibrosis (CF) who developed gastrointestinal symptoms of Crohn's disease (CD). Two patients developed enteroenteric or enterocutaneous fistulas. The diagnosis of CD is based on typical endoscopic, radiologic, and histological features of epithelioid granulomas in two children.

Adolescent↗

[Variants of radial hemimelia with and without vitium cordis (Holt-Oram syndrome) in 2 families].

In two families radial hemimelia is inherited as a dominant trait. The first proposita suffered from bilateral radial aplasia, the 2nd propositus exhibited (pseudo)phocomelia. In this case the diagnosis was Holt-Oram-syndrome. The affected mothers showed unilateral hypoplasia of the thumb only. Cases like these ones are likely to be overlooked or misinterpreted. The recurrence risk is 50%. Similar observations are quoted. The cause of "variable expressivity" is unknown.

Child↗

[Intralobar lung sequestration. Diagnosis using the duplex-scan technic].

A 3 7/12 year old boy, suffered from a intralobar sequestration of the lung and recurrent pneumonia. In the area of the right lower lobe, a plain film showed a limited density. The ultrasonographic evaluation demonstrated a homogenous tumor above the diaphragm. We found pulsatile flow patterns by pulsed doppler sonography within the arteries feeding the sequestration. Aortography finally clearly showed the anomalous arteries leading from the descending aorta to the sequestrum. Bronchoscopy and bronchography demonstrated, that the sequestered area of the lung had no open connexion to the rest of the bronchial tree. Surgical treatment is the only possible method to remove the cause of the recurrent infections.

Aortography↗

[Cerebral ultrasound diagnosis in brain abnormalities].

Gray scale ultrasonography of the brain was performed in 93 infants with cerebral malformations. The most common malformation was the Chiari-malformation (56 children), characterized by the caudal displacement of the cerebellar vermis into the foramen magnum. The rest of the cerebellum, pons and medulla oblongata were displaced caudally and dysplastic. All children with Chiari-syndrome showed more or less severe hydrocephalus. The Dandy-Walker-malformation (3 children) was characterized by a huge retrocerebellar cyst communicating with the fourth ventricle. The cerebellar vermis was dysplastic. In alobar holoprosencephaly (2 children) a large singular midline ventricle could be shown. Both thalami and plexus chorioidei were fused in the midline. Absence of the falx cerebri, interhemispheric fissure, corpus callosum and septum pellucidum was characteristic for alobar holoprosencephaly. Stenosis of the Sylvian aqueduct (4 children) was characterized by enlarged lateral ventricles and third ventricle, whereas the fourth ventricle was normal in size. Porencephalic cysts (7 children) were spheric echofree lesions of various size, usually located symmetrically in both cerebral hemispheres. In hydranencephaly (4 children) both hemispheres were replaced by echofree space occupying bubbles. In 5 children with agenesis of the corpus callosum no corpus callosum could be demonstrated by sonography. Coronal sections displaced the typical bull's head shape, formed by the enlarged third ventricle and the side ventricles as well as randomly arrayed sulci around the lateral ventricles, which are pathognomonic for agenesis of the corpus callosum. Agenesis of the septum pellucidum (5 children) was characterized by fusion of the frontal horns of the lateral ventricles. One child with aneurysmatic malformation of the vein of Galen showed dilated sinus rectus and pulsations of a cyst, located behind the third ventricle. Pulsed doppler recording showed pulsatile arterial flow patterns within the cyst.

Agenesis of Corpus Callosum↗

[Sonographic diagnosis of hypertrophic pyloric stenosis in childhood].

In most cases, diagnosis of hypertrophic pyloric stenosis is now based on real-time ultrasound examination. We employed the criteria stated in literature for sonographic diagnosis of hypertrophic pyloric stenosis, to evaluate our results. We studied 15 patients with symptoms of hypertrophic pyloric stenosis via real-time ultrasound, as well as 15 healthy infants. 10 patients were boys and 5 were girls, between 19 and 83 days of age (average age 45 days). 15 healthy infants of the same age were studied (average age 41 days) for comparison. In the healthy group, the average transverse pyloric diameter was 1.0 cm (0.6-1.5 cm); the mean wall thickness was 0.24 cm (0.2-0.3 cm) and mean pyloric length was 1.29 cm (1.0-1.6 cm). Analysis of the results in hypertrophic condition showed that the mean transverse pyloric diameter was 1.6 cm (1.2-1.9 cm). The wall thickness ranged from 0.4 to 0.8 cm with an average of 0.56 cm. The muscle length ranged from 1.7 to 2.5 cm with an average of 2.1 cm. On comparing the data of both groups we found significant differences in wall thickness and length. There was no false negative scan. Sonographic diagnosis was confirmed in all cases by surgical intervention. Our results agree with those obtained by other authors.

Female↗