Upgrading end-of-line residues of the red seaweed Gelidium sesquipedale to polyhydroxyalkanoates using Halomonas boliviensis.

Agar extraction from Gelidium and Gracilaria red seaweed species produces hundred thousand ton of carbohydrate-rich residues annually. Gelidium sesquipedale waste biomass obtained after agar extraction, still contained 44.2 % w/w total carbohydrates (dry-weight basis). These residues were biologically up-graded to poly-3-hydroxybutyrate (P3HB) after saccharification of their carbohydrate fraction to simple sugars. A combined hydrolysis treatment using sulfamic acid followed by enzymatic hydrolysis with cellulases produced a glucose-rich hydrolysate with a negligible content of inhibitors. With this treatment a sugar yield of circa 30 % (g glucose/g biomass) was attained. The algal hydrolysates were assessed as carbon source for the production of P3HB by the halotolerant bacteria Halomonas boliviensis. A cell concentration of 8.3 g L-1 containing 41 % (w/w) of polymer and a yield (YP/S ) of 0.16 gpolymer/gglucose were attained in shake flask assays. In this work, cellulose-rich seaweed waste was shown to be an upgradable, sustainable source of carbohydrates.

Chances and shortcomins of adenovirus-mediated ATP7B gene transfer in Wilson disease: proof of principle demonstrated in a pilot study with LEC rats.

BACKGROUND & AIMS: Wilson disease (WD) is an autosomal recessively inherited copper storage disease due to mutations in the ATP7B gene. It results in impaired biliary copper excretion followed by liver injury leading to cirrhosis. In parallel, copper accumulates in other tissues e. g. basal ganglia of the brain inducing motoric disorders. Phenotypical cure of Wilson disease by liver transplantation raised the question whether gene therapy may represent a successful alternative treatment procedure. To examine the principle feasibility of this approach we investigated the effects of gene transfer using an adenoviral vector construct expressing the human ATP7B cDNA in an established rodent model for WD, the Long-Evans Cinnemon rat (LEC). METHODS: Transduction efficiency was assessed by RT-PCR, Western blot and immunofluorescence analysis. The therapeutic effect was estimated by analyzing holoceruloplasmin and its ferroxidase activity in serum, and the copper content of excrements. Changes in copper homeostasis were determined by positron emission tomography (PET). RESULTS: Successful, but temporary gene transfer was clearly detectable on RNA and protein levels. In parallel the temporary therapeutic effect was documented by restoration of serum holoceruloplasmin and of its ferroxidase activity. Additionally the Ad-ATP7B treated LEC rat revealed a higher (64)Cu content in stool. PET was able to visualize differences in (64)Cu distribution between wild type and LEC rats, indicating its principle usefulness as analytical tool. CONCLUSION: The data demonstrate proof of principle of successful gene therapy in an experimental model of WD. As a consequence of successful but only transient therapeutic effect of adenoviral gene transfer we can now focus more efficient and permanent gene transfer strategies.

["Ambulatory" approach to selective coronary angiography: our results and experience]. / "Ambulantní" prístup k selektivní koronarografii: nase výsledky a zkusenosti.

The authors present a group of their first 110 patients subjected to selective coronarography during an average 7-hours hospitalization period. In selection of patients they respect the criteria ruling out the following approach: age above 70 years, distance of the patients domicile above 80 km or inaccessibility of catheterization laboratory within 45 minutes, serious aortic valve disease, unstable coronary syndromes, insulin treated diabetes mellitus, renal insufficiency, cardiac insufficiency, grade III or IV, severe left ventricular dysfunction, history of malignant arrhythmia, acute myocardial infarction or cerebrovascular attack during month preceding examination, coronary finding calling for urgent revascularization, complicated course of examination and mental inability of patient. All coronarographies were performed by a 4F catheterization instrument, no complications were recorded and readmission to hospital was not called for. An early check-up by telephone was accepted by half the patients. When all the above criteria are respected the authors recommend this procedure as a standard one without the need of hospital admission.

Long-term outcomes of balloon dilation of esophageal strictures in children.

RATIONALE AND OBJECTIVES: The purpose of this study was to determine the long-term success of the use of angioplasty balloons for dilation of esophageal strictures in children. MATERIALS AND METHODS: The authors studied 24 children who had undergone fluoroscopically guided angioplasty balloon dilation of esophageal strictures. Ten children had stricture of the anastomosis after surgical repair of esophageal atresia; five had achalasia; three had strictures due to gastroesophageal reflux; three had corrosive strictures; two had congenital stenosis; and one had stricture after radiation therapy. Success was defined as resolution of the dysphagia a year after dilation. RESULTS: A year after the last dilation, 15 of the 24 children had no dysphagia. Patients in whom treatment was successful included eight children with strictures after surgery for esophageal atresia, one with achalasia, three with strictures caused by gastroesophageal reflux, one with a corrosive stricture, one with congenital stenosis, and one with a stricture resulting from radiation therapy. CONCLUSION: The long-term results of balloon catheter dilation are highly successful in patients with stricture after surgical repair of esophageal atresia and stricture due to gastroesophageal reflux. Dilation is not as valuable in the treatment of esophageal strictures resulting from other causes.

[Catheterization therapy of the left main coronary artery in chronic stenosis of the right coronary artery. What are the limits of interventional cardiology?]. / Katetrizacní lécba stenózy kmene levé vencité tepny pri chronickém uzáveru pravé vencité tepny. Kde jsou hranice intervencní kardiologie?

The authors give an account of a 59-year-old patient with a significant stenosis of the left main coronary artery and occlusion of the right coronary artery, occlusion of the ramus interventricularis anterior after failure of surgical revascularization and with a left ventricular ejection fraction of 20%. The authors performed a successful percutaneous coronary angioplasty of the left main coronary artery with an implantation of a stent. They discuss contemporary possibilities and limitations of catheterization treatment of stenosis of the left main coronary artery.

[Treatment of significant coronary stenoses using stents without preliminary balloon angioplasty: present status, results, perspectives]. / Osetrení významných koronárních stenóz pomocí stentu bez predchozí balonkové angioplastiky: soucasný stav, nase výsledky, perspektivy.

The authors present an account on a group of 57 patients where they tried to treat 58 significant coronary stenoses by means of direct stenting without predilatation. In two patients they were able to implant the stent only after previous dilatation, in one instance postdilatation was necessary because of a residual stenosis of more than 20% and in one instance it was necessary to implant another stent on account of distal dissection. Direct stenting was successful in 93%. At the significance level of p < 0.05 significantly shorter fluoroscopic and total intervention times were achieved as compared with the control group where the standard procedures of stenting after previous balloon angioplasty was used. The authors conclude that direct stenting without previous dilatation is a safe method which can be used with the contemporary instrumental equipment in approximately 20% of carefully selected lesions.

Influence of bidirectional superior cavopulmonary anastomosis on pulmonary arterial growth.

Right-sided BSCA provides for satisfactory pulmonary arterial growth in infants and children with complex congenital heart defects, and it could enhance the growth of a small right pulmonary artery. The growth of the left pulmonary artery, particularly in younger patients, needs close attention to confirm the safe role of BSCA in long-term palliation.

Double aortic arch in d-transposition of the great arteries complicated by tracheobronchomalacia.

Simultaneous occurrence of d-transposition of the great arteries and aortic arch malformations is very rare. A case of this malformation, the fourth case reported in the literature, is described. Despite successful surgery, tracheobronchomalacia was fatal at the age of 7 months.

[Surgery of achalasia in childhood. The thoracic or abdominal approach?]. / Operace achalázie v detském veku. Hrudní nebo brisní prístup?

The authors present their experience with surgical treatment of achalasia of the oesophagus in child age. During a nine-year period (1984-1992) they operated 11 children with achalasia. The group comprised 6 boys and 5 girls. The mean age at the time of operation was 9.5 years. The youngest patient was operated at the age of 14 months and the oldest one at the age of 15 years. The main clinical symptoms at the time of establishment of the diagnosis were: vomiting in 91%, dysphagia in 64%, the children did not thrive and lost weight in 36%; they suffered from relapsing bronchopneumonia in 27%, chronic bronchitis in 9%, bronchial asthma in 9% and one female patient was treated and followed up on account of anorexia nervosa. In six patients a modification of Heller's operation was performed with left-sided thoracotomy which in three patients was supplemented by anti-reflux Belsey Mark IV plastic operation. During the last three years five patients were operated from an abdominal approach and myotomy was supplemented by Nissen fundoplication. At present the authors prefer and abdominal approach and supplement myotomy of the distal oesophagus by Nissen fundoplication.

[Magnetic resonance imaging of the postoperative status in children with congenital heart defects]. / Zobrazení pooperacních stavu detí s vrozenou srdecní vadou magnetickou rezonancí.

The justification of examinations by magnetic resonance and the results of visualization of cardiac and vascular structures in relation to the performed surgical operation were evaluated retrospectively in 24 children after surgery of congenital heart disease. Thirteen patients had an operation of coarctation or a double aortic arch and four had an operation of an anomaly of the branch of the pulmonary artery-its loop or atresia. Seven patients had operations, mostly complicated inborn heart disease, of the common arterial trunk and transposition of the large arteries with a conduit, tricuspidal atresia after Fontan's or Glenn's modified operation and ligature of an aortal aneurysm. At the time of examination the patients were 4 to 18 years old. Spin echo sequences obtained by basic reconstructions of T1 and possibly T2 weighted images on a 1.5 T Magnetom apparatus Siemens proved an excellent visualization in the area of the large arteries and the capacity of detailed visualization of cardiac structures, incl. changes of the vascular wall in relation to the performed operation. Magnetic resonance examinations provided accurate information on central anastomoses and conduits as well as on changes of the vascular lumen. The investigation confirmed that magnetic resonance is suitable for visualization of postoperative changes and findings of morphological structures in particular at sites of cardiovascular anastomoses where magnetic resonance supplements the visualization capacity of echocardiography and substitutes invasive examinations where only an angiographic examination is needed. In eight instances the examination was supplemented by invasive assessment of haemodynamic conditions.

[Diagnostic imaging methods in neonates with diseases of the urinary tract]. / Uplatnení diagnostických zobrazovacích metod u novorozencu s onemocnením mocových cest.

The actually used examination algorithm of imaging methods was checked in 35 neonates examined in the course of two years (1991 and 1992) on account of suspected diseases of the urinary pathways. It involved sonographic examination of 28 children, voiding cystourethrography (34x) and intravenous urography (18x). Ascendent pyelography and irrigography were performed three times, one neonate was examined by computed tomography. The procedure used as a basic sonographic prenatal screening in 11 children, in 20 clinical suspicion of disease of the urinary pathways, and four neonates were examined on account of an anorectal malformation or abnormality of the external genitalis. The most frequent finding--in 18 children--was hydronephrosis. It was associated with signs of subrenal obstruction (8x), with ren duplex (5x) and with a megaureter (6x). The association of the sonographic finding of dilatation of the intrarenal urinary pathways with absent contrast filling during excretory urography was observed in a child with hydronephrosis and subrenal obstruction and in four children with a multicystic dysplastic kidney. In individual cases the authors detected a valve of the posterior urethra, a sigmoid-vesical fistula, agenesis of the penis and stenosis of the urethra. Eleven neonates had a normal findings of the urinary tract.

[Long-term follow-up of the quality of life in children after surgery for vascular compression of the respiratory tract]. / Kvalita zivota detí dlouhodobe po operaci pro útlak dýchacích cest cévní strukturou.

The aim of this study is the analysis of long-term results of surgery for vascular airway compression. Out of those operated within last 14 years at our institution, thirty two have a longer than five years follow up, being now 9.1 +/- 3.0 years following surgery. All are in excellent clinical condition. One quarter of patients show occasional stridor and one third frequent respiratory infections. Eighty percent have some lung function abnormality, 72% features of central airways obstruction. Pulmonary perfusion scintigraphy demonstrated in 19% asymmetric pulmonary blood flow. Magnetic resonance imaging showed relationship between vessels and airways. Although long-term outcome of surgery for vascular airway compression is excellent, high percentage of pathologic lung functions is of concern and substantiates the need for close long-term follow-up.

[Takayasu's arteritis with primary involvement of the pulmonary artery]. / Takayasuova arteritida s provotním postizením plicnice.

The authors demonstrate a 23-year-old women in the fourth month of pregnancy with complaints and findings typical for Takayasu's arteritis. The onset of the disease can be safely located to the area of the pulmonary artery where the aorta and its branches were still unaltered. In the course of nine years not only a severe occlusive stage developed in the area of the thoracic aorta and its branches but there was also progression as regards the affection of the pulmonary artery. In the area of the subclavicular artery on the left balloon dilatation was performed. The collateral arterial blood supply of the left lung from the left coronary artery is also unusual.

Intermediate to late results of surgical relief of vascular tracheobronchial compression.

Since 1979, a total of 69 infants and children aged 0.1 to 11.9 (median 0.7) years required surgical intervention for: double aortic arch (26), anomalous origin of innominate artery (26), right aortic arch with left ligamentum arteriosum (9), pulmonary artery sling (5), retroesophageal right subclavian artery (3). Before operation, the morbidity was high: 84% of children were symptomatic in the first trimester of life with 24 patients requiring mechanical ventilation. Seven children had an accompanying heart defect. Left thoracotomy was the preferred approach. There were 2 early (asphyxic brain damage, postoperative pneumonia) and 2 late (tracheomalacia, complex heart defect) deaths. Five patients (2 originally operated elsewhere) needed reoperation for persistent symptoms. All 65 survivors are well 1 month to 11.9 (mean 3.9 +/- 3.62) years after surgery. Tracheobronchoscopy, magnetic resonance imaging, and lung function testing were helpful for postoperative evaluation. Minor tracheal compression was revealed in 4 patients despite their good clinical condition.

Ultrastructure of rabbit tracheal epithelium after the administration of Hexabrix--a hexaiodated contrast agent for tracheobronchography.

The ultrastructure of the tracheal epithelium after the application of 1 ml of Hexabrix (a hexaiodated contrast agent for tracheobronchography) into the airways was studied. Severely altered pseudostratified columnar epithelium was found in the trachea. Eighty-one +/- 5% of the goblet cells had been stimulated to discharged mucus. Forty-three +/- 3% of them were completely exhausted and had degenerated. The ciliated cells displayed marked signs of pathological alteration up to vacuolar degeneration. The ciliary border above the epithelium was severely damaged. The mean number of cilia per micron 2 fell to 3.3 +/- 0.5, but the proportion of intact cilia did not drop below 95%. As morphological signs of impaired self-cleaning ability of the airway epithelium, a large amount of inspissated mucus and numerous bacteria were found in the area of the impaired ciliary border. Coarse grained contrast substance of supreme electron density was intermingled with the condensed mucus. The most pronounced damage to the cells was observed at the sites of direct contact of the contrast agent with the cell membranes.

[Clinical morphology of ventricular septal defects]. / Klinická morfologie defektu kómórového septa.

The authors give an account of morphological characteristics of different types of ventricular septal defects. They demonstrate their typical pictures obtained by angiocardiographic and echocardiographic examination. The investigation is based on analysis of 295 children with a cinematographically confirmed diagnosis and 75 necroptic preparations of the heart with a ventricular septal defects.

[The infundibular septum in congenital heart defects. Morphologico-angiocardiographic study of the double-outlet heart ventricle]. / Infundibulární septum u vrozených srdecních vad. Morfologicko-angiokardiografická studie dvojvýtokové pravé komory.

The authors investigated the infundibular septum in 40 archival heart preparations with a double outlet right ventricle. They compared the post-mortem findings with the previous angiocardiographic examination and the skiagram of the preparation made post mortem. They present also the corresponding findings obtained by echocardiography. The infundibular septum was in all instances the dominating intracardiac structure influencing the direction of the blood flow from the ventricles into outflow tracts in the direction towards the large arteries. In 15 instances the infundibular septum was shifted in an anterosuperior direction beneath the orifice of the pulmonary artery and it derived the blood current from the left ventricle through the defect in the ventricular septum into the aorta. The restricted blood flow through the aorta led to its hypoplasia, which in 11 instances was associated with coarctation or interruption of the aorta. In seven instances of posteroinferior shift the infundibular septum was straight. These cases were associated with coarctation or interruption of the aorta. In ventricular septum and with stenosis of the pulmonary artery. Based on these findings the authors assume that the shift of the infundibular septum in a double outlet right ventricle has a decisive position as regards haemodynamic manifestations of the defect and in the development of outflow tracts and large arteries. Last not least, information on the infundibular septum is also important for surgery of the defect.

[Anomalous origin of the right coronary artery from the pulmonary artery. Preoperative diagnostic finding in the aortopulmonary window]. / Anomální odstup pravé koronární artérie z plícnice. Predoperacne diagnostikovaný nález u aortopulmonálního okna.

A rare case of the abnormal origin of the right coronary artery is demonstrated in a two-year-old boy with an aortopulmonary window and subvalvular membranous aortal stenosis. The diagnosis was established by angiography before operation by injection of contrast substance into both great arteries. Surgical repair of the complicated defect comprised also derivation of the orifice of the right coronary artery into the aorta. The long-term postoperative follow-up indicates relatively favourable results with signs of impaired myocardial perfusion in the area supplied by the right coronary artery.