Katetrizační uzávěr defektu septa síní typu ostium secundum je v léčbě této vrozené srdeční vady metodou volby, preferovanou před chirurgickým uzávěrem. Embolizace okluderu do plicního nebo systémového řečiště je nejčastější závažnou a potenciálně fatální komplikací tohoto katetrizačního výkonu. Prezentujeme případ úspěšné urgentní perkutánní extrakce okluderu embolizovaného do rizikové anatomické lokalizace, s využitím smyčkového katétru a endomyokardiálního bioptomu.
Percutaneous treatment of secundum type atrial septal defect has become the method of choice in treatment of this congenital heart disease. Embolization of the septal occluder device is recognised as the most common severe complication of the procedure. We report a case of successful urgent percutaneous retrieval of an occluder device using loop catheters and endomyocardial bioptome following embolization to a hazardous anatomical location.
- Keywords
- bioptom, palpitace, extrakce okluderu,
- MeSH
- Aorta diagnostic imaging pathology MeSH
- Middle Cerebral Artery pathology MeSH
- Biopsy instrumentation MeSH
- Stroke etiology complications MeSH
- Heart Septal Defects, Atrial diagnosis complications therapy MeSH
- Dyspnea etiology MeSH
- Echocardiography, Transesophageal MeSH
- Embolism diagnosis epidemiology etiology MeSH
- Middle Aged MeSH
- Humans MeSH
- Paresis etiology MeSH
- Septal Occluder Device * adverse effects MeSH
- Cardiac Catheterization methods instrumentation MeSH
- Brachiocephalic Trunk diagnostic imaging pathology MeSH
- Check Tag
- Middle Aged MeSH
- Humans MeSH
- Female MeSH
- Publication type
- Case Reports MeSH
BACKGROUND: Cysteine peptidases of clan CA, family C1 account for a major part of proteolytic activity in the haematophagous monogenean Eudiplozoon nipponicum. The full spectrum of cysteine cathepsins is, however, unknown and their particular biochemical properties, tissue localisation, and involvement in parasite-host relationships are yet to be explored. METHODS: Sequences of cathepsins L and B (EnCL and EnCB) were mined from E. nipponicum transcriptome and analysed bioinformatically. Genes encoding two EnCLs and one EnCB were cloned and recombinant proteins produced in vitro. The enzymes were purified by chromatography and their activity towards selected substrates was characterised. Antibodies and specific RNA probes were employed for localisation of the enzymes/transcripts in tissues of E. nipponicum adults. RESULTS: Transcriptomic analysis revealed a set of ten distinct transcripts that encode EnCLs. The enzymes are significantly variable in their active sites, specifically the S2 subsites responsible for interaction with substrates. Some of them display unusual structural features that resemble cathepsins B and S. Two recombinant EnCLs had different pH activity profiles against both synthetic and macromolecular substrates, and were able to hydrolyse blood proteins and collagen I. They were localised in the haematin cells of the worm's digestive tract and in gut lumen. The EnCB showed similarity with cathepsin B2 of Schistosoma mansoni. It displays molecular features typical of cathepsins B, including an occluding loop responsible for its exopeptidase activity. Although the EnCB hydrolysed haemoglobin in vitro, it was localised in the vitelline cells of the parasite and not the digestive tract. CONCLUSIONS: To our knowledge, this study represents the first complex bioinformatic and biochemical characterisation of cysteine peptidases in a monogenean. Eudiplozoon nipponicum adults express a variety of CLs, which are the most abundant peptidases in the worms. The properties and localisation of the two heterologously expressed EnCLs indicate a central role in the (partially extracellular?) digestion of host blood proteins. High variability of substrate-binding sites in the set of EnCLs suggests specific adaptation to a range of biological processes that require proteolysis. Surprisingly, a single cathepsin B is expressed by the parasite and it is not involved in digestion, but probably in vitellogenesis.
- MeSH
- Gastrointestinal Tract parasitology MeSH
- Hydrolysis MeSH
- Host-Parasite Interactions MeSH
- Carps parasitology MeSH
- Cathepsin B chemistry genetics isolation & purification metabolism MeSH
- Cathepsin L chemistry genetics isolation & purification metabolism MeSH
- Proteolysis MeSH
- Recombinant Proteins analysis genetics isolation & purification MeSH
- Gene Expression Profiling MeSH
- Trematoda enzymology genetics MeSH
- Introduced Species MeSH
- Animals MeSH
- Check Tag
- Animals MeSH
- Publication type
- Journal Article MeSH
- Research Support, Non-U.S. Gov't MeSH
Schistosomiasis caused by a parasitic blood fluke of the genus Schistosoma afflicts over 200 million people worldwide. Schistosoma mansoni cathepsin B1 (SmCB1) is a gut-associated peptidase that digests host blood proteins as a source of nutrients. It is under investigation as a drug target. To further this goal, we report three crystal structures of SmCB1 complexed with peptidomimetic inhibitors as follows: the epoxide CA074 at 1.3 Å resolution and the vinyl sulfones K11017 and K11777 at 1.8 and 2.5 Å resolutions, respectively. Interactions of the inhibitors with the subsites of the active-site cleft were evaluated by quantum chemical calculations. These data and inhibition profiling with a panel of vinyl sulfone derivatives identify key binding interactions and provide insight into the specificity of SmCB1 inhibition. Furthermore, hydrolysis profiling of SmCB1 using synthetic peptides and the natural substrate hemoglobin revealed that carboxydipeptidase activity predominates over endopeptidolysis, thereby demonstrating the contribution of the occluding loop that restricts access to the active-site cleft. Critically, the severity of phenotypes induced in the parasite by vinyl sulfone inhibitors correlated with enzyme inhibition, providing support that SmCB1 is a valuable drug target. The present structure and inhibitor interaction data provide a footing for the rational design of anti-schistosomal inhibitors.
- MeSH
- Hemoglobins chemistry MeSH
- Hydrolysis drug effects MeSH
- Protease Inhibitors chemistry MeSH
- Cathepsin D antagonists & inhibitors chemistry genetics MeSH
- Crystallography, X-Ray MeSH
- Drug Delivery Systems MeSH
- Humans MeSH
- Peptidomimetics chemistry MeSH
- Peptides chemistry MeSH
- Helminth Proteins antagonists & inhibitors chemistry genetics MeSH
- Schistosoma mansoni enzymology genetics MeSH
- Schistosomiasis mansoni drug therapy enzymology genetics MeSH
- Structure-Activity Relationship MeSH
- Animals MeSH
- Check Tag
- Humans MeSH
- Animals MeSH
- Publication type
- Journal Article MeSH
- Research Support, Non-U.S. Gov't MeSH
- Research Support, U.S. Gov't, Non-P.H.S. MeSH
Cílem práce bylo retrospektivní zhodnocení klinických a anatomických rysů u dětí s pozdní manifestací vrozené hraniční kýly po novorozeneckém období. V souboru bylo 11 chlapců a 7 dívek, věk v době diagnózy se pohyboval v rozmezí od 2 do 78 měsíců. Brániční kýla se diagnostikovala u 15 dětí pri neakutních projevech a u tří dětí při urgentní manifestaci. Posterolaterální defekt v bránici byl u 11 dětí na levé straně a u 7 dětí na straně pravé. Kýlní vak se nalezl u 11 dětí. Herniovanými orgány do hrudníku byly střevní kličky u 12 dětí, játra u 7, slezina u 6, žaludek u 5 a dále ledvina, pankreas a omentum. Defekt v bránici se u 17 dětí uzavřel primární plastikou, v jednom prípade se použila záplata z Goretexu. Strangulace střeva si u jednoho chlapce vyžádala resekcí nekrotické části střeva a chlapec zemřel měsíc po operaci na multiorgánové selhání pri dlouhodobé resuscitační péči. Ostatní děti se zahojily bez komplikací. Vrozená brániční kýla se musí diferenciálně diagnosticky zvážit u každého dítěte s neobvyklými respiračními nebo gastrointestinálními symptomy a abnormálním RTG nálezem na snímku hrudníku. Akutní uskrinutí vrozené brániční kýly po novorozeneckém období je život ohrožující onemocnění, pri kterém se kombinuje gastrointestinální obstrukce s respiračním a oběhovým selháním.
The aim of the work was to evaluate clinical and anatomical features in children with late manifestations of inborn diahragma hernia after the newborn period. The group included 11 boys and 7 girls at the age of two to 78 months during the time of diagnosis. The diaphragma hernia was diagnosed in 15 children during non-acute maiúfestations and in three children affected by emergencies. A posterior-lateral defect of diaphragma was on the left side in 11 children and in 7 children on the left. A hernia sac was found in 11 children. Organs protruding into thorax included intestinal loops in 12 children, liver in 7 subjects, spleen in 6 individuals, stomach in five and kidney, pancreas and omentum in one each. The defect in diaphragma was occluded by a primary plasty in 17 children, while a patch from Goretex was used once. An intestinal strangulation required resection of necrotic part of intestine, but the boy died one month later for a multi-organ failure despite long-term resuscitation care. The other children were cured up without complications. The inborn diaphragma hernia should be considered in differential diagnosis of every child with unusual respiratory or gastrointestinal symptoms and abnormal X-ray picture of thorax. Acute strangulation of inborn diaphragma hernia after the newborn period is a life-threatening disease, where the gastrointestinal obstruction is combined with respiratory and circulation failure.
