Severe type I plasminogen deficiency is the underlying cause of ligneous conjunctivitis (LC). Furthermore, pseudomembranes may also be found on other mucous membranes (gastrointestinal tract, ...bronchial system, genital tract). In very rare cases, congenital hydrocephalus has been associated with the more severe forms of the disease and may even precede LC. The pathophysiological mechanism is unclear at present. It is advisable to look for plasminogen deficiency in patients with congenital hydrocephalus, because obstruction of ventriculoperitoneal shunts is possible when such a condition is overlooked. Here, we report a case of LC with hydrocephalus. This report reemphasizes the association of LC with hydrocephalus which is not well known.
Zusammenfassung
Hintergrund
Rotaviren sind weltweit die häufigste Ursache von schweren Durchfallserkrankungen im Kindesalter. Sowohl in den Industrie- als auch in den Entwicklungsländern sind ...Rotavirusinfektionen mit einer hohen Morbidität verbunden.
Ergebnisse
Zwei orale Rotaviruslebendimpfstoffe (Rotateq®, Rotarix®), die in Europa zugelassen sind, haben ihre Wirksamkeit und Sicherheit vorher bei vielen Tausenden von Kindern weltweit erwiesen. Die Einführung der Rotavirusimpfung in den allgemeinen Impfkalender hat in mehreren Ländern (Österreich, USA etc.) bereits nach 1–2 Jahren zu einer drastischen Reduktion der Rotaviruserkrankungen und Hospitalisationen wegen Rotavirusgastroenteritis geführt. Vorbild in Deutschland sind hier die Bundesländer Sachsen, Mecklenburg-Vorpommern, Brandenburg und Thüringen.
Schlussfolgerung
Es ist zu hoffen, dass in baldiger Zukunft die Rotavirusimpfung durch die STIKO (Ständige Impfkommission) in allen Bundesländern empfohlen wird und dann auch die Frage der Kostenübernahme durch die Krankenkassen geklärt ist.
Children with cancer or stem cell transplantation (SCT) are at considerable risk to develop life threatening viral infections. Due to both underlying disease and immunosuppressive therapy lymphocyte ...number and function are low and the cellular immunity against viral infections is restricted or missing. As immunosuppressive treatment regimens and mismatched or T-cell-depleted stem cell products are being used increasingly, viral infections will become an even greater problem in the future. PCR-based methods have become an indispensable tool for early recognition, preemptive therapy, and monitoring therapeutic responses by qualitative and quantitative approaches. Assays are now available that allow for parallel screening of the 16 most common viral agents. Responses to antiviral therapy are often limited in immunocompromised patients and mainly depend on the time of their initiation. Most antiviral agents have a toxicity profile that may become clinically relevant and curtail antiviral therapy. New options for treatment are therefore warranted. For the next future, these may include the transfer of specific T-cells and other immunotherapeutic approaches. This article provides the recommendations of the Infectious Diseases Working Party of the German Society for Pediatric Hematology/Oncology (GPOH) and the German Society for Pediatric Infectious Diseases (DGPI) for diagnosis and treatment of viral infections in children with cancer or post HSCT. They are based on the results of clinical trials, case series and expert opinions using the evidence criteria set forth by the Infectious Diseases Society of America (IDSA).
Anion exchange plays an important role in renal ion transport and acidification. To further understand the molecular nature of renal epithelial anion exchange, we used a monoclonal antibody to the ...membrane domain (52 kDa) of human erythrocyte band 3 protein to immunocytochemically search for this polypeptide in the rabbit kidney. In cryostat sections, a subpopulation of cells in the cortical and outer medullary collecting tubules showed immunoreactivity; labeling was restricted to the basolateral membrane. Proximal tubules and thick and thin limbs of Henle showed no immunoreactivity. Approximately 11% of cells in the cortical, but 43% of cells in the medullary, collecting tubule were positive for band 3. To determine the type of cells that were band 3 positive, mitochondria-rich (intercalated) cells were identified by their positive histochemical staining for succinic dehydrogenase activity and by their ability to bind peanut lectin at the apical membrane. In the cortical collecting tubule, the majority of mitochondria-rich cells bound peanut lectin but were band 3 negative; the remainder were band 3 positive but lectin negative. This distribution was reversed in the inner stripe of the outer medulla: all mitochondria-rich cells were band 3 positive and lectin negative. Thus mitochondria-rich cells are of at least two types, each of which has a distinct axial distribution pattern. Given available information about in vitro HCO3 transport properties of rabbit collecting tubules, it is likely that the lectin-positive, band 3-negative mitochondria-rich cells secrete HCO3, whereas the lectin-negative, band 3-positive cells reabsorb HCO3 (secrete H).
In an effort to discover a potent ultrashort-acting mu opioid analgetic that is capable of metabolizing to an inactive species independent of hepatic function, several classes of 4-anilidopiperidine ...analgetics were synthesized and evaluated. One series of compounds displayed potent mu opioid agonist activity with a high degree of analgesic efficacy and an ultrashort to long duration of action. These analgetics, 4-(methoxycarbonyl)-4-(1-oxopropyl)phenylamino-1-piperidinepropanoi c acid alkyl esters, were evaluated in vitro in the guinea pig ileum for mu opioid activity, in vivo in the rat tail withdrawal assay for analgesic efficacy and duration of action, and in vitro in human whole blood for their ability to be metabolized in blood. Compounds in this series were all shown to be potent mu agonists in vitro, but depending upon the alkyl ester substitution the potency and duration of action in vivo varied substantially. The discrepancies between the in vitro and in vivo activities and variations in duration of action are probably due to different rates of ester hydrolysis by blood esterase(s). The SAR with respect to analgesic activity and duration of action as a function of the various esters synthesized is discussed. It was also demonstrated that the duration of action for the ultrashort-acting analgetic, 8, does not change upon prolonged infusion or administration of multiple bolus injections.
The objective of this study was to evaluate the ability of weighted-incidence syndromic combination antibiograms (WISCAs) to inform the selection of empirical antibiotic regimens for suspected ...paediatric bloodstream infections (BSIs) by comparing WISCAs derived using data from single hospitals and from a multicentre surveillance dataset.
WISCAs were developed by estimating the coverage of five empirical antibiotic regimens for childhood BSI using a Bayesian decision tree. The study used microbiological data on ∼2000 bloodstream isolates collected over 2 years from 19 European hospitals. We evaluated the ability of a WISCA to show differences in regimen coverage at two exemplar hospitals. For each, a WISCA was first calculated using only their local data; a second WISCA was calculated using pooled data from all 19 hospitals.
The estimated coverage of the five regimens was 72%-86% for Hospital 1 and 79%-94% for Hospital 2, based on their own data. In both cases, the best regimens could not be definitively identified because the differences in coverage were not statistically significant. For Hospital 1, coverage estimates derived using pooled data gave sufficient precision to reveal clinically important differences among regimens, including high coverage provided by a narrow-spectrum antibiotic combination. For Hospital 2, the hospital and pooled data showed signs of heterogeneity and the use of pooled data was judged not to be appropriate.
The Bayesian WISCA provides a useful approach to pooling information from different sources to guide empirical therapy and could increase confidence in the selection of narrow-spectrum regimens.
Chronic metabolic acidosis (CMA) in the rabbit upregulates carbonic anhydrase (CA) IV in the proximal convoluted tubule (PCT). This study was designed to assess CA IV expression in a model of CMA in ...the mouse, i.e., congenital deficiency in CA II CA(II)D. In female CA(II)D mice, CA IV specific activity but not CA IV immunoreactivity was upregulated in the renal cortex, specifically in microdissected PCTs. Western blot analysis showed higher expression of CA IV immunoreactive protein in renal membranes from males than in those from females.
Infection by the Epstein–Barr virus (EBV) in immunocompetent individuals seems mainly confined to antigen-experienced memory B cells. However, a recent report shows that EBV
+ post-transplant ...lymphoproliferative disease might arise not only from memory B cells but also from naı̈ve and germinal center (GC) B cells. Intriguingly, some of the EBV-positive B-cell clones seem to carry non-functional Ig-V-region genes as a result of deleterious somatic mutations acquired during the GC reaction. Given that such GC B cells are destined to die by apoptosis in the absence of EBV, these findings suggest that transformation by EBV might bypass negative selection of B cells within GCs.
The processes by which chloride is transported by the cortical and outer medullary collecting tubule have been most extensively studied using in vitro microperfusion of rabbit tubules. Chloride ...appears to be transported by three major mechanisms. First, Cl can be actively reabsorbed by an electroneutral Cl-HCO3 exchanger localized to the apical membrane of the HCO3-secreting (beta-type) intercalated cell. Cl exits this cell via a basolateral Cl channel. This anion exchange process can also operate in a Cl self-exchange mode, is stimulated acutely by beta-adrenergic agonists and cAMP, and is regulated chronically by in vivo acid-base status. Second, Cl can diffuse passively down electrochemical gradients via the paracellular pathway. Although this pathway does not appear to be selectively permeable to Cl, it is large enough to allow for significant passive reabsorption. Third, Cl undergoes recycling across the basolateral membrane of the H+-secreting (alpha-type) intercalated cell. HCO3 exit from this cell brings Cl into the cell via electroneutral Cl-HCO3 exchange; Cl then exits the cell via a Cl channel. Cl transport is thus required for acidification and alkalinization of the urine. Both of these processes exist in the cortical collecting tubule. Their simultaneous operation allows fine tuning of acid-base excretion. In addition, these transport systems, when functioning at equal rates, effect apparent electrogenic net Cl absorption without changing net HCO3 transport. These systems may play an important role in regulating Cl balance.
Plasma values for u-PA in children Zeitler, P; Schuster, V
European journal of pediatrics,
12/1999, Letnik:
158 Suppl 3, Številka:
S3
Journal Article
Recenzirano
Urokinase-type plasminogen activator (u-PA) is one major activator of plasminogen. It is also involved in tissue remodelling, angiogenesis, cell migration, and tumour metastasis. In adults, increased ...u-PA levels have been identified in patients with chronic liver disease. No data exist for u-PA plasma levels in children. In the present study, u-PA plasma levels were measured by ELISA in 95 healthy children and adolescents aged 7 months to 17 years. We found a median value of 1.06 ng/ml u-PA (range 0.43-15.78 ng/ml), which is similar to that found in adults (2-20 ng/ml). No differences between males and females were recorded. In addition, we determined u-PA plasma levels of 16 patients with severe or mild type I plasminogen deficiency and found a median value of 1.06 ng/ml (range 0.69-7.7 ng/ml).
Normal plasma u-PA values in healthy children and adults are virtually no different from those reported in adults.
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