Identification and application of quality elements, indicators, and criteria for assessment of impact on habitats in marine Natura 2000 areas.

The EU Habitat Directive adopted in 1992, requires member states of the European Union to protect species and habitats considered to be of 'Community Interest' and listed in annexes to the directive. The appropriate environmental assessment of "plans and projects" is an important part of the conservation process. Despite several amendments and guidelines supporting the implementation of the Habitat Directive, science based operational procedures, indicators, and impact criteria for assessing potential negative impacts on marine Natura 2000 areas are still lacking.

Submerged aquatic vegetation: Overview of monitoring techniques used for the identification and determination of spatial distribution in European coastal waters.

Coastal waters are highly productive and diverse ecosystems, often dominated by marine submerged aquatic vegetation (SAV) and strongly affected by a range of human pressures. Due to their important ecosystem functions, for decades, both researchers and managers have investigated changes in SAV abundance and growth dynamics to understand linkages to human perturbations. In European coastal waters, monitoring of marine SAV communities traditionally combines diver observations and/or video recordings to determine, for example, spatial coverage and species composition.

A spatial model for nutrient mitigation potential of blue mussel farms in the western Baltic Sea.

Worldwide, coastal and marine policies are increasingly aiming for environmental protection, and eutrophication is a global challenge, particularly impairing near-coastal marine water bodies. In this context, mussel mitigation aquaculture is currently considered an effective tool to extract nutrients from such water bodies. Mussel mitigation farming using longline systems with loops of collector material is a well-developed technology and considered promising in the western Baltic Sea.

From the Children's Oncology Group: Evidence-Based Recommendations for PEG-Asparaginase Nurse Monitoring, Hypersensitivity Reaction Management, and Patient/Family Education.

PEG-aspariginase is a backbone chemotherapy agent in pediatric acute lymphoblastic leukemia and in some non-Hodgkin lymphoma therapies. Nurses lack standardized guidelines for monitoring patients receiving PEG-asparaginase and for educating patients/families about hypersensitivity reaction risks. An electronic search of 6 databases using publication years 2000-2015 and multiple professional organizations and clinical resources was conducted.

Response to cyclosporine in steroid-resistant nephrotic syndrome: discontinuation is possible.

Background: Steroid-resistant nephrotic syndrome (SRNS) is still regarded as a serious disease although treatment with cyclosporine (CSA) has improved outcome. However, the duration of treatment in responders is unclear, and treatment of patients with genetic causes is a matter of debate.

Methods: Thirty-six patients with SRNS were studied retrospectively.

Clinical and molecular characterization of patients with heterozygous mutations in wilms tumor suppressor gene 1.

Background And Objectives: The Wilms tumor suppressor gene 1 (WT1) plays an essential role in urogenital and kidney development. Genotype/phenotype correlations of WT1 mutations with renal function and proteinuria have been observed in world-wide cohorts with nephrotic syndrome or Wilms tumor (WT). This study analyzed mid-European patients with known constitutional heterozygous mutations in WT1, including patients without proteinuria or WT.

Mussels as a tool for mitigation of nutrients in the marine environment.

Long-line mussel farming has been proposed as a mitigation tool for removal of excess nutrients in eutrophic coastal waters. A full-scale mussel farm optimized for cost efficient nutrient removal was established in the eutrophic Skive Fjord, Denmark where biological and economic parameters related to nutrient removal was monitored throughout a full production cycle (1 yr). The results showed that it was possible to obtain a high area specific biomass of 60 t WW ha(-1) eqvivalent to a nitrogen and phosphorus removal of 0.

Hypoxia in the Baltic Sea: biogeochemical cycles, benthic fauna, and management.

Hypoxia has occurred intermittently over the Holocene in the Baltic Sea, but the recent expansion from less than 10 000 km(2) before 1950 to >60 000 km(2) since 2000 is mainly caused by enhanced nutrient inputs from land and atmosphere. With worsening hypoxia, the role of sediments changes from nitrogen removal to nitrogen release as ammonium. At present, denitrification in the water column and sediments is equally important.

Effects of bioturbation on the fate of oil in coastal sandy sediments--an in situ experiment.

Effects of bioturbation by the common lugworm Arenicola marina on the fate of oil hydrocarbons (alkanes and PAHs) were studied in situ during a simulated oil spill in a shallow coastal area of Roskilde fjord, Denmark. The fate of selected oil compounds was monitored during 120 d using GC-MS and bioturbation activity (feces production and irrigation) was measured regularly during the experiment and used as input parameters in a mechanistic model describing the effects of A. marina on the transport and degradation of oil compounds in the sediment.

Percutaneous endoscopic gastrostomy in children on peritoneal dialysis.

Objective: Insertion of percutaneous endoscopic gastrostomies (PEG) in patients on chronic peritoneal dialysis (PD) has been reported to be contraindicated due to an increased risk of morbidity and mortality. However, no systematic survey on this topic has yet been published.

Design: Retrospective multicenter study.

Type I IgE receptor, interleukin 4 receptor and interleukin 13 polymorphisms in children with nephrotic syndrome.

Polymorphisms in the genes encoding the high-affinity IgE receptor, the interleukin 4 (IL4) receptor and IL13 can be associated with the development of asthma and allergy. Although several studies have described an association between atopy and idiopathic childhood nephrotic syndrome (NS), it is not clear whether this association is of a causal nature. Furthermore, it is not known whether these polymorphisms are associated with the clinical course of NS.

Antenatal oligohydramnios of renal origin: postnatal therapeutic and prognostic challenges.

Urinary tract anomalies (UTA) including polycystic kidney disease nowadays can be detected antenatally by ultrasound. The concomitant presence of oligohydramnios has been regarded as a severe risk factor for renal dysfunction and pulmonary hypoplasia, although clinical data after birth are scarce. We report the postnatal course and long-term follow-up of 10 infants with oligohydramnios due to congenital UTA from two pediatric nephrology centers.

Unfavorable response to cyclophosphamide in steroid-dependent nephrotic syndrome.

Development of steroid dependency represents a significant therapeutic challenge in steroid-sensitive nephrotic syndrome. Previous studies have shown conflicting results concerning the benefit of a 12-week treatment with cyclophosphamide (CPO), with 24%-67% of patients achieving long-term remission. We therefore analyzed the clinical response of 20 consecutive children with steroid-dependent nephrotic syndrome (SDNS) (12 male, median age at start of treatment 5.

Functional characterization of the molecular defects causing nephrogenic diabetes insipidus in eight families.

X-Linked nephrogenic diabetes insipidus (NDI) is a rare inherited disorder characterized by the excretion of abnormal large volumes of diluted urine mainly caused by mutations in the V2 vasopressin receptor (AVPR2) gene. By screening NDI patients for mutations within the AVPR2 gene we have identified three novel (I46K, F105V, I130F) and four recurrent (D85N, R106C, R113W, Q225X) mutations. In addition, a recurrent missense mutation (A147T) within the aquaporin-2 gene was identified in a female patient with autosomal recessive NDI associated with sensorineural deafness.

Iron homeostasis in relapsing steroid-sensitive nephrotic syndrome of childhood.

Aim: Urinary transferrin loss is a typical feature in relapse of the idiopathic nephrotic syndrome, however, the impact on serum iron homeostasis and hematological parameters has not been studied systematically so far.

Patients And Methods: Therefore, we investigated serum iron (Fe), erythropoietin (EPO), ferritin (FN), transferrin (TF), total iron-binding capacity (TEBK), transferrin saturation and the soluble transferrin receptor (sTFR) combined with hematological parameters (hemoglobin, MCV, MCH) in 42 children with relapsing, steroid-sensitive nephrotic syndrome (NS) in remission (RM, n = 26) and relapse (RL, n = 16), including 13 patients who were studied in both states. Thirty-three age-matched healthy children served as controls.

Preemptive liver transplantation in primary hyperoxaluria type 1: timing and preliminary results.

Preemptive isolated liver transplantation (PLTX) can cure the metabolic defect in primary hyperoxaluria type 1 (PH1) but there are no uniformally accepted recommendations concerning the timing of this transplantation procedure. We have performed PLTX successfully in 4 children (age 3-9 years) with PH1 with no mortality or morbidity due to the transplantation procedure. Plasma and urinary oxalate levels normalised rapidly and renal function remained stable including one patient with advanced chronic renal failure who showed a stable course for more than 24 months.

[The treatment with levamisole of frequently recurring steroid-sensitive idiopathic nephrotic syndrome in children].

Background And Objective: The treatment of frequently relapsing steroid-sensitive nephrotic syndrome in children with established immunosuppressive drugs (steroids, cyclophosphamide, cyclosporin A) sometimes presents problems because of the expected incidence of side effects. Stimulation of the immune system with the anthelminthic drug levamisole in this disease has been documented. Aim of this study was to assess in a prospective but uncontrolled series of observations its value and side effects.

[Acute reversible encephalopathy with brain edema and serial seizures in pseudohypoparathyroidism].

A 16 year old patient with the typical clinical signs of Albright's hereditary dystrophia developed series of epileptic seizures with loss of consciousness, tonic muscle contractions and bite of the tongue. After termination of the seizures there was coma without focal neurological signs. CT scan revealed diffuse brain edema.