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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. The lack of a generic and operational methodology complicates the management of plans and projects with potential impact on marine Natura 2000 areas. In this study, generic methods for the assessment of marine aquaculture in the inner Danish waters in relation to Natura 2000 areas was developed and applied for assessment of nine existing marine fin fish farms, in accordance with the latest methodological guidance on the provisions of Article 6(3) and (4) of the Habitat Directive. The applied methodology is based on high resolution 3D hydrodynamic- and ecosystem modelling (MIKE by DHI), that describes the dynamical physical, chemical, and biogeochemical processes and changes of marine ecosystems in time and space. To our knowledge, this is the first study that formulates operational biological quality elements, key indicators, concrete and generic impact criteria, and assessment procedures for operational assessment across several distinct marine habitat types. The method represents a generic, operational, transparent, and science-based assessment tool, that simplifies management, and is widely applicable for quantification of environmental impacts from various marine activities and eutrophication related pressures across geographical zones and different marine habitat types in marine Natura 2000 areas., Competing Interests: Declaration of competing interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2024 Elsevier Ltd. All rights reserved.)

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. While these techniques provide very useful data, they are rather time consuming, labor-intensive, and limited in their spatial coverage. In this study, we compare traditional and emerging remote sensing technologies used to monitor marine SAV, which include satellite and occupied aircraft operations, aerial drones, and acoustics. We introduce these techniques and identify their main strengths and limitations. Finally, we provide recommendations for researchers and managers to choose the appropriate techniques for future surveys and monitoring programs. Integr Environ Assess Manag 2022;18:892-908. © 2021 SETAC., (© 2021 SETAC.)

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. Besides several spatially limited field studies, a suitable spatial model for site-specific implementation is still lacking. In this study, we present a modular spatial model, consisting of a spatial and temporal habitat factor model (Module 1), blue mussel growth model (Module 2), mussel farm model (Module 3), and an avoidance of food limitation model (Module 4). The modules integrate data from in situ monitoring, mussel growth experiments, and eco-physiological modelling for the western Baltic Sea, to estimate spatially explicit nutrient reduction potentials. The model is flexible with respect to farm setups and harvest times and considers natural variability, model uncertainty, and required hydrodynamics. Modelling results proved valid at all scales and modules, and point out key areas for efficient mussel mitigation farms in Danish, German and Swedish areas. Modelled long-term mean mitigation potentials for harvest in November reach up to 0.88 t N /ha and 0.05 t P /ha for a farm setup using 2 m depth-range of the water column and 3.0 t N /ha and 0.17 t P /ha using up to 8 m, respectively. For Danish water bodies, we demonstrate that in efficient areas, mitigation farms (18.8 ha, 90 km collector substrate in loops with 2 m depth-range) required <3.6% of the space to extract the target nitrogen loads for good ecological status. The developed approach could prove valuable for implementing environmental policies in aquatic systems, e.g. in situ nutrient mitigation, aquaculture spatial planning, and habitat suitability mapping., Competing Interests: Declaration of competing interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2020. Published by Elsevier B.V.)

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. Evidence sources were reviewed for topic applicability. Each of the final 23 sources was appraised by 2 team members. The GRADE (Grading of Recommendations Assessment, Development and Evaluation) system was used to assign a quality and strength rating for each recommendation. Multiple recommendations were developed: 4 relating to nurse monitoring of patients during and after drug administration, 8 guiding hypersensitivity reaction management, and 4 concerning patient/family educational content. These strong recommendations were based on moderate, low, or very-low-quality evidence. Several recommendations relied on generalized drug hypersensitivity guidelines. Additional research is needed to safely guide PEG-asparaginase monitoring, hypersensitivity reaction management, and patient/family education. Nurses administering PEG-asparaginase play a critical role in the early identification and management of hypersensitivity reactions.

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. Median age at presentation was 3.2 (range, 0.06-15.0) and median follow-up 15.5 years (range, 1.8-27.7), respectively; 23 (64%) had focal segmental glomerulosclerosis (FSGS) on biopsy. In 33/36 patients (92%), genetic testing was performed for at least three most common genes known to be mutated in SRNS., Results: Nineteen patients (53%), especially those with minimal change nephrotic syndrome (MCNS) at initial biopsy (p < 0.002), entered complete remission with CSA monotherapy, including one patient with compound heterozygous NPHS1 and dominant ACTN4 mutation, respectively. Ten patients entered partial remission (28%, all FSGS), including two with NPHS2 mutations. Seven patients (six FSGS, one MCNS) did not respond to treatment. In 15 of 19 responders to CSA, treatment was stopped after a median of 3.1 years (range, 0.5-14) and no further relapses occurred in 11/15 (73%) patients with median follow-up of 9.7 years., Conclusions: CSA monotherapy is effective in SRNS. Discontinuation of CSA is possible in many patients with complete remission.

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., Design, Setting, Participants & Measurements: Retrospective analysis of genotype, phenotype, and treatment of 53 patients with WT1 mutation from all pediatric nephrology centers in Germany, Austria, and Switzerland performed from 2010 to 2012., Results: Median age was 12.4 (interquartile range [IQR], 6-19) years. Forty-four of 53 (83%) patients had an exon mutation (36 missense, eight truncating), and nine of 53 (17%) had an intronic lysine-threonine-serine (KTS) splice site mutation. Fifty of 53 patients (94%) had proteinuria, which occurred at an earlier age in patients with missense mutations (0.6 [IQR, 0.1-1.5] years) than in those with truncating (9.7 [IQR, 5.7-11.9]; P<0.001) and splice site (4.0 [IQR, 2.6-6.6]; P=0.004) mutations. Thirteen of 50 (26%) were treated with steroids and remained irresponsive, while three of five partially responded to cyclosporine A. Seventy-three percent of all patients required RRT, those with missense mutations significantly earlier (at 1.1 [IQR, 0.01-9.3] years) than those with truncating mutations (16.5 [IQR, 16.5-16.8]; P<0.001) and splice site mutations (12.3 [IQR, 7.9-18.2]; P=0.002). Diffuse mesangial sclerosis was restricted to patients with missense mutations, while focal segmental sclerosis occurred in all groups. WT occurred only in patients with exon mutations (n=19). Fifty of 53 (94%) patients were karyotyped: Thirty-one (62%) had XY and 19 (38%) had XX chromosomes, and 96% of male karyotypes had urogenital malformations., Conclusions: Type and location of WT1 mutations have predictive value for the development of proteinuria, renal insufficiency, and WT. XY karyotype was more frequent and associated with urogenital malformations in most cases., (Copyright © 2015 by the American Society of Nephrology.)

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 0.6-0.9 and 0.03-0.04 t ha(-1)yr, respectively. The analysis of the costs related to establishment, maintenance and harvest revealed that mussel production optimized for mitigation can be carried out at a lower cost compared to mussel production for (human) consumption. The costs for nutrient removal was 14.8 € kg(-1)N making mitigation mussel production a cost-efficient measure compared to the most expensive land-based measures., (Copyright © 2014 Elsevier Ltd. All rights reserved.)

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. Phosphorus is currently buried in sediments mainly in organic form, with an additional contribution of reduced Fe-phosphate minerals in the deep anoxic basins. Upon the transition to oxic conditions, a significant proportion of the organic phosphorus will be remineralized, with the phosphorus then being bound to iron oxides. This iron-oxide bound phosphorus is readily released to the water column upon the onset of hypoxia again. Important ecosystems services carried out by the benthic fauna, including biogeochemical feedback-loops and biomass production, are also lost with hypoxia. The results provide quantitative knowledge of nutrient release and recycling processes under various environmental conditions in support of decision support tools underlying the Baltic Sea Action Plan.

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. The chemical analytical data and model results indicated that A. marina had profound and predictable effects on the distribution, degradation and preservation of oil and that the net effect depended on the initial distribution of oil. In sediment with an oil contaminated subsurface-layer A. marina buried the layer deeper in the sediment which clearly enhanced oil persistence. Conversely, A. marina stimulated both the physical removal and microbial degradation of oil compounds in uniformly oil contaminated sediments especially in deeper sediment layers (10-20 cm below the surface), whereas the fate of oil compounds deposited in surface layers (0-5 cm) mainly was affected by removal processes induced by wave actions and other bioturbating infauna such as Nereis diversicolor, Corophium volutator and Hydrobia spp. present in the experimental plots., (Copyright © 2010 Elsevier Ltd. All rights reserved.)

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., Setting: 23 pediatric dialysis units associated with the working group Arbeitsgemeinschaft für Pädiatrische Nephrologie (APN)., Data Source: A structured questionnaire on clinical details of PD patients who had undergone PEG insertion or open gastrostomy (OG) since 1994 was distributed to all pediatric dialysis units of the APN., Results: 27 PD patients (20 males) from 12 centers in whom PEG insertion was performed after Tenckhoff catheter introduction were evaluated. Age at intervention ranged from 0.25 to 10.9 years (median 1.3 years). Most patients were malnourished, with standard deviation score (SDS) for body weight between -4.2 and -0.6 (median -2.2). Major complications were early peritonitis < 7 days after PEG in 10/27 (37%) patients, episodes of fungal peritonitis in 7/27 (26%) patients, 4 cessations of PD and change to hemodialysis, and 2 associated deaths. However, in 14 patients, no such problems were encountered and, in 4 patients, early peritonitis effectively treated with intraperitoneal antibiotics was the only major complication. Thus, in 18/27 (67%) patients, PD was successfully reinitiated shortly after PEG insertion. Among all participating centers, only two OG procedures were reported during the study period, illustrating a clear preference for the PEG over the OG procedure among members of the APN., Conclusion: PEG insertion following PD initiation carries a high risk for fungal peritonitis and potential PD failure; however, complication rates in this largest reported series were lower than previously described. Antibiotic and antifungal prophylaxis, withholding PD for 2 - 3 days, and gastrostomy placement by an experienced endoscopy team are suggested precautions for lowering the risk of associated complications. When gastrostomy placement does not occur prior to or at the time of initiating PD, the risks and benefits of percutaneous versus open placement must be carefully weighed.

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. A total of 84 children (52 male and 32 female; mean age 12.1 years) with NS were included in the present study. Of these, 78 could be classified as either atopic or non-atopic. Atopy was defined by elevated IgE levels (>100 k-units/l) and/or a positive history of atopy (33 of 78 patients). DNA was extracted from blood collected in EDTA tubes, and polymorphisms at positions 50 and 551 of the IL4 receptor, position 110 of IL13 and position 181 of the high-affinity IgE receptor were investigated by sequence-specific PCR or direct sequencing. Although we noted a strong tendency towards a higher allele frequency of polymorphisms in children with atopy and NS compared with children with NS but without atopy (IL4 50, 30% compared with 18%; IL4 551, 39% compared with 31%; IL13 110, 45% compared with 33%; IgE 181, 12% compared with 13%), these differences did not reach statistical significance. There were no differences in the frequency of polymorphisms between the different clinical courses of NS (frequent relapsers, steroid-dependent or steroid-resistant NS). We conclude that polymorphisms in the IL4 receptor, the high-affinity IgE receptor and IL13 do not seem to predict the clinical course of NS, despite the fact that serum IgE elevations are more frequent in patients with NS than in normal control subjects. The investigated polymorphisms may contribute to the IgE switch in patients with NS.

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. The underlying final diagnoses were autosomal-recessive polycystic kidney disease (ARPKD, n = 2), familial tubular dysgenesis (n = 2) and bilateral renal hypoplasia (n = 6) including 3 children with posterior urethral valves. Two children died in the neonatal period while 8 children are currently alive at a median age of 2.5 (range 1.1-10) years. In the postnatal period, respiratory failure necessitating mechanical ventilation occurred in 7 infants (including the 2 non-survivors). All surviving children had chronic renal failure, which could be managed conservatively in 6 children (median GFR 45 (range 15-53) ml/min/1.73 m2) while 2 reached end-stage renal disease; one undergoing preemptive kidney transplantation and one peritoneal dialysis. Seven of 8 children reached normal developmental milestones. In conclusion, the presence of antenatal oligohydramnios in infants with UTA does not always carry a poor prognosis. The high incidence of perinatal complications, the complexity of underlying causes and the prevalence of postnatal chronic renal dysfunction calls for a multidisciplinary approach in the management of these children.

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 5.9 years, range 3.2-14.7 years) treated at our institution with CPO (2 mg/kg per day) for 12 weeks since 1989. Median duration of follow-up was 5.8 (range 1.1-9.25) years. Only 6 of 20 children (30%) showed a long-term remission of >2 years, while 14 of 20 (70%) developed relapses again. Of these, 12 patients (86%) again developed steroid dependency, requiring further alternative treatment. Our data show that a 12-week course of CPO leads to unfavorable results in the majority of patients with SDNS. We therefore conclude that there is a need for further optimization of therapy in SDNS.

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. Selected clinical data of the NDI patients were compared with the results from the in vitro studies. Functional analysis of I46K and I130F revealed reduced maximum agonist-induced cAMP responses as a result of an improper cell surface targeting. In contrast, the F105V mutation is delivered to the cell surface and displayed an unchanged maximum cAMP response, but impaired ligand binding abilities of F105V were reflected in a shifted concentration-response curve toward higher vasopressin concentrations. As the extracellularly located F105 is highly conserved among the vasopressin/oxytocin receptor family, functional analysis of this residue implicates an important role in high affinity agonist binding.

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., Results: Fe, TEBK and TF were significantly reduced in RL compared to RM in cross-sectional as well as in paired studies while ferritin, hematological parameters and EPO levels remained unchanged. A significant increase, however, of the soluble transferrin-receptor could be demonstrated in cross-sectional analysis comparing RL to RM and healthy controls (3568+/-713 mg/ml vs 2625+/-576 vs 2646+/-697; p < 0.001 respectively) as well as in paired analysis of 13 patients in RL and RM (p < 0.001)., Conclusion: We conclude that transient transferrin and iron deficiency occurs in RL of INS but this seems to be counterbalanced by upregulation of the sTFR, a mechanism that might be important in preventing the development of iron deficiency anemia during the active nephrotic state.

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. Although treatment must be individualised in this severe metabolic disorder and PLTX has to be viewed as invasive procedure, we feel PLTX should be offered and discussed not too late in the treatment of PH1 to prevent or at least delay the progression to end stage renal disease and systemic oxalosis.

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., Patients and Methods: 25 patients (15 boys, ten girls; median age 10 [3.5-22] years) were given levamisole, 2 mg/kg/48 h. Before this treatment was started eight of the children/adolescents (32%) had frequent relapses and 17 (68%) had become steroid-dependent. Treatment was started during steroid-induced remission and continued for 3-24 (median 6) month, while steroids were discontinued after four weeks., Results: Relapse frequency per patient month was reduced from a mean of 0.5 (0.33-0.83) before to 0.31 (0-0.67) during levamisole administration (P < 0.001). In 12 patients (48%) no or considerably fewer relapses were observed. Patients with exclusively frequent relapses responded to levamisole better than those with steroid dependence (7/8 [87.5%] vs. 5/18 [27.7%], P = 0.01). Side effects were reversible leukopenia in two patients and nonspecific skin rash as well as epigastric pain in one patient., Conclusion: Levamisole is an efficacious addition or alternative, with a low incidence of side effects, in the treatment of frequently relapsing nephrotic syndrome, particularly so in yet steroid-dependent patients.

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. Electroencephalographic studies showed generalized slowing. In laboratory tests the only abnormalities were marked hypocalcemia (1.15 mmol/l) and hyperphosphatemia. Blood parathyroid hormone (PTH) was elevated. PTH-Test confirmed the diagnosis of pseudohypoparathyroidism. The patient was treated with calcium and 1,25-dihydroxy-cholecalciferol. After few days the severe encephalopathy, CT and electroencephalographic changes were completely reversible. Hereditary disturbances of the parathyroid hormone metabolism are rare diseases. Hypocalcemia must be included into the differential diagnosis of seizures and brain edema to avoid invasive diagnostic and irrational treatment.