Visserij in cijfers 2003

Deze jaarlijkse rapportage over de Nederlandse visserij bevat gegevens over de bedrijfsresultaten en financiële positie van de kottervisserij in 2003, met vergelijkende cijfers van voorgaande jaren. Naast de bedrijfseconomische gegevens van de kottervisserij, wordt ook ingegaan op resultaten van de grote zeevisserij en op ontwikkelingen in de buitenlandse handel. This annual report on Dutch fisheries contains data on the business results and the financial position of cutter fishing in 2003, with comparative figures from previous years. Alongside the economic data of cutter fishing, results of large-scale high sea fishing and developments in foreign trade are also examined

Serological and genetic complement alterations in infection-induced and complement-mediated hemolytic uremic syndrome

Background: The role of complement in the atypical form of hemolytic uremic syndrome (aHUS) has been investigated extensively in recent years. As the HUS-associated bacteria Shiga-toxin-producing Escherichia coli (STEC) can evade the complement system, we hypothesized that complement dysregulation is also important in infection-induced HUS. Methods: Serological profiles (C3, FH, FI, AP activity, C3d, C3bBbP, C3b/c, TCC, αFH) and genetic profiles (CFH, CFI, CD46, CFB, C3) of the alternative complement pathway were prospectively determined in the acute and convalescent phase of disease in children newly diagnosed with STEC-HUS or aHUS. Serological profiles were compared with those of 90 age-matched controls. Results: Thirty-seven patients were studied (26 STEC-HUS, 11 aHUS). In 39 % of them, including 28 % of STEC-HUS patients, we identified a genetic and/or acquired complement abnormality. In all patient groups, the levels of investigated alternative pathway (AP) activation markers were elevated in the acute phase and normalized in remission. The levels were significantly higher in aHUS than in STEC-HUS patients. Conclusions: In both infection-induced HUS and aHUS patients, complement is activated in the acute phase of the disease but not during remission. The C3d/C3 ratio displayed the best discrepancy between acute and convalescent phase and between STEC-HUS and aHUS and might therefore be used as a biomarker in disease diagnosis and monitoring. The presence of aberrations in the alternative complement pathway in STEC-HUS patients was remarkable, as well

Genetic drivers of kidney defects in the digeorge syndrome

BACKGROUND The DiGeorge syndrome, the most common of the microdeletion syndromes, affects multiple organs, including the heart, the nervous system, and the kidney. It is caused by deletions on chromosome 22q11.2; the genetic driver of the kidney defects is unknown. METHODS We conducted a genomewide search for structural variants in two cohorts: 2080 patients with congenital kidney and urinary tract anomalies and 22,094 controls. We performed exome and targeted resequencing in samples obtained from 586 additional patients with congenital kidney anomalies. We also carried out functional studies using zebrafish and mice. RESULTS We identified heterozygous deletions of 22q11.2 in 1.1% of the patients with congenital kidney anomalies and in 0.01% of population controls (odds ratio, 81.5; P = 4.5×1014). We localized the main drivers of renal disease in the DiGeorge syndrome to a 370-kb region containing nine genes. In zebrafish embryos, an induced loss of function in snap29, aifm3, and crkl resulted in renal defects; the loss of crkl alone was sufficient to induce defects. Five of 586 patients with congenital urinary anomalies had newly identified, heterozygous protein-Altering variants, including a premature termination codon, in CRKL. The inactivation of Crkl in the mouse model induced developmental defects similar to those observed in patients with congenital urinary anomalies. CONCLUSIONS We identified a recurrent 370-kb deletion at the 22q11.2 locus as a driver of kidney defects in the DiGeorge syndrome and in sporadic congenital kidney and urinary tract anomalies. Of the nine genes at this locus, SNAP29, AIFM3, and CRKL appear to be critical to the phenotype, with haploinsufficiency of CRKL emerging as the main genetic driver

Acceptability of liquid human growth hormone (hGH) (Norditropin SimpleXx) in adults and children with GH deficiency and children with chronic renal disease.

OBJECTIVE: Human growth hormone (GH) is well established as a treatment for growth hormone deficiency. Recently, a new liquid GH formulation (Norditropin SimpleXx®) has become available that does not require reconstitution before subcutaneous self-administration with a new delivery system (NordiPen™) and an optional auto-injector (NordiPenmate™). In this study the acceptability and tolerability of and compliance with the liquid formulation were investigated DESIGN AND PATIENTS: An open 6-week multicentre trial in 53 patients, including adults and children, was performed in The Netherlands. Acceptability and tolerability of the liquid GH formulation were assessed by questionnaires and compliance by both questionnaires and re-collection of GH cartridges MAIN OUTCOME MEASURES AND RESULTS: The mean daily dosage of GH was 0.24 mg/m2 (0.03 to 0.82 mg/m2) in adults and 1.13 mg/m2 (0.65 to 1.77 mg/m2) in children. Most patients (91% of adults and 89% of children) preferred to continue on the new GH formulation rather than the previously used formulation. This was mainly due to the easier and less time-consuming procedure and the good tolerance of the drug. Self-reported compliance also improved, but this could not be proven due to poor return of study cartridges CONCLUSIONS: The use of the liquid GH formulation Norditropin SimpleXx® with the NordiPen™ device and optional use of the NordiPenmate™ device was well accepted by both adults and children. This was mainly due to the avoidance of reconstitution and the ease of handling of the injection device

Pediatric acute kidney injury in the ICU: an independent evaluation of pRIFLE criteria

OBJECTIVE: The present study was undertaken to evaluate the practicability of the proposed pediatric RIFLE (pRIFLE) criteria in a patient population at risk for acute kidney injury (AKI) and to analyze the prevalence and association of AKI as defined by pRIFLE with mortality. DESIGN: Retrospective, descriptive cohort study. SETTING: Single-center, 9-bed PICU facility. PATIENTS: Children with respiratory failure requiring mechanical ventilation for more than 4 days admitted between January 2002 and December 2006. INTERVENTIONS: None. MEASUREMENTS AND RESULTS: Data of 103 patients were studied. Median age was 4.5 years (range 1 month-17 years). Six patients received renal replacement therapy. Seventeen patients (17%) died. Sixty patients (58%) developed AKI by pRIFLE. Mean time to attainment of the first RIFLE stratum was 1.9 +/- 1.6 days. By pRIFLE, 34 of the 60 patients fulfilled the maximum AKI criteria on the first day after admission based on the estimated creatinine clearance criterion. Patients with AKI according to the pRIFLE scoring system had five times higher mortality than patients without AKI (25 vs. 5%, P < 0.05). CONCLUSIONS: We observed a high incidence of significant AKI in a PICU population at risk, which was associated with high mortality. Pediatric RIFLE criteria may guide in the early identification of patients at risk for AKI and in the initiation of therapy

Nephrotic syndrome in The Netherlands: a population-based cohort study and a review of the literature

Nephrotic syndrome (NS) is a clinical diagnosis with proteinuria, hypoalbuminaemia and oedema. NS is rare in children, and its incidence in The Netherlands is unknown. The aim of this study was to estimate the incidence of idiopathic NS in the Netherlands. All paediatric patients (age 0–18 years) with a newly diagnosed NS in the Netherlands were registered by the Dutch Pediatric Surveillance Unit during the years 2003 until 2006, secondary NS was excluded. All paediatricians filled out questionnaires about the first clinical findings of the patients and incidences were calculated. A literature review on incidences of childhood NS was conducted. The incidence of NS in children in the Netherlands in the years 2003 until 2006 was 1.52/ 100, 000 children/ year. The median age at diagnosis was 3.88 years with a mean age of 5.08 years. A significant male:female ratio of 2.04:1 was found. This prospective study of NS in the Netherlands revealed an incidence of 1.52:100, 000 children/year, and is similar to the incidences found all over the world