The UN Convention on the rights of persons with disabilities and its interpretation

This paper explores the United Nations Convention on the rights of persons with disabilities (CRPD) from a phenomenological perspective. It argues for complementing the predominant juridical approach to the CRPD with attention to the extra-juridical dimension of the constitution of its meaning. The core argument is that disabled people's collectives should be recognised and admitted as important stakeholders and contributors in the community of interpretation that gives the CRPD its meaning. After briefly introducing the CRPD, the first part of the paper highlights the ubiquity of interpretation and the limits of its juridical regulation. The second part explores some extra-juridical factors that influence the interpretation of the CRPD. Two cases are considered: the socially embedded materiality of the interpretive work of the CRPD Committee; and the politics of interpretation inherent in the CRPD's translation between languages. The latter is backed up by comparing the English, French, Russian and Bulgarian versions of several CRPD provisions. In conclusion, some methodological and programmatic inferences are drawn from the analysis. In particular, it is argued that disabled people's civic self-organising is indispensable for sustaining the interpretation of the CRPD along transformative and emancipatory lines.     

Left ventricular assist device support reverses altered cardiac expression and function of natriuretic peptides and receptors in end-stage heart failure

OBJECTIVE: Atrial (ANP) and B-type natriuretics peptides (BNP) via their guanylyl cyclase-A (GC-A) receptor not only regulate arterial blood pressure and volume but also exert local antihypertrophic, antifibrotic and lusitropic effects in the heart. To elucidate whether cardiac hypertrophy/insufficiency and reversal is associated with changes in the local responsiveness to NPs, we compared the mRNA expression of ANP, BNP and receptors and the responsiveness of GC-A to ANP in left ventricular tissue obtained from 10 patients with congestive heart failure (CHF) before and after hemodynamic unloading by left ventricular assist device (LVAD) support. METHODS AND RESULTS: Quantitative "real time" RT-PCR demonstrated that the mRNA expression levels of ANP, BNP and the NP-metabolizing NPR-C receptor were both markedly increased in human failing hearts. GC-A mRNA expression levels were not different from nonfailing hearts, but cGMP production by GC-A in response to ANP was nearly abolished. Reversal of cardiomyocyte hypertrophy during LVAD support was accompanied by normalization of ANP, BNP and NPR-C mRNA levels and a significant recovery of GC-A responsiveness to ANP. CONCLUSION: In CHF patients, increased local clearance by NPR-C receptors and diminished responsiveness of cardiac GC-A might impair the local antihypertrophic effects of natriuretic peptides and contribute to the progression of cardiac hypertrophy and insufficiency. Reverse remodeling during LVAD support reverses these changes and can thereby recuperate the local protective effects of ANP and BNP.     

Restdysplasie im Kleinkindesalter

Objective

Restoration of a congruent, stable, mobile hip joint with normal function.

Indications

Persistent acetabular dysplasia of congenital or neuromuscular origin in children with still open triradiate cartillage.

Contraindications

Age under 18 months, closed triradiate cartilage, femoral head deformity.

Surgical technique

Incomplete supraacetabular transiliac osteotomy, the correction is performed by reshaping the acetabulum taking advantage of the inherent flexibility of the triradiate cartilage and the periacetabular bone. A triangular bioresorbable xenograft is used to maintain the correction until bony healing.

Postoperative management

Cast immobilization is not necessary, non-weight bearing for 4 weeks after surgery, then full weight bearing.

Results

In all, 31 hips in 20 patients were surgically treated for persistent acetabular dysplasia with the Dega acetabuloplasty. The results were retrospectively studied. The mean age at surgery was 42 months (range 31–67 months). The mean follow-up was 31 months (range 12–60 months). The acetabular angle before surgery measured 31.5° (range 27–44°) and was corrected to 14° (range 8–20°) at last follow-up. All patients showed at last presentation normal hip range of motion and had no complaints. None of the patients had functional restrictions. Severe complications were not observed. Transitory inguinal swelling was observed in only 3 cases in the immediate postoperative period and resolved completely without sequelae.     

The freshwater cyanobacterium Lyngbya aerugineo-coerulea produces compounds toxic to mice and to mammalian and fish cells

Despite a growing awareness of the presence of cyanobacterial toxins, knowledge about the ability of specific species to produce toxic compounds is still rather limited. It was the overall goal of the current work to investigate if probes derived from the freshwater species Lyngbya aerugineo-coerulea (Kutz.) Gomont, a cyanobacterium frequently found in southern Europe and not previously investigated for the presence of bioactive compounds, were capable of eliciting in vivo and in vitro toxicity. The cyanobacterial extract revealed signs of neuro- as well as hepatotoxicity in mice, although these signs could not be explained by the well-known respective cyanobacterial neuro- and hepatotoxins saxitoxin and microcystin. Cytotoxicity was elicited by the cyanobacterial extract in all mammalian cell lines tested. As well, the rainbow trout liver cell line, RTL-W1, was found to be susceptible to the cytotoxic effects of the extract, although the cytotoxicity was dependent on temperature. In contrast, the cyanobacterial growth medium elicited cytotoxicity independent of temperature, leading to morphological changes indicative of alterations to the cytoskeleton. Overall, the results suggest that Lyngbya aerugineo-coerulea is an important cyanobacterium to be considered for its potential to cause health risks on environmental exposure of it to mammals and fish. Applying a combination of mammalian and piscine cell line bioassays is a unique approach that, combined with chemical analysis, could be used in the future to identify the structure and cellular mechanisms of the as-yet-unknown toxic Lyngbya aerugineo-coerulea metabolites in particular and to screen cyanobacterial extracts for their toxicity in general.     

Successful emergency pancreatoduodenectomy in a non-trauma patient

In the past decades considerable improvements in diagnostic imaging procedures, surgical technique and perioperative intensive care treatment have lead to a significant decrease in perioperative mortality and morbidity after pancreatic resection. This lead to an expansion of the indications for pancreatoduodenectomy. Little data exist in the literature on emergency pancreatoduodenectomy in non-trauma patients. We present a 43-year-old woman with extensive bleeding from papilla of Vater after endoscopic retrograde cholangiopancreatography (ERCP) with papillosphincterotomy. During the surgical exploration a tumor mass on the head of the pancreas was observed and a pancreatoduodenectomy was performed. On day 7 after the operation pancreatic leakage was observed and conservative therapy was administrated. Two weeks later the patient was released from the hospital in good health. Emergency pancreatoduodenectomy may be considered in institutions with extensive experience in these procedures, where cooperation of the invasive gastroenterologists and the abdominal surgeons, long experience of the surgeon and intensive reanimation care are present.     

Calcium-sensing receptor attenuates AVP-induced aquaporin-2 expression via a calmodulin-dependent mechanism

Recent evidence suggests that arginine vasopressin (AVP)-dependent aquaporin-2 expression is modulated by the extracellular calcium-sensing receptor (CaSR) in principal cells of the collecting duct, but the signaling pathways mediating this effect are unknown. Using a mouse cortical collecting duct cell line (mpkCCD(cl4)), we found that increasing the concentration of apical extracellular calcium or treating with the CaSR agonists neomycin or Gd(3+) attenuated AVP-dependent accumulation of aquaporin-2 mRNA and protein; CaSR gene-silencing prevented this effect. Calcium reduced the AVP-induced accumulation of cAMP, but this did not occur by increased degradation of cAMP by phosphodiesterases or by direct inhibition of adenylate cyclase. Notably, the effect of extracellular calcium on AVP-dependent aquaporin-2 expression was prevented by inhibition of calmodulin. In summary, our results show that high concentrations of extracellular calcium attenuate AVP-induced aquaporin-2 expression by activating the CaSR and reducing coupling efficiency between V(2) receptor and adenylate cyclase via a calmodulin-dependent mechanism in cultured cortical collecting duct cells.     

Angiosarcoma of the heart

Two cases of angiosarcomas of the heart are described. In one the tumour, which arose from the right atrium, was demonstrated during life by angiography. In the other, diagnosed only at necropsy, the tumour arose from the right ventricle. Both cases illustrate many of the typical features of this rare tumour and the difficulties of antemortem diagnosis.     

Pathogenic effect of osteopetrosis virus strain MAV-2(0) on guinea fowl pancreas

Osteopetrosis virus strain MAV-2(0) in guinea fowl pancreas induces early unspecific changes in separate acini and the development of neoplasia among apparently unaffected acinar tissue. The tumors were classified as serous and mucinous adenomas and adenocarcinomas, as well as poorly differentiated carcinomas. Foci of phenotypically altered acinar cells were observed in single experimental birds.