The peroxisome biogenesis disorders (PBDs) are currently difficult-to-treat multiple-organ dysfunction disorders that result from the defective biogenesis of peroxisomes. Genes encoding Peroxins, which are required for peroxisome biogenesis or functions, are known causative genes of PBDs. The human peroxin genes PEX3 or PEX16 are required for peroxisomal membrane protein targeting, and their mutations cause Zellweger syndrome, a class of PBDs. Lack of understanding about the pathogenesis of Zellweger syndrome has hindered the development of effective treatments. Here, we developed potential Drosophila models for Zellweger syndrome, in which the Drosophila pex3 or pex16 gene was disrupted. As found in Zellweger syndrome patients, peroxisomes were not observed in the homozygous Drosophila pex3 mutant, which was larval lethal. However, the pex16 homozygote lacking its maternal contribution was viable and still maintained a small number of peroxisome-like granules, even though PEX16 is essential for the biosynthesis of peroxisomes in humans. These results suggest that the requirements for pex3 and pex16 in peroxisome biosynthesis in Drosophila are different, and the role of PEX16 orthologs may have diverged between mammals and Drosophila. The phenotypes of our Zellweger syndrome model flies, such as larval lethality in pex3, and reduced size, shortened longevity, locomotion defects, and abnormal lipid metabolisms in pex16, were reminiscent of symptoms of this disorder, although the Drosophila pex16 mutant does not recapitulate the infant death of Zellweger syndrome. Furthermore, pex16 mutants showed male-specific sterility that resulted from the arrest of spermatocyte maturation. pex16 expressed in somatic cyst cells but not germline cells had an essential role in the maturation of male germline cells, suggesting that peroxisome-dependent signals in somatic cyst cells could contribute to the progression of male germ-cell maturation. These potential Drosophila models for Zellweger syndrome should contribute to our understanding of its pathology.
eCrCl-AKI can provide relatively accurate estimates for fluctuating CrCl. eCrCl-AKI enables more stable and earlier classification of AKI than Cr, at least in the simulation study. The more widespread use of eCrCl-AKI in actual clinical settings of AKI is necessary to evaluate this formula.
AdjLpst can detect patients of the EX group more accurately than other tests. Because AdjLpst is related to plasma refilling, it may indicate removable fluid overload. AdjLpst in conjunction with BIS may contribute to more adequate fluid management.
Key words:subcutaneously fixed artery, vascular access, repeated cannulation, brachial vein, ultrasound-guided cannulation 〈Abstract〉Generally, hemodialysis patients with a subcutaneously fixed artery proceed to arteriovenous graft implantation when their superficial veins are exhausted. Inserting a double lumen hemodialysis catheter, however, could be disadvantage in terms of infection control once the graft has failed due to infection. Under real-time ultrasound guidance, we repeatedly cannulated the brachial vein in two patients whose graft failed due to infection and succeeded in avoiding the insertion of a double lumen catheter until the infection was controlled. Tips for ultrasound-guided cannulation are as follows:1)Locations of the brachial vein, artery and nerve were fully observed in advance. 2)After the brachial vein was visualized on short axis view, the ultrasound probe was slanted and set perpendicular to the hemodialysis needle, which improved visualization of the needle echo,
We report on three patients with postrenal acute kidney injury (AKI) showing a remarkably low level of cystatin C (CysC) compared with that of creatinine (Cr). The levels of Cr and CysC (Cr/CysC) were respectively as follows: 12.16 mg/dl/1.26 mg/l, 17.92 mg/dl/0.95 mg/l and 18.94 mg/dl/0.55 mg/l. The causes of urinary tract obstruction were benign prostatic hypertrophy, urinary bladder carcinoma and urethral stenosis due to radiation therapy for bladder carcinoma. Renal function was promptly recovered after relief of the obstruction. It is considered that the discrepancy strongly indicated AKI because of urinary tract obstruction and encouraged relief of the obstruction in order to recover renal function. Although the precise mechanism for the discrepancy was not determined, the maintenance of glomerular filtration and proximal tubular reabsorption of CysC long after the cessation of Cr excretion because of urinary tract obstruction seemed to be involved. This finding may be beneficial for the diagnosis and reversal of postrenal AKI and provides new insight into the process of postrenal AKI.
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