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21.
Background Hereditary pancreatitis is an important cause of chronic pancreatitis, which may result in endocrine and exocrine failure.
This may necessitate simultaneous pancreas and kidney transplant (SPK). Bladder drainage of the exocrine secretions may cause
problems.
Aim To report one such case and its surgical correction.
Methods A 20-year-old male with insulin-dependent diabetes mellitus secondary to idiopathic chronic pancreatitis had a SPK with bladder
drainage. Urological and metabolic complications secondary to the drainage of pancreatic secretions, rich in proteolytic enzymes
required convertion from bladder to enteric drainage.
Results He was able to discontinue his pancreatic enzyme supplements, ceased to have steatorrhoea and gained weight. He was referred
to the €pean Registry of Hereditary Pancreatitis and Familial Pancreatic Cancer (€PAC), hereditary pancreatitis was confirmed
by genetic analysis.
Conclusion Enteric-drained pancreas transplantation is a successful treatment for exocrine as well as endocrine pancreatic failure and
should be considered as a treatment option in patients with chronic pancreatitis. 相似文献
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Human platelet membrane glycoproteins IIb and IIIa (GPIIb and IIIa) were incorporated into phospholipid vesicles by the reverse-phase technique to assess the ability of GPIIb and IIIa to function as a Ca2+ channel. Movement of Ca2+ across the lipid bilayer was quantitated by injection of proteoliposomes with encapsulated Fura-2 into Ca2+ buffers and measurement of Fura-2 fluorescence as an indicator of Ca2+ influx. Reciprocally, to assess the function of proteins in an inside-out orientation, Ca2+-loaded vesicles were injected into Ca2+-free buffer and Ca2+ efflux monitored by a calcium electrode. Incorporation of the IIb-IIIa complex produced significant facilitation of Ca2+ movement across the lipid bilayer. No net transmembrane Ca2+ movement was seen with dissociated IIb and IIIa. Movement of Ca2+ was proportional to the transmembrane Ca2+ gradient. Ca2+ movement into the vesicles was inversely proportional to extravesicular NaCl from 25 to 150 mmol/L, analogous to several studies in the intact platelet. Adenosine triphosphate had no effect on Ca2+ movement into or out of the vesicles. Specific inhibition of a Ca2+ shift into the vesicles was seen with M148, a monoclonal antibody to IIb/IIIa, while no inhibition was observed with a panel of other anti-IIb/IIIa monoclonal antibodies. This suggests that a specific site on the complex or orientation of the complex is essential for calcium channel function. These data demonstrate that the GPIIb/IIIa complex can serve as a passive Ca2+ channel across a phospholipid bilayer and has the potential to play a role in Ca2+ flux across the platelet plasma membrane. 相似文献
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G. Yoon B. Baskin M. Tarnopolsky K. M. Boycott M. T. Geraghty E. Sell S. Goobie W. Meschino B. Banwell P. N. Ray 《Neurogenetics》2013,14(3-4):181-188
We describe the clinical and genetic features of a well-characterized cohort of patients with autosomal recessive hereditary spastic paraplegia (ARHSP) in the province of Ontario. Patients with documented corticospinal tract abnormalities were screened by whole gene sequencing and multiplex ligation probe amplification for mutations in nine genes known to cause ARHSP. Of a cohort of 39 patients, a genetic diagnosis was established in 17 (44 %) and heterozygous mutations were detected in 8 (21 %). Mutations were most frequent in SPG7 (12 patients), followed by SPG11 (10 patients), PNPLA6 (SPG39, 2 patients), and ZFYVE26 (SPG15, 2 patients). Although there are associations between some clinical manifestations of ARHSP and specific genes, many patients are tested at an early stage of the disease when phenotype/genotype correlations are not obvious. Accurate molecular characterization of well-phenotyped cohorts of patients will be essential to establishing the natural history of these rare degenerative disorders to enable future clinical trials. 相似文献
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Peter A Lazzarini Julia Geraghty Ewan M Kinnear Mark Butterworth Donna Ward 《Journal of foot and ankle research》2013,6(1):1-11
Background
Research is a major driver of health care improvement and evidence-based practice is becoming the foundation of health care delivery. For health professions to develop within emerging models of health care delivery, it would seem imperative to develop and monitor the research capacity and evidence-based literacy of the health care workforce. This observational paper aims to report the research capacity levels of statewide populations of public-sector podiatrists at two different time points twelve-months apart.Methods
The Research Capacity & Culture (RCC) survey was electronically distributed to all Queensland Health (Australia) employed podiatrists in January 2011 (n?=?58) and January 2012 (n?=?60). The RCC is a validated tool designed to measure indicators of research skill in health professionals. Participants rate skill levels against each individual, team and organisation statement on a 10-point scale (one?=?lowest, ten?=?highest). Chi-squared and Mann Whitney U tests were used to determine any differences between the results of the two survey samples. A minimum significance of p?<?0.05 was used throughout.Results
Thirty-seven (64%) podiatrists responded to the 2011 survey and 33 (55%) the 2012 survey. The 2011 survey respondents reported low skill levels (Median?<?4) on most aspects of individual research aspects, except for their ability to locate and critically review research literature (Median?>?6). Whereas, most reported their organisation’s skills to perform and support research at much higher levels (Median?>?6). The 2012 survey respondents reported significantly higher skill ratings compared to the 2011 survey in individuals’ ability to secure research funding, submit ethics applications, and provide research advice, plus, in their organisation’s skills to support, fund, monitor, mentor and engage universities to partner their research (p?<?0.05).Conclusions
This study appears to report the research capacity levels of the largest populations of podiatrists published. The 2011 survey findings indicate podiatrists have similarly low research capacity skill levels to those reported in the allied health literature. The 2012 survey, compared to the 2011 survey, suggests podiatrists perceived higher skills and support to initiate research in 2012. This improvement coincided with the implementation of research capacity building strategies. 相似文献29.
Richard James Dallalana Ryan A. McMahon Ben East Liam Geraghty 《International journal of shoulder surgery》2016,10(2):59-66
Purpose:Glenoid component malposition is associated with poor function and early failure of both anatomic and reverse total shoulder arthroplasty. Glenoid positioning is challenging particularly in the setting of bone loss or deformity. Recently, the use of computer assistance has been shown to reduce implantation error. The aim of this study is to evaluate the accuracy of patient-specific instrumentation in cases of anatomic and reverse shoulder replacement in vivo.Methods:Twenty patients underwent total shoulder arthroplasty using a computed tomography (CT)-based patient-specific instrumentation (PSI) system, ten anatomic and ten reverse. Preoperative three-dimensional digital templating of glenoid component position was undertaken and surgery then performed using a custom-made guide. Postoperative CT scans were used to compare final implanted component position to the preoperatively planned position in the same patient.Results:Final component position and orientation closely reflected the preoperatively templated position. Mean deviation in the glenoid version from planned was 1.8° ±1.9° (range, 0.1°–7.3°). Mean deviation in inclination was 1.3° ±1.0° (range, 0.2°–4.5°). Mean deviation in position on the glenoid face was 0.5 ± 0.3 mm (range, 0.0–1.3 mm) in the anteroposterior plane and 0.8 ± 0.5 mm (range, 0.0–1.9 mm) in the superoinferior plane. Actual achieved version was within 7° of neutral in all cases except for one where it was deliberately planned to be outside of this range.Conclusion:PSI in both anatomic and reverse shoulder arthroplasty is highly accurate in guiding glenoid component implantation in vivo. The system can reliably correct bony deformity. 相似文献
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