Glycogen storage disease type Ia (GSDIa) is due to the deficiency of glucose-6-phosphatase activity in the liver, kidney, and intestine. Although significant progress has been achieved in the ...management of patients with GSDIa, complications still emerge. The potential for development of liver adenomatosis and kidney failure makes these patients candidates for simultaneous liver-kidney transplantation (SLKT). Herein, we describe such a transplantation in a patient affected by this rare storage disease.
A 25-year-old female patient with GSDIa developed hepatic adenoma and kidney failure despite dietary therapy. The patient underwent an SLKT from a cadaveric donor.
The operative time was 8 hours without hemotransfusion. Only a transitory lactic acidosis was observed. Laboratory results normalized on postoperative day 7. The patient was discharged on postoperative day 9. After 4 months, the patient is in good condition with well-functioning kidney and liver allografts.
Patients with end-stage renal disease secondary to GSDIa should be considered for SLKT, especially when the disease is in an early stage.
Low dose of dopamine is commonly used after kidney transplantation as a reno-protective agent, although its benefits are controversial. Dopamine may increase renal blood flow, decrease resistive ...index (RI), and induce urine output in normal kidneys. Many authors hypothesized that the vasculature of a denervated renal transplant may not respond to dopamine in the same fashion as healthy native kidneys, which led us to find other drugs to attenuate the ischemia-reperfusion (I/R) injury. Fenoldopam is a selective dopamine1 (DA1) receptor agonist, most of the activity of which resides in the R-enantiomer, which also shows weaker alpha 2-adrenoceptor antagonist activities. Fenoldopam produces a vasidilatory effect in vascular beds that are rich in vascular DA1 receptors, producing increased renal blood flow at doses that do not affect blood pressure. In addition to its renal vasodilator activity, fenoldopam is natriuretic, possibly resulting from a direct effect of DA1 receptors on the proximal convoluted tubule. In animals with spontaneous or drug-induced renal failure, fenoldopam improves renal function. The aim of this study was to investigate the possible effects of fenoldopan mesylate in recent kidney transplants. Creatinine, blood urea nitrogen, urine output, and renal vascular resistive index (IR) were measured using Doppler ultrasound. Two groups of patients with no statistical differences in demographic data were treated with dopamine or fenoldopan, showing no significant difference but a trend favoring the fenoldopan group.
Recently observations of rhabdomyolysis in patients treated with tacrolimus have been reported. The authors present a kidney transplant patient who had an epileptic seizures, severe rhabdomyolysis, ...and acute renal failure. The patient was initially immunosuppressed with tacrolimus and chimeric CD25 monoclonal antibody. After intensive therapy with plasmapheresis, CVVH, and dialysis, the patient completely recovered at 11/2 year his serum creatinine is 1.2 mg/dL.
Delayed graft function and acute renal failure after kidney transplant negatively influence graft outcome. It has been reported that pretransplantation peritoneal dialysis (PD) instead of ...hemodialysis (HD) correlated with better short-term graft outcome in adult kidney recipients. In this study the impact of PD versus HD was evaluated among pediatric kidney recipients. This study suggested that different forms of dialysis pretransplantation did not affect early graft function among pediatric kidney recipients.
Summary
Delayed graft function (DGF) is a frequent complication of kidney transplantation (KT) that may affect both short‐ and long‐term graft outcome. It has been reported that pretransplantation ...peritoneal dialysis was correlated with a better recovery of graft function than hemodialysis in adult kidney recipients. However, the effect of pretransplantation dialysis mode (PDM) seemed to be unclear on the early outcome of KT in pediatric recipients. In this study, the potential impact of PDM on early graft function was evaluated in 174 pediatric patients who underwent KT by using cadaveric donors. The primary outcome parameter was the time to reach a serum creatinine (SCr) level 50% of the pretransplantation value T1/2(SCr), while DGF was defined as a T1/2(SCr) >3 days after KT (n = 40). By stratifying kidney recipients for normal function graft or DGF, this latter group showed a significantly higher body weight (BW) on the day of KT (P = 0.014), body surface area (BSA) (P = 0.005), warm ischemia time (WIT) (P = 0.022), early SCr on the day 1 after KT (P < 0.001), and T1/2(SCr) (P < 0.001), whereas lower urine volume (UV) collected in the first 24 h after KT (P < 0.001) and fluid load (P < 0.001) occurred. Univariate exponential correlation that was carried out between T1/2(SCr) and all the other variables had shown a better value than the linear correlation for BW (R2 = 0.28 vs. R2 = 0.04), BSA (R2 = 0.29 vs. R2 = 0.03), and SCr (R2 = 0.51 vs. R2 = 0.28). In a multivariate regression analysis performed by entering T1/2(SCr) as dependent variable and following a forward stepwise method, cold ischemia time (CIT) (P = 0.027) but not PDM (P = 0.195) reached significance. In a Cox regression analysis carried out with T1/2(SCr) as dependent variable, neither CIT nor PDM gained significance. This study suggests that PDM does not affect early graft function in pediatric kidney recipients.
Delayed graft function and acute renal failure after kidney transplant negatively influence graft outcome. It has been reported that pretransplantation peritoneal dialysis (PD) instead of ...hemodialysis (HD) correlated with better short-term graft outcome in adult kidney recipients. In this study the impact of PD versus HD was evaluated among pediatric kidney recipients. This study suggested that different forms of dialysis pretransplantation did not affect early graft function among pediatric kidney recipients.
Patients undergoing lower extremity amputation are perceived to be at high risk for deep vein thrombosis (DVT). DVT can cause micro or macro pulmonary embolism and often the post-thrombophlebitic ...syndrome. The chronic condition can affect patient quality of life and his residual working capacity. Usually the echo-Doppler or the color-Doppler is used as a prevention and diagnostic method, identifying patients at high risk. Following the Authors examine and report the Literature opinion about the topics.
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