Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0020500 (hyperoxaluria)
 912 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

One hundred and sixty three children who received total parenteral nutrition (TPN), including 7 cases of short bowel syndrome, were studied to evaluate the role of TPN in the management of infants with extremely short bowel. Three of the seven were died of sepsis related with central venous catheter (CV catheter) during the period of malabsorption when TPN was necessary. Two children of other diseases were died of catheter sepsis, 5 out of 163 in total, making the mortality late of TPN 3%. Incidence of CV catheter related complications was significantly less frequent in Broviac catheter when compared with conventional Silastic catheter (p less than 0.01). Another significant complication of TPN in cases of short bowel syndrome was hepatic dysfunction. Cholestatic liver dysfunction seemed to be cleared when enteral feeding was started even with TPN going on. Oral feeding should be started in the early postoperative period with concomitant TPN covering the fluid loss. A case of copper deficiency with high output jejunostomy and a case of urolithiasis with hyperoxaluria complicated with short bowel were reported.
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PMID:[Long-term TPN for short bowel syndrome]. 314 68

Primary hyperoxaluria type 1 (PH 1) is complicated by a high rate of early end-stage renal failure (ESRF). In ESRF combined liver kidney transplantation has emerged as treatment of choice for teenagers and adults. In chronic renal failure (CRF) and for small children the situation is less clear. We report on three isolated liver transplantations and show the data of young children from the European Registry for liver transplantation in PH 1. Patient #1 developed ESRF at 3 months of age. Deficiency of alanine:glyoxylate aminotransferase proved PH 1. Progressive bone disease developed and the boy received a living related liver graft (LRLTx) at age two. Due to recurrent cholangitis kidney transplantation (KTx) is currently not feasible. Plasma oxalate decreased after LRLTx indicating correction of the metabolic defect. Patient #2 was diagnosed at the age of 14 months. He had nephrocalcinosis and hyperglycolic hyperoxaluria. Two years later he developed ESRF. At 5 years of age isolated liver transplantation was performed as a first step of therapy. Due to prolonged warm ischemia time organ function was poor. A severe bleeding complicated the course. The child died four weeks after transplantation from untreatable CMV septicemia. Patient #3 was evaluated for failure to thrive at 6 months of age. Urinary oxalate/creatinine ratio was 705 mumol/mol and gave rise to the diagnosis of PH 1. Renal failure slowly progressed to a creatinine clearance of 20 ml/min/1.73 m2 at 8 years, when liver transplantation (LTx) was performed. Four months later, GFR has not changed. Liver function and urinary oxalate/creatinine ratio are normal. Slowly deteriorating chronic renal failure can be stabilized through isolated liver transplantation and thus the rapid need for KTx will at least be delayed. Even more important, normalization of the oxalate metabolism prevents extrarenal oxalate deposits during renal failure.
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PMID:Transplantation procedures in primary hyperoxaluria type 1. 883 45

With advancements in the operative techniques, patient survival following liver transplantation (LTx) has increased substantially. This has led to the acceleration of pre-existing kidney disease because of immunosuppressive nephrotoxicity making additional kidney transplantation (KTx) inevitable. On the other hand, in a growing number of patients on the waiting list to receive liver, long waiting time has resulted in adverse effect of decompensated liver on the kidney function. During the last two decades, the transplant community has considered combined liver kidney transplantation (CLKTx) to overcome this problem. The aim of our study is to present an overview of our experience as well as a review of the literature in CLKTx and to discuss the controversy in this regard. All performed CLKTx (n = 22) at our institution as well as all available reported case series focusing on CLKTx are extracted. The references of the manuscripts were cross-checked to implement further articles into the review. The analyzed parameters include demographic data, indication for LTx and KTx, duration on the waiting list, Model for End-Stage Liver Disease (MELD) score, Child-Turcotte-Pugh (CTP) score, immunosuppressive regimen, post-transplant complications, graft and patient survival, and cause of death. From 1988 to 2009, a total of 22 CLKTx were performed at our institution. The median age of the patients at the time of CLKTx was 44.8 (range: 4.5-58.3 yr). The indications for LTx were liver cirrhosis, hyperoxaluria type 1, polycystic liver disease, primary or secondary sclerosing cholangitis, malignant hepatic epithelioid hemangioendothelioma, cystinosis, and congenital biliary fibrosis. The KTx indications were end-stage renal disease of various causes, hyperoxaluria type 1, polycystic kidney disease, and cystinosis. The mean follow-up duration for CLKTx patients were 4.6 +/- 3.5 yr (range: 0.5-12 yr). Overall, the most important encountered complications were sepsis (n = 8), liver failure leading to retransplantation (n = 4), liver rejection (n = 3), and kidney rejection (n = 1). The overall patient survival rate was 80%. Review of the literature showed that from 1984 to 2008, 3536 CLKTx cases were reported. The main indications for CLKTx were oxalosis of both organs, liver cirrhosis and chronic renal failure, polycystic liver and kidney disease, and liver cirrhosis along with hepatorenal syndrome (HRS). The most common encountered complications following CLKTx were infection, bleeding, biliary complications, retransplantation of the liver, acute hepatic artery thrombosis, and retransplantation of the kidney. From the available data regarding the need for post-operative dialysis (n = 673), a total of 175 recipients (26%) required hemodialysis. During the follow-up period, 154 episodes of liver rejection (4.3%) and 113 episodes of kidney rejection (3.2%) occurred. The cumulative 1, 2, 3, and 5 yr survival of both organs were 78.2%, 74.4%, 62.4%, and 60.9%, respectively. Additionally, the cumulative 1, 2, 3, and 5 yr patient survival were 84.9%, 52.8%, 45.4%, and 42.6%, respectively. The total number of reported deaths was 181 of 2808 cases (6.4%), from them the cause of death in 99 (55%) cases was sepsis. It can be concluded that there is still no definitive evidence of better graft and patient survival in CLKTx recipients when compared with LTx alone because of the complexity of the exact definition of irreversible kidney function in LTx candidates. Additionally, CLKTx is better to be performed earlier than isolated LTx and KTx leading to the avoidance of deterioration of clinical status, high rate of graft loss, and mortality. Shorter graft ischemia time and more effective immunosuppressive regimens can reduce the incidence of graft malfunctioning in CLKTx patients. Providing a model to reliably determine the need for CLKTx seems necessary. Such a model can be shaped based upon new and precise markers of renal function, and modification of MELD system.
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PMID:A single center experience of combined liver kidney transplantation. 1993 Mar 23

Our objective is to study the outcomes and complications of peritoneal dialysis (PD) including comparison of self-care PD with home-care assisted PD during a five-year period. A retrospective study of PD data at King Saud University-affiliated hospital in Riyadh from January 1, 2009, to December 31, 2013. One hundred and eleven patients were included (female 55%). The average age was 47.4 (1-83) years. Twenty-one (18.91%) patients were on continuous ambulatory PD and 90 (81.08%) on automated PD. The mean time on PD was 23.5 (3-60) months. At the end of five years, 47 (42.34%) patients were continuing on PD, 12 (10.81%) had renal transplant, 33 (29.73%) patients were transferred to hemodialysis, and two (1.8%) patients were transferred to other centers. Seventeen patients died during this period giving a mortality rate of 7.13 deaths/100 patient-year during the five-year period. Six patients died due to cardiovascular causes, while five had sepsis. There was one death each due to prostate cancer, hyperoxaluria, and toxic epidermal necrolysis. Three patients died suddenly at home. Peritonitis rate was one episode/35.28 patient/month or one episode/2.94 patient/year. We compared the results for patients doing the dialysis themselves [56 (50.45%)] "self-care PD" to 55 (49.5%) patients assisted by a family member or other caregivers "assisted PD." We found no significant difference in the incidence of complications, technical outcome, mortality, and peritonitis episodes. However, we found a high prevalence of diabetes mellitus and significant increase in exit site infection in assisted PD. Our study suggests that PD patients in Saudi Arabia have a good overall outcome. Furthermore, assisted PD showed good patient and technique outcome.
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PMID:Appraising the outcome and complications of peritoneal dialysis patients in self-care peritoneal dialysis and assisted peritoneal dialysis: A 5-year review of a single Saudi center. 2945 10