Renal allograft failure related to a lower extremity vascular access--a case report.
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Adult↗
Biomedical subjects
L W Ernest van Heurn
Publications and source records attributed to L W Ernest van Heurn.
Redox-active iron released during machine perfusion predicts viability of ischemically injured deceased donor kidneys.
Redox-active iron, catalyzing the generation of reactive oxygen species, has been implicated in experimental renal ischemia-reperfusion injury. However, in clinical transplantation, it is unknown whether redox-active iron is involved in the pathophysiology of ischemic injury of non-heart-beating (NHB) donor kidneys. We measured redox-active iron concentrations in perfusate samples of 231 deceased donor kidneys that were preserved by machine pulsatile perfusion at our institution between May 1998 and November 2002 using the bleomycin detectable iron assay. During machine pulsatile perfusion, redox-active iron was released into the preservation solution. Ischemically injured NHB donor kidneys had significantly higher perfusate redox-active iron concentrations than heart-beating (HB) donor kidneys that were not subjected to warm ischemia (3.9 +/- 1.1 vs. 2.8 +/- 1.0 micromol/L, p = 0.001). Moreover, redox-active iron concentration was an independent predictor of post-transplant graft viability (odds ratio 1.68, p = 0.01) and added predictive value to currently available donor and graft characteristics. This was particularly evident in uncontrolled NHB donor kidneys for which there is the greatest uncertainty about transplant outcomes. Therefore, perfusate redox-active iron concentration shows promise as a novel viability marker of NHB donor kidneys.
Adenosine↗
Hirschsprung's disease: healthcare meets the needs.
OBJECTIVE: The objective of this study was to examine the types of healthcare services used by children, adolescents, and adults with Hirschsprung Disease (HD) in relation to the severity of the initial defect (mild, severe), whether additional care was needed, the provision of information, transfer to adult care, and satisfaction with the care provided. METHODS: Three hundred twenty (71%) HD patients, ages 6 to 54, completed a questionnaire that assessed the use of healthcare services, the need for more healthcare, the provision of information, the transfer to adult care and satisfaction with the provided care. RESULTS: In 6 months, 45% of the children, 14% of the adolescents, and 15% of the adults consulted a medical specialist. Compared with patients with a mild form of HD in the age range of 6 to 16 years, only the more severely afflicted adult patients visited medical professionals more often (10% vs 29%) (P < .05). Of the children, the adolescents, and the adults 23%, 8%, and 6% respectively consulted a nonmedical professional. Less than 15% of all patients whould have liked more treatment. In 6 months 51% of the children, 24% of the adolescents, and 21% of the adults received treatment information, of which respectively 14, 8, and 20% wished they had received more information. Three (12%) patients who needed adult care encountered problems with the transfer. Almost all patients were satisfied with the care provided. CONCLUSIONS: There is good access to medical healthcare services, especially for children. The only lacuna in the healthcare system we revealed was a lack of information, particularly for adult patients. Most parents and patients reported to be very satisfied with the provided care.
Adolescent↗
The Currarino triad: the variable expression.
BACKGROUND: The Currarino triad is a relatively unknown hereditary disorder linked to the 7q36 region and characterized by an anorectal malformation, sacrococcygeal defect, and a presacral mass. PURPOSE: The aim of the study was to report the highly variable expression in patients with the Currarino triad and provide a guideline for the clinician if the Currarino triad is suspected. METHODS: We examined the symptoms and associated anomalies in 5 families with the Currarino triad by retrospective chart review. RESULTS: Fifteen patients had Currarino-associated anomalies. This included anorectal malformation in 9, a presacral mass in 9, and a sacral bone defect in 15. Tethered cord was present in 4 patients. There were 12 symptomatic patients. Constipation was diagnosed in all of them. Other symptoms included recurrent urinary tract infections caused by bladder dysfunction in 5, abnormal gait in 1, and another patient who initially presented with meningitis. Two carriers of the genetic defect had no symptoms or anomalies. CONCLUSIONS: The phenotypical expression of the gene mutations causing the Currarino triad can vary from asymptomatic to patients presenting with the complete triad. Presence of a crescent-shaped defect of the sacral bone may be used as initial screening method. As the penetration of the genetic defect is variable, DNA screening is the only method to fully exclude the triad.
Abnormalities, Multiple↗
Anorectal malformations: does healthcare meet the needs?
OBJECTIVES: The first aim was to identify the types of healthcare services used by children, adolescents, and adults with anorectal malformation (ARM) in relation to the severity of their disease and to examine whether additional care was needed. The second aim was to evaluate specific areas in the healthcare system, including provided information, transfer from pediatric to adult care, and satisfaction with the provided care. METHODS: Three hundred eighty-six (61%) patients with ARM, ages 6 to 52, completed a questionnaire that assessed their use of healthcare services and the need for additional services. Also, questions were asked about specific areas in the healthcare system. Clinical and sociodemographic characteristics were extracted from medical records. RESULTS: In the preceding 6 months 50% of the children, 24% of the adolescents, and 24% of the adults consulted a medical specialist. Compared with patients with a mild form of ARM in the age range of 6 to 16 years, the more severely afflicted patients visited medical professionals more often (18% vs. 32%). Particularly, adolescents in the age range of 12 to 16 years with a severe form of the disease more often visited the pediatric surgeon than their peers with a mild form (2% vs. 16%). Twenty-three percent of the children, 7% of the adolescents, and 8% of the adults consulted a nonmedical professional. Twenty percent of the children, 13% of the adolescents, and 17% of the adults would have liked additional or more treatment of a nonmedical professional. In 6 months, 40% of the children, 24% of the adolescents, and 20% of the adults received treatment information. One third of the adult patients who were transferred to "adult" surgeons encountered transfer problems. Almost all patients were satisfied with the care provided. CONCLUSIONS: There is good access to medical healthcare services, especially for children. However, more psychosocial and paramedical care is considered necessary. As could be expected, children and adolescents with a severe form of the disease reported to have visited a medical specialist more often. Although healthcare for patients with ARM may be improved at certain points, most parents and patients were very satisfied with the care provided.
Adolescent↗
Apoptotic cell death is initiated during normothermic ischemia in human kidneys.
Ischemic damage plays an important role in post-transplant organ failure. Activation of the apoptotic cascade is crucially involved in post-ischemic inflammation resulting in tissue damage and organ dysfunction. Here we investigate the initiation of the apoptotic cascade during normothermic ischemia in human kidneys using a model for normothermic ischemia with kidneys nephrectomized because of renal cell carcinoma. Ex vivo, kidneys were stored at 37 degrees C, and consecutive biopsies were taken from disease-free tissue. Pro- and anti-apoptotic proteins were assessed by Western blotting and immunofluorescence. During normothermic ischemia the pro-apoptotic proteins Bax and activated caspase-9 increased with ischemia time, whereas caspase-8 was not activated. The anti-apoptotic proteins Bcl-2 and cFLIP decreased in time. Data on Bcl-2 and Bax were supported by immunofluorescence for Bcl-2 and activated Bax. However, activation of the central effector caspase-3, essential for execution of the apoptotic process, was not detected. In conclusion, during normothermic ischemia the apoptotic cascade in the human kidney is initiated, but not fulfilled. Our data show that the duration of ischemia significantly correlates with activation of the apoptotic cascade. These findings provide insight in the initiation of apoptotic cell-death during warm ischemia and may be useful in the assessment of ischemic injury.
Apoptosis↗
Outcome of nonheart-beating donor kidneys with prolonged delayed graft function after transplantation.
Nonheart-beating donor (NHBD) kidneys are frequently associated with delayed graft function (DGF), with a deleterious effect on kidney function and allograft survival. The influence and the duration of DGF on the outcome of NHBD kidneys are assessed. All recipients of an NHBD kidney in the period 1993-2003 were reviewed. Excluded from analysis were patients with primary nonfunction (PNF). One hundred and five patients with a functioning NHBD graft were reviewed: 23 (22%) had immediate function (group 1), 40 (38%) had DGF < or = 2 weeks (group 2), 31 (30%) had DGF 15 days to 4 weeks (group 3) and 11 (10%) had DGF for > 4 weeks (group 4). Creatinine clearance at 3 months was higher in groups 1 and 2 versus group 4 (p = 0.015 and p = 0.006, respectively) and was higher in group 2 versus group 4, at 1 year (p = 0.01). Graft survival was 95%, 98%, 97% and 89%, respectively, at 1 year and 95%, 85%, 77% and 89%, respectively, at 5 years, which was not significantly different. The duration of DGF in NHB kidneys has a negative effect on creatinine clearance, but no effect on graft survival.
Adult↗
Reduction of circulating redox-active iron by apotransferrin protects against renal ischemia-reperfusion injury.
BACKGROUND: Warm ischemia-reperfusion (I/R) injury plays an important role in posttransplant organ failure. In particular, organs from marginal donors suffer I/R injury. Although iron has been implicated in the pathophysiology of renal I/R injury, the mechanism of iron-mediated injury remains to be established. The authors therefore investigated the role of circulating redox-active iron in an experimental model for renal I/R injury. METHODS: Male Swiss mice were subjected to unilateral renal ischemia for 45 min, followed by contralateral nephrectomy and reperfusion. To investigate the role of circulating iron, mice were treated with apotransferrin, an endogenous iron-binding protein, or iron-saturated apotransferrin (holotransferrin). RESULTS: Renal ischemia induced a significant increase in circulating redox-active iron levels during reperfusion. Apotransferrin, in contrast to holotransferrin, reduced the amount of circulating redox-active iron and abrogated renal superoxide formation. Apotransferrin treatment did not affect I/R-induced renal apoptosis, whereas holotransferrin aggravated apoptotic cell death. Apotransferrin, in contrast to holotransferrin, inhibited the influx of neutrophils. Both apo- and holotransferrin reduced I/R-induced complement deposition, indicating that the effects of transferrin are differentially mediated by its iron and protein moiety. Finally, apotransferrin, in contrast to holotransferrin, dose-dependently inhibited the loss of renal function induced by ischemia. CONCLUSIONS: Redox-active iron is released into the circulation in the course of renal I/R. Reducing the amount of circulating redox-active iron by treatment with apotransferrin protects against renal I/R injury, inhibiting oxidative stress, inflammation, and loss of function. Apotransferrin could be used in the treatment of acute renal failure, as seen after transplantation of ischemically damaged organs.
Animals↗
Portoenterostomy for biliary atresia: Long-term survival and prognosis after esophageal variceal bleeding.
BACKGROUND/PURPOSE: Portoenterostomy is the procedure of choice in patients with biliary atresia. The authors analyzed the long-term survival rate and complications of the procedure in children operated on at The University of Hong Kong Medical Centre between 1979 and 2000. METHODS: Seventy-seven consecutive patients were analyzed by retrospective chart review. The patients were divided into groups according to postoperative bile flow, decade of operation, and age at operation. The data were analyzed statistically. RESULTS: The overall actuarial transplant-free survival rate was 68% at 10 years after operation. For patients with poor, partial, and good postoperative bile flow, transplant-free survival rate was 0%, 22%, and 96%, respectively (P <.001). Age and decade of operation were not significant risk factors. Esophageal variceal bleeding occurred in 13 patients and was a prognostic indicator for end-stage liver failure (P =.044); the poor prognosis of patients with variceal bleeding, however, was related to poor or partial initial bile drainage. CONCLUSIONS: The postoperative serum bilirubin level is the most important predictor of long-term survival in patients with portoenterostomy for biliary atresia. Esophageal hemorrhage is not an absolute indication for urgent liver transplantation in patients with good bile drainage.
Bile↗
The mannose-binding lectin-pathway is involved in complement activation in the course of renal ischemia-reperfusion injury.
Ischemia-reperfusion (I/R) is an important cause of acute renal failure (ARF). The complement system appears to be essentially involved in I/R injury. However, via which pathway the complement system is activated and in particular whether the mannose-binding lectin (MBL)-pathway is activated is unclear. This tempted us to study the activation and regulation of the MBL-pathway in the course of experimental renal I/R injury and in clinical post-transplant ARF. Mice subjected to renal I/R displayed evident renal MBL-depositions, depending on the duration of warm ischemia, in the early reperfusion phase. Renal deposition of C3, C6 and C9 was observed in the later reperfusion phase. The deposition of MBL-A and -C completely co-localized with the late complement factor C6, showing that MBL is involved in complement activation in the course of renal I/R injury. Moreover, the degree of early MBL-deposition correlated with complement activation, neutrophil-influx, and organ-failure observed in the later reperfusion phase. In serum of mice subjected to renal I/R MBL-A, levels increased in contrast to MBL-C levels, which dropped evidently. In line, liver mRNA levels for MBL-A increased, whereas MBL-C levels decreased. Renal MBL mRNA levels rapidly dropped in the course of renal I/R. Finally, in human biopsies, MBL-depositions were observed early after transplantation of ischemically injured kidneys. In line with our experimental data, in ischemically injured grafts displaying post-transplant organ-failure extensive MBL depositions were observed in peritubular capillaries and tubular epithelial cells. In conclusion, in experimental renal I/R injury and clinical post-transplant ARF the MBL-pathway is activated, followed by activation of the complement system. These data indicate that the MBL-pathway is involved in ischemia-induced complement activation.
Animals↗
Critical factors affecting quality of life of adult patients with anorectal malformations or Hirschsprung's disease.
OBJECTIVES: The first objective was to compare the quality of life of adult patients with anorectal malformations (ARM) or Hirschsprung's disease (HD) and to compare both groups with healthy people. And the second objective was to examine the factors that affect the quality of life of patients with ARM or HD, using a theoretical model in which patients' background characteristics explain quality of life via mediating disease-specific functioning and psychosocial functioning. METHODS: Three hundred and forty-one patients completed a questionnaire, which assessed sociodemographic characteristics, disease-specific and psychosocial functioning, and quality of life. Clinical factors were extracted from the medical records. RESULTS: Patients with ARM or Hirschsprung's disease did not differ in their quality of life. Compared to healthy people, both patient groups reported more limitations in their "overall" physical quality of life, but only patients with ARM reported impaired quality of life on several specific domains (e.g., physical role-functioning, pain). The model was largely accepted. Most striking were the strong effects of the psychosocial functioning factors in contrast to weak effects of the disease-specific "constipation" and "fecal continence" factors. CONCLUSIONS: The quality of life of patients with ARM or Hirschsprung's disease was found to be comparable. Compared to healthy people, both patient groups encountered "overall" physical health problems, but only patients with ARM reported additional pain and limitations in role functioning due to physical problems. It appeared that psychosocial functioning had the most important effect on the quality of life of patients with ARM or Hirschsprung's disease, while fecal incontinence and constipation had almost no effect on their quality of life.
Adaptation, Psychological↗
Extreme short bowel syndrome in a full-term neonate--a case report.
Massive small bowel resection often leads to long-term parenteral nutrition. The authors present a term-born, 3-day-old boy with midgut volvulus in whom only 17 cm of small bowel was left after resection. This patient was weaned from parenteral nutrition after 7 months. Temporary parenteral nutrition-associated cholestasis was treated with ursodeoxycholic acid.
Anastomosis, Surgical↗
Cholangitis after hepatic portoenterostomy for biliary atresia: a multivariate analysis of risk factors.
Cholangitis occurred in 59% of 77 patients who underwent the portoenterostomy procedure for biliary atresia between 1980 and 2000. Good postoperative bile drainage was associated with a lower risk of cholangitis than partial (odds ratio, 5.72; 95% CI, 2.89-11.3) or poor (odds ratio, 3.29; 95% CI, 1.89-5.7) bile drainage. Cholangitis was not an independent risk factor for death or liver transplantation.
Biliary Atresia↗
Quality of life in patients with anorectal malformation or Hirschsprung's disease: development of a disease-specific questionnaire.
PURPOSE: Hirschsprung's disease and anorectal malformation are congenital diseases of the digestive tract with sequelae into adulthood. The quality of life of patients with these diseases is largely unknown. The aim of the study was 1) to construct a self-report disease-specific instrument to assess the quality of life in these patients and 2) to evaluate its psychometric performance. METHODS: An age-specific (6 and 7 years, 8-11 years, 12-16 years, and >17 years) questionnaire called the Hirschsprung's disease/anorectal malformation quality-of-life instrument was constructed. This questionnaire consists of 39 to 42 items, grouped into 10 to 11 scales that cover physical, emotional, and social functions as well as disease-related symptoms. Generic quality-of-life data were obtained in addition. A national sample of 715 patients aged six years and older completed the questionnaire (response rate, 61.9 percent). RESULTS: Multitrait scaling analyses confirmed the hypothesized scale structure with exception of the scales related to diet for the two youngest groups. Cronbach's alpha ranged (with exception of the diet scales) from 0.62 to 0.91 for children (8-11 years), from 0.69 to 0.82 for adolescents (12-16 years) and from 0.57 to 0.91 for adults. Selective scales were able to discriminate between subgroups of adult patients known to differ in disease and disease severity. Relevant scales of the adult version showed substantial correlations (> 0.40) with comparable scales of the SF-36. In the two youngest age groups the differences between subgroups of patients were less significant, but in the expected direction. CONCLUSIONS: With the exception of the scales related to diet, the Hirschsprung's disease/anorectal malformation quality-of-life instrument is an instrument with promising reliability and validity, to measure the disease-specific quality of life of patients with anorectal malformation or Hirschsprung's disease.
Adolescent↗
Metastasized angiosarcoma of the spleen in a 2-year-old girl.
Primary angiosarcoma of the spleen is rare and the prognosis is very poor. The authors present a 2-year-old girl with spontaneous rupture of splenic angiosarcoma. At diagnosis there were liver metastases. After splenectomy she received chemotherapy with ifosfamide, vincristine, and actinomycine D combined with a partial liver resection. She remained in complete remission over 2 years from diagnosis.
Antineoplastic Combined Chemotherapy Protocols↗