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Biomedical subjects

R Reding

Publications and source records attributed to R Reding.

At least 37 records · Page 2Linked to original sources

Cricopharyngeal achalasia: case reports and review of the literature.

Primary cricopharyngeal achalasia (a = absence, chalasia = relaxation) is a rare cause of swallowing disorders in newborns. Two cases are reported which were successfully treated by a myotomy of the cricopharyngeal muscle. A thorough history is essential in differential diagnosis as well as observation of the feeding infant. Presence of anatomical obstruction to swallowing and existence of neurological defects should be ruled out. Cineradiography with lateral views by an experienced radiologist is the best diagnostic technique. Esophageal manometry may provide information regarding other esophageal dyskinetic problems. However, these studies are difficult to perform in neonates and infants. Endoscopy may be helpful to exclude vocal cord paralysis or mechanical obstruction. Balloon dilatation has been reported as being successful in several reports; however no comparison of efficacy has been made in any series between dilatation of the upper esophagus and surgical myotomy which remains in our mind, the optimal treatment of cricopharyngeal achalasia.

Deglutition Disorders↗

Epstein-Barr virus infection in sixty pediatric liver graft recipients: diagnosis of primary infection and virologic follow-up.

BACKGROUND: Epstein-Barr virus (EBV) is an important cause of infection after pediatric liver transplantation. Earlier detection of EBV could result in shortening the delay in diagnosis and allow better management of a pediatric high risk population. OBJECTIVES: To determine the timing of EBV primary infection after graft and to compare the performances of different assays for an early detection of the virus. METHODS: Sixty pediatric liver graft recipients were followed. Kinetics of appearance of different EBV serologic parameters (anti-EBV-IgG, -IgM and -IgA, and anti-EBV nuclear antigen-IgG) and of the viral DNA in peripheral blood lymphocytes by PCR were compared. RESULTS: Thirty-six patients had a primary EBV infection. The first PCR and IgM positive result appeared after a mean delay of 56 and 61 days, respectively, and preceded the IgG response (mean delay, 143 days). Most of the studied patients (13 of 16) developed anti-EBV-IgA and only 3 developed anti-EBNA-IgG during the follow-up period. CONCLUSIONS: EBV primary infection occurred in most cases during the first 2 months after graft. The IgG response was delayed. The best performance was obtained by PCR. However, the IgM test compared well with the PCR and could be a more widely accessible measure to follow regularly.

Adolescent↗

Hemodynamic changes in patients with Alagille's syndrome during orthotopic liver transplantation.

UNLABELLED: Children with Alagille's syndrome are at increased perioperative risk during orthotopic liver transplantation due to the cardiopulmonary abnormalities and the hemodynamic changes associated with this procedure. We studied 16 children with Alagille's syndrome who underwent 21 orthotopic liver transplantations. Peripheral pulmonary stenosis was present in all subjects. Right ventricular pressures were increased in 15 cases. Caval clamping resulted in a mean decrease of 15 +/-9 mm Hg in systolic blood pressure, 5 +/- 3 mm Hg in mean pulmonary artery pressure, and 4 +/- 3 mm Hg in central venous pressure. Systolic blood pressure decreased by 16 +/- 13 mm Hg, whereas mean pulmonary artery pressure and central venous pressure increased by 3 +/- 4 mm Hg and 1 +/- 4 mm Hg, respectively, at portal vein unclamping. There was no correlation between severity of pulmonary artery stenosis and hemodynamic changes. Veno-venous bypass used in four cases resulted in smaller hemodynamic changes. Time to extubation and duration of intensive care unit stay were unrelated to severity of pulmonary artery stenosis. IMPLICATIONS: Some children with Alagille's syndrome require liver transplantation. In our study, associated pulmonary artery stenosis did not dramatically increase perioperative risk. Veno-venous bypass decreased intraoperative hemodynamic changes in these patients.

Alagille Syndrome↗

[The role of intuition in medical malpractice].

In a retrospective analysis of 502 surgical malpractice claims, the contribution of intuition was assessed. It was found that intuition, as a logical and directed intellectual approach, had an overall positive effect; this was not the case with an attitude of autistic intuition. However, judgement based on the physicians intuition alone did never result it negligence or misconduct.

General Surgery↗

Early hepatocyte, endothelial, and bile duct cell injury after pediatric liver transplantation from cadaveric or living-related donors.

BACKGROUND: When compared with cadaveric grafts (Cad), the potential advantages of pediatric orthotopic liver transplantation (OLT) from living-related (LR) donors may include better graft quality, shorter ischemic time, appropriate preparation of the recipient, and better immunologic compatibility. METHODS: The aim of this study was to analyze early hepatocyte, endothelial, and bile duct cell injury following pediatric OLT using LR (n=15) or uncomplicated Cad reduced-size (n=10) grafts. Median (range) total ischemic times were 190 min (105-261) versus 760 min (418-948) in LR and Cad groups, respectively (P<0.001). RESULTS: The post-OLT cytolytic profile, assessed daily during the first 7 days using both plasma glutamate-pyruvate transaminase and serum alpha-glutathione S-transferase, showed significantly higher levels of both parameters for the 10 uncomplicated Cad cases when compared with the 15 LR grafts (P<0.001). The evaluation of hepatic endothelial cell function during the first week after OLT, using serum hyaluronic acid levels, suggested lower endothelial injury in the LR grafts, when compared with the Cad grafts (P=0.059). Bile duct cell injury, as assessed using plasma gamma-glutamyl transferase levels, was similar in both groups, with a progressive increase at the end of the first week after OLT, which was correlated with a similar incidence of early acute rejection in both groups (80% in the LR group vs. 62% in the Cad group, NS). CONCLUSION: (1) The hepatocellular and endothelial cell damage was reduced after OLT with LR grafts, which may be related to shorter ischemic time when compared with Cad grafts; (2) the putative immunologic advantage for LR grafts was not confirmed in terms of incidence of acute rejection.

Adult↗

Delayed primary closure of the abdominal wall after cadaveric and living related donor liver graft transplantation in children: a safe and useful technique.

Due to the shortage of size-matched liver donors, relatively oversized liver grafts (even after ex situ volume reduction) are frequently used for liver transplantation in children. This was recently observed when livers from large, living related donors were procured for transplantation in very small recipients. Given that abdominal hyperpressure can compromise vascular flow in the new graft, primary closure of the abdomen was delayed by temporary Silastic prosthetic closure in selected cases. The new technique was original in that the skin was closed, avoiding fluid loss and reducing the risk of infections reported with other techniques, and in that reoperation allowed for a delayed, but primary-type, closure (fascia and skin) that resulted in an esthetically correct aspect. Over a period of 7 years, 330 pediatric liver transplantations were performed, and delayed prosthetic closure was achieved successfully and safely in 47 cases. The present report outlines this clinical experience.

Abdominal Muscles↗

Pediatric liver transplantation: from the full-size liver graft to reduced, split, and living related liver transplantation.

Between 1984 and 1996, the authors performed 499 liver transplants in 416 children less than 15 years old. The overall patient survival at 10 years was 76.5%. It was 71.3% for the 209 children grafted in 1984-1990; 78.5% for biliary atresia (n = 286), 87.3% for metabolic diseases (n = 59), and 72.7% for acute liver failure (n = 22). The 5-year survival was 73.6% for the 209 children grafted in 1984-1990 and 85% for the 206 grafted in 1991-1996. Scarcity of size-matched donors led to the development of innovative techniques: 174 children who electively received a reduced liver as a first graft in our center had a 5-year survival of 76% while 168 who received a full-size graft had a survival of 85% (NS). Results of the European Split Liver Registry showed 6-month graft survival similar to results obtained with full-size grafts collected by the European Liver Transplant Registry. Extensive use of these techniques allowed the mortality while waiting to be reduced from 16.5% in 1984-1990 to 10% in 1991-1992. It rose again to 17% in 1993, leading the authors to develop a program of living related liver transplantation (LRLT). The legal and ethical aspects are analyzed. Between July 1993 and October 1997, the authors performed 53 LRLTs with 90% survival. In elective cases, a detailed analysis was made of the 45 children listed for LRLT between July 1993 and March 1997 and the 79 registered on the cadaveric waiting list during the same period. Mortality while waiting was 2% and 14.5% for the LRLT and cadaveric lists, respectively. The retransplantation rate was 4.6% and 16.1% for LRLT and cadaveric transplants, respectively. Overall post-transplant survival was 88% and 82% for children who received a LRLT or a cadaveric graft, respectively. Overall survival from the date of registration was 86% and 70% (P < 0.05) for LRLT or cadaveric LT respectively. The 2-year post-transplant survival in children less than 1 year of age at transplantation was 88.8% and 80. 3% with a LRLT or cadaveric graft, respectively; patient survival after 3 months post-transplant was 95.8% and 91.9% for stable children waiting at home, 93.7% and 93.7% in children hospitalized for complications of their disease, and 89.5% and 77.7% for children hospitalized in an intensive care unit at the time of transplantation for children who received a LRLT or cadaveric graft, respectively. It is concluded that LRLT seems to be justified for multidisciplinary teams having a large experience with reduced and split liver grafting.

Child↗

Intraabdominal pyloromyotomy through the umbilical route: a technical improvement.

BACKGROUND/PURPOSE: Performing a pyloromyotomy through a supraumbilical skin fold incision will leave an almost invisible scar and therefore has definitive cosmetic advantages. This alternative approach may be related to technical difficulties in delivering a large pyloric tumor when compared with the conventional pyloromyotomy through a right upper quadrant incision. However, in situ (intraabdominal) myotomy can help overcome this inconvenience. METHODS: Of 122 cases of infantile hypertrophic pyloric stenosis operated on between January 1990 and August 1996, 29 underwent a pyloromyotomy performed intraabdominally through the umbilical route. The medical records of these babies were reviewed. RESULTS: Twenty-three boys and six girls (median age, 30 days; range, 17 to 70 days) underwent surgery. The median hospital stay was 2.5 days. There were two intraoperative technical complications (small mucosal perforation) and one postoperative wound complication (abcess formation) requiring local drainage. CONCLUSIONS: In situ pyloromyotomy through the umbilical route is an elegant alternative in cases of a large pyloric tumor.

Female↗

Use of granulocyte macrophage colony stimulating factor in children after orthotopic liver transplantation.

BACKGROUND/AIMS: Bacterial infections complicate the course of up to 80% of pediatric liver transplant recipients, and in some cases, neutropenia, surgical complications and/or antibiotic resistance prevent successful control of sepsis. The aim of the present study was to evaluate the safety and efficacy of granulocyte macrophage colony stimulating factors (GM-CSF) in treating neutropenia following pediatric orthotopic liver transplantation. METHODS: Among a cohort of 430 pediatric orthotopic liver transplantation recipients, 13 children (12 months to 15 years, median 2 years, 10 males) received 15 courses of GM-CSF, 5 microg x kg(-1) x d(-1) subcutaneously, during their post-transplant course. In nine cases, the initial neutrophil count was below 1000/mm3. Ten patients were infected. Three received GM-CSF for severe sepsis without neutropenia. The mean duration of treatment was 16.3 days (range 4-49). RESULTS: In all but one neutropenic patient the neutrophil count increased above 1500/mm3 and the mean neutrophil count increased from 1392+/-1912/mm3 (range 130-7170, median 640) to 4508+/-2459/mm3 (range 350-9630, median 4390) (p<0.01). Only one neutropenic patient (FK506 related) failed to respond to treatment. No rejection episode was induced by treatment, no side effects were noted, and patients with sepsis were cured. CONCLUSION: In these patients, GM-CSF was safe, it achieved a significant increase in neutrophilic count, and was beneficial in patients with severe bacterial infections. This compound may prevent infectious complications in neutropenic patients and may benefit patients with severe sepsis with or without neutropenia.

Adolescent↗

Early signs and risk factors for the increased incidence of Epstein-Barr virus-related posttransplant lymphoproliferative diseases in pediatric liver transplant recipients treated with tacrolimus.

BACKGROUND: Posttransplant lymphoproliferative disease (PTLD) is a life-threatening condition the incidence of which in pediatric solid organ transplantation may be related to the immunosuppressive load. It has been suggested that tacrolimus, a new and potent immunosuppressor, causes an increased incidence of this syndrome. METHODS: The incidence, early signs, and risk factors for lymphoproliferative disease were reviewed in a cohort of 89 pediatric liver transplant recipients treated with tacrolimus. RESULTS: Eighteen patients (20%) developed a PTLD-16 concomitant to a primary Epstein-Barr virus (EBV) infection and 2 with previous immunity against EBV. Three additional patients had preliminary signs of PTLD concomitant to primary EBV infection, but did not develop individualized lymphoid masses. Six patients died (6.7% of all tacrolimus-treated patients). Mean tacrolimus blood level during the 3 months preceding EBV infection reached 11.8+/-1.8 ng/ml in PTLD patients versus 9.4+/-3.4 ng/ml in non-PTLD patients (0.05<P<0.1). Previous OKT3 or antithymocyte globulin treatment was also significantly associated to PTLD. There was no association with age, rejection episodes, steroid-resistant rejection, prior cytomegalovirus infection, HLA mismatch, living donor or cadaveric organ transplantation, United Network for Organ Sharing status at the time of orthotopic liver transplant, and primary or rescue tacrolimus treatment. A significant increase of total gamma-globulin level occurred in PTLD patients, and mono/oligoclonal production was significantly associated to PTLD. CONCLUSION: In EBV-infected pediatric liver transplant recipients, use of OKT3 or antithymocyte globulin and high tacrolimus blood levels are risk factors for a significant increase in the incidence of PTLD. An increase in total gamma-globulin level and appearance of mono/oligoclonal immunoglobulin production are the major preliminary signs of the syndrome.

Child↗

Cavocaval liver transplantation without venovenous bypass and without temporary portocaval shunting: the ideal technique for adult liver grafting?

The influence of the implantation technique on the outcome was studied prospectively in a series of 116 consecutive adult patients undergoing primary liver transplantation during the period January 1991-June 1994. Thirty-eight patients (32.8%; group 1) underwent classical orthotopic liver transplantation (OLT) with replacement of the recipient's inferior vena cava (R-IVC) and with venovenous bypass (VVB). Thirty-nine patients (33.56%) had a piggy-back OLT with preservation of the R-IVC (group 2); bypass was used in 17 of them (43.6%) because of poor hemodynamic tolerance of R-IVC occlusion. Thirty-nine patients (33.6%) had OLT without VVB and with side-to-side cavocaval anastomosis (group 3). The three techniques were performed irrespective of the anatomical situation and of the status of the recipient at the time of transplantation. The following parameters were assessed in all patients: implantation time, blood product use, morbidity (e.g., hemorrhagic, thoracic, gastrointestinal, neurological, and renal complications), and outcome. Thirty-one patients underwent detailed intraoperative hemodynamic assessment. The early (< 3 months) post-transplant mortality of 10.3% (12/116 patients) was unrelated to the implantation technique. Group 3 had a significantly shorter mean implantation time, a reduced need for intraoperative blood products, and a lower rate of reoperation due to intra-abdominal bleeding. After excluding two immediate perioperative deaths and eight patients requiring early retransplantation because of primary nonfunction, the frequency of immediate extubation was significantly higher in group 3. Detailed hemodynamic assessment did not show a difference between 6 group 1 patients and 17 group 3 patients, indicating that partial lateral clamping of the IVC fulfills the function of venous bypass. Similar results were obtained in 6 group 2 patients who did not have IVC occlusion. Cavocaval OLT has become our preferred method of liver implantation. It allows the transplantation to be performed without VVB, regardless of the anatomical situation and of the condition of the patient at the time of transplantation. Moreover, it avoids all of the potential complications and costs of VVB.

Adult↗

Clinical pharmacokinetics of Neoral in pediatric recipients of primary liver transplants.

Pediatric liver transplant recipients constitute a population characterized by a particularly unpredictable and poor bioavailability of cyclosporin (CyA). Even though several adult studies show that the new oral formulation of CyA, Neoral (NEO), produces better bioavailability and blood level predictability, few data describe its pharmacokinetics in children. We performed a complete analysis of the pharmacokinetics of NEO in ten small children after primary liver transplantation. Three pharmacokinetic profiles were set up with data obtained from tests taken during i.v. administration of CyA, after the first oral NEO dose, and after the last NEO dose before discharge from the hospital. The mean half-lives obtained were 8.1, 7.7, and 6.9 h, respectively, and the bioavailabilities were 22% and 21% for the first and last NEO doses. A large interpatient variability was observed. This was due, in part, to episodes of diarrhea that interfered with the pharmacokinetic evaluation and, in part, to the variability of post-transplant hepatic function. There was a good correlation between CyA trough levels and their related AUCs for both NEO profiles (r = 0.93 and r = 0.74, respectively). We conclude that, even though the pediatric OLT population remains more unpredictable than that of adults, NEO has a relatively rapid half-life and a remarkably improved bioavailability.

Administration, Oral↗

Arteriomesenteric syndrome as a cause of duodenal obstruction in children with cerebral palsy.

Two cases of gastroduodenal outlet obstruction caused by arteriomesenteric compression in children who have cerebral palsy are reported. Clinical symptoms of gastrointestinal obstruction include recurrent postprandial nausea and vomiting, upper abdominal distension, and pain. In such patients, multiple predisposing factors can contribute to the development of arteriomesenteric compression, including marked weight loss, supine position, and severe scoliosis. Upper gastrointestinal x-rays using barium contrast allow diagnostic confirmation. In our experience, this cause of acute gastroduodenal outlet obstruction may usually resolve after conservative treatment using a jejunal feeding tube passed beyond the compression, left lateral positioning, and renutrition.

Adolescent↗

Hirschsprung's disease: a 20-year experience.

During the period from 1972 to 1992, 59 children received surgical treatment at the University of Louvain Medical School for biopsy-proven Hirschsprung's disease (HD). The extent of aganglionosis was as follows: short segment restricted to the rectosigmoid or descending colon (n = 44, 75%); long segment (n = 9,15%); ultra-short segment (n = 3, 5%); unknown length because of death without autopsy (n = 3, 5%). The median age at operation was 7 months for short-segment disease compared with 14 months for those with long-segment disease. Surgical procedures used for short-segment disease were Swenson with colostomy (n = 16), Swenson-Pellerin without colostomy (n = 27), Duhamel (n = 1), and for long-segment disease were Martin (n = 3), Swenson-Deloyers (n = 2), Swenson-Boley (n = 2) and ileostomy only in = 2). Lynn's sphincteromyotomy was performed in the three ultra-short cases. There were six deaths (10%) at a median age of 86 days (range, 28 to 1545 days), three had long-segment disease, and the others were not classified because of death before curative surgery. Enterocolitis (EC) was the most common cause of death (five cases) and was also the major source of morbidity after curative surgery (12 of 44, 27%) in short-segment patients, three of seven (43%) in long-segment patients. The functional success of the procedure was evaluated in 70% of the surviving patients (37 of 53; mean follow-up, 8.7 years; range, 1.2 to 21.5), using a novel semiquantitative scoring system, specifically designed for children who have HD. This system assesses normal stool evacuation, abdominal distention, soiling, and severe incontinence. The results were compared with those from a population of 39 healthy children and adolescents and demonstrated progressive improvement in function during childhood and adolescence (P = .04) for patients treated for short-segment disease. However, function was found to be consistently poorer in all age groups when compared with healthy controls (5 to 10 years, P < .01; 10 to 15 years, P < .05; > 15 years, P < .01).

Enterocolitis↗

[Related living donor for liver transplantation in children: results and impact].

Encouraging results of alternative techniques used for liver transplantation in children (liver reduction) and the persistent lack of a sufficient number of cadaver donors has favored the development of living related donor liver transplantation. This program, which began after a long preparative period concerning the ethical questions involved, has included 32 children during the first 30 months. Results have been excellent. All children who underwent elective transplantations (n = 18) are still living. Among the 14 patients whose condition required hospitalization before transplantation, 86% have survived. Vascular complications and graft loss due to primary dysfunction or chronic rejection have been reduced, but 22% of the patients have biliary stenosis. In the donors, there has been no severe complication or sequelae. Use of related living donor livers has increased the number of grafts available for children on the waiting list for cadaver livers. The resulting gain in waiting time has also reduced the risk of death before transplantation. In our experience, the combination of the two transplantation programs using living donors and cadaver livers has had a positive impact on global management of children referred for liver transplantation, whatever the option chosen by the parents.

Adult↗