Extraperitoneal placement of renal allografts in children weighing less than 15 kg.

PURPOSE: Extraperitoneal renal transplantation is not routine in small recipients, in whom transperitoneal engraftment is the norm. The outcome of extraperitoneal placement of renal allografts in children weighing less than 15 kg. was evaluated at 2 institutions. MATERIALS AND METHODS: We retrospectively reviewed all pediatric renal transplantations at 2 institutions from 1988 to 2000 and identified 29 children 14 to 72 months old (mean age 29.2) weighing less than 15 kg. (range 8 to 14.8, mean 11.2). All children underwent allograft placement extraperitoneally via a modified Gibson and low midline retroperitoneal incision in 27 and 2, respectively. A concurrent procedure was done via the same incision during 2 ipsilateral and 2 bilateral nephrectomies. RESULTS: Of the 29 patients 25 have a functioning renal allograft. In 2 cases the initial allograft was lost due to early postoperative thrombosis and acute rejection in 1 each. Two patients with a functioning allografts died of medical complications greater than 2 years after transplantation. One child required reexploration secondary to fascial dehiscence and an additional recipient required pyeloureterostomy due to ureteral necrosis after living related donor transplantation. CONCLUSIONS: Extraperitoneal renal transplantation is technically feasible in children who weigh less than 15 kg. This approach preserves the peritoneal cavity, limits potential gastrointestinal complications and allows the confinement of potential surgical complications, such as bleeding and urinary leakage. In addition, this approach provides complete access to the retroperitoneum to enable concurrent retroperitoneal surgery, such as nephrectomy, to be performed safely. We recommend that extraperitoneal renal engraftment should become routine in children weighing less than 15 kg. rather than using the more common transperitoneal approach for allograft placement.

Survival after first esophageal variceal hemorrhage in patients with biliary atresia.

OBJECTIVE: To determine the influence of the new onset of esophageal variceal hemorrhage (EVH) on transplant-free survival in children with biliary atresia and to examine variables that predicted survival after the onset of EVH. METHODS: Retrospective chart review of 134 patients with biliary atresia who underwent portoenterostomy between 1973 and 1992 at a single institution; 29% had EVH. RESULTS: The risk of death or need for liver transplantation was 50% at 6 years after the initial episode of EVH. Patients with a serum bilirubin concentration < or =4 mg/dL at the first episode of EVH had transplant-free survival of >80% for 4 years after this episode, those with bilirubin levels >4 to 10 mg/dL had 50% survival at 1 year, and those with bilirubin levels >10 mg/dL had 50% survival at 4 months. The risk of death or transplant for a child with EVH and total serum bilirubin levels >10 mg/dL was 12.0 (95% CI: 6.0, 24.1), 4 to 10 mg/dL was 7.2 (3.1, 16.7), and < or =4 mg/dL was 0.6 (0.1, 3.1) times the risk of a same-aged child who did not have EVH. CONCLUSIONS: Children with biliary atresia and first EVH episode have a variable prognosis related to total serum bilirubin concentration at the time of the episode.

Life-threatening dog attacks: a devastating combination of penetrating and blunt injuries.

BACKGROUND/PURPOSE: Children often are the victims of dog attacks. Although bite injuries sustained in an attack characteristically are attributed to the penetrating component of the bite, the blunt nature of a bite may represent the most serious and devastating component of injury. The purpose of this study was to characterize a group of children suffering life-threatening dog bites and examine the predominant aspect of injury. METHODS: Thirty-nine children were admitted to the trauma service at a regional pediatric trauma center with the diagnosis of dog bite injury over a 6-year period (1994 through 1999). Patient demographics, site and description of injury, and surgical procedures performed were recorded from a chart review. RESULTS: Mean age of the 35 children included for analysis was 5.4 years (range, 0.8 to 17 years). Twenty-five (71%) injuries occurred in the head and neck region. Eight (23%) children sustained life-threatening injuries. Of these, blunt force was the predominant injury in 6. This resulted in 1 (20%) arterial occlusion requiring vascular reconstruction, 2 (40%) permanent neurologic injuries (stroke, spinal cord transection), and 1 (20%) death (exsanguination). CONCLUSIONS: On evaluation of a dog attack, the focus generally is on the obvious penetrating aspect of the bite. Yet, we found the blunt component of injury can have devastating consequences reflected in acute arterial, brain, and spinal cord injury. Even in the absence of significant penetrating trauma, further evaluation should be considered to exclude occult blunt arterial or neurologic injury.

Synthesis and structure-activity relationships of benzophenone hydrazone derivatives with insecticidal activity.

A broad range of benzophenone hydrazone derivatives was prepared and tested against selected chewing insect pests, allowing the analysis of structure-activity relationships. Good activity was found only when the aromatic rings were substituted at the 4-positions with an halogen atom and a triflate or perhaloalkoxy group. In contrast, a number of substituents on the hydrazone part led to active compounds, the best results being achieved with acyl-type substituents. The excellent laboratory and greenhouse activity of the best representatives was confirmed in semi-field trials against Spodoptera littoralis.

Neonatal cholestasis.

In simplest terms, cholestasis is defined as a decrease in bile flow. The clinical manifestations of cholestasis occur because of accumulation of substances normally excreted in the bile; namely bilirubin, bile acids, and cholesterol. Accumulation of bilirubin leads to jaundice and dark urine. Accumulation of bile acids is associated with pruritus, and accumulation of cholesterol causes hypercholesterolemia and xanthomas. There are many causes of cholestasis in early infancy ranging from normal physiologic jaundice to complete biliary obstruction associated with biliary atresia.

Hepatoblastoma in a neonate: a hypervascular presentation mimicking hemangioendothelioma.

Congenital heart failure in the neonate supported by classic imaging findings may allow the implementation of medical therapy for presumed hemangioendothelioma without obtaining a tissue diagnosis. This case report describes a neonate with these classic clinical and radiographic findings but who underwent surgery for failing medical treatment and was diagnosed as having a hepatoblastoma by pathology. This case supports the need to obtain tissue confirmation before beginning medical therapy.

Cervical spine trauma in the injured child: a tragic injury with potential for salvageable functional outcome,.

BACKGROUND/PURPOSE: Cervical spine injuries are uncommon in children, and, therefore, presumptive immobilization and diagnosis remain controversial. The purpose of this study was to review the author's experience with cervical spine injuries in children to determine the incidence, injury mechanism, pattern of injury, and subsequent functional outcome. METHODS: Fifty-two children over a 6-year period (1994 to 1999) with a cervical spine injury secondary to blunt trauma were identified (1.3% incidence). The functional independent measure (FIM) was assessed at the time of discharge in each of 3 categories: communication, feeding, and locomotion. RESULTS: Mean age of the study children was 10.7 +/- 0.7 years. Eight children (15%) were less than 5 years old, and 4 (8%) were less than 2 years old. The mechanism of injury included motor vehicle crash (52%), falls (15%), bicycle accidents (11%), sports-related injuries (10%), pedestrian accidents (8%), and motorcycle crashes (4%). Seven patients died yielding an overall mortality rate of 13%. Injuries were distributed along the cervical spinal cord as follows: 5 atlanto-occipital dislocations, 28 C1 to C3 injuries, 17 C4 to C7 injuries, and 2 ligamentous injuries. FIM scores were recorded for 18 patients. Seventeen communicated independently, 14 fed themselves independently, and 12 had independent locomotive function. CONCLUSIONS: Cervical spine injuries occur in children across a spectrum of ages. Although atlanto-occipital dislocation is a highly lethal event, children with C1 to C7 injuries have a high likelihood of reasonable independent functioning.

Operative management of injured children at an adult level I trauma center.

BACKGROUND: The geographic distribution of trauma centers results in a significant number of children being treated in adult centers. The emphasis on nonoperative management of pediatric blunt trauma has heightened concern that in adult trauma centers, an aggressive operative approach will continue to be used. We hypothesized that pediatric commitment at a Level I trauma center results in appropriate nonoperative care of injured children as established by regional pediatric trauma centers. METHODS: The records of 1,792 consecutively treated children admitted to the trauma service during a 6-year period (January of 1990 to December of 1995) were reviewed. Patients were stratified into one of three age groups: 0 to 5, 6 to 11, and 12 to 17 years of age. RESULTS: Mean age of the study patients was 10.0 +/- 0.1 years, 1,147 were boys (64%), and their mean Injury Severity Score was 7.3 +/- 0.3. The injury mechanism was blunt in 1,550 (87%) and 132 (7%) required laparotomy. In the 0- to 5-year-old blunt mechanism group, 6% underwent laparotomy or thoracotomy from 1990 to 1992. In comparison, only 1% of this age group had a laparotomy from 1993 to 1995 (p < 0.05, Fisher's exact test). A similar trend was found in the 6- to 11-year-old children after blunt injury (4% laparotomy rate from 1990 to 1992; 2% from 1993-1995). CONCLUSION: There has been a declining trend in the operative management of blunt pediatric trauma, especially in children less than 6 years old, whereas the operative management of penetrating injuries has remained stable. These data confirm that pediatric commitment in a Level I trauma center results in nonoperative treatment of injured children commensurate with that established in regional pediatric trauma centers.

Hepatocyte transplantation in acute liver failure.

The majority of patients with acute liver failure (ALF) die waiting for orthotopic liver transplantation (OLT). No other treatment modality is shown to improve survival. This study was conducted to assess the safety and feasibility of hepatocyte transplantation (HT) and subsequent engraftment and function of donor cells. Functional and structural integrity of cryopreserved and thawed human hepatocytes were assessed by their morphological characteristics, induction of P-4501A1 transcription, and survival in vivo by xenotransplantation into rats. Five patients with severe ALF underwent intrasplenic (4 patients) and/or intrahepatic (2 patients) HT through angiography under cyclosporine immunosuppression. All patients had grade III to IV encephalopathy and factor V levels less than 0.5 U/mL, were ventilator and dialysis dependent, and were not OLT candidates. Three of the 5 patients who survived 48 hours after HT had substantial improvement in encephalopathy scores, arterial ammonia levels, and prothrombin times. Clinical improvement was paralleled by an increase in aminopyrine and caffeine clearances. All 3 patients lived substantially longer than expected based on clinical experience after HT (12, 28, and 52 days) but eventually died. Postmortem examination showed the presence of transplanted hepatocytes in liver and spleen by light microscopy and fluorescent in situ hybridization (FISH). Cryopreserved and thawed human hepatocytes can be transplanted into recipients with ALF with some acceptable but definite complications. Engraftment of donor hepatocytes was proven by histological examination and FISH by both transjugular biopsy and at autopsy. Improvement in brain edema, encephalopathy grade, and clearance of antipyrine and caffeine suggested function, albeit with a 24- to 72-hour delay posttransplantation.

Nonoperative management of solid organ injuries in children results in decreased blood utilization.

BACKGROUND: The administration of blood products to injured children has been recognized as a potential risk of nonoperative management. The purpose of this study was to evaluate blood utilization in the management of solid organ injuries in pediatric blunt abdominal trauma victims. METHODS: One hundred sixty-one children (< or =16 years old) with solid organ injuries over an 8-year study period (1990 through 1997) were identified from the trauma registries at 2 urban regional trauma centers. RESULTS: Mean age of the study patients was 7.9+/-0.4 years, 95 (59%) were boys, and their mean injury severity score (ISS) was 17.8+/-1.2. Patients were divided into 4-year study cohorts (1990 through 1993 and 1994 through 1997) to examine changes in operative management and blood utilization. For each time period examined, those treated nonoperatively received fewer blood transfusions (46% v 9% and 44% v 13%, P<.05 by Fisher's Exact test), and the hospital length of stay was shorter (12.3+/-2.1 v 5.0+/-0.7 and 7.8+/-1.9 v 4.2+/-0.4 days, P<.0001 by analysis of variance/Scheffe's) compared with the laparotomy cohort. CONCLUSIONS: The appropriate nonoperative management of injured children actually reduces the risks of receiving blood transfusion and decreases the length of hospital stay compared with aggressive operative intervention. Blood transfusion should be reserved only for those injured children with solid organ injuries who are hemodynamically unstable.

Esophageal varices: current management in children.

The treatment of esophageal variceal hemorrhage is still the subject of some controversy. The main causes of portal hypertension in children are portal vein thrombosis or cirrhosis, most commonly caused by biliary atresia. Many treatment options are available including endoscopic, radiographic, and surgical strategies. In general, children with presinusoidal obstructions have preserved hepatic synthetic function, and, therefore, treatment options include endoscopic strategies or portosystemic shunts, each with advocates. For children with advanced liver disease, liver transplantation offers the only chance for cure, so primary treatment of variceal bleeding should be by endoscopic means or transjugular intrahepatic portosystemic shunt (TIPS). Each modality has specific advantages and disadvantages, and treatment recommendations must therefore be tailored to the individual on a case-by-case basis, largely dependent on the expertise and experience of the health care team.

Use of rifampin for severe pruritus in children with chronic cholestasis.

BACKGROUND: Rifampin has been proposed to reduce pruritus in children and adults with chronic cholestasis; however, there is a paucity of published data regarding the use of rifampin in children. METHODS: In an open trial, 24 children were evaluated during a 6-year period. Diagnoses included 13 patients with extrahepatic biliary atresia (54%), six with Alagille's syndrome, three with Byler's disease, and one each with primary sclerosing cholangitis and alpha1-antitrypsin deficiency. All patients had severe pruritus that had not responded adequately to at least 2 months of therapy with ursodeoxycholic acid, diphenhydramine, or phenobarbital and local skin care measures. Treatment was initiated with rifampin, 10 mg/kg per day in two divided doses for 18+/-20 months, and the effect on the severity of pruritus was assessed by a clinical scoring system. RESULTS: Ten patients showed a complete response, 12 a partial response, and 2 no response. Complete response was more common in extrahepatic cholestasis (64% vs. 10%), whereas partial response was more common in intrahepatic cholestasis (80% vs. 29%). Treatment was associated with reduction of gamma-glutamyl transpeptidase. No clinical or biochemical toxicity of rifampin was observed. CONCLUSIONS: We conclude that for more than 90% of children with chronic cholestasis and severe pruritus unresponsive to other treatments, rifampin appears to be a safe and effective therapy.

Histological identification of purified and cryopreserved allogeneic hepatocytes following transplantation in a murine model without host immunosuppression.

Hepatocyte transplantation is a conceptually attractive alternative to whole organ grafting for some inborn metabolic errors and for fulminant liver failure. However, studies of the immunogenicity of transplanted allogeneic hepatocytes have yielded contradictory results. In these experiments, the effect of purification and cryopreservation of the hepatocytes on the ability of these cells to engraft in the mouse allogeneic recipients without immunosuppression was studied. BALB/cByJ mouse crude (unpurified), modified (purified or cryopreserved), or dead (irradiated) hepatocyte preparations labeled with fluorescein dye CFSE were infused either into the portal vein or into the spleen parenchyma of the recipient CBA mice. A histological examination revealed normal appearance of engrafted modified hepatocytes with no signs of acute rejection up to 21 days posttransplant. Many of the intrasplenically implanted hepatocytes migrated into the hepatic sinusoids. The modified hepatocytes showed intact ultrastructural appearance 7 days after transplantation. The numbers of inoculated crude hepatocytes rapidly declined with signs of dense infiltration of mononuclear cells in the graft indicating destructive response. The fluorescence of dead hepatocytes was undetectable. These results suggest that reduced immunogenicity may be responsible for the longer survival time of inoculated, purified or cryopreserved hepatocytes with no adverse morphological effects.

Correction of hypozincemia following liver transplantation in children is associated with reduced urinary zinc loss.

Zinc deficiency is a relatively common problem in children with chronic liver disease. We have previously shown inappropriate urinary zinc excretion in children with chronic liver disease and hypozincemia. This study was designed to determine if zinc deficiency and inappropriate urinary zinc losses are corrected in children with liver disease by liver transplantation. Thirty-three patients (age 1-19 years) underwent 35 liver transplants for acute and chronic liver disease. At the time of transplant, 17 patients had low plasma zinc (hypozincemic) (plasma zinc, 45.4 +/- 1.8 microg/dL), whereas 18 had normal plasma zinc (75.7 +/- 3.8). Before transplant, patients with zinc deficiency had higher urinary zinc to creatinine ratio compared with those with normal zinc status (6.6 +/- 1.9 vs. 2.2 +/- 0.6; P =.03) and lower serum albumin concentrations (low: 2.8 +/- 0.1 vs. normal: 3.3 +/- 0.2; P =.02). After transplant, there was a significant reduction in urinary zinc losses in the hypozincemic group followed by normalization of plasma zinc levels by 7 days posttransplant. These data suggest that the abnormal renal zinc homeostasis that is present in approximately 50% of pediatric patients undergoing liver transplant is rapidly improved and biochemical zinc deficiency is reversed after liver transplantation.

Driveway crush injuries in young children: a highly lethal, devastating, and potentially preventable event.

BACKGROUND/PURPOSE: The aim of this study was to investigate driveway-related injuries in children, identify associated risk factors, and evaluate outcome compared with other mechanisms of blunt trauma. METHODS: A 6-year review (1991 to 1996) of pediatric (age less than 18 years) pedestrian injuries treated at two urban trauma centers was conducted: one regional pediatric trauma center and one level I trauma center with pediatric commitment. Five hundred twenty-seven children injured in pedestrian accidents were identified from the trauma registry; 51 children (10%) sustained traumatic injuries as a result of being struck in their driveway. Data are reported as mean +/- SEM. RESULTS: Children less than 5 years of age (n = 41) had an injury severity score (ISS) of 12.3+/-2.3, 15 (37%) sustained closed head injury, 13 (37%) had torso trauma, 19 (46%) skeletal trauma, and eight (20%) died. Children > or = 5 years old (n = 10) had an ISS of 10.7+/-2.4, three (30%) sustained closed head injury, four (40%) torso trauma, six (60%) skeletal trauma, and none died. In contrast, all other pediatric pedestrian accidents analyzed over the same time period had a mortality rate of only 2% (11 of 476). CONCLUSIONS: Pediatric driveway trauma carries a significant risk of head injury and a 10-fold increase in mortality in children under 5 years of age when compared with all other pediatric pedestrian accidents. More emphasis must be placed on injury prevention and public education to prevent this devastating mechanism of injury in these young, vulnerable children.

Adult living donor liver transplantation using a right hepatic lobe.

BACKGROUND: Living donor liver transplantation has gained wide acceptance as an alternative for children with end-stage liver disease. The standard left lateral segment used in this operation does not provide adequate parenchymal mass to broaden its application to larger children or adults. METHODS: We report two cases of adult to adult living donor liver transplantation using a right hepatic lobe in patients with chronic liver disease. RESULTS: Both recipients experienced excellent initial graft function and have normal liver function 4 and 9 months postoperatively. Both donors are alive and well and returned to normal life 4 weeks postoperatively. CONCLUSIONS: Our initial experience suggests that this technique is a safe and reliable option for adults with chronic end-stage liver disease. A conservative application of this procedure in the adult population could significantly reduce the mortality on the adult waiting list.

Fulminant Epstein-Barr viral hepatitis: orthotopic liver transplantation and review of the literature.

Acute hepatic failure caused by primary Epstein-Barr virus (EBV) infection has been reported in the literature in 16 cases, with an overall mortality of 87%. We report a case of fulminant hepatic failure in an immunocompetent young girl caused by primary EBV infection that was treated by orthotopic liver transplantation. After transplantation she has been treated with low-dose immunosuppression, a pooled gammaglobulin preparation containing anti-EBV antibodies, and anti-viral therapy. The patient is presently doing well 2 years after transplantation without evidence of clinical EBV infection, primary immunodeficiency, or lymphoproliferative disease.

Is routine pathological evaluation of pediatric hernia sacs justified?

BACKGROUND/PURPOSE: Herniorrhaphy is the most common general surgical procedure performed on children, and hernia sac material is one of the most common tissue specimens microscopically examined in the authors' surgical pathology laboratory. The risk of accidental vas deferens ligation has prompted the recommendation that all hernia sacs be examined pathologically. The authors hypothesized that the incidence of unrecognized vas deferens or epididymis ligation is actually very low and may not warrant routine pathological examination of all pediatric hernia sacs. METHODS: Over a 3-year period (1994 to 1996), pathology reports from all hernia repairs at the authors' institution were reviewed. A total of 1,494 inguinal hernia sacs were pathologically evaluated from 1,077 pediatric patients (417 were bilateral). Pathological diagnoses not affecting clinical management (ie, chronic inflammation, irritated hernia sacs, embryonal remnants, adrenal cortical rests) were classified as incidental findings. Identification of true vas deferens was classified as a positive finding. RESULTS: The study population had a mean age of 3.9 +/- 0.1 years and 963 (89%) were boys. The incidence of vas deferens injury from herniorrhaphy was found to be 0.13% (2 of 1,494), and these were recognized by the pediatric surgeon in the operating room. CONCLUSIONS: When vas deferens injury is suspected, the sample should always be sent to the pathology department for confirmation. However, no occult carcinoma or other pathology was identified, and the remainder of the histological findings did not change the clinical treatment of any child. Given a fixed cost of pathological analysis, elimination of routine hernia sac examination may result in substantial annual savings. Therefore, in the current era of cost containment, recommendations for routine pathological examination of excised pediatric hernia sacs should be reevaluated.