Fibrin clot lysis by tissue plasminogen activator (tPA) is impaired in plasma from pediatric patients undergoing orthotopic liver transplantation.

Large vessel thrombi can present life-threatening complications following orthotopic liver transplantation (OLT) in pediatric patients. We investigated the thrombolytic response to tissue plasminogen activator (tPA) of stored, pooled plasma (days 4-14 postoperatively) from 41 patients (mean age 4 years, 9 months) who underwent OLT at the Hospital for Sick Children, Toronto between 1986 and 1990. Trace-labeled fibrin clots were prepared by recalcifying 500-microliters aliquots of patient plasma spiked with 125I fibrinogen and then incubated at 37 degrees C in patient plasma in the presence or absence of tPA (0.1 or 0.3 mg/ml). At the end of the incubation period, the extent of clot lysis and concentrations of fibrinogen, plasminogen, and alpha 2 antiplasmin were determined. Pooled adult plasma was used as a control. Fibrin clot lysis in OLT plasma was significantly reduced compared with controls (P < 0.01). Initial concentrations of plasminogen were significantly reduced in OLT plasma. To determine if the low plasminogen levels limited the thrombolytic effect of tPA, we supplemented OLT plasma with purified plasminogen. Fibrin clots placed in OLT plasma containing adult levels of plasminogen showed a similar lytic response as adults. In summary, the reduced fibrinolytic response of OLT fibrin clots to tPA was due to low concentrations of plasminogen and corrected by plasminogen supplementation.

Lymphoproliferative disorders after organ transplantation in children.

BACKGROUND: After organ transplant, patients are at risk of posttransplant lymphoproliferative disorders (PTLD). The purpose of this study was to analyze 26 pediatric cases of PTLD observed at our institution between 1988 and 1996, and to evaluate the validity of the Society for Hematopathology Workshop (SHPW) 1997 classification in our patient population. METHODS: Charts were reviewed for analysis of incidence, clinical course, and outcome. Tissue samples were classified by a pathologist according to SHPW recommendations. RESULTS: By morphology, 20 were monomorphic, 5 polymorphic, and 1 hyperplastic. Assessment of lineage by morphology, molecular studies, and immunophenotyping did not correlate in six cases. By immunophenotyping, 12 were B cell, 4 T cell, 8 mixed B/T cells, and 2 undetermined. The 20 patients evaluable for treatment efficacy were treated with various therapeutic combinations, including immunosuppressive drug reduction, acyclovir/ganciclovir, interferon-alpha, immunoglobulins, surgery, and local irradiation. No patient received systemic chemotherapy. Thirteen patients achieved complete remission and 3, partial; 1 died 5 days after starting therapy, and 3 of progressive disease. Adverse prognostic factors included low platelet or neutrophil counts; stage III-IV and SHPW morphology were marginally significant. CONCLUSIONS: The majority of patients eligible for treatment can be cured with immunosuppressive drug reduction and antiviral drugs, along with surgery and irradiation when indicated. Systemic chemotherapy or innovative approaches may have a role in unresponsive cases. Morphologic SHPW grouping is feasible and seems to have clinical relevance. However, correlation with clonality and immunophenotyping is not always possible, necessitating modifications including segregation of descriptive morphology from clonality and cell origin.

Treatment of primary liver graft nonfunction with prostaglandin E1.

Primary nonfunction following orthotopic liver transplantation is characterized by rapidly rising serum transaminases, minimal bile production, and severe coagulopathy, which can progress to hypoglycemia, hepatic encephalopathy, and acute renal failure. Untreated it has a mortality of over 80% and to date the only treatment has been retransplantation. As a result of the beneficial effect of Prostaglandin E1 infusion in patients with fulminant hepatic failure, this trial was conducted to determine whether PGE1 would be of value in primary nonfunction. We have encountered 16 cases of primary nonfunction in 94 liver transplants, an incidence of 17%. Initially in the program, there were 6 occurrences of nonfunction that did not receive PGE1; 3 underwent retransplantation (2 survivors), 2 died awaiting another liver, and in one recovery of hepatocellular function occurred with supportive care but the patient died of cytomegalovirus infection. Ten patients received PGE1 within 4-34 hr of transplantation. Within 12 hr of treatment, 8 patients responded with a significant fall in the AST (129 U/hr) whereas, in the untreated group, the AST continued to rise (267 +/- 102 U/hr) at the same rate as prediagnosis (337 +/- 95 U/hr). At the conclusion of the infusion (4-7 days) in the 8 responders, there were significant decreases in AST (4386 +/- 546 U/L to 102 +/- 21 U/L), prothrombin time (22 +/- 2 to 12 +/- .4 sec) and partial thromboplastin time (45 +/- 3-29 +/- 4 sec), and significant increases in coagulation factor V (26 +/- 8 to 95 +/- 12%) and factor VII (10 +/- 5 to 61 +/- 4%). No serious side effects occurred, although 2 patients developed diarrhea, and abdominal cramps. Two patients treated with PGE1 were retransplanted at 10-36 hr and were considered nonresponders. Graft survival was 80% in the PGE1-treated group and 17% in the untreated group (P less than 0.05) and patient survival was 90% and 33%, respectively. This study suggests a potential benefit of PGE1 in the treatment of primary nonfunction.

NOF-11: a one-year pediatric randomized double-blind comparison of neoral versus sandimmune in orthotopic liver transplantation.

BACKGROUND: Although cyclosporine (CsA) has been a mainstay in liver transplantation immunosuppression the original formulation [Sandimmune (SIM)] has variable absorption, particularly in children. Neoral is a new formulation of CsA that may have improved biovailability that would be advantageous in children. This study was undertaken to assess the pharmacokinetics (PK) and effects on outcome of Neoral versus Sandimmune (SIM) in primary pediatric liver transplant recipients. METHODS: Thirty-two patients were randomized to receive Neoral (17 patients) or SIM (15 patients) in the early posttransplant period (days 1-7) in a double-blind fashion. Intravenous CsA was instituted immediately posttransplant followed by Neoral or SIM as soon as the patient was tolerating oral fluids (days 1-7). PK were compared after the first dose (1-7 days), 3 weeks, and 6 and 12 months posttransplant. In addition, side effects, effect of age and food on absorption, and rejection episodes were assessed by intent to treat analysis. Notable characteristics of this study include the use of a central laboratory for all sample analyses and the assessment of renal function using radioisotopic evaluation of glomerular filtration rates. RESULTS: At baseline the two groups were comparable. Neoral resulted in higher peak levels of CsA and total drug exposure with comparable time to peak drug levels at days 1-7 and week 3. This trend was maintained at 6 and 12 months. Time on i.v. CsA was reduced in the Neoral group (8.4 vs. 11.1 days) and the weight adjusted daily dose of SIM required to achieve target trough levels was about 2-fold more than Neoral from day 22 onward. In addition, biopsy proven and treated and steroid-resistant rejection episodes were fewer in the Neoral group (6 vs. 12; P=0.01 and 1 vs. 8: P=0.004, respectively). Side effects were comparable in both treatment groups. CONCLUSIONS: Neoral was well tolerated and had greater biovailability than SIM without any increase in the incidence of side effects. In addition fewer episodes of rejection were observed with Neoral versus SIM. We conclude that Neoral is the CsA formulation of choice for use in pediatric liver transplant recipients.

Circulating dermatan sulfate and heparan sulfate/heparin proteoglycans in children undergoing liver transplantation.

The liver produces dermatan sulfate (DS), heparan sulfate (HS) and heparin glycosaminoglycans (GAG) and in the presence of hepatic disease, tissue levels of the DS GAG increase dramatically. We hypothesized that in children undergoing liver transplantation plasma levels of DS would be increased. Plasma from children undergoing liver transplantation were tested preoperative, intra operative and post operative at 24-48 h, and 1-3 weeks. Fluctuating levels of DS, HS and heparin anticoagulant activity were detected at all timepoints. The anticoagulant activity was purified and gel chromatography of the material displayed a mean Mr 110,000 D. Reductive elimination decreased the mean Mr 24,000 D indicating the activity resides on a proteoglycan (PG). The purified material was subjected to further chromatography and two peaks of anticoagulant activity resolved, compatible with at least two separate PGs, one with DS GAG chains and the additional PG(s) with HS and heparin GAG chains.

Hirschsprung disease, postaxial polydactyly, and atrial septal defect.

We report on an infant girl with Hirschsprung disease, postaxial polydactyly, and atrial septal defect who was born to a consanguineous Iraqi couple. A similar condition of aganglionic megacolon, postaxial polydactyly, and ventricular septal defect with a presumed autosomal recessive (AR) inheritance was reported by Laurence in two sibs [Laurence et al.; J Med Genet 12: 334-338, 1975].

Spontaneous pneumothorax in cystic adenomatoid malformation. Unusual clinical and histologic features.

Pneumothorax is a rare presentation of congenital cystic adenomatoid malformation (CCAM) in the newborn period and is presumed to be due to resuscitative measures. A previously well three-week-old baby presented with spontaneous tension pneumothorax due to CCAM. In the lung resection specimen, a malformation was seen, which in addition to the histologic changes of CCAM, showed diffuse vascular proliferation in the interstitium and lining of air space by type 2 pneumocytes. We propose that this is a new variant of CCAM rather than one of the classic three types. The unusual clinical manifestation may be related to the unusual histologic features.

Distal splenorenal shunts for the treatment of severe thrombocytopenia from portal hypertension in children.

Profound thrombocytopenia resulting from portal hypertension may exacerbate gastrointestinal bleeding, precipitate spontaneous bleeding, preclude surgical intervention for associated disorders, and severely limit life-style because of the danger of splenic injury. Although splenectomy can reverse the thrombocytopenia, the procedure should be avoided in children. We reviewed our experience with distal splenorenal shunting (DSRS) in children, particularly when performed for the sole purpose of reversing severe thrombocytopenia resulting from portal hypertension. DSRS was performed in 11 children between the ages of 7 and 15 years: five for severe thrombocytopenia (group 1), four for advanced hypersplenism and congenital hepatic fibrosis prior to renal transplantation (group 2), and two for esophageal bleeding (group 3). One child in group 1 with severe heart disease and Child's class C cirrhosis due to hepatitis C died of progressive cardiac failure and was excluded from further analysis. Of the eight remaining patients in groups 1 and 2, four children had congenital hepatic fibrosis, two had portal vein thrombosis, one had hepatitis B, and one had Wilson's disease. After DSRS, the mean platelet count increased from 37,000 +/- 18,000 to 137,600 +/- 81,000 (P = 0.01). The platelet count improved significantly in all seven children with presinusoidal portal hypertension or stable cirrhosis but did not increase in the child with hepatitis B and Child's class B cirrhosis. The white blood cell count increased from an average of 3.3 +/- 1.1 to 5.4 +/- 2.6 (P= 0.02). There were no postoperative complications in this group. The improved platelet count allowed the four children with congenital hepatic fibrosis and renal failure to undergo renal transplantation with full posttransplant immunosuppression including azathioprine. Postoperative Doppler ultrasound examination demonstrated shunt patency at 6 months in all cases. Spleen size decreased appreciably in all children in groups 1 and 2. All children were able to resume full activity including contact sports. In summary, DSRS effectively controls profound thrombocytopenia resulting from presinusoidal portal hypertension or stable cirrhosis without sacrificing the spleen and should be the treatment of choice for this condition.

Congenital absence of the portal vein: two cases and a proposed classification system for portasystemic vascular anomalies.

Congenital absence of the portal vein (CAPV) is an unusual condition that often is associated with other anomalies. This is the first report of reduced-size liver transplant in a patient with CAPV. Because the presence of this rare congenital portasystemic shunt, there was no portal-systemic pressure differential, and thus an absence of collateral vessels in the pretransplant state. As a result, surgery was complicated by severe mesenteric edema caused by an increase in portal pressure when the allograft was implanted. The morbidity associated with CAPV usually results from associated conditions, but if transplantation is necessary, careful management of mesenteric congestion is crucial to success. The authors' experience and a review of the literature indicate that the CAPV can be classified into one of two groups of portasystemic anomalies.

Results of liver transplantation in children with unresectable liver tumors.

Liver cancer is an uncommon indication for liver transplantation in children. Between 1986 and 1995, five children with hepatocellular cancer (HCC), three with hepatoblastoma (HEP), and one with sarcoma were referred to the transplant service. All nine tumors were considered unresectable. Four of the five children with HCC had underlying predisposing conditions (2 hepatitis B, 1 biliary atresia, 1 tyrosinemia). Preoperative evaluation of all patients included careful radiological screening and pretransplantation laparotomy for staging. Two patients with HCC were excluded from further consideration because of intraabdominal spread. Three patients had transplantation (mean age, 6.0 +/- 7.1 years), and all have survived for 1 to 5 years with no evidence of recurrence. Three patients with HEP were assessed (mean age 2.0 +/- 1 years); two had stage 4 disease and one had stage 3. All three received preoperative chemotherapy. The two with stage 4 had thoracotomies as part of their assessment. Two of three patients had a significant decrease in the size of the primary tumor during the waiting period. These two patients and one with stage 4 disease have survived more than 2 years since transplantation, with no recurrence. The third patient had recurrence within 2 months of transplantation. In summary, liver transplantation should be considered for all children who have unresectable hepatic malignancies, given the 83% survival rate and no evidence of tumor recurrence. Stage 4 disease in HEP does not necessarily exclude patients from transplantation. Early referral is encouraged so that tumor spread beyond the liver is minimized.

Lymphoproliferative disease after pediatric liver transplantation.

Posttransplant lymphoproliferative disease (LPD) is a serious complication, associated with considerable morbidity and mortality. Herein the authors report their experience with LPD in a series of pediatric liver recipients (from 1986 to 1993). A total of 95 transplants were performed in 78 patients. Only the 66 patients who survived at least 30 days were included in the analysis. There were seven cases of LPD (incidence, 10.65). Seven of the 43 patients who received OKT3 had LPD, compared with none of the 23 patients who did not receive OKT3 (P < .05). The total cumulative dose and the duration of therapy both correlated with occurrence of LPD. However, the dose per kilogram did not correlate with the development of LPD. The median time from transplant to diagnosis was 90 days. All cases were immunoblastic B-cell lymphomas, and all tumors were positive for the Epstein-Barr viral genome (EBV). Four patients never treated for LPD died; it was discovered incidentally during autopsy in two, during retransplantation in one, and within 5 days of death in one. The other three were treated with decreased immunosuppression, acyclovir, gamma globulin, and alpha-interferon. All three were cured of LPD, but one died of neurological complications after retransplantation. LPD may be interpreted as a symptom of a chronically overimmunosuppressed state, associated with a high mortality, from a variety of causes. LPD should be suspected for any patient whose clinical condition is deteriorating with no clear evidence of rejection, and should lead to a decrease in the amount of immunosuppression used.

Unconventional treatment of neuroenteric cyst in a newborn.

Neuroenteric cysts are uncommon congenital malformations that can require early surgical treatment. The authors report on an unusual treatment of a very large neuroenteric cyst that involved most of the small bowel and extended into the chest. A 1-day-old boy was admitted because of abdominal distension. The prenatal ultrasound results at 8 and 36 weeks had been normal. Examination showed right upper quadrant fullness and mild respiratory distress. A malformed sternum and asymmetric upper thoracic vertebra were seen on the initial x-rays. The possibility of a midthoracic right paravertebral mass was raised. Abdominal ultrasound findings were consistent with a large bowel duplication cyst. Laboratory results were all normal except the bilirubin level, which was (59 mmol/L). During laparotomy, the second part of the duodenum was found to enter a dilated cyst, and the terminal ileum arose from the cyst. The total length of the intact small bowel was 20 cm including a competent ileocecal valve. The site of the biliary duct entering the cyst was not clear. The surgical procedure involved partial resection of the anterior wall of the cyst, creation of an enteric tube from the posterior cyst wall to communicate between the duodenum and the ileum, and addition of another 25 cm of length to the small bowel. An enterocutaneous fistula was created with the proximal portion of the cyst because the site of the papilla of Vater was suspected to enter this part of the cyst. A postoperative HIDA scan showed good uptake with no excretion into the gut or the proximal pouch.(ABSTRACT TRUNCATED AT 250 WORDS)

Coagulation plasma factor levels are early indicators of graft nonfunction following liver transplantation in children.

Following liver transplantation, the decision to retransplant in cases in which graft function is marginal must be taken early. Plasma coagulation factor monitoring was evaluated as an early predictor of graft failure requiring retransplantation in the first posttransplant week. Plasma levels of fibrinogen, factors V, VII, VIII, IX, antithrombin III, protein C, and plasminogen were measured in all patients at 0, 12, 24, 48, 72, 96, and 120 hours posttransplant in 46 patients who received 56 grafts and results were compared between livers that failed early (group 1) and those that functioned adequately (group 2). Six grafts were included in group 1: one patient died before retransplantation, four were retransplanted once, and one patient was retransplanted twice. Three grafts had primary nonfunction (PNF), 2 had obstructed portal veins, and 1 had a long period of warm ischemia during the initial transplant. In group 1, plasma levels of factor V were significantly lower than in group 2 at 24, 48, and 72 hours posttransplant (21.2% +/- 14.2%, 12.4% +/- 4.5%, and 13.0% +/- 5.0% v 39.1% +/- 23.9%, 48.8% +/- 31.9%, and 60.9% +/- 25.9%; P less than .05, P less than .01, and P less than .005, respectively). Similarly, plasma levels of factor VII were significantly lower in group 1 over the same period of time (7.3% +/- 2.7%, 4.2% +/- 1.8%, and 4.7% +/- 2.5% v 27.4% +/- 17.1%, 34.1% +/- 21.6%, and 34.8% +/- 18.6%, respectively; P less than .005).(ABSTRACT TRUNCATED AT 250 WORDS)

Surgical complications in children after liver transplantation.

The frequency of surgical complications after liver transplantation remains high. Sixty transplants were done in 48 patients during 4 years. Eleven patients were retransplanted (re-transplant rate, 20%) for primary nonfunction (6), arterial thrombosis (3), warm ischemia (1), and rejection (2). Right pleural effusions were drained in 13 patients and left ones in 2. Forty-eight re-explorations excluding retransplantation were performed in 20 patients. Twelve laparotomies were for control of postoperative intraabdominal bleeding. The majority of these patients (8/10, 80%) were transplanted with reduced-size grafts. Early postoperative vascular complications were detected in 12 grafts (5 portal vein occlusions, 7 arterial thromboses). All 5 patients with portal vein (PV) occlusions were reexplored, and PV flow was reestablished in all 5. Biliary leaks were diagnosed in 6 patients and were associated with arterial thromboses in 2 cases. Reoperation was required in 4 of 6 patients. Bowel perforation occurred in 4 patients; 2 small bowel, 1 duodenum, and 1 colon. There was 1 postoperative bowel obstruction requiring laparotomy. Two splenectomies were required in 4 patients with splenic infarction. Resection of part of a transplanted liver was done in 1 patient to exclude septic infarcts. Pancreatitis was diagnosed in 4 patients and one required laparotomy for control of pancreatic hemorrhage. Intraabdominal abscesses required open drainage in 2 patients and percutaneous drainage in 4. Seven thoracotomies were done in 6 patients: 5 open lung biopsies, 1 for control of hemorrhage, and 1 for diaphragmatic plication. The current high survival rates following liver transplantation require aggressive surgical management of a myriad of complications and numerous procedures are necessary both as treatment modalities and as diagnostic aids.

A modified Sugiura operation for bleeding varices in children.

Objections to portal systemic shunting in children with life-threatening hemorrhage from esophageal varices include the high incidence of postshunt encephalopathy with neurologic and psychiatric sequelae and the inability to provide an adequate shunt in very young children. We have operated on eight children in the past 4 years for bleeding varices. The causes were: portal vein thrombosis (3), congenital hepatic fibrosis (2), chronic active hepatitis (2), and cystic fibrosis (1). The ages at operation were between 2 and 17 years. These children underwent various modifications of an operation described by Sugiura. The operation we have developed is done through a single thoracoabdominal incision, dividing and anastomosing the esophagus with a stapler, preserving the vagal innervation to the pylorus and antrum, and wrapping the fundus around the distal esophagus at the site of the anastomosis. The venous drainage of the lower esophagus and of the upper stomach is divided. The operation is therefore shorter and simpler, but adheres to the principles enunciated by Sugiura. Complications include one significant postoperative anastomotic leak and one symptomatic esophageal stricture. Longterm results have been gratifying with no evidence of rebleeding from esophageal varices. We believe that our modification of the original Sugiura operation is the preferred therapy of bleeding esophageal varices when surgical intervention is indicated because it preserves the normal structure and function of the upper gastrointestinal tract as well as the portal venous drainage to the liver.

The diagnosis of pneumonia in the immunocompromised child: use of bronchoalveolar lavage.

Between January 1987 and December 1988, 26 immunocompromised children (aged 15 months to 17 years) underwent bronchoalveolar lavage (BAL) for evaluation of pneumonia (chemotherapy for malignancy, 12; orthotopic liver transplantation, 9; other hematologic disease, 5). Bilateral diffuse pulmonary disease was present in 25 children. All were receiving broad spectrum antibiotics. In addition, five were receiving antiviral therapy and two were receiving antifungal therapy. Sixteen patients underwent rigid and 10 underwent flexible bronchoscopy. Two lavages of 10 to 20 mL of normal saline were obtained from involved subsegmental bronchi of both lungs in each patient. Second wash samples from each lung were sent for bacterial and viral cultures, silver staining for pneumocystis, and direct electronmicroscopy analysis for viral particles. Samples were considered satisfactory if they contained an abundance of alveolar macrophages and only small numbers of upper respiratory tract epithelial cells. Alveolar macrophages were present in 21 (81%) of the BAL samples. A specific infectious agent was identified in 15 of these patients (cytomegalovirus [CMV], 6; Pneumocystis carinii, 4; gram-positive cocci, 3; Candida albicans, 2), and therapy was modified in 12. In the five patients in whom BAL samples were contaminated with upper respiratory tract cells no infectious agents were isolated. Because of continued clinical deterioration, open-lung biopsies were performed in three patients in whom BAL had identified CMV and in three patients in whom no organisms had been obtained. Lung biopsies did not identify any new infectious agents, although in the latter group specific histological diagnosis of a noninfectious process was made (hemorrhagic infarct, bronchiolitis obliterans, and lymphoma).(ABSTRACT TRUNCATED AT 250 WORDS)

Unilateral pulmonary agenesis with esophageal atresia and distal tracheoesophageal fistula: report of two cases.

The combined anomalies of pulmonary agenesis with esophageal atresia and distal tracheoesopahgeal fistula are an exceedingly rare and highly lethal association. The longest survivor in the literature is 10 months. We describe two neonates with this association who underwent primary repair shortly after birth and who are alive and well at 24 and 18 months. One of these neonates also demonstrated duodenal atresia. The prime goal in the management of these patients is early protection and preservation of respiratory units. This goal is best accomplished by primary repair of the tracheoesophageal lesion, when feasible, rather than by more complex procedures.

Colonic atresia combined with Hirschsprung's disease: a diagnostic and therapeutic challenge.

Three new cases of patients with co-occurrence of colonic atresia and Hirschsprung's disease and a review of the literature (n = 5) are presented in this report. All patients (n = 8) except one were full-term infants who had no other significant anomalies. The preterm infant had associated tetralogy of Fallot in addition to Hirschsprung's disease and colonic atresia. Six patients had atresia of the ascending colon, and two had atresia of the colon to splenic flexure. All colonic atresias were diagnosed neonatally; however, there was mean delay of 15.4 months (range, 1 mo to 5 years) in diagnosing associated Hirschsprung's disease. There were two deaths. A careful examination of the resected specimen to rule out Hirschsprung's disease is recommended. Performing a rectal biopsy must be considered for patients who initially were treated for colonic atresia and who have a slow return of normal gut function.

Prevention of biliary cirrhosis following hepatic arterial thrombosis after liver transplantation in children by using ursodeoxycholic acid.

Hepatic artery thrombosis (HAT) after liver transplantation is a severe complication that often requires retransplantation. The authors have adopted a different approach, aimed at treating the perioperative HAT complications aggressively and early, with ursodeoxycholic acid (UDCA), to try to preserve the original graft. Eighty-six liver transplants were performed in 73 children (age range, 4.5 months to 17.5 years; median, 2.6 years). HAT occurred eight times, in seven patients (9.3%). Patients with HAT were significantly younger and smaller (mean age, 0.8 +/- 0.4 v 4.8 +/- 5.3 years; P < .02; mean weight, 7.4 +/- 0.8 v 18.7 +/- 16.2 kg; P < .05). The incidence of HAT varied significantly according to the method of arterial reconstruction used: 4 of 16 (25%) when a donor iliac artery interposition graft to the aorta was used, 4 of 61 (6.6%) when the native hepatic artery was used, and 0 of 9 when the donor celiac axis was anastomosed directly to the aorta (P < .05). The incidence of HAT was not significantly different when reduced-size grafts were used. Early retransplantation was performed in three of the eight patients; two survived. All other patients were treated for 4 to 6 weeks with broad-spectrum antibiotics and amphotericin. Five patients were treated with UDCA, three immediately after the acute event and two after 4 and 6 months (respectively) post-HAT. The patients who had UDCA immediately post-HAT had histologically normal liver biopsy specimens. Results of liver function tests have been normal. One of these patients required transhepatic stenting of a common bile duct stricture for several months.(ABSTRACT TRUNCATED AT 250 WORDS)

Survival after reduced-size liver transplantation is dependent on pretransplant status.

Orthotopic liver transplantation (OLT) in children is characterized by unique problems including a shortage of compatible-size donors resulting in long waiting periods, significant deterioration while waiting, and death before transplantation. To improve the chances of obtaining an organ for the sickest patients, reduced-size liver transplantation (RSLT) was offered to all hospital-bound children starting in July 1988. Since then, 68 OLTs were performed in 58 children. Thirty-six RSLTs were done in 30 children (42% of total 86 OLT, 53% since 1988). The mean weight of the RSLT patients was 13.5 +/- 10.4 kg versus 23.8 +/- 21.9 kg in the full-size (FSLT) group (P < .05). Twenty-five of 39 transplants (71.4%) done in children < 10 kg were RSLTs in comparison to only 10 of 47 (21.3%) in patients > 10 kg (P < .0005). Since 1988, 25 of 34 (73.5%) of all transplants in children < 10 kg have been RSLTs. Average donor to recipient weight ratio in the RSLT group was 4.21:1 versus 1.17:1 in the FSLT group (P < .0001). RSLT was done as a primary procedure in 26 patients and as a retransplant in 10. Mean blood product replacement was significantly higher in the RSLT group both intraoperatively (515.7 +/- 490.9 v 177.2 +/- 278.3 mL/kg, P < .005) and during the first 24 hours postoperation (50.5 +/- 81.8 mL/kg v 16.4 +/- 28.5 mL/kg, P < .05).(ABSTRACT TRUNCATED AT 250 WORDS)