Liver transplantation in an adult with adenomatosis and congenital absence of the portal vein: a case report.

Congenital absence of the portal vein (CAPV) is a rare congenital anomaly in which the superior mesenteric veins (SMV) and splenic veins converge and bypass the liver, effectively draining directly into the systemic venous circulation via the inferior vena cava (IVC), or alternatively the renal or iliac vein, creating a native portosystemic shunt. Portosystemic shunting results in clinical manifestations of hepatic encephalopathy as well as a predisposition to focal nodular hyperplasia and tumors, including adenomas, hepatoblastoma, and hepatocellular carcinoma (HCC), by the disruption of enterohepatic blood flow. Historically, CAPV has been thought to be a rare condition found mainly at autopsy, however, in recent years due to advances in radiological techniques, CAPV detection has increased. Herein we describe a patient with known CAPV who initially underwent hepatic resection for HCC. During surveillance, additional masses were discovered and were identified as recurrent HCC. Unfortunately, this patient was not a candidate for further resection or locoregional therapy. We demonstrate that transplantation is a challenging but technically viable option for treatment of HCC complicating adenomatosis-associated CAPV.

Genetic diversity and antibiotic resistance in Shigella dysenteriae and Shigella boydii strains isolated from children aged <5 years in Egypt.

Diversity within Shigella dysenteriae (n=40) and Shigella boydii (n=30) isolates from children living in Egypt aged <5 years was investigated. Shigella-associated diarrhoea occurred mainly in summer months and in children aged <3 years, it commonly presented with vomiting and fever. Serotypes 7 (30%), 2 (28%), and 3 (23%) accounted for most of S. dysenteriae isolates; 50% of S. boydii isolates were serotype 2. S. dysenteriae and S. boydii isolates were often resistant to ampicillin, chloramphenicol and tetracycline (42%, 17%, respectively), although resistance varied among serotypes. Pulsed-field gel electrophoresis separated the isolates into distinct clusters correlating with species and serotype. Genetic differences in trimethoprim/sulfamethoxazole and ß-lactam-encoding resistance genes were also evident. S. dysenteriae and S. boydii are genetically diverse pathogens in Egypt; the high level of multidrug resistance associated with both pathogens and resistance to the most available inexpensive antibiotics underlines the importance of continuing surveillance.

Feasibility of upfront dose-intensive chemotherapy in children with advanced-stage Hodgkin's lymphoma: preliminary results from the Children's Cancer Group Study CCG-59704.

BACKGROUND: Treatment strategies involving dose intensification have recently demonstrated improvements in cure compared with older trials. However, dose-intensive therapy is associated with increased acute and long-term toxicities, particularly in pediatric patients. The Children's Cancer Group initiated this pilot study to assess the feasibility and toxicity of a moderate dose-intensive regimen, BEACOPP (bleomycin, etoposide, doxorubicin, cyclophosphamide, vincristine, procarbazine and prednisone), in children and adolescents with advanced-stage Hodgkin's lymphoma (HL). PATIENTS AND METHODS: Children with stage IIB or IIIB with bulk disease, or stage IV were eligible. Induction consisted of four cycles of escalated dose BEACOPP. The rapidity of response, defined as >70% reduction in disease burden, was assessed after two and four cycles. Rapid responders then received consolidation therapy as per gender-specific guidelines to reduce the risk of gender-specific long-term toxicities of therapy, i.e. females received four cycles of COPP/ABV (cyclophosphamide, vincristine, procarbazine, prednisone, doxorubicin, bleomycin and vinblastine) without radiation therapy and males received two cycles of ABVD (doxurubicin, bleomycin, vinblastine and dacarbazine) with involved field radiation therapy (IFRT). Slow responders received four cycles of BEACOPP and IFRT. RESULTS: Ninety-nine patients were enrolled. Myelosuppression was frequent. Non-hematological grade 4 toxicities included allergic reaction (two patients), hypotension (one), mucositis (four), infection (three), seizure (one) and elevated transaminases (one). Typhlitis developed in four patients; three recovered and completed dose-modified chemotherapy, while one died of sepsis associated with grade 4 neutropenia. A rapid response was achieved by 45 and 72% of patients after two and four cycles, respectively. There are no disease progressions or secondary malignancies to date. There is only one reported relapse to date. Median follow-up for the cohort is 6 months. CONCLUSIONS: BEACOPP chemotherapy is feasible and generally well tolerated in children with advanced-stage HL. The absence of reported progressive disease and only one relapse to date is encouraging.

Use of unconventional therapies by children with cancer at an urban medical center.

PURPOSE: The aim of this study was to determine the prevalence, modalities, and determinants of use of unconventional therapies among children with cancer receiving conventional treatment at an urban academic medical center in the United States. PATIENTS AND METHODS: We interviewed the parents of patients and/or patients who were receiving or had received conventional therapy for treatment of childhood cancer. Of 78 patients/ parents asked, 75 consented to the interview, which included demographic factors, life events, and use of unconventional therapies. All participants also consented to the abstraction of chart data for this study. RESULTS: Overall, 84% of respondents reported the use of one or more unconventional therapies. The most commonly used modalities were changes in diet, nutritional and herbal agents, and mind/body treatments. Most users had tried more than one unconventional modality. No difference in use was seen by cancer diagnosis, race/ethnicity, socio-economic status, or educational attainment of the respondent. Of the therapies used, 50% were not reported to the physicians. Of patients reporting use of an unconventional approach, 85% were concurrently enrolled on clinical trials for primary treatment of their cancer. CONCLUSIONS: The use of unconventional therapies is highly prevalent among children with cancer and is not associated with demographic or clinical factors or participation in clinical trials. The possibility that an unconventional treatment may interact with a protocol treatment underscores the need for more information about the use of such therapies among all patients.

Transjugular intrahepatic portosystemic shunt for recurrent hepatic hydrothorax.

For many years, pleural effusions have been recognized as a complication of cirrhosis, occurring in approximately 5.5% of patients. Recent studies have confirmed that small defects in the diaphragm allow for passage of ascitic fluid into the pleural space. Successful management of these patients is challenging, as many of the treatment options can be associated with increased morbidity. The initial treatment should focus on eliminating and preventing the recurrence of ascites with diuretics and water and salt restriction. For those patients who do not respond medically, more invasive techniques have been used including serial thoracentesis, chest tube placement, chemical pleurodesis, and peritoneovenous shunts. We present a patient with recurrent pleural effusions secondary to hepatic cirrhosis who was unsuccessfully treated medically, and subsequently treated with thoracentesis, chest tube drainage and pleurodesis, with ultimate resolution after transjugular intrahepatic portosystemic shunt placement.

Basimeningeal/spinal axis primitive neuroectodermal tumor without intraaxial lesion: radiological features.

Over a 12-year period, the authors encountered 2 patients with spinal axis/basimeningeal primitive neuroectodermal tumor (PNET) involving the spinal axis extramedullary, intradural compartment and the basimeninges. In neither patient was an intraaxial focus demonstrated. A 48-year-old woman had computed tomography (CT) findings of hydrocephalus and later abnormal basimeningeal enhancement. Cervical myelography revealed a low cervical intradural block. A 10-year-old boy was evaluated initially with non-contrast-enhanced CT of the brain, which revealed hydrocephalus. Contrast-enhanced CT revealed abnormal basimeningeal enhancement implicating cisternal obstruction. Subsequent magnetic resonance examination of the entire spine with and without gadolinium enhancement revealed diffuse abnormal enhancement of the spinal axis intradural, extramedullary compartment. While intraaxial PNETs are well recognized, the patients described here represent a distinctly unusual pattern of intradural spinal meningeal and basimeningeal infiltration by PNET in the absence of a demonstrable intraaxial lesion.

Randomized study of intensive MOPP-ABVD with or without low-dose total-nodal radiation therapy in the treatment of stages IIB, IIIA2, IIIB, and IV Hodgkin's disease in pediatric patients: a Pediatric Oncology Group study.

PURPOSE: To determine whether the addition of low-dose total-nodal irradiation (TNI) in pediatric patients with advanced-stage Hodgkin's disease who have received eight cycles of alternating mechlorethamine, vincristine, procarbazine, and prednisone (MOPP) and doxorubicin, bleomycin, vinblastine, and dacarbazine (ABVD) will improve the event-free survival (EFS) and overall survival (OS) when compared with patients who have received chemotherapy only. PATIENTS AND METHODS: At diagnosis, 183 children and adolescents with stages IIB, IIIA2, IIIB, and IV Hodgkin's disease were randomized to receive eight cycles of alternating MOPP-ABVD with or without low-dose TNI. RESULTS: Of 183 patients, four were rendered ineligible before treatment was initiated. One hundred sixty-one of 179 patients (90%) were in complete remission (CR) at the completion of eight cycles of alternating MOPP-ABVD; 81 were in the chemotherapy-only group and proceeded to observation off therapy, whereas 80 of 161 were to receive combined modality therapy (CMT). Nine of 80 patients randomized at the time of diagnosis to receive CMT did not receive radiation (RT) because of a protocol violation, but were monitored for EFS and OS and included in all analyses. The estimated EFS and OS rates at 5 years for the 179 eligible patients are 79% and 92%, respectively. The actuarial EFS at 5 years was 80% for patients who received CMT and 79% for patients who received MOPP-ABVD only. The OS for the former group is estimated to be 87% and for the latter patients 96%. Age < or = 13 years of age at diagnosis and the attainment of a clinical CR after three cycles of chemotherapy were associated with a statistically significant improved EFS. CONCLUSION: Our results indicate that after the delivery of eight cycles of MOPP-ABVD, the addition of low-dose RT does not improve the estimated EFS or OS in pediatric patients with advanced-stage Hodgkin's disease.

Chronic active mediastinitis in a 7-year-old boy: MRI findings.

A 7-year-old child with a right mediastinal mass was found to have chronic active mediastinitis. The mass spontaneously involuted after biopsy. MRI showed the infiltrative nature of the mass.

Laminin stimulates expression of two mitochondrial proteins during neurite outgrowth.

Differential hybridization was utilized with mRNA from NG108-15 cells cultured on either tissue culture plastic or laminin for 4 hr to identify genes whose mRNA was increased by laminin, a potent stimulator of neurite outgrowth. Two of the 16 laminin-induced clones, cytochrome b and chargerin II, are mitochondrial proteins. Northern blotting confirmed that laminin increased the mRNA levels of cytochrome b and chargerin II several fold. Antibody to chargerin II stained both processes and cell bodies of the cerebellar Purkinje cells and localized in the mitochondria of NG108-15 cells, which also showed increased protein levels in the presence of laminin. In addition, higher levels of chargerin II protein were detected in the newborn brain compared to the adult. However, inhibitors of mitochondrial protein synthesis did not affect laminin-mediated neurite outgrowth. These data suggest that the increased synthesis of mitochondrial enzymes observed with laminin treatment may not be necessary for the formation of neurites.

Severe osteoporosis before and after lung transplantation.

STUDY OBJECTIVE: Osteoporosis and/or fractures have been reported as a complication of kidney, heart, liver, bone marrow, and, most recently, lung transplantation (LTx). We measured bone mineral density (BMD) before and after LTx to determine the frequency and severity of preoperative and postoperative osteoporosis. PATIENTS AND METHODS: We conducted a cross-sectional study of BMD in 100 patients with various diagnoses before (n = 55) and after (n = 45) LTx. RESULTS: Age-matched mean spine and femoral BMDs were severely depressed before and after LTx placing 45% of the pre-LTx and 73% of the post-LTx patients at or below the fracture threshold (2 SDs below the mean). As expected, patients with cystic fibrosis had lower (p < 0.05) mean spine and femoral BMD than patients with COPD or other lung diseases both before and after LTx. Nevertheless, considerable osteoporosis was present in the patients with COPD and other lung diseases before and after LTx. The best predictors of pre- and post-LTxZ scores were body mass index (r = 0.56 to 0.59, p < 0.001) and cumulative steroid dose/per kilogram (r = 0.49-0.51, p < 0.001), respectively. Osteoporosis has resulted in 3 symptomatic fractures before and 12 after LTx. CONCLUSIONS: Osteoporosis appears to be widespread in patients with end-stage respiratory failure before LTx and LTx, while often life-saving, has an adverse impact on BMD. As patients live longer after LTx, osteoporosis-related fractures may compromise quality of life by causing pain and debilitation.

Vesiculated erythrocytes as a determination of splenic reticuloendothelial function in pediatric patients with Hodgkin's disease.

PURPOSE: To determine the effect of low-dose total, or subtotal, nodal irradiation (TNI/sub-TNI) on splenic reticuloendothelial function in pediatric patients with Hodgkin's Disease (HD). PATIENTS AND METHODS: Pediatric and adolescent patients with advanced stage HD were accrued from two Pediatric Oncology Group studies and subdivided into three groups: the first had chemotherapy (CT) only; the second received chemotherapy and low-dose (2,100 cGy) TNI or subtotal TNI (sub-TNI); the third underwent staging laparotomy with splenectomy followed by CT, with or without low-dose radiotherapy. Vesiculated erythrocyte counts (VRBC) were performed on all patients using Nomarski interference phase optics at the conclusion of therapy. RESULTS: The mean VRBCs were 3.2%, and 3.8% for the non-splenectomized patients who received chemotherapy only, and chemotherapy plus low-dose splenic irradiation, respectively. For those who underwent splenectomy before chemotherapy, the VRBC was 36.7%. Statistical analysis revealed no difference in vesiculated erythrocyte percentages between the CT only group and the CT + TNI/sub-TNI patients; however, there was a significant difference between both of these groups and the splenectomized patients. CONCLUSIONS: Our results indicate that the addition of low-dose splenic irradiation to chemotherapy in children and adolescents with advanced-stage Hodgkin's disease does not adversely affect splenic reticuloendothial function.

Improved results of lung transplantation for patients with cystic fibrosis.

Patients with cystic fibrosis pose particular challenges for lung transplant surgeons. Earlier reports from North American centers suggested that patients with cystic fibrosis were at greater risk for heart-lung or isolated lung transplantation than other patients with end-stage pulmonary disease. During a 3 1/2 year period, 44 patients with end-stage lung disease resulting from cystic fibrosis underwent double lung transplantation at this institution. During the same interval, 18 patients with cystic fibrosis died while waiting for lung transplantation. The ages of the recipients ranged from 8 to 45 years, and mean forced expiratory volume in 1 second was 21% predicted. Seven patients had Pseudomonas cepacia bacteria before transplantation. Bilateral sequential implantation with omentopexy was used in all patients. There were no operative deaths, although two patients required urgent retransplantation because of graft failure. Cardiopulmonary bypass was necessary in six procedures in five patients and was associated with an increased blood transfusion requirement, longer postoperative ventilation, and longer hospital stay. Actuarial survival was 85% at 1 year and 67% at 2 years. Infection was the most common cause of death within 6 months of transplantation (Pseudomonas cepacia pneumonia was the cause of death in two patients), and bronchiolitis obliterans was the most common cause of death after 6 months. Actuarial freedom from development of clinically significant bronchiolitis obliterans was 59% at 2 years. Results of pulmonary function tests improved substantially in survivors, with forced expiratory volume in 1 second averaging 78% predicted 2 years after transplantation. Double lung transplantation can be accomplished with acceptable morbidity and mortality in patients with cystic fibrosis.

With modern imaging techniques, is staging laparotomy necessary in pediatric Hodgkin's disease? A Pediatric Oncology Group study.

PURPOSE: To determine whether the information gained from staging laparotomy can be predicted by imaging and/or clinical factors in children with Hodgkin's disease. PATIENTS AND METHODS: Between 1986 and 1991, 216 consecutive pediatric patients with Hodgkin's disease underwent laparotomy and were treated on two concurrent protocols in a multiinstitutional cooperative group. All patients had computed tomography (CT) of the chest, abdomen, and pelvis. Clinical factors studied included sedimentation rate, B symptoms, histology, number and location of involved sites, sex, mediastinal involvement, and age. Pretreatment CTs were centrally reviewed in 88 cases for the presence and size of both supradiaphragmatic and infradiaphragmatic lymph nodes, intrinsic spleen lesions, and splenic size. Models were generated that were predictive of any abdominal disease, splenic involvement, extensive splenic involvement, and upstaging at the laparotomy. False-positive and false-negative rates were calculated. RESULTS: For the end point of any abdominal disease, a model based on B symptoms, histology, sedimentation rate, and number and location of involved sites was highly significant (P < .0001). However, the success in predicting abdominal disease in an individual patient was limited: false-negative rate, 26%; false-positive rate, 32%. Highly significant models based on clinical factors and/or radiographic findings were also generated to predict splenic involvement, extensive splenic involvement, and upstaging with laparotomy, but they also had high false-positive and false-negative rates. CONCLUSION: Laparotomy findings cannot be predicted accurately in the majority of patients based on knowledge of CT findings and clinical factors.

Intensive chemotherapy and low-dose radiotherapy for the treatment of advanced-stage Hodgkin's disease in pediatric patients: a Pediatric Oncology Group study.

Sixty-two patients with advanced-stage Hodgkin's disease and a median age of 12 years (range, 3 to 22 years) were treated with four cycles of mechlorethamine, vincristine, procarbazine, and prednisone (MOPP) alternating with four cycles of doxorubicin, vinblastine, bleomycin, and dacarbazine (ABVD) followed by low-dose radiotherapy (RT). We determined the feasibility, immediate safety, and rapidity of response of patients to this regimen, as well as the relationship between prognostic factors and the rate of complete remission (CR), event-free survival (EFS), and overall survival. Therapy was well tolerated, and the major toxicity was hematopoietic. At the end of chemotherapy, 54 of 62 patients (87%) were in CR by clinical restaging, with a biopsy of residual disease where necessary. The actuarial 3-year EFS is 77% (SE, 11%), with a median follow-up of 35 months, and the survival is 91% (SE, 7%). With respect to EFS, female patients and those with stage II or III disease fared statistically better than males and patients with stage IV disease, respectively. Six patients have died: three of progressive Hodgkin's disease, one of secondary acute myelocytic leukemia (AML), one of secondary non-Hodgkin's lymphoma (NHL), and one of overwhelming bacterial sepsis. The Pediatric Oncology Group (POG) is currently engaged in a randomized study of these eight cycles of chemotherapy with and without RT to assess the role of RT in achieving comparable results.

Erythrocyte characteristics in childhood acute leukemia.

Children with acute leukemia often have erythrocytes with "fetal-like" features. To examine the relationship of the type and phase of the leukemia to this observation, we studied 39 children with newly diagnosed acute lymphocytic leukemia (ALL) and 5 with acute nonlymphocytic leukemia (ANLL). In addition, 22 patients were evaluated during chemotherapy, 3 off therapy, and 12 at the time of relapse. Macrocytosis and/or anisocytosis was noted in 70% of patients with ALL at the time of first diagnosis, 80% of patients with new ANLL, and greater than 90% of patients with ALL while on treatment. F cells were increased in 25% of ALL and 80% of ANLL at diagnosis and in 60% of ALL during chemotherapy. Hb F levels were elevated in 8, 40, and 30% of these groups, respectively. Nonleukemic controls for chemotherapy (six patients with osteogenic sarcoma) all had macrocytosis and/or anisocytosis, and 80% had increased F cell proportions. Features at relapse on chemotherapy were similar to those during treated remissions. Abnormal RBCs are more frequent at the time of diagnosis in ANLL, in which they may belong to the malignant clone, than in ALL, in which stress erythropoiesis and/or leukemic factors may be contributory. During chemotherapy, drug-related erythrocyte changes are added to those of leukemia itself. Thus, leukemia, chemotherapy, and the combination lead to erythrocytes with fetal-like features.

Thrombocytopenia in a bisexual adolescent male with a history of intravenous drug use.

Immunologically mediated thrombocytopenia has been reported as a manifestation of human T-lymphotropic virus type III (HTLV-III) infection and intravenous (IV) drug abuse. The case report describes thrombocytopenia in a bisexual adolescent male with antibody to the HTLV-III virus and a history of infrequent IV drug abuse. The case underscores the need to consider HTLV-III infection and/or IV drug abuse in the differential diagnosis of thrombocytopenia in an adolescent.