Search

Congenital agenesis of the right liver (ARL) is a rare condition that is generally asymptomatic. Congenital anomalies of the liver are generally diagnosed with current cross-sectional imaging modalities such as ultrasound, computed tomography, and magnetic resonance imaging (MRI) before surgery. However, discovery of a congenital anomaly of the liver only at time of surgery remains a possibility. Herein reported is the case of a 68-year-old woman undergoing laparoscopic cholecystectomy for symptomatic gallbladder stones as diagnosed on preoperative ultrasounds. Upon laparoscopic exploration of the upper abdomen, the right liver was not found; the gallbladder was located in the right subdiaphragmatic region posterior to the medial segment of the liver. A posterolateral interposition of the hepatic flexure of the colon was also found. Cholecystectomy was completed under laparoscopy. A postoperative MRI confirmed right liver agenesis. We discuss the technical difficulties of performing a laparoscopic cholecystectomy in the case of ARL and the advantages of a laparoscopic approach.

Aneurysms of pancreaticoduodenal arteries represent only 2% of all aneurysms of digestive arteries. Occurrence of these aneurysms are favoured by stenosis or occlusion of the celiac axis. Aneurysm rupture is frequent and carries a mortality rate of 20%. Computed tomography with intravenous contrast and selective coeliomesenteric arteriography can make the diagnosis of this disease which can be treated by either surgery or embolotherapy. We report the case of a patient with a ruptured aneurysm of the pancreaticoduodenal arcades mimicking a perforated duodenal ulcer, and successfully treated by surgical ligation.

Background: Cancer is the second most important cause of death for children aged less than 15 years in France, unintentional injuries being the leading cause. The aim of the present study was to estimate the incidence of childhood cancer from six Childhood Cancer Registries covering 32% of France., Procedure: Incident cancer cases diagnosed between 1990 and 1999 in children (0-14 years) resident in the administrative areas covered by each Registry were included. Annual age-standardized rates (ASRs) were adjusted by the world population. The estimated annual percent change (EAPC) was used to measure trend towards changes in the annual age-standardized incidence rate., Results: With 4234 registered cases, the ASRs per million children were 137.5 for all cancers combined, 42.3 for leukemia, 29.1 for central-nervous-system tumors, 15.6 for lymphomas, 14.1 for sympathetic-nervous-system tumors, and 9.1 for renal tumors. The ASR of all cancers combined was slightly higher in males (145.8 per million children) than in females (128.7 per million children) with an M/F ratio of 1.2. No significant incidence trend was observed, with an EAPC of +0.2% [IC 95% (-2.5; +3.0); P = 0.89]., Conclusions: The estimated incidence rates are similar to those reported in previous studies in European and North American countries. These results will contribute to the development of National Registration of Childhood Cancer in France and support the national research program on childhood cancer., (2004 Wiley-Liss, Inc.)

The presacral venous plexus results from anastomoses between the lateral and median sacral veins, and courses into the pelvic fascia covering the anterior aspect of the body of the sacrum. The presacral venous plexus is not directly visible during rectal surgery, and injuries to this plexus may be life-threatening. Dissection of the retrorectal plane or anchoring of the rectum to the sacral promontory as in rectal prolapse surgery exposes the patient to the risk of injury to the presacral venous plexus. The aim of this study was to identify some avascular areas in the anterior aspect of the sacrum in order to lower the occurrence of such injuries during rectal surgery. The pelvis of 10 fresh cadavers was dissected after injection of a colored resin into the inferior vena cava, and the presacral venous plexus was studied. Four avascular tetragonal areas were common to all the specimens. The corners of a square with a side of 3 cm, centered on the anterior aspect of the body of sacrum, were always contained in the avascular areas. The upper side of this square was parallel to a line passing through the sacral promontory, at a 3 cm distance from it. Staples or sutures should be placed in the avascular areas to avoid injuries to the presacral venous plexus.

Background: Rectal tumors are rarely metastatic. Moreover, hematogenous spread is rare in ovarian cancer whose dissemination frequently occurs through peritoneal or lymphatic ways., Case: A 55-year-old female presented with a rectal metastasis that appeared 20 years after the treatment of a primary clear cell carcinoma of the ovary. The cytokeratin 7 positive/cytokeratin 20 negative immunophenotype assessed the ovarian origin of the rectal tumor. Because of the integrity of the rectal serosa and the uninvolved mesenteric lymph nodes, we hypothesize our rectal metastasis to come from the hematogenous way., Conclusion: This is the first reported case of hematogenous rectal metastasis in epithelial ovarian carcinoma and that, with so late delay.

Unlabelled: Self-expanding metallic stents is an alternative treatment to colostomy that is the treatment of choice in acute tumoral left colonic obstruction., Aim of the Study: To compare morbidity, mortality, length of hospital stay and treatment performed after desobstruction using the two methods., Patients and Methods: Thirty-three patients admitted for acute obstruction of the left colon were retrospectively separated in two groups depending on the type of intervention performed to treat the obstruction ("colostomy" group: 17 patients and "self-expanding stent group": 16 patients). We studied complications after desobstruction, hospital courses and surgical strategy performed after the acute phase., Results: Time between desobstruction and colectomy was shorter in the "self-expanding stent group" than in the "colostomy group" (18.5 days versus 73 days). Age superior than 75 years and colostomy were the two main factors predicting the risk of definitive colostomy (P < 0.05). Global mean hospital stay was longer in the colostomy group (32.7 days versus 19.3 days, P = 0.02). Two perforations and one local recurrence occurred in the "self-expanding stent group"., Conclusions: Self-expanding metallic stent can decrease the permanent colostomy rate and the number of interventions. The recurrence rate seems to be theoretically increased with the stenting method. Then, colostomy must be done for patients in curative situation. The self-expanding metallic stent should be used as a palliative care.

Introduction: Hafnia alvei is a commensal organism of the digestive tract. It is best known as an agent of pediatric gastro-enteritis. In adults, this organism may exceptionally be responsible for extra-intestinal infections. In such cases, infections occur mainly in unusually susceptible patients, due to an underlying condition., Case Report: A 69 year-old man who had recently undergone surgery for a gastric stromal tumor was admitted to hospital because of H. alvei bacteremia accompanied by liver and right iliac muscle abscesses. The course was favourable with a 60 day treatment of fluoroquinolone., Commentary: H. alvei is usually considered as moderately or non pathogenic for humans. However, recent descriptions of severe community or nosocomial infections due to H. alvei have challenged this belief. The actual role of H. alvei in human disease remains to be defined.

The Lyon-Marseille-Curie-Est (LMCE) of France cooperative group has previously reported successive series of unselected stage four children older than 1 year at diagnosis with metastatic neuroblastoma (LMCE 1 and 3). The goal of LMCE 5 study was to increase progression free survival rate as compared to LMCE 1 and 3. Based on improvements reported with post induction chemotherapy, the LMCE 5 used post induction for all children, but omitted total body irradiation and immunomagnetic purging in megatherapy regimen for all children. Twenty-five sequentially diagnosed children received an induction regimen which compared with previous induction included an increased dose of etoposide and cyclophosphamide, delivered similar dose of cisplatinum, and deleted doxorubicin and vincristin. After surgery treatment was stratified based on response and eligible children received etoposide carboplatin (LMCE 5A : n=10)+/-doxorubicin (LMCE 5B-C n=13) followed by megatherapy (melphalan without total body irradiation and unpurged peripheral blood stem cell rescue). The increase in drug doses during induction did not improve remission rate. The progression free survival at 6 years is 8%. It is significantly worse than LMCE 3, and equivalent to LMCE 1 study though toxic death rate has decreased with increasing experience. Failure to improve the response rate during induction and reducing the megatherapy regimen may be the main factors in this disappointing result. Modified strategies for induction, non toxic alternative to total body irradiation, and post megatherapy regimen should be developed.

Purpose: To prospectively evaluate fluoroscopic-assisted insertion of self-expanding metallic stents before surgery or for palliative treatment of soft tissue colorectal obstruction. Materials and methods. From January 1999 to October 2000, 18 consecutive patients with colorectal obstruction were included in the study. Treatment with self-expanding metallic stent was either the first line of treatment before surgery (n=8) (group I) or purely palliative (n=10) (group II). Colic stenosis was located proximal to the sigmoid in seven cases., Results: Technical success was achieved in 83.3% of cases and colic decompression was observed after 48 hours in all patients with a stent. Thirty days mortality and stent related complications were respectively 0% and 37.5% for group I, and 20% and 50% for group II. All complications were minor except for one colic perforation by a guidewire., Conclusion: Stent insertion was effective and provided relief of colic obstruction in the majority of cases. Randomized studies would be necessary to demonstrate a definitive reduction in mortality and morbidity with this technique as compared to the classical surgical approach.

The interest in the topic of pain in children is increasing within the paediatric community after a long period of misrecognition, often under-valuation, sometimes negligent or even denial. A lack of knowledge concerning pharmacology and toxicity of antalgic drugs and poorly adapted galenic presentations induced delay and complications in their use in children. The recent progress is supported by a better semiology, development of adequate pain scales, availability of adapted drugs, an effort at education of caregivers and public sensitization. In primary care the objective is to lead the practitioner to identify and consider pain in his/her preventive and curative strategies. In hospitals efforts are needed to improve the use of protocols and evaluations, the systematic practice of preventive analgesia and the quality of interprofessional cooperation within care teams. Managed care organizations are today an effective system to promote these practices.

The authors report the MR features of a non functioning and hemorragic islet-cell tumor of the pancreas. This tumor was composed of a central cystic component with a fluid-fluid level seen on T1- and T2-weighted images and a peripheral hypervascular soft tissue component which showed hyperintensity on T2-weighted images with fat saturation.

RGS proteins negatively regulate heterotrimeric G proteins at the plasma membrane. RGS2-GFP localizes to the nucleus, plasma membrane, and cytoplasm of HEK293 cells. Expression of activated G(q) increased RGS2 association with the plasma membrane and decreased accumulation in the nucleus, suggesting that signal-induced redistribution may regulate RGS2 function. Thus, we identified and characterized a conserved N-terminal domain in RGS2 that is necessary and sufficient for plasma membrane localization. Mutational and biophysical analyses indicated that this domain is an amphipathic alpha-helix that binds vesicles containing acidic phospholipids. However, the plasma membrane targeting function of the amphipathic helical domain did not appear to be essential for RGS2 to attenuate signaling by activated G(q). Nevertheless, truncation mutants indicated that the N terminus is essential, potentially serving as a scaffold that binds receptors, signaling proteins, or nuclear components. Indeed, the RGS2 N terminus directs nuclear accumulation of GFP. Although RGS2 possesses a nuclear targeting motif, it lacks a nuclear import signal and enters the nucleus by passive diffusion. Nuclear accumulation of RGS2 does not limit its ability to attenuate G(q) signaling, because excluding RGS2 from the nucleus was without effect. RGS2 may nonetheless regulate signaling or other processes in the nucleus.

The authors report three cases of endometriosis of the rectus abdominis muscle, presenting as a mass of the abdominal wall associated with pain during menstruation in women with a history of cesarean section. Treatment consisted in wide surgical resection followed by prosthetic abdominal wall repair in one recurrent case. Abdominal wall endometriosis is a rare disease that can be explained by grafting of endometrial cells to the abdominal wall during laparotomy for pelvic surgery, particularly cesarean section. Isolation of the abdominal wall during cesarean section and irrigation of the abdominal wall at the end of the operation are two theoretical measures designed to prevent endometrial cell engraftment. When abdominal wall endometriosis occurs, only radical surgical resection can prevent recurrence.

The implication of environmental carcinogens in childhood cancer is still unknown. To assess a possible link between DNA damage and alterations of the tumor suppressor gene p53, blood samples of 21 children with malignancies were examined for the presence of micronuclei in lymphocytes using the cytokinesis blocked micronucleus assay (CBMA). The constitutional karyotypes were analyzed for chromosome abnormalities and the presence of anti-p53 antibodies in blood sera was evaluated by an enzyme-linked immuno sorbent assay (ELISA). A control group of 20 children was also included. The rates of micronucleated cells were 5.1 per thousand+/-3.9 and 2.4 per thousand+/-2.3 for the cancer and control groups, respectively. The difference between the groups were statistically significant (P<0.05 by the Mann-Withney rank sum test). Two children in the cancer group showed extensive chromosome breakage in lymphocytes. The sera of two other children from the cancer group and of one child from the control group contained anti-p53 antibodies. Chromosome breakage and anti-p53 antibodies from the five children were associated with increased micronucleated cell rates. The results of the present study suggest that genotoxic events can occur in the lymphocytes of children with a cancerous state.

Purpose: To tailor postinduction therapy for stage 4 neuroblastoma in children who are older than 1 year at diagnosis according to status after induction., Patients and Methods: From March 1987 to December 1992, 99 patients who were consecutively admitted were included in the Lyon-Marseille-Curie East of France (LMCE)3 strategy. After induction with the French Society of Pediatric Oncology NB87 regimen and surgery, patients who were in complete remission immediately proceeded to consolidation therapy with vincristine, melphalan, and fractionated total-body irradiation (VMT). All other patients underwent a postinduction strategy before VMT, either an additional megatherapy regimen or further chemotherapy with etoposide/carboplatin., Results: The progression-free survival (PFS) is 29% at 7 years from diagnosis, which compares favorably with that of a similar cohort of 72 patients previously reported by our group (LMCE1; PFS of 20% at 5 years and 8% at 14 years, P =.004). In the multivariate analysis, only age younger than 3 years at diagnosis (P =.0085) and achievement of complete or very good partial remission after NB87 and surgery (P =.00024) remained significant. The PFS of the 87 patients who were included in the postinduction strategy was significantly better than that of the comparable 62 patients on the LMCE1 study (32% v 11% at 7 years; P =.005)., Conclusion: The progressive improvements in the LMCE results over the last 10 years suggest that improvements in supportive care measures and increases in each component of this strategy (induction, postinduction, consolidation) may all contribute to increased survival rates.

RGS (regulator of G protein signaling) proteins are GTPase-activating proteins that attenuate signaling by heterotrimeric G proteins. Whether the biological functions of RGS proteins are governed by quantitative differences in GTPase-activating protein activity toward various classes of Galpha subunits and how G protein selectivity is achieved by differences in RGS protein structure are largely unknown. Here we provide evidence indicating that the function of RGS2 is determined in part by differences in potency toward G(q) versus G(i) family members. RGS2 was 5-fold more potent than RGS4 as an inhibitor of G(q)-stimulated phosphoinositide hydrolysis in vivo. In contrast, RGS4 was 8-fold more potent than RGS2 as an inhibitor of G(i)-mediated signaling. RGS2 mutants were identified that display increased potency toward G(i) family members without affecting potency toward G(q). These mutations and the structure of RGS4-G(i)alpha(1) complexes suggest that RGS2-G(i)alpha interaction is unfavorable in part because of the geometry of the switch I binding pocket of RGS2 and a potential interaction between the alpha8-alpha9 loop of RGS2 and alphaA of G(i) class alpha subunits. The results suggest that the function of RGS2 relative to other RGS family members is governed in part by quantitative differences in activity toward different classes of Galpha subunits.

Background and Study Aims: Endoscopic sphincterotomy results in a continuous flow of bile into the duodenum and consequently leads to an increase in the frequency of enterohepatic bile acid cycling. Because bile acids are the driving force of biliary secretion, sphincterotomy may affect bile genesis. The present study was undertaken to determine the influence of endoscopic sphincterotomy on bile composition., Patients and Methods: The cholesterol saturation index and the bile acid pattern were determined in the gallbladder bile of lithiasis patients with (group III) or without sphincterotomy (group I), and in the hepatic bile of patients with gallbladder in situ who were checked at 3 months after the endoscopic procedure (group II). Stones from each patient were examined for chemical composition and microstructure., Results: All the patients had cholesterol stones. After endoscopic sphincterotomy the molar percentages of cholesterol in the gallbladder bile of group III and in the hepatic bile of group II were significantly lower (-31% and -46% respectively) than in group I. Similarly, the cholesterol saturation index in the hepatic bile (0.79) and the gallbladder bile (0.86) from patients who had undergone sphincterotomy indicated undersaturation whereas bile from group I was oversaturated (1.25). On the other hand, endoscopic sphincterotomy did not modify the hydrophobicity index of the bile acid pool, even though deoxycholate content increased., Conclusions: Endoscopic sphincterotomy causes a marked decrease in the lithogenicity of bile and thus may prevent the risk of recurrence of cholesterol lithiasis.

The authors report 2 cases of congenital mesothelial cyst of the diaphragm diagnosed in adults. The differential diagnosis will be discussed and the literature reviewed.

Study Aim: The aim of this retrospective study was to report a series of 28 patients with adenocarcinoma (ADK) arising in Barrett's esophagus (BE), treated by esophagectomy., Patients and Methods: From 1992 to 1998, 28 patients were operated on for ADK in BE. There were 27 men and one woman (mean age: 65 years) classified as ASA I (n = 2), ASA II (n = 15) and ASA III (n = 11). Eighteen patients had a long story of gastro-esophageal reflux disease which required surgical repair in five of them. Dysphagia was the main symptom (n = 19). Surgical procedures included 15 Ivor Lewis operations, nine esophagectomies without thoracotomy and four esophagogastrectomies by the left thoracic approach., Results: Pathological examination of the specimens showed an EBO with adenocarcinoma (n = 27) and a high grade dysplasia (n = 1). Among five patients with a previously known BE, three under endoscopic surveillance had high grade dysplasia (n = 1) and limited T1 tumor (n = 2), while the other two patients without surveillance developed an invasive tumor (T3N1). There were three postoperative deaths (mortality rate: 10%), all arising from pulmonary failure. Median survival was 16.6 months. All patients resumed a normal diet. The actuarial survival rates were 63%, 42% and 15.2% respectively at 1, 2 and 4 years. A multivariate analysis could identify 3 prognostic factors: ASA score previously known BE under surveillance, length of BE., Conclusion: Adenocarcinoma arising in BE is very often diagnosed too late. Patients with high risk BE require an endoscopic survey. High grade dysplasia detected in two successive examinations by two different pathologists may require prophylactic esophagectomy, but local endoscopic management presently under evaluation could be efficient in the future.

Background: Adequate treatment of pain in children with cancer is a critical issue, and is of equal importance as discussions concerning chemotherapy, surgery and radiotherapy., Objective: To evaluate the treatment of refractory pain by peridural analgesia., Methods: Seven children (1-15 years) with solid tumors were treated with long term epidural analgesia for refractory pain. Catheters were inserted in epidural space (L1-L2) and infused with sufentanil, bupivacaine and clonidine., Results: Three out of five children with good response to peridural therapy could be discharged. A 12-month-old infant had a poor response. Treatment was discontinued in a teenager boy because of patient refusal. The side effects were: early catheter displacement in two patients and a bacterial contamination in one. Serious adverse effects related to high doses of opiates were not observed. However, toxicity of bupivacaine was observed in three patients leading to treatment discontinuation in one., Conclusion: Long-term epidural analgesia looks promising in selected children with refractory pain.

Aim of this study was to analyse prognostic factors of improved survival after resection of colorectal cancer. We studied 715 patients by retrospective review operated for colorectal adenocarcinoma. Survival was analyzed by the Kaplan-Maier method. Comparisons were made by log rank analysis. The overall survival is 75% at 1 years, 41.0% at 5 years, 29.7% at 10 years. A significant difference was noted in the survival rate according to age of the patients (p < 0.01), preoperative serum level of carcinoembryonic antigen (CEA) (p < 0.05), the performance status (p < 0.05), intestinal obstruction (p < 0.01), clinicopathological stage of the tumour (p < 0.05). Other factors including the sex, the clinical diagnosis of anaemia, the site of the tumour and histological grade had no apparent influence on survival. To define high-risk groups of recurrence is important for adjuvant therapy and follow-up study.

Administration of combination chemotherapy to children with metastatic neuroblastoma induces profound myelosuppression resulting in chemotherapy treatment delays and febrile neutropenic episodes. The objective of this randomised multicentre study was to assess the incidence, duration and severity of neutropenia when filgrastim is added to induction chemotherapy administered to patients with metastatic neuroblastoma. In this study, 59 patients with metastatic neuroblastoma were randomised to receive chemotherapy (control group, n = 28) or chemotherapy plus filgrastim (filgrastim group, n = 31). Chemotherapy consisted of four cycles of cyclophosphamide, vincristine and doxorubicin (CADO) alternating at 21-day intervals with cisplatin and etoposide (CDDP-VP16). Filgrastim was administered subcutaneously at a dose of 5 micrograms/kg/day from day 7 for up to 14 days. The incidence of neutropenia (absolute neutrophil count [ANC] < 0.5 x 10(9)/l) in the filgrastim group was not reduced after the first CADO course. However, significant reductions were observed after courses 2, 3 and 4. The duration of neutropenia and of intravenous antibiotic use were significantly reduced in the filgrastim group over the whole study period (9 days versus 26 days, P < 0.001; 12 days versus 20 days, P = 0.04, respectively). However, the duration of hospitalisation and the incidence of febrile neutropenia were not significantly reduced. Compliance to treatment was good and the ability to administer chemotherapy without treatment delays was significantly better in the filgrastim group (P < 0.05). Event-free survival was greater in the filgrastim than in the control group (2.4 years versus 1.3 years; P = 0.072). Filgrastim is a beneficial adjunct to combination induction chemotherapy used in the treatment of metastatic neuroblastoma.

Objectives: The objective of this study was to characterize the clinical picture of Behçet's disease (BD) in children., Study Design: A questionnaire was completed by five BD specialists from Turkey, France, Iran, or Saudi Arabia. We first reviewed 86 cases retrospectively with a specially designed computerized database and then selected 65 who met the criteria of the International Study Group for BD, which include buccal aphthosis plus at least two among recurrent genital aphthosis, eye lesions, skin lesions, and positive pathergy test. The remaining 21 patients, who had features suggestive of BD but did not fulfill the international criteria, were analyzed separately and then compared with the other 65 patients., Results: BD affected boys and girls equally. The clinical picture frequently included mucocutaneous lesions. Uveitis was less frequent than in adults but carried a poor prognosis, especially in male patients (p < 0.001). The mortality rate (3%) was related to large vessel involvement. Familial cases were particularly frequent (15%). Erythema nodosum and skin hypersensitivity were common in Turkish patients, whereas neuro-BD was more frequent in French and Saudi Arabian patients. Patients who did not fulfill the international criteria had significantly less genital aphthosis (p < 0.01), less skin lesions or hypersensitivity (p < 0.01), and less uveitis (p < 0.01)., Conclusion: BD in children is similar to BD in adults. The high frequency of familial cases calls for further investigation of the immunogenetic factors that may favor early expression of the disease.

This paper describes our approach in analyzing the impact of the TOP-FORUM hypermedia in a pediatric oncology care unit. The impact of this technology is realized through the study of accommodation and assimilation adoption. Accommodation refers to the technological adoption and Assimilation refers to the professional adoption. Results show that accommodation depends on information and formation of the users. Assimilation is more difficult to evaluate because it depends on human, social and organizational problems.

Purpose: NB87 was designed to test the efficacy of a short, non cross-resistant, induction protocol for unselected patients over 1 year of age with stage 4 neuroblastoma. A secondary objective was to compare in a randomized study the toxicity of two modalities of cisplatin administration., Patients and Methods: A total of 183 patients received two cycles of alternating sequences: cyclophosphamide 300 mg/m2/d on days 1 to 5, vincristine 1.5 mg/m2/d on days 1 and 5, and doxorubicin 60 mg/m2/d on day 5 (CADO); and cisplatin 40 mg/m2/d and etoposide 100 mg/m2/d on days 1 to 5 (CVP), followed by surgery of the primary tumor (126 patients). Ninety-one were randomized to receive cisplatin either as bolus (BO; n = 48) or continuous infusion (CI; n = 43). International Neuroblastoma Staging System (INSS) and Response Criteria (INRC) were used with emphasis on skeletal evaluation by meta-iodobenzylguanidine (MIBG)., Results: Hematotoxicity was predominant, with a higher incidence of neutropenia (P = .01) for CADO and of thrombocytopenia for CVP (P < .001). Severe infections, as well as nonhematologic toxicities, occurred more often after the first sequence. Gastrointestinal complications were predominant during both courses of CVP. The toxic death rate, including surgery, was 3%. Complete remissions (CRs) were less frequent on MIBG (45%) compared with marrow (66%) or other metastases (61%). Combining all metastatic sites resulted in a 39% CR rate. After surgery, the final CR rate was 42%. Nephrotoxicity was minimal in both arms (92% normal clearance for CI v 82% for BO). Hearing loss greater than 40 dB at 6,000 to 8,000 Hz was reported equally in both arms (n = 6 for CI v n = 5 for BO)., Conclusion: Intensified chemotherapy using CADO/CVP increases CR rates despite a shorter induction duration. However, the rate of MIBG normalization remains unsatisfactory and could be raised through the dose-intensive use of agents such as cyclophosphamide.

The host immune responses have been suggested to play a role in liver injury occurring in patients with chronic hepatitis C. In order to explore the relationship between the relative proportions of intrahepatic and peripheral blood lymphocytes (IHL, PBL), the levels of viremia, and the histological hepatitis activity score, three-color fluorescence-activated cytometric analysis was performed for 36 patients with chronic hepatitis C and six control subjects without chronic hepatitis. The liver biopsy was performed before any antiviral therapy. Each liver specimen was divided into two parts: one for histological examination and one for immunological analysis. Tricolor CD45 was used to improve "lymphogating." Fluorescein isothiocyanate- or phycoerythrin-conjugated monoclonal antibodies with specificity for CD3, CD4, CD8, and CD20 (lymphocyte subpopulations), for CD69 (activated lymphocytes), and for CD16/56 (natural killer cells) were used. The livers of patients with chronic hepatitis C contained a greater proportion of CD4+ lymphocytes that exhibited marked expression of CD69 than in control subjects (20.7 +/- 7.3% vs 10.2 +/- 4.6%, P = 0.027). Moreover, in patients with chronic hepatitis C, the proportion of CD4+ IHL correlated with the histological hepatitis activity evaluated by the Knodell score (r = 0.48, P = 0.004). No correlation was found between the percentage of CD4+ IHL and the level of viremia or transaminase activities. Our findings clearly indicate that a cellular immune response does take place in HCV-infected livers and could thus contribute to the outcome of hepatitis C virus infection.

1070 myeloablative procedures followed by stem cell rescue for neuroblastoma are reviewed. These 1070 procedures are part of the European Group for Blood and Marrow Transplant (EBMTG) registry from the last 17 years (in 4536 patients). In 1070 neuroblastoma patients, survival at 2 years was 49%, at 5 years, 33% and relapses were observed as late as 7 years post-BMT (bone marrow transplant). However, 5-year survivors after megatherapy with BMT for stage 4 disease do have an 80% chance of becoming a long-term survivor. When BMT had been used in first complete response (CR1) no salvage was possible, whereas 15% survivors may be seen if BMT is used for the first time at relapse. Infants with stage 4 neuroblastoma had a 17% toxic death rate and indication in this group is exceptional and not recommended. In a matched cohort (17 allogeneic and 34 autologous), autologous stem cell rescue (SCR) was shown to be at least equal to allogeneic SCR. Multivariate analysis of clinical prognostic factors in children with stage 4 disease over 1 year showed that event-free survival was mainly influenced by two adverse factors before the megatherapy procedure: persisting skeleton lesions (99Tc and/or mIBG scan positive) as well as persisting bone marrow (BM) involvement.

The diagnosis of a metastatic kidney tumor arising in a 2-month infant is discussed between atypical mesoblastic nephroma and clear cell sarcoma. The precocity of distant metastases, their location in bone marrow, liver and thoracic soft tissues, and their association with myelofibrosis set up an original clinical presentation which seems to have never been described elsewhere. Treatment strategy with surgery of the primary followed by a polychemotherapy combining vincristin-etoposide-ifosfamide and the short term follow-up are reported.

We report two-cases of brain tumors, one childhood medulloblastoma and one adult glioblastoma with an unusual chromosomal abnormality: a t(1;19)(q23;q13). We analyzed these karyotypes using fluorescence in situ hybridization (FISH) and wonder if this chromosomal aberration could represent a particular entity in these brain tumors like t(1;19) in ALL.

The aim of this phase II study was to determine the efficacy of high-dose ifosfamide with moderate dose etoposide in childhood osteosarcoma. From January 1992 to January 1995, 27 children (15 male, 12 female) with relapsed or refractory evaluable osteosarcoma were included in a phase II study of two courses of ifosfamide 3g/m2/day and etoposide 75 mg/m2/day for 4 days. Median age was 14 years (7-19 years). All but one had received high-dose methotrexate and doxorubicin as first-line treatment. 22 patients had previously received ifosfamide. This regimen was given as first-line in 1 patient, second-line in 23 and third-line in 3. Evaluable disease was lung metastases in 21 patients, local relapse in 5 and adenopathy in 1. There were six complete responses, seven partial responses, three minor responses, six stable disease and five progressive disease (including one mixed response). Response rate was 48% (95% confidence interval, 29-67%). Duration of response was not available (10 responding patients had other treatments). Response rate was equivalent in the subgroup of 22 patients who had previously received ifosfamide (4 CR, 6 PR). Among 3 patients who received the phase II regimen as third-line chemotherapy, there was 1 PR. All but 4 patients had a well tolerated grade 4 neutropenia. Transient mild confusion or seizures were each observed once. 5 patients are alive 15-31 months after the beginning of chemotherapy. This combination of drugs at this dosage has tolerable toxicity, is efficient and deserves evaluation in phase III studies.

Pulmonary blastoma is a rare malignant tumor. A new case is reported in a 3 years 6 month-old girl. An apparent clinical remission was first obtained after a surgical treatment followed by a conventional chemotherapy during six months. Afterwards the persistence of a microscopic residual pulmonary disease lead us to deliver successfully an intensive chemotherapy followed by autologous peripheral blood stem cells reinjection. The child remains disease free 12 months after graft. Problems set by the histogenesis of this tumor, its unspecific clinical and paraclinical features and the role of conventional and intensive chemotherapy followed by autologous bone marrow transplantation are discussed on the basis of a review of 50 cases in the literature.

From January 1979 to May 1995, 18 patients (4 men, 14 women) with a mean age of 75.4 +/- 12.5 yr underwent surgery for a complicated paraesophageal hiatus hernia. In 5 patients, the complication was the first sign of the diagnosis. Thirteen patients had a history of digestive, respiratory, or cardiac symptoms (mean duration of symptoms 74 mo.; range 2-240 mo.); 9 of them were aware that they had a hiatus hernia. Ten patients presented with acute obstruction (associated with a perforation in 1 case, jaundice in 1 case, and righy lower lobe pneumonia in 1 case). Hemorrhage occurred in 6 patients (hematemesis 4 cases, melena 2 cases). One patient had a perforation and another had an abscess of the lower right lobe. Surgery was performed via a transabdominal approach in all cases (5 times as an emergency, 12 times as a delayed emergency procedure, and once as an elective procedure). The procedure was delayed in 13 cases because of successful nasogastric decompression. All patients underwent reintegration of the stomach, diaphragmatic repair and gastropexy. An antireflux procedure was performed in 14 cases. Seven patients had an ancillary procedure (including one splenectomy following decapsulation). There were no postoperative deaths. Two patients who underwent emergency surgery developed a benign complication. The outcome of 17 patients is known; none of them developed a recurrence. One patient who did not undergo an antireflux procedure presented with gastroesophageal reflux; another experienced pain during eructation. In conclusion, nearly two-thirds of all patients who present with an acute complication can benefit from medical preparation before surgery, a strategy that improves results.

Thirteen epidemiological studies of the relations between electromagnetic fields and childhood cancers have been published. Eleven have shown some associations between the presumed intensity of exposure to residential magnetic fields and the childhood cancers incidence, either for all cancers or for the 3 most frequent types (leukaemias, brain tumours and lymphomas). These associations are not often significant because of the weak statistical power of these studies in relation with both the low incidence of cancers in childhood, and specially of each particular type, and the little number of subjects considered to be exposed at a high level exposure for residential magnetic fields. All these studies fall in with the same difficulties, particularly for identification and assessment of the exposure to magnetic fields and for the manner to take into account all the potential confounders. So even significant associations do not imply their causality, all the more as the carcinogenicity of extremely low frequency electromagnetic fields, although biologically conceivable, has never been experimentally proven. Today it is impossible to claim or invalidate the influence of residential magnetic fields in the genesis of childhood cancer. Setting up of new epidemiological studies based on large number of cases issued from population based registries and conducted with a best defined methodology seems to be highly desirable.

Between 1979 and May 1994, 42 patients (11 males, 31 females, mean age 72.8 +/- 12.4 years) underwent surgery for paraoesophageal hiatal hernia. In 37 cases, clinical signs had been observed for 64 months on the average: digestive (32), respiratory (15), cardiac (9). Nine patients had iron deficiency anaemia. Eighteen patients had a complication (acute obstruction: 10, haemorrhage: 6, perforation: 2, lung infection: 2). In 5 cases, the complication was the inaugural sign. Surgery was performed in a emergency situation in 5 cases and deferred emergency in 12 and surgery was elective in 25. An antireflux procedure was used in 36 cases (Lortat-Jacob (2), Toupet (5), Dor (2), Nissen-Rossetti (27)). Another procedure was also needed in 12 patients. There were no postoperative deaths and complications occurred in 6 patients (14.9%). Outcome was known in 40 patients with a mean follow-up of 40.5 months (range 3 to 140 months). Functional results was very good in 35 patients (87.5%), good in 3 (7.5%) and poor in 2 (5%). The most satisfactory immediate results were obtained for patients who had elective surgery and in those with a deferred emergency. Use of the Nissen-Rossetti procedure did not have any deleterious effect on these results and no long-term inconveniences.

We used a noninvasive monitor of arterial pressure to determine whether respiratory changes in arterial pressure were closely correlated with airflow obstruction in asthmatic patients during bronchial challenge with methacholine. To validate the noninvasive measurement of respiratory changes in arterial pressure, a preliminary study in 6 subjects with normal cardiovascular and respiratory systems was done during cardiac catheterization for suspected coronary artery disease. There were no significant differences between inspiratory falls in systolic pressure measured noninvasively and those measured from intraaortic pressure. In 11 otherwise healthy asthmatic patients we measured finger arterial pressure, end-expiratory lung volume (FRC), and forced expired volume (FEV1) during baseline and bronchial challenge in the supine posture. Finger arterial pressure was also measured in 11 normal control subjects seated and supine. Normal subjects had an inspiratory fall in systolic pressure (IFSP) of 3.2 mm Hg supine and 5.1 mm Hg seated (p < 0.01). Asthmatic patients when bronchodilated (seated FEV1 = 83 +/- 7% of predicted) had an IFSP of 5.9 mm Hg supine (p < 0.01 compared with supine normal subjects). During bronchial challenge (average fall in FEV1 = 22%), IFSP increased to 16.1 mm Hg (p < 0.001 compared with baseline). In asthmatic subjects, there was a significant correlation between IFSP and FEV1 (mean r = -0.92 +/- 0.05, p < 0.01), and the average change in IFSP/change in FEV1 was -0.38 mm Hg per percentage change in FEV1. During subsequent bronchodilation, IFSP decreased with a similar time course as relaxation of airway smooth muscle, assessed by the breath-to-breath fall in FRC.(ABSTRACT TRUNCATED AT 250 WORDS)

Background: Chronic administration of theophylline can be used for treating asthma; it may represent a severe risk when some other drugs are prescribed by unaware physicians., Case Report: A 4 yr-8-mo-old boy was admitted suffering from status epilepticus which occurred 3 hours after vomiting. This patient had asthma and was given sustained-release formulation of theophylline and, independently by another physician, two anti-H1 drugs: ketotifen and mequitazine. Serum theophylline concentration measured 9 hours after the onset of vomiting was 28 micrograms/ml; the calculated concentrations were 45.3 +/- 4, 40.2 +/- 4 and 33.4 +/- 4 micrograms/ml after the last administration of theophylline, the onset of vomiting and the onset of seizures, respectively. The patient was given IV fluids plus clonazepam and recovered completely., Conclusion: Severe poisoning can be seen with moderately increased blood levels of theophylline when this drug is chronically administered. Association of other drugs such as anti-H1 could represent an additional risk.

From November 1990 to September 1992, 72 patients underwent a video-assisted thoracic surgery (VATS), 47 men and 25 women, of mean age 49 years. Video-assisted thoracic surgery was indicated for the following: 44 spontaneous pneumothorax, 6 benign tumors of the lung or the mediastinum, 4 pleural effusions, one broncho-pleural fistula and one sympathectomy; 17 diagnostic procedures (lung or mediastinal lymph-nodes biopsies). In one patient, it was necessary to associate a mini-thoracotomy and 5 patients went on to a formal thoracotomy. There was no mortality and the morbidity rate was 5.5%. Some conditions are required: one-lung ventilation, ability to convert to a formal thoracotomy at any moment. It is contra-indicated in cases of single lung or complete obliteration of the pleural space due to dense adhesions. The place of VATS is now well defined for the treatment of spontaneous pneumothorax. It is discussed for resection of pulmonary diseases depending on their size, location and histologic type.

From january 1986 to december 1992, 71 patients underwent direct colo-anal anastomosis as described by Parks (CAA) after total rectal resection for carcinoma: 49 men and 22 women with a mean age of 64 years (range 37-82). In 67 cases, the indication was for adenocarcinoma of the mid and low rectum, and in 4 cases for carcinoma of the upper rectum associated with a low rectal benign tumour (6 Dukes A, 36 Dukes B, 21 Dukes C, 8 Dukes D). A diverting colostomy was constructed in all cases. One patient died from pulmonary embolism (mortality: 1.4%). Anastomotic leakage occurred in 6 cases (8.5%). None of these cases required reoperation and all colostomies have been closed. Local recurrence occurred in 12 cases (17%) 6 to 34 months after CAA, of whom 4 were treated by abdominoperineal resection. Eleven patients died from local recurrence (3 cases) or distant metastasis (8 cases). Actuarial survival at 1, 2, 3, 4 and 5 years was 92%, 88%, 78%, 75% and 69% respectively. From the functional point of view, one patient underwent abdomino-perineal resection for incontinence 3 years after CAA. All the other patients were fully continent, with a mean stool frequency of 2 per day, and good gas-stool discrimination. Twenty per cent of patients presented soiling, 20% with stool frequency, and 12% with urgency. Long term functional and oncological results make CAA a good alternative to abdomino-perineal resection for mid and low rectal carcinoma.

This survey includes 25 children with Wilms' tumor undergoing high-dose chemotherapy associated with autologous bone marrow transplantation (ABMT) in the period June 1984-December 1991 and enrolled in the European Bone Marrow Transplantation Registry for Solid Tumors. At diagnosis, 12 children presented stage IV disease, 5 stage III, 3 stage II, and 5 stage I. Before ABMT, 21 children had 1 to 4 relapses (median 1); 13 achieved a second or subsequent complete remission (CR), four stage IV children failed to respond to first line treatment and achieved either CR (3 patients), or partial remission (PR) after second line therapy. At high-dose chemotherapy, 17 children were in CR and 8 had measurable disease. Seven different high-dose regimens were administered, even if 20 children received melphalan mostly associated with vincristine and 8 involved field radiotherapy. Three children died early of pneumonitis; 2 developed an acute transient renal failure, 1 a chronic renal failure. Out of the 8 children with target disease at graft, 2 died of toxicity, 5 achieved CR, 1 obtained PR, and only 1 is presently alive in CCR at 39 months after ABMT. Of the 17 children grafted in CR, 8 are alive event-free at 14-90 months (median 34) from ABMT; 7 relapsed at 3-23 months (median 7 months); 1 died of toxicity and 1 was lost to follow-up in CR at 12 months. A salvage attempt with high-dose chemotherapy in children with resistant or poor prognosis recurrent Wilms' tumor seems to be justified. An international cooperative protocol taking into account the increased risk of lung and renal toxicity is necessary.