Cross-cutting view of current challenges in paediatric solid organ and haematopoietic stem cell transplantation in Europe: the European Reference Network TransplantChild.

The low prevalence of European paediatric transplanted patients and scarcity of resources and expertise led to the need for a multidisciplinary network able to improve the quality of life of paediatric patients and families requiring a solid organ or haematopoietic stem cell transplantation. The European Reference Network (ERN) TransplantChild is one of the 24 ERNs established in a European legal framework to improve the care of patients with rare diseases. ERN TransplantChild is the only ERN focused on both solid organ and haematopoietic stem cell paediatric transplantation, based on the understanding of paediatric transplantation as a complex and highly specialised process where specific complications appear regardless the organ involved, thus linking the skills and knowledge of different organ disciplines. Gathering European centres of expertise in paediatric transplantation will give access to a correct and timely diagnosis, share expertise and knowledge and collect a critical mass of patients and data that increases the speed and value of clinical research outcomes. Therefore, the ERN TransplantChild aims for a paediatric Pan-European, Pan-transplant approach.

Outcome of non-visualization of fetal gallbladder on second-trimester ultrasound: cohort study and systematic review of literature.

OBJECTIVES: To investigate the ultrasound characteristics and outcome of fetuses with non-visualization of the fetal gallbladder (NVFGB) followed in our tertiary university hospital, and to provide a comprehensive review of the literature on prenatal findings and outcome of NVFGB. METHODS: NVFGB was defined as non-visualization of the gallbladder on two targeted ultrasound examinations performed within a 1-week period. First, we reviewed the medical records of NVFGB cases managed in our center over a 9-year period. Then, we performed a systematic review of the literature to identify studies on NVFGB. The incidence of chromosomal anomalies, later visualization of the gallbladder, gallbladder agenesis, cystic fibrosis and biliary atresia was assessed in fetuses with isolated and non-isolated NVFGB. The role of hepatic enzyme measurements in the diagnosis of cystic fibrosis and biliary atresia in fetuses with NVFGB was also reviewed. RESULTS: Sixteen cases of NVFGB were followed in our center, in 10 (62.5%) of which it was an isolated finding. The incidence of biliary atresia was 12.5% and that of gallbladder agenesis was 12.5%, while no case of cystic fibrosis was reported. The gallbladder was visualized later in pregnancy or postnatally in 43.8% and 25.0% of cases, respectively. A total of seven studies, including our cohort, involving a total of 280 NVFGB cases, met the inclusion criteria for the systematic review. Overall, 20.5% of fetuses had an associated ultrasound anomaly, and the incidence of chromosomal anomaly in this group was 20.4%. In cases with isolated NVFGB, the incidence of chromosomal anomaly was 1.9%. In fetuses with normal karyotype and isolated NVFGB, the gallbladder was later visualized in 70.4% of cases, while the incidence of gallbladder agenesis, cystic fibrosis and biliary atresia was 25.2%, 3.1% and 4.8%, respectively. In fetuses with non-isolated NVFGB, the incidence of cystic fibrosis and biliary atresia was 23.1% and 18.2%, respectively. The negative predictive value of amniotic fluid enzyme levels for the prediction of severe disease (including biliary atresia or cystic fibrosis) ranged between 94% and 100% when evaluated before 22 weeks' gestation, and dropped to 88% after 22 weeks. CONCLUSIONS: In cases with persistent NVFGB, the risk of a severe postnatal condition should be considered. A detailed ultrasound scan should be offered and parents tested for cystic fibrosis gene mutation. An invasive procedure for karyotyping and measurement of liver enzyme concentrations before 22 weeks constitutes a reasonable work-up. Copyright © 2019 ISUOG. Published by John Wiley & Sons Ltd.

No visualización de la vesícula biliar fetal en la ecografía del segundo trimestre del embarazo: estudio de cohortes y revisión sistemática de la literatura sobre el resultado postnatal OBJETIVOS: Investigar las características ecográficas y los resultados de los fetos con no visualización de la vesícula biliar fetal (NVFGB, por sus siglas en inglés) a los que se ha dado seguimiento en un hospital universitario terciario, y ofrecer una revisión exhaustiva de la literatura sobre los hallazgos prenatales y los resultados de la NVFGB. MÉTODOS: La NVFGB se definió como la no visualización de la vesícula biliar en dos exámenes ecográficos específicos realizados en un período de una semana. Primero, se revisó los registros médicos de los casos de NVFGB tratados en este hospital durante un período de 9 años. Luego, se realizó una revisión sistemática de la literatura para identificar estudios sobre NVFGB. Se evaluó la incidencia de anomalías cromosómicas, la visualización posterior de la vesícula biliar, la agenesia vesicular, la fibrosis quística y la atresia biliar en fetos con NVFGB aislada y no aislada. También se examinó la función de las mediciones de las enzimas hepáticas en el diagnóstico de la fibrosis quística y la atresia biliar en fetos con NVFGB. RESULTADOS: Se siguieron dieciséis casos de NVFGB en este centro hospitalario, lo cual fue un hallazgo aislado en 10 de ellos (62,5%). La incidencia de atresia biliar fue del 12,5% y la de agenesia vesicular del 12,5%, mientras que no se reportó ningún caso de fibrosis quística. La vesícula biliar se visualizó más tarde en el embarazo o después del parto en el 43,8% y 25,0% de los casos, respectivamente. Un total de siete estudios cumplieron los criterios de inclusión para la revisión sistemática, incluidos los de la cohorte del mencionado hospital, con un total de 280 casos de NVFGB. En total, el 20,5% de los fetos presentaban una anomalía ecográfica asociada, y la incidencia de anomalías cromosómicas en este grupo fue del 20,4%. En los casos con NVFGB aislada, la incidencia de anomalías cromosómicas fue del 1,9%. En los fetos con cariotipo normal y NVFGB aislada, la vesícula biliar se visualizó posteriormente en el 70,4% de los casos, mientras que la incidencia de agenesia vesicular, fibrosis quística y atresia biliar fue del 25,2%, 3,1% y 4,8%, respectivamente. En los fetos con NVFGB no aislada, la incidencia de fibrosis quística y atresia biliar fue del 23,1% y 18,2%, respectivamente. El valor predictivo negativo de los niveles de enzimas del líquido amniótico para la predicción de una enfermedad grave (como la atresia biliar o la fibrosis quística) se situó entre el 94% y el 100% cuando se evaluó antes de las 22 semanas de gestación, y bajó al 88% después de las 22 semanas. CONCLUSIONES: En casos con NVFGB persistente, se debe considerar el riesgo de una condición postnatal severa. Se debe ofrecer una ecografía detallada y la madre y el padre deben someterse a pruebas para detectar mutaciones del gen de la fibrosis quística. Un examen diagnóstico razonable incluye un procedimiento invasivo para el cariotipado y la medición de las concentraciones de enzimas hepáticas antes de las 22 semanas.

Impacts of donor-specific anti-HLA antibodies and antibody-mediated rejection on outcomes after intestinal transplantation in children.

AMR is a risk factor for graft failure after SBTx. We studied impact of DSAs and AMR in 22 children transplanted between 2008 and 2012 (11 isolated SBTx, 10 liver inclusive Tx, and one modified multivisceral Tx). Three patients never developed DSA, but DSAs were found in seven in the pre-Tx period and de novo post-Tx in 19 children. Pathology revealed cellular rejection (15/19), with vascular changes and C4d+. Patients were treated with IV immunoglobulins, plasmapheresis, and steroids. Rescue therapy included antithymocyte globulins, rituximab, eculizumab, and bortezomib. Pathology and graft function normalized in 13 patients, graft loss occurred in two, and death in seven. At the end of the follow-up, 15 children were alive (68%), 13 with functioning graft (59%). Prognosis factors for poor outcome after Tx were the presence of symptoms at AMR suspicion (P +.033). DSAs were often found following SBTx, mostly de novo. Resistant ACR or severe AMR is still difficult to differentiate, with a high need for immunosuppression in both. DSAs may precede development of severe disease and pathology features on the graft: relationship and correlation need to be better investigated with larger groups before and after Tx.

Long-term outcome of children with patent processus vaginalis incidentally diagnosed by laparoscopy.

INTRODUCTION: Patent processus vaginalis (PPV) might be incidentally diagnosed during laparoscopy. The aims of this study were to determine the prevalence and the natural history of PPV, i.e. its possible development into symptomatic inguinal hernia. INCLUSION CRITERIA: children <16years undergoing laparoscopy for pathologies other than processus vaginalis (PV) related, from 10/2000-10/2005. EXCLUSION CRITERIA: past or present history of PV-related pathologies. The internal inguinal rings were documented during laparoscopy. Follow-up was provided by phone inquiry and clinical examination if needed. Median follow-up was 10.5years (range 7.1-12.8). RESULTS: 416 patients were included. Median age at laparoscopy was 12.4years (range 3days-18.1years). Forty-three PPV (33 unilateral, 5 bilateral) were found in 38 patients (9.1%). Four children with PPV presented later with an ipsilateral inguinal hernia (10.5%, 95%CI [3%; 25%]), at a median age of 16.0years (range 11.8-17.3), at a median of 22.5months (range 12-50) after initial laparoscopy, as compared to no patient in the population with obliterated PV (0%, 95%CI [0%; 1%]). CONCLUSION: 9.1% of the observed pediatric population showed an asymptomatic PPV, and 10.5% of these children later developed an inguinal hernia. None of the children with obliterated PV developed a hernia.

Early and late complications after liver transplantation for propionic acidemia in children: a two centers study.

Propionic acidemia (PA) is a severe metabolic disorder with cardiac and neurologic complications and a poor quality of life. Liver transplantation (LT) was thus proposed in PA to increase enzyme activity. We studied retrospectively LT in PA in two European centers. Twelve patients underwent 17 LTs between 1991 and 2013. They developed severe, unusual and unexpected complications, with high mortality (58%). When present, the cardiomyopathy resolved and no acute metabolic decompensation occurred allowing dietary relaxation. Renal failure was present in half of the patients before LT and worsened in all of them. We suggest that cardiac and renal functions should be assessed before LT and monitored closely afterward. A renal sparing immunosuppression should be used. We speculate that some complications may be related to accumulated toxicity of the disease and that earlier LT could prevent some of these consequences. As kidney transplantation has been performed successfully in methylmalonic acidemia, a metabolic disease in the same biochemical pathway, the choice of the organ to transplant could be further discussed.

Assessment and outcome of children with intestinal failure referred for intestinal transplantation.

BACKGROUND & AIMS: Chronic intestinal failure (CIF) requires long term parenteral nutrition (PN) and, in some patients, intestinal transplantation (ITx). Indications and timing for ITx remain poorly defined. In the present study we aimed to analyze causes and outcome of children with CIF. METHODS: 118 consecutive patients referred to our institution were assessed by a multidisciplinary team and four different categories were defined retrospectively based on their clinical course: Group 1: patients with reversible intestinal failure; group 2: patients unsuitable for ITx, group 3: patients listed for ITx; group 4: patients stable under PN. Analysis involved comparison between groups for nutritional status, central venous catheter (CVC) related complications, liver disease, and outcome after transplantation by using non parametric tests, Mann-Whitney tests, Kruskal-Wallis, Wilcoxon signed rank tests and chi square distribution for percentage. RESULTS: 118 children (72 boys) with a median age of 15 months at referral (2 months-16 years) were assessed. Etiology of IF was short bowel syndrome [n = 47], intractable diarrhea of infancy [n = 37], total intestinal aganglionosis [n = 18], and chronic intestinal pseudoobstruction [n = 17]. Most patients (89.8%) were totally PN dependent, with 48 children (40.7%) on home-PN prior to admission. Nutritional status was poor with a median body weight at -1.5 z-score (ranges: -5 to +2.5) and median length at -2.0 z-score (ranges: -5.5 to +2.3). The mean number of CVC inserted per patient was 5.2 (range 1-20) and the mean number of CRS per patient was 5.5 (median: 5; range 0-12) Fifty-five patients (46.6%) had thrombosis of ≥2 main venous axis. At admission 34.7% of patients had elevated bilirubin (≥50 µmol/l), and 19.5% had platelets <100,000/ml, and 15% had both. Liver biopsy performed in 79 children was normal (n = 4), or showed F1 or F2 fibrosis (n = 29), bridging fibrosis F3 (n = 20), or cirrhosis (n = 26). Group 1 included 10 children finally weaned from PN (7-years survival: 100%). Group 2 included 12 children with severe liver disease and associated disorders unsuitable for transplantation (7-years survival: 16.6%). Group 3 included 66 patients (56%) who were listed for small bowel or liver-small bowel transplantation, 62/66 have been transplanted (7 years survival: 74.6%). Factors influencing outcome after liver-ITx were body weight (p < .004), length (p < .001), pre-Tx bilirubin plasma level (p < .001) and thrombosis (p < .01) for isolated ITx, Group 4 included 30 children (25.4%) with irreversible IF considered as potential candidates for isolated ITx. Four children were lost from follow up and 3 died within 2 years (survival 88.5%). Among potential candidates, the following parameters improved significantly during the first 12 months of follow up: Body weight (p.0001), length (p < .0001) and bilirubin (p < .0001). CONCLUSIONS: many patients had a poor nutritional status with severe complications especially liver disease. PN related complications were the most relevant indication for ITx, but also a negative predictor for outcome. Early patient referral for Tx-assessment might help to identify and separate children with irreversible IF from children with transient IF or uncomplicated long-term PN, allowing to adapt a patient-based treatment strategy including or not ITx.

Mucormycosis as a rare cause of severe gastrointestinal bleeding after multivisceral transplantation.

Mucormycosis, an emerging fungal infection in solid organ transplant patients, is mostly located in rhino-orbito-cerebral, pulmonary, and cutaneous areas, or disseminated with poor prognosis. A 4-year-old girl with chronic intestinal pseudo-obstruction syndrome underwent a modified multivisceral transplantation, including half of the stomach, the duodeno-pancreas, the small bowel, and the right colon. On postoperative day 5, a digestive perforation was suspected. Surgical exploration found a small necrotic area on the native stomach, which was externally drained. The next day, massive gastric bleeding occurred. During the emergency laparotomy, 2 hemorrhagic ulcers were found and resected from the transplanted stomach. Pathology and fungal culture showed mucormycosis caused by Lichtheimia (formerly Absidia) ramosa in both the transplanted and native stomach. High-dose intravenous liposomal amphotericin B was immediately started. No other site of fungal infection was found. The child recovered, and 3 years after transplantation, is alive and well, off parenteral nutrition. The originality of this case is the very early presentation after transplantation, the unusual site, and the complete recovery after rapid medico-surgical management. The origin of the fungus and treatment are discussed.

Staged abdominal closure after small bowel or multivisceral transplantation.

Following paediatric SBMT, size discrepancy between the recipient's abdomen and the graft may lead to ACS, graft dysfunction, and death. We report our experience with SAC in these patients. Between 04/1993 and 03/2009, 57 children underwent 62 SBMTs. When abdominal wall tension seemed excessive for safe PAC, SAC was performed, using a Silastic® sheet and a vacuum occlusive dressing. Transplantations with SAC (23 combined liver and small bowel [CLB]) were compared with those with PAC [14 ISB and 25 CLB]. Indications for transplantation, preoperative status (after stratification for ISB/CLB transplants), age at transplantation, donor-to-recipient weight ratio, reduction in bowel and/or liver, and incidence of wound complications were not different in both groups. Post-operative intubation, stay in intensive care unit, and hospital stay were prolonged after SAC. Two deaths were related to ACS after PAC, none after SAC. Since 2000, one-yr patient survival is 73% after ISB transplantation and 57% vs. 75% after CLB transplantation with PAC vs. SAC, respectively (NS). SAC safely reduces severe ACS after paediatric SBMT and can be combined with graft reduction for transplantation of small recipients.

[Biliary atresia: a condition requiring urgent diagnosis and treatment]. / Atrésie des voies biliaires : une urgence diagnostique et thérapeutique.

Every neonatal jaundice lasting more than 2 weeks needs urgent investigations, beginning with examination of stools colour, and blood tests with total and conjugated serum bilirubin. If neonatal cholestasis (NC) is confirmed, vitamin K should be immediately injected, and the child should be referred to a specialised centre for investigations and treatment. Biliary atresia (BA) is the first cause of NC. Its diagnosis is urgent, since the chance of success of the conservative surgical treatment (Kasai operation or variants) decreases rapidly as the age at surgery increases. Normal ultrasound scans cannot rule out BA. After prompt work-up looking for the main other causes of NC, BA can often be strongly suspected before surgery, and is confirmed by operative findings and cholangiogram if needed. In case of failure to restore the biliary drainage, biliary cirrhosis progresses and leads to liver transplantation, generally in the first years of life. Currently, more than 90 % of children with BA can live, with a close to normal quality of life for most of them. Early diagnosis and treatment of BA contribute to decrease the needs for liver transplantation in infancy and childhood.

[Surgical therapy of abdominal cystic lymphangioma in adults and children]. / Le traitement chirurgical du lymphangiome kystique abdominal chez l'adulte et chez l'enfant.

BACKGROUND: Cystic lymphangioma is a rare malformative benign tumor of the lymphatic vessels. In the abdomen it generally develops as a mesenteric and/or retroperitoneal cyst, but any organ can be involved. The present retrospective study aims to define the symptoms, complications and differences noted between adults and children suffering from abdominal cystic lymphangioma; it is based on patients who underwent surgery for this condition at the Geneva University Hospital. PATIENTS AND METHODS: Since 1995, 16 patients (9 adults and 7 children) were surgically treated for abdominal cystic lymphangioma. Their medical files were reviewed retrospectively. The follow-up was based either on the last physical examination or on a telephone interview with the patients. RESULTS: The mean follow-up was 45 months. The most common presenting symptom was abdominal pain (38%). Ultrasonography was the most efficacious diagnostic modality. The lesions were mostly micropolycystic (44%), and found in retroperitoneal locations (50%). The surgical excision was complete in 14 patients and partial in 2 patients. These last 2 were the only ones who developed complications after the surgery, including one recurrence. CONCLUSIONS: A total surgical excision, if feasible without a major sacrifice of adjacent organs, seems to be the best therapeutic option to minimize the risk of recurrence of symptomatic abdominal cystic lymphangiomas. In our clinical experience, the presentation and evolution of the abdominal cystic lymphangioma seemed to be similar in adults and children.

European biliary atresia registries: summary of a symposium.

Biliary atresia (BA) is a rare but potentially devastating disease. The European Biliary Atresia Registry (EBAR) was set up to improve data collection and to develop a pan-national and interdisciplinary strategy to improve clinical outcomes. From 2001 to 2005, 100 centers from 22 countries registered with EBAR via its website (www.biliary-atresia.com). In June 2006, the first meeting was held to evaluate results and launch further initiatives. During a 5-year period, 60 centers from 19 European countries and Israel sent completed registration forms for a total of 514 BA patients. Assuming the estimated incidence of BA in Europe is 1:18,000 live births, 35% of the expected 1488 patients from all EBAR participating countries were captured, suggesting that reporting arrangements need improvement. At the meeting, the cumulative evaluation of 928 BA patients including patients from other registries with variable follow-up revealed an overall survival of 78% (range from 41% to 92%), of whom 342 patients (37%) have had liver transplants. Survival with native liver ranged from 14% to 75%. There was a marked variance in reported management and outcome by country (e.g., referral patterns, timing of surgery, centralization of surgery). In conclusion, EBAR represents the first attempt at an overall evaluation of the outcome of BA from a pan-European perspective. The natural history and outcome of biliary atresia is of considerable relevance to a European population. It is essential that there is further support for a pan-European registry with coordination of clinical standards, further participation of parent support groups, and implementation of online data entry and multidisciplinary clinical and basic research projects.

Liver function tests in children with blunt abdominal traumas.

BACKGROUND: Previous studies have suggested that there are cut-off values for liver function tests (LFTs) beneath which significant liver injury can be excluded after blunt abdominal trauma in children. Our objective is to test this hypothesis in our patient population. METHODS: The LFTs of all consecutive patients admitted in Geneva from January 1, 2001 to December 31, 2004 following blunt abdominal trauma were analysed and compared to radiological (ultrasound and/or computed tomography scan) findings and final outcome. RESULTS: Of 115 patients identified, sixteen had radiological evidence of liver injury. These patients had significantly (p < 0.01) increased aspartate aminotransferase (AST) and alanine aminotransferase (ALT) values (474 +/- 369 IU/l and 442 +/- 383 IU/l, respectively) compared to patients without liver injury (AST 88 +/- 161 IU/l and ALT 68 +/- 137 IU/l). Among the sixteen patients with liver injury, ten (63 %) had AST < 450 IU/l and seven (44 %) had ALT < 250 IU/l. Two patients had radiological evidence of OIS grade 3 liver injury with AST as low as 95 and 92, and ALT of 80 and 86, below all cut-off values recommended in the literature. CONCLUSION: In our experience, low LFT values at admission could not rule out significant liver injury. The diagnosis of such lesions still relies on clinical and radiological findings, as do other intra-abdominal organ injuries.

[Prenatal diagnosis of congenital mesoblastic nephroma. A case report]. / Diagnostic anténatal d'un néphrome mésoblastique congénital.

UNLABELLED: Antenatal ultrasounds allow the detection of renal tumors, especially renal mesoblastic nephromas, but only the pathological analysis of the surgical specimen can confirm this diagnosis postnatally. OBSERVATION: We report the prenatal discovery of a mesoblastic nephroma because of premature labour. Postnatal early surgery was decided because of possible complications in this premature infant. Histology revealed mesoblastic nephroma. COMMENTS: We point out the diagnostic elements of congenital mesoblastic nephroma, especially in what is related to arterial hypertension and hypercalcemia, histology and cytogenetics.

Outcome of liver disease in children with Alagille syndrome: a study of 163 patients.

BACKGROUND AND AIMS: Various opinions have been expressed as to the long term prognosis of liver disease associated with Alagille syndrome (AGS). PATIENTS AND METHODS: We reviewed the outcome of 163 children with AGS and liver involvement, investigated from 1960 to 2000, the end point of the study (median age 10 years (range 2 months to 44 years)) being death, liver transplantation, or the last visit. RESULTS: At the study end point, of the 132 patients who presented with neonatal cholestatic jaundice, 102 remained jaundiced, 112 had poorly controlled pruritus, and 40 had xanthomas; cirrhosis was found in 35/76 livers, varices in 25/71 patients, and liver transplantation had been carried out in 44 patients (33%). Forty eight patients died, 17 related to complications of liver disease. Of 31 patients who did not present with neonatal cholestatic jaundice, five were jaundiced at the study end point, 17 had well controlled pruritus, and none had xanthomas; cirrhosis was found in 6/18 patients, varices in 4/11, and none underwent liver transplantation. Nine patients died, two of liver disease. In the whole series, actuarial survival rates with native liver were 51% and 38% at 10 and 20 years, respectively, and overall survival rates were 68% and 62%, respectively. Neonatal cholestatic jaundice was associated with poorer survival with native liver (p=0.0004). CONCLUSIONS: The prognosis of liver disease in AGS is worse in children who present with neonatal cholestatic jaundice. However, severe liver complications are possible even after late onset of liver disease, demanding follow up throughout life.

Adult-to-adult living related liver transplantation: initial experience.

The number of adult patients on the liver transplantation waiting lists is growing steadily. Adult living related liver transplantation (LRLT) represents the ultimate means to expand the donor pool. The success of this model of "small for size" grafting relies on strict donor and recipient selection. The choice of the graft (2 left and 4 right hepatectomies) was made on the minimal ratio between estimated graft and recipient body weights (0.8-1%), necessary to meet the recipient's metabolic demands. Our experience with six adults is reported. Donor morbidity was minimal (one wound infection); there was no mortality. Four (66%) recipients are doing well, two died of infectious complications. All recipients had a complicated post-transplant course. Due to its complexity, both in donor and recipient, LRLT should only be developed very carefully in experienced liver transplant centers.

[Coordinated organization of symptoms management and support in all the stages of cancer disease: putting in place pluridisciplinary structures of supportive oncological care]. / Organisation coordonnée de la prise en charge des symptômes et du soutien à toutes les phases de la maladie cancéreuse: vers la mise en place de structures pluridisciplinaires de soins oncologiques de support.

The organization of the management of pain and other symptoms all along the cancer disease, of psychological support and palliative care is a complex question that does not correspond to any perfectly established model, both in France and abroad. Different structures are implied in there care and coexist with an insufficient coordination: cancerology structures, structures of chronic pain management, structures of psycho-oncology, structures of palliative care. Some other assistances are more or less isolated inside the hospital: nutritional support, social assistance, action against tobacco and other addictions, volunteer work. Because of the evolution of practices and mentalities over the last ten years, the highlights of evident interfaces and complementary activities, the notions of "continuous care" and "integrated care" inside conventional departments, the budgetary and organizational restraints, it is now possible to propose a model of hospital structure adapted to the problem of supportive care. The creation is proposed from preexisting structures, consultations, units, departments of supportive oncological care according to the size of the institution. The structure should comply with some specifications, sometimes regulations, and to coordinate at best different competencies in the interest of the patients and medical teams : pain and symptoms management (of which palliative care is an important part), psycho-oncology, rehabilitation (functional rehabilitation, nutrition, social work, fights against addictions). A pooling of technology settings is one of its interest. The model can be applied in other domains than cancerology and in most health institutions.

Orthotopic liver transplantation for mitochondrial respiratory chain disorders: a study of 5 children.

BACKGROUND: Liver involvement in mitochondrial respiratory chain disorders (MRCD) frequently ends in liver failure and death. Because of the high risk of extrahepatic, particularly neuromuscular, manifestations of the disease, the indication of orthotopic liver transplantation (OLT) in these patients remains controversial. We report on 5 such children in whom OLT was carried out, in an attempt to help clarify the matter. PATIENTS: Patients 1 and 2 presented with fulminant liver failure at ages 7 and 6 months respectively. Emergency liver transplantation was performed before etiological investigations were completed. Retrospective examination of the explanted livers showed defects in complexes I, III and IV. In patient 1, severe neurological deterioration occurred 2 months after OLT with fatal outcome 9 months later. Patient 2 is alive 22 months after OLT with moderate motor impairment. Patients 3, 4 and 5 presented with progressive liver failure before 6 months of age. Surgical liver biopsies displayed a 50% defect in complex IV (patient 3), a defect in complexes I, IV (patient 4) and in complexes I, III, IV (patient 5). Because there was no clinical extrahepatic involvement on investigations, OLT was carried out in these patients. Patient 3 died of multiple organ failure soon after OLT, patients 4 and 5 are alive respectively 21 months and 12 months after OLT with normal neurological examination. CONCLUSION: OLT may be a valid therapeutic option in infants with delayed liver cell failure due to MRCD, only after performing in emergency a thorough inves tigation to exclude clinically significant extrahepatic, especially neuromuscular, involvement.

[Update on kidney tumors in children: role of the pediatric surgeon]. / Actualités dans les affections tumorales du rein del'enfant: rôle du chirurgien pédiatre.

The surgical treatment of malignant renal tumours in children (nephroblastomas) is part of a multimodal therapeutic strategy, defined by the International Society of Paediatric Oncology protocol. Resection of the primary tumour and determination of the post-operative stage determine subsequent treatment. The modalities of the surgical procedure are described together with the particular cases concerning infants under the age of 6 months, extension to the renal vein and inferior vena cava, emergency surgery, bilateral, tumours or tumour in a solitary kidney, lymph node involvement, metastatic nephroblastomas and complications of surgery.