Long-term outcome of liver transplantation in childhood: A study of 20-year survivors.

We report the results of a study of survival, liver and kidney functions, and growth with a median follow-up of 24 years following liver transplantation in childhood. From 1988 to 1993, 128 children underwent deceased donor liver transplantation (median age: 2.5 years). Twenty-year patient and graft survival rates were 79% and 64%, respectively. Raised serum aminotransferase and/or γ-glutamyl transferase activities were present in 42% of survivors after a single transplantation. Graft histology (35 patients) showed signs of chronic rejection in 11 and biliary obstruction in 5. Mean total fibrosis scores were 4.5/9 and 3/9 in patients with abnormal and normal serum liver tests, respectively. Glomerular filtration rate was <90 mL·min-1 in 35 survivors, including 4 in end-stage renal disease who were undergoing dialysis or had undergone renal transplantation. Median final heights were 159 cm for women and 172 cm for men; final height was below the target height in 37 patients. Twenty-year survival after childhood liver transplantation may be close to 80%, and final height is within the normal range for most patients. However, chronic kidney disease or altered liver biochemistries are present in over one third of patients, which is a matter of concern for the future.

Organization in response to massive afflux of war victims in civilian practice - experimental feedback from the November 2015 Paris terrorist attacks.

The arrival of a large number of war-weapon casualties at a civilian trauma center requires anticipation. A plan defining the management principles and the respective roles of the involved physicians and nurses and their interaction with each other is essential. Uni-directional patient flow associated with adequate numbers of staff physicians and nurses under the leadership of a medical director is essential to prevent the overwhelming of the trauma center. Routine and regular interaction between the pre-hospital medical flow control system and the medical director, on one hand, and between surgical teams and the medical director, on the other, are necessary to know when to apply "damage control" surgical techniques. Based on the feedback of a level 1 trauma center that received 53 victims of the November 13, 2015 terrorist attack in Paris, we present the factors of success, and the stumbling blocks.

[Duodenal duplication revealed by acute pancreatitis]. / Duplication duodénale révélée par une pancréatite aiguë.

INTRODUCTION: Duodenal duplications are rare congenital malformations whose revealing signs are highly variable and nonspecific. OBSERVATION: We report the case of a female infant who presented with neonatal acute pancreatitis complicated by recurrent ascites, profound hypoalbuminemia responsible for pleural and pericardial effusions, revealing a duodenal duplication cyst. The unusual and original clinical presentation as well as the difficulty detecting the duplication radiologically delayed the diagnosis. A prolonged medical treatment with octreotide, albumin infusions, and exclusive parenteral nutrition led to an almost total disappearance of the ascites before surgery. The outcome was favorable after surgical removal of the duplication with 1 year of follow-up. CONCLUSION: The diagnosis of duodenal duplication can be difficult and it may be necessary to repeat the ultrasound examinations. Surgical resection is delicate, especially when there is an abundant pancreatic ascites. Therefore, an adequate prolonged medical treatment to reduce this ascites is recommended before the surgery.

[Pediatric liver tumor: What to do?]. / Conduite à tenir devant une tumeur hépatique de l'enfant.

Two-thirds of pediatric liver tumors are malignant, but pseudotumors such as abscesses or hematoma can simulate a tumor. The pediatrician is often the first to discover a hepatic mass in a child. The diagnostic gamut varies depending on the child's age. Before the age of three years, the main diagnoses are hepatoblastoma and hemangioma, while after the age of three, hepatocarcinoma, sarcoma, focal nodular hyperplasia, and adenoma are more frequent. The laboratory findings to search for are alpha-fetoprotein whatever the age (increased in hepatoblastoma and hepatocarcinoma), beta-hCG, and urinary catecholamines in infants. Liver function is usually normal. Ultrasonography is the first-line examination to request. It confirms the hepatic location of the mass, differentiates solid from cystic tumors (cystic mesenchymal hamartoma and undifferentiated sarcoma), hypervascular findings (hemangioma in the infant, focal nodular hyperplasia in the older child), portal or hepatic thrombosis suggesting a malignant tumor, and findings of portacaval fistula predisposing to focal nodular hyperplasia and adenoma. At the end of this clinical, biological, and ultrasound examination, the pediatrician will refer the patient to a specialized center for further investigation and management, which are at best performed by pediatric oncologists, surgeons, and radiologists. Diagnostic confirmation and extension work-up will require CT or MRI depending on the patient's age and clinical state and the availability of equipment.

Ovarian tumors in children and adolescents: a series of 41 cases.

OBJECTIVE: Pictorial review with a detailed semiological analysis of ovarian tumors in children and adolescents to provide a relevant diagnostic approach. PATIENTS AND METHODS: Retrospective study (2001-2011) of 41 patients under the age of 15 who underwent surgery for an ovarian mass with a definite pathological diagnosis. RESULTS: Sixty-two percent of the lesions were benign, 33% were malignant and 5% were borderline. Germ cell tumors were most frequent (77.5%), followed by sex cord stromal tumors (12.5%) and epithelial tumors (7.5%). Malignant tumors were more frequent in children between 0 and 2 years old. On imaging, calcifications and fat were specific for germ cell tumors; the presence of a mural nodule was predictive of a mature teratoma (P<0.001). Predictive factors for malignancy were clinical, including abdominal distension (P<0.01) or a palpable mass (P=0.05), biological, including increased hCG and/or AFP levels (P<0.001) and radiological, including tumors larger than 12 cm (P<0.05), tumoral hypervascularity (P<0.01) and voluminous ascites (P<0.01). CONCLUSION: This semiological analysis confirms the role of imaging in diagnosing the etiology of ovarian lesions in children and adolescents and emphasizes the importance identifying tumoral hypervascularity, which, in addition to classic criteria, is highly predictive of malignancy.

[Congenital portosystemic shunts: experience of the western region of France over 5 years]. / Shunts porto-systémiques congénitaux : expérience du Grand-Ouest sur cinq ans.

INTRODUCTION: Congenital portosystemic shunts (CPS) are rare vascular malformations that lead to liver metabolic modifications. The aim of this study was to describe the management and outcome of CPS in children who were followed in the western region of France and to compare our experience to a literature review. PATIENTS AND METHODS: This retrospective study reports nine children with CPS who were followed in the western region of France between 2008 and 2013. RESULTS: Among these nine children, six were detected by prenatal ultrasonography, one had cholestatic jaundice during the postoperative period, one had cholestatic jaundice during the first days of life, and one had dyspnea and fatigue. Two children had liver tumors. The CPS was intrahepatic for four children and extrahepatic for two children. Three others had a patent ductus venosus. Among the intrahepatic shunts that were discovered before the age of 2 years, three closed spontaneously. The five other shunts were closed surgically or by interventional radiology techniques, after angiography for four children. After closure of eight shunts, the outcome was favorable for six children. Two children presented complications after surgery: portal hypertension and portal thrombus. CONCLUSION: When CPS is suspected, the diagnosis should be confirmed and complications should be investigated. Shunt closure restores portal flow and prevents complications.

The long-term outcome of hepatic artery thrombosis after liver transplantation in children: role of urgent revascularization.

Hepatic artery thrombosis (HAT), one of the most severe complications of pediatric orthotopic liver transplantation (OLT), often compromises graft and/or child survival. Of 590 OLT performed in 516 children over a 20-year period, 45 were complicated by early HAT, during the first 2 weeks after transplantation. Systematic Doppler ultrasonographic detection of HAT allowed successful surgical revascularization in 19 instances, resulting in a 20-year graft survival rate of 77% versus 24% of cases when revascularization was not attempted or failed. A combination of surgical emergency revascularization, biliary interventional radiology, biliary surgery and/or retransplantation resulted in an 80% 20-year patient survival rate, identical to that of transplanted children who did not experience early HAT. The majority of long-term survivors with their initial graft had normal liver tests, no biliary dilation on ultrasonography and minimal or moderate fibrosis on liver histology. A failed attempt at revascularization did not significantly alter patient survival. Despite these encouraging results, for the children and their parents to overcome the entire process in terms of reoperations, repeated radiological interventions, number of hospitalizations and emotional stress, remains an ordeal of such magnitude that it justifies renewed efforts to progress in the prevention of this complication.

Congenital portosystemic shunts in children: recognition, evaluation, and management.

Congenital portosystemic shunts are present in one in 30,000 children. Among the associated risks of severe complications are neonatal cholestasis, benign and malignant liver tumors, hepatopulmonary syndrome, portopulmonary hypertension, and encephalopathy. They can be detected on prenatal ultrasonograms, during the investigation of a positive galactosemia screening test in neonates or of a complication, or be found fortuitously on an abdominal ultrasound. Small intrahepatic shunts may resolve spontaneously within one year of age, but other shunts such as extrahepatic, persistent ductus venosus or persisting intrahepatic shunts, must be closed in one or two steps, by interventional radiology techniques or surgically. The plasticity of the intrahepatic portal system allows revascularization of the liver after shunt closure, even when no intrahepatic portal structures can be detected on imaging studies. This leaves little or no place for liver transplantation in the management of these children.

[What is the value of the chest radiography in making the diagnosis of children pneumonia in 2011?]. / Quel est l'intérêt de la radiographie du thorax dans le diagnostic d'une pneumonie de l'enfant en 2011 ?

UNLABELLED: According to the French national health authority guidelines, the diagnosis of childhood pneumonia is based on clinical suspicion and radiological confirmation. The chest radiography is an expensive procedure, and potentially deleterious by its radiating character. We have attempted to clarify its diagnostic value in community acquired pneumonia in children through a literature review using the Pubmed search engine with the following keywords: "pneumonia, child, radiograph". CONCLUSION: The indication of chest radiography in severe pneumonia achieved unanimity among the various scientific societies. In contrast, in mild forms, tendency of the available data is to not recommend the routine use of chest radiography; further randomised and prospective studies are necessary to confirm this trend. Finally, because of the frequency of atypical presentations in children, chest radiography retains all its usefulness in the etiologic diagnosis of fever of unknown origin.

[Observatory of the elderly over 80 years supported by the mobile emergency and resuscitation service]. / Observatoire du sujet âgé de plus de 80 ans pris en charge en urgence par le service mobile d'urgence et de réanimation.

Emergency medical services (EMS) received an increasing number of calls for patients aged 80 and older. The goal of the present study was to evaluate outcome and functional dependence of patients aged 80 and older who EMS managed in the prehospital theater. This prospective study was conducted over 1 year (September 2007-August 2008), all consecutive patients aged 80 and older managed by a medical team during the study period were included. Characteristics of patients, including previous health status and functional dependence, were recorded on-the scene by the attending physician. Three-month mortality was recorded, as well as ADL score. Data are expressed as mean values±standard deviations, medians and interquartile ranges (IQRs), and percentages and compared using univariate and multivariate analysis. P<0.05 was considered the threshold for significance. Five hundred twenty-three patients were included. Mean age was 86 ± 5. Median ADL index was 2 (IQR 0-9), and 63% of patients were living at home. At 3 months, the survival rate was 66% (n=273) and the proportion of patients living at home was 64% (P=0.9), the median ADL index of survivors was 2 (IQR 0-8) vs 1 (IQR 0-6) initially for this subpopulation, P=0.01. Our study confirms utility and efficacy of full access of elderly persons to advanced life support especially for self-patients and not restricted based on aging per se. The development and daily use of tools for rapid assessment of autonomy should enable practitioners to innovate and thus, adapt their management.

[Transrectal drainage of pediatric pouch of Douglas abscesses under suprapubic US guidance: technique and results]. / Drainage par voie transrectale sous contrôle échographique sus-pubien des abcès du douglas chez l'enfant: technique et résultats.

Pelvic abscesses are not always amenable to percutaneous drainage. Transrectal drainage of pouch of Douglas abscesses, performed blindly by surgeons for many years, is not familiar to radiologists. The purpose of this article is to describe our technique for the drainage of pouch of Douglas abscesses under suprapubic US guidance and report our results in a series of 7 pediatric patients with deep pelvic abscess not amenable to percutaneous drainage after failure of antibiotics. The procedure was performed under general anesthesia. All procedures were successful and without complication. The drainage catheter was well tolerated in all cases with short post-drainage hospital stay. After failure of medical management, this technique can be used irrespective of patient age or sex using US transducers and drainage catheters routiney available in any radiology department.

[Results of a national survey about the use of sedation scales in emergency prehospital medicine]. / Résultats d'une enquête nationale évaluant l'utilisation des scores de sédation en médecine préhospitalière.

The primary goal of sedation in emergency prehospital care is to guarantee the security of the mechanically ventilated patients by optimising their adaptation to the respirator. If the French prehospital guidelines are well codified, their applicability in routine clinical practice seem to be rather empirical. The aim of this national survey was to evaluate the use of the clinical sedation scales by the prehospital physicians. This prospective and clinical practice survey was begun in January 2005. An anonymous questionnaire was sent to the physicians working in the 377 Mobile Intensive Care Unit of the 105 French Emergency Medical Service System. The total response rate from physicians was 28% (n=497). Only 29% of the physicians (n=145) declared to use a sedation scale for a mechanically ventilated patient. The Ramsay score was used in 97% of the cases (n=141).The principal reasons given by the physicians for not using the sedation scales were their ignorance in 57% of the cases (n=200) and the systematic choice of a deep sedation in 42% of the cases (n=147). For 18% of them (n=62), the use of sedation scores was considered too complicated. The final results show that the utilisation ratio of the sedation scores is very low in emergency prehospital medicine and suggest that an effort toward improving the use of sedation in prehospital emergency medicine is necessary.

Acquired renal cystic disease after liver transplantation in children.

To our knowledge, the development of renal cystic disease that may contribute to kidney dysfunction has never been reported after liver transplantation. Herein we have reported on the fortuitous finding of renal cystic lesions upon computed tomographic scans (CT) in 33 (30%) of 108 pediatric liver transplant recipients who were the subjects of a prospective study evaluating long-term kidney dysfunction at 10 years after liver transplantation. The renal lesions had 2 different appearances: that of simple renal cysts and that of round lesions that were spontaneously hyperdense before contrast injection. These high-density lesions had a low signal on T2 weighted sequences, but 70% of them had been missed at ultrasonography. Their aspect upon CT and magnetic resonance favored cystic lesions filled with hemorrhagic or milk calcium content. Both types of cystic lesions were associated in 14 children. The renal lesions were significantly associated with moderate renal dysfunction, biopsy-proven chronic liver graft rejection, and thrombosis of the retrohepatic vena cava. The physiopathology of these lesions is undetermined. Two important questions need to be clarified with respect to the risk of progression of renal dysfunction associated with individual volume changes and/or increased number of renal cysts, as well as the risk of renal cancer as has been reported in dialyzed patients with acquired cystic kidney disease.

[Noninfectious febrile inflammatory syndromes in children: diagnosis and usefulness of diagnostic procedures]. / Syndromes inflammatoires fébriles non infectieux de l'enfant: diagnostic, contribution des examens complémentaires.

OBJECTIVE: To determine the causes and to quantify the benefits obtained from further diagnostic investigations in children presenting with a non infectious inflammatory fever. METHODS: The records of 62 children aged from two-months to 15 years (median: four years) admitted to a paediatric department between 1990 and 2000 for the evaluation of a fever associated to an inflammatory syndrome, defined as temperature over 38 degrees C with an increase of the erythrocyte sedimentation rate (ESR) more than 20 mm/h and/or a serum C-reactive protein level (CRP) > 20 mg/L, and excluding overt infectious diseases, were retrospectively reviewed. RESULTS: Of these patients, 79% children (49 cases) had inflammatory systemic disease, 3.2% (two cases) had malignancy, and 17.8% (11 cases) had undiagnosed disorders. The most frequent disease was Kawasaki disease (22 children), especially in young children. Increase of ESR above 100 mm/h and of CRP above 100 mg/L was present in 59% of Kawasaki disease, 71% of idiopathic juvenile arthritis, 100% of malignancies and 7% of unknown diagnoses. Increase of ESR below 50 mm/h and of CRP below 50 mg/L was present in 75% of hemophagocytic syndromes and 46% of unknown diagnosis. The polymorphonuclear count, hepatic function evaluation, triglycerides levels, abdominal ultrasound, abdominal computed tomography, echocardiography, biopsies were useful diagnosis tools. Technetium scintigraphy was helpful only when abnormalities were found on physical examination. CONCLUSION: The diagnosis of Kawasaki disease must be quickly suspected in febrile young children with inflammatory syndrome without infection. ESR and CRP values, abdominal ultrasound and echocardiography are helpful tools for the diagnostic procedure.

[Sonography of the pediatric abdomen: liver and biliary tract]. / Echographie abdominale de l'enfant: foie et voies biliaires.

Ultrasound is a routine imaging modality that is quite valuable to assess the pediatric abdomen. The objective of this article is to describe the sonographic appearance of the liver and bile ducts in children. Our goal is to emphasize normal variants and specific pediatric diseases, even if some of them may be encountered in adults.

[Cyclic filling cystourethrography in the study of febrile urinary tract infection in children]. / Cystographie à remplissages répétés dans l'exploration des infections urinaires fébriles chez l'enfant.

PURPOSE: To assess the value of cyclic voiding cystourethrography (VCUG) for the detection of reflux in a large population of children with urinary tract infection. MATERIALS AND METHODS: 234 patients (67% less than 3 years of age) underwent VCUG with two fillings in 214 cases and three fillings in 139 cases. Appearance or increase in the grade of reflux compared with previous filling was expressed as "modification of the radiology report" (MRR). The amount of contrast material, the duration of fluoroscopy and the number of films were recorded. RESULTS: The rate of reflux was 18.4% at the first filling, 16% at the second filling with 9.8% MRR and 14.7% at the third filling with 10% MRR. These results were the same for children younger and older than 3 years. For children under three years, if one considered reflux grade higher than 1, the MRR was 9.6% at the second filling and 7.5% at the third while it was 5.8% and 3% for older children. On average, the use of contrast material increased 50% with a third filling, number of films was not modified and the time of fluoroscopy increased by 6 sec per filling. CONCLUSION: VCUG is recommended in all children. The detection of reflux higher than grade 1 is more frequent with multiple fillings in children under 3 years. The increase in radiation exposure and cost seems negligible.