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Introduction: Venous thromboembolism (VTE) poses a significant challenge in cancer patients, particularly those with advanced malignancies. The management of recurrent VTE is complicated by the need for effective anticoagulation while addressing the underlying cancer progression., Cases: We present two clinical cases from the gastroenterology department at Percy French military hospital involving patients with progressive malignant digestive diseases. Patient 1, a 62-year-old woman, developed recurrent pulmonary embolism despite appropriate anticoagulation with low molecular weight heparin (LMWH). After treatment adjustments, she ultimately succumbed to tumor progression. Patient 2, a 54-year-old man hospitalized for pulmonary embolism, faced upper gastrointestinal bleeding and delayed anticoagulation initiation. Although he showed initial improvement with immunotherapy and stabilization of thrombotic events, he experienced oncological progression and recurrent VTE, leading to palliative care., Discussion: These cases illustrate the difficulties of managing recurrent VTE, even with curative anticoagulation and dose escalation. In case of VTE recurrence, it is essential to investigate for cancer progression and ensure patient adherence to treatment. A comprehensive management strategy should involve both the malignancy and the thrombotic complications., Conclusion: The management of recurrent VTE in cancer patients requires a multidisciplinary approach to evaluate the benefit-risk ratio of anticoagulation adjustments. These clinical cases highlight the necessity for integrated care that addresses both oncological and thrombotic concerns, emphasizing the importance of timely intervention and collaboration among healthcare providers., (Copyright © 2024 Elsevier Masson SAS. All rights reserved.)

Introduction: Osteoid osteoma is a benign osteogenic tumour traditionally treated by surgical excision or percutaneous CT-guided procedures. We describe three cases of osteoid osteomas of which the locations were difficult to access, or for which the procedure was potentially unsafe, involving treatment with zoledronic acid infusions., Case Description: We report here three male 28-to-31-year-old patients with no medical history who had osteoid osteomas located at the second cervical vertebra, the femoral head, and the third lumbar vertebra respectively. These lesions were responsible for inflammatory pain requiring daily treatment with acetylsalicylic acid. Given the impairment risk, all of the lesions were ineligible for surgical or percutaneous treatment. Patients were successfully treated by 3 to 6 monthly zoledronic acid infusions. All patients experienced complete relief of their symptoms allowing aspirin discontinuation, without any side effects. In the first two cases, CT and MRI control showed nidus mineralization and bone marrow oedema regression, correlating with the pain decrease. After 5years of follow-up, there had been no recurrence of the symptoms., Conclusion: In these patients, monthly 4mg zoledronic acid infusions have been safe and effective in the treatment of inaccessible osteoid osteomas., (Copyright © 2023 Société française de rhumatologie. Published by Elsevier Masson SAS. All rights reserved.)

Drosophila insulators were the first DNA elements found to regulate gene expression by delimiting chromatin contacts. We still do not know how many of them exist and what impact they have on the Drosophila genome folding. Contrary to vertebrates, there is no evidence that fly insulators block cohesin-mediated chromatin loop extrusion. Therefore, their mechanism of action remains uncertain. To bridge these gaps, we mapped chromatin contacts in Drosophila cells lacking the key insulator proteins CTCF and Cp190. With this approach, we found hundreds of insulator elements. Their study indicates that Drosophila insulators play a minor role in the overall genome folding but affect chromatin contacts locally at many loci. Our observations argue that Cp190 promotes cobinding of other insulator proteins and that the model, where Drosophila insulators block chromatin contacts by forming loops, needs revision. Our insulator catalog provides an important resource to study mechanisms of genome folding.

Purpose: Anal canal duplication is a rare malformation characterized by a second perineal opening positioned behind the anus, which is generally observed at 6 o'clock in the lithotomy position. The purpose was to describe six new cases of anal canal duplication (in addition to our previously reported series of six patients) with the aim of providing further clinical information about this anomaly., Methods: We described 6 new cases of anal canal duplication in terms of symptoms, anatomical disposition, imaging results, and histopathology. Clinical details of these cases and those already reported (n = 12) were summarized and compared to existing literature., Results: A total of 12 cases were reported over 34 years. 17% of the patients were male, constituting the only subgroup to present a communication with the digestive tract. A single patient, diagnosed at 12 years, was symptomatic. Half of the patients had at least one associated malformation. All patients underwent surgery, either with a posterior sagittal or perineal approach., Conclusion: Diagnosis of anal canal duplication should be suspected when a perineal opening positioned behind the anus is present, and necessitates further exploration by a comprehensive clinical examination and imaging. Surgery is always required, typically performed via a posterior sagittal approach. The postoperative course is usually uncomplicated.

The exceptional health challenges generated by the COVID-19 pandemic have sparked a wave of mobilization among medical students. We were four students to provide support to the Migrant Care Units of the Vulnerability and Social Medicine Department of Unisanté. This two-month experience, very different from the academic framework we were used to during our studies, allowed us to discover a clinic grappling with social and cross-cultural issues, and taught us the mutual benefits of an interprofessional collaboration with experienced nurses. Through a few clinical vignettes, we would like to share the challenges of the care we have seen, as well as the significant lessons we have learned from them., Competing Interests: Les auteurs n’ont déclaré aucun conflit d’intérêts en relation avec cet article.

2'-O-Methylation (Nm) represents one of the most common RNA modifications. Nm affects RNA structure and function with crucial roles in various RNA-mediated processes ranging from RNA silencing, translation, self versus non-self recognition to viral defense mechanisms. Here, we identify two Nm methyltransferases (Nm-MTases) in Drosophila melanogaster (CG7009 and CG5220) as functional orthologs of yeast TRM7 and human FTSJ1. Genetic knockout studies together with MALDI-TOF mass spectrometry and RiboMethSeq mapping revealed that CG7009 is responsible for methylating the wobble position in tRNAPhe, tRNATrp and tRNALeu, while CG5220 methylates position C32 in the same tRNAs and also targets additional tRNAs. CG7009 or CG5220 mutant animals were viable and fertile but exhibited various phenotypes such as lifespan reduction, small RNA pathways dysfunction and increased sensitivity to RNA virus infections. Our results provide the first detailed characterization of two TRM7 family members in Drosophila and uncover a molecular link between enzymes catalyzing Nm at specific tRNAs and small RNA-induced gene silencing pathways., (© The Author(s) 2020. Published by Oxford University Press on behalf of Nucleic Acids Research.)

Objective: To identify predictors of and factors associated with the performance of antireflux surgery during the first year of life in children born with esophageal atresia., Study Design: All patients were included in a French registry for esophageal atresia. All 38 multidisciplinary French centers completed questionnaires about perinatal characteristics and one-year outcome for children born with esophageal atresia., Results: Of 835 infants with esophageal atresia born in France from 2010 to 2014, 682 patients, excluding those with long-gap esophageal atresia, were included. Three patients had type I, 669 had type III, and 10 had type IV esophageal atresia. Fifty-three children (7.8%) received fundoplication during the first year of life. The median age at the time of the end-to-end esophageal anastomosis was 1.1 day (range 0-15). Multivariate analysis identified three perioperative factors that predicted the need for early antireflux surgery: anastomotic tension (P = .004), associated malformations (P = .019), and low birth weight (P = .018). Six other factors, measured during the first year of life, were associated with the need for antireflux surgery: gastroesophageal reflux (P < .001), anastomotic stricture (P < .001), gastrostomy (P < .001), acute life-threatening event (P = .002), respiratory complications (P = .045), and poor nutritional status (P < .001)., Conclusions: Gastroesophageal reflux disease, low birth weight, poor nutrition, and surgical anastomosis difficulties predicted the performance of antireflux surgery in the first year of life in infants with esophageal atresia., (Copyright © 2019 Elsevier Inc. All rights reserved.)

Glomous tumors are rare and benign, generally affecting the fingers. Other localizations have nevertheless been described. We report the case of a patient who presented a supra-patellar glomous tumor provoking a pain-induced limp. Magnetic resonance imaging confirmed the diagnosis. The patient underwent complete surgical resection of the tumor followed by total resolution of the pain. Glomous tumors in an atypical localization may go unnoticed, with the risk of late or erroneous diagnosis. Symptoms are easily resolved with simple resection., (Copyright © 2019. Published by Elsevier Masson SAS.)

The transition from a pediatrician to a general practitioner builds on a strong relationship with the pediatrician. Informed consent and decision making in children and adolescents are greatly improved by a trustful relationship with healthcare providers. In Switzerland, minors can access their medical file without parental consent if considered competent. Electronic files can be helpful but may lead to breeches in confidentiality : transmission of data concerning the family or parent's health status not relevant for the adolescent's medical follow-up, parental access to the medical files. A simple transmission file can be given to the adolescent at the end of pediatric follow up. Visits to mark the end of the pediatric follow-up and the beginning of the GP follow-up should be systematically offered to young people with special needs., Competing Interests: Les auteurs n’ont déclaré aucun conflit d’intérêts en relation avec cet article.

Deciphering the rules of genome folding in the cell nucleus is essential to understand its functions. Recent chromosome conformation capture (Hi-C) studies have revealed that the genome is partitioned into topologically associating domains (TADs), which demarcate functional epigenetic domains defined by combinations of specific chromatin marks. However, whether TADs are true physical units in each cell nucleus or whether they reflect statistical frequencies of measured interactions within cell populations is unclear. Using a combination of Hi-C, three-dimensional (3D) fluorescent in situ hybridization, super-resolution microscopy, and polymer modeling, we provide an integrative view of chromatin folding in Drosophila . We observed that repressed TADs form a succession of discrete nanocompartments, interspersed by less condensed active regions. Single-cell analysis revealed a consistent TAD-based physical compartmentalization of the chromatin fiber, with some degree of heterogeneity in intra-TAD conformations and in cis and trans inter-TAD contact events. These results indicate that TADs are fundamental 3D genome units that engage in dynamic higher-order inter-TAD connections. This domain-based architecture is likely to play a major role in regulatory transactions during DNA-dependent processes.

Small RNAs are potent regulators of gene expression. They also act in defense pathways against invading nucleic acids such as transposable elements or viruses. To counteract these defenses, viruses have evolved viral suppressors of RNA silencing (VSRs). Plant viruses encoded VSRs interfere with siRNAs or miRNAs by targeting common mediators of these two pathways. In contrast, VSRs identified in insect viruses to date only interfere with the siRNA pathway whose effector Argonaute protein is Argonaute-2 (Ago-2). Although a majority of Drosophila miRNAs exerts their silencing activity through their loading into the Argonaute-1 protein, recent studies highlighted that a fraction of miRNAs can be loaded into Ago-2, thus acting as siRNAs. In light of these recent findings, we re-examined the role of insect VSRs on Ago-2-mediated miRNA silencing in Drosophila melanogaster. Using specific reporter systems in cultured Schneider-2 cells and transgenic flies, we showed here that the Cricket Paralysis virus VSR CrPV1-A but not the Flock House virus B2 VSR abolishes silencing by miRNAs loaded into the Ago-2 protein. Thus, our results provide the first evidence that insect VSR have the potential to directly interfere with the miRNA silencing pathway.

Background: The aim of the present national prospective population-based study was to assess the early morbidity of esophageal atresia (EA)., Methods: All 38 multidisciplinary French centers that care for patients with EA returned a specific questionnaire about the 1-year outcome for each patient. This information was centralized, checked, and entered into a database., Results: From the total population of 307 EA patients born in 2008 and 2009, data about the 1-year outcome were obtained from 301 (98%) patients, of whom 4% were lost to follow-up and 5% died. Medical complications occurred in 34% of the patients: anastomotic leaks (8%), recurrent tracheoesophageal fistula (4%), and anastomotic stenosis (22%); all of the latter group needed dilation (median, 2 dilations/patient). A new hospitalization was required for 59% of patients (2.5 hospitalizations/patient) for digestive (52%) or respiratory (48%) reasons. Twelve percent of patients required antireflux surgery at a median age of 164 days (range, 33-398 days), and 1% underwent an aortopexy for severe tracheomalacia. The weight/age Z-score was -0.8 (range, -5.5 to 3.7 months) at 12 months. Fifteen percent of patients were undernourished at 12 months of age, whereas 37% presented with respiratory symptoms and 15% had dysphagia at the last follow-up. Significant independent factors associated with medical complications were anastomotic esophageal tension (p = .0009) and presence of a gastrostomy (p = .0002); exclusive oral feeding at discharge was associated with a decreased risk of complications (p = .007)., Conclusions: Digestive and respiratory morbidities remain frequent during the first year of life and are associated with difficult anastomosis and lack of full oral feeding.

Background: Spatially resolved exposure models are increasingly used in epidemiology. We previously reported that, although exhibiting a moderate correlation, pregnancy nitrogen dioxide (NO2) levels estimated by the nearest air quality monitoring station (AQMS) model and a geostatistical model, showed similar associations with infant birth weight., Objectives: We extended this study by comparing a total of four exposure models, including two highly spatially resolved models: a land-use regression (LUR) model and a dispersion model. Comparisons were made in terms of predicted NO2 and particle (aerodynamic diameter<10 μm, PM10) exposure and adjusted association with birth weight., Methods: The four exposure models were implemented in two French metropolitan areas where 1026 pregnant women were followed as part of the EDEN mother-child cohort., Results: Correlations between model predictions were high (≥ 0.70), except for NO2 between the AQMS and both the LUR (r = 0.54) and dispersion models (r = 0.63). Spatial variations as estimated by the AQMS model were greater for NO2 (95%) than for PM10 (22%). The direction of effect estimates of NO2 on birth weight varied according to the exposure model, while PM10 effect estimates were more consistent across exposure models., Conclusions: For PM10, highly spatially resolved exposure model agreed with the poor spatial resolution AQMS model in terms of estimated pollutant levels and health effects. For more spatially heterogeneous pollutants like NO2, although predicted levels from spatially resolved models (all but AQMS) agreed with each other, our results suggest that some may disagree with each other as well as with the AQMS regarding the direction of the estimated health effects., (Copyright © 2014 Elsevier Ltd. All rights reserved.)

Defects in miRNA biogenesis or activity are associated to development abnormalities and diseases. In Drosophila, miRNAs are predominantly loaded in Argonaute-1, which they guide for silencing of target RNAs. The miRNA pathway overlaps the RNAi pathway in this organism, as miRNAs may also associate with Argonaute-2, the mediator of RNAi. We set up a gene construct in which a single inducible promoter directs the expression of the GFP protein as well as two miRNAs perfectly matching the GFP sequences. We show that self-silencing of the resulting automiG gene requires Drosha, Pasha, Dicer-1, Dicer-2 and Argonaute-2 loaded with the anti-GFP miRNAs. In contrast, self-silencing of the automiG gene does not involve Argonaute-1. Thus, automiG reports in vivo for both miRNA biogenesis and Ago-2 mediated silencing, providing a powerful biosensor to identify situations where miRNA or siRNA pathways are impaired. As a proof of concept, we used automiG as a biosensor to screen a chemical library and identified 29 molecules that strongly inhibit miRNA silencing, out of which 5 also inhibit RNAi triggered by long double-stranded RNA. Finally, the automiG sensor is also self-silenced by the anti-GFP miRNAs in HeLa cells and might be easily used to identify factors involved in miRNA biogenesis and silencing guided by perfect target complementarity in mammals.

Accidental foreign body ingestion by children is not exceptional and occurs in most cases in children less than 5 years old. The impact is not well known. We present the case of a 31-month-old boy affected by peritonitis caused by several bowel perforations after swallowing three magnets at different times. A single foreign body magnetic ingestion does not often cause trouble, but if the ingestions are spaced out, then consequential complications can occur. This is why, in case of stomachaches in a child less than 3 years or a child with behavior problems, it is important to perform standard abdominal X-ray examination., (Copyright © 2012. Published by Elsevier SAS.)

The rise in obesity in many countries has led to the emergence of nutritional information policies that aim to change people's diets. Changing an individual's diet is an ambitious goal, since numerous factors influence a person's food-choice decisions, many of which are made unconsciously. These frequently subconscious processes should not be underestimated in food-choice behavior, as they play a major role in food diet composition. In this review, research in cognitive experimental psychology and neuroscience provides the basis for a critical analysis of the role of pleasure in eating behaviors. An assessment of the main characteristics of nutritional policies is provided, followed by recent findings showing that food choices are guided primarily by automatic emotional processes. Neuroimaging and behavioral studies, which provide new insights into the relationships between emotions and food both in lean persons and in persons with eating disorders, are reported as well. Lastly, the argument is presented that future nutritional policies can be more effective if they associate healthy food with eating pleasure., (© 2012 International Life Sciences Institute.)

When listening to speech in everyday-life situations, our cognitive system must often cope with signal instabilities such as sudden breaks, mispronunciations, interfering noises or reverberations potentially causing disruptions at the acoustic/phonetic interface and preventing efficient lexical access and semantic integration. The physiological mechanisms allowing listeners to react instantaneously to such fast and unexpected perturbations in order to maintain intelligibility of the delivered message are still partly unknown. The present electroencephalography (EEG) study aimed at investigating the cortical responses to real-time detection of a sudden acoustic/phonetic change occurring in connected speech and how these mechanisms interfere with semantic integration. Participants listened to sentences in which final words could contain signal reversals along the temporal dimension (time-reversed speech) of varying durations and could have either a low- or high-cloze probability within sentence context. Results revealed that early detection of the acoustic/phonetic change elicited a fronto-central negativity shortly after the onset of the manipulation that matched the spatio-temporal features of the Mismatch Negativity (MMN) recorded in the same participants during an oddball paradigm. Time reversal also affected late event-related potentials (ERPs) reflecting semantic expectancies (N400) differently when words were predictable or not from the sentence context. These findings are discussed in the context of brain signatures to transient acoustic/phonetic variations in speech. They contribute to a better understanding of natural speech comprehension as they show that acoustic/phonetic information and semantic knowledge strongly interact under adverse conditions., (Copyright © 2010 Elsevier Inc. All rights reserved.)

Mixed epithelial and stromal tumour of the kidney (MESTK) is a rare kidney neoplasm that occurs almost exclusively in perimenopausal women. Long-term oestrogen replacement appears to play a major role in its pathogenesis. Around 70 cases have been described in the international literature, none of which involve male children. Herein, we describe an atypical case of MESTK diagnosed in a 12-year-old prepubertal boy who presented with hematuria. Pathology and immunohistochemistry revealed a typical MESTK. The child was free of disease at 2-year follow up after a partial nephrectomy and tumour excision., (Crown Copyright (c) 2009. Published by Elsevier Ltd. All rights reserved.)

A French foreign legionnaire returning from Djibouti developed feverish polyarthritis with acral purpura. Diagnostic workup demonstrated gonococcemia contracted during unprotected fellatio. Based on this case report, diagnostic and therapeutic management is described.

Multicellular organisms evolved sophisticated defence systems to confer protection against pathogens. An important characteristic of these immune systems is their ability to act both locally at the site of infection and at distal uninfected locations. In insects, such as Drosophila melanogaster, RNA interference (RNAi) mediates antiviral immunity. However, the antiviral RNAi defence in flies seems to be a local, cell-autonomous process, as flies are thought to be unable to generate a systemic RNAi response. Here we show that a recently defined double-stranded RNA (dsRNA) uptake pathway is essential for effective antiviral RNAi immunity in adult flies. Mutant flies defective in this dsRNA uptake pathway were hypersensitive to infection with Drosophila C virus and Sindbis virus. Mortality in dsRNA-uptake-defective flies was accompanied by 100-to 10(5)-fold increases in viral titres and higher levels of viral RNA. Furthermore, inoculating naked dsRNA into flies elicited a sequence-specific antiviral immune response that required an intact dsRNA uptake pathway. These findings suggest that spread of dsRNA to uninfected sites is essential for effective antiviral immunity. Notably, infection with green fluorescent protein (GFP)-tagged Sindbis virus suppressed expression of host-encoded GFP at a distal site. Thus, similar to protein-based immunity in vertebrates, the antiviral RNAi response in flies also relies on the systemic spread of a virus-specific immunity signal.

Introduction: The ex utero intrapartum treatment (EXIT) procedure uses uteroplacental gas exchange after caesarean section for foetus oxygenation. This placental support allows establishing an airway in the newborn in case of obstruction. Maintenance of placental perfusion requires uterine relaxation. A halogenated agent is classically use while nitroglycerine is used in the modified EXIT procedure., Case Report: We present the case of a newborn with a giant thyroid teratoma diagnosed on ultrasound at 20 weeks' gestation. At 32 weeks' gestation, a modified EXIT procedure was performed. The EXIT was successful and newborn was operated on the following day. Currently, the child is 32-month-old, has no sequelae and benefits from thyroid hormone substitution., Discussion: The EXIT procedure allows managing airway obstruction, even when complex, at birth. Nevertheless, prolonged uterine relaxation increases the risk of flooding. Because of its short half-life, nitroglycerine reduces this risk. In this case report we chose the modified EXIT procedure because the mother was young and primipara and foetal prognosis was poor., Conclusion: Currently, the EXIT procedure is the technique of choice in the management of a foetal cervical mass. An exhaustive preoperative foetal workup is necessary to choose the classical or modified EXIT procedure.

Drosophila Gcn5 is the catalytic subunit of the SAGA and ATAC histone acetylase complexes. Here, we show that mutations in Gcn5 and the ATAC component Ada2a induce a decondensation of the male X chromosome, similar to that induced by mutations in the Iswi and Nurf301 subunits of the NURF nucleosome remodelling complex. Genetic studies as well as transcript profiling analysis indicate that ATAC and NURF regulate overlapping sets of target genes during development. In addition, we find that Ada2a chromosome binding and histone H4-Lys12 acetylation are compromised in Iswi and Nurf301 mutants. Our results strongly suggest that NURF is required for ATAC to access the chromatin and to regulate global chromosome organization.

Polyorchidism is an urogenital curiosity defined by the presence of more than two testes confirmed by histology. This anomaly is extremely rare and only a hundred world cases were described in the literature. Although it can remain asymptomatic, polyorchidism is often associated to processus vaginalis anomalies in childhood (hernia, hydrocele) and undescended testis. The review of the literature finds cases of polyorchidism revealed by testis torsion and an increased risk of malignancy and infertility. We report the case of polyorchidism in a 14 year old child, at whom a painful testis mass indicate surgical exploration and documented the histological diagnosis of polyorchidism. Through this observation and review of the literature, authors describe pathological and managment findings of polyorchidism.

Neonatal splenic injury is a rare but serious condition, due to the risk of haemorrhagic shock. We report on the case of a newborn infant with a neonatal respiratory distress that first evoked materno-fetal infection. Clinical deterioration, with anemia and abdominal distension, led then to the proper diagnosis. Dystocia seems to be the most likely cause of the splenic rupture in this report. Medical treatment is advocated as first line, while surgical treatment may be necessary in some cases. In the case surgery is inevitable, a conservative approach is preferable.

Intestinal perforations in preterm newborn are characterized with high morbidity and mortality rates. They often are associated with necrotizing enterocolitis and seldom correspond to idiopathic spontaneous intestinal perforation. Perforations upstream of an intestinal organic obstruction (atresia), or of a functionnal obstruction (meconium-ileus, Hirschsprung disease) have been considered to be rare in preterm newborns. We report a case of caecal perforation with a pneumoperitoneum which occurred at 5 days of life, in a 28-week gestational age infant, that was treated by cecostomy. There were no signs of necrotizing enterocolitis. At 43 days of life, the preoperative contrast enema study revealed a left colon transition zone, suggesting a Hirschsprung disease, which was confirmed by rectal biopsies. Transanal pull-through was performed. There were no postoperative complication. This case demonstrates that an isolated intestinal perforation in very preterm newborn can reveal a Hirschsprung disease, especially if it occurs in the caecum.

The transverse colon is an exceptional location of intestinal duplication. Perforated duplications are rarely described in neonates. Meconium peritonitis (MP) can originate from prenatal perforated intestinal duplication. The authors report a case of a baby girl with prenatal diagnosis of MP. Rapid worsening of clinical aspects at birth and the presence of a pneumoperitoneum on systematic abdominal plain radiographs led to urgent surgery on the 1st day of life. Laparotomy showed a perforated necrotizing tubular duplication of the transverse colon. Removal of the duplication followed by limited segmental colonic resection and double colostomy were carried out. Follow-up was uneventful.

A 5 month-old baby developed non-ceasing intra-peritoneal bleeding after extensive surgical biopsy for an hepatoblastoma. A single recombinant activated factor VII injection following enlarged hepatectomy helped to resolve quickly this life-threatening haemorrhagic syndrome.

Background: Intussusception due to lymphoma is a challenging condition for pediatric surgeons. The aim of this study is to report seven cases of this entity and to discuss its management., Case Report: Six boys and one girl, 3-15-years-old, were admitted for intussusception secondary to a lymphoma. All patients underwent laparotomy: biopsy of massive abdominal tumor 6 and 8 weeks following resection of an intussusception (two cases), ileal resection of non-reductible intussusception (one case), right hemicolectomy for tumor of the appendix (one case), tumorectomy of localized ileal tumor (two cases), enlarged mesenteric lymph node biopsy associated with simple reduction of intussusception (one case). All children were successfully treated with protocol chemotherapy with a 15-month to 13-year follow-up. No relapse was observed., Conclusion: Surgeons should be aware of operative sights of ileal lymphomas. Diagnosis of lymphoma may be difficult after manual reduction of intussusception. A sample of any abnormality (mesenteric lymph node, peritoneal fluid) should be taken. Intestinal resection allows to reduce the intensity of chemotherapy but must be as limited as possible: ileal resection in cases of complicated intussusception, tumorectomy "in sano" in cases of ileal parietal isolated tumor. Reduction of intussusception alone (with no resection of ileal tumor) seems to be effective if diagnosis of lymphoma is possible from peripheral samples (peritoneal fluid, pleural effusion, mesenteric lymph node, bone marrow biopsy...).

Purpura fulminans is a thrombotic disease that can occur during infections, disseminated intravascular coagulation or in the context of an acquired or congenital protein C or S deficiency. Here we report the case of a 4-year-old child who developed, 5 days after a chickenpox infection, large painful ecchymotic, necrotizing and retiform plaques on the lower extremities. Laboratory analyses revealed very low protein S levels as well as anticardiolipin antibodies. Aggressive treatment by low-molecular-weight heparin, steroids, intravenous immunoglobulins and fresh frozen plasma was able to prevent the extension of the lesions and to correct the coagulation abnormalities. No lesions required skin grafting. As in our patient, an acquired protein S deficiency is probably responsible for most cases of purpura fulminans occurring after varicella, but the concomitant presence of antiphospholipid antibodies may also play a role., (Copyright 2004 S. Karger AG, Basel)

Introduction: Leydig cell hyperplasia (LCH) of the testis is rarely described in children. The authors report a case of incidental discovery of LCH on ultrasound examination., Case Report: The authors report the case of a 9-year-old boy presenting with isolated and painless increased volume of the left testis, with no clinically palpable mass. Scrotal ultrasound revealed an echogenic mass, 12 mm in diameter, with a solid appearance and several hypoechoic areas, without calcification, situated in the lower pole of the testis. Tumour markers and gonadotropin axis hormonal assessment were normal. On surgical exploration, the testis had a macroscopically normal appearance; opening of the tunica albuginea revealed the lesion and enucleation was performed. Histological examination confirmed the presence of LCH, 5 mm in diameter. The postoperative course was uneventful. Physical examination and scrotal ultrasound have remained normal with a follow-up of two years., Discussion: The authors recall the characteristics of LCH, which usually presents, in children, in the form of signs of precocious puberty or more rarely by gynaecomastia. The lesion is rarely palpable. Scrotal ultrasound reveals a homogeneous mass with several hypoechoic nodules. An endocrine assessment must always be performed (frequent elevation of LH). Surgery should be as conservative as possible (enucleation-resection). Histological diagnosis may be difficult.

The transfer of anti-human cytomegalovirus (HCMV) effector T cells to allogeneic bone marrow recipients results in protection from HCMV disease associated with transplantation, suggesting the direct control of CMV replication by T cells. IE1 and pp65 proteins, both targets of CD4(+) and CD8(+) T cells, are considered the best candidates for immunotherapy and vaccine design against HCMV. In this report, we describe the purification of a 165-kDa chimeric protein, IE1-pp65, and its use for in vitro stimulation and expansion of anti-HCMV CD4(+) and CD8(+) T cells from peripheral blood mononuclear cells (PBMC) of HCMV-seropositive donors. We demonstrate that an important proportion of anti-HCMV CD4(+) T cells was directed against IE1-pp65 in HCMV-seropositive donors and that the protein induced activation of HLA-DR3-restricted anti-IE1 CD4(+) T-cell clones, as assessed by gamma interferon (IFN-gamma) secretion and cytotoxicity. Moreover, soluble IE1-pp65 stimulated and expanded anti-pp65 CD8(+) T cells from PBMC of HLA-A2, HLA-B35, and HLA-B7 HCMV-seropositive blood donors, as demonstrated by cytotoxicity, intracellular IFN-gamma labeling, and quantitation of peptide-specific CD8(+) cells using an HLA-A2-peptide tetramer and staining of intracellular IFN-gamma. These results suggest that soluble IE1-pp65 may provide an alternative to infectious viruses used in current adoptive strategies of immunotherapy.

The authors present a series of six anal canal duplications (ACD), duplications of the alimentary tract located along the posterior side of the anal canal, with a perineal opening just behind the anus. Five asymptomatic duplications were diagnosed before the age of one year, by simple perineal inspection. A twelve-year-old girl presented with perineal and anal pains and diarrhoea. Fistulography revealed a tubular structure in five cases and a cystic structure in one case, behind the normal anal canal, in one case communicating with it. A presacral sacrococcygeal teratoma was found in two children and in one case it was visualised by preoperative US in an infant with a lumbosacral myelomeningocele. Surgical excision was performed by a perineal approach in 5 cases, by a combined sacral and perineal approach in the last case, because of the associated teratoma. Non-invasive preoperative investigations, consisting of a pelvic X-ray, US examination, barium enema and fistulography, are sufficient in most cases; MRI is reserved for special indications. Surgical treatment restores a normal perineal aspect, without sequelae, and avoids complications like those described in other types of digestive duplications: infection, ulceration, bleeding, malignant changes during later adult life. Associated anomalies are frequently described in the literature, especially presacral tumours (16%) and anorectal malformations (21%); they can influence the management, the surgical approach and the functional prognosis.

Background: Jejunal intussusception is uncommon in comparison with ileocolic form. It is more frequent in children over 2 years of age and has an atypical subacute presentation. An underlying anatomical cause is usually found., Case Report: A 14-year-old boy was admitted for abdominal pain with bilious vomiting. The physical examination was normal, with only the ultrasonography showing an intussusception in the left hypochondrium. At laparotomy the diagnosis of jejunal intussusception was made; its reduction was impossible. A resection and end to end anastomosis was performed. The anatomopathology examination found a polyp in ectopic gastric mucosa., Conclusion: Jejunal intussusception must be better understood as its diagnosis could be made too late. Surgical exploration is the treatment of choice because of the usual underlying anatomical cause.

An electrophoretic karyotype of the filamentous fungus Podospora anserina has been obtained using contour-clamped homogeneous electric field gel electrophoresis. Six chromosomal bands were separated with one migrating as a doublet. The size of the chromosomes was estimated to be between 3.8 and 6.0 megabase pairs (mb) using the chromosomes of Schizosaccharomyces pombe as size standards, giving a total genome size of about 34 mb for the P. anserina genome. Homologous probes were used to assign five of the seven linkage groups (LGs) to chromosomal bands on the gel. Analysis of reciprocal translocation strains allowed us to complete the karyotype. In decreasing size order, the P. anserina chromosomes are LG I (6.0 mb); LG II (5.5 mb); LG V (5.1 mb); LG III (4.9 mb); LGs VI and VII (4.3 mb) and LG IV (3.8 mb).

One hundred and nineteen dairy cows were given 0, 50 or 100 g sorbitol per day in the diet, in 4 trials. Three trials were conducted at the beginning of lactation, with maize silage, grass silage or grass silage and hay based diets. In the fourth trial conducted in mid-lactation, the concentrate had been enriched with fat. No sorbitol or fructose was found in blood. Sorbitol decreased free cholesterolemia (P less than 0.01), ester-cholesterolemia (P less than 0.10) and glycemia (P less than or equal to 0.05). No changes in performance were noted.