Search

Acute pain is one of the most common symptoms in children admitted to the Pediatric Emergency Department (PED) and its management represents a real clinical challenge for pediatricians. Different painful procedures can be very stressful for young children and their perception of pain can be enhanced by emotional factors, such as anxiety, distress, or anger. Adequate procedural sedation reduces anxiety and emotional trauma for the patient, but it reduces also stress for operators and the time for procedures. We have reviewed the literature on this topic and the drugs covered in these papers were: midazolam, fentanyl, ketamine, and dexmedetomidine. There are several routes of administering for these drugs to provide analgesia and anxiolysis to children: oral, parenteral, or intranasal (IN). Intravenous (IV) sedation, since it involves the use of needles, can be stressful; instead, IN route is a non-invasive procedure and generally well tolerated by children and it has become increasingly widespread. Some medications can be administered by a mucosal atomizer device (MAD) or by drops. The benefits of the atomized release include less drug loss in the oropharynx, higher cerebrospinal fluid levels, better patient acceptability, and better sedative effects. IN midazolam has a sedative, anxiolytic and amnesic effect, but without analgesic properties. Fentanyl and ketamine are mainly used for pain control. Dexmedetomidine has anxiolytic and analgesic properties. In conclusion, IN analgo-sedation is a simple, rapid and painless option to treat pain and anxiety in the PED requiring brief training on the administration process and experience in sedation.

It has taken almost twenty years to complete the vast research project that has resulted in this astonishingly beautiful and fascinating book. Emilie d’Orgeix (Ecole Pratique des Hautes Etudes, Par...

International audience; The state secretariat papers, including a huge variety of maps and sketches, reveal the complexity of governing a territory as vast as that ruled by Napoleon I. In terms of state administration, state secretariat reports show attempts to rationalise the territorial and administrative units making up the Empire. These papers reveal not only a distillation of the disagreements which occupied public administrators at the local level, both in France and beyond the pre-imperial frontiers, such as in Italy, but also how central government took note of these inter-communal conflicts and tried to resolve them.; Le fonds de la secrétairerie d’État, à travers de nombreuses cartes et croquis, illustre la complexité à gouverner sur un territoire aussi vaste que celui sur lequel règne Napoléon Ier. D’abord à visée normative, les rapports de la secrétairerie veulent rationaliser les unités territoriales et administratives qui composent l’Empire. Au fil du temps, ces écrits concentrent les contentieux qui animent le quotidien des acteurs publics à l’échelle locale, aussi bien en France qu’au-delà de ses frontières pré-impériales, par exemple en Italie. Et comment le pouvoir central tente d’en rendre compte et de résoudre ces conflits communaux.

Haemophilia A is a rare X-linked recessive bleeding disorder caused by deficiency or functional defects in coagulation factor VIII (FVIII). Here, we report two cases of challenging diagnosis of haemophilia A because of unusual presentation. The first case is a 10-month-old female, admitted to our hospital because a neck mass appeared within the previous 24 h, who had a past medical history consistent with recurrent spontaneous haematomas but no family history of bleeding disorders. Despite several radiological evaluations, only the histology of the mass defined the presence of a haematoma. Chromosomal analysis revealed a normal female karyotype and a de-novo mutation into the FVIII intron 22 associated with a skewed X chromosome inactivation. The second case is a male neonate with a history of seizures who underwent brain MRI that showed a suspicious vascular malformation on the quadrigeminal cistern, causing cerebellum compression and hydrocephalus. The clinical conditions of the child progressively worsened and blood tests revealed a severe deficit of FVIII levels. The radiological images were re-evaluated; vascular anomalies were excluded and the diagnosis of haematoma was made. Family history was negative for coagulation disorders. Molecular studies revealed a rearrangement of the FVIII gene involving intron 22. The haemophilia A diagnosis can be challenging. Lack of family history, difficulties in detecting haematomas by imaging techniques, female sex and neonatal age represent misleading factors that can delay the diagnosis.

Background Data on the epidemiology of bacteremias and invasive fungal diseases (IFD) in children with acute myeloid leukemia (AML) are scarce. Design and Methods In a multi-center, retrospective study, we analyzed proportion, rate per 1,000 person-days at risk, and cumulative risk of bacteremias and IFD in children with AML. Results Between January 1998 and December 2005, 240 children were treated for AML at 8 Italian Centers, for a total of 521 treatment courses and 63,232 person-days at risk. Bacteremia was observed in 32% of treatment courses and IFD was seen in 10% (P

« Talent » est un terme couramment utilisé dans les études sur les modes de recrutement entre la fin de l’Ancien Régime et la Restauration, mais les insuffisances dans sa définition entravent son utilité comme catégorie d’analyse. Se penchant sur le monde de la topographie militaire, cet article reconstitue concrètement le savoir-faire qui y est appelé « talent », ainsi que son usage en tant que moyen de contrôle de l’accès au service. Par le biais de ce concept, une autre lecture des conflits pour les modes de formation dans les armes savantes devient possible, et les oppositions au modèle de dépersonnalisation et centralisation incarné par l’École polytechnique se clarifient. “Talent” is the term commonly used in studies about the method of recruting between the end of the Old Regime and the Restoration. Ye t the definition of this term is not precise enough to be useful as an analytical category. This article reconstructs the expertise known as “talent” as well as its use as a means of controlling access to posts. With this concept, another interpretation of the struggle for the formation a elite is possible, and the opposition to the centralized and depersonalized Ecole Polytechnique more comprehensible.

Study Objective To assess the diagnostic accuracy of office hysteroscopy by comparing hysteroscopic findings with histologic findings on hysterectomy specimens in postmenopausal women with breast cancer treated with tamoxifen. Design Retrospective clinical study (Canadian Task Force classification II-2) Setting University-affiliated hospital. Patients Thirty-three women. Intervention Record review. Measurements and Main Results Compared with histologic diagnoses of uteri, hysteroscopic findings showed diagnostic sensitivity of 97%, specificity of 100%, positive predictive value of 100% and negative predictive value of 96%. The diagnostic accuracy of hysteroscopy is the same in patients taking tamoxifen as in the general population. Conclusion Hysteroscopy is a powerful method for diagnosing endometrial disease because it provides a direct view of the uterine cavity, reveals focal lesions, and enables targeted biopsies to be performed at the same time.

Idiopathic thrombocytopenic purpura in children remits spontaneously in the majority of cases but most children require treatment. Between 1995 and 2005, 265 children (0-15 years old) have been consecutively observed and treated: 28 children with high doses of methylprednisolone (HDMP) (15 mg/kgx4 days), 63 with HDMP (7.5 mg/kgx4 days), 37 with HD dexamethasone (DXM) pulses, 29 with low doses of MP, and 51 with different doses of intravenous immunoglobulins (IVIG) (0.4 or 0.8 g/kg). Fifty-seven children have not been treated because of a platelet count>or=10x10(9)/L and no significant bleeding. Two hundred forty-four (92.1%) children reached a persistent CR, 237 (89.4%) after a first-line treatment or the wait and see strategy. No statistically significant differences in CR related to different treatments have been observed. IVIG and HDMP (7.5 mg/kg for 4 days) are the best treatments to reach quickly safe platelet levels>or=30x10(9)/L (3-6 days) and CR (7-11 days). Among non-responding (NR) patients, seven have been splenectomized and three reached stable CR. These results emphasize differences with adult ITP.

BACKGROUND: Deeply infiltrating endometriosis (DIE) is recognized as a specific entity responsible for pain. The distribution of locations and their contribution to surgical management has not been previously studied. METHODS: Medical, operative and pathological reports of 241 consecutive patients with histologically proven DIE were analysed. DIE lesions were classified as: (i) bladder, defined as infiltration of the muscularis propria; (ii) utero-sacral ligaments (USL), as DIE of the USL alone; (iii) vagina, as DIE of the anterior rectovaginal pouch, the posterior vaginal fornix and the retroperitoneal area in between, and (iv) intestine, as DIE of the muscularis propria. RESULTS: A total of 241 patients presented 344 DIE lesions: USL (69.2%; 238); vaginal (14.5%; 50); bladder (6.4%; 22); intestinal (9.9%; 34). The proportion of isolated lesions differed significantly according to the DIE location: 83.2% (198) for USL DIE; 56.0% (28) for vaginal DIE; 59.0% (13) for bladder DIE; 29.4% (10) for intestinal DIE (P < 0.0001). The total number of DIE lesions varied significantly according to the location (P < 0.0001). In 39.1% of cases (9/23) intestinal lesions were multifocal. Only 20.6% (seven cases) of intestinal DIE were isolated and unifocal. CONCLUSIONS: Multifocality must be considered during the pre-operative work-up and surgical treatment of DIE. We propose a surgical classification based on the locations of DIE. Operative laparoscopy is efficient for bladder, USL and vaginal DIE. However, indications for laparotomy still exist, notably for bowel lesions.

Objective : To assess the intervention related risk in HIV-infected women, undergoing caesarean section (CS). Setting : Tertiary care center for high risk obstetrics and infectious diseases in pregnancy. Patients and methods : Thirty-three HIV-positive women and one hundred and sixty-eight controls, cross matched for age, weight, parity, obstetrical characteristics at delivery and indication for CS, were prospectively recruited for the study. Infection related characteristics, as mode of acquisition, drug abuse, immune status and stage of disease were also recorded. Complications of the intervention were evaluated according to infectious status and risk factors. Results : Post-operative complications were significantly more frequent among HIV-infected mothers. More advanced disease and maternal age were the risk factors statistically related to complications. Discussion : According to our data, CS carries a higher chance of post-operative complications in HIV-infected women, these complications being also related with the severity of infection (stage and duration of the disease). Given the characteristics of the population in the study (mode of acquisition, prevalence of early stage of the disease), the rate of complication is still relatively low compared to non-western, malnourished, drug-abusers groups.

The venous thromboembolism is a serious and complex pathological condition especially for its potential complications. It is related to both congenital and acquired factors. Among the congenital factors, different mutations have been identified leading to a reduced activity of enzymes (even higher than 50%) involved in the homocysteine-methyonine methabolic pathway, such as the methylenetetrahydrofolate reductase enzyme (MTHFR). The homozygotic TT genotype is found in approximately 10–12% of the population and is associated with a 25% higher homocisteine level in these patients than those without this mutation. The heterozygotic genotype is found in 40% of the population. The aim of this study is to define in children the correlation between venous thrombosis and MTHFR mutations. Between december 2002 and april 2008 nine children aged between 1–132 months were admitted in the Department of Hematology of Children Hospital Bambino Gesù in Rome. They were affected by deep venous thrombosis and were submitted to thrombophilic screening and vascular imaging. The MTHFR polymorphism C677CT was found in all the patients (four in homozygosis and seven in heterozygosis) and also in their parents. Four heterozygous and three homozygous patients presented increased plasmatic and urinary levels of homocysteine. Since venous thrombosis was only referred in the clinical history, two patients did not received treatments. Nine were treated with low molecular weight heparin (enoxaparin 100 U/kg s.c. × 2 daily), then seven continued the treatment with oral anticoagulant for 6–12 months and all of them performed long term profilaxis with folic acid and B group vitamins. Only one patient presented further thrombotic event and restarded the oral anticoagulant therapy. These data confirm that the measurement of homocysteinemia and MTHFR may be indicated in unexplained idiopathic venous thrombosis or recurrent episodes or venous thrombosis occurred at an early age or at an uncommon site. It is to be discussed whether to perform anticoagulant profilaxis in all patients with MTHFR mutation, in homozygosis or heterozygosis, with or without hyperhomocysteinaemia, with previous thrombotic events, with other thrombotic risk factors (pregnancy, oral contracceptives, sepsis and immobilization). In addition in presence of hyperhomocysteinemia, it might be reccomended to perform therapy with folic acid and B6 vitamin.

Introduction: Myelodysplatic syndromes (MDS) are characterized by ineffective and dysplastic hematopoiesis and peripheral cytopenias. Moreover, autoimmune phenomena, mainly directed against RBC, are described in early MDS, i.e. refractory anemia (RA) and RA with ringed sideroblasts (RARS). We already reported an autoimmune reactivity against erythroblasts in 50% of RA and RARS adult patients, along with peripheral signs of hemolysis, increased BM erythroblasts; and efficacy of steroid therapy. It has become evident that there are significant differences between MDS in children and adults. Most importantly, the cure is the aim of therapy in all children with MDS, while therapeutic possibilities are often limited in adults. MDS and myeloproliferative disorders are rare in childhood and there is no widely accepted system for their diagnosis and classification. Aims of this study were: To evaluate BM autoimmunity in 9 pediatric patients with MDS by the same method used in adults, named mitogen-stimulated-direct antiglobulin test (MS-DAT), to correlate BM MS-DAT positivity with clinical parameters and therapy. Methods: MS-DAT was performed by stimulating BM with PMA and PHA and antibodies were detected in supernatants by competitive solid phase ELISA. Results: We demonstrated the presence of anti-erythroblast autoimmunity in roughly half of pediatric MDS patients, as already reported in adult MDS. More precisely, 4 out of 9 patients showed positive MS-DAT in BM. At variance with adult MDS, positive patients do not show increased erythroblast counts and peripheral signs of hemolysis (i.e higher reticulocytes, indirect bilirubin, and LDH, and lower haptoglobin) compared with MS-DAT negative ones. One of the four patients with anti-erithroblasts autoimmunity has RA, the other three show peripheral and medullary signs of refractory cytopenia with multilineage dysplasia (RCMD). Two out of four were successfully treated with steroid therapy (with a follow up respectively of 6 and 1 months). Discussion: Although the short follow up and the limited number of patients does not allow definitive conclusions, we retain useful to investigate anti-erythroid autoimmunity in all pediatric MDS in order to identify selected patients with low risk MDS who display autoimmune phenomena. In these cases immunosoppressive therapy may be an effective treatment option.

International audience