[Hemihypertrophy and scoliosis revealing a Chiari 1 malformation with syringomyelia]. / Hémi-hypertrophie et scoliose révélatrices d'une malformation de Chiari de type 1 avec syringomyélie.

We report the case of a 9-year-old boy with progressive thoracic scoliosis and crossed hemihypertrophy who was discovered with a Chiari 1 malformation and syringomyelia. These disorders are connected by complex physiopathological mechanisms; their association deserves attention. This observation reviews the importance of the clinical examination, particularly the neurological exam, in childhood scoliosis. The features suggesting a neurogenic background of spine deformation should be sought. Scoliosis with hemihypertrophy can be the sign of an underlying neurological abnormality.

[A case of Kabuki syndrome admitted for acute diarrhea and growth retardation in a French hospital in tropical area]. / Syndrome de Kabuki en milieu tropical chez un nourrisson hospitalisé pour diarrhée et stagnation pondérale.

The authors report on a 6-month-old girl with Kabuki syndrome, admitted for acute diarrhoea and growth retardation at the Mayotte hospital. From this case, they try to explain the way of understanding and management in front of dysmorphic features.

[Children's soft tissue infections in tropical countries. Prospective study in Mayotte]. / Les infections des parties molles de l'enfant en milieu tropical. Etude prospective hospitalière à Mayotte.

In France and Europe, soft tissue infections are secondary to chickenpox infection. In tropical countries, soft tissue infections seem to be different and are more frequent. We conducted a prospective and descriptive study in children hospitalised for cellulitis. We studied characteristics of our population and we tried to individualize risk factors for deep soft tissue infections. 54 children were included over a six-month period. Blood cultures were positive in 10% and local culture in 62%. Pathogenic organisms to be found, were first Staphylococcus aureus (78%) and secondly alpha-haemolytic streptococcus. Average rate hospitalisation was 4.5 days (1-28). Despite intravenous antibiotherapy, more than one third of patients had had a deep soft tissue infection (myositis, abscess, or arthritis). As regards the overall population, deep soft tissue infections associated with cellulitis were more frequent in children over six. Association with arthritis was found only in children under two. Severe malnutrition seems to be a notable risk factor for myositis. Soft tissue infections are still frequent in tropical countries. Deep soft tissue infections are encountered in more than one third of the cases, specially in children over six, and with Staphylococcus aureus. These results justify a systematic hospitalisation. If severe malnutrition is present, association with myositis should be suspected.

[Confirmed chikungunya in children in Mayotte. Description of 50 patients hospitalized from February to June 2006]. / Chikungunya confirmé chez l'enfant à Mayotte a propos de 50 cas hospitalises, Février-Juin 2006.

The French Indian Ocean island Mayotte was hit by an outbreak of chikungunya in January 2005. The purpose of this retrospective study is to report data recorded over a five-month period (February - June 2006) in the pediatric-neonatal department of the Hospital Center in Mayotte. The study cohort includes a total of 50 children in whom chikungunya was confirmed by molecular tools. Mean age was 9.3 years and the male-to-female sex ratio was 1:5. The main symptoms were intense pain (88%), high fever (82%), and skin rash (80%) that was less common in children under 2 years of age. Neurological complications were observed in 46% of patients including hypotonia (22%) that occurred mainly in newborns, meningitis syndrome (18%) and convulsions (16%) that occurred mainly in children over 2 years of age. Infectious complications included pneumonia (4%), pyelonephritis (2%), and possible nosocomial septicemia due to Pseudomonas (6%). The main hematological abnormalities were lymphopenia (27%) and thrombopenia (16%). Serum CRP values were moderately high (mean, 25 mg/l). Elevated AST (24%) and ALT (10%) values were observed. High CSF protein levels were noted in 30% of cases. A total of 25 children required hospitalization for more than 10 days. There were two deaths in newborns infected before the seventh day of life. The main risk factors for hospitalization longer than 10 days were premature birth and age at the time of chikungunya infection.

[Malignancy risk and Wiedemann-Beckwith syndrome: what follow-up to provide?]. / Risque tumoral et syndrome de Wiedemann-Beckwith: quelle surveillance proposer?

Wiedemann-Beckwith syndrome (WBS) is a syndrome of excessive growing with a high predisposition to developing embryologic tumours within the first years of life. This risk is evaluated between 7.5 and 10%; it varies with the mechanisms of mutations involved. These take place in two distinct domains of 11p15, which are under parental printing. Emerging techniques of cytogenetic and molecular biology now have shown correlations between genotypes and phenotypes, and can identify the 30% of WBS who are especially at risk of developing tumours. A specific follow-up, integrating the specificity of developing tumours of each 11p15 mutations involved, is now proposed to patients with WBS.

[Update on HIV infection in Mayotte]. / Le VIH à Mayotte.

Mayotte is a small French island located in the Indian Ocean between Madagascar and Mozambique. It is one of the four Comorian Islands and has a population of about 200,000. The first cases of AIDS were diagnosed in 1989. Since then, the number of serological tests performed annually has stabilized at around 14000. However the number of new cases and treatment reports appears to be increasing slowly. Five of the 15 cases diagnosed in 2005 were at the AIDS stage. In 2006, 74 people were treated at the Mayotte hospital including 5 children. The mean age of the 69 adult patients was 38 years. Contamination was heterosexual for 71% of the adult cases, homosexual in 13% and transfusional in 3%. Women accounted for 59.5% of adult patients because of antenatal screening. All cases in Mayotte involved HIV type 1 infection. Forty-nine patients are undergoing treatment. Viremia is undetectable in 74% as compared to 85% in 2005. This decrease is due to a drop in attendance from 7.2 in 2005 to fold 4.5 in an island environment where HIV is still considered as a shameful disease.

[Vaccination of children against hepatitis B in Mayotte, French Comoros Island]. / Vaccination de l'enfant contre l'hépatite B à Mayotte, île française des Comores.

Hepatitis B virus (HBV) is responsible for a worldwide mortality of 1 million people each year. It constitutes a major public health problem, especially in highly endemic zones, where it concerns the youngest children, primarily by a mother to child transmission, with a strong risk of chronic hepatitis infection and hepatocellular carcinoma. Immunisation of children versus HBV is known to be efficient and safe. In Mayotte, a French overseas territory in Indian Ocean, immunisation versus HBV has been introduced since 1993 in the vaccine schedule, starting at day 1 of life. We report hereby the local experience and practice on HBV infection, state of vaccine coverage, and difficulties met with this major public health issue.

[Multifocal infection due to Mycobacterium intracellulare: first case of interferon gamma receptor partial dominant deficiency in tropical French territory]. / Infection multifocale à Mycobacterium intracellulare: premier cas de déficit partiel dominant du récepteur de l'interféron gamma en milieu tropical français.

Nontuberculous mycobacterial infections are rare in immunocompetent children, and usually present as adenitis. We report a case of a 6-year-old girl with a multifocal chronic osteomyelitis and pulmonary localisation due to Mycobacterium intracellulare associated with an autosomal dominant mutation of interferon gamma receptor 1 gene (INFGR1) leading to a syndrome of mendelian predisposition to mycobacteria infections by partial deficiency of intracellular signalisation of gamma interferon. This child has been cured with anti-mycobacteria drugs and gamma interferon. This report focus on the importance of looking for a susceptibility of the host to infectious diseases, which can lead to a specific treatment. As far as we know, this is the first case described in a tropical area.

The characteristic appearance of non-alcoholic duct destructive chronic pancreatitis: a report of 2 cases.

We report 2 patients with an unusual form of chronic pancreatitis, both of whom were treated for clinical suspicion of pancreatic malignancy. The surgical specimens revealed a dense lymphoplasmacytic infiltration of the main and interlobular branches of the pancreatic duct, causing sclerosis of the duct wall, diffuse irregular lumenal narrowing, extensive parenchymal fibrosis, and organ enlargement. Neither case showed calcifications, fat necrosis, or cyst formation, features usually seen in alcoholic pancreatitis, nor was there any evidence of neoplasia. One patient had an unusual form of acalculous cholecystitis, but without cystic duct inflammation or fibrosis. Both patients recovered well from the surgical procedure and have not had any complications or relapse of their symptoms. To the best of our knowledge, these cases are representative of the recently described non-alcoholic duct destructive chronic pancreatitis, which is thought to be immune-mediated.

[Severe forms of eosinophilic meningitis in infants of Mayotte. Apropos of 3 cases]. / Formes graves de méningites à éosinophiles chez le nourrisson à Mayotte. A propos de 3 observations.

BACKGROUND: Eosinophilic meningitis caused by Angiostrongylus cantonensis is widespread in Southeast Asia and the Pacific islands. Adults develop transient meningitis with a benign course, whilst severe or fatal disease may occur in pediatric patients. CASE REPORTS: Three infant girls, aged 8 to 11 months, living on the island of Mayotte, developed fever, hypotonia, coma (2 cases), and, for one of them, seizures. Eosinophilia was detected in the peripheral blood and cerebrospinal fluid. Secondary, flaccid quadraplegia (1 case) or paraplegia (2 cases) with absence of deep tendon reflexes, urinary retention and anal incontinence were noted. Three patients had autonomic dysfunction. Computerized tomography showed enlarged ventricles and cerebral subarachnoid spaces. One patient had sequelae. Two patients could not be followed. Retrospectively, the diagnosis of angiostrongylus infection was established for two infants by a serological study. CONCLUSION: We report three new cases of infants with severe Angiostrongylus cantonensis infection in the French island of Mayotte (Comoro Islands). In this Indian Ocean area, eosinophilic meningitis seems to occur exclusively in infants and with severe radiculomyeloencephalitic forms.

A new model for recognizing and rewarding the educational accomplishments of surgery faculty.

Faculty members' educational endeavors have generally not received adequate recognition. The Association for Surgical Education in 1993 established a task force to determine the magnitude of this problem and to create a model to address the challenges and opportunities identified. To obtain baseline information, the task force reviewed information from national sources and the literature on recognizing and rewarding faculty members for educational accomplishments. The group also developed and mailed to surgery departments at all U.S. and Canadian medical schools a questionnaire asking about the educational endeavors of the surgery faculty and their recognition for such activities. The response rate after two mailings was only 56%, but the responses reaffirmed the inadequacy of systems for rewarding and recognizing surgeon-teachers and surgeon-educators, and confirmed that the distinction between the roles of teacher and educator was rarely made. The task force created a four-tier hierarchical model based on the designations teacher, master teacher, educator, and master educator as a framework to offer appropriate recognition and rewards to the faculty, and endorsed a broad definition of educational scholarship. Criteria for various levels of achievement, ways to demonstrate and document educational contributions, appropriate support and recognition, and suggested faculty ranks were defined for these levels. The task force recommended that each surgery department have within its faculty ranks a cadre of trained teachers, a few master teachers, and at least one educator. Departments with a major commitment to education should consider supporting a master educator to serve as a resource not only for the department but also for the department's medical school and other medical schools. Although this model was created for surgery departments, it is generalizable to other disciplines.

Who should teach medical students surgery?

BACKGROUND: Medical schools are undergoing major curricular reform, partly in attempts to increase the number of graduates pursuing careers in the generalist disciplines. These reforms have often resulted in a shortening of the surgery clerkship, decreasing students' experiences in several domains important to the generalist. METHODS: A seven-question survey of clerkship directors of US medical schools was administered to measure the magnitude of curriculum change during the past 5 years affecting the surgery and family practice clerkships. The survey also addressed attitudes about the purpose of the surgery clerkship. RESULTS: There was an 80% (103 of 129) response rate. Between 1989 and 1994, surgery clerkships decreased on average from 11 to 10.2 weeks (P <0.05) while family practice clerkships increased from 4.2 to 6.8 weeks (P <0.05). Ninety-one percent of clerkship directors felt the primary goal of the clerkship should be to train generalists. CONCLUSIONS: The length of the surgery clerkship has decreased at several institutions. In order to ensure an appropriate educational experience for medical students, surgeons must participate actively in curriculum reform within medical schools and highlight their unique role in training generalists.

[Acquired aphasia in a child with epilepsy (Landau-Kleffner syndrome). Comments apropos of a case with 1-year follow-up]. / Aphasie acquise de l'enfant avec épilepsie (syndrome de Landau-Kleffner). Réflexions à propos d'une observation durant un an.

Authors report a case of Landau-Kleffner syndrome in a 6 year old boy. Landau-Kleffner syndrome is a rare disorder characterized by the combination of acquired aphasia and epileptic abnormalities like diffuse spikes-and-waves in sleep EEG. Seizures are associated in 50 to 80% of cases and generally disappear at puberty. Behavior disorder ranges from minor psychomotor disturbances to psychotic-like features. Onset appears at an age between 3 to 7 years. The relationship between aphasia and epilepsy remains unclear, even if language improvement frequently follows EEG improvement. The hypothesis of an underlying encephalitis could explain the whole syndrome but is not yet validated. Therapy should associate antiepileptic drugs, corticosteroid treatment and speech therapy, but no controlled study is available to confirm this protocol. Aphasia recovery is generally incomplete. The evolution of behavior disorder is not well documented. In the reported case, one year after onset, sleep EEG again became normal, behavior disturbances had disappeared, but spoken language was still absent.

Effects of exogenous cytokines on intravascular clearance of bacteria in normal and splenectomized mice.

BACKGROUND: Pretreatment with interleukin-1 (IL-1), granulocyte colony-stimulating factor (G-CSF), and granulocyte macrophage colony-stimulating factor (GM-CSF) can improve alveolar macrophage bactericidal activity against pneumococci. These effects vary in eusplenic and asplenic mice. Likewise, these cytokines have been shown to improve survival after an aerosol pneumococcal challenge. Mice dying in these studies had positive blood cultures and disseminated infection. The purpose of this study was to determine the effect of cytokine pretreatment on intravascular clearance of bacteria from eusplenic and asplenic mice. METHODS: Two weeks after splenectomy or sham operation, mice were pretreated for various times with IL-1, G-CSF, or GM-CSF or their corresponding vehicles. Mice then received tail-vein injections of bacteria (0.1 mL), and quantitative blood cultures were performed 15 and 30 minutes thereafter. RESULTS: Splenectomized mice had impaired clearance of both pneumococci and Pseudomonas compared with sham-operated mice (p < 0.05). IL-1 enhanced clearance in splenectomized mice (p < 0.001) but not in sham-operated mice (p not significant). G-CSF enhanced bacterial clearance in sham-operated mice (p < 0.01) but not in splenectomized mice (p not significant). GM-CSF enhanced clearance in both groups (p < 0.001). CONCLUSION: The net effects of exogenous cytokine therapy for infections depends on the state of the host defenses at the time of therapy. These agents may be useful as adjuvants for the treatment of infections, but further study is warranted.

Granulocyte-macrophage colony-stimulating factor (GM-CSF) enhances pulmonary defenses against pneumococcal infections after splenectomy.

BACKGROUND: Splenectomized individuals are at risk for pneumococcal sepsis. Alveolar macrophage bactericidal function is depressed after splenectomy. Granulocyte-macrophage colony-stimulating factor (GM-CSF) has pronounced effects on the number and function of macrophages. We hypothesized that GM-CSF treatment could improve alveolar macrophage bactericidal activity against pneumococci, and improve survival with pneumococcal infection. METHODS: Two weeks after splenectomy or sham operation, mice were treated with GM-CSF or saline twice daily for varying times. Alveolar macrophages were obtained by bronchopulmonary lavage, and bactericidal activity was measured. Survival was assessed after pneumococcal aerosol challenge. RESULTS: Alveolar macrophage bactericidal activity was improved with GM-CSF treatment in both eusplenic and asplenic mice (p < 0.001). GM-CSF treatment improved survival in both groups (p < 0.001). CONCLUSIONS: GM-CSF can augment alveolar macrophage function and provide protection against pneumococcal infections. It may be a useful adjuvant therapy for normal and splenectomized individuals.

Modifying the host response to injury. The future of trauma care.

It is beyond the scope of this article to describe all of the contributions of molecular biology to increasing our understanding of the pathophysiology of inflammation and the response to injury. This review focuses on those aspects that are clinically relevant. In addition to providing quantities of recombinant proteins, recent advances in molecular and cellular biology have provided other tools to help differentiate the pathophysiology of the host response to injury and infection. Hybridoma technology has facilitated the development of specific antibodies that are used to block the activity of a specific factor or toxin. Receptor and signal transduction biology has provided further insight into the activity and function of various factors and mediators. Studies at the level of the gene have shed light on the phylogenic relationship among various factors. Transgenic animals can be used to determine the effects of excess factor production; conversely, genetic "knockouts" are useful in determining the pathophysiology associated with the absence of a particular factor. It is clear that as our understanding of the complex interactions leading to inflammation increases, we will be able to take advantage of this knowledge to more effectively treat patients.