[Intoxication from accidental ingestion of cannabis: analysis of eight cases]. / À propos de 8 cas d'intoxication accidentelle au cannabis chez des nourrissons.

Consultations at pediatric emergency units for acute consciousness alterations is frequent. Miscellaneous causes include cranial trauma, meningoencephalitis, metabolic disorders, drugs, or other intoxications. We report here eight cases of infants who were brought to the emergency division due to acute consciousness failure after accidental ingestion of hashish, confirmed by urinary dosage of Δ9-tetrahydrocannabinol. This series of under 24-month-old infants only emphasizes the value of screening for cannabis in urine in cases of abnormal consciousness and/or abnormal behavior in an infant.

[A 2-month-old baby with Mediterranean spotted fever]. / Une fièvre boutonneuse méditerranéenne chez un nourrisson de 2 mois.

INTRODUCTION: Mediterranean spotted fever is an endemic rickettsiosis in southern France. We report here the case of a 2-month-old baby who developed rickettsiosis after a tick bite. CLINICAL FACT: A 2-month-old baby was hospitalized in the pediatric ward for fever with maculopapular rash extending to the palms and plantar surfaces and an eschar after a tick bite. Rickettsiosis serology examined after 48 h of fever was negative, but Rickettsia (spotted group) PCR taken from the lesion at the bite site was positive. A 1-week treatment with clarithromycin was started. Fever and rash disappeared 3 days after treatment initiation. DISCUSSION: Mediterranean spotted fever is endemic in southern France. It is, therefore, important to consider this diagnosis and search for tick bite signs or an eschar when a patient presents with fever and maculopapular rash. The treatment of choice consists in doxycycline or macrolides. CONCLUSION: Around the Mediterranean sea in particular, Mediterranean spotted fever should be considered as a possible cause of febrile disease with rash, to allow for a specific antibiotic treatment as fast as possible and to avoid dangerous complications, even though few cases have been reported below the age of 3 months.

Management of young children in contact with an adult with drug-resistant tuberculosis, France, 2004-2008.

SETTING: Drug-resistant tuberculosis (DR-TB) is increasing worldwide and may be a source of diagnostic and therapeutic problems in young exposed children. In France exposed children are systematically treated with 3-month isoniazid-rifampicin prophylaxis. OBJECTIVE: To describe the characteristics and management of children aged <2 years in contact with an adult case of DR-TB in France over a 5-year period (2004-2008). METHODS: Children were retrospectively identified by sending questionnaires to all the members of the Paediatric Infectious Diseases Group and the Paediatric Pulmonology Group of the French Paediatric Society. RESULTS: Ten children, all infants, in contact with an adult case of DR-TB were identified: six cases of DR-TB (mean age 4.6 months), one case of TB infection and three cases of exposure (mean age 3.1 months). The children were mainly in contact with poly- or multidrug-resistant TB. Time to initiation of appropriate treatment was 39 days for TB disease and 58 days for TB infection or exposure. One child with TB infection developed TB disease due to failure to adapt prophylaxis. Treatment was variable and centre-dependent. Short-term follow-up showed complete recovery of all children. CONCLUSION: Management of young children in contact with adult DR-TB requires rapid identification of the drug resistance profile. Molecular techniques should be used to reduce delays in initiating appropriate treatment.

Cyanosis revealing hepatopulmonary syndrome in a child with dyskeratosis congenita.

We report the case of a 5-year-old child with dyskeratosis congenita who presented cyanosis and dyspnea at exertion. He had severe hypoxemia with elevated alveolar-arterial oxygen gradient in the setting of liver disease. Technetium-99m-labeled macroaggregated albumin scan showed abnormally high uptake in the brain, confirming hepatopulmonary syndrome.

Mitochondrial biochemical activities and heteroplasmy evolution in established D. subobscura cell line.

A mutant strain of drosophila (D. subobscura) has two types of mitochondrial genomes: a small population (20%) identical to that of the wild strain (15.9 kb) and a predominant population (80%) which has undergone a 5-kb deletion affecting more than 30% of the coding zone. Two cell lines were established from homogenates of embryos from mutant and wild strains. The activities of the respiratory complexes measured in the different cell lines are much lower than in the flies, indicating a glycolytic metabolism. Various modifications of the medium composition did not change this metabolic pathway. The mutant cell line has two types of populations of mitochondrial genomes and the heteroplasmy is equivalent to that measured in the mutant strain. However, the biochemical characteristics differ from those observed in the flies (i.e., the decrease of complex I and III activities), and the various systems of compensation for the consequences of the deletion that are showed in the mutant strain are no longer observed. Furthermore, in contrast with observations made on mutant flies, the heteroplasmy appears unstable in the mutant cell lines: after 60 or so generations, it progressively decreases until it disappears completely. The limited importance of mitochondrial energy metabolism in cells may explain the low impact of the mutation on the established cell line, in contrast to what is seen in the mutant strain.

[Paravertebral streptococcal myositis complicated by an epidural abscess in a 5-year-old girl]. / Myosite paravertébrale à Streptococcus pyogenes A beta hémolytique compliquée d'un abcès épidural chez une enfant.

CASE REPORT: A 5-year-old girl was hospitalised for fever, abdominal and lumbar pain, associated with general impairment state and a whitlow. One of the blood cultures and CSF grew A beta haemolytic Streptococcus, muscular echography and MRI showed paravertebral myositis, which was complicated by an epidural abscess. The outcome was good with medical treatment alone. DISCUSSION: Streptococcal myositis is a rare and severe skeletal muscle infection caused by A beta haemolytic Streptococcus. It is characterized by a muscle necrosis, without abscess formation. It has to be distinguished from pyomyositis, usually caused by Staphylococcus aureus, where a muscle abscess occurs, which must be treated by surgical drainage and antibiotics. Prognosis of this infection is poorer than other muscle infections such as pyomyositis, with a high mortality rate. The diagnosis is difficult and often delayed. Practitioners should keep in mind this diagnosis, even if symptoms are non specific, in front of an undetermined infectious syndrome associated with pain, and make an echography or nuclear magnetic resonance imaging to confirm the diagnosis.

Distribution of the C282Y and H63D polymorphisms in hereditary hemochromatosis patients from the French Basque Country.

The distribution of HFE mutations was studied in patients from the French Basque Country with hereditary hemochromatosis (HH). The C282Y mutation was underrepresented but H63D seemed to demonstrate the highest prevalence when compared with other European countries. In addition, symptomatic HH was rarer in autochthonous Basques. This profile is interesting to consider in view of population genetics and should be associated with the search for non-HFE mutations.

Genetic hemochromatosis is a rare disease entity among French Basques: a center-based study from the general hospital of Basque County.

A center-based study from the general hospital of Basque country has been performed to evaluate the importance of genetic hemochromatosis among French Basques. A sample of 37 patients from 34 families fulfilling the diagnosis criteria of hemochromatosis was obtained. Only four of them were of Basque origin: two homozygotes for C282Y, one homozygote for H63D, and one heterozygote for C282Y. These results suggest a significant lower prevalence of genetic hemochromatosis in Basques than in people from other French regions (P=0.001). They underline further the biological specificity of this population.

Reduced incidence and prevalence of atopy in rheumatoid arthritis. Results of a case-control study.

OBJECTIVE: To determine the cumulative incidence and the point prevalence of atopy in patients with rheumatoid arthritis (RA). PATIENTS AND METHODS: A standardized questionnaire was sent to 300 RA patients. Questions concerned previous or present characteristics of atopy (hay fever, asthma and constitutive eczema) and RA. RA patients were matched with genetically unrelated controls (sister- or brother-in-law, neighbour or friend). The same questionnaire (except for questions about RA) was sent to the control subjects. In cases of atopy, patients, controls and the treating physicians were contacted by a physician to check the validity of the responses. RESULTS: Paired responses were obtained in 173 cases. Information about atopy was obtained for 69 other RA patients. The characteristics of RA were similar for patients who responded and those who did not respond. The frequency of atopy was significantly lower in RA patients than in controls, both for cumulative incidence (RA 7.5%, controls 18.8%; P: < 0.01) and point prevalence (RA 3.5%, controls 16.2%; P: < 0.0001). The clinical manifestations of atopy stopped before the onset of RA in eight of the 17 RA patients with an allergic condition, and there was no subsequent relapse. No effect of RA treatment could account for the remission of atopy. CONCLUSION: These data support the concept that atopy protects against the future development of RA and that the two diseases could counterbalance one another.

Characteristics of deaths in a department of oncohaematology within a general hospital. A study of 81 cases.

We aimed to perform a prospective analysis of the main characteristics of deaths occurring in the oncohaematology department of a general hospital. From November 1995 to February 1997, a total of 81 patients died in our unit, 50 of whom (61.7%) were male. Their mean age was 67.8 (range 19-96) years. Underlying diseases were: multiple myeloma (9 cases), acute myeloid leukaemia (22), lymphoma (14), chronic lymphocytic leukaemia (6), acute lymphoblastic leukaemia (4), myelodysplastic syndromes (3), solid tumours (11), and other (12). The previous disease duration ranged from 5 days to 276 months (mean 31.9 months). The duration of the last hospital stay varied between 0 (death on arrival or on way to hospital) and 40 days (mean 9.3 days). Two patients died in the emergency unit just before entering our department (1 suicide). Only 15 patients had been admitted for the first time. In 70% of these cases death appeared predictable, as the consequence of refractory or end-stage disease. In these cases, all the "do not resuscitate" orders were in place at least 48 h before death. About half the patients died without any relative present. The frequencies of the clinical complaints evaluated were the following: pain necessitating opiates 27%; infection- or disease-related fever 40%; dyspnoea 44%; haemorrhage 20%; CNS disturbances 25%. The percentages of use of therapy tools chosen as indicators were: benzodiazepines 80%; chemotherapy 46%; anti-infectious agents 47%; transfusions 42%; major analgesics 27%; and steroids 40%. The circumstances and quality of patient deaths must be regularly evaluated so that palliative care in the final stages of life can be improved.

Biochemical and molecular consequences of ethidium bromide treatment on Drosophila cells.

KC167 Drosophila cells were incubated with low concentrations of ethidium bromide (200 ng/ml), causing changes in mitochondrial DNA (mtDNA) content (2-184% of that of controls). SSCP (single strand conformational polymorphism) analysis of mtDNA indicated that the incubation with ethidium bromide also generated mutations. Compared with controls, there were marked reductions in the activities of respiratory complexes III and IV measured in these cells, and in respiration and ATP synthesis capacities measured in isolated mitochondria. These reductions matched that in mtDNA content. In contrast, no link could be demonstrated between mtDNA content and steady-state concentrations of the transcripts of genes COIII and Cyt b.

A leptomeningeal metastasis revealed by sciatica.

UNLABELLED: Meningeal metastatic disease usually occurs as a complication of a brain tumor and is exceptionally isolated in patients with solid tumors. We report the case of a 74-year-old woman admitted for mechanical S1 sciatica refractory to drug therapy. She had been treated for breast cancer three years earlier. Physical findings were pain upon hyperextension of the lumbar spine and absence of the ankle jerks. Analysis of cerebrospinal fluid sampled during an intrathecal glucocorticoid injection showed 1 g/L of protein and 11 normal cells per mm3. Grade 3 L5-S1 spondylolisthesis was seen on plain radiographs, computed tomography scans, and magnetic resonance imaging scans. At that point, the patient developed sphincter dysfunction and motor loss in the left lower limb in the distribution of several nerve roots. Findings were normal from a myelogram and a magnetic resonance imaging study of the brain. A repeat cerebrospinal fluid analysis showed 1.1 g/L of protein and 5 cells/mm3. Because of the discrepancy between the clinical and imaging study findings, the patient was transferred to a neurology department. A third cerebrospinal fluid study showed numerous adenocarcinoma cells, and a repeat magnetic resonance imaging demonstrated a mass in the dural sac opposite L2. A program of monthly intrathecal methotrexate injections was started. A fatal meningeal relapse occurred eight months later. CONCLUSION: This case shows that a leptomeningeal metastasis can cause isolated nerve root pain, and demonstrates the diagnostic value of magnetic resonance imaging and cerebrospinal fluid cytology in patients with atypical symptoms, particularly when there is a history of malignant disease.

Descriptive epidemiology of non-Hodgkin lymphomas in a southwestern French hematology center: absence of significant relationship with hepatitis C virus infection.

From January 1995 to June 1998, 136 new cases of non-Hodgkin lymphomas (NHLs) were seen in our center which serves all the French Basque Country (300000 inhabitants). The crude and standardized incidence rates were respectively 13.2 and 7.6 cases/100000/year. The distribution of histologic subtypes according to the REAL classification showed a good correlation with the previous published data. Eleven patients (8%) were HIV-positive and two (1.4%) were HCV carriers. Hence, in our region, contrary to Italy, there is no evidence of relationship between HCV and NHLs.

De novo and secondary acute myeloid leukemia in patients over the age of 65: a review of fifty-six successive and unselected cases from a general hospital.

Fifty-six patients older than 65 (median age: 77, range: 65-91) were treated in our general hospital, between January 1991 and November 1995 with the diagnosis of AML. Twenty-five were de novo AML (45%). The other cases which were considered as secondary AML (sAML) occurred after: myelodysplastic syndromes (19 cases: 34%), myeloproliferative disorders (7: 13%) or therapy of malignancies (5: 9%). Distribution of FAB subtypes was: M0: 4, M1: 8, M2: 14, M4: 10, M4eo: 1, M5: 10, M6: 1, unclassified: 8. Twenty-seven patients (48%) (de novo: 18, sAML: 9) received conventional "3 + 5" or "3 +7" induction chemotherapy +/- consolidation and maintenance (group 1). Low-dose Ara-C was given in eighteen cases (32%) (group 2), miscellaneous single agents were prescribed in seven cases and two patients received only supportive care. Sixteen early deaths (ED) (<1 month) occurred (29%). Distribution of age, WHO-PS, hyperleukocytosis, ED and median survival (MS) (3 months) was equivalent in de novo and sAML. Fifteen CR were achieved including twelve patients from group 1 (no difference between age <75 versus >75 years) and three from group 2. The MS of CR patients was 13 months. Eleven patients survived more than one year (de novo: 8). M5 subtype, fever >38 degrees C, high LDH level, WHO-PS>2, CR non achievement were predictive of reduced survival. Besides conventional induction, investigational therapies currently remain the best antileukemic modalities. Age per se should not be an exclusion criteria for treatment. Our data underline the high frequency of sAML in the community. Of note, this common type AML is largely excluded from many trials.

Developmental changes in heteroplasmy level and mitochondrial gene expression in a Drosophila subobscura mitochondrial deletion mutant.

Eighty percent of DNA molecules are deleted in the mitochondrial population of an adult mutant strain of D. subobscura. Both intact and deleted genomes are autonomous monomers. The heteroplasmy level, which is lower in germ tissue, increases from the oocytes (60%) to the third larval instar (83%), and is then maintained throughout the life of the fly. The mtDNA/nuclear DNA ratio is on average two-times greater in the heteroplasmic strain than in the wild-type strain, irrespective of the stage, but the cellular content of mitochondria is elevated only in the embryos and pupae of the mutant strain. The steady state concentrations (SSCs) of the transcripts affected by the deletion are greatly reduced at the larval and adult stages, and less so at the pupal stage of the mutant strain compared with the wild-type. The SSCs of these transcripts are identical in the two strains at the embryonic stage. The fusion transcript, indicating that the deleted genome is expressed, was detected at all stages. The mechanisms involved in the changes in the heteroplasmy level during the course of development and in its maintenance from the third larval instar onwards are discussed.

Epidemiology of myelodysplastic syndromes in a French general hospital of the Basque country.

Epidemiologic studies concerning myelodysplastic syndromes (MDS) are rare. The estimated incidence varies between 1 and 12.6/100,000/year. The aim of this work was to compare our own experience with these data. Our general hospital represents a structure with 1197 beds which serves a population of 290,000 individuals (French Basques). Most of the inhabitants live in a rural environnement. Twenty percent of the population are aged over 65. During a 4-year period (1993-1996), 90 new cases of MDS were diagnosed on bone marrow studies in our laboratory. Among FAB subtypes refractory anemia (RA) represented 27 cases (31%), RA with ring sideroblasts (RARS): 21 (23%), RA with excess of blasts (RAEB) and in transformation (RAEB-t): 22 (24%), chronic myelomonocytic leukemia (CMML): 10 (11%). Ten cases were unclassifiable (11%). Therapy-related MDS were seen in 8 patients. The sex ratio was 1 and the mean age of the patients was 74.3 (range: 23-96), 37% of them being 80 years or older. The calculated incidence was 7.7/100,000/year for the entire cohort and 31.4/100,000/year for people over 65.

Cultured neonatal rat cardiomyocytes and 99mTc-sestamibi to assess the direct effects of cardioplegia.

The efficacy of cardioplegia in neonatal myocardial protection is still a matter of debate. 99mTc-sestamibi cellular accumulation reflects sarcolemmal and mitochondrial electrical gradients. It was used to monitor the direct effects of two cardioplegic solutions, modified St Thomas' Hospital and Bretschneider, on normoxic and metabolically-inhibited cultured cells. Cellular accumulation of 99mTc-sestamibi, expressed by the ratio between intra and extra cellular concentrations, was assessed in three different sets of neonatal rat cardiomyocytes. Cells were either treated with different concentrations of modified St Thomas' solution (50, 75, 100%), they were treated or recovering from a treatment with modified St Thomas and Bretschneider solutions at 50% concentrations, or were recovering from treatment with modified St Thomas' and Bretschneider solution at 50% concentrations mixed with metabolic inhibitors. Cardioplegia depressed the tracer accumulation in a concentration-dependent manner. This effect was independent of the type of cardioplegia (120-min uptake, as a percentage of control values, modified St Thomas' 68+/-12 and Bretschneider 59+/-7) and was rapidly reversible. Cardioplegia was unable to prevent the depression of tracer accumulation induced by metabolic inhibitors and even induced a deleterious effect (120-min uptake, as a percentage of control values, metabolic inhibitors 69+/-12, metabolic inhibitors + modified St Thomas 38+/-14, metabolic inhibitors + Bretschneider 43+/-6) during recovery after 30 min of metabolic inhibition. It was concluded that cardioplegia has an apparent detrimental effect on neonatal cardiomyocytes accumulation of 99mTc-sestamibi during recovery from an ischaemic-like insult.

Release of mast cell mediators and nitrites into knee joint fluid in osteoarthritis--comparison with articular chondrocalcinosis and rheumatoid arthritis.

In order to address the issue of the role of mast cells and nitric oxide (NO) in joint effusions occurring in the course of osteoarthritis (OA), synovial fluids collected from the knee of patients with OA, articular chondrocalcinosis and rheumatoid arthritis (RA) were studied for number of mast cells, and histamine, tryptase, phospholipase A2 and nitrite content. Mast cell counts are elevated in synovial fluid from OA patients when compared with RA. Histamine content in synovial fluid parallels the number of mast cells. Tryptase levels are elevated in OA in comparison with both other conditions, but do not reach the level of significance. Identical phospholipase A2 levels are recorded in three groups. Nitrite concentrations are also higher in synovial fluid from OA patients when compared with RA patients. These results suggest that mast cells in association with various inflammatory cells, may contribute to inflammation and cartilage breakdown in OA.