Severe hyperhomocysteinemia and peripheral neuropathy as side effects of nitrous oxide in two patients with sickle cell disease.

Nitrous oxide (N2O) is a widely used anesthetic agent. We report two patients with sickle cell disease (SCD) who presented with complications following the use of N2O. Patient 1, a 15-year-old girl, presented severe hyperhomocysteinemia, pancytopenia, vitamin B12 deficiency, and peripheral polyneuropathy after massive use of N2O for pain management. At the 1-year follow-up, hyperhomocysteinemia and B12 deficiency had resolved, but she had persisting mild symptoms of polyneuropathy. Patient 2, a 17-year-old boy, presented only severe hyperhomocysteinemia, only partially corrected by initial B12 supplementation. Careful monitoring of N2O use, especially in patients with SCD, is mandatory to prevent complications.

Biosecurity assessment of Argentinian pig farms.

The pig industry is growing very fast in Argentina with an increasing need for replacement animals, feedstuff and transportation of animals. One of the main competitive advantages of the Argentinian pig industry is its being free of most major pig diseases. Within this context, applying measures aimed to reduce the risk of introduction and spread of pathogens is critical. The aim of the present study was to assess the biosecurity of Argentinian pig farms. Two types of farms were assessed: firstly, all official suppliers of high-genetic-value (n = 110) and secondly, a sample from commercial farms (n = 192). Data on the external and internal biosecurity practices applied on the farms was collected with a questionnaire. Data was analysed using a correspondence analysis and a hierarchical clustering analysis, which allowed identification of types of farms with regard to the biosecurity measures applied. Key variables characterizing the clusters were identified through an indicator value analysis. In addition, the external biosecurity of the farms was evaluated by using risk assessment tools with respect to the potential introduction of porcine epidemic diarrhoea virus. Results made evident three clusters: the first one which, amongst other measures, applied several barriers to prevent the entry of people, trucks and other vehicles, and could be considered as a group of high biosecurity, and the two other groups which applied a lower number of external and internal biosecurity measures. The results of the risk assessment showed that the routes with the highest risk of disease introduction were: replacement animals, vehicles transporting feed or animals, and visitors. The assessment of the external biosecurity showed that most Argentinian farms were not prepared for the contingency of a pathogen such as porcine epidemic diarrhoea virus. Special efforts should be made in official suppliers of high-genetic-value farms with poor biosecurity scores since they are at the top of the pig production chain and can be key for the spread of diseases.

[Congenital syphilis: a case report]. / Syphilis congénitale: à propos d'un cas.

Syphilis is a re-emerging infectious disease in Western Europe. Congenital syphilis is a potentially serious pathology affecting newborns of infected mothers. This disease is easily curable by a simple antibiotic treatment. Because of systematic antenatal screening it should no longer exist in industrialized countries. Nevertheless, we report a case of a six-week-old infant with a delayed diagnosis of congenital syphilis. Physicians, especially gynaecologists, obstetricians and paediatricians, have to be vigilant in order to allow for early diagnosis and appropriate treatment of congenital syphilis.

Influence of acute normovolaemic haemodilution on the dose-response relationship, time-course of action and pharmacokinetics of rocuronium bromide.

BACKGROUND: Acute normovolaemic haemodilution (ANH) is an effective strategy for avoiding or reducing allogeneic blood transfusion. We aimed to study its effect on the pharmacological profile of rocuronium. METHODS: In two study centres, 28 patients undergoing major surgery with ANH were matched with 28 control patients. In the dose-response groups, using the mechanomyograph, neuromuscular block of six consecutive incremental doses of rocuronium 50 microg kg(-1), followed by 300 microg kg(-1), was evaluated. In the pharmacokinetics groups, serial arterial blood samples were withdrawn for rocuronium assay after a single dose of rocuronium 600 microg kg(-1). RESULTS: ANH resulted in a shift to the left of rocuronium dose-response curve. Rocuronium effective dose(95) (ED(95)) was 26% lower (P<0.05) in the ANH group [283.4 (92.0) microg kg(-1)] compared with the control group [383.5 (127.3) microg kg(-1)]. Times from administration of last incremental dose until 25% of first response of train-of-four (TOF) recovery (Dur(25)) and 0.8 TOF ratio recovery (Dur(0.8)) were 28% longer in the ANH group [39.9 (8.4), 66.7 (14.2) min] compared with the control group [31.1 (6.6), 52.1 (15.8) min] (P<0.01, P<0.05), respectively. Volume of distribution was higher (P<0.01), central clearance was lower (P<0.05) and terminal elimination half-life was longer (P<0.0001) in the ANH group [234.97 (47.11) ml kg(-1), 4.70 (0.94) ml kg(-1) min(-1), 77.29 (12.25) min] compared with the control group [181.22 (35.73) ml kg(-1), 5.71 (1.29) ml kg(-1) min(-1), 56.86 (10.05) min, respectively]. CONCLUSION: ANH resulted in prolongation of rocuronium time-course of action, thus careful monitoring of neuromuscular block is recommended in patients who undergo ANH.

[Lysinuric dibasic protein intolerance: characteristic aspects of bone marrow involvement]. / Intolérance aux protéines dibasiques avec lysinurie: aspect caractéristique de l'atteinte médullaire.

BACKGROUND: The marrows of patients with lysinuric protein intolerance (LPI) are generally considered as normal, even though autoerythrophagocytosis has been observed in some of them. CASE REPORTS: Lysinuric protein intolerance was recognized in two 12 and 15-year-old brothers who had been diagnosed following an immuno-hematological investigation. Clinical history had been characterized by a neonatal macrophage activation syndrome (hepatosplenomegaly, pancytopenia, hypofibrinogenemia and hypertriglyceridemia). A putative diagnosis of familial lymphohistiocytosis had been ruled out because of unusual clinical and immunological course. Both brothers had displayed chronic aversion to high-protein foods, failure to thrive, osteoporosis and developmental delay. Metabolic investigations had revealed chronic hyperammonemia while cationic aminoaciduria (lysine, arginine and ornithine) was only present during L-citrulline supplementation. Bone marrow examinations had been performed during the neonatal period and during later metabolic investigations. They both displayed a peculiar red cell and granulocytes phagocytosis by histiocytes and granulocytes precursors. CONCLUSIONS: This aspect of bone marrow could be considered as a specific sign of LPI. This report suggests that appropriate metabolic investigations should be performed in any unexplained macrophage activation syndrome.

[Lethargic form of acute intestinal intussusception in an infant]. / Forme léthargique d'invagination intestinale aiguë du nourrisson.

BACKGROUND: Intussusception encephalopathy, a misleading form in which neurological symptoms are obvious, is classic but rarely described. CASE REPORT: A 21 month-old infant was admitted because he had suffered from fluctuating consciousness and apathy for a few hours. Vomiting occurred soon afterwards. Search for dehydration, meningitis, encephalitis, poisoning... was negative; the abdomen was tender leading to ultrasonography that showed a sausage-shaped tumor. The ileocolic intussusception was successfully reduced with a gas enema. CONCLUSION: A striking degree of lethargy associated with vomiting may overshadow to a considerable extent the classical intestinal manifestations. A possible endogenous opioid poisoning by massive secretion of endorphins during pain's paroxysm is one of the hypotheses explaining this type of presentation.

[Microangiopathic anemia following thrombopenic purpura]. / Anémie microangiopathique succédant à un purpura thrombopénique idiopathique.

BACKGROUND: Chronic relapsing microangiopathic hemolytic anemia is rare in children. This report describes a case associated with thrombocytopenia following idiopathic thrombocytopenic purpura. CASE REPORT: A 4 year-old girl was admitted for acute idiopathic thrombocytopenic purpura (platelet count: 12,000/mm3) without anemia or fragmented red cells. The patient was given intravenous gammaglobulins without success, followed by prednisone (2 mg/kg/day). The platelet count was normalized, but decreased when the treatment was discontinued. The patient developed acute intracranial hypertension at the age of 5 yr 8 mo, following two cerebral hematomas. The platelet count was 9,000/mm3. A second course of intravenous gammaglobulins and prednisone was unsuccessful, so a splenectomy was performed. One year later, the patient was admitted because of diffuse purpura, anemia and jaundice. Hematologic findings were: Hb 8.4 g/dl, reticulocytes 448,200/mm3, fragmented red cells 16%, platelets 15,000/mm3, WBC 22,400/mm3. Seroimmunologic investigation showed a high titer of antinuclear antibodies. Examination for viral etiology was negative. Intravenous gammaglobulins had a transient effect on platelets, reticulocytes and fragmented red cells. The patient was then given vincristine plus prednisone; they were only effective when high doses were used. A second intracerebral hemorrhage occurred when the patient was given low doses of drugs. After 3 other hematologic relapses, the vincristine was stopped without further complication. CONCLUSION: The criteria for systemic lupus erythematous were not satisfied, despite the presence of antinuclear antibodies. A congenital deficiency of an unidentified plasma factor that reverses microangiopathic hemolysis and thrombocytopenia was not demonstrated in this patient, who could not be given fresh frozen plasma.

Lead encephalopathy mimicking a cerebellar tumor.

Encephalopathy is a rare but severe complication of lead poisoning, mainly due to cerebral edema. Usually diffuse, symptoms and signs are sometimes focal, suggesting a false diagnosis of tumor, particularly cerebellar tumor. We report such a case diagnosed by MR imaging in which early treatment for edema avoided neurosurgical exploration. Only six cases have been published since Biemond and Van Creveld's first report in 1939. Several data--clinical and experimental--providing specific involvement of cerebellum in lead poisoning are emphasized.

Comparing measures of poverty and relative deprivation: an example for Belgium.

"This paper presents three different poverty standards....These alternative measures are...compared on a sample composed of 6,380 Belgian households. Such an analysis can be expected to provide some further insight into the problem of measuring poverty...."

Inheritance and wealth composition.

"This paper studies the effects of variables pertaining to alternative bequest motives on the composition of households' portfolio. It relies on a 1986 survey on the assets structure of 5,600 French households. The main conclusion is that bequest motives indeed influence the composition of households' wealth.... In general, however, for lack of data but also because households have mixed bequest motives, one cannot establish a clear relation between specific models of inheritance on the one hand and wealth pattern on the other hand."

[The association of pulmonary hemosiderosis and celiac disease. Apropos of a new case in a child]. / Association hémosidérose pulmonaire et maladie coeliaque. A propos d'une nouvelle observation chez l'enfant.

A new case of pulmonary hemosiderosis with coeliac disease is reported. This is an extremely rare combination of which only nine instances have been published over the last 20 years. Three of the reported cases occurred in children. Apart from a marked predominance of males, the combination has no specific features. Firm evidence of a causal relationship between the two diseases is lacking but treatment with a gluten-free diet alone apparently had beneficial effects on the lung disease in two patients. Three pathogenic hypotheses are discussed herein: deposition of circulating immune complexes involving food allergens on the basement membrane of alveolar capillaries; reaction between antireticulin antibodies and an alveolar basement membrane antigen; or effect of adenovirus 12, a potential causative factor for celiac disease. Patients with idiopathic pulmonary hemosiderosis should routinely have tests for gluten intolerance, for instance a lactulose-mannitol intestinal permeability test. Lastly, other concomitant food allergies are reported.

Changes in serum K+ in healthy and in asthmatic subjects during exercise.

Adrenergic mechanisms modulate exercise-induced changes in blood serum K+ concentration ([K+]). Impairment of these same mechanisms may be associated with bronchial hyper-reactivity. If this is accurate, asthmatic subjects should show disturbed K+ regulation during exercise. We measured [K+] and FEV1 in 13 healthy control and in 13 asthmatic subjects pre-exercise, at peak exercise (within 1 min of stopping exercise), and 10 min postexercise. This was done on 2 separate days, one with and one without bronchodilator (BD) pretreatment. Both groups were equally fit, exercising to the same O2 consumption and heart rate. Resting [K+] was normal for both groups (two-day averages were 4.00 +/- 0.07 and 4.09 +/- 0.07 mmol/L, mean +/- SEM, in control and asthmatic subjects, respectively). Without BD pretreatment, at peak exercise, [K+] in control subjects rose by 0.56 +/- 0.08 compared with 0.96 +/- 0.09 in asthmatics (p less than 0.01). After exercise, [K+] returned to baseline (4.12 +/- 0.08) in control subjects but remained elevated in asthmatics (4.60 +/- 0.12, p less than 0.01). Although FEV1 was unchanged in control subjects, in asthmatics it fell after exercise (p less than 0.01). With BD pretreatment: peak exercise [K+] increased by 0.55 +/- 0.09 in control subjects, and by 0.49 +/- 0.01 in asthmatics (p less than 0.01). By 10 min postexercise, it returned to baseline in both groups (4.15 +/- 0.11 for control subjects and 4.32 +/- 0.07 for asthmatics). The asthma group's fall in FEV1 was also abolished. These data indicate that postexercise K+ remains elevated in asthmatics, supporting the suggestion that their adrenergic function is impaired.