Massive recurrent post-tonsillectomy bleedings revealing a transient factor XIII deficiency in a 10-year-old boy. A case report.

A previously healthy 10-year-old boy was hospitalized for a left cervical abscess associated with massive tonsillar hypertrophy. He underwent abscess drainage and bilateral tonsillectomy. At H36 post-surgery, he presented with tonsillar hemorrhage requiring surgical revision. Hemorrhage relapsed 2 days later, with a total of 7 episodes, 5 of which required surgical revisions. Laboratory investigations were normal except for a markedly low factor XIII (FXIII) activity at 7%. After administration of a single dose of 40 IU/kg plasma-derived FXIII (Fibrogammin®) I.V., the bleeding stopped with no further recurrence. FXIII activity gradually normalized (75%) at 6 weeks, confirming the transient character of factor XIII deficiency. Severe congenital FXIII deficiency (FXIIID) (<1%) is very rare (1:2,000,000 births), whereas partial congenital deficiency and/or acquired deficiency may be more frequent but likely underreported. Acquired FXIIID may result from impaired synthesis (liver failure) or increased consumption (surgery, sepsis, leukemia, Henoch-Schönlein, inflammatory bowel disease, stroke, disseminated intravascular coagulation). FXIII replacement in form of fresh frozen plasma (FFP) or plasma-derived FXIII may be necessary for the presence of bleeding.

[Management of pediatric septic shock]. / Prise en charge du choc septique chez l'enfant.

Septic shock is a frequent admission cause in intensive care unit. In spite of the important progresses in the understanding of his physiopathology, mortality due to septic shock is about 20%. Recently, it has been demonstrated that an early goal-directed therapy permitted to improve the patient prognosis. With a good hemodynamic management and early antibiotherapy, mortality could be reduced.

Effects of chronic alcoholism on the amygdaloid complex. A study in human and rats.

The effect of chronic alcoholism on the amygdaloid complex was studied in 16 humans and 10 rats. Eighteen patients whose death was due to extraneural causes were selected as controls with 3 rats. The alcoholic cases, in addition to the data collected in their clinical history, showed, microscopically confirmed, liver cirrhosis or steatosis. The alcoholics and controls were divided into 4 groups: 35-44 years old (4 cases), 45-54 (5 cases), 55-64 (5 cases) over 65 (2 alcoholics and 4 controls). The alcoholic ingestion in the rats (Wistar, 10 weeks old) was 3 ml at a concentration of 30% in water solution administered by esophagic intubation, for 48 (5 rats) or 58 weeks (5 rats). To judge the state of the amygdaloid nuclei, a neuronal count and caryometry were carried out. The numerical data obtained in this study were analyzed statistically. The results in humans have paralleled those obtained in rats and the behaviour of the different nuclei of the amygdala was uniform and can be summarized as follows: 1) ethanol provoked a prominent and early loss of neurons, and 2) the remainder of non-affected neurons did not react in order to compensate for this neuronal loss.

Tautomeric equilibrium of pyridoxine in water. Thermodynamic characterization by 13C and 15N nuclear magnetic resonance

A remarkable temperature dependence on the 13C NMR and 15N NMR chemical shifts of pyridoxine in water (pH = 7.0) has been observed. C-3, C-6, and N-1 were the most sensitive nuclei to the temperature effect. This dependence has been explained on the basis of an equilibrium shift thermally induced between the neutral and the dipolar form of this molecule. The thermodynamic characterization of tautomeric equilibria that interconvert quickly on the NMR time scale can be carried out from the observed average 13C NMR and 15N NMR chemical shifts at different temperatures (5-90 degrees C). We have developed a new method for the estimation of the thermodynamic parameters of a given equilibrium by fitting the experimental data to a theoretical curve. This new method allows us to improve the fitting results on our previously proposed methodology. We show that there are linear correlations between the average chemical shifts obtained from different nuclei at the same temperature. This indicates that the parameters of the pure forms are related among them. We have carried out a simultaneous multiple function curve fitting of all data obtained from the most sensitive signals together using these linear correlations as restricted conditions in order to diminish the number of independent parameters to fit. To test the new methodology, we have studied the thermodynamics of the tautomeric equilibrium of pyridoxine in water. We have obtained delta H degree values ranging from -23.6 +/- 1.3 to -25.8 +/- 1.7 kJ/mol for this equilibrium depending on the used data set. This kind of methodology has, among others, the following advantages: It allows the use of a great number of experimental points from different signals in the fitting process, it yields very precise and accurate values of the tautomeric process, and it allows the resolution of the problem with only 13C NMR data in some cases saving NMR time.

[Hydatidosis in Catalonia. Study of patients younger than 20 years of age (1977-1985)]. / Hidatidosis en Catalunya. Estudio en pacientes menores de 20 años 1977-1985).

To evaluate the epidemiological features of human hydatidosis during the first two decades of life, and to assess whether it exists autochthonously in Catalonia, we analysed the patients younger than 20 years who had been diagnosed and/or treated between 1977 and 1985 in 46 hospitals (representing 95% of the hospital beds in Catalonia where surgical therapy for hydatid disease is available). Ninety-five cases were detected in 87 patients. The mean yearly incidence rate was 0.53/100,000. In 81 patients hydatidosis was diagnosed for the first time in their lives, in 11 for the second time, and in three for the third time. Among the first there was a predominance of exclusively pulmonary cysts (41.9%) over those with exclusively hepatic localization (32%) and with simultaneous hepatic and pulmonary localization (13%). Surgical operations were undertaken in 112 instances, with a mortality rate of 2.2%. It was estimated, by the actuarial method, that the likelihood of a second diagnosis of hydatid disease after the surgical removal of all the cysts found at the time of the first diagnosis was 4.2% two years after the last operation, 9.5% after four years, and 24.6% after 7 years. The incidence rate in the immigrant population was 3.03 times higher than the recorded one for Catalonia. Sixteen patients born in Catalonia reported not to have ever traveled out of it; this information was confirmed by the separate parents report. This would confirm the occurrence of autochthonous hydatid disease.

[Treatment in 2003 of septic shock in children in the first two hours (excluding newborns)]. / Approche thérapeutique en 2003 du choc septique de l'enfant dans les deux premières heures (à l'exclusion du nouveau-né).

Despite new understandings in pathophysiology, sepsis mortality remains high in children. Recently, it has been demonstrated that early goal directed therapy may decrease septic shock mortality. The aim of this paper is to propose practical clinical guidelines based on earlier consensus recommendations. Septic shock must be rapidly suspected and early recognized. Bases of treatment are maintenance of adequate oxygenation with use of artificial ventilation if necessary, larger and faster volume resuscitation than recommended before, empiric antibiotherapy and early use of vasopressive agents associated with corticosteroids in particular situations. Treatment efficacy must be regularly assessed during first hours of resuscitation. Taking into account pediatric particularities and results of adult studies, pediatricians who take care of children at beginning of septic shock may reasonably hope to decrease mortality if they keep in mind specific therapeutic goals.

[Bilateral jugular thrombosis in Lemierre syndrome]. / Un cas de syndrome de Lemierre avec thrombose veineuse jugulaire bilatérale.

An adolescent presented with a rhinosinusitis complicated with bilateral jugular veins and left superior ophthalmic vein thrombosis and respiratory distress with pulmonary hypertension. Blood culture was positive for Haemophilus influenzae and sinus puncture for Streptococcus constellatus. Evolution was under control after 1 week of appropriate antibiotherapy, antithrombotic and anti-inflammatory treatment. He had no neurologic, respiratory or ophthalmologic sequelae 6 months later. Despite lack of pharyngitis or isolation of anaerobic species on blood cultures, the picture was considered compatible with Lemierre syndrome. The risk for such a complication should be considered in cases of severe otorhinolaryngologic infection in young adults.

[Apparent life-threatening event in infants: think about star anise intoxication!]. / Malaise du nourrisson pensez à une intoxication à l'anis étoilé

UNLABELLED: In previous years, several publications have reported cases of infants presenting neurological and gastrointestinal symptoms after ingestion of star anise tea. Such teas are sometimes given in various cultures for the treatment of infant colic pains. In most cases, the cause of intoxication was contamination of Chinese star anise (Illicium verum) by Japanese star anise (Illicium anisatum). Indeed, the toxicity of Illicium anisatum, also known as Shikimi, is caused by its content in potent neurotoxins (anisatin, neoanisatin, and pseudoanisatin), due to their activity as non-competitive antagonists of GABA receptors. The main reasons explaining the frequent contaminations are the strong macroscopic resemblance of the 2 substances, as well as the fact that the fruits are often sold partially broken or in ground form. Therefore, in most cases, chemical analysis is required to determine the possible adulterations. CASE REPORT: A 2-month-old infant, in good general health, was brought to the emergency unit after 3 consecutive episodes of central cyanosis and tetany of the limbs with spontaneous recovery the same afternoon. The child was also very irritable, regurgitated a lot, and positioned himself in opisthotonos. Between these episodes, the neurological exam showed some perturbations (horizontal nystagmus and Bell's phenomenon, hypertony of the extensor muscles, and mild hypotony of the axial flexor muscles) with slow improvement over the following hours. The remaining clinical exam, the laboratory work (complete blood count, renal, hepatic, and muscular tests, capillary blood gas, plasmatic amino acids, and urinary organic acids), and the electroencephalogram findings were all normal. In the course of a detailed interview, the parents reported having given 3 bottles to their child, each one containing 200 mL of an infusion with 4 to 5 fruits of star anise, in the hours preceding the symptoms to relieve colic pains. The last seizure-like event took place approximately 8h after the last ingestion. We could prove the ingestion of anisatin, the toxic substance found in Japanese star anise, and the contamination of Chinese star anise by the Japanese species. Indeed, the anisatin analysis by liquid chromatography and mass spectroscopy (LC-MS) in a urine sample taken 22 h after the last infusion ingestion showed trace amounts of the substance. In another urine sample taken 33 h after ingestion, no anisatin could be detected. Furthermore, the analysis of the fruit sample gave an anisatin concentration of 7800 µg/kg while the maximum tolerance value in Switzerland is 1000 µg/kg. CONCLUSION: The evaluation of ALTE in infants should always include the possibility of intoxication. Star anise is generally considered a harmless medicine. Nevertheless, it can sometimes cause a severe intoxication resulting in various neurological and gastrointestinal symptoms. To prevent such events, not only the parents, but also the care personnel and pharmacists must be informed about the possible adverse effects caused either by the overdose of Chinese star anise or by the eventual contamination of herbal teas with Japanese star anise. A better control of the substances by the health authorities is also necessary.

Adrenovascular alterations from neurogenic stress modified by denervation of the adrenal gland in rat.

The application of a painful stimulus to restrained rats provokes conspicuous adrenovascular alterations (vasodilatation, edema, small hemorrhages). Hypophysectomy does not prevent the adrenovascular response to stress, or the abdominal vagotomy. On the contrary, splanchnicotomy practically abolishes such response. These results show that the vascular alterations, consequence of the action of a neurogenic stress, are not caused by a hypophysohormonal response but by a sympathetic one.

Effect of cyanamide on transaminases and other serum proteins in the rat.

The changes of the serum biochemical features induced by cyanamide, a drug used in the pharmacological treatment of alcoholism, were studied in Wistar rats. Sixty five Wistar rats were divided into 6 groups, 4 experimental and 2 control. Two experimental groups received cyanamide intraperitoneally, at a dose of 1 and 16 mg per kg of body weight, for 8 weeks. One experimental group received CCl4 and the other one CCl4 and cyanamide for 13 weeks. In addition to a delay in the increase of body weight as compared with the control group, the rats receiving cyanamide underwent a marked dispersion of the transaminase and LDH values. This could be explained by the double effect of cyanamide: its capacity to depress the cellular activity (lowered synthesis of proteins, transaminase and LDH among them) and cellular damage. Therefore, some animals showed a marked depression of protein synthesis, causing low enzyme values while cytolysis prevailed in the rest as seen in higher enzyme values. When cyanamide and CCl4 were employed simultaneously, the cyanamide tended to diminish the higher enzyme values caused by CCl4 when used alone. These findings can explain why patients on cyanamide may develop severe liver damage without serious alterations in tests for liver function.

Thermodynamic constants for tautomerism, hydration, and ionization of vitamin B6 compounds in water/dioxane.

The macroscopic deprotonation constants of phenol, pyridine, p-nitrophenol, salicylaldehyde, 4-pyridinaldehyde, pyridoxine, 3-hydroxypyridine, 5-deoxypyridoxal, pyridoxal, and pyridoxal 5'-phosphate have been determined at 25 degrees C in water/dioxane mixtures. Many of the hydration and tautomeric constants and microscopic pK values of these compounds have also been measured under the same conditions. These values are discussed with reference to Hammett's and Marshall's equations and a general equation that predicts these equilibrium constants in the media under discussion has been formulated. The significance of these findings on the chemistry of vitamin B6 and its importance in the study of the catalytic pathways of vitamin B6-dependent enzymes are also discussed.

Criteria to recognize the structure and micropolarity of pyridoxal 5'-phosphate-binding sites in proteins.

We show that polarography can be a useful tool for the recognition of the mode of binding of pyridoxal 5'-phosphate to proteins. Furthermore we discuss the possibilities of other techniques that can be used to study this subject.

Disseminated histoplasmosis in an adopted infant from El Salvador.

UNLABELLED: An HIV-negative 4-month-old infant recently adopted from El Salvador was admitted for high fever. Hepatosplenomegaly, anaemia, leucopenia, thrombocytopenia, increased transaminases, and diffuse interstitial pulmonary infiltration were present on admission. Granulomas were seen in bone marrow and liver biopsies without any organism. Disseminated histoplasmosis was diagnosed 2 weeks later when bone marrow and blood cultures taken on admission became positive for Histoplasma capsulatum and when histoplasmic antigen was detected in blood. The outcome was good after treatment with amphotericin B followed by itraconazole which was administered for a 6-month period without significant toxicity. CONCLUSION: Disseminated histoplasmosis is very rarely seen in Europe but should be suspected in case of unexplained fever in immigrants from the endemic areas in the world, particularly when hepatosplenomegaly and pancytopenia are present. Bone marrow examination and culture, blood cultures, and antigen testing are the mainstays of the diagnosis.

Studies on the pyridoxal phosphate site in glycogen phosphorylase b.

It is usually accepted that the adduct formed by reaction of pyridoxal phosphate with amines in aprotic solvents is a good model to stimulate some properties of the pyridoxal phosphate site in glycogen phosphorylase. The chemical structure of this adduct was not very well established. An aldimine structure is supported by the infrared, electronic absorption and nuclear magnetic resonance spectra given in this work. Therefore, we conclude that, at neutral pH, the pyridoxal phosphate is bound to the glycogen phosphorylase through a Schiff base structure and embedded in a hydrophobic environment. The polarographic measurements reported in this paper could explain the fact that, at neutral pH, the pyridoxal phosphate can not be reduced onto phosphorylase by NaBH4.

[Diabetes and ignored disorders of glucose tolerance in primary care. Evidence from opportunistic detection. Group for the Study of Diabetes in Tarragona]. / Diabetes y trastornos ignorados de la tolerancia a la glucosa en atención primaria. Evidencia mediante detección oportunista. Grup per a l'Estudi de la Diabetis a Tarragona (GEDT).

OBJECTIVE: To investigate the level of attendance for care of people with glucose tolerance disorders, both known and not, and to discuss their real impact on Primary Care. DESIGN: Simple prospective randomised sampling. Opportunist detection among spontaneous users over 40, excluding pregnant women. SETTING: Primary Health Care. PATIENTS AND OTHER PARTICIPANTS: Data was recorded using a pre-designed survey, which included the recognised Diabetes (DM) risk factors. MEASUREMENTS AND MAIN RESULTS: Of the 183 cases evaluated, DM was already known in 13 (7.1%). 15 (8.2%) with unknown DM and 27 (14.7%) with GIT. A strong link was demonstrated with the average age and a marked tendency to overweight and obesity only significant when comparing DM with the rest. As to the risk factors researched, there was a significant association with antecedents of prior tolerance abnormality, DM in pregnancy, hyperglucaemiant medicines and ischaemic disease, in cases where family antecedents of definite diabetes were not found. CONCLUSIONS: The frequency of attendance of users with diabetes and unknown glucose tolerance disorders is very high in spontaneous primary care consultations. These results suggest that opportunist detection should be encouraged in primary health care centres.

[Arterial hypertension in the Baix Ebre region (Tarragona)]. / La hipertensión arterial en la comarca del Baix Ebre (Tarragona).

The results of a cross-sectional study for the evaluation of the prevalence of hypertension in the Baix Ebre region (Tarragona) are reported. 628 individuals from a randomly selected sample of 670 gave their consent to participate. The study was based on interview and examination at the patients home after getting an appointment by post. The prevalence of hypertension was 31.84 +/- 3.64%, and that of borderline hypertension 16.56 +/- 2.97%. Only 57% of hypertensives were previously known as such, and only 18.5% of these were being correctly treated. There was a significant association of hypertension with age (basically systolic blood pressure for women); also with alcohol intake, obesity and family history of hypertension or cardiovascular disease. There was no significant correlation with sex, residence in rural or urban areas, emigration, marital status, occupational status, social and professional level, education, or with the coexistence of hypertension in the spouse. The high prevalence of hypertension was a remarkable finding, consistent with its recognized importance as a first rate health problem.

[A study of the prevalence of alcohol consumption in the Baix Ebre region (Tarragona)]. / Estudio de la prevalencia del consumo de alcohol en la comarca del Baix Ebre (Tarragona).

A cross-sectional study was carried out in the Baix Ebre region about the prevalence of alcohol use, within a wider study of hypertension and other risk factors. The following characteristics were considered for the elaboration of the present study: non drinker, moderate drinker and heavy drinker, as well as the amount of alcohol intake related with demographic, socioeconomical and cultural variables such as: age, sex, urban or rural medium, marital status, place of origin, working status, social and professional level, and educational status. 64.2% of the whole population greater than or equal to 20 years were usual alcohol users, and so were 79.4% of males and 48.2% of females. According to the definition of heavy or excessive drinker, such were 21.8% of males and 2.6% of females. Males drank a higher alcohol amount, with a mean and standard deviation of 47.1 +/- 35.5. In females, these figures were 19.4 +/- 12.4 g per day. The rate of alcohol drinkers was higher in young individuals, in those with active working status, in those with medium social and professional level, and in those with medium and high educational status.

[Acute respiratory distress syndrome in children]. / Le syndrome de détresse respiratoire aiguë (SDRA) chez l'enfant.

The acute respiratory distress syndrome (ARDS) encountered in a child may be either due to a primary lung infection or may be secondary to a systemic inflammatory response of varying origin. Therapy is based on: 1) the mechanical ventilation strategy aimed at maintaining the functional residual capacity by alveolar recruitment using positive end expiratory pressure and to limit secondary pulmonary lesions by using small tidal volumes, 2) prone positioning as soon as sufficient stability is achieved; 3) optimizing tissue oxygen delivery by cardiac support; 4) correction of any other organ dysfunction. If this conventional approach is not sufficient experimental therapies may be tempted given the vital risk. For instance inhaled nitric oxide and high frequency oscillation ventilation may be a valuable support. Newer techniques, such as partial liquid ventilation, are being developed and could become useful therapeutic options. After the acute phase a close medical follow-up is mandatory. Because of the possibility of a chronic respiratory insufficiency with negative consequences on the right ventricular function, these patients may need long term oxygen therapy and diuretics. Cardiac echography helps orientation in maintaining or discontinuing this long term therapy by estimating the arterial pulmonary pressure.